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