B E H A V IO R A L P E D IA T R IC S
RichardW.Olmsted, Editor
Emotional adjustment of adolescents and young adults with cystic fibrosis Twenty-seven adolescents and young adults with cystic fibrosis were studied to evahtate the psychological impact of this chronic illness. At first glance, most patients appeared to function adequately on a daily basis. However, four sources of psychological stress, leading to emotional disturbance, were identified: altered physical appearance causing distorted body images and denial of sexuality, strained interpersonal relationships resuhing in isolation and mental strain, conflicts in upbringing, and increased awareness of t/~efuture and of death. GuMelines for the physician treating these young adults and their families include: (1) encouragement for greater involvement by the patient's father; (2) assisting the mother to find outside interests and to allow more independence to the patient," (3) stressing communication about cystic fibrosis within the home," (4) emphasizing outside aetivity for each patient," (5) repeated discussions of the patient's concerns while emphasizing his strengths; (6) anticipation of problems, specific to cystic fibrosis, such as sterility in males," and (7) encouragement of interpatient communication.
Ivy R. Boyle, M.D., Paul A. di Sant'Agnese, M.D.,* Sallyaun Sack, Ph.D., F r a n c e s Milliean, M.D., and Lueas L. Kulezyeki, M.D.,
Bethesda, Md.,
and Washington, D.C.
THE CHRONICITY and eventual fatal outcome of cystic fibrosis are sources of severe psychological stress for patients and their families. Several studies 1-' have explored the psychological impact of this disease on children. Now, however, many CF patients are living to adolescence and to early adulthood, and they often are able to remain physically active. These older patients face new demands. Needs for independence and for peer relationships take on greater significance, and decisions about work, family, and future become paramount, In order to deal with their concerns, CF patients need physicians to whom they can turn for understanding and guidance. Physicians must be aware of the special problems of these patients in order to give them appropriate help and support. Accordingly, we have undertaken a From the Pediatric Metabolism Branch, National Institute of Arthritis, Metabolism, and Digestive Diseases, National Institutes of Health, and Children's Hospital. *Reprint address: P,A. di Sant'Agnese, M.D., Building 10, Room 8N250, National Institutes of Health, Bethesda, Md. 20014.
318
The Journal of P E D l A T R I C S Vol. 88, No, 2, pp. 318-326
study of our adolescent and young adult CF population and have examined their emotional strengths and weaknesses and their ability to cope with chronic illness in the hope of being able to devise guidelines for the physician caring for patients with this illness.
MATERIALS AND M E T H O D S Twenty-seven patients, ages 13 to 30 years, with CF diagnosed by sweat tests, duodenal intubation, and evidence of chronic pulmonary disease, were studied (Table I). Twenty-two patients were followed at the National Institutes of Health and five (Cases-1,6,7,14, and 22) at Children's Hospital of the District of Columbia. Each patient had a psychiatric interview and was evaluated for performance at school or work, quality of interpersonal relationships, interactions with members of his family, and style of coping with illness. Psychological tests administered included the Wechsler Adult Intelligence Scale (for patients over t6 years of age) or the Wisconsin Intelligence Scale for Children (for those under 16), the Thematic Apperception Test, the Rohrshach Test, and the Draw-a-Person Test.
