Tuesday, May 31, 2011

Adele - Rolling In The Deep

Just because I really, really, really love this song, and the video.  Enjoy!


Friday, May 20, 2011

5-Question Friday!

1. Do you and your spouse go to bed at the same time?
Nope!  The Hubster sleeps with a CPAP now, but he didn't for a long time, and well, snoring noises don't exactly lull me to sleep.  These days, either he goes to bed first because he has to be up by 5:30 a.m. to get ready for work...or, he falls asleep watching t.v., and I fall asleep reading in the other room...and then eventually we both end up going to bed. 

2. A question for the ladies...What kind of facial hair do you like on your man?
B has had a very small beard and a moustache for a long time now, and that is fine with me.  I think if he shaved it off now he'd look weird. And since I'm a long-time married woman I don't look at facial hair on other guys!

3. What's the worst vacation you've ever taken?
Hmmm.  I have not taken many vacations.  There have a been a few vacations which, looking back, were not very enjoyable.  B and I went to Washington D.C. some years ago, b.k. (before kids), and while there are a few parts of that trip I remember in a positive light, for the most part I just remember endless walking, being very hot, and thus very thirsty for most of that trip.  He really, really, really wants to take the kids to Washington D.C., he thinks they would find it "very interesting".  I think it would be he**.....what eleven-year-old would ever find endless walking to multiple historical buildings "very interesting"????  Every time B mentions taking a trip to D.C. I just keep quiet and look preoccupied with something out the window.

4. What's the first-ever blog you followed?
Well, the first blog I ever read consistently was "Confessions of a CF Husband", authored by Nathan Lawrenson.  I learned about his blog from a church friend, and I started reading that blog just as N. Lawrenson's wife Tricia, who has cystic fibrosis, was going into the hospital and ended up having their daughter Gwyneth very, very prematurely.  I think that was in late 2007, early 2008.  I think in 2008 Tricia was re-listed for lung transplant, and did eventually get a new set of lungs....amazing story, just really inspiring.  Their daughter is a doll, and the blog overall is excellent.  As far as the first blog where I actually clicked on that "follow" button...hmm.  That I don't remember.  I've followed a lot of blogs in the past few months!  Sometimes I become a regular reader of a blog and then forget to actually "follow" it. 

5. Do you enjoy amusement parks?
Um, no.  Not really.  I don't enjoy walking around in the sun on really hot days, and usually those are the kinds of days where one would want to go to an amusement park.  I also am not big on crowds, and I don't do roller coasters (if I want to plummet to earth and defy gravity, I'd rather jump out of an airplane - not be strapped into a piece of metal or wood where the person next to me might throw up on me.).  I went to a park with some friends during high school, and they went on the biggest coaster at that park at the time.  I stayed on the ground and counted how many seconds it took the cars to get to the top of the first hill...far too much suspense for me.  I know, I'm boring!

Have a great weekend,

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-

Tuesday, May 17, 2011

Trivial Tuesday!

Just some trivial bits and bobs for your blog-reading amusement!  Wouldn't want you to check in here and be bored or anything.

1. In Shakespeare's time, mattresses were secured on bed frames by ropes...when you pulled on the ropes the mattress tightened, making the bed firmer to sleep on. That's where the phrase, "good night, sleep tight" came from.

2. The term "the whole 9 yards" came from W.W.II fighter pilots in the Pacific. When arming their airplanes on the ground, the .50 caliber machine gun ammo belts measured exactly 27 feet, before being loaded into the fuselage. If the pilots fired all their ammo at a target, it got "the whole 9 yards."

3. The name Jeep came from the abbreviation used in the army for the "General Purpose" vehicle, GP

4. The first toilet ever seen on television was on "Leave It To Beaver."

5. Thirty-five percent of the people who use personal ads for dating are already married.

6. The world's termites outweigh the world's humans 10 to 1.

7. On average, 100 people choke to death on ball point pens every year, so be careful.

8. One of the longest one-syllable words in the English language is screeched. (Strengths is another one.)

9. No word in the English language rhymes with month, orange, silver or purple.

10. "Dreamt" is the only English word that ends in the letters "mt".

