Summer is a very "mixed bag", I think, for parents of special-needs kids.
In one sense, it is definitely a break, a much-needed break, from the school environment and all that that environment means, for parents of children with special needs. Since she is at home, I know obviously where she is and I am assured that she is not around any unnecessary food and/or people/kids who will not treat her appropriately. I still worry about whether she is losing some of what she had been learning, in math skills especially, but there hasn't been too much regression in skills over summers so far, so I'm not too concerned.
However, I know Sophie misses the constant activity and the routine of the school day, and she also misses being around other kids. So for her, summer isn't always a great thing.
Summer is a time of year that seems to make it especially noticeable how challenging it is for children-with-special needs to have true "friends". By "friends" I guess I am thinking back to my own childhood, and how I and my neighborhood friends used to spend our days - roaming the neighborhood, going in and out of each others' houses, going to the pool, playing hide-and-seek. While I do understand that times have changed, and things are not the same as they were 30 years ago, still I clearly see that for Sophie, this ease of having friends is not immediately possible.
For a "typical" child to have a friendship with Sophie - one which looks anything like that between two "typical" children - they would be required to meet her in the middle, somewhere between their cognitive level, and hers. Sophie is 9, but has fun doing things that I suspect are more enjoyable for kids a year-or-two-younger. So while I would dearly love for my wonderful, funny, sweet daughter to have true friends, I would be surprised at this point to come upon any "typical" child who knows how - or has been taught how - to adjust their play skills so that both they and Sophie have fun. While I do notice and it breaks my heart, I sincerely hope that Sophie does not notice the neighborhood kids playing, choosing to play without her. If she does notice, I hope and pray that she doesn't understand it enough that it hurts her. I can't force kids to play with her, and I am too wary of kids being mean to her (which, again, I notice even if she doesn't) to want to push the issue.
School districts these days, for the most part, are instructed to practice the concept of "inclusion", where children with a diagnosis are still able to spend part of their day in the classroom with their same-age "typical" peers. And for the most part, this is a good thing. Unfortunately, though, there is still a piece missing in the inclusion effort, and that is, teaching "typical" children how to meet their special-needs peers halfway, in terms of spending enjoyable time together. Sophie does not often have kids to play with, at recess time at school. I have images of her standing by herself while outside. Micro-managing her entire school day, and/or forcing other kids to play with her by demanding the school do something, seems to be the solutions that jump to mind. Not the greatest solutions, and she does seem to have days where she plays with kids, so....
Here is a "for-instance", of how one child can learn how to interact with a special-needs child: Sophie spends part of every school day in the Intervention Room, which is where special-needs kids spend time learning the subjects which they need taught at a pace different from their mainstream peers. In Sophie's room there is a student who in non-verbal and is in a wheelchair. "Typical" children would take one look at her and assume that there is no way for them to interact with her in a positive, "play" manner - but as I learned, they would be wrong. Sophie is high-functioning enough to be able to help out, in the Intervention Room, in small ways. One of the ways she helps with this particular student is by pushing her around the room, in her wheelchair, when the student becomes upset. It seems that this student prefers movement, sound, action around her - it is enjoyable for her and without it, she often starts to cry. Is this something that "typical" students could help with? Yes, in a supervised way of course. It is a way that they could interact, positively, with a special-needs peer.
Ah summer....supposed to be such a carefree time filled with sunshine and ice-cream cones....doesn't always work that way.
Later,
Jen
Showing posts with label Prader-Willi Syndrome. Show all posts
Showing posts with label Prader-Willi Syndrome. Show all posts
Sunday, July 14, 2013
Friday, October 26, 2012
The "food life" of the PWS* parent
[*PWS = Prader-Willi Syndrome]
The topic sometimes comes up, among PWS parents, of "how have your/your family's eating habits changed since your child was diagnosed with PWS?". Because Prader-Willi Syndrome has almost everything to do with the scheduled consumption of food by one's child, and the calorie-count of that food, it is often a standard consequence that the eating/food preparation habits of everyone in the household are affected, both for better and for worse. Some parents are able to change their life 100% with the entrance of the PWS diagnosis, and they devote all of their time to becoming nutrition and supplement experts. Most parents, though, are doing the best they can, keeping their child-with-PWS as healthy and slim as they can while muddling through as far as feeding the rest of the family. "Food" and its preparation becomes a source of questioning and recalculating of eating habits that have been in practice for many years before the entrance of the PWS diagnosis.
Take me, for example.
Like a good number of PWS parents, I sneak snacks. I eat stuff I shouldn't, when I know Sophie won't see me. Eating in the late hours of the day when she is definitely in bed is now a bad habit of mine. I sometimes eat when I'm not at all hungry, because if I don't, I know that I'll become hungry in a moment when Sophie should not be eating, and to eat in front of her at that time would be cruel.
Then there is the "recipe collection". I have probably 100 back issues of food-based magazines; "Cooking Light", "Everyday Food", to name a few. There are several stacks of cookbooks on various shelves around the house. I have a binder bursting with several hundred recipes clipped from magazines and printed off from the internet.
But...do I actually "cook", anymore? As in, follow a recipe, make something "from scratch", spend any prolonged time in the kitchen?
Not really, for various PWS-related reasons.
Any extra time spent in the kitchen, when Sophie is home, in problematic. She likes to hang out in the kitchen and sometimes wants to help me, even if what I'm making is really simple. So then I worry, should she even be IN the kitchen at all? If she's watching me warm this up or make that, will she at some point sneak into the kitchen and try to make it herself (because I am ALWAYS telling her that this or that food item, if eaten without cooking it first, will make her very very sick - so as to somehow deter her from eating raw food product. Except then, will she just use her clever brain, take the food anyway, and try to cook it herself??). There is also the concern that, if something is cooking for a few hours and sending tantalizing smells all through the house, does that bother Sophie and cause her added stress? Do I open all the windows to decrease the smells? Should I just not cook or bake much at all?
And then, with all these cookbooks and food magazines and clipped recipes, of course it is now mandatory to consider the calorie-count-per-serving for whatever recipe. Sophie is eight years old now, and with the passing years I have kind of lost my desire to make much of anything - there is no longer any justification for making a recipe "just because it tastes or looks good" or "just because it was a comfort food when I was a kid", if one serving equals 200, 300, 400+ calories. Certainly Sophie's portion can be adjusted so that she can at least have some, and the rest of her meal would be suitably lightened up. That isn't hard to do, and I have certainly done that on occasions when it has been necessary. It gets tiring and depressing, though, to approach every recipe with that precaution in mind. Really, the more recipes I read, I start to question why any recipe, for any main dish, side dish, dessert, whatever, needs to be made with full-fat or full-calorie ingredients. If the population of the U.S. is in the midst of an obesity epidemic, then why does any company or chef or food magazine or cookbook author produce recipes with seemingly no regard for the calorie-count-per-serving? I have looked at recipes where the count is 600 calories-per-serving, and the serving size is one cup. Now, obviously I am not going to make that particular recipe, as that is not healthy for anyone in my house let alone Sophie, plus her portion size for such a recipe would be so minute that it wouldn't even be worth it to put any on her plate. But even though I know right away that I won't be making that recipe, I just have to wonder why anyone would make that recipe. Why put your health at risk for the sake of that plateful of "whatever"? Is it worth it? Why spend the money on the ingredients to make something which, even as it tastes good, is increasing your weight or your risk of heart disease? Why....
And on, and on....all these questions from looking at just one recipe.
Looking at food magazines and cookbooks etc etc is fraught with conflict, questions, self-doubt and second-guessing, anymore. I know of families where the entire family has gone vegetarian/vegan/gluten-free/sugar-free/carb-free/all-or-some-of-the-above, as a result of the PWS diagnosis. One family switched to juiced-everything, and says that not only has this helped their child-with-PWS, the whole family feels so much healthier. It seems inevitable that with the PWS diagnosis, simply walking into one's kitchen or eating anything at all, is an action which is so much less clear-cut than for the "typical" person or family. Food consumption becomes a guilty pleasure, or a hoped-for cure-all for your child's diagnosis, or it's just a every-two-hour-chore with no joy in it at all. I watch cooking shows sometimes and drool, not just because of what is being cooked but because of the person's freedom to cook whatever, without having to agonize over the calories and ingredients of the recipe. The people on the cooking show actually enjoy what they are doing! I'd like to get back to that, somehow. I'd like to be able to cook and bake in my kitchen without feeling like I have to do it in the middle of the night or only when Sophie is at school, so as not to cause her any extra distress.
Well, off to figure out what to make for dinner.... :-).
Later,
Jen
The topic sometimes comes up, among PWS parents, of "how have your/your family's eating habits changed since your child was diagnosed with PWS?". Because Prader-Willi Syndrome has almost everything to do with the scheduled consumption of food by one's child, and the calorie-count of that food, it is often a standard consequence that the eating/food preparation habits of everyone in the household are affected, both for better and for worse. Some parents are able to change their life 100% with the entrance of the PWS diagnosis, and they devote all of their time to becoming nutrition and supplement experts. Most parents, though, are doing the best they can, keeping their child-with-PWS as healthy and slim as they can while muddling through as far as feeding the rest of the family. "Food" and its preparation becomes a source of questioning and recalculating of eating habits that have been in practice for many years before the entrance of the PWS diagnosis.
Take me, for example.
Like a good number of PWS parents, I sneak snacks. I eat stuff I shouldn't, when I know Sophie won't see me. Eating in the late hours of the day when she is definitely in bed is now a bad habit of mine. I sometimes eat when I'm not at all hungry, because if I don't, I know that I'll become hungry in a moment when Sophie should not be eating, and to eat in front of her at that time would be cruel.
Then there is the "recipe collection". I have probably 100 back issues of food-based magazines; "Cooking Light", "Everyday Food", to name a few. There are several stacks of cookbooks on various shelves around the house. I have a binder bursting with several hundred recipes clipped from magazines and printed off from the internet.
But...do I actually "cook", anymore? As in, follow a recipe, make something "from scratch", spend any prolonged time in the kitchen?
Not really, for various PWS-related reasons.
Any extra time spent in the kitchen, when Sophie is home, in problematic. She likes to hang out in the kitchen and sometimes wants to help me, even if what I'm making is really simple. So then I worry, should she even be IN the kitchen at all? If she's watching me warm this up or make that, will she at some point sneak into the kitchen and try to make it herself (because I am ALWAYS telling her that this or that food item, if eaten without cooking it first, will make her very very sick - so as to somehow deter her from eating raw food product. Except then, will she just use her clever brain, take the food anyway, and try to cook it herself??). There is also the concern that, if something is cooking for a few hours and sending tantalizing smells all through the house, does that bother Sophie and cause her added stress? Do I open all the windows to decrease the smells? Should I just not cook or bake much at all?
And then, with all these cookbooks and food magazines and clipped recipes, of course it is now mandatory to consider the calorie-count-per-serving for whatever recipe. Sophie is eight years old now, and with the passing years I have kind of lost my desire to make much of anything - there is no longer any justification for making a recipe "just because it tastes or looks good" or "just because it was a comfort food when I was a kid", if one serving equals 200, 300, 400+ calories. Certainly Sophie's portion can be adjusted so that she can at least have some, and the rest of her meal would be suitably lightened up. That isn't hard to do, and I have certainly done that on occasions when it has been necessary. It gets tiring and depressing, though, to approach every recipe with that precaution in mind. Really, the more recipes I read, I start to question why any recipe, for any main dish, side dish, dessert, whatever, needs to be made with full-fat or full-calorie ingredients. If the population of the U.S. is in the midst of an obesity epidemic, then why does any company or chef or food magazine or cookbook author produce recipes with seemingly no regard for the calorie-count-per-serving? I have looked at recipes where the count is 600 calories-per-serving, and the serving size is one cup. Now, obviously I am not going to make that particular recipe, as that is not healthy for anyone in my house let alone Sophie, plus her portion size for such a recipe would be so minute that it wouldn't even be worth it to put any on her plate. But even though I know right away that I won't be making that recipe, I just have to wonder why anyone would make that recipe. Why put your health at risk for the sake of that plateful of "whatever"? Is it worth it? Why spend the money on the ingredients to make something which, even as it tastes good, is increasing your weight or your risk of heart disease? Why....
And on, and on....all these questions from looking at just one recipe.
Looking at food magazines and cookbooks etc etc is fraught with conflict, questions, self-doubt and second-guessing, anymore. I know of families where the entire family has gone vegetarian/vegan/gluten-free/sugar-free/carb-free/all-or-some-of-the-above, as a result of the PWS diagnosis. One family switched to juiced-everything, and says that not only has this helped their child-with-PWS, the whole family feels so much healthier. It seems inevitable that with the PWS diagnosis, simply walking into one's kitchen or eating anything at all, is an action which is so much less clear-cut than for the "typical" person or family. Food consumption becomes a guilty pleasure, or a hoped-for cure-all for your child's diagnosis, or it's just a every-two-hour-chore with no joy in it at all. I watch cooking shows sometimes and drool, not just because of what is being cooked but because of the person's freedom to cook whatever, without having to agonize over the calories and ingredients of the recipe. The people on the cooking show actually enjoy what they are doing! I'd like to get back to that, somehow. I'd like to be able to cook and bake in my kitchen without feeling like I have to do it in the middle of the night or only when Sophie is at school, so as not to cause her any extra distress.
Well, off to figure out what to make for dinner.... :-).
Later,
Jen
Friday, August 5, 2011
Get S through summer camp: check
Today was S's last day of summer camp. It is somewhat of an accomplishment for both her and us to get through all six weeks successfully. She went to day camp six weeks out of the summer, for four days a week, 8 a.m. until 2 p.m. This being the last day for this summer camp, I now have the few weeks up until school starts to keep her entertained. (Yes, that makes me nervous.)
The staff of this camp are just phenomenal. This is more than your average summer day camp. This particular camp is a program run through our county's Board of Developmental Delay. There are just over 100 spots in this camp, and the application process is on a first-application-received, first-applicant-guaranteed-a-spot basis. I have heard that some parents fill out the application the day they receive it in January, and overnight it to the intake person that day, just to make sure their child gets a spot. The staff members of this camp are special people, because they do what so many people refuse to do - they work one-on-one with children with disabilities, and treat them with respect, kindness, and love. Today being the last day of camp, the campers and counsellors put together a talent show, with each group doing a routine to a M.ichael Jackson song. It was sooooo cute, the kids did so well and had fun. The people working with the kids in this camp didn't look at them and say, 'oh, they can't do that'. They picked a song, practiced with the kids, and put on a well-done show for all the parents. It was truly wonderful to see.
This camp is another reason why we are so, so blessed to live where we live. S does well at this camp, thank God. She does not enjoy the drive there/back home as it highway driving, but she enjoys herself while she is there, and I doubt I could keep her nearly as well entertained and appropriately busy here at home. So this camp ensures her sanity during the summer - and mine.
As I said, the staff of the camp is phenomenal. The lead teachers are all either special-ed teachers or intervention specialists, so they know their way around an IEP/504 plan, and the goals/parameters of those documents are followed and worked on as much as possible over the summer. The counsellors are all college students who are focusing their studies on areas matching the needs of this camp - nursing, special ed, occupational/speech/physical therapy and the like. And yet, this is still very much a summer camp, with all the accompanying craft projects and silly songs and swimming! The kids have a fantastic time in a safe environment where the staff is aware of their challenges yet helps the kids work through those challenges so they can still enjoy themselves. So my daughter, who could not possibly go to a regular summer camp, day-based or otherwise, can still say she is "going to camp" like any other kid.
