- Email: [email protected]
Quality of life in female patients with bladder exstrophy-epispadias complex: Long-term follow-up
Accepted Manuscript Quality of life in female patients with bladder exstrophy-epispadias complex: longterm follow-up Anna Bujons, Diana M. Lopategui, Nelly Rodríguez, Clara Centeno, Jorge Caffaratti, Humberto Villavicencio PII:
S1477-5131(16)30060-2
DOI:
10.1016/j.jpurol.2016.05.005
Reference:
JPUROL 2193
To appear in:
Journal of Pediatric Urology
Received Date: 18 January 2016 Accepted Date: 17 May 2016
Please cite this article as: Bujons A, Lopategui DM, Rodríguez N, Centeno C, Caffaratti J, Villavicencio H, Quality of life in female patients with bladder exstrophy-epispadias complex: long-term follow-up, Journal of Pediatric Urology (2016), doi: 10.1016/j.jpurol.2016.05.005. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT ESPU Prague 2015 Quality of life in female patients with bladder exstrophy-epispadias complex: long-term follow-up Anna Bujons *, Diana M. Lopategui, Nelly Rodríguez, Clara Centeno, Jorge Caffaratti, and Humberto Villavicencio
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Pediatric Urology Department, Fundació Puigvert, Barcelona, Spain
* Corresponding author. C/ Cartagena, 340–350, 08022 Barcelona, Spain.
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E-mail address: [email protected]
Summary Introduction: Bladder exstrophy-epispadias complex (BEEC) is a congenital malformation that requires multiple surgeries during childhood and
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life-long follow-up. It often presents with conditions that have the potential to impact quality-of-life (QoL) and psychosocial functioning of affected patients, such as incontinence and sexual dysfunction. The aim of this study is to examine the QoL, urinary continence, sexual function, and overall health in a long-term series of female patients with BEEC.
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Method: A retrospective review was performed of female patients with BEEC born between 1964 and 1996. Thirty-three patients were asked to complete four validated questionnaires to evaluate their QoL regarding urinary continence and sexual activity (ICIQ, Potenziani-14, and PISQ-12 questionnaires). Nineteen
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patients completed and returned the questionnaires. The overall QoL was assessed with the SF-36 questionnaire, and demographics were evaluated. Statistical analysis was performed to compare the general QoL with that of the
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general population.
Mean (range) age, years Previous history
26 (18–50) Total number of surgeries,
13 (3–16)
mean (range) Initial surgery, n
Primary closure
16
Cystectomy +
2
ureterosigmoidostomy Cystectomy + cutaneous
1
1
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Lithiasis, n
8
Ileal reservoir
33%
Colonic reservoir
67%
Mitrofanoff conduit
67%
Renal
2
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Bladder enlargement or reservoir
Osteotomy
Bladder
4
Vesicoureteral reflux
36%
Chronic renal failure
6%
31%
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Genital prolapse Rectal prolapse Continence status, n (%)
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Normal continence Occasional leak
Mild incontinence, little QoL
15%
12 (63) 7 (37) 5
impact, n
Moderate incontinence, big
2
QoL impact, n Young-Dees
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Continence management, n
7
cervicourethroplasty
Silimed perimitrofanoff
3
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constrictor
Sexual function and fertility
Education level, n
Bulking agents
4
Closed bladder neck
7
Autocatheterization
14
Incontinent
6
Sexually active
84%
Not in a relationship
10%
Dyspareunia
42%
C-section, n
5
Abortions, n
3
High school
11
Higher education
8
2
ACCEPTED MANUSCRIPT Occupational status, n
Active workers
12
Unemployed
5
Invalidity status
2
Results: The median age of the patients was 26 years (range 18–50) (Table). A
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low to moderate impact of urinary incontinence on QoL was reported by 30% of patients in the ICIQ. Also as a result of urinary incontinence, 84% of patients
reported a moderate to severe impact on their sexual lives. Twelve patients got married with eight gestations and five births. SF-36 reported general QoL
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comparable with that of the general population in five out of eight items.
Differences were seen in the mental health, emotional role, and physical functioning items (p<0.001). The main factors for the differences were poor
reported by 70% of patients.
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body image, anxiety, and urinary incontinence. A satisfactory social life was
Conclusion: Questionnaire studies on BEEC consistently report a high rate of patients not answering, 43% in the present study. The rarity of the disease determines a small sample size, which diminishes statistical power and could potentially conceal small differences with controls. Despite these limitations, the
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present findings are consistent with previous studies on BEEC with validated QoL questionnaires: adult women with BEEC suffer psychosocial impact mainly from incontinence, and also from gynecological complications, but their
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resilience and coping mechanisms allow them to achieve a quasi-normal QoL. Female patients with BEEC reported a normal QoL in five of eight items in the SF-36 questionnaire. Urinary incontinence was the main factor for the
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moderately decreased QoL according to specific questionnaires.
KEYWORDS
Long-term follow-up; Quality of life; Female patients;
Exstrophy-epispadias
Introduction
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ACCEPTED MANUSCRIPT Bladder exstrophy-epispadias complex (BEEC) is a congenital malformation presenting with pubic diastasis, anterior displacement of internal genitals and labia majora, and protrusion of the bladder through a defect of the lower abdominal wall [1]. It is one of the most severe and challenging conditions in pediatric urology. Most of the affected patients require multiple surgeries during
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childhood, and life-long follow-up by a specialized multidisciplinary care team. This congenital defect can affect patients’ quality of life (QoL) both in the short and long term, and its psychosocial impact is not well understood [2].
