Does Standard of Care Change for Adult Sickle Cell Warriors?
More Programs and Publications Featuring Dr. Marwan Shaikh
In this program:
Do adult sickle cell disease patients have one standard of care for all? Dr. Marwan Shaikh and family nurse practitioner Maya Bloomberg explain the treatment that has been used as the gold standard of care, newer treatment options, and an online resource for finding the latest sickle cell disease research information.
Transcript
Interviewer:
With all of the new treatments we're seeing that fortunately, patients with sickle cell disease are starting to live longer. So I would ask you for our aging warriors, is there a specific type of care?
Maya Bloomberg, APRN:
It's so individualized, it's really...there's no one-size-fits-all. I think the gold standard really is hydroxyurea (Hydrea), because it's the only one that we have, we have data from 1980 where you got the 1985 on. They started doing the research back in the 1980s, and then that's what ultimately led to the approval. So I think a lot of times people are a little hesitant with hydroxyurea, because it wasn't created for sickle cell. It is a cancer drug, however, we're not using that, and we have so much research that really justifies its use in the fact that it makes you live longer and prevents complications and decreases your transfusion need. But now we'll have more therapy, so I think this is where we really need to speak to our patients and to shared decision-making and see what are their goals out of the treatment, what are problems in their life that they're not able to achieve, is paying their big issue in which case maybe crizanlizumab or Adakveo is a good option to them. Or are they constantly being transfused and having rapid hemolysis or red cell breakdown where hydroxyurea, which could hit the hemoglobin and the pain or voxelotor (Oxbryta), which helps improve the anemia could be a good option.
So again, I think this is where having combination therapy is so helpful that I think we really just need to have these conversations with our patients, see what they think their big issue is because we can prescribe something. But it doesn't mean that the patient is going to take it, so I find that including patients in the decision-making is just when you include them, they're a little bit more eager, and excited about being a part of that plan. And at the end of the day, that helps them, it's a win-win situation, but definitely it's individualized. There's no way to say every patient's going to be on this product because everyone...hemoglobin is different, everyone's pain level is different, maybe their platelets comes different, maybe to have ulcers which prevent them from using hydroxyurea, it really takes more of the dissections if they got what that best plan of care would be.
Dr. Marwan Shaikh:
Yeah, and then I think...well, now you mentioned about the Hydrea, it's a wonderful drug, and as you mentioned, it's definitely a chemo drug. And kind of on that note, I'd like to mention that even though it is a chemotherapy drug, it works differently than a lot of other traditional chemotherapies, it's not like chemotherapy for breast cancer, which you can have severe analogy, a vomiting, losing your hair, severe fatigue, a lot of people get away with Hydrea with no side effects, and they do well with it for years. Yes, you could have a little nausea...yes, you can still have some fatigue, but in general and most people, so it's a milder medicine and it's very well-tolerated. And I think a lot of people do have some hesitation because of that chemotherapy part, and I think we have long-term data with this, all these other agents are newer like we mentioned, but we have over 17 years, 17 years something that have your data was published 2010, a lot, and so now re-siting another 11 years after that. And people have done really well with this medicine and tolerated it well without any clear increased risk of leukemia, because people think about this drug causing leukemia down the road. And there's data saying that in certain scenarios and certain other conditions like myeloproliferative disorders or other cancers disorders, yeah, it could progress to...diseases could progress to leukemia. But in sickle cell disease and non-cancerous things, there's no good data that I know.
Definitely saying Hydrea can lead to leukemia.
Maya Bloomberg, APRN:
And we're literally putting babies on it, we have a baby health study where they literally will put babies on hydroxyurea really was when we're bringing the uses and the benefits of starting the patient, the therapy. If the risks really did outweigh the benefits, we wouldn't be putting babies on it, and we now have new research that's showing that hydroxyurea can actually reverse heart damage or prevent damage. So there really are so many benefits to hydroxyurea that when patients don't want to use it, and I know it's going to be beneficial just because it's a chemo drug. That's where I send them to the SCinfo.org. It's a wonderful website. It's actually put on by Emory. because I think in light of COVID we saw how fast this information can spread that having a reliable website and knowing what is reliable is very important, so I send a lot of patients, to SCinfo.irg or you can go to my Instagram, I post a lot of information that way. But the Emory website, they have it broken down for patients, providers, if you want to look at research, it's a really nice concise way to learn about sickle cell and research happening in an understandable, and most importantly, a reliable and factual way.
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