"They had the lunch lady coming up to assist": The experiences of menarche and menstrual management for adolescents with physical disabilities.

BACKGROUND: Menarche is a pivotal time in an adolescent's life but can be experienced differently by those with physical disabilities. Parents typically serve as the primary educators and support for their daughters during this time. Little is known about the parent's perspective of their daughter's experience preparing for menarche and learning to manage menses. OBJECTIVE: We sought to understand the parent's perspective of the experience of their daughter with a physical disability around menarche and their preferences for health care provider support. METHODS: Individual semi-structured interviews were conducted with 21 parents of a daughter with a physical disability ages 7-26. Interviews were coded by 2 reviewers using Grounded Theory, with disagreements resolved by consensus. RESULTS: Six themes emerged regarding the parent's perception of the experiences, including 1) variation in emotional responses to menarche, 2) parent's perception of their daughter's experience with menses and menstrual symptoms, 3) cross-section of disability and menstrual management, 4) menstrual management at school, 5) parental knowledge correlating to daughter's preparation for menarche, and 6) desires for health care provider support. CONCLUSIONS: All parents reported that their daughters faced challenges during menarche, ranging from emotional distress to dealing with the inaccessibility of hygiene products. Managing periods at schools was particularly burdensome. Parents who were better educated about what to expect were better able to prepare their daughters, but had difficulties finding informed, supportive providers. Health care providers should provide both anticipatory guidance and holistic, respectful, and equitable options for the management of menstrual symptoms.

Codevelopment of an illustration representative of people living with spina bifida for health educational materials.

BACKGROUND: The lack of health education resources specific to people with disabilities contributes to disparities in outcomes. Developing user-centered materials with representative images tailored to people with disabilities could help improve knowledge and outcomes. OBJECTIVE: As a first step in developing an online sexual health resource for adolescents with physical disabilities, we sought end-user feedback to create illustrated characters for use in educational materials. METHODS: Two styles of characters were developed by the research team, which included a professional disability artist. Verbal and online survey feedback was obtained at the Spina Bifida Association's Clinical Care Conference. A new image was created incorporating initial feedback. The new image and favored image from the first round were then tested through an online survey advertised on the Spina Bifida Association's Instagram story feed. Open-ended comments were organized by categories and overlapping themes. RESULTS: Feedback was obtained from 139 audience members and 25 survey respondents from the conference and 156 Instagram survey respondents. Themes included depiction of disability, nondisability diversity, other physical appearance, emotional response, and design style. Most frequently, participants suggested the inclusion of characters with a range of accurately depicted mobility aids and of characters without mobility aids. Participants also wanted a larger, more diverse group of happy, strong people of all ages. CONCLUSIONS: This work culminated in the codevelopment of an illustration that represents how people impacted by spina bifida view themselves and their community. We anticipate that using these images in educational materials will improve their acceptance and effectiveness.

Acoustic radiation force imaging (ARFI) in the non-distended bladder does not predict abnormal urodynamic parameters in children.

INTRODUCTION: Acoustic radiation force imaging (ARFI) is a recently developed form of ultrasound imaging that allows in vivo measurement of tissue stiffness. This technology could be useful at predicting bladder compliance in children. We hypothesize that tissue stiffness, as measured by ARFI, correlates with abnormal bladder compliance and capacity in patients with bladder dysfunction. METHODS: Patients who presented for cystometrography (CMG) underwent ARFI of the bladder wall. Nine bladder wall shear wave speed (SWS) measurements were acquired using point and 2D ultrasound shear wave elastography. The mean for each ARFI technique was correlated to bladder compliance, calculated using Wahl's dimensionless number. ARFI parameters also were correlated with bladder capacity. RESULTS: A total of 25 patients were enrolled. Mean age at time of enrollment was 4.2±3.9 years (range two months to 15 years). There was no significant correlation between bladder compliance and point shear wave speed measurements (r=-0.22, p=0.31) or 2D shear wave speed measurements (r=-0.35, p=0.1). A total of 19 patients had bladder capacity below expected bladder capacity (EBC). There was no significant correlation between bladder capacity and point shear wave speed measurements (r =-0.08, p=0.7) or 2D shear wave speed measurements (r=-0.36, p=0.09). CONCLUSIONS: Our results did not demonstrate a significant correlation between bladder wall ARFI shear wave measurements and bladder compliance or bladder capacity. Further studies are warranted to determine whether ARFI may be used to predict abnormal urodynamic parameters in children.

The transition to independence and adult care for women with Turner syndrome: Current status and priorities of 1338 women and parents.

