What is the recommended vaccination schedule for a patient with sickle cell disease after autosplenectomy (removal of the spleen due to disease)?

Vaccination Schedule for Sickle Cell Disease Patients After Autosplenectomy

Patients with sickle cell disease and autosplenectomy should receive PCV13 first, followed by PPSV23 at least 8 weeks later, along with meningococcal conjugate vaccine (MenACWY), Haemophilus influenzae type b (Hib) vaccine, and annual inactivated influenza vaccine, with all encapsulated bacterial vaccines ideally administered at least 2 weeks after functional splenic loss is confirmed. 1

Core Vaccination Algorithm

Pneumococcal Vaccination Sequence

• Administer PCV13 as the initial pneumococcal vaccine for all sickle cell patients aged ≥2 years with autosplenectomy, regardless of prior vaccination status 1
• Wait at least 8 weeks, then administer PPSV23 to provide broader serotype coverage against encapsulated bacteria 1
• Give a second dose of PPSV23 exactly 5 years after the first dose to maintain protective antibody levels 1
• Revaccinate with PPSV23 every 5-6 years thereafter for lifelong protection, as antibody levels decline more rapidly in asplenic patients 1, 2

The sequential PCV13-then-PPSV23 strategy produces superior antibody responses compared to PPSV23 alone, with children receiving this sequence showing higher antibody levels to all PCV7 serotypes 1. This prime-boost approach is critical because invasive pneumococcal disease rates fell by 93% in children with sickle cell disease after PCV implementation 1.

Meningococcal Vaccination

• Administer quadrivalent meningococcal conjugate vaccine (MenACWY) for patients aged ≥2 months with sickle cell disease and autosplenectomy 1
• Do NOT use MCV4-D in children <2 years if they need concurrent pneumococcal vaccination, as this reduces antibody response to pneumococcal serotypes; instead, wait ≥4 weeks after PCV13 completion 1
• Revaccinate with MenACWY every 5 years due to waning immunity 1
• Consider meningococcal serogroup B vaccine (MenB) as an additional protection, particularly given that serogroup B now accounts for 40% of meningococcal cases 1, 2

Mortality rates from meningococcal infection in asplenic patients range from 40-70%, making this vaccination essential 1.

Haemophilus Influenzae Type B (Hib)

• Administer one dose of Hib vaccine to all unvaccinated persons aged ≥5 years with sickle cell disease and autosplenectomy 1
• No revaccination needed if the patient completed the primary Hib series in infancy (by age 6 months) plus booster (6-8 months later), or received one dose after age 14 months 1

Annual Influenza Vaccination

• Administer inactivated influenza vaccine (IIV) annually to all sickle cell patients with autosplenectomy aged ≥6 months 1, 3
• Never use live attenuated influenza vaccine (LAIV/nasal spray) in this population 1, 3
• Annual influenza vaccination reduces mortality by 54% in asplenic patients and prevents secondary bacterial pneumonia and sepsis 3

Timing Considerations

Post-Autosplenectomy Timing

• Ideally wait at least 2 weeks after confirming functional asplenia before administering vaccines to ensure optimal antibody response 1, 2
• If vaccination timing is uncertain, vaccinate as soon as autosplenectomy is documented rather than delaying, as the infection risk is immediate and lifelong 2, 4
• The 2-week interval allows for adequate antibody formation, which generally takes 9 days 1, 4

For Children Under 2 Years

• Follow standard CDC immunization schedule for PCV13 in children <2 years with sickle cell disease 1
• Delay PPSV23 until age ≥2 years as younger children have inherently reduced antibody response 2, 4
• Consider reimmunization after age 2 years for children vaccinated before this age 4

Critical Pitfalls to Avoid

Common Errors

• Do not skip the PCV13-first sequence: Giving PPSV23 before PCV13 produces inferior immune responses and may cause hyporesponsiveness to subsequent PCV13 1
• Do not co-administer MCV4-D with PCV in children <2 years: This significantly reduces pneumococcal antibody response 1
• Do not use only PPSV23: The conjugate vaccine (PCV13) is essential for optimal T-cell dependent immunity 1
• Do not forget the 5-year PPSV23 booster: Antibody levels wane significantly, and the second dose is strongly recommended 1

Special Considerations for Sickle Cell Disease

• Recognize that autosplenectomy creates functional asplenia requiring the same vaccination approach as surgical splenectomy 1, 3
• Patients with autosplenectomy have 87.5% infection rates more than once yearly compared to 50% in those without autosplenectomy, emphasizing the critical need for vaccination 5
• All routine vaccines for immunocompetent persons should also be given according to CDC schedules; no standard vaccine is contraindicated except LAIV 1

Additional Protective Measures

Antibiotic Prophylaxis

• Strongly consider lifelong prophylactic phenoxymethylpenicillin (oral penicillin V), particularly in the first 2 years post-autosplenectomy when infection risk is highest 2, 4
• Provide emergency standby amoxicillin for patients to keep at home and start immediately with fever >101°F (38°C), malaise, or chills 2, 4
• Use erythromycin for penicillin-allergic patients 4

Patient Education

• Counsel patients that infection risk is lifelong, with overwhelming post-splenectomy infections reported more than 20 years after splenic loss 2, 4
• Provide written information about their asplenic status and infection risk 2, 4
• After animal bites, immediately start a 5-day course of co-amoxiclav due to susceptibility to Capnocytophaga canimorsus 2, 4
• Notify primary care providers formally of the patient's asplenic status to ensure coordinated long-term care 2, 4