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Published on 21 April 2020

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COVID-19 rapid guideline summary: cystic fibrosis

The purpose of this guideline (NG170) is to maximise the safety of patients with cystic fibrosis during the COVID-19 pandemic, to protect staff from infection and to enable services to make the best use of available resources.

Patient communication and minimising infection risk
NG170 discusses the importance of communicating with patients, their families and carers to support their mental wellbeing during the COVID-19 pandemic to alleviate any anxieties or concerns they might have about the virus. NG170 suggests signposting patients to the Cystic Fibrosis Trust which has information for patients on the virus as well as the NHS guidance on mental health and wellbeing aspects of COVID-19. In addition, NG170 reminds health professionals how some patients, families or carers might also need specialist psychological or social support during the current pandemic.

It is recommended that health professionals minimise face-to-face contact for consultations and make use of either via telephone or video instead and to contact patients via email or text messaging if possible. Health professionals should explore the provision of equipment for home spirometry testing and if appropriate weighing scales and facilities to collect samples remotely. However, NG170 advises that routine childhood vaccinations should continue at GP surgeries. NG170 also suggest using electronic rather than paper prescriptions need to explore alternative delivery routes for prescriptions and medicines such as pharmacy delivery services, via the post, using NHS volunteers or having drive-through pick-up points for medicines.

Patients or family members and carers should contact their cystic fibrosis team if they suspect that the patient has COVID-19 symptoms to ensure that they are appropriately assessed. Alternatively they must contact the NHS111 online coronavirus service but if seriously ill to contact emergency services. NG170 also reminds staff that pulmonary disease exacerbations may be difficult to differentiate at the initial presentation.

Patients not known to have COVID-19
NG170 stresses the need for patients and their families or carers need to follow the government advice on shielding and social distancing stringently but where this is not possible, for instance, they have a caring role, to adopt shielding measures.

Where a face-to-face appointment is deemed necessary, health professionals need to ensure appropriate measures are in place to prevent cross-infection and follow the NICE guideline on cystic fibrosis. In addition, patients are advised to attend alone or with no more than one family member and to avoid public transport if possible.

Patients known or suspected of having COVID-19
In cases where a patient has symptoms of COVID-19 on first presentation, NG170 advises health professional staff to follow the government guidance on investigation and initial clinical management of possible cases which provides information on testing and isolating patients.

Additionally, all healthcare staff having contact with patients either known or suspected of being infected, should follow the UK government guidance on infection prevention and control. Teams are advised to report known or suspected cases of COVID-19 to the UK cystic fibrosis registry.

Due to the risk of spreading the virus, NG170 recommends that infected patients carry out airway clearance in well-ventilated rooms, away from other family members as the sputum generated is a potentially infectious aerosol. Other family members should avoid entering the room until enough time has passed for the aerosol to clear. NG170 suggests that teams reassure patients that nebulisers are safe to use because the nebulised fluid does not contain virus particles from the patient. Furthermore, family members or carers supporting patients with equipment such as masks, need to use appropriate hand hygiene.

Treatment and care planning
NG170 advises patients and their families or carers to continue with all the usual self-care measures such as airway clearance, prophylactic treatments, cystic fibrosis transmembrane conductance regulator therapies (CFTR) and home exercise. This will include any advice on the course of action for an exacerbation. Treatments should be continued to be prescribed for no more than 30 days and clinical teams need to be aware that cystic fibrosis patients may be at a greater risk of rapid deterioration if they contract COVID-19.

Modification to usual care
NG170 recommends that specialist cystic fibrosis centres maintain contact with network and outreach clinics via telephone or video consultations and that centres maintain a sufficient number of inpatient beds for those requiring urgent hospital admission. It advises that day-case facilities may still be required for administration of first doses of intravenous antibiotics and for flushing totally implantable intravenous devices although the flushing frequency can be reduced. Finally, it is necessary to ensure that teams retain sufficient clinical expertise and capacity to support the care and needs of patients as safely as possible and wherever practicable at home.

When reviewing patient care plans, NG170 advises teams to consider patients on a case-by-case basis and to involve the multidisciplinary team in any decision-making and that any changes are recorded and fully communicated with patients, family members or carers. Hospital lung function testing can be done only where the results have a direct impact on patient care and make greater use of home spirometry.

Patients can still access CFTR therapies under the NHS England policy for these drugs. NG170 reminds teams that the data collection agreement has a minimum requirement of annual assessments and that liver function test and eye monitoring are only required when clinically indicated. Data reporting by staff (for example, cystic fibrosis quality dashboard quarterly returns) can be reduced provided the deadline of 31 January 2021 is still met. It is also recommended that the transition of young people is deferred until the pressures of the COVID-19 pandemic have passed.

Healthcare workers
NG170 suggests that all NHS staff involved in all aspects of patient care (including receiving, assessing and caring) who have known or suspected COVID-19 infection or who live in a household where there is another who is known or suspected to have COVID-19, should follow the government guidance on self-isolation. If self-isolating are still able to work, suggested roles include undertaking telephone or video consultations and participation in virtual multidisciplinary meetings. These staff can also help identify patients who can be monitored remotely and those who might require more support as well as performing any routine tasks which can be done remotely.

NG169 is available online and interested readers should check this version for further updates.



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