Female sexual health care in cystic fibrosis

Fifty five teenage girls with cystic fibrosis and their mothers were interviewed to assess the provision of sexual health information. Parents were the most common source of information for adolescents. The cystic fibrosis doctor was identified as the key resource for parents. Yet few parents had spoken to their doctor about these issues, and 96% requested more information. This information was wanted before puberty by mothers, and from puberty onwards by girls.     

Global and specific disease-related information needs of cystic fibrosis patients and their families

A cross-sectional survey of 60 families with a child with cystic fibrosis was undertaken to assess how much family members wanted to be told about the illness, to determine specific information needs concerning selected medical and psychosocial topics, and to determine whether this information had been provided in the past. Self-administered index measures were designed for this purpose. Most family members wanted complete and comprehensive information about cystic fibrosis. A minority of family members claimed they had not received basic medical information. Fathers and siblings wanted more medical information than mothers and patients. The majority of parents and patients wanted a great deal more information on the possible effects of cystic fibrosis on the patient's career, social life, marriage, and reproductive capacity. In general, family members had been given more medical than psychosocial information. Index measures were independent of the effects of patients' age, Shwachman score, and period since diagnosis. Social class was significantly associated with fathers' index measures of their specific information needs and information not given previously.     

Cystic fibrosis and family stress: effects of age and severity of illness

Stress and adjustment in mothers of children with cystic fibrosis was compared with that in a control group of mothers of healthy children. Mothers of children in four age groups were included: preschool, middle childhood, early adolescence, and late adolescence. Mothers of children with cystic fibrosis did not report significantly higher levels of stress than did the control group mothers; nor did they report greater feelings of inadequacy as parents. However, mothers of children with cystic fibrosis in two age groups, preschool and early adolescence, scored higher on a measure of depression than did mothers of healthy children in the same age groups. The relationship of illness severity to maternal stress and adjustment was examined in the cystic fibrosis group. The mother's subjective rating of the child's illness severity was a better indicator of her reported stress than was the Schwachman clinical rating. It appears that many mothers are able to adapt to the presence of cystic fibrosis in the family, although certain periods in the child's life and perceived increases in illness severity are associated with increased maternal distress.     

Adolescents with cystic fibrosis: psychosocial adjustment

Patients aged between 12 and 16 years with cystic fibrosis were interviewed, as were their parents, to find out if psychosocial problems existed. All 27 of them appeared to be well adjusted and none had special problems at school; they were not socially isolated, and family relationships seemed to be good. Contributing factors were good communications patterns within the family and perhaps denial of the ultimate outcome of the disease, since few patients wished to know more about cystic fibrosis. Comparison with a group of healthy adolescents and their mothers showed that the patients had a tendency (not significant) towards depression. Among the mothers however, the incidence of depression was slightly greater. It was concluded that psychiatric intervention should only be undertaken cautiously with adolescents, but that special help should be given to the mothers as they appeared to carry the main burden of coping with the illness.     

Adolescents with cystic fibrosis: psychosocial adjustment.

Patients aged between 12 and 16 years with cystic fibrosis were interviewed, as were their parents, to find out if psychosocial problems existed. All 27 of them appeared to be well adjusted and none had special problems at school; they were not socially isolated, and family relationships seemed to be good. Contributing factors were good communications patterns within the family and perhaps denial of the ultimate outcome of the disease, since few patients wished to know more about cystic fibrosis. Comparison with a group of healthy adolescents and their mothers showed that the patients had a tendency (not significant) towards depression. Among the mothers however, the incidence of depression was slightly greater. It was concluded that psychiatric intervention should only be undertaken cautiously with adolescents, but that special help should be given to the mothers as they appeared to carry the main burden of coping with the illness.     

Plasma and urinary catecholamines in patients with cystic fibrosis

In 43 patients with cystic fibrosis (age 8-23 yr, 26 boys and 17 girls) attending a summer camp in a mountain rehabilitation center and in 25 parents (heterozygotes) plasma epinephrine, norepinephrine, dopamine and plasma activity of dopamine-beta-hydroxylase were determined as well as the 24-h excretion of the free urinary amines (epinephrine, norepinephrine, dopamine), their O-methylated products (metanephrine, normetanephrine, 3-methoxytyramine) and the urinary phenolic acids (vanilmandelic and homovanillic). Also the metabolic breakdown product of serotonin in urine, the 5-hydroxyindoleacetic acid, was determined. Significantly elevated plasma dopamine (0.03-0.45 nmol/liter for controls versus 1.70-2.21 nmol/liter for cystic fibrosis) and slightly higher plasma norepinephrine levels were found in patients with cystic fibrosis. An increased 5-hydroxyindoleacetic acid excretion was noticed in adolescent patients which correlated with the disease state and the extent of lung involvement. No abnormalities of plasma amine levels were seen in the parents of the patients. Despite controversial results, CF patients seem to have an alteration in catecholamine metabolism which is reflected in higher plasma dopamine levels.     

