T. Lunnon-Wood

Successful cardiac transplantation in Barth syndrome - : single-centre experience of four patients

Abstract:  We describe four patients with Barth syndrome who have undergone successful orthotopic heart transplantation. Patients are one, seven, 12.5 and 14.7 yr post‐transplantation. One episode of severe infection occurred. Renal dysfunction and coronary allograft vasculopathy do not appear accelerated over non‐Barth patients. Despite withholding purine synthesis inhibitors, these patients have not demonstrated an increased rate of rejection.

Perceived quality of life of children after successful bridging to heart transplantation.

BACKGROUND Mechanical circulatory support is increasingly used to bridge children with end-stage heart failure to transplant. Quality of life (QoL) has not been systematically evaluated in children bridged to heart transplant. METHODS All children transplanted for cardiomyopathy during 2001 to 2008 and currently being followed at our center (n = 84) had QoL assessed during 2006 to 2009, at a median of 3 years post-transplant, using a validated generic measure (PedsQL4.0). RESULTS Twenty-six children, aged 2.7 to 18 (median 7.4) years who were bridged to transplant, were compared with 58 children, aged 2.0 to 18.0 (median 13.0) years, who were transplanted in the same era without bridging. There were no significant differences between the 2 groups on any domains of QoL assessed by children or parents, although the small number of bridged patients increases the likelihood of a Type II error. Bridged children who were younger (r = 0.48, p = 0.02) or more recently transplanted (r = 0.42, p = 0.04) were scored by their parents as having poorer emotional QoL. Regression analysis indicated that age at transplant was the only medical or demographic variable associated with parent-reported total QoL scores (β = 0.27, p = 0.01). With few links between QoL scores and medical or demographic factors, other subjective psychologic factors may be of greater salience in determining QoL. CONCLUSIONS Despite greater severity of illness, children who required mechanical bridging to transplantation report a QoL comparable to that of other children undergoing heart transplantation. Younger children may require greater psychologic support to reach their full potential in terms of QoL.

Psychological benefits for children and adolescents who have undergone transplantation of the heart from participation in the British Transplant Games.

The British Transplant Games are held annually for children and adults who have received transplanted organs. The aim of our pilot study was to determine whether participation in such an event had an impact on psychological well-being. We asked 26 children participating in the Games to complete a specifically designed questionnaire before and after the event. The 13 questions covered areas such as state of mood, perceived physical health, fatigue, anxiety, and confidence. There were mean improvements over time on 10 of the 13 questions, and the change in overall score was significant (p = .036). For the majority of young people, participation in the Transplant Games was associated with a positive impact on psychological functioning in the short term. Further evaluation is now required to determine whether such benefits are maintained in the longer term.

Experiences of adolescent lung transplant recipients: A qualitative study

Many young transplant recipients experience psychological distress and adjustment difficulties, yet there is little research investigating lung transplantation from the recipients’ perspective. This qualitative study aimed to explore experiences of young people who underwent lung transplantation. Semi‐structured interviews were conducted with six lung transplant recipients (aged 15‐18). Interviews were analysed using IPA, a qualitative research approach examining how people make sense of their major life experiences. The analysis revealed three master themes: “Living with Dodgy Lungs” outlined how participants dealt with their experiences, managing through accepting or discussing their feelings with others, although talking was often difficult. “The Big Deal” reflected participants’ experiences of the process, their expectations, and the contrast of their lives pre‐ and post‐transplant. Inherent in their accounts was the profound meaning ascribed to transplantation, the emotional turmoil, and impact on their lives. “A Sense of Self” illustrated participants’ developing identities within their social contexts and at times isolating experiences. The results highlight key areas where adolescent lung transplant recipients could be supported by clinicians, enabling the promotion of psychological well‐being. Examples include supporting identity integration post‐transplant, facilitating social inclusion, considering alternative means of support, and involving adolescents in healthcare decisions.