37.1 Introduction
The birth of a child with an imperforate anus is not only a surgical challenge, but also a shocking event for a partnership and the whole family. Neither par- ents nor physicians, who have no regular contact with such cases, can imagine how this event (and the ef- forts for a definitive solution by pull-through surgery) will influence the social system around this handi- capped individual. A diagnosis of anorectal anomaly (anorectal malformation, ARM) no longer means a death sentence, and over the last 6 decades, surgi- cal treatment has became more and more successful, especially after the introduction of the current gold standard of posterior sagittal anorectoplasty (PSARP) [6]. For this reason, secondary issues have become more and more relevant. There are methods of im- proved follow-up [18,31] and coping with this special situation by both patients and families.
The view of a surgeon, of course, differs from that of a family. Unlike the surgeon, the families struggle with the daily care of the child after the pull-through surgery and live with the long-term consequences.
Some surgeons even think that there are no further problems at all, if there are no surgical problems or complaints. An evaluation of the surgical results with the outcome of continence is usually performed only in their own hospital. Furthermore, the continence scores and their interpretation are quite divergent be- tween different centres. Hopefully, the Krickenbeck standards [13] will help to rectify the current situa- tion (Tables 37.1–37.3). On the other hand, unfortu- nately a lot of patients and parents tolerate major and minor disabilities or suppress the problems.
Table 37.1 Standards for diagnosis: International classification (Krickenbeck)
Major clinical groups Rare/regional variants Perineal (cutaneous) fistula
Rectourethral fistula • prostatic • bulbar Rectovesical fistula Vestibular fistula Cloaca No fistula Anal stenosis
Pouch colon Rectal atresia/stenosis Rectovaginal fistula H-fistula
Others
Table 37.2 International grouping (Krickenbeck) of surgical procedures for follow-up
Operative procedures Perineal operation Anterior sagittal approach Sacroperineal procedure Posterior sagittal ano- rectoplasty (PSARP) Abdominosacroperineal pull-through
Abdominoperineal pull-through Laparoscopically assisted pull-through Associated conditions Sacral anomalies
Tethered cord Contents
37.1 Introduction . . . 459
37.2 Materials and Methods . . . 461 37.3 Results . . . 461
37.3.1 Demographics and Classification . . . 461 37.3.2 Methods of Aftercare . . . 462
37.3.3 Degree of Contentment . . . 462 37.3.4 Continence Function (HAQL) . . . 463 37.3.4.1 Soiling . . . 463
37.3.4.2 Constipation . . . 464 37.3.5 Impact on the Family . . . 464 37.4 Discussion . . . 464
37.4.1 A Call for Further Collaboration . . . 466 37.4.1.1 Benefit for the Surgeon . . . 466 37.4.1.2 Benefit for the Patient . . . 467 37.5 Conclusion . . . 467
References . . . 468
37 ARM: Aftercare and Impact
from the Perspective of the Family
Ekkehart W.D. Jenetzky and Nicole Schwarzer
Table 37.3 International classification (Krickenbeck) for post- operative results
1. Voluntary bowel movements Yes/no
Feeling of urge, capacity to verbalise, hold the bowel movement
2. Soiling Yes/no
Grade 1: Occasionally (once or twice per week) Grade 2: Every day, no social problem Grade 3: Constant, social problem
3. Constipation Yes/no
Grade 1: Manageable by changes in diet Grade 2: Requires laxatives
Grade 3: Resistant to laxatives and diet
In the last 2 decades, however, in several coun- tries organisations of concerned people have been established (Table 37.4). For such rare conditions like ARM they have become an indispensable support and information network for the secondary issues that ac- company this congenital and, in spite of successful surgery, chronic condition. Collaboration between parents and patients on one side and physicians on the other is a promising development that has made this article possible. This is the first time that a con- tribution from a self-help organisation has appeared in a standard textbook on anorectal malformations (ARM).
