Medical Update: A Downward Slope
I want to begin by saying a gesture: thank you. Thank you for being respectful of our privacy as we have waited to share this journey. Thank you for the constant support. Thank you for the messages when I have been quiet, checking in on me. Thank you can’t suffice, but it is an expression to try to express our gratitude. I struggled with how to write an update, only wanting to spread positivity. However, I realize the beauty in this journey is the peaks and valleys. Here is my medical update as of now, its been a slow, but steady, downward slope. A peak is coming, I will always hope for that.
Since returning from the initial transplant evaluation, at University of Pittsburgh Medical Center, it has been hard to adjust to everything. There has been a slow, yet persistent, decline in my health since this summer. It is easy to escape into the “normalcy” of everyday life and try to avoid reality. I am grateful for a medical care team that works hard to honor my wishes to remain outpatient while we go through the transplant evaluation. They are all the reason I can try to cling onto “normalcy”.
At Pittsburgh the testing and appointments were heavily scheduled. Each day was a grueling task of retelling my story over and over again… memories that are painful for my mom and me. However, it is important that no detail is missed or forgotten. Once we met with the transplant team, it became clear that my health is fragile. Due to the systemic impacts of this rare, and to this day unnamed and undocumented, medical mystery that has been ravaging my body, it makes the question of transplant become serious. The question becomes: is it a viable option? The surgeon expressed his concerns… He was worried how I do not just have intestinal failure, but a lengthy list of issues. I have bladder issues, spinal cysts, severe adrenal issues, Ehlers-Danlos due to a mutated collagen gene, a bleeding disorder that makes me bleed too much, an atrophied diaphragm…the list goes on and on. Due to this, the transplant team took a step back. It was emotional to realize what led them to currently putting a pause on listing me: the intensity of how diseased the rest of my body is. Ever since Pittsburgh, I have been trying to wrap my mind around the two possible outcomes I am now looking at in the opinion of UPMC as of now:
- The first outcome could be that I am listed as a transplant candidate, even with the concerns of the systemic disease that is ongoing. The wait for the donor call begins. Once I transition into the role of a post-transplant patient, after undergoing the major operation, I begin the months long process of recovering and healing in Pittsburgh. After the intense recovery process I will be given the go-ahead to head back to Wisconsin, this time bringing home an abdomen full of beautiful organs: a second chance at life. At this point our main goal of transplant is not to cure me, but a better quality of life.
- The second outcome could be no transplant at this time. Due to the concerns over the systemic process and surviving transplant, as well as being able to thrive post transplant, I am declared to not be a candidate for transplantation at UPMC. This choice would lead to what the transplant surgeon was discussing: shifting our focus to improving quality of life. This choice would leave us a bit heartbroken, as we feel we have fought too hard, and come too far, to accept this diseased state.I want a chance at a better life. This option would bring up new questions: Do we pursue evaluation to be listed at another center? Do we succumb to quality of life focus? Do we allow palliative care to become the main focus? Do we travel to other hospitals for more opinions? Is this the right choice?
Due to all of these unknowns, I am not finished being evaluated for transplant. Yet again, my family and I are living moment to moment. We wait for more information and decisions to be made by the intestinal team at UPMC. The tentative plan is to be admitted to UPMC for a minimum of three weeks for more evaluation. I am fearful about this due to a brand new hospital with entirely new staff, and the center being across the country from my loved ones. I am grateful I was able to negotiate out of being admitted directly that day in December, as I would’ve been kept inpatient over the holidays. There is always something to be thankful for, and the gift of making it home for the holiday season and New Year is something I have endless gratitude for.
