Asplenia and hyposplenia vaccination and prophylaxis

Disclaimer

These guidelines have been produced to guide clinical decision making for the medical, nursing and allied health staff of Perth Children’s Hospital. They are not strict protocols, and they do not replace the judgement of a senior clinician. Clinical common sense should be applied at all times. These clinical guidelines should never be relied on as a substitute for proper assessment with respect to the particular circumstances of each case and the needs of each patient. Clinicians should also consider the local skill level available and their local area policies before following any guideline. 

Read the full PCH Emergency Department disclaimer.

Aim

To provide guidance to PCH clinical staff on the vaccination schedule and prophylactic antibiotic therapy for children who have no spleen (asplenia) or a non-functioning spleen (hyposplenia).

Background1,2 

Patients with no spleen (asplenia) or a reduced/absent functional spleen (hyposplenia) are at life-long risk of severe infection with encapsulated bacteria (Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae type b) and influenza virus. Vaccination and empiric antibiotic management is warranted to decrease morbidity and mortality. The risk of infection is greatest within 3 years of splenectomy or, in the setting of congenital asplenia, the first 3 years of life. These patients include, but are not limited to:
  • Post-traumatic or elective splenectomy
  • Congenital asplenia, including hyposplenia or polysplenia
  • Cancer-related asplenia
  • Splenic infarction and or sequestration e.g. sickle cell disease.

Assessing splenic function

There is no consensus regarding the best method for assessing splenic function and if there is concern regarding hyposplenism, a cautious approach and adherence to this guideline is recommended. Some investigations that may be useful to assist with identifying patients with hyposplenism include:

  • Ultrasound+/- Doppler to assess spleen size and blood flow
  • Howell Jolly bodies (intracellular nuclear remnants in erythrocytes) and other changes on a peripheral blood film
  • Other imaging modalities such as MRI/CT can further delineate anatomy in heterotaxy syndrome with polysplenia and rarely technetium 99m sulfur colloid scintigraphy considered.

Key Points1,2

  • Education: All patients and families must be provided with education (both verbal and written) and an Asplenia/hyposplenia management plan (PDF 134kb) and the Asplenia and Hyposplenia vaccination and prophylaxis Health Fact sheet (PDF 140kb). A copy should be given to the patient/family, GP and any other care providers. A referral for further advice can be obtained from the Specialist Immunisation Clinic at Perth Children’s Hospital.
  • The patient should be strongly encouraged to wear a Medic Alert bracelet (external site).
  • Further resources are available for patients and families on the Spleen Australia website (registration not currently available for patients in WA).
  • Management of suspected infection: Urgent presentation to hospital when febrile (temperature > 38°C) or with rigors or acutely unwell is essential in patients with asplenia/hyposplenia. In this situation, blood cultures should be taken and broad spectrum empiric antibiotics commenced as per local guidelines (Refer to the ChAMP Guideline for Presumed Bacteraemia/Sepsis).
  • Prophylactic antibiotics should be administered until patients are at least 5 years of age and for at least 2 years following splenectomy (see below). Lifelong antibiotic prophylaxis may be warranted, given patients are at lifelong risk of severe sepsis; this should be addressed on a case by case basis.
  • Vaccinations: Individuals with asplenia/hyposplenia importantly should be up to date with routine vaccinations (see online WA immunisation schedule), particularly Haemophilus influenzae B (Hib) and require additional pneumococcal, meningococcal, and annual influenza vaccination, due to their increased risk of overwhelming sepsis. Refer to the Asplenia/hyposplenia management plan.
    • For elective splenectomy, vaccination should be administered at least 2 weeks before surgery is performed. However, if this is not possible, start vaccinations approximately 1 week after surgery, prior to discharge, with a vaccination plan in place.
    • For patients who have not been immunised prior to splenectomy and will be receiving immunosuppressive chemotherapy, vaccination advice from the treating Oncology Consultant or Immunisation service, Infectious Diseases should be sought.
    • Household/close contacts of individuals with asplenia should be up to date with age appropriate recommended vaccinations, including annual influenza vaccination. The use of live vaccines in contacts of immunocompromised persons is safe, and strongly recommended.
  • A PCH Clinical Alert must be in place for all asplenic / hyposplenic patients. The treating team should complete a ‘Clinical Alert notification form’ for entry onto webPAS by Clinical Coding. Refer to the Clinical Alerts Policy in the PCH Operational Manual.

