Dr. Craig Kessler

Physicians who take care of ITP are now just recognizing the fact that ITP is really a disease of aging. The level of fatigue that a younger individual may experience with ITP is minimal compared to the level of fatigue that a much older individual may experience with ITP.

Narrator

Welcome to the PDSA podcast, Bruised but Not Broken, Living with ITP. The diagnosis of a bleeding disorder like immune thrombocytopenia may leave you wondering, how can I really live my life with ITP? PDSA's podcast, Bruised but Not Broken: Living With ITP, brings empowering stories, the latest research and treatment updates, lifestyle tips, and answers to the real-life questions the ITP community is asking. Here's your host for this episode, Barbara Pruitt.

Barbara Pruitt

Today, at the PDSA Annual Conference. We're in Philadelphia. In a previous episode, I interviewed Rand and Lib, who are two older ITP patients, and i asked them about living with ITP. At the conference, we have a great opportunity to get some expert medical advice from some of our medical advisors. So today, we have with us Dr. Craig Kessler. Dr. Kessler is a professor of medicine and pathology and the section chief of hematology and the director of the coagulation laboratory at Georgetown University in Washington, D.C. He's a graduate of Tulane University Medical School, and he received his specialty training in hematology and oncology at the John Hopkins Hospital. He's an international expert in disorders of coagulation. He also has expertise in the treatment of hematological malignancies. So Dr. Kessler also serves on our PDSA Board of Medical Advisors. So Dr. Kessler, thank you so much for joining me today.

Dr. Craig Kessler

Thank you, Barbara.

Barbara

It's been great to see you through the years and we've always appreciated your input and your expertise. And today's topic is a little bit different. You've been treating ITP patients for years. And as you've watched your patients age, what have you noticed? What have you seen with them?

Dr. Kessler

You know, physicians who take care of ITP are now just recognizing the fact that ITP is really a disease of aging. Now, we have sort of a bimodal distribution of ITP patients. So we know that there are young individuals with ITP. And frequently, the young individuals have a very different clinical presentation and duration of disease. And as patients age, the presentations of their ITP are different. And also, the ability to treat ah the ITP becomes a little bit more difficult than it is in younger individuals. So over the years, I think that physicians have have begun to realize that the ITP that they were taught about in their years of medical education is very different from the ITP that we actually see in our clinical practice in the older individual.

Barbara

When you're talking about this, it reminds me of something because I know that a lot of patients are diagnosed with ITP at an older age. I know it can strike anyone at any age, but is there a comparison between maybe a person that was just diagnosed at an older age compared to a person that's had it for years and is now older?

Dr. Kessler

Yes, there's a definite difference because typically the older individual who presents with ITP will very frequently have an underlying disease which is contributing to the development of ITP. So frequently in the older individual, you're going to have other disease states that either have been recognized and treated before or have never been recognized. And then the development of the treatment plan or the diagnostic plan in the older individual would require that you look for secondary causes of ITP. And so for instance, the older individual with ITP will frequently have an underlying a lymphoproliferative malignancy, something like chronic lymphocytic leukemia, an indolent lymphoma. Even solid tumor malignancies can be associated with the development of ITP. In younger individuals, they'll present maybe with an autoimmune disease right rather than a malignancy. So in the treatment and the diagnosis of ITP in the older individual, it's critical that the physician look for underlying causes that are also treatable. In other words, you may be able to raise a platelet count with many of the treatment modalities that we have available for ITP in the elderly individual, but unless you get rid of the underlying disease that may be contributing to the development of ITP, you really haven't completed the treatment or the diagnostic ah parameters of this disease.

Barbara

Right. It's predominantly secondary ITP you see mostly with elderly patients.

Dr. Kessler

That's correct.

Barbara

Well, that makes sense. So with a person that is diagnosed earlier with ITP, maybe in their 30s or 40s and continue to have ITP through their older years, do you see that the ITP, does it progress? Does it get any worse with age?

Dr. Kessler

Many of our treatment modalities now for young individuals, we can eradicate the ITP and put the patient into complete remission. But as they get older, then sometimes the disease will recur. And that's that's a problem that we have with aging. It's a system that I i like to call immunosenescence, so that as we get older, parts of our immune system um become tired, fatigued, and it allows for other disease processes to become apparent. Our immune systems become less efficient and we as we age. And when you lose components of the immune function, then it allows other abnormalities to become apparent.

