Rescued by a Gila Monster……and the creativity of scientists. (we hope!)

The magnitude of this story is perfectly stated by a friend
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….“I think life sometimes is measured in moments.  you are born in a moment, you die in a moment, you can fall in love in a moment.  everything can change in a moment.  most of our strongest memories are really moments frozen in time, good and bad.
for you, perhaps, this was one of those moments. maybe everything changed.”

Thursday, March 13 was that moment.

Katie and I sat nervously in the office of the endocrinologist. She had just been weighed, so she was quite disappointed and disgruntled before trying to pull herself up and onto the exam table. That is never an easy feat. She tried to step onto the narrow shelf that pretends to be a step stool to the table. Facing the table, she lifted one foot onto the step, lost her balance, grabbed hold of the table and tried to turn around to plop her behind on the table, but was unwilling to move either of her feet. I had her step down, turn around, hold onto me and lift her foot up behind her to step onto the step so she could then, just sit down. I imagine reading all of that was as tedious for you as living through it is for me. This is a routine we do with each appointment. Finally, she was situated, I settled into a chair next to the exam table and coached Katie to look at the doctor when he speaks to her and to answer his questions. Usually she sits and cries. Which is understandable. Every time we leave the office I feel as if I am the most incompetent mother to have ever walked the planet. Endocrinology appointments are not our favorite thing. Our exercise log is accepted with extreme skepticism, her blood sugars are way out of control, and we feel like there is nothing we do that is right.

March 13th changed all of that.

When the exam room door opened a doctor we had never seen before entered the room, introduced himself and by his actions and words it was obvious that he had already studied Katie’s case. He reviewed her A1C levels and smilingly told us that he was pleased that they had remained so steady, that it was much lower than when she was referred to them. There was no change in the number that day than the previous three visits, but the attitude of the two different doctors was like night and day. I wanted to bow down and kiss the man’s feet at that moment, not only for not berating us, but for praising us, which was a first. He looked at her exercise record and said she was doing a great job at being active. Then the moment came. The moment that quite possibly made everything change. His next words were.

“You are doing everything you can do, what needs to be done is to take off some of the weight,  but the Prader-Willi complicates it all, I think I can help with that. I would like to try, if you are willing, some different medications, we will keep her on her insulin and start her on a pill and a weekly injection, her blood sugars should improve, we will be able to lower her insulin and get her off of some of the insulin eventually. Maybe by the end of the year she will need no more insulin. The injection given once a week is Bydureon, some people find that there is a decrease in appetite with it, with the Prader-Willi we don’t know what the effect will be.”

My mind:  WHAT!!!!?????   A decrease in appetite!? gulp, puke, sob, sob, sob, CAN THIS BE POSSIBLE?! don’t fall apart Anne—ask rational questions!

Me: Is that like Byetta? I have read about Byetta’s possible effects on appetite with Prader-Willi.

Dr.: Yes, the same family of,  it is a weekly form of exenatide.

Me: Yes, we would love to try it.

My mind:  sorry, mind went blank here, swirling with the enormity of the possibility of the radical changes this could mean for her.

NO HUNGER! What a wild thought. No grinding of her teeth all night long as she dreams she is eating, no sneaking cheese or pancakes or pizza into her pocket when I blink, NO HUNGER……or even the idea of decreased hunger would be acceptable and welcome. Acceptable and welcome, what a silly phrase for me to use when basically my entire being is shouting, “Oh my God, Praise the Lord, let this really work, Yes, Yes, Yes!!! ”

We listened to the instructions, I made sure much of it was written down, I knew my reeling brain was not absorbing anything other than the possibility of decreased hunger.

We walked out of the office giggling with delight.

As we drove home I told Katie that she needed to really work out and keep good records of food intake, insulin amounts, and blood sugars. I also stated that it will be interesting to know if she feels any different when she is on the new medicine. I told her to let me know if she feels anything different.

Here’s a thought.

If a person has never had the feeling of satiety, will one know what it is if it happens? If one has not experienced something before how do you know it, recognize it for what it is if it does happen?

Here’s the condensed nutshell, please read the attached links to have your mind completely blown, but the basic nutshell is this, a doctor found out that the spit of Gila Monsters affects blood sugar. Some other people found out that injecting this into folks with PWS showed a decrease in their appetite. Now, you and I would think that news should automatically be shouted from the mountaintops, but scientific types thought that the study was too limited to have any real worth. Fortunately, other people are continuing to study this and more fortunately there is a doctor in Rock Island, Illinois who knows all these things and has possibly changed our lives forever.

