Overview

The purpose of this guideline is to maximise the safety of patients with cystic fibrosis and make the best use of NHS resources, while protecting staff from infection.

This guideline focuses on what you need to stop or start doing during the pandemic. Follow the usual professional guidelines, standards and laws (including those on equalities, safeguarding, communication and mental capacity), as described in making decisions using NICE guidelines.

On 7 October 2020, we withdrew our recommendations on reducing or deprioritising cystic fibrosis registry data entry, limiting transplant services and deferring transition to adult services because these emergency measures are no longer needed.

This guideline is for:

• health and care practitioners
• health and care staff involved in planning and delivering services
• commissioners

The recommendations bring together

• existing national and international guidance and policies
• advice from specialists working in the NHS from across the UK. These include people with expertise and experience of treating patients with cystic fibrosis during the current COVID-19 pandemic.

NICE has also produced a COVID-19 rapid guideline on arranging planned care in hospitals and diagnostic services, which should be read alongside this guideline.

We developed this guideline using the interim process and methods for developing rapid guidelines on COVID-19 in response to the rapidly evolving situation. We will review and update the recommendations as the knowledge base develops using the interim process and methods for guidelines developed in response to health and social care emergencies.

1. Communicating with patients and minimising risk

1.1.

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.

1.2.

Be aware that some patients, families or carers may need specialist psychological or social work support in the context of COVID-19.

1.3.

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.

However, note that routine childhood vaccinations should continue to take place at the GP surgery.

1.4.

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.

1.5.

Be aware that symptoms of COVID-19 and pulmonary disease exacerbations may be difficult to differentiate at initial presentation.

2. Treatment and care planning

2.1.

Tell patients, their families and carers to continue with all their usual self-care arrangements including, for example:

• airway clearance techniques
• prophylactic medication, including oral and inhaled antibiotics, and mucoactive agents
• cystic fibrosis transmembrane conductance regulator (CFTR) therapies
• diet, vitamins and pancreatic enzyme replacement therapy
• home exercise (following UK government guidance on shielding and protecting people defined on medical grounds as extremely vulnerable from COVID-19).

2.2.

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.

2.3.

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.

2.4.

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.

2.5.

Monitor patients with cystic fibrosis closely, because they may be at greater risk of rapid deterioration if they contract COVID-19.

3. Modifications to usual care and service delivery

3.1.

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).

3.2.

Specialist cystic fibrosis centres should maintain contact with network and outreach cystic fibrosis clinics using telephone or video consultations and meetings.

3.3.

Specialist cystic fibrosis centres should maintain sufficient inpatient beds and facilities for those patients for whom a hospital admission is essential.

3.4.

Specialist cystic fibrosis centres should maintain sufficient access to day-case facilities 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. [amended 7 October 2020]

3.5.

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.

3.6.

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.

3.7.

Discuss the risks and benefits of changing treatment plans with patients, their families and carers.

3.8.

Only carry out lung function tests in hospital if the results will have a direct impact on patient care. Use home spirometry if possible.

3.9.

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.

4. Healthcare workers

4.1.

Healthcare workers with known or suspected COVID-19, or who live in a household in which another person is known or suspected to have COVID-19, should self-isolate and only return to work in accordance with UK government guidance on self-isolation for households with possible infection.

4.2.

If a healthcare professional needs to self-isolate, ensure that they can continue to help if they are well enough to do so by:

• enabling telephone or video consultations and multidisciplinary team meetings
• identifying patients who are suitable for remote monitoring and follow up, and those who are vulnerable and need support
• carrying out tasks that can be done remotely, for example, updating the cystic fibrosis registry.

4.3.

Cystic fibrosis teams should keep in touch with staff who are self-isolating, to support their mental wellbeing.

4.4.

Provide all staff with visible leadership and supportive messaging, to maintain morale.

4.5.

Take account of the information on the NHS Employers website about good partnership working and issues to consider when developing local plans to combat COVID-19.

Update information

7 October 2020: We withdrew our recommendations on reducing or deprioritising cystic fibrosis registry data entry, limiting transplant services and deferring transition to adult services (recommendations 3.10 to 3.12) because these emergency measures are no longer needed.

We also updated recommendation 1.6 to bring it in line with the latest shielding advice and added a link to Royal College of Paediatrics and Child Health guidance. In recommendation 3.4 we clarified the need for day-case facilities.

Your responsibility: The recommendations in this guideline represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, professionals and practitioners are expected to take this guideline fully into account, alongside the individual needs, preferences and values of their patients or the people using their service. It is not mandatory to apply the recommendations, and the guideline does not override the responsibility to make decisions appropriate to the circumstances of the individual, in consultation with them and their families and carers or guardian.

Local commissioners and providers of healthcare have a responsibility to enable the guideline to be applied when individual professionals and people using services wish to use it. They should do so in the context of local and national priorities for funding and developing services, and in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities. Nothing in this guideline should be interpreted in a way that would be inconsistent with complying with those duties.

Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.