Literature evaluating the effect of BEEC on QoL is still very scarce: a
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meta-analysis by Diseth et al. [3] showed that only 0.8% of studies on BEEC
look into the psychosocial or QoL implications of the disease. Several studies found BEEC to have a significant impact on mental health and QoL, particularly
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regarding sexual function and anxiety disorders [2], with patients scoring lower than the general population on questionnaires assessing general health perception and physical activity limitations [4].
Over the last decade, research on BEEC has led to better care management and reconstructive techniques, ultimately improving the morbidity and mortality of BEEC. Patients now reach adulthood aiming for a good QoL,
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including normal sexual function and acceptable urinary continence. We designed a retrospective study to evaluate the QoL of adult females with BEEC, including parameters of urinary continence, sexual function, and
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general health. Our aim is to quantify the overall result of treatment of BEEC and follow-up into functional adulthood, not the result of any particular surgical technique.
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Material and methods The study population was selected from the patients followed at the pediatric unit of a third-level urology-specialized center. Selection criteria included diagnosis of BEEC, female gender, and age >18 years. The study was approved by the ethics committee of our center. The method to collect data was the patients’ self-completion of four validated questionnaires: Short Form-36 Health Survey (SF-36, Spanish version), Pelvic Organ Prolapse/Urinary Incontinence Sexual Questionnaire (PISQ-12), International Consultation on Incontinence Modular Questionnaire (ICIQ-SF), and POTENZIANI-14.
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ACCEPTED MANUSCRIPT Generic QoL questionnaires are instruments designed to evaluate the impact of disease conditions on an individual’s daily functioning, regardless of the underlying disease. They have been used and validated in many different clinical scenarios to assess the effect of specific entities, as well as to make comparisons between diseases and with controls from a healthy population.
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The SF-36 questionnaire evaluates health-related QoL providing a profile of the health state in eight different dimensions: physical functioning, physical role,
body pain, general health, vitality, social functioning, emotional role, and mental health. Every concept is scored based on the answer to a total of 36 different
and best possibly imaginable health state [5].
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items in a numerical scale from 0 to 100, equivalent, respectively, to the worst
The PISQ-12 evaluates the sexual functioning of women with genital
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prolapse or urinary or fecal incontinence. It has been validated to the Spanish language and sociocultural environment. It consists of 12 items analyzing the impact of the symptoms on the patient’s sexual function, and provides a numeric score for each item from 0=always, to 4=never, and a total score from 0 to 48. Lower scores are equivalent to a greater sexual dysfunction caused by the symptomatology [6].
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The ICIQ-SF (see Appendix) has been validated for the diagnosis of urinary incontinence [7], and POTENZIANI-14 evaluates the impact of urinary incontinence on QoL in women.
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Patient characteristics were recorded in a clinical interview, including demographic data, medical and surgical previous history, childhood development, academic and job history, relationships, and physical and sexual
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function. The patients fulfilling the study criteria were contacted by telephone and informed regarding the methodology and purpose of the study. Those who agreed to participate were sent the four questionnaires and a consent form via e-mail.
We identified 33 BEEC female adult patients followed at our institution, 19 (57%) of whom completed and returned all questionnaires. Data collection and all statistical analyses were performed with SPSS software version 15. Comparisons of the subjects’ scores on questionnaires with general population normalized scores were performed with the Student’s ttest. 5
ACCEPTED MANUSCRIPT Results Of 33 eligible patients from our institution, 19 (57%) agreed to participate in the study and completed the questionnaires fully. Their age ranged from 18 to 50 years, with a mean of 26 years. All the 19 patients included in the study underwent multiple surgeries
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after the primary defect closure, with a mean total of 13 interventions (range 3– 16). Urological history is summarized in Table 1. The initial intervention was a primary closure in 16 patients (84.3%), and cystectomy with primary urinary diversion in three, with ureterosigmoidostomy in two cases, and cutaneous
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ureterostomy in one; together with osteotomy in eight cases. A secondary
urinary diversion with creation of a urinary reservoir was required in 67% of the patients: four received an ileal reservoir and nine a colonic reservoir. Thirteen of
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the patients also received a Mitrofanoff conduit.
Other relevant events include nephrolithiasis in two patients and bladder stones in three. Seven had some degree of vesicoureteral reflux at some point of their life, and one presented chronic renal failure. Serious and common complications were mainly genital (13 cases, 31% of the patients) or rectal prolapse (three cases).
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With the purpose of urinary continence, several different methods had been used by the patients in the cohort: seven underwent a Young-Dees type cervicourethroplasty, three a silimed periurethral constrictor implantation
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(placed perimitrofanoff), four bulking agents, seven closure of the bladder neck, and 14 (74%) performed intermittent self-catheterization. Despite use of such methods, six patients were incontinent according to the ICIQ-SF questionnaire
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(score higher than 0 [7], see the full questionnaire in the Appendix), and the mean score of the questionnaire was 10.3. For the POTENZIANI-14 questionnaire, the mean score was 10.3,
equivalent to a mild impact of incontinence in QoL. Of the patients, 63% scored a 0, interpreted as perception of normal continence. Occasional leaks were reported by 37% of patients: five perceived mild incontinence with little impact on QoL (two to three times a week during nighttime; score=8) and two perceived moderate incontinence with a big impact in QoL (spontaneous leaks, more than once daily, during sexual intercourse, score=15).