Transitioning to adult health care and functioning is crucial for youth and young adults with special health care needs, such as those with Turner syndrome (TS). The International Turner Syndrome Consensus Group developed clinical practice guidelines to improve this transition. This study sought to evaluate how effectively they have been implemented and whether they align with the priorities of women with TS and families of girls and women with TS. A web-based survey was offered to those affiliated with major TS support organizations, with 1338 successfully responding (n = 543 women [≥18 years]; n = 232 parents of a woman [≥18 years]; and n = 563 parents of a girl with TS [<18 years]). Findings demonstrated that while most women transitioned to adult primary care providers, follow-up with key specialists was lacking. Women and families prioritized flexibility in appointments and designating one provider to oversee all their TS-related care. They identified health care coverage as a barrier to receiving care. The transition process to adult care and independence commonly occurred after girls become legal adults. Together, these findings serve to inform strategies to improve the delivery of transitional care for girls and women with TS.

Sexual Function of Men and Women With Spina Bifida: A Scoping Literature Review.

INTRODUCTION: As most adults with spina bifida are either sexually active or interested in becoming sexually active, providers should understand how spina bifida impacts sexual function and options for treatment. OBJECTIVES: The objective of this study is to summarize the current literature describing how features of spina bifida impact sexual function in men and women, effective available treatment options for sexual dysfunction, and to identify research gaps. METHODS: Searches were conducted in PubMed, CINAHL Complete, PsychInfo, Cochrane Central, Scopus, and Web of Science Core Collection databases using keywords related to spina bifida and sexual function. 34 primary research studies were included. RESULTS: Most men (56-96%) can achieve an erection, although it may be insufficient for penetration. Although 50-88% ejaculate, it is often dripping, retrograde, or insensate. Twenty percent to 67% achieve orgasm. Generally, men with lower lesions and intact sacral reflexes have better outcomes, although some men with all levels of lesion report good function. Sildenafil is efficacious at treating erectile dysfunction for most men. The "TO-MAXimize sensation, sexuality, and quality of life" procedure may improve sexual function in selected men with low-level lesions. Female sexual function and treatment is less well understood. Women may experience decreased arousal, difficulties with orgasm, and pain. No treatment has been studied in women. Bowel and bladder incontinence during intercourse appears to be bothersome to men and women. Although both men and women have diminished sexual satisfaction, their sexual desire appears to be least impacted. Present studies are limited by studies' small, heterogeneous populations, the misuse of validated questionnaires in the sexually inactive population, and the lack of a validated questionnaire specific to people with spina bifida. CONCLUSIONS: Spina bifida impacts the sexual function of both men and women. Future studies should seek a better understanding of female sexual function and treatment, use validated questionnaires appropriately, and ultimately create a validated sexual function questionnaire specific to this population. Streur CS, Corona L, Smith JE, et al. Sexual Function of Men and Women With Spina Bifida: A Scoping Literature Review. Sex Med Rev 2021;9:244-266.

Opioid prescribing is excessive and variable after pediatric ambulatory urologic surgery.

BACKGROUND: Acute pain after surgery is one of the most frequent indications for opioid prescribing in children. Opioids are often not stored or disposed of safely after their use, placing children and others in the home at risk for accidental ingestion or intentional misuse. We currently lack evidence-based guidelines for post-operative pain management after common ambulatory pediatric urologic procedures. Thus, each surgeon must decide if and how much opioid to prescribe based on his/her own assumptions of perceived post-operative pain. OBJECTIVES: As part of an effort to establish opioid prescribing guidelines across two academic centers, the objectives of this study were to evaluate current variability in pediatric urologists' opioid prescribing factors and identify patients at greatest risk of being prescribed high doses of opioids after common ambulatory pediatric urologic procedures. METHODS: We retrospectively evaluated post-operative opioid prescribing patterns after common ambulatory pediatric urology procedures (circumcision, orchiopexy, and hernia/hydrocele) at two major children's hospitals. Specifically, we evaluated if and how much opioid was prescribed for all children (18 years or younger) between 2016 and 2017. Bivariate analysis was performed using Kruskal-Wallis Test and Wilcoxon Rank Sum. Multivariable logistic regression was performed to determine patient, surgeon, and procedural factors that predicted the prescription of a high dose of opioids (greater than the median number of doses prescribed for that procedure). RESULTS: Over the two-year period, 811 circumcisions and 883 inguinal surgeries (inguinal orchiopexy and hernia/hydrocele) were performed. 94% of patients undergoing circumcision and 97% of those undergoing inguinal surgery were prescribed opioid analgesia. The median number of doses prescribed for circumcision was 20; for inguinal surgeries, 23.75% of patients received 15 opioid doses or more. Patients ages 0-2 years, who represented the largest age group (41% of all patients), received significantly more opioid doses than all other age groups, followed by those >10 years (p < 0.01). There was significant variation in opioid prescribing patterns by provider (p < 0.01) (Figure 1) On multivariable logistic regression, younger age, pill form, and earlier year were all associated with a greater number of opioid doses prescribed for all surgeries. CONCLUSIONS: Across two institutions without a formal post-operative opioid prescribing policy for ambulatory pediatric urologic procedures, we observed considerable variability in provider prescribing patterns, with nearly all patients receiving an opioid, and those 0-2 years receiving the highest number of doses. This highlights the need for evidence-based guidelines for post-operative pain management after ambulatory pediatric urologic surgeries.

