Sickle cell disease (SCD) is a chronic, genetic blood disorder which causes painful attacks (sickle cell crises) that can be difficult for patients to manage. Patients with SCD over their lifetime can have problems which affect practically every part of the body.  SCD changes normal, round red blood cells into ones shaped like crescent or half-moons. Normally, healthy red blood cells carry oxygen through the body. However, people with SCD do not have enough normal cells to carry the right amount of oxygen. This causes the sickled cells to get stuck and block blood vessels, which stops the oxygen from getting through the body and causes a lot of pain. When there is a blockage, the hands, feet, abdomen, back, or chest are affected.

Along with pain, symptoms of SCD can include fatigue, shortness of breath, rapid heart rate, paleness or jaundice, swelling of the hands or feet, and an increased rate of infections. Sometimes, SCD can lead to damage of many organs throughout the body, delayed growth, vision problems, and strokes.

Center of Excellence in Sickle Cell Disease

BMC’s Center of Excellence in Sickle Cell Disease is the largest center of its kind in New England, serving more than 450 patients annually, from newborns to adults. The center’s multidisciplinary team is committed to providing patients across their lifespan with highly personalized care to manage their symptoms associated with the disease so they can live as normal a life as possible.

The SCD team includes physicians and a nurse practitioner from pediatric and adult hematology, adult and pediatric primary care, and other specialties as well as nurses, social workers, case management and a patient navigator to help guide patients through their care. The program focuses on providing consistent care between the Emergency Department, inpatient services, and outpatient clinics.  The program utilizes the pediatric STAR unit and the Infusion Center in the adult Hematology/Oncology clinic for outpatient transfusions and pain management. 

Features of the Center

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Specialty Clinics

Hematology

Patients with blood disorders receive consultation and treatment for a broad range of benign conditions, such as hemoglobinopathies (sickle cell anemia and thalassemia), hemochromatosis, bleeding and thrombotic disorders (von Willebrand's disease and hemophilia), hypercoagulable states, myeloproliferative disorders, myelodysplasia and anemia, and malignancies such as leukemia, lymphoma and myeloma.

Treatments & Services

Treatment of Sickle Cell Disease

Treatment of young patients usually includes antibiotics from 2 months to 5 years of age to help prevent infections. Children and adults should be seen regularly in the outpatient clinic to assess for symptoms, screen for complications of the disease and promote general health and well-being.  We recommend appropriate immunizations across the lifespan. Many patients living with SCD experience pain on a daily basis and using the right combination of therapies can help to treat this.  Developing a pain management plan with a physician or pain management specialist is encouraged at the center. 

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Diagnostics and Tests

Sickle cell disease can be diagnosed by a blood test. Every state requires that all babies, before they leave the hospital, are tested for SCD as part of their newborn screening. 

Our Team

Elizabeth S Klings, MD

Assistant Professor of Medicine, Boston University School of Medicine, Boston MA

Special Interests

Pulmonary complications of sickle cell disease, Pulmonary Hypertension, Chronic dyspnea

Adult Clinic

Lillian E McMahon, MD

Associate Professor of Pediatrics and Medicine, Boston University School of Medicine

Special Interests

Adult Sickle Cell Hematology

Martin H Steinberg, MD

Professor of Medicine, Pediatrics, Pathology and Laboratory Medicine, Boston University School of Medicine

Special Interests

Sickle Cell Disease

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Charlene M Sylvestre, NP

Instructor of Medicine, Boston University School of Medicine

Special Interests

Adult Sickle Cell Hematology

Pediatric Clinic

Robyn T Cohen, MD

Director, Pediatric Pulmonary & Allergy Clinic
Associate Professor of Pediatrics, Boston University School of Medicine

Special Interests

Asthma, Sickle Cell Lung Disease, Recurrent Pneumonia, Chronic lung disease of prematurity, Obstructive Sleep Apnea

Patricia L Kavanagh, MD

Assistant Professor of Pediatrics, Boston University School of Medicine, Boston MA

Special Interests

pediatric emergency medicine

Caitlin M Neri, MD

Instructor of Pediatrics, Boston University School of Medicine, Boston MA

Special Interests

Pediatric Hematology/Oncology, Sickle Cell Disease, Chronic Pain, Palliative Care, General Pediatric Hematology

Amy E Sobota, MD, MPH

Pediatric Hematologist and Medical Oncologist

Special Interests

sickle cell disease, iron deficiency, menstrual disorders, general pediatric hematology

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Social Workers

Mandi Curtis, LCSW

Abby Jeffers, MSW, LCSW

Hematologists

Lillian E McMahon, MD

Associate Professor of Pediatrics and Medicine, Boston University School of Medicine

Special Interests

Adult Sickle Cell Hematology

Charlene M Sylvestre, NP

Instructor of Medicine, Boston University School of Medicine

Special Interests

Adult Sickle Cell Hematology

Additional Information

Research Overview

The physicians and staff of the BMC’s Center of Excellence in Sickle Cell Disease are also affiliated with the Boston University School of Medicine (BUSM). For more information on research studies, please visit the Center of Excellence in Sickle Cell Disease BUSM website.

Affiliations

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As the principal teaching affiliate of Boston University School of Medicine (BUSM), Boston Medical Center is devoted to training future generations of healthcare professionals.  Learn more about Boston University School of Medicine.