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Amyotrophic lateral sclerosis (ALS) is a degenerative disease involving both upper and lower motor neurons, and leading relentlessly to progressive muscle atrophy and weakness, dysphagia and dysarthria. In most cases, patients die within 3–5 years of the disease’s onset, usually due to respiratory failure (
There is no effective cure for ALS, so medical treatment is focused on physical symptom management (
Amyotrophic lateral sclerosis is overwhelming and challenging for caregivers and families as well. When
Patients with ALS have to cope with serious restrictions and changes in their daily life. They need assistance with eating and dressing, nursing care, and mobility, that is often provided by a family member, and the patient’s partner in nearly 80% of cases (
Although many studies have explored how patients and their caregivers cope with ALS, the literature completely lacks any research on the specific psychological impact of such a devastating disease on the patients’ children. It is important to investigate this issue in the case of ALS because the consequences of a parent’s severe illness on their offspring can sometimes be dramatic, as already reported for a variety of diseases. In particular, the impact on children of a parent suffering from a chronic medical condition (CMC), i.e., a disease lasting at least 3 months and involving one or more apparata (
According to the extant literature, a CMC in a parent involves the whole family and has a great impact on the parent’s children (
More in general, children of an ill parent were reported to experience high levels of stress and to be at higher risk of health-related and social-emotional problems, such as somatic complaints, social isolation, excessive concern about becoming ill themselves, anxiety, depressive affects, and low self-esteem (
Among the studies on children with parents suffering from neurological CMCs, the case of MS has been the most thoroughly investigated (
Given these premises, the present study had three main objectives. First, we aimed to verify whether school-age children and adolescents who have a parent with ALS are more at risk of problem behavior, as measured by self-report questionnaires, than children of healthy parents. Consistently with the extant literature on CMCs in parents, we expected children with a parent who had ALS to display more internalizing and/or externalizing problems than controls. Second, we aimed to explore the psychological adjustment of children with a parent suffering from ALS by administering a performance-based task, the Rorschach test, scored using the Comprehensive System (CS;
The study group consisted of 23 children with a parent suffering from ALS. The mean age of the children was 10.62 years (
Twenty-three children matched with the study group for age, gender, and birth-order formed a control group. They were recruited from the general population of children attending primary or secondary schools in the same area as the study group.
All participants were Caucasian and none of their parents were single. The participants’ demographic details are shown in
Demographic characteristics of the study sample and control group.
Parents’ characteristics | ||
Ill parent (n = 12) | Spouse (n = 12) | |
Mothers ( |
5 (41.7%) | 7 (58.3%) |
Fathers ( |
7 (58.3%) | 5 (41.7%) |
Age (mean, SD) | 45.12 (6.5) | 42.75 (3.2) |
Age range (years) | 31.5–52.5 | 38.0–47.7 |
Duration of illness, years (mean, SD) | 6.93 (4.2) | |
ALSFRS (mean, SD) | 26 (8.4) | |
Limb onset ( |
11 (91.7%) | |
Bulbar onset ( |
1 (8.3%) | |
Gender ( |
||
Female | 6 (26.1%) | 6 (26.1%) |
Male | 17 (73.9%) | 17 (73.9%) |
Age (mean, SD) | 10.62 (4.0) | 10.74 (3.9) |
Age range (years) | 5.0–17.6 | 5.0–17.5 |
Number of children participating in the study per family ( |
||
1 | 5 (41.7%) | 18 (90%) |
2 | 3 (25%) | 1 (5%) |
3 | 4 (33.3%) | 1 (5%) |
Children aged 11 years and over (
The YSR is a self-administered questionnaire, part of the Achenbach System of Empirically Based Assessments (ASEBAs;
The CBCL is a rating scale, also part of the ASEBA, and it is the parallel form of the YSR for parents. It provides parent-reported information on a broad range of emotional and behavioral difficulties in a child during the previous 6 months. Like the YSR, the CBCL includes 118 items for measuring the eight syndrome scales and the three general dimensions of Internalizing, Externalizing, and Total Problems, plus 20 competence items (referring to the child’s participation in hobbies, games, sports, jobs, chores, friendship, and activities).
