EDMARC HELPS A HAPPY LITTLE GIRL COME BACK TO HER FAMILY
~ by Peyton’s Mom
On August 8th of 2006 our first daughter, Peyton, was welcomed into the world. She was tiny at only five pounds, but she was beautiful and perfect otherwise. It didn’t take her long to catch up; eating was her favorite experience. She could never get enough. She was a happy baby, usually smiling and cooing to anyone whose attention she could catch. She was everything we could have dreamed of and more.
Our experiences raising Peyton were amazing, she brought so much light into our lives. The joy that we felt as her parents was unexplainable, but underneath that joy were also some concerns. You see, as Peyton grew, there were some signs that she was not developing as typically as most of her peers. She was becoming ill more frequently, developing strings of infections and viruses, and, other times, and spiking frequent fevers with no identified cause. Around the age of two her muscles began losing tone, and she began lagging in milestones. Despite the increased difficulties she was facing, she was still the happy baby we had loved from the moment we knew of her existence.
By the age of three, things started to change rather quickly for her. She began losing stamina, often opting out of playing, even during activities that she loved. When she was up to it, she tried her best to give it her all, but when she was down, she spiraled quickly. She had a much tougher time recuperating from illnesses and had even begun to lose some previously mastered skills. It was becoming clearer by the day that something was wrong, but no one knew what, not even her doctors.
At three and a half, Peyton had stopped consistent eating and drinking. At first it was hard to tell if it was just a stage, or something more concerning. But it didn’t take long to realize there was more to it. She had started becoming dehydrated and it was clear that Peyton, who used to love food, was no longer feeling hungry. There were so many changes in our little girl that were coming so fast, but nothing would prepare us for what lay ahead.
Not long after Peyton began struggling with her ability to eat and drink, she began having seizures. She was diagnosed with complex partial epilepsy and placed on antiepileptic medications. It seemed as though we had just fallen into a landslide we couldn’t get out of, and up until that point, no one could tell us why. It was then that a geneticist told us of her suspicions that our daughter could be suffering from a genetic disorder called Mitochondrial Disease.
Mitochondrial Disease is a metabolic disease that affects individuals at the cellular level. It is genetic, it is progressive, and often fatal. In individuals with Mitochondrial Disease, the mitochondria in the cells are defective and cannot provide enough cell energy for proper cellular function. Without properly functioning cells, you cannot have properly function organ systems. It is a multi-systemic disease that is often neuro-muscular in nature.
While Peyton went through the proper testing process to diagnose or rule out Mitochondrial Disease, her body continued to struggle. She was in and out of the hospital for gastrointestinal issues (GI) issues, hypoglycemic events, and difficult-to-treat seizures. Her nutritional struggles were getting harder to manage, and she had begun to lose weight as a result. The decision was made to place a temporary feeding tube, a NG tube, while we waited on the results of her testing.
At four and a half, we found the answer why our sweet girl was struggling so much, and much to our sorrow, the geneticist was correct. She was suffering from the effects from Mitochondrial Disease, a disease which had very few treatment options, and no cure.
Even after learning the name of what Peyton was struggling with, she still had such immense battles to face. There were so few doctors who understood the disease and so few places for us to turn in order to understand what was going on in our daughter’s small body. She had a G-tube placed for nutrition and hydration and, by that point, had completely lost all desire to eat and drink. Her G-tube feedings often failed, as her belly would fill with formula that could not be digested.
After meeting with one of the top Mitochondrial Disease specialists, and after several inpatient stays at CHKD, it became clearer that our daughter’s digestive tract was quite affected by the disease. Mitochondrial Disease often affects the organs that require the most energy. Running a GI tract on mitochondria that are dysfunctional would be like trying to run a car on a battery that needs to be replaced. Though it is important to point out that the disease affects everyone differently, most of our daughter’s affects were in her gastro-intestinal tract and neuro-muscular system. She had developed gastro paresis, meaning her stomach did not empty in a timely manner. This made it difficult to feed directly into her stomach. She also had slow colon motility.
She eventually had a special feeding tube placed to bypass her stomach and feed directly into her small intestine. We thought things would begin looking up for her from there. Unfortunately, that would only shine more light on the problems our daughter was facing. She began to have chronic pseudo obstructions, places where the peristalsis or contractions in her small intestine would stop, causing portions of her small intestine to fill with air and fluid. This was not only a painful experience, but one that would often prevent her from being fed as it would cause her nutrition to actually reverse its flow into her stomach. Over time the episodes would come closer and closer together and it became more and more painful for her to be fed through her gut. She was becoming weaker, losing weight, having more frequent seizures, and not functioning well at all.
By the end of 2011, when she was five years old, Peyton ended up in a series of lengthy hospitalizations in order to try any means to maximize her enteral feedings. By the beginning of 2012, it was clear that her GI tract was no longer able to function well enough to provide her the nutrition, electrolytes, or dextrose that she needed for survival. The difficult decision was made to place a permanent IV or central venous line so that she could receive IV nutrition, dextrose, hydration, and medications. It seemed that Peyton’s body was becoming more frail by the moment. The little girl who once spent her days twirling herself dizzy now spent most of her days hooked up to IV’s and needing supplementary oxygen in order to catch her breath. By February of that year, she had developed significant autonomic dysfunction, chronic bone marrow suppression, and a T/B cell deficiency, along with her already difficult-to process-disorders. There were days we feared how much more she could possibly take.
Unfortunately, in late February, that fear would be tested. Peyton developed a life threatening blood stream infection from bacteria seeping from her digestive tract. She was at a higher risk for this type of infection due to the fact that she could no longer be fed through her gut. Bacteria was more likely to build up and break through the delicate vessel walls as opposed to being washed through her digestive tract as it should be. The situation was unavoidable, but one that put her at a high risk for life threatening infections no matter how careful we were.
