Impaired physical function following pediatric LT.

The purpose of this article is to investigate the spectrum of physical function of pediatric liver transplantation (LT) recipients 12-24 months after LT. Review data were collected through the functional outcomes group, an ancillary study of the Studies of Pediatric Liver Transplantation registry. Patients were eligible if they had survived LT by 12-24 months. Children ≥ 8 years and parents completed the Pediatric Quality of Life Inventory™ 4.0 generic core scales, which includes 8 questions assessing physical function. Scores were compared to a matched healthy child population (n = 1658) and between survivors with optimal versus nonoptimal health. A total of 263 patients were included. Median age at transplant and survey was 4.8 years (interquartile range [IQR], 1.3-11.4 years) and 5.9 years (IQR, 2.6-13.1 years), respectively. The mean physical functioning score on child and parent reports were 81.2 ± 17.3 and 77.1 ± 23.7, respectively. Compared to a matched healthy population, transplant survivors and their parents reported lower physical function scores (P < 0.001); 32.9% of patients and 35.0% of parents reported a physical function score <75, which is > 1 standard deviation below the mean of a healthy population. Physical functioning scores were significantly higher in survivors with optimal health than those with nonoptimal health (P < 0.01). There was a significant relationship between emotional functioning and physical functioning scores for LT recipients (r = 0.69; P < 0.001). In multivariate analysis, primary disease, height z score < -1.64 at longterm follow-up (LTF) visit, > 4 days of hospitalization since LTF visit, and not being listed as status 1 were predictors of poor physical function. In conclusion, pediatric LT recipients 1-2 years after LT and their parents report lower physical function than a healthy population. Findings suggest practitioners need to routinely assess physical function, and the development of rehabilitation programs may be important.

Youth and young adults with spina bifida: their utilization of physician and hospital services.

OBJECTIVE: To describe current patterns of health care utilization of youth and young adults who have spina bifida (SB) and provide evidence to guide the development of health care for this growing population. DESIGN: We conducted a secondary analysis of health services utilization data from the Canadian Institute for Health Information to determine the rates and patterns of health care utilization, because comprehensive health care has been recognized as critical to positive health outcomes. SETTING: Participants were identified from 6 publicly funded children's treatment centers. PARTICIPANTS: Health records from youth (n=164; age range, 13.0-17.9y) and adults (n=120; age range, 23.0-32.9y) with SB contributed to this study. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: The rates of outpatient physician visits and hospital admissions for the youth and adult groups were calculated. The proportion with a "medical home" was also calculated. RESULTS: The annual rates of outpatient physician visits per 1000 persons were 8031 for youth and 8524 for adults with SB. These rates were approximately 2.9 and 2.2 times higher, respectively, than for their age-matched peers. On average, 12% of youth and 24% of adults with SB had a medical home. The annual rates of hospital admissions per 1000 persons were 329 for youth and 285 for adults with SB. Rates of admissions were 19.4 and 12.4 times higher, respectively, for these groups than for the general population. CONCLUSIONS: It appears that persons with SB are accessing health services more often than their age-matched peers, and few have a medical home. We recommend that seamless medical care be provided to all adults with SB, coordinated by a primary care provider, to facilitate comprehensive care.

Quantitative Musculoskeletal Imaging of the Pediatric Shoulder.

ABSTRACT: Pediatric acquired and congenital conditions leading to shoulder pain and dysfunction are common. Objective, quantitative musculoskeletal imaging-based measures of shoulder health in children lag recent developments in adults. We review promising applications of quantitative imaging that tend to be available for common pediatric shoulder pathologies, especially brachial plexus birth palsy and recurrent shoulder instability, and imaging-related considerations of musculoskeletal growth and development of the shoulder. We highlight the status of quantitative imaging practices for the pediatric shoulder and highlight gaps where better care may be provided with advances in imaging technique and/or technology.

Handrim kinetics and quantitative ultrasound parameters for assessment of subacromial impingement in wheelchair users with pediatric-onset spinal cord injury.

