ITP in an Adolescent Girl That Has Persisted for 23 Years
Christy was eleven years old in 1984 when her mother was diagnosed with Hodgkin’s disease. Only a few weeks later Christy came down with strep throat. It was at this time that she also began to notice bruises under her waistband and on her legs. After one evening at volleyball practice, bruises were all over her arms, "They were everywhere!" Christy was taken to her family doctor. He did a blood count that revealed her platelet count was down to 2,000 (normally the platelet count is over 150,000). The platelets were so low "They thought they had done it wrong," says Christy. The second blood count confirmed the low platelets. But otherwise, Christy was completely healthy. At eleven years old, she was diagnosed with ITP.
"At this time, it was more of a worry for my parents," Christy remembers. The doctors then wanted to do a procedure called a "bone marrow aspiration". This test is sometimes done to rule out leukemia or another cause of a very low platelet count. "This was absolutely the worst thing," Christy adds now, years later. To add to the fear of an 11 year-old, her mother had undergone a bone marrow aspiration the previous month. She remembers this as the "horrible, most painful thing."
Things that she remembers from this time in her life are visits with her hematologist in Tulsa, blood tests each week, staying in the classroom during recess, and basically "knowing she couldn’t do anything." In addition, she was taking prednisone. As a side effect, she gained 30 pounds in about two months. But it had no effect on her platelets, so she stopped taking it after two months. Even though her platelet count continued to stay very low, at about 10,000 to 15,000, Christy made the decision to continue to do the things she loved. Even though it was against the advice of her doctor and parents, she played softball and basketball. But she did have some complications from her ITP. Once, while swinging at school, Christy fell and hit her head. Her head was extremely swollen and she was rushed to the hospital where they gave her a platelet transfusion. Tests showed that she had no bleeding in her brain, but her face bulged with a big bruise, so she stayed home from school for the next week.
Two years after her diagnosis, at age 13, Christy had a continuously low platelet count, always about 10,000, but she had few bleeding or bruising symptoms. But because her platelet count was so low, and because she was an active teenager, Christy was advised to try another option: splenectomy. She underwent the surgery to have her spleen removed in 1986, and the surgery occurred without complications. After one week, her platelets jumped to 100,000 but then dropped back to 10,000 after another week. It was initially exciting when the platelets rose, but when they dropped, "It was disappointing to everyone, even the doctors." She thinks the splenectomy was a "huge waste of pain, suffering, and an organ." In her words, this whole ordeal was "a let-down".
At age 15, in 1988, Christy received a call from her doctor. She was told that there was a new treatment for ITP available. So Christy made her way to the city for this new treatment. The treatment was IVIG (Intravenous immune globulin). A side effect that sometimes occurs with the IVIG is called "chemically-induced meningitis"; this happened to Christy. She describes this as ten hours of "feeling like my head was splitting open"; she also had "severe joint pain", as well as ten hours of nausea and vomiting. With the IVIG treatment, Christy’s platelet count rose from 10,000 to 240,000. Her platelets remained above 100,000 for more than four weeks. She explains, "I couldn’t tell any difference in the way I felt, but this thoroughly excited all of us". After the four weeks of elevated platelets, the count dropped back to her usual 10,000. She and her family were disappointed that there was not a total recovery, and she would "just as soon never have IVIG therapy again."
For the next 9 years of her life, Christy was asymptomatic even though her platelet count remained about 10,000 to 15,000. She played high school basketball, went to college, graduated with a degree in Social Work, began her career in a hospital, and was married in 1992. Her husband states, "She even had less bruising as time went by."
During this time she stopped having menstrual periods. She was not on any type of birth control, and was content with this. However, around 1996, when she and her husband began thinking about having children, she was given two medications to help her restart her menstrual periods and to induce ovulation. With the medications, Christy resumed her menstrual cycles, but this also caused occasional excessive bleeding during menstruation. Several times she required platelet transfusions to stop the bleeding. These always seemed to help, but one time she developed shaking chills during the transfusion. Overall, she believes that the platelet transfusions are the best therapy for her when she has severe bleeding.
Also problems would arise when she had her annual routine lab work at her hospital. On one occasion, the doctor "freaked out" when he saw how low her platelets were. She simply explained about ITP and the doctor calmed down. Soon, however, the hospital staff became familiar with her ITP, but they still always remark on how low her platelet counts are.
