Shubhra Mukherjee

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.

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.

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.

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.

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.

Disability Stigma in Rehabilitation

In the 26 years since the Americans with Disabilities Act was passed, we have seen changes in access, knowledge, and a move towards equity for people with disabilities; however, societal attitudes and implicit and explicit biases continue to impact the way that people with disabilities are viewed, represented, and treated. A simple definition of stigma from Merriam Webster is “a set of negative and often unfair beliefs that a society or group of people have about something” [1]. In his classic book, Stigma: On the Management of a Spoiled Identity [2], Erving Goffman, a sociologist by training, described and delineated the concept of stigma and how it impacts society. He described complex phenomena that include devaluation and marginalization. Although critiqued and refined over the years, the concept of stigma that Goffman described is a robust and important one to consider. Stigma, and more specifically disability stigma, is part of the social, psychological and ethical context in which physical medicine and rehabilitation (PM&R) clinicians practice. This columnwill explore the concept of disability stigma and its impact on the practice of rehabilitation medicine. This idea for a column is based in large part on a course that I teach to undergraduates at Northwestern University called “Disability and Global Health,” in which we define, discuss, and consider disability stigma in various contexts around the world. We also discuss disability rights and disability ethics in the United States. One of the first reading assignments for the course is an article by Lerita Coleman, “Stigmadan enigma demystified” [3]. Coleman writes that stigma has at least 3 components: