Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents.

BACKGROUND: Infantile spasms (IS; West syndrome) is a severe form of encephalopathy that typically affects infants younger than 2 years old. Pediatricians, pediatric neurologists, and other pediatric health care providers are all potentially key early contacts for families who have an infant with IS. The objective of this article is to assist pediatric health care providers in the detection of the disease and in the counseling and guidance of families who have an infant with IS. METHODS: Treatment guidelines, consensus reports, and original research studies are reviewed to provide an update regarding the diagnosis and treatment of infants with IS. Web sites were searched for educational and supportive resource content relevant to providers and families of patients with IS. RESULTS: Early detection of IS and pediatrician referral to a pediatric neurologist for further evaluation and initiation of treatment may improve prognosis. Family education and the establishment of a multidisciplinary continuum of care are important components of care for the majority of patients with IS. The focus of the continuum of care varies across diagnosis, initiation of treatment, and short- and long-term needs. Several on-line educational and supportive resources for families and caregivers of patients with IS were identified. CONCLUSIONS: Given the possibility of poor developmental outcomes in IS, including the emergence of other seizure disorders and cognitive and developmental problems, early recognition, referral, and treatment of IS are important for optimal patient outcomes. Dissemination of and access to educational and supportive resources for families and caregivers across the lifespan of the child with IS is an urgent need. Pediatric health care providers are well positioned to address these needs.

Cognitive outcomes in children who present with a first unprovoked seizure.

PURPOSE: To determine the long-term cognitive and educational outcomes in children prospectively identified at the time of a first unprovoked seizure. METHODS: A cohort of children with a first unprovoked seizure was enrolled and followed for a mean of 15 years. Cognitive function and educational outcomes were determined 10 or more years after the first seizure via standardized neuropsychological tests, school records, and structured interviews. Children with symptomatic etiology were excluded from the analysis. When available, siblings of study subjects were recruited as normal controls. Primary educational outcome was defined as enrollment into special education services or grade repetition. RESULTS: Twenty-eight percent of (43 of 153) of children with a single seizure and 40% (42 of 105) of children with epilepsy received special education service or repeated a grade (p = 0.05). There was a statistically significant trend in which the children with more seizures tended to require special education or repeat a grade more often (28% in single seizure group vs. 34% in 2-9 seizure group vs. 64% in ≥10 seizure group; p = 0.004). Of 163 subjects who completed neuropsychological testing, children with single seizures tended to score higher than children with epilepsy on Wide Range Achievement Test-3 (WRAT) reading (p = 0.08), Test of Non-Verbal Intelligence-II (TONI-II) (p = 0.02), and Wechsler Intelligence Scale for Children (WISC)/Wechsler Adult Intelligence Scale (WAIS) (p = 0.07). There was no statistically significant difference between children with a single seizure and sibling controls. CONCLUSION: The results suggest that children with a single seizure represent a group that is distinctly different from children with epilepsy and are more similar to sibling controls. In contrast, even children with very mild epilepsy have significantly worse educational outcomes.

Infantile spasms: a U.S. consensus report.

The diagnosis, evaluation, and management of infantile spasms (IS) continue to pose significant challenges to the treating physician. Although an evidence-based practice guideline with full literature review was published in 2004, diversity in IS evaluation and treatment remains and highlights the need for further consensus to optimize outcomes in IS. For this purpose, a working group committed to the diagnosis, treatment, and establishment of a continuum of care for patients with IS and their families­the Infantile Spasms Working Group (ISWG)­was convened. The ISWG participated in a workshop for which the key objectives were to review the state of our understanding of IS, assess the scientific evidence regarding efficacy of currently available therapeutic options, and arrive at a consensus on protocols for diagnostic workup and management of IS that can serve as a guide to help specialists and general pediatricians optimally manage infants with IS. The overall goal of the workshop was to improve IS outcomes by assisting treating physicians with early recognition and diagnosis of IS, initiation of short duration therapy with a first-line treatment, timely electroencephalography (EEG) evaluation of treatment to evaluate effectiveness, and, if indicated, prompt treatment modification. Differences of opinion among ISWG members occurred in areas where data were lacking; however, this article represents a consensus of the U.S. approach to the diagnostic evaluation and treatment of IS.

The personal and financial impact of repetitive or prolonged seizures on the patient and family.

Individuals with epilepsy consistently report diminished quality of life. The clinical characteristics of seizures and the unpredictable nature of seizure occurrences are some factors that affect quality of life. Prolonged or repetitive seizures can impose psychologic comorbidities, social issues, and lifestyle restrictions that can affect quality of life of patients and their caregivers and family members, who also bear the considerable indirect costs of seizures, including time away from work or school, and even loss of employment. The availability and use of an at-home medication to terminate prolonged or repetitive seizures or in seizure emergencies improves quality of life for patients and their families. Fewer visits to the emergency department are associated with a reduction in the financial burden to families and the health care system. This article discusses factors that contribute to the personal and financial impact of prolonged seizures on adult and pediatric patients, their families, and caregivers.

Emergency management of seizures in the school setting.

Effective seizure management in the school setting is a critical issue for students with seizures, as well as their parents, classmates, and school personnel. The unpredictable nature of seizures and the potential outcomes of experiencing a seizure in school are sources of anxiety for students with seizures. The ability to respond appropriately to a seizure is of concern to parents and school personnel. Implementation of a seizure emergency treatment plan empowers school personnel to quickly treat the child. Diazepam rectal gel is commonly used in seizure emergency treatment plans. It is safe and effective in terminating seizures and reduces the time to treatment and the need for emergency department visits when used in the school setting, and can be administered by medical and delegated to trained nonmedical personnel. School nurses should be aware of the laws and professional recommendations that pertain to rectal medication administration in schools for optimal emergency seizure management.