In 1997, Christy’s doctor called to tell her about a new drug that was being tested for ITP. The drug had already been tested extensively in animals and had just been approved by the FDA for studies in people. Usually new drugs are tested first in normal volunteers, but because this drug had a potential risk of suppression of the immune system, the FDA and the company together decided that the initial tests should be done in patients with ITP. Christy was asked to be the first person ever to receive this drug because she had persistent, severely low platelet counts, and also because she was otherwise very healthy, very intelligent, and very calm. The discussions about this experiment were extensive. The "consent form" that she had to sign, agreeing to participate in this research, explained in detail all the possible complications that could occur. The morning of the brief IV infusion was tense, in spite of everyone’s effort to remain calm. But then the long day of continuous careful checks became oppressively boring. Christy was checked carefully over the next 6 weeks. There were no complications from the medicine, but also no increase in her platelet count. In 1998, Christy received 12 infusions of this experimental drug, again with no response. Christy’s participation in this search for new treatments was just one more part of her effort to eventually overcome ITP.
In 2001, at age 28, Christy became pregnant. She and her husband are excited and happy, but they are not without worry. Christy says, "It’s scary when you think about the future, having kids of your own, and always in the back of your mind is that bleeding thing. And you know there is a chance that the baby will be born with thrombocytopenia." She simply does not want her child to have to go through IVIG treatments.
Regarding her 17 years with ITP, Christy thinks that, "The biggest thing is the fear of the unknown. ITP could be life-threatening, but we don’t view it that way." For example, she may wonder if she’s having bleeding in her brain when she has a headache, but she says, "Thinking this way will ruin your life." She still rides horses, four-wheelers, goes snow skiing, hunts with a rifle, and plays softball. But at the same time, she won’t do things by herself. Christy and her husband say, "There is no need to take unnecessary risks." She says, "You can’t worry constantly, but you also want to be aware of the possibilities as well as the signs and symptoms so you can be treated."
Christy likes to believe that "Your attitude determines how you do." When asked about the most frustrating aspect of her illness, Christy quickly responds with "not knowing how it was acquired, and not having any effective treatment." She is aware of many changes that ITP has brought to her life. She admits it has restricted some activities, there have been some complications, and there is a constant worry in the back of her mind. But she also feels that this experience has helped her to learn so much, meet so many interesting people, and that she is now more aware of herself and everything around her.
When asked what advice she would give to someone newly diagnosed with ITP, she had a lot to say. She first warns, "Sometimes the treatment is worse than the disease." And, "If you are not having an acute problem, then why suffer the side effects of treatments?" She then adds, "But if there is a problem, go and get treatment." Christy suggests finding all available information and, "If there is something out there that works for you, do it." In conclusion, she stated, "The more we know, the more we know how to react, the more comfortable we feel, and we can make good decisions."
Christy update: October 2001
Christy’s pregnancy was uncomplicated. Her platelet count remained very low, as it always is, throughout her pregnancy (3,000-16,000), but she had no bleeding symptoms. She was scheduled for elective induction of labor at 37 weeks, when her baby seemed fully mature. This allowed her to have IVIG treatment to increase her platelet count, to prevent excessive bleeding at the time of delivery. On September 19, when her platelet count was 3,000, she received a very slow infusion of IVIG, 0.8 gram/kg (40% of a typical full 2 day course) following medication with benadryl and hydrocortisone to prevent side effects. This time Christy had no headache or nausea, and the next day her platelet count was 74,000 and it was 107,000 on the following day – so she received no more IVIG. Labor was induced on September 25 and she could have epidural anesthesia, since her platelet count was still in a safe range, 83,000. Christy’s baby daughter was born that evening, with an uncomplicated vaginal delivery. She was robust and healthy. Her platelet count was 88,000 at birth and 73,000-101,000 over the next three days. Christy and her baby went home on September 28, and her baby’s platelet count became normal two weeks later.
Some doctors have recommended routine cesarean section for mothers with ITP, to prevent the risk of birth trauma for an infant who may be severely thrombocytopenic. But severe thrombocytopenia in infants born to mothers with ITP is rare, and the increased risks from cesarean section do not appear to be worth any possible benefit. Some doctors have recommended procedures to determine the infant’s platelet count before birth, so a cesarean section can be done when the infant is severely thrombocytopenic. But these procedures are difficult and have risks, and often the platelet counts are not accurate. Therefore the standard practice is what was done for Christy: vaginal delivery with careful attention to the baby’s platelet count immediately at birth and for the first several days of life.
