“Few people outside medicine realize that what tortures doctors most is uncertainty, rather than the fact they often deal with people who are suffering or who are about to die. It is easy enough to let somebody die if one knows beyond doubt that they cannot be saved – if one is a decent doctor one will be sympathetic, but the situation is clear. This is life, and we all have to die sooner or later. It is when I do not know for certain whether I can help or not, or should help or not, that things become so difficult.”–Henry Marsh, Do No Harm: Stories of Life, Death and Brain Surgery
I have Ehlers Danlos Syndrome, a genetic connective tissue disorder that affects every fiber of my body, mind and spirit. I was misdiagnosed for thirty-five years. It wasn’t until my two year old daughter exhibited extreme symptoms, and I fought for her like I never had for myself, that I found answers for my own suffering. After her first Chiari decompression failed and her little cervical spine became more unstable I set out to find the best care possible.
It was world renowned neurosurgeon Ben Carson’s book, Take the Risk: Learning to Identify, Choose and Live with Acceptable Risk that guided me in those adrenaline filled months of searching for any kind of help and hope for Danica. The entire book addresses medical, spiritual and practical life situations, but it was these four questions he always asks himself when facing an important surgical decision that helped me most:
1. What’s the BEST thing that can happen if we do operate?
2. What’s the WORST thing that can happen if we do operate?
3. What’s the BEST thing that can happen if we don’t operate?
4. What’s the WORST thing that can happen if we don’t operate?
Danica’s case was complex, and she was not even three years old. All the neurosurgeons we consulted with agreed she needed another decompression and that she needed fusion but only one team of doctors, a neurosurgeon and a brilliant orthopedic surgeon, were willing to actually TAKE THE RISK to give Danica the best chance at some kind of childhood. This risk saved her life.
There was a period of several months between a commitment to operate and the actual surgery when one of the surgeons took a step back, and we thought the chance for help was gone. There is an incredible amount of trust needed on both sides of these relationships. I thought he was reconsidering the odds of success in Danica’s case and us as parents. I later found out there were all kinds of things happening behind the scenes at the hospital and in his personal life that had nothing to do with us. I began to understand dedicated physicians are professionals making critical life or death decisions and then heading home to a family and all the important and mundane things this requires. I approached every new doctor I met with this newfound compassion and respect.
Danica’s surgery was groundbreaking. The surgeon I mentioned above created a 3-D model of her skull and fusion hardware that would grow with her tiny head and neck. Because it was a teaching hospital dozens of orthopedic and neurosurgery residents learned from her case directly and through the resulting medical literature. The very personal risk we took and the professional risk the doctors and hospital took resulted in many lives being changed.
These same surgeons recognized the similarity in Danica and I. They sent us to genetics for a diagnosis that would change everything. I finally had an answer for a lifetime of my own widespread pain. Once Danica was stable enough in her recovery I began my own search for doctors who were willing to take the risk on me.
This began with a general practitioner who sat across from me while I sobbed my story, looked into my eyes and believed me. Over time the trust we built in one another became critical in my care. She stretched the boundaries of the recommended and acceptable doctor-patient relationship, because I needed this level of involvement. She fought for me in situations I simply could not advocate for myself. This commitment was not hers alone. At every point her husband and young children were sacrificing something personal as well. She ran a one doctor practice. I was a patient who needed more than a twenty minute appointment every sixty to ninety days. I needed her sometimes weekly. I needed pre and post-surgical care. I needed her to research and try and understand all my comorbid conditions. I needed referrals and coordination of care with genetics, cardiology, hematology, urology, gynecology, endocrinology, oncology, neurology and neurosurgery. I taxed her and her small staff in every possible way, but she never wavered in her support.
I’ve seen the most dedicated specialists in the United States to treat my Chiari, Cervical Instability, Tethered Spinal Cord, Tarlov Cysts, Intracranial Hypertension, Autoimmune Encephalitis, Endometriosis, Dysautonomia/POTS, Mast Cell Activation Disorder and Bleeding and Clotting Disorders. Every one of these doctors has stepped outside what could be a comfortable, career climbing and lucrative practice of medicine to treat patients like me. They are as rare as the zebra patients they care for. They listen closely, examine the entire body and try to understand the systemic result of Ehlers Danlos Syndrome.
