Developmental and behavioral problems in preschool-aged primary ciliary dyskinesia patients

Akkuş, Pınar; Hızal, Mina Gharibzadeh; Bahadur, Evin İlter; Özmert, Elif Nursel; Polat, Sanem Eryılmaz; Özdemir, Gökçenur; Karahan, Sevilay; Yalçın, Emine; Doğru, Deniz; Ki̇per, Nural; Özçeli̇k, Uğur

doi:10.1007/s00431-019-03382-z

Cited by 13 publications

(5 citation statements)

References 32 publications

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“…Research on chronic pediatric disease indicates a greater presence of clinical anxietydepression in these patients compared to their healthy peers [12][13][14][15] more behavioural problems [15][16][17] and a worse perceived psychological well-being [15,18]. In addition, these patients appear to report lower self-esteem [19][20][21].…”

Section: Introductionmentioning

confidence: 94%

Primary ciliary dyskinesia and psychological well-being in adolescence

et al. 2020

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Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disease with low prevalence in pediatrics. Health studies have not sufficiently analyzed the role of psychological variables in rare diseases such as PCD. This paper studies the psychological characteristics of a group of pediatric patients diagnosed with PCD compared to their healthy peers. The sample consisted of 48 preadolescents-adolescents, aged 9-18 years (M = 12.96; SD = 2.71), with similar distribution by sex, and 25% of the patients having dyskinesia. Clinical anxiety-depression, self-esteem and psychological well-being were evaluated using questionnaires: the Adolescent Psychological Well-being Scale (BIEPS-J), the Hospital Anxiety and Depression Scale (HADS) and the Rosenberg Self-Esteem Scale (RSE). Data were analysed using descriptive, mean comparison (t-test) and diffuse comparative qualitative analysis (QCA). The results show no differences were found between healthy and PCD patients in the variables analyzed, except for social ties showing the latter greater well-being in this aspect. In QCA models, the variables that best explained the high or low levels of well-being were depression and selfesteem, and primary ciliary dyskinesia was not a necessary condition for presenting low levels of well-being. In conclusion, our results highlight the need to explore psychological aspects in pediatric patients with rare diseases.

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Section: Introductionmentioning

confidence: 94%

Primary ciliary dyskinesia and psychological well-being in adolescence

et al. 2020

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“…(Arikan et al, 2015) Furthermore, a study by Zengin Akkus et al (2019) reported delay in fine motor skills, based on the Ages and Stages Questionnaire, when comparing pre-school children with CF to healthy controls. (Zengin Akkus et al, 2019) Conversely, Gruber et al (2010) reported that pre-school children with CF performed better at motor skills than healthy peers. (Gruber, Orenstein, Paul, Huls, & Braumann, 2010) Although a significant relation between pulmonary function and physical activity has been found in children with CF, (Schneiderman et al, 2014) no studies have investigated the effect on motor delay in this population.…”

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confidence: 99%

Motor Performance in South African Children with Cystic Fibrosis

Corten

Morrow

2019

Physical & Occupational Therapy In Pediatrics

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Aims: This study aimed to investigate motor performance in children with cystic fibrosis (CF) and the relationship with respiratory and anthropometric outcome measures. Methods: A cross-sectional exploratory study investigated 12 children with CF, mean (SD) age 6.17 (0.67) years, using the Movement Assessment Battery for Children 2 nd edition (MABC-2), spirometry, body weight, height, body mass index and age related anthropomorphic z-scores. Results: MABC-2 total scores indicated 9/12 (75%) children performed below average (<50 th percentile), of which 4/12 children (33.3%) had motor delay and 2/12 (16.7%) were at risk for motor delay. The balance subscale showed the lowest scores, with 5/12 (41.7%) participants performing at or below the 5 th percentile and a median (IQR) percentile score of 9.00 (5.00-62.50). A significant negative correlation was found between the manual dexterity subscale and both height and height for age z-scores (p=0.017 and p=0.019 respectively), as well as peak expiratory flow in liter (p=0.027). The balance subscale scores were positively correlated with forced expiratory volume in %predicted (p=0.048). No other significant correlations were found. Conclusion: Children with CF may be at risk for delayed motor development, particularly their balance skills. Poor lung function might affect motor development but further research is recommended.

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“…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][26][27] .…”

Section: Discussionmentioning

confidence: 99%

Is Quarantine for COVID-19 Pandemic Associated with Psychological Burden in Primary Ciliary Dyskinesia? A Prospective Study.

Riccio

Borrelli

Fioretti

et al. 2020

Preprint

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Background: Primary ciliary dyskinesia (PCD) is a rare disorder with variable disease progression. Information on the psychological impact of the Coronavirus Disease 2019 (COVID-19) quarantine in PCD, a condition with characteristics of personal and familiar increased stress due to frequent pulmonary exacerbations and high risk of lung function deterioration, is lacking. We designed a prospective study to assess the psychological burden and parental stress during the COVID-19 lockdown in Italy in a PCD population.Methods: Questionnaires assessing psychological well-being were administered to 10 PCD patients <15 years-old’s mothers (Group A: Parenting Stress Index-Short Form), to 17 PCD patients >=15 years-old (Group B: Psychological General Well-Being Index) and to 27 controls. We compared the PCD pulmonary exacerbations from the beginning of the outbreak and the frequency of weekly chest physiotherapy sessions during quarantine to the same period in 2019. Results: Seventy percent of Group A cases did not present parental stress levels. Ninety percent of Group B subjects did not show scores indicating distress. Groups A and B showed no significant difference versus controls in parental stress and psychological well-being, respectively. In the whole PCD population pulmonary exacerbations from the beginning of outbreak were less than in the same period of 2019 (p<0.05), and weekly chest physiotherapy sessions significantly increased during the quarantine compared to the same period of 2019 (p<0.05).Conclusions: We found that during COVID-19 quarantine, a PCD population in Italy neither developed psychological distress nor had a psychological burden significantly different than controls. We speculate that the low pulmonary exacerbation rate, explained by lower exposure to infectious triggers or by improved compliance to weekly chest physiotherapy, likely contributes to the psychological well-being of PCD patients and their families. The evaluation of the psychological burden and parental stress is a valuable tool for measuring the emotional impact of PCD and ultimately improving the medical care to PCD patients and their families, especially when exceptional events such as a pandemic occur.

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Developmental and behavioral problems in preschool-aged primary ciliary dyskinesia patients

Cited by 13 publications

References 32 publications

Primary ciliary dyskinesia and psychological well-being in adolescence

Primary ciliary dyskinesia and psychological well-being in adolescence

Motor Performance in South African Children with Cystic Fibrosis

Is Quarantine for COVID-19 Pandemic Associated with Psychological Burden in Primary Ciliary Dyskinesia? A Prospective Study.

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