COVID-19 in People With Primary Ciliary Dyskinesia

Background: The current pandemic of Coronavirus disease 2019 (COVID-19) caused by
SARS-CoV-2 virus is a public health emergency of international concern. The severity of
COVID-19 ranges from asymptomatic to severe. Patients with chronic lung disease such as
primary ciliary dyskinesia (PCD) are thought to be at increased risk, but no data support
this. Disease course and optimal treatment strategies might differ depending on the
underlying disease. The lack of knowledge hampers optimal treatment, and it leads to
insecurity and fear among persons affected by PCD and their family. PCD patient
organisations initiated the idea of a survey that gathers essential knowledge on effects
of COVID-19 on people with PCD and makes it available to patients, physicians, and policy
makers in real time.

Aims: To set up an online surveillance system of COVID-19 for people with PCD. For people
with PCD, the study aims to:

1. Describe incidence of COVID-19, symptoms, course of disease, duration of illness,
treatments, and outcomes;

2. Identify risk factors for prognosis for a severe disease course;

3. Assess the experiences of patients, and their wishes and needs such as availability
of preventive measures, medicines, therapies, and official communication and
government policies in relation to the COVID-19 outbreak.

Methods: The study population includes all patients with PCD who can be contacted via
patient organisations. Local organisations invite their contacts using social media and
mailing lists. People with PCD can participate via a link on the study website
(www.covid19pcd.ispm.ch), where they can read the study information and give consent.
Once registered, participants receive a link via e-mail to the baseline questionnaire
with questions on prior COVID-19, PCD diagnosis and treatment, and environment and living
conditions. 7 days after completing the baseline questionnaire, and in regular intervals
thereafter, participants receive a link to a short follow-up questionnaire with questions
on COVID-19, current symptoms, and contact behaviour. Throughout the study, occasional
questionnaires are sent out focusing on emerging questions of interest such as official
information and policy issues, availability of treatments, or anxieties and fears.
Occasional questionnaires can also focus on topics relevant for people with PCD that are
not related to COVID-19. In case of hospitalisation, patients or family members are asked
to obtain a hospital report. Results are continuously analysed and summaries are
published on the study website. Specific analyses are possible on request to inform
involved persons, physicians, and policymakers.

This study uses an innovative approach to assess information relevant for risk, disease
course, treatments, and outcomes of COVID-19 directly from persons with a rare lung
disease and make the information available in real time. It will inform and empower
patients and be a resource to relieve anxiety and provide hands-on evidence to physicians
and policymakers. It adds no burden to health care professionals and will provide people
with PCD and their families specialised information, which they can pass on to their
physicians.

Current status

By October 2024, 771 people with PCD from 49 countries registered to participate in
COVID-PCD. Results from the study, publications, and plain language summaries are listed
on the study website (https://covid19pcd.ispm.ch/en/publications/)