Lupus, ITP, and Antiphospholipid Antibody Syndrome

My story is a little different in that ITP isn't the only chronic illness I have. In 1989, I was diagnosed with systemic lupus erythematosus, a chronic, incurable autoimmune disease where the body makes antibodies against any soft tissue in your own body. In my case, I had more inconvenient symptoms than life-threatening: extreme fatigue, rashy with discoid lupus lesions, and significant joint pain. These symptoms were brought under control with anti-inflammatory medicines and plaquenil, an antimalarial. After one year, I was able to taper off of the NSAIDs, and function essentially as prior to the onset of disease.

In 1992, my disease took a severe downturn. People at work asked how I got the large bruise on the back of my leg. I was surprised; it was a huge, purple bruise about 5 inches in diameter. I excused myself to the restroom and found a number of smaller bruises on my thighs. I was concerned, but not worried. I returned to my meeting, and on three occasions in the next couple hours my nose began bleeding. I returned to the restroom and saw bruises on my ear lobes when I removed my clip-on earrings. I went to my office and asked a couple of friends if they thought I should phone my doctor. One of them asked about the 'rash' on my wrist. On both wrists, I had bright red dots (which I learned later were petichiea). They encouraged me to call my doctor, just to be safe.

My rheumatologist who had been treating me for the previous 3 years for lupus returned my call within 10 minutes. I described to her about the bruises, nose bleeds and rash. She said, quite calmly, come to my building and go up to the hematologist's office as soon as possible for a blood test. I hung up the phone, grabbed my purse, and told the secretary I'd be back in an hour or so.

A blood test revealed that my platelet count was 8,000 and I was told I would have to be admitted to the hospital immediately. During my 5 day stay at the hospital, I was put on 100 mg of prednisone, which raised by platelet level from a low 2,000 to about 37,000. A bone marrow biopsy was done on the second day confirmed that I was producing platelets.

Over the next six months, I was unsuccessful in tapering the prednisone below 20-25mg while maintaining a platelet count of at least 80,000. My doctors were apprehensive about risking a drop below 60,000 because of the original severity of my ITP. I was told that I should consider having a splenectomy. At the time, I could find very little information that contraindicated a splenectomy, but was very apprehensive. After three months of research, I agreed to the splenectomy.

Two weeks before my pre-surgery consultation with the surgeon, I started to experience severe pain in the calf of my right leg. Over the course of the two days, the pain began to be constant, and woke me at night. It became nearly impossible for me to walk at all on it. I called my rheumatologist again.

My rheumatologist asked me to come in immediately. She saw me soon after I arrived, and examined my leg. My calf was swollen to more than twice the normal. I was hurried over to the hospital for a scan of my leg; a large clot was found and I was admitted to the hospital. I can recall my mom saying, when I called to tell her I was in the hospital with a clot, "No, we are worrying about you bleeding." Thus, I began to learn more than I cared to know about antiphospholipid antibody syndrome.

I learned that antiphospholipid antibody syndrome, or APS, can occur by itself or secondarily with other autoimmune diseases such as lupus. Characteristic diagnosis of the disease includes evidence in blood tests of one or more of the following: lupus anticoagulant, anticardiolipin antibody, false positive syphilis test, and false positive Russell viper venon test. Symptoms from APS include thrombosis, thrombocytopenia, and recurrent miscarriages. When the thrombocytopenia and thrombosis occur together, it becomes imperative to control the ITP because the only treatment for clots is take blood thinners. Also, APS is chronic (ITP and clots can reoccur at any time), so therapy must be for the duration of life; requiring therapeutic doses of blood thinner prophylatically.

I was convinced by my doctors that since I was now a 'clotter' too, a splenectomy was especially called for because, even with the 50% success rate, it was a risk that must be taken to at least slow down the destruction of platelets. So, to make a hugely complicated story more abbreviated, the initially successful splenectomy failed after 5 months. Now, on 60mg of prednisone, my doctors recommended Imuran. After a month of research and a month of suffering on 60mg, I reluctantly agreed. The Imuran was very successful. Over the next 4 months, I tapered down to below 5mg and my platelets helped steady at over 350,000. I continued to take the blood thinner to prevent clots from developing.

In 1994 I approached my rheumatologist about getting pregnant. She arranged for me to see specialists at Johns Hopkins and the National Institute of Health. I coordinated a team of a high-risk obstetrician, a hematologist, and my rheumatologist. I was put on a relatively new blood thinner, low molecular heparin, that even the doctors at JHU and NIH only had limited experience with. I was counseled on all of the possible outcomes, including death - for me or the baby. With the very thorough guidance and monitoring of my doctors, I did have a healthy baby in 1996.

The baby's platelets were normal at birth, but to be careful, the neonatologist ordered that the baby continue to be monitored over the next 3 months. I have been told that what happened next is very rare. Within less than a month, my baby's platelets had rose as high at 850,000. The neonatologist and pediatrician actually had predicted this: evidently, the baby's system, while in utero, had overcompensated for the antibodies that had passed throughout the placenta. Then, as the maternal influence on the baby wore off, the baby's platelets gradually returned to normal. It is still amazing to me - the wonder of a healthy human body to overcome!

It is now 1998, and I have lived with the potential 'time bomb' of controlling my body's fight over bleeding and clotting. I have experienced a total of four clots in my leg and clots in my lungs; the last clot was while I was on coumadin, an oral blood thinner. This is not necessarily unheard of, to clot while 'coumadinized', but fairly rare. So, now I must take daily heparin injections, a much more effective blood thinner. I continue on the Imuran and a minimal dose of prednisone.

There is so much more known about antiphospholipid antibody syndrome than when I was first diagnosed. I, as well as my doctors, have learned and experienced a lot. It continues to be frustrating as well confusing; to live everyday wondering if a bruise will pop up or if I feel the pain of a clot. I don't think the fear will ever go away. I try to focus on the fact that I have a beautiful baby, and still am here to enjoy life. I feel a great compulsion to communicate my experiences, and to help those facing similar challenges as I have and continue to do.

Tricia (THANKINS@loudoun.gov)