BACKGROUND: Most manual wheelchair users with pediatric-onset spinal cord injury (SCI) will experience shoulder pain or pathology at some point in their life. However, guidelines for preservation of the upper limb in children with SCI are limited. RESEARCH QUESTION: What are the relationships between manual wheelchair handrim kinetics and quantitative ultrasound parameters related to subacromial impingement in individuals with pediatric-onset SCI? METHODS: Subacromial impingement risk factors including supraspinatus tendon thickness (SST), acromiohumeral distance (AHD), and occupation ratio (OR; SST/AHD) were measured with ultrasound in 11 manual wheelchair users with pediatric-onset SCI. Handrim kinetics were acquired during the stroke cycle, including peak resultant force (FR), peak rate of rise of resultant force (ROR) and fractional effective force (FEF). Variability of handrim kinetics was computed using the coefficient of variation and linear regression was performed to assess correlations between handrim metrics and quantitative ultrasound parameters. RESULTS: Peak resultant force significantly increased 1.4 % and variability of FEF significantly decreased 8.0 % for every 0.1 cm increase in AHD. FEF decreased 3.5 % for every 0.1 cm increase in SST. Variability of peak resultant force significantly increased 3.6 % and variability of peak ROR of resultant force significantly increased 7.3 % for every 0.1 cm increase in SST. FEF variability significantly decreased 11.6 % for every 0.1 cm increase in SST. Peak ROR significantly decreased 1.54 % with every 10 % increase in OR. FEF variability significantly decreased 1.5 % with every 10 % increase in OR. SIGNIFICANCE: This is the first study to investigate relationships among handrim kinetics and shoulder structure in manual wheelchair users with pediatric-onset SCI. Associations were identified between subacromial impingement risk factors and magnitude and variability of wheelchair handrim kinetics. These results indicate the critical need to further explore the relationships among wheelchair handrim kinetics, shoulder joint dynamics, and shoulder pathology in manual wheelchair users with pediatric-onset SCI.

Health outcomes among youths and adults with spina bifida.

OBJECTIVE: To describe the health and health-related quality of life (HR-QoL) outcomes of youths and young adults with spina bifida. STUDY DESIGN: One global rating of self-rated health and 2 generic measures of HR-QoL were administered to a group of youths and young adults with spina bifida. HR-QoL was measured using the Health Utilities Index Mark 3 (HUI3) and the Assessment of Quality of Life version 1 (AQoL). RESULTS: Data was obtained from 40 youth (mean age 16.0 years) and 13 young adults (mean age 26.6 years). Most youth rated their overall health as either excellent or very good (65%) compared with fewer adults (23%) (P = .007). The mean HR-QoL scores for youths versus adults were 0.57 versus 0.36 (P = .03) for the HUI(3) and 0.37 versus 0.25 for the AQoL (P = .09). HUI(3) and AQoL scores were correlated with level of anatomic lesion (rho = 0.64 and rho = 0.42, respectively). CONCLUSIONS: The HR-QoL of youths and young adults with spina bifida was low on measures that are aggregated using societal values (the HUI3 and AQoL). This is in contrast to their single global self-ratings of health, which were more favorable. These findings underscore the distinction between ratings of HR-QoL based on societal values versus the personal lived experiences of adults with childhood-onset disability.

2023 updates to the spina bifida transition to adult care guidelines.

PURPOSE: This article provides an update to the 2018 Spina Bifida Association's Transition to Adult Care Guidelines. METHODS: A workgroup of topic experts was convened including authors from the initial guideline workgroup. The workgroup reviewed and updated the primary, secondary, and tertiary outcome goals, clinical questions, and guideline recommendations based on a literature review. RESULTS: Twenty-two additional articles were identified from the literature search. Updated references included observational studies describing transition to adult care outcomes, transition care model initiatives, and a validated self-management assessment tool. CONCLUSION: Structured transition initiatives increase the likelihood of establishing with adult care, decrease acute care use for young adults with spina bifida, and have the potential to improve quality of life and optimize chronic condition management. However, there is still a need to implement structure transition practices more broadly for this population using these recommended guidelines.

Mobility guidelines for the care of people with spina bifida.

The Spina Bifida Association (SBA) is the organization that represents the needs of the population with spina bifida (SB). They are tasked with advocacy, education, optimizing care, and providing a social voice for those with spina bifida. In response to the tenet of optimizing care they were tasked with developing up to date clinical care guidelines which address health care needs for those impacted by spina bifida throughout their lifespan. This article will discuss the SB Mobility Healthcare Guidelines from the 2018 Spina Bifida Association's Fourth Edition of the Guidelines for the Care of People with Spina Bifida.