Probably a future update of Christy’s story will tell about a second child.
Christy update: June 2003
Since having her first child, a beautiful baby girl, in September of 2001, Christy has been doing very well. Family life has been wonderful. She is very excited when speaking of her daughter, who is also doing well. Career-wise, Christy is currently the director of social service at a regional hospital; health-wise, she is doing well. Christy’s platelet count has been over 30,000 since the delivery.
For almost two years now, Christy has had no health problems, nor bleeding episodes. She has her platelet count done two to three times yearly, based upon how she feels and the presence of symptoms, such as bruises. When asked how often she thinks about ITP, Christy responds, “I don’t think about it unless symptoms arise…maybe twice a month.” Here, Christy emphasizes that it is “crucial that the [ITP] patient knows the signs and symptoms of ITP” so that when they appear the patient can get them checked out and deal with the situation immediately to prevent the problem from getting out of control.
Having 19 years of experience with ITP, when asked what advice she would give to others diagnosed with ITP, Christy replies, “Patients should not get too consumed with the diagnosis and literature. Take it day-by-day and not get too overwhelmed with everything. Also find a physician you can trust and who is knowledgeable about the disease.” Having experimented with new treatments and clinical trials, Christy also advises that “a lot of times treatments are worse than the disease itself.” Therefore she suggests that the patient should not be too aggressive with treatments, rather, gauge the treatments according to how she feels.
Christy update: November 2006
We last spoke to Christy in June 2003. Within the last couple of years, Christy has had a few episodes of heavy bleeding related to her menstrual cycle. At these times when she has required treatment, she prefers to receive platelet transfusions rather than IVIG therapy to stop the bleeding. This is because she experienced a very painful side effect of IVIG treatment, known as chemical-induced meningitis, when she was a teenager. Whenever she has a platelet transfusion, her bleeding stops within a couple of days and her life returns to normal.
In August 2005 Christy experienced her worst bleeding episode of the last few years. She was experiencing very heavy menstrual bleeding that would not stop. At this time her platelet count was only 5,000 and she could not avoid IVIG treatment. She required 5 days of IVIG treatment in all. On the third day of IVIG, her platelet count increased to 202,000. The next day her platelet count increased to 381,000, and on the fifth and final day of her treatment her platelet count was 498,000. (Christy did experience a severe headache, nausea, vomiting, and a stiff neck with this treatment.) She also had to take two weeks off of work because of this episode.
Christy has always been very calm and practical when it comes to her ITP, and her advice for others with ITP continues to be to not get consumed by all of the possible treatment options. Christy continues to get her platelet counts when she has symptoms (tiredness, bruising, or bleeding), and receives treatment when it is necessary. She certainly hasn’t let ITP dictate how she lives her life. One of Christy’s next big plans is to have another baby. “Hopefully soon,” she says, “as I don’t seem to be getting any younger.”
Christy update: December 2010
For the first two years, after we last wrote about Christy in November 2006, she did remarkably well. She had no major bleeding episodes; only mild, scattered bruises or petechiae. Her only serious bleeding episode since we last spoke was in November and December of 2008. She describes the episode as “by far the worst bleeding that I’ve ever had.” It started about a month before Christmas when she started having multiple episodes of heavy vaginal bleeding. She had three visits to the hospital and required six blood transfusions and three platelet transfusion during this time. Christmas came, and she spent most of her time in the house feeling too weak to get out. The day after Christmas, she was so tired of being stuck in the house that she decided to go with her husband to feed their cows. Her only excursion outside of the truck they were riding in resulted in her feeling so light-headed that it wasn’t long until she went back to the truck to rest. The next thing she remembers is waking up to her frantic husband shaking her, screaming at her, and threatening to start CPR. He drove her to the hospital where her hemoglobin was extremely low at 4.8, well below the normal of 12g/dl, the result of continued vaginal bleeding. Her platelets were less than 6,000, too low for the machine to accurately count. She stayed in the hospital for 3 days where she received 6 more blood transfusions and IVIG. Even with premedication of Benadryl and steroids, she again suffered from the torturous side effects of headache and nausea from chemically-induced meningitis that IVIG infusions can cause. Eventually her platelet count rose to above 100,000 and her bleeding slowed to a manageable rate. To stop her bleeding completely her doctors recommended a hysterectomy. She wasn’t ready for this yet so the next option was high-dose birth control pills to stop the bleeding and regular birth control pills to control future bleeding. Christy’s periods have been controlled since then and she’s had no more serious bleeding and no more need for blood transfusions or IVIG in the past 2 years.
