Spinal cord issues in adult patients with MPS: transition of care survey.

PURPOSE: This study aims to raise awareness of the need for research and appropriate guidelines for managing spinal cord issues in adult patients with mucopolysaccharidosis (MPS) and transition of these patients from pediatric to adult care. METHODS: Pediatric/adult neurosurgeons, orthopedic spine surgeons, and treating physicians with expertise in metabolic disorders and spinal cord issues were invited to complete a survey to assess their experience with spinal cord problems in MPS and their opinion on transitioning routes from pediatric to adult care. RESULTS: Twenty specialists completed the survey; 16 had treated spinal cord issues in patients with MPS. Foramen magnum and cervical stenosis (87%), atlanto-axial instability (67%), and lumbar spine instability (33%) were the main spinal cord issues encountered; 28% had treated adult patients for one or more spinal cord issues. In 40% of cases, this concerned an intervention or procedures performed during childhood. The main specialist responsible for the care of adult patients with MPS differed considerably between institutions and included both pediatric and adult specialists (30% pediatric neurosurgeons, 10% pediatric spine orthopedic surgeons, 30% adult spine neurosurgeons, 20% general adult surgeons). The preferred option (> 50%) for the transition of care was an interdisciplinary team of pediatric and adult specialists. CONCLUSIONS: Further work needs to be done to address problems of managing spinal cord issues in adult patients with MPS. Currently, the responsibility for the care of patients with MPS with spinal cord issues is inconsistent. The best strategy for transitioning these patients from pediatric to adult care is likely an interdisciplinary approach.

The Minimally Important Clinical Difference.

The minimally important clinical difference (MCID) is the smallest measureable change in outcome score that will make a difference to the patient. MCID is usually estimated either as the smallest difference that can be detected statistically, or as the difference between patients who respond positively and negatively to an anchor question.• MCID is the least a surgeon can do which benefits the patient from the patient's perspective.• MCID is necessary information in power analyses used to design new studies comparing treatments.

Pediatric SCI/D caregiver mental health and family dynamics in Colombia, South America.

OBJECTIVE: This study examined the connections between family dynamics and the mental health of caregivers of youth with spinal cord injuries/disorders (SCI/D) caregivers from Colombia, South America. It was hypothesized that lower family functioning would be associated with poorer caregiver mental health. METHODS: A cross-sectional study of self-report data collected from caregivers through the Hospital Universatario Hernando Moncaleano Perdomo in Neiva, Colombia. Thirty caregivers of children with SCI/D from Nevia, Colombia who were a primary caregiver for ≥3 months, providing care for an individual who was ≥6 months post-injury/diagnosis, familiar with the patient's history, and without neurological or psychiatric conditions. Caregivers' average age was 41.30 years (SD = 10.98), and 90% were female. Caregivers completed Spanish versions of instruments assessing their own mental health and family dynamics. RESULTS: Family dynamics explained 43.2% of the variance in caregiver burden and 50.1% of the variance in satisfaction with life, although family dynamics were not significantly associated with caregiver depression in the overall analysis. Family satisfaction was the only family dynamics variable to yield a significant unique association with any index of caregiver mental health (satisfaction with life). CONCLUSIONS: If similar findings emerge in future intervention research, interventions for pediatric SCI/D caregivers in Colombia and other similar global regions could benefit from including techniques to improve family dynamics, especially family satisfaction, given the strong potentially reciprocal connection between these dynamics and caregiver mental health. IMPLICATIONS FOR REHABILITATION: The degree of disability resulting from SCI/D can vary greatly depending on the severity and level of the lesion, though permanent impairment is often present that profoundly impacts both physical and psychological functioning. Very little is known about the impact of pediatric SCI/D in developing countries, despite the high rates of injury reported in these areas. Family interventions could contribute significantly to the lives of children with SCI/D and their families.

A sexualidade depois de lesão medular: uma análise qualitativa-descritiva de uma narrativa biográfica / Sexuality after spinal cord injury: an analysis of biographical narrative

Esta pesquisa qualitativo-descritiva investigou aspectos da sexualidade, a partir da análise de conteúdo, em uma narrativa autobiográfica de uma jovem com lesão medular adquirida. As categorias analisadas nos resultados foram: (1) Mudanças cotidianas decorrentes da deficiência adquirida, (2) Reconhecimento da identidade de deficiente e (3) Manifestações afetivas e sexuais depois da deficiência. A lesão medular pode influenciar diretamente a vida sexual, mas não impossibilita a expressão da sexualidade. A percepção de um corpo erótico e desejável relacionou-se ao suporte emocional familiar e à autoestima. A narrativa explicita que a sexualidade é um aspecto importante na reestruturação da vida após a lesão medular e, portanto, o aconselhamento sexual deveria ocorrer na reabilitação da pessoa com deficiência (AU)

This descriptive qualitative study investigated sexuality issues by applying content analysis to an autobiographical narrative of a young woman with acquired spinal cord injury. The categories analyzed in the results were the following: (1) Everyday changes arising from becoming disabled, (2) Recognition of the identity of disabled person and (3) Emotional and sexual manifestations after the disability. Spinal cord injury can directly affect the sex life, but does not preclude the expression of sexuality. The perception of an erotic and desirable body was positively related to the family’s emotional support and to self-esteem. The narrative makes clear that sexuality is an important aspect of the restructuring of life after spinal cord injury and, therefore, sexual counseling should occur in the rehabilitation of the disabled person (AU)

A sexualidade depois de lesão medular: uma análise qualitativa-descritiva de uma narrativa biográfica / Sexuality after spinal cord injury: an analysis of biographical narrative

Esta pesquisa qualitativo-descritiva investigou aspectos da sexualidade, a partir da análise de conteúdo, em uma narrativa autobiográfica de uma jovem com lesão medular adquirida. As categorias analisadas nos resultados foram: (1) Mudanças cotidianas decorrentes da deficiência adquirida, (2) Reconhecimento da identidade de deficiente e (3) Manifestações afetivas e sexuais depois da deficiência. A lesão medular pode influenciar diretamente a vida sexual, mas não impossibilita a expressão da sexualidade. A percepção de um corpo erótico e desejável relacionou-se ao suporte emocional familiar e à autoestima. A narrativa explicita que a sexualidade é um aspecto importante na reestruturação da vida após a lesão medular e, portanto, o aconselhamento sexual deveria ocorrer na reabilitação da pessoa com deficiência

This descriptive qualitative study investigated sexuality issues by applying content analysis to an autobiographical narrative of a young woman with acquired spinal cord injury. The categories analyzed in the results were the following: (1) Everyday changes arising from becoming disabled, (2) Recognition of the identity of disabled person and (3) Emotional and sexual manifestations after the disability. Spinal cord injury can directly affect the sex life, but does not preclude the expression of sexuality. The perception of an erotic and desirable body was positively related to the family’s emotional support and to self-esteem. The narrative makes clear that sexuality is an important aspect of the restructuring of life after spinal cord injury and, therefore, sexual counseling should occur in the rehabilitation of the disabled person