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ACCEPTED MANUSCRIPT Regarding academic and occupational history, 11 (57.9%) patients had graduated high school and eight (42.1%) obtained college degrees. Twelve (63.2%) were active workers, five unemployed, and two had invalidity status. Scores obtained in the SF-36 questionnaire are summarized in Table 2, with reference scores from a general Spanish population, and p-values of the
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comparison between the scores from our cohort and the norm scores from controls.
According to the SF-36 QoL questionnaire, mean scores in our study
group do not differ significantly from those of the control population in most of
health (p<0.001), and emotional role (p<0.0001).
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the evaluated QoL fields, except for physical functioning (p<0.002), mental
Of the patients, 84% were sexually active. At the time of the study, 42%
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reported having dyspareunia and one had genital prolapse. Two patients did not have partners. Five patients gave birth, all by C-section, and there were three abortions. The mean score for the PISQ-12 questionnaire was 8.55 (scores≤12 are interpreted as severe sexual dysfunction). Discussion
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BEEC places a significant burden on patients, and constitutes one of the most challenging pathologies for the pediatric urologist. Its management implies a close medical follow-up, several surgeries during childhood, and potential socially impairing issues such as incontinence, scars, and physical differences
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from peers. In this study we aimed to quantify the impact of the condition on the QoL of BEEC adult patients, using validated psychosocial questionnaires.
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The questionnaires used to assess QoL have been repeatedly validated and widely used in many different pathologies, including previous studies in BEEC [4,8]. Rate of response in this group was 57%: 19 patients out of the 33 we contacted fully completed the questionnaires. In the context of BEEC QoL assessment, this constitutes a high response rate and an advantage over previous single and multiple center studies [1,9,10]. We found BEEC patients to score normal values in all but three of the SF-36 fields. Scores lower than in the healthy population were those obtained in physical functioning, mental health, and emotional role. These results are consistent with previous studies using the SF-36: Wittmeyer et al. [4] assessed 25 adult patients in a French cohort, showing scores comparable with those of 7
ACCEPTED MANUSCRIPT healthy individuals in all fields except for general health perception and physical functionality. Gupta et al. [9] followed 15 males and six females, reporting scores below the norm only in general health, and even higher than controls in physical functioning. Jochault-Ritz et al. [8] found adult BEEC patients to have normal scores globally and in the physical and pain dimensions, but lower in the
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others, especially social functioning. In the present cohort, academic and occupational performance was
above the populational average, with all patients graduating from high school
and almost half of them pursuing college education. This finding is consistent
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with previous studies on BEEC in young adults [4,11,12].
The extraordinary adaptability and resilience of BEEC patients is highlighted by their close to normal QoL scores, together with their uneventful
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academic and occupational development and their social relationships (with only 10% of patients without a partner in our study). It has been repeatedly reported how proper medical and psychosocial support aids young women affected by BEEC to develop emotional and practical mechanisms to cope with their serious condition, and to manage their condition so that it does not prevent them from taking part in physical and social activities, personal relationships, or
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maternity [13–15]. Despite this remarkably normal functioning, treatment of BEEC targeted towards the best possible QoL still poses many challenges, with patients most concerned about continence and the impact of their body image
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and sexual dysfunction on intimate relationships [1,10,16]. The familial impact of the condition poses a challenge which can affect the patient’s and parents’ mental health and family unit [4,17]. This and other issues specific to BEEC
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patients might not be detected with QoL questionnaires or a typical clinical interview [18], despite having an important psychosocial impact. For the appropriate QoL study and care of BEEC patients, issues such as void difficulties interfering with social interactions, impact of the condition on identity, and dissatisfaction with physical appearance must be addressed. It is strongly advisable for this purpose to complement use of questionnaires with other instruments, such as specific psychosocial interviews using more open questions [19]. Urinary incontinence is one important component affecting the daily functioning of BEEC patients. This condition causes an important impact on the 8
ACCEPTED MANUSCRIPT socialization and QoL of affected patients [20]. It affects one-third of our study population, constituting a poor outcome of their surgeries, as current reconstructive techniques have reported success rates up to 88% in achieving continence [21]. However, they score only a mild impact on their QoL from incontinence. It might be reasonable that with better surgical outcomes, the
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impact on QoL could be even milder. Literature generally shows incontinence as one important concern in BEEC patients, and it has been correlated to
poorer QoL scores [4,8,13]. However, despite the big impact of incontinence among other components of BEEC, affected patients manage to achieve a
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better overall QoL score than those with other urological conditions that might seem milder, such as stress incontinence [20] or kidney stones [16]. A
suggested explanation is that BEEC incontinent patients have always lived with
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this condition. Thus, during childhood, they developed strategies to minimize the interference it causes on their daily functioning. This helps them overcome the detrimental effect of incontinence on QoL observed when it debuts in adults [2]. The finding is consistent in other congenital causes of incontinence such as spina bifida [22,23].
