Sickle cell warriors depend on their providers and local health care systems to access the care they need – especially during pain crises. Whether you are a doctor, nurse or community health worker, you play an important role in advocating for these rare disease patients. Learn more about the patient experience and research into this condition.

Emergency Care and Pain Crises

Pain crises are one of the most common reasons why people with sickle cell seek emergency care. A pain crisis, or a vaso-occlusive crisis, occurs when sickle cells block blood flow. It can be caused by factors including dehydration, stress, temperature and/or an infection or illness – but it doesn’t always have a clear cause.

Per the National Heart, Lung, and Blood Institue, emergency presenting symptoms may include:

• Severe pain
• Fatigue, shortness of breath, dizziness and irregular heartbeat
• Fever
• Chest pain, coughing, fever and shortness of breath
• Sudden weakness, numbness on one side of the body, confusion, or trouble speaking, seeing, or walking
• Priapism

What Providers Need to Know

A sickle cell pain crisis can be life-threatening. When treating a patient with sickle cell disease:

• Take their pain seriously: Per the Centers for Disease Control and Prevention, patients presenting with pain need prompt treatment. NSAIDS may be effective for treating mild or moderate pain crises, but severe crises may require opioid treatment. The National Alliance of Sickle Cell Centers Opiate Dosing Calculator can help providers create a care plan.
• Review existing pain management plans: Many sickle cell patients will be able to provide you with their medical history and current pain medicines. Some may have a pain management plan in their electronic medical record or on hand. SCDAA and MedicAlert have recently partnered to provide patients in select states with a MedicAlert Smart Medical ID Card, which providers can scan to access the patient’s health profile. Take these resources seriously. They could make a life-saving difference.
• Contact the patient’s primary care physician or sickle cell specialist as needed: The patient’s existing care team will be able to answer questions about treatment plans, care needs and more. Sickle cell specialists understand that their patients may need to go to the emergency room frequently and are prepared to provide support in these situations.

Expanding Your Adult Sickle Cell Practice

Sickle cell disease is no longer just a pediatric focus. We need to meet the needs of a growing adult sickle cell population. You can help bridge the gap by deepening your understanding of sickle cell disease. We encourage providers to find support for their practice through the National Alliance of Sickle Cell Centers and the Sickle Cell Adult Provider Care Network. Provider-focused education is available through the National Heart, Lung, and Blood Institute’s Blood Diseases and Disorders Education Program. Interested in doing research? Keep an eye out for grant opportunities released by government partners at grants.gov.