Management of Sickle Cell Disease

Diagnostic Workup and Initial Management

Patients with suspected acute chest syndrome should undergo a complete blood count with reticulocyte count, oxygen saturation monitoring, and blood cultures if febrile, as recommended by the American Thoracic Society 1
Aggressive management, including supplemental oxygen, broad-spectrum antibiotics, pain management, and considering exchange transfusion for moderate to severe cases, is recommended for patients with signs of acute chest syndrome, according to the American College of Chest Physicians 1
Key clinical features of acute chest syndrome include respiratory symptoms, fever, chest pain, decreasing hemoglobin, hypoxemia, and arm and leg pain 2, 3
A chest X-ray is required for patients with respiratory symptoms, fever, or hypoxemia to diagnose acute chest syndrome (new pulmonary infiltrate) 4
Regular monitoring of SpO2 allows for early detection of acute chest syndrome, characterized by a new pulmonary infiltrate on chest radiograph, fever, and/or respiratory symptoms, often accompanied by a decrease in hemoglobin and hypoxemia, according to the American Thoracic Society and the American College of Cardiology 4, 2

Indefinite anticoagulant therapy should be considered for patients with confirmed venous thromboembolism and RHC-confirmed pulmonary hypertension if no additional risk factors for hemorrhage exist, as recommended by the American College of Cardiology 1
Phosphodiesterase-5 inhibitor therapy should be avoided in patients with sickle cell disease who have pulmonary hypertension, according to the American Heart Association 1

Hydroxyurea is recommended as first-line therapy for patients with increased mortality risk, defined by tricuspid regurgitant velocity >2.5 m/second, NT-pro-BNP >160 pg/ml, or RHC-confirmed pulmonary hypertension, according to the National Heart, Lung, and Blood Institute 1
Chronic transfusion therapy should be considered for patients who are not responsive to or not candidates for hydroxyurea, as recommended by the American Society of Hematology 1

Opioid analgesics should be administered promptly using scheduled dosing or patient-controlled analgesia to manage pain in patients with sickle cell crisis, with a strength of evidence rated as high, according to the American Pain Society 5
Aggressive pain management with opioids as needed, using scheduled dosing or patient-controlled analgesia, is also recommended, with a strength of evidence based on high-quality evidence 6, 7, 8, 9
Adjunctive non-opioid analgesics should be considered to avoid under-treatment of pain, which can worsen the crisis, according to the American Society of Hematology 5

Patients with sickle cell disease who report acute chest pain require emergency transfer to an acute care setting, with immediate evaluation to exclude acute chest syndrome, which is a leading cause of death in this population 2, 3
The American Academy of Pediatrics recommends assessment for splenic sequestration, characterized by a rapidly enlarging spleen and hemoglobin drop >2 g/dL, in patients presenting with pain crisis 6
The American Academy of Pediatrics also recommends assessment for transient aplastic crisis, characterized by worsening anemia with reticulocyte count <1%, in patients presenting with pain crisis 6
Regular transcranial Doppler screening is recommended for stroke risk assessment, as reported by the American Academy of Pediatrics 6
Daily assessment by a hematologist for admitted patients with sickle cell disease and fever is recommended to monitor for signs of acute chest syndrome and inspect IV sites regularly for signs of phlebitis, according to the American Society of Hematology 4

Patients should be aware of the signs and symptoms of emergencies, such as fever, respiratory symptoms, and worsening pain, and seek medical attention promptly, as recommended by the National Heart, Lung, and Blood Institute 5
The American Academy of Pediatrics recommends educating patients on the importance of hydration, avoiding extreme temperatures, and adhering to medication regimens, as well as considering hydroxyurea for patients with frequent episodes of acute chest syndrome, to reduce the risk of vaso-occlusive crises 4, 1
The use of pneumococcal vaccination and prophylactic antibiotics has decreased the incidence of bacteremia in febrile children with sickle cell disease, highlighting the importance of preventive measures in reducing the risk of serious infections 4

With optimal multidisciplinary care, survival into the 7th decade can be expected for patients with sickle cell disease, according to the American Society of Hematology 4
Life expectancy has improved significantly but remains approximately 20 years less than the general population, as reported by the Centers for Disease Control and Prevention 4