Note: I found these trivial facts at the website http://www.dan.hersam.com/.  I don't know him, but you'd be surprised how nearly impossible it is to find website with, simply, lists of facts.  The world wide web is THE biggest collection of millions and millions of pieces of information...and yet, there are very few websites that just list facts about 'stuff'.  I guess I'll just start reading encyclopedias - you know, those collections of books we all turned to for information before the internet came along?  :-)


Sunday, May 15, 2011

the "Versatile Blog" Award!

I just have to thank my blog friend Rachel over at "Picture This" (http://www.rachelpicturethis.com/)- not just for this award but for being patient w/me until I FINALLY got it onto my blog here!!  Weeks and weeks and weeks and weeks ago (well, okay, maybe not THAT far back but it has definitely been at least three weeks) she gave this award out.  I love reading Rachel's blog because she is a caring, interesting person with a great sense of humor, and her blog is one of substance and honesty (two things not automatically found on every single blog!).  So I hope you'll check her blog out as well!

Yay!  Thank you Rachel!  So now what I have to do is tell you 7 things about myself, and then pass this award on to other bloggers.  So here are 7 things, ummmmmm....

1.  I am the youngest of 10 children; my mother was pregnant for about 20 years.  Not straight through obviously - there are approximately 2-yr gaps between us all I think.   I had a great childhood.  I could never have had a big family of my own, but they are fun.

2.  I went to an all-girls high school which had a brick wall around it (at one time it was a convent).  In retrospect it was a great experience and was definitely a benefit in terms of being ready for college.  At the time though, it was a bit challenging as I was not from a wealthy family but many of my classmates were.

3. At some point during my childhood I actually made up a code and then kept a journal in that code.  Seriously.  It was fun.

4.  Speaking of journals, I have 6-7 notebooks/journals, and a handful of "started" journals...and I'm not sure whether I should keep them or burn them.  I'm not sure I'd want the Hubster reading them if I kicked the bucket, as they are mostly full of childish whining and complaining from a much different time in my life!  I may feed them to the firepit this summer, I doubt there's much to be gained from re-reading them.

5.  I have a firm belief that I have a good singing voice.  I probably don't, and hopefully my friends and family will prevent me from auditioning for "Middle-Aged American Idol" should such a show ever exist.

6.  I think when I was about 8, my mom gave me a box of socks for a birthday present.  I must have really liked it as that is the one birthday present that really stands out in my memory of childhood birthdays!  Probably it was really cool because, instead of being hand-me-down socks they were all 100% new!

7.  Someday I would like to get a pilot's license.  Looking at the earth from up in the sky is pretty cool.  If I don't get a pilot's license I might at least skydive once.

I am not sure at the moment to which blogs I will pass the Versatile Blog award on...I'll have to think on it.

Thanks for reading!

Friday, May 13, 2011

5-Question Friday!


[Well, durn it, I didn't think there were going to be 5QF's today so that's why I posted about parental diplomacy below!  It's a pretty funny post if I do say so myself...so make sure you scroll down and read that one too!:-)]

Here goes;
1. Tube socks, calf socks, crew, ankle, none? (From@ThisDaddys_Blog)
Um.  Tube socks, really that's a choice these days?  I don't think I have ever worn a pair of tube socks, to be honest.  I was born in the early 70s so I think that particular "fashion trend" had, sadly, died off by the time I would have been old enough to rock the tube socks.  Anyway...It's crew socks during the cold months and ankle socks for the warm months.  I can't wear the flip-flops anymore because, sigh, I have very flat feet and if I wear those then I'm pretty much in serious pain by the end of the day!

