Background

Cystic Fibrosis (CF) is a genetic disorder, primarily affecting the lungs as well as the pancreas, liver, kidneys and intestines.  In patients with CF, the cells that are responsible for producing mucous, sweat and other fluids are impaired. As a result of this, secretions become thick and sticky, impacting the function of certain organs in the body. 1 in 2500 babies in Australia are born with CF each year.

Symptoms of CF include frequent chest infections, poor weight gain and growth, intestinal obstructions, infertility (commonly in males) and diabetes. Treatment includes intensive daily physiotherapy, enzyme replacement medication, salt and vitamin supplements, exercise and a high calorie diet. Insulin and blood glucose monitoring is required for patients that develop diabetes. Many patients progress to requiring a lung transplant resulting in immunocompromise. CF patients are at high risk of infections, including some that are vaccine preventable.

Immunisation recommendations

Patients with CF are recommended to complete the routine immunisation schedule as per the National Immunisation Program (NIP) as well as some additional funded immunisations.

Secondary school vaccines are available on the NIP and should be administered to all children with CF including Year 7 (12-13 years of age): Boostrix® (diphtheria–tetanus–pertussis) and Gardasil® 9 (human papillomavirus) and in Year 10 (14-19 years) Nimenrix® (meningococcal ACWY).

Influenza

Patients with CF are funded to receive influenza vaccines annually from 6 months of age. 2 doses of the age-appropriate vaccine are required in the first year of receiving the vaccine [refer to MVEC: Influenza vaccine recommendations].

Pneumococcal

Prevenar 13® (pneumococcal conjugate) should be given 6 weeks, 4 months, 6 months (additional dose) and 12 months.

A dose of Pneumovax® 23 (pneumococcal polysaccharide) should be should be given at 4 years of age. A second dose should then be given at least 5 years later.

Further booster vaccines are required if being worked up for a lung transplant [refer to MVEC: Solid organ transplant recipient]

COVID-19

Patients with CF are at an increased risk of severe symptoms of COVID-19 disease if they become infected. A primary course of vaccination is recommended for all individuals aged 6 months and over, with booster doses recommended for some individuals, depending on age and additional risk factors.

Other vaccines to consider

Varicella

Currently a single dose of varicella vaccine is funded on the NIP. The combination MMRV (ProQuad/Priorix-tetra) is scheduled for 18-months of age, however giving 2 doses of the varicella vaccine can enhance protection. A second varicella vaccine dose can be obtained with a private script. As the varicella vaccine is a live-attenuated vaccine, doses should be separated by a minimum of 4 weeks.

Hepatitis A

CF patients are at risk of associated liver disease. The Royal Children’s Hospital funds a 2-dose course of Hepatitis A vaccines for their patient cohort. Administration can be commenced from 12 months of age.

Resources

Authors: Rachael McGuire (SAEFVIC Research Nurse, Murdoch Children’s Research Institute) Nigel Crawford (Director SAEFVIC, Murdoch Children’s Research Institute) and Nadine Henare (Nurse Coordinator- Immunisation, The Royal Children’s Hospital)

Reviewed by: Francesca Machingaifa (MVEC Education Nurse Coordinator)

Date: August 5, 2022

Materials in this section are updated as new information and vaccines become available. The Melbourne Vaccine Education Centre (MVEC) staff regularly reviews materials for accuracy.

You should not consider the information in this site to be specific, professional medical advice for your personal health or for your family’s personal health. For medical concerns, including decisions about vaccinations, medications and other treatments, you should always consult a healthcare professional.