Caregivers, Patients With Cystic Fibrosis, Primary Ciliary Dyskinesia at Elevated Risk of Mental Illness

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There was an increase in depression in the cystic fibrosis group, as well as an increase in anxiety in cystic fibrosis caregivers during the pandemic.

A new study based in the Netherlands show patients with cystic fibrosis or primary ciliary dyskinesia, as well as their caregivers, were at an increased risk of developing mental illnesses like depression or anxiety during the ongoing COVID-19 pandemic.1

A team, led by Lieke Noij, MD, Department of Pediatric Pulmonology, Emma Children's Hospital, Amsterdam UMC, evaluated the impact of the pandemic on depression, anxiety, and resilience in patients with cystic fibrosis or primary ciliary dyskinesia and their caregivers in the Netherlands.

The Pandemic

The COVID-19 pandemic and some of the associated byproducts such as social isolation and stress, has made a negative impact on the collective mental health of the population. This is particularly true for patients with cystic fibrosis or primary ciliary dyskinesia, a rare hereditary syndrome that results in ciliary dysfunction and leads to neonatal respiratory distress, chronic wet cough, and recurrent airway infections.

In the quantitative, longitudinal, questionnaire screening study, the investigators examined adolescent patients aged 12-17 years and caregivers of children aged 0-17 years with cystic fibrosis, as well as adolescents, adults, and caregivers of children with primary ciliary dyskinesia. Each participant completed a questionnaire on depression, anxiety, and resilience between September 2020 and February 2021, the second wave of the COVID-19 pandemic.

The majority of participants previously were part of a pre-existing cohort between January 2019 and March 2020 with an annual mental health screening program for depression and anxiety, following the International Committee on Mental Health in Cystic Fibrosis guidelines.

The investigators measured the psychosocial impact of COVID-19 using the Exposure and Family Impact Survey (CEFIS) Part 2 and compared depression and anxiety results to participant prepandemic scores using a mixed model analyses. The team also collected demographic factors, including information on gender, marital status, region of residence, and level of education for all participants.

Finally, each responder was asked if they had received psychological support in the previous year, are currently receiving psychological support, and whether they feel the need for psychological support.


The study included 110 patients, 10 with cystic fibrosis, 31 with primary ciliary dyskinesia, 52 cystic fibrosis caregivers, and 17 primary ciliary dyskinesia caregivers. However, while all 110 completed the questionnaires, only 87 participants had prepandemic outcome data available.

The results show the prevalence of symptoms of depression and anxiety in patients and their caregivers was high both before and during the pandemic.

There was an increase in depression in the cystic fibrosis group (PHQ-9, 2.75; 95% confidence interval [CI], 0.82-4.68), as well as an increase in anxiety in cystic fibrosis caregivers (GAD-7 scores ≥5, 1.03; 95% CI, 0.09-1.96) during the pandemic.

However, resilience was within the normal range for all of the groups and CEFIS scores showed a low to normal impact.

“[Patients with cystic fibrosis] and [patients with primary ciliary dyskinesia] and their caregivers were at risk of elevated depression and anxiety symptoms both before and during the pandemic, which emphasizes the importance of mental health screening and psychological care in cystic fibrosis and primary ciliary dyskinesia],” the authors wrote.