This is me today, minus the oxygen (because despite the fact that I’m about to write the tell all on why I’m kintrsukouri I’m still vain enough to not want you all to have *that* picture in your head).
More than 15 years ago I started having strange symptoms that no doctor could explain and since none of them seemed serious no one took them seriously. They would run tests and when things came back negative they would shrug and say, “Hmmmm… Well that’s weird! I don’t know,” and that was that.
Dropped from 19% body fat to 1% in 9 days? Weird. “Well you don’t have any parasites or infections, your thyroid is fine, and you haven’t had a reaction to any food… So the good news is you have a great excuse to go shopping! Congratulations, you’re super thin!”
Headache that lasted for 9 months? “It’s not a brain tumor and it’s not a migraine, so here’s a bunch of narcotics. They should help with the pain…”
Heart rate spiking to the mid-100s while my blood pressure tanks to 60/40? “Well… I’ve never seen that before… I really don’t know what to tell you.”
Fainting all the time for no apparent reason? “Just try to sit down before you fall down.” (I obviously didn’t think of that!)
Swollen blue arm? Unable to walk across the parking lot because one leg feels like it weighs 30 pounds more than the other? Blue-grey hands and feet? Red, lacy rash on your skin? Unable to breathe? Too weak to walk around the house? “Huh. Do you have a mental disorder I should be aware of?!” (No… But if you ask me that again the jury will agree that homicide was justifiable!)
Then about 6 years ago I was in a car accident that left me with enough of the right kind of injuries that triggered a symptom that could not be ignored: a veinous blood clot extending from my neck down the length of my left arm. As soon as it was discovered I started on an anticoagulant treatment and within 2 days was in the emergency room with pulmonary embolisms. It seemed weird that the clot would break into chunks rather than dissolve slowly, but beyond that I didn’t give it a second though, took my anticoagulants like a good patient, went to the courthouse with my soldier 6 days before he left for Afghanistan, and dealt with deployment. Nothing seemed to be improving with my condition but it didn’t seem strange to me because I thought it was just stress from the deployment. Then he stepped on an IED and we lived for the next 5 months in two different hospitals.
Stress makes many things worse, especially autoimmune and dysautonomic conditions. I didn’t know that yet, but I was about to learn all about it. As soon as my new husband was injured, my health started to decline as well. Almost every doctor I saw for myself while he was inpatient thought that I was just ill because he was in critical condition and my body was just reacting to the stress. Although I was reacting to the stress of seeing my new husband in the condition he was in, the stress wasn’t the source of the problems I was having but no one knew what to do to help me.
One night I collapsed on the floor outside his hospital room and was taken to the emergency room by his nursing staff. They called the mamma of another patient whom I was close with and she came to me in the emergency room. She was there when I was told by a nurse and a doctor in that hospital ER that I was being a big baby and making things up to not deal with what was really happening in my life. They refused to do any tests or provide any care:
“Do you know what your real problem is? You’re not dealing with your feelings and doing tests or giving you treatment won’t change that. All they will do is cover up your real problem, not fix it, and I don’t believe in covering problems but addressing them.”
Thank God for my quick-witted friend: “I see you’re wearing glasses. How do you justify wearing them when they don’t actually fix your vision just cover up the fact that you’re half blind?! How exactly are you addressing your vision when you’re just using the glasses to cover up your deficit?” I don’t know what I would have done to that doctor if she wasn’t there (I’m pretty sure she saved his life that night). I left the ER without having been given anything but a hard time and a headache.
A couple days later I had to go first thing in the morning to have my blood checked to make sure the anticoagulants were thinning the blood enough but not too much. The nurses at the little clinic I was at told me to go to the ER immediately because they knew there was something seriously wrong. Having had such a stellar experience at the one ER, I decided to go to the ER at the hospital across the street from where my husband was. It was 9:30 in the morning. As soon as the hospital staff at that ER started doing blood work they knew there was something wrong: I was on anticoagulants but there was clotting present in the blood. They ordered more tests and found that after 8 months of anticoagulation there was still clotting in my arm and there were indications of clotting elsewhere. They wanted to admit me and perform vascular surgery. I said no knowing that if I didn’t go back to my husband’s side I might lose him (that’s another story for another day).