For weeks I found myself weeping at random moments at the thought of what this could mean for Katie’s life, for my life, for the lives of everyone, everywhere affected by PWS. I tried to tell people who deserved to know the excitement. People who always, sincerely inquire after her well-being. People who love her.  Mostly, all I could do was bawl, I would start out by telling them I had news about Katie, then I would break down. I probably scared the crap out of them. Some I never even told, because the enormity was simply too overwhelming. For those of you to whom I said, “wait until my next blog post”. This is the post you have been waiting for. Read it and weep!

Here is an aside to all PWS parents. I want you to know some of the things these people said to me. To let you know that there truly are people who “get it”.

*This is HUGE!

*I can’t even imagine the changes this will make in your lives.

*sob-she won’t be hungry?-sob-sob-sob

*oh, Anne -hug

* Monster to the rescue…Does that mean there are now gila monster farms, like chicken farms? (I know, not quite showing the enormity, but shows that a sense of humor has always been appreciated by me.)

*I’m very happy for you. And hopeful.

*I do grasp it, and that is incredible news!
*wow, just think what this means
*I never thought we’d see it.
*nothing…..nothing could be better news
With all of the focus on obesity these days, I feel like it is a boon for PWS. Researchers are paying attention to us and wanting to know more about the inner workings of satiety vs. hunger. It has given me hope that someday Katie would benefit from their intelligence and studies. I honestly did not expect it to happen during my lifetime, but I had hopes that it would be in Katie’s lifetime. We still don’t know exactly how much affect it has on hunger. I still watch her chewing in her sleep. Her pockets still show signs of pilfered food. Her need to know what will be eaten at the next meal and the time of said meal has remained the same. What has changed is this, after being on this regime for five weeks her blood sugars had decreased dramatically and she lost NINE pounds. She continues to lose weight and her blood sugars are doing well with very small amounts of insulin.
I am not a scientist, but revere them greatly and think our society is screwed up with its worship of entertainers and athletes. Give me a scientist any day of the week. I might not understand what they are talking about, but I would love to listen to their thought processes and probe them with questions.

My utmost respect and gratitude goes out to the Gila Monster, Dr. John Eng, and Dr. Rameshkumar Raman.

CAVEAT: This might not prove to be the total solution, but the fact that this discovery has been made certainly gives me  hope that more and greater discoveries are on the horizon.

~”It really is a beautiful lizard,” Eng said. “Like many other animal species it is under pressure from development and other environmental concerns. “The question is, what other animal has something to teach us that can be of future value? And plants, too? We will never know their value if they are gone.”

http://legacy.utsandiego.com/news/business/20050429-9999-1b29amylin.html

http://www.ncbi.nlm.nih.gov/pubmed/21632815

http://www.pwsausa.org/research/provigil.htm

http://www.healio.com/endocrinology/pediatric-endocrinology/news/print/endocrine-today/%7Bdda4e2f5-5107-4bd2-838b-614ce5c41c39%7D/exenatide-may-increase-satiety-in-adults-with-prader-willi-syndrome

http://public.ukcrn.org.uk/search/StudyDetail.aspx?StudyID=11280

http://clinicaltrials.gov/ct2/show/NCT01444898

http://www.rdshp.org/research.php

http://www.nytimes.com/2014/01/15/health/seeking-clues-to-obesity-in-rare-hunger-disorder.html

 

 

 

Humbled by generosity

 

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  I have people in my life who astound and humble me with their generosity. People who have given unexpected, greatly appreciated gifts- not because it was a gift giving occasion, but simply because they thought they had something I would like or could use. People who have helped me financially when a need was noticed. Some examples of generosity towards me: a girlfriend buying me Jujubes from the candy counter at the movie theater when we were children, a friend sharing paper, stamps and ink to encourage a new hobby for me and Katie, practical items such as soap, Crystal Light, and sugar-free canned fruit, a couple thinking I could use a Kitchen-Aid mixer, a woman I barely knew offering me a lovely linen tunic, books, purses, and jewelry showing up in my mailbox, fellow gardeners splitting and sharing plants, cold hard cash to use as I see fit, offers of frequent flyer miles to a girl’s beach vacation, neighbors we barely knew offered the use of their car after ours was lost in a fire, gift cards to the grocery store, restaurants and coffee houses, cheery plants and flowers….this list could continue for pages, this is just to let you know some of the random things people have bestowed upon me.
  That list did not include the incredible amount of time others have given of themselves for my benefit. Women have converged upon my house to clean, cook, iron, toss me in the shower, and dress me for my son’s rehearsal dinner and graduation parties. A woman with five children of her own takes Katie bowling once a week. There are people who endure endless phone calls and emails that have prevented me from needing years of psychotherapy. People who encourage life: cheerleaders of my swimming passion, readers of my ramblings, people who want to laugh, dance, sing, play, and talk.
  These kind people who are my family and friends humble me with their generosity. I agree with what Felix Frankfurter stated, “Gratitude is one of the least articulate of the emotions, especially when it is deep.”  Deep gratitude leaves me choked up, unable to express how truly appreciative I am. I very much appreciate the time people devote to me, I adore the gifts bestowed upon me, I value and frugally utilize their financial support, but what moves me greater than what these folks do for me is the thought that they do.
Oh, to have a heart able to see need and a soul willing to share.
How does that happen? I see a benevolent God blessing people with a spirit of generosity, hearts filled with tenderness,
and souls guided by love.
  I see those people placed in my life…..and I see a glimpse of the bigger picture. Only enough that my limited understanding can comprehend, but it explains why deep gratitude is so inarticulate. It is bigger than me. It is more precious than words can express, but words are what I have to give, so I will continue to gratefully say, “thank you”……..always, Anne