The German branch (Selbsthilfeorganisation für Menschen mit Anorektalfehlbildungen eV, SoMA eV) of support groups for people with ARM recently
Table 37.4 Eleven currently known support groups for anorectal malformations (in 2005)
Country Name Established Members Contact
Norway NFA: Norsk Forening
for Analatresi
1981 300 http://www.analatresi.no
e-mail: have a look on website
Chile Asociacion Nacional de
ninos con malformacio- nes anorectales de Chile
1986 450 no website available
e-mail: marioantoniovarela@vtr.net
USA PTN: Pull-thru Network 1988 700 http://www.pullthrough.org
e-mail: info@pullthrough.org
Germany SoMA: Self-help-or-
ganisation for people with anorectal malformations
1989 500 http://www.soma-ev.de
e-mail: info@soma-ev.de
Finland AH-POTILAAT-RY:
Finnish association for Anus atresia and Hirschs- brung disease patients
1991 100 http://www.ah-potilaat.org
e-mail: info@ah-potilaat.org
The Netherlands Dutch Association
“Vereniging Anusatresie” 1993 350 http://www.anusatresie.nl e-mail: info@anusatresie.nl
Italy AIMAR: Associazione
Italiana per le Mal- formazioni AnoRettali
1994 500 http://www.romacivica.net/aimar/
e-mail: dalia.aminoff@agcm.it
Taiwan Imperforate Anus
Family Association 1994 200 http://www2.mmh.org.tw
e-mail: mmhss@ms2.mmh.org.tw
Israel Israelian Parents Organisation 1996 50 http://www.itmut.info
e-mail: phili@itmut.info
Portugal APMAR: Associacao
Portuguesa de Malfor- macoes Anorectais e Patologias Associadas
2003 ? http://www.apamar.org
e-mail: info@apamar.org
Australia PCAA:
Paediatric Continence Association of Australia
2004 200 http://www.pcaa.org.au
performed an exhaustive survey of member families.
Some of the results of this ground-breaking research will be presented in this article. The focus of this con- tribution is on the application of non-surgical meth- ods, contentment and continence function according to the Krickenbeck criteria and the impact on families as seen by parents.
Collaborative international studies will become possible when cooperation between professionals and support groups is improved further. A visionary goal is a standardised follow-up chart and regular registra- tion of each individual in their country-specific net- work (Table 37.4).
37.2 Materials and Methods
This investigation was approved by an independent ethics committee at the Charité – University Medi- cine in Berlin. The study was conducted between the autumn of 2004 and spring 2005. It was a postal sur- vey addressed to member parents involving a stan- dardised questionnaire (total 23 pages). Parents were included if their children were between 3 and 17 years of age. The intention was to collect epidemiological data regarding type of ARM, type of post-surgical therapy, degree of contentment, persisting needs, psy- chiatric screening, quality of life (QOL), faecal conti- nence and family impact, using descriptive statistics.
The final sample consisted of 104 (28%) polled families who are members of the parent network from all over Germany. Of course this is a selected sample of very committed parents, with one in three partici- pating couples having an academic degree.
Evaluation of classification, aftercare methods and degree of contentment was carried out in collabora- tion with the Italian association for ARM (Associazi- one Italiana per le Malformazioni AnoRettali). The results of the Italian questionnaire were based on a sample size of 209 (with no age limits), which is about double our sample [1].