I argued with 13 different doctors over the course of about 6 hours including a hospital administrator, the head of cardiology, and the head of surgery, and in the end I signed paperwork saying that I was aware that leaving the hospital meant I could die and they had advised me that it was in my best interest to stay. They even brought in behavioral health and legal to try to get me declared incompetent or temporarily insane so that they could treat me. This was the first time doctors recognized that there was more going on than what was on the surface and because of where my life was at that moment I had to choose between getting help for me or being help for my husband. When I was finally released from the ER it was 10:00 at night.
The stress continued to wear down my body and caused further blood related symptoms to arise. I was covered in bruises all the time to the degree that my husband was repeatedly accused of beating me; it didn’t matter that his hands had been broken and weren’t yet healed enough or strong enough to be able to punch anything because I was bruised and it was assumed he had to be the source. I could pinch myself and leave a black mark, which would seem to indicate lack of platelets, but there were clots circulating in my blood at the same time.
Two months after that long day at the ER, my husband was discharged from inpatient care and we were able to go home for him to continue recovery from there. He got better and better, little by little, and I got worse. I was covered in bruises all the time, my limbs would swell when the weather was warm and they would shrivel and turn blue-grey when it was cool, and despite having been on anticoagulation for over 2 years I was still showing signs of clotting. I found myself referred to a hematologist who recognized telltale signs and was able to test for and confirm the diagnosis of a blood disease where my blood clots very easily; we didn’t know then how easily but at least there was an explanation beginning to emerge.
Shortly after my diagnosis, we were moved so my husband could finish his rehabilitation with the best prosthetists the military had to offer. At our new home I was referred to a new hematologist who tried to remove the diagnosis of my disease, claiming it was an unrelated rheumatological condition. The hematologist both sent me to rheumatology, who disagreed with his change to my diagnosis, and he changed my anticoagulation treatment to a once daily pill that only thins the blood for about 8 hours; he only “allowed” me to remain on anticoagulation therapy because of the swelling I experienced that he couldn’t explain. For the 18 months we were at that hospital my health declined significantly.
I had been able to stay active up to that point but within a month of the prescription change I began to lose mobility and more severe symptoms began to pop up. I began to see red, lacy rashes on my left lower leg and foot; the left leg felt heavy every time I walked more than a few steps and I slowly lost the ability to stand the pain of a shoe. I would collapse randomly but frequently, passing out for no known reason. I was hospitalized when my heart rate was between 30 and 40 beats per minute but my blood pressure seemed fine, and again when my blood pressure was around 60/37 and my heart rate was normal. I would bleed an inordinate amount from small wounds and the doctors at the ER wouldn’t believe I could have lost as much blood as I knew I had; I had devised clever ways to measure my blood loss because when it would happen and I would show up at the ER they told me I was crazy and ordered psych evaluations. At one point the hospital kept me for a week just to run dozens of tests, repeatedly, because they couldn’t figure out what was wrong with me but saw a plethora of strange symptoms and I appeared to be dying. My one legged husband was perfectly capable of running, swimming, scuba diving, and doing a great many things while my ability to go grocery shopping or stand in the kitchen to cook dwindled.
By the time my husband was finished with his recovery and we were transferred to our current duty station 2 years ago, I was no longer able to wear real shoes. Most days I was not able to walk unless I was wearing compression socks or stockings. My arms, hands, legs, and feet would swell when we flew or when it was warm, but when it was cold they would appear to shrivel and turn a dark grey-lavender. The red and lacy rash on the left foot was no longer lacy or red: it had turned dark violet, almost black in places, and it covered the sole of my foot, the sides and top of the foot, my great toe, my ankle, and my lower leg. Every time I saw someone in the medical field they asked if I had diabetes because of the condition of my leg and foot; upon learning I’m not diabetic no new thoughts were ever brought forward. Open sores began to appear in the darkest spots, especially around my heel, ankle, and toe and the skin would break down, leaving a hole that would either become infected or necrotic.
By October of 2015 I was using my husband’s unused crutches and wheelchair at home and at work because I could no longer walk on my own. I began contemplating requesting the amputation of my lower leg because I knew I would have more mobility. Instead, I met a doctor who was able to diagnose the violet rash with the dying skin and give me a treatment plan; the medicine he prescribed didn’t stop everything but did make the pain slightly decrease. When I finally saw my new hematologist 18 months ago, there was no pulse in my left foot and he sent me to see a vascular surgeon to get a vascular study and surgery consult.
