Biomedical subjects

P J M Helders

Publications and source records attributed to P J M Helders.

At least 19 recordsLinked to original sources

PubMed2006

Examining the psychometric characteristics of the Dutch childhood health assessment questionnaire: room for improvement?

The aim of this study was to examine the psychometric characteristics of the childhood health assessment questionnaire-disability index (CHAQ-DI). Seventy-six patients with juvenile idiopathic arthritis (JIA), age range 4.8-15.8 years, completed a CHAQ questionnaire one or more times. In total, 321 CHAQ questionnaires were available for analysis. Factor analysis and correlation were used to analyse the data. The analysis indicated that 12 items could be removed from the original 30 items of the CHAQ-DI. Also the addition of "aids and assistance" to the overall scoring method of the CHAQ-DI did not contribute to the overall measuring concept of the CHAQ-DI. The psychometric characteristics of the CHAQ-DI could be improved by removing 12 items from the original 30 items. Moreover, a simple scoring method, without the addition of aids and assistance to the total CHAQ-DI improves sensitivity to change of the CHAQ-DI.

Adolescent↗

PubMed2006

Early gross motor development of preterm infants according to the Alberta Infant Motor Scale.

OBJECTIVE: To systematically examine gross motor development in the first 18 months of life of preterm infants. STUDY DESIGN: A total of 800 preterm infants (356 boys), ages between 1 and 18 months and corrected for degree of prematurity, were assessed with the use of the Alberta Infant Motor Scale. RESULTS: Comparison of the mean Alberta Infant Motor Scale scores of the preterm infants with the norm-referenced values derived from term infants revealed that as a group, the preterm infants scored significantly lower at all age levels, even with full correction for degree of prematurity. CONCLUSIONS: In general, preterm infants exhibit different gross motor developmental trajectories compared with term infants in the first 18 months of life. The gross motor developmental profile of preterm infants may reflect a variant of typical gross motor development, which seems most likely to be specific for this population. As a consequence, adjusted norms should be used for proper evaluation and clinical decision-making in relation to preterm infants.

Analysis of Variance↗

PubMed2006

Measuring patients' perceptions on their functional abilities: validation of the Haemophilia Activities List.

Recently, the Haemophilia Activities List (HAL), a haemophilia-specific self-assessment questionnaire to assess a patient's self-perceived functional ability, was introduced and a limited pilot study warranted its further development. The present study finalizes the HAL and assesses the convergent and construct validity, as well as the internal consistency of its definitive version. Three questionnaires (HAL, Dutch-Arthritis Impact Measurement Scales 2 and the Impact on Participation and Autonomy questionnaire) were completed by 127 patients with severe haemophilia (<1% clotting activity), as well as four performance tests (button test, 50 metre walking test, timed-up-and-go test and figure-8 walking test). After removal of 15 non-informative items from the provisional HAL, three components within the questionnaire were identified (upper extremity activities, basic lower extremity activities and complex lower extremity activities). The internal consistency of these components was high (Cronbach's alpha = 0.93-0.95), as was internal consistency for the seven domains of the HAL (alpha = 0.61-0.96). The convergent validity of the HAL when compared to the other two questionnaires was good (r = 0.47-0.84). The construct validity of the HAL when compared to the four performance tests was generally lower (r = 0.23-0.77). The final version of the HAL has good internal consistency and convergent validity and gives the clinician insight into a patient's self-perceived ability to perform activities of daily life. It is likely that self-assessment instruments (questionnaires) and performance tests consider different concepts of functional health status and it is therefore recommended that both types are included when clinicians assess a patient's functional abilities.

Activities of Daily Living↗

PubMed2006

Pain and functional limitations in patients with severe haemophilia.