Barbara

The immune system kind of gets tired, like we do as we age. Do you monitor your older patients more frequently than you do like a younger patient with ITP? Do they need to be seen more frequently?

Dr. Kessler

Definitely. And the reason for that is multiple reasons. One is that the older individual who presents to you will have many other medical comorbidities and they have hypertension, diabetes, obesity, heart disease, et cetera. And every treatment modality that we have is going to exacerbate those underlying medical comorbidities. Corticosteroids will raise blood pressure, will raise blood sugar, increase obesity. So even the simplest approach to the treatment of ITP in the older individual needs more close surveillance of the medical comorbidities, not just the ITP.

Barbara

Right. And I can see that you as a physician would feel more like a general physician for your ITP patients when they're dealing with all these different things because they can upset one of the other functions or one of the other problems that they are having. So it puts more weight on your shoulders too. You're not just taking care of their ITP, but you're looking at all the other factors involved with it.

Dr. Kessler

That's absolutely correct. And it takes a village, so to speak, because you really do need to bring in other medical subspecialists who appreciate the problems associated with ITP, but also the side effects of the treatment modalities that we have available. And frequently, we can't use those common treatment modalities that work in younger individuals because of the exacerbation of the medical comorbidities in older individuals.

Barbara

So one of the most important things for a patient would be that there's great communication between you and those other specialists that that are treating them. And I know that's not always easy. I mean, in a facility like yours, where you're at a medical school and a hospital, you probably have better availability to those other specialists. But for people that might have one doctor in one town and one doctor in another town, that's going to be more challenging, I should say.

Dr. Kessler

Well, ideally, there's communication among all of the physicians. In reality, that's not always the case.

Barbara

Which puts more on the patient's shoulders to make sure they're communicating well sometimes.

Dr. Kessler

Definitely. I think patient education is extremely important. And quite truthfully, ah the topics of discussion ah that the hematologist has with the patient who's older with ITP is very different from the discussion that you would have with a younger individual where you may be talking about pregnancy issues, autoimmune disease, cetera. In the older individual, you really have to start talking about, well, this is the workup that we're going to have to do. You may need a bone marrow exam. In the younger individual, we may not need a bone marrow exam to make a diagnosis. But if we're looking for an underlying lymph or proliferative malignancy, we need to have a bone marrow. Completely different discussion. And up to now, the guidelines have not really focused in on how you approach an older individual with ITP from a younger individual with ITP.

Barbara

Right. So that's something that needs to be addressed in the future. Do you speak to your patients? I mean, I'm sure you do regarding aging in their ITP at like what point? And I mean, do you see signals with the patient that they're starting to age? If this is a person, a patient that's had ITP for a long period of time, At what point do you kind of decide, you know, we need to have a discussion about aging?

Dr. Kessler

Well, I think that for, and at least in in my approach to taking care of ITP patients, we're always talking about future potential problems. The patient really has to be alerted to what could happen, whether it's related to the complications of prior treatments or to the introduction of other medications that might need to be used ah in an individual who has chronic ITP. Just as an example, ah we know that there are diseases that are immunosuppressive, and we know that there are treatments that are immunosuppressive, and ITP is oftentimes associated with complications of an immunosuppressive disease. So if you added a an immunosuppressive drug for another disease in an individual who has had chronic ITP but in remission, you can actually have that ITP reemerge as as a definite complication of that person's care. Wow. They really have to be educated and it takes a lot of effort to educate our patients. It takes a lot of time to educate.

Barbara

Well, I know myself as an ITP patient, I've always had the feeling that I needed to be proactive. And if I was referred to a specialist for something, I want to make sure that everything's okay. If I saw something or we saw something in the future that it could be staved off quickly instead of having it grow larger and become a bigger problem and then be more more of a problem with my ITP. I think as a patient, we have to be very proactive. And of course, their doctors are that way too.

