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Adult Sickle Cell

Active Research Studies

Learning More about Sickle Cell Disease and Treatments through Clinical Research

Patients in the Adult Sickle Cell Disease Clinic have been very interested in participating in local and national studies. Investigators from University Hospitals and Case Western Reserve University School of Medicine have developed these studies together, or are collaborating with investigators at other centers across the nation.

These studies are only possible because of the interest and enthusiasm of people with sickle cell disease in seeking to understand how their disease works and how we can make it better.

Medical Therapy

Alkali Therapy in Subjects with Sickle Cell Disease (SCD) – Evaluation of Efficacy, Safety, and Beneficial Effects (Jane Little, MD and Tom Hostetter, MD)

  • Bicarbonate keeps the blood neutral, not too acid or too basic
  • Bicarbonate levels are unusually low in patients with HbSS (sickle cell anemia)

    We want to see whether oral sodium bicarbonate (baking soda) supplements, given over three months, will improve acid and salt balance in the blood. We will also test whether the kidneys work better, or whether red blood cells are less fragile, when bicarbonate levels in the bloodstream are more normal.

Hematopoietic Stem Cell Transplant for Sickle Cell Disease (Jane Little, MD and Marcos deLima, MD)

  • Transplantation of non-sickle stem cells into patients with sickle cell disease may be used to treat sickle cell disease, but half of the transplants do not take.
  • New approaches have allowed safe stem cell transplant from less than fully matched or unrelated non-sickle cell donors into patients with sickle cell disease.

    We are testing whether one month of pre-treatment of people with sickle cell disease with immune suppressants before a stem cell transplant is safe. We are asking whether this pre-treatment helps the sickle cell disease patient hold onto the donor cells more successfully after transplant.

Relieving Symptoms

Comparison of Patient Centered Outcomes for People with SCD in the Acute Care Setting (Jane Little, MD, local Principal Investigator)

  • Rapid and adequate treatment of pain is critically important for people with sickle cell disease.
  • Pain treatment can vary between acute care clinics (infusion centers) and the Emergency Department (ED)

    The purpose of this study is to compare the care sickle cell patients receive during a vaso-occlusive pain crisis in the ED with the care they receive when going to the acute care clinic. In this study, participants agree to contact a study team member (within three days) whenever they receive treatment for a vaso-occlusive pain crisis or go to the emergency department or acute care clinic for any reason.

    We are one of four sites across the nation that are participating in this important study that was developed at Johns Hopkins (Case Western Reserve University, Johns Hopkins University, Medical College of Wisconsin, Baton Rouge).

The effects of a single music therapy session on the pain of adult patients with sickle cell disease: a mixed methods study
Principal Investigator: Samuel Rodgers-Melnick, MT-BC
Co-Investigators: Tara Pell, MA, MT-BC; Deforia Lane, PhD, MT-BC; Jane Little, MD

  • Rapid and adequate treatment of pain is critically important for people with sickle cell disease
  • Music therapy has been proven to help people with other diseases manage pain

    We want to test whether a single 20-minute music therapy intervention with a music therapist affects pain intensity, pain relief, and mood of adult patients with sickle cell disease in the acute care clinic. We will compare this with standard care and listening to music without a therapist present.

The effects of music therapy on transition outcomes in young adult patients with sickle cell disease
Principal Investigator: Samuel Rodgers-Melnick, MT-BC
Co-Investigators: Tara Pell, MA, MT-BC; Deforia Lane, PhD, MT-BC; Jane Little, MD

  • Young adults, who have transitioned from pediatric care within the last five years, face exceptional challenges

    Here, we want to find out if music therapy improves young adult patients’ knowledge of their disease, ability to manage their disease, adherence to medications, and trust in their healthcare team.

Understanding the Disease

Sickle Cell Disease (SCD) Biochip: Towards a Simple and Reliable Way to Monitor Sickle Cell Disease. (Jane Little, MD and Umut Gurkan, PhD)

  • Red and white blood cells are abnormally “sticky” to each other and to the blood vessel wall in sickle cell disease

    We are asking whether small amounts of blood (spare blood, left over after blood counts have been analyzed) from people with sickle cell disease can be analyzed for stickiness. The blood samples are run through tiny channels in the lab, and then analyzed for what they tell us about disease activity. We hope this will eventually be a rapid test that may guide treatments in sickle cell disease. In this study, we are working with engineering experts from Case Western Reserve University.

Stress Erythropoiesis in Anemia (Jane Little, MD)

  • Red blood cells in sickle cell disease may differ from other red cells because they are always exposed to the stress of anemia

    We are asking patients for extra blood during their routine clinic blood draws, which we can then test for evidence of changes from anemia that may help us to understand how anemia affects red cell production in sickle cell disease. In this study, we are working with experts at Pennsylvania State College.