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Tuesday, January 23, 2007

The truth about Growth Hormone Deficiency in Children

After reading about this issue, and being personally involved for over 21 years I wanted to have a place for honest communication for parents. As you will notice, I am not a proficient writer but I do write from experience and with high hopes that this little blog can help people suffering.

I have read the good, the bad and the very biased information about growth hormone use for children for many years. I am a mother whose child was one of the first children to ever use the "new" growth hormone medicine. It was an agonizing decision.

My interest was not focused on getting my daughter taller, even though that was nature's little alert signal telling me something was wrong, rather I was more worried about her life. I wanted her heart to be strong, her lungs to work well and her bones to hold up throughout her lifetime. Today she is healthy, no longer on the medicine- and has given me 2 healthy grandsons (ages 2 and 1- oh they are cute!!!!) (Revison on Jan. 2011- she is now back on GH- so parents be aware!)

I have been around a while and experienced many issues with her. So if you have a question, comment or concern-feel free to shout.

Details of some of my experience with this-is provided next. Post your comments and questions so that everyone can learn. Thanks!

June 4th- one of the happiest days in my life. The birth of my first child, Missy. As the pediatrician checked her over and all was "A-OK", I joyfully and sincerely said my prayers of thanks. For the next 18 months her father and I watched over every move she made and marveled at every silly thing. We just knew our child was the best child in the entire world! She was given one hundred percent of our attention and every toy imaginable, including toys from supermarket aisles.

I admit it, she was spoiled rotten...until December 29th of the following year. Our second daughter Elaine was born. The reality of having two perfect children exhausted our head sizes even further and put a fast reality check on our spoil the child bank account!

As the girls began growing up I naturally (despite my best efforts not to) started comparing the two. When Missy was 3 years old, Elaine was almost as tall as her "big" sister. I mentioned my concern to the girl's pediatrician. He said, "Don't worry…"

Two years later Missy had not improved. I had gotten tired of the don't worry be happy scenario. Elaine was now the same size as Missy. I was beginning to panic. The pediatrician now said I was being hysterical and over anxious, and not to bother him about such concerns! Well this poor man paid for his lack of experience and tact as I unleashed a truly panicked mother's verbal rage.

The nurse took me aside. She told me to come in without an appointment on the same date each month for several months, simply to measure my daughter. And laying her job on the line, said she would give me the referral I needed to see a specialist if the measurements showed that Missy wasn't growing normally. (Long story –military nurse outranked doctor).

Anyway, six months later, Missy was referred to a Pediatric Endocrinologist. My mothers instincts were … unfortunately …right! The specialist examined her thoroughly, and took a complete medical history of both my husband's family and mine. After a battery of tests and more than 4 weeks time, we had a diagnosis. She had a pituitary disorder causing her to be deficient in both thyroid and growth hormones.

Great! I thought, let's get the pills and get started! You see I too had not grown normally… thyroid pills "fixed me" and I was anxious to "fix" her. Boy was I WRONG! What was facing us now was terrifying. She did need thyroid pills but growth hormone was new (this was many years ago) and required regular injections. The doctor explained that the long term effects of this "new" medicine were not known (at the time). She could go without growth hormone and be perfectly proportioned but stand only about 4 feet 2 inches tall as an adult. My husband and I were terrified. Short ok...but heart, lungs, bones...what was going on? What do we do? Experimental, shots, unknowns, only opportunity...the urgency and the unknown were literally taking our breath away. The pediatric endocrinologist suggested that we go home, discuss it thoroughly, and get back to her with our decision.

So we packed up the kids, put on our brave faces and headed home. The 45 minute drive seemed to last for hours. We tried to be happy with the kids, singing and playing games to keep them busy. Occasionally my husband and my eyes would meet but we quickly looked away fearing that we would loose control and break into tears. The decision was a tough one and we could not make it alone. Lesson #1- you are never too old to call Mom and Dad!

We love our daughters, they are our life. To have Missy healthy at the height of 4 feet 2 as opposed to the uncertain possibilities was strongly considered. Yet to pre-determine her future, to live her entire life the size of a child was an aspect we had to seriously consider. More importantly to us were the other (the real deal) aspects which growth hormone affects; her heart size and strength, her lung development and strength, her bone strength, immune system function, and much more....we had a lot to learn, very fast.

I called my father who was a hospital director with access to people and inside knowledge. It was a serious call, and a serious decision. His first born grandchild's life was in the balance. He and my mom tackled the job with the fierceness of a protective lion. After several weeks of agonizing waiting, my father finally called. He said, (and this is verbatium because I will never forget his exact words), "It's safe. I can’t tell you what to do but if it were me I would go ahead with the treatments." He relayed all of the information he had gathered piece by piece. I knew that this very protective grandfather would never make such a definitive statement without complete faith. My husband and I talked some more...and then we called the doctor. We were going ahead with the growth hormone treatments. We made an appointment for the next day, to learn how to give her the shots.

