It never left. It must have been there before I had my first flare. I remember as a young woman getting blue fingers at times but it was only an inconvenience, poor circulation I thought. Now I know that getting those blue fingers is called Raynaud's and it can often be one of the many symptoms of lupus and scleroderma.
The illness made itself known in 1988. I began having problems with my normal stamina. Climbing stairs or any mild exertion began leaving me strangely winded. My bronchial felt dry and tight. I had episodes where my breathing muscles were clenching from my diaphragm upward and my pulse was hard in my abdomen. I was admitted to UBC hospital in Vancouver.
As an apparently healthy twenty-six-year-old woman without any known condition, I was stricken by something that made no sense. A strange and terrible collection of symptoms developed: severe resting tachycardia of 120 which was double my normal, extreme abdominal pain, hair loss, a seizure and other neurological problems. At that time, I was told I had an autoimmune disease affecting soft tissue and blood vessels. The internist called it Mixed Connective Tissue Disease. He said my antinuclear antibodies (ANA) were high. It had a speckled and nuclear pattern, the latter was seen in high frequency with systemic sclerosis.
Somehow my neurological system was being affected. At one point, I had a seizure and lost consciousness. Then I began having trouble controlling my facial muscles. My neck and spine would vibrate. I could not rest. They ordered an electromyography to be done on my muscles and the report came back indicating a myopathic process.
While in the hospital, I was put on Propanalol to lower my heart rate. The doctor talked about the use of antimalarials and blood thinners but started with Prednisone. It did not help so the doctor took me off it. Only now do I know that steroids are often ineffective with systemic sclerosis and perhaps the reason the doctor was talking about blood thinners was a little known condition called Antiphospholipid Syndrome (APS) . Instead of using blood thinners, he chose aspirin four times a day as it would additionally have an anti-inflammatory effect.
At that time, I hadn't known about APS. It is a blood disorder where the blood clots too readily. In its severest forms, it is called Catastrophic Antiphospholipid Syndrome. Obstruction in the large blood vessels can occur and an acute respiratory distress syndrome can be a major feature early on in many patients. The twenty year experience they have with this syndrome has shown that the central nervous system is particularly at risk and that neurological problems in general are associated with it. Now years later, I have the suspicion that this might be at least part of the reason why I had struggled with neurological problems, why they arose again years later in the same fashion and why it wasn't discovered by my small town doctor who had no information on my health history. CAPS is extremely rare. Close to half of those affected die soon after the syndrome begins. Strangely, if one survives the initial stages, complete recovery is frequent.
It has been almost two decades later that I have found out I am positive for anticardiolipin antibodies which is a type of antiphospholipid. This clotting disorder seems to be a part of what has been for me a primarily vascular, neurological, and respiratory disease--and shows itself in the strangest ways. Recently, I had a wisdom tooth pulled and when I removed the gauze after an hour, there was only a tiny spot of blood. It did not even bleed through to the other side of the gauze. That would be a good thing you would think. And it would be if I felt well. But I suffer continuously with breathing problems and my system at its very core level does not function normally any more.
When I was twenty-six I was lucky, in a small way, to the extent that it remitted for the most part. I was given sixteen years to live my life. Whatever this mixed thing was, it faded. A few months after it had begun, the extreme distress abated. The last thing that hung on was the difficulty with my facial muscles but that finally went away as well and I put it all behind me. Only now do I regret being lax with what was insidious. I did not want to believe that it could ever return like that again. But it did. A whole sixteen years later and with vengeance. And now, in the age of the internet, I read somewhere that it can remain mild for twenty years and then a medical crisis can occur.
In hindsight I know that it had never truly left me. It was always there, a ghostly illness lurking in the shadows. Although it had settled down for awhile, gradually it was making itself known again. Little things creeping up here and there. More and more I noticed a weakness in my bronchial. I began to dread flu season as I would have difficulty recovering and would always seem to get what I thought was bronchitis. But it wasn't that. My bronchial's were always dry. It was as though the ability to produce normal mucus secretions was diminished.
Sinusitis would take hold and I noticed that vascular abnormalities were becoming more pronounced. I ignored my heart pounding hard and fast at times while laying in bed at night. There was no rhyme or reason to it. It would just work hard at times. And the blue fingers, well now they had companions, blue feet. The real strange thing was that it wasn't just due to cold temperatures. My hands and feet would sometimes turn blue in the warmth of the house or car. I joked about carrying around a barbecue chicken in the grocery store because my fingers were white and although I tried shaking and rubbing them, the circulation just would not get going again.
One time I developed what a doctor in Prince George thought was mumps on one side. I told him I had the mumps when I was a kid. But he thought I had them again. I had noticed that every so often I was starting to get a weird blurry dizzy like feeling on the right side of my head just above my neck and behind my ear. It was unsettling but I shrugged it off. There were a few times that I had been plagued by painful bladder cystitis that was not related to any infection, just another part of the mystery. These seemingly disconnected problems were probably related. Somehow, I knew that it all had something to do with that autoimmune thing but I did what many people would do, ignore it.
No one wants to accept an illness but that was the worst thing that I could have done. I moved around a lot and I did not take my health history with me. I did not even mention it to doctors. I simply wanted to forget it. It was not going to come back.
After all those years with only small signals that something was wrong, the MCTD came out of its apparent remission.
Again I began losing my stamina. I had the same seizing up of breathing muscles and the vibrating down my spine. I developed a strange weakness in the blood vessels just below my thyroid. When I bent down to pick something up it felt as though the tissue was sagging. When I tried to started the lawn mower, my heart would pound extremely hard and I would get a hard pulse in the front of my neck where I had the weakness.