How to Discuss Sexual Health With Girls and Young Women With Spina Bifida: A Practical Guide for the Urologist.

OBJECTIVE: To provide urologists with a practical guide for how to provide sexual health counseling to girls and women with spina bifida. METHODS: The recommendations and research of several sources were synthesized to create this guidance, including clinical guidance from the Spina Bifida Association and American College of Obstetricians, the current literature on the sexual health of girls and women with spina bifida, and the multidisciplinary experience of the authors. RESULTS: Sexual health education should be viewed by urologists as a continuous discussion, starting in early childhood and gradually building through adolescence. Developing a plan for when and how to bring it up, utilizing parents as educational partners, identifying who will provide the detailed one-on-one counseling if not the primary urologist, establishing a referral network for specialized care (eg, adolescent gynecologist, physical therapist, or sex therapist), becoming familiar with how spina bifida impacts sexual health, and being prepared for challenges are key to providing these girls and women with competent sexual health education. Urologists should also screen for abuse at each visit and be familiar with reporting and resources for when abuse is identified. CONCLUSION: This guidance can serve to direct urologists in providing competent sexual health education to girls and women with spina bifida. This will ensure these girls and women receive the basic education they need, and that they can be referred to appropriate sexual health experts as indicated.

Trends in Procedures to Initiate Renal Replacement Therapy among People Living with Spina Bifida.

PURPOSE: Given the increasing prevalence of chronic kidney disease in people with spina bifida, we sought to determine if this is associated with an increase in end stage kidney disease. We examined population based data to measure the frequency of procedures to establish renal replacement therapy-a marker for end stage kidney disease-among patients with spina bifida. MATERIALS AND METHODS: We used the Healthcare Cost and Utilization Project State Inpatient Database and State Ambulatory Surgery and Services Database from Florida, Kentucky, Maryland and New York (2000 to 2014), which include encounter level data. With a diagnosis code based algorithm we identified all procedural encounters made by patients with spina bifida. We determined the percentage of these encounters that were for facilitating renal replacement therapy (ie arteriovenous anastomosis, renal transplantation). We assessed for changes over time in this percentage with the Cochran-Armitage trend test. Bivariate analysis was performed using chi-square test. RESULTS: Of all procedures performed on patients with spina bifida over this time the proportion of procedures performed to establish renal replacement therapy significantly decreased in both the inpatient and outpatient settings (p=0.042 and p <0.001, respectively). People with spina bifida undergoing procedures to establish renal replacement therapy were, on average, young adults (mean age 34.5 and 36.0 years) with a high prevalence hypertension (75.8% of inpatients, 68.6% of outpatients). CONCLUSIONS: The frequency of surgeries to initiate renal replacement therapy among people with spina bifida undergoing procedures is low and is not increasing. This highlights the importance of consistent care throughout adolescence and young adulthood, and hypertension screening.

"I Tell Them What I Can Feel and How Far My Legs Can Bend": Optimizing Sexual Satisfaction for Women With Spina Bifida.