Dr. Kessler

Take a look at a patient that I saw last week, a lady who came in with a history of breast cancer, intermission, and she had low platelet counts. the question is, and she had received a line of treatment for her disease in the past that caused her to be immunosuppressive. It's a category of drugs called checkpoint inhibitors. These are very common drugs now being used in the cancer realm. So the patient presents to you with thrombocytopenia. You have to decide now, is this related to recurrent breast disease? Is this related to the past exposure to chemotherapy so that now her bone marrow isn't working to make platelets anymore? This is a condition called myelodysplastic syndrome. This is a very common cause of thrombocytopenia in the older individual. Or is this ITP related to her recent exposure to these new drug modalities called checkpoint inhibitors. It's a completely different discussion than if somebody comes at a younger age with maybe a remote history of lupus or Hashimoto's thyroiditis or some other autoimmune disease.

Barbara

Right. That sounds like a difficult thing to address, trying to figure that out with that patient.

Dr. Kessler

And also the diagnosis is going to be important because if the patient has a low platelet count, that prevents you from immediately being able to biopsy any lesions that you would see in the body that might be related to her prior breast cancer or whatever. So it's not just a discussion on what's happening now, but it's a discussion of future events and also how will you tailor your treatment for that person's ITP that won't make things worse.

Barbara

Right. You have to keep safety in mind. Right. On another topic, which I think is ITP patients, we don't want to hear about, but it it has become something that's been discussed quite a bit, that there can be a cognitive decline in patients with ITP as they get older. Tell me about that.

Dr. Kessler

This is an area which is emerging as a topic that patients don't want to know about. And also physicians have very difficult issues abilities to quantitate. So there's never been a good prospective, controlled, randomized trial to see whether or not individuals who have ITP are more likely to develop early dementia than those individuals without ITP. And again, we don't know whether there certain medications that were used to treat ITP might increase the likelihood of developing dementia. So as an example, we know that um individuals who have had chemotherapy agents for treatment of many diseases many years down the road have an earlier onset of dementia. They may call it brain fog or or some other euphemism, but in actuality it's probably early onset dementia. If they've been on corticosteroids for many, many years and they've developed hypertension and diabetes because of corticosteroids, they may also have early onset of ischemic dementia. That means that the blood supply to the brain has been has been compromised by earlier treatment. And also what we don't have a good handle for is whether or not there's a series of microbleeds that can occur in the brain when platelet counts are extremely low or when trauma occurs in the patient who has ITP, trauma to the head. Or even more likely is the ubiquitous use of non-steroidal anti-inflammatory agents or the occasional use of aspirin which will then increase the risk of bleeding in individuals who have very low platelet counts. The bleeds may not be large enough to cause any obvious neurological symptoms, but over time, there's a cumulative effect of all of these microbleeds on the brain. And these individuals are very likely to have some evidence of dementia or some other cognitive compromise.

Barbara

What kind of signs do you look for in your patients that would lead you to think that there's maybe some kind of cognitive deficiency? Or do you rely on family members to alert you to things? Or a combination of both, I would think.

Dr. Kessler

The evaluation of dementia is extremely difficult because it's time-consuming. You have to know your patient. You have to spend time with your patients. You have to maybe even provide them with some kind of a cognitive test. And that's difficult time-wise, and it's very difficult to quantitate from one time to the next. Typically, it's done maybe on a yearly basis or every six-month basis. We really have to rely on family observation because rarely does the individual himself realize that there's any cognitive decline or there's a lot of denial around the possibility that there could be cognitive decline. Can you do the New York Times so crossword puzzle as rapidly now as you did when you were 50 years old or 40 years old? Most patients don't use that kind of a parameter. It's like, can you remember your social security number? Can you remember your telephone number? Those kinds of things. I can't remember my own wife's telephone number because I only hit send or or some other mechanism. We don't thinking those terms anymore. Can you keep your checkbook up to date most people are on computerized banking so you don't really know so it really takes a lot of effort to be able to understand what the patient's perception is of his own cognition and then what does the spouse or the family think about the individual's behavior pattern

Barbara

Well if you have an older itp patient whether they were recently diagnosed or diagnosed years ago. Would it be prudent to have some sort of cognitive testing done with a psychologist or something? Would that be called for?

Dr. Kessler

Ideally, it would be useful. However, many of the medications that we use for the treatment of ITP make people cognitively impaired because corticosteroids can change personalities and can change your cognitive capacity. on The medications that that we're using for ITP these days is secondary drugs. We haven't seen this with TIPO RA drugs, TIPO mimetics as much, but certainly all of the immunosuppressive drugs that we use have all been associated already with increased fatigue, increased cognitive ah capacity, and many other side effects that can affect the patient's behavior.