The evening before "lesson day" was horrible. We sat down with both girls, one kid for mom's lap and one for dad's lap. We explained the situation as best we could in 5 year old language. We were surprised that Missy did not act concerned. Later, we tucked the girls in for the night, and then sat side by side holding hands in silence on the couch for hours.

There were no words left to say, only tears. My thoughts raced...I will be brave. I have to be strong so that Missy will not be afraid. Oh God, please help me make it through this!  Please let this decision be the best one for her...How is she going to react? Is she going to hate us? Can I actually push that needle into her tiny little leg? My mind was racing out of control. All I could do was hang on and keep going...cook, clean, clean, clean,clean and clean some more-anything distracting. My house looked great, I looked a mess!

Lesson Day- June 9th. My husband’s 28th Birthday! The activities helped distract Missy. Yet, every smile on her little round face brought doubt in our hearts...would she be the same sweet child after all of this started? What if our decision is the wrong choice? What if this stuff hurts her instead of helping her? I kept telling myslef to trust my faith. We were put in this extraordinary doctors hands for a reason...trust my guidance. But oh it was so hard at this point! We dressed the girls for the doctor's office and began what seemed to be the shortest ride back to the hospital.

I DON'T WANT TO BE HERE, I thought, as we pulled into the hospital parking lot. But being the adult, I composed my nerves, gathered Missy in my arms went to the office. Dad gathered up Elaine, and put his arm around my shoulder playing with Missy's arm which was clinging to me. My throat ached with a fierce gripping sensation. Trembling hands signed my daughters name on the waiting list, as I nervously tried to smile.

She jumped a little each time a name was called out, but quickly returned to play when she heard an unknown name. After about 10 minutes we heard....MISSY, the nurse's voice echoed...I looked at my husband. I was quietly taking deep breaths to try and keep control and he noticed. I remained with Elaine in the waiting room because as the nurse put it- we don't need to scare her too and mom looks like she needs to stay out here. Thank you Lord, I have the role of the ostrich! Without a word my husband Jim gathered her up in his arms, kissed her cheek, and headed down the hall. 
About 30 minutes later, the love of my life reappeared. His face was pure / see thru white with Missy in his arms. She was smiling. She thought it was all over. Poor Dad was trying to keep his “public” composure as he had just given his baby her first shot with a nurse evaluating his skills. On the drive home he gathered his strength back and praised her. He tried to explain it would happen again- but she mentally ignored and blocked it....we just let her! So much easier at this point!

The next evening I picked her up and said, "OK baby, now it's time to get your shot". This is when reality struck her with a vengeance.

My husband took the "stuff" required to do an injection into the bathroom. When faced with the reality and the inevitable, she began manically kicking and screaming like a wild child. I had never seen anything this fierce from her or any other child ever before. We had to enclose her in the bathroom just to try and contain her. I stood in the hall leaning on the closed bathroom door with tears flooding my face. I gripped my mouth with both hands HARD to hide my own gasping sounds as I heard my baby yell and beg her father to love her...pleeeeeease don't daddy...I'll be a good girl..I'll be your bestest girl......I PROOOOMISE! Screams, wrestling around sounds, more begging ... it seemed like forever....then silence....

I heard the door unlock and I scrambled to my feet and away from the door. My husband appeared. He was pale, shaking, and holding her in his arms. And without words, he handed her to me and walked quickly to our bedroom closing the door behind him. Her face was soaking wet, hair drenched, arms sweaty, and just upset as she could be. So for the next half hour to hour, (mistake #1) I held her, rocked her and tried to assure her of our love and explain our motives.

Just FYI- The old style of injection (1985) was indeed a painful one. A large needle was used. We had to push the needle all of the way, sometimes hitting the bone and pull it back to make sure it was in her muscle. (Today they use small insulin needles and the pain is minor. Unfortunately, as one of the "first" children to benefit from modern medicine, she was also one who taught lessons for future children to benefit from. A sad but necessary part of safe medicine.)

Missy continued her fight each shot night for 18 long months. She finally got to the point where she would tolerate her dad doing the shot (after a lesser battle) but not me. My job was to console her. We had to explain to all of the neighbors that the blood curdling screams at 8:00 p.m. ..... meant shot time.