QOL and continence were measured using a paedi- atric disease-specific instrument, the Hirschsprung’s disease/ARM quality-of-life instrument (HAQL) de- veloped by Hanneman et al. [8]. We translated the HAQL into German according to the guidelines. The HAQL and a five-item index described by Ditesheim and Templeton [5] are the only QOL scores assessing faecal incontinence in children [29]. The strong rela- tionship between incontinence and QOL was demon- strated by Rothbarth et al. [24] on two other scales in 32 adult patients. Hanneman et al. [8] evaluated the HAQL on 534 patients aged 6–17 years. Whereas we
only used the parent version of the HAQL (initially only for families with 6- to 11-year-olds) consisting of 46 items with 12 additional items for children with stomas, this questionnaire covered the following do- mains: laxative and constipating diet (each two items), diarrhoea (two items), constipation (one item), faecal continence (eight items), urinary continence (four items), social functioning (three items), emotional functioning (six items), body image (two items) and physical symptoms (nine items). We transformed the answers into two of three qualitative outcome do- mains according to the Krickenbeck agreement: soil- ing and constipation (see Table 37.3).
To evaluate the impact on the family with a chroni- cally ill child, the Anglo-American impact-on-fam- ily scale (IFS) [27] was originally developed with 33 items ordered in 4 levels. In 2001, Ravens-Sieberer et al. [22] validated a German version of this self-re- port scale on 273 families with a child affected by dif- ferent chronic conditions, such as epilepsy, diabetes and neurological disabilities from mild psychomotor problems to severe mental retardation. We used this so-called FaBel (Familien-Belastung-Fragebogen) to estimate the impact of the specific disease (ARM) on the family in several areas. We stratified the score for each scale into four categories: “better” if the result was less then one standard deviation (SD) below the mean of the reference population (< mean – 1×SD) and “worse” or “much worse” if the score was raised more than one or two (“much worse”) standard de- viations (> mean + 1×SD or > mean + 2×SD, respec- tively), respectively, above the reference population of chronic disabled children. The results of our family study can thus be compared with a reference popula- tion (i.e. families with different chronic conditions).
Five scales could be separated: financial burden and job problems (4 items), daily social impact, partner- ship, leisure time (15 items), personal strain, worries regarding the future (5 items), coping problems of the parents (3 items) and concerns regarding siblings (6 items). The first four scales built up the total impact score (27 items). This questionnaire is available in four languages, English, German, Spanish and Italian.
37.3 Results
37.3.1 Demographics and Classification
The sample consisted of 104 responses from parents
of the German self-help organisation SoMA eV. In
78% of cases the mother completed the questionnaire,
in 7% the father and in 15% both parents. In 19% of
cases only one parent was living together with the child. About 30% of the families had no other chil- dren, while 42% have one further child. One-third of the parents have a tertiary academic degree.
The age range of the affected child was 3–17 years, with a median of 7 years (mean: 8 years). Most cases were aged between 5 (25th percentile) and 11 years old (75th percentile) and 60% were male.
For classification regarding the ARM we used the descriptive Wingspread classification [28] as well as the fistula classification [17]. The type of fistula was not known by the parent in 18% of cases. About 19%
had a rectobulbar fistula and 20% a rectovesical or a bladder-neck fistula. A perineal fistula was reported in 13%. There was no fistula in 8%. In 7 out of 12 girls with a vaginal fistula there was no recognised cloacal malformation, although it was likely to be an unrec- ognised vestibular fistula (4 cases) or cloacal malfor- mation (3 cases). Therefore, depending on the correct diagnosis, 8–12% of participants had a vestibular fis- tula and 16–19% had a cloacal anomaly.
About 5% had a different diagnosis from ARM, such as Hirschsprung’s disease, or the diagnosis was unknown, and 5% could not define the level of the ARM according to Wingspread. According to the Wingspread classification, 17% had low ARM, 11%
had an intermediate ARM and 46% a high ARM [28].
The data from Italy were quite similar, but only 8%
had a rectovesical fistula, whereas in 18% no fistula was diagnosed. We also assessed in detail the associ- ated malformations and, for example, VATER (Verte- bral, Anal, Tracheo-Esophageal, Renal anomalies) or VACTERL (Vertebral, Anal, Cardiac, Tracheo-Esoph- ageal, Renal and Limb anomalies) malformations were present in 24%.
