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.
Normally flexible and smooth red blood cells pass swiftly through the spleen. The spleen receives about 3 to 5 % of the total blood volume per minute. The immunologically competent portion of the spleen (white pulp) receives a relatively small portion of blood flow as compared to the splenic filter portion (red pulp).In most older children and adults with Hg SS disease the repetitive intrasplenic sickling and local infarction eventually results in scarring, fibrosis, and a non-functional spleen (called functional asplenia or auto splenectomy). In infants and young children (up to age 5-6 years), some splenic congestion and splenomegaly is not unusual.
Acute splenomegaly, pallor, or lethergy can be the first clinical signs of a potentially life threatening splenic sequestration crisis.
Splenic sequestration refers to an acute condition of intrasplenic pooling of large amounts of blood. Children with sickle cell disease between ages 5 months and two years represent most cases of splenic sequestration. During severe sequestration crisis, the blood-filled spleen may enlarge to the point of filling the entire abdomen. The child’s hemoglobin may drop acutely (to as low as 1-3g/dl) resulting in hypovolemic shock and death within hours of initial onset. Prompt treatment with volume expanders and blood transfusion to reverse the hypovolemic shock can help remobilize the blood sequestered in the spleen and lead to regression of the splenomegaly over a fairly short period of time.
Minor sequestration events are common in young children with sickle cell disease. Some cases are associated with viral illnesses. Mild episodes are characterized by moderate splenomagaly and often a 1-3g/dl decrease from the patient’s baseline hemoglobin and/or thrombocytopenia.
Due to overall less sickle related infarction, the spleens of older children and young adults with Hg SC or sickle beta thalassemia may remain enlarged (persistent splenomegaly) or retain the ability to enlarge. Therefore, they are also at risk for sequestration.
Because sequestration tends to recur and because of the acute nature of potentially catastrophic symptoms and fatal outcome, Splenectomy should be considered if the child has had more than one episode.