Stop Bullies

October is a favorite month of mine and is known for many things:  crunchy, colorful leaves underfoot, apples, pumpkins, sweatshirts, bonfires, football, breast cancer awareness and anti-bullying campaigns.

Forgot gym suit at home. Fever and vomiting. Need lunch money. Sprained ankle from falling down stairs. These are a few of the phone calls one expects to receive occasionally regarding a middle school child. Never, ever did it occur to me that I would get a call from school telling me that my daughter had been set on fire, I had no idea how to respond. If it had been any of the aforementioned reasons, I had pat responses ready for those calls. Instead, dumbfounded, I leaned my body against the kitchen wall where the phone was hanging and listened as the compassionate voice on the other end assured me that she was fine. That was the part my befuddled brain grabbed hold of: that she was fine, the rest was just details. I was told that a boy had brought lighter fluid to school, poured the fluid over her long, blond, frizzy curls and set her afire, but she was fine. The woman was combing the burned hair out of her head while she was speaking to me. In a calm, comforting voice she told me that there were no burns on the scalp or neck or face, that she was fine. When I untangled the knots from the phone cord, I hung up the receiver, leaned back against the wall in a stupor and thought, “what do I do now? They said she is fine, so I guess I don’t need to go get her, but gosh, I do want to check on her to make sure. I’ll just buzz up there, make sure she is fine and be on my way.” I seriously almost did not go see her simply because it was not a scenario I had ever rehearsed in my head and I didn’t know what to do! October is ANTI-BULLYING month and this is a glimpse of how bullying affects people. It makes the parents of the bullied completely astonished, flummoxed, aghast and horrified.

When I arrived at the school, the teacher who had phoned me was just finishing combing the charred hair from her head. There were no burns anywhere! With lighter fluid in her thick, frizzy hair it is amazing that she was not burned and had no inhalation signs. My darling girl said that she heard the boy tell another boy what he was going to do to someone and she was trying to get through the crowd to go get help, she didn’t know it was going to be her in flames moments later. She said there was a “whoosh” of noise as the hair took fire and that angel wings were batting her head to put the fire out. She assured me she was fine. She seemed nervous and kept looking at the big, round office clock. After being doused with an accelerant and torched, the thought that worried her was that she was going to be late to class. She was sent on her way with a pass excusing her tardiness. I visited with the principal to find out what should happen next. I was told that the boy had already been arrested, the school had filed charges against him so I would not need to do that and that the on-site liaison officer had removed him from the premises. There would be a trial that I would be allowed to attend and I should expect a call from a Victim’s Advocate who would tell me where I needed to be and when. I left the school stunned.

As Paul Harvey says, “and now, the rest of the story….” My first visit to the county courthouse was with my mother by my side. We sat on a wooden bench in the hallway waiting for our turn to enter the courtroom. I fidgeted while trying to appear calm and nonchalant, as if sitting outside of a courtroom was an everyday occurrence for me. My mother provided a distraction by offering me mints, tissues and newsy tidbits about the family. We both jumped when a young boy was escorted past us, the metal of his shackles clanking as he shuffled by with his arms hanging forward to where his hands were cuffed to the chains. My mother grabbed my hand and I held on tightly as we watched him being led into the courtroom we were waiting to enter for the hearing. Heat rushed through my body and I needed to gulp for air as I realized this was the monster who had set my girl afire.

