21 June 2001 – 22 June 2001
See also: Benjamin
Hello, I want to tell you about my son, Elijah Stephen Guinn. He was born on June 21, 2001. He was 22 inches long, and he weighed 7 lbs, 3 oz. He was lovely and beautiful- just a gorgeous site to behold! He had velvety soft, bronze skin, a ton of jet black, silky hair, long graceful hands and feet – just perfect. There was only one problem… he wasn’t able to breathe.
From the moment he was born, he struggled to take a breath, but was unable to draw a full one on his own. He was very weak all over, in fact. This was obvious by the way he tumbled out into the world, all floppy- like a rag doll. How could this be? We had waited with eager anticipation for our first son to be born – we had so many plans and dreams for him – but this? This was not foreseeable by any means. We were very shocked.
I knew that I had a (very distant) cousin who had a couple of baby boys die in infancy 12 years prior, and she was said to have been a carrier for something called “Centro Nuclear Myopathy” (CNM). I really didn’t know much about the details of any of this, I just knew it happened. Shortly after my Cousin’s second son, Gregory, passed away, my entire maternal family was “tested” for CNM to determine whether or not they were carriers for the disorder. This was in 1988, before the gene for CNM / MTM was discovered. Based on these tests, I was said to have been a ‘carrier’. I was 17 years old. Unfortunately, I was far removed from knowing my cousin, and the concept of having children was a long way off, so I tucked this knowledge away in the back of my mind – probably thinking that I would never use it again.
In July of 2000, these same researchers contacted my cousin again, and said that they were looking over all of the data they had collected from our family, and they noticed that they had done all of the testing completely wrong. They said that all of the results were skewed and inaccurate, and that if we truly wanted to know the real results everyone would have to be retested. One problem: originally the testing was all a part of ‘research’ and so was done at no cost to us. Now, they were not researching it, so we would have to retest at OUR cost – at the hefty price of around $2500 per person. We were talking about over 30 people, so this was out of the question!!
Later that year, in September of 2000, I became pregnant with Elijah. I was elated! It had been 6 long years since I had my last child (I have a beautiful, 6-year old daughter). How thrilling!! My husband had just gone through a kidney transplant in March of that year, and, as a result, I was now pregnant!! When we found out we were having a boy, we were elated!! Our daughter, LeeAnn, was especially happy- she had always wanted a brother!!!
My mother, on the other hand was not nearly as excited. In fact, she became quite worried. You see, her grandmother had lost 11 boys just after they were born, her own mother lost a couple of boys just after birth, her sister lost 6 boys – including 1 1/2 sets of twins. This was such a common occurrence in my mother’s family. My mother, herself, had a miscarriage with her second boy at around 12-16 weeks along. So, it was no wonder that she was alarmed.
Back to Elijah – the city we lived in was very small and had only one complete hospital system, but it was not adequate enough for such a special child as Elijah. He needed a Neonatal ICU, but there was none. He also needed concise, up to date information about his disorder and on how to care for him. But, they had no information about CNM (other than the fact that it was also called, Myotubular Myopathy, and that the outlook was grim). You see, they only had very old, outdated, incorrect, (20+ year old) information on the subject. Based on this, they incorrectly assumed that there was no hope for my precious baby boy. They told us as much, and they pressured us to make a decision. They told us that all of his cells were dying before our eyes and that there was nothing we could do about it. They told us that if we ever wanted to hold him without all the wires and tubes connected, while he was still alive, then we needed to make a decision, and quickly, too – or else the decision would be made for us, by Elijah. We were dumbfounded. Was this really happening?
How could there be NOTHING that could be done for my son in this day and age, with all of the technology available? We didn’t want our baby to die! We came there to bring a new LIFE home to our family, how could this be happening?
The doctors pressured us relentlessly. We wanted a second opinion, so they called the children’s hospital in the closest major city – Ft Worth, TX. The people there also said they could do nothing for him. What??? This was almost unbelievable. No one could help our son?
We were so tired and confused. On one hand, there was our tiny, perfect baby – looking around at us, turning his head toward us when we spoke, and holding our hands. On the other hand, there were those who are the “experts”, the ones who “know” what is going on, telling us that Elijah was brain dead. What a tumultuous time. We were so tired (labor, alone, is exhausting enough – but this, too??). It was almost too much to bear. We kept putting off our ‘decision’ over and over again, trying to stall for any time that we could get.
Unfortunately, our fatigue and confusion caught up with us. Unable to bear the enormous pressure that we were under, and being massively confused, we relented, and gave the doctors a time that we would make the ‘decision’. We have regretted this moment ever since…
At 5:00pm on June 22, 2001, we signed the papers and Elijah was taken off of the ventilator. My husband and I, and our daughter bundled him up and carried him off to a private room, where we held him and rocked him and kissed him and loved him as much as we could. He was so sweet. We could hardly stand it. But we were told there was nothing else that could be done, that this was inevitable, that he was dying even while on the ventilator. Why? Why did it have to be this way? I hated the colors that he turned and watching him struggle to breathe. My daughter kept trying to blow into his mouth to give him breaths to keep him alive. It was so unbearable. I told him I loved him a million and one times, and I kissed him just as many… I prayed a prayer over him, committing him into the arms of Jesus. I sang the song “I will arise and go to Jesus” over him. It was agonizingly heart wrenching. After a while, our extended family was asked into the room and we all took turns holding him as we cried, prayed, hugged and kissed Elijah, and held him ever so tightly.
He was gone before we knew it – there was no exact moment. What seemed on one hand to last hours, and on the other hand, only for seconds, was neither. He was pronounced dead, in my arms on June 22, 2001 at 5:50pm- just 50 minutes after being taken off of the ventilator, and less than 24 hours after he was born. I was numb. I watched mindlessly as the people around me bustled about whispering, and gathering up all of his belongings, cutting off a lock of his hair, etc. It was so surreal. I reluctantly allowed the doctor to take him out of my arms… That was the last time I saw him until we prepared his body for viewing at the funeral home.
Three weeks later, my husband found the Myotubular Myopathy Resource Group website. There, we discovered, for the first time, that there were kids (of ALL ages) that had MTM and were alive and well – how could this be? Maybe Elijah’s was more severe? We quickly found this wasn’t true. That was the moment when we realized, for the first time, that we had been lied to – not intentionally, but all the same. Why weren’t we given the information that these other parents had? Where was our support, when we desperately needed it? All we had was incorrect information and assumptions, and dashed hopes and dreams, and now, an endless ache that will forever be with us… Part of all of us died along with Elijah. We learned of such painful things which young families should not have to know. The sting of it all was made even worse as we discovered that all of the pressuring and ill advice we were given was all based on wrong, outdated information.
This is one reason why we are joining forces with other families and organization in reaching out to all families affected by MTM / CNM – to help in supporting them, educating them, and helping them to connect with more families who are experiencing this disorder. We whole-heartedly support organizations like the Joshua Frase Foundation and the Myotublar Trust, as well as, the fundraising campaign of the Foye family (the “Centronuclear Myopathy Project- moving from hope to cure”) in their drives and desires to find a cure for Centronuclear / Myotubular Myopathy, and to help to educate people about it. Through these contacts, it is our hope that others will not have to go through what our family has had to endure.
The Guinn Family
Richard, Connie, LeeAnn, (Elijah)