Project Description

To whom it may concern,

We are aware that in Europe there is a wide variation between countries in vaccine prioritization for SARS-CoV-2 in fragile individuals. Patients affected by Primary Ciliary Dyskinesia (PCD) are eligible to be vaccinated in some countries and not in others. PCD is a rare, genetic, severe multi-organ disease, characterized by impaired mucociliary clearance due to ineffective airway cilia motility. The choice of management strategy for PCD patients, in case of lack of evidence based guidelines, is based on expert consensus and is often extrapolated from cystic fibrosis (CF) for similarities on respiratory complications. Evidence of SARS-CoV-2 infection in patients affected by CF showed that COVID-19 viral infection increased the risk of respiratory complications especially in patients with advanced lung disease.1People with CF aged under 18 have also been shown to be at risk of hospitalization from SARS-CoV-2 infection.2Moreover, the medium and long-term impact of COVID-19 amongst infected patients is still not known. Therefore, the ‘Global Registry Harmonization Group’ (GRHG) for CF stated that people with CF need aggressive vaccine distribution strategies. In a similar way to CF patients, individuals with PCD suffer from recurrent respiratory infections and persistent viscid bronchial secretions, leading to irreversible lung damage that may progress to bronchiectasis and respiratory failure.3Similarly to other rare diseases, data on COVID-19 infection are scarce. The only available study, based on a longitudinal online survey, showed a low incidence rate of COVID-19 infection in subjects affected by PCD and overall severity was moderate.4However, results can be explained by the careful shielding behaviour of PCD patients, with half of the participants reporting that they were not attending school or work , which may also explain why PCD patients had less pulmonary exacerbations during the COVID-19 quarantine.5Moreover, the PCD patients have less recognized treatment options than other rare diseases, which is a worrisome factor in case of COVID-19 infection.6Therefore, we strongly believe that PCD patients must be considered a fragile population at higher risk of hospitalization in case of COVID-19 infection.

We are aware that some countries were able to get ahead in delivery of COVID-19 vaccines but global equitable access should be a priority for all age groups. Nevertheless, the disparity in prioritization for people with PCD across the globe is a real concern.

In quality of ERN-LUNG PCD Core members, we believe that patients with PCD need a global prioritization of vaccine distribution. Moreover, strategies should be clearly stated so that patients know how, when and where they can receive vaccination.

Heymut Omran

On behalf of ERN-LUNG PCD Core members



  1. McClenaghan E, Cosgriff R, Brownlee K, Ahern S, Burgel PR, Byrnes CA, Colombo C, Corvol H, Cheng SY, Daneau G, Elbert A, Faro A, Goss CH, Gulmans V, Gutierrez H, de Monestrol I, Jung A, Justus LN, Kashirskaya N, Marshall BC, McKone E, Middleton PG, Mondejar-Lopez P, Pastor-Vivero MD, Padoan R, Rizvi S, Ruseckaite R, Salvatore M, Stephenson AL, Filho LVRDS, Melo J, Zampoli M, Carr SB; Global Registry Harmonization Group. The global impact of SARS-CoV-2 in 181 people with cystic fibrosis.J Cyst Fibros. 2020 Nov;19(6):868-871.
  2. Bain R, Cosgriff R, Zampoli M, Elbert A, Burgel PR, Carr SB, Castaños C, Colombo C, Corvol H, Faro A, Goss CH, Gutierrez H, Jung A, Kashirskaya N, Marshall BC, Melo J, Mondejar-Lopez P, de Monestrol I, Naehrlich L, Padoan R, Pastor-Vivero MD, Rizvi S, Salvatore M, Filho LVRFDS, Brownlee KG, Haq IJ, Brodlie M. Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study.J Cyst Fibros. 2021 Jan;20(1):25-30.
  3. Halbeisen FS, Goutaki M, Spycher BD, Amirav I, Behan L, Boon M, Hogg C, Casaulta C,  Crowley S, Haarman EG, Karadag B, Koerner-Rettberg C, Loebinger MR, Mazurek H, Morgan L, Nielsen KG, Omran H, Santamaria F, Schwerk N, Thouvenin G, Yiallouros P, Lucas JS, Latzin P, Kuehni CE. Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study.Eur Respir J. 2018 Aug 23;52(2):1801040
  4. Pedersen ESL, Goutaki M, Harris AL, Dixon L, Manion M, Rindlisbacher B; COVID-PCD patient advisory group, Lucas JS, Kuehni CE. SARS-CoV-2 infections in people with PCD: neither frequent, nor particularly severe.Eur Respir J. 2021 Apr 8:2004548.
  5. Riccio MP, Borrelli M, Fioretti MT, Del Bene M, Bravaccio C, Poeta M, Santamaria F. Is Quarantine for COVID-19 Pandemic Associated with Psychological Burden in Primary Ciliary Dyskinesia?Int J Environ Res Public Health 2020 Nov 3;17(21):8099.
  6. Crowley S, Azevedo I, Boon M, Bush A, Eber E, Haarman E, Karadag B, Kötz K, Leigh M, Moreno-Galdó A, Mussaffi H, Nielsen KG, Omran H, Papon JF, Pohunek P, Priftis K, Rindlisbacher B, Santamaria F, Valiulis A, Witt M, Yiallouros P, Zivkovic Z, Kuehni CE, Lucas JS. Access to medicines for rare diseases: beating the drum for primary ciliary dyskinesia. ERJ Open Res. 2020 Sep 14;6(3):00377-2020.