37.3.2 Methods of Aftercare
The possible methods of aftercare available are given in Table 37.5. Only 25% of the families received regu- lar follow-up, with 15% believing no aftercare was re- quired; 60% did not receive any aftercare! The result of the received aftercare was judged to be good in 70%
of cases, moderate in most other cases (27%). Two- thirds of the families perceived a great need for more follow-up treatment. Diapers and enemas are the most frequently applied methods (51%), and enema management has increased considerably in recent years. The 51% usage of diapers shows that treatment aiming for so-called social continence was inadequate and may need to be improved. The rates were lower only in patients with low ARM (28%) or perineal fis- tula (17%). In cases of vesical and bladder-neck fistula (62%) and vaginal fistula (83%) the diaper and enema rate was higher.
37.3.3 Degree of Contentment
The degree of contentment of the parents (Table 37.6) depended on one hand on the final outcome, but also on the way the surgeon dealt with and informed the parents and patients. Some parents reported that the surgeon was ignorant regarding basic maternal in- stincts like breast feeding, which was especially im- portant to these families. In 66% the parents received no advice from nutritional specialists, although it is well known how important this issue is, especially in colonic diseases.
Another important issue was the communication of prognosis and diagnosis, which predetermined the expectations in the first place and therefore the later
Table 37.5 Methods of aftercare. Please note that this table does not include surgical follow-up and other professional issues (see Table 37.6)
Diapers 51% Anal tampons 6%
Enemas Enemas (rectal) 51%
Alternative Homeopathy 22%
Microenemas (klysma) 18% Osteopathy 9%
Enemas (Malone) 3% Acupuncture 1%
Medication
Suppository 17% Reflex zone therapy 6%
Laxative medication 19% Something different 23%
Constipating medication 11% Physiotherapy 21%
Bladder medication 18% Diet 16%
Other medication 7% Nothing 7%
Bladder catheter 18% Biofeedback 6%
degree of contentment. That is why we asked the par- ents if and how they were informed about the mal- formation and its consequences. Almost half of the parents received no information (4%) or insufficient information (42%) about the malformation prior to surgery. Regarding the information about functional prognosis, 63% of the respondents answered this question by saying that they felt that they were either not informed (27%) or insufficiently informed (39%).
It was also necessary to consider the psychological strain on the parents in the hospital. It is worse when the surgeon could not imagine what huge psychologi- cal strain the initial weeks in hospital imposed upon the parents. In the long term only very few families (17%) were offered psychological support.
In more than 40% of the cases follow-up was either not offered by the attending surgeon or the follow-up supplied was not satisfactory. That level is too high and can be improved upon. Given that the surgery could also be a financial burden, the rate of referral to a social worker was too low. The initial attending sur- geon should have in mind all the ancilliary services (see Table 37.6) and at least have proposed their use, as the information of further supporting facilities im- proved the degree of contentment of the families.
Concerning how valuable the support given by other parents is, it is surprising that 21% of the par- ents were not offered any support by other families.
For example, the parents were not informed about self-help groups. Our members confirmed that even
when they asked, they did not receive any informa- tion about how to contact us or other parents groups despite the high level of contentment demonstrating the importance of this kind of support.
37.3.4 Continence Function (HAQL)
In the outcome classification of Krickenbeck, three domains are separately evaluated: voluntary bowel movement, soiling and constipation. With the data of our survey we obtain from the simplified definition of
“voluntary bowel movement” according krickenbeck- agreement (Table 37.3), because we see inconsistency in this item. But ist was possible to operationalise the soiling domain with data from the HAQL. The con- stipation domain of the new Krickenbeck classifica- tion works with several assumptions. For instance, that the constipation is treated and that the order of treatment is observed (diet→medication→irrigation).
However, some parents perform colonic irrigation as preventive therapy as well as to manage soiling. This has to be considered, if judgements with this assess- ment method are made.
37.3.4.1 Soiling
Twenty-two percent of parents reported no soiling, but 48% (grade 1) experienced occasional soiling.
