N Letter

Background

Children with neutropenia are at increased risk of bacterial and fungal infections. Preventing infections is this group is therefore a priority and we should be aiming to protect these children against vaccine preventable diseases (VPDs) according to the National Immunisation Program (NIP).

There is a lack of direct data to base vaccine recommendations in this group. This group of patients is not addressed in the Australian Immunisation Handbook. A number of international expert groups have however published recommendations in this area (see resources).

Inactivated vaccines are considered safe, and international guidelines recommend they be administered to patients with neutropenia as per routine immunisation schedules. Given patients with neutropenia are at increased risk of bacterial infections, some also recommend additional pneumococcal vaccines, as for patients at increased risk of invasive pneumococcal disease (IPD).

Live-attenuated viral vaccines are contraindicated in patients who are severely immunocompromised, due to the risk of unchecked viral replication and vaccine associated disease. Neutropenia itself, however, is not a contraindication to live-attenuated viral vaccines as patients with an isolated neutropenia would be expected to handle viral infections, and live-attenuated viral vaccines in most circumstances. International guidelines recommend these vaccines be given to patients with congenital or cyclical neutropenia, or neutropenia in general. However, some recommend against these vaccines in patients with undefined phagocytic cell defects or phagocytic cell defects undefined at a molecular level, and others recommend prior immunology review. This is because some conditions which affect neutrophil number or function may also affect lymphocyte number or function. Live-attenuated viral vaccines are contraindicated in patients with leucocyte adhesion disorder, Chediak Higashi syndrome or other defects in cytolytic granule release.

Live bacterial vaccines (including the BCG vaccine) are contraindicated in patients who are immunocompromised including those with some phagocytic cell defects. This is due to an increased risk of vaccine associated adverse events in this group.

Our recommendations are based on these international guidelines and local consensus.

Recommendations

Inactivated vaccines

Inactivated vaccines are recommended as per the NIP. We also recommend additional pneumococcal vaccines, as for patients at high risk of invasive pneumococcal disease (see resources).

Live-attenuated viral vaccines

Neutropenia itself is not a contraindication to live-attenuated viral vaccines. In cases of an undefined neutropenia some caution should be exercised, given neutropenia can occur in the setting of conditions which also affect lymphocyte number and function

For patients with congenital neutropenia or cyclical neutropenia caused by a known molecular defect (which is not associated with any other immune dysfunction), live-attenuated viral vaccines are recommended as per the NIP.

A number of patients with chronic moderate-severe neutropenia (neutrophil count <1 x10^9/L) will not have a defined molecular defect, the cause may be unclear, or they may have suspected immune neutropenia for which there is no diagnostic test. In these patients, an evaluation for an underlying immune defect is recommended prior to administration of live-attenuated viral vaccines (see MVEC Flowchart: Live attenuated viral vaccines in patients with chronic neutropenia). If there are no concerning features on history, examination or screening investigations, live-attenuated viral vaccines are recommended, as per the NIP. If any abnormalities or concerns are raised, a formal immunology consult should be sought prior.

In patients for whom the neutropenia is expected to be transient, or further investigation is planned in the near future (e.g. bone marrow examination or molecular studies), consideration should be given to delaying live-attenuated viral vaccines until this information is available.

Live-attenuated viral vaccines are contraindicated in patients with leucocyte adhesion disorder, Chediak Higashi syndrome or other defects in cytolytic granule release.

Live bacterial vaccines

Live bacterial vaccines (e.g. the BCG vaccine, oral typhoid vaccine) are generally contraindicated in patients with neutropenia. Typhoid protection can be provided by using an inactivated vaccine. If there is a high risk of Tuberculosis in an individual with neutropenia, BCG vaccine should be discussed with both an immunisation specialist and haematologist.