Obesity and anthropometry in spina bifida: What is the best measure.

OBJECTIVE: Diagnosis of obesity using traditional body mass index (BMI) using length may not be a reliable indicator of body composition in spina bifida (SB). We examine traditional and surrogate measures of adiposity in adults with SB, correlated with activity, metabolic disease, attitudes towards exercise and quality of life. DESIGN: Adult subjects with SB underwent obesity classification using BMI by length and arm span, abdominal girth and percent trunk fat (TF) on dual energy X-ray absorptiometry (DXA). Quality of life measures, activity level and metabolic laboratory values were also reviewed. RESULTS: Among eighteen subjects (6 male, 12 female), median age was 26.5 (range 19-37) years, with level of lesion 16.7% ≤L2, 61.1% L3-4, and 22.2% ≥L5, respectively. Median weight was 71.8 (IQR 62.4, 85.8) kg, similar between sexes (P = 0.66). With median length of 152.0 (IQR 141.8, 163.3) cm, median conventional BMI was 29.4 m/kg2, with 7 (43.8%) subjects with BMI >30. Median BMI by arm span was 30.2 m/kg2, abdominal girth of 105.5 cm, and TF 45.7%. More subjects were classified as obese using alternate measures, with 9 (56.3%) by arm span, 14 (82.4%) by abdominal girth and 15 (83.3%) by TF (P = 0.008). Reclassification of obesity from conventional BMI was significant when using TF (P = 0.03). No difference in quality of life measures, activity level and metabolic abnormalities was demonstrated between obese and non-obese subjects. CONCLUSIONS: Conventional determination of obesity using BMI by length is an insensitive marker in adults with SB. Adults with SB are more often classified as obese using TF by DXA.

Transition to adulthood in spina bifida: changing roles and expectations.

Survival to adulthood for people with Spina Bifida now exceeds 85% due to improvements in medical and surgical management. Rates remain lower than expected for community participation, healthy lifestyle choices, employment and independent living. The importance of transition programming to help adolescents with disabilities prepare for adult life roles is now understood. Literature currently is mainly conceptual or descriptive, but informs the process of developing transition program models. The need for competent and effective adult care providers is discussed. Both the transition to adulthood and the transfer of care to adult care clinics are important and distinct components of spina bifida lifespan care.

Care for Adults with Spina Bifida: Current State and Future Directions.

The care for adults with spina bifida is an important area to study. As increasing numbers of patients with spina bifida survive into adulthood, they expect to thrive and receive the best possible care into adulthood to maintain their health. Understanding the health needs in this emerging and changing population will help clinicians provide the best anticipatory care for adults with spina bifida and continue to improve outcomes. This will also impact pediatric care by improving the ability to determine preventive methods from early on and understand the impacts of pediatric care and decisions over the lifespan.

A snapshot of the adult spina bifida patient - high incidence of urologic procedures.

INTRODUCTION: To describe the urologic outcomes of contemporary adult spina bifida patients managed in a multidisciplinary clinic. MATERIAL AND METHODS: A retrospective chart review of patients seen in our adult spina bifida clinic from January 2004 to November 2011 was performed to identify urologic management, urologic surgeries, and co-morbidities. RESULTS: 225 patients were identified (57.8% female, 42.2% male). Current median age was 30 years (IQR 27, 36) with a median age at first visit of 25 years (IQR 22, 30). The majority (70.7%) utilized clean intermittent catheterization, and 111 patients (49.3%) were prescribed anticholinergic medications. 65.8% had urodynamics performed at least once, and 56% obtained appropriate upper tract imaging at least every other year while under our care. 101 patients (44.9%) underwent at least one urologic surgical procedure during their lifetime, with a total of 191 procedures being performed, of which stone procedures (n = 51, 26.7%) were the most common. Other common procedures included continence procedures (n = 35, 18.3%) and augmentation cystoplasty (n = 29, 15.2%). Only 3.6% had a documented diagnosis of chronic kidney disease and 0.9% with end-stage renal disease. CONCLUSIONS: Most adult spina bifida patient continue on anticholinergic medications and clean intermittent catheterization. A large percentage of patients required urologic procedures in adulthood. Patients should be encouraged to utilize conservative and effective bladder management strategies to reduce their risk of renal compromise.

Transition to adult care for patients with spina bifida.

