Recurrent TTP in a Young Woman who was Initially Diagnosed with ITP
Bridget first noticed bruises on the tops of her feet while visiting relatives over Christmas break during her junior year in college. A few days before she had noticed her urine was a reddish color, suggesting to her that she may have blood in her urine. She was feeling tired and the bruises on the top of her feet could not be explained. She had never seen a doctor since she had been a child, so she planned a visit with her pediatrician.
Her doctor ordered a complete blood count (CBC), which revealed that her platelet count was low (40,000, normal is over 150,000) as well as her red blood cells (hematocrit 24%, normal is over 38%). She was admitted to the hospital that day, July 12, 1989. Another blood test confirmed the low platelets and anemia.
When she saw a hematologist at the hospital, she had not had any neurological problems, such as memory or visual problems, and she had no kidney abnormalities except for the blood in her urine. She did not feel sick; she had no symptoms except for the bruises and fatigue. In order to rule out bone marrow cancer (leukemia), she had a bone marrow biopsy. Bridget describes this procedure as a large needle that is pushed into her breast bone and then removed quickly. She recalls that this was quite painful and afterwards it felt as if it was hard to take a breath or two, but then her breathing returned to normal. She did feel sore in her chest for a week. The bone marrow was normal. (Bone marrow aspirations are now usually done in the hip bone.) This led to a diagnosis of ITP (idiopathic/immune thrombocytopenic purpura). The treatment of ITP begins with steroids to try to boost the platelet count. Bridget was treated with a common steroid, prednisone. Her initial dose was 60 mg daily, but it was then increased to 160 mg daily.
After 15 days in the hospital, Bridget’s platelet count had returned to 190,000, within the normal range. She went home, but the steroid treatment was continued with the intention of gradually decreasing the dose. She was also given immuran for two months as an additional treatment for the ITP. Over the course of a year, Bridget decreased her prednisone dose from 160 mg per day to a 3 mg daily dosage. She stopped prednisone after 12 months
While the steroids did boost her platelets to within the normal range, Bridget had many side effects. Over that year she suffered from memory and concentration problems. She also experienced violent mood swings, one in particular where she threw a fit at her doctor. She also experienced stomach pains. In addition, she had some physical changes which included the formation of a hump on the back of her neck, and her face became round and puffy. Because of all of these problems with the steroids, Bridget dropped out of college for a semester. In her own words, Bridget exclaims that "steroids drive you nuts!"
Over the next six years, Bridget was normal. Although it took about a year for the side effects of the steroid therapy to wear off, her body and mind returned to the way they were before treatment. She did, however, state that once you have thrombocytopenia (a low platelet count), "You are always afraid that you are going to crash again". During this time, Bridget finished medical school, was married, and gave birth to a healthy baby girl without complications.
Interesting is the fact that during this time, while she was in medical school, Bridget sought out information about ITP. She also ran across a different, yet similar disease, TTP (thrombotic thrombocytopenic purpura). While the information on TTP was brief, it was clear to Bridget that TTP was a much more serious disease. This insight into Bridget’s thoughts becomes important on June 28, 1995. Bridget was visiting relatives when she again noticed bruising and fatigue, just like before. She also had a new symptom, one she hadn’t had with her "ITP" 6 years earlier: she was experiencing blurring of her vision. She immediately assumed that this was a "crash" and was prepared for another round with ITP. She began prednisone on her own and then spoke to a hematologist by telephone. Her assumption was that this was simply another episode of the ITP, and since she was a doctor, the hematologist assumed that her diagnosis was correct. When Bridget arrived at the city hospital the next day, the hematologist met her in his office.
The CBC was repeated, demonstrating an extremely low platelet count (7,000). Again, she was also anemic (hematocrit 25%). The hematologist was puzzled by the anemia; he assumed she must have had intestinal bleeding caused by the low platelet count. Also, Bridget looked sick and sweaty; she vomited in the office; she was admitted to the hospital. Then the doctor looked at Bridget’s blood cells with a microscope. He was shocked to see that, in addition to the absence of platelets, all the red cells were severely broken and fragmented. This was not ITP; this was TTP! Therefore he thought that her first illness, 6 years ago, must also have been TTP. That was probably why she was anemic then. In fact, her doctors in 1989 had considered TTP but had dismissed this diagnosis because of her response to prednisone, without plasma exchange. Typically, plasma exchange treatments are essential for patients with TTP.
Bridget did not look very sick when she entered the hospital, but in fact she was critically ill. Her serum LDH level was extremely high (2823, normal is less than 200), evidence for severe blood cell destruction. When asked the difference in treatment between the first episode and the second, Bridget remembers hearing TTP and being very afraid. Also she says that instead of just administering steroids and "hanging out", the doctors and nurses seemed to be hurrying. A surgeon was called to put in a large venous catheter in preparation for plasma exchange therapy. Bridget describes the placement of the femoral (in the groin) catheter as the least traumatic option for catherization. When asked about the catheterization, she describes it as a "sharp pain". Plasma exchange was begun immediately.
