GUIDELINES FOR MANAGEMENT OF PATIENTS WITH AN ABSENT OR DYSFUNCTIONAL SPLEEN

People with an absent or dysfunctional spleen are at increased risk of severe infection. The risk is greatest in the first 2 years following splenectomy, but persists throughout life. The commonest infection is with Streptococcus pneumoniae, but other organisms also present significant risks e.g. Haemophilus influenzae type b (Hib) and Neisseria meningitidis. In January 1994 the Chief Medical Officer wrote to all doctors regarding the consequences of splenectomy. Further advice was issued in March 2001. The 2006 edition of the book Immunisation Against Infectious Disease (The Green Book) also gives information on immunisation of individuals with an absent or dysfunctional spleen. The following procedures should be followed for all asplenic/hyposplenic patients: - 1. MEDICAL RECORDS

These should be flagged and the patient should carry a card or wear a bracelet/necklet stating the risk of infection.

2. VACCINATION

The patients GP should be informed of any vaccines given in hospital. Ideally the vaccines should be given 4 to 6 weeks before elective splenectomy where this is not possible they should be given up to 2 weeks before. If it is not practicable to vaccinate 2 to 6 weeks before splenectomy, immunisation should be delayed until at least 2 weeks after the operation as functional antibody responses may be better at this time. However, if the patient leaves hospital before this time, the vaccination should be given before discharge if delaying is likely to result in failure to vaccinate. Vaccination should be given at least 2 weeks before the initiation of immunosuppressive chemotherapy or radiotherapy. If this is not possible, immunisation can be delayed for at least 3 months after completion of treatment in order to maximise the response to the vaccine. Immunisation of these patients should not be delayed if this is likely to result in a failure to vaccinate. A table from The Green Book 2006, p51 detailing the immunisation of individuals with asplenia or splenic dysfunction is reproduced in Appendix 1.

Approved at Medicines Management Committee 8th November 2007 Page 1 of 6 Issue Number 2 Review Date: October 2009

Approved at Medicines Management Committee 8th November 2007 Page 2 of 6 Issue Number 2 Review Date: October 2009

The following vaccines are recommended: - a) Pneumococcal Vaccine

Adults A single injection of pneumococcal polysaccharide vaccine (PPV) should be given to adults who have not received a dose of this vaccine in the last 5 years. Reimmunisation is currently recommended every 5 years. Children Children under 2 years should be immunised according to the routine childhood immunisation schedule (Reproduced in Appendix 2) with pneumococcal conjugate vaccine (PCV). A dose of PPV [Pneumovax II] is then given after the 2nd birthday, at least 2 months after the last dose of PCV [Prevenar]. Children aged 2 years to under 5 years who have already been fully vaccinated with PCV according to the routine childhood schedule should receive an additional dose of PCV at the time they become hypo/asplenic, followed by a single dose of PPV 2 months later. Children aged 2 years to under 5 years who have not already received a full course of PCV should be given 2 doses of PCV 2 months apart followed by a dose of pneumococcal polysaccharide vaccine (PPV) 2 months after the final PCV dose. Children aged 5 years and older should follow the schedule for adults. A gap of at least 2 months should be left after the final dose of PCV before the PPV is given. Reimmunisation with PPV is recommended every 5 years.

b) Meningococcal C conjugate vaccine.

Children under 2 years should follow the routine immunisation schedule see Appendix 2. Adults, and children of 2 years and over, who have not previously been immunised with meningococcal C conjugate vaccine (Men C vaccine) according to the UK schedule should be given 2 doses 2 months apart. These doses are given together with Hib vaccine, which is also recommended (see below), as the combined Hib/Men C vaccine Menitorix. Single component Hib vaccine is no longer available in the UK. Individuals who have been fully immunised with Men C and Hib vaccine according the UK schedule previously, but then develop splenic dysfunction, or require a splenectomy, should be given one dose of Menitorix. Vaccination can be performed at the same time as pneumococcal vaccination. See also Hib Catch Up Programme 10/9/07 to 3/3/09 below.

Approved at Medicines Management Committee 8th November 2007 Page 3 of 6 Issue Number 2 Review Date: October 2009

c) Haemophilus influenzae type b vaccine (Hib)

Children under 2 years should follow the routine immunisation schedule see Appendix 2. Adults and children of 2 years or over, who have not previously been immunised with Hib vaccine according to the UK schedule should be given 2 doses 2 months apart. These doses are given as the combined Hib/Men C vaccine Menitorix. Single component Hib vaccine is no longer available in the UK. Individuals who have been fully immunised with Men C and Hib vaccine according the UK schedule previously, but then develop splenic dysfunction, or require a splenectomy, should be given one dose of Menitorix. Vaccination can be performed at the same time as pneumococcal vaccination. Hib Catch Up Programme 10/9/07 to 3/3/09 All children born between 13/3/03 and 3/9/05 are scheduled to be included in this catch up (See Appendix 2). Take individual immunisation history and any routine scheduled immunisations into account when prescribing Hib containing vaccines for children who become hypo/asplenic.

d) Influenza vaccine

Should be given annually.

3. ANTIBIOTICS

The first 2 years after splenectomy is the period of highest risk, so antibiotic prophylaxis should be given for that time, and should also be given to children up to 16 years of age. Recommended dosages: Adult phenoxymethyl penicillin 500 mg B.D. Child 6 - 12 250 mg B.D Child 8 250 - 500 mg daily Child 2 - 8 250 mg daily Child

Approved at Medicines Management Committee 8th November 2007 Page 4 of 6 Issue Number 2 Review Date: October 2009

If the patient starts to feel unwell he/she should be warned to seek medical attention immediately, even if taking prophylactic antibiotics.

