A multi-site cultural and linguistic adaptation of a hypospadias decision aid for Latinx communities.

INTRODUCTION: Latinx, Spanish-speaking (LSS) patients are more likely to experience decisional conflict and regret about healthcare decisions than non-Hispanic, white, English-speaking patients. OBJECTIVE: To adapt the Hypospadias Hub (Hub), a rigorously developed and tested web-based decision aid (DA), for LSS parents. METHODS: Guided by the Ecological Validity Model (EVM), a heuristic framework was followed to culturally adapt the Hub (see Extended Summary Figure). In stage 1, recommendations were obtained from a focus group with members of the institution's Latinx Community Review Board (Latinx CRB) and semi-structured interviews with pediatricians with Latinx-focused practices. In stage 2, preliminary cultural modifications were made, the Hub was translated into Spanish, and a second focus group with the Latinx CRB was convened to review the revised Hub. In stage 3, semi-structured interviews with LSS parents of healthy boys (i.e., without hypospadias) ≤ 5 years old were conducted to identify any cultural adaptations and/or usability issues regarding the revised Hub. In stage 4, based on parents' feedback, final revisions to the Hub were made. The focus groups and parent interviews were conducted in Spanish; then, the recordings were professionally transcribed in Spanish and translated into English. Interviews with pediatricians were conducted in English; then, the recordings were professionally transcribed. Three coders conducted a qualitative content analysis to identify areas for revision. Changes were applied iteratively. RESULTS: Participants included 3 Latinx CRB members (2 women, 1 did not disclose gender; mean age = 48.3, SD = 21.2), 3 non-Latinx pediatricians (2 women, 1 man; mean age = 49.6, SD = 9.1), and 5 Latinx mothers (mean age = 34.0, SD = 1.26). Participants recommended: 1) featuring video testimonials from Latinx families or including Spanish voice-overs/subtitles; 2) diversifying racial/ethnic/geographic representation and including extended families in photographs/illustrations; 3) adding information about health insurance coverage and circumcision, 4) reassuring parents that the condition is not their fault, 5) considering cultural values (e.g., reliance on expert advice), and 6) clarifying medical terminology. Feedback related to seven EVM dimensions: concepts, content, context, goals, language, metaphors, and methods. DISCUSSION: Participants perceived the Hub to be informative in guiding parents' treatment decisions. Revisions were reasonable and acceptable for a linguistic and cultural adaptation for LSS parents. CONCLUSIONS: We identified and implemented preliminary cultural modifications to the Hub and applied user-centered design methods to test and revise the website. The product is a culturally appropriate DA for LSS parents. Next, English and Spanish-versions of the Hub will be tested in a randomized controlled trial.

Parental decision regret after pediatric urologic surgeries compared to decisions of everyday life.

INTRODUCTION: Parents are at risk of decision regret (DR) for decisions affecting their children. The Decision Regret Scale (DRS) measures medical DR but lacks context outside of healthcare. OBJECTIVE: To compare parental DR 1) between common pediatric urologic surgeries and everyday decisions and 2) with preference to make a different choice. METHODS: We conducted a cross-sectional online survey of randomly selected parents >1year (y) after their children underwent: orchiopexy (males ≤10y), open ureteral reimplant (OUR, females 2-6y), open pyeloplasty (OP, ≤2y), or robotic pyeloplasty (RP, 5-17y) (2017-2021). Higher DRS scores indicate increased DR (none: 0, mild: 1-25, moderate: 30-50, strong: 55-75, very strong: 80-100). Parents completed DRS on four decisions: their child's surgery, most recent/current romantic relationship, most recent leased/purchased car, and most recent purchased meal. Parents reported if they would make the same choice (yes/no). Nonparametric statistics were used. RESULTS: We surveyed 191 parents (orchiopexy n = 52, OUR n = 50, OP n = 51, RP n = 38). The median parent age was 36y (mothers: 86%). Some DR was reported for all decisions, but with significant differences in DR severity. The lowest median DRS score was seen with surgery (orchiopexy 0 [IQR 0-10], OUR 0 [IQR 0-5], OP 0 [IQR 0-0], RP 0 [IQR 0-0]), with no difference between surgery groups (p = 0.78). This was followed by relationship (0, IQR 0-20), car (15, IQR 0-25), and meal (20, IQR 0-30, p < 0.001). Most parents did not report any DR regarding surgery (orchiopexy 69%, OUR 74%, OP 76%, RP 76%, with no difference between surgery groups p = 0.85, Summary Figure). Comparatively, 59% of parents did not have any regret about their relationship, 37% their car, and 28% their meal (p < 0.001). All surgical DR was mild or moderate. No parent (0%) would have chosen differently for their child's surgery versus 4-12% for non-surgical decisions (p < 0.001). Overall, increasing DR corresponded to increasing desire to have made a different choice (DRS≤10: 0%, DRS 45-50: 32%, DRS 55-60: 66%, DRS≥75: 100%, p < 0.001). CONCLUSION: Parental DR varied between urological surgical and non-surgical decisions. It was lowest after surgery. Some regret was reported after every decision, but the subset of parents with regret was smallest after surgical decisions. Positive DRS scores do not necessarily correspond to parents wishing they made a different choice.

