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\begin{document}

\title{Psychological status of mothers of children with cystic fibrosis and
primary ciliary dyskinesia}



\author[1]{Tugba Ramasli Gursoy}%
\author[2]{Tugba Sismanlar Eyuboglu}%
\author[1]{Ayse Aslan}%
\author[3]{Nagehan Emiralioğlu}%
\author[4]{Sevgi Pekcan}%
\author[5]{Eda Buyukkaya}%
\author[6]{Melih Hangul}%
\author[7]{MEHMET KOSE}%
\author[1]{Sebnem Soysal}%
\author[1]{Işıl Budakoğlu}%
\affil[1]{Gazi Universitesi Tip Fakultesi}%
\affil[2]{Gazi University Faculty of Medicine}%
\affil[3]{Hacettepe University Faculty of Medicine}%
\affil[4]{Necmettin Erbakan University Meram Medical Faculty Hospital}%
\affil[5]{Necmettin Erbakan University Meram Faculty of Medicine}%
\affil[6]{Erciyes Üniversitesi Tıp Fakültesi}%
\affil[7]{Erciyes Universitesi Tip Fakultesi}%


\vspace{-1em}



  
  \date{June 14, 2024}


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\selectlanguage{english}
\begin{abstract}
Objectives: We aimed to investigate depression, burnout, attitude, and
burden of caregivers of children with cystic fibrosis (CF), and
especially caregivers of children with primary ciliary dyskinesia (PCD)
due to limited number of studies on this topic, and to compare them
according to their children's clinical status. Methods: Clinical
features and demographic data of children and their families were asked
to caregivers in four pediatric pulmonology centers. Beck Depression
Inventory (BDI), Maslach Burnout Inventory (MBI), Zarit Caregiver Burden
Scale (ZCBS), and Parental Attitude Research Instrument (PARI) were
administered to caregivers in both groups. Results were compared between
the two groups. Results: In total, 131 children with CF and 39 with PCD
and their caregivers were involved in study. All primary caregivers were
mothers in both groups. Depression, burnout, and burden scores of
mothers of children with CF were significantly higher than mothers of
children with PCD (p=0.017, p=0.024, p=0.038, respectively). Burnout was
higher in both CF and PCD groups with low family income (p=0.022,
p=0.034). Number of hospital visits in the previous six months was
correlated with burnout in both CF and PCD groups (r=0.207, p=0.034;
r=0.352, p=0.044). Conclusions: Although mothers of children with CF
have higher depression, burnout, burden, and negative attitudes towards
children than mothers of children with PCD, it is disregarded in mothers
of children with PCD. Psychological problems of mothers of children with
CF and PCD may increase with frequent hospital visits, hospitalizations,
low family income, number of children, and chronic disease in another
child.%
\end{abstract}%



\sloppy


\textbf{Psychological status of mothers of children with cystic fibrosis
and primary ciliary dyskinesia}

Tugba Ramasli Gursoy\textsuperscript{1}, MD, Tugba Sismanlar
Eyuboglu\textsuperscript{1},~MD, Ayse Tana Aslan\textsuperscript{1}, MD,
Nagehan Emiralioglu\textsuperscript{2}, MD, Sevgi
Pekcan\textsuperscript{3}, MD, Eda Zeynep Buyukkaya\textsuperscript{4},
MD, Melih Hangul\textsuperscript{5}, MD, Mehmet Kose\textsuperscript{5},
MD, Sebnem Soysal\textsuperscript{6}, PhD, Isil Irem
Budakoglu\textsuperscript{7}, MD

\textsuperscript{1}Department of Pediatric Pulmonology, Gazi University
Faculty of Medicine, Ankara, Turkey

\textsuperscript{2}Department of Pediatric Pulmonology, Gaziantep Cengiz
Gokcek Maternity and Children Hospital, Gaziantep, Turkey

\textsuperscript{3}Department of Pediatric Pulmonology, Necmettin
Erbakan University Meram Medicine Faculty, Konya, Turkey

\textsuperscript{4}Department of Pediatrics, Necmettin Erbakan
University Meram Medicine Faculty, Konya, Turkey

\textsuperscript{5}Department of Pediatric Pulmonology, Erciyes
University Faculty of Medicine, Kayseri, Turkey

\textsuperscript{6}Department of Pediatrics, Gazi University Faculty of
Medicine, Ankara, Turkey

\textsuperscript{7}Department of Medical Education, Gazi University
Faculty of Medicine, Ankara, Turkey

\textbf{*New institution informations of authors:}

Nagehan Emiralioglu: Department of Pediatric Pulmonology, Hacettepe
University Faculty of Medicine, Ankara, Turkey

Melih Hangul: Department of Pediatric Pulmonology, Gaziantep Cengiz
Gokcek Maternity and Children Hospital, Gaziantep, Turkey

\textbf{Funding/Support:} No funding was secured for this study.

