COVID-19 in People With Primary Ciliary Dyskinesia

The COVID-PCD is a participatory research project that aims to study how COVID-19 affects people with primary ciliary dyskinesia (PCD). The study is advertised through patient support groups and participants register online and answer a baseline questionnaire with details on PCD diagnosis, habitual symptoms, and COVID-19 episodes occurring before study entry. A short weekly follow-up questionnaire includes questions on incident SARS-CoV-2 infections, current symptoms, social contact behaviour, and physical activity. Occasionally, participants receive extra questionnaires focused on special topics. The study is hosted at the University of Bern and recruitment started on May 30th, 2020.

Study Overview

• Status: Recruiting
• Conditions: Primary Ciliary Dyskinesia

Detailed Description

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 2023, 759 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/)

• Study Type: Observational
• Enrollment (Estimated): 1000

Contacts and Locations

• Study Contact: Name: Claudia E Kuehni, Prof.; Phone Number: +41 31 684 35 07; Email: claudia.kuehni@unibe.ch
• Study Contact Backup: Name: Leonie D Schreck, MSc; Phone Number: +41 31 684 56 75; Email: Leonie.schreck@unibe.ch

Study Locations

• Switzerland
  • Bern, Switzerland, 3012
    • Recruiting
    • Institute of Social and Preventive Medicine (ISPM), University of Bern
    • Contact: Claudia E Kuehni, Prof.; Phone Number: +41 (0)31 684 35 07; Email: claudia.kuehni@ispm.unibe.ch
    • Contact: Leonie D Schreck, MSc.; Phone Number: +41 (0)31 684 56 75; Email: leonie.schreck@unibe.ch

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

The study includes all persons with primary ciliary dyskinesia who can be contacted via patient organisation networks worldwide.

Description

Inclusion Criteria:

• People of any age with reported suspected or confirmed Primary Ciliary Dyskinesia who gave consent to participate in the study

Exclusion Criteria:

• People who report not to have suspected or confirmed Primary Ciliary Dyskinesia

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
SARS-CoV-2	Number of people with PCD who report an infection with SARS-CoV-2	6 months
COVID-19	Number of people with PCD who report an infection with SARS-CoV-2 and report symptoms	6 months
COVID-19 hospitalisation	Number of people with PCD who report a hospital stay due to COVID-19	6 months
COVID-19 intensive care	Number of people with PCD who report a hospital stay due to COVID-19 and treatment in intensive care	6 months
COVID-19 fatal outcome	Number of people with PCD who reported an infection with SARS-CoV-2 and died	6 months

Collaborators and Investigators

• Sponsor: University of Bern
• Collaborators: University of Southampton; Selbsthilfegruppe Kartagener Syndrom und Primäre Ciliäre Dyskinesie; Verein Kartagener Syndrom und PCD; PCD Support UK; PCD Foundation; PCD Australia; European Lung Foundation; Associazione Italiana Discinesia Ciliare Primaria Sindrome di Kargagener APS; Asociación Nacional de Pacientes con Discinesia Ciliar Primaria; Association Dyskinésie Ciliaire Primitive ADCP; Universität Luzern
• Investigators: Principal Investigator: Claudia E Kuehni, Prof., University of Bern

Publications and helpful links

Helpful Links

• Study website

Study record dates

Study Major Dates

• Study Start (Actual): May 31, 2020
• Primary Completion (Estimated): May 31, 2030
• Study Completion (Estimated): May 31, 2030

Study Registration Dates

• First Submitted: October 23, 2020
• First Submitted That Met QC Criteria: October 23, 2020
• First Posted (Actual): October 26, 2020

Study Record Updates

• Last Update Posted (Actual): October 11, 2023
• Last Update Submitted That Met QC Criteria: October 9, 2023
• Last Verified: October 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Central Nervous System Diseases; Nervous System Diseases; Respiratory Tract Diseases; Neurologic Manifestations; Congenital Abnormalities; Genetic Diseases, Inborn; Otorhinolaryngologic Diseases; Movement Disorders; Abnormalities, Multiple; Ciliopathies; Dyskinesias; Ciliary Motility Disorders
• Other Study ID Numbers: COVID-PCD

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED
• IPD Plan Description: Researchers who wish to use data should submit a concept sheet describing the planned analysis and send it to Prof. Claudia Kuehni (Claudia.kuehni@unibe.ch). The concept sheet will be discussed with the patient advisory group and if agreed, a partial dataset will be prepared by the research team.

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No