Sickle Cell Disease: Applying Comprehensive Care to Patients

EP: 1.Overview of Sickle Cell Disease: Pathophysiology and Clinical Burden

EP: 2.Progression of SCD and Management Throughout a Patient’s Life

EP: 3.Sickle Cell Disease Global Prevalence

EP: 4.Sickle Cell Disease and Newborn Screening

EP: 5.Management of SCD: Educating Patients on Symptoms and Lifestyle Modification

EP: 6.Sickle Cell Disease: Overview of the Comprehensive Care Team

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EP: 7.Sickle Cell Disease: Applying Comprehensive Care to Patients

EP: 8.Patient Follow-up and Pain Management in Sickle Cell Disease

EP: 9.Treatment Goals and Guidelines in Sickle Cell Disease

EP: 10.Hydroxyurea Use in Sickle Cell Disease

EP: 11.Sickle Cell Disease Management: L-Glutamine Therapy

EP: 12.Crizanlizumab Use in Sickle Cell Disease

EP: 13.Sickle Cell Disease Management: Voxelotor Therapy

EP: 14.Factors in Selecting Disease-Modifying Therapy in SCD

EP: 15.Understanding the Risk-Benefit Ratio of Gene Therapy in SCD

EP: 16.Educating Patients on Gene Therapy in Sickle Cell Disease

EP: 17.Sickle Cell Disease: Unmet Needs and Future Directions in Care

Transcript:

Ifeyinwa Osunkwo, MD: As we wrap up this session, I want us to think about the next session: treatment options for the management of sickle cell disease. In this section, we’re going to talk about how a comprehensive team works together in the medical home or in the life span clinic to address sickle cell disease complications. What are the actual interventions we do? What are the treatment options available? Who should get them? Who shouldn’t get them? What are the pros and cons of therapy, the potential adverse effects, and how well do they work?

We’re going to go on to section 2, which is treatment options for the management of sickle cell disease, starting with Dr Desai. Dr Smith had talked about the life span and comprehensive care team. Dr Desai, share with us your experience and how you implemented this [when you were] at The Ohio State [University Comprehensive Cancer Center]? What did that look like?

Payal Desai, MD: There’s a great paper that a lot of experts across the country contributed to that said, what are the essential parts of what makes a sickle cell center, what are the more adjunct pieces that you can consider, and what are the next-level things? I wouldn’t call any of them luxuries because really, what are the core basics to have a true comprehensive model that will provide comprehensive care? I think about sickle cell disease in terms of a biophysiological model—you have the biology piece, and you need somebody who understands sickle cell to deal with a sickle cell piece. That includes monitoring therapies, thinking about blood clots, and sickle cell complications. For us, we had a partnership with a primary care [physician], so we had a home visitation program for patients who were complicated and older with multiorgan failure, who we couldn’t see because of retinopathy, and who had to get to dialysis. They would do some home visits for the patient, and then we had a second integrated primary care. As you talked about, not just the gout and the hypertension which can sometimes be disease-related, but are they getting their breast cancer screening? Who’s looking at patients for colon cancer? What about the other things you think about in terms of primary care? We had that piece covered from the biology side.

The second part is the social work: looking at the patient’s home, at what support structure they have, to see if they have food and transportation. You can’t think about medicine and hydroxyurea if you’re figuring out how to get your family food and your kids to school, or making sure that they’re staying in school. They have reports for their teachers to know how to take care of their sickle cell disease, so [we have to] prioritize other factors that play a huge role. With psychiatry, we think about depression and anxiety—no medication is going to work for the treatment of sickle cell if you don’t treat the underlying depression. It doesn’t matter how good the therapy is, the pain medication doesn’t work, the hydroxyurea doesn’t work. If you have untreated mental health issues, they can’t take the place of that.

The other essential parts to think about are the environment. That self-advocacy piece is almost bringing that patient in—thinking about what the community health workers are doing, how are you supporting that piece, how are the patients themselves advocating for themselves and participating in care? I think about all those pieces, and that’s what we’re trying to bring together with a patient care coordinator, a social worker, with inpatient-outpatient coordination. When people discharge, sometimes there’s mix-up of medications, even in the best situations. We looked at that, and 25% of people were like, “I thought I was supposed to be taking this, and it looks like my list is different.” In a comprehensive center, you’re approaching that plus starting to build those next things when you understand how to bring a nephrologist into the situation, how to start having retinopathy screening. We brought in eye cameras, so you can do undilated exams in the clinic if patients can’t get to their ophthalmologist. We need to think about how to use technology to help you bring those other specialties into your clinic for our patients.

Ifeyinwa Osunkwo, MD: I love what you just said. You summarized, like a pizza pie, how sickle cell care should be given. It should be done with the disease-magnifying therapy, medical complication assessment, psychosocial, and then the self-advocacy and pulling in different specialists to support the overall organ function and treatment of the individual patient.

Transcript edited for clarity.