Table 4.
Diagnosis and treatment of acute complications of SCD.
| Complication | Clinical presentation and evaluation | Treatment |
|---|---|---|
| Fever | May be a manifestation of an acute and sometimes life-threatening complication such as acute chest syndrome (ACS) or osteomyelitis. CBC with WBC differential, reticulocyte count, blood culture, and sensitivity. Perform urine culture and lumbar puncture for CSF analysis if urinary tract infection or meningitis is suspected respectively. Chest X-ray if signs of ACS |
Immediate medical attention Temperature ≥39.5°C: admit to hospital for a 7-day course of IV antibiotics and close monitoring. Temperature ≥38.5°C: administer IV antibiotics with coverage against Streptococcus pneumoniae and gram-negative enteric organisms (e.g., ceftriaxone, 75-100 mg/kg), then subsequent outpatient care and follow-up within 24hr. Continue with an oral antibiotic is feasible in patients who do not appear ill or toxic looking. Subsequent antibiotic change should be based on culture and sensitivity results |
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| Vaso-occlusive crises (VOC) | Manifests as sudden or gradual excruciating pain, most commonly in limbs, back, chest, and abdomen. Triggers include infections, stress, and cold exposure, among others Determine associated symptoms, location, and severity of pain Severity of pain is based on the patient report. Use a pain severity assessment scale such as the Wong-Baker facies |
Treatment is individualized. Depends on severity of pain, patient or caregiver knowledge of predictably effective agents and doses, and previous adverse events. Initiate analgesics within 30 minutes of triage. Mild pain: non-opioid analgesics. Paracetamol 15mg/kg/dose 4 to 6hrly (maximum = 60mg/kg/day) +/- Ibuprofen 5-10mg/kg/dose 6-8 hrly OR diclofenac 1mg/kg PO/suppository 8hrly +/- adjuvants (i.e. anxiolytics, antidepressants) Moderate pain: non-opioid analgesics as above alternated with an opioid, e.g., codeine 0.5-1mg/kg every 3-4 h, dihydrocodeine 1mg/kg/dose 8hrly, oral oxycodone 0.15-0.20 mg/kg every 3-4 h, hydrocodone 0.15-0.20 mg/kg every 3-4 h, and tramadol IV 2 hr, +/- adjuvants Ketorolac 0.5 mg/kg/dose 6 hr (max=30mg/dose, 60mg/day). Avoid use with other NSAIDs. Severe pain: Oral morphine sulfate 0.2-0.4mg/kg/dose (max=20mg/dose) or diamorphine 0.1mg/kg/dose in IV infusion or IM/SC stat for immediate pain relief, then maintenance oral morphine 0.2-0.4mg/kg/dose 3-4 hourly. IV diclofenac 1mg/kg/dose 8 hourly after opioids Morphine IV or SC (0.05–0.15mg/kg over 10 minutes) 2-4hr or hydromorphone IV 0.015-0.020 mg/kg 3-4hr. In case of opioid-induced respiratory depression, give naloxone IV 10 μg/kg (Max=8mg). Patient-controlled analgesia (PCA) may be provided Consider ketamine infusion at analgesic dose (0.1–0.3 mg/kg/hr, Max=1 mg/kg/hr) if pain is refractory to opioids When using morphine, administer laxatives (e.g., lactulose, senna) because it can cause constipation. Treat pruritus (itching) after opioid administration using oral antihistamines, and nausea/vomiting using antiemetics Avoid meperidine (risk of neurotoxicity – dysphoria, irritable mood, clonus, and seizures) unless it is the only effective opioid (0.75-1.0 mg/kg 3-4hr) Reassess patient after every 15-30 minutes to determine effectiveness of pain medication (+adverse events) and adjust accordingly Oral hydration. If unable to drink fluids, provide IV hydration at maintenance fluid rate Oxygen therapy if SPO2 <95% on room air Adjunctive nonpharmacological approaches (e.g., local warm application, massage, reassurance, and distraction through stories and play) Psychosocial support Rule out and treat malaria or bacterial infections (including osteomyelitis, septic arthritis) if confirmed, avascular necrosis, ACS Do not transfuse with blood unless other indication is present |
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| Splenic sequestration crisis | Due to sudden progressive enlargement of the spleen caused by pooling of blood in the spleen. Quick drop in Hb level ≥2 g/dL below the baseline value. Can cause hypovolemic shock and death. Common in children <6 years with HbSS and some older children and adults with HbSC or HbSβ-thal CBC and reticulocyte count (reticulocytosis, elevated circulating nucleated RBCs, anemia, and thrombocytopenia) |
10ml/kg of packed RBCs or 15ml/kg of whole blood over 2-4hr to raise Hb to stable level, but not exceeding 8g/dl. Risk of hyperviscocity. Immediate IV fluid resuscitation if hypovolemia – normal saline 20ml/kg Monitor vitals Analgesics for pain Investigate and treat infections, malaria Monitor splenic size 12-24hr Splenectomy if ≥2 episodes occur, and in patient with chronic hypersplenism |