As recommended by
The CBCL and the Teacher’s Report Form in the ASEBA have shown good validity and reliability characteristics in the Italian population (
The children’s personality characteristics were assessed using the Rorschach test, a performance-based task consisting of 10 official inkblots (five made with black ink on a white background, two with black and red ink on a white background, and three with multicolored ink on a white background). Forty-five Rorschach protocols were collected because one child in the study group refused to complete the Rorschach task. Exner’s CS was used to administer and interpret the Rorschach protocols (
To obtain inter-scorer reliability, 10 protocols set were selected at random and scored independently by the two judges. Inter-scorer reliability was calculated by percentage of agreement and iota for 10 response segments. Iota is chance-corrected reliability coefficient that is equivalent to Cohen’s kappa for a multivariable test scored by two or more raters (
Rorschach inter-scorer agreement on coding segments.
Variable | % Agreement | Iota (Kappa) |
---|---|---|
Whole responses | 0.975 | 0.793 |
Location and space (two variables) | 0.954 | 0.895 |
DQ (+, o, v/+, v) | 0.894 | 0.732 |
Determinants (11 variables) | 0.978 | 0.835 |
FQ (None, +, o, u, -) | 0.673 | 0.512 |
Pairs | 0.954 | 0.895 |
Contents (27 variables) | 0.987 | 0.830 |
P | 0.954 | 0.795 |
Z score | 0.899 | 0.825 |
CS special scores (14 variables) | 0.989 | 0.587 |
To prevent chance findings arising from multiple comparisons, we restricted the Rorschach variables analyzed in this study (
Several indexes were used to assess respondents’ ideational processes and cognitive resources, namely variables regarding reality testing (X-%, distorted form; X+%, conventional form; XA%, very good form quality percent; Xu%, usual form), and variables regarding thought problems (i.e., Sum6, Number of Special Score; WSum6, Weighted Sum of Special Score; Level 2, Raw Number of Lv2 Special Score). We also used the intellectualizing index (2AB + [Art + Ay]) to examine variables that might reflect defensive mechanisms, such as the tendency to take refuge in fantasy (Ma/Mp), or to minimize emotional experiences. All these variables were analyzed as frequencies (considering the number of responses), and/or as categories when the frequency exceeded a cutoff validated in the CS.
Finally, we analyzed the Psychopathological Constellation indexes. When positive, these indexes point to the presence of depressive symptoms (DEPI), the risk of suicidal behavior (S-CON, Suicide Constellation), inadequate coping abilities (CDI, Coping Deficit Index), obsessive cognitive processing (OBS, Obsessive Style Index), paranoid thoughts (HVI, Hypervigilance Index), and thought disturbance (PTI, Perceptual Thinking Index).
Patients with possible, probable, or definite ALS – according to the El Escorial criteria for its clinical diagnosis (
We compared the study and control groups for the measures derived from the YSR, CBCL, and Rorschach CS, using the non-parametric statistic of the Mann–Whitney
To check the consistency between the YSR completed by the children in the study group and the CBCL completed by their parents, we used Pearson’s product–moment correlations to assess relative agreement (
First we examined whether the children with a parent who had ALS differed from the controls in terms of emotional and behavioral problems and competences, as measured by the YSR (
Comparison of emotional and behavioral problems and competences (YSR) between children with and without a parent suffering from ALS.
