What complications of sickle cell disease should an Acute Care Nurse Practitioner (ACNP) be aware of?

Complications of Sickle Cell Disease for ACNPs

An ACNP caring for patients with sickle cell disease must be aware of both life-threatening acute complications—particularly acute chest syndrome, stroke, and acute splenic sequestration crisis—as well as chronic multi-organ complications that significantly impact morbidity and mortality. 1, 2

Life-Threatening Acute Complications

Acute Chest Syndrome

• Acute chest syndrome is the leading cause of death in sickle cell disease, occurring in more than 50% of hospitalized patients with vaso-occlusive crisis 2, 3
• Characterized by new onset respiratory symptoms (chest pain, fever, tachypnoea, cough, wheeze) combined with new pulmonary infiltrates on chest X-ray 1
• Can develop rapidly after initial presentation, requiring continuous monitoring and low threshold for ICU admission 2, 3
• Incentive spirometry every 2 hours significantly reduces development of acute chest syndrome and should be initiated for all admitted patients 2, 3

Neurological Complications

• Stroke occurs in up to 10% of children with sickle cell disease and requires urgent evaluation for any acute neurologic symptom beyond transient mild headache 1, 2
• Common presentations include hemiparesis, aphasia, seizures, severe headache, cranial nerve palsy, stupor, or coma 2
• Adults experience both ischemic stroke and intracranial hemorrhage 1
• "Silent" infarctions occur in 40% of adults on MRI and may be associated with cognitive impairment 1
• All children under 17 years require regular transcranial Doppler screening to assess stroke risk 1

Acute Splenic Sequestration Crisis

• Potentially life-threatening complication in children aged 5 months to 2 years characterized by massive splenomegaly, acute anemia (Hb 10-30 g/L), and hypovolemic shock 1
• Requires careful transfusion to avoid acute overtransfusion while managing rapid progression to shock 2

Infection-Related Complications

• Patients with hyposplenism are particularly vulnerable to gram-negative sepsis, including urinary tract infection, biliary sepsis, and non-typhi salmonella infection 1, 2
• Blood cultures should be obtained if temperature reaches ≥38.0°C, with prompt antibiotic initiation 2, 4
• Infections commonly precipitate other sickle cell crises and are a leading cause of morbidity and mortality 3, 4

Other Acute Complications

Vaso-Occlusive Pain Crises

• Affects fingers in infancy (dactylitis), progressing to long bones, sternum, ribs, or back in older children and adults 1
• Requires prompt pain control with opioids within 30 minutes of triage, as delays worsen morbidity 2, 3

Aplastic Crisis

• Commonly triggered by parvovirus B19 infection (or Epstein-Barr virus or streptococcal infections) 1
• Aplasia may last 5-10 days 1

Priapism

• Occurs as "stuttering" or acute fulminant episodes due to sickling localized to the penis 1
• May result in permanent impotence if prolonged 1

Chronic Multi-Organ Complications in Adults

Renal Complications

• Hyposthenuria affects all patients, making them prone to dehydration which precipitates sickling 1
• Proteinuria occurs in 40% of adults 1
• Renal insufficiency develops in 20% of adults 1

Cardiorespiratory Complications

• Chronic lung disease with restrictive pattern affects 70% of patients 1
• Pulmonary hypertension occurs in 6% of adults and represents significant morbidity and mortality 1
• Obstructive sleep apnea and congestive cardiac failure are common 1

Musculoskeletal Complications

• Avascular necrosis of the femoral head causes chronic pain and disability 1
• Osteomyelitis risk is increased 1

Other Chronic Complications

• Cholelithiasis from chronic hemolysis 1
• Retinopathy requiring ophthalmology referral for proliferative disease 1
• Leg ulcers 1
• Erectile dysfunction 1

Transfusion and Treatment-Related Complications

• Acquired red cell antibodies complicate future transfusions 1
• Iron overload from chronic transfusions 1
• Neutropenia from hydroxycarbamide treatment 1
• Hyperhaemolysis 1

Critical Management Principles

Early Recognition and Monitoring

• Maintain high index of suspicion for acute chest syndrome, which can develop after initial presentation 2, 3
• Document baseline oxygen saturation and maintain SpO2 above baseline or ≥96% (whichever is higher) 2, 3
• Keep patients normothermic as hypothermia increases sickling 2

Hydration Management

• Aggressive hydration is crucial due to impaired urinary concentrating ability 2, 3
• Meticulous fluid balance monitoring to prevent both dehydration and overhydration 2

Multidisciplinary Coordination

• Patients should be managed in clinical networks with specialist hematology involvement 1, 2
• Low threshold for ICU admission with severe complications or clinical deterioration 2, 3
• Planned surgery should occur at designated sickle centers with exchange transfusion capability 2

Common Pitfalls to Avoid

• Do not delay opioid administration for severe pain—this worsens morbidity 2, 3
• Do not use "as needed" dosing for severe pain; use scheduled around-the-clock dosing or patient-controlled analgesia 2
• Do not overlook hypoxia, as it precipitates sickling and worsens vaso-occlusion 2, 3
• Do not miss early signs of acute chest syndrome in patients with chest or thoracoabdominal pain 2, 3
• Do not underestimate infection risk—fever requires immediate blood cultures and antibiotics 2, 4