So days like today are bittersweet. Like other special-needs parents, as my daughter gets older I find myself repeatedly going through the scenario of saying goodbye to people who I have trusted to take care of my daughter, keep her safe, help her learn and develop, and help her have a good time. That is HUGE for any special-needs parent. I hope we will see many of the same counsellors and staff back next summer...but I don't know, maybe we won't. Anyone who sees my daughter for the great person she is, is someone who comes to mean a lot to me - and it is hard to keep saying goodbye to these people. I wonder if they know how meaningful their presence in my daughter's life is? I know they get a paycheck...but believe me, they don't do this job just for that. They do this job because they see in these kids what is so often overlooked by the general public - they see a person.
Thanks for reading.
Later,
Jen
The staff of this camp are just phenomenal. This is more than your average summer day camp. This particular camp is a program run through our county's Board of Developmental Delay. There are just over 100 spots in this camp, and the application process is on a first-application-received, first-applicant-guaranteed-a-spot basis. I have heard that some parents fill out the application the day they receive it in January, and overnight it to the intake person that day, just to make sure their child gets a spot. The staff members of this camp are special people, because they do what so many people refuse to do - they work one-on-one with children with disabilities, and treat them with respect, kindness, and love. Today being the last day of camp, the campers and counsellors put together a talent show, with each group doing a routine to a M.ichael Jackson song. It was sooooo cute, the kids did so well and had fun. The people working with the kids in this camp didn't look at them and say, 'oh, they can't do that'. They picked a song, practiced with the kids, and put on a well-done show for all the parents. It was truly wonderful to see.
This camp is another reason why we are so, so blessed to live where we live. S does well at this camp, thank God. She does not enjoy the drive there/back home as it highway driving, but she enjoys herself while she is there, and I doubt I could keep her nearly as well entertained and appropriately busy here at home. So this camp ensures her sanity during the summer - and mine.
As I said, the staff of the camp is phenomenal. The lead teachers are all either special-ed teachers or intervention specialists, so they know their way around an IEP/504 plan, and the goals/parameters of those documents are followed and worked on as much as possible over the summer. The counsellors are all college students who are focusing their studies on areas matching the needs of this camp - nursing, special ed, occupational/speech/physical therapy and the like. And yet, this is still very much a summer camp, with all the accompanying craft projects and silly songs and swimming! The kids have a fantastic time in a safe environment where the staff is aware of their challenges yet helps the kids work through those challenges so they can still enjoy themselves. So my daughter, who could not possibly go to a regular summer camp, day-based or otherwise, can still say she is "going to camp" like any other kid.
So days like today are bittersweet. Like other special-needs parents, as my daughter gets older I find myself repeatedly going through the scenario of saying goodbye to people who I have trusted to take care of my daughter, keep her safe, help her learn and develop, and help her have a good time. That is HUGE for any special-needs parent. I hope we will see many of the same counsellors and staff back next summer...but I don't know, maybe we won't. Anyone who sees my daughter for the great person she is, is someone who comes to mean a lot to me - and it is hard to keep saying goodbye to these people. I wonder if they know how meaningful their presence in my daughter's life is? I know they get a paycheck...but believe me, they don't do this job just for that. They do this job because they see in these kids what is so often overlooked by the general public - they see a person.
Thanks for reading.
Later,
Jen
Saturday, June 11, 2011
Prader-Willi Syndrome and Inflatables
I am consistently amazed at the things that come up, about which I never would have thought twice if I did not have a child with special needs. The learning curve, as we weave PWS into our lives, has been one of mountainous proportions...or I could say, it is a curve as high and bouncy as the inflatable S tried to scale the other day.
The PTA for my daughters' school has, for the past few years, paid to have four huge inflatable 'things' brought into the cafetorium. Throughout the day, each grade level has a chance to go wild and crazy on these things. The picture below is from Google Images, but there was one of those at the school that day.
Now, the PTA at the school is great. They really do quite a bit for the kids, and provide a great deal of help to all the teachers there. So I don't have a problem with this activity being provided for the school at large, even though there are special-needs-kids in the school population who could not possibly play on these things, for various reasons. When S brought the flyer home announcing this "Fun Day", I know I groaned inwardly and may have done so audibly as well....I just wasn't sure how S would do on these things. Plus, even though there are only two grade levels going through at a time, we're still talking about 200 kids cycling through 4 inflatables over about an hour's time. The noise level is incredible...there are kids moving, running, bouncing, flinging everywhere. They all definitely have fun, but for a child with low muscle tone and poor overall muscle strength, how would this work?
So, I went up to the school Tuesday afternoon, to supervise/help her with this. She was excited! She has been on inflatables before so she is familiar with the sort-of-out-of-control environment that happens to groups of kids when these things are around. So, first she lined up for this rock-wall-climbing thing; squares on the incline to put your foot on, with ropes to pull yourself up. I told her she could try it but if she found she couldn't make it up and over, to just stop and slide down. She did a great job trying, and made it about halfway up - but the combination of trying to balance on the footholds AND pull herself up with the rope was too much. So that one will have to wait 'til she's older and stronger.
Then, she lined up to try this obstacle course thing...have you ever watched that show "Wipe.Out"? This inflatable reminds me of what contestants go through on that show, on a much smaller scale of course!
It looked pretty much like the photo above, although the one at the school had a net roof on it. The beginning is at the left side of the picture. As soon as you get kids moving through it, the whole thing is moving and bopping and you really have to keep your balance. And of course, you have to be able to move fast, or you are going to get squashed by kids starting after you but landing on top of you as you both exit at the same time. Sophie did not enjoy this one either; I give her a lot of credit for wanting to try it, but this is another one that will wait until she's a bit older and stronger!
So I told S that, for that day, she would stick the two inflatable she could go on and enjoy without hurting herself! These were two slides which looked like the top photo, one a little higher than the other. The other students had wait a few seconds longer for her to climb up, and then get situated to slide down, and then "dismount" at the bottom - but it went okay. S enjoyed herself and, oddly enough, did NOT tire herself out as much as I thought she would. I will probably have to go up to the school every time the PTA plans this activity, though - or make it mandatory that an aide stay with her the entire time, I guess. I mean, she can definitely participate on at least some of these - but if she had not been supervised I'm pretty sure she would have gotten hurt.
Another instance of life with PWS - walking that fine line between wanting to have my child participate as much as she's able, yet also wanting her to be safe and/or wanting the rest of the world to just slow down so that she can participate.
Thanks for reading-
Later,
Jen
The PTA for my daughters' school has, for the past few years, paid to have four huge inflatable 'things' brought into the cafetorium. Throughout the day, each grade level has a chance to go wild and crazy on these things. The picture below is from Google Images, but there was one of those at the school that day.
Now, the PTA at the school is great. They really do quite a bit for the kids, and provide a great deal of help to all the teachers there. So I don't have a problem with this activity being provided for the school at large, even though there are special-needs-kids in the school population who could not possibly play on these things, for various reasons. When S brought the flyer home announcing this "Fun Day", I know I groaned inwardly and may have done so audibly as well....I just wasn't sure how S would do on these things. Plus, even though there are only two grade levels going through at a time, we're still talking about 200 kids cycling through 4 inflatables over about an hour's time. The noise level is incredible...there are kids moving, running, bouncing, flinging everywhere. They all definitely have fun, but for a child with low muscle tone and poor overall muscle strength, how would this work?
So, I went up to the school Tuesday afternoon, to supervise/help her with this. She was excited! She has been on inflatables before so she is familiar with the sort-of-out-of-control environment that happens to groups of kids when these things are around. So, first she lined up for this rock-wall-climbing thing; squares on the incline to put your foot on, with ropes to pull yourself up. I told her she could try it but if she found she couldn't make it up and over, to just stop and slide down. She did a great job trying, and made it about halfway up - but the combination of trying to balance on the footholds AND pull herself up with the rope was too much. So that one will have to wait 'til she's older and stronger.
Then, she lined up to try this obstacle course thing...have you ever watched that show "Wipe.Out"? This inflatable reminds me of what contestants go through on that show, on a much smaller scale of course!
It looked pretty much like the photo above, although the one at the school had a net roof on it. The beginning is at the left side of the picture. As soon as you get kids moving through it, the whole thing is moving and bopping and you really have to keep your balance. And of course, you have to be able to move fast, or you are going to get squashed by kids starting after you but landing on top of you as you both exit at the same time. Sophie did not enjoy this one either; I give her a lot of credit for wanting to try it, but this is another one that will wait until she's a bit older and stronger!
So I told S that, for that day, she would stick the two inflatable she could go on and enjoy without hurting herself! These were two slides which looked like the top photo, one a little higher than the other. The other students had wait a few seconds longer for her to climb up, and then get situated to slide down, and then "dismount" at the bottom - but it went okay. S enjoyed herself and, oddly enough, did NOT tire herself out as much as I thought she would. I will probably have to go up to the school every time the PTA plans this activity, though - or make it mandatory that an aide stay with her the entire time, I guess. I mean, she can definitely participate on at least some of these - but if she had not been supervised I'm pretty sure she would have gotten hurt.
Another instance of life with PWS - walking that fine line between wanting to have my child participate as much as she's able, yet also wanting her to be safe and/or wanting the rest of the world to just slow down so that she can participate.
Thanks for reading-
Later,
Jen
Thursday, May 19, 2011
Still recovering
Have you ever gone through surgery, and after that you'll have friends and family say that they wish you a smooth and quick recovery? I know I have said that to people, because I do wish that for them.
I'm not recovering from any surgery, not literally. I'm recovering from more a figurative procedure, where the life I thought I would live was removed, and replaced with a life that I never, ever saw coming.
I think I am still recovering from that day back in 2004, when a voice on the end of my phone line told me that blood tests had confirmed the diagnosis of Prader-Willi Syndrome for our younger daughter. I think I will be recovering from that moment for possibly the rest of my life.
I think my entire universe shifted that day, in that moment. It only took a few seconds for that doctor's voice to say those words...but I will forever be feeling the ripple effect from that. In the space of a few short weeks, from the time S arrived until that phone call, we went from being your average, garden-variety parents to being special-needs parents. I am no longer the person I was, in the early days of that year. I don't know who that old Jen is. I'm not sure I would recognize her. I know I would probably take issue with large chunks of that old Jen's personality.
But, at the same time, I am still "growing into" my new skin. I'm still recovering from the loss of what I thought life with two children would be like. I know, it's been 7 years - that is a long time! But the first year of the PWS diagnosis is incredibly busy. We quickly got involved with taking S to multiple doctors, seeing county service representatives, on and on. We were simultaneously learning about the syndrome, trying to make sure S got enough calories and was growing, parent our older daughter, answer the many questions of friends and family...simply adjust. It was mind-numbing. Shortly after S turned a year old, I began to volunteer for the PWS organization in my state, and that has added a whole extra layer to our PWS journey - a very fulfilling but very time-consuming level. We have planned four fundraisers. I have been a mentor to other newly-diagnosed families. Believe me, I wouldn't trade any of this! I love doing all of this. But I will admit, that it has been an extremely busy seven years. There really has not been much time for "just living", just breathing, just adjusting to this life.
So it occurred to me today that just possibly, I am still recovering from that moment when the PWS reality entered our lives. Maybe that is why some days I just feel numb, or like I could just sit for hours and hours and do nothing else, not even really think about much. Because PWS throws something new at us on a very, very regular basis - which means that even as we are still wrapping our minds around PWS, we are also still having to recalibrate our family life. S isn't finished throwing curveballs at us, because she still has quite a bit of developing to do, so the surprises and challenges will keep coming - without the hope that one day, she'll be fully independent and will be able to handle things on her own. So here we are, with some parts of our future with PWS somewhat predictable...and the rest of it something we'll just have to keep recovering from.
Added on to all of that, is the process of parenting an 11-year-old who looks older every day. She'll be a teenager soon. She'll be wanting to date, sooner than I'm comfortable with. Someday before I know it she'll be driving. Oh, my. By the time AARP comes looking for me, I'll be positively catatonic.
Thanks for reading-
Later,
Jen
I'm not recovering from any surgery, not literally. I'm recovering from more a figurative procedure, where the life I thought I would live was removed, and replaced with a life that I never, ever saw coming.
I think I am still recovering from that day back in 2004, when a voice on the end of my phone line told me that blood tests had confirmed the diagnosis of Prader-Willi Syndrome for our younger daughter. I think I will be recovering from that moment for possibly the rest of my life.
I think my entire universe shifted that day, in that moment. It only took a few seconds for that doctor's voice to say those words...but I will forever be feeling the ripple effect from that. In the space of a few short weeks, from the time S arrived until that phone call, we went from being your average, garden-variety parents to being special-needs parents. I am no longer the person I was, in the early days of that year. I don't know who that old Jen is. I'm not sure I would recognize her. I know I would probably take issue with large chunks of that old Jen's personality.
But, at the same time, I am still "growing into" my new skin. I'm still recovering from the loss of what I thought life with two children would be like. I know, it's been 7 years - that is a long time! But the first year of the PWS diagnosis is incredibly busy. We quickly got involved with taking S to multiple doctors, seeing county service representatives, on and on. We were simultaneously learning about the syndrome, trying to make sure S got enough calories and was growing, parent our older daughter, answer the many questions of friends and family...simply adjust. It was mind-numbing. Shortly after S turned a year old, I began to volunteer for the PWS organization in my state, and that has added a whole extra layer to our PWS journey - a very fulfilling but very time-consuming level. We have planned four fundraisers. I have been a mentor to other newly-diagnosed families. Believe me, I wouldn't trade any of this! I love doing all of this. But I will admit, that it has been an extremely busy seven years. There really has not been much time for "just living", just breathing, just adjusting to this life.
So it occurred to me today that just possibly, I am still recovering from that moment when the PWS reality entered our lives. Maybe that is why some days I just feel numb, or like I could just sit for hours and hours and do nothing else, not even really think about much. Because PWS throws something new at us on a very, very regular basis - which means that even as we are still wrapping our minds around PWS, we are also still having to recalibrate our family life. S isn't finished throwing curveballs at us, because she still has quite a bit of developing to do, so the surprises and challenges will keep coming - without the hope that one day, she'll be fully independent and will be able to handle things on her own. So here we are, with some parts of our future with PWS somewhat predictable...and the rest of it something we'll just have to keep recovering from.
Added on to all of that, is the process of parenting an 11-year-old who looks older every day. She'll be a teenager soon. She'll be wanting to date, sooner than I'm comfortable with. Someday before I know it she'll be driving. Oh, my. By the time AARP comes looking for me, I'll be positively catatonic.
Thanks for reading-
Later,
Jen
Thursday, May 12, 2011
{Re-post} "What is Prader-Willi Syndrome??"
Q. "Prader-what? I've never heard of Prader-Willi Syndrome; what is it? How does it affect your daughter?"
A. Without a doubt, every parent or close family member of an individual with PWS has gotten this question multiple times...and has probably also developed their own "stock answer" for it!
Prader-Willi Syndrome is a genetic syndrome affecting the 15th chromosome pair. It occurs in 1 out of every 12,000 - 15,000 births - which makes it both relatively rare, and also more common than you might think! PWS is the most common genetically-based cause of obesity in children. It is found in both genders and all ethnicities.
PWS looks one way from birth to ages 2-6: Infants have extremely low muscle tone, a weak-to-nonexistent cry and almost no ability to coordinate feeding/breathing. Many infants with PWS have stays in the NICU, and many are discharged with either naso-gastric or direct gastric tubes for feeding. Infants with PWS are generally extremely lethargic and sleepy, and must be fed on a strict schedule as otherwise they would sleep to the possible point of starvation; infants w/PWS are generally labelled "failure to thrive" at this stage.