Study group ( |
Control group ( |
Cohen’s |
||||||
Scale | SD | SD | ||||||
Anxious/Depressed | 8.86 | 3.30 | 5.00 | 2.88 | 35.0 | -2.92 | 0.003** | 1.25 |
Withdrawn/Depressed | 4.43 | 2.34 | 2.14 | 1.70 | 41.0 | -2.65 | 0.007** | 1.12 |
Somatic Complaints | 4.50 | 4.16 | 2.79 | 1.67 | 81.0 | -0.79 | 0.442 | 0.54 |
Social Problems | 4.50 | 2.95 | 2.86 | 2.14 | 64.0 | -1.58 | 0.119 | 0.64 |
Thought Problems | 3.57 | 3.32 | 1.86 | 1.41 | 67.5 | -1.43 | 0.158 | 0.67 |
Attention Problems | 6.36 | 3.52 | 4.64 | 1.98 | 78.0 | -0.93 | 0.364 | 0.60 |
Rule-Breaking Behavior | 2.00 | 0.88 | 2.36 | 2.90 | 80.5 | -0.822 | 0.423 | -0.17 |
Aggressive Behavior | 8.07 | 3.73 | 5.64 | 3.10 | 58.5 | -1.84 | 0.066 | 0.70 |
Internalizing | 17.79 | 7.03 | 9.93 | 4.71 | 31.0 | -3.08 | 0.001** | 1.31 |
Externalizing | 10.07 | 3.91 | 8.00 | 5.55 | 61.5 | -1.68 | 0.095 | 0.43 |
Total problems | 47.93 | 17.94 | 30.57 | 13.25 | 41.0 | -2.62 | 0.008** | 1.10 |
Activities | 6.74 | 2.98 | 8.03 | 2.50 | 77.0 | -0.97 | 0.345 | -0.47 |
Social | 6.86 | 1.57 | 7.56 | 1.92 | 72.5 | -1.18 | 0.248 | -0.40 |
School | 2.94 | 2.36 | 2.32 | 0.45 | 94.0 | -0.19 | 0.863 | 0.36 |
Total Competence | 15.93 | 3.37 | 17.91 | 3.94 | 71.0 | -1.24 | 0.227 | -0.54 |
Analyzing the scores for Internalizing, Externalizing, and Total Problems in terms of their clinical relevance, we found 6 (42.9%) of 14 children with a parent who had ALS were in the clinical range on the Internalizing scale, 4 (28.6%) were borderline, and only 4 (28.6%) were in the normal range. Conversely, none of the 14 children in the control group was in the clinical range, 2 were borderline, and 12 were in the normal range. The difference between the two groups was statistically significant (χ2 [2,
No such differences emerged between the groups in terms of their distribution in the Externalizing (χ2 [2,
The personality characteristics (derived from the Rorschach CS) of the children in the two groups were compared using the Mann–Whitney
Mann–Whitney
Study group ( |
Control group ( |
||||||||
---|---|---|---|---|---|---|---|---|---|
CS indexes | Interpretation (after |
SD | SD | Cohen’s |
|||||
SumV | Self-disgust, shame, emotional distress, guilt | 0.73 | 0.70 | 0.04 | 0.21 | 113.0 | -3.92 | <0.001*** | 1.34 |
FC | Controlled emotional experiences | 0.64 | 0.85 | 1.96 | 1.67 | 126.0 | -3.01 | 0.002** | -0.99 |
An | Preoccupations concerning body, health or illness | 1.16 | 0.25 | 0.66 | 0.14 | 183.5 | -1.79 | 0.078 | 2.47 |
MOR | Negative or blemished features about the self and the world | 1.73 | 1.39 | 0.65 | 1.56 | 104.5 | -3.57 | <0.001*** | 0.73 |
GHR | Positive self-image | 1.64 | 1.62 | 3.04 | 1.99 | 146.5 | -2.45 | 0.013* | -0.77 |
XA% | 64.23 | 16.52 | 91.39 | 9.01 | 25.0 | -5.19 | <0.001*** | -2.04 | |
Distorted perceptions | 34.00 | 15.34 | 7.52 | 7.43 | 18.0 | -5.35 | <0.001*** | 2.20 | |
Xu% | 36.41 | 14.11 | 59.00 | 12.32 | 57.5 | -4.44 | <0.001*** | -1.71 | |
Sum6 | 4.64 | 5.20 | 1.00 | 1.31 | 103.0 | -3.49 | <0.001*** | -0.48 | |
