Well sibling psychological adjustment to chronic physical disorder in a sibling: how important is maternal awareness of their illness attitudes and perceptions?

The psychological adjustment of healthy siblings was investigated in relation to their attitudes and perceptions about their brother's or sister's chronic physical disorder, to their mothers' awareness of these attitudes and perceptions, and to three other maternal factors (maternal distress, maternal social support, and amount of care demanded by the physical disorder). Sixty-two well siblings and mothers of children with a range of chronic physical disorders completed standardised questionnaires. The majority of siblings did not appear to have adjustment problems, although the sample had slightly increased rates of emotional symptoms compared to the general population. Mothers rated well siblings as having more negative attitudes and perceptions about the physical disorder than reported by siblings themselves. A multiple regression analysis indicated that better sibling adjustment was associated with higher maternal awareness of their attitudes and perceptions. These findings support Varni and Wallander's (1998) model that emphasises the role of relationship and attitude variables in child adjustment to chronic physical disorder. The implications of these findings for clinical practice are discussed.

Estimating resources for setting up an HV service for postnatal depression.

Postnatal depression (PND) presents a significant health burden for mothers and their offspring. Research has indicated that screening for PND increases detection (Holden et al, 1989) and also that counselling by health visitors (HVs) is an effective intervention. This article describes a pilot PND screening and treatment project which aimed to assess the resource and organizational implications of introducing new health visiting services modelled on research findings compared with existing practice. The pilot project included approximately 400 new mothers in an inner city multicultural area and confirmed previous findings regarding prevalence of PND (19%) and the effectiveness of brief counselling interventions. It resulted in significantly reduced estimates of the HV resources that would be required to introduce screening as part of the core health-visiting programme. The project also revised expected training requirements and challenged existing concerns about its appropriateness for minority cultural groups.

Educational progress, behaviour, and motor skills at 10 years in early treated congenital hypothyroidism.

AIM: To assess educational attainments, behaviour, and motor skills at 10 years of age in a group of children with congenital hypothyroidism identified by neonatal screening. SUBJECTS: 59 children with congenital hypothyroidism born in 1978-81, 31 cases with pretreatment thyroxine (T4) values of 40 nmol/l or below (group I) and 28 less severe cases with T4 values over 40 nmol/l (group II), together with 59 classroom control children matched for age, sex, social class, and main language spoken at home. METHODS: The Neale analysis of reading ability; the child health and education study written test of mathematics; Rutter behaviour questionnaires for parents and teachers; the Oseretsky test of motor proficiency (short form). RESULTS: On all measures the congenital hypothyroidism children in group I had less satisfactory scores for educational attainments, behaviour, and motor skills than those in group II and controls. For reading the differences were small and did not reach statistical significance, but the deficits in mathematics and total motor skills were statistically significant (p < 0.01). There were more striking and statistically significant differences in behaviour scores, particularly with respect to attentional difficulties. Although less striking, these were also apparent in the group II children with mild hypothyroidism. CONCLUSIONS: At the age of 10 years severe congenital hypothyroidism is associated with some mild impairment in educational and motor attainments. Behaviour problems are also common, even in some children with less severe congenital hypothyroidism.

Pain, quality of life, and coping in sickle cell disease.

This study examined the frequency and severity of sickle related pain, its impact on quality of life, and methods of coping for 25 children with sickle cell disease, aged 6-16 years. Subjects were matched with non-affected peers and asked to complete the Central Middlesex Hospital Children's Health Diary for four weeks. Results indicated that sickle pain occurred on average one in 14 days, and total summary pain scores indicated significantly greater pain than for controls. Children with sickle cell disease could discriminate sickle pain and did not adopt sick role responses to ordinary childhood ailments. Nearly all sickle pain was dealt with at home. Sickle pain resulted in over seven times increased risk of not attending school and was highly disruptive of social and recreational activities. Careful assessment of sickle pain in the home environment is an essential part of a community focused pain management service, which effectively supports children's resilience and improves their quality of life.

Psychological adjustment and family functioning in a group of British children with sickle cell disease: preliminary empirical findings and a meta-analysis.

Sickle cell disease (SCD) is a family of inherited blood disorders of variable severity which have in common haemolytic anaemia, recurrent painful crises, end-organ failure and the risk of reduced life expectancy. In Britain, the condition predominantly occurs among families of African or Afro-Caribbean origin. This study examines the effects of the condition on the psychological adjustment and family functioning of 39 children with SCD and 24 control children. Participants were assessed on measures of behaviour, depression, self-esteem, IQ and reading skills, family relationships and maternal mental health. Assessment on the Family Environment Scale showed that SCD children came from families who reported more cohesion and less conflict than did the families of controls. Maternal mental health showed no significant differences between the SCD group and controls. Children with SCD showed an IQ deficit of five points, a difference that was not statistically significant. However, a meta-analysis of six studies in the literature, including this one, did show a highly significantly decreased intellectual ability. SCD children did not show significant differences from controls on measures of depression and self-esteem. However, differences in behavioural problems were found between the three groups, with the SC group showing more behavioural problems. Results of regression analyses suggest that maternal mental health is associated with children's behavioural problems.