Patients with severe haemophilia often experience pain caused by haemophilic arthropathy, and it is known that pain acts as a predictor of disability. The role of pain in functional limitations in these patients, however, has not been systematically investigated. The aim of this study was to describe pain as experienced by patients with haemophilia and to address the role of pain in subsequent functional limitations. Seventy-eight adult patients (18-70 years) with severe haemophilia completed one questionnaire on pain and one on self-perceived functional abilities. Two-thirds of the patients suffered from more than one painful joint, the ankle being mentioned most frequently (n=43). Analgesics were used by 36% of the patients indicating having pain. Six of 10 most frequently mentioned pain descriptors were of evaluative nature. Evaluative qualities of pain were associated with pain intensity, which in turn was associated with the impact of pain on daily life. Pain outcomes, after adjusting for age and treatment modality, explained 3-22% of the variance in self-perceived functional abilities. Data on radiological joint damage (Pettersson scores) were retrieved for a subgroup of 28 younger patients on prophylaxis and no significant correlations with pain parameters were found. Over two-thirds of the patients with severe haemophilia had one or more painful joints. Pain plays a small, but significant role in functional limitations. In the identified subgroup, arthropathy, as measured by the Pettersson score, seemed to have no role in pain experience. Promoting analgesic use might decrease the impact of pain on functional limitations.

Activities of Daily Living↗

PubMed2006

Physical fitness, functional ability and quality of life in children with severe haemophilia: a pilot study.

In the Netherlands comparable levels of sports-participation between persons with haemophilia and healthy controls have been reported. This raises the question if children with haemophilia under the currently available prophylaxis do reach comparable levels of physical fitness and health-related quality of life (HRQoL) as their healthy peers. The aim of this study was to investigate the level of physical fitness, functional ability and quality of life and to determine the feasibility to safely test the exercise capacity of boys with severe haemophilia A. Thirteen subjects participated in this study. Physical fitness was determined using the measurement of maximal oxygen uptake (VO2peak) attained during a graded maximal exercise test to volitional exhaustion. Joint health, physical activity levels and health-related quality of life (Haemo-Qol) were also measured. Mean VO2peak was 1.86+/-0.77 L min-1 (Z-score: -0.39+/-1.61) which was not significantly different from reference values. Relative VO2peak was 47.42+/-8.29 mL min-1 kg-1 (Z-score: -0.52+/-1.43), which did not differ significantly from reference values either. One boy suffered a joint bleeding one day after the test. Haemo-Qol scores in parents and children ranged from 3.2% to 36.7% (100% reflects poor outcome). Relationship between the child or parent reports of Haemo-QoL and both absolute and relative VO2peak ranged from R=0.00 and R=0.4. Exercise testing in children with severe haemophilia A was a safe procedure. Patients with severe haemophilia A with good joint health and no limitations of activities have comparable physical fitness and physical active lifestyle with healthy peers and good HRQoL.

Adolescent↗

PubMed2005

Physiologic response of the six-minute walk test in children with juvenile idiopathic arthritis.

OBJECTIVE: To determine the physiologic response of the 6-minute walk test (6-mwt) in children with juvenile idiopathic arthritis (JIA). METHODS: Eighteen children with JIA (age 7-17 years; 6 boys, 12 girls) performed a 6-mwt and a maximal exercise test. RESULTS: The physiologic response of the 6-mwt was on average between 80% and 85% of the peak values of heart rate and oxygen uptake (VO2peak) during the maximal exercise test, except for the minute ventilation, which had a mean percentage of 68.5%. Backward regression analysis showed that height and distance walked were the best predictors of VO2peak during cycling (R2 = 0.883, P < 0.001). During the 6-mwt, the difference between the first and second minute was significant in every variable, except for heart rate. The range of walking distance of children with JIA was comparable with that of healthy elderly people. CONCLUSION: The physiologic response of the 6-mwt is at a submaximal, intense level of exercise. The course of the responses during the 6-mwt was normal. The 6-mwt can be regarded as a good test for measuring functional exercise capacity.

Adolescent↗

PubMed2005

Epilepsy surgery does not harm motor performance of children and adolescents.