But then came the day when dad (he was in the military) had to leave for three months. I had been dreading this, it was now my turn...alone. Missy was quiet all day. I thought it was because she was sad about her daddy leaving. I was right, but there was so much more going on in that head of hers! That evening I prepared the syringe and her silent plan came into full bloom. I went into the living room where she was watching television. She ran. I pulled her out from underneath the bed...she ran again and again. She was fighting me with the panic and furry of a drowning child. She was hitting, biting, clawing, and punching me in the face. I couldn't let go of my hands to give her the shot without her escaping. So I just held on, and it literally took all of my strength to do so and I was not a weak person.

I held on to this squirming child for 45 minutes as we had been trying to do what everyone told us to do...and that was...patience and undertanding-with firmness and consistency (mistake #2).  But after being abused for almost an hour I got mad and popped her rear end. As this was not a common occurrence for her, I got her attention ...quickly! She immediately halted the fight. We were both dripping wet and exhausted. She relaxed her muscle just enough for me to safely give her the shot. Afterwards, we talked for a long time. We came to an understanding, and we both grew up a little that night.

The battles were finally over. From that night forward, she did her best to tolerate the injections with only a few occasional whimpers. No more running and hiding. A few weeks later, we got a BIG blessing-we began using the small insulin needles. Months later, Jim finally returned home. He was shocked at her progress. I don't know who was more proud, Missy or her daddy. The smiles were genuine and frequent. But wait...she had one more trick up her sleeve. Two weeks after daddy's arrival it was Mother's Day. Again she was quiet all day long. I thought, "Oh no what is going on her head now?"

Elaine colored mountains of pictures for me all day long. I bet she colored 3 dozen pictures of me.  Jim took us all out for dinner. When we got home it was time to get ready for bed and that meant time to do the shot. Missy quietly came to me in the kitchen. She looked up at me with nervous innocence, and big round eyes special only to a child. She said, "Happy Mother's Day Mommie! ....for your present I'm going to do my shot all by myself", and she produced a prepared needle from behind her back. I never believed, not for a second that she could do this. But "going along with it" I followed her to the family room. She sat down on the floor where she had other things already lined up and prepared. She was visibly trembling. Slowly she began to wipe her leg with an alcohol pad. She wiped and she wiped and she wiped some more. It was the cleanest injection site in the world! Then she picked up the syringe and made sure her fingers were holding it just right. She held it over her leg for a minute and then closed her eyes tight. Her bottom lip was sucked in so hard that it was invisible. Her tiny hand was shaking...Then she opened her eyes, and thrust the needle in her thigh. She whimpered slightly as she pulled the needle out. Then she looked up at me , "Happy Mommie's Day" she yelled with the BIGGEST toothless grin from ear to ear.

I cried and cried and then cried some more. Mother's Day 1988, I was given the gift of sacrificial love from the tiniest of sources...my baby girl. 

Since this experience, my life took not just one but two more dramatic turns. And due to these events the Lord and I have had some loud conversations. Some of which I am terribly ashamed of!

You see my baby daughter Elaine, broke her arm about 2 months after Missy gave me my amazing Mother's Day gift. Apparently, it was not an ordinary broken arm. As a matter of fact it looked so bad that the young X-ray technician thought we had been abusing her by breaking her arm hundreds of times! We were questioned and threatened by military "experts" regarding possible child abuse because the bones looked so horrible. But knowing that we are push over parents, we knew something was terribly wrong. After many conversations, guarantees from my husbands CO that we were ok people and not abusers- we were released from the military hospital but warned to come back on Monday for follow up (this was Friday). Hearing the "review" of child abuse and distorted bones, we knew they did not know what was going on with her and left the hospital and drove directly to Children's Hospital.

After hours and hours and many people looking at her and the x-rays, the ER staff called in the Chief of Orthopedics at 4:30 a.m.! He examined her, looked at her x-rays and left. After about 45 minutes he returned and told us that she had a rare condition called McCune-Albright Syndrome. Among other things it causes a bone deterioration (to put it simply). Finally, someone had answers! Now what?

No treatments. No cures. Yes it does continue to affect more bones etc., etc. The words were so horrible I began blocking their entrance into my brain. What do you mean there is nothing ....nothing that can be done? This is not in my personal being...nothing....call Mom and Dad...they will know.

The more I serached for answers, the worse the news - nothing could be done. How exactly do you tell a mother to just sit and watch their child deteriorate? God, I hope you offer a special place in heaven for Mom's who help their children pass or face challenges in life which us mere spoiled healthy people can only fear.

There is always a reason and a path in life. And so if something was lucky about Missy having her problem, it was this... I had met some incredible Mom's with kid's like mine. I networked with hundreds or even thousands of families and physicians from throughout the country. So when we got this horrible news, I immediately picked up the phone to call my friend Mary. She gave me the name of a nurse in Virginia, who gave me the name of another nurse at the National Institutes of Health, who knew of only 18 cases in the country, and gave me the name and number of a mom with a daughter like mine. Only 18 known cases and I found the connection within 3 hours.

