Sickle_Cell_Disease_Flashcards
What prophylactic measures are recommended for patients with Sickle Cell Disease?
Prophylactic measures for Sickle Cell Disease include immunisation against encapsulated organisms (e.g., S. pneumoniae and H. influenzae type B), daily oral penicillin, and daily oral folic acid.
How should vaso-occlusive crises be minimized in Sickle Cell Disease?
Vaso-occlusive crises should be minimized by avoiding exposure to cold, dehydration, excessive exercise, undue stress, or hypoxia.
What is the recommended management for acute crises in Sickle Cell Disease?
Management of acute crises in Sickle Cell Disease includes oral and IV analgesia (avoid morphine < 12 years), good hydration (oral or IV), treating infection with antibiotics, and providing oxygen if there is reduced saturation.
When is exchange transfusion indicated in Sickle Cell Disease?
Exchange transfusion is indicated for acute chest syndrome, priapism, and stroke in Sickle Cell Disease.
What chronic problems management options are available for children with recurrent hospital admissions due to Sickle Cell Disease?
Children who have recurrent hospital admissions (>3 in 12 months) for acute chest syndrome or vaso-occlusive crises could benefit from hydroxycarbamide (stimulated HbF production).
What should be monitored when using hydroxycarbamide for Sickle Cell Disease?
When using hydroxycarbamide for Sickle Cell Disease, monitor for white blood cell suppression.
What surgical intervention may be considered for Sickle Cell Disease and what prophylaxis is needed?
Splenectomy may be considered for Sickle Cell Disease, with immunisation against encapsulated organisms needed.
What is a potential cure for severe cases of Sickle Cell Disease?
Bone marrow transplant may be considered as a potential cure for severe cases of Sickle Cell Disease.
What is the prognosis for patients with the most severe form of Sickle Cell Disease?
The prognosis for patients with the most severe form of Sickle Cell Disease can include premature death due to complications, with 50% dying before the age of 40.