This study was presented as poster presentation in 41th European Cystic
Fibrosis Conference 2018, Belgrade, Serbia.

\textbf{Correspondence} : Ayse Tana ASLAN, MD, Department of Pediatric
Pulmonology, Gazi University Faculty of Medicine, 06560, Besevler,
Ankara, Turkey. {[}aysetugbapp@gmail.com{]}, +903122026023.

\textbf{Key words:} children, cystic fibrosis, mothers, primary ciliary
dyskinesia, psychology

\textbf{Short title:} Psychological status of mothers of children

\textbf{Psychological status of mothers of children with cystic fibrosis
and primary ciliary dyskinesia}

\textbf{Objectives:} We aimed to investigate depression, burnout,
attitude, and burden of caregivers of children with cystic fibrosis
(CF), and especially caregivers of children with primary ciliary
dyskinesia (PCD) due to limited number of studies on this topic, and to
compare them according to their children's clinical status.

\textbf{Methods} : Clinical features and demographic data of children
and their families were asked to caregivers in four pediatric
pulmonology centers. Beck Depression Inventory (BDI), Maslach Burnout
Inventory (MBI), Zarit Caregiver Burden Scale (ZCBS), and Parental
Attitude Research Instrument (PARI) were administered to caregivers in
both groups. Results were compared between the two groups.

\textbf{Results} : In total, 131 children with CF and 39 with PCD and
their caregivers were involved in study. All primary caregivers were
mothers in both groups. Depression, burnout, and burden scores of
mothers of children with CF were significantly higher than mothers of
children with PCD (p=0.017, p=0.024, p=0.038, respectively). Burnout was
higher in both CF and PCD groups with low family income (p=0.022,
p=0.034). Number of hospital visits in the previous six months was
correlated with burnout in both CF and PCD groups (r=0.207, p=0.034;
r=0.352, p=0.044).

\textbf{Conclusions:} Although mothers of children with CF have higher
depression, burnout, burden, and negative attitudes towards children
than mothers of children with PCD, it is disregarded in mothers of
children with PCD. Psychological problems of mothers of children with CF
and PCD may increase with frequent hospital visits, hospitalizations,
low family income, number of children, and chronic disease in another
child.

\textbf{Introduction}

Chronic diseases in childhood affect the lives of the children as well
as their caregivers. Although caregiving is a normal part of being a
parent, the level of care needed by children with chronic diseases
increases the burden and stress on the caregiver and affects the
routines of family life and socialization.\textsuperscript{1}Caregiver's
psychological status, such as depressive symptoms, can also negatively
affect the child's condition and outcomes.\textsuperscript{2}

Cystic fibrosis and PCD have a significant impact on health and quality
of life of patients.\textsuperscript{3} The pathogenesis in both primary
ciliary dyskinesia (PCD) and cystic fibrosis (CF) includes impaired
mucociliary clearance leading to intermittent or chronic pulmonary
infections. Although PCD has similar characteristics to CF, lung disease
is usually milder than in CF.\textsuperscript{4} Patients with PCD have
been shown to have similar respiratory function and magnetic resonance
imaging findings as patients with mild CF.\textsuperscript{5} Patients
with PCD generally have a longer life span and better
prognosis.\textsuperscript{6} On the other hand, while CF can be
diagnosed before clinical findings begin with newborn screening,
patients with PCD can be diagnosed late with clinical
findings.\textsuperscript{7} The frequency of chronic respiratory
symptoms can potentially result in lifestyle restrictions in
PCD.\textsuperscript{8} The caregivers of children with CF experience
depression, unwillingness, dissatisfaction, and sometimes even negative
feelings for the child due to long-term care. These emotions cause
stress, which leads to physical, emotional, and social
exhaustion.\textsuperscript{9} As most of the morbidity and mortality in
CF is associated with respiratory infection and disease, clinical
severity of lung disease plays a significant role in determining the
caregiver's mood. The psychological status of caregivers of children
with CF affects their daily tasks and their children's compliance with
treatment regimens.\textsuperscript{10} In addition to the stress of
managing daily care, parents also have to deal with health system
issues.\textsuperscript{11} Family relationships and the psychological
functioning of the child with CF and family members may be affected.
Social relationships can also be difficult to maintain, given the
commitment to daily care and the uncertainty of the disease. Employment
issues can contribute to financial distress, which has far-reaching
effects on the functioning of the family
system.\textsuperscript{12}Although many studies are evaluating the
psychological status of caregivers of children with CF, studies on
caregivers of children with PCD are very limited.

