CURRENT SERIES:
The Challenges and Treatment of Sickle Cell Disease

Sickle Cell Disease: Addressing Complications and Quality of Life

A comprehensive discussion on sickle cell disease complication management that addresses disease severity and quality of life.


Neil B. Minkoff, MD: Ahmar, we’ve talked a lot about the different things we can use as therapies, and that you can use as therapies that we can support. But what other precautions can patients be doing in terms of preventing complications besides medical and pharmacy benefit stuff?

Ahmar U. Zaidi, MD: We discussed earlier a little bit about the role of antibiotics in preventing infection complications in patients, and that goes until about the age of 5. After that there’s really no benefit of antibiotics unless patients are in the setting of an acute fever. Beyond that, we of course provide patients with pain medications, both NSAIDs [nonsteroidal anti-inflammatory drugs] and opioids, to help them control their own pain at home. We often drive home the point with patients that hydration, hydration, hydration is going to keep you out of the hospital. Keep hydrated, keep drinking water. And that tends to correlate quite nicely with how frequently patients are coming into the hospital.

We do see that patients who live in less temperate environments and more extreme temperatures, particularly the cold, tend to have more pain, particularly in the winter months. Over the summer, the number 1 reason for coming to the emergency department with pain at our children’s hospital was a sickle cell patient jumping into a pool because of the extreme temperature change and then coming out of the pool and having the wind hit them as the water’s still on them. So we talk to patients about managing temperature control. For example, for a sickle cell patient going to the OR [operating room], we ask the OR to keep it a little bit on the warmer side, things like that. There is an effect of altitude on sickle cells, particularly high altitude, plane flights, areas that are quite above sea level, tend to cause more compilations.

Neil B. Minkoff, MD: How does the severity change over time?

Ahmar U. Zaidi, MD: It’s really a cumulative effect of what damage the sickle erythrocytes are doing to your body. We see that patients have these additive issues, where you’re having repeated pain episodes and that plays again into that psychosocial component where patients start having, for example, PTSD [post-traumatic stress disorder] with every subsequent pain episode. That makes it even more difficult to manage that pain episode on a subsequent visit. We see that cumulatively as you have more endothelial dysfunction, you have more immune dysregulation, we have a body that’s heading very rapidly toward multi-organ dysfunction and failure.

Neil B. Minkoff, MD: Now I want to ask you if the severity or how it’s being presented to you affects management style or the number of steps that a patient needs to go through to get to different therapies.

Maria Lopes, MD, MS: Well, I would say the severity certainly impacts what the options are but also then at that point, usually you’re now even higher cost and the damage is done. From a case management standpoint, there’s a lot of care coordination as you have multi-organ failure, perhaps even more referrals. It’s unfortunate that’s where the resources then become used in terms of the attention to the patient, where perhaps had we been more effective early on in controlling pain and more patient education around their own responsibility and accountability for what they should be doing from a day to day perspective, we’d have better outcomes.

John C. Stancil, RPh: Absolutely, and it moves from the pharmacy benefit to more of a medical benefit at that time. Pharmacy costs are probably treating the end organ damage, the comorbidities that come with the progression of the disease, but it certainly moves to that more costly area of our program, which is the medical side with hospitalizations and ED [emergency department] visits.

Neil B. Minkoff, MD: Are there other steps we should be taking for quality of life?

Ahmar U. Zaidi, MD: I think that the most forgotten about component of this disease is that psychosocial component. That tremendously affects quality of life. And I don’t think providers are doing a great job at addressing that, and a lot of that is driven by the fact that providers don’t have resources to address those psychosocial issues that patients may be facing. There are centers that have psychologists and social workers, but those are few and far in between. So we do struggle with providing the support that’s necessary to maintain the quality of life.

Neil B. Minkoff, MD: Is there a payer role?

John C. Stancil, RPh: Yes, I think we’re transitioning to a managed care environment or program, and part of that program is to identify what we call—we don’t use the term social determinants—healthy opportunities. And there’s the realization that that impacts the quality of life more so than drug therapy will, that if you address those social determinants, that transportation need, that food need, those things have a greater impact on the quality of life that a patient can live or has the ability to live than any program or the services you’re providing.

Maria Lopes, MD, MS: I totally agree, there’s the component of understanding what’s happening in the home or lack thereof, including if there is heat, if there is shelter, if there is transportation, if there is a caregiver, as well as where the access to care is occurring. Because perhaps the emergency department for some of these patients and the churn that occurs is where you’re getting your care repeatedly, which is certainly not the most appropriate setting.

John C. Stancil, RPh: I think the reality is that this is actually a disease that’s been neglected. Certainly, there are other pediatric or child-born diseases that are getting a lot more attention. There have been emerging therapies. There’s the lack of comprehensive care around that patient population to address those needs.

Maria Lopes, MD, MS: Maybe hemophilia is actually a good model, right? We’re bringing in all the other resources that really help the patient.

 
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