Steps to improve care coordination for sickle cell disease

Sickle cell disease is a genetic disorder where the body produces abnormal red blood cells called sickled cells, which have a crescent-like shape that resembles a farming tool called a sickle. These abnormal red blood cells are ineffective at transporting oxygen, have a short lifespan in the bloodstream, and can stick together to block blood vessels.

A sickle cell crisis (also called a vaso-occlusive crisis) occurs when sickled cells block blood vessels. This deprives tissues and organs of oxygen-rich blood and is often intensely painful. A sickle cell crisis can also lead to serious complications, including organ damage, injury to the lungs, and stroke. Over time, repeated crises can lead to many long-term health problems as damage accumulates.

Many treatment plans for sickle cell disease focus on preventing sickle cell crises, preventing complications, and improving quality of life. Medications and lifestyle changes can reduce the frequency of crises.

A select number of individuals are candidates for stem cell transplant (bone marrow transplant) or gene therapies. These approaches have the potential to cure sickle cell disease, but each involves considerable risks and side effects. People who receive these treatments will need to undergo conditioning therapy, intense chemotherapy that destroys bone marrow to eradicate any cells that carry the mutated gene causing sickle cell disease.

Regardless of how you approach treatment, sickle cell disease is best treated by a multidisciplinary team made up of different healthcare providers who specialize in different aspects of care.

Healthcare providers on a multidisciplinary team

Treatment for sickle cell disease is typically overseen by a hematologist, a medical doctor who specializes in blood disorders. In addition to a hematologist, a sickle cell disease care team can include:

• Primary care provider (for adults) or pediatrician (for children and adolescents)
• Pain management specialist
• Clinical social worker
• Counselors or other mental health specialists
• Pulmonologist (for lung and respiratory conditions related to sickle cell disease)
• Cardiologist (heart specialist)
• Nephrologist (kidney doctor)
• Orthopedist (for bone and joint complications)
• Neurologist (to evaluate stroke risk)
• Ophthalmologist (eye doctor)
• Genetic counselor

Coordinating care for sickle cell disease

“Care coordination” refers to the ability of your different healthcare providers to communicate with each other, share information, and provide comprehensive care. Research has identified care coordination as a significant challenge for many individuals living with sickle cell disease. Research has also demonstrated that care coordination has the ability to improve treatment outcomes, such as reducing hospital readmissions following a sickle cell crisis.

Strategies for care coordination

One of the best approaches for coordinating care is to work with a sickle cell team that is based in a single location, such as a medical clinic or hospital. However, the ability to take this approach can be limited by where you live and what healthcare resources are accessible in that area.

Here are two additional strategies that can help.

Designate a coordinator

Designate one healthcare provider as your "point person" or coordinator. This healthcare provider should maintain an overview of the different aspects of your care and treatment, and they should have contact information for all providers you have worked with. This may be a hematologist, a primary care provider, or a nurse coordinator.

Keep a personal health record

This is a file that includes all paperwork and documents related to sickle cell disease as well as other aspects of your health and healthcare. This file should contain:

• Copies of test results, evaluations, and workups from specialists
• Treatment records, including medications prescribed
• Contact information for all of your healthcare providers
• Insurance information
• Copies of bills, receipts, explanation of benefits documents, and other information related to the financial aspects of treatment
• An individualized care plan that outlines steps for emergency treatment during a sickle cell crisis

You can also include your own notes from appointments and information provided by healthcare providers, such as patient education materials. It also helps to keep a diary or journal, where you track symptoms and treatment.

While this can take time and effort, it can also save you time and effort. Organization can help you find relevant information quickly when contacting your insurance provider or if you are referred to a specialist.