June 2008 - Prader-Willi Syndrome Association (South Africa)

two of the most important things you can do for us…I could feel sharp teeth tearing at
my stomach like piranhas and still do. I know I need someone to keep the cupboards
locked and I need someone to keep me active and to control my weight. I want to
have some fun in my life…. If the government has money to fund crisis situations why
can they not prevent situations from becoming a crisis? I need full support NOW
before I get into a crisis situation. I want to live and I am sure you want the same for
your child.”
John died shortly after this was written.
We did not find a cure in his lifetime, but I
have promised my own son Matt, who is
27 years old, that in his lifetime we will
find a drug to abate the deadly hunger.
Meanwhile, we need all of the help,
support, and compassion you can muster
as a dietician to make the long road to
weight control a little easier.§
1 calorie = 4,186kJ
* Wenk: Die sg “fridge lock” werk vir ons. Die slot
sluit self d.m.v. „n draaibeweging en sluit oop m.b.v.
„n sleutel.
Tip: The so-called fridge lock works for us. The
lock closes by turning it and has to be opened with
a key.
Precautions Advised When Starting Growth Hormone With PWS
From The Gathered View, March-April 2008
We advocate a sleep study before the start of growth hormone (GH) on infants,
children and adults with PWS and a follow-up study 6-8 weeks later.
If obstructive sleep apnea (OSA) worsens on GH, temporarily stopping GH is
recommended until the cause is understood. Frequently OSA can be corrected by
removing the adenoids and tonsils or lowering the GH dose (in the face of an
abnormally high IGF-1). We also recommend taking precautions during bouts of
upper respiratory infections.
Dr. Merlin Butler also recommends obtaining a thyroid function test and cortisol levels
(in a.m.) before starting growth hormone treatment. He has done a recent study on
cortisol levels in 63 subjects with PWS and found one of four infants with PWS had a
low cortisol level. There has been some discussion about adrenal hypofunction in a
subset of PWS.
Studies have shown that in most individuals with sleep-disordered breathing due to
PWS, GH can actually improve (or at least doesn‟t worsen) the apnea (Haqq et al,
2004; Miller et al, 2006; Festen et al, 2006).
Withholding GH from those with sleep apnea may be detrimental on several levels,
thus the recommended approach is monitoring children with PWS closely when
starting GH to make sure that they do not worsen.§
Jennifer Miller, M.D., M.S., Endocrinologist,PWSA (USA) Clinical Advisory Board
Merlin G. Butler, M.D., Ph.D., Chair, PWSA (USA) Scientific Advisory Board
Daniel J. Driscoll, M.D., Ph.D., Chair, PWSA (USA) Clinical Advisory Board
People With Strength Volume 11 Issue 2 Page 14