Marjolein Verhoef

High prevalence of incontinence among young adults with spina bifida: description, prediction and problem perception

Study design:Cross-sectional study.Objectives:To study the prevalence of incontinence, problem perception and determinants of urinary and faecal incontinence in young adults with spina bifida.Setting:Nation-wide study in the Netherlands.Participants:A total of 179 of 350 invited patients participated, including 37 patients with spina bifida occulta and 142 with spina bifida aperta, of whom 119 had hydrocephalus; 41% were male and mean age was 20.4 (range 16–25 years).Methods:Data were collected from interviews, physical examination, neuropsychological tests and medical records.Results:Urinary and faecal incontinence was common in young adults with spina bifida (60.9 and 34.1%, respectively), regardless of the bladder and bowel management they used. The majority of urinary and faecal incontinent patients perceived this as a problem (69.7 and 77.0%, respectively). Spina bifida aperta, hydrocephalus and a level of lesion of L5 or above were associated with patients suffering from urinary and/or faecal incontinence. Predictors of perceiving urinary incontinence as a problem were, in addition to being incontinent, not having hydrocephalus and having a level of lesion of L5 or above. The only predictor of perceiving faecal incontinence as a problem was the frequency of incontinence.Conclusion:A majority of young adults with spina bifida suffer from urinary and faecal incontinence and most of them perceive their incontinence as a problem. Therefore, further efforts are important to improve urinary and faecal continence.

Secondary impairments in young adults with spina bifida

The aim of this study was to examine the prevalence of secondary impairments in young adults with spina bifida and to relate the prevalence to the type of spina bifida and the level of lesion. This cross‐sectional study is part of the ASPINE (Adolescents with Spina Bifida in the Netherlands) study. Data were collected on medical history, hydrocephalus (shunt: yes/no), neurological level of lesion (International Standards for Neurological and Functional Classification of Spinal Cord Injury), visual acuity (Landolt rings), spasticity (Modified Ash worth Scale), contractures (range of motion), scoliosis (deviation from perpendicular), ambulation (Hoffer criteria), pressure sores and blood pressure (physical examination), epilepsy, pain, incontinence and sexuality (questionnaire), and cognitive functioning (Raven Standard Progressive Matrices). In total, 179 patients with spina bifida participated (41% male, age range 16 to 25 years, mean 20 years 9 months, SD 2 years 11 months). These were 37 patients with spina bifida occulta, 119 patients with spina bifida aperta and hydrocephalus (AHC+) and 23 patients with spina bifida aperta without hydrocephalus (AHC−). Of our patient group, 73 had a high‐level lesion (L2 and above), 68 a mid‐level lesion (L3 to L5), and 38 a low‐level lesion (S1 and below). Both subdivisions were strongly related with patients with higher lesions more often having hydrocephalus. Most secondary impairments were found for patients with AHC+, and patients with AHC− were mostly comparable to patients with spina bifida occulta. According to level of lesion, most medical problems were found in the high‐level lesion group. However, all subgroups suffered from health problems.

Cognitive status of young adults with spina bifida

The cognitive status of 168 Dutch young adults (103 females, 65 males; mean age 20 years 9 months, age range 16 to 25 years) with spina bifida (SB) was examined. The main purpose was to establish the effect of the type of SB (occulta or aperta) and the effect of hydrocephalus (HC) within the group with SB aperta (AHC+). Results indicated, on average, a lower cognitive status of persons with AHC+ (n=111) than of persons with SB occulta (n=37) and of persons with SB aperta without HC (AHC-; n=20). Almost half the young adults with AHC+ had cognitive impairments of some sort. These included more domain specific impairments (70%) as well as a more general cognitive deficit (30%). Cognitive status of persons with SB occulta and of those with AHC- was similar to that in the healthy population. The presence of associated pathology, rather than SB per se, has a negative effect on cognitive status.

Functional independence among young adults with spina bifida, in relation to hydrocephalus and level of lesion

Knowledge about the level of functional independence that can be expected in adulthood might support decisions on the treatment of newborn infants with spina bifida. This study determined functional independence among young adults with spina bifida and its relationships with pathological characteristics known from birth (hydrocephalus and level of lesion). Data were collected from medical records and by physical examination. Functional independence was assessed on six domains (self‐care, sphincter control, transfers, locomotion, communication, and social cognition) using the Functional Independence Measure (FIM). Participants were 165 patients with spina bifida (69 males, 96 females; age range 16 to 25y, mean 20y 9mo [SD 2.9]; 117 with hydrocephalus). Patients without hydrocephalus were independent for all FIM domains except sphincter control, as were patients with hydrocephalus with a lesion level below L2. Most patients with hydrocephalus and a lesion at L2 or above were dependent as regards sphincter control (98%), locomotion (79%), and self‐care (54%), and quite a few needed support in transfers (38%), social cognition (29%), and communication (15%).

Restrictions in social participation of young adults with spina bifida

Purpose. To determine participation restrictions of young adults with spina bifida (SB) in relation to health condition and activity limitations. Method. A total of 179 persons aged 16–25 years and born with SB participated in a cross-sectional study. The main outcome on four domains of participation (independent living, employment, education and partner relationships) was assessed using a structured questionnaire. Results. At the mean age of 21 years only 16% were living independently, more than one-third of the participants went to special secondary education, 53% of those who finished education did not have a regular job and 71% did not have a partner. Health condition variables (type of SB, hydrocephalus and level of lesion) and to a lesser extent activity limitations (wheelchair dependence and incontinence) were significant determinants for having participation restrictions. Perceived hindrances in participation included long-distance transportation (19–36%), accessibility (10–42%), physical impairments (22–40%), emotional barriers (20–32%) and financial limits (3–17%). More severe SB, defined as hydrocephalus, high level of lesion and wheelchair dependence, was related with more experienced hindrances due to long-distance transportation accessibility of buildings. Conclusions. Many young adults with spina bifida experience participation restrictions. Severity of SB was negatively related to participation. Social integration should be a major focus in the professional guidance of youngsters with physical disabilities.