Accuracy in reporting incontinence in adults with spina bifida: A pilot study.

BACKGROUND: The accuracy of self-reported urinary incontinence (UI) and fecal incontinence (FI) among adults with spina bifida (SB) is unknown. We aimed to quantify the accuracy of self-reported recall incontinence in the last 4 weeks using prospective diary data. METHODS: Adults with SB were enrolled via patient advocacy groups in a larger 30-day smartphone-based ecological momentary assessment study of daily well-being and incontinence. We examined agreement between yes/no questions on exit questionnaires ("In the last 4 weeks, did you leak any urine and get your underwear, pads, pull-ups or disposable underwear wet?") and 30-day diaries. Collected data included a non-validated 4-item UI Negativity scale (UIN: 0-100, 0 = no impact). Inter-rater reliability was assessed with Cohen's kappa (>0.60 = substantial). FI was analyzed similarly. Sub-groups were too small for statistical analysis. RESULTS: Median age of 88 adults was 35 years old (70% female, 53% shunted, 71% community ambulators). Among 81 adults reporting UI in the last month, 79 (98%) had diary-documented UI (UI agreement), 2 (2%) did not (Summary Table). Among 7 adults reporting no UI in the last month, 5 (71%) did not record UI in their diaries (agreement in no UI), 2 (29%) did. Both adults reporting no UI on exit questionnaires, despite contrary diary data, experienced single UI episodes (negativity: 0 and 6). In contrast, the UI agreement group recorded UI on median 18 days (median negativity: 22). Sensitivity of the recall UI question was 0.98 (specificity 0.71, kappa 0.69). Among 66 adults reporting FI in the last month, 65 (98%) had diary-documented FI (FI agreement), 1 (2%) did not. Among 22 adults reporting no FI in the last month, 17 (77%) did not record FI in their diaries (agreement in no FI), 5 (23%) did. Five adults reporting no FI on exit questionnaires, despite contrary diary data, experienced a median single episode (median negativity: 8). In contrast, the FI agreement group recorded FI on median 5 days (median negativity: 31). Sensitivity of the recall FI question was 0.93 (specificity 0.94, kappa 0.81). Results were unchanged when analyzing the first and last 28 days of data. COMMENT: Sensitivity/specificity of single incontinence questions approach those reported for women with UI but without SB. Diaries may best serve SB adults with bothersome incontinence. CONCLUSIONS: Self-reported incontinence accurately and reliably captures UI and FI among adults with SB. It may minimize less bothersome incontinence, supporting its use in screening for clinical practice and research.

Prevalence of sexual abuse and intimate partner violence in adults with spina bifida.