Volume 88 Number 2
Emotional adjustment in cystic fibrosis
3 19
Whenever possible the patient's mother was interviewed (21 out of 25 mothers were seen) and was asked about herself, her family, and her relationship with the patient. An attempt was also made to see fathers of our patients. Because of scheduling difficulties only four fathers were seen. The 27 patients came from 25 families. Two patients were sisters (Cases 16 and 19) and two were identical twin brothers (Cases 10 and 11). Nineteen of our patients (five over age 18) were still living with their parents. Of those not living with parents (eight patients) six were married and two lived alone. RESULTS Pertinent clinical data as well as the results of this study are summarized in Table I. Daily timctioning. Judging the patients on daily performance, our initial impression was that they were doing quite well. Intellectual functioning was above average: IQ's ranged from 82 to 150 with a mean of 113 and a standard deviation of 14.6 (standard error of mean: 2.8). Seventy per cent of the adolescents were at their proper high school level, and 11 of the 13 adults had had some college training. Only three patients (Cases 3,6, and 14), all adolescents, were homebound: although theoretically attending school, all three were actually far below their peer group in performance. Two thirds of the patients studied were competently handling all daily tasks of living and were able to function satisfactorily at school or work, or at home (Table I). Of this group, 70% were honor students in school, and those working were successful in their jobs. Interestingly, of all patients performing well on the basis of their clinical score,'". " physical condition was rated "poor" (below 50) for three and only "fair" (below 60) for another seven. Thus even patients who were physically quite ill were often able to function adequately in daily life. Psychological stresses. In contrast to their good daily performance, however, emotional adjustment in these patients was often impaired. Interviews as well as psychological tests revealed much anxiety and depression. There was no relationship between IQ and the patient's ability to cope emotionally. We felt that there were four main sources of stress causing psychological difficulties: altered physical appearance, strained interpersonal relationships, conflicts in upbringing, and increased awareness of the future. Physical appearance. Most of the patients looked significantly different from others of their age group. Seventy per cent of the adolescents were short, and development of secondary sexual characteristics was often delayed. Later on, even if growth achieved normal limits, there
I
i
I
Fig. 1. Figures drawn by a normal control subject, age 15 years, IQ 116. were outstanding differences associated with chronic respiratory illness, such as coughing, excessive thinness, and obvious difficulty breathing. In interviews, all patients expressed dissatisfaction with their bodies. Although most were adept at using clothing and hairstyles to disguise their emaciation, a few had simply given up. Two adolescents (Cases 5 and 6) preferred to remain in the house, far from the stares of others. One (Case 6) would not even take baths; she so disliked looking at her body. The clearest view of the patients' body concept was obtained from the figure drawings. Of 27 patients, 17 made drawings showing striking denial of sexual differences. Their male and female figures, except for length of hair and possibly the addition of a skirt, were indistinguishable. In Fig. 1 are "normal" figure drawings of a healthy adolescent, age 15 years, with no physical deform-
320
Boyle et al.
The Journal of Pediatrics February 1976
Table I
Percentile Case No.
Sex
Age (yr)
Weight
Clinical score*
IQ
l 2 3
M F F
13 13 14
3 95
RichardW.Olmsted, Editor
Emotional adjustment of adolescents and young adults with cystic fibrosis Twenty-seven adolescents and young adults with cystic fibrosis were studied to evahtate the psychological impact of this chronic illness. At first glance, most patients appeared to function adequately on a daily basis. However, four sources of psychological stress, leading to emotional disturbance, were identified: altered physical appearance causing distorted body images and denial of sexuality, strained interpersonal relationships resuhing in isolation and mental strain, conflicts in upbringing, and increased awareness of t/~efuture and of death. GuMelines for the physician treating these young adults and their families include: (1) encouragement for greater involvement by the patient's father; (2) assisting the mother to find outside interests and to allow more independence to the patient," (3) stressing communication about cystic fibrosis within the home," (4) emphasizing outside aetivity for each patient," (5) repeated discussions of the patient's concerns while emphasizing his strengths; (6) anticipation of problems, specific to cystic fibrosis, such as sterility in males," and (7) encouragement of interpatient communication.
Ivy R. Boyle, M.D., Paul A. di Sant'Agnese, M.D.,* Sallyaun Sack, Ph.D., F r a n c e s Milliean, M.D., and Lueas L. Kulezyeki, M.D.,
Bethesda, Md.,
and Washington, D.C.
THE CHRONICITY and eventual fatal outcome of cystic fibrosis are sources of severe psychological stress for patients and their families. Several studies 1-' have explored the psychological impact of this disease on children. Now, however, many CF patients are living to adolescence and to early adulthood, and they often are able to remain physically active. These older patients face new demands. Needs for independence and for peer relationships take on greater significance, and decisions about work, family, and future become paramount, In order to deal with their concerns, CF patients need physicians to whom they can turn for understanding and guidance. Physicians must be aware of the special problems of these patients in order to give them appropriate help and support. Accordingly, we have undertaken a From the Pediatric Metabolism Branch, National Institute of Arthritis, Metabolism, and Digestive Diseases, National Institutes of Health, and Children's Hospital. *Reprint address: P,A. di Sant'Agnese, M.D., Building 10, Room 8N250, National Institutes of Health, Bethesda, Md. 20014.