The aim of this study was to evaluate the depression, burnout, caregiver
burden, and attitude of caregivers of children with CF, and especially
the caregivers of children with PCD due to the limited number of studies
on this topic, and the relationship between the clinical characteristics
of children, sociodemographic characteristics, and the psychological
status of their caregivers.

\textbf{Materials and Methods}

All children with CF and PCD and their primary caregivers who were
referred to four pediatric pulmonology centers and agreed to participate
in the study were recruited between July 2017 and September 2019.
Informed consent forms~were obtained from the~primary caregivers before
data collection. The study was performed according to the principles of
the Declaration of Helsinki and approved by the Ethics Committee of the
Faculty of Medicine (No. 2017/984; June 16, 2017). CF was diagnosed with
genetic analysis and/or sweat chloride test, and PCD was diagnosed with
genetic analysis and/or high-speed video microscopy and/or transmission
electron microscopic examination, and clinical findings.

Questionnaires regarding the children's clinical features and
demographic data about the children and their families were administered
to the caregivers. Information collected about the children included
age, sex, ethnicity, body mass index (BMI), pulmonary function tests
(PFT) results, history of sibling death, follow-up duration, number of
hospital visits (both emergency and outpatient clinics) and
hospitalizations in the previous 6 months, duration of hospitalizations
in the previous 6 months, and the number of exacerbations in the last
year. Information on the presence of bacterial colonization in the
sputum culture of children both during hospitalization or outpatient
clinic visits were obtained from their medical records. Colonization was
defined as bacterial growth in a sputum sample with no clinical signs or
symptoms.\textsuperscript{13} In the children's PFT results who were
adaptable to PFT technique, FEV\textsubscript{1} (forced expiratory
volume in the first second), FVC (forced vital capacity),
FEF\textsubscript{25-75} (25-75\% of forced expiratory flow) percent
predicted by age, and FEV\textsubscript{1}/FVC values were obtained from
their medical records. The PFTs were performed according to the American
Thoracic Society-European Respiratory Society ATS-ERS
guidelines.\textsuperscript{14}

Data of the caregivers were obtained from the demographic questionnaire.
Information on factors that might affect the psychological status of the
caregiver was collected such as their ages, ethnicity, higher education
levels, occupational status, family income, physical or psychiatric
disorders and treatments, consanguinity, the number of children, chronic
disease in another child (if any), family type (nuclear or extended),
the family members who were the primary caregivers, any persons helping
with the children's care (defined as assistants), and any work or family
problems (such as dismissal, inability to care for other children,
parental problems) related to the children's hospitalization. Monthly
family income levels were classified as below the hunger threshold,
between the hunger and poverty thresholds, and above the poverty
threshold according to 2016 data of the Turkish Statistical
Institute.\textsuperscript{15} The occupational status of the mothers
was classified as homemakers, and blue and white-collar workers.
Salaried professionals, which refers to general office workers and
management, were defined as white-collar workers. Workers who performed
manual labor, earned hourly wages or were paid piece wages according to
the amount of work done were defined as blue-collar workers.

The Beck Depression Inventory (BDI), Maslach Burnout Inventory (MBI),
Zarit Caregiver Burden Scale (ZCBS), and the Parental Attitude Research
Instrument (PARI) were used for psychological measurements and attitudes
towards the children of the primary caregivers. The Turkish versions of
these scales have been validated and used in many previous
studies.\textsuperscript{16-19} The modified Shwachman-Kulczycki Score
(mSKS) was used for evaluating the clinical status of children with CF.

