When I Was Diagnosed By My Own Dr. Shaun Murphy
One day I sat down and researched the incidence of all my wild conditions and used a little high school statistics to figure out the likelihood of a person on Earth having all the conditions I have. The number I arrived at was staggering: I am one of 1.2 people likely to have all my conditions.
Someone I shared that number with scoffed at me saying it was a terrible and depressing thing for me to discover but I disagreed. I certainly understand now why it is so difficult for doctors to get a handle on what is happening with me when I’m such a rarity. I call myself a “Baby Pegasus Unicorn” (BPU) of a patient because I am that rare. And I’m perfectly OK with that.
Livedoid vasculopathy (LV) is not connected to or caused by other conditions. What it looks like initially is levido reticularis: a vasculitis that looks like red to purple mottled, reticulated, lace-like patterns on the skin that is caused by swelling of venules and capillaries due to micro-clotting. One doesn’t have to have a diagnosis of a blood condition to experience microclotting: the smallest capillaries and venules in the body are three to four µm across while a single red blood cell can be five to 10 µm across, so even a single red blood cell that is oversized, two cells together, or very thick plasma (from a condition or from dehydration) can slow or stop the flow. When that happens in an entire area along a limb or the torso, it can cause levido reticularis. Most of us have seen it in fair-skinned folks who have gotten cold or in babies when they feel a draft. Initially it was the diagnosis I received when a red, somewhat lacy spot that changed shapes seemingly randomly appeared on the top of my left foot. While levido reticularis isn’t supposed to be particularly painful, my rash was. I knew something was wrong, especially when my symptoms worsened.
Within several months of the first patch of red lace appearing on my foot, several other patches appeared around the same foot, onto the ankle, and even eventually in a string like pattern around my calf. The spots were extremely painful to the touch and nothing seemed to soothe them. Still my doctors insisted it was “just levido.” When the middle of the spots started to turn purple and little weeping ulcers appeared with black spots of necrosis in the middle, like craters, every doctor I asked was baffled. No one had a clue what was going on with my simple case of levido reticularis. I did everything I could to heal the ulcerations but nothing standard worked. Wearing compression stockings did seem to help a little, but when they healed it seemed to take forever and I was left with white star — like scars that almost looked like a flake of thin wax on my skin.
By the time the necrotic ulcers and white scars started to appear, doctors became wary of using the words levido reticularis, because it simply wasn’t accurate to what I was experiencing. Of course I had already begun to quit wearing regular shoes at all because of the pain. They put pressure on the ulcers all over my foot, and the use of mobility aids such as a cane, crutches, or a wheelchair became normal.
When I was finally sent to a new dermatologist right after relocating to a new state, I found one of the partners was autistic and had one of those incredible memories like Shaun in ABC’s “The Good Doctor.” I cried the first time I watched an episode because I swore the character could have been based on my doctor, and it broke my heart to think that so many brilliant minds might be swept aside because of the word “autistic” in a chart or file. This partner wasn’t actually the doctor I had been assigned, but she was stumped and asked if she could take a picture, then sent it to someone. It was my very own real-life Shaun. He recognized it immediately and could recite verbatim the definitions he had seen and read about this condition. He knew exactly what tests to perform to verify the diagnosis. He also began a treatment plan before the diagnosis was even confirmed because he wanted to see me get better.
Yes, I was saved by my very own Shaun Murphy.
I had only experienced the LV symptoms for 18 months, but the pain was so severe and the damage so vast in that short period of time that my entire lifestyle had to change with it. I couldn’t be active and be at the gym regularly when I could barely walk in my own home. I was hardly able to make it through the day at work, so I began having to rely on a housekeeper to keep my house clean because a housekeeper costs less than a personal shopper and chef (I still had to do those things). At first, the treatment plan I was allowed me to sustain all the activities of daily living we all hold dear. It was miraculous, but it didn’t last. By the end, I couldn’t sleep through the pain and even the breeze from a ceiling fan on the next floor up or the heat radiating from my dog or cat walking past would leave me screaming in agony. The damage from the LV along with damage from widespread catastrophic clotting that had taken place due to another condition (antiphospholipid antibody syndrome) was so extreme, I ended up having my left leg amputated at mid-calf. It was high enough to cut away all the LV and tissue that had been damaged, but low enough to allow me to have a long residual limb, commonly called a stump. It was only 20 months between my diagnosis of LV and the amputation of my lower leg. I thought then I was done with the LV, but sadly I was wrong.
About nine months after my amputation, some pink lacy patterns began to appear on the end of my stump coinciding with the use of a negatively pressured socket designed to help with healing. I knew immediately what it was because it had been a part of my life for so long, and a part of the cause for my world being completely turned upside down. While I know many amputees experience levido reticularus, I haven’t met a prosthetist who has fitted an amputee with LV.
Of course I showed the LV to my prosthetist and my surgeon, but they both pooh-poohed me and said, “It’s just a little levido.” Although I know there is a vast difference between levido reticularus and livedoid vasculopathy, I’ve only ever met the one amazing doctor who knew what it was. I barely understand this condition myself, but here I am — the expert in the room with everyone I’ve ever met save one.
I know at some point my amputation may need to be shortened, or this condition will keep me from being able to wear a prosthetic for long periods of time — if at all. I know right now I am one of a very small number of people who do know what this disease is and what it does. I am one of an even smaller number of people who know what it is like to be an amputee suffering from it.
How can I expect the other experts in the room to understand this diagnosis, let alone its implications when it’s not something they have ever seen before or will likely ever see again?
How can I expect other people with LV to ever be able to get a diagnosis — especially considering that of the hundreds of doctors I’ve met with all my “weird and wild” conditions, I’ve only ever met one Shaun Murphy.