The impact of epilepsy surgery on motor performance, activities of daily life (ADL) and caregiver assistance was assessed in 37 children (age range 0.1-15.4 years) with pharmacologically untreatable epilepsy, 17 of whom were also diagnosed as having spasticity of cerebral origin. All patients underwent epilepsy surgery between 1996 and 2001 at the Wilhelmina University Children's Hospital and were assessed using a standard protocol at fixed intervals: before surgery and 6 months, 1 year and 2 years after surgery. The type of surgery was hemispherectomy (n = 14) and temporal (n = 14), frontal (n = 4), parietal (n = 2) and central (n = 2) resection. One child underwent callosotomy. Engel's classification was used to determine seizure outcome. Impairments were measured in terms of muscle strength, range of motion and muscle tone. Motor performance of infants and children without spasticity was measured using the Movement Assessment Battery for Children (M-ABC). The Gross Motor Function Measure (GMFM-88) was used in children with spasticity, the severity of motor disability in this group being determined by means of the Gross Motor Function Classification System (GMFCS). Daily activities and caregiver's assistance were measured in all children using the Pediatric Evaluation of Disability Inventory (PEDI). Twenty-four months after surgery 74% of the children could be classified as Engel class 1, indicating a significant seizure reduction. Impairments revealed some decrease in muscle strength and range of motion in the group with spasticity. Scores improved statistically significantly at group level on M-ABC and GMFM (P < 0.05). Improvement in activities of daily life and caregiver's assistance could not be measured in children without spasticity because of the ceiling effect of the PEDI, but children with spasticity improved significantly with respect to these parameters (PEDI) (P < 0.05). Hence, epilepsy surgery does not harm motor performance in children with or without spasticity.

Activities of Daily Living↗

PubMed2005

[Systematic review of the effects of therapy in infants with the KISS-syndrome (kinetic imbalance due to suboccipital strain)].

OBJECTIVE: To establish the effects of manual therapy, chiropractic, or osteopathic treatment of the KISS-syndrome (kinetic imbalance due to suboccipital strain) in infants with positional preference, plagiocephaly, and colic. DESIGN: Systematic review of the literature. METHOD: PubMed, Embase and the Cochrane Library were searched for articles on the effects of manual therapy, chiropractic and osteopathy on the KISS-syndrome. Experts in the field of manual medicine and osteopathy were asked to provide relevant articles. The bibliography in a textbook of manual therapy for children was hand-searched for additional references to the KISS-syndrome. RESULTS: No clinical trials were found that evaluated the effects of manual therapy or osteopathy on either the KISS-syndrome or its symptoms. Pooled analysis of two randomised clinical trials on the effects of chiropractic in infantile colic showed no statistically significant difference between active and control treatments. In addition, we found that 22% of infants showed short episodes of apnoea during manual therapy of the spine, and that one case has been described in which such apnoea resulted in death. CONCLUSION: Given the absence of evidence of beneficial effects of spinal manipulation in infants and in view of its potential risks, manual therapy, chiropractic and osteopathy should not be used in infants with the KISS-syndrome, except within the context of randomised double-blind controlled trials.

Apnea↗

PubMed2005

Thoracic excursion measurement in children with cystic fibrosis.

BACKGROUND: Measurement of thoracic excursion (TEM) during maximal ventilation is part of the physiotherapy assessment in children with cystic fibrosis (CF). OBJECTIVES: The purpose of our study was to examine the reliability of TEM and its relation with pulmonary function. METHODS: Thoracic excursions were measured using a measuring tape. Thirty children participated in an intra-observer and inter-observer reliability study. Reliability was determined by calculating the typical error in repeated measurement, limits of agreement and correlation coefficients. Cross-sectional data from the annual check-ups were used to measure the relation between TEM and pulmonary function. RESULTS: In the intra-observer reliability study the typical error was 0.31 cm, the limits of agreement were +/-0.86 cm. Pearson's r and ICC were 0.96 and 0.95, respectively. In the inter-observer reliability study these values were 0.56 cm, +/-1.55 cm, 0.85 and 0.85, respectively. TEM correlated significantly with height (0.31, P<0.001), FVC (0.44, P<0.001), FEV1 (0.41, P<0.001) and TLC (0.19, P<0.05), and there was a significant inverse correlation with RV%TLC (-0.45, P<0.001). CONCLUSIONS: TEM is a reliable assessment tool. Thoracic excursion is significantly, although moderately correlated with pulmonary function.

