WHAT HAPPENS FOLLOWING RECOVERY FROM AN EPISODE OF TTP?
Before we talk about recovery, we must say that some patients with TTP still die. Most of our patients who died never had a chance for effective treatment; patients who have begun plasma exchange treatment have almost always survived. Survival is much, much better than before PEX began as a treatment in the 1980s. Before that only 10% of patients survived. Survival is even more likely in 2015 than it was only 10 years ago, because of the more frequent use of steroids – sometimes in very high doses – and also rituximab when the initial response to PEX is not sufficient. We expect that over 90% of our patients will survive. But, of course, this expectation is for patients who are diganosed promptly and treated properly.
Initially, the greatest concern of TTP survivors is whether they will have another episode, described as a “recurrent episode” or a “relapse”. Ten years ago we estimated that 35-40% of patients would relapse. Now, with more frequent use of rituximab, we estimate that only 20-30% of patients will relapse. Most recurrent episodes occur within the first year following recovery from an initial episode. But one of our patients had her first relapse 14 years after her initial episode. We do not think that there is any good way to predict the risk for relapse. Some doctors measure plasma ADAMTS13 activity when their patients are completely well, with no health problems and normal blood counts. They feel that a very low value predicts a high risk for relapse. We measure ADAMTS13 activity once a year in our patients, and we do not think that low activity, even undetectable activity, is a good predictor for relapse. Patients who do relapse almost always do very well, because their disease was responsive to treatment the first time and because there is no delay in diagnosis the next time.
The most important part of preventing serious problems with a relapse episode is immediate medical care. Common symptoms that suggest a relapse are the same symptoms that a patien had with his or her previous episode. These may be bruising easily, dark urine, trouble with concentration, headaches described as a migraine. The symptoms may only be a sense of weakness and fatigue. The symptoms may be similar to common illnesses - stomach pain and diarrhea, fever and cough. For any and all of these symptoms, the most important thing to do is to get an immediate platelet count (usually described as part of a "complete blood count" or CBC). Then the next important thing is to learn the result immediately - not the next day. If the platelet count is low (less than 150,000 or more importantly less than 50,000), hospital care is immediately required.
We began group meetings for TTP survivors in 1996. These have been very successful and they have continued two-three times a year since then. Perhaps the most important benefit of these meetings is for patients to better understand what TTP is. TTP is rare and unfamiliar to almost everyone – therefore it’s very difficult for our patients to talk about TTP with their family and friends. The opportunity to talk with each other at these group meetings is very help and very reassuring. This has been a great help to our patients, to better understant what happened to them and to understand that other people have had the same experience. This removes a sense of isolation, which is a major problem for people with rare disorder.
For us, the most important result of these patient group meetings is to better understand the long-term problems that some of our patients have. Twenty years ago, before these meetings began, we thought that recovery from TTP was complete – that the only risk was for relapse. We have learned from our patients that some, maybe most, patients do not feel that they have recovered completely, physically and mentally. They tell us about persistent issues with memory, concentration and endurance. Because of these issues we began annual evaluations of our patients in 2004. We have done some different tests each year.
From these tests we have learned that recovery from TTP may not be 100% complete. We have learned that some of our patients suffer from severe depression. Then we have been able to help them understand what depression is and how it can be effectively treated. We help them find counselors or doctors who are experts at managing patients with depression. We have learned that some of our patients have persistent problems with memory and concentration, exactly as they had described to us. We describe these as cognitive problems. We have worked with occupational therapist and psychologist to know what to suggest for our patients, but the things we can do are very limited. Mostly, we recommend simple things like keeping lists, writing everything down, trying to simplify and organize their lives. There are not disabling problems. Out patients have returned to their normal jobs and activities. The issues are that things may be a little more difficult.
We have also learned that some of our patients have minor but persistent kidney problems and that this can lead to high blood pressure. We have learned that some of our patients develop another autoimmune disorder – systemic lupus erythematosus (usually called lupus).
Because TTP most often occurs in young women, some of who are pregnant when the first diagnosis is made, the question about safety of subsequent pregnancies is critical. Our experience is that TTP can recur during pregnancy, but this is uncommon. Most women have uncomplicated, successful pregnancies. During the past 20 years, 10 of our patients have had 17 subsequent pregnancies. Fourteen of these pregnancies resulted in healthy babies (one woman had early miscarriages with both of her pregnanciesl; another lost her pregnancy at 20 weeks). Only two women had a recurrent episode of TTP. In both of these women, the TTP recurred 1 and 5 weeks after they had a healthy baby, and they recovered promprtly. This experience has taught us not to discourage women who wish to become pregnant. We recommented very careful prenatal care because other complications of pregnancy, such as high blood pressure, seem to occur more commonly after recovery from TTP.
The most important thing we have learned from following our patients for up to 20 years is that many patients need some continuing advice or support. Not all patients need continuing support; some patients seem truly, perfectly health - with no depression, no cognitive problems, no high blood pressure and no problems with subsequent pregnancies. But we feel that most patients do require some advice and support over time. The most important thing that our patients can have is a good doctor who is sympathetic to their problems and who is accessible when problems occur.
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