Last week I had an appointment with my primary Gastroenterology doctor at Children’s Hospital of Wisconsin. After much discussion it is clear that the decline we saw this summer has continued. We touched bases on all of the main issues. Having to confess to the struggles I try to hide every day is emotionally painful. It came down to the main issues: pain, nausea, wound breakdown, my GJ tube, and concerns over Endocrinology issues. This Friday I will have a surgical scope, with biopsies, to investigate. I will receive infusions to prevent bleeding. They will see how my Gastro-Jejunal tube appears inside of my stomach. My GJ tube has been causing severe pain, along with severe wound breakdown. Tragically, my ileostomy wound opened up again. This wound has been opening up since its revision in May of 2017. Its been countless rounds of silver nitrate treatments to cauterize it, and trials of medical grade honey. Needless to say, it has been an ongoing battle. We will begin consulting with Dermatology to try and address the wound breakdown. My primary GI doctor consulted with the Irritable Bowel Disease team due to the discovery of Diversion Colitis at UPMC. My Chronic Intestinal Pseudo Obstruction continues to lead to complications that prove to be challenging.
Then we discussed the severity of my adrenal issues, and the adrenal insufficiency recently endured. This recent bout of adrenal insufficiency made me miss a week of schoolwork, a week of being unable to function. The labs ran at that time showed a cortisol level lower than one. However, that was not all. Last week Endocrinology contacted us regarding labs. They showed my Pituitary Gland is struggling, as well as my thyroid. A concern is that my brain is struggling to communicate to my glands. These are all new systems to be affected by this disease. It proves the need for some kind of medical intervention, sooner rather than later, to stabilize this health storm. As a result there will be more labs, along with an MRI of my brain. The signs of these issues have also been very challenging to deal with: fatigue, weight gain, fluid retention, headaches, mental confusion, lower back pain, the list goes on.
We never wish for new issues, and more systems to cease to work, but part of us hopes that having this information will help guide the teams to discoveries as to what is the underlying cause. Having a disease this rare and advanced that medicine cannot even name it, is hard. There is no road map… this journey has just been full of watching systems fail and trying to put band-aids on where we can. Whatever process this is has been an evil one. It is scary to face the unknown. A challenging part is that these diseases are invisible: meaning I appear fine. Sometimes that deceives even myself. It is a blessing, and a curse, to not immediately see the level of intensity that is happening inside of my body. I always can feel the disease working its way through my body. Despite this, I will always believe that positive actions are stronger than any disease. Therefore, I remind myself I endure these changes in order to live life. Remembering that makes all of this so worth it.
My multi-disciplinary team of dedicated care providers will have a care conference on February 2nd. My parents and myself will be present as we discuss all of the concerns and assess the decline that has happened. After presenting everything, the team will reassess and see if we can find any leads, or if any of my genetic variations could explain these issues. We hope and pray that by having everyone’s mind on my case at once, and my parents and myself present to advocate as needed, we can get new ideas. This team approach is due to Dr Gisela Chelimsky and her amazing care, rounding up everyone to help me.
One constant thing that remains a priority is to find a way to live the most joy-filled life. That will be a main factor in these massive decisions that must be made. My goal will be to live life as vibrantly as I can. I find peace in knowing I have a family, and support group, behind me that will not give up the fight for better health, and a long future ahead of me.
I choose positive and good vibes! It is important to make every little thing a victory. I celebrate the victory of working on stairs once again and walking independently. Walking is a challenge with my slow gait, but I take joy in knowing there was a time when we were worried I wouldn’t walk again. I celebrate the victory of progress in academics, despite the mental fog from pain control. The progress cannot be mentioned without expressing gratitude for the team of teachers and staff, they have supported me and have made all of this possible. I celebrate a new goal: making a small activity or outing every week. Having a trip outside of the home, or a movie outing scheduled, provides immense motivation on the days I am very sick, which have been increasingly frequent. Life is about victories and joy, not sadness or focusing on tragic events. Life is about finding the peace within the fight.
This fight is far from over, and despite further issues, I am not giving up. I have fear about the future, but I also have an immense amount of faith and excitement for what is to come.
Thank you for being there, through the good and the bad. I dream of celebrating medical victories. I pray they will be coming soon. Thank you for patience during this period of processing everything.