Adolescent↗

PubMed2005

Is physical fitness decreased in survivors of childhood leukemia? A systematic review.

The aim of this review is to determine whether physical fitness, assessed by peak oxygen uptake (VO(2peak)) measurement, is reduced in survivors of acute lymphoblastic leukemia (ALL) compared to healthy children. A systematic literature search (up to June 2004) was performed using Medline, Sportdiscus, Cinahl, Embase, Cochrane and PEDro database and reference tracking. The VO(2peak) (ml kg(-1) min(-1)) reached during a maximal exercise test until volitional exhaustion was used as the main outcome for this review. In all, 17 studies were identified in the literature. Data from three studies (102 ALL survivors, age ranging from 7 to 19 years) were pooled in a meta-analysis. Although there was a significant heterogeneity between the included studies (P=0.0006), the standardized mean difference (SMD) value of -0.61 (P=0.07) indicated that VO(2peak) tended to be reduced in survivors of childhood ALL compared to healthy control subjects, that is, decrease of -5.97 ml kg(-1) min(-1) (95% confidence interval (CI): (-12.35, 0.41); P=0.07) or -13% (95 % CI: (-27, 0.004)). Physical fitness tends to be reduced in survivors of ALL during childhood, which suggests the need for this population group to engage in regular physical activities with the purpose of increasing their functional capacity. Although more research is needed, this functional improvement might ameliorate the quality of life of ALL survivors as physical and outdoors activities are an essential part of daily routine during childhood.

Child↗

PubMed2005

The relationship between generalized joint hypermobility and motor development.

PURPOSE: The aim of this study was to investigate the relationship between the presence and localization of joint hypermobility and delay in motor development. METHODS: A retrospective chart review was performed. All children younger than 2.5 years of age and between four and 12 years of age with generalized joint hypermobility were included. Generalized joint hypermobility was assessed using the criteria of Bulbena. In children between one and 2.5 years of age, motor development was measured using the Bayley Scales of Infant Development. In children four to 12 years of age, the Movement Assessment Battery for Children was used. RESULTS: Data from 72 children were available for analysis. In nine of 16 children younger than 2.5 years of age, a delay in motor development was found without a significant association between the delay in motor development (yes/no) and the Bulbena score (odds ratio: 0.4; 95% confidence interval: 0.07-2.1). In children between four and 12 years of age, a severe delay in motor development was present in 14 of 56 children (25%), while 12 of 56 children (21%) were at risk and 30 of 56 (54%) were age appropriate. No significant association between delay in motor development and the Bulbena score was found (odds ratio: 1.3; 95% confidence interval: 0.8-2.1). The age of independent walking was not significantly associated with the number of hypermobile joints (Bulbena score) (linear regression coefficient: 0.3; 95% confidence interval: -1.5 - 2.1). CONCLUSIONS: Although severe delays in motor development may be observed in approximately one third of children with generalized joint hypermobility, there is no association between the amount of generalized joint hypermobility and delay in motor development.

Child↗

PubMed2005

The use of a disability model in haemophilia research.