BACKGROUND: There is a high reported rate of sexual dysfunction among women with spina bifida, but little is known about the etiology of this or how sexual satisfaction could be improved. AIM: To identify, through the words of women with spina bifida, perceived causes of diminished sexual satisfaction and recommendations to optimize partnered sexual encounters. METHODS: In this qualitative study, we conducted semi-structured individual interviews with 22 women with spina bifida (median age 26.5 years, range 16-52 years) who have had a romantic partner. Using Grounded Theory, interviews were independently coded by 3 reviewers. Disagreements were resolved by consensus. OUTCOMES: We identified overlapping themes of issues women experienced during sexual intimacy and strategies they learned to improve sexual encounters. RESULTS: 7 salient themes emerged from the data: (i) fear of rejection with resulting difficulty setting boundaries and the risk of coercion; (ii) conflict between spontaneity and self-care in sexual encounters; (iii) worry about incontinence during sex; (iv) trial and error in learning optimal sexual positions; (v) decreased genital sensation; (vi) safety considerations; and (vii) sharing advice with other women with spina bifida. CLINICAL IMPLICATIONS: As sexual satisfaction is influenced by physical features as well as psychological, interpersonal, and sociocultural factors, optimizing sexual satisfaction of women with spina bifida is best managed with a holistic approach utilizing a biopsychosocial model. STRENGTHS & LIMITATIONS: The sample included women with a diverse range of functional impairments. Women were forthright with their comments and thematic saturation was reached. Recruitment was primarily from a single Midwestern institution, which may have limited sampled perspectives. CONCLUSION: While women with spina bifida encounter challenges during sexual encounters, strategies focused on improving communication with partners and addressing specific physical considerations can potentially enhance their sexual experiences. Streur CS, Schafer CL, Garcia VP, et al. "I Tell Them What I Can Feel and How Far My Legs Can Bend": Optimizing Sexual Satisfaction for Women With Spina Bifida. J Sex Med 2020;17;1694-1704.

"He told me it would be extremely selfish of me to even consider [having kids]": The importance of reproductive health to women with spina bifida and the lack of support from their providers.

BACKGROUND: As more women with spina bifida (SB) enter their reproductive years, the number having children is significantly increasing. However, little is known about their understanding of their ability to get pregnant or their experiences in considering, planning, or interacting with providers during a pregnancy. OBJECTIVE: We sought to determine what women have been told and understand about their reproductive health, their attitudes towards having children, and their experiences interacting with providers when seeking reproductive health care. METHODS: In this exploratory study employing qualitative research methods and following Grounded Theory, interviews with women with SB 16 years or older were transcribed verbatim and analyzed by three coders. RESULTS: Interviews of 25 women with SB ages 16-52 (median 26) revealed the following themes about their reproductive health perceptions and experiences: 1) poor understanding of reproductive health and potential, 2) interest in having a family, 3) facing provider's opposition to their reproductive goals, 4) going into pregnancy and delivery unprepared, 5) the importance of provider support for reproductive goals. Five women experienced an unintended pregnancy. CONCLUSIONS: Although having children is important to most women with SB in this study, they report a poor understanding of their reproductive potential with several noting unintended pregnancies. They feel uninformed and unprepared during pregnancy and face discouragement from providers. Those experiencing supportive providers report a more positive experience. This demonstrates the urgent need to educate women with SB about their reproductive health and the providers who care for them how to support and counsel these women.

"If Everyone Else Is Having This Talk With Their Doctor, Why Am I Not Having This Talk With Mine?": The Experiences of Sexuality and Sexual Health Education of Young Women With Spina Bifida.

INTRODUCTION: Women with spina bifida are sexually active, but most never discuss this topic with providers. AIM: To determine what women with spina bifida understand about their sexual health, how they learned about it, what questions they have, and their experiences with their sexuality. METHODS: For this qualitative study, women with spina bifida ages 16 and older without marked developmental delay were individually interviewed. 25 women with spina bifida participated (mean age 27.1 years, range 16-52). Interviews were independently coded for themes by 3 reviewers, using Grounded Theory, with disagreements resolved by consensus. MAIN OUTCOME MEASURES: We identified overlapping themes regarding the women's perception and experience of their sexuality and sexual health education. RESULTS: 17 of the 25 (68%) participants had been or were currently sexually active. 5 themes emerged regarding their understanding of their sexuality and their sexual experiences: (i) being perceived as asexual, (ii) sources for sex education, (iii) need for spina bifida-specific sex education, (iv) impact of spina bifida-specific features on sexual encounters, and (v) perceived relationship between low sexual self-confidence and risk for sexual assault. CLINICAL IMPLICATIONS: Women with spina bifida are sexual beings, but they are perceived as asexual by providers, which prevents them from getting adequate sexual health education and leaves them with misconceptions and unanswered questions, as well as vulnerable to sexual abuse. STRENGTH & LIMITATIONS: The strengths of this study include the diversity of women interviewed, including their age, severity of disability, and experiences with their sexuality, as well as the ability to reach thematic saturation. The limitation of this study is that most women received treatment at a single Midwestern tertiary referral center in the United States. CONCLUSION: Including sexual health discussions in the usual care of women with spina bifida is critical to enhancing their sexual confidence and experience and preventing sexual abuse. Streur CS, Schafer CL, Garcia VP, et al. "If Everyone Else Is Having This Talk With Their Doctor, Why Am I Not Having This Talk With Mine?": The Experiences of Sexuality and Sexual Health Education of Young Women With Spina Bifida. J Sex Med 2019;16:853-859.