Bridget describes her first experience with plasma exchange to be about four hours long. She had six or seven units of plasma put in through one tube and the same amount removed from another. She said it felt as if she had a tingling or shaking feeling throughout the four hours experience.
In addition to plasma exchange, methylprednisolone, an IV steroid, was also given. She received plasma exchange once a day for eight days. At this time, her platelet count had risen to 232,000 and had been in the normal range for four days. She stayed in the hospital for two more days to ensure recovery, and was discharged on July 7, 1995. Her doctor had planned to continue with intermittent plasma exchange after she left the hospital. Because of the risk for infection with a venous catheter in the groin area, it was removed and a new catheter was inserted just below her collar bone. But it was the wrong size of catheter- too small! Plasma exchange requires a large catheter; the surgeon had put in a small catheter, the more common size used for antibiotic and chemotherapy solutions. Instead of putting in another catheter, her doctor decided just to wait and see if Bridget’s platelet count would remain normal. It did.
At this point, Bridget knew she had just recovered from her second episode of TTP. Yet she was able to return to her work and family life quickly. She had no symptoms of TTP. She was once again in remission. She did however begin to notice some changes in her ability to concentrate, and her memory was a little hazy at times. She needed to look things over a couple more times to remember them. Although these subtle symptoms were occasionally troublesome, she continued to become an excellent physician. She says, "I just had to try harder."
One year later, in June and July of 1996, Bridget was in the later stages of her second pregnancy. She was aware of the risks of a relapse of TTP associated with pregnancy, but she was confident because her first pregnancy had been uncomplicated. To be safe, her doctors checked her blood counts often. On June 26, her platelet count was 190,000, but on July 2, her platelet count had dropped to 139,000. While 139,000 is close to normal, the change over just seven days was scary. Bridget was very concerned about another recurrence of TTP but she was determined to not receive plasma exchange while pregnant and expose her unborn child to plasma. She was only 33 weeks pregnant; seven weeks to go. Over the next six weeks her platelet count gradually decreased to 51,000, but the count did not fall every week; sometimes it increased a bit. Then on August 21, Bridget had a healthy baby girl; on that day her platelet count fell to 11,000, her hematocrit fell to 28%, and her LDH level increased sharply to 683. She also had difficulty concentrating and had blurred vision. All of these abnormalities indicated recurrent TTP. Plasma exchange was begun and continued for six days. Her platelet count steadily rose to 218,000. She had survived her third episode of TTP.
Bridget is a very intelligent and insightful doctor. She successfully combines her professional role with her responsibilities as a mother and wife. But she believes that her experiences with TTP have given her a "low freak-out threshold." Every time she feels a little off-kilter, she is worried that she is having another episode. Every time she feels this way, she tries to sleep, and thinks that this seems to help. When asked about herbal remedies, she responded quite quickly with Billberry tea. She uses this tea as a preventive measure. When asked about the effectiveness of the Billberry tea, she said that it doesn’t hurt, and she hasn’t had another episode of TTP, so it will be continued.
Bridget believes that the biggest effects of TTP on her life are that her illness has caused her to be more tired, she has to read things two or three times to remember them, and she is worried about relapses all of the time. It is constantly in the back of her mind. She calls it a "health-related paranoia". She always wonders if she is crashing again. But she also believes that these experiences have made her tough. The advice she has to offer to those who have been recently diagnosed with TTP is that, "You CAN get better if you think you can." And that the best thing you can do is to find someone else who has the illness, in order to support each other.
Bridget Update: July 2003
Since her third episode of TTP, Bridget has led a normal life. Career-wise, Bridget is an excellent physician; health-wise, she is doing well. Although Bridget says that it is stressful to be a working mom with two active children and a professional husband who has his own career, she is happy with her current situation.
When she gets tired, fatigued, and feels abnormal, she gets a check-up, which includes a complete blood count (CBC) and checking the red blood cells under a microscope for any signs of broken, damaged cells. Fortunately, the lab values and examinations have always been normal. Now, Bridget thinks about TTP on a weekly basis, whereas a couple of years back she would think about it daily.
Given her experience with TTP, Bridget finds it most helpful to have a doctor whom she trusts and who knows what to do with TTP. She asserts that TTP-patient group meetings are also beneficial because of the shared experiences, the opportunity to talk to doctors who are knowledgeable about TTP, and to learn the latest information about TTP.
With respect to alternative medicines, it is important to note that the herbal Billberry tea is specific to Bridget’s case. Bridget discussed Billberry tea with her doctor, and together they weighed the potential benefits and harms (see comments by Dr. George). Bridget emphasizes that it is very important that if a patient is using herbal remedies she must talk to her physician so together they can best evaluate whether the specific herbal therapy is beneficial or harmful, since herbal chemicals may adversely react with prescribed medicines.