Most haemophilia research is medically orientated. However, assessment of the impact of disease on the individual is different when viewed from a rehabilitation perspective. Several models are available to explore functioning and health from this perspective. The disablement process (DP) is such a model, and the aim of this study was to introduce this process in haemophilia research to see whether this type of research could lead to meaningful data. Forty-three adult patients with severe haemophilia participated in this study in which the three 'main pathway' domains of the DP (impairments, functional limitations and disability) and two additional factors (intra-individual and risk factors) were addressed. Three questionnaires (HAL, Dutch-AIMS2 and IPA) were incorporated, and Pettersson scores for 21 patients were retrieved. Step-wise and hierarchical regression analysis was used to assess relationship between the various domains. Arthropathy explained 48% of the variance in functional limitations and nearly 25% of the variance in disability. Functional limitations explained 54% of the variance in disability. Patients identified pain as an important aspect of health which addressed 22% and 13% of the variance in functional limitations and disability respectively. Age was correlated with arthropathy (r = 0.85; P < 0.001), whereas psychological health correlated with pain (r = 0.67; P < 0.001). Both variables were also correlated with functional limitations and disability. Analyses adjusting for the effects of age and psychological health were subsequently performed resulting in more insight in the associations within the DP. The use of the DP in haemophilia research proved to be useful.

Activities of Daily Living↗

PubMed2005

Determinants of functional independence and quality of life in children with spina bifida.

OBJECTIVE: To investigate determinants of functional independence and study which functional abilities were determinants for 'health-related quality of life' in children with myelomeningocele. DESIGN: Cross-sectional study by means of clinical assessment, 'disability' measurement and questionnaires. Uni- and multivariate logistic regression models were used to investigate factors that were determinants for these outcomes. Results were expressed as odds ratios (OR) and 95% confidence intervals (95% CI). SETTING: Outpatient spina bifida clinic at a university hospital. SUBJECTS: One hundred and twenty-two children with myelomeningocele. Mean age 7.9; range 1-18 years. MAIN MEASURES: Functional independence as measured by the Pediatric Evaluation of Disability Inventory (PEDI), and quality of life as measured by the Spina Bifida Health Related Quality of Life Questionnaire. RESULTS: Lesion level below L3 (OR 0.4, 95% CI 0.1-1.0), mental status of IQ > or =80 (OR 4.2, 95% CI 1.2-14.9), having no contractures in lower extremities (OR 3.4, 95% CI 1.3-8.8), and having normal strength of knee extensor muscles (OR 4.1, 95% CI 1.4-11.5) were most strongly associated with independence in self-care. Mental status (OR 16.1, 95% CI 2.8-93.9), having no contractures in lower extremities (OR 1.5, 95% CI 1.4-5.3), and normal strength in knee extensors (OR 11.0, 95% CI 1.3-97.0) were the most important determinants for independence in mobility. Concerning functional abilities, being independent with regard to mobility was the most important determinant for 'health-related quality of life' (OR 5.3, 95% CI 1.6-17.4). CONCLUSIONS: In children with myelomeningocele, good muscle strength, mental ability and being independent in mobility appeared to be much more important for daily life function and quality of life than other medical indicators of the disorder.

Activities of Daily Living↗

PubMed2004

Spinal fusion in children with spina bifida: influence on ambulation level and functional abilities.