2. Was your childs (children's) name a mutual decision or was it a debate? If you don't have children, do you plan on giving your spouse a say, or do you have a name you MUST have, regardless if your other half likes it or not?! (From @momtodh)
Well.  If you talk to the Hubster, he will say that Elder Daughter's name was his idea.  I however, clearly remember where we were (in a friend's car, driving back from dinner, on an evening early in the year we got pregnant w/her) when the topic of future children's names came up and I told the friend that we thought this one particular name would be what we would use for a daughter.  Clearly remember this scenario.  Hubster does not.  Hmph.  As for Younger Daughter, eesh, that for some reason was much harder.  It took us several months to decide on her name.  We went through books, I made a few lists, we talked about it...then one day he walked into the living room and threw out a suggestion....and that was it.  After S was born, and we started down the PWS road, I remember thinking that it was quite fitting that her name means "wisdom", as we definitely need that and are developing it at a pretty fast clip, living with PWS!  [If we had ever had a boy, I really like the name Daniel.]

3. What is your favorite type of weather? (From@TheSpunkyDiva)
I love it when it is sunny, but just cool enough to put on a comfy sweatshirt.  I'm not one for really hot days, but at the same time while I do love to snuggle in a bunch of warm blankets and read a good book, usually by March I am really, really tired of the cold.

4. If left to your own devices, what time would you wake up every day? (From Sandy at "Pardon My Dust")
Oh, I would get up at 7 a.m. every day, are you kidding me?
NOT.  I am so not a morning person.  If I didn't have to get up early I'd probably sleep til about 10 a.m. every day.

5. Have you bought your cemetery plots yet? (From Pam at "Troop Petrie")
Um, no.  This is one of the "I'm a responsible adult, who also has children, so I really should grow up and take care of this detail" things which I/we, erm, just haven't gotten to.  Along with the whole make-a-will, choose-a-guardian, set-up-a-special-needs-trust, stuff that keeps me up at 3 a.m. worrying.  Plus I am not sure on the cremation vs. burial thing.  Basically the whole topic gives me the heebs.

Thanks for reading!

Dear U.N.: Parents Know Diplomacy

Exhibit A:  See that photo right there?  (Well, yes, of course you see it, duh.)  See the "Ks" and the "Ss" alternating from day to day?

That, my friends, is a fine example of how parents get really, really skilled at diplomacy.  The U.N. ain't got nothin' on me when it comes to mediating between two warring factions!

Here's the story:  Along about the time when S became mobile and could walk from the car to the side door on her own, K started to take issue with which one of them entered the side door FIRST, after of course I got them both to move out of the way so I might actually get the darn door unlocked for pete's sake.  K denies that she started all this...probably will deny it 'til her old age...but she was obviously old enough to care about this issue long before S gave two hoots about who went in the side door FIRST.

I got really tired of all this.  When we get home from somewhere I just want to get in the door!  Who cares who goes in first??  I mean, yes I remember being a kid, yes I probably had go-rounds with my siblings about stupid stuff just like this, all those years ago.  But I really just found all of this frustrating and tedious and could we PLEASE JUST GO IN THE HOUSE and I'm sure the neighbors are getting a kick out of listening to all this and GIRLS! JUSTGOINTHEHOUSEALREADY and FINE.  I'll be going in the house now.  You two can just stand out here on the driveway and fight.  Sheesh.  Really? Seriously, we have to fight about this?

Well.  Obviously I couldn't just leave them outside.  Added in to all this was the issue of which girl got to let the dog out of his crate (we put him in his crate when we go places - not for vacations obviously, just errand-running).  Lovely, one more thing I had to "keep track of" in my tired brain. 

I resisted keeping track of this stuff, for many months.  I wanted both girls to just not care about this stuff...to just start doing that "No, YOU go first", "no no, I insist, YOU go first" back-and-forth that you see high-society, affected, well-mannered folks do in movies or whatever.  Like that would ever happen with a 5-6-7 yr old and a 9-10-11 yr. old.  In my dreams.

So, my solution?  Yep, it's that calendar pictured up there.  It hangs in the kitchen and the girls and I refer to it every. time. we go anywhere together where we'll all be arriving back home together.  There have been times when we've all forgotten to consult the calendar, so the girls have waited outside on the driveway by the door while I go in and look at the calendar to see whose turn it is to go in first (yes, I'm serious).  I then let them know whose turn it is, and that child then enters first.  Whoever's turn it is to enter first also gets to let the dog out of his crate. 

There are a few others parts of our day-to-day, with which the letter on the calendar might come in handy.

