Patient A, a Black woman 26 years of age, presents with two days of escalating bone pain centered in the lower back and shins, radiating to the hips. She reports recent stress as the result of the breakup of a long-term relationship.

The patient notes that she noticed marked lethargy and mild localized pain at home a few days ago. The pain intensified from mild to moderate, and she noted mood changes. Today, the pain intensified to severe, leading her to seek emergency care. Her laboratory tests show elevated reticulocyte count and lactate dehydrogenase, with a marked rise in C-reactive protein.

After assessment and laboratory testing, Patient A is diagnosed with HbSS sickle cell disease, defined by inheritance of a sickle cell gene from each parent. The underlying single nucleotide mutation causes hemoglobin to polymerize when deoxygenated, producing sickled red cells that increase blood viscosity and occlude the microvasculature. The resultant ischemia explains the acute and chronic pain patterns and multi-organ risks.

In the emergency department, Patient A is started on intravenous fluids. To address her severe pain, parenteral morphine and corticosteroids are initiated. After she stabilizes, a parenteral NSAID is added to reduce opioid requirements and facilitate transition to oral therapy. Transfusion is not required for this episode, as she did not have severe anemia. If severe anemia were present—particularly with infection or splenomegaly—blood transfusion would be considered.

Hydroxyurea at a daily dose 15 mg/kg of is initiated as the backbone of disease modification to reduce pain crises, transfusion needs, and mortality. The patient is counseled on the importance of adherence, noting clinical response often begins after three to six months; more frequent follow-up and encouragement are planned to support adherence. L-glutamine is discussed as an option to reduce acute complications and hospitalizations, but use is deferred.

Prevention counseling focuses on avoidance of dehydration, extreme temperatures, high altitudes (including flying), and low-oxygen states from intense exercise or athletic training.

Hematopoietic stem cell transplantation is the only known cure for sickle cell disease. It requires close donor-recipient HLA matching and is suitable for a limited subset of patients. Patient A is informed about indications and risks, with agreement to revisit this option if a suitable donor is identified.

Close follow-up was arranged to titrate hydroxyurea as necessary and monitor clinical response. Pain management follow-up is also scheduled to optimize long-acting and rescue medications and incorporate adjuvants, as needed.

At two-week follow-up, Patient A reports improved baseline pain and no new crises. She is adhering to hydration and temperature-avoidance guidance, remains adherent to hydroxyurea therapy, and feels supported by the outlined plan. Ongoing monitoring is planned to evaluate response over the next three to six months, with consideration of adding crizanlizumab or L-glutamine if breakthrough crises persisted despite optimized hydroxyurea therapy.