Genital prolapse is another important issue in this patient population. It
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was present in 31% of our sample, and has been reported in up to 50% in other series [1]. It is particularly concerning related to pregnancy. Even though BEEC patients’ deliveries are always managed with C-sections, the patients are
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expected to have some degree of genital prolapse, especially after a second pregnancy. In a series of 14 pregnant women published by Giron et al. [24], 50% of patients had genital prolapse after delivery, and four spontaneous
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abortions occurred resulting from genital prolapse. Another study on 12 patients by Dy et al. [14] reported genital prolapse in three patients. Sexual function is an important health dimension to assess in the long-
term follow-up of these patients. Conditions associated to BEEC can severely affect it, such as the aforementioned incontinence and genital prolapse. Dyspareunia is also an important concern, affecting 42% of our patients and up to 50% in other studies [15]. It is related to the anatomical particularities of BEEC: most patients have a narrow introitus, requiring surgery or the use of vaginal dilators [1]. Even with the challenges posed by BEEC, studies
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ACCEPTED MANUSCRIPT consistently find patients developing sub-normal sexual function [1,15,25], 90% of the patients in our study have a partner and 84% are sexually active. As it is a rare disease, research on BEEC is always done on small sample sizes, in terms of statistical power. This is the case in the present study. The effect of diminished statistical power could potentially conceal small
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differences with controls, and this possibility of bias always has to be taken into account. However, consistency with previous publications gives some support to our findings, along with the fact that smaller statistic differences are less likely to translate into clinical significance.
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Another potential limitation of the study is the proportion of subjects
responding to the questionnaires, being 57%. Even though this may seem a low rate of response, it is similar to or even higher than rates in previous studies on
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BEEC functionality and QoL [1,9,10]. Studies have been conducted into the reasons for the high number of patients not responding, but these are not yet clarified. However, there is no association with parameters such as gender, age, reference center, or total number of surgeries [8]. This phenomenon, constant throughout the literature, raises concern about the possibility of a nonresponse bias: if the patients refusing to participate do so because they find
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their condition too distressing to discuss, that could possibly skew the results towards the more positive results of those patients willing to participate [16]. Conclusions
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We used four validated psychosocial questionnaires to assess health-related QoL, sexual function, incontinence, and impact of incontinence on QoL in adult females with BEEC. The scored for women with BEEC regarding QoL differed
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from those of the general population in only three out of eight health items: physical functioning, emotional role, and mental health. We conclude that the patients present good general QoL, regardless of having endured several surgical interventions during their life and often facing medical challenges such as incontinence and genital prolapse. Patients develop normally socially and professionally, reaching a high level of education. Similarly, they are able to establish intimate relationships and develop a satisfactory social life. Urinary incontinence, poor body image, and sexual dysfunction are the main factors affecting patients’ QoL. Medical attention should target those fields 10
ACCEPTED MANUSCRIPT for improvement. However, the personal resilience and adaptation mechanisms of these patients help them to overcome those challenges and achieve a normal QoL. Conflict of interest None.
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Funding None. References [1]
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exstrophy. BJU Int 2015;115(4):633–8. [2]
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Schaeffer AJ, Yenokyan G, Alcorn K, Furth SL, Diener-West M, Wu AW,
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et al. Health related quality of life in adolescents with bladder exstrophyepispadias as measured by the Child Health Questionnaire-Child Form 87. J Urol 2012;188(5):1924–9. [3]
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Table 1. Main urological history of the patients in the cohort Initial surgery
Primary closure Cystectomy + ureterosigmoidostomy Osteotomy Lithiasis (33%) Renal Bladder Bladder ampliation/ Ileal Reservoir (67%) Colonic Mitrofanoff Vesicoureteral reflux 36% Genital prolapse 31% Rectal prolapse 15% 13
84.3% 10.5% 42% 10% 21% 33% 67% 67%
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Table 2. SF-36 mean scores and SD in the general healthy population, mean and SD scores in the BE patient cohort, and p-value of the differences with normal values Exstrophic bladder patients, mean (SD)
p
95%CI
80 (18.8) 94.4 (12.8) 96 (14.1) 91.1 (25.7) 77.7 (17.3) 69.9 (18.4) 82.3 (24.8) 90.1 (25.2)
70 (18.5) 85 (25.6) 92 (25.8) 88 (25.3) 48 (15.4) 70 (18.5) 90 (25.1) 50 (15.6)
=0.21 <0.002 =0.22 =0.6 <0.001 =0.98 =0.178 <0.001
1.15-18.5 3.56-15.24 -2.42-10.4 -8.52-14.7 21.18-36.8 -8.42-8.22 -18.91-3.5 28.73-51.4
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General health Physical functioning Social functioning Physical role Mental health Vitality Body pain Emotional role
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Spanish general population, mean (SD)
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Health parameter
Appendix. Translation of the ICIQ-SF Spanish version
Number
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ICIQ-SF (Spanish version)
Date
Day
Month
Year
CONFIDENTIAL
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There is many people who leak urine on a certain moment in time. We are trying to determine the number of people presenting this problem and to which point they are concerned about their situation. We would very much appreciate if you answer the following questions, regarding your situation in the past four weeks.
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1. Please, write your date of birth Day
2. Your are (mark which)
Female
14
Month Male
Year
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0
Once a week or less
1
2
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Two or three times a week
Once every day
3
More than once every day
4
5
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Continuously
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4. We would like to know your perception regarding the amount of urine you think you leak. Quantity of urine you usually leak (either wearing protection or not) (mark one) None
0
A very small amount
2
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A moderate amount 4
A large amount 6
5. This urine leakage you experiment, how much they affect your daily life? (please, circle a number between 0 –they do not affect me- and 10 –they affect me a lot): 2
3
4
5
6
7
8
9
10 A lot
ICI-Q score: add the scores of questions 3 + 4 + 5 =
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0 1 Nothing
15
ACCEPTED MANUSCRIPT 6. When do you leak urine? (mark all that apply to you) I never leak urine
6.2
I leak urine before I reach the WC
6.3
I leak urine when I cough or sneeze
6.4
I leak during my sleep
6.5
I leak when I do physical activity/exercise
6.6
I leak urine after I urinated and put my clothes on
6.7
I leak urine without apparent reason
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6.1
6.8
I continuously leak urine
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Thank you very much for answering this questions.