Recommended vaccination schedule for children with asplenia / hyposplenia

1. Streptococcus pneumoniae (Pneumococcus)

Due to higher disease burden and the potential for poorer antibody responses, children diagnosed with asplenia/hyposplenia at age ≤ 12 months are recommended to receive an additional 13vPCV dose (4th dose) and 3 lifetime doses of 23vPPV. Catch-up schedules for children diagnosed at an older age are outlined in Table 1.

Table 1: Pneumococcal vaccines (derived from AIH 10th edition)3

Current age  Number of 13vPCV doses previously received Age at presentation   Age at 1st PCV dose Number of 13vPCV doses required #+ Number of 23vPPC doses required 
< 5 years None
< 12 months  N/A  4 3 lifetime doses **
12 - 59 months
 N/A
 2
 1
< 12 months  N/A
 3
12 - 59 months
 < 12 months  2
 > 12 months  1
 2  < 12 months  N/A  2
12 - 59 months  < 12 months  2 (if 2nd dose received at age > 12 months, 1 further 13vPCV dose is required)
 > 12 months  -
 3  < 12 months  N/A  1
 12-59 months  < 12 months  1 (if 2nd and 3rd doses received at age > 12 months, no furhter 13vPCV doses are required)
 4  N/A  N/A  - 
5 to 17 years  0  N/A  N/A  1 3 lifetime doses **
 >1 (excluding Prevenar7 and Prevenar10)  N/A  N/A  -

# 13vPCV (Prevenar13)/ 23vPPV (Pneumovax23); route IM, dose 0.5ml. The minimum interval between 13vPCV dose(s) is 1 month if aged <12 months, and 2 months if aged ≥12 months. The minimum interval between 13vPCV (recommended to be given first) and 23vPPV is 2 months. If 23vPPV is given first, a minimum interval of 12 months is recommended before 13vPCV can be given.

** The first 23vPPV dose should be given at age 4-5 years and at a minimum of 2 months after the last 13vPCV dose. The second dose of 23vPPV is recommended 5 years after the first and at a minimum of 2 months after 13vPCV. The third 23vPPV dose should be approximately 10 years after the second, or at age 18–20 years, whichever is later.

+ Menactra (MenACWY) should not be co-administered with 13vPCV as Menactra may interfere with the immune response against some pneumococcal serotypes. If co-administered, the 13vPCV dose should be repeated a minimum of 8 weeks later. Menveo and Nimenrix can be co-administered with 13vPCV.

2. Neisseria meningitidis (Meningococcus)

All patients require additional vaccines against N. meningitidis, which are hospital funded, including vaccinations against meningococcal ACWY (4vMenC) and meningococcal B (MenBV).

Table 2: Meningococcal ACWY vaccines (4vMenC) (derived from AIH 10th edition)3

Age at commencement  Recommended brand(s)# Number of primary 4vMenC doses# Recommended interval between primary doses  Timing of booster 4vMenC doses
6 weeks to 5 months   Menveo OR
 Nimenrix
 4 8 weeks; 4th dose at 12 months of age or 8 weeks after 3rd dose, whichever is later 3 years after completing primary schedule, then every 5 years thereafter
6 - 8 months  Menveo OR
 Nimenrix
 3 8 weeks; 3rd dose at 12 months of age or 8 weeks after 2nd dose, whichever is later
3 years after completing primary schedule, then every 5 years thereafter
9 -11 months  Menveo OR 
 Nimenrix OR
 3 8 weeks; 3rd dose at 12 months of age or 8 weeks after 2nd dose, whichever is later
3 years after completing primary schedule, then every 5 years thereafter
> 12 months  Menveo OR 
 Nimenrix OR
 2 8 weeks If primary course completed at ≤ 6 years of age - 3 years after completing primary schedule, then every 5 years thereafter. If primary course completed at ≥ 7 years of age – every 5 years after completing primary schedule.

