Helping Families Cope With The Severe Stress Of Dravet Syndrome.
Epilepsies; Myoclonic/nursing; Epilepsies Myoclonic/physiopathology; Epilepsies Myoclonic/psychology; Family; Family Health; Female; Humans; Male; Stress Psychological/etiology; Transition To Adult Care
Dravet Syndrome; Family; Help; Management; Support
Abstract
A child with Dravet syndrome shakes family life to the core. Dravet syndrome usually has three phases: (1) up to 1-1½ years: with episodes of febrile status epilepticus but normal development; (2) age 1½ to ~6-10 years: with frequent seizures of varying types, developmental stagnation, behavioural and sleep problems; (3) after ~10 years: improvement in seizures, deteriorating gait, intellectual disability but some developmental gains. Complete seizure control is rare-simply prescribing medication is inadequate to help families. Based on structured interviews with 24 families and confirmed by more informal discussions with other families, we suggest strategies for coping with this catastrophe. A child with Dravet syndrome usually means that one parent cannot work-financial pressures should be anticipated. In Stage 1, the approach to status should include a written protocol. An indwelling catheter for rapid venous access may be helpful. In Stage 2, assistance finding qualified babysitters is required, and the extended family needs encouragement to help. Appropriate equipment, rescue medication and protocols should travel with the child. Siblings may benefit from a system of one parent "on call." An internet support group provides an invaluable lifeline. In Stage 3, family isolation may be extreme-respite care and personal time for parents are important. Death from status, accidents and SUDEP (sudden unexplained death in epilepsy) occurs in 15%. Fear of SUDEP needs to be addressed. Moving from paediatric to adult care is frightening; an epilepsy transition clinic is useful. Attention to these realities may improve the quality of life for both child and family.
Camfield P; Camfield C; Nolan K
Canadian Journal Of Neurological Sciences
2016
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
DOI: 10.1017/cjn.2016.248
Living with cerebral palsy and tube feeding: A population-based follow-up study
Child; Humans; Community Health Planning; Adult; Attitude to Health; Questionnaires; Follow-Up Studies; Survival Analysis; Activities of Daily Living; quality of life; adolescent; Preschool; Adaptation; Psychological; Caregivers/psychology; infant; Parents/psychology; Nutritional Failure; Cerebral Palsy/mortality/psychology/therapy; Enteral Nutrition/adverse effects/psychology; Gastrostomy/adverse effects/psychology; Jejunostomy/adverse effects/psychology; Nova Scotia/epidemiology; Precipitating Factors
OBJECTIVES: To assess the impact of surgically placed feeding tubes on children with severe cerebral palsy (CP) and their families and to determine the survival of these children after initiation of tube feeding (TF). METHODS: Virtually all children from Nova Scotia who had gastrostomy or jejunostomy procedures between the years 1980 and 1998 and who had been diagnosed with CP were identified. Caretakers of those children who had TF initiated in the last 8 years were evaluated by using a semi-structured interview. Names of children who had not had recent follow-up visits were submitted to the provincial Vital Statistics office to determine whether they had died. Data from patients who were tube-fed between 1980 and 1989 were then used in combination with data from the more recent cases to create a survival curve. RESULTS: A total of 61 children were identified; 16 had died. Forty of 45 eligible families were interviewed; 90% were pleased with the effect of TF on their child and family life. Negative reports were associated with increased stress related to feeding. Survival rates after gastrostomy and/or jejunostomy were 83% after 2 years and 75% after 7 years. CONCLUSIONS: In children with severe CF, initiation of TF improved the quality of life for both the child and family in 90% despite frequent minor complications.
1999
Smith SW; Camfield C; Camfield P
The Journal Of Pediatrics
1999
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1016/s0022-3476(99)70125-3" target="_blank" rel="noreferrer">10.1016/s0022-3476(99)70125-3</a>
Assessing the impact of pediatric epilepsy and concomitant behavioral, cognitive, and physical/neurologic disability: Impact of Childhood Neurologic Disability Scale
Child; Female; Humans; Male; Questionnaires; Follow-Up Studies; Longitudinal Studies; Reproducibility of Results; Disability Evaluation; Diagnostic and Statistical Manual of Mental Disorders; adolescent; Preschool; Chronic disease; Child Behavior Disorders/diagnosis/etiology; Cognition Disorders/diagnosis/etiology; Epilepsy/complications/diagnosis
Epilepsy has a significant impact on a child's life, the extent to which is based on four factors: epilepsy, cognition, behavioral, and physical/neurologic function. This study evaluates the ability of the 44-item Impact of Childhood Neurologic Disability Scale (ICND) to assess each of these four realms. Parents of children (aged 2 to 18 years) with epilepsy rated their child's overall quality of life and completed the ICND. External validation compared the ICND with (1) neurologists' reports of children's behavior, cognitive abilities, physical/neurologic disability, and epilepsy; and (2) parents, teachers, and children's ratings on six 'criterion standard' questionnaires. Families of 68 children with epilepsy only and 29 children with 'epilepsy-plus' (additional cognitive, behavioral, or physical/neurologic disability; 39 males, 58 females; mean age at testing 10 years 3 months [SD 4.5] age range 2 to 17 years) participated. Internal consistency was excellent (Cronbach's alpha=0.92) as was test-retest reliability (intraclass correlation=0.89). Caregivers distinguished the impact of each of the four realms. Scores were negatively related to quality of life (Pearson's r=-0.59). Children with high ICND scores had more difficulties at home and school. Their parents saw them as less rewarding and adaptable and the children saw themselves as less intelligent and less popular with more emotional problems. In addition, children with 'epilepsy-plus' had significantly higher total ICND scores as well as markedly elevated scores within each of the four realms when compared with the epilepsy-only group. It is concluded that the ICND is an accurate, quick measurement tool reflecting the impact of behavior, cognitive learning ability, physical/neurologic disability, and epilepsy on children and their families.
2003
Camfield C; Breau L; Camfield P
Developmental Medicine And Child Neurology
2003
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1017/s0012162203000306" target="_blank" rel="noreferrer">10.1017/s0012162203000306</a>