To our knowledge, this is the first prospective study on the levels of psychological burden in a PCD population compared to healthy subjects during the imposed lockdown period for COVID-19 in Italy. This study was designed starting from the assumption that PCD is a chronic disorder with potential psychological effects also on the intra-familiar relationships because of patients’ frequent need of medical consultations, their own perception of being sick, and the possible effects of denial and rejection of the disease by patients and/or parents. Our main finding is that, overall, during the COVID-19 quarantine, PCD patients did not show scores indicating distress, and only some of them reported more difficulties related to self-control and general health. Among PCD parents, only 20% had high levels of stress. Finally, when compared to healthy subjects, the current PCD population did not show significantly different psychological burden or parental stress level during the quarantine. Patients with PCD and their families, as well as controls, strictly observed the stringent preventive measures ordered by the Italian Government. We speculate that the quarantine, avoiding the risk of exposure to SARS-CoV-2, gave PCD patients a great sense of security. Despite the access to the healthcare service was fairly limited, the telemedicine facility at our hospital warranted a continuous medical surveillance through the remote contact.
Some data from the current study deserve a comment. The finding of less PCD lung exacerbations during the COVID-19 quarantine period imposed in Italy, quite novel to the best of our knowledge, is not surprising, and might be likely explained by the strict adherence to the preventive measures for reducing viral transmission. These consisted of a) social distancing with avoidance behaviours; b) the adoption of the smart working model that allowed workers to work at home with a flexible time schedule; c) schools closure for limiting contacts with or among kids and their teachers; d) education to frequent handwashing; e) respiratory (especially, while coughing or sneezing) and environmental hygiene measures [22]. Since health care institutions are major foci of disease, and the nosocomial SARS-CoV-2 transmission is a concrete possibility, several hospitals, also including our, imposed to patients to limit the access to healthcare services during the quarantine unless urgent or serious problems were claimed by emails or telephone calls. The detection of respiratory pathogens in the PCD lower airways is associated with frequent exacerbations, and fuels the vicious cycle of infection, inflammation and lung damage that further increases bacterial growth [9]. Therefore, the absence of stress perception during the quarantine found in the vast majority of our study population might be related to less symptoms or signs of pulmonary exacerbation. As our patients during the COVID-19 quarantine period had a lot more free time, we speculate that the smart working model or school closure likely resulted in improved compliance to chest physiotherapy. This could represent an additional reason for explaining, at least in part, the reduced number of lung infections [23].
In PCD, retention of airways mucus and growth of biofilms caused by abnormal ciliary structure and/or function result in early recurrent airways infection. These are, indeed, a common issue also in non-PCD subjects [9]. The diagnostic delay and delayed start of PCD treatment may eventually result in patients’ uncertainty or anxiety about their disease outcome. This, combined with the need of continued care, may negatively impact on patients’ emotional status and place them and their families at risk for impaired psychosocial functioning. Research on well-being and psychological issues of PCD has focused on the emotional burden of the condition, including patients’ concern about current and future health [24]. Several studies have highlighted that PCD patients of any age are at risk of experiencing anxiety or depression, or reduced self-esteem, even though ultimately they may not present worse rates of psychological well-being than healthy peers [25–27].
The primary goal of public health measures for controlling a pandemic is to prevent person-to-person spread of the infection by separating people. Isolation is an unpleasant experience, and individuals report not only boredom or sense of frustration, but also feelings such as sadness, fear, or nervousness due to reduced social contacts and loss of daily tasks [28]. During disease outbreaks, anxiety can also rise following repeated media reporting on everyday rate of new cases or deaths. In addition to this, being unable to get regular medical care and prescriptions may be a matter of concern, especially for patients with chronic disorders [29]. In PCD patients and their families the uncertainty about the future, combined with the fear that a respiratory exacerbation or any additional medical problem could occur and not be addressed because hospital visits were blocked, might increase patients’ or parents’ level of anxiety. However, social distancing likely halted the transmission of all respiratory pathogens also including SARS-CoV-2, and this might have a beneficial effect on PCD patients and family well-being.
We chose to evaluate the stress levels of a parent of a PCD patient younger than 15 years rather than the patient him/herself. In fact, we believe that remote assessment of the psychological state in younger subjects could result unreliable because of the difficulties of self-monitoring and self-awareness at that age. Actually, PCD parents may exhibit a great emotional and stress load (27) which, conversely, during COVID-19 quarantine did not appear increased compared to healthy peers. Nevertheless, in the current PCD mothers’ group, the PSI-SF total score and the scores of other subscales appeared higher than the control group score, although the difference was not statistically significant. The data suggest that in Italy the emergency condition associated with COVID-19 increased the stress levels in the general population, reducing the gap with subjects with an underlying chronic condition such as PCD. Actually, greater care, greater hygiene and staying at home might represent a protective factor for people with chronic respiratory diseases, paradoxically affecting the levels of psychological well-being in their parents. Comparing the scores obtained in each subscale, we found that the percentage of subjects with clinically relevant scores of the “Difficult Child” subscale, was greater in the group of PCD mothers than in the control group. Moreover, 30% of PCD mothers had T-scores above 85th pc in the subscale relating to DC. The DC subscale represents the perception that a parent has of his/her own child, seen as a "difficult child" for any problematic behaviour. High scores on this scale indicate the presence of characteristics which may make it difficult for parents to play their role. This finding suggests that some of our mothers felt themselves more stressed during the quarantine period in the daily management of their kids when compared to healthy kids.
Also in the group of PCD patients older than 15 years, the evaluation of the psychological impact of the COVID-19 quarantine did not reveal clinically significant differences versus the control group. Nevertheless, when we compared the scores obtained in each subscale, we found that the percentage of subjects with clinically relevant scores associated to “Anxiety”, “Depression”, “Positivity and well-being” and “Vitality”, was greater in the control group than in PCD patients. Conversely, more PCD subjects showed clinically significant scores in the subscales related to “Self-control” and “General health”. These data indicate that in the healthy controls anxiety and depression levels were increased because of the concerns of the outbreak, with a consequent reduction in the perception of well-being and vitality, as recently reported [30]. In PCD patients, on the other hand, self-control was decreased, perhaps because of the fear of getting infected. The general sense of health might be perceived as more precarious, but stress levels, mood and perception of anxiety were stable.
Our study has strengths and limitations. First, we provided the novel information of the psychological effects of quarantining patients with PCD that was lacking in the literature. An additional strength is the availability of detailed medical records from a well-defined cohort of PCD patients. This allowed to compare the data collected during the COVID-19 lockdown period to the same period of the previous year. Moreover, we designed a prospective, observational study with a control group who adhered strictly to the isolation preventive measures as well as PCD patients. Yet, the study has some limitations. First, due to the unexpected nature of the COVID-19 outbreak we could not evaluate the psychological burden in the previous year and thus ignore the level of emotional stress in the pre-COVID-19 period both in PCD patients and controls. Second, the study population from a single-centre in Southern Italy was small. Actually, designing a study of a large and homogeneous population of patients with a rare condition like PCD is not easy. However, these findings should be hopefully confirmed by data from larger PCD population in countries with even different sociocultural background.