In honor of National Sickle Cell Month, we spoke to Dr. Geetha Puthenveetil, a pediatric hematologist and director of blood and donor services at CHOC, about the multidisciplinary care and other resources available to patients with sickle cell disease at CHOC.
Q: What is sickle cell disease?
A: Sickle cell disease is an inherited disorder wherein the red blood cells are misshapen. Instead of their standard round shape, the red blood cells are shaped like sickles, or crescent moons. This can lead to painful episodes and even hospitalization when these misshapen red blood cells get caught up in small blood vessels when they try to carry oxygen to different parts of the body. Sickle cell disease is usually diagnosed at birth during newborn screening tests.
Q: How do patients with sickle cell benefit from CHOC’s comprehensive red cell clinic?
A: I started this clinic for patients with a number of red cell disorders—including sickle cell disease, thalassemia, aplastic anemia, and Diamond-Blackfan Anemia. The clinic is attended by a cardiologist, endocrinologist, and hematologist and provides comprehensive care for patients.
Our multidisciplinary clinic offers continuity of care and expedited service for patients and families that they don’t find elsewhere. Since their entire care team is in one place, families can get more immediate answers to their questions. All members of the team are involved in creating the care plan for how best to treat each patient.
Q: What should parents know about the hematology program at CHOC?
A: Along with other centers, we are a part of a growing number of clinical trials to decrease pain crises in patients with sickle cell, and their amount and length of hospitalizations. We are also part of international study looking at standards of patient care.
Patients can also benefit from neuropsychology evaluations. Our pediatric neuropsychologist is specially trained in working with patient with sickle cell disease, and can help evaluate if their medic al condition is affecting their cognitive functioning, and lay the groundwork for treatment.
We also offer a support group for families of patients with sickle cell. Families who are new to their diagnosis or our program have the opportunity to be mentored by more experienced families who can relate to what they’re going through. This group is open to all caregivers of our patients, from teachers and principals to babysitters and day care operators, who are interested in learning more about sickle cell, and what they can do to offer better care.
Q: What aspect of pediatric hematology/oncology are you most passionate about?
A: No two patients are alike. Each and every sickle cell patient has their own challenges and battles, and each requires a unique strategy. As a hematologist/oncologist, you need to constantly think outside the box.
Despite all the health challenges my patients are facing, they always keep me laughing. In pediatrics, you’re not just treating the child, but the whole family. It adds to the challenge of providing care, but it’s essential to engage their entire environment in order to create the best outcomes for them.
Q: What impact do blood donors have on patients with sickle cell?
A: Blood donors are of utmost importance, and they are crucial for patients with sickle cell disease. Sickle cells have a shorter life span than normally-shaped cells, which can lead to anemia, or a low red blood cell count. Our patients depend on the generosity of blood donors.
Patients with sickle cell can develop antibodies after their great number of transfusions, so our team is focusing on red blood cell genotyping, so we will be able to more closely match them to specific and regular blood donors. Our ultimate goal is having patients develop fewer antibodies because they’d be better matched with their donor at a gene level.