Mild impairment of neuro-otological function in early treated congenital hypothyroidism.

Pure tone audiometry, tympanometry, acoustic stapedial reflex thresholds (ASRTs), and auditory evoked brain stem responses (AEBRs) were carried out in 38 children with early treated congenital hypothyroidism aged 10-12 years, together with tests of vestibular function (electronystagraphy, rotational, and caloric tests). Sensorineural hearing loss with thresholds of greater than 15 dB was detected in 18 children (10 at 8 kHz only); only two children had more than 40 dB hearing loss, each in one ear. Raised ASRTs were found in eight children and two children had abnormal AEBRs. Of the 29 children tested, 12 had an abnormality of vestibular function. Although not significant at the 5% level, there was a tendency for the abnormalities to be more prevalent and severe in the children with more severe hypothyroidism, as judged by pretreatment plasma thyroxine. It is concluded that (i) mild abnormality of hearing is still common in children with congenital hypothyroidism despite early treatment but this is much less severe than that found before neonatal screening and (ii) mild abnormalities of vestibular function may be common in early treated congenital hypothyroidism.

Intellectual development at 10 years in early treated congenital hypothyroidism.

Fifty nine children born between 1978 and 1981 with congenital hypothyroidism detected by neonatal screening were assessed at 10 years using the Wechsler intelligence scale for children, together with 59 matched classroom controls. Thirty one children with severe hypothyroidism who had pretreatment plasma thyroxine concentrations of 40 nmol/l or less had a mean (SD) full scale IQ score of 104.7 (15.1), compared with a mean (SD) score of 114.6 (16.3) for the 28 less severely affected children who had pretreatment thyroxine levels greater than 40 nmol/l, and mean (SD) scores of 114.5 (12.8) and 114.8 (13.8) respectively for the 31 and 28 control children. In the hypothyroid children the IQ scores at 10 years were closely related to the IQ scores at 5 years and at 3 years. It is concluded that the deficit in IQ score found at 3 and 5 years in children with severe hypothyroidism is still evident at the age of 10 years.

Relation between biochemical severity and intelligence in early treated congenital hypothyroidism: a threshold effect.

OBJECTIVES: To assess whether early treatment of congenital hypothyroidism fully prevents intellectual impairment. DESIGN: A national register of children with congenital hypothyroidism who were compared with unaffected children from the same school classes and matched for age, sex, social class, and first language. SETTING: First three years (1982-4) of a neonatal screening programme in England, Wales, and Northern Ireland. SUBJECTS: 361 children with congenital hypothyroidism given early treatment and 315 control children. MAIN OUTCOME MEASURES: Intelligence quotient (IQ) measured at school entry at 5 years of age with the Wechsler preschool and primary scale of intelligence. RESULTS: There was a discontinuous relation between IQ and plasma thyroxine concentration at diagnosis, with a threshold at 42.8 nmol/l (95% confidence interval 35.2 to 47.1 nmol/l). Hypothyroid children with thyroxine values below 42.8 nmol/l had a mean IQ 10.3 points (6.9 to 13.7 points) lower than those with higher values and than controls. None of the measures of quality of treatment (age at start of treatment (range 1-173 days), average thyroxine dose (12-76 micrograms in the first year), average thyroxine concentration during treatment (79-234 nmol/l in the first year), and thyroxine concentration less than 103 nmol/l at least once during the first year) influenced IQ at age 5. CONCLUSIONS: Despite early treatment in congenital hypothyroidism the disease severity has a threshold effect on brain development, probably determined prenatally. The 55% of infants with more severe disease continue to show clinically significant intellectual impairment; infants with milder disease show no such impairment. The findings predict that 10% of early treated infants with severe hypothyroidism, compared with around 40% of those who presented with symptoms in the period before screening began, are likely to require special education.

Increased plasma thyroid stimulating hormone in treated congenital hypothyroidism: relation to severity of hypothyroidism, plasma thyroid hormone status, and daily dose of thyroxine.

