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COVID-19 Nice guideline summary: Cystic fibrosis

Nice has published a rapid guideline for the care of patients with cystic fibrosis (CF) during the COVID-19 pandemic, including those with suspected or confirmed cases of the virus

Unplanned learning

It may be useful to print this summary as a resource for your pharmacy team – PDF available here.

The recommendations in this guideline are designed to protect staff from infection and ensure the safety of patients with cystic fibrosis (CF), while making the best possible use of resources and enabling services to match capacity to patient need during the pandemic.

It can be hard to distinguish between symptoms of COVID-19 and symptoms of pulmonary disease exacerbation. If it is suspected that a person with CF has COVID-19, they should contact their CF team as soon as symptoms develop, to ensure there is an accurate assessment. NHS 111 or NHS 111 online should also be contacted, or 999 in an emergency.

Communicating with patients and minimising risk

Communicating with patients and their families or carers can help to support their mental wellbeing. Remind patients that charities such as the Cystic Fibrosis Trust and support groups including NHS volunteers are available and can help to relieve anxiety about COVID-19. There is also UK government guidance available on mental health during the pandemic.

When communicating with patients, pharmacists are reminded to minimise face-to-face contact as much as possible.

CF patients not known to have COVID-19

Until the pandemic has passed, patients, families and carers are advised to follow the relevant parts of UK government advice on shielding and protecting people defined on medical grounds as extremely vulnerable to COVID-19.

CF patients known or suspected to have COVID-19

If a patient has symptoms of COVID-19, airway clearance should be carried out in a well-ventilated room. Where possible, this should be done away from other members of the household. Others should not enter the room until a sufficient amount of time has elapsed, in order for the aerosol droplets that may have been generated to pass. UK government guidance on infection prevention and control should also be followed. Sputum generation may potentially create an infectious aerosol.

Advise patients, their family and carers that using nebulisers to administer an antibiotic will not carry virus particles from the patient as the aerosol comes from the fluid in the nebuliser. However, a contaminated droplet can be formed if the aerosol coalesces with a contaminated mucous membrane: therefore appropriate hand hygiene needs to be practised when handling a nebuliser mask.

Planning treatment and care

Advise patients to continue with their usual self-care arrangements, such as:

  • Prophylactic medication, including oral and inhaled antibiotics and mucoactive agents
  • Airway clearance techniques
  • Cystic fibrosis transmembrane conductance regulator (CFTR) therapies
  • A healthy diet, vitamins and pancreatic enzyme replacement therapy
  • Home exercise, while following UK government guidance on shielding and protecting people defined on medical grounds as extremely vulnerable to COVID-19.

If symptoms of an exacerbation develop, patients should manage this the same way as previously, including taking rescue medication and informing their cystic fibrosis team.

Equipment

Advise patients to clean all equipment (eg mouth pieces and face masks used for nebulised treatment or non-invasive ventilation) regularly with detergent and warm water, or as per manufacturer instructions.

Modifications to usual care

Patients with CF can still access CFTR therapies under the NHS England policy statement. Any changes to a patient’s individual treatment plan should be recorded, stating the reason for the modification, and should include all relevant members of the multidisciplinary care team. It should also take into account the clinical circumstances and preferences.

Patients with CF should be monitored closely due to being at greater risk of rapid deterioration if they contract COVID-19. Conduct liver function testing and eye monitoring only when clinically essential. Use home spirometry if possible, and only carry out lung function tests in hospital if the results will have a direct impact on patient care.

Nice has also advised doctors that patients should be dispensed medication to meet their clinical needs for no more than 30 days, as quantities higher than this can risk disrupting the supply chain during this period.

This summary is intended as a quick resource for pharmacy teams on the Nice COVID-19 rapid guideline: Cystic fibrosis. For more detailed information, please see the full guideline.

References

National Institute for health Care and Excellence (2020) Nice COVID-19 rapid guideline: Cystic fibrosis. Nice guideline (NG170).

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