Over the years, Christy has been asked to participate in various clinical trials of new drugs to treat ITP. In recent years she has declined to participate in any because she thought she would want to have another child. Recently, she and her husband have decided that are blessed to have their daughter, and given her trouble with menstrual bleeding, have decided to not have any more children. Therefore in January of 2010 she agreed to be part of a trial involving a drug that stimulates platelet production (romiplostim; the brand name is Nplate). At first she was very optimistic that the drug would help her because it has had such high success rates in other patients, even patients with very prolonged, severe low platelet counts like Christy has had. She was also intrigued by how few side effects patients were experiencing. She began making the trips to Oklahoma City every few weeks, with her husband along so he can take her out to eat when she is done with her appointment. Initially her platelet counts went up to as high as 62,000, but gradually, even with increasing dosage of the drug, her platelet counts have dwindled back down to around 2,000. In hindsight, she still thinks that participating in the drug trial was worth it, just on the chance that it might have worked. She would like to tell other ITP patients to do their homework when thinking about starting a new drug or participating in a drug trial. Find out the benefits and side effects. About participating in drug trials: Christy’s advice is to do it because there is not a whole lot to lose and there is so much that might be gained.Christy continues to live her life without hindrance. She is very active, even enjoying Tae Kwon Do with her nine year-old daughter, who has no signs of ITP. She has also been known to take a skiing trip with a platelet count of 7,000 (though she “forgot” to tell her doctors about the trip ahead of time). She continues to only think about ITP when she is symptomatic. She states it may be in the back of her mind at times, but she doesn’t focus on it because it can “drive you crazy worrying about it.” She doesn’t want to let ITP control her life and refuses to live in fear of a low platelet count. Christy believes as long as she is happy and doing alright, she is content to live with ITP.
Comments by Dr. George
Christy is a wonderful person. I think she knows as much about ITP as anyone, hematologists included. She and I recently gave a talk on ITP at her hospital. My part was the usual doctor material on ITP; Christy’s part was terrific. For true education about a chronic health problem, there is no substitute for learning directly from a patient. The important messages in Christy’s story are that you can have a happy, successful life, even with severe thrombocytopenia and even with occasional episodes of severe bleeding. Christy is calm and sensible, and I assume she was also calm and sensible as a teenager, years before I first met her. In this story, she may appear to have been a rebellious adolescent but I doubt that. Her determination to have a normal active life is only normal. What are the alternatives? Only worse. Christy’s calmness comes through in her description of interactions with doctors who were unfamiliar with ITP and alarmed by her severe thrombocytopenia. Most patients with ITP experience this sometime. Her calmness was also apparent in the story of her participation as the first human subject to be treated in a phase I clinical trial of a new agent for the treatment of ITP. (This agent was effective in a few other patients with ITP, but its development was discontinued.) Participation in clinical trials is very important for patients with a chronic illness that has no effective treatment, such as ITP. The patient may not benefit, but the process is critical for progress to effective management. The process can also be very educational and supportive for the participating patients. This has been Christy’s rationale for her participation in the current clinical trial with romiplostim. Although Christy’s platelet count has not increased with romiplostim treatment during this clinical trial, I still think her participation in the study is important for her, as I think participation in clinical trials are important for all patients. Christy has had severe bleeding episodes in the past and even though her platelet count has not increased with romiplostim, I think that it is probable that the increased platelet production that is caused by romiplostim provides more platelets whenever there is some trauma or risk for bleeding – even though the platelets don’t remain in the circulation and get counted. Also the continuous evaluations over months and years provide a very clear picture of Christy’s ITP. This can be important information whenever future treatments may be considered.
Of course Christy was selected to tell her story because hers is a happy one – but it also includes some scary episodes of severe bleeding. Some patients with ITP suffer much more, but too often the severe problems receive the most attention. Christy’s experience is common. Her most important message is optimism. Her story is even more optimistic than when we first recorded it in 2001. Although she has times when her platelet count was higher with an apparent spontaneous remission, and now her platelet count is very low again, Christy is perfectly comfortable with her life. People around her would never know that she has a significant health problem. Christy’s story may seem exceptional but I think it could also be a typical story of a young person with ITP.
[First written July 2001. Updated November 2006. Reviewed January 2007. Updated December 2010.]
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