Many times over the previous two years I had been given ultrasounds on my left leg and every time I was told that blood was flowing so I had nothing to worry about. When I had my vascular study, the technician didn’t just note if blood was flowing: the study looked at the blood pressure and wave forms at 5 different levels between my toes and upper thigh. Although blood was flowing, the blood pressure was only about 60% of the flow in my right leg and the wave forms were pitiful. At the end of the exam he had found a 5 inch blood clot completely occluding (blocking) my left upper common iliac artery just below the aorta. The clot had to have been forming for at least 2 years to have become so complete and for my body to have made collaterals (new vessels) around the area of the blockage to provide blood flow.
My anticoagulant was changed immediately and the anticoagulant change by that doctor who wanted to toss the diagnosis was blamed for the clotting. Surgery was scheduled to remove the clot with the possibility of bypass or stent placement. When all was said and done with that surgery, the clot was removed, the artery widened, and a patch was placed to replace destroyed arterial wall. Within days the skin that had been dying on my foot and the rash began to heal; within a few months it seemed like everything was going to be okay. The wounds were healing, the rash was disappearing, and I could even wear a real shoe for a few hours everyday.
Finally it seemed we had found a solution and a path to healing.
We were wrong.
A year ago I was at my ballet studio when I suddenly lost the ability to do the simplest barre exercises: rond de jambe, dégagé, tendu plié. Just as suddenly I was able to complete a complicated floor sequence with perfect precision and form, and with the most spectacular grand jeté attitude I will ever perform. When I got home my husband asked me how ballet was and as I opened my mouth I froze. I couldn’t understand what he was saying and I couldn’t form words myself; I couldn’t close my mouth or move. I was terrified that I was having a stroke and was seen immediately but I was assured I was not having a stroke but probably just had an anxiety attack. It was 6 months later when it was discovered that I had lesions on my brain indicative of a stroke.
A few weeks later, I took a short weekend trip to The City for a wedding. When I left I was able to walk and was wearing shoes once more; by the time I returned home I was back in flip flops and needed a cane to walk. Within a few weeks of that trip I was hospitalized with necrosis on my foot once more and IV anticoagulants were used to stop the clotting. Once again, there seemed to be a reason the clotting began: changes in cabin pressure along with remaining seated in cramped seats for a long period of the time causes DVTs in persons without clotting diseases. Obviously that HAD to be the reason I was clotting once more so once the foot began to heal, I was discharged with the belief that I was once again on the road to recovery. How wrong we were.
Within a couple weeks I was no longer able to sleep because of the pain, swelling, purple rash, and infected sores that had once again taken hold on my foot. I couldn’t walk up the stairs to bed, I had to crawl. Not that it mattered if I could get to the bedroom because I was in so much pain I couldn’t sleep anyway. I would walk back and forth over the same 5 feet of carpet for hours because the pressure on the bottom of my foot felt better than resting it. When the pain was at its worst my husband would find me lying on the kitchen or bathroom floor screaming in agony, holding my foot, unable to bear the pain. Those nights were both the best and worst of nights because even though the pain was unbearable I would eventually pass out from exhaustion and pain:
I learned that passing out from pain isn’t as restful as sleep but it is more restful than no sleep.
My husband during this whole time did everything he could to help but it wasn’t much. Sometimes he could hold my foot and the pain would magically seem to disappear for long enough for me to sleep for 10 or 15 minutes at a time. Other times all he could do was sit up with me while the pain destroyed my sanity. The doctors offered me every pain relieving prescription available but there is no pain medicine that can overpower the pain the body feels when flesh is dying. The best any of them can do is dull the pain for a short time, but the longer the pain is lived with the less effective the medicine is. I heard him tell someone many months later that he was genuinely concerned that I was legitimately going to completely lose my mind from the pain.
By Thanksgiving week it was clear that clotting had to be continuing because my great toe and part of my foot were necrotic. I was hospitalized and scheduled for an emergency surgery to clean out the clot that was found in the same location the last clot had been. It was supposed to take no more than an hour and a half but it was over 8 hours before the surgeon came out to see my family. He said he had never seen anything as bad in another patient in his career. There was scarring down the length of my leg indicative of many clots over many years, and he had to perform a multitude of angioplasties down my leg and into my foot to finally allow the blood to flow to my toes. A stent was placed where the 5 inch clot had been and the arterial patch was replaced as well as it had been clotted upon extensively.
No one could figure out why I had so much clotting in my leg and foot. I was within therapeutic ranges on my anticoagulant so I shouldn’t have been clotting at all.