Level 2 | 0.82 | 1.30 | 0.04 | 0.21 | 158.5 | -2.96 | 0.003** | 0.84 | |
Alog | Thought disturbance | 1.32 | 2.15 | 0.17 | 0.65 | 127.0 | -3.42 | <0.001*** | 0.72 |
CTM | 0.86 | 1.21 | 0.00 | 0.00 | 138.0 | -3.59 | <0.001*** | 1.01 | |
WSum6 | 19.95 | 23.88 | 2.74 | 4.47 | 88.0 | -3.82 | <0.001*** | 1.00 | |
M- | 1.27 | 1.72 | 0.26 | 0.45 | 157.0 | -2.48 | 0.013* | 0.80 | |
2AB + (Art + Ay) | Minimizing emotional experiences by intellectualizing | 1.59 | 1.47 | 0.83 | 1.07 | 169.5 | -1.99 | 0.049* | 1.39 |
PTI | Perceptual-thinking index | 1.95 | 1.50 | 0.09 | 0.29 | 57.0 | -4.92 | <0.001*** | 1.72 |
DEPI | Depression index | 4.23 | 1.19 | 3.57 | 0.95 | 169.0 | -1.97 | 0.050 | 0.61 |
Chi-square comparison of categorical variables in the Rorschach Comprehensive System (CS) between children with and without a parent suffering from ALS.
Study group ( |
Control group ( |
||||||
---|---|---|---|---|---|---|---|
CS indexes | Interpretation (according to |
Freq. | Freq. | Chi-square | p | ||
SumV > 0 | Self-disgust, shame, emotional distress, and guilt | 13 | 59.1 | 1 | 4.3 | 15.72 | <0.001*** |
GHR < PHR | Healthy and adaptive understanding of others | 17 | 77.3 | 8 | 34.8 | 8.22 | 0.004** |
Negative or blemished features about the self and the world | 10 | 45.5 | 4 | 17.4 | 4.13 | 0.042* | |
XA% > 0.90 | 0 | 100 | 16 | 69.5 | 23.75 | <0.001*** | |
X-% > 0.14 | Distorted perceptions | 21 | 95.5 | 5 | 21.7 | 25.05 | <0.001*** |
X-% > 0.25 | 16 | 72.7 | 2 | 8.7 | 19.21 | <0.001*** | |
M > 0 | 12 | 54.5 | 6 | 26.1 | 3.79 | 0.051 | |
Level 2 > 0 | Thought disturbance | 9 | 40.9 | 1 | 4.3 | 8.70 | 0.003** |
Ma < Mp | 0 | 0 | 4 | 17.4 | 4.20 | 0.040* | |
PTI > 2 | Index of disturbed thinking and distorted perceptions | 9 | 40.9 | 0 | 0 | 11.76 | 0.001** |
DEPI > 4 | Higher probability of subclinical or clinical depression | 10 | 45.5 | 4 | 17.4 | 4.13 | 0.042* |
CDI > 3, DEPI > 4 | Tendency toward affective problems | 3 | 13.6 | 0 | 0 | 3.76 | 0.053 |
The children with a parent suffering from ALS had several variables that, interpreted in accordance with the CS, were indicative of a less adequate psychological functioning than the children in the control group. They showed signs of more self-disgust, shame, emotional distress, and guilt (SumV, SumV > 0), more negative or blemished features about the self and the world (MOR, MOR > 1), more preoccupation with the body, health or illness (An nearing statistical significance,
The children in the study group revealed other differences vis-à-vis the control children too. They had less unconventional, uncommon, or creative views (Xu%), they more frequently exhibited human movements indicative of general mental abilities, including planning, imagination, and empathy (M > 0). They also tended to minimize emotional experiences by intellectualizing [2AB + (Art + Ay)]. None of the children with a parent suffering from ALS used fantasies as an integral part of their defense system (Ma < Mp).