BACKGROUND: People with disabilities and chronic medical conditions are known to be at higher risk of sexual abuse (SA) and intimate partner violence (IPV). People with spina bifida (SB) are vulnerable, but little is known about the prevalence of abuse in this population. OBJECTIVE: To evaluate the prevalence and risk factors of SA and IPV in adults with SB. METHODS: An anonymous international cross-sectional online survey of adults with SB asked about history of SA ("sexual contact that you did not want") and IPV ("hit, slapped, kicked, punched or hurt physically by a partner"). RESULTS: Median age of the 405 participants (61% female) was 35 years. Most self-identified as heterosexual (85%) and were in a romantic relationship (66%). A total of 19% reported a history of SA (78% no SA, 3% preferred not to answer). SA was more frequently reported by women compared to men (27% vs. 5%, p < 0.001) and non-heterosexual adults compared to heterosexuals (41% vs. 15%, p < 0.001). Twelve percent reported a history of IPV (86% no IPV, 2% preferred not to answer). IPV was more frequently reported by women compared to men (14% vs. 9%, p = 0.02), non-heterosexuals compared to heterosexuals (26% vs. 10%, p = 0.002), and adults with a history of sexual activity versus those without (14% vs. 2%, p = 0.01). CONCLUSION: People with SB are subjected to SA and IPV. Women and non-heterosexuals are at higher risk of both.

Urinary and Fecal Incontinence During Sexual Activity Is Common and Bothersome Among Adults With Spina Bifida.

OBJECTIVE: To evaluate the prevalence of and risk factors for urinary fecal incontinence (UI, FI) during sexual activity (UIS, FIS) among adults with spina bifida (SB). METHODS: An international online survey of adults with SB was administered through SB clinics and SB organizations via social media. Adults with a history of masturbation or partnered sexual activity were included. The primary outcome was ever experiencing UIS/FIS. Nonparametric tests and logistic regression were used for analysis. RESULTS: A total of 341 adults met inclusion criteria (median age: 36years, 59% female, 52% shunted, 48% community ambulators). Baseline UI in the last 4weeks was reported by 50% and FI by 41%. Nineteen (5%) had a urostomy. Eight (2%) had a colostomy. Overall, 93% had a history of partnered genital contact. Among adults without a diversion, UIS was more common than FIS (70% vs 45%, P < .001). Among adults without a urostomy, UIS was more common among women (76% vs 62%, P = .01) and those with baseline UI (84% vs 50%, P < .001). UIS was not associated with age, shunt, ambulatory, or catheterization status (P >=.32). On bivariate analysis, female sex and baseline UI were independent predictors of UIS (P <=.001). Among adults without a colostomy, FIS was associated with female sex (50% vs 39%, P = .046), baseline FI (59% vs 32%, P < .001), community ambulation (52% vs 40%, P = .04), but not age, shunt, or MACE status (P >=.27). On multivariate analysis, baseline FI was independently associated with FIS (P < .001). Among adults with UIS/FIS, 29% experienced UIS "almost always" to "always," compared to 5% for FIS (P < .001). Virtually all adults found UIS/FIS bothersome (>=96% for each), even when incontinence occurred "almost never." UIS/FIS mostly occurred before and/or during orgasm than afterward (P < .001). UIS was reported by 53% of adults with a urostomy (100% bothersome). FIS was reported by 38% of adults with a colostomy (100% bothersome). CONCLUSION: Incontinence during sexual activity is a common problem for men and women with SB. Baseline incontinence is an independent, but not absolute, predictor of both. While FIS is less frequent than UIS, both are virtually always bothersome.

Objectified kidney ultrasound echogenicity and size metrics as potential predictors for kidney function in children.