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S1477-5131(16)30060-2
DOI:
10.1016/j.jpurol.2016.05.005
Reference:
JPUROL 2193
To appear in:
Journal of Pediatric Urology
Received Date: 18 January 2016 Accepted Date: 17 May 2016
Please cite this article as: Bujons A, Lopategui DM, Rodríguez N, Centeno C, Caffaratti J, Villavicencio H, Quality of life in female patients with bladder exstrophy-epispadias complex: long-term follow-up, Journal of Pediatric Urology (2016), doi: 10.1016/j.jpurol.2016.05.005. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT ESPU Prague 2015 Quality of life in female patients with bladder exstrophy-epispadias complex: long-term follow-up Anna Bujons *, Diana M. Lopategui, Nelly Rodríguez, Clara Centeno, Jorge Caffaratti, and Humberto Villavicencio
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Pediatric Urology Department, Fundació Puigvert, Barcelona, Spain
* Corresponding author. C/ Cartagena, 340–350, 08022 Barcelona, Spain.
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E-mail address: [email protected]
Summary Introduction: Bladder exstrophy-epispadias complex (BEEC) is a congenital malformation that requires multiple surgeries during childhood and
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life-long follow-up. It often presents with conditions that have the potential to impact quality-of-life (QoL) and psychosocial functioning of affected patients, such as incontinence and sexual dysfunction. The aim of this study is to examine the QoL, urinary continence, sexual function, and overall health in a long-term series of female patients with BEEC.
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Method: A retrospective review was performed of female patients with BEEC born between 1964 and 1996. Thirty-three patients were asked to complete four validated questionnaires to evaluate their QoL regarding urinary continence and sexual activity (ICIQ, Potenziani-14, and PISQ-12 questionnaires). Nineteen
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patients completed and returned the questionnaires. The overall QoL was assessed with the SF-36 questionnaire, and demographics were evaluated. Statistical analysis was performed to compare the general QoL with that of the
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general population.
Mean (range) age, years Previous history
26 (18–50) Total number of surgeries,
13 (3–16)
mean (range) Initial surgery, n
Primary closure
16
Cystectomy +
2
ureterosigmoidostomy Cystectomy + cutaneous
1
1
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Lithiasis, n
8
Ileal reservoir
33%
Colonic reservoir
67%
Mitrofanoff conduit
67%
Renal
2
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Bladder enlargement or reservoir
Osteotomy
Bladder
4
Vesicoureteral reflux
36%
Chronic renal failure
6%
31%
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Genital prolapse Rectal prolapse Continence status, n (%)
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Normal continence Occasional leak
Mild incontinence, little QoL
15%
12 (63) 7 (37) 5
impact, n
Moderate incontinence, big
2
QoL impact, n Young-Dees
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Continence management, n
7
cervicourethroplasty
Silimed perimitrofanoff
3
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constrictor
Sexual function and fertility
Education level, n
Bulking agents
4
Closed bladder neck
7
Autocatheterization
14
Incontinent
6
Sexually active
84%
Not in a relationship
10%
Dyspareunia
42%
C-section, n
5
Abortions, n
3
High school
11
Higher education
8
2
ACCEPTED MANUSCRIPT Occupational status, n
Active workers
12
Unemployed
5
Invalidity status
2
Results: The median age of the patients was 26 years (range 18–50) (Table). A
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low to moderate impact of urinary incontinence on QoL was reported by 30% of patients in the ICIQ. Also as a result of urinary incontinence, 84% of patients
reported a moderate to severe impact on their sexual lives. Twelve patients got married with eight gestations and five births. SF-36 reported general QoL
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comparable with that of the general population in five out of eight items.
Differences were seen in the mental health, emotional role, and physical functioning items (p<0.001). The main factors for the differences were poor
reported by 70% of patients.
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body image, anxiety, and urinary incontinence. A satisfactory social life was
Conclusion: Questionnaire studies on BEEC consistently report a high rate of patients not answering, 43% in the present study. The rarity of the disease determines a small sample size, which diminishes statistical power and could potentially conceal small differences with controls. Despite these limitations, the
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present findings are consistent with previous studies on BEEC with validated QoL questionnaires: adult women with BEEC suffer psychosocial impact mainly from incontinence, and also from gynecological complications, but their
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resilience and coping mechanisms allow them to achieve a quasi-normal QoL. Female patients with BEEC reported a normal QoL in five of eight items in the SF-36 questionnaire. Urinary incontinence was the main factor for the
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moderately decreased QoL according to specific questionnaires.
KEYWORDS
Long-term follow-up; Quality of life; Female patients;
Exstrophy-epispadias
Introduction
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ACCEPTED MANUSCRIPT Bladder exstrophy-epispadias complex (BEEC) is a congenital malformation presenting with pubic diastasis, anterior displacement of internal genitals and labia majora, and protrusion of the bladder through a defect of the lower abdominal wall [1]. It is one of the most severe and challenging conditions in pediatric urology. Most of the affected patients require multiple surgeries during
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childhood, and life-long follow-up by a specialized multidisciplinary care team. This congenital defect can affect patients’ quality of life (QoL) both in the short and long term, and its psychosocial impact is not well understood [2].