Among the pathological constellations of the Rorschach CS, the DEPI (i.e., the intensity of depressive affects) was significantly higher in the study group, and significantly more children in the study group (10 of 22, 45.5%) than in the control group (4 of 23, 17.4%) came above the clinical threshold (DEPI > 4) indicating a higher probability of subclinical or clinical depression. Three children in the study group, but none in the control group, had a DEPI > 4 associated with a CDI ≥ 4 (i.e., a positive CDI): this association – which neared statistical significance (
The children in the study group did not differ significantly from the controls in the other psychopathological constellations, i.e., the S-CON, the HVI, and the OBS.
We compared the two groups of children in terms of their emotional and behavioral problems and competences as reported by their healthy parents using the CBCL (
On the other hand, parents of the children in the study group returned significantly lower scores than parents of children in the control group for all competence scales of the CBCL except School (
Then we applied Pearson’s correlation and ICCs to examine the agreement between the scores obtained by the children in the study group and their healthy parents. Moderate to high Pearson’s correlations were found for Internalizing, Externalizing, and Total Problems (
Our study investigated the emotional and psychological impact of having a parent with ALS on school-age children and adolescents, in terms of adjustment, problem behavior, and personality characteristics, because many studies have explored how patients and caregivers cope with ALS, but none have investigated the psychological impact of the disease on patients’ children.
Our findings clearly showed that, compared with a control group of offspring of healthy parents, children who have a parent with ALS experience several emotional and behavioral adverse consequences in terms of global emotional and behavioral problems, internalizing problems, anxiety, and depression, and clinically significant indexes relating to their personality characteristics.
The first aim of our study was to see whether children of a parent with ALS are more at risk of problem behavior than a control group of children with healthy parents. Specifically, children of a parent with ALS reported more emotional and behavioral problems in the YSR, as measured by the Total Problems scale, and more Internalizing problems than the control group. They also had significantly higher scores on two syndrome scales in the internalizing domain of the YSR (Anxious/Depressed and Withdrawn/Depressed), and scores that neared a statistically significant difference on the Aggressive behavior scale of the YSR. Notably, all the corresponding effect sizes were either large (for Anxious/Depressed, Withdrawn/Depressed, and Total Problems) or very large (for Internalizing Problems), indicating that the differences between the scores obtained by the two groups were very clear. A substantial percentage of children with an ALS parent fell within the clinical (42.9%) and borderline (28.6%) ranges for Internalizing problems according to the YSR cutoffs; the difference vis-à-vis the control group was statistically significant.
On the whole, our results are in line with the extant literature suggesting that children can be strongly affected by having a chronically ill parent, especially in terms of internalizing problems (
Having a parent with ALS did not increase the scores for somatic complaints in our study group, as reported in other studies concerning CMCs, and MS in particular.
Contrary to our expectations, we found no significant differences between our study and control groups in terms of externalizing problems (as reported in many other studies on children with parents suffering from CMCs). To clarify this result, we should consider that the Externalizing score in the YSR consists of two clinical subscales for Rule-Breaking Behavior and Aggressive Behavior. While there were clearly no differences between our two groups of children for the first subscale, on the second the study group revealed higher levels of aggressive behavior than the controls, and the difference neared statistical significance with a medium effect size (
To sum up, we found that children and adolescents with a parent suffering from ALS had high levels of internalizing problems but few or no externalizing problems. Looking at the broader picture, having a parent with ALS globally affected the children’s adjustment – given their significantly higher YSR scores for Total Problems. But analyzing the YSR subscales comprising the Total Problems score showed that the study group’s specific reaction pattern was due essentially to internalizing problems and, to a lesser extent, to aggressive behavior.
More in general, our findings are in line with other reports on the profound effect of having a parent with a CMC on a child’s functioning, though the combinations of internalizing and externalizing problems may vary.
The second aim of our study was to test the impact of ALS in parents on their offspring’s personality characteristics using the Rorschach CS, in much the same way as
First, the Rorschach CS confirmed the substantial impact of ALS in a parent in terms of internalizing behavior and depression in their offspring. The DEPI of the Rorschach CS, which indicates the intensity of depressive affects, was significantly higher in our study group than in the control group, and a significant proportion of the former children (45.5%) scored above the clinical threshold pointing to a higher likelihood of subclinical or clinical depression. It is worth emphasizing that the percentage of children above the clinical threshold for depression in the Rorschach CS (45.5%) was very similar to the percentage of children identified by the YSR as having internalizing problems above the clinical cut-off (42.9%). In other words, the self-report and projective measures converged in indicating that more than 40% of children with a parent suffering from ALS have internalizing-depressive problems.