The BDI consists of 21 items and measures symptoms related to emotional,
cognitive, physical, and motor functions in depression. The overall
depression score was obtained by summing all the items' scores.
Cronbach's alpha value of the inventory was found as
0.80.\textsuperscript{16} Scores between 0-9 were classified as no
depression, between 10-18 as mild depression, between 19-29 as moderate
depression, 30 and above as severe depression. The scores that can be
obtained from the scale vary between 0-63.\textsuperscript{20}

The MBI assesses burnout and consists of 22 items divided into three
subscales: emotional exhaustion, depersonalization, and personal
accomplishment. Emotional exhaustion indicates the stress dimension of
burnout and refers to a decrease in the individual's emotional and
physical resources. Depersonalization represents the interpersonal
dimension of burnout and refers to negative, rigid attitudes toward
people, and unresponsiveness to work. Personal accomplishment refers to
the sense of competence and achievement in one's work. A low score
indicates that the person tends to evaluate themself negatively.
Cronbach's alpha value of the inventory was found as
0.93.\textsuperscript{17,21} Each subscale was scored according to the
reference ranges of the MBI: for emotional exhaustion, between 0-16 as
low, between 17-26 as moderate, 27 or above as high; for
depersonalization, between 0-6 as low, between 7-12 as moderate, 13 or
above as high; for personal accomplishment, 31 or less as low, between
32-38 as moderate, and 39 or above as high. The total scores obtained
range from 0-54 for emotional exhaustion, 0-30 for desensitization, and
0-48 for personal accomplishment. Burnout was defined using the updated
Maslach-recommended criteria of ``high emotional exhaustion and high
depersonalization'' or ``high emotional exhaustion and low personal
accomplishment''.\textsuperscript{22}

The ZCBS consists of 22 items and assesses caregivers' perceived burden.
Each item is scored on a 5-point Likert scale (0=\emph{never} ,
1=\emph{rarely} , 2=\emph{sometimes} , 3=\emph{quite often} ,
4=\emph{almost always} ). A total score between 0-20 points indicates no
burden, between 21-40 illustrates mild burden, between 41-60 shows
moderate burden, and between 61-88 points indicates severe burden.
Cronbach's alpha value of the ZCBS was found as
0.83.\textsuperscript{18,23}

PARI was used to determine the attitudes of caregivers towards their
children. PARI, which is a Likert-type scale, consists of 60 items and
five subscales. These subscales are over-protectiveness, democratic
attitude, rejection of the homemaker role, marital conflict and
strictness. For each statement, one of the options ''I find it very
appropriate'' (4), ''I find it quite suitable'' (3), ''I find it
somewhat appropriate'' (2), ''I do not find it suitable at all'' (1) is
selected. The option number marked for each item is evaluated as the
item score. Responses to items 2, 29, and 44 are scored in reverse. A
separate score is obtained for each subscale. There is no total score. A
high score from the subscale indicates that the attitude reflected by
that dimension is approved. The highest scores that can be obtained from
the subscales are 64 for over-protectiveness, 36 for democratic
attitude, 52 for rejection of the homemaker role, 24 for marital
conflict, and 64 for strictness. Except for democratic attitude, high
scores from the subscales indicate the presence of a negative parental
attitude. The Cronbach's alpha coefficient of the scale was found as
0.89.\textsuperscript{19, 24}

The mSKS is calculated in four domains, including general activity,
physical examination findings, nutritional status, and radiological
findings. Each parameter was scored from 5 (i.e. severely impaired) to
25 (i.e. normal), which resulted in a total score categorised as
excellent (86-100), good (71-85), mild (56-70), moderate (41-55) and
severe (\textless{}40).\textsuperscript{25,26}

In the descriptive statistics section, categorical variables are
presented as numbers, percentages, and continuous variables as mean \selectlanguage{ngerman}±
standard deviation and median (range). Pearson's Chi-square test and
Fisher's exact test were used to evaluate categorical variables. The
Mann--Whitney U test was used for comparative analysis between two
independent variables for data that did not conform to normal
distribution, and the independent sample t-test was used for data with
normal distribution. In comparisons of three and more variables, one-way
analysis of variance (ANOVA) was performed where parametric test
conditions were ensured, and the Kruskal--Wallis H test was performed
where parametric test conditions were not ensured. The relationship
between data that did not conform to normal distribution was evaluated
using Spearman's correlation test, and data with normal distribution
were evaluated using Pearson's correlation test. P-values less than 0.05
were considered statistically significant.