School nurses' experience with administration of rectal diazepam gel for seizures.

The purpose of this study was to determine school nurses' knowledge of state and school district policies, their experience regarding the administration of rectal diazepam gel in the school, and the perceived benefits and barriers of providing this treatment. Four hundred nineteen nurses responded to a survey conducted during the National Association of School Nurses Annual Meeting. Seventy-one (18%) nurses surveyed had administered rectal diazepam gel in a school setting, while 54 (13%) nurses reported that either their state practice act or school district prohibited them from giving rectal medications in the school. Medication administration benefits, such as early intervention for treatment of acute seizure emergencies, were noted. Barriers were also identified, with lack of privacy as the most frequently listed. Scope of practice as it pertains to administering medication in the school and the extent to which delegation of duties can be used in the situation of administering rectal medication in a seizure emergency remain issues for school nurses.

Rectal diazepam gel in the home management of seizures in children.

This study assessed the utility of rectal diazepam gel in the home management of prolonged or repetitive seizures in children. Thirty-eight children being prescribed rectal diazepam gel by their clinician were prospectively recruited. Seizures, rectal diazepam use, emergency department visits, and quality of life data before and after study entry were recorded. The 38 children included 14 (37%) with complex febrile seizures, and 24 with epilepsy (n = 22) or a single seizure (n = 2). There were 23 (61%) children with prolonged seizures and 15 (39%) with repetitive seizures. During the 6-month follow-up period, 12 children experienced 26 seizures which met the criteria for rectal diazepam administration. Rectal diazepam gel was administered to 8 children on 19 occasions. In 16 (84%) of these episodes, seizures stopped and no emergency department visit was required. Parental stress was decreased between baseline and 6 months in both the overall group and in all the subgroups. Home use of rectal diazepam gel is effective in aborting seizure activity, often avoiding an emergency department visit. Its use reduces morbidity and costs associated with hospital visits and provides parents a treatment option for home management of prolonged or repetitive seizures.

Mortality following a first unprovoked seizure in children: a prospective study.

In a prospective study, 407 children with a first unprovoked seizure were followed for a mean of 14.2 years. To date, nine have died. Death was unrelated to seizures in four subjects who had no further seizures and were on no medications. The remaining five subjects all had multiple seizures and were on medications. Treatment following the first seizure would not have altered mortality in any of the nine cases.

Childhood brain tumors: parental concerns and stressors by phase of illness.

The objective of this study is to identify common problems and helpful resources important to parents of children with brain tumors by illness phase and to determine associations with stress. Parents with a child diagnosed within the past 10 years were surveyed regarding healthcare provider interactions, medical information/education, health care utilization and psychosocial concerns. Survey items were rated as problems or helpful, and for importance at each phase of illness. Stress was recorded from 0 to 10 for each phase; associations with demographic characteristics and items were tested statistically. A total of 139 parents from 87 families responded, with 45 mother-father pairs. Half reported unmet informational needs as most important during diagnosis (etiology), recurrence (complementary therapy), end of life (dying process), and remission (long-term effects). Mothers experienced greater stress than fathers during adjuvant treatment (p = .009). Stress increased (p < .05) during diagnosis and hospitalization/surgery with being married, at hospital discharge because of changes in child's personality/moods, during adjuvant treatment with unmet informational needs regarding stopping treatment, during recurrence regarding employment concerns, and during remission with unmet informational needs regarding life-time expectations. Stressors changed across phases of illness. Married respondents appeared at increased risk for stress. Further work is needed to tailor and evaluate interventions to decrease stress during illness phases.

Febrile seizures.

Febrile seizures are the most common seizure type in childhood. The prognosis is usually benign. Diagnostic evaluation focuses on excluding other causes of seizures and fever, especially central nervous system infections. For most febrile seizures, whether simple or complex, education and counseling are the primary modes of treatment. Given our current state of knowledge, a rational plan of treatment focuses on counseling and preventing prolonged febrile convulsions. An understanding of the natural history and prognosis enables physicians to reassure the families of children with febrile seizures and provide appropriate counseling and management while avoiding unnecessary diagnostic and therapeutic interventions.

Childhood brain tumors: children's and siblings' concerns regarding the diagnosis and phase of illness.

Our objective was to identify commonly reported problems and helpful resources important to children with brain or spinal cord tumors and siblings during phases of illness. Affected children and siblings from 40 families responded to a regional survey. Items encompassed four general content areas: health care provider interactions, medical information/education, health care utilization, and psychosocial. Children rated individual items as helpful, a problem, and for importance; relative frequencies were derived. One-third or more affected children reported important problems: hospitalization/surgery--help with schoolwork; hospital discharge--help with changes in physical activity, appearance, moods, special needs at school; adjuvant treatment--Internet information, help with moods; and remission--socializing. One-third or more siblings reported important problems: diagnosis--information about etiology and prognosis, manner of physician and parents in providing information; hospitalization/surgery--information about prognosis, help with schoolwork; hospital discharge--help with schoolwork; adjuvant treatment--help with changes in sister or brother's appearance, physical activity, moods, and information about the treatment; end of life--treatment of sister's or brother's pain, information about dying, family harmony, support from friends, help with schoolwork, and preparation for the death. Consideration of problems and helpful resources will provide the framework for developing and evaluating intervention strategies at each phase of illness.