So it's very difficult to actually determine what's actually happening here. You really have to peel the onion and look at each aspect of the patient's health.

Barbara

And dive into it. I've read that there's one hypothesis about cognitive difficulties. That could be a result of, like you said, the fatigue or steroid-induced issues on general illness in particular. But what do you think about that? Are there any other things that might contribute to to that other than the drugs that you were talking about?

Dr. Kessler

There's a new theory that's emerging about ITP being ah part of an inflammatory process in the body. and we know that ah inflammatory states in the body cause release of certain types of hormones or proteins, even from, say, lymphocytes. These are called ae interferons, for instance, or they're called cytokines. And we know that these cytokines are responsible for a large amount of the clinical vague clinical problems that patients have with ITP. And in the older individual, these are exaggerated. So for instance, the level of fatigue that a younger individual may experience with ITP is minimal compared to the level of fatigue that a much older individual may experience with ITP. That may be the primary presentation that the patient comes in to see the physician is fatigue and a low platelet count. And so ah this is very likely a response of the immune system part of which is the cause of ITP. So our treatment then is focused on the suppression of those cytokines rather than necessarily focused on the production of platelets from the bone marrow, such as you would have with a TPO-RA drug.

Barbara

And the suppression of the cytokines would be trying to eliminate inflammation in the body? Right. You had mentioned about the brain bleeds. That's the monkey on all of us, you know, on our back as a patient. But I know I've experienced two microbleeds haven't affected me as far as I know. I mean, we found them just as a happenstance. But you mentioned that the continuation of little microbleeds could also lead to a cognitive decline. Would that be considered like a mini stroke?

Dr. Kessler

Well, the way that we define stroke, it would not be defined as a stroke. So medically, a stroke would have an obvious neurological abnormality, either sensory or motor. Cognitive strokes, we don't really we don't consider that to be a stroke unless there's a major bleed into the brain that causes compression of the of the brain tissue. That's very obvious when you do x-rays on the brain. So cognitive decline is not considered to be a sign of a stroke.

Barbara

But an accumulation of microbleeds, that could cause the cognitive decline, correct?

Dr. Kessler

Yes.

Barbara

I've also heard that the cognitive decline decline could affect your psychomotor function. Is that the same as hand-eye coordination?

Dr. Kessler

I think it's difficult to say. Each individual, if there is psychomotor impairment, those individuals have to be evaluated by a neurologist and by specialized scanning techniques. Because it may not be that the ITP is causing these issues. It may be other problems associated with aging, like arteriosclerosis, or actually having had a stroke, or related to their underlying diseases that they've had. So it's slightly different. It could be also alcohol-related. It could be related to nutrition. There are many things that could be causing those kinds of impairments. I would put ITP probably at the lower end of the differential diagnostic table here. If I saw something like that, I'd suspect something else.

Barbara

Well, if you suspected the decline could be related to ITP, and I know some of the decline could be attention-related, Are there drugs that maybe would be helpful in some of these decline that you could see?

Dr. Kessler

I think you have to understand why the cognitive decline is occurring. If you feel that it's related to fatigue, that it's related to other underlying psychiatric issues, if it's related to other medications, there are some maneuvers that can be done to improve patient's cognitive focus. So for instance, there are a set of medications that can be used to try to improve people's focus. None of the ones that are advertised on television that have these brain health labels. right None of those have have ever really been validated and in good clinical trials. But I'm talking about drugs such as a drug called Provigil or NuVigil. These are drugs that have been used many years by the military, for instance, and by airline pilots to try to increase their focus. And I've used these drugs at times in patients who have severe fatigue associated with their ITP or the treatment of their ITP. And they can take these medications and and their focus can be improved. Temporarily, it has to be a drug used on a repetitive basis, but they can actually improve their quality of life and the quality of their cognitive capacity. The unfortunate thing is that insurance doesn't usually pay for them.

Barbara

Yeah, that is unfortunate. Is it something that If you started it, you you have to continue it. Can you stop it or take it only occasionally?

Dr. Kessler

You can take it occasionally. It works pretty fast. Airline pilots or military pilots use them only when they're long-haul missions, for instance. And so the effect is pretty immediate, and there's no hangover effect of the drug. Yyou have the immediate improvement, but it's only temporary.

