摘要
Background:The presence in a family of a child or children with epidermolysis bullosa (EB) may have profound psychological implications for other family members. Objectives:To assess the impact of the presence of EB in one or more children on the personal relationships between their parents. Methods:Standardized questionnaires were used. Results:In general, the presence of a child severely affected with EB had profound effects on many aspects of marriage. This included a lack of interest in participating in activities as couples junctional EB (JEB), 45%; recessive dystrophic EB(RDEB), 25% , a lack of energy to invest in such pursuits (JEB, 82%; RDEB, 50%), limitations in opportunities for sharing nonintimate physical activities (reported by most parents having children with some type of generalized EB), and negatively altered parental sex life (JEB, 55%; RDEB, 39%). This is consistent with the fact that 10%, 64%, 25%and 36%of parents of an affected child with EBsimplex (EBS), JEB, dominant dystrophic EB(DDEB) and RDEB,respectively,characterized theirrelation ship sas couples as revolving almost exclusively around the day-to-day care of their affected children. The severity of disease in an affected child clearly influenced parental decisions about having more children:24%and 64%of parents of children with JEBand RDEB,respectively,chose not to have additional children,compared with 26%and 54%of parents with children having EBSor DDEB. This choice was most often pursued via tubal ligation; less often, alternative means of surgical sterilization were chosen. Divorce was common among parents of children with EB (range:17%in EBS to 31%in JEB) and, with the exception of parents of children with EBS, was usually directly attributed by one or both parents to the profound impact that this disease had exerted on their marriage. Conclusions:Physicians caring for children with EB need to give more consideration to the many psychological factors that may contribute to their patients’well being. They may need to assist these children’s parents in seeking support and counselling to prevent destruction of the family unit.
Background:The presence in a family of a child or children with epidermolysis bullosa (EB) may have profound psychological implications for other family members. Objectives:To assess the impact of the presence of EB in one or more children on the personal relationships between their parents. Methods:Standardized questionnaires were used. Results:In general, the presence of a child severely affected with EB had profound effects on many aspects of marriage. This included a lack of interest in participating in activities as couples junctional EB (JEB), 45%; recessive dystrophic EB(RDEB), 25% , a lack of energy to invest in such pursuits (JEB, 82%; RDEB, 50%), limitations in opportunities for sharing nonintimate physical activities (reported by most parents having children with some type of generalized EB), and negatively altered parental sex life (JEB, 55%; RDEB, 39%). This is consistent with the fact that 10%, 64%, 25%and 36%of parents of an affected child with EBsimplex (EBS), JEB, dominant dystrophic EB(DDEB) and RDEB,respectively,characterized theirrelation ship sas couples as revolving almost exclusively around the day-to-day care of their affected children. The severity of disease in an affected child clearly influenced parental decisions about having more children:24%and 64%of parents of children with JEBand RDEB,respectively,chose not to have additional children,compared with 26%and 54%of parents with children having EBSor DDEB. This choice was most often pursued via tubal ligation; less often, alternative means of surgical sterilization were chosen. Divorce was common among parents of children with EB (range:17%in EBS to 31%in JEB) and, with the exception of parents of children with EBS, was usually directly attributed by one or both parents to the profound impact that this disease had exerted on their marriage. Conclusions:Physicians caring for children with EB need to give more consideration to the many psychological factors that may contribute to their patients'well being. They may need to assist these children's parents in seeking support and counselling to prevent destruction of the family unit.