Perceived health in young adults with spina bifida

The aims of this study were to compare the perceived health of young adults with spina bifida with a population without disability, and to determine the effect of the disease characteristics and resulting impairments on perceived health. This cross‐sectional study is part of the Adolescents with Spina Bifida in the Netherlands study. Data were collected by physical examination and a questionnaire. In total, 179 patients (age range 16‐25y) participated in the study and perceived health data were completed for 164 participants (92 females, 72 males; mean age 20y 7mo [SD 2y 9mo]). Twenty‐six participants had spina bifida occulta and 138 had spina bifida aperta, of whom 115 also had hydrocephalus. Perceived health was measured with the Medical Outcome Study 36‐item Short‐form Health Survey (SF‐36), a generic health status measure. SF‐36 scores of young adults with spina bifida were below those of an age‐matched population group for six of the eight domains. This difference was largest for the physical functioning domain. Although these differences were statistically significant they were small. Findings for the emotional health domains (vitality, mental health, role problems due to emotional problems) did not differ at all from the population group.

Work participation among young adults with spina bifida in the Netherlands

The aim of this study was to: (1) assess work participation among young adults with spina bifida, (2) identify problems perceived in finding employment, and (3) examine which determinants are related to work participation. This cross‐sectional study was a follow‐up study to the Adolescents with SPina bifida In the Netherlands (ASPINE) study. Data regarding work participation and problems finding employment were collected with questionnaire developed by the authors. Data on disease characteristics were taken from the ASPINE database. Responses of 136 participants were analyzed (77 females, 59 males; mean age 26 years 1 month [SD 3y1mo], range 21–32y). Twenty participants had spina bifida occulta and 116 had spina bifida aperta, 96 of whom also had hydrocephalus. Work participation rate was 62.5%, of which 22.4% was in a sheltered workplace. Significant determinants of having paid work for at least 1 hour a week were: level of education, level of lesion, hydrocephalus, IQ, functional independence, and ambulation. Significant determinants of full‐time employment were the same, plus sex and type of spina bifida. In a multivariate backward logistic regression analysis, however, only level of education remained a significant predictor of work participation. Sex, level of education, and self‐care independence were significant predictors of full‐time employment. This study shows the importance of educational support and self‐care independence training for children with spina bifida.

IS COGNITIVE FUNCTIONING ASSOCIATED WITH SUBJECTIVE QUALITY OF LIFE IN YOUNG ADULTS WITH SPINA BIFIDA AND HYDROCEPHALUS

OBJECTIVE To test the hypothesis that cognitive functioning is associated with subjective quality of life of young adults with spina bifida and hydrocephalus (SBHC). DESIGN Cross-sectional multi-centre study in The Netherlands. SUBJECTS A total of 110 young adults with SBHC (16-25 years old, 63% female). METHODS Cognitive domains measured were intelligence (Raven Standard Progressive Matrices), memory (Wechsler Memory Scale) and executive functioning (Wisconsin modified Card Sorting Test (WmCST), Trail Making Test A and B (TMT) and UNKA word production test). Subjective quality of life was measured with a visual analogue scale. Correlations and hierarchical regression analysis controlling for age, gender and functional independence were applied. RESULTS The TMT score was significantly associated (-0.25) with subjective quality of life. In the hierarchical regression analysis both the WmCST and TMT scores were significant determinants of subjective quality of life (Beta values 0.24 and -0.31 respectively). Intelligence, memory and word production were not related to subjective quality of life. All 5 cognitive variables together explained a significant additional 14.6% of the variance of subjective quality of life (total explained variance 19.9%). CONCLUSION Executive functioning was associated with subjective quality of life in young adults with spina bifida and hydrocephalus. This finding underlines the importance of examining cognitive functioning of persons with SBHC in addition to medical and functional status in medical care and outcome research.

Developmental trajectories of receptive and expressive communication in children and young adults with cerebral palsy.

The aim of this study was to determine the developmental trajectories of expressive (speech) and receptive (spoken and written language) communication by type of motor disorder and intellectual disability in individuals with cerebral palsy (CP).

Developmental Trajectories of Mobility and Self-Care Capabilities in Young Children with Cerebral Palsy

OBJECTIVE To describe development of mobility and self-care capabilities in young children (aged 1-4 years) with cerebral palsy, and to examine whether the development of mobility and self-care capabilities differs by cerebral palsy severity in terms of 5 distinct Gross Motor Function Classification System (GMFCS) levels. STUDY DESIGN This prospective longitudinal cohort study included 100 children with cerebral palsy (aged 1.5 or 2.5 years at baseline) and their parents. Mobility and self-care capabilities were assessed by the Pediatric Evaluation of Disability Inventory during yearly assessments from inclusion up to age 4.5 years. Longitudinal data for 92 children were available for analysis. Repeated-measures analyses with random coefficient analysis were performed using linear mixed models. RESULTS Despite large variations among individuals in the development of mobility and self-care capabilities in young children with cerebral palsy, distinct developmental trajectories were found for children in different GMFCS levels. The estimated change per month differed significantly by GMFCS level for both outcomes. CONCLUSIONS This longitudinal study provides an evidence base for prognosis in daily mobility and self-care skills in young children with cerebral palsy. The developmental trajectories for GMFCS levels can be helpful in communication between professionals and also in discussions of expectations and goal setting with families regarding mobility and self-care in the daily life of young children with cerebral palsy in neonatal follow-up and pediatric practice.