Precautions To Take When Starting Growth Hormone With PWS

We advocate a sleep study before the start of growth hormone (GH) on infants, children and adults with Prader Willi syndrome, and then a follow up study 6-8 weeks later.  If there is worsening of obstructive sleep apnea (OSA) on GH temporarily stopping the GH is recommended until the cause is understood.  Frequently the OSA can be corrected by removing the adenoids and tonsils or lowering the dose of GH (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 AM) 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 monitoring the child with PWS closely when starting GH to make sure that they do not worsen is the recommended approach.   

Jennifer Miller, M.D., M.S.  –  Endocrinologist on the PWSA (USA) Clinical Advisory Board
Merlin G. Butler, M.D., Ph.D. – PWSA (USA) Scientific Advisory Board Chairperson
Daniel J. Driscoll, M.D., Ph.D.  --  PWSA (USA) Clinical Advisory Board Chairperson

edited: 03/23/2010