I stayed in the hospital for a little more than a week and was on ordered bed rest at home for another 6 weeks in the hopes that keeping me flat would allow the artery to heal and acclimate to having a stent. As had happened previously, my foot improved quickly and I enjoyed a couple weeks free from agonizing pain. But by the time it was time for a follow up vein study before being released back to work, my great and second toe began to turn purple again; I was clotting down the length of my leg once more.
The doctors were baffled and panicked. They had no explanation as to why I was clotting again and they could find no suggestions in their researching and seeking wisdom of doctors nationwide. I had my own personal symposium of vascular surgeons, cardiologists, and hematologists discussing my case because my surgeons were not going to give up on finding me help. No one had a clue what to do next so it was decided that my specialists would work together on a referral to Mayo Clinic in the hopes that perhaps they had seen another case like mine.
At the same time my referral was being written and processed, the hematologist decided to try an experimental treatment for patients with my disease that on occasion has helped stop catastrophic clotting and I was scheduled for 4 rounds of a chemotherapy designed to attack the bone marrow and b-cells that create the antibodies in my blood that cause clotting. Aside from being utterly exhausting, the chemotherapy was a complete failure.
When I made it to Mayo Clinic a month later I was hopeful that they would be able to give me some answers and solutions. Although their thoroughness was able to verify that I had had a stroke, they were as baffled by my history as every other doctor had been and ordered many of the previous tests to be redone, not trusting that they could ALL be negative. In the end, I left with more questions and no new answers. They had even made some recommendations that disregarded the clear patterns illustrated in 5 and a half years of medical documentation from 5 cities and 9 hospitals. Disillusioned and distraught I came home.
Within 6 weeks of my visit to Mayo Clinic, I was hospitalized again because my great and second toe had progressed beyond some tissue death with necrosis to full blown gangrene. This was 4 months ago. I had 5 vascular surgeries in the course of about 10 days; the first 2 were one day apart and the final 3 were also done on consecutive days. In the time between the first 2 and the other 3 my body had clotted through areas in the artery that had just been cleared. My vascular surgeons were doing everything they could to clear the clotting and save my foot.
Meanwhile, my hematologist had one last idea to treat and stop the clotting: plasmapheresis. Plasmapheresis is similar to dialysis except instead of cleaning waste from the blood all the plasma is removed from the body and replaced with synthetic plasma. The average human adult has between 5 and 5.5 liters of blood in their body; the plasmapheresis machine has 2 liters of blood in it at any given moment. Rapid blood loss is painful and dangerous, so the body goes into shock and you feel like you’re dying within 60 seconds of the machine turning on because 2/5th of the blood your body is depending on is no longer available. While the amount of synthetic plasma that is pumped into the body equals 3 liters, up to 4.5 liters of plasma can be removed, leaving your body in shock and disarray from being 1.5 liters shy of where it was 90 minutes earlier. If this sounds uncomfortable, believe me it is far worse.
The shock to my body after the first treatment was such that I was unable to move, speak, eat, or function without the machines I was connected to in the ICU. My body was so worn that when the second treatment happened the next day I was unable to lift my head or speak when the plasmapheresis nurse came to administer the treatment. On the day of the third treatment I told my nurse I didn’t think I could handle the treatment again. She asked me if I would feel that way if this was finally the treatment that would stop the clotting and allow me to live. I told her I would rather die than do that treatment once a week for the rest of my life. She convinced me to give it one more shot, telling me to think of my husband and all he endured and survived to stay with me. Inside of the first few minutes my body was so distressed that they inverted my bed in order to keep enough blood in my chest and head to (hopefully) prevent a heart attack or death. I imagine bleeding to death would have been better than what that treatment was like because keeping a body alive without enough blood is about as painful as having a limb rotting and dying for 3 years. I made it through that treatment with an ICU nurse holding one hand and a chaplain holding the other. The last two treatments were endured solely because I was too stubborn to quit on my husband.
After the treatments were over it again seemed like I was going to be well. The gangrene on my foot had stopped growing in size and it appeared the circulation was improving. I was an hour away from being sent home when I felt a sharp stab I knew all too well and my last three toes on the left foot started to turn purple. An ultrasound confirmed that I had occluded an artery in my ankle once again. The vascular surgeon was visibly distraught when he told me there was nothing else he could do for me and it was time to amputate the foot.
After 18 months of the best efforts and knowledge of doctors across the country working to stop the clotting and save my foot and life, they finally got to the point I had been waiting for and a date was scheduled.