Literature evaluating the effect of BEEC on QoL is still very scarce: a
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meta-analysis by Diseth et al. [3] showed that only 0.8% of studies on BEEC
look into the psychosocial or QoL implications of the disease. Several studies found BEEC to have a significant impact on mental health and QoL, particularly
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regarding sexual function and anxiety disorders [2], with patients scoring lower than the general population on questionnaires assessing general health perception and physical activity limitations [4].
Over the last decade, research on BEEC has led to better care management and reconstructive techniques, ultimately improving the morbidity and mortality of BEEC. Patients now reach adulthood aiming for a good QoL,
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including normal sexual function and acceptable urinary continence. We designed a retrospective study to evaluate the QoL of adult females with BEEC, including parameters of urinary continence, sexual function, and
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general health. Our aim is to quantify the overall result of treatment of BEEC and follow-up into functional adulthood, not the result of any particular surgical technique.
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Material and methods The study population was selected from the patients followed at the pediatric unit of a third-level urology-specialized center. Selection criteria included diagnosis of BEEC, female gender, and age >18 years. The study was approved by the ethics committee of our center. The method to collect data was the patients’ self-completion of four validated questionnaires: Short Form-36 Health Survey (SF-36, Spanish version), Pelvic Organ Prolapse/Urinary Incontinence Sexual Questionnaire (PISQ-12), International Consultation on Incontinence Modular Questionnaire (ICIQ-SF), and POTENZIANI-14.
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ACCEPTED MANUSCRIPT Generic QoL questionnaires are instruments designed to evaluate the impact of disease conditions on an individual’s daily functioning, regardless of the underlying disease. They have been used and validated in many different clinical scenarios to assess the effect of specific entities, as well as to make comparisons between diseases and with controls from a healthy population.
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The SF-36 questionnaire evaluates health-related QoL providing a profile of the health state in eight different dimensions: physical functioning, physical role,
body pain, general health, vitality, social functioning, emotional role, and mental health. Every concept is scored based on the answer to a total of 36 different
and best possibly imaginable health state [5].
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items in a numerical scale from 0 to 100, equivalent, respectively, to the worst
The PISQ-12 evaluates the sexual functioning of women with genital
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prolapse or urinary or fecal incontinence. It has been validated to the Spanish language and sociocultural environment. It consists of 12 items analyzing the impact of the symptoms on the patient’s sexual function, and provides a numeric score for each item from 0=always, to 4=never, and a total score from 0 to 48. Lower scores are equivalent to a greater sexual dysfunction caused by the symptomatology [6].
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The ICIQ-SF (see Appendix) has been validated for the diagnosis of urinary incontinence [7], and POTENZIANI-14 evaluates the impact of urinary incontinence on QoL in women.
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Patient characteristics were recorded in a clinical interview, including demographic data, medical and surgical previous history, childhood development, academic and job history, relationships, and physical and sexual
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function. The patients fulfilling the study criteria were contacted by telephone and informed regarding the methodology and purpose of the study. Those who agreed to participate were sent the four questionnaires and a consent form via e-mail.
We identified 33 BEEC female adult patients followed at our institution, 19 (57%) of whom completed and returned all questionnaires. Data collection and all statistical analyses were performed with SPSS software version 15. Comparisons of the subjects’ scores on questionnaires with general population normalized scores were performed with the Student’s ttest. 5
ACCEPTED MANUSCRIPT Results Of 33 eligible patients from our institution, 19 (57%) agreed to participate in the study and completed the questionnaires fully. Their age ranged from 18 to 50 years, with a mean of 26 years. All the 19 patients included in the study underwent multiple surgeries
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after the primary defect closure, with a mean total of 13 interventions (range 3– 16). Urological history is summarized in Table 1. The initial intervention was a primary closure in 16 patients (84.3%), and cystectomy with primary urinary diversion in three, with ureterosigmoidostomy in two cases, and cutaneous
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ureterostomy in one; together with osteotomy in eight cases. A secondary
urinary diversion with creation of a urinary reservoir was required in 67% of the patients: four received an ileal reservoir and nine a colonic reservoir. Thirteen of
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the patients also received a Mitrofanoff conduit.
Other relevant events include nephrolithiasis in two patients and bladder stones in three. Seven had some degree of vesicoureteral reflux at some point of their life, and one presented chronic renal failure. Serious and common complications were mainly genital (13 cases, 31% of the patients) or rectal prolapse (three cases).
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With the purpose of urinary continence, several different methods had been used by the patients in the cohort: seven underwent a Young-Dees type cervicourethroplasty, three a silimed periurethral constrictor implantation
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(placed perimitrofanoff), four bulking agents, seven closure of the bladder neck, and 14 (74%) performed intermittent self-catheterization. Despite use of such methods, six patients were incontinent according to the ICIQ-SF questionnaire
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(score higher than 0 [7], see the full questionnaire in the Appendix), and the mean score of the questionnaire was 10.3. For the POTENZIANI-14 questionnaire, the mean score was 10.3,
equivalent to a mild impact of incontinence in QoL. Of the patients, 63% scored a 0, interpreted as perception of normal continence. Occasional leaks were reported by 37% of patients: five perceived mild incontinence with little impact on QoL (two to three times a week during nighttime; score=8) and two perceived moderate incontinence with a big impact in QoL (spontaneous leaks, more than once daily, during sexual intercourse, score=15).