Resources

• MVEC Flowchart: Live attenuated viral vaccines in patients with chronic neutropenia
• Australian Immunisation Handbook: Risk conditions for Pneumococcal Disease
• Australian Immunisation Handbook: Vaccination for people who are immunocompromised
• 2013 IDSA Clinical practice guideline for vaccination of the immunocompromised host
• Medical advisory committee of the immune deficiency foundation: Recommendations for live viral and bacterial vaccines in immunodeficient patients and their close contacts 2014
• Canadian Immunisation Guide: Immunization of immunocompromised persons
• Martire B, Azzari C, Badolato R, Canessa C, Cirillo E, Gallo V, et al. Vaccination in immunocompromised host: Recommendations of Italian Primary Immunodeficiency Network Centers (IPINET). Vaccine. 2018;36(24):3541-54

Background

Many people may find the experience of having a needle an unpleasant one. However, for some people, the experience is much more difficult, presenting as a real phobia characterised by both severe and persistent anxiety and fear. As a result, this patient group often avoid needle-related experiences. This needle avoidance extends to the area of immunisation, resulting in the individual being left susceptible to vaccine preventable diseases. This can result in further procedures involving needles should they become unwell.

Generally speaking, it is recommended that children and adults with anxiety, intellectual disabilities and needle phobia’s avoid attending large scale vaccination sites for immunisation. These sites are loud and busy and can increase distress. Smaller settings such as a GP clinic or pharmacy may be preferred. It is important to have a discussion with your immunisation provider prior to the appointment in order to make an individualised plan for how to approach the immunisation experience.

Strategies to manage needle phobia and immunisation- information for individuals and care givers

• needle phobia is real and it’s ok to be scared
• do not criticise the child or individual, this can make it worse
• with young children, it may be best not to tell them until you arrive at clinic, as this will stop them overthinking the needle
• use words such as “arm medicine” or “vaccine” and avoid using words like “needle” or “injection” when descibing what is about to happen
• the use of rewards for children may help encourage bravery
• the application of local anaesthetic cream patches to the site a minimum of 45 minutes prior to appointment (available for purchase from chemists), may help to settle the fear of pain
• needle phobia can be a lifelong problem if not treated appropriately.

Specialist phobia treatments

Specialist trained psychologists and more recently hypnotherapists have had significant success in giving an individual mechanisms and strategies to help overcome or manage their needle phobia. Considering either pathway is recommended as a long term strategy to overcome needle phobia.

Strategies to manage needle phobia and immunisation- information for health care providers

Effective preparation, distraction techniques, pain management strategies and addressing the core reason for the phobia are all important steps in ensuring successful immunisation.

Children and adolescents

• encourage parents not to over discuss the vaccine/needle with the children prior to arrival as this can build up their fear
• have a plan in place with the parents prior to their arrival at the clinic (in known phobic children)
• carry out pre-immunisation checks and side effect explanation with parents when the child is out of the room or prior to arrival
• minimise discussions in front of the child as this can increase distress
• an individualised approach is more likely to lead to success
• do not rush or attempt to force children to be vaccinated, this is likely to result in failure to vaccinate
• take you time with clearly distressed patients, calmly talk them through the procedure and tips to manage their anxiety (deep breathing, looking away or counting).

People with autism spectrum disorder (ASD)

The following strategies may assist in managing needle phobia in individuals with ASD:

• discuss the individual’s communication style and capabilities with their parent/carer
• explain everything you are going to do using clear, simple language
• involve the individual as much as possible by considering their likes/dislikes
• provide support and positive reassurance
• remove distracting/disturbing stimuli
• try not to stop stimming behaviours (eg. rocking, flapping) that may help the individual deal with distress
• do not attempt to restrain the individual
• older individuals with ASD can be very difficult to immunise due to significant needle phobias. Restraining these children can result in injury to the carer, child or health care provider
• have a low threshold to refer to a specialist immunisation provider for sedation.

Distraction techniques

Young children or the intellectually disabled:

• bubbles upon entering the room and before and after needle
• musical toys
• if able have one person blowing bubbles or creating noise with toy intermittently to maintain attention of child
• TV on with kids shows
• child watching their favourite show on parents’ phone.

Older children, adolescents or adults:

• phone/iPad with headphones watching their favourite show or music (give them time to settle into watching it)
• make conversation eg. sport, upcoming holidays
• countdown so they know when to be ready
• strongly encourage them to look away.

