It's not uncommon to notice changes in the energy level of your child with Prader-Willi Syndrome. There are multiple reasons for this, and each child is different, hence this syndrome being spectrum. In a previous entry I talked about supplements. It's important to remember that manufacturers (of vitamins & supplements) do not need to register their product with the FDA, nor get the FDA's approval, before producing & selling to the public. When choosing a supplement for energy maintenance, consider the source. If your doctor can give you some recommendations, as did ours, please take their advice. Doing your own research into your child's supplements can make a huge difference.
By now you are probably aware of the different phases of Prader-Willi Syndrome. Some of these phases present at different times, child to child, due to their course of treatment. A big factor is the use of Human Growth Hormone. GH has now been used for about 13 years, getting approval from the FDA in 2000,to treat patients with Prader-Willi Syndrome. Some children may show a deficiency of GH, but even with no such deficiency, GH is prescribed to help with over-all body composition & growth. It has been documented that the use of GH in individuals with PWS improves lean body mass, decreases body fat, increases bone mineral density & normalizes height. Growth hormone also influences the body's conversion of food into energy.
A physical characteristic of a child or adult with PWS is short stature, obesity & some level of mental disability. There is also a reduction in muscle mass that is associated with diminished strength, physical function & energy expenditure. This is now somewhat avoidable with the use of growth hormone injections. Studies suggest it has positive effects on behavior and development. It has been noted that children who start GH therapy before the insatiable appetite begins (between ages 2 & 3), that hunger is delayed until age 7, 8 or 9.
Supplements, diet & growth hormone all play a major roll in the over-all energy of an individual with PWS. We keep a fairly strict diet with Claire, keeping to this daily ratio: 25% protein, 35% fat & no more than 40% carbohydrates. It's not easy - it's actually REALLY difficult. Before our first appointment with Dr. Miller, I got the idea to make a menu board for our family. I spent hours upon hours finding, creating, re-doing favorite recipes that met the calorie restrictions I was under the assumption we needed to stick to. I organized it by color, laminated it all, included nutritional content per serving. Here's a picture...
I was so excited to show off my pretty menu board to Dr. Miller. However, she said to me "Oh, we don't count calories, we just balance." My bubble was burst.
Nonetheless, when I returned home I went through the recipes, tossed some out, and started searching for more or tweaking the ones we love. I also started doing a bunch of research on 'healthy' foods.
Since adopting the 'Balancing Diet', we all have noticed that it's not so bad! There is a noticable increase energy, and I have lost 15 lbs in 6 months. Food is essential to the proper functioning of your body, but those with PWS need a little more help. Each time Claire's Growth Hormone is increased, we see an increase in her energy, sometimes more than others. A few weeks ago I asked the doctor if we could add B12 to Claire's regimine- she said yes, told us which kind to get & the proper dosage. There was no difference in her energy, which was quite poor at the time. After a blood test, we learned that Claire actually had a pretty severe case of anemia. We added gentle iron (non-constipating) & after about 5 days, she is back to her silly, funny, energetic self! We took away the B12 & will add it in the future if we feel it's necessary.
Because of the plethora of information on the internet, it's easy to fall prey to fad diets, pills, supplements, juices, etc. Our doctor just put out a very informative PSA that I think everyone should read - and remember. PSA: Warning
There is no cure for Prader-Willi Syndrome. It is mostly about treating the symptoms of the disorder as they arise, and each person is very different. We consider ourselves extremely lucky thus far that Claire has been so healthy. She is in 4 therapy sessions a week, is in horseback riding therapy (hippotherapy), plays with children her age, has the regular childhood illnesses, and is the JOY of our lives. We trust our doctor, and that is a big deal. You know your child, and you may know best in some situations, but I urge you to consult with the professionals when making decisions on the course of care for your child. What works for one child, may actually hurt another. Diets included.
Most of you moms recognize the word Pitocin. Pitocin is a drug used to induce labor, or create stronger contractions during labor & is also sometimes used to control bleeding after birth. Pitocin is the chemical brand name of the naturally occuring, and quite powerful, hormone Oxytocin (not to be confused with the pain killer Oxycontin, or Oxy). It is often referred to as the "trust hormone" & is released when we hug or kiss a loved one and plays a huge role in bonding.
There have been several studies conducted in France on the use of Oxytocin in adults on the autism spectum. They found that the adults socialized more often after taking Oxytocin, they understand the behaviors of others & can respond appropriately. They have also found that this hormone, even in a synthetic form, enhanced autitic adults' abilty to understand emotions in speech & can lessen the common symptom of repetitive behaviors. Some behaviors of children & adults with Prader-Willi Syndrome mimic those of autistic tendencies, and therefore, some of these studies have been very useful in the treatment of Prader-Willi. Autism is more common in the most rare form of PWS (imprinting defect), but a lot of the symptomatic issues are common in all three types.
Speech delays are very common, this is why early interevention with ALL types of therapy are necessary, even before there are any true signs there may be a specific issue. This may sound silly, but it is very likely there will be some sort of delay, and it's better to approach the care of your child's PWS as preventative, rather than the "catch-up" type, as I like to refer to it. This will cause you to really stand up to coordinators, or case managers, for your child's first 3 years of therapy if you choose to do it through the state. Most states do not work in a manner of preventative therapy - it's always the "catch-up" aprroach, and personally I think it's negligent.
We spent countless hours fighting for the proper care & the correct amount of therapy sessions for our daughter. There were also set-backs when we needed to ask for a new therapist because we knew the sessions were virtually useless. When I couldn't make any more calls or argue with another case manager, because my emotions were getting the best of me, AJ would take over. Luckily, he is calmer than me, and has a way with words. I found that they tend to listen to the man of the house with a little more respect... so if you have that in your back pocket, don't be afraid to use it.
The reason I'm highlighting speech delays in this post is because the use of Oxytocin in kids with Prader-Willi Syndrome is showing improvement in speech & socialization. It's also believed to help with anxiety, irritability, social communication, behaviors & possibly even some food issues. Individuals with PWS have been found to have a deficit of oxytocin-producing neurons and decreased oxytocin receptor gene function. Oxytocin is produced by the hypothalamus, where most of the issues with PWS begin, so it makes sense to try to bring it back to it's normal level.
Our speech therapist, who we really respect & trust, has told us that Claire has social anxiety & this is why, in part, she is practically non-verbal 3 months out from her 3rd birthday. We know she will talk, and she CAN talk, she just needs to form some new pathways in her brain that allow her to feel comfortable in social settings, enough to speak. It's definitely baby steps, but I see improvements weekly.
Oxytocin helps with creating that trust within herself to want to have conversations with others. She used to remove herself from play groups and go hide in the closet, or just go into a different room, away from other little kids her age. Now, she's trying to grab everyone's hand and dance with them, or lead them to a toy. She may not be saying anything, but her non-verbal communication has improved significantly. Do not be discouraged if progress seems to be slow - there is no timeline for which this all needs to happen. Afterall, Einstein didn't speak until he was 4.
