Appendix 12 – Unusual or recurrent infections
(based on Nottingham guidelines), guidance on standard and additional immunisations, GP and parent letter/template
Background:
Vulnerability to URTIs – 30% increased risk of death from sepsis (Garrison, 2005) – Impaired immune competence: • Reduced T and B lymphocyte subpopulations • Reduced neutrophil chemotaxis • Thymic abnormalities. • Alterations of Ig subclasses.
Note for recurrent Respiratory infections - a lot of Down Syndrome aspiration is silent and this is the difficulty in detecting it on history alone. Certainly if any child with Down Syndrome has above average respiratory issues compared with a child without Down Syndrome, then aspiration MUST be sought and do not hold back on videofluoroscopy (advice from the Brompton respiratory physicians 2018)
Prophylactic antibiotics
If significant infection history, it may still be appropriate to commence prophylactic antibiotics even when immunological tests are normal. These should be considered in children with frequent infections either to be used throughout the year or just September to April.
Options – e.g. azithromycin 10mg/kg od 3 days per week. The need for prophylactic antibiotics should be reviewed at each visit. If stopping, suggest choosing to do this in late spring/early summer.
General management: Consider using double the length of the usual antibiotic course. Maximise immunity by ensuring appropriate vaccines have been given (as per childhood schedule and any additional vaccines – see below).
Immunological investigations: Immunoglobulins, Functional Antibodies, Prevnar (pneumococcal) Antibodies and lymphocyte subsets are sometimes done routinely in some DS pathways/boroughs at the 12m review. In Barnet/RFH we are planning to have a low threshold for immune testing and discussing with our immunology teams and not test routinely in every child.
Eg we would test at any time if ‐ with ≥ 4 infections in 6 months requiring GP visits/ill-health > 5 days or admission for sepsis or there was an unusual infection.
Timing of immunology blood tests is at least 1 month after completion of the 13 month routine Hib/ MenC/ PCV booster immunisations.
Persistent lymphopenia/hypogammaglobulinaemia/recurrent infections:
The question relates to interpretation of lymphocyte subsets in patients with T21. We all talk about the cohort being 'relatively immunodeficient' however we don’t have specific knowledge about the range of 'normal' Lymphocyte subsets (LSS) and Immunoglobulins (Igs).
Refer to the local haematology clinic/immunology clinic but also consider referral to GOSH immunology:
Immunologist at GOSH, “We try to see T21 patients at least once here if they have been detected to have lymphopenia/hypogammaglobulinaemia/recurrent infections. We are also happy to see them if they haven't had an LSSMEM/IgGAM/vaccine responses checked and/or history of infections. We see quite a wide spectrum of immunodeficiency in children with T21.
We have a specific clinic where we see children and young people with syndromes like Down Syndrome.
If they are referred into the Immunology Department - even if, as above, with no LSS/IgGAM/vaccine responses done elsewhere - we will triage them into the most appropriate clinic and see the child.
IMMUNISATIONS:
In addition to the normal UK immunisations schedule - https://www.gov.uk/government/publications/pre-school-vaccinations-a-guide-to-vaccinations-from-2-to-5-years -
Annual influenza vaccine via GP/practice nurse (from 6 months to 2 years this is the inactivated injected form, after 2 years it is the nasal flu vaccine) for every person with Down Syndrome AND their household members. https://www.nhs.uk/conditions/vaccinations/child-flu-vaccine/
Pneumococcal vaccines – Babies in the UK are given the PCV (pneumococcal conjugate vaccine, also known as Prevenar 13) routinely as part of the UK immunization program ie Babies born on or after the 1 January 2020 have 2 injections, which are usually given at 12 weeks old and 1 year old. Babies born before this date will continue to be offered 3 doses, at 8 and 16 weeks and a booster at 1 year.
For further information/poster - https://assets.publishing.service.gov.uk/government/uploads/system/uploads/attachment_data/file/735239/Which_flu_vaccine_should_children_flu_vaccine_2018_.pdf
An additional pneumococcal immunisation is now universally agreed (from Autumn 2019 onwards) for every child with Down Syndrome and not only those at “high-risk:” Via GP/practice nurse.
Age 2-5yrs: single dose of Pneumovax II (also known as PPV 23 as it protects against 23 different pneumococcal strains).
Age >5 years: a single dose of Pneumovax II which lasts 5 years and then needs repeating.
The PPV vaccine is not effective under 2 years of age, hence why they receive the PCV.
Pneumovax II (PPV23) needs to be REPEATED EVERY 5 YEARS until 65 years of age (but should not be repeated within 5 years of the last one). (Green book on Immunisation for “at-risk” groups) but should not be repeated within 5 years.
If pneumococcal antibodies are tested (based on a clinical reason for checking) and still low despite recent Pneumovax II, please discuss with an immunology consultant locally or at GOSH.