# Menveo/Niminrix; route IM, dose 0.5ml. 4vMenC and MenBV can be given at the same time

Menveo or Niminrix are the preferred brands over Menactra. Menactra should not be co-administered with 13vPCV as Menactra may interfere with the immune response against some pneumococcal serotypes. If co-administered, the 13vPCV dose should be repeated a minimum of 8 weeks later. Menveo and Nimenrix can be co-administered with 13vPCV.

Table 3: Meningococcal B vaccine (MenBV) (derived from AIH 10th edition)3

Age at commencement  Recommended brand Number of primary MenBV doses+ Interval between doses 
6 weeks to 5 months   Bexsero  4 8 weeks; 4th dose at 12 months of age or 8 weeks after 3rd dose, whichever is later
6 - 11 months  Bexsero

 3 8 weeks; 3rd dose at 12 months of age or 8 weeks after 2nd dose, whichever is later
12 months to 9 years  Bexsero

 2 8 weeks
> 10 years  Bexsero OR

 2 8 weeks
Trumenba  3 At 0, 1 and 6 months

+All MenBV vaccines (Bexsero & Trumenba) route IM, dose 0.5ml. 4vMenC and MenBV can be given at the same time. Prophylactic use of paracetamol is recommended when MenBV is administered to children < 2 years of age. There is an increased risk of fever when MenBV is co-administered with other routine vaccines, however this is not a contraindication.

3. Influenza

Table 4: Influenza vaccine (derived from AIH 10th edition1)3

Age £ Influenza vaccine brand Route Dose Number of doses required if receiving the Influenza vaccine for the first time Number of doses required if previously received one or more doses of influenza vaccine
6 months to <3 years FluQuadri Junior IM 0.25mL 2 doses, minimum 4 weeks apart 1 dose
3 years to < 9 months Fluarix Tetra or FluQuadri IM 0.5mL 2 doses, minimum 4 weeks apart 1 dose
9 years and over Fluarix Tetra or FluQuadri IM 0.5mL 1 dose* 1 dose

£ All children with asplenia require annual influenza vaccine at the start of each influenza season, given their increased risk of severe infection.

* If the child/adolescent with asplenia has had a solid organ or haematopoietic stem cell transplant, 2 doses of influenza vaccine are recommended during their first year of influenza vaccination after transplantation.

4. Haemophilus influenzae B vaccine (derived from the AIH 10th edition)3

Age at diagnosis Vaccine history Number of further primary doses required Number of booster doses required at age > 18 months of age, or 2 months after the last dose, whichever is later
  Number of previous Hib doses Current age (months Age at time of 1st Hib vaccine (months    
6 weeks to < 5 years No previous Hib doses  < 7 months  N/A  3  1
 7 - 11 months  N/A  2  1
 12 - 17 months  N/A  1  1
 18 - 59 months  N/A  1  -
1 previous Hib dose  < 12 months  < 7 months  2  1
 7-11 months  1  1
 12-17 months  < 12 months  1  1
 > 12 months  -  1
 18-59 months  < 18 months  -  1
 > 18 months  -  -
2 previous Hib doses  < 12 months  < 7 months  1  1
 < 17 months  7-11 months  -   1
 12 - 17 months  > 12 months  -   -
 18 - 59 months  < 12 months  -  1
 > 12 months  -   - 
3 Hib doses received previously  All 3 doses at < 12 months
  