The aim of this study was to determine the influence of spinal fusion on ambulation and functional abilities in children with spina bifida for whom early mobilization was stimulated. Ten children (three males and seven females) with myelomeningocele were prospectively followed. Their mean age at operation was 9.3 years (standard deviation (SD): 2.4). Spinal curvature was measured according to Cobb. Pelvic obliquity and trunk decompensation were measured as well. The ambulation level was scored according to Hoffer, and functional abilities, as well as the amount of caregiver assistance, were documented using the Pediatric Evaluation of Disability Inventory. All patients were assessed before surgery and three times after surgery, with a total follow-up duration of 18 months after surgery. After spinal fusion, magnitude of primary curvature decreased significantly (p=0.002). Pelvic obliquity and trunk decompensation did not change. In spite of less immobilization as compared with other reported experiences, ambulation became difficult in three out of four patients who had been able to ambulate prior to surgery. Functional abilities and amount of caregiver assistance concerning self-care (especially regarding dressing upper and lower body, and self-catheterization) and mobility (especially regarding transfers) showed a nonsignificant trend to deterioration within the first 6 months after surgery, but recovered afterwards. From pre-surgery to 18 months after surgery, functional skills on self-care showed borderline improvement (p=0.07), whereas mobility did not (p=0.2). Mean scores on caregiver assistance improved significantly on self-care (p=0.03), and borderline on mobility (p=0.06), meaning that less caregiver assistance was needed compared with pre-surgery. The complication rate was high (80%). In conclusion, within the first 6 months after spinal fusion, more caregiver assistance is needed in self-care and mobility. It takes about 12 months to recover to pre-surgery level, while small improvement is seen afterwards. After spinal fusion, ambulation often becomes difficult, especially in exercise walkers. These findings are important for health-care professionals, in order to inform and prepare the patients and their parents properly for a planned spinal fusion.

Activities of Daily Living↗

PubMed2004

Functional consequences of hemispherectomy.

Using the International Classification of Functioning Disability and Health (ICF) (WHO, 2001), impairments, activities and social participation are reported in 12 children (mean age at surgery 5.9 years) who were investigated before and three times over a 2-year period after hemispherectomy. Impairments were assessed (i) in terms of seizure frequency (Engel classification) and seizure severity (HASS) and (ii) with respect to muscle strength (MRC), range of motion (JAM score) and muscle tone (modified Ashworth scale). Activities were assessed in terms of gross motor functioning (GMFM) and self-care, mobility and social function (PEDI). Participation was assessed in terms of epilepsy-related restrictions and quantified by means of the Hague Restrictions in Childhood Epilepsy Scale (HARCES). Nine out of 12 children could be classified as free of seizures (Engel class I), and in the remaining three seizure frequency was Engel class III. HASS scores showed maximum improvement in 10 out of 12 children and near-maximum improvement in the two remaining children. Muscle strength and muscle tone on the side of the body contralateral to the hemispherectomy, which were already decreased preoperatively, decreased even further in the first 6 months after surgery, but returned to the presurgical baseline thereafter, except for the distal part of the arm. Range of motion was abnormal prior to operation and remained so after operation. Mean GMFM increase was 20% after 2 years (95% confidence interval 10-33); all five dimensions improved statistically significantly (P < 0.05). Mean PEDI increase was more than 20 scale points (95% confidence interval 10-35); again, all domains improved significantly (P < 0.05). In nearly all children, HARCES scores had normalized 2 years after surgery. In conclusion, decrease of seizure frequency and severity widens the scope of motor and social functioning, which overrides the effects of remaining motor impairments.

Child↗

PubMed2004

Functional consequences of haemophilia in adults: the development of the Haemophilia Activities List.

Several instruments can be used to evaluate the functional status of patients with haemophilia, but none of these instruments is specific for haemophilia. We developed a haemophilia-specific self-assessment questionnaire to evaluate and monitor a patient's perceived functional health status: the Haemophilia Activities List (HAL). In three separate but interlinked substudies, the questionnaire was constructed and tested for face, expert, and convergent validity, as well as internal consistency and patient-evaluated relevance. Items for the questionnaire were collected by interviewing 162 patients, using the McMaster-Toronto Arthritis Patient Preference Disability Questionnaire (MACTAR). The items were combined to generate the first version of the questionnaire [HAL(1)]. This version was evaluated and commented on by two focus groups (patients and caregivers), and then the questionnaire was adapted on the basis of these comments, forming the final version, HAL(2). This version was then validated in a pilot study with 50 consecutive patients using the Dutch Arthritis Impact Measurements Scales 2 (Dutch-AIMS2) and the Impact on Participation and Autonomy (IPA) questionnaires. The HAL(2) showed good convergent validity (Pearson correlation 0.80-0.91; P < 0.01), and the internal consistency was good for six of the eight domains (Cronbach's alpha 0.83-0.95). Patients considered the content of the HAL to be more relevant to their situation than the content of the other questionnaires (P < 0.01). Three major factors (upper extremity function, lower extremity function, key activities/major problem activities) were identified by factor analysis. The questionnaire seems to be a useful tool to identify problematic activities as part of the functional health status of patients with haemophilia. The construct validity, test-retest reliability, and responsiveness of the HAL will be established in the future.

