Supplements

Ellie has quite the cocktail in the morning of supplements.  Every morning I make sure she takes various supplements that have proven to be effective in individuals with PWS.  Each of them work in different ways, but benefit her.  As she becomes older, we will continue to research supplements and talk with her doctors about what could benefit her.  I wanted to share with you a list of the supplements Ellie takes, or has taken in the past.  These give her the best possible outcome, in addition to some medication, for fighting PWS.  Also, these supplements are not given to just "try," we consult and are followed by leading endocrinologists in the field, and they know Ellie is taking them, and advise us on her dosages.  Please make sure anytime you seek to begin any supplement that you consult your physician first.


Carnitine - when child with PWS is taking less than 20 oz of formula or breast milk a day, then they need carnitine supplementation.  Until that time, those things are both in breast milk and formula.  "Carnitine is a natural antioxidant that improves cellular energy metabolism. Low carnitine levels may occur due to metabolic diseases or can be due to inadequate nutritional intake of carnitine-containing foods [Stephnes et al, 2007]. Carnitine supplementation has not been studied in individuals with PWS, but in other conditions has been shown to improve hypotonia, ataxia, activity levels, and alertness. Infants and young children with Down syndrome, who have muscle hypotonia and delayed growth similar to that seen in individuals with PWS, have significantly lower carnitine levels than unaffected children of the same age, and supplementation with L-carnitine results in significant increases in visual memory and attention in this population [Seven et al, 2001]. A subset of individuals with PWS have been found to have low serum carnitine levels [Miller et al, 2010].  Carnitine is available in a variety of formulations, including L-carnitine (available over the counter or as a prescription) and carnitine fumerate. Although no formal studies of carnitine supplementation have been done, we have anecdotally heard that some individuals have improvements in gross or fine motor skills and alertness with carnitine supplementation" (Information from Miller handout).

B-12 - I had been talking with another PWS mom and reading some info about B-12 on the PWS listserv, and wanted to give B-12 a shot.  I asked Dr. Miller if we could, but more importantly if it was safe.  She agreed that B-12 is remarkably non toxic and did not see anyway that it could hurt her, and also mentioned that when testing B-12 in individuals, it is most always high in everyone with PWS (from infants through adults).  We discussed that would could see improvement with balance, endurance and language.  There are two forms of B-12 that are absorbed the best, injection and oral methyl strips.  We tried the oral strips.  I got super nervous, because there was a week where Ellie was extremely clumsy.  It was as if her body had to work to respond to the B-12...after that week, Ellie's balance has improved and she had a huge spurt in language development.  

Probiotic - probiotics help with constipation (something individuals with PWS tend to combat due to their low muscle tone), and also help if the individual has been sick and been on antibiotics for a period of time.

Fish Oil (Omega 3 fatty acids - DHA) - when child with PWS is taking less than 20 oz of formula or breast milk a day, then they need omega 3 fatty acid supplementation.  Until that time, those things are both in breast milk and formula.  "Individuals with PWS may have very caloric and fat restricted diets. Because of this, the diet may be low in healthy omega 3 fatty acids. This is highly important for children due to the developing brain. Recently, infant and toddler formulas have been supplemented with similar amounts of fatty acids found in breast milk. Therefore, starting fish oil supplementation generally begins after 1 year of age when a child’s diet is primarily whole milk and solid foods or if the diet is low in fat" (Information from Miller handout).

Vitamin D - Ellie recently had lab work that showed she was Vitamin D deficient...remember that yucky winter we had with no sun?  We are currently giving her a Vitamin D gummy and she loves it...and it is also strengthening her bones, which is critical...it is common that individuals PWS can have osteoporosis (low bone mineral density).  The reasons for the increased prevalence of osteoporosis in PWS are not totally clear, but decreased production of sex and growth hormones and hypotonia most likely contribute to the increased tendency to develop osteoporosis (http://www.fpwr.org/fpwr-funded-projects/osteoporosis-in-individuals-with-pws-and-the-role-of-vitamin-d-receptor/) 

Iron - Iron deficiency is something relatively commonly in kids with PWS, and Ellie was deficient when her sleep study came back and it showed she had may leg kicks during the night.  Her lab work showed that the was slightly low in iron and so we started supplementation at a very low dose.  Iron is a supplement that you have to be extremely careful about, because it can be toxic, if too much is given.  Ellie no longer needs iron supplementation.  I am glad, because thing it can do is stain your teeth!

CoQ10 - If a child with PWS has excessive daytime sleepiness and is sleeping through feeds, could benefit from this supplement.  It helps with daytime alertness.  "Some parents also see an increase in activity and attentiveness after supplementing their PWS child with CoQ10. However, while some parents feel that their child demonstrates improvements with CoQ10, others feel that it has no effect. Also, parents should be clear that CoQ10 supplementation is not a substitute for growth hormone treatment which has definitely been shown to have multiple benefits for individuals with PWS by many well designed research studies" (Information from Miller handout).