Knowledge of cystic fibrosis in patients and their parents

A standardized questionnaire was used to assess knowledge about cystic fibrosis in 28 patients with cystic fibrosis (aged 10 to 21 years) and in the parents of 25 of these patients. Knowledge of disease pathophysiology and treatment was generally comprehensive and detailed; knowledge of the genetics was fair. However, there were conspicuous deficits in the awareness of reproductive risks and of male sterility. Significant predictors of patient knowledge were patient age, sex, and educational level and parental age. Predictors of parent knowledge were Shwachman score, socioeconomic status, and sex of responding parent. Patients rely heavily on parents for information about cystic fibrosis. More than one third of both patients and parents sought more information about the disease and its implications.     

Persistent or recurrent pneumonia in Saudi children seen at King Khalid University Hospital, Riyadh: clinical profile and some predisposing factors.

Children with persistent or recurrent pneumonia without an apparent cause constitute an important clinical category with much morbidity and mortality and can be perplexing and frustrating to the treating physician and the parents. To identify the clinical profile and predisposing factors in this group of patients, 18 Saudi children were studied. Their ages ranged from 3 months to 12 years (mean age 5.7 years) with male preponderance--12 boys and 6 girls (M:F ratio 2:1). About 44.4% had immune and inherited metabolic disorders. Anatomical abnormalities were found in four (22.2%). Two had measles as a predisposing factor. None had tuberculosis or pertussis. One child each had pulmonary candidiasis and laryngeal papilloma, probably contracted from their mothers as congenital infections. Though the pattern seems to follow that found in developed countries, it is noteworthy that cystic fibrosis was not identified in any of our patients.     

Transition from paediatric to adult care for persons with cystic fibrosis: patient and parent perspectives

OBJECTIVES: To gauge the perspectives of adolescents and adults with cystic fibrosis (CF) and their parents regarding the transition from paediatric to adult-oriented health care. METHODS: Cross-sectional survey using an anonymous, semi-structured questionnaire. The study population consisted of adolescents and adults attending a paediatric and an adult CF clinic in Cape Town, South Africa and their parents. RESULTS: Forty-seven of the 61 subjects completed the questionnaire (response rate 77%). Autonomy in health care was 'extremely important' to most persons with CF. Transfer at the age of 16-18 years of age was the preferred option for most respondents. Whereas over 80% of parents felt their children needed more CF-related information, only 38% of adolescents expressed this need (P < 0.05). Adolescents also reported little need for general health information. More than 80% of respondents were 'unsure' about transfer. Over 90% felt that a transition clinic would be useful. As viewed by the respondents, its main purpose would be to provide information about the adult clinic and an opportunity to meet the CF doctor in the adult clinic. CONCLUSION: There are significant concerns about the transition process in this population. Given the expressed need for autonomy and a transition clinic, the basis for a smoother transition in the future has been laid.     

Testing carrier status in siblings of patients with cystic fibrosis

Altogether 114 parents of patients attending a cystic fibrosis clinic and 27 regional genetics units were surveyed for their views on whether healthy siblings should be tested for carrier status during childhood. Most parents wanted to know their child's carrier status and felt it was their right; almost all would tell the children if they were carriers. However, 37% of the units never tested siblings and 40% said the parents had no right to this knowledge. Furthermore, 60% would withhold the information from parents.     

Parent interview findings regarding the impact of cystic fibrosis on families

An assessment of the impact of cystic fibrosis (CF) was conducted with 43 families. Semistructured parental interviews on family functioning, parent-child interactions, sibling and peer relationships, and medical issues were coded by two independent raters to identify "major," "minor", or "no" problems. Of the 62 questions presented, only 8 were viewed by more than 10% of parents as "major problems." The impact of hospitalization upon parents was the most prevalent "major problem." Parental communication was a "major problem" for 28% of the mothers but for only one father. Ten to 15% of the parents described "major problems" related to: their marital relationship, accepting the illness, feeling they should do more for their child with CF, feeling their other children had been deprived or complained about inattention, or their relationship with the ill child's grandparents. While most families were generally coping successfully, health care professionals should be alert to specific areas of potential problems.     

