July 21, 2015

Meeting Sickle Cell’s Challenges in Kind

Proactive approach required to prevent complications

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Sickle cell disease brings no shortage of management challenges, including a host of clinical complications, frequently spotty treatment adherence and a need for intensive patient education. In response, Cleveland Clinic Children’s Sickle Cell Program relies on a proactive, team-based strategy to ensure excellent care despite such hurdles.

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“We are well-equipped to handle the challenges our patients present,” says Grace Onimoe, MD, a pediatric hematologist who specializes in sickle cell disease and leads the program.

Along with a core team of two nurse practitioners, a social worker and a child psychologist, Dr. Onimoe works with pediatricians and general practitioners to coordinate care and obtain subspecialty expertise from across Cleveland Clinic Children’s when needed for the condition’s diverse complications. They also work in collaboration with staff from the American Sickle Cell Anemia Association (ASCAA), which has an office on Cleveland Clinic’s main campus.

“Our team provides extensive education and works closely with families to overcome barriers to successful management,” Dr. Onimoe says.

Complications can occur at any time

Approximately 1,000 infants with sickle cell disease are diagnosed in the U.S. each year through a screening blood test given at birth. In the most common form — sickle cell anemia — complications are common and can be severe. The disease’s other forms tend to be milder, but complications and exacerbations can occur at any time and include:

  • Acute pain
  • Anemia
  • Infections
  • Stroke
  • Vision problems
  • Kidney dysfunction
  • Lung problems
  • Heart dysfunction

Proactivity is paramount

A proactive, patient-centered protocol helps prevent complications. The team provides ongoing care for a sizable population of patients, each of whom is seen for comprehensive assessments every two to three months, depending on genotype and frequency of complications.

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“Our goal is to keep patients as healthy as possible,” says Dr. Onimoe. Because complications are unpredictable, the team is always on standby Monday through Friday to provide rapid evaluation and triage of crises. Nights and weekends, a pediatric hematologist is on call.

Management ranges from meds to BMT

A full complement of medications is considered for symptom management, along with iron overload therapy. The FDA-approved agent hydroxyurea is used to reduce the frequency of pain crises and increase the hemoglobin count.

Blood and marrow transplantation (BMT) may be considered for selected patients with frequent, severe complications and uncontrolled pain. Consultation with Cleveland Clinic Children’s expert team of BMT specialists is readily available to the sickle cell population.

Unfortunately, a lack of donors often prevents BMT from being used. “In northern Ohio, most sickle cell patients are African-American, and finding a bone marrow match in the African-American population is challenging,” Dr. Onimoe notes. She adds, however, that haploidentical transplantation — now being offered at Cleveland Clinic Children’s — may be considered on a research basis for patients who have no matched donors.

Softening the impact of socioeconomic issues

The Sickle Cell Program also has protocols designed to minimize the impact of the socioeconomic issues common to patients.

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Medication noncompliance is a common problem. When prescribing hydroxyurea, which must be taken daily, Dr. Onimoe gradually introduces the drug to families. Blood tests at each visit reveal whether the drug is being taken as prescribed. “We don’t leave things to chance,” she says.

Helping patients’ parents understand the importance of regular assessments can also be challenging, particularly when a parent is reluctant to take time off work for fear of losing a job. In cases like these, Dr. Onimoe calls on a counselor from the ASCAA for help in educating families and identifying resources to eliminate barriers to care.

Optimizing care through research

In an ongoing quest to provide better treatment options, this year the sickle cell program expects to gain access to investigational protocols offered through various organizations involved in sickle cell research. Resident physicians are also working on several sickle cell projects aimed to optimize patient care.

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