Plasma thyroid stimulating hormone (TSH) concentrations obtained during the first four years of treatment in 418 children with congenital hypothyroidism, identified by neonatal screening, were examined in relation to paired measurements of plasma thyroxine (n = 1945), free thyroxine (n = 836), triiodothyronine (n = 480), and free triiodothyronine (n = 231), and estimated daily dose of thyroxine at the time of blood sampling. Overall, plasma TSH was above 7 mU/l in 1280 out of 2960 samples (43%); the percentage was not related to severity of hypothyroidism at diagnosis. Mean values for thyroxine and free thyroxine, and to a lesser extent free triiodothyronine, were consistently lower in samples with TSH concentrations over 7 mU/l and this was the case in patients with either severe or less severe hypothyroidism. Raised TSH concentrations were also associated with lower mean doses of thyroxine (micrograms/kg/day) but here the mean doses of thyroxine in children with severe hypothyroidism were higher than in the children with less severe hypothyroidism. The mean dose of thyroxine associated with low/normal TSH values was highest in the first 6 months and fell progressively. Thyroxine dose was significantly related to thyroxine and free thyroxine concentrations but not to triiodothyronine and free triiodothyronine and the latter appeared to be of limited value as measures of plasma thyroid hormone status during treatment.

Psychosocial aspects of sickle cell disease (SCD) in childhood and adolescence: a review.

This paper reviews the literature on the psychological and social aspects of Sickle Cell Disease (SCD) and discusses the clinical implications of its impact on children and their families. Sickle Cell Disease is a family of blood diseases including sickle cell anaemia (SS), SC disease (SC), and sickle B thalassaemia (SBThal). Research on the psychological and social aspects of SCD, particularly in the UK, has been limited and of varying methodological quality. The psychosocial adaptation of children and adolescents with SCD and their families has been associated with the personality and developmental stage of the child, family attitudes and behaviour, socioeconomic status, and social and environmental support. Concerns about the quality of interpersonal relationships within families have also led to investigations of family characteristics and social networks, and some research studies have pointed to different ways of coping associated with specific network and family structures.

Rising IQ scores in British children: recent evidence.

Between 1987 and 1990 IQ scores for 333 5-year-old white school children were obtained using the Wechsler Pre-School and Primary Scale of Intelligence (WPPSI) and the results were compared with 112 children assessed in 1967. The mean full scale IQ score of 113.3 was 8 points higher than the mean of 105.1 in the 1967 study. These findings indicate that the rate of increase of IQ scores was comparable and probably higher than that reported in the U.S.A. for the period 1932-78. It is important that this large increase is taken into account for correct interpretation of IQ test results.

Congenital hypothyroidism detected by neonatal screening: relationship between biochemical severity and early clinical features.

The relationships between biochemical severity of hypothyroidism (as judged by plasma thyroxine) and the clinical and radiographic findings at diagnosis were evaluated in 449 infants born in 1982-4 with congenital hypothyroidism identified by neonatal screening. Details of pregnancy, delivery, and the neonatal period were also examined and compared with the findings in a normal population of 36,727 infants born in 1988. Infants with plasma thyroxine values of 30 nmol/l or less had a significantly higher incidence of prolonged jaundice, feeding difficulties, lethargy, umbilical hernia and macroglossia, showed more severe delay of bone maturation on a knee radiograph, and had a higher proportion of thyroid agenesis on isotope scan. In contrast, an ectopic or hypoplastic gland was more common in infants with plasma thyroxine values above 30 nmol/l. Prevalence of illness in pregnancy and mode of delivery was not related to severity of hypothyroidism and were similar to figures for the normal population. Induction of labour, gestation over 40 weeks, and birth weight above 3500 g were significantly more common in the hypothyroid infants. Perinatal illness and congenital malformations were more common in the infants with low plasma thyroxine values at diagnosis.

Intelligence, motor skills and behaviour at 5 years in early-treated congenital hypothyroidism.

The cognitive functioning, motor skills and behaviour of 5-year-old children with early-treated congenital hypothyroidism was assessed. The study group was 57 children with congenital hypothyroidism (CH) diagnosed by neonatal screening in N.E. and N.W. Thames regions between 1978 and 1981 along with 51 non-affected controls matched for age, sex, social class and language background. Small differences in I.Q. and behaviour between the patients and the controls were not statistically significant. However, children with CH showed significant deficits in motor skills (M 79.9 SE 3.7) compared to the controls (M 99.8 SE 4.0) (P = 0.0003). Deficits were particularly marked for balance. In addition, children with more severe hypothyroidism at diagnosis (Plasma thyroxine less than 20 nmol/l) did significantly less well in respect to I.Q. and motor skills than those with less severe hypothyroidism (plasma thyroxine greater than 60 nmol/l). These findings provide further evidence for the importance of the severity of hypothyroidism in determining the outcome for intelligence and motor skills in children with early-treated congenital hypothyroidism. Deficits in motor skills, particularly in relation to balance, suggest that early impairment of the vestibular system may occur despite early treatment.