Asplenia, Hyposplenia and Complement Deficiency 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 CAHS clinical disclaimer.
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Aim
To provide guidance to Perth Children's Hospital (PCH) clinical staff on the vaccination schedule and prophylactic antibiotic therapy for children who have no spleen (asplenia), a poorly functioning spleen (hyposplenia), or a related immunodeficiency.
These patients include, but are not limited to, those who have:
- Post-traumatic or elective splenectomy.
- Congenital asplenia, including hyposplenia or polysplenia.
- Splenic trauma, including those managed conservatively or with splenic artery embolisation.
- Splenic infarction and or sequestration e.g. sickle cell disease.
- Complement deficiency (either classical or alternative pathway).
Background1,2,4
Patients with asplenia or hyposplenia are at life-long risk of severe infection with encapsulated bacteria (Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae type b) and influenza virus. Patients are at higher risk of infections and of more severe clinical illness for a given infection.
Patients with complement deficiency are at variable risk of invasive encapsulated bacterial infection, depending on the nature of the complement pathway defect.
The risk of infection is greatest within 3 years of splenectomy or, in the setting of congenital asplenia, the first 3 years of life. In most cases, vaccination and empiric antibiotic management is warranted to decrease morbidity and mortality.
Assessing splenic function
There is no best practice diagnostic test for assessing splenic function and the tests currently available have recognised limitations. If there is a possibility of hyposplenism, we recommend a cautious approach and adherence to this guideline.
If there is uncertainty about the extent of splenic function or hyposplenism, discuss with Infectious Diseases.
Some investigations that may be useful to assist with identifying patients with hyposplenism include:
- Blood tests
- Presence of Howell Jolly bodies (intracellular nuclear remnants in erythrocytes) on a peripheral blood film, which is to be specifically requested on the pathology form (and will need to be requested >4 weeks after splenic artery embolisation to reassess function).
- Other measurements of immune function pertinent to which the spleen contributes are possible including IgM memory B cells – discuss with immunology.
- Imaging
- Ultrasound+/- Doppler to assess spleen size and blood flow.
- Other imaging modalities such as magnetic resonance imaging (MRI) or computerised tomography (CT) can further delineate anatomy in heterotaxy syndrome with polysplenia. Technetium 99m sulphur colloid scintigraphy may be considered.
Assessing complement function
Investigation of patients with suspected complement deficiency (e.g. due to invasive encapsulated bacterial infection or family history) should be performed by or in consultation with a clinical immunologist to optimise test selection, timing of collection and interpretation of results.
Management of patients with asplenia/hyposplenia2
1. Education
- All patients and families must be educated regarding:
- The need for early investigation and management of any febrile illness or acute illness where the patient is unwell.
- The symptoms and signs of bacterial infection and when to give standby antibiotics (see below).
- All patients and families should be provided with written information, including a management plan and health fact sheet. This should also be provided to the patient’s general practitioner (GP) and any other care providers.
- The patient should be strongly encouraged to wear a Medic Alert bracelet.
- Further patient resources are available on the Spleen Australia website and healthcare providers are strongly recommended after obtaining patient consent to register patients with Spleen Australia to assist families with management of asplenia.
- All patients should be referred to the Specialist Immunisation Clinic, Department of Infectious Diseases, Perth Children’s Hospital, for further education optimisation of antibiotic prophylaxis and immunisations.
2. Management of suspected infection
- Urgent presentation to hospital when febrile (temperature > 38°C) or with rigors, or when acutely unwell, is essential in patients with asplenia / hyposplenia / complement deficiency. In this situation, blood cultures should be taken and broad spectrum empiric antibiotics commenced as per local guidelines (Refer to the Sepsis and Bacteraemia - ChAMP Guideline).
3. Prophylactic antibiotics (see Appendix 1 for recommendations)
- Daily dosing of amoxicillin or phenoxymethylpenicillin reduces the risk of invasive bacterial infections in patients with asplenia.
- Longer term or lifelong antibiotic prophylaxis should be considered given patients are at lifelong risk of severe sepsis; this should be addressed on an individual basis.
- Particularly high-risk individuals are those who are immunosuppressed, those who had splenectomy in the setting of haematological conditions or graft-versus-host-disease, and those with a history of previous post-splenectomy infection, especially invasive pneumococcal disease. Antibiotic prophylaxis until the age of 16 years at a minimum should be considered in these high-risk groups.
- Minimum recommended duration:
- Congenital asplenia – 5 years of age
- Hyposplenia from sickle cell anaemia or similar – 16 years of age
- Post splenectomy – 3 years post operative
- Patients following splenic trauma including those who receive splenic artery embolisation may require prophylactic antibiotics on a case-by-case basis.
4. Vaccinations (see Appendix 2 for recommendations)
- These patients should be up to date with routine vaccinations (see online WA immunisation schedule), particularly Haemophilus influenzae B (Hib), and annual influenza vaccination, and require additional pneumococcal and meningococcal, vaccines as per the ‘medically at risk’ recommendations in the Australian Immunisation Handbook due to their increased risk of overwhelming sepsis.
