An investigation on parenting stress of children with cystic fibrosis

Cystic Fibrosis (CF) is the most common serious genetic disease in Caucasian populations, resulting in clinical symptoms in multiple body systems as lung, sinus, gastrointestinal and reproductive tracts. Standard of care includes monitoring pulmonary function, nutritional status, airway clearance, and infection control [1]. Newer oral agents that are based on specific mutations are providing physicians with the ability to target underlying CFTR gene defects, thus leading to an increased life expectancy over 50 years [2] in patients with CF.

However, there is no evidence that chronic time consuming conventional therapies can be stopped and CF still represents a paradigmatic clinical scenario requiring a multidimensional approach for patients and their families among chronic diseases [3].

With regard to families, it has seemed interesting to investigate the degree of stress in parents of children affected by this disease, as caregivers’ psychological wellness is hardly ever taken into consideration during the treatment paths of pediatric patients. In addition, since parenting stress is a common part of parenting experience and it usually occurs when either parents’ commitment exceeds their own internal resources, we have considered understanding how a parent of a child with CF may feel in the light of both their practical and emotional commitment involved.

There is evidence that shows that caregivers of children with a chronic illness report significantly higher general parenting stress than caregivers of healthy children [4‐7]. Furthermore, parenting stress is crucial to understand any family dysfunction and/or psychopathology [8]. However, it still remains unclear whether or not and to what extent parental distress is a major feature of cystic fibrosis [9, 10].

The aim of the study is to understand whether this disease may induce a stress pattern in parents of children and adolescents with CF, analyze parental stress severity as well as identify any further correlations with additional factors.

Table 1 shows the characteristics of both parents and children.

Statistical tests were performed for each variable. As expected, there were more mothers than fathers as key responders (p < 0.0001) as well as married versus unmarried parents (89.36% > 10.64%, p < 0.0001). More frequently, parents had a “Middle School” education level (53.19%, p = 0.0005) and 51.32%% reported a regular job.

As to CF children’s features, their mean age was about 8 years and 54.35% of these were males. In addition, children with no (40.43%, p = 0.0344) or one (42.55%, p = 0.0161) sibling were more frequent whilst the most frequent birth order was “first” child (59.57%, p < 0.0001). Finally, in terms of disease severity, the most frequent degree was “low” whereas “severe” was significantly less frequent compared to other severity degrees.

Table 2 reports two groups: Mother and Father.

The Mother’s group was composed of 31 female individuals aged between 21 and 53 years (mean 37.71 ± 8.17, of 8.17 years), while the Father’s group included 16 individuals aged between 28 and 55 years, (mean of 41.13 years ±7). There were no statistical differences between the two groups. Similarly, there was no significant difference (p = 0.209) among the sub-categories with normal stress: PD (74.47% = 35/47), P-CDI (55.32% = 26/47), DC (70.21% =33/47) and DEF (70.21% = 33/47).. Overall the majority of the population reported normal stress. However 1 out of 4 parents reported high stress with no major difference between mothers and fathers. The evaluation on variables revealed no significant differences between mothers and fathers in terms of high stress. These results are similar in each of the following sub-categories: PD (12.5% =2/16 < 32.26% =10/31, p = 0.263), P-CDI (50% = 8/16 > 41.94% = 13/31, p = 0.828), DC (31.25% = 5/16 > 29.03% = 9/31, p = 0.858) and DEF (25% = 4/16 < 32.26% = 10/31, p = 0.858).

In addition for high stress, there were not any remarkable differences among PD (21.28%), P-CDI (25.53%) and DC (23.41%) subscales. A similar result was reported for normal stress (74.47, 55.32 and 70.21%, respectively), while for suspicious stress (4.26, 19.15 and 6.38% respectively) there was a significant difference (p = 0.0033). Finally, also the cases with high stress including suspicious stress (25.53,44.68 and 29.79% respectively) bore no significant differences (p = 0.12). In short, PD, P-PCI and DC subscales had similar association pattern.

Table 3 shows the characteristics of parents and their children, considering parents with elevated or suspicious stress only.

In particular, only 13 in 47 parental questionnaires revealed increased stress. One of these questionnaires fell in the defensive response category with an exceptionally low stress score (PSI-SF score = 40). In this case, the individual was a married female of 37 with no work income and a high school diploma, and a male child of 2 years with a severe disease.

Table 3 shows a significant presence of female (69.23%, p = 0.0027), married subjects (84.62%, p < 0.0001), patents unemployed (69.23%, p = 0.0027) and individuals with a middle school education level (61.54%, p = 0.0084). Among the children’s characteristics, the most frequent feature was ill children with one sibling (53.85%, p = 0.0375).

Table 4 reports two groups: a group of parents with normal stress (PNS) (32.29% males and 64.71% females and an age range of 21–51 years, a mean of 38.53 and a standard deviation of 8.82 years), and a second group of parents with suspicious or elevated stress (PES) (30.67% males and 69.23% females, an age range of 28–58 years, a mean of 39.77 and a standard deviation 5.25 of years).

A significant presence of children with one sibling in the PNS group was observed compared to the PES group with 52.94% > 7.69% (p = 0.013). In addition, a significant presence of children with low Clinical Severity was revealed in the PNS group, while a remarkable number of children with a severe disease was observed in the PES group compared to the PNS group with 30.77% > 2.94%, (p = 0.0252).