Impact of the Image Gently® Campaign on Computerized Tomography Use for Evaluation of Pediatric Nephrolithiasis.

PURPOSE: The Image Gently® campaign was launched by several radiological societies in 2007 to promote safe imaging in children. A goal of the campaign was to reduce ionizing radiation exposure in children. Given the recurrent nature of kidney stones, affected children are at risk for unnecessary ionizing radiation exposure from computerized tomography. We sought to determine whether the Image Gently campaign led to a decrease in the use of computerized tomography for evaluating children with nephrolithiasis. We hypothesized that the campaign was the primary cause of a reduction in the use of computerized tomography. MATERIALS AND METHODS: We analyzed medical claims data from 2001 to 2015 identifying children with nephrolithiasis covered by the same commercial insurance provider. Using a difference in differences design, we estimated changes in computerized tomography use after the campaign started among patients less than 18 years old compared to a control group age 18 years or older with nephrolithiasis. RESULTS: We identified 12,734 children and 787,720 adults diagnosed with nephrolithiasis. Before 2007 quarterly rates of computerized tomography use during a stone episode (per 1,000 patients) were increasing at a parallel rate in children and adults (5.1 in children vs 7.2 in adults, p = 0.123). After the Image Gently campaign started the use of computerized tomography decreased in both groups but at a slightly higher rate in adults (difference in differences 2.96, 95% CI 0.00 to 5.91, p = 0.050). CONCLUSIONS: Although there has been a reduction in the use of computerized tomography among children with nephrolithiasis, given a similar trend seen in adults this change cannot be primarily attributed to the Image Gently campaign.

"I Don't Know What I'm Doing I Hope I'm Not Just an Idiot": The Need to Train Pediatric Urologists to Discuss Sexual and Reproductive Health Care With Young Women With Spina Bifida.

INTRODUCTION: Although pediatric urologists have taken responsibility for initiating discussions on sexual and reproductive health with spina bifida patients, research shows that very few girls and women with spina bifida have ever discussed this topic with any physician. AIM: We sought to better understand pediatric urologists' gaps in knowledge and training needs in the sexual and reproductive health education of women with spina bifida with the goal of creating a tool kit to equip providers to have these discussions. METHODS: In this qualitative study, pediatric urologists were interviewed separately about their current practices, perceived barriers, knowledge gaps, and recommendations for the tool kit until thematic saturation was reached. The interviews were recorded and transcribed verbatim, then analyzed using grounded theory by 3 independent reviewers. MAIN OUTCOME MEASURES: To evaluate the perspectives and practices of pediatric urologists, we identified the overlapping themes of the interviews. Consensus on themes was reached. RESULTS: 10 Pediatric urologists participated in the study, including 5 men and 5 women, of whom 4 were fellows and 6 were attending physicians (mean years of practice 18, range 6-31 years). The mean number of patients followed up in the respective spina bifida clinics or by the provider was 434 (range 24-1,500). The following themes regarding pediatric urologists' experience providing sexual and reproductive health education to women with spina bifida emerged. Pediatric urologists': (i) lack of formal training; (ii) knowledge gaps such as spina bifida sexuality, fertility, and pregnancy experience; (iii) barriers to having sexual and reproductive health conversations such as lack of comfort and lack of time; (iv) facilitators of these conversations such as a long-term relationship with the patient and the patient's own initiative; (v) desire to learn and provide competent care; and (vi) recommendations for a web-based tool kit that would include content to address the knowledge gaps and training about how to start sexual and reproductive health conversations. CLINICAL IMPLICATIONS: These findings can provide the beginning concepts for the development of training on providing sexual and reproductive health education for pediatric urologists' care for women with spina bifida. STRENGTHS & LIMITATIONS: This study gives the perspectives of 10 pediatric urologists with a diversity of backgrounds, but all of whom care for a large number of spina bifida patients. This does not give the perspectives of the spina bifida women themselves, which will be evaluated in the next phase of the study. CONCLUSION: Pediatric urologists are not trained and do not feel prepared to provide sexual and reproductive health education for girls and women with spina bifida. However, they do see it as their scope of practice and wish to acquire competence in this area. Streur CS, Schafer CL, Garcia VP, et al. "I Don't Know What I'm Doing… I Hope I'm Not Just an Idiot": The Need to Train Pediatric Urologists to Discuss Sexual and Reproductive Health Care With Young Women With Spina Bifida. J Sex Med 2018;15:1403-1413.