BACKGROUND: Kidney echogenicity is typically determined subjectively but may have a quantifiable relationship to kidney function. Similarly, kidney length has been shown to correlate with kidney function. This study sought to quantify echogenicity using readily available software. Secondarily, we aimed to evaluate the correlation between quantified echogenicity and kidney length to the estimated glomerular filtration rate (eGFR) in children with acute kidney injury (AKI) and chronic kidney disease (CKD). METHODS: In a single-center retrospective observational study, echogenicity index (EI) was determined using a ratio of right kidney to liver mean pixel density. The kidney length ratio (KLR) was determined by the actual to predicted lengths of both kidneys. Both variables were correlated to eGFR using correlation analyses and predictive capacity was determined with receiver operating characteristic curve (ROC) analysis. RESULTS: Of 94 subjects, 46% (43/94) had AKI, 28% (26/95) had CKD and 26% (25/95) were controls. The higher the EI the lower the eGFR (r = - 0.46, p < 0.0001). EI between 1.0 and 1.1 predicted an eGFR < 90 ml/min/1.73m2 with an AUC of 0.71-0.78 while an EI between 1.1 and 1.2 predicted an eGFR < 60 ml/min/1.73m2 with AUC of 0.75-0.80. Overall, the larger the KLR the lower the eGFR (r = - 0.25, p 0.018). CONCLUSION: We have developed an accessible methodology to quantify kidney echogenicity. Overall, there was an inverse correlation between EI and eGFR in pediatric CKD and AKI. However, these correlations did not persist within subgroups which could be due to small sample size and heterogeneity of etiologies. Overall, KLR had a weaker correlation to eGFR, compared to EI. Despite these correlations, both EI and KLR had "fair" to "good" performance as a biomarker for an eGFR < 60 ml/min/1.73m2.

Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

People with spina bifida use their MACE on long-term follow-up: A single institutional retrospective cohort study.

OBJECTIVE: While the Malone antegrade continence enema (MACE) facilitates bowel movements in patients with spina bifida (SB) and neuropathic bowel, little is known about its long-term use. We aimed to assess long-term MACE use and potential risk factors for disuse. METHODS: All patients with SB who underwent MACE procedures at our institution were retrospectively reviewed. Main outcome was MACE disuse (no longer catheterizing the MACE for antegrade enemas) based on self-report on a clinic questionnaire, or medical record for patients last seen before introducing the questionnaire 5 years ago. Survival analysis used two timeframes: time after surgery (Analysis 1) and chronological age: accounting for older children reaching adulthood earlier (Analysis 2). RESULTS: Overall, 411 patients (54% female, 78% shunted, 65% augmented) underwent a MACE procedure at median 7.9 years old (median follow-up: 8.4 years). Thirty-three (8%) patients no longer used their MACE. Most common reasons for doing so were channel/stomal stenosis (61%) and excision at colostomy or other abdominal surgery (12%). Bowel management afterwards included oral agents ± enemas (55%), Chait tube (30%), colostomy (12%). After correcting for differential follow-up, 90% of participants used their MACE at 10 years and 87% at 15 years after surgery. Based on chronological age, 97% used their MACE at 15 years old, 92% at 20 and 81% at 30 (Summary Figure). On multivariate analysis, umbilical MACEs were 2.4 times more likely to be disused than right lower quadrant MACEs (p = 0.04). Without correcting for chronological age (Analysis 1), patients undergoing MACE surgery at older ages were more likely to stop MACE use (p = 0.03). However, after accounting for chronological age (Analysis 2), patients undergoing a MACE procedure at older ages were no more likely to stop its use (p = 0.47, Figure). Gender, SB type, shunt status, mobility status, bladder augmentation or a urinary catheterizable channel were not associated with stopping MACE use (p ≥ 0.10). COMMENT: Participants were regularly followed in multi-disciplinary SB clinics. We did not assess continence, satisfaction or long-term urinary channel use, making it premature to recommend optimal stomal locations. CONCLUSIONS: Most patients with SB followed by a multi-disciplinary team continue using their MACE; 1% stopped MACE use annually, particularly after adolescence. This strongly suggests it is an effective bowel management method and transitioning to self-care plays a role in maintaining long-term MACE use. Umbilical MACEs may be at high risk of disuse, but all people with a MACE can benefit from support as they transition to adult care.

Sexual activity and function of adult men with spina bifida.