My mother and I were invited in. It was not a stately courtroom like I had imagined, more like a classroom with church pews; there was no aura of American justice or majesty. We sat and listened as the judge read the name and the charges and declared the date of a trial. I was unable to take my eyes off of the boy. I thought my boring eyes would somehow be able to see what was going on inside of his being and give me an explanation for his orneriness. I was disappointed, my eyes saw no answers. I inhaled to shore up my courage, took my mom’s hand and rose to leave. We were stopped by the kind lady from Victim’s Advocacy who told me she would be calling me with further details. We walked out of the second-rate courtroom, our heavy footsteps on the old marble floors echoing the leaden beats of our weary hearts.

The days passed without any comment regarding being set afire except for occasionally when the frizzed ends of hair would not cooperate and cuddle in with the other springy curls or if she was recounting to someone how it was “really nice for the angels’ wings to bat out the fire”.

The Victim’s Advocate phoned to provide me with details for the day of the trial and informed me that as the “proxy victim” I would be allowed the chance to speak my piece before the judge.

I stewed and fretted and fussed and finally knew exactly what I wanted from the judge. I wanted this boy’s life to be forever changed. I wanted this boy to be horrified by his action of that day. I wanted him to hear how fortunate he was that angels had batted out the fire before serious harm had been done to his victim.  I wanted the judge to have the courage to turn this boy’s life on end and present him an opportunity to live a life he never imagined, a life that would free him from his unfortunate situation. Community members who knew the boy had shared with me bits of information regarding this young man’s circumstances. He lived alone with a mother who was extremely unstable. He had a history of bad behavior. My hope was that this act of pyromania would open doors for him. I carefully searched for the perfect words, crafted graphically descriptive sentences and practiced reciting my plea for the judge.

The fateful judgement day arrived. My dear sister and I sat side by side on the pew bench in the nondescript courtroom eyeing the hapless mother at the end of the row in front of us. She clung to the man who was seated beside her. I don’t know what his exact role was, but he worked sometimes at my school visiting “difficult” children. She frequently sent us hateful glares that we pretended to not see. I felt like my nervousness caused a thickness to the air and wondered how anyone else was able to breathe properly. The judge entered. The boy was ushered to a seat at a small wooden table perpendicular to the pews. He maintained a cocky tilt of his head, grinned at his mother and avoided eye contact with me. The judge called the session to order, the charges were announced and I was offered the opportunity to speak. Looking back on this moment, I hope I sounded more like the stoic Perry Mason or the passionate Eli Stone than the spurned Ally McBeal. I stood on weak legs with knocking knees, inhaled deeply and started my carefully prepared speech. When my mouth opened no words came out and a parched tongue was glued to the roof of my mouth. The judge watched me expectantly, the kid nonchalantly, the mother hatefully, the man from school sheepishly and my sister encouragingly. The look on my sister’s face, her confidence in my ability to speak my mind allowed me to try again when all I wanted was to collapse on the pew and pretend I did not have volumes to say regarding the monster who set my girl on fire. I inhaled a deep, ragged breath, wet my lips as best as possible with that parched tongue, opened my mouth and described for everyone to hear how fortunate this boy was that my daughter did not have serious repercussions from his act. I said that although she was not injured the possibilities for extreme injury caused by his actions were there. Burns to the head and neck are extremely dangerous and I described in detail why they are and how lucky she was (and he was) that none of those things happened to her even though his actions could have caused that severity of injury. I wanted everyone within earshot to realize this was a very serious offense. Next I told the judge my opinion of the options available to this boy. I said justice would be that he be held accountable for the severe action he chose that day, without considering that she ended up being fine despite his effort. I said that it would not be suitable for him to be detained in the Mary Davis Center with other juvenile delinquents who could give him more ideas of crimes to commit. I looked directly at his delusional mother and said it would be an injustice to send him home with her to continue to live in a situation which brought him to this place at this time. I pleaded with the judge to give this boy a chance at a new life, a different life, a life that would encourage him to become an upstanding, responsible member of society. I begged the judge to allow this young man the opportunity to reside at Arrowhead Ranch where boys learn responsibility, community, family, loyalty, and how to live a worthwhile life. Looking directly into his eyes,  I gave this judge a successful option. I presented this judge with the challenge to change a young man’s future. Then I succumbed to my wobbly knees and plopped onto the bench next to my sister where we sat stoically until the end of the trial and listened with grave sadness as this judge took the coward’s way and sentenced this boy to a life of imprisonment living with an unstable mother in an unstable environment that would stunt his future and never present him with the opportunities this situation could have provided him.  Evil flourishes when good men don’t stop it.

A sad day for all.

ugly duckling