Of the 30% of parents whose children had daily soil- ing, 9% reported social problems (grade 3) and 21%
(grade 2) did not have major social problems.
Considering the degree of satisfaction with the treatment by the primary surgeon, we examined whether there was any relationship between conti- nence results and parental satisfaction. Regarding constipation, the majority (80%) were satisfied in cases of no constipation or grade 1. However, in grades 2 and 3 the satisfaction with the first-line surgeon fell to 60%. High contentment was present when there was no soiling (80%), but the soiling grade (grade 1 and 3) were equally distributed (50%) between satisfied and unsatisfied parents. Even the parents in grade 2 with soiling every day but without social problems showed a high rate of contentment (83%). In conclusion, con- tentment with the primary surgeon depended only in part on the continence outcome.
These continence results have to be put in the context of the type of malformation, as even 80% of perineal fistula children had constipation. The major- ity of these were treated by dietary changes or laxa- tives. In children with perineal fistula the outcome for
Table 37.6 Degree of satisfaction with aftercare by different specialists
Follow-up by specialist Not
offered Not
satisfied Satisfied Surgeon/clinic perform-
ing pull-through 10% 32% 58%
Other surgeon 24% 17% 59%
Urologist 46% 15% 39%
Paediatrician 10% 25% 65%
Nursing staff 39% 12% 49%
Physiotherapist 44% 6% 50%
Ergotherapist 69% 3% 28%
Psychological support 83% 7% 10%
Dietician 67% 12% 21%
Stoma therapist 50% 6% 44%
Social worker 92% 4% 4%
Alternative medicine 55% 7% 38%
Other parents 21% 4% 75%
soiling was divided equally between none, occasional or daily soiling. With rectobulbar fistula, most cases needed enemas (grade 3) or at least laxatives (grade 2) because of constipation. Occasional or daily soiling was equally named without major social impact. The cases of so-called vaginal fistula, as well as vestibular fistula and rectovesical fistula usually needed enemas for constipation, and occasional soiling occurred.
In 43% of children with high ARM, bowel action frequency was more than four times a day on two or more days each week. This feature should not be classified as “diarrhoea” (39% of high ARM with thin stool at least occasionally). The risk for pseudo-incon- tinence has to be considered, especially in this specific type of malformation.
37.3.4.2 Constipation
Twenty-four percent of parents thought that there was no constipation, but 8% (or one-third) of them still perform enemas; 6% managed the constipation with diet (grade 1) and 19% with laxative medica- tion (grade 2). The vast majority (41%) used different kinds of enemas (grade 3) to deal with the constipa- tion. In 10% the constipation was not treated in any way.
37.3.5 Impact on the Family
In general, the total impact on the family was similar to that for other chronic conditions (Table 37.7). Only 7% had a significantly better and 14% a significantly worse total score (> mean + 1×SD). The financial im- pact was diverse. Half of the families perceived less financial burden than families of children with other chronic conditions, although there was a significantly worse subgroup (> mean + 2×SD) of 6%.
Looking at single items of the impact-on-family scale, some results were of specific interest: For in- stance, 66% had serious concerns regarding the fu- ture of the child (item number 26; 33% even strongly agreed). More than the half of the parents reported that travelling to hospitals strained them (item num- ber 24) or that they felt like they were living on a roller coaster (item number 27). About 50% did not want further children because of the ill child (item num- ber 15). Thirty percent of families had to give things up because of the child’s illness (item number 20), but the vast majority (95%) still tried to treat the affected child as normal (item number 17). In complex cases in particular there needed to be support for coping
with the situation, as 66% of parents of cloacal cases were worried about the future of their child.