Over the past few months one of my trusted surgeons has faced a firestorm of criticism for some of the decisions he’s made. I’m able to look at the situation from many different perspectives. I understand the position of a large hospital and peer review and the vulnerability of everyone involved. I also know for sure this one man’s willingness to TAKE THE RISK to try and try again and again to find something sustainable to bring relief for my intracranial hypertension SAVED MY LIFE. There was no one who was willing to take on EDS patients and our specific vascular neurosurgery needs. We were suffering. We were hopeless. I was so hopeless I wanted to die. He humbly sat by my bedside when something failed and would rethink the anatomy and the engineering, and he wouldn’t give up. He was the crack in the doorway shining a little light on the wasteland of pain. Would medicine ever move forward without this kind of courage?
I’ve had more than several dozen surgeries of all kinds. Every time I’m given a rundown of the possible complications and risk. I sign my name. My husband or father or sister or friend, whoever happens to be with me that day, watches me sign my name. I’m grateful for access to their specialized care. I’m grateful they are brave enough to operate on someone as high risk as I am when literally no one else will.
Two hundred and forty days ago I had my last surgery. It was my third in two months, and I was desperate. My surgeon came and proposed something new, and I was all in. Yes, I trusted him. But I also trusted the hospital to support whatever decision my doctor and I made together. I expected them to support us that day and in the weeks and months following.
Much like when Danica’s surgeon stepped back from her case for a time, I don’t pretend to know all of the personal and professional things happening behind the scenes of this particular situation. But I do know it’s not easy for anyone involved. I hold the hospital more accountable than my own surgeon, because he is human and they are a large organization with a responsibility to back him up when something gets really hard in his family or his practice. He is a pioneer, but he is not rogue. I also hold patients like myself accountable. This delicate relationship only works if we are all trusting one another.
I’m now faced with new information about the surgery I accepted then. I know I made the exact right choice for me that day. I know I’ve had the most well life possible because of that decision. I’m needing to make a new decision about the most recent shunt and placement of it in my body and the risk involved. And this is okay. My family and I are asking ourselves the same questions above that we used to decide about Danica’s surgery so long ago.
In all the confusion and the new recommendation for removal of my lumboatrial shunt I reached out to my Ohio general practitioner, and she called me on Sunday, her day off, to discuss. I emailed my counselor, and she called me and spent an unscheduled hour to talk me through it all. I texted another trusted neurosurgeon who’s been inside my body over and over again even placing several lumboperitoneal shunts early in my IH journey, and he texted me back.
One of the most critical and controversial issues in my care is the ability to access specialists when faced with emergency situations. I believe this too has saved my life. In a perfect world our doctors would receive timely information through HIPPA approved channels of communication, but this just isn’t happening. For a special group of people the rules can’t apply. The physicians need to be trusted with the discernment to give their cell phone number to someone who might never be connected through the answering service. They need to be able to send an email and check on someone post-surgery. When the majority of their patients travel from all over the United States and even the world they understand this kind of follow up is the most practical. This kind of personal medicine works. This too is a risk.
Dr. Carson writes in the aforementioned book, “Faith by definition is a risk.” I have moments and even hours where I feel peace about the new surgical decision I think God is asking me to make. Admittedly, the rest of the time I am a wreck. The most wonderful thing about the foundation I stand on through all this is I cannot be moved even when I am doubting and certainly not feeling what I should. My God is unchanging and nothing I say or do alters His care for me. I am safe. My days were written when there were none of them. Danica is safe. Delaney is safe. Dan is safe. We are resting in the shadow of the Almighty God. No one can pluck us from His hand. Carson continues to write:
“We all have the choice. But only when someone takes the risk of faith can he or she begin to experience the best consequence and the best rationale I know for belief in God. That’s the privilege of a personal relationship with the Creator of the universe, who wants to offer His wisdom and guidance to help us deal with all the other risks we face in our dangerous world.”
We are all going to die. I’m more aware of the fragility of life, because I’m faced with inherently riskier situations. But my time here will not be cut short. I won’t be taken too soon. My purpose will be fulfilled.
I have Ehlers Danlos Syndrome, a genetic connective tissue disorder that affects every fiber of my body, mind and spirit, and I’ve had access to doctors and surgeons who are courageous enough to TAKE THE RISK to give me this precious life.
I don’t take a minute for granted.
Please pray for my family and I. This is something we did not expect to be facing. Now that I’m in Tucson the difficulty of travel to the east coast doctors who treat me is much more expensive and difficult. I am not privately insured. These were both huge calculated risks we took in moving here. Over the next few days I will be making a decision, and we will need your help. Please pray for the surgeons I appreciate and care for so deeply. Please pray for the other patients making hard choices too. Thank you. Our Hope remains.
(Dan and I were able to grab sweet moments of live music in Tuscon last Wednesday and see the band Need to Breathe in concert. Much of their music is part of the soundtrack of our lives, but this song made me cry. If God is on our side who can be against us?)