The PWS diagnostic process is usually begun shortly after birth, and while there are obvious clinical signs which SHOULD alert a neonatalogist/pediatrician to PWS, a definitive PWS diagnosis involves bloodwork.
This complex syndrome, characterized by poor feeding and slow weight gain at first, takes a 180-degree turn sometime between the ages of 2 and 6 years old. The child with PWS begins to experience hyperphagia, which is an uncontrollable appetite and 24/7 feelings of hunger even after the child has eaten a regular meal. The metabolism is affected with this syndrome, such that individuals with PWS gain weight twice-as-fast on half the calories. This, combined with the hyperphagia, requires that the food/caloric intake of a person with PWS be strictly monitored at all time; without supervision it is possible for the individual to either consistently over-eat (which for them can look like the average person's 2,000 calorie day) to the point of morbid obesity/diabetes/heart complications. Individuals with PWS require restricted access to food and constant supervision; without these measures it is then possible for them to consume enough food at one sitting to cause their stomach to rupture, leading to death.
Individuals with PWS often also experience: Cognitive delays, gross-motor delays (walking), moderate-to-severe scoliosis, speech delays, dental issues....in PWS the hypothalamus is affected, and this small endocrine organ is extremely important to hunger/satiety, pain sensation, bodily temperature regulation, fluid balance, emotions, puberty and fertility. So, you can see that once the hypothalamus is affected, as it is with PWS, this causes many, many ripples of consequence.
My own personal take on this complex, challenging, sometimes aggravating syndrome? It sucks. I love my S-girl and I don't need to "fix" her, but this syndrome puts her, and all the other individuals who have it, through h-e-double-hockey-sticks.
[The above was originally posted on my blog some months ago. I am re-posting it because my blog is connected to "Blog Frog", and internet blogging service which then allows others from around the country to see this post. Because this is Prader-Willi Syndrome Awareness Month, I re-posted this to build awareness of PWS. :-) ]
Thanks for reading!
Later,
Jen
A. Without a doubt, every parent or close family member of an individual with PWS has gotten this question multiple times...and has probably also developed their own "stock answer" for it!
Prader-Willi Syndrome is a genetic syndrome affecting the 15th chromosome pair. It occurs in 1 out of every 12,000 - 15,000 births - which makes it both relatively rare, and also more common than you might think! PWS is the most common genetically-based cause of obesity in children. It is found in both genders and all ethnicities.
PWS looks one way from birth to ages 2-6: Infants have extremely low muscle tone, a weak-to-nonexistent cry and almost no ability to coordinate feeding/breathing. Many infants with PWS have stays in the NICU, and many are discharged with either naso-gastric or direct gastric tubes for feeding. Infants with PWS are generally extremely lethargic and sleepy, and must be fed on a strict schedule as otherwise they would sleep to the possible point of starvation; infants w/PWS are generally labelled "failure to thrive" at this stage.
The PWS diagnostic process is usually begun shortly after birth, and while there are obvious clinical signs which SHOULD alert a neonatalogist/pediatrician to PWS, a definitive PWS diagnosis involves bloodwork.
This complex syndrome, characterized by poor feeding and slow weight gain at first, takes a 180-degree turn sometime between the ages of 2 and 6 years old. The child with PWS begins to experience hyperphagia, which is an uncontrollable appetite and 24/7 feelings of hunger even after the child has eaten a regular meal. The metabolism is affected with this syndrome, such that individuals with PWS gain weight twice-as-fast on half the calories. This, combined with the hyperphagia, requires that the food/caloric intake of a person with PWS be strictly monitored at all time; without supervision it is possible for the individual to either consistently over-eat (which for them can look like the average person's 2,000 calorie day) to the point of morbid obesity/diabetes/heart complications. Individuals with PWS require restricted access to food and constant supervision; without these measures it is then possible for them to consume enough food at one sitting to cause their stomach to rupture, leading to death.
Individuals with PWS often also experience: Cognitive delays, gross-motor delays (walking), moderate-to-severe scoliosis, speech delays, dental issues....in PWS the hypothalamus is affected, and this small endocrine organ is extremely important to hunger/satiety, pain sensation, bodily temperature regulation, fluid balance, emotions, puberty and fertility. So, you can see that once the hypothalamus is affected, as it is with PWS, this causes many, many ripples of consequence.
My own personal take on this complex, challenging, sometimes aggravating syndrome? It sucks. I love my S-girl and I don't need to "fix" her, but this syndrome puts her, and all the other individuals who have it, through h-e-double-hockey-sticks.
[The above was originally posted on my blog some months ago. I am re-posting it because my blog is connected to "Blog Frog", and internet blogging service which then allows others from around the country to see this post. Because this is Prader-Willi Syndrome Awareness Month, I re-posted this to build awareness of PWS. :-) ]
Thanks for reading!
Later,
Jen
Tuesday, May 10, 2011
May is Prader-Willi Syndrome Awareness Month!
Well, it won't show up on your general calendar...yet! In honor of PWS Awareness Month, here are some facts about the syndrome:
- Prader-Willi syndrome (PWS) is the most common known genetic cause of life-threatening obesity in children.
- How common is PWS? Well, estimates range from 1:8,000 births to 1:25,000 births, with the most likely figure being 1:15,000 births. Taking the most current population figures for just the United States, and using the 1:15,000 births ratio, this means that in the U.S. alone there are almost 21,000 individuals with PWS. Many cases go or have gone undiagnosed or misdiagnosed because genetic testing for PWS is not always immediately suggested by neonatalogists or pediatricians caring for infants with clinical signs of the syndrome.
- All individuals with PWS generally experience low muscle tone, short stature if not treated with growth hormone, incomplete sexual development, and a chronic feeling of hunger that, coupled with a metabolism that utilizes drastically fewer calories than normal, can lead to excessive eating and life-threatening obesity.
- The early stage of PWS - up to approximately 2 years of age - is characterized by "failure to thrive". Infants are generally unable to feed via bottle or breast and many parents rely on a naso-gastric or gastric tube for feeding; weight gain is slow and the chew-swallow-breathe reflexes take time to develop.
- Between the ages of 2 and 6, children with PWS generally begin to experience "hyperphagia", or a constant belief that they are starving. This leads to extreme anxiety about the next meal or snack, even as the child's body (the hypothalamus, specifically) is unable to register that the stomach is full. Thus, individuals with PWS must have constant supervision, especially in any situation where food is accessible.
Prader-Willi syndrome was first formally described in the medical literature in 1956 by Swiss endocrinologists A. Prader, H. Willi, and A. Labhart. The genetic abnormality was discovered in 1981 by David Ledbetter while he was a graduate student.- Individuals with PWS are (generally speaking) on growth hormone therapy, which means that a daily injection of growth hormone is administered. GH therapy in individuals with PWS helps to increase lean body mass, decrease body fat, increase bone mineral density, and normalize height. GH therapy has also been shown to improve strength, agility, and motor-skills development. (S has been on GH therapy since she was 10 months old. She would not be doing as well as she is without it - but there are families where GH therapy has been used for their child and there weren't the most positive results. To date GH is the only "pharmaceutical" help which has been proven to benefit individuals with PWS. However, it is not a cure.)
I am writing this post on a day when, as I checked my e-mail early this morning, I read a message from a PWS chapter leader in another state. She relayed the sad news that a 31-year-old woman with PWS passed away over the weekend. The parents, while devastated, have made the extremely compassionate and giving decision to donate their daughter's brain to research.
This young woman was only 31. That is so, so young. In my own state, our PWS community lost a young woman - just 21 years old - in November. Another adult with PWS, who I think was only in her 50s, passed away a few weeks ago. My heart goes out to their families....their childrens' lives were far too short.
When I hear of any individual with PWS passing away, their relatively young ages always, always make me incredibly sad. My heart is gripped with fear and unquantifiable loss, when I think of how PWS may, someday, take my own daughter's life. I may outlive my youngest child. Someday this syndrome may leave my older daughter without any living siblings. PWS sucks.
For more information about Prader-Willi Syndrome or to donate towards research and quality-of-life projects, please visit the website of PWSA(USA), http://www.pwsausa.org/.
Thank you for reading!
Later,
Jen
Tuesday, April 26, 2011
The long day that might be tomorrow
Tomorrow is our appointment at our PWS clinic. For those of you who aren't familiar with what this means, our clinic days are the days we take S down to our Children's Hospital, to see a team of doctors etc. who specialize in PWS. Tomorrow we will be checking in with the geneticist who is a PWS expert, a behavioral psychologist, and a dietitian. We may possibly also see the endo but I took S for a separate appointment with him in January, so we may just say hi to him. I really like all the people we see; they are great humans and they really care about the kids they work with.
However, these are not my favorite days. I do not look forward to these days. I will be greatly relieved if we can even get out the door without S fighting me about what she should wear. I will be shocked if we get through the day without the "S needs to lose weight" talk. I have to imagine that, for many a PWS parent, that talk becomes a bit inevitable. I'm sure that various PWS parents who might read my blog, may be gasping in horror right now that we have allowed S's weight to get into an unacceptable range. I do not fault the doctors or the dietician for telling us that we need to address the issue; that is their job. They have charts to look at, growth curves to map out, percentiles which go strictly by the numbers and must be adhered to. They are just doing their job. They are going to grill me about her diet, what we feed her, how much exercise she gets. It is going to be grueling, and we will simultaneously be trying to keep S amused throughout all this. We will try to explain S's current behavioral challenges, and we will try to make the best decision possible as to whether a small dose of medication - Zol.oft, Proza/c, like that - is looming. How bad does it have to get, just trying to basically live with a child with behavioral issues, before medication becomes necessary? I don't know. It's not something I really wanted to have to do for S. But the truth is, it is becoming harder and harder to have her get through consecutive days, and have her mood/demeanor/personality remain stable. And no, I don't really think these are behaviors she's exhibiting because of the food dyes/preservatives/gluten in her food or too many carbs in her diet. I mean, if the dietician tomorrow says, "try this diet with S, I have personally seen it cause positive behavioral changes", then we'll see, but I don't really know where this is headed. I just know that I live on pins and needles all. day. long. with her, wondering what seemingly unimportant thing is going to set her off into an epic, ugly meltdown. The meltdowns are becoming harder to bounce back from, for both her and the rest of us!
My older daughter K, is also going to have an interesting day tomorrow. The 4th, 5th, and 6th grade classes are taking the first part of our state's standardized testing tomorrow, with the second half for her grade on Friday. I really am curious as to how this will go, as the math prep-book which she's been bringing home since January has been causing her a significant amount of stress. There were several evenings where the only way she was going to get the math pages done was if B or I sat with her and coached her through the problems. What will she do for those sections of the test tomorrow (or Friday, not sure which day math is)? Her specific 6th-grade class has also been learning how to do a research paper. This is complicated by the fact that her teacher, who is somewhat of a computer geek, is having them do this using computer software specifically focused on writing papers. So, not only is she having to learn what a research paper even is, and all the parts of the process that come before writing one, she is having to learn how to use this software. I like her teacher a lot but this was NOT a good combination, not for K and probably not for others in the class either. What's wrong with good 'ol index cards for pete's sake??
So, I'm going to bed "early", in the hopes that I might actually sleep.
Later,
Jen
However, these are not my favorite days. I do not look forward to these days. I will be greatly relieved if we can even get out the door without S fighting me about what she should wear. I will be shocked if we get through the day without the "S needs to lose weight" talk. I have to imagine that, for many a PWS parent, that talk becomes a bit inevitable. I'm sure that various PWS parents who might read my blog, may be gasping in horror right now that we have allowed S's weight to get into an unacceptable range. I do not fault the doctors or the dietician for telling us that we need to address the issue; that is their job. They have charts to look at, growth curves to map out, percentiles which go strictly by the numbers and must be adhered to. They are just doing their job. They are going to grill me about her diet, what we feed her, how much exercise she gets. It is going to be grueling, and we will simultaneously be trying to keep S amused throughout all this. We will try to explain S's current behavioral challenges, and we will try to make the best decision possible as to whether a small dose of medication - Zol.oft, Proza/c, like that - is looming. How bad does it have to get, just trying to basically live with a child with behavioral issues, before medication becomes necessary? I don't know. It's not something I really wanted to have to do for S. But the truth is, it is becoming harder and harder to have her get through consecutive days, and have her mood/demeanor/personality remain stable. And no, I don't really think these are behaviors she's exhibiting because of the food dyes/preservatives/gluten in her food or too many carbs in her diet. I mean, if the dietician tomorrow says, "try this diet with S, I have personally seen it cause positive behavioral changes", then we'll see, but I don't really know where this is headed. I just know that I live on pins and needles all. day. long. with her, wondering what seemingly unimportant thing is going to set her off into an epic, ugly meltdown. The meltdowns are becoming harder to bounce back from, for both her and the rest of us!
My older daughter K, is also going to have an interesting day tomorrow. The 4th, 5th, and 6th grade classes are taking the first part of our state's standardized testing tomorrow, with the second half for her grade on Friday. I really am curious as to how this will go, as the math prep-book which she's been bringing home since January has been causing her a significant amount of stress. There were several evenings where the only way she was going to get the math pages done was if B or I sat with her and coached her through the problems. What will she do for those sections of the test tomorrow (or Friday, not sure which day math is)? Her specific 6th-grade class has also been learning how to do a research paper. This is complicated by the fact that her teacher, who is somewhat of a computer geek, is having them do this using computer software specifically focused on writing papers. So, not only is she having to learn what a research paper even is, and all the parts of the process that come before writing one, she is having to learn how to use this software. I like her teacher a lot but this was NOT a good combination, not for K and probably not for others in the class either. What's wrong with good 'ol index cards for pete's sake??
So, I'm going to bed "early", in the hopes that I might actually sleep.
Later,
Jen
Saturday, April 23, 2011
Prader-Willi Syndrome: Community = Room for Everyone
I still remember, quite vividly, many of the moments and experiences of the first six months after S was diagnosed. I remember sitting at the dining-room table, on the phone, taking notes as I listened to the geneticist tell me the news. I had never heard of Prader-Willi Syndrome before, neither had my husband, neither had any of my family.
We weren't alone, though. Even before our second appointment with the geneticist, after she had evaluated S, the bloodwork had been drawn, and the diagnosis was certain, I had landed at the website for the national Prader-Willi Syndrome organization PWSA(USA). We had taken with us to the geneticist's office, printouts of pages from that website, to help us ask as many of the right questions as possible. It was significant, I thought, that the geneticist also had printouts...also from that website. After that appointment and through the next months, we would begin meeting family after family, both from our own state and elsewhere, who were on their own - but a similar - PWS journey.
We had found our new "family", our new community which extended around the globe. At this point I can't even begin to accurately count how many families I have met in real life, online, on Facebook, through message boards, and at conferences, who feel like my family. I mean, I know we might not be best friends were we to hang out in real life - but it is immediately understood, among PWS parents (and any extended family who truly try to understand the PWS life), that we all "get it". We all know what's involved in living with PWS. We all know there are good days and bad days. We all know, I think, that being a special-needs-parent does not automatically mean we are "Perfect PWS Parents"...rather it means we all try hard to give our children good, happy, healthy lives, but sometimes life doesn't cooperate with those efforts.