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| Aplastic crisis | Acute acquired red cell aplasia caused by parvovirus B19 infection Presents with weakness and easy tiredness, fever, facial erythema, headache, severe anemia, and low reticulocytopenia (<2% of total RBC count) |
Isolate patient – droplet precautions Transfuse with RBCs. Target to achieve the patient's steady-state Hb level |
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| Acute chest syndrome | Caused by pulmonary infection, sequestration of RBCs in pulmonary vasculature, and fat embolism. May follow VOC or surgery Associated with high mortality Presents with cough, chest pain, difficulty in breathing, ± fever. Examination may be normal or reveal signs of respiratory distress, hypoxemia, wheezing, percussion dullness. Consider two or more of the above. New opacity on a chest X-ray Perform CBC, blood grouping and cross-matching, CRP, blood gas analysis if in respiratory failure |
Hospital admission Oxygen therapy if low O2 saturation (target SPO2 >95%) Antibiotics: Third generation cephalosporin (e.g., ceftriaxone 80-100 mg/kg/day for 7-10 days) combined with an oral macrolide for Mycoplasma and Chlamydophila coverage: azithromycin 5-10mg/kg (max dose 500mg) once daily for 5 days or erythromycin 5-10 mg/kg/dose (max dose 500mg) 6 hourly for 7-10 days Bronchodilators (e.g., nebulized salbutamol 2.5 mg for children <5 years, 5mg for older children) Analgesics If Hb concentration is >1.0 g/dL below baseline, consider simple blood transfusion with 10mL/kg of packed RBCs or 20ml/kg of whole blood. Urgent exchange transfusion if rapid progression of ACS Optimal hydration (avoid pulmonary edema) Monitor vitals and for acute anemia Incentive spirometry every 2–4 hours while awake |
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| Acute stroke | Ischemic and hemorrhagic stroke are mostly common in children and adults respectively Headache, vomiting, seizures, sensory/motor neurological deficits (paresis, hemiplegia, paraplegia, facial droop, aphasia), altered level of consciousness/coma MRI and MRA, acute brain infarct or hemorrhage CT, acute hemorrhage in the brain Exclude meningitis |
Monitor vitals, maintain normal temperature Exchange blood transfusion within 4 hours if acute stroke is confirmed by neuroimaging Initiate long-term blood transfusion every 3-4 weeks and/or Hydroxyurea (if monthly transfusion unavailable) Physiotherapy in the long-term |
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| Priapism | Presents as a sustained and painful erection unrelated to sexual stimulation. Lasts ≥4 hours (fulminant or major) or repeated painful erections lasting more than 30 minutes and up to 4-6 hours (stuttering) Erectile dysfunction and impotence can result from delay in diagnosis and treatment |
At onset (<2hr): encourage extra oral fluids Oral or parenteral analgesia Attempt to urinate Warm birth Exercise (e.g., walking) Anxiolytics if anxious (e.g., lorazepam 0.05mg/kg/dose 8-12hr, Max= 2mg/dose) If >2hr, catheterize if unable to urinate, consult urologist or surgeon – to consider irrigation and aspiration of the corpus cavernosum and intracorporeal etilefrine or phenylephrine injection If no response to initial treatment, consider simple or exchange blood transfusion |
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| Multisystem organ failure (MSOF) | Associated with VOC and characterized by respiratory, hepatic, and renal failure Unexpected and rapid deterioration, usually after several days of in-hospital treatment for severe VOC, at a time when pain is beginning to improve Fever, non-focal encephalopathy, ACS. Rapid decline in Hb and platelet count. Marked elevations in liver enzymes, total and direct bilirubin, blood coagulation screening tests. Elevated serum creatinine (± oliguria and hyperkalemia) |
Rapid diagnosis and treatment Simple or exchange blood transfusion to Hb 10g/dL Supplemental oxygen and mechanical ventilation (if needed) Renal replacement therapy (e.g., hemodialysis) for acute renal failure (if needed) |
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| Acute severe anemia | Hemoglobin <5g/dl or acute drop of Hb by >2g/dl from baseline/steady state or acutely symptomatic anemia Rule out malaria, bacterial infections, splenic or hepatic sequestration, and aplastic crisis |
Immediately transfuse with packed RBCs 10ml/kg if symptomatic or Hb <5g/dl Transfuse to steady-state Hb if asymptomatic and Hb >5g/dl Treat patient according to other underlying diagnoses |