What care should be done for a person with hereditary spherocytosis (HS) and post-splenectomy (removal of the spleen)?

From the Guidelines

Care for a person with spherocytosis who has undergone splenectomy should focus on preventing infections and managing anemia, with a strong emphasis on vaccination against encapsulated organisms, including pneumococcal, Haemophilus influenzae type b, and meningococcal vaccines, as well as annual influenza vaccines, as recommended by the most recent guidelines 1.

Key Recommendations

• Patients should receive vaccinations against encapsulated organisms before splenectomy if possible, or at least 14 days after splenectomy, as stated in the guidelines 1.
• Vaccination programs should include pneumococcal vaccines (PCV13 and PPSV23), Haemophilus influenzae type b, meningococcal vaccines, and annual influenza vaccines, as suggested by the evidence 1.
• Patients should carry a medical alert card/bracelet indicating their asplenic status and seek immediate medical attention for any fever (≥101°F/38.3°C), as they are at risk for overwhelming post-splenectomy infection (OPSI) 1.
• Antibiotic prophylaxis should be considered in the event of any sudden onset of unexplained fever, malaise, chills, or other constitutional symptoms, especially when medical review is not readily accessible, with options including amoxicillin, levofloxacin, or moxifloxacin, as recommended by the guidelines 1.
• Regular complete blood count monitoring is necessary to assess for anemia, though most patients experience improved red cell survival after splenectomy, and folic acid supplementation (1 mg daily) may be beneficial, especially if there is ongoing hemolysis 1.

Prevention of Infections

• Patients should be educated about the increased risk of venous thromboembolism and gallstones, with monitoring for these complications, as suggested by the evidence 1.
• Malaria prophylaxis is strongly recommended for travelers to endemic areas, as stated in the guidelines 1.
• Patients should be aware of the risks of OPSI and the importance of prompt medical attention in case of fever or other symptoms, as emphasized by the guidelines 1.

Management of Anemia

• Regular monitoring of complete blood counts is necessary to assess for anemia, and folic acid supplementation may be beneficial in cases of ongoing hemolysis, as suggested by the evidence 1.
• Patients should be educated about the risks of anemia and the importance of regular monitoring, as stated in the guidelines 1.

From the Research

Care for a Person with Spherocytosis and Splenectomy

To manage a person with spherocytosis who has undergone splenectomy, several key considerations must be taken into account:

• Vaccination: Vaccination against encapsulated organisms such as Streptococcus pneumoniae, Haemophilus influenzae type b, and Neisseria meningitidis is crucial before splenectomy, as it reduces the risk of overwhelming post-splenectomy infection (OPSI) 2, 3.
• Antibiotic Prophylaxis: Daily antibiotics for at least 2 years post-splenectomy and emergency antibiotics in case of infection can help prevent OPSI 2.
• Education and Awareness: Patients should be educated about the risks of OPSI and the importance of seeking medical attention immediately if they develop symptoms of infection 4, 2.
• Medical Alert and Vaccination Card: Asplenic patients should carry a medical alert and an up-to-date vaccination card to inform healthcare providers of their condition 2.
• Travel and Animal Handling Precautions: Asplenic patients are at increased risk of severe malaria and OPSI from animal bites, so specific advice around travel and animal handling is necessary 2.
• Regular Follow-Up: Regular follow-up with a healthcare provider is essential to monitor for potential complications and provide ongoing education and support 4, 5.
• Management of Underlying Condition: The underlying condition of hereditary spherocytosis should be managed appropriately, with consideration of the risks and benefits of splenectomy 6.

Prevention of Infections

Preventing infections is a critical aspect of care for a person with spherocytosis and splenectomy:

• Registry Programs: Participation in registry programs, such as Spleen Australia, can help reduce the risk of infection by providing education, clinical guidance, and annual vaccination reminders 4.
• Vaccination Reminders: Annual vaccination reminders can help ensure that patients receive necessary vaccinations to prevent infections 4.
• Emergency Preparedness: Patients should be prepared for emergency situations by carrying a medical alert and having a plan in place for seeking medical attention if symptoms of infection develop 2.