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Considering Complications of Sickle Cell Disease


Neil B. Minkoff, MD: What’s the level of severity of the cerebral vascular incidents that you mentioned?

Ahmar U. Zaidi, MD: It ranges. We have a fair number of individuals who have silent strokes that are caught just on routine MRIs [magnetic resonance imaging], and we have patients who walk into our emergency department with limb paralysis. Although the majority of CNS [central nervous system] incidents are ischemic, there’s a small incidence of patients who have hemorrhagic incidents that tend to be a little bit more severe in their presentation.

Neil B. Minkoff, MD: And in infectious disease complications?

Ahmar U. Zaidi, MD: Absolutely. We used to see a lot of infectious disease complications, and the initiation of penicillin prophylaxis in the individuals who were less than 5 years old really altered that dynamic. We hardly ever see fulminant sepsis in individuals who have been on antibiotic prophylaxis and have been vaccinated appropriately against the incapsulated organisms that the spleen should be fighting but can’t in sickle cell disease because of their hyposplenic or poor spleen function.

John C. Stancil, RPh: Do you think the use of prophylactic antibiotics is maximized in this patient population, especially the pediatric population?

Ahmar U. Zaidi, MD: I think we do a reasonable job of ensuring that our patients under 5 remain on antibiotics. It really helps to have parents who are engaged and involved in the care of these individuals. Is there room for improvement? Absolutely. Is our adherence 100%? Probably not.

John C. Stancil, RPh: And I understand it’s a CMS quality measure, but it’s voluntary reporting. So I’m not sure we know what we’re doing other than in the individual practice.

Ahmar U. Zaidi, MD: That’s very true.

Maria Lopes, MD, MS: I think also as you get into it, these strokes are happening in children, young adolescents.

Ahmar U. Zaidi, MD: Absolutely.

Maria Lopes, MD, MS: Not in their 40s.

Ahmar U. Zaidi, MD: That’s correct.

Maria Lopes, MD, MS: So we have the ability to actually predict who is at higher risk of a stroke. If they have access to the right care, they can have transcranial Dopplers.

Ahmar U. Zaidi, MD: Absolutely.

Maria Lopes, MD, MS: Because then there is something we can do to prevent the stroke.

Ahmar U. Zaidi, MD: That’s right.

Maria Lopes, MD, MS: And that’s really a huge hallmark here.

Ahmar U. Zaidi, MD: That’s right, absolutely. This imaging biomarker, transcranial Doppler, has been just huge in reducing our stroke incidents. We’re able to stratify quickly who’s at risk for a stroke. The issue becomes getting patients to the transcranial Doppler appointment; getting patients to become compliant still is an issue.

John C. Stancil, RPh: Yes.

Neil B. Minkoff, MD: A lot of the things we’ve been talking about so far are acute complications, right? A vaso-occlusive crisis, pain from that or pain related to the inflammation, the cerebral vascular incidents, and so on. But there’s a chronic component to this, right? So how do those progress and evolve?

Ahmar U. Zaidi, MD: The major chronic complication of sickle cell disease is chronic pain. And essentially what we’re seeing there is a component of neuropathic pain that doesn’t respond tremendously well to opioids, but we don’t really have anything better to treat it with at this point.

A large number of patients deal with chronic neuropathic pain. It’s an underrecognized entity by providers. It’s difficult to test for and even more difficult to treat. On top of that, with the endothelial dysfunction that we discussed earlier, patients tend to run into chronic issues of pulmonary hypertension, which is a very prolific problem in our aging sickle cell population that’s quite difficult to manage at times.

And then the last one that I want to touch on is an entity called avascular necrosis, which tends to occur in the hip and shoulder joints of individuals with sickle cell disease, particularly in the subtype hemoglobin SC, where we basically have sickling occurring in the small vessels of these joint spaces. That tends to cause quite a bit of decrease in the quality of life with these patients.

Neil B. Minkoff, MD: I hear all of that and think about the move from the acute complications, which we touched on, to the chronic, I have a couple of well-known payers who are very thoughtful about how are those impacting your work, but also how that goes upstream to the employers or the government as payers.

John C. Stancil, RPh: Well as I’ve mentioned earlier, my pediatric or child population represents about $22 million spent for about 3000 patients. With 4000 patients, that number goes up to almost $41 million. So as that disease progresses, and we’re not having modalities that can treat the upstream progression of this disease or preventing it from getting worse as they age, then we’re going to see higher costs.

Maria Lopes, MD, MS: It also appears that we’re focused on symptoms, and yet the opportunity is to focus on disease modifying therapies.

John C. Stancil, RPh: Right.

Maria Lopes, MD, MS: And so as payers, many times what we end up seeing is the emergency department visits, the hospitalizations, the cycling through the emergency department, which then brings case management usually into the fold because now these patients are high cost. Hopefully we’ll have the ability to connect them to centers of excellence, connect them to specialists to deal with the social determinants, including transportation or housing. There’s also sometimes a behavioral health component to this that maybe you can speak to.

Ahmar U. Zaidi, MD: Sure.

Maria Lopes, MD, MS: That obviously has an impact on the way these patients function, their attitudes toward certain treatments, and even patient accountability, that care for themselves. So this all bubbles up into even bigger issues around compliance, adherence, follow-up, and even access to care in some of the rural areas.

Ahmar U. Zaidi, MD: Absolutely. I want to add on to that, that’s very well said. In sickle cell disease we focus, you’re right, on the biology. But truly, through and through, this is a biopsychosocial disease. And unless you’re addressing all 3 components of this disease, you’re not going to make any real improvement.

Neil B. Minkoff, MD: I want to come back to that in just one second, but I did want to ask you a follow-up before we go there, which is, is this a disease state where you’ve had other payers or employers be addressing the cost of it, and what are we doing about it?

Maria Lopes, MD, MS: I would say employers not so much because I think we have to remember that as we look at commercial versus Medicaid versus Medicare, a lot of these patients may not make it to the ripe old age of 65. Some of them may be duals. But this disproportionately affects Medicaid because of the disability component, which can happen very early on. And so I don’t think it’s top of mind for employers. When you run the data on the commercial side, usually sickle cell disease is not in your top 10, probably not even in your top 20.

Neil B. Minkoff, MD: Right.

Maria Lopes, MD, MS: But in Medicaid it certainly is.

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