To me, the word "community" implies inclusion of all realities and all perspectives. In the PWS community there is room for all kinds of families. There is room for parents whose child is in an extremely healthy, stable phase of PWS. There is also room for parents and families where the individual with PWS is definitely not in a calm, stable phase and the family is struggling on a day-to-day basis to get through. There is room for families whose child has never had a bite of fast-food pass their lips, and there is room for families where the parents are okay with the occasional fast-food or restaurant meal. There is room for families where the parents have decided to have the whole family go vegan/gluten-free/low-carb or whatever....and there is room for families who have found success without doing those things. In this community, in my opinion, it is perfectly okay for PWS parents to vent, to express frustration with PWS, to be open about the sometimes horrifically bad days PWS can cause for an entire family. It is also perfectly okay to brag about the successes of a child with PWS, because oftentimes those successes are hard-won.
While I would never provide scads of detail, to parents of a newly-diagnosed child, of every difficult/heart-breaking/challenging/utterly frustrating situation PWS can cause, I am not going to sugarcoat anything. And I think every PWS parent has the right to say "hey, this really sucks" if they need to. Some days, PWS does suck. If it were easy and effortless and our children never suffered, sure, then parents would not have much to feel angry/frustrated/depressed about. But I firmly believe that even as we all fiercely love our children, that love and acceptance can indeed coexist just fine with honesty about how difficult living with PWS can be. I believe in keeping it real, in telling it like it is, when appropriate and to those parents who are ready to hear it. Every PWS family out there IS going to face their own variety of PWS challenges....that is the nature of the PWS beast. It is okay to comment on those challenges, and I think the last people who should ever be judgemental about the venting comments of a PWS parent is...another special-needs parent. Parents who are having a rough time with their child should always feel okay with talking about it; because there are definitely other families out there who are also going through a rough time (that's not a "glass half-empty" prediction, that is a fact - I've seen it happen many times). If a parent who is going through a hard time with their child-with-PWS is "scolded" for talking about their less-than-sunshiny experiences, what's the consequence? That parent is made to feel alone, as if they are doing something wrong with their child, that the complex challenges of PWS are completely controllable and anything less than total calm in their house must be because of their bad parenting.
What parent EVER needs THAT kind of response??
We are all on the same PWS sailboat....when another parent starts to sink with the weight of the PWS life, give them a hug and a life preserver, don't make them walk the plank.
Later,
Jen
We weren't alone, though. Even before our second appointment with the geneticist, after she had evaluated S, the bloodwork had been drawn, and the diagnosis was certain, I had landed at the website for the national Prader-Willi Syndrome organization PWSA(USA). We had taken with us to the geneticist's office, printouts of pages from that website, to help us ask as many of the right questions as possible. It was significant, I thought, that the geneticist also had printouts...also from that website. After that appointment and through the next months, we would begin meeting family after family, both from our own state and elsewhere, who were on their own - but a similar - PWS journey.
We had found our new "family", our new community which extended around the globe. At this point I can't even begin to accurately count how many families I have met in real life, online, on Facebook, through message boards, and at conferences, who feel like my family. I mean, I know we might not be best friends were we to hang out in real life - but it is immediately understood, among PWS parents (and any extended family who truly try to understand the PWS life), that we all "get it". We all know what's involved in living with PWS. We all know there are good days and bad days. We all know, I think, that being a special-needs-parent does not automatically mean we are "Perfect PWS Parents"...rather it means we all try hard to give our children good, happy, healthy lives, but sometimes life doesn't cooperate with those efforts.
To me, the word "community" implies inclusion of all realities and all perspectives. In the PWS community there is room for all kinds of families. There is room for parents whose child is in an extremely healthy, stable phase of PWS. There is also room for parents and families where the individual with PWS is definitely not in a calm, stable phase and the family is struggling on a day-to-day basis to get through. There is room for families whose child has never had a bite of fast-food pass their lips, and there is room for families where the parents are okay with the occasional fast-food or restaurant meal. There is room for families where the parents have decided to have the whole family go vegan/gluten-free/low-carb or whatever....and there is room for families who have found success without doing those things. In this community, in my opinion, it is perfectly okay for PWS parents to vent, to express frustration with PWS, to be open about the sometimes horrifically bad days PWS can cause for an entire family. It is also perfectly okay to brag about the successes of a child with PWS, because oftentimes those successes are hard-won.
While I would never provide scads of detail, to parents of a newly-diagnosed child, of every difficult/heart-breaking/challenging/utterly frustrating situation PWS can cause, I am not going to sugarcoat anything. And I think every PWS parent has the right to say "hey, this really sucks" if they need to. Some days, PWS does suck. If it were easy and effortless and our children never suffered, sure, then parents would not have much to feel angry/frustrated/depressed about. But I firmly believe that even as we all fiercely love our children, that love and acceptance can indeed coexist just fine with honesty about how difficult living with PWS can be. I believe in keeping it real, in telling it like it is, when appropriate and to those parents who are ready to hear it. Every PWS family out there IS going to face their own variety of PWS challenges....that is the nature of the PWS beast. It is okay to comment on those challenges, and I think the last people who should ever be judgemental about the venting comments of a PWS parent is...another special-needs parent. Parents who are having a rough time with their child should always feel okay with talking about it; because there are definitely other families out there who are also going through a rough time (that's not a "glass half-empty" prediction, that is a fact - I've seen it happen many times). If a parent who is going through a hard time with their child-with-PWS is "scolded" for talking about their less-than-sunshiny experiences, what's the consequence? That parent is made to feel alone, as if they are doing something wrong with their child, that the complex challenges of PWS are completely controllable and anything less than total calm in their house must be because of their bad parenting.
What parent EVER needs THAT kind of response??
We are all on the same PWS sailboat....when another parent starts to sink with the weight of the PWS life, give them a hug and a life preserver, don't make them walk the plank.
Later,
Jen
Thursday, April 14, 2011
Prader-Willi Syndrome and the IEP*
*IEP = Individual Education Plan. This is a legal document which is developed by a team of individuals including the parents/guardians of a special-needs child, the school principal, the school Intervention Specialist, any physical/occupation/speech therapists, and sometimes if necessary the school nurse...and possibly other school district or school staff members. The IEP is a legally binding plan for any and all therapeutic, academic, behavioral supports and accomodations a child may need in order to participate in the "free and appropriate public education" (FAPE) every state is mandated to provide for every child. The Individuals with Disabilities Education Act (IDEA) drives the structure of, and policies/procedures for developing, an IEP. (Warning: that is a very simplified explanation!)
An alternative title for this post would be "Thankful Thursday". Brad and I had S's IEP meeting this morning. At this point the only thing that makes me nervous about these meetings is hoping I will remember to bring up everything which S might need, over the next school year. I don't really need to be that nervous about it, as we can call a meeting at any point to make any changes to S's IEP. And fortunately, our school district has thus far been really great in working with us to make sure S is learning appropriately and is in a food-secure environment. Some of the details of her IEP:
-she does not EVER go anywhere in the building without adult supervision. We have to make it clear that she must be accompanied by an adult and not a classmate; I don't want another seven-year-old to have the responsibilty for keeping her out of lockers, lunch boxes, or trash cans if she suddenly decides to search for food.
-she must have 1:1 direct supervision during lunch and snack. We had to clarify this today, as the wording for this accomodation was simply going to be "supervision", which to me could easily be interpreted by an aide as "I can stand 10 feet away from her, supervise 10 other kids at the same time, and she'll still be 'supervised'". S needs someone sitting right next to her; having someone stand 5 feet away and possibly get distracted is still too much opportunity for her to snag a classmate's food.
-This time around, for this IEP covering the next year, we made it a point to add a behavioral detail to the document. S has not yet, thankfully, had a meltdown during school hours. There have been plenty of times since the school year began that a meltdown would start almost as soon as she came home, usually triggered by something really trivial. While I'm glad for the school staff's sake that she hasn't had a meltdown at school, I would not be surprised if, at some point in the next year, these started to happen. So I had to let them know what a meltdown looks like for her - and for those of you who have children without diagnosed behavioral issues, S's meltdowns are nothing like your average tantrum. She completely loses control, screams, and has started kicking/hitting out of sheer anger. It is, to put it bluntly, an ugly moment. These moments are only going to get more challenging as she gets older and bigger. So, the school staff needs to be aware of this possibility, and they need to have a plan in place. They also need to know that restraining her during these meltdowns is a really, really bad idea.
We also talked with the staff about where S is at, academically. They have stated bluntly that at this point, they see absolutely no reason to hold her back and have her repeat any of the next couple grades. She is behind in math and reading, and does need accomodations in those subjects. At the same time, her progress since the beginning of the year has been phenomenal - she is getting really good at her reading, and can read quite a few of the Dr. Suess books on her own - so at this point the consensus is that she is gaining so much from advancing through the grades with her peers, that it is greatly to her benefit to keep on this way. We are glad for that. In S's case, and perhaps for many children with PWS, she may never be able to learn at the same level as her peers. But continuing on in the same classroom, spending as much time as possible with those peers, is an incredible motivator for her.
We are so incredibly thankful that we landed in this school district. I mean, obviously my husband and I have tried to go about all of this as calmly and firmly as possible, and made ourselves quite visible to the school staff over the years. But I still feel fortunate that the school district has responded more than adequately to what we need to keep S safe at school. I know many, many families of children with PWS have problems - serious, long-lasting problems - obtaining appropriate services for their child from their school district; and sadly sometimes this happens simply because the school district doesn't want to take the time to understand the child's needs, and needs to face a potential lawsuit before they'll do what they were supposed to do all along.
I hope you have lots to be thankful for today.
Later,
Jen
An alternative title for this post would be "Thankful Thursday". Brad and I had S's IEP meeting this morning. At this point the only thing that makes me nervous about these meetings is hoping I will remember to bring up everything which S might need, over the next school year. I don't really need to be that nervous about it, as we can call a meeting at any point to make any changes to S's IEP. And fortunately, our school district has thus far been really great in working with us to make sure S is learning appropriately and is in a food-secure environment. Some of the details of her IEP:
-she does not EVER go anywhere in the building without adult supervision. We have to make it clear that she must be accompanied by an adult and not a classmate; I don't want another seven-year-old to have the responsibilty for keeping her out of lockers, lunch boxes, or trash cans if she suddenly decides to search for food.
-she must have 1:1 direct supervision during lunch and snack. We had to clarify this today, as the wording for this accomodation was simply going to be "supervision", which to me could easily be interpreted by an aide as "I can stand 10 feet away from her, supervise 10 other kids at the same time, and she'll still be 'supervised'". S needs someone sitting right next to her; having someone stand 5 feet away and possibly get distracted is still too much opportunity for her to snag a classmate's food.
-This time around, for this IEP covering the next year, we made it a point to add a behavioral detail to the document. S has not yet, thankfully, had a meltdown during school hours. There have been plenty of times since the school year began that a meltdown would start almost as soon as she came home, usually triggered by something really trivial. While I'm glad for the school staff's sake that she hasn't had a meltdown at school, I would not be surprised if, at some point in the next year, these started to happen. So I had to let them know what a meltdown looks like for her - and for those of you who have children without diagnosed behavioral issues, S's meltdowns are nothing like your average tantrum. She completely loses control, screams, and has started kicking/hitting out of sheer anger. It is, to put it bluntly, an ugly moment. These moments are only going to get more challenging as she gets older and bigger. So, the school staff needs to be aware of this possibility, and they need to have a plan in place. They also need to know that restraining her during these meltdowns is a really, really bad idea.
We also talked with the staff about where S is at, academically. They have stated bluntly that at this point, they see absolutely no reason to hold her back and have her repeat any of the next couple grades. She is behind in math and reading, and does need accomodations in those subjects. At the same time, her progress since the beginning of the year has been phenomenal - she is getting really good at her reading, and can read quite a few of the Dr. Suess books on her own - so at this point the consensus is that she is gaining so much from advancing through the grades with her peers, that it is greatly to her benefit to keep on this way. We are glad for that. In S's case, and perhaps for many children with PWS, she may never be able to learn at the same level as her peers. But continuing on in the same classroom, spending as much time as possible with those peers, is an incredible motivator for her.
We are so incredibly thankful that we landed in this school district. I mean, obviously my husband and I have tried to go about all of this as calmly and firmly as possible, and made ourselves quite visible to the school staff over the years. But I still feel fortunate that the school district has responded more than adequately to what we need to keep S safe at school. I know many, many families of children with PWS have problems - serious, long-lasting problems - obtaining appropriate services for their child from their school district; and sadly sometimes this happens simply because the school district doesn't want to take the time to understand the child's needs, and needs to face a potential lawsuit before they'll do what they were supposed to do all along.
I hope you have lots to be thankful for today.
Later,
Jen
Thursday, April 7, 2011
Prader-Willi Syndrome: Ten bits of advice for parents of newly diagnosed kids
I have had many opportunities, over the past 4 years or so, to speak with parents shortly after their little ones are first diagnosed. I can only hope that what I have been able to tell them, and my answers to their questions, have been of some help in those first crazy and emotional weeks and months after first learning of their child's PWS diagnosis. Below is a list of ten things which have all, at one point or another, made it into my conversations with parents just beginning the PWS journey. A warning for readers - I do not consider myself to be a "perfect PWS parent". Parents who have been living this PWS journey for even just a few years know that once you get beyond the top 15 basic things to do for a child with PWS, the lifestyle choices of any particular family come into play....so if you are a PWS parent reading this, please keep that in mind - every family is different, and I know you would be able to add considerably to this list! Also, these are only in the order in which I thought of them tonight, not in order of importance.
1. HAVE HOPE. Seriously, have hope! The diagnosis is a shock and it will change how you thought your life would look with children - but HAVE HOPE for your child's future. They WILL be happy, and so will you! Allow yourselves time to adjust to all this. And, know that adjusting to 'all this' may take a few years...or more. That's okay!
2. Expect to be pretty busy, that first year post-diagnosis. Doctor's appointments, therapies, etc. However...
3. DO NOT DRIVE YOURSELVES CRAZY, feeling like you should have accomplished that to-do list yesterday. As long as you know what you have to do for your child, and go about it at your own pace, you and your child will be fine. So many parents, I think, start to feel like if they don't do a, b, or c right away, or start GH therapy immediately, that their child will suffer immensely. Not true! My daughter started GH shots at 10 months - which, these days, is about 5 months (at least) later than kids are starting it now. You know what? She still saw and sees the benefits from GH! Even adults with PWS, who have never been able do GH therapy before, see benefits from the shots! So, if GH therapy is something you choose to pursue for your child, yes get the process started, and stay on top of everything that needs to be done - there is no reason these days for the process to take longer than 5-8 months at the most. But once you know you are doing what you need to, to get your child going with the shots, then take a breath and relax. (and then go schedule those other doctor appointments!!) (kidding on that last bit. Sit down and breathe!)
4. If you are choosing to pursue GH therapy for your child, find an endocrinologist who is familiar with PWS and is not afraid to consider GH therapy for infants/children under 2. GH therapy for very young children is still a concept with which some endocrinologists may be unfamiliar. There are circumstances in which GH therapy may have to be delayed or discontinued - but initially, your endo should be willing to at least start the process, have your child do a sleep study, etc. If they aren't willing to begin the process, find another endocrinologist, or at least find an endo who is willing to consult via phone/e-mail with another endo or PWS expert regarding GH therapy for your child. The age of your child is not enough to deny treatment with GH shots!
5. If you are parents to more than your child with PWS, I strongly suggest instituting some basic "food rules" in your house. (Even if you have only one child, these still make sense!) These are pretty general, and in my house have helped regulate our food consumption and maintain some sanity. These rules are: 1) Eating and snacking times are the same for everyone; there is NO free-range food consumption. If you're hungry, you wait 'til the next scheduled meal or snack-time, to eat. No-one is to ask for food or snacks outside of scheduled snack and meal times. and 2) There is no sharing of food, and no eating off of anyone else's plate. 3) The kitchen and food areas are off-limits to everyone except parents (kids usually don't need to be sipping water or juice all day, so they don't need to be going in and out of the kitchen for that, either).