37.4 Discussion
Failure of the initial pull-through surgery for ARM due to preventable reasons is now unacceptable. For instance, Peña et al. [19] and Moss [16] report cases where the neoanus was outside of the muscle com- plex, or where more complex situations (cloaca) were not recognised during the initial treatment. During the primary treatment, even the correct location of the stoma has to be considered. Therefore it is ap- propriate that parents have to be well informed and ask questions about receiving adequate surgery by an experienced surgeon. New or old surgical strate- gies with major modifications compared to the gold standard should only be evaluated under scientific circumstances (i.e. controlled and registered trials).
According to the EUROCAT group [4], the inci- dence of ARM in Germany ranges between 0.02%
(Saxony-Anhalt) and 0.05% (Mainz). Only 30% of the ARM are isolated and not associated with other mal- formations. For instance, the prevalence of VATER and VACTERL of 24% in our group is similar to the 15% overall rate of VACTERL reported in the EU- ROCAT study, although they reported only 5% with cloacas, which is in contrast to our group (15%). We know of several cases were the diagnosis of cloaca was not made by the first-line surgeon, with serious results for the care of the child. Particularly complex cases like cloaca, which can sometimes be detected in prenatal ultrasound screening, should only be han- dled in specialised and experienced centres and not by every surgeon. In these cases social workers should
Table 37.7 Social, financial and psychological impact on the family
Impact-on-family
scale (items) Better Similar* Worse
Financial burden (4) 46% 33% 21%
Daily social impact (15) 15% 72% 13%
Personal strain (5) 5% 79% 16%
Parental coping
problems (3) 12% 71% 17%
Concerns regard- ing siblings (6)
31% 52% 17%
Total impact score (27) 7% 79% 14%
*Mean reference population ± one SD
be involved regularly because at least 60–70% of these cases later receive disability status. In this context we refer to our special subgroup analysis of 28 cloacal cases, which was presented at the 43rd Meeting of the German Society for Pediatric Surgery in Bremen this year [25].
One issue that the recent Krickenbeck conference tried to tackle is the lack of commonly accepted stan- dards for classification [21] procedures and evalua- tion of outcome. Outcome results in particular, such as continence scores and QOL instruments, differ and are used divergingly [12]. Not only are the samples bi- ased because of incomplete cases or results from only one centre, but also the judgement of “good” or “fair”
results differs greatly and is often only evaluated by the physician but not by the patient or legal guardian, which would be more appropriate. The conclusions are therefore of limited value.
Regarding our results, further questions could be addressed. The HAQL as a disease-specific ques- tionnaire reports many more details than are used in pragmatic, new standards for continence evaluation [13]. Of course faecal continence is a pivotal point for QOL. Therefore the patients need to be treated care- fully, including life-long follow-up. There are other issues, however, that can be optimised. Interestingly, two publications using scales that were applied in our study (FaBel: [9]) raise the question: “Does healthcare meet the needs?” Satisfying needs for access to medi- cal healthcare services and adequate surgery could be achieved; however, more psychosocial and paramedi- cal care should be offered according to Hartman and colleagues [9]. Thyen et al. suggested that the fam- ily burden is a direct measure for unmet healthcare needs [30].
According to Trajanovska and Catto-Smith [29]
there are only two QOL indices that also assess faecal incontinence. They missed in their review the QOL scale of Bai et al. [2], where the authors concluded that “somatic and psychological care and long-term follow-up are necessary to improve the quality of life”.
The strong relationship between QOL and continence function is already conditioned through the construc- tion of the QOL scales, where continence function is a major domain in the instrument. Therefore the other domains (social acceptance or psychological problems) should be viewed as separate.
Just recently Funakosi et al. [7] pointed out, that half (6 out of 11) of the children with ARM in the 12- to 16-year age group suffered from significant de- pression. The 7- to 12-year age group with ARM were not so badly affected. Similarly, Pfeiffer et al. [20]
explored by self-report 30 children with ARM (aged
10–16 years) and reported that recurrent, long hos- pitalisations alter personality development towards internalising disorders and low self-esteem. These self-reported results differ in some way from parental views analysed by Ludman and Spitz [14]. They anal- ysed specific coping strategies and reasoned that with adolescents, covert denial or eventual acceptance of the disability continues, but in general the children are able to adapt themselves to society.