More individuals with spina bifida are living into adulthood, and unique challenges arise as they age. These patients have multiple organ system involvement in addition to physical impairments, disabilities, cognitive involvement, and psychosocial challenges. There is a growing need for transitional care for adults with spina bifida. This article explores the 5 key elements for a transition program to adult care: preparation, flexible timing, care coordination, transitional clinic visits, and health care providers who are interested in taking care of adults with disabilities.

Quality of life related to urinary continence in adult spina bifida patients.

INTRODUCTION: To analyze the correlations of bladder management technique, ambulatory status and urologic reconstruction on quality of life (QOL) as affected by urinary symptoms in adult spina bifida (SB) patients. MATERIAL AND METHODS: Sixty-six adult SB patients completed the RAND 36-Item Health Survey (mSF-36) and Incontinence Quality of Life (I-QOL). Demographic information, history of urinary reconstruction, and bladder management techniques were reviewed and analyzed with respect to survey scores. RESULTS: Mean age of patients was 32.3 (SD ±7.2) years and 44 patients (66.7%) were female. Forty-five patients (68.2%) were mainly ambulatory, 21 (31.8%) use a wheelchair and 10 (15.2%) had urologic reconstruction, while 56 (83.3%) did not. Twelve patients (18.2%) void, 42 (63.6%) perform clean intermittent catheterization (CIC), 4 (6.1%) use an indwelling catheter, 3 (4.5%) have an ileal conduit (IC) and 5 (7.6%) mainly use diapers. Mean mSF-36 General Health score was 56.5 (SD ±22.9) and mean I-QOL Sum score was 50.9 (SD ±21.7), where lower scores reflect lower QOL. mSF-36 and I-QOL scores did not significantly correlate with bladder management technique, ambulatory status or urologic reconstruction. A correlation was noted between I-QOL scales and most mSF-36 scales (all p <0.02). CONCLUSIONS: In our cohort study of adult SB patients, bladder management technique and urologic reconstruction did not correlate with urinary (I-QOL) or general health (mSF-36) domains, although I-QOL and mSF-36 scores correlated closely, suggesting urinary continence is significantly related to general QOL. However, we are unable to identify a single factor that improves either urinary or general QOL.

Pain management in a pediatric rehabilitation setting.

This article reviews the current understanding of pain evaluation as applied to children who have chronic illness and disabilities. Utilizing a collaborative medical approach, psychiatric principles of management are discussed. Case scenarios are presented to outline application of general strategies of clinical management.

Risk of bladder cancer in patients with spina bifida: case reports and review of the literature.

PURPOSE: This article presents four cases of patients with spina bifida who developed bladder cancer while under our care and provides a literature review on the incidence, initial presentation, possible etiologies, and role for screening for bladder cancer in the neurogenic bladder population. METHODS: Case reports of four patients are presented followed by a literature review of the current available studies. RESULTS: Patients with spina bifida present with bladder cancer at an atypically young age with very advanced disease. The dire prognosis associated with bladder cancer in these patients demands that we provide better treatment, diagnosis, and prevention modalities. However, the potential morbidity, financial burden, and lack of proven benefit discourage cystoscopic screening in this patient population. Until we have more data on how to best serve spina bifida patients, this population should receive careful and regular urologic follow-up. CONCLUSION: Given the atypical young age of presentation and very advanced nature of bladder cancer in the spina bifida population, the authors strongly recommend that any new bladder changes, such as including increased urinary leakage, pain, recurrent infections, or increased gross hematuria, prompt immediate urologic referral for endoscopic evaluation and biopsy as indicated.

Barriers to community participation: Teens and young adults with spina bifida.

This study identified the baseline participation rates for 101 teens and young adults ages 10-32 years old with a diagnosis of spina or lipomenigocele bifida in various domains: school, employment, community activities, physical activity and peer social relationships. The goal of the study was also to identify barriers to community participation. Our findings demonstrate that overall participation is low in several domains. Community participation is low with only 30% partaking in an organized community activity at least once a week. Multiple individual, family, and environmental barriers were identified by participants and their family. The most frequent barriers identified were low motivation (38%), lack of information (25%) and time constraints (21%). Barriers need to be addressed on an individualized basis as well as addressing the community as a whole. Future plans are to intervene based on the barriers and reassess participation at 6 months and a year with the goal of increased long term participation, employment, quality of life and social relationships.