6. This kind of goes along with #5: If there are multiple children in the home, start to gradually work everyone into a snack-and-meal routine - i.e. the idea that these happen only at specific times. Children are resilient and do indeed soak up knowledge, but it still takes time for them to learn that they are no longer going to be able to walk around the house munching out of a box of Gold.Fish crackers all day. I think kids also need time to learn that while sharing of toys is great, they are no longer encouraged/allowed to share food, period - not with their 'typical' siblings and not with their sibling who has PWS.
7. I always think it is important to tell parents of newly-diagnosed newborns and infants these things:
-their child WILL roll over, sit up, cruise the furniture, and walk;
-their child WILL talk, sing, and smile;
-their child WILL laugh, and play;
-their child WILL be able to do many of the things all children do (including fight with their siblings yay!).
The PWS diagnosis does not mean the end of happiness in your family! Your child will amaze you and thrill you and bring you to happy tears with their hard-won accomplishments. They are spectacular kids! Living with PWS will take you down a 'road less travelled'....where you still have other families and much joy to accompany you.
8. Join up with the national PWS organization in the U.S., PWSA(USA) - visit http://www.pwsausa.org/ for more information. There is also an international PWS organization, IPWSO - visit http://www.ipwso.org/ to find out more. There are also many state chapters in the U.S., and the office at PWSA(USA) can also connect you with those and with another family in your state. Try to meet, visit with, or somehow connect with other families near you if you can. Personally, I feel that our Sophie would not be doing as well as she is if my husband and I had isolated ourselves and tried to do this alone. Find other families and maintain your connections to the PWS community, even when you feel like you're in a 'calm PWS phase'. It's important!
9. The role of extended family: Your extended family members can prove to be amazingly supportive...or not! If you spend a significant amount of time with extended family, you will need to find a way to educate them about the 'policies and procedures of PWS'. In our society food is so pervasive - well beyond the basic needs of human survival. Food is used to show love, to soothe boo-boos, to celebrate anything and everything - especially with extended family. Most of this will be affected by the requirement of PWS, that food not be readily available or used to treat/reward your child. It takes some diplomacy, tact, patience and time to convey this message to extended family....and, sad to say, sometimes extended family members are not always able or willing to understand this. Be prepared to adjust your family's activities as necessary, to keep your child safe.
10. You can expect your family's eating habits to change, because of your child's PWS diagnosis. My own family does still eat out, and we do still go to family holiday dinners, but our eating habits have definitely been affected by the presence of PWS in our house. Living with PWS will, whether you like it or not, change the way you think about food and how/when/why/in what quantities it is consumed in your house. This is good though! You will eventually find yourself making healthier choices for yourself more often, because you are paying such close attention to food ingredients and calorie counts for your child's sake. Don't feel like you have to make food changes overnight, and don't worry - you will still be able to have treats or whatever. But, understand that for your own sanity and your child's health, safety, and well-being, those changes are part of this journey.
Well, as always when I'm writing about PWS, I could go on...and on...and on....but I'll stop now. Thank you for reading and for any feedback.
Later,
Jen
1. HAVE HOPE. Seriously, have hope! The diagnosis is a shock and it will change how you thought your life would look with children - but HAVE HOPE for your child's future. They WILL be happy, and so will you! Allow yourselves time to adjust to all this. And, know that adjusting to 'all this' may take a few years...or more. That's okay!
2. Expect to be pretty busy, that first year post-diagnosis. Doctor's appointments, therapies, etc. However...
3. DO NOT DRIVE YOURSELVES CRAZY, feeling like you should have accomplished that to-do list yesterday. As long as you know what you have to do for your child, and go about it at your own pace, you and your child will be fine. So many parents, I think, start to feel like if they don't do a, b, or c right away, or start GH therapy immediately, that their child will suffer immensely. Not true! My daughter started GH shots at 10 months - which, these days, is about 5 months (at least) later than kids are starting it now. You know what? She still saw and sees the benefits from GH! Even adults with PWS, who have never been able do GH therapy before, see benefits from the shots! So, if GH therapy is something you choose to pursue for your child, yes get the process started, and stay on top of everything that needs to be done - there is no reason these days for the process to take longer than 5-8 months at the most. But once you know you are doing what you need to, to get your child going with the shots, then take a breath and relax. (and then go schedule those other doctor appointments!!) (kidding on that last bit. Sit down and breathe!)
4. If you are choosing to pursue GH therapy for your child, find an endocrinologist who is familiar with PWS and is not afraid to consider GH therapy for infants/children under 2. GH therapy for very young children is still a concept with which some endocrinologists may be unfamiliar. There are circumstances in which GH therapy may have to be delayed or discontinued - but initially, your endo should be willing to at least start the process, have your child do a sleep study, etc. If they aren't willing to begin the process, find another endocrinologist, or at least find an endo who is willing to consult via phone/e-mail with another endo or PWS expert regarding GH therapy for your child. The age of your child is not enough to deny treatment with GH shots!
5. If you are parents to more than your child with PWS, I strongly suggest instituting some basic "food rules" in your house. (Even if you have only one child, these still make sense!) These are pretty general, and in my house have helped regulate our food consumption and maintain some sanity. These rules are: 1) Eating and snacking times are the same for everyone; there is NO free-range food consumption. If you're hungry, you wait 'til the next scheduled meal or snack-time, to eat. No-one is to ask for food or snacks outside of scheduled snack and meal times. and 2) There is no sharing of food, and no eating off of anyone else's plate. 3) The kitchen and food areas are off-limits to everyone except parents (kids usually don't need to be sipping water or juice all day, so they don't need to be going in and out of the kitchen for that, either).
6. This kind of goes along with #5: If there are multiple children in the home, start to gradually work everyone into a snack-and-meal routine - i.e. the idea that these happen only at specific times. Children are resilient and do indeed soak up knowledge, but it still takes time for them to learn that they are no longer going to be able to walk around the house munching out of a box of Gold.Fish crackers all day. I think kids also need time to learn that while sharing of toys is great, they are no longer encouraged/allowed to share food, period - not with their 'typical' siblings and not with their sibling who has PWS.
7. I always think it is important to tell parents of newly-diagnosed newborns and infants these things:
-their child WILL roll over, sit up, cruise the furniture, and walk;
-their child WILL talk, sing, and smile;
-their child WILL laugh, and play;
-their child WILL be able to do many of the things all children do (including fight with their siblings yay!).
The PWS diagnosis does not mean the end of happiness in your family! Your child will amaze you and thrill you and bring you to happy tears with their hard-won accomplishments. They are spectacular kids! Living with PWS will take you down a 'road less travelled'....where you still have other families and much joy to accompany you.
8. Join up with the national PWS organization in the U.S., PWSA(USA) - visit http://www.pwsausa.org/ for more information. There is also an international PWS organization, IPWSO - visit http://www.ipwso.org/ to find out more. There are also many state chapters in the U.S., and the office at PWSA(USA) can also connect you with those and with another family in your state. Try to meet, visit with, or somehow connect with other families near you if you can. Personally, I feel that our Sophie would not be doing as well as she is if my husband and I had isolated ourselves and tried to do this alone. Find other families and maintain your connections to the PWS community, even when you feel like you're in a 'calm PWS phase'. It's important!
9. The role of extended family: Your extended family members can prove to be amazingly supportive...or not! If you spend a significant amount of time with extended family, you will need to find a way to educate them about the 'policies and procedures of PWS'. In our society food is so pervasive - well beyond the basic needs of human survival. Food is used to show love, to soothe boo-boos, to celebrate anything and everything - especially with extended family. Most of this will be affected by the requirement of PWS, that food not be readily available or used to treat/reward your child. It takes some diplomacy, tact, patience and time to convey this message to extended family....and, sad to say, sometimes extended family members are not always able or willing to understand this. Be prepared to adjust your family's activities as necessary, to keep your child safe.
10. You can expect your family's eating habits to change, because of your child's PWS diagnosis. My own family does still eat out, and we do still go to family holiday dinners, but our eating habits have definitely been affected by the presence of PWS in our house. Living with PWS will, whether you like it or not, change the way you think about food and how/when/why/in what quantities it is consumed in your house. This is good though! You will eventually find yourself making healthier choices for yourself more often, because you are paying such close attention to food ingredients and calorie counts for your child's sake. Don't feel like you have to make food changes overnight, and don't worry - you will still be able to have treats or whatever. But, understand that for your own sanity and your child's health, safety, and well-being, those changes are part of this journey.
Well, as always when I'm writing about PWS, I could go on...and on...and on....but I'll stop now. Thank you for reading and for any feedback.
Later,
Jen
Sunday, March 27, 2011
Depends on the listener
Talking about life with S, and about life with Prader-Willi Syndrome, is always influenced by the audience.
I guess this might be obvious, and no surprise. But it is something that really crystallized in my brain just now, as a result of a F.acebook post I just read. Another PWS mom posted a link to an article about a family whose youngest-of-four-children has Down's Syndrome. What surfaced most clearly for me, with this article, is the mom's emphasis on how life with her daughter-with-DS is just...life. It is what it is, and since they have three other children, life is just whatever it is with a house full of children. In other words, their daughter's DS diagnosis doesn't define the family's life, and while the mom does write about it sometimes on her blog, it doesn't direct the blog's content. They live family life just like any other family.
So, given that the article ran in the family's area newspaper, and thus had the general public as it's audience, part of the message here seems to be that children with DS or any diagnosis are children just like any other children, and life with DS is just like "regular" family life. Thus the ultimate, if subconscious, outcome of an article like this might be better overall acceptance of individuals with special needs...and for that, I am intensely grateful to this family and this mom, for putting themselves out there into the public eye.
I kind of wonder, if this mom were trying to raise funds for a DS non-profit, for research or quality-of-life projects, would her message have been different? Because folks, let me tell you - regardless of how smoothly her family has adjusted to her daughter's diagnosis, I bet there are still aspects of DS with which she might wish her daughter didn't have to contend. That's the challenge with education about syndromes which affect children - it is part consolation, part brutally honest education, and all of it is true. Here in my house, because of PWS, our good times with S do happen - and those times are really, really good - and thank God for that because the bad times can be really, really hard.
Talking about Prader-Willi Syndrome, and S's life with it, and our adjustment to it as a family, is very much influenced by my audience. Some examples:
Talking to a parent of one of S's classmates, about an upcoming holiday party: "I have to count S's calorie intake very carefully, so I'd appreciate it if the holiday party treats weren't 100% junkfood. And, please don't offer her seconds."
Talking to the parent of one of S's classmates, about a potential playdate: "Yes, S can come over to play with your child....but, guess what, I have to come with her, for her safety. Plus, here are a few pamphlets for you to read about PWS, before we come over." (I'm not sure I would do the pamphlet thing - but I would have to go with S, and I would have to educate the parent, because American kids in general snack too much so I'm sure that would come up during most playdates.)
Talking to parents of younger children with PWS, sometimes newly diagnosed: "S has done 100% better than we ever expected. Life here is calm, and the food aspect of PWS has been manageable." This is a true statement, for us/S/our family. I'm not going to lay out all the tough parts of PWS to parents who have just started down the PWS path - getting the diagnosis and learning about the syndrome is enough info for them at that point.
Talking to neighbors: "Yes, S can play for a bit....but please do NOT offer her any snacks. Which means, that if your child starts jones-ing for a juicebox, please let me know and I'll take S home."
Talking to S's teachers: "The only thing that is keeping S from having a "negative food episode" is opportunity. She is not to go ANYWHERE in the building unsupervised, and she must be supervised by an adult, not a classmate. If she starts to have a meltdown please do not restrain her, as that only makes it worse."
Talking to any group which is totally unfamiliar with PWS: "Because of PWS my child feels constantly hungry. No, it doesn't matter what or how often she eats. Yes, because of this she potentially might eat until her stomach bursts. Yes this has happened to individuals in the PWS community, with fatal outcomes. No, appetite suppressants do not work, nor does any stomach-altering surgery. Yes, your donations are extremely important, not only for research projects but also for quality-of-life efforts, like PWS-only activities where our kids don't have to worry about pervasive, anxiety-producing food opportunities or gaining 10 pounds in one weekend. Yes, S is doing well but PWS still sucks. No, there is no "cure"....yet."
And those are just some examples....I could think of many more. To fellow families with younger children with PWS, I always want to emphasize the "livability" of PWS - because THAT'S TRUE. You adjust, and it becomes your "normal" as a family. It's do-able, and you will have lots and lots of regular moments with your family. But to the general public? No, this isn't "just like normal life", as most of society knows it. This is hard. My daughter struggles, minute by minute, with what PWS does to her body. She still has a right to, and deserves, acceptance and understanding from society as a whole; but that doesn't change her daily reality, or make it less worthy of funding for quality-of-life funding or research projects.
Thanks for reading!
Later,
Jen
I guess this might be obvious, and no surprise. But it is something that really crystallized in my brain just now, as a result of a F.acebook post I just read. Another PWS mom posted a link to an article about a family whose youngest-of-four-children has Down's Syndrome. What surfaced most clearly for me, with this article, is the mom's emphasis on how life with her daughter-with-DS is just...life. It is what it is, and since they have three other children, life is just whatever it is with a house full of children. In other words, their daughter's DS diagnosis doesn't define the family's life, and while the mom does write about it sometimes on her blog, it doesn't direct the blog's content. They live family life just like any other family.
So, given that the article ran in the family's area newspaper, and thus had the general public as it's audience, part of the message here seems to be that children with DS or any diagnosis are children just like any other children, and life with DS is just like "regular" family life. Thus the ultimate, if subconscious, outcome of an article like this might be better overall acceptance of individuals with special needs...and for that, I am intensely grateful to this family and this mom, for putting themselves out there into the public eye.
I kind of wonder, if this mom were trying to raise funds for a DS non-profit, for research or quality-of-life projects, would her message have been different? Because folks, let me tell you - regardless of how smoothly her family has adjusted to her daughter's diagnosis, I bet there are still aspects of DS with which she might wish her daughter didn't have to contend. That's the challenge with education about syndromes which affect children - it is part consolation, part brutally honest education, and all of it is true. Here in my house, because of PWS, our good times with S do happen - and those times are really, really good - and thank God for that because the bad times can be really, really hard.
Talking about Prader-Willi Syndrome, and S's life with it, and our adjustment to it as a family, is very much influenced by my audience. Some examples:
Talking to a parent of one of S's classmates, about an upcoming holiday party: "I have to count S's calorie intake very carefully, so I'd appreciate it if the holiday party treats weren't 100% junkfood. And, please don't offer her seconds."
Talking to the parent of one of S's classmates, about a potential playdate: "Yes, S can come over to play with your child....but, guess what, I have to come with her, for her safety. Plus, here are a few pamphlets for you to read about PWS, before we come over." (I'm not sure I would do the pamphlet thing - but I would have to go with S, and I would have to educate the parent, because American kids in general snack too much so I'm sure that would come up during most playdates.)
Talking to parents of younger children with PWS, sometimes newly diagnosed: "S has done 100% better than we ever expected. Life here is calm, and the food aspect of PWS has been manageable." This is a true statement, for us/S/our family. I'm not going to lay out all the tough parts of PWS to parents who have just started down the PWS path - getting the diagnosis and learning about the syndrome is enough info for them at that point.