Second, the structural summary of the children in our study group (obtained with the Rorschach CS) revealed several indicators of difficult adjustment and psychological pain. In their projective responses, these children showed more self-disgust, shame, emotional distress, and guilt. They also experienced more negative or blemished features about the self and the world, an undesirable self-image, less well controlled or modulated emotional experiences, and a less healthy and adaptive understanding of others. The Rorschach CS also generated projective indexes of preoccupations with the body, health and illness that did not emerge from the YSR. A psychodynamic interpretation of this inconsistency might be that these children were worried and scared about their parent’s body, health or illness (as indicated by the Rorschach projections), and they coped with their concern by unconsciously adopting defense mechanisms such as repression, denial, or dismissal. In fact, previous research has shown that physical evidence of a parent’s illness and treatment (e.g., a mother’s hair loss due to treatment for breast cancer;
Overall, the results obtained with the Rorschach CS are particularly congruent with the extant literature on children whose parents have advanced or terminal disease.
Another finding emerging from the Rorschach CS in the present study is that our study group had higher scores in several indexes relating to thought problems. The children in the study group proved less adequate in reality testing, they had more distorted perceptions and signs of serious mediational impairment, along with direct evidence of thought disturbance emerging from several scores included in the PTI (
In our opinion, these indexes should be seen as depending on traumatic events in the children’s history, possibly due to the stress associated with a parent’s illness. Consistently with this interpretation,
Other studies have identified thought problems connected with post-traumatic experiences in the children of ill parents.
The third aim of our study was to explore how parents see their children in terms of problem behavior and competences. According to previous literature, it is common for children and adolescents to report experiencing more problems than their parents perceive in them (
In short, our study shows that school-aged children and adolescents who have a parent with ALS are vulnerable and liable to suffer mainly in terms of internalizing behavior and depressive symptoms, and thought problems as a reaction to their stressful condition. Strikingly, about 40% of our sample had scores in the clinical range for internalizing/depressive problems. The healthy parents seemed to confirm this picture and are probably aware of the impact of their spouse’s ALS on their children.
Several limitations apply to this study. First, the sample size was not large enough to allow for any reliable generalization of our results. Second, the age range of the children considered was rather wide. The small number of children considered also prevented us from assessing any influence of moderators relating to the children (e.g., age and gender) or the situation (e.g., features of the parent’s illness, the patient’s neuropsychological and psychopathological profile, parental functioning, and socioeconomic status) that could help to explain the differences in problem behavior between the study group and the controls. A last, potential limitation lies in that we considered more than one child from the same family, thus violating the assumption of statistical independence of our observations. Having said that, we must remember that ALS is a relatively rare disease and it is consequently very difficult to obtain a large number of participants.
Despite its limitations, to the best of our knowledge this exploratory study is the first to address the impact of ALS in a parent on a child’s adjustment. We believe that the severe psychological consequences of having a parent with ALS emerging from the present study mean that the families affected may need support to cope with such an overwhelming disease.
Design and conceptualization of the study: AP, GS, VC. Coding of data: AP, FB. Statistical analysis and interpretation of the data: VC. Drafting the manuscript: VC, FB. Revising the manuscript for intellectual content: VC, FB, EB, MS, MM, CF, GQ, GS, AP. Final approval of the version to be published: VC, FB, EB, MS, MM, CF, GQ, GS, AP. Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved: VC, FB, EB, MS, MM, CF, GQ, GS, and AP.
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
We are grateful to all participants for their efforts. We also thank Viviana Cristillo, Teresa Ferrantino, Carla Volo, Valentina Parrino, and Frances Coburn for their skillful assistance. This study received no funding from third parties.