Pelvic ultrasonography in girls with precocious puberty, congenital adrenal hyperplasia, obesity, or hirsutism

Real-time ultrasonography of the pelvic organs was performed on 151 girls with various complete and incomplete forms of precocious puberty, 20 girls with congenital adrenal hyperplasia, 20 with hirsutism, 18 with obesity, and 133 age-matched normal girls. Uterine and ovarian volumes were calculated and the ovarian morphologic picture was classified as homogeneous, nonhomogeneous (less than three small cystic areas), microcystic (four or more small cystic areas less than 9 mm in diameter), follicular (at least one cystic area greater than 9 mm), and macrocystic (large cystic area greater than 20 mm). Ultrasound imaging allowed an easy distinction between true precocious puberty and premature thelarche or idiopathic premature adrenarche. It was also helpful in the diagnosis of transient sexual precocity, although in these cases the differential diagnosis of precocious puberty can be difficult. In postmenarcheal patients with congenital adrenal hyperplasia, ultrasound study showed a low uterine volume and, frequently, a macrocyst in the ovary. In hirsute girls and in a few obese patients, ovaries had an increased volume and a microcystic structure, similar to those in polycystic ovary syndrome. Pelvic ultrasonography can be useful not only in diagnosing disorders in sexual development but also for greater understanding of the pathogenesis of these and other disorders.     

Vocal communication between parents and infants

Mothers and fathers of 20 boys and 20 girls representing all social classes were filmed when playing with their 6-month-old infants in a standardized playing situation. The vocal behavior of the parents and their infants was analyzed for sex differences. In addition, vocal activity was compared in parents and infants. Mothers showed a higher vocal activity than fathers together with their infants. Mothers also received more vocal activity from their infants than the fathers did. Fathers took more vocal initiatives towards girls than towards boys. Parents were vocally more active than infants. Comparing the vocal irritability of boys and girls, no differences were found. Fathers and mothers did not differ in vocal responsiveness to vocal irritability. Girls received more vocal initiations from fathers than boys while mothers treated boys and girls vocally equally.     

Gender affects self-evaluation in children with cystic fibrosis and their healthy siblings

AIM: To determine whether self-esteem among children with cystic fibrosis (CF) and their healthy siblings differs from that of a healthy reference group and whether there are differences within and between sibling pairs. METHODS: All Swedish CF children 6-14 y old with a healthy sibling in the same age range (n=65) were invited to participate, 55 sibling pairs and their parents taking part in the study. Five aspects of the children's self-concept-physical characteristics, skills and talents, mental well-being, relations to parents and family, and relations to others-were assessed by the "I think I am" self-evaluation questionnaire. Severity of illness was assessed by means of the Shwachman Clinical Evaluation System. RESULTS: Whereas self-evaluation did not differ between groups at a general level, healthy girls as well as those with CF scored lower than girls in the reference group on the "mental well-being" and "relations to parents and family" subscales. Comparison of gender combinations (sick girl/healthy boy, sick girl/healthy girl, sick boy/healthy boy, sick boy/healthy girl) suggested that girls pay a cost of a lesser sense of psychological well-being and feelings of inadequacy in relation to their parents and family. The Shwachman score of the sick child was not related to the level of self-esteem. CONCLUSION: When CF is present among siblings, girls seem to carry more of the family pain than boys, a finding that calls for an increased awareness of the girls' situation by members of care teams.     

13C and H2 breath tests to study extent and site of starch digestion in children with cystic fibrosis.

BACKGROUND: Starch is an important source of energy for children with cystic fibrosis, but little is known about their capacity to digest it. METHODS: A 13C breath test was used to measure starch digestion and oxidation in 16 children with cystic fibrosis (median [range] age, 7.9 [4-15] years; 7 girls, 9 boys) and 5 normal healthy control subjects (median age, 8.3 [7-13] years; 3 girls, 2 boys). A test meal of 13C flour and lactulose was consumed and breath samples were obtained half-hourly thereafter for 6 hours to measure 13C enrichment by isotope ratio mass spectrometry and H2 by electrochemistry. The test was repeated on 10 children with cystic fibrosis when they were taking pancreatic supplements. RESULTS: The median (range) cumulative percentage 13C dose recovery (cPDR), was 35% (18-52%) in control subjects, 18% (9-33%) in children with cystic fibrosis without enzymes, and 29% (22-51%) in those with pancreatic supplements. cPDR differed significantly between healthy control subjects and children with cystic fibrosis without enzymes (p = 0.01) and between children with cystic fibrosis with and without enzymes (p < 0.0001), but there was no difference between control subjects and children with cystic fibrosis taking enzymes (p = 0.5). Eight children with cystic fibrosis had a cPDR within control range, and in six there was a second peak in 13CO2 enrichment coincident with an increase in H2. CONCLUSIONS: Starch digestion and oxidation are diminished in children with cystic fibrosis, but pancreatic enzymes restored them to near normal levels. A second peak in 13CO2 enrichment, suggestive of colonic starch fermentation was absent in healthy children, but present in some children with cystic fibrosis and abolished by pancreatic enzymes.     