Talking to neighbors: "Yes, S can play for a bit....but please do NOT offer her any snacks. Which means, that if your child starts jones-ing for a juicebox, please let me know and I'll take S home."
Talking to S's teachers: "The only thing that is keeping S from having a "negative food episode" is opportunity. She is not to go ANYWHERE in the building unsupervised, and she must be supervised by an adult, not a classmate. If she starts to have a meltdown please do not restrain her, as that only makes it worse."
Talking to any group which is totally unfamiliar with PWS: "Because of PWS my child feels constantly hungry. No, it doesn't matter what or how often she eats. Yes, because of this she potentially might eat until her stomach bursts. Yes this has happened to individuals in the PWS community, with fatal outcomes. No, appetite suppressants do not work, nor does any stomach-altering surgery. Yes, your donations are extremely important, not only for research projects but also for quality-of-life efforts, like PWS-only activities where our kids don't have to worry about pervasive, anxiety-producing food opportunities or gaining 10 pounds in one weekend. Yes, S is doing well but PWS still sucks. No, there is no "cure"....yet."
And those are just some examples....I could think of many more. To fellow families with younger children with PWS, I always want to emphasize the "livability" of PWS - because THAT'S TRUE. You adjust, and it becomes your "normal" as a family. It's do-able, and you will have lots and lots of regular moments with your family. But to the general public? No, this isn't "just like normal life", as most of society knows it. This is hard. My daughter struggles, minute by minute, with what PWS does to her body. She still has a right to, and deserves, acceptance and understanding from society as a whole; but that doesn't change her daily reality, or make it less worthy of funding for quality-of-life funding or research projects.
Thanks for reading!
Later,
Jen
Thursday, March 24, 2011
Prader-Willi Syndrome and birthdays
[Note: If you are new to this blog, and want to know more about Prader-Willi Syndrome, please visit http://www.pwsausa.org/.]
One of the first issues that often arise, in the lives of families and children living with PWS, is what to do about the birthday cake for the child's first birthday.
In any other families, this is a total NON-issue, right? You just get a cake, or make one...baby smashes it or smears frosting all over their cute face (I have pictures of both my kids like this, so adorable), and life goes on.
But, when a child has PWS, the parents have been told from Day 1 of being diagnosed, that Food Is The Enemy. The child - while totally uninterested in food in the first two years at least - has a faulty hypothalamus, and so at some point will begin to experience a constant, biting, gnawing feeling of hunger. The parents' caution signals in regards to anything food-related go up immediately, of course! And then...along comes the child's First Birthday, or the first birthday post-diagnosis. For S's first few birthdays, we did do the Angel-Food cakes or the Jello desserts. We've moved away from that at this point - our reasoning being, birthdays come once a year, and since her diet is not made up of birthday cake, it is okay for her to have a piece of her own cake - whatever flavor she's requested - on her birthday.
The birthdays of her peers have begun to present some unforeseen, although unsurprising, challenges. S is in first grade now, and so she's begun to receive invites to her classmates' birthday parties. On the one hand, I am thrilled with this development, as it shows that at this point, she is able to interact appropriately with her peer group, and develop relationships with them. This will, I hope, make her years in gradeschool with these kids somewhat easier. Her extreme awareness of and obsession with food, and her occasional behavioral issues, have not proven an obstacle so far to her making friends (well, at her age I'd call it more of a "Friendship Lite" situation).
On the other hand...birthday parties are a challenge. I will be attending, with her, any of her friends' birthday parties for the foreseeable future. (Maybe I should forewarn the kid's mom that I want a treat bag too? Maybe with some G.odiva chocolates in it?) PWS is so complex that it is easier for me to just go with S, than try to warn the parent of all the food issues that might come up for S during the party. I have also recently learned that I may need to talk with the child's parent, regarding the activities during the party; S was recently invited to a birthday party which was held at a recreation center. The invite simply said the kids should wear clothes comfortable for running around in. Well, we arrive at the rec center, go up to the 2nd floor...to a gymnastics area...yep, you got it, the high bars, the balance beams, the big mats for doing lots and lots of somersaults, the whole shebang. If I had known that was the nature of the party, I would not have taken S to it.
S has never taken a gymnastics class and is not anywhere near being able to do gymnastics, owing to low muscle tone, gross motor skill challenges, and muscle/general fatigue. It was an hour of watching all these other kids bounce and hop and climb ropes to the ceiling and swing on ropes and flip around on the low bars...while S and I walked around the area, I tried not to cry (not quite successful there), and I helped her do what she was able. She still had fun, and I sucked it up as quickly as possible and we moved on. I'm sure birthday girl's dad thought I was a little wacko; I/we made a choice early in the school year not to educate S's classmates' parents as to her diagnosis (I probably will choose differently for 2nd grade) so they could not have known that S wouldn't be able to fully participate. As for my reaction to the whole situation, as I said I almost cried outright, half-way through. I know S has physical challenges owing to PWS - that has been clear from Day 1 of all this. It's just that, the difference between her and her peers in this area is not, usually, quite this glaring - and not as "public". There were other parents who had stayed for the party as well, just to be there....I'm sure they also were thinking "what the heck is wrong with that mom, and why isn't her child able to do all this stuff?". Lovely, lovely afternoon. I did let her have a piece of birthday cake.
I, however, have learned my lesson. Birthday parties have turned out to be one of those "harder than expected" things, in our journey with PWS. S brought home another birthday party invite yesterday; I will now be helping her with her inevitable, PWS-based anxiety about an event that is still two weeks in the murky (for her) future, and I'll be calling the parent to inquire as to the party activities. I am dreading the day she brings home an invite to a birthday pool party....if we can't avoid taking her to that one, my treat bag for that one better be pretty awesome!!
Thanks for reading!
Later,
Jen
One of the first issues that often arise, in the lives of families and children living with PWS, is what to do about the birthday cake for the child's first birthday.
In any other families, this is a total NON-issue, right? You just get a cake, or make one...baby smashes it or smears frosting all over their cute face (I have pictures of both my kids like this, so adorable), and life goes on.
But, when a child has PWS, the parents have been told from Day 1 of being diagnosed, that Food Is The Enemy. The child - while totally uninterested in food in the first two years at least - has a faulty hypothalamus, and so at some point will begin to experience a constant, biting, gnawing feeling of hunger. The parents' caution signals in regards to anything food-related go up immediately, of course! And then...along comes the child's First Birthday, or the first birthday post-diagnosis. For S's first few birthdays, we did do the Angel-Food cakes or the Jello desserts. We've moved away from that at this point - our reasoning being, birthdays come once a year, and since her diet is not made up of birthday cake, it is okay for her to have a piece of her own cake - whatever flavor she's requested - on her birthday.
The birthdays of her peers have begun to present some unforeseen, although unsurprising, challenges. S is in first grade now, and so she's begun to receive invites to her classmates' birthday parties. On the one hand, I am thrilled with this development, as it shows that at this point, she is able to interact appropriately with her peer group, and develop relationships with them. This will, I hope, make her years in gradeschool with these kids somewhat easier. Her extreme awareness of and obsession with food, and her occasional behavioral issues, have not proven an obstacle so far to her making friends (well, at her age I'd call it more of a "Friendship Lite" situation).
On the other hand...birthday parties are a challenge. I will be attending, with her, any of her friends' birthday parties for the foreseeable future. (Maybe I should forewarn the kid's mom that I want a treat bag too? Maybe with some G.odiva chocolates in it?) PWS is so complex that it is easier for me to just go with S, than try to warn the parent of all the food issues that might come up for S during the party. I have also recently learned that I may need to talk with the child's parent, regarding the activities during the party; S was recently invited to a birthday party which was held at a recreation center. The invite simply said the kids should wear clothes comfortable for running around in. Well, we arrive at the rec center, go up to the 2nd floor...to a gymnastics area...yep, you got it, the high bars, the balance beams, the big mats for doing lots and lots of somersaults, the whole shebang. If I had known that was the nature of the party, I would not have taken S to it.
S has never taken a gymnastics class and is not anywhere near being able to do gymnastics, owing to low muscle tone, gross motor skill challenges, and muscle/general fatigue. It was an hour of watching all these other kids bounce and hop and climb ropes to the ceiling and swing on ropes and flip around on the low bars...while S and I walked around the area, I tried not to cry (not quite successful there), and I helped her do what she was able. She still had fun, and I sucked it up as quickly as possible and we moved on. I'm sure birthday girl's dad thought I was a little wacko; I/we made a choice early in the school year not to educate S's classmates' parents as to her diagnosis (I probably will choose differently for 2nd grade) so they could not have known that S wouldn't be able to fully participate. As for my reaction to the whole situation, as I said I almost cried outright, half-way through. I know S has physical challenges owing to PWS - that has been clear from Day 1 of all this. It's just that, the difference between her and her peers in this area is not, usually, quite this glaring - and not as "public". There were other parents who had stayed for the party as well, just to be there....I'm sure they also were thinking "what the heck is wrong with that mom, and why isn't her child able to do all this stuff?". Lovely, lovely afternoon. I did let her have a piece of birthday cake.
I, however, have learned my lesson. Birthday parties have turned out to be one of those "harder than expected" things, in our journey with PWS. S brought home another birthday party invite yesterday; I will now be helping her with her inevitable, PWS-based anxiety about an event that is still two weeks in the murky (for her) future, and I'll be calling the parent to inquire as to the party activities. I am dreading the day she brings home an invite to a birthday pool party....if we can't avoid taking her to that one, my treat bag for that one better be pretty awesome!!
Thanks for reading!
Later,
Jen
Tuesday, March 22, 2011
Prader-Willi Syndrome questions, #7: What has been the biggest adjustment?
Q. What has been the biggest adjustment for your family, living with your daughter's diagnosis? What has been easier/harder than you expected?
A. I think my answers to these questions change, somewhat, as time goes by. S is only 7, so there are parts of PWS which we have not had to tackle yet! And I am trying to think in general terms here...I'm "wordy" to start with, and PWS is a complex syndrome which affects each child and family a little differently.
I think the biggest adjustment has been, simply, adjusting to the idea that S has special needs! This has changed who we are as parents, because we have an extra level of care we need to provide for S. We can't just "coast" with her - and by that I mean, we will never be able to assume much, about her future. For our older daughter, who does not have special needs, B and I can assume lots of things: that she'll be able to make friends with her peers, that she'll be able to finish high school, go to college, find a job which will support her without the help of state-run programs, move out into her own place, maintain a 'normal' relationship with boyfriend, and if she chooses, to eventually get married and have children. (And yes, I know we can all think of situations, among family/friends, where the "normal" kids in the family have not been able to do these things. But, I think generally, most parents assume their kids will do these things, and in most cases, their assumptions/expectations for their kids are fulfilled.)
S, on the other hand, will never be completely independent...I do have hope that research will discover things to help our children! But, for now, I am going on the assumption that S will always need some kind of supervision. This is neither good nor bad - it is, however, a reality to which we've had to adjust, as parents.
How we handle food issues, and how we even think about food, has been another adjustment! I think one of the most useful bits of advice to give to parents of newly-diagnosed children, is this: "start paying attention to the nutrition labels on every single bit of packaged food you buy, and buy a book which gives calorie counts for fresh foods"! The dietitian and the endocrinologist we see with S told us when we needed to really start counting her calories - and reading the nutrition labels for the calories-per-serving (among other info) was really enlightening. I was shocked to learn that there are over 100 calories per 1/4 cup of raisins...RAISINS! Do you know how often little kids eat raisins or dried fruit? I think because of the concentrated sugar, dried fruit is actually pretty high in calories. So we quickly cut that out of S's diet. We have made lots of other changes; we use Splenda although I've never been crazy about artificial sweeteners, we make good and constant use of measuring cups to make sure we're not giving S too much, things like that.
I know I have thought more about "food" in the past 7 years, than I ever expected. I have also become MUCH more aware of how ever-present food is in our society here in the states (maybe in other countries too, but I'm not well-travelled!). It is EVERYWHERE - ads about food everywhere you look - on buses, in magazines, on the radio, in the coupons we get in the junk mail. There are whole tv channels about cooking food. School fundraisers, generally, revolve around the sale of some food item. Classroom parties are, generally, junkfood-fests. Although teachers are getting away from this a bit, in many classrooms 'good' students are still rewarded with food. Want to show someone you love them? Give them a food-based gift. Halloween/Easter/Thanksgiving/Christmas? Holidays which for most people would not be quite the same without the food element. So, figuring out how to navigate through all of this, and still keep S healthy, has been a huge challenge, and will continue to be one.
I could go on, but I'll stop here. Thanks for reading!
Later,
Jen
A. I think my answers to these questions change, somewhat, as time goes by. S is only 7, so there are parts of PWS which we have not had to tackle yet! And I am trying to think in general terms here...I'm "wordy" to start with, and PWS is a complex syndrome which affects each child and family a little differently.
I think the biggest adjustment has been, simply, adjusting to the idea that S has special needs! This has changed who we are as parents, because we have an extra level of care we need to provide for S. We can't just "coast" with her - and by that I mean, we will never be able to assume much, about her future. For our older daughter, who does not have special needs, B and I can assume lots of things: that she'll be able to make friends with her peers, that she'll be able to finish high school, go to college, find a job which will support her without the help of state-run programs, move out into her own place, maintain a 'normal' relationship with boyfriend, and if she chooses, to eventually get married and have children. (And yes, I know we can all think of situations, among family/friends, where the "normal" kids in the family have not been able to do these things. But, I think generally, most parents assume their kids will do these things, and in most cases, their assumptions/expectations for their kids are fulfilled.)
S, on the other hand, will never be completely independent...I do have hope that research will discover things to help our children! But, for now, I am going on the assumption that S will always need some kind of supervision. This is neither good nor bad - it is, however, a reality to which we've had to adjust, as parents.
How we handle food issues, and how we even think about food, has been another adjustment! I think one of the most useful bits of advice to give to parents of newly-diagnosed children, is this: "start paying attention to the nutrition labels on every single bit of packaged food you buy, and buy a book which gives calorie counts for fresh foods"! The dietitian and the endocrinologist we see with S told us when we needed to really start counting her calories - and reading the nutrition labels for the calories-per-serving (among other info) was really enlightening. I was shocked to learn that there are over 100 calories per 1/4 cup of raisins...RAISINS! Do you know how often little kids eat raisins or dried fruit? I think because of the concentrated sugar, dried fruit is actually pretty high in calories. So we quickly cut that out of S's diet. We have made lots of other changes; we use Splenda although I've never been crazy about artificial sweeteners, we make good and constant use of measuring cups to make sure we're not giving S too much, things like that.
I know I have thought more about "food" in the past 7 years, than I ever expected. I have also become MUCH more aware of how ever-present food is in our society here in the states (maybe in other countries too, but I'm not well-travelled!). It is EVERYWHERE - ads about food everywhere you look - on buses, in magazines, on the radio, in the coupons we get in the junk mail. There are whole tv channels about cooking food. School fundraisers, generally, revolve around the sale of some food item. Classroom parties are, generally, junkfood-fests. Although teachers are getting away from this a bit, in many classrooms 'good' students are still rewarded with food. Want to show someone you love them? Give them a food-based gift. Halloween/Easter/Thanksgiving/Christmas? Holidays which for most people would not be quite the same without the food element. So, figuring out how to navigate through all of this, and still keep S healthy, has been a huge challenge, and will continue to be one.
I could go on, but I'll stop here. Thanks for reading!
Later,
Jen
Saturday, March 12, 2011
Prader-Willi Syndrome questions, 6A - How to help
[Further thoughts on the answer to this question, originally posted here.]