Finally, Table 5 shows both univariate and a multivariate linear regression analysis between Total stress evaluated with the standardized questionnaire PSI-SF, and the independence variables associated to parents such as Age, Gender parents, Civil Status, Job, Education level, and between Total stress and the independent variables associated to children such as Children’s Age, Age at diagnosis, Number of siblings, Birth order and Clinical Severity.

The univariate analysis pointed out a significant positive association between Total stress and children’s Clinical Severity, indicating that parent stress was directly proportional to child Clinical Severity. In the multivariate analysis, i.e. considering simultaneously for children: age, gender, age at diagnosis, clinical severity, number of siblings and birth order, it resulted that the Number of siblings was a negative predictor of Total stress variable, while Birth order was a significant positive predictor of Total stress variable; in other words, an increased number of siblings represents a predictor of a decrease of parenting stress level, vice versa for Birth order.

Finally we observed in Total stress variable a significant presence of parents with normal stress (72.34% = 34/47) in contrast to parents with high stress (25.53% = 12/47) including subject with suspicious stress (2.13% = 1/47), i.e. it resulted: 72.34% (34/47) > 27.66% (13/47), p < 0.0001.

In this study we analyzed parenting stress of children affected by Cystic Fibrosis through a biopsychosocial approach. The latter, which is supported both by the World Health Organization (WHO) and many data [13, 14], indicates that healthcare professionals should provide adequate healthcare assistance to children with the disease as well as a psychological support to their parents in order to improve their overall quality of life. The care should include biomedical and psycho-social interventions to achieve a positive impact on patient’s life.

Contrary to our expectations [15‐18], the study revealed a significant presence of parents with normal stress level. We hypothesize that this result may be attributed to the low Clinical Severity of the children enrolled in this study (Tables 4 and 5). In addition, we found a positive correlation between Clinical severity and Total stress, i.e. it was observed an increasing of abnormal stress in parents connected to an increasing of clinical severity in children. This result was not detected in a previous review [4]. We also investigated on additional parameters to identify possible correlations with parenting stress such as education levels, civil status, number of siblings, clinical status, etc. A significant correlation was found between stress levels and number of siblings in a multivariate analysis, i.e. the presence of more children in the same family is associated with high parental stress levels. This may be due to the higher commitment of parents in having to manage more children, when repeated hospitalizations cause more stress [19]. In terms of Birth order, the study has shown that parents of children with chronic illness bear higher stress level compared to other parents, and in particular, a high Birth order in a family with a child with the disease, implies parents with high or suspicious stress, whereas a low Birth order in a family predicts parents with normal stress levels. It has been recently shown that the mother first born interaction differs from the mother second born interaction in healthy children [20].

Our results may suggest that the increased distress observed in parents of children with chronic disease is probably affected by the great expectations that parents have on their first child.

In the event of a sick, the parents need a mental re-adjustment between their expectations of an ideal child and the real child status. The Clinical Severity analysis revealed a correlation between parental stress levels and degree of clinical severity, that is parental stress is higher in parents with a child with a severe disease and less in parents with a child with low Clinical Severity.

With regard to the total scores among stress sub-scales (Parenting Distress – PD, Parent-Child Dysfunctional Interaction P-CDI, Difficult Child (DC), the lowest score of parents with normal stress value was reported in the P-CDI sub-scale. P-CDI sub-scale measures parents’ expectations and interactions with their child. High scores in P-CDI may indicate a parent’s feelings of disappointment either caused by the child or by a lack of proper bonding with their child [21]. The set of questions made it possible to investigate the parent-child relationship and to point out whether the parent perceived the relationship with their child to be different from the one they expected in a no sick child, (i.e. “My child is not able to do as much as I expected”, “When I do things for my child, I feel that my efforts are not much appreciated”, “My son does not seem to learn as fast as most children do!”). It is clear that the illness event in these parents also negatively influences parent-child dynamics [22].

Similarly, none of the sub-categories has shown any remarkable difference between fathers and mothers in terms of high stress (including stress to be assessed and high stress) 25.0 < 29.03%. In this case, a higher level of stress in mothers would have been more likely expected as they are normally more involved in the history of the disease. As they are nearly always hospitalized together with the child, these mothers could be overloaded with more stress [18, 23].

However, despite the small number of parents with clinically elevated or suspicious stress (27.66%), their features were nonetheless analyzed.

Considering parents with high or suspicious stress levels only (Table 3), a higher presence of mothers was observed as opposed to fathers (69.23% > 30.77), the mean age was of about 40 years, married individuals were the majority (84.62%), and there was a substantial presence of individuals without regular paid job (69.23%) and a middle school education level (61.54%). Finally, among the examined parents, high stress was detected in parents having a second child (53.85%) with Cystic Fibrosis, i.e. an ill child with one sibling.

This study has shown that, despite the fact that a child’s chronic disease inevitably puts a heavy stress on a family, making them vulnerable and emotionally exposed, an effective multidisciplinary care plan, that takes into account the emotional and sympathetic aspect of caregivers, would help parents development of internal resources to allow them to cope with stress. Such comprehensive model of management with a regularly provided psychological support appears to be a key factor for coping and for achieving a strong therapeutic alliance between healthcare professionals and patients, in other words a strong psychological support fits particularly well in the biopsychosocial model of comprehensive are in this progressively worsening disease.