- 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. Obtain an inpatient consult from the Specialist Immunisation Clinic, Department of Infectious Diseases to assist with developing a formal plan.
- For more complex immunisation needs (e.g. children with asplenia also undergoing transplant), refer to the Specialist Immunisation Clinic, Department of Infectious Diseases.
- Household and close contacts of these patients should be up to date with recommended vaccinations, including annual influenza vaccination.
5. Clinical Alert
- A PCH clinical alert must be in place for all patients with asplenia / hyposplenia or complement deficiency.
- The treating team should complete a Clinical Alert eReferral. Refer to the Clinical Alerts Policy in the PCH Operational Manual (internal WA Health only).
6. Travel
Patients are at increased risk of severe disease and complications if they contract Plasmodium falciparum malaria, Salmonella infection and Babesiosis (rare tick infection). If travelling to endemic areas, Infectious Diseases advice should be sought prior to travel on antimalarial prophylaxis, mosquito avoidance and additional travel vaccines required.
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 amoxicillin/clavulanic acid is recommended (refer to ChAMP Skin and Soft Tissue Infections Paediatric Empiric Guidelines).
References
- Spelman D, Buttery J, Daley A et al. Guidelines for the prevention of sepsis in asplenic and hyposplenic patients. Internal Medicine Journal 2008; 38:349-356
- 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. 155:308-317.
- Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook, Australian Government Department of Health, Canberra, 2018. Accessed from: Australian Immunisation Handbook
- Skattum L, van Deuren M, van der Poll T, Truedsson L. Complement deficiency states and associated infections. Molecular Immunology. 2011/08/01/ 2011;48(14):1643-1655. doi: https://doi.org/10.1016/j.molimm.2011.05.001
- Spleen Australia. Medical Guidelines for Individuals with Asplenia or Hyposplenism. January 2019, V2.
- eTG complete: Antibiotic. Prevention of infection in patients with asplenia or hyposplenism. March 2021
Appendices
Appendix 1: Antibiotic prophylaxis5,6
Long term prophylactic antibiotics should be administered based on durations above. Lifelong prophylaxis should be considered and addressed on a case-by-case basis.
See the Asplenia/ Hyposplenia health fact sheet. Refer to Infectious Diseases, Specialist Immunisation Clinic for an ongoing management plan.
Standby antibiotic supply5,6
A prescription for standby antibiotics should be supplied in case 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 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.
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Age at diagnosis
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Standby antibiotic
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All ages
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Amoxicillin /Clavulanate 25 mg/kg/dose of amoxicillin component (to a maximum of 875 mg) twice daily (use Duo forte or Duo 400 preparations) until medical review.
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If low-risk allergy to penicillins
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For children 3 months or older:
Cefuroxime 15 mg/kg/dose (to a maximum of 500 mg) twice daily until medical review.
For children less than 3 months of age, discuss options with Infectious Diseases team.
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If high-risk allergy to penicillins
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Discuss options with Infectious Diseases team.
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Appendix 2: Recommended vaccination schedule for children with asplenia / hyposplenia / complement deficiency
1. Streptococcus pneumoniae (Pneumococcus)
Due to higher disease burden and the potential for poorer antibody responses, all patients are recommended to complete a 4-dose primary course of the WA scheduled pneumococcal vaccine (additional dose to be given at 6 months of age or at diagnosis if older). Pneumococcal catch-up schedules for children are outlined in Table 1.
Table 1: Pneumococcal vaccines3
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Current age
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Number of PCV doses previously received
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Age at presentation
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Age at 1st PCV dose
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Number of PCV doses required π
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< 5 years
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None
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< 12 months
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n/a
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4
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12-59 months
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n/a
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2
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1
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< 12 months
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n/a
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3
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12-59 months
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< 12 months
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2
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≥ 12 months
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1
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2
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< 12 months
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n/a
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2
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12-59 months
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< 12 months
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2 (If 2nd dose received at age ≥ 12 months, 1 further PCV dose is required)
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≥ 12 months
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-
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3
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< 12 months
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n/a
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1
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12-59 months
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< 12 months
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1 (If 2nd & 3rd doses received at age ≥ 12 months, no further PCV doses are required)
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4
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n/a
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n/a
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-
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5 to 17 years
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0 to ≤ 3 (excluding Prevenar7 and Prevenar10)
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n/a
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n/a
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1
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PCV = pneumococcal conjugate vaccine
πThe minimum interval between PCV dose(s) is 1 month if aged <12 months, and 2 months if aged ≥12 months. Children should receive their last dose of PCV after they reach 12 months of age (as a booster dose), with a minimum interval of 2 months after the previous dose.
2. Neisseria meningitidis (Meningococcus)
All patients require additional vaccines against N. meningitidis, which are nationally funded, including vaccinations against meningococcal ACWY (MenACWY) and meningococcal B (MenB).