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ACCEPTED MANUSCRIPT Regarding academic and occupational history, 11 (57.9%) patients had graduated high school and eight (42.1%) obtained college degrees. Twelve (63.2%) were active workers, five unemployed, and two had invalidity status. Scores obtained in the SF-36 questionnaire are summarized in Table 2, with reference scores from a general Spanish population, and p-values of the
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comparison between the scores from our cohort and the norm scores from controls.
According to the SF-36 QoL questionnaire, mean scores in our study
group do not differ significantly from those of the control population in most of
health (p<0.001), and emotional role (p<0.0001).
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the evaluated QoL fields, except for physical functioning (p<0.002), mental
Of the patients, 84% were sexually active. At the time of the study, 42%
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reported having dyspareunia and one had genital prolapse. Two patients did not have partners. Five patients gave birth, all by C-section, and there were three abortions. The mean score for the PISQ-12 questionnaire was 8.55 (scores≤12 are interpreted as severe sexual dysfunction). Discussion
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BEEC places a significant burden on patients, and constitutes one of the most challenging pathologies for the pediatric urologist. Its management implies a close medical follow-up, several surgeries during childhood, and potential socially impairing issues such as incontinence, scars, and physical differences
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from peers. In this study we aimed to quantify the impact of the condition on the QoL of BEEC adult patients, using validated psychosocial questionnaires.
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The questionnaires used to assess QoL have been repeatedly validated and widely used in many different pathologies, including previous studies in BEEC [4,8]. Rate of response in this group was 57%: 19 patients out of the 33 we contacted fully completed the questionnaires. In the context of BEEC QoL assessment, this constitutes a high response rate and an advantage over previous single and multiple center studies [1,9,10]. We found BEEC patients to score normal values in all but three of the SF-36 fields. Scores lower than in the healthy population were those obtained in physical functioning, mental health, and emotional role. These results are consistent with previous studies using the SF-36: Wittmeyer et al. [4] assessed 25 adult patients in a French cohort, showing scores comparable with those of 7
ACCEPTED MANUSCRIPT healthy individuals in all fields except for general health perception and physical functionality. Gupta et al. [9] followed 15 males and six females, reporting scores below the norm only in general health, and even higher than controls in physical functioning. Jochault-Ritz et al. [8] found adult BEEC patients to have normal scores globally and in the physical and pain dimensions, but lower in the
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others, especially social functioning. In the present cohort, academic and occupational performance was
above the populational average, with all patients graduating from high school
and almost half of them pursuing college education. This finding is consistent
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with previous studies on BEEC in young adults [4,11,12].
The extraordinary adaptability and resilience of BEEC patients is highlighted by their close to normal QoL scores, together with their uneventful
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academic and occupational development and their social relationships (with only 10% of patients without a partner in our study). It has been repeatedly reported how proper medical and psychosocial support aids young women affected by BEEC to develop emotional and practical mechanisms to cope with their serious condition, and to manage their condition so that it does not prevent them from taking part in physical and social activities, personal relationships, or
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maternity [13–15]. Despite this remarkably normal functioning, treatment of BEEC targeted towards the best possible QoL still poses many challenges, with patients most concerned about continence and the impact of their body image
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and sexual dysfunction on intimate relationships [1,10,16]. The familial impact of the condition poses a challenge which can affect the patient’s and parents’ mental health and family unit [4,17]. This and other issues specific to BEEC
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patients might not be detected with QoL questionnaires or a typical clinical interview [18], despite having an important psychosocial impact. For the appropriate QoL study and care of BEEC patients, issues such as void difficulties interfering with social interactions, impact of the condition on identity, and dissatisfaction with physical appearance must be addressed. It is strongly advisable for this purpose to complement use of questionnaires with other instruments, such as specific psychosocial interviews using more open questions [19]. Urinary incontinence is one important component affecting the daily functioning of BEEC patients. This condition causes an important impact on the 8
ACCEPTED MANUSCRIPT socialization and QoL of affected patients [20]. It affects one-third of our study population, constituting a poor outcome of their surgeries, as current reconstructive techniques have reported success rates up to 88% in achieving continence [21]. However, they score only a mild impact on their QoL from incontinence. It might be reasonable that with better surgical outcomes, the
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impact on QoL could be even milder. Literature generally shows incontinence as one important concern in BEEC patients, and it has been correlated to
poorer QoL scores [4,8,13]. However, despite the big impact of incontinence among other components of BEEC, affected patients manage to achieve a
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better overall QoL score than those with other urological conditions that might seem milder, such as stress incontinence [20] or kidney stones [16]. A
suggested explanation is that BEEC incontinent patients have always lived with
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this condition. Thus, during childhood, they developed strategies to minimize the interference it causes on their daily functioning. This helps them overcome the detrimental effect of incontinence on QoL observed when it debuts in adults [2]. The finding is consistent in other congenital causes of incontinence such as spina bifida [22,23].
