Our Journey Raising Two Children with Special Needs

This blog chronicles our life raising two children, Nicholas 15, diagnosed with Prader-Willi Syndrome and Weston 18, diagnosed with Autism/Asperger's/ADHD. It's the ups, the downs, the joys, the sorrows and most importantly, the beauty of living a life less perfect, a life more meaningful.




Wednesday, March 18, 2015

Testosterone and Homeostasis

One thing about being the parent of a child diagnosed with Prader Willi Syndrome.....like it or not, it improves your vocabulary.

Yes, thanks to Nicholas, and the inordinate amount of time I spend in physician's offices, I have learned the meaning of a host of rather impressive technical terms. Multi-syllable words that, more often than not, describe a very simple process.

I am surprised just how easily these long, silly words have found their way into my vocabulary. They spew from my lips without much thought, that is until I begin to explain some of Nick's issues to others and am met with raised eyebrows, blank stares, quizzical looks or plain old hysterical laughter.

The latest silly word to enter my vocabulary is homeostasis, a fancy word that means nothing more than maintaining stability, balance....the status-quo.

Why do I know this word?

Let me explain.

Last week we met with Nick's Endocrinologist. This is Nick's hormone doctor. Children diagnosed with Prader Willi Syndrome possess a malfunctioning hypothalamus.

According to Healthline:

"The hypothalamus is a section of the brain responsible for hormone production. The hormones produced by this area of the brain govern body temperature, thirst, hunger, sleep, circadian rhythm, moods, sex drive, and the release of other hormones in the body.

This area of the brain is small, but involved in many necessary processes of the body including behavioral, autonomic, and endocrine functions. The hypothalamus' primary function is homeostasis, which is to maintain the body's status quo system-wide."

Nick is 13-years-old and up until now, growing normally. His height and weight were proportional to other children his age. His hypothalamus was doing its job keeping things in balance.

Lately, however, Nick's height has slowed and weight increased, despite a strict diet and consistent growth hormone dose. Things are becoming unbalanced.

So, since Nick's body does not manufacture the hormones he needs, his endocrinologist must determine what hormones to start and when.

At birth, we began growth hormone shots to help Nick to grow in a healthy manner. We give these injections to Nicholas at home every night and for the most part, it works.

But now, Nick is entering puberty, and since his body is not producing testosterone, his growth has slowed. We must now introduce this hormone into his body and begin a course of testosterone shots.

Although he may never be able to sexually reproduce, testosterone will help Nick's body to develop in a healthier way by increasing his muscle mass and metabolism, helping his bones to become longer and stronger and managing his height/weight ratio so that it is maintained in a healthy proportion.

As with any new drug, there are, of course, side effects.

One of them is increasing the bone age of a child. This means the child grows too much, too quickly resulting in the fusing of the child's bone plates and the halting of growth.

There is also a risk of increasing behaviors. Since children diagnosed with PWS already experience poor emotional control, this may become extreme and will need to be monitored by caregivers in and outside of the home.

Increased behaviors during teen years are however, a "rite of passage" for most and I, for one, do not want to deny my son this important opportunity.

He will receive a low dose intra-muscular injection of testosterone once every four weeks at his pediatrician's office. This will continue for six months. His dose will then increase, doubling in size and continuing for another six months. After that, a maintenance dose is established for the rest of his life. Thankfully, there are other delivery options, like a patch that he can wear on his skin to alleviate a lifetime of injections at the doctor's office.

And so as it is with all things Nicholas, I have become like his hypothalamus, I am the regulator of homeostasis....the balancer of systems, the maintainer of the status-quo. It will be important for me to evaluate this treatment and ensure it benefits him physically without pushing him emotionally over the edge.

It will be yet another big decision I need to evaluate in the ongoing care of my children.



p.s. I am not sure homeostasis can be achieved in a household that now possesses two teenaged boys.....oy!