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Table 1 - Common Complications and Treatment Options

Sickle Cell Disease (Learning Scope No. 417)

Table 1: Common Complications & Treatment Options19-22

Complication - Pathophysiology

Signs and Symptoms

Diagnostic Tests and Differential Diagnosis

Treatment Options

Hand-foot syndrome - vaso-occlusion

Symmetric hand-foot swelling

First manifestation of sickle cell disease. Consider osteomyelitis, CBC and X-ray

Fluids and pain management

Infection - decreased splenic function. Pneumococcus is the most common cause of sepsis.

Chills, fever, pain, cough, dysuria, headache, flank pain, atypical pain

Sepsis, pneumonia, UTI, meningitis, osteomyelitis; do a CBC, WBC differential, (check for elevated bands and total WBC count), blood cultures, UA

Empirically treat with antibiotics until cultures are known. Prevent infections with immunizations and prophylactic penicillin up until age 5.

Stroke - vaso-occlusion in children, aneurysm in adults. Most common in Hb SS

Headache, paresthesias, loss of motor function, aphasia

Stroke, aneurysm, meningitis; CT scan, MRI-MRA and LP. Annual TCD screening on all SS, SCD 0-thal children

Treat etiology - transfusion or exchange transfusion. Transfuse acutely and chronic transfusion program for prevention.

Acute chest syndrome - fat embolism, infection, infarction

Chest pain, dyspnea, cough, fever, acute hemolysis, falling hematocrit, new infiltrate on chest X-ray

Acute chest syndrome, pneumonia - do a CXR, ABG, cultures, CBC, bronchoscopy

Treat empirically with antibiotics, and exchange or simple transfusion, prevent with pre-op transfusion when indicated and incentive spirometry. Hydroxyurea as preventive

Splenic or hepatic sequestration - trapping of sickled red cells

Abdominal pain and swelling, falling hematocrit, pallor, weakness, shock, increased jaundice

Splenic or hepatic sequestration, gallstones, hepatitis. Ultrasound or CT, CBC and chem profile

Transfusion for sequestration treatment and prevention. Hydroxyurea.

Surgery for gallstones or recurrent splenic sequestration.

Gallstones - increased bilirubin from hemolysis

Abdominal pain, RUQ pain, fatty food intolerance, increased jaundice.

Ultrasound or CT, CBC and chem profile - high direct bilirubin and ALP.

Surgery: remove gallbladder if symptomatic.

Pain episode or "crisis" - vaso- occlusion

Pain in extremities, low back "typical crisis pain," chest pain may be rib pain

Pain crisis - look for precipitating causes such as infection, dehydration, acidosis. Do a CBC, reticulocyte count, chem profile and UA

Hydration with IV D5W. Multimodal pain management. Hydroxyurea may prevent recurrent crisis.

Aplastic crisis - bone marrow shut down from infection, low RBC count and not reticing, transient low platelets and WBCs

Weakness, lethargy, pallor, dyspnea,

headache, abdominal pain, viral syndrome picture

Aplastic crisis, do a CBC with reticulocyte count, parvovirus titers, erythropoietin level, ferritin, B12 and folate levels

Transfusion support until bone marrow responds. Give oral folate 1mg daily as supplement.

Multi-organ system failure - vaso-occlusion

Acute decline after routine pain crisis - multiple organ failure, sudden rise in creatinine, fall in platelets and hematocrit

Multi-organ system failure - evidenced by renal, hepatic, failure, ARDS, DIC-CBC, chem profile, coagulation profile, UA, CXR, ABGs, cultures

Exchange or simple transfusions can be lifesaving. Supportive care until organs recover.

Pseudo-addiction - inadequate pain treatment

Increasing ER visits for pain



Consider inadequate pain management, infection, increased anemia. Assess psychosocial aspects

Provide good pain management - believe the patient's pain level. Consider hydroxyurea therapy to prevent pain. Case management.

Bone infarction - vaso-occlusion. Most common in Hb SC

Focal bone pain, swelling, erythema, heat

Consider bone infarction or osteomyelitis. If hip or shoulder pain, consider avascular necrosis (AVN). Do a CBC and X-ray the area. Consider bone scan if pain does not resolve.

For bone infarction and AVN, treat with long-acting NSAIDs.

Joint replacement surgery.

Priapism - vaso-occlusion

Painful prolonged erection in male patients

CBC, UA, urine culture if infection.

Hydration, morphine, nitropaste, warm bath, irrigation, aspiration, transfusion, shunt procedure.

Pseudoephedrine and clonidine or nifedipine for prevention

Leg ulcer - decreased peripheral circulation

Non-healing leg ulceration

CBC, bone X-ray, bone scan if suspect osteomyelitis, consider cellulitis

Unna boots, wet to dry, leg elevation, prevent leg lesions. Oral zinc supplements.

Antibiotics only if osteomyelitis or cellulitis.

Retinopathy - vaso-occlusion. Most common in Hb SC

Decreased vision, floaters, flashes

Retinal detachment, hemorrhage or progressive retinopathy

Annual eye examinations and laser surgery

Nephropathy - vaso-occlusion

Hypertension, edema, elevated BUN, creatinine, proteinuria

24-hour urine for microalbinuria, creatinine clearance, CBC, chem profile, renal ultrasound, autoimmune workup, renal biopsy

ACE-ARB treatment, erythropoietin for anemia, low protein diet, dialysis, hydroxyurea as preventive

Delayed growth and puberty - hypermetabolic state

Small stature, delayed puberty

Dietary history and physical examination

Increased calories, consider hydroxyurea

Pulmonary hypertension

Dyspnea on exertion, chest pain, high LDH


Hydroxyurea, chronic transfusions

Iron overload from repeated transfusions

Liver and endocrine failure, heart block

High ferritin, liver biopsy, SQIDD

Desferoxamine parenteralor oral deferasirox


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