Q. My grandchild/niece/nephew/friend's child has just been diagnosed with PWS. How best can I be of help to them??
In addition to learning about the syndrome and respecting the decisions the parents make in the care of their child with PWS, there are some additional ways to help the individual with PWS and their immediate family:
-Keep your communication simple - at least until you get to know the child/adult better, and know at what cognitive level he/she functions.
-Approach the child/adult with "subdued emotion". PWS is a syndrome which is on the autism spectrum; actually, individuals with PWS are often also diagnosed with autism. What this means in terms of "subdued emotion" is that individuals with PWS are often uncomfortable with excessive emotion. It is as if that level of feeling - happy or sad - is more than they can handle.
-Don't take it personally when the individual with PWS responds in a grumpy/mean/hostile way, to something you've asked or said. Being able to regulate emotions is something which is managed by the hypothalamus, and since that gland is the one affected by PWS, emotions can swing between calm and happy, to upset and angry, over seemingly small things.
Finally, just a few words of advice on what not to say to parents of a child with PWS:
Later,
Jen
Q. My grandchild/niece/nephew/friend's child has just been diagnosed with PWS. How best can I be of help to them??
In addition to learning about the syndrome and respecting the decisions the parents make in the care of their child with PWS, there are some additional ways to help the individual with PWS and their immediate family:
- Because children with PWS are at their best when they live in an environment of routine and structure, be consistent when you spend time with that child (or adult!) with PWS. If you say you are going to do a certain activity with them, then do that activity with them. Do not ever, ever, ever mention something in passing to that child, if you have even the slightest doubt about being able to act on it. Also, once the child knows about an upcoming activity, they will often become obsessive about it. So, if at all possible, don't talk about events or activities with the child, which are happening any further into the future than that day or possibly the next day!
- Parents of children with PWS often find that attending family parties or holiday gatherings are extremely stressful situations - because these events so often center around a huge meal or a big table packed with food! Obviously this creates not just an anxious situation for the child with PWS (if you felt you were starving, constantly, wouldn't you feel overwrought at such a display?), but a dangerous one as well. Children with PWS cannot sense when their stomach is full-to-bursting, and so when well-meaning relatives keep slipping the child "a little of this" and "a little of that"...well, pretty soon that child will need to be rushed to the emergency room. Thus, if a parent of a child with PWS tells you that in order for their family to be able to also enjoy a family party or a holiday gathering, some adjustments to the menu and the eating schedule would be necessary, listen to them. Yes, it may mean that some eating traditions will have to change...so what? You can make new ones, right? And, what is more important: being able to see your grandchild/niece/nephew/family friends, or having a table overstuffed with way more food than anyone should eat?
-Keep your communication simple - at least until you get to know the child/adult better, and know at what cognitive level he/she functions.
-Approach the child/adult with "subdued emotion". PWS is a syndrome which is on the autism spectrum; actually, individuals with PWS are often also diagnosed with autism. What this means in terms of "subdued emotion" is that individuals with PWS are often uncomfortable with excessive emotion. It is as if that level of feeling - happy or sad - is more than they can handle.
-Don't take it personally when the individual with PWS responds in a grumpy/mean/hostile way, to something you've asked or said. Being able to regulate emotions is something which is managed by the hypothalamus, and since that gland is the one affected by PWS, emotions can swing between calm and happy, to upset and angry, over seemingly small things.
Finally, just a few words of advice on what not to say to parents of a child with PWS:
- "She'll grow out of it!" Ummm, no, she/he won't grow out of it. It's a genetic syndrome, it's part of him/her forever.
- "Hmmmm, he/she is always hungry and asking for food? Wow, maybe that's what my dog has!" Yes, indeed, this is a comment that is made.
- "You worry about (fill in the blank), with your child with PWS?? Oh, that's nothing - all parents worry about their children!" Really? Do you worry about your child ever having real friends, once her challenges become obvious to her peers? Do you worry about your child eating out of a trash can at school? Do you have to lock your refrigerator and cupboards, because your child will get up in the middle of the night and possibly have a fatal eating episode? I have found it exceedingly frustrating when other parents say "oh, my parenting experience with my non-diagnosis children is just like your experience with S!", because in essence what they are saying is that they don't think my experiences are real, that I don't have any extraordinary challenges on a daily basis. And while I know people sometimes say things in an effort to make me "feel better", or in an effort to seem understanding, honestly I can say that most parents of special-needs-children simply want to be heard. They want their experiences to be validated and accepted for the very real things they are. We aren't kidding, folks. PWS sucks, and this is hard. It IS an out-of-the-ordinary life. My child's constant hunger is NOT like your "normal" toddler asking for a snack a few times a day: for S this is HER LIFE, for your toddler it is A PHASE.
Later,
Jen
Friday, February 25, 2011
Prader-Willi Syndrome Questions, #6: What can I do to help my newly-diagnosed relative/family friend?
Q. My grandchild/niece/nephew/friend's child has just been diagnosed with PWS. How best can I be of help to them??
A. This is an excellent question, as obviously it is more than just the child, and the child's immediate family, who will be affected - both short-term and long-term - by the Prader-Willi Syndrome diagnosis. [As a side note - to any of my own family who might be reading this...rest assured you have been very supportive of our routines with S! If the families and friends of all PWS families could be supportive as you have been, that would be great!!]
The first thing that those involved in the child's life should do, is learn about the syndrome. The best way to do this is to visit http://www.pwsausa.org/, which is the website for PWSA(USA), the national Prader-Willi Syndrome organization in the United States. There is also an international organization, IPWSO, which also has a website, http://www.ipwso.org/. Both of these organizations are well-equipped to not only provide immediate and crisis-based assistance, but they are a great place to start in terms of connecting with local/state organizations, which will then put you in contact with families or parent mentors near you, if you wish. I strongly encourage parents, caregivers, and all extended family members of the individual with PWS to connect with these organizations and local PWS organizations, and stay connected permanently by maintaining membership. The support and advice gained from connecting with other families is, in my opinion, crucial to providing the child-with-PWS and his/her caregivers with much needed, and appropriate, support.
The second thing you should do, as you learn about the syndrome, is take everything you learn about it with a nice helping of objectivity! PWS is very much a "spectrum" syndrome, which means that descriptions of its symptoms are of necessity very comprehensive and will cover every last medical/behavioral/psychological observation of individuals with PWS, if they happen in enough cases. This does not mean that every single one of those will happen with the child you know. Those comprehensive descriptions should be used as education, not as prediction.
The third thing you should plant in your brain, as you are supportive of the parents of the newly-diagnosed individual, is that THE PARENTS ARE THE EXPERTS ABOUT THEIR CHILD.
Let me repeat this, because it is important: THE PARENTS ARE THE EXPERTS ABOUT THEIR CHILD. They are the people living with the syndrome, trying to raise the child with PWS, day-in and day-out. If you are spending time with the family, you must absolutely respect the policies and procedures, systems and routines that the parents have put in place for their child with PWS. Raising a child with this syndrome is, without a doubt, going to look different than perhaps what you did with your children - and that is okay, it is necessary for the health and safety of the child with PWS for the parent/caregivers to do exactly what they are doing. If you have concerns, ask the parents in a respectful way, for the purposes of educating yourself but NOT with the goal of changing what the parents are doing. Further, if the parents/caregivers of the child with PWS have gotten to the phase where their child is on a restricted-calorie diet, and every calorie is counted, DO NOT feed that child anything without first asking the parent/caregiver. This means, do not even feed the child a cookie...or a juice box...or even a stick of gum. Nothing. Calories are calories are calories, and when a parent is counting calories for their child with PWS, your action of giving that child food/drink of any kind is an act of disrespect to what that parent is trying to do for their child!
I think there is more I can say in answer to this question - but I'll stop here for now.
Later,
Jen
A. This is an excellent question, as obviously it is more than just the child, and the child's immediate family, who will be affected - both short-term and long-term - by the Prader-Willi Syndrome diagnosis. [As a side note - to any of my own family who might be reading this...rest assured you have been very supportive of our routines with S! If the families and friends of all PWS families could be supportive as you have been, that would be great!!]
The first thing that those involved in the child's life should do, is learn about the syndrome. The best way to do this is to visit http://www.pwsausa.org/, which is the website for PWSA(USA), the national Prader-Willi Syndrome organization in the United States. There is also an international organization, IPWSO, which also has a website, http://www.ipwso.org/. Both of these organizations are well-equipped to not only provide immediate and crisis-based assistance, but they are a great place to start in terms of connecting with local/state organizations, which will then put you in contact with families or parent mentors near you, if you wish. I strongly encourage parents, caregivers, and all extended family members of the individual with PWS to connect with these organizations and local PWS organizations, and stay connected permanently by maintaining membership. The support and advice gained from connecting with other families is, in my opinion, crucial to providing the child-with-PWS and his/her caregivers with much needed, and appropriate, support.
The second thing you should do, as you learn about the syndrome, is take everything you learn about it with a nice helping of objectivity! PWS is very much a "spectrum" syndrome, which means that descriptions of its symptoms are of necessity very comprehensive and will cover every last medical/behavioral/psychological observation of individuals with PWS, if they happen in enough cases. This does not mean that every single one of those will happen with the child you know. Those comprehensive descriptions should be used as education, not as prediction.
The third thing you should plant in your brain, as you are supportive of the parents of the newly-diagnosed individual, is that THE PARENTS ARE THE EXPERTS ABOUT THEIR CHILD.
Let me repeat this, because it is important: THE PARENTS ARE THE EXPERTS ABOUT THEIR CHILD. They are the people living with the syndrome, trying to raise the child with PWS, day-in and day-out. If you are spending time with the family, you must absolutely respect the policies and procedures, systems and routines that the parents have put in place for their child with PWS. Raising a child with this syndrome is, without a doubt, going to look different than perhaps what you did with your children - and that is okay, it is necessary for the health and safety of the child with PWS for the parent/caregivers to do exactly what they are doing. If you have concerns, ask the parents in a respectful way, for the purposes of educating yourself but NOT with the goal of changing what the parents are doing. Further, if the parents/caregivers of the child with PWS have gotten to the phase where their child is on a restricted-calorie diet, and every calorie is counted, DO NOT feed that child anything without first asking the parent/caregiver. This means, do not even feed the child a cookie...or a juice box...or even a stick of gum. Nothing. Calories are calories are calories, and when a parent is counting calories for their child with PWS, your action of giving that child food/drink of any kind is an act of disrespect to what that parent is trying to do for their child!
I think there is more I can say in answer to this question - but I'll stop here for now.
Later,
Jen
Saturday, February 19, 2011
When I exhale
Do you remember that movie, I think it came out in the mid-90's perhaps, it was called "Waiting to Exhale"? I liked the movie, but the thing that has really stuck with me all these years is not the movie's subject but its title.
"Waiting to exhale". What does that phrase mean? I think it means that moment when circumstances in one's life finally ease up - there's money in the bank, or one's body is finally healthy again, or a troubling relationship is either resolved or in permanent, soul-easing disconnect....that moment when the weight which has been hanging over your head like a 9,000 pound anvil, keeping you constantly on edge, finally disappears. And, having been taking short, panicky breaths for hours/days/years, you finally feel like you can fully breathe in...and fully, totally, exhale. The stress melts away from your neck and shoulders, your mind empties for a moment, maybe even you sit down and just sit, and think about absolutely nothing...because God knows your thoughts have been running a mile-a-minute for what feels like eternity.
Do you know when I exhale, on a day-to-day basis?
When my child-with-special-needs is either in the competent care of someone I trust, or when she is finally in bed. When I finally, after a long and emotionally draining day, have a moment to myself to just sit, just think about nothing, just be.
I have to imagine that many parents who have children with special needs may have this same brand of "exhale moments". This is especially true when the child's diagnosis-based behavior issues are becoming or have been severe. Moments like these, when the primary caregiver can "exhale" even for an hour, are also called "respite". Not every parent of a special-needs-child is able to obtain respite, and some who can easily get it, through social services of one kind or another, may even decide they don't need it. But, bluntly speaking, most special-needs parents probably do need respite assistance, and they should always, always use it.
And with all due respect to parents everywhere, please do not tell me that parenting my special-needs child is just like parenting any "normal" child - it isn't. I have one of each - a child with no diagnosis or special need of any kind, and one with Prader-Willi Syndrome. My "normal" child is the older of the two - by 4.5 years - so I was very well aware of what parenting a "normal" child looked like, before S came along. I know for a fact, from second-by-second experience, how very much different it is to be a good parent to S compared to my first 4.5 years of parenting. While I do breathe a bit easier when my older child is finally in bed, it is in NO WAY the same kind of "exhale" I experience once Sophie is finally in bed. Parenting S is a daily emotional roller-coaster. I am almost literally on the edge of my seat, for all the minutes and hours she is at home and awake, not knowing what is going to happen with her next. Is she going to have a planet-sized meltdown about food, about something, about anything? Is she going to go use the bathroom, and end up sitting there picking at her skin if I let her sit too long? Is she going to spend the remainder of the day being exceedingly nasty to me - just because that is part of the PWS behavior issues and for no other reason? She is 7 now, so this is no longer about "it's just a phase" or "it's the terrible twos" or "it's the terrible threes". No, this is part of Prader-Willi Syndrome. Her tantrums can be epic,.they can poison the atmosphere of the entire house (although she seemingly recovers with no residual effects). Her PWS-based behavior issues can be extremely unpleasant to be around, and that is not going to change without medication, I believe. I have been in contact with enough parents who have (even just slightly) older children with PWS, to know that these behaviors are part of the syndrome. So they are here to stay, it's not a phase - or, even if it lessens somewhat, I'm aware that changes in the body or changes in routine or changes in circumstance can bring it on again. It is probably a bit like living with someone who, at the drop of a hat and for no obvious reason, turns on you and becomes verbally abusive. S can and does turn on me, the syndrome causes her emotions to swing from very loving to just plain mean - and she's only 7. I am her primary caregiver, I am with her more than anybody else including her father. I am on the recieving end of whatever mood she's got going, and while I love her and know she's a gift from God, this drains me. Although I expect that I will outlive her, I have many years ahead of me of trying to figure her out, trying to anticipate/head off if I can, the things that set her off.
I have many days ahead, of waiting to exhale.
Later,
Jen
"Waiting to exhale". What does that phrase mean? I think it means that moment when circumstances in one's life finally ease up - there's money in the bank, or one's body is finally healthy again, or a troubling relationship is either resolved or in permanent, soul-easing disconnect....that moment when the weight which has been hanging over your head like a 9,000 pound anvil, keeping you constantly on edge, finally disappears. And, having been taking short, panicky breaths for hours/days/years, you finally feel like you can fully breathe in...and fully, totally, exhale. The stress melts away from your neck and shoulders, your mind empties for a moment, maybe even you sit down and just sit, and think about absolutely nothing...because God knows your thoughts have been running a mile-a-minute for what feels like eternity.
Do you know when I exhale, on a day-to-day basis?
When my child-with-special-needs is either in the competent care of someone I trust, or when she is finally in bed. When I finally, after a long and emotionally draining day, have a moment to myself to just sit, just think about nothing, just be.
I have to imagine that many parents who have children with special needs may have this same brand of "exhale moments". This is especially true when the child's diagnosis-based behavior issues are becoming or have been severe. Moments like these, when the primary caregiver can "exhale" even for an hour, are also called "respite". Not every parent of a special-needs-child is able to obtain respite, and some who can easily get it, through social services of one kind or another, may even decide they don't need it. But, bluntly speaking, most special-needs parents probably do need respite assistance, and they should always, always use it.