FAMILY RESPONSE TO CYSTIC FIBROSIS

The effects of cystic fibrosis on 50 Victorian families with one or more affected children were examined in a study carried out between August 1972 and February 1973. The social and emotional problems experienced by family members, particularly the patients and mothers, are reported on. At all ages, socially embarrassing symptoms and awareness of being different create major problems for the patient. The uncertainty of prognosis and doubts about the prospects of employment are specially relevant for the teenager. It is suggested that the patient be given ample opportunity, from early adolescence onward, to discuss all aspects of his disease, and that more appropriate provision should be made for the total care of adult patients. Many problems, such as depression, feelings of isolation and the physical demands of constant treatment, which the parents of children with cystic fibrosis experience, are probably similar to those confronting parents of other chronically ill and handicapped children. But special difficulties stem from the genetic basis of cystic fibrosis and from the knowledge of an eventually fatal outcome. The roles of professional members of the medical team are touched on, particularly in relation to genetic counselling and the provision of practical and emotional support to parents. Comments are made on the establishment of lay organisations. The financial strain associated with obtaining regular medical attention was looked at, and suggestions are made for minimizing the economic problems of certain vulnerable families.     

Effects of newborn screening of cystic fibrosis on reported maternal behaviour.

Screening for cystic fibrosis is highly controversial. Concerns have been expressed that newborn screening may cause mothers, who had considered their child to be healthy before diagnosis, to overprotect their child. Some critics of screening also suggest that a period of delay from onset of symptoms to diagnosis may help a mother adjust to the reality of the child''s lethal condition. This study compared the strength of overprotective child rearing attitudes of 29 mothers whose children were screened (13 had symptomatic children and 16 asymptomatic children) with the attitudes of 29 mothers whose children were diagnosed after the onset of symptoms. Results indicate that newborn screening had not increased a mother''s tendency to overprotect her child with cystic fibrosis and in some cases the tendency had decreased. Further, delay in diagnosis when screening was not conducted usually caused mothers considerable personal distress.     

Influence of education and residence on the parental search for pediatric surgical information on the internet

BACKGROUNDThe internet is a valuable tool for access to health-related information. There is limited literature regarding its use by parents of children with surgical conditions. AIMTo investigate internet usage by parents seeking information about the surgical conditions of their offspring in relation to epidemiological factors such as family residential area and parental educational level and to subsequently review the literature regarding this topic. METHODSAn anonymous questionnaire about internet usage was completed by eligible parents of children who were admitted to our clinic for minor surgical procedures during a six-month period. RESULTSOur results demonstrated that the internet has been mostly used by mothers for children’s health information. Google was the most commonly used search engine, while pediatricians were the first parental choice for ‘live’ information. Only one-quarter of the parents informed their doctor about the information found online. Nine of ten parents had a positive opinion of an official website managed by the doctors of our clinic. Our results mostly agreed with the international literature. CONCLUSIONIn conclusion, the establishment of official websites (designed and managed by specialists) that parents can access to receive appropriate health information is mandatory in the internet era.     

Incidence of Cystic Fibrosis in Ethnic Italians and Greeks and in Australians of Predominantly British Origin

The incidence of cystic fibrosis among the Australian born children of parents born in Italy was 1: 3 625 live births and among Australian born children of parents born in Greece 1: 3726. These incidences were significantly lower than the incidence for cystic fibrosis of 1: 2021 in children born to Australian born parents, about 90% of whom have ancestors born in the British Isles. The figures suggest there are significant variations in the incidence of cystic fibrosis in different European populations.     

Incidence of cystic fibrosis in ethnic Italians and Greeks and in Australians of predominantly British origin

The incidence of cystic fibrosis among the Australian born children of parents born in Italy was 1:3 625 live births and among Australian born children of parents born in Greece 1:3 726. These incidences were significantly lower than the incidence for cystic fibrosis of 1:2 021 in children born to Australian born parents, about 90% of whom have ancestors born in the British Isles. The figures suggest there are significant variations in the incidence of cystic fibrosis in different European populations.