Table 2: Meningococcal ACWY vaccines3
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Age at commencement
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Recommended brand(s)#
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Number of primary Men ACWY doses#
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Recommended interval between primary doses
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Timing of booster MenACWY doses
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6 weeks to 5 months
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Nimenrix
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4
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8 weeks; 4th dose at 12 months of age or 8 weeks after 3rd dose, whichever is later
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3 years after completing primary schedule, then every 5 years thereafter
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6-8 months
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Nimenrix
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3
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8 weeks; 3rd dose at 12 months of age or 8 weeks after 2nd dose, whichever is later
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3 years after completing primary schedule, then every 5 years thereafter
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9-11 months
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Nimenrix
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3
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8 weeks; 3rd dose at 12 months of age or 8 weeks after 2nd dose, whichever is later
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3 years after completing primary schedule, then every 5 years thereafter
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≥ 12 months
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Nimenrix
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2
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8 weeks
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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.
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# Nimenrix; route IM, dose 0.5 mL. MenACWY and MenB vaccines can be given at the same time.
Table 3: Meningococcal B vaccine3
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Age at commencement
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Recommended Brand
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Number of primary MenB doses+
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Interval between doses
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Timing of booster MenB doses
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6 weeks to 5 months
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Bexsero
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4
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8 weeks; 4th dose at 12 months of age or 8 weeks after 3rd dose, whichever is later
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Single booster dose 3 years after completing the primary schedule
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6-11 months
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Bexsero
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3
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8 weeks; 3rd dose at 12 months of age or 8 weeks after 2nd dose, whichever is later
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Single booster dose 3 years after completing the primary schedule
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12 months – 9 years
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Bexsero
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2
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8 weeks
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If primary course completed at ≤ 6 years of age – single booster dose 3 years after completing the primary schedule
If primary course completed at ≥ 7 years of age – single booster dose 5 years after completing the primary schedule
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≥ 10 years
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Bexsero
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2
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8 weeks
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A single booster dose 5 years after completing the primary schedule
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+ MenB vaccine (Bexsero) route IM, dose 0.5 mL. MenACWY and MenB vaccines can be given at the same time. Prophylactic use of paracetamol is recommended when MenB is administered to children < 2 years of age. There is an increased risk of fever when MenB is co-administered with other routine vaccines, however this is not a contraindication.
3. Influenza
Table 4: Influenza vaccine3
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Age£
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Influenza vaccine
brand
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Route
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Dose
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Number of doses required if receiving the influenza vaccine for the first time
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Number of doses required if previously received one or more doses of influenza vaccine
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6 months to < 9 years
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Refer to the WA Immunisation Schedule seasonal influenza vaccine recommendations
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IM
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0.5 mL
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2 doses, minimum 4 weeks apart
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1 dose#
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9 years and over
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Refer to the WA Immunisation Schedule seasonal influenza vaccine recommendations
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IM
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0.5 mL
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1 dose*
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1 dose
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£ All children with asplenia require annual influenza vaccine at the start of each influenza season, given their increased risk of severe infection.
# One dose is required in subsequent years even if only one dose was given in the first year of influenza immunisation.
* 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 type b (Hib)
All patients require review of their Hib vaccination status. Catch-up schedules are outlined in table 5.
Table 5: Haemophilus influenzae b vaccine.3
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Age at diagnosis
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Vaccine history
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Number of further primary doses required#
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Number of booster doses required ≥ 18 months of age, or 2 months after the last dose, whichever is later
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Number of previous Hib doses |
Current age |
Age at time of 1st Hib vaccine |
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6 weeks to < 5 years
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No previous Hib doses
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< 7 months
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n/a
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3
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1
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7-11 months
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n/a
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2
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1
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12-17 months
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n/a
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1
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1
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18-59 months
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n/a
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1
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-
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1 previous Hib dose
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< 12 months
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< 7 months
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2
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1
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7-11 months
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1
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1
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12-17 months
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< 12 months
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1
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1
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≥ 12 months
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-
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1
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18-59 months
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< 18 months
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-
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1
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≥ 18 months
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-
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-
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2 previous Hib doses
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< 12 months
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< 7 months
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1
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1
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≤ 12 months
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7-11 months
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-
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1
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12-17 months
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< 12 months
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-
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1
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12-17 months
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≥ 12 months
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-
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-
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18-59 months
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< 12 months
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-
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1
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≥12 months
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-
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-
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3 Hib doses received previously
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All 3 doses at < 12 months
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-
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1
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≥ 1 dose at 12-17 months
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-
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-
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≥ 5 years
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Primary course complete
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-
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-
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Never received a dose of Hib-containing vaccine or primary course incomplete
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-
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1
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#For all Hib containing vaccines; route IM, dose 0.5 mL. The minimum interval between primary doses for Hib is 4 weeks. Hib age-appropriate combination vaccines are acceptable, e.g. DTPa- HepB-IPV-Hib (e.g. Infanrix-hexa or Vaxelis) 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.
Glossary of vaccination abbreviations
- PCV – Pneumococcal conjugate vaccine
- MenACWY – Meningococcal quadrivalent vaccine covering serogroups A, C, W-135 and Y
- MenB – Meningococcal vaccine covering serogroup B
| Approved by: |
PCH Medication Safety Committee |
Date: |
Sep 2025 |
| Endorsed by: |
CAHS Drugs and Therapeutics Committee |
Date: |
Oct 2025 |
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