 -  1
 > 1 dose at 12-17 months  -  -
 > 5 years Primary course complete

 -   - 
Never received a dose of Hib-containing vaccine or primary course incomplete  -   1

For all Hib containing vaccines; route IM, dose 0.5ml. The minimum interval between primary doses for Hib is 4 weeks. Hib age appropriate combination vaccines are acceptable, e.g DTPa- HepB-IPV-Hib (Infanrix-hexa) on schedule at 6 weeks, 4 and 6 months. Monovalent Hib vaccines (Hiberix & Act-HIB) are registered for use in infants and children from age 2 months – 5 years.

5. Antibiotic prophylaxis 4,5

Prophylactic antibiotics should be administered until patients are at least 5 years of age and for at least 2 years following splenectomy. Lifelong prophylaxis may be warranted and must be addressed on a case by case basis.

Refer to the Asplenia/Hyposplenia Management Plan. Further advice can be sought from an Infectious Diseases Specialist.

Age at diagnosis  Prophylactic antibiotic
 < 1 year Amoxicillin 20mg/kg/dose (up to 250mg) once daily OR
Phenoxymethylpenicillin (Penicillin V) 62.5mg twice daily
 1 - 5 years Amoxicillin 20mg/kg/dose (up to 250mg) once daily OR
Phenoxymethylpenicillin (Penicillin V) 125mg twice daily
 > 5 years Amoxicillin 20mg/kg/dose (up to 250mg) once daily OR
Phenoxymethylpenicillin (Penicillin V) 250mg twice daily
 If confirmed penicillin allergy  Roxithromycin 4mg/kg/dose (up to 150mg) once daily

Standby antibiotic supply 4,5

Standby antibiotics should be supplied if immediate access to medical care is not available (e.g. whilst on holiday or if living in remote areas). In this circumstance if a patient with hyposplenia/asplenia develops a fever (temp > 38°C) or rigors or is acutely unwell, they should commence the standby antibiotic immediately and seek urgent hospital/medical review.
Age at diagnosis  Standby antibiotic
 All ages < 1 year Amoxicillin /Clavulanate 25mg/kg/dose of amoxicillin component (to a maximum of 875mg) twice daily (use DUO forte or DUO 400 preparations)
 If penicillin allergic Clarithromycin 7.5mg/kg/dose twice daily OR
Clindamycin 10mg/kg/dose three times daily

6. Travel

All hyposplenic/asplenic patients travelling to developing countries may be at risk of Plasmodium falciparum malaria, Salmonella infection and Babesiosis (rare tick infection). Infectious Diseases advice should be sought on antimalarial prophylaxis, mosquito avoidance and additional travel vaccines required prior to travel.

7. Animal bites

There is an increased risk of severe sepsis following dog, cat or other animal bites/severe scratches (e.g. due to Capnocytophaga canimorsus, streptococcal sp, anaerobic bacteria). Early medical attention and antibiotic prophylactic treatment with Augmentin duo (Amoxycillin/clavulanic acid) is recommended.

References

  1. Spelman D, Buttery J, Daley A et al. Guidelines for the prevention of sepsis in asplenic and hyposplenic patients. Internal Medicine Journal. 2008. 38 pp349-356.
  2. Davies, JM, Lewis, PN et al. Review of guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. British Journal of Haematology. 2012.155.pp308-317.
  3. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook, Australian Government Department of Health, Canberra, 2018. Accessed from: https://immunisationhandbook.health.gov.au/
  4. Spleen Australia. Medical Guidelines for Individuals with Asplenia or Hyposplenism. February 2016
  5. Royal Children’s Hospital. Paediatric Asplenia/Hyposplenia Guidelines: Melbourne July 2014

Approved by:  PCH Clinical Practice Advisory Committee Date:  20 Sep 2018
Endorsed by:  Executive Director, Nursing Services Date:  10 Oct 2018


Review date:   Sep 2021


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