\textbf{Results}

One hundred thirty-one children with CF and 39 children with PCD and
their primary caregivers were enrolled in the study. Ethnicity of all
patients were Caucasian. Sixty-six (50.4\%) of the children with CF and
20 (51.3\%) of the children with PCD were female (p=0.087). The mean age
of the children was 6.5 ± 5.3 years in the CF group, and 10.4 ± 4.6
years in the PCD group (p=0.001). The numbers of hospital visits and
hospitalizations were higher and the length of hospitalizations was
longer in the previous 6 months in the CF group than in the PCD group
(p=0.006, p=0.011, and p=0.001 respectively). The sociodemographic
features and clinical characteristics of the children in the CF and PCD
groups are displayed in Table 1.

All children's primary caregivers were their mothers. Fifty-seven
(43.5\%) of the mothers in the CF group and 19 (48.0\%) in the PCD group
had an assistant -- in all cases, a relative. Consanguinity were present
in 67 of the mothers (51.1\%) in the CF group and 17 (43.5\%) in the PCD
group. The sociodemographic characteristics of the children's mothers
are shown in Table 2.

The median mSKS of the CF group was 90 (min: 10, max: 100). The mSKS was
excellent in 66.7\% of children, good in 17.5\%, mild in 10.6\%,
moderate in 3.0\%, and severe in 2.2\%.

Only one mother in the PCD group with moderate depression and two
mothers in the CF group with mild and severe depression, respectively,
were receiving antidepressant treatment. A comparison of the mothers'
mean BDS, MBI, ZCBS, and PARI scores is given in Table 3.

There were no significant differences in BDI, MBI, ZCBS, and PARI scores
according to the mothers' educational levels and occupational status,
family type, the number of children, presence of an assistant, and work
and family problems related to their children's hospitalization in each
group (p\textgreater{}0.05). The BDI scores were higher in mothers who
had another child with chronic disease (p=0.046), low family income
(p=0.014), and children with colonization (p=0.008) in the CF group.
Burnout was higher in both the CF and PCD groups with low family income
(p=0.022 and p=0.034, respectively). The ZCBS scores were higher in
mothers with a history of sibling death in the CF group (p=0.002). The
mSKS was lower in children with CF with bacterial colonization
(p=0.028). Democratic attitude was lower in mothers with consanguinity
in the CF group (p=0.016). Over-protectiveness and strictness scores
were higher in mothers with high family income in the CF group (p=0.041
and p=0.024, respectively). Rejection of the homemaker role scores was
higher in mothers who had another child with chronic disease in the PCD
group (p=0.001).

There were no correlation between the ages of children with CF and PCD
and BDI, MBI, ZCBS, and PARI scores of their mothers. The number of
hospital visits, hospitalizations, and the duration of hospitalizations
in the previous 6 months were negatively correlated with the children'
FEV\textsubscript{1} (r= -0.443, p=0.001; r= -0.469, p=0.001; r= -0.478,
p=0.001, respectively) and FVC (r= -0.496, p=0.001; r= -0.362, p=0.007;
r= -0.361, p=0.007, respectively) in the PFT results of children with
CF. Also, the number of hospitalizations in the previous 6 months were
negatively correlated with BMI in the PCD group (r= -0.238, p=0.043).
Correlations between children's hospital visits and length of
hospitalization with mothers' scores are given in Table 4.

In the CF group, the BDI scores of mothers were correlated with mSKS,
burnout, ZCBS and over-protectiveness scores (r= -0.311, p=0.004;
r=0.483, p=0.001; r=0.259, p=0.010; r= -0.267, p=0.005, respectively).
The mSKS of children was correlated with burnout, democratic attitude,
and marital conflict scores of mothers (r= -0.232, p=0.033; r=0.290,
p=0.007; r=0.348, p=0.001 respectively). The burnout scores of mothers
were correlated with ZCBS, democratic attitude, and strictness scores
(r=0.468, p=0.001; r= -0.242, p=0.015; r= -0.222, p=0.026,
respectively). The ZCBS scores of mothers were correlated with
over-protectiveness and strictness scores (r= -0.243, p=0.014; r=
-0.339, p=0.001, respectively). The over-protectiveness scores of
mothers were correlated with rejection of the homemaker role, marital
conflict, and strictness scores (r=0.431, p=0.0001; r=0.474, p=0.001;
r=0.713, p=0.001 respectively).

