Early and detailed characterisation of coronavirus disease 2019 (COVID-19) has emerged
principally through publications from China, where the disease was first identified.
As the pandemic spread to Lombardia, Northern Italy and then globally,
evaluating the impact on people with cystic fibrosis has become imperative, because
the prevalence of this inherited condition is much higher in populations derived from
Europe than in other populations.
Since the beginning of the outbreak in Lombardia, severe acute respiratory syndrome
coronavirus 2 (SARS-CoV-2) infections have been reported in ten patients with cystic
fibrosis out of 42 161 people in the region known to have been infected. The number
of infected patients in Lombardia make up a substantial proportion of the 101 739
total SARS-CoV-2 infections in Italy (according to available data on March 31, 2020).
The Italian patients with cystic fibrosis were resident in endemic areas and acquired
the infection from family members. These observations, as well as data from other
European countries (five patients with cystic fibrosis in France have been reported
to have SARS-CoV-2 infection, seven in the UK, five in Germany and three [one transplanted]
in Spain) suggest few patients with cystic fibrosis, mainly adults, are becoming infected
with SARS-CoV-19, without apparent effect on cystic fibrosis disease severity. From
these data, it is not possible to identify factors that might be protective, for example
use of long-term antibiotic therapy such as azithromycin.
The few reported cases of SARS-CoV-2 infection in people with cystic fibrosis might
reflect the efforts of families across Europe to minimise social contacts. This effort
might be considered a success, but there is no room for complacency and the directive
for social shielding of both the patient and family members remains clear and important.
More data should be collected to better characterise the impact of COVID-19 on patients
with cystic fibrosis. The European Cystic Fibrosis Society (ECFS) is supporting the
collection and timely sharing of data from across Europe. The ECFS Patient Registry,
in cooperation with national cystic fibrosis registries, has established a data collection
and reporting system, contributing to an international harmonised dataset, to identify
factors that predict severity of COVID-19.
Viral respiratory tract infections are more severe in patients with cystic fibrosis
than in the general population, with an increased risk of complications and a negative
impact on lung function. During the 2009 influenza pandemic, H1N1 virus caused substantial
morbidity in patients with cystic fibrosis, and in a subgroup with severe lung disease,
H1N1 infection was associated with respiratory deterioration, mechanical ventilation,
and even death.4, 5
The situation in Italy has been monitored carefully to inform management strategies
and provide clear advice for patients with cystic fibrosis and their families. Consistent
with the Italian Government, the Cystic Fibrosis Center in Milan promptly recommended
self-isolation for these patients. Recommendations for preventive measures that were
already established in this population, such as using face masks and practising adequate
hand hygiene, were reinforced. The cystic fibrosis team in Milan cancelled routine
clinic appointments to prevent unnecessary hospital visits and viral spread. Procedures
such as respiratory function testing and bronchoscopy were put on hold. Phone calls
and email contacts were used to monitor the clinical condition of patients and to
provide psychological support immediately after the lockdown.
These measures have subsequently been adopted by cystic fibrosis centres across Europe.
There is no doubt that cancelling routine cystic fibrosis clinics and monitoring according
to ECFS standards of care
will have a negative impact on the wellbeing of patients with cystic fibrosis over
time. It is important that patients and their families are provided with tools to
support self-monitoring during this period. This self-monitoring might include transmission
to clinicians of spirometry and oxygen saturation data that are recorded at home.
Support should also include the capacity to carry out respiratory culture at home
and send to the laboratory securely.
Home visits by health-care professionals present a risk of SARS-CoV-2 transmission
and virtual clinics should be used to provide families with advice on all aspects
of cystic fibrosis care management, including airway clearance and maintaining exercise.
In case cystic fibrosis team members are seconded to work on the front line of tackling
this pandemic, it is imperative that lines of communication remain open and that patients
with cystic fibrosis do not feel isolated. For patients with more severe cystic fibrosis,
access to lung transplantation might be compromised.
People with cystic fibrosis have a phenotypic spectrum ranging from excellent respiratory
health to chronic airways disease with productive cough and respiratory compromise.
The clinical features of COVID-19 (dry cough, myalgia, and fever) are quite distinct
from the symptoms of cystic fibrosis. Therefore, most COVID-19 in people with cystic
fibrosis should be recognisable, but it is possible that mild disease might be labelled
as within the normal spectrum of symptoms for that individual. A low threshold for
testing is therefore needed in this population.
Many isolated families are concerned about collection of medicines and food during
this pandemic. Most countries have established systems to ensure deliveries to people
who are isolated, often using volunteers, but as the pandemic progresses, maintaining
these systems will be a priority for patients with cystic fibrosis and their families.
A further concern is the halt to the development of new therapies for cystic fibrosis.
No new clinical trials are being initiated in Europe and recruitment of patients to
ongoing trials is being stopped. Patients in existing trials are being supported by
reducing study visits with the agreement of regulatory bodies such as the European
Medicines Agency and by providing study medication to the home.
As national advice changes rapidly to respond to the pandemic, it is important that
cystic fibrosis organisations place this advice in context and present information
for patients and their families that is based on clear evidence and not conjecture.
This pandemic is imposing an extraordinary level of stress on health services and
the consequences include the trauma of witnessing so many deaths, even of young and
healthy people, and the impact on the whole community of fear, isolation, and uncertainty
for the future. Following the situation in Italy, a community-oriented plan for the
next pandemic is clearly needed.
People with cystic fibrosis and their families have invested considerable time and
energy to maintain good health and, now, on the cusp of remarkable new therapies to
transform their condition, they face a global pandemic, the effect of which is unclear.
Early data suggest that most patients with cystic fibrosis are doing an exceptional
job avoiding SARS-CoV-19 infection, but they must remain dedicated to this task, as
data are gathered from across Europe to better understand factors that affect the
severity of COVID-19 in people with cystic fibrosis.
For information from Italy on COVID-19 see https://www.salute.gov.it/nuovocoronavirus