Administration techniques

• prepare the vaccine out of view of the individual or before they come into room
• avoid showing the individual the prepared vaccine and needle prior to administration
  • place the tray containing vaccines out of sight
  • hold the vaccine to your side or behind you back until about to inject
  • encourage the individual to close their eyes, look away or focus on the distraction method
• don’t over discuss the needle and administration of the vaccine
  • do necessary pre-vaccination checks and side effects only
  • make the process as quick as possible to give the patient minimal time to over-think the needle
• encourage adult individuals to bring a support person if they wish
• a phobia in adolescents or adults can lead to a vasovagal or faint response. It is important that the individual is supported to lay down if needed.

Sedation

If immunisation attempts are unsuccessful, a referral for vaccines to be administered under sedation may be considered.

Paediatric services

• The Royal Children’s Hospital – Referrals for this service can be sent to Nurse Practitioner Immunisation via [email protected]
• Monash Health – Referrals can be submitted as a HealthLink referral
• Family Immunisation and Travel Specialists (FITS) – Make an appointment via webpage / web referral

Adult services

• Monash Health – Referrals can be submitted as a HealthLink referral.

Resources

RCH resources

• The Royal Children’s Hospital Clinical Practice Guidelines for Procedural Pain Management
• The Royal Children’s Hospital Be Positive: Nitrous Oxide
• The Royal Children’s Hospital comfort kids- for kids
• The Royal Children’s Hospital comfort kids- for parents
• The Royal Children’s Hospital Be Positive: Immunisations
• RCH Kids Health Info Podcast: Fear of needles. What helps?

Other resources

• Dr Daryl Cheng’s Needle Phobia interview with The House of Wellness at Channel 7
• RACV: Scared of needles? Try these simple tips
• The Conversation: Is your child frightened of needles? Here’s how to prepare them for their COVID vaccine

There are a number of neurological conditions that are associated with an increased risk of vaccine preventable diseases. These include:

1. Epilepsy and seizure disorders
2. Developmental delay and intellectual disability
3. Cerebral palsy
4. Neuromuscular disorders
5. Hydrocephalus and children with ventricular-peritoneal shunts

Children with any of these underlying conditions should have routine vaccinations according to the Australian National Immunisation Program Schedule.

In children where epilepsy is unstable, or there is a progressive neurological disorder of unclear aetiology, specialist consultation is recommended to advise on the appropriate vaccination course. In Victoria, referrals for this service should be made to SAEFVIC.

Influenza vaccination

All children with neurologic and neurodevelopmental conditions are at high risk of severe and complicated influenza infection.

This includes disorders that do not have an obvious compromise in respiratory function such as intellectual disability and epilepsy. Influenza infection can also markedly increase seizures in children with epilepsy.

These children should all be offered annual influenza vaccination from 6 months of age. Two doses are required in the first year the vaccine is received if less than 9 years of age [see Resources].

Pneumococcal vaccination

Children with CSF leaks and intracranial shunts are at higher risk of invasive pneumococcal disease. This includes brain inflammation (meningitis) and blood infection (bacteraemia). These children should be offered additional vaccines, including an extra pneumococcal conjugate vaccine to optimise protection.  See the Pneumococcal chapter in the Australian Immunisation Handbook for the latest recommendations [see Resources]

Human Papillomavirus (HPV) Vaccination

Transmission of the HPV virus is primarily through sexual intercourse and can also occur via non- penetrative sexual contact.

There is some evidence for potential virus transmission via viral particles on hands.

HPV infection is associated with the potential development of serious disease such as cervical and anogenital cancers. The infection is also the primary cause of anogenital warts.

As transmission can be varied, children with neurodevelopmental conditions should be offered HPV vaccination as per the routine secondary school program.

Resources

• The Australian Immunisation Handbook: Pneumococcal chapter
• HPV Cancer Council
• MVEC: Influenza Vaccine Recommendations
• MVEC: Human Papilloma Virus (HPV)
• MVEC: SAEFVIC