Internal Medicine

0521779407-19 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:21

Sickle Cell Syndromes 1347

■Elevated transcranial Doppler, use chronic transfusion program to

prevent first stroke

Stroke, use exchange transfusion followed by maintenance trans-

fusions with iron chelation regimen

■Pain crisis use incentive spirometry, narcotic analgesics as appropri-

ate

■Acute chest syndrome use empiric antibiotics to cover chlamy-

dia, mycoplasma, pneumococcus, oxygen supplementation, bron-

chodilators, incentive spirometry simple or exchange transfusion,

mechanical ventilation, inhaled NO as indicated

■Cholecystitis, perform elective cholecystectomy after resolution of

acute episode

■Osteomyelitis use empiric antibiotics to cover salmonella and staph

■Priapism use exchange transfusion and operative shunting proce-

dure if necessary

specific therapy

■Hydroxyurea therapy follow CBC every 2 weeks, fetal hemoglobin

level monthly, LFTs monthly, birth control for males and females

■Chronic transfusion therapy, monitor for alloantibodies, Fe, ferritin

monthly. Start parenteral deferoxamine when iron overload present.

■Cholecystectomy can be done laparoscopically, most safely done

with preoperative simple or exchange transfusion. Similar guidelines

pertain to other operative procedures.

follow-up

■Patients on hydroxyurea monitor for toxicity as above, visits every

two weeks, tapering to every month once chronic stable dose is

achieved

■Patients on chronic transfusion typically get 2–3 units of packed cells

every 3–4 weeks.

■Relatively asymptomatic adults can be seen every 6 months

complications and prognosis

■Sickle trait is not a disease, and has no measurable morbidity or

mortality associated with it.

■SS patients have average life expectancy in the 45–50 year range.

Those with increased morbidity (higher rates of pain, acute

chest syndrome, pulmonary hypertension, etc.) have a shorter life

expectancy. This can be influenced by the use of prophylactic hydrox-

yurea.