Delivering Integrated Care Management
The IHTC pharmacy and healthcare professionals interact on a daily basis at our center to maximize coordination and quality of care. Our pharmacists and physicians are on call and available 24 hours a day, seven days a week.
The IHTC pharmacy and healthcare professionals effectively coordinate ongoing care by proactively communicating with our patients to manage clotting factor needs, therapy compliance, and bleeding episodes.
Aplastic Crisis & Intervention
An aplastic crisis should not be confused with hemolytic anemia, which is a constant feature of sickle cell disease. The aplastic crisis is temporary cessation of red cell production. Because of the markedly shortened red cell survival time in patients with sickle cell disease, a precipitous drop in hemoglobin occurs in the absence of adequate reticulocytosis.
Among patients with sickle cell disease, severe aplastic crisis is frequently (86% of cases) associated with parvovirus B 19 infections (childhood fifth disease). Other causes may be EBV or Strep infection as well as use of hydroxyurea. Patients typically present with increased fatigue, pallor, activity intolerance or shortness of breath. Pallor may replace the typical jaundice of sickle cell disease. The period of viremia lasts about one week. The CBC reveals a lower than relative normal hemoglobin and a decreased reticulocyte count. The aplasia usually persists for 5-10 days. Pancytopenia does not usually occur. The treatment is management of symptoms of anemia with simple transfusion using extended antigen-typed, leukodepleted PRBCs. Recovery is heralded by increased peripheral NRBCs followed by reticulocytosis. This phase of recovery is also often marked by hyperbilirubinemia, and called a “hyperhemolytic” phase. The incidence of recurrent parvovirus B 19 is very low. IgM titers begin to rise by about day 7 and peak by day 21. As normal erythroid function returns, IgG rises and remains elevated, indicating past infection. If the patient develops an aplastic crisis off folic acid supplementation, it is wise to initiate or resume 1mg daily, as folate deficiency will slow recovery. Children in the general community do not require isolation. Patients admitted to the hospital are often managed in isolation. Hematologically normal people who are infected by parvovirus usually do not develop red cell aplasia or other serious complications.