OBJECTIVE: Data on sexual function of men with spina bifida (SB) is limited. We aimed to assess sexual activity and erectile dysfunction (ED) in a large international sample of men with SB. METHODS: Men with SB (≥18yo) were recruited in an international online survey via clinics and social media. We collected data on demographics, ambulation (Hoffer classification), penile rigidity (Erection Hardness Score), sexual activity and ED (International Index of Erectile Function). Non-parametric tests were used. RESULTS: A total of 162 men (median age 35, 62% shunted, 38% community ambulators) reported sexual desire similar to the general population (p = 0.82), but 55% were dissatisfied with their sex life (Summary Table). Overall, 36% reported full penile rigidity with erections, more commonly with better ambulation (p = 0.01), 69% had ever experienced orgasm and 84% ejaculated. In 44 men (27%) attempting sexual intercourse in the last 4 weeks, 59% had ED (11% severe, 7% moderate, 14% mild-moderate, 27% mild). In this group, 91% of men reporting less than full penile rigidity had ED, compared to 30% with full penile rigidity (p = 0.001). Overall, partnered non-genital contact in the last 3 months was reported by 56%, solo masturbation: 62%, partnered intercourse: 48% (31% vaginal). Of 54 men who used phosphodiesterase type 5 inhibitors (PDE5I), 80% reported improved erections, 56% improved intercourse. Overall, 40% reported non-genital erogenous zones as most pleasurable, especially with poorer ambulation (p = 0.002, chest/nipples: 73%). COMMENT: Strengths of this study include anonymous, voluntary, online participation maximizing participation of a heterogenous, international population. Whenever available, we compared findings to published values for the general population. Since romantic and sexual activity is a complex intersection of interest, opportunity and ability, a more comprehensive assessment was beyond the study's scope. Future work will focus on the interplay with issues like incontinence. CONCLUSIONS: ED was frequent among men with SB, especially in men with poorer ambulation. PDE5 inhibitors may be beneficial. Partnered sexual activity was reported by half of the men, although it may not involve penetrative intercourse.

Building a prototype of a continence goal-selection tool for children with spina bifida: Patient, parental and urology provider recommendations.

INTRODUCTION: No tools exist to help children with spina bifida (SB) describe their incontinence concerns and to help their providers account for them when evaluating management success. Our aim was to understand patients', parents', and clinicians' preferences about how a SB continence goal-selection tool (MyGoal-C) should look and function, and to create a tool prototype. METHODS: We used a qualitative research approach integrated with human-centered design methods. We recruited children with SB (8-17 years old), parents ( ≥ 18 years old) of children with SB (8-17 years old) in clinic and online, and urology healthcare providers at our institution. We surveyed children and parents, and conducted parent and provider Zoom-based prototyping sessions to iteratively design the app. Design researchers analyzed online activities using affinity diagramming, group analysis and modeling activities. Provider sessions were analyzed with qualitative thematic analysis based on grounded theory. Recruitment continued until saturation was reached. RESULTS: Thirteen children with SB participated (median age: 10 years old, 4 female, 9 shunted, 10 using bladder catheterizations). Thirty-seven parents participated (33 mothers, median age: 43 years old). Children and parents unanimously recommended an app and their comments generated 6 major theme domains: goal-setting process, in-app content, working toward goals, urology provider check-in, app customization, and using big data to improve future functionality. Twenty-one of the parents participated in 3 prototyping sessions with 6 breakout groups. The remaining 16 parents and 13 children then completed the Prototype Testing Survey, leading to a refined prototype and a visual flow map of the app experience (Figure). Feedback from 11 urology healthcare providers (7 female, 6 advanced practice providers) generated 8 themes: patient engagement/autonomy, clearly displaying results, integration into clinic workflow, not increasing clinical staff burden, potential clinician bias, parental involvement, limitations of the app, and future app adaptation. These cumulative data allowed for a construction of a final app prototype. COMMENT: Findings of our study lay the foundation for creating a goal-selection app that meets preferences and needs of children, parents, and providers. Next steps involve building the app, testing its usability and assessing it prospectively in a clinical setting. CONCLUSIONS: Children with SB and parents preferred an app to help them set and track continence goals. All stakeholders, including urology providers, offered complementary and mutually reinforcing feedback to guide the creation of an app prototype that would ultimately be integrated into a clinic visit.