In the PCD group, the BDI scores of mothers were correlated with burnout
and ZCBS scores (r=0.582, p=0.001; r=0.622, p=0.001, respectively). The
ZCBS scores of mothers were correlated with burnout and marital conflict
scores (r=0.488, p=0.009; r=0.388, p=0.031, respectively). The burnout
scores of mothers were correlated with marital conflict scores (r=
0.407, p=0.026). The democratic attitude scores of mothers were
negatively correlated with over-protectiveness and strictness scores
(r=-0.394, p=0.025; r= -0.617, p=0.001, respectively). The rejection of
the homemaker role of mothers was correlated with marital conflict and
strictness scores (r=0.457, p=0.010; r=0.657, p=0.001, respectively).
The marital conflict of mothers was correlated with strictness scores
(r=0.483, p=0.006).

\textbf{Discussion}

Although there are many studies investigating the psychological status
of caregivers of children with CF, data on caregivers of children with
PCD are limited. This study showed that depression, burnout, burden, and
negative attitudes of mothers of children with CF were higher than
mothers of children with PCD, but these problems were also quite common
in mothers of children with PCD. Mothers of children with CF and PCD
with lower family income had more burnout. Rejection of the homemaker
role was higher in mothers of children with PCD who had another child
with chronic disease. Frequent hospitalizations were negatively
associated with BMI in children with PCD. Frequent hospital visits were
correlated with burnout of mothers in children with CF and PCD.

In a study evaluating the depression and anxiety levels of patients with
CF and their caregivers, it was found that 20-34\% of mothers had
depression in the evaluations made with different scales. Mothers'
depression has been found to be associated with children's recent
intravenous antibiotic therapy and physiotherapy.\textsuperscript{27} In
our study, the depression level of the mothers of children with CF was
higher than the mothers of children with PCD. Depression in mothers of
children with CF was associated with the presence of chronic disease in
another child, low family income, and colonization in their children. In
a study of 326 caregivers of children with CF, a higher burden was found
in the caregivers of older patients and patients with \emph{P.
aeruginosa} infection.\textsuperscript{28} In our study, the caregiver
burden of mothers of children with CF was higher than mothers of
children with PCD. It was observed that as the number of hospital visits
and duration of hospitalization of patients with CF increased, their
respiratory functions were also negatively affected. The caregiver
burden was increased in the mothers of children with CF who had a
history of sibling death and frequent hospitalizations. Frequent
hospital visits, long and frequent hospitalizations, and lower clinical
scores may affect the psychological status of the mothers of patients
with CF and may affect the care of their children.

Carotenuto et al.\textsuperscript{29} found that mothers of children
with PCD have higher levels of stress compared with mothers of healthy
children. PCD is highly stressful and depressing for affected children
and their families. In our study, all mothers of children with PCD had
caregiver burden, approximately one in three mothers had depression, and
one in ten mothers had burnout. As the depression level of mothers of
children with PCD increased, burnout and caregiver burdens are also
increased. Like children with CF, children with PCD need frequent
clinical evaluation and treatment, including chest physiotherapy and
inhaled therapies, and antibiotics for recurrent respiratory infections.
All these daily practices can lead to caregiver burden, burnout, and
depression. In mothers caring for an ill child, burnout increases due to
an increased sense of responsibility, concerns about the health of the
child, limited social activities, and the inability to find time for
their own needs. Due to burnout and caregiver burden, mothers may feel
insignificant and withdrawn from their environment and have an
introverted and depressed mood. These negatively affected moods of
mothers may affect the care of their children, adherence to treatment,
and the course of the disease. The higher depression, burnout, burden
and negative attitudes of mothers of children with CF than mothers of
children with PSD may be related to the younger age of children with CF
and their greater need for maternal care for all daily practices.