The United Nations will be calling me any day now and giving me some diplomatic assignment, dealing with two countries whose leaders are acting like spoiled brats, I just know it.  Not that I think my kids are spoiled brats...they definitely aren't...but I bet we can all think of countries where some parent-type discipline might work wonders. (Time-outs for Kim Jong Il....no internet/news for him for 2 years....hmmmmm....).

Thanks for reading!

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!

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:

  1. Prader-Willi syndrome (PWS) is the most common known genetic cause of life-threatening obesity in children.
  2. 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.
  3. 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.
  4. 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.
  5. 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.

  6. 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.
  7. 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!

Monday, May 9, 2011

Hi out there

You know, sometimes I come up to the computer, and I have so much I want to say I don't know where to start.

So, I'll start with thanking everyone who commented on that last post....I very much appreciate your kindness and support.  It has just been a rough few weeks; I just had gotten to a point where I had just had enough.  Do you ever have those moments, where you just feel like not saying anything to anyone, ever again?  That's where I had gotten to....so I just couldn't come here and write much, because in my day-to-day life I've had a few very negative reactions to things I had said.  I realize that having opinions about things, and then actually voicing those opinions, does make me vulnerable to negative reactions - but I've had too many of those, too close together.  I got pretty nervous about saying much of anything, about anything!  But, I'm not the kind of person who will just shut down for good.  So here I am.  Hopefully I won't piss anybody off this week, but I am treading veeeerrrry carefully.

Our visit at our PWS clinic with Sophie a week or two ago went really well, thankfully!  Her BMI number went down a bit - probably helped some by her having gotten just a wee bit taller - so that was really good news. And, while we do need to work on her weight, I was still glad to see that her weight has stayed pretty consistent over the past year-and-a-half.  This is a good thing - she's been weighed at various doctor's appointments over the past six months and it has stayed about the same.  Big jumps in weight for children with PWS are a big cause of concern, and mean a big decrease in the amount of calories the child can have in a day.  So, I'm glad we are not in that place.  We did talk with the behavioral psychologist about S's recent behavior issues.  It was good to talk with her but I'm not sure she gave us suggestions that were altogether helpful.  Well, I shouldn't say that - we have tried one of her suggestions and, most days, it has helped;
S earns a quarter when she gets herself ready for school quickly, with no dawdling or yelling at people.  She has earned about $1.50 so far, and we may use this technique for bedtime routines as well.  I didn't think it would work for S because she doesn't exactly understand money at this point.  But, because she absolutely LOVES animals, I borrowed an idea from another PWS family and told her that the money she earns could be used to buy supplies for a local animal rescue, and then we could go there to drop off the supplies, and then she could visit with the animals!!   She seemed to like that idea, and I know she would enjoy it, so we'll see.  At any rate, a psychologist can't actually prescribe medications, so if we needed to go that route we will have to call her back and get a referral to a psychiatrist.  But, that is an option I'd like to put off, if we can.

Mother's Day for me was pretty nice, I have to say!  (Rachel, thank you for the Mother's Day wishes!! :-) )
I did a fundraiser at my church in the morning - sold my "Zen.tangle" artwork.  People responded pretty well to it and seemed to like it.  I did miss going to lunch with Brad and the girls and his parents, but he ordered some take-out Oli.ve Garden for me which I ate at my in-law's house.  We then had Chinese for dinner, so yay, a whole day without having to make a meal!  Plus Brad and the girls got me some lovely-smelling shower gels, a food processor, and two cds (Adele and Sugarland).  I'm not sure if I'll keep the food processor, in truth I really may not cook enough to get enough use out of it!  So it is still in the box.  I always aspire to cook more but it just never seems to happen!

Well, thanks for reading my meandering thoughts.  I am better but I'm still feeling pretty burned from various situations.  But I will continue writing!


Wednesday, May 4, 2011

I would pray, but...

I'm guessing God's just as frustrated with me as everyone else seems to be, these days.

Yah, this sucks as a post...but it was a thought I had, and pretty much sums things up to cover the past few days.  Weeks, months, years?  Who knows, who really knows.