Barbara

It sounds like something a college kid would want to get a hold of.

Dr. Kessler

It's probably better than caffeine.

Barbara

All right. Would it interfere with your sleeping?

Dr. Kessler

There are some side effects, insomnia, maybe bad dreams, maybe. So all of these drugs have their own profile of complications.

Barbara

Right. Like everything else. Exactly. Do you ever discuss with your patients end of life decisions, what they should be thinking about, whether it's do not resuscitate, DNR, power of attorney, healthcare surrogates, anything like that?

Dr. Kessler

Well, I think, again, when you're an adult physician taking care of aging individuals, this comes up inevitably. It's really the bandwidth of the physician as to how detailed the discussion is going to be.

And it's also important that the patient and his family are interested in discussing it because frequently they're not interested in discussing it.

Barbara

Or they don't have the ability to discuss it with that family member because it's kind of a taboo subject. A lot of people don't want to talk about it

Dr. Kessler

And a lot of people aren't sophisticated enough to realize that the disease that they have may be life-threatening. So I think it's an important topic for any aging patient, no matter what the disease problem is that they're seeing a physician for.

Barbara

What other advice comes to mind when you're speaking to your older patients?

Dr. Kessler

I think there are two major areas. The first one, which I think is extremely important, is the need for physical therapy. So even the normal non-coagulopathic, non-ITP patient, as they age, they become physically unfit, their proprioception, their nervous systems age, they may be tripping over things a little bit more. They may even have a misinterpretation of their own physical ability. And they may try to do things at home like change light bulbs on a stool or on on a ladder. They may have difficulty getting out of a bathtub and trip over the lip of the tub. All of these issues are physical fitness issues, but they're related to the aging nervous system. In the ITP patient, it's going to be an exaggerated risk to be on a ladder changing light bulbs with your head extended or whatever. So I advocate in all of my aging patients that if they have the resources to get a physical treatment, a therapist or physical fitness counselor or somebody who can actually develop core strength, help them develop core strength, to help them to to develop strength in muscle groups so that if they do have a fall, they can protect themselves. They know how to get up off the ground if they fall. These are the issues that we need to emphasize in the and the aging population.

The other area that I think is extremely important, and most physicians don't spend a lot of time on this, is ah to make sure that the ITP aging patient is vaccinated appropriately, particularly if they've had a splenectomy, for sure, because those individuals have extremely exaggerated risks of death, immediate death when they have infection.They lose the protection that the spleen provides them with. But also other things like we know ah pneumonia vaccination should be given to even individuals who have not had splenectomies. We know that the COVID vaccine is protective against the severity of COVID if you get it. We know COVID can produce exaggerated decreases your platelet count. The vaccines don't so much, but the infection does. We know that individuals should be vaccinated against shingles. The shingles vaccine is extremely important for aging individuals.

If you haven't had diphtheria tetanus shot over years, you should be revaccinated as an older individual. Measles, as you know, ah from recent media reports, is becoming an increasing a problem. And if you're aging and and you have had many, many years' distance from your original vaccinations, you're at risk. So vaccinations are extremely important. So those two, physical therapy and vaccinations, are key, I think, to healthy aging ah population population particularly if you have ITP.

Barbara

Well, that's great information and advice. I know I've been very diligent about my vaccines through the years and I'm always making sure I'm up to date, but my hematologist is always making sure that I'm up to date. But exercising has always been an issue for me. I'm not very I'm not very devoted to it. I really know that there's always room for improvement. and And I have to say that I have noticed a difference in my body as I've gotten older. There are changes. I'm not as physically fit as I used to be. So I think both of those are very good advice for all of us. Well, I can't thank you enough. This is a great opportunity for me to talk to you face-to-face. Most of my podcasts we're we doing online. And I just see whoever I'm interviewing on my computer screen. So this is a great treat for me, you know, to be interviewing you here. and And I, again, I thank you for all that you do for the ITP community and for the PDSA as a whole. You've been terrific. I know you've been; gosh, you've been on the medical advisory board for, what, 15, 20 years?

Dr. Kessler

I think 15. But I want to thank you, too, because what you're providing the education here for patients is invaluable. And i'm very happy to have been invited to participate. So thank you.