Burnout caused by prolonged stress is quite common among parents of
children with chronic illnesses.\textsuperscript{30} Children with PCD
usually have a normal life span. Lung functions usually deteriorate more
slowly than in CF.\textsuperscript{31} In our study, the level of
burnout was associated with low family income and the frequency of
hospital visits of their children in mothers of both children with CF
and PCD. Anxiety about the unmet daily financial needs of their children
due to low family income may increase the burnout level of mothers.
Hospital visits and hospitalizations of children with PCD are, as
expected, less than for children with CF, and their respiratory function
is also significantly better. The BMIs of children with PCD who had
frequent hospitalizations were decreased. Growth monitoring is
important, especially in children with PCD who have frequent
hospitalizations.

Caring for a child with a chronic illness can pose significant
challenges for caregivers, due to uncertainty about the aggravation of
the disease, financial costs associated with care, compliance with
treatment programs, impact on family life due to frequent outpatient
visits, and hospitalization.\textsuperscript{28} In a study conducted
with caregivers of 44 children with CF and 19 with PCD, the burden of
caregivers of children with CF was higher than for PCD in relation to
pulmonary function and quality of life.\textsuperscript{32} In our
study, mothers of all children with CF and PCD had caregiver burden.
Families have to cope with daily life difficulties, implementation of
complex treatment regimens, regular clinic visits, care costs,
disruptions in plans, as well as uncertainties about the progression of
chronic disease. In addition, problems in the marital relationship, the
decrease in the life expectancy of the child, and the decrease in the
time allocated to the family are significant sources of
stress.\textsuperscript{33} In a study evaluating 36 children with CF,
mothers of children with CF with frequent hospital visits or
hospitalizations were found to be more overprotective, rejecting the
role of homemaker, and experiencing marital
conflicts.\textsuperscript{34} In our study, marital conflict was higher
in mothers of children with PCD with burnout and caregiver burden.
Rejection of the role of homemaker was more common in mothers who had
other children with chronic disease and in mothers with marital
conflict. The time the mother spends with their children can reduce the
time spent with other family members, which may lead to rejection of the
role of homemaker, affecting family relationships and thus increasing
conflict. Mothers who have strict rules can cause unrest in the home and
marital problems due to the increased stress associated with their
child's illness and compliance with their treatment. This situation can
be exacerbated, especially in mothers who have other children with
chronic disease.

The nature of overprotection has been defined as exhibited protective
behavior that is excessive in light of the child's developmental stage
and has been associated with parental stress. Parental stress has been
found to be associated with a child's disease
outcomes.\textsuperscript{35} In our study, mothers of children with PCD
who were overly controlling, intrusive, and strictly disciplined had a
negative democratic attitude. Fears and anxieties of mothers about the
future of the child may lead to overprotective, stricter, and
authoritarian behaviors. Democratic attitude was lower in the mothers of
children with PCD when the mothers had more children. The fact that
mothers spend more time with their ill children and protect them more
may cause them not to show the same attention to their healthy children.

The mean of the consanguineous marriages is 23.2 \% (8.9-42.6) in our
country according to 2016 data of the Turkish Statistical
Institute.\textsuperscript{15} In our study, consanguinity were present
in 51.1\% of CF and in 43.5\% in PCD families. The higher rates of
consanguineous marriages in our study compared to the general population
may be associated with the increased incidence of both diseases in
consanguineous marriages.

Previous research on well-being and psychological issues of PCD and CF
has focused on the emotional burden of the condition, including
patients' concern about current and future health. The issue has been
recently further enriched with the contribution of several studies
conducted during the current SARS-CoV-2 pandemic, an event that has had
an impressive impact on the quality of life of patients with chronic
conditions and their families.\textsuperscript{36,37}

The limitations of our study were that all primary caregivers were
mothers and the questionnaires were administered cross-sectionally.

In conclusion, mothers of children with CF and PCD had various
difficulties related to the children's disease in our study. Although
the psychological status of mothers of children with PCD was better than
mothers of children with CF, their problems may increase with frequent
hospitalizations, low family income, number of children, and chronic
disease in another child. It is important not to neglect the
psychological problems that mothers of children with PCD may experience
due to difficulties associated with care. Screening the psychological
status of mothers of children with CF and PCD and providing them with
psychological and social support may help reduce their anxiety about
their children's health and benefit the care of their children.

\textbf{Acknowledgments}

The authors declare that they have no known competing financial
interests or personal relationships that could have appeared to
influence the work reported in this paper. The authors have no conflicts
of interest relevant to this article to disclose.

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