And with all due respect to parents everywhere, please do not tell me that parenting my special-needs child is just like parenting any "normal" child - it isn't. I have one of each - a child with no diagnosis or special need of any kind, and one with Prader-Willi Syndrome. My "normal" child is the older of the two - by 4.5 years - so I was very well aware of what parenting a "normal" child looked like, before S came along. I know for a fact, from second-by-second experience, how very much different it is to be a good parent to S compared to my first 4.5 years of parenting. While I do breathe a bit easier when my older child is finally in bed, it is in NO WAY the same kind of "exhale" I experience once Sophie is finally in bed. Parenting S is a daily emotional roller-coaster. I am almost literally on the edge of my seat, for all the minutes and hours she is at home and awake, not knowing what is going to happen with her next. Is she going to have a planet-sized meltdown about food, about something, about anything? Is she going to go use the bathroom, and end up sitting there picking at her skin if I let her sit too long? Is she going to spend the remainder of the day being exceedingly nasty to me - just because that is part of the PWS behavior issues and for no other reason? She is 7 now, so this is no longer about "it's just a phase" or "it's the terrible twos" or "it's the terrible threes". No, this is part of Prader-Willi Syndrome. Her tantrums can be epic,.they can poison the atmosphere of the entire house (although she seemingly recovers with no residual effects). Her PWS-based behavior issues can be extremely unpleasant to be around, and that is not going to change without medication, I believe. I have been in contact with enough parents who have (even just slightly) older children with PWS, to know that these behaviors are part of the syndrome. So they are here to stay, it's not a phase - or, even if it lessens somewhat, I'm aware that changes in the body or changes in routine or changes in circumstance can bring it on again. It is probably a bit like living with someone who, at the drop of a hat and for no obvious reason, turns on you and becomes verbally abusive. S can and does turn on me, the syndrome causes her emotions to swing from very loving to just plain mean - and she's only 7. I am her primary caregiver, I am with her more than anybody else including her father. I am on the recieving end of whatever mood she's got going, and while I love her and know she's a gift from God, this drains me. Although I expect that I will outlive her, I have many years ahead of me of trying to figure her out, trying to anticipate/head off if I can, the things that set her off.
I have many days ahead, of waiting to exhale.
Later,
Jen
Tuesday, February 15, 2011
Prader-Willi Syndrome Question 5: How do you structure meals and snacks?
Q. How do you structure your daughter's meals and snacks - do you keep her on a schedule?
A. Yes, S is very much on a schedule. She eats about every two hours or so, except for the hours between dinner and bedtime. She has three meals a day and two snacks.
In order to be successful at managing the PWS lifestyle for S, we had to do this. There could no longer be a pattern of just open snacking all day long - not for us, and not for her sister, either. K was only 4.5 years old when S was born, but by the time S turned 2, we were on a schedule. We all eat at the same times - if S can't eat, then no-one eats. It may sound harsh - and believe me, there are many times when I regret having skipped a meal, because I am of course feeling incredibly hungry but I know it would be a mistake to eat before S can eat too - but this has worked very well for us, and it is a system which I recommend to any other PWS families I have opportunity to speak with, especially if there are other children in the family. The child-with-PWS needs to have it made clear, as early on as possible, that they cannot eat or snack all day long - this is so important to teach them, because of course they will be getting that hunger signal constantly. I have tried to explain to S that the signal is just a mistake - that it's Prader-Willi Syndrome tricking her into thinking she's hungry. I've even tried to explain to her that her stomach still has food in it from the last time she ate, that her stomach needs at least two hours to digest before putting more food in...at this point she is too young to really get these explanations. She may not be able to really understand PWS until she's much older, and perhaps not even then, so trying to explain it to her may or may not help.
So, the very best thing parents of children with PWS can do, is to get on a schedule for eating and snacking. That way, the clock becomes their ally - when their child begins to ask about eating and becomes anxious (as I would too, if I felt like I were starving and were worried about obtaining more food), the parent/caregiver can point to the clock and say, we will eat again in _____________minutes. I used to use the timer on the microwave or oven, because setting the timer for the next time S could have something to eat seemed to help her think about something else for a while. It was as if the timer was then doing the worrying for her, and she could do something else and just wait for the beep. She now knows how to tell time - plus we have chiming clocks in the house - so she doesn't need the timers as much anymore. Getting on a schedule of eating every two hours or so also helps to keep the blood sugar levels constant - so that whatever intensity of hunger S is feeling isn't made more extreme by a huge dip in blood sugar levels. There has to be structure to the eating schedule for kids with PWS, because food is, ultimately, everything to them. Yes, they do have hobbies, and can do some of the things other kids can do - but as a parent I do not ever, ever mess around when it comes to food with S. I have learned to be prepared, to take snack with me if necessary, to plan outings/errands around the time between meals and snacks, because I understand what part food plays in S's daily life. It's not always fun, and I've spent more time thinking about "food" in the past 7 years than I ever have before, but thus far it has been manageable, thank God.
Later (and thanks Rachel for the question!),
Jen
A. Yes, S is very much on a schedule. She eats about every two hours or so, except for the hours between dinner and bedtime. She has three meals a day and two snacks.
In order to be successful at managing the PWS lifestyle for S, we had to do this. There could no longer be a pattern of just open snacking all day long - not for us, and not for her sister, either. K was only 4.5 years old when S was born, but by the time S turned 2, we were on a schedule. We all eat at the same times - if S can't eat, then no-one eats. It may sound harsh - and believe me, there are many times when I regret having skipped a meal, because I am of course feeling incredibly hungry but I know it would be a mistake to eat before S can eat too - but this has worked very well for us, and it is a system which I recommend to any other PWS families I have opportunity to speak with, especially if there are other children in the family. The child-with-PWS needs to have it made clear, as early on as possible, that they cannot eat or snack all day long - this is so important to teach them, because of course they will be getting that hunger signal constantly. I have tried to explain to S that the signal is just a mistake - that it's Prader-Willi Syndrome tricking her into thinking she's hungry. I've even tried to explain to her that her stomach still has food in it from the last time she ate, that her stomach needs at least two hours to digest before putting more food in...at this point she is too young to really get these explanations. She may not be able to really understand PWS until she's much older, and perhaps not even then, so trying to explain it to her may or may not help.
So, the very best thing parents of children with PWS can do, is to get on a schedule for eating and snacking. That way, the clock becomes their ally - when their child begins to ask about eating and becomes anxious (as I would too, if I felt like I were starving and were worried about obtaining more food), the parent/caregiver can point to the clock and say, we will eat again in _____________minutes. I used to use the timer on the microwave or oven, because setting the timer for the next time S could have something to eat seemed to help her think about something else for a while. It was as if the timer was then doing the worrying for her, and she could do something else and just wait for the beep. She now knows how to tell time - plus we have chiming clocks in the house - so she doesn't need the timers as much anymore. Getting on a schedule of eating every two hours or so also helps to keep the blood sugar levels constant - so that whatever intensity of hunger S is feeling isn't made more extreme by a huge dip in blood sugar levels. There has to be structure to the eating schedule for kids with PWS, because food is, ultimately, everything to them. Yes, they do have hobbies, and can do some of the things other kids can do - but as a parent I do not ever, ever mess around when it comes to food with S. I have learned to be prepared, to take snack with me if necessary, to plan outings/errands around the time between meals and snacks, because I understand what part food plays in S's daily life. It's not always fun, and I've spent more time thinking about "food" in the past 7 years than I ever have before, but thus far it has been manageable, thank God.
Later (and thanks Rachel for the question!),
Jen
Wednesday, February 9, 2011
Prayer warriors needed!!!! Please pray for EllyAnnah
Hello everyone,
PLEASE PRAY for baby EllyAnnah, and infant with Prader-Willi Syndrome. She was born 8 week premature in late July 2010, spent about 9 weeks in the NICU and was finally able to go home in late September. Due to illness Elly was rushed back to the hospital on December 7, was discharged Dec 18, was rushed back on Dec 30, and is still there. In recent days her condition has taken a turn for the worse, as she has had high fevers, low blood-oxygen levels, and high respirations-per-minute numbers. [Update: She has spiked a fever again today 2/10] The right ventricle in her heart is now having trouble because Elly has decreased ability to expel CO2. She is battling pneumonia and a staph infection. Her progress is slowed due to complications of the syndrome. Contracting pneumonia is especially dangerous for our children with the syndrome. Please keep EllyAnnah, her mother, and all their family in your prayers. Please keep all individuals with Prader-Willi Syndrome in your prayers...as I've said before, the syndrome is extremely challenging and, while the general public may not know much about it (yet), our kids still need your much-appreciated support and praryers.
Thank you,
Jen
PLEASE PRAY for baby EllyAnnah, and infant with Prader-Willi Syndrome. She was born 8 week premature in late July 2010, spent about 9 weeks in the NICU and was finally able to go home in late September. Due to illness Elly was rushed back to the hospital on December 7, was discharged Dec 18, was rushed back on Dec 30, and is still there. In recent days her condition has taken a turn for the worse, as she has had high fevers, low blood-oxygen levels, and high respirations-per-minute numbers. [Update: She has spiked a fever again today 2/10] The right ventricle in her heart is now having trouble because Elly has decreased ability to expel CO2. She is battling pneumonia and a staph infection. Her progress is slowed due to complications of the syndrome. Contracting pneumonia is especially dangerous for our children with the syndrome. Please keep EllyAnnah, her mother, and all their family in your prayers. Please keep all individuals with Prader-Willi Syndrome in your prayers...as I've said before, the syndrome is extremely challenging and, while the general public may not know much about it (yet), our kids still need your much-appreciated support and praryers.
Thank you,
Jen
Tuesday, February 8, 2011
Prader-Willi Syndrome Question 4: Is there a cure for Prader-Willi Syndrome?
Q. Is there a cure for Prader-Willi Syndrome?
A. No, there currently is no cure for PWS. Since this syndrome is genetically-based, any kind of "cure" will involve altering the pathways of many different signals sent from the hypothalamus to the rest of the body. Finding a cure for PWS involves lots and lots and lots and lots of research! The tough part about that, is that because the PWS community itself is not large, pharmaceutical companies (who would stand to make the most money from any drug-based therapies seen as a 'cure' for PWS) would not initially be making a ton of money - and so they aren't chomping at the bit to fund PWS research, unfortunately. That is why fundraising efforts involving the PWS community are SO important, because compared to the amount of funding given to research efforts and quality-of-life supports for something like cancer, PWS is woefully UNDER-FUNDED. This is very frustrating to parents and caregivers of individuals who struggle with PWS - we'd like a cure too. Quality-of-life is just as much an issue for my child, even though she's not undergoing chemo or radiation or losing all her hair!
Sometimes people have asked whether appetite suppressants, or surgeries like bariatric surgery, are of any help for people with PWS - as these would seem to address the extreme, constant, deadly hunger sensations which are such a threat to those with PWS. Unfortunately, appetite suppressants and surgeries have proven unsuccessful with, and actually very dangerous for, people with PWS. You see, PWS is not really about appetite, or the size of the stomach. It involves an erroneous signal being consistently sent from the hypothalamus to the hunger centers in the brain, and the signal is constantly giving the "you're hungry, you're hungry" message. The message keeps coming even when the person's stomach is full to the brim with food. Appetite suppressants don't turn off that signal; and surgeries which alter the stomach's size definitely do not turn off that signal.
How do I personally feel, as a parent of a child with PWS, about there someday being a "cure"? Well....of course I would love for there to be some kind of drug that S could take, or safe surgical procedure that could be done, which could relieve her of even half of what she goes through every day. I would especially love it if some smart researcher could formulate a drug which could turn off that hunger signal...that, for S, is the hardest part of this. No surprise there - of course it is hard for her to think about much else, when her brain is telling her she is starving, every second of every day! But I am also realistic about the "cure" idea. PWS is genetic, which means it is embedded in every single cell of her body. Whatever might be found to help the PWS community will involve some pretty complex research and, at this rate of funding, is still some years down the road. So I do have hope for some kind of cure, but I know that we have to wait...hopefully something will be discovered in S's lifetime.
While much, much more research is needed, I am encouraged to know that there is research currently being done on PWS, how it happens, how the genes in question work exactly, what role the hypothalamus plays in satiety and obesity, and what might be done to address the challenges caused by PWS. The general population should be excited by PWS research as well, because if some of these questions about the "mechanics" of PWS can be answered, then questions about obesity itself will hopefully get answered as well. If we can better understand why we eat what we eat, I think we may all be better off.
For more information about Prader-Willi Syndrome, and to donate towards research and quality-of-life resources, visit http://www.pwsausa.org/ OR http://www.pwsaohio.org/.
Later,
Jen
A. No, there currently is no cure for PWS. Since this syndrome is genetically-based, any kind of "cure" will involve altering the pathways of many different signals sent from the hypothalamus to the rest of the body. Finding a cure for PWS involves lots and lots and lots and lots of research! The tough part about that, is that because the PWS community itself is not large, pharmaceutical companies (who would stand to make the most money from any drug-based therapies seen as a 'cure' for PWS) would not initially be making a ton of money - and so they aren't chomping at the bit to fund PWS research, unfortunately. That is why fundraising efforts involving the PWS community are SO important, because compared to the amount of funding given to research efforts and quality-of-life supports for something like cancer, PWS is woefully UNDER-FUNDED. This is very frustrating to parents and caregivers of individuals who struggle with PWS - we'd like a cure too. Quality-of-life is just as much an issue for my child, even though she's not undergoing chemo or radiation or losing all her hair!
Sometimes people have asked whether appetite suppressants, or surgeries like bariatric surgery, are of any help for people with PWS - as these would seem to address the extreme, constant, deadly hunger sensations which are such a threat to those with PWS. Unfortunately, appetite suppressants and surgeries have proven unsuccessful with, and actually very dangerous for, people with PWS. You see, PWS is not really about appetite, or the size of the stomach. It involves an erroneous signal being consistently sent from the hypothalamus to the hunger centers in the brain, and the signal is constantly giving the "you're hungry, you're hungry" message. The message keeps coming even when the person's stomach is full to the brim with food. Appetite suppressants don't turn off that signal; and surgeries which alter the stomach's size definitely do not turn off that signal.
How do I personally feel, as a parent of a child with PWS, about there someday being a "cure"? Well....of course I would love for there to be some kind of drug that S could take, or safe surgical procedure that could be done, which could relieve her of even half of what she goes through every day. I would especially love it if some smart researcher could formulate a drug which could turn off that hunger signal...that, for S, is the hardest part of this. No surprise there - of course it is hard for her to think about much else, when her brain is telling her she is starving, every second of every day! But I am also realistic about the "cure" idea. PWS is genetic, which means it is embedded in every single cell of her body. Whatever might be found to help the PWS community will involve some pretty complex research and, at this rate of funding, is still some years down the road. So I do have hope for some kind of cure, but I know that we have to wait...hopefully something will be discovered in S's lifetime.
While much, much more research is needed, I am encouraged to know that there is research currently being done on PWS, how it happens, how the genes in question work exactly, what role the hypothalamus plays in satiety and obesity, and what might be done to address the challenges caused by PWS. The general population should be excited by PWS research as well, because if some of these questions about the "mechanics" of PWS can be answered, then questions about obesity itself will hopefully get answered as well. If we can better understand why we eat what we eat, I think we may all be better off.
For more information about Prader-Willi Syndrome, and to donate towards research and quality-of-life resources, visit http://www.pwsausa.org/ OR http://www.pwsaohio.org/.
Later,
Jen
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