Shruti Chaturvedi, MBBS, is a hematologist and an associate professor of medicine at Johns Hopkins Medicine in Baltimore. She specializes in the care of adults with non-cancerous hematologic disorders.
Immune thrombocytopenia (ITP) is a rare condition that makes it harder for your blood to clot. As a result, you may bruise and bleed more easily. This happens because ITP can cause you to have lower levels of platelets circulating in your blood, which affects clotting.
Most often with ITP, your immune system attacks platelets by mistake. In adults, there's usually no clear cause for this, but it can be linked with certain infections, medicines, pregnancy, cancers, or immune system disorders. In children, it can be due to viral infections, including chickenpox, flu, and other respiratory viruses. ITP can be acute (going away within a few months), intermediate (lasting three to 12 months), or chronic (lasting more than a year). We spoke with Shruti Chaturvedi, MBBS, a hematologist with Johns Hopkins Medicine, about how to take charge of ITP symptoms, manage or prevent treatment side effects, and live well with the condition.
Q: When you have ITP, what can you do to manage symptoms like bruising and bleeding?
A: There are two types of things one can do to minimize these ITP symptoms. The first is to work with your physician to make sure that your platelet count is in what we consider the safe range from the bleeding standpoint. For most people, this means a platelet count greater than 30,000 to 50,000 per cubic millimeter. The other thing that one can do is minimize trauma if you can. For example, if your platelet count is very low, avoid playing contact sports or taking medications that can interfere with platelet function or lead to bleeding. Apart from that, some bruising is inevitable if the platelet counts are running low. And how much someone bruises also depends on other features, like whether you have thin skin, your age and activities, etc.
Q: What supplies should you keep on hand to manage minor bleeding?
A: I would suggest keeping Band-Aids with you at all times. I'd also suggest a styptic pencil for use, for example, if you cut yourself while shaving. I tell all of my menstruating patients to keep their period supplies on hand. Particularly for those who are having menstrual periods where the bleeding may be increased because the platelet counts are low, there are medications that your doctor can prescribe that can make the period bleeding lighter.
Q: When should you call your doctor about bleeding?
A: If you have any bleeding that is unusual for you, you should call your doctor. What we worry the most about is potentially life-threatening bleeding where someone is losing a lot of blood, particularly in the gut (showing up as bloody or dark bowel movements) or a critical location like the brain.
Q: What are some common treatment side effects?
A: It depends on the medication. One class of medications that is used in a lot of patients with ITP is steroids such as prednisone or dexamethasone. These are very effective in the short term in bringing the platelet count up and reducing the risk of bleeding. But they also carry the highest rate of side effects, particularly if these medications are used for a long time. The side effects include things like out-of-control blood pressure or blood sugar, increased appetite, weight gain, thinning skin, and weakening of bones. If someone is needing steroids for weeks or months, it's worth having the conversation with your doctor about other options.
The other class of medications that come with side effects are longer-term medications. There is one class of medications called TPO. These are thrombopoietin mimetics or thrombopoietin receptor agonists. These include medicines like avatrombopag (Doptelet, Doptelet Sprinkle), eltrombopag (Promacta), and romiplostim (Nplate). On the whole, they are very well-tolerated with minimal side effects, although with Promacta, sometimes people have liver function abnormalities.
The other important class of medicines are those that suppress the immune system to stop it from attacking your platelets. These include medicines like rilzabrutinib (Wayrilz) and rituximab (Rituxan, Ruxience, Truxima). Now, when we suppress the immune system, patients can be at an increased risk of infections, which is a major side effect. If you're on immunosuppressive treatment, it's advisable to pursue good hand-washing practices and stay away from people who are obviously sick. While we recommend getting all of the appropriate vaccinations, understand that if you got vaccinated after receiving an immunosuppressive treatment, your response to vaccination may not be as good as everyone else's. So, keep all of the other infection-prevention practices going.
Q: If you have chronic ITP, can you expect the condition to change over time?
A: Chronic ITP comes in different flavors. There are some people out there who live with slightly low platelet counts – for example, platelet counts in the 60,000 to 80,000 range – which don't truly put them at the risk of bleeding. These individuals may never need treatment.
Living with that type of chronic ITP is very different from living with a chronic ITP where your platelet counts are consistently below 20,000 or 30,000 and you need to be on treatment most of the time. Some patients, unfortunately, despite these treatments, still have significant fluctuations requiring treatment changes over time.
Occasionally, we will find that someone in the first group who has had pretty stable platelet counts – for sometimes years, even decades – will suddenly have a drop in their counts that needs treatment. It's also possible, but somewhat less common, where someone who has had more difficult-to-treat ITP for a while may achieve a lasting response on or off treatment. So ITP can change.
Q: What should you know if you have a child with chronic ITP?
A: Some children will require longer-term treatment with medications, and it can be frightening for parents. Your child now has a chronic illness, and they're also sometimes restricted from doing the things that they most love.
The biggest takeaway for parents is that ITP is very much manageable. Fortunately, there are more and more clinical studies and clinical trials that are directly investigating ITP treatments in children. So your children are now able to get treatments that have been tested in the right population rather than treating them like tiny adults, which they're not.
Q: If you need surgery to remove your spleen (splenectomy), what safety measures should you take afterward?
A: Taking the spleen out used to be a second- or third-line treatment. Now it's been pushed out to eighth-, ninth-line, and beyond, because we have a lot of medications that are effective. If a doctor decides that their patient needs a splenectomy, then the patient should get vaccinated against certain infections that the spleen is particularly important in fighting before they get the surgery. These are meningococcal infection, pneumococcal infection, and haemophilus influenza.
After someone's spleen has been taken out, they have a lifelong risk of serious infection. We advise all of our patients who do not have spleens that if they have a fever of 100.4 degrees F or higher that lasts for longer than an hour with or without other symptoms, they should seek medical attention. And they should absolutely stay up to date with any booster vaccinations that are recommended.
Q: If you don't live near a hematologist who's knowledgeable about ITP, what steps can you take to find one?
A: You could reach out to patient support organizations such as the PDSA (Platelet Disorder Support Association), which can either direct you to resources within your area or provide you with additional information that you may not be getting from your medical team. Also, telemedicine is very feasible. You should, if you really want, be able to get a second opinion through telemedicine or a video consultation with a physician with expertise in ITP who practices within your state.
Q: Along with a hematologist, what other health care professionals might someone need on their team?
A: It depends on the individual and what they're experiencing. Some people with ITP also have an underlying condition (for example, lupus) and they may benefit from having someone to treat that condition, like a rheumatologist.
Some patients with ITP also struggle with symptoms that are not bleeding. Some of this is fatigue, stress, anxiety, or poor quality of life related to ITP. If that is truly impacting someone's life, they may actually benefit from a mental health professional, whether it's a therapist or a psychiatrist, to help them work through this.
The more that we recognize and talk about the symptoms beyond bleeding, the more we can actually start to help our patients who are struggling with them. The platelet count is the easiest thing to treat, but it's only the tip of the iceberg.