Everything escalated. My nose felt hot and dry and developed thick bloody mucus. I had severe breathing problems in my upper bronchial and my blood pressure remained high. I was in the hospital for a week and the doctor said a heart enzyme test was positive. Later, he said it was a false positive. He said I had tracheaitis. I wanted to get home after a week of being on Pulmacort four times a day in the hospital. It didn't help me though and again I found myself on Prednisone--and again it didn't help and again I was put on a heart drug, this time Metropolol. It lowered my heart rate but not my blood pressure and still I was in distress. Breathing problems, abdominal pain, I could not even walk around.
A wheeze developed in my upper bronchial area and I developed a cyst on my thyroid where I felt a lot of throbbing pain in the blood vessel near the area. I had a low grade fever, started soaking my clothes and sheets at night. Later I was told I had a staph aureaus infection. During all of this, the only way I could sleep (and it wasn't anything near what one could call sleep) was with sleeping pills. They would only keep me out for three or four hours. I would wake up with my heart beating hard and fast. (I was no longer on the Metropolol). My breathing continued to be labored and I now was starting to lose control over my hands and arms. My balance and coordination were going as well and I had the same abnormal hair loss that I had when I was twenty-six. This time around things became much worse. I developed shooting pains in my head and then I started having severe chest pain.
Because I was suffering so much, I went, in desperation, to the hospital saying: "I think I have anxiety disorder" just to get in and get some kind of help. I might as well have signed my own death sentence. After that I lost all credibility and my stay in the hospital was a nightmare. The nurses knew something was terribly wrong. I was soaking through my clothes and sheets. My heart rate was 125-150. I had low grade fever. But the doctor, for whom I at first had the utmost respect, led me down a dark path where I took drugs that had nothing to do with my illness and made me even more sick from their side effects. As a forty-two-year-old woman I would learn the bitter lesson that some doctors have little real concern for their patients and that they yield a lot of power that they sometimes abuse. Lesions on my MRI were cast off even though I have learned that they were written up as possibly due to small vessel disease or vasculitis.
I hold extreme regret for the day I walked into the hospital and said those six little words. But I had tried to fight for three months on my own. Struggling with Pulmacort, Metropolol and sleeping pills that only put me out for a few hours. Still, I fought and tried to go to physiotherapy where the therapist took one look at my dark purplish fingers on my left hand and said: "I think you should go back to your unit and show them."
One nurse said to me, "You need to go to a larger hospital. Something more is going on than we know." I knew that. They knew that--but the doctor? He ruined my life. He said to me things like: "You have had every test! There is nothing wrong with you. No doctor will see you." I do not know what he wrote down but I know it affected the way I was treated afterwards.
I left that hospital in a wheelchair. Today, I am severely disabled, in part due to the underlying condition and in part because I had forced myself to take drugs that had severe adverse affects.
Sometimes, I wish I had never left Vancouver. Then at least I would have had my history and a doctor who would have known me for years, a doctor who saw me give birth to my children and shared in my joy. One who could put the puzzle together. One who cared.
It is a hard lesson to learn-that some of those people in power are not so nice as we'd like to think. We want to think that the justice system is just, that doctors are people of good moral conduct, compassionate and thorough. Yet if you speak with friends or browse the internet, you sometimes hear startling stories that shake up your whole belief system.
Protect yourself. Make sure that you get copies of your test results. Know your health history and take it with you when you move.
Remember this: Some test results reported as normal are not normal at all. One instance is relayed by a doctor who routinely uses Holter monitors on his patients who suffer from M.E. (Myalgic Encephalomyelitis) The cardiologist or computer is basing the report on the number of ischemic events (which is not related to the heart problems caused by ME.)
I had often wondered about my Holter monitor test. When this came out of remission, I knew my heart rate was constantly high and it would beat hard for no reason yet obviously it was being reported as normal. Also, do not let your doctor shrug off your positive ANA results if you are indeed suffering. Yes, it is true that a small percentage of normal healthy individuals can have a positive ANA but it is only about five percent, and then, too, it is usually low titres. It certainly is not a big deal if you feel well otherwise. But if you are having problems and levels are high, then it means something.
If I can make others aware that the medical system is not so cozy as they might have once thought; if I can spare others from walking painfully down a dark path of uncertainty; it will not save me from my suffering, but it will provide satisfaction in knowing that others might be spared.
I still do not have any proper answers. I have my own theories as to what went wrong and I am waiting to see a rheumatologist. I have a second-hand document that gives my first diagnosis at twenty-six. It says: Dx: Mixed Connective Tissue Disease Ddx: Lupus, Systemic Sclerosis, Hyperthyroidism and Post Viral Syndrome. It mentions an EMG and a myopathic process. I wish I had had this little summary when I first went to the little hospital where I had the positive heart enzyme test. Then maybe it would not have been disregarded as being a false positive. Maybe then someone would have had a clue. Maybe I could say it never left, this mixed thing, and I am okay. But it never left and I am not okay. Maybe someone else will be.
Email: [email protected]
Story edited 10-11-06 JTD
Story posted 10-26-06 SLE
ISN Senior Artist: Sherrill Knaggs
Story Editor: Judith Devlin
Antinuclear Antibodies (ANA)
Mixed Connective Tissue Disease
Types of Scleroderma
What is Scleroderma?
SCLERO.ORG is the world leader for trustworthy research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. We are a service of the nonprofit International Scleroderma Network (ISN), which is a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team, or Volunteer. Donations may also be mailed to:
International Scleroderma Network (ISN)
7455 France Ave So #266
Edina, MN 55435-4702 USA
Email [email protected] to request our Welcome email, or to report bad links or to update this page content.