Four patients (three male and one female) had urinary incontinence. In the female patient, urinary continence improved after bowel management programme. However, all the male patients required clean intermittent catheterization for neurogenic bladder, two owing to sacral abnormality and the remaining one occurring as a postoperative complication of previous ARM reconstruction. Only one female patient in our study has delivered a child; the remaining six were seeking treatment before marriage. The male patients were concerned about cleanliness, sexual relationship and embarrassment at job. Their fear of embarrassment at job disappeared when they achieved continence with bowel management programme.
The type of ARM determines the number of surgeries needed for repair. The long-term functional outcome of patients of ARM depends on anatomy of birth defect and function after the reconstruction 2. While patients of ARM with mild defect usually develop adequate bowel control, many others have long-lasting problems related to defecation affecting their QoL, which is an important endpoint in medical care 1. Clinicians should inform, treat and refer their patients to the appropriate caregiver 2.
Paediatric surgeons should be aware of deficits in psychological functioning and therefore not only enquire about their patient’s physical well-being but also about their patients’ emotional and social well-being. This is especially important in adolescent and adults as they are reported to have lower levels of psychosocial functioning than children 2–4.
Patients with ARM have to learn to live with a variety of problems, and it remains unclear whether these problems remain consistent over time 2. In Indian circumstances, probably parents of patients having a colostomy are initially anxious that their child is not passing stool through the normal route.
Therefore, when reconstructive surgery is done, they heave a sigh of relief. Some amount of incontinence or soiling in the initial phase does not bother them, or they wait in the hope that this will improve with time. However, as the child enters into adolescence the psychosocial problems increases, and in adulthood job and matrimonial issues cause anxiety and low self-esteem. In this scenario, the previous treating surgeon is usually not available.
It is difficult to classify faecal continence. Frequency and consistency of stool, amount of uncontrolled loss, rectal sensation, possibility of holding back defecation, discrimination between formed, loose or gaseous stool and need for therapy are considered to be important factors defining faecal continence 5.
It is important to know and understand whether the adults are content with their status or continent after the repair of high ARM. In a long-term study in patients aged 18 years and older, all patients operated on for high ARM had some form of incontinence. Normal faecal continence was taken as producing faeces once or twice a day of normal consistency at the proper time and place without soiling in between and without taking dietary measures, anal aids or medicine. Nobody could fulfil these criteria, although most patients (84%) were satisfied with the achieved level of continence with some measures such as drugs, dietary or anal aids 5. This study concluded that adults after the ARM repair although content (satisfied) were not continent as per the criteria mentioned.
Chronic difficulty in defecation affects the QoL. Most studies found a positive association between disease-specific functioning and QoL, directing attention towards alleviating symptoms in improving psychosocial functioning 4,6,7. Relationship between disease-specific functioning and QoL remains unclear 2. Although adults are reported to have lower level of psychosocial functioning 8,9, a few studies reported less faecal problems with adults 9–11. In our study, patients showed improvement in QoL scores and better self-esteem the moment soiling disappeared. Overall the scores almost doubled (mean: 1.26–3.04 for QoL and 2.7–5.1 for Kelly’s score).
Another important goal of management is the preservation of sexual and urological function. There seems to be a close relationship between psychosocial development, urological function and sexual activity. Analysis of 55 patients, 18–56 years of age, showed that 35% of females and 69% of males lived alone. Twenty-six per cent of females became pregnant and 32% males fathered children 12. Twenty-one patients suffered mucosal prolapse, 18 had megasigmoid/megacolon, 17 had anal stenosis, 14 had permanent neurogenic bladder dysfunction, 23 had recurrent urinary tract infection, 37 patients had to be reoperated and 41 patients needed means of aftercare to achieve social continence 13.
Urinary incontinence may improve with just bowel management as seen in our female patients or require clean intermittent catheterization when secondary to a neurogenic bladder. In our study, only one female had delivered a child. The remaining six were seeking treatment before getting married. Males were concerned about cleanliness, sexual relationship and embarrassment at job. The fear of embarrassment at job disappeared when they achieved continence with bowel management programme. Two patients were happy with MACE and felt confident being independent of relatives for bowel washes. One patient is still requiring rectal washes but is not willing to undergo another surgery for MACE.
Probably these were the few patients who approached various clinicians in the hope of improving their QoL. There might be many others who have exhausted their resources and have lost hopes of improvement or are in depression.
Long-term follow-up of patients of ARM is essential, which might be lifelong at times for some patients. It is important to establish an association or a forum with adult clinicians, so as to have a transition of care and improve their psychosocial, sexual and colorectal functional problems.
The authors acknowledge the contribution of Dr Shubhanka Kala in assessing quality of life of patients; Dr Prema Menon for helping in drafting the manuscript; and Arpita Chhipa for collection of the data.
Conflicts of interest
There are no conflicts of interest.
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