Activities of Daily Living↗

PubMed2004

Spina bifida at the sacral level: more than minor gait disturbances.

OBJECTIVE: To investigate functional outcome in two groups of children with sacral level paralysis: myelomeningocele (MMC) versus lipomyelomeningocele (LMMC). Additionally both groups were compared with each other and when possible with reference values. DESIGN: Cross-sectional study by means of (1) clinical assessment, and (2) disability measurement. SETTING: Spina bifida outpatient clinic at a university hospital in the Netherlands. SUBJECTS: Sample of 30 children with MMC and 14 with LMMC. Mean age (SD) 6.0 (4.9) and 8.4 (4.9) years respectively. MAIN MEASURES: Muscle strength, ambulation level, motor performance (Bayley Scales of Infant Development (BSID) and Movement Assessment Battery for Children), and the Pediatric Evaluation of Disability Inventory (PEDI). RESULTS: The majority of patients in both groups were normal ambulant, 14/21 (67%) in MMC and 9/14 (64%) in LMMC. Ambulation was strongly associated with muscle strength of hip abductors (odds ratio (OR): 13.5, 95% confidence interval (CI) 2.5-73.7), and ankle dorsal-flexor muscles (OR: 110, 95% CI 8.9-135.9). No significant differences were found in lesion and ambulation level. Muscle strength and motor performance were significantly lower in the MMC group than in the LMMC group (p < 0.05). PEDI scores were comparable in both groups. Most problems were noted in mobility skills and caregiver assistance in self-care, especially regarding bladder and bowel management. CONCLUSIONS: Gross motor and functional problems were seen in both groups. The MMC group showed more muscle weakness and motor problems. However, in both groups caregiver assistance was needed for a prolonged period, especially regarding bladder and bowel management. These findings need special attention, particularly in children who attend regular schools.

Activities of Daily Living↗

PubMed2003

Osteogenesis imperfecta in childhood: effects of spondylodesis on functional ability, ambulation and perceived competence.

We studied the effects of spondylodesis on spinal curvature, functional outcome, level of ambulation and perceived competence in 11 children with osteogenesis imperfecta (OI). Mean age at surgical intervention was 13.1 years (SD 2.5 years) and follow-up amounted to 3.4 years (SD 2.3 years). Spinal curvature was measured according to Cobb. The level of ambulation was scored according to the modified criteria of Bleck. Functional abilities and the amount of parental assistance were scored using the Dutch version of the Pediatric Evaluation of Disability Inventory (PEDI). Perceived competence was measured using the Harter Self-Perception Profile for Children. The amount of fatigue, spinal pain and presence of subjective dyspnea were scored with a visual analog scale. The median progression per year before spondylodesis was 6.1 degrees (interquartile range 2.9 degrees -12.9 degrees ) and after the spondylodesis it was 5.0 degrees (interquartile range 1.6 degrees -11.0 degrees ). No significant progression or regression in the level of ambulation was found. Perceived competence improved slightly. In the total score of the perceived competence, a borderline significant increase was found ( P-value 0.068). We concluded that spinal fusion in children with OI does not materially influence functional ability and level of ambulation. Self-perceived competence seemed to improve after surgery. The amount of pain, fatigue and subjective dyspnea seemed to diminish after spinal surgery. Progression of scoliosis proceeded, as did development of spinal curvature at the junction of the spondylodesis. Therefore, oral or intravenous bisphosphonates before and after spinal surgery should be considered.

Activities of Daily Living↗