The surgery went well. Normally patients on anticoagulation are taken off their therapy up to 3 days before the scheduled surgery in order to minimize the risk of bleeding out; I was taken off my treatment a few hours beforehand. My surgeon told me later that he hadn’t performed surgery on a patient who didn’t bleed since working on cadavers… Until me. He also said he had never seen so many blood vessels in a single leg before either; my clotting had been so extensive for so long that my body had made hundreds of collaterals to keep blood moving down my leg; my calf looked like Swiss cheese.
The surgery seemed to be successful. I didn’t have any signs of clotting, my incision was clean, my stump was long, and I was finally free from the feeling of dying tissue. At the first dressing change, my nurse wrapped the bandages too tightly, insisting that they needed to be that tight. I was in agony as my knee swelled above the wrappings and my stump burned but she refused to loosen my bandages insisting that they were supposed to be that tight. By the time the shift change happened, the damage had already been done and there was blistering along the incision. Blistering at the sight of a surgery is bad news for people that have healthy circulation; for me it proved to be disastrous. While I recovered in the hospital and began rehabilitation we watched the healing closely. For a while everything seemed to be healing, although slowly, until one day the skin along the entire incision turned to dry gangrene and had to be removed. Still the surgeon hopes my body would be able to heal and I would be able to avoid another surgery.
Because all of this was happening and could clearly be tied to clotting, I miraculously was able to get an appointment with one of the world’s leading experts on my blood disease; her waiting list is over 18 months long but she squeezed me in as soon as I was out of the hospital. This doctor poured over my entire medical history before I even had met her and when I did finally see her the appointment lasted 2 and a half hours. We went through everything. All the strange symptoms I had been dealing with for years were finally addressed and explained as she diagnosed me with another handful of rare conditions that, she explained, were all ultimately caused by blood clotting that had gone on undetected and untreated for years. A few changes to my medications and a new anticoagulant (that has not been approve for my disease) finally stopped all the symptoms of ongoing clotting. Thank God she made time to see me instead of asking me to wait another 18 months for help. I don’t think I would have lived to see her if I had had to wait.
Meanwhile, the open end of my stump began to rot. I could smell the smell of decomposition coming out of my skin. I could taste it. Eating made me sick and I felt like death. But because my temperature never went over 100F and my blood cultures looked like I was healthy, no one was willing to do anything; I should mention that this was when my surgeon was on vacation. The day he returned he saw me and didn’t even get the bandages off of my oozing, gaping wound before declaring that he would be seeing me first thing the next morning for an amendment (shortening) and debriding the wound. During the surgery more blood cultures were taken but as before they showed no signs of any major infections. Bone and tissue samples that were rubbed on petri dishes, however, showed 2 antibiotic resistant infections: a strong strain of staph and a plant based pathogen that is rarely seen in humans but when it is seen is a devil to eliminate.
Since that last surgery and the discovery of the infections, I have been sequestered at home, quarenteened, out of fears that I will get further infections or illnesses from the general public; I am allowed to go to the doctor and that is all. It is not that I was allowed out and about when I was first discharged from the hospital but at least I was allowed to have physical therapy outside of my livingroom. My stump has shown no signs of infection but the healing has been so slow that I have a wound vac to keep the area clean, dry, and pulled close to help encourage healing. A home nurse will come by at least once a day to administer antibiotics via my chest port, check my vitals, take blood samples, and change dressings on the port or the vac when needed.
Despite all that has improved and the answers I have finally gotten about my blood condition and the other conditions I’ve developed because of it, I am still a mystery to my doctors. Being limited in my activities and location, being closely watched daily for weeks going into months, and having regular blood workups have highlighted some other interesting symptoms in my body that have yet to be diagnosed. It is amazing to me to know where I have come from to get to where I am now with my health and even more so to realize that despite the answers I still manage to pose new questions.
I guess I shouldn’t be surprised I am such a mystery: I always knew I was something special. The other day I decided to find out how special I am and worked up some numbers with the incidence of 7 of the 8 rare conditions I have (no data exists for the incidence of the 8th). As it turns out, I am 13-hundred-millionths of 1% of the approximately 7.5 billion people on this planet likely to have these 7 conditions; had I worked in the incidence of common conditions the number would have been even smaller.
This makes me 1 of about 10 people (statistically) on Earth living with what I do every day.
I didn’t find it upsetting or surprising even to realize exactly how special I am. In a strange way I find it comforting. Being so rare explains why it has taken so long to get answers and honestly I am just happy that I survived long enough to get them.
This is me.
This is my life.
And this life is beautiful!