Looking back at her experience with TTP, both as a physician and as a patient, Bridget offers a suggestion that has helped her. She emphasizes the importance of keeping good track of one’s symptoms. When she feels abnormal, tired, fatigued or sees any symptoms of TTP, she gets a check-up, including a CBC. Bridget believes that “if you have had TTP you can walk away from it” if you keep a good track of your symptoms and are familiar with the signs and symptoms of TTP. Therefore, if something feels wrong and “your symptoms” appear, get a check-up.
Bridget Update: September 2006
Despite being very busy with her family and her medical practice, Bridget has been doing very well during the past three years. Her family life is “fun”, and her medical practice is going well despite being stressful at times.
Bridget continues to be in good health. In fact, her last episode of TTP occurred ten years ago! She says that some weeks she doesn’t think about TTP at all, but during stressful times she thinks about it more often. “I don’t think I worry about it anymore---I just hope I’m never a fourth time repeater.” Bridget does get “paranoid” when she gets tired or bruises easily, however. At these times, she gets a CBC (complete blood count) to check her platelets, to prove to herself that she is okay. Over the last year, Bridget had her platelets checked twice, and they were above 200,000 (in the normal range) each time.
In Bridget’s last update in 2001, she said that one of the biggest effects of TTP on her life was that her memory and concentration had diminished somewhat. Today, she still has some memory and concentration problems at times, but she doesn’t have a problem like she had experienced before. Bridget thinks that any issues she does have with remembering things and staying focused is probably 90% related to her busy schedule as a physician, mother, and wife.
Bridget has an interesting perspective in that she has experience as both a patient and a physician. She likens having TTP to someone who has had a stroke. “You always kind of worry whether you will have another one. You never see it coming and often can’t do anything to ward it off anyway. I’m like my patients who have had strokes in the past; the farther away they get from the episode, the less they worry about it.” She only wishes doctors and patients knew how to prevent TTP and its relapses. Bridget encourages people with TTP to teach their doctors what the disease is. Most doctors have not cared for patients with TTP, so it is important for the patient to find a physician who really knows about the disease and how best to treat it.
Bridget Update: March 2011
At this time, Bridget says, "I don't have much to add. Luckily, I've had no more episodes of TTP to report. The further away from the episodes I get, the less I worry - or even think about TTP. My baby will turn 15 this year, so it will be my 15th anniversary since having my last TTP episode. Sounds like a success story to me!"
Comments by Dr. George
Although in some patients it may seem that both TTP and ITP have occurred, careful review of the records from Bridget’s first hospitalization suggests that her first illness was also TTP. Her doctors thought she had TTP from the appearance of fragmented red cells on her blood smear, but when she responded completely to prednisone, without plasma exchange, they concluded that she had ITP. However responses of TTP to prednisone alone have been described. Her second episode was more typical, with mild neurologic abnormalities. Although we often describe patients as having TTP-HUS, Bridget only had very minimal evidence for kidney involvement. The problem with her second pregnancy was puzzling. Was this a third episode of TTP? That certainly had to be the first assumption. But the slow decrease of her platelet count during the last 7 weeks of her pregnancy was also consistent with the incidental thrombocytopenia of pregnancy, which occurs, in 5% of healthy pregnant women near the time of delivery. She did not have high blood pressure, the sign of preclampsia, which can be a cause for more severe thrombocytopenia. When her platelet count fell to 11,000 on the day of delivery, accompanied by a decreased hematocrit, increased LDH, and neurologic symptoms, the diagnosis of TTP was clear and treatment for TTP was essential. However, plasma exchange may not have been appropriate if Bridget had previously always been healthy, if she had not previously had TTP. In otherwise normal women thrombocytopenia, sometimes even this severe, can occur around the time of delivery, and in almost all women the platelet count recovers without treatment after delivery. In a woman with no previous illnesses, it may have been appropriate to wait several hours, or even a day, to see if these abnormalities may spontaneously improve. The key issue with Bridget was that she had had TTP twice before. Therefore her doctors were confident that she had TTP again.
The Billberry tea was not my recommendation, but I did not discourage Bridget from using it. Doctors must appreciate, as Bridget certainly does, that many patients who have difficult diseases try a variety of remedies. These may be called alternative or complementary treatments; they may be herbs, diets, physical manipulations or spiritual experiences. Patients must tell their doctors they use these treatments, and doctors must understand their potential value and risks. My view is that if Billberry tea helps Bridget, I support her use of it. I know of no harm that it can cause.
With all of her experience as both a physician and patient, Bridget is insightful and always very positive. She is an excellent physician. Her patients are lucky to have her as their doctor.
Update 2011: Bridget continues to come to our Support Group meetings occasionally. Some years ago, she said that she was reluctant to come to these meetings because they stirred her bad memories of TTP episodes. Now she's past this concern and she's a wonderful contributor because she has the wisdom of both perspectives - patient and physician.
[First written July 2001. Last updated March 2011.]
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