Psychosocial outcomes of bone marrow transplant for individuals affected by Mucopolysaccharidosis I Hurler Disease: patient social competency.

AIM: To explore the frequency with which children and young people participate in social activities with peers, when they are affected by Mucopolysaccharidosis I Hurler Disease (MPS IH) post bone marrow transplant (BMT). This was investigated in relation to patient age, and in comparison with a normative sample. Patient withdrawal, adaptive and social skills are also described in terms of patient age and in comparison with a normative sample. METHOD: Forty-four individuals affected by MPS IH post BMT participated in this investigation. Their ages ranged from 16 months to 25 years. Semi-structured interviews with patients' mothers were utilized, which included the Behaviour Assessment System for Children and a socialization questionnaire. Normative data for the socialization questionnaire were collected from 46 mothers of children not affected by chronic illness or disability. RESULTS: A one-way ANOVA revealed that children not affected by disability or chronic illness (mean = 20.63) participated overall in social activities more frequently than children aged under 12 years (mean = 14.87) and over 12 years (mean = 13.25) who were affected by MPS IH post BMT (F = 21.01, P < 0.001). Young people aged 12 years and over affected by this condition were found to participate in social activities the least. A pattern also emerged, which indicated greater withdrawal and less well-developed adaptive and social skills for MPS IH patients aged 2.5-5 years and 12 years and over, but scores were within the normal range for those aged 6-11 years. CONCLUSION: These data illustrate a lack of social competency and a tendency towards inhibition and withdrawal in this patient group, particularly among the very young children and the adolescents and young adults. Further research is needed to explore these issues longitudinally and to examine the role played by the family, and indeed disability, in the quality and quantity of social interaction experienced by these individuals.

The effect on ambulatory blood pressure of working under favourably and unfavourably perceived supervisors.

AIMS: To investigate the role played by employees' perceptions of their supervisors' interactional styles as a possible source of workplace stress that may be associated with increased morbidity and mortality rates from cardiovascular disorders in workers in the lower strata of organisational hierarchies. METHODS: A controlled, quasi-experimental, field study of female healthcare assistants. Allocation to the experimental and control groups was based on participants' responses to a supervisor interactional style questionnaire. Experimental participants (n = 13) reported working under two divergently perceived supervisors at the same workplace, on different days. The control group (n = 15) worked either under one supervisor, or two similarly perceived supervisors. Ambulatory blood pressure was recorded every 30 minutes, over a 12 hour period for three days. RESULTS: The control group showed a 3 mm Hg difference in systolic blood pressure (SBP) and a non-significant difference in diastolic blood pressure (DBP; mean difference 1 mm Hg) between the two supervisor conditions. The experimental group showed significantly higher SBP (15 mm Hg) and DBP (7 mm Hg) when working under a less favoured compared to a favoured supervisor. The degree of divergence in perceptions of supervisors shows a significant positive relation with the difference in blood pressure between the two workdays. Divergence in perceptions of interpersonal fairness is the strongest predictor of difference in blood pressure. CONCLUSION: An unfavourably perceived supervisor is a potent workplace stressor, which might have a clinically significant impact on supervisees' cardiovascular functioning.