Parental information: Pneumovax (aka PPV): https://www.medicines.org.uk/emc/files/pil.1061.pdf
(As discussed with Dr Elliman, Clinical lead for National NIPE & NBS Screening Programmes, Public Health England and Professor Helen Bedford, Senior Lecturer in Children's Health with the Centre for Epidemiology and Biostatistics at the UCL Institute of Child Health 2019).
Include in every report to the GP about the recommended extra vaccinations (Appendix 12) – annual flu vaccine and the extra pneumococcal vaccine. (There will also be routine letters being distributed around Autumn to GPs to remind them about these vaccinations due for ALL children with Down Syndrome). Ideally too, ensure that these children are given the first batches of flu vaccine that is made available for the GPs.
Also have a statement at the end of each report about the increased risk of infection and that in Down Syndrome these infections can sometimes present atypically. There is information in the new insert for the PHCR/red book about this as well.
Chickenpox and vaccines:
Vaccines for children with Down Syndrome are under review (2019) and more guidance will be published, including e.g. the VZV vaccine against chickenpox. For now, professionals need to have a low threshold to consider this vaccine if evidence of immunocompromise, as there have been serious cases of VZV pneumonitis in DS. Also inform GP and parents, that sometimes children with Down Syndrome don’t show the typical signs and symptoms of chickenpox as other children, so professionals need to consider chickenpox even if it presents in an atypical way, be aware of the complications and monitor them more closely.
Covid-19 and vaccines: RCPCH and JVCI and DSA websites
Further research:
Archives of Diseases of Childhood (Nov. 2018) Towards Evidence based medicine for paediatricians: Do children with Down syndrome benefit from extra vaccine?
All children with Down Syndrome should have annual influenza vaccines from 6 months of age.
23 valent pneumococcal vaccine from 2 years.
Consideration should be given to meningococcal ACWC (and Meningococcal B if not already given.)
Long term immunogenicity of vaccines unknown in this group and may require boosters
Recurrent vaccine preventable diseases may have negative impact on long term outcome.
Royal Free London NHS Foundation Trust
Edgware Community Hospital, Child Health HQ,
Burnt Oak Broadway, Burnt Oak, Edgware HA8 0AD
Letter to GP Date:
Dear Dr
Immunisation and Infection: Recommendations for Children with Down Syndrome (Revised Oct. 2020)
Re: Name:
Date of birth:
Address:
NHS number:
MRN Number
We are writing with regard to your patient who has Down Syndrome.
Children with Down Syndrome are at increased risk of infections, such as pneumonia and sepsis, secondary to altered immune function. Furthermore, there is often a suboptimal antibody response to vaccination, and children with Down Syndrome may not be able to maintain appropriate long-term immunity. We recommend the following advice in line with guidance by the national Down Syndrome Medical Interest Group (DSMIG.)
Children with Down Syndrome should receive all the immunisations in the normal UK schedule. In addition, the following immunisations are recommended in children with Down Syndrome,
Annual influenza vaccine before the winter months (injectable vaccine for infants). Influenza vaccine should also be considered for carers and family members.
Pneumococcal polysaccharide vaccine (PPV 23, also known as Pneumovax II), which is protective against 23 strains, is now recommended for all children with Down Syndrome over 2 years of age. It is not effective in children under 2 years of age.
Varicella vaccine if there is any suggestion of lowered immunity
If antibiotics are prescribed, double the length of the usual antibiotic course should be considered.
Yours sincerely,
Dr Christine Jenkins & Dr Ella Rachamim
MB BS BSc FRCPCH FRCP DRCOG DCH MRCGP MB BS BSc MRCPCH
Consultant Paediatrician, Community Child Health Specialist Doctor in Community Paediatrics
c.c. Parents/Carers for information
Royal Free London NHS Foundation Trust
Edgware Community Hospital, Child Health HQ,
Burnt Oak Broadway, Burnt Oak, Edgware HA8 0AD
Letter to Parents/Carers Date:
Dear Parents/ Carers,
We enclose a copy of a letter we have sent to your GP regarding immunisations for your child.
Many children with Down Syndrome have lowered immunity and are more prone to infections which can sometimes be serious. It is therefore recommended that they have:
Influenza (flu) vaccine every year. Influenza vaccine should also be considered for carers and family members.
Pneumococcal polysaccharide vaccine (PPV 23, also called Pneumovax II) if over 2 years of age.
All the immunisations in the normal UK schedule.
If you would like your child to have the additional vaccines, please take this letter to your GP surgery and make an appointment with the practice nurse or health visitor to discuss further.
Yours sincerely,
Dr Christine Jenkins & Dr Ella Rachamim
MB BS BSc FRCPCH FRCP DRCOG DCH MRCGP MB BS BSc MRCPCH
Consultant Paediatrician, Community Child Health Specialist Doctor in Community Paediatrics
c.c. GP for information
(Revised Oct. 2020)