She ended up developing sepsis, which took a toll on her body. Sepsis is a full body inflammation that occurs as a result of a blood stream infection. It leads to poor circulation and low perfusion to organ systems. In short, it made her already fragile body even sicker, much sicker than we had ever seen her.
Her condition led her doctors to want to do everything they thought they could to try another large effort to rehabilitate Peyton’s GI tract. This would not only prove to be one of the most painful experiences of her life, but one that ultimately showed just how fragile she had become. Not only did the tiny volume of feedings dilate her entire digestive tract with air, pushing her bowels into her chest cavity, but she also developed hepatitis from the work that her liver was being asked to do, functioning as her only working digestive organ. It was clear at that point there was very little anyone could do to force Peyton’s body to function as it should. It was one of the scariest times of our lives, one in which we realized for the first time we weren’t sure how long she would be able to put up a fight against Mitochondrial Disease.
It was then that our pediatrician called us, unexpectedly, to meet with him without the kids. We weren’t sure what to think, but we did know one thing — in all of our years as parents, we had never been asked to show up without the kids. It certainly made for an uneasy situation. When we arrived at his office he spoke with us each gently. He shared with us his concerns regarding Peyton’s rapid decline and her uncertain prognosis. Then he said something that, as parents, we never expected to hear. It took the air clear out of my lungs. It was a feeling I will never forget. He told us that he wanted to refer her to a pediatric hospice program called Edmarc. At that moment, all five years of her life flashed before my eyes. If it had ever been evident and tangible that we could lose her, it was then. Little did we know, that referral would not be indicative of an imminent end, but the mark of a new beginning for our daughter.
It wasn’t long after hearing about Edmarc for the first time that we would begin using Edmarc’s services. In a small room, my husband and I sat on one side of a table, and on another sat our pediatrician and a couple of members of Edmarc, who we would eventually come to know as our daughter’s biggest allies. I came slightly guarded, unsure what to expect, not quite grasping yet that we had all come there for the same reason, to protect Peyton’s best interests.
They started off by asking us how they could help and what we needed. I gave the same response I gave most health care professionals that asked at that time, which I believe was just a short list of things that would make her care more manageable at home. They were unsurprised by the needs and promised to do their best to see that they were met. For the first time in her life I was not being asked to do the advocating, but instead we had a liaison who would be helping us to make sure our daughter was receiving the proper care. I was finally able to relax a little.
It was only a short time after meeting with Edmarc for the first time that we realized they were not only going to be there to help with her increased nursing needs, but also to help provide Peyton with something that she had been lacking for quite some time — a proper quality of life. Living in and out of the hospital for years had been difficult for all of us, and though we tried our best to maintain a childhood for her that every parent hopes for, the truth was it was far from anything we had wished for her, no matter what we tried. Though there was nothing to blame but the disease itself, it was way beyond time for her to have everything she deserved, and she deserved so much more than the life she was living.
With the loving guidance and approval of the social worker Edmarc appointed to our family, we found it possible to help Peyton figure out her own desires and hopes within her life. Things that were important to her to accomplish and things that she wanted for herself, as well as things that she did not. Edmarc helped her to find her voice, one that had been lost amongst the commotion of people trying to fix her body and forgetting about her person.
Edmarc is so much more than what meets the eye, so much more than what we thought was being introduced to our daughter’s life those years ago. It is a full-on comprehensive approach to building quality in the face of tragedy. It is the flicker of light that was all but lost when hope seemed just outside of our grasp.
It has nearly been three years since we were referred to Edmarc. Peyton is now eight years old. There is no way we could have predicted the positive outcome it would have in Peyton’s life. For a service that most people think of when they think of the end of life, pediatric hospice services has only brought more life to our daughter. When Peyton needs a pick-me-up, she knows that Edmarc will be there to support her. Be it through annual festivities, personal visits from Santa, or a special trip to a local venue, they know how to make her and her fellow Edmarc recipients feel like a child should, one with as few worries and pains to focus on as possible.
Though Peyton’s disease still greatly greatly affects her, and she requires more medical intervention now than she did when she began services with Edmarc, I can say with the upmost gratitude that she is stable. Her hospitalizations have been reduced from an average of twelve or more a year, to one or two. Edmarc and its team of nurses help to keep both our wishes and Peyton’s to stay home as much as possible at heart. This has been one of the biggest blessings to her life.
Over the past three years, I have watched our daughter’s confidence and self-esteem flourish as she realized through Edmarc’s help that what she wanted was just as much, if not more important, than what everyone wanted for her. I truly believe this is one of the biggest reasons our daughter has renewed her fighting spirit. She is now the same happy little girl she was all those years before her disease tried to stifle that. She is the same happy girl we caught glimpses of for those years, but longed to hold onto before those moments slipped back into misery.
A few weeks ago, when she was not feeling her best, I was back and forth on the phone with one of her favorite Edmarc nurses. As we formulated a plan to get her back on her feet, she lay there staring at me, definitely not her typical self. She was pale, her eyes were at half mast, and she was devoid of energy. When I got off the phone she looked at me and said “Mommy, I just want you to know that even when I am sick, I am still happy.” At that moment my heart filled with an emotion I had not felt in quite some time. I felt complacent. Of all the difficult choices we had to make in her life, ones with such uncertain outcomes, we were tangibly seeing the positive result of a choice that was made for her, a place we could not have gotten to without Edmarc’s help. Despite the fact that Peyton will continue to live out her days with a chronic and debilitating disease, she can live well. She can live a positive life, a memorable life, because of Edmarc.