4. FOREIGN TRAVEL

Malaria poses a threat to people without a functioning spleen. The importance of taking anti-malarial prophylaxis and other precautions (insect repellents, correct clothing and screens at night) should be emphasised. Asplenic/hyposplenic patients travelling to counties in which Group A meningococcal disease is common e.g. those travelling to sub-Saharan Africa, India and Nepal, will require meningococcal quadrivalent A, C, W135, Y vaccine. Pilgrims travelling to Saudi Arabia for Hajj or Umrah will also require the quadrivalent vaccine as outbreaks of meningococcal W135 infection have occurred there. Boosters for those at ongoing risk are required every 5 years. N.B. A period of at least 2 weeks should be left between vaccination with meningococcal C conjugate vaccine and subsequent vaccination with A, C, W135, Y meningococcal vaccine.

5. OTHER MEASURES

Asplenic patients are particularly susceptible to infection following animal bites and insect bites, and should be alerted to this, so that they attend promptly for appropriate management. Capnocytophaga canimorsus may follow animal (particularly dog) bites. The infection responds to a five-day course of co-amoxiclav. Babesiosis is a rare tick borne infection which can affect asplenic patients following a tick bite. I have no functioning spleen cards can be downloaded from the Department of Health website (www.dh.gov.uk). A patient information leaflet can also be downloaded from the same site. References 1. Department of Health. Asplenic patients and immunisation. C.M.Os Update 1994; 1:3. 2. Department of Health. Meningococcal immunisation for asplenic patients. Current Vaccine & Immunisation Issues. 9

March 2001. 3. Department of Health Update on Immunisation Issues - CMOs Update August 2002. 4. Department of Health. The Pneumococcal Immunisation Programme for Older People & Risk Groups. 31 March

2005. 5. Department of Health. Important Changes to the Childhood Immunisation Programme CMO 12 July 2006. 6. Department of Health. Immunisation Against Infectious Disease (The Green Book) 2006.

7. Update of Guidelines for the Prevention and Treatment of Infection in Patients with an Absent or Dysfunctional Spleen.

Clinical Medicine (Journal of the Royal College of Physicians of London, Vol 2, No. 5, Sept/Oct 2002, p 440 - 443).

8. Department of Health. Haemophilus Influenzae Type b [Hib] Vaccine for Young Children----Catch-up Programme. 23 July 2007.

APPENDIX 1

Approved at Medicines Management Committee 8th November 2007 Page 5 of 6 Issue Number 2 Review Date: October 2009

Immunisation of individuals with asplenia or splenic dysfunction

Suggested schedule for immunisation in individuals with splenic dysfunction and immunosuppression.

Age at which asplenia or splenic dysfunction or immunosuppression is acquired

Vaccination schedule Where possible, vaccination course should ideally be started at least two weeks before surgery or commencement of immunosuppressive treatment. If not possible, see advice in pneumo chapter.

Month 0 Month 2 Month 4

Under two years Routine immunisation schedule should be followed.*

Over two to under five years (fully vaccinated including booster)

Booster dose of Hib/MenC vaccine Booster dose of PCV

Single dose of PPV None

Over two to under five years (unvaccinated or partially vaccinated)

First dose of Hib/MenC vaccine First dose of PCV

Second dose of Hib/MenC vaccine Second dose of PCV

Single dose of PPV

Five years and older (and previously vaccinated with Hib, MenC, PCV vaccines)

Booster dose of Hib/MenC vaccine Single dose of PPV

Five years and older (unvaccinated)

First dose of Hib/MenC vaccine Single dose of PPV

Second dose of Hib/MenC vaccine

PCV = pneumococcal conjugate vaccine, PPV = pneumococcal polysaccharide vaccine

Taken from the 2006 edition of The Green Book (Immunisation Against Infectious Diseases) p51 * In addition a dose of PPV is given after the 2nd birthday (at least 2 months after the last dose of PCV). See page 303 of The Green Book

APPENDIX 2

Approved at Medicines Management Committee 8th November 2007 Page 6 of 6 Issue Number 2 Review Date: October 2009

Schedule for the UKs routine childhood immunisations

When to immunise What vaccine is given How it is given

Two months old Diphtheria, tetanus, pertussis (whooping

cough), polio and Hib (DTaP/IPV/Hib) One injection

Pneumococcal (PCV) One injection Three months old Diphtheria, tetanus, pertussis (whooping

cough), polio and Hib (DTaP/IPV/Hib) One injection

MenC One injection Four months old Diphtheria, tetanus, pertussis (whooping

cough), polio and Hib (DTaP/IPV/Hib) One injection

MenC One injection

PCV One injection

Twelve months old Hib/MenC One injection

Around 13 months old Measles, mumps and rubella (MMR) One injection

PCV One injection Three years four months to five years old *

Diphtheria, tetanus, pertussis and polio (DTaP/IPV or dTaP/IPV)

One injection

Measles, mumps and rubella (MMR) One injection

Thirteen to 18 years old Tetanus, diphtheria and polio (Td/IPV) One injection

Taken from the 2006 edition of The Green Book (Immunisation Against Infectious Diseases) p80. *Between 10/9/07 and 3/3/09, the routine childhood schedule includes a Hib catch-up programme for children born between 13/3/03 and 3/9/05. [These children will not have received the dose of Hib/Men C which is now scheduled at 12 months old]. Between age 3 years 4 months and 5 years, the above children are scheduled to receive DTaP/IPV/Hib as their pre school booster instead of DTaP/IPV or dTaP/IPV. Or, if they have already received their pre school booster without Hib, they are scheduled to receive a dose of Hib/Men C.