Barbara

Well, thank you. I appreciate that. And I will probably call on you again, and we'll talk about another subject that's really pertinent to our community.

Dr. Kessler

It was a pleasure.

Barbara

Thank you so much.

Narrator

How do you live your life with a bleeding disorder like ITP? From working in the kitchen with knives to navigating sharp corners in your house, going out to eat in a restaurant, traveling on a plane, attending a sporting event, even dancing at a wedding. ITP patient Barbara Pruitt shares her tips and tricks for moving through life with ITP for more than 60 years. Here's her lifestyle lesson for the day.

Barbara Pruitt

Well, I just wrapped up with Dr. Craig Kessler on this episode, and I thought it was really great. And here with me now is my husband, Peter Pruitt.

Peter Pruitt

Hi there. So you finished up with Craig. Craig was one of your doctors you saw when we were living in D.C. That's right. Yeah, he was wonderful man does wonderful work for us here at PDSA.

Barbara

He does. He's great. He's very personable and really very caring, and appreciated talking to him.

Peter

What talking about?

Barbara

We're talking about aging because I think it's something that hits all of us. I mean, you can't stop it. It kind of creeps up on you. And before you know it, here we are. We're getting old. And by the way, happy birthday, dear.

Peter

So you brought that up because we're talking about aging.

Barbara

Right. Today's your birthday. You're a year old. I'm not telling anybody how old I am.

Peter

No, you don't have to. we know I could tell them that I'm 69 today.

Barbara

We know you're old.

Peter

So what were the takeaways? Is aging, I need to worry about this with you and ITP?

Barbara

Yeah. Well, I mean, he brought up a couple of very interesting topics that, you know, it's different when a person is diagnosed as an older person with ITP because they're very frequently at secondary ITP and they're primary diagnosis needs to be handled along with their ITP, so there has to be a lot of coordination with their specialists and stuff. For patients with ITP as we get older, we're facing aging issues and need to be more cognizant of falling. And one of the things he really emphasized is that we should have physical therapy. We need to keep moving and keeping our muscles strong. And i know that's something that I've pretty much avoided through my life. I'm I've always said, I don't like to sweat. I've kind of avoided avoided exercising, haven't I?

Peter

Well, I know my primary care physician now when he does my physical every year, makes me stand on one foot. He makes me walk and he's like, stand on one foot. You know, you you only get like 15 seconds. You may want to think about physical therapy. I'm like, I'm not doing physical therapy but

Barbara

Well, now maybe you should.

Peter

As we age, our balance goes away. And I think your point, and think what you what you're saying is that when you add on the aging process we have on top of ITP, it leads to whole other set of issues that you as an elderly patient, are you elderly? I decided we're older. Older. Elderly is a different thing.

Barbara

That's always 20 years beyond what we are as elderly.

Peter

But you need to be concerned that you be concerned about these things.

Barbara

Yeah. I can stand on my one foot for longer than you can. sure can. It's because I do yoga. See, but I don't sweat. I do yoga, but I don't sweat. Well, i I do need to be more concerned about falling and tripping and stuff.

Peter

We all do it.

Barbara

Yeah. And like you said, it's like... Older. But like you said, tripping over a rug. You know, I mean, I always was worried about that with my grandparents and with my own parents. But now I guess I'm creeping up on that stage of life where I need to be worried about it for myself. And vaccines was another thing that he mentioned. Keeping up to date with all of our vaccines, because as we get older and you get hit with some kind of a disease or something, it's going to hit you a lot harder the older you are. So you don't have the ability to rebound the way we used to.

Peter

Great. Sounds like a good episode.

Barbara

It was. It was great. So hang in there, listen to our next episode, and we hope to see you then.

Narrator

Thanks for listening to the PDSA podcast. Bruised but Not Broken: Living with ITP made possible by our presenting sponsor, Amgen. Special thanks to Gus Majorga for composing our theme music.

To see what's coming up, visit our website at pdsa.org and subscribe wherever you get your podcasts. Please share this podcast through social media with anyone who you think might benefit from the information and stories we share with the ITP and other platelet disorders community. As always, please speak with a healthcare care professional before making any treatment decisions. But know that PDSA.org is a wealth of information and resources to help you navigate life with ITP and other platelet disorders. Remember, you are not alone.