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 1 of 1.2 people likely to have all my conditions.
All of them.
1 person of 1.2 people out of somewhere in the neighborhood of 7,530,103,737 people living today on this Earth.
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. When we think of rarities in this world, as far as animals are concerned (after all, we are animals too) creatures that are endangered and those that I know have gone extinct. The last male northern white rhino died recently; sure some scientists may have been able to find some way to somehow bring back their numbers but it is still sad to think about. I could literally name hundreds of animals that in the last few hundred years have gone endangered or extinct, but none is likely as famous as the Dodo bird.
The Dodo is the unapologetic mascot of the extinct and the obsolete. Somehow I don’t find it to be very majestic so you will excuse me if I find for myself a much more appropriate mascot for myself…
A Baby Pegasus Unicorn
I am that rare… And I’m perfectly okay with that. How rare am I?
I am a writer on The Mighty right now, a place to find voices on all things chronic and rare. Yet, there is no place for me to designate that I suffer from a condition called Livedoid Vasculopathy. Fantastic. I’m the first one on The Mighty, I guess!
Score one for the BPU.
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 microclotting. One doesn’t have to have a diagnosis of a blood condition to experience microclotting: the smallest capillaries and venules in the body are 3-4 µm across while a single red blood cell can be 5-10 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 so 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 caused by putting 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 that 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 that the character could have been based on my doctor and it broke my heart to know that so many brilliant minds would 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 and 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, and he had a treatment plan beginning before his diagnosis was even confirmed because he wanted to see me get better.
Yep, I was saved by my very own Shaun Murphy.
I had only had 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 (so someone had to do those things). At first with the treatment plan I was on I was able to sustain all the activities of daily living we all hold dear again. 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 that I ended up having my left leg amputated at midcalf; 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 9 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 that 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 that 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 that at some point my amputation may need to be shortened or that 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 does and of an even smaller number of people who know what it is like to be an amputee suffering from it.
Being a subscriber and a writer for The Mighty, you might think that I would find a lot of patients who, like me, find the sunny positives of a life of chronic illness and disease but sadly that’s not the case. I understand that my condition is rare enough that no one really wants to spend money, time, or effort researching ways to treat and overcome it; there’s no money to be made in that. I understand that there is no reason to blame the doctors who missed this diagnosis before I met my Shaun Murphy because I can hardly expect anyone to hold every word they’ve read on every rare condition captive for just the right moment to magically save the day when there will probably never be another patient to walk through their door with it after me. I was frustrated and angry when I couldn’t get help but I didn’t quit looking for it and when I found it I didn’t quit trying to save my foot either, I gave the doctors a chance to do their best to save me.
In all that time I could have let myself become embittered or disenfranchised with all medical providers because something rare continued to be unrecognized. I could have become one of those chronic patients who angrily pickets in online forums and social media, who writes about the complete and utter failure of all areas of healthcare because I did not simply stick with modern medical practitioners or practices while seeking healing, or who bemoans every attempt to treat similar symptoms as utter failures to be avoided, touting my experiences as a degree in everything not to try. If I did, I’d be a dodo.
*chortle… get it?!*
It is foolish to be angry because people I sought help from couldn’t help me. They didn’t know what they didn’t know, it wasn’t that they didn’t try.
Holding onto the anger from something like that is like drinking poison and expecting the other person to die: it only hurts you.