Genital prolapse is another important issue in this patient population. It
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was present in 31% of our sample, and has been reported in up to 50% in other series [1]. It is particularly concerning related to pregnancy. Even though BEEC patients’ deliveries are always managed with C-sections, the patients are
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expected to have some degree of genital prolapse, especially after a second pregnancy. In a series of 14 pregnant women published by Giron et al. [24], 50% of patients had genital prolapse after delivery, and four spontaneous
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abortions occurred resulting from genital prolapse. Another study on 12 patients by Dy et al. [14] reported genital prolapse in three patients. Sexual function is an important health dimension to assess in the long-
term follow-up of these patients. Conditions associated to BEEC can severely affect it, such as the aforementioned incontinence and genital prolapse. Dyspareunia is also an important concern, affecting 42% of our patients and up to 50% in other studies [15]. It is related to the anatomical particularities of BEEC: most patients have a narrow introitus, requiring surgery or the use of vaginal dilators [1]. Even with the challenges posed by BEEC, studies
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ACCEPTED MANUSCRIPT consistently find patients developing sub-normal sexual function [1,15,25], 90% of the patients in our study have a partner and 84% are sexually active. As it is a rare disease, research on BEEC is always done on small sample sizes, in terms of statistical power. This is the case in the present study. The effect of diminished statistical power could potentially conceal small
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differences with controls, and this possibility of bias always has to be taken into account. However, consistency with previous publications gives some support to our findings, along with the fact that smaller statistic differences are less likely to translate into clinical significance.
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Another potential limitation of the study is the proportion of subjects
responding to the questionnaires, being 57%. Even though this may seem a low rate of response, it is similar to or even higher than rates in previous studies on
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BEEC functionality and QoL [1,9,10]. Studies have been conducted into the reasons for the high number of patients not responding, but these are not yet clarified. However, there is no association with parameters such as gender, age, reference center, or total number of surgeries [8]. This phenomenon, constant throughout the literature, raises concern about the possibility of a nonresponse bias: if the patients refusing to participate do so because they find
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their condition too distressing to discuss, that could possibly skew the results towards the more positive results of those patients willing to participate [16]. Conclusions
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We used four validated psychosocial questionnaires to assess health-related QoL, sexual function, incontinence, and impact of incontinence on QoL in adult females with BEEC. The scored for women with BEEC regarding QoL differed
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from those of the general population in only three out of eight health items: physical functioning, emotional role, and mental health. We conclude that the patients present good general QoL, regardless of having endured several surgical interventions during their life and often facing medical challenges such as incontinence and genital prolapse. Patients develop normally socially and professionally, reaching a high level of education. Similarly, they are able to establish intimate relationships and develop a satisfactory social life. Urinary incontinence, poor body image, and sexual dysfunction are the main factors affecting patients’ QoL. Medical attention should target those fields 10
ACCEPTED MANUSCRIPT for improvement. However, the personal resilience and adaptation mechanisms of these patients help them to overcome those challenges and achieve a normal QoL. Conflict of interest None.
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Funding None. References [1]
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Table 1. Main urological history of the patients in the cohort Initial surgery
Primary closure Cystectomy + ureterosigmoidostomy Osteotomy Lithiasis (33%) Renal Bladder Bladder ampliation/ Ileal Reservoir (67%) Colonic Mitrofanoff Vesicoureteral reflux 36% Genital prolapse 31% Rectal prolapse 15% 13
84.3% 10.5% 42% 10% 21% 33% 67% 67%
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Table 2. SF-36 mean scores and SD in the general healthy population, mean and SD scores in the BE patient cohort, and p-value of the differences with normal values Exstrophic bladder patients, mean (SD)
p
95%CI
80 (18.8) 94.4 (12.8) 96 (14.1) 91.1 (25.7) 77.7 (17.3) 69.9 (18.4) 82.3 (24.8) 90.1 (25.2)
70 (18.5) 85 (25.6) 92 (25.8) 88 (25.3) 48 (15.4) 70 (18.5) 90 (25.1) 50 (15.6)
=0.21 <0.002 =0.22 =0.6 <0.001 =0.98 =0.178 <0.001
1.15-18.5 3.56-15.24 -2.42-10.4 -8.52-14.7 21.18-36.8 -8.42-8.22 -18.91-3.5 28.73-51.4
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General health Physical functioning Social functioning Physical role Mental health Vitality Body pain Emotional role
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Spanish general population, mean (SD)
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Health parameter
Appendix. Translation of the ICIQ-SF Spanish version
Number
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ICIQ-SF (Spanish version)
Date
Day
Month
Year
CONFIDENTIAL
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There is many people who leak urine on a certain moment in time. We are trying to determine the number of people presenting this problem and to which point they are concerned about their situation. We would very much appreciate if you answer the following questions, regarding your situation in the past four weeks.
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1. Please, write your date of birth Day
2. Your are (mark which)
Female
14
Month Male
Year
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0
Once a week or less
1
2
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Two or three times a week
Once every day
3
More than once every day
4
5
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Continuously
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4. We would like to know your perception regarding the amount of urine you think you leak. Quantity of urine you usually leak (either wearing protection or not) (mark one) None
0
A very small amount
2
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A moderate amount 4
A large amount 6
5. This urine leakage you experiment, how much they affect your daily life? (please, circle a number between 0 –they do not affect me- and 10 –they affect me a lot): 2
3
4
5
6
7
8
9
10 A lot
ICI-Q score: add the scores of questions 3 + 4 + 5 =
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0 1 Nothing
15
ACCEPTED MANUSCRIPT 6. When do you leak urine? (mark all that apply to you) I never leak urine
6.2
I leak urine before I reach the WC
6.3
I leak urine when I cough or sneeze
6.4
I leak during my sleep
6.5
I leak when I do physical activity/exercise
6.6
I leak urine after I urinated and put my clothes on
6.7
I leak urine without apparent reason
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6.1
6.8
I continuously leak urine
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Thank you very much for answering this questions.
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