My next call was to that mom in Ohio. She waited patiently as I sobbed and literally talked me through my pain and fear. She truly saved my sanity! There were many battles because Elaine wanted to learn how to ride a bike, roller skate and do all the things that kids with "normal" bones can do. I tried to stop her. She snuck around and did them all anyway. UGH!!! As it turned out, we were blessed, it appeared that Elaine was only mildly affected with the syndrome. Her symptoms were only slowly progressive. We found out why... later.

Five years after her diagnosis, she had gotten up three times to go the bathroom and before midnight. By this time in my daughter's lives I had become an intensely observant mother. So, not taking anything for granted, I took her to the pediatrician the next day. I thought she might have a bladder infection. The pediatrician literally took one look at her, smelled her breath and said she wanted to do some lab work. I thought nothing of it as they have to do labs to confirm a bladded infection. But then the pediatrician said "come with me" and she personally walked us to the lab! This was not normal I thought! But I didn't say a word as she stood there while we all waited for the results. She looked at the paperwork and said "let's go back to my office". I just obediantly followed her, thinking that it may be something about the MAS bone issue. When we arrived at her office she asked us to sit down and said, "Elaine has diabetes."
Elaine didn't know what diabetes was. But upon hearing the words I gasped. Elaine looked at me and saw tears streaming down my face. This little baby all of ten years of age, put her tiny arm on my back and began patting me, trying to console me! Then she quietly asked, "am I going to die?" I could only hug her and say "no baby, you are going to be alright". The doctor then asked that I call my husband from work so that she could talk with us together. (My husband and I surmized, years later, that the developing diabetes may have somehow stunted the progression of the bone disease but we don't know for sure because there is only one other case like hers that I have ever heard of...and have never been able to speak with that family.)



Elaine did not experience the terror and shame most children going through similar problems experience. Due to the fast networking of other children "just like me" I was able to help her feel normal despite her multiple challenges, thanks to my work with The MAGIC Foundation.

Over the years a few people have commented to me;  why I seem to give so many details about Missy's medical problems and skip over many details about Elaine. I was bothered by this. And in thinking about it I realized that due to the intense emotional overload of having not one but two affected children, one of which is life threatening, I can only deal with so much. And the more serious the problem, the more I tend to internalize. Eventually, I will reach out for help.

Each time I had called Mary at MAGIC. And each time I found exactly what I needed to help my children. Today, Missy stands 5'3" tall. She is beautiful, intelligent, and will never experience the medical complications associated with her disorder. Elaine is equally as beautiful and intelligent. She has a fighter's independent spirit, and an I can do anything attitude. Many people have commented on what a good job her father and I have done in raising two physically challenged, yet emotionally stable children. In all honesty, we were just really blessed.

We could not have done it without our parents, each other, a great deal of faith nor without the support of the entire MAGIC family. I personally, could not have made it without Mary Andrews. There is nothing, absolutely nothing more comforting than another parent who can say those words from experience...everything will be ok. It feels as real and as reassuring as the big bear hugs our parents gave us when we were children. And now that medical technology has once again pushed forward, the important role of growth hormone in the adult body is being recognized, and addresses. We are all in this together. And since the issues are "rare", if we don't band together, there will be no one for those who follow.

This is the point of my establishing this blog.

6 comments:

Jamie said...
This comment has been removed by a blog administrator.
Melanie said...

Hi, you posted a comment on my blog today (rockinralstons). I just read your story and now I'm sitting here with tears rolling down my cheeks! Ahhh! My daughter was just diagnosed with RSS. It's a little scary. What I think I'm the most scared about though is the Growth Hormone Therapy. But, I have a little over a year (she's only 9 months old and we won't start it until she's 2 years old). Anyways, I just wanted to say hi and thank you for sharing your story. By the way, our Endocrinologist is Mark W. Parker in Charlotte, NC. He's great.

Courtney said...

I cried reading this, and that's crazy consdering who it's about!

Jodi said...

Jamie, I'm so glad you are posting this. You know the awful times we went through after Max stopped gh, only to find he was ghd, too. We thought he "only" had RSS, and a complicated case of it at that. Now people can read your/your daughter's story and be aware of what to look for. The only thing I would add to your list of "symptoms" is severe hypoglycemia. That was the indicator for our son. Remember all those passing out issues?

ghdmom2 said...

Good point Jodi- thanks! Hypoglycemia is a serious indicator....although- in my daughter's case....the passing out was something else! But if a newborn in particular has hypoglycemia- they SHOULD be tested asap. If not- they need a new ped!

ghdmom2 said...

Mark Parker ROCKS!