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The purpose of this Guidelines summary is to maximise the safety of patients with cystic fibrosis and make the best use of NHS resources, while protecting staff from infection. It will also enable services to match capacity to patient needs if services become limited because of the COVID-19 pandemic.

This summary should be viewed in conjunction with NICE clinical guidance 78—cystic fibrosis: diagnosis and management.

Communicating with patients and minimising risk

  • Communicate with patients, their families and carers, and support their mental health and wellbeing to help alleviate any anxiety and fear they may have about COVID-19. Signpost to charities (such as the Cystic Fibrosis Trust), support groups (including NHS Volunteer Responders), and UK government guidance on the mental health and wellbeing aspects of COVID-19
  • Be aware that some patients, families or carers may need specialist psychological or social work support in the context of COVID-19
  • Minimise face-to-face contact to reduce the risk of infection by:
    • using telephone, video or email consultations whenever possible
    • cutting non-essential face-to-face appointments
    • contacting patients via text message, telephone or email
    • using electronic prescriptions rather than paper
    • providing home spirometry and, where appropriate, weighing scales
    • providing facilities to collect samples remotely
    • using different methods to deliver prescriptions and medicines to patients, for example, pharmacy deliveries, postal services, NHS Volunteer Responders, or introducing drive-through pick-up points for medicines
  • Tell patients, their families and carers that they should contact their cystic fibrosis team if they think the patient may have COVID-19, to ensure that their symptoms are appropriately assessed. They should do this as soon as they have symptoms. They should also contact the NHS 111 online coronavirus service or call NHS 111. In an emergency they should call 999 if the patient is seriously ill
  • Be aware that symptoms of COVID-19 and pulmonary disease exacerbations may be difficult to differentiate at initial presentation

Patients not known to have COVID-19

  • Tell patients, their families and carers to follow relevant parts of UK government guidance on shielding and protecting people defined on medical grounds as extremely vulnerable from COVID-19, until the risks associated with the COVID-19 pandemic have passed. This includes:
    • advice for other members of the household, noting the recommended shielding support for the patient and the need to follow UK government guidance on social distancing for everyone in the UK stringently. If this is not possible, for example, if they have a caring role for the patient, they may need to adopt shielding measures for themselves
    • advice for visitors who are providing essential care
    • advice on getting assistance with foods and medicines while shielding
    • support for mental wellbeing
  • For patients who still need to attend face-to-face appointments, ensure that existing arrangements to prevent cross-infection include COVID-19. See the NICE clinical guideline on cystic fibrosis
  • If patients need to attend face-to-face appointments, ask them to go alone if they can, or with no more than 1 family member or carer, to reduce the risk of contracting or spreading infection with COVID-19. They should avoid using public transport if possible

Patients known or suspected to have COVID-19

  • If a patient has symptoms of COVID-19 on presentation or admission, follow UK government guidance on investigation and initial clinical management of possible cases. This includes information on testing and isolating patients
  • All healthcare workers involved in receiving, assessing and caring for patients who have known or suspected COVID-19 should follow UK government guidance on infection prevention and control. This contains information on using personal protective equipment (PPE), including visual and quick guides for putting on and taking off PPE
  • Cystic fibrosis teams should report known or suspected cases of COVID-19 to the UK cystic fibrosis registry reporting hotline on the same day by emailing registry@cysticfibrosis.org.uk to request a call back
  • Ensure that relevant members of the cystic fibrosis team are involved in decisions about the care of patients with suspected COVID-19, including escalation of treatment
  • Patients with symptoms of COVID-19 should carry out airway clearance in a well-ventilated room, separate from other household members if possible, unless the patient needs assistance. This is because sputum generation is a potentially infectious aerosol generating procedure for COVID-19. Advise other family members not to enter the room until enough time has passed for aerosols to clear: follow UK government guidance on infection prevention and control
  • Explain to patients, their families and carers that when a nebuliser is used to administer an antibiotic, the aerosol comes from the fluid in the nebuliser chamber and will not carry virus particles from the patient. Tell families and carers to use appropriate hand hygiene when helping patients with their nebuliser mask. This is to prevent spread from a contaminated droplet that could be formed if the aerosol coalesces with a contaminated mucous membrane

Treatment and care planning

  • Tell patients, their families and carers to continue with all their usual self-care arrangements including, for example:
  • Tell patients, their families and carers to wash their hands and clean equipment such as face masks and mouth pieces used for nebulised therapies, or face masks used for non-invasive ventilation, by regularly using washing-up liquid or following the manufacturer’s cleaning instructions
  • Prescribe usual quantities of medicines to meet the patient’s clinical needs, normally 30 days’ supply. Prescribing larger quantities of medicines puts the supply chain at risk
  • Tell patients, their families and carers to follow the advice they have previously been given about what to do if they have an exacerbation, including taking rescue medication and contacting their cystic fibrosis team
  • Monitor patients with cystic fibrosis closely, because they may be at greater risk of rapid deterioration if they contract COVID-19

Modifications to usual care and service delivery

  • Think about how to modify usual care to reduce patient exposure to COVID-19 and make best use of resources (for example, workforce, facilities and equipment)
  • Specialist cystic fibrosis centres should maintain contact with network and outreach cystic fibrosis clinics using telephone or video consultations and meetings
  • Specialist cystic fibrosis centres should maintain sufficient inpatient beds and facilities for those patients for whom a hospital admission is essential
  • Day-case facilities may be necessary for procedures such as administering first doses of intravenous antibiotics for courses to be delivered at home and flushing totally implantable intravenous devices. Flushing frequency may be reduced from usual practice. If no alternative is possible, think about carrying out a home visit.
  • Ensure that sufficient clinical expertise and capacity remains within the cystic fibrosis team so that patients have the support they need, and their care can be safely managed out of hospital as much as possible
  • When modifying individual patients’ treatment plans:
    • take their clinical circumstances and preferences into account
    • involve all relevant members of the multidisciplinary team in the decision
    • record the reasoning behind each decision
  • Discuss the risks and benefits of changing treatment plans with patients, their families and carers
  • Only carry out lung function tests in hospital if the results will have a direct impact on patient care. Use home spirometry if possible
  • Be aware that patients with cystic fibrosis can still access cystic fibrosis transmembrane conductance regulator (CFTR) therapies under the NHS England policy statement for these drugs (refer to devolved national policies if appropriate). Note that the assessments and data collection frequencies recommended in the data collection agreement are the ideal, and the minimum requirement is annual assessment and reporting. Conduct liver function testing and eye monitoring only when clinically essential
  • Reduce the burden on frontline staff by following advice from NHS England and NHS Improvement about which data are not required (for example, the cystic fibrosis quality dashboard quarterly returns), and think about de-prioritising updating the cystic fibrosis registry (although note that centres will still need to meet the deadline of 31 January 2021)
  • Be aware that NHS England and NHS Improvement and NHS Blood and Transplant are working closely to enable limited access to transplants where this is feasible. However, given the severe capacity constraints because of managing the COVID-19 pandemic, the selection of patients from the waiting list for transplant will be considered on a case-by-case basis only. Referrals will only be seen in exceptional circumstances.
  • Defer the transition of young people to adult services until the pressures associated with the COVID-19 pandemic have passed

© NICE 2020. COVID-19 rapid guideline: cystic fibrosis. Available from: nice.org.uk/guidance/ng170. All rights reserved. Subject to Notice of rights.

NICE guidance is prepared for the National Health Service in England. All NICE guidance is subject to regular review and may be updated or withdrawn. NICE accepts no responsibility for the use of its content in this product/publication.

Published date: 09 April 2020.