318
The Journal of P E D l A T R I C S Vol. 88, No, 2, pp. 318-326
study of our adolescent and young adult CF population and have examined their emotional strengths and weaknesses and their ability to cope with chronic illness in the hope of being able to devise guidelines for the physician caring for patients with this illness.
MATERIALS AND M E T H O D S Twenty-seven patients, ages 13 to 30 years, with CF diagnosed by sweat tests, duodenal intubation, and evidence of chronic pulmonary disease, were studied (Table I). Twenty-two patients were followed at the National Institutes of Health and five (Cases-1,6,7,14, and 22) at Children's Hospital of the District of Columbia. Each patient had a psychiatric interview and was evaluated for performance at school or work, quality of interpersonal relationships, interactions with members of his family, and style of coping with illness. Psychological tests administered included the Wechsler Adult Intelligence Scale (for patients over t6 years of age) or the Wisconsin Intelligence Scale for Children (for those under 16), the Thematic Apperception Test, the Rohrshach Test, and the Draw-a-Person Test.
Volume 88 Number 2
Emotional adjustment in cystic fibrosis
3 19
Whenever possible the patient's mother was interviewed (21 out of 25 mothers were seen) and was asked about herself, her family, and her relationship with the patient. An attempt was also made to see fathers of our patients. Because of scheduling difficulties only four fathers were seen. The 27 patients came from 25 families. Two patients were sisters (Cases 16 and 19) and two were identical twin brothers (Cases 10 and 11). Nineteen of our patients (five over age 18) were still living with their parents. Of those not living with parents (eight patients) six were married and two lived alone. RESULTS Pertinent clinical data as well as the results of this study are summarized in Table I. Daily timctioning. Judging the patients on daily performance, our initial impression was that they were doing quite well. Intellectual functioning was above average: IQ's ranged from 82 to 150 with a mean of 113 and a standard deviation of 14.6 (standard error of mean: 2.8). Seventy per cent of the adolescents were at their proper high school level, and 11 of the 13 adults had had some college training. Only three patients (Cases 3,6, and 14), all adolescents, were homebound: although theoretically attending school, all three were actually far below their peer group in performance. Two thirds of the patients studied were competently handling all daily tasks of living and were able to function satisfactorily at school or work, or at home (Table I). Of this group, 70% were honor students in school, and those working were successful in their jobs. Interestingly, of all patients performing well on the basis of their clinical score,'". " physical condition was rated "poor" (below 50) for three and only "fair" (below 60) for another seven. Thus even patients who were physically quite ill were often able to function adequately in daily life. Psychological stresses. In contrast to their good daily performance, however, emotional adjustment in these patients was often impaired. Interviews as well as psychological tests revealed much anxiety and depression. There was no relationship between IQ and the patient's ability to cope emotionally. We felt that there were four main sources of stress causing psychological difficulties: altered physical appearance, strained interpersonal relationships, conflicts in upbringing, and increased awareness of the future. Physical appearance. Most of the patients looked significantly different from others of their age group. Seventy per cent of the adolescents were short, and development of secondary sexual characteristics was often delayed. Later on, even if growth achieved normal limits, there
I
i
I
Fig. 1. Figures drawn by a normal control subject, age 15 years, IQ 116. were outstanding differences associated with chronic respiratory illness, such as coughing, excessive thinness, and obvious difficulty breathing. In interviews, all patients expressed dissatisfaction with their bodies. Although most were adept at using clothing and hairstyles to disguise their emaciation, a few had simply given up. Two adolescents (Cases 5 and 6) preferred to remain in the house, far from the stares of others. One (Case 6) would not even take baths; she so disliked looking at her body. The clearest view of the patients' body concept was obtained from the figure drawings. Of 27 patients, 17 made drawings showing striking denial of sexual differences. Their male and female figures, except for length of hair and possibly the addition of a skirt, were indistinguishable. In Fig. 1 are "normal" figure drawings of a healthy adolescent, age 15 years, with no physical deform-
320
Boyle et al.
The Journal of Pediatrics February 1976
Table I
Percentile Case No.
Sex
Age (yr)
Weight
Clinical score*
IQ
l 2 3
M F F
13 13 14
3 95