It is encouraging that already in 1983, Martinius [15] had considered the prevention of psychological disturbances in children with ARM. He stated that in 4- to 5-year-olds with anal atresia there were symp- toms related to detachment anxiety, bonding, appetite and continence. Nowadays we should revise his con- clusion that psychologically caused incontinence (en- copresis) should be considered in cases where sphinc- ter functioning can be proven. Today it is known that the physiological function of continence and rectal reservoir is a complex interaction that is not only de- fined by external sphincter function, but also by other muscles (levator), receptor density and innervation.
At that time patients were treated by methods other than PSARP, for instance the Rehbein procedure, which itself caused a high level of incontinence. Fur- thermore, the concept of pseudo-incontinence should be considered in such cases.
It should be realised that dirty underwear is not only a warning sign, but also a burden. Occasional soiling may be a satisfying outcome for a malforma- tion with a poor prognosis; nevertheless, it can be still a stressful experience for the affected person.
The expected benefit of biofeedback on children with defecation disorders is not scientifically founded, according to the Cochrane review by Brazelli and Griffith [3]. In accordance with these results, parents only rarely use this method, although it is recom- mended by some specialists. By contrast, according to the Cochrane review of 16 randomised trials with a total of 843 children, the benefit achieved by the com- bination of laxatives and behavioural interventions (e.g. toilet training) was scientifically proven. Stan- dardised care after surgery with primarily non-inva- sive methods could be better established in the view of parents.
The tendency of some surgical experts to aim for
continence through “experimental” surgery is not
appreciated by most parents, because of repeated
experiences with adverse outcomes. Re-do surgery
for patients with injuries caused by accidents should
be separate from the necessities of our patients with
ARM because basic anatomy and requirements are
not comparable. In addition, bowel control is not
only a physical issue but also a question of strategy and psychology. This area of expertise differs from the surgical approach.
Owing to a lack of professional support and no standard guidelines, adults with persisting inconti- nence have to sort out through self study ways to deal with the continence problems. We support Hassink et al. [10] in calling for a stepwise protocol of pro- cedures for earlier and more efficient ways of bowel control. Of course referral to self-help groups for adults should be a standard opportunity for everyone interested. There are some other further problems, like sexuality, which were not examined in our study but are still important. The care for adolescents and adults is more self-determined, and long term follow- up could be improved through routine cooperation between professionals and associations of affected people.
Hassink et al. [11] has already stated that “parents play a crucial role in the life of a child suffering from an anorectal malformation (ARM), since their guid- ance contributes to the degree to which the child learns to cope with his or her disability.” The question can also be raised: Who guides the parents? In order to remove this deficiency, parents are considering the welfare of their children in parental networks.
37.4.1 A Call for Further Collaboration Regrettably at the moment, it is quite exceptional if a paediatric surgeon informs the family of an affected child about the existence of self-help groups (see Ta- ble 37.4). But in our view it is a sign of a confident, competent and supportive surgeon, if he/she does so. This advisory information should just become a standard, with information on social concerns, pos- sible psychological support or the exact description of the situation (exact classification of the deformity, treatment and continence function) given in the phy- sician’s letter. In ARM this self-evident information should be communicated regularly. Whether the fam- ily then becomes a member of a self-help group is an independent decision that every family has to make on its own. Such a communication could obviate an arduous and long search by many families for fellow sufferers. We have learned that even professionals in ARM surgery denied the existence of such initiatives against better knowledge. Why does this anxiety and prejudice exist? Substantial thought patterns are:
1. “My patients do not need aftercare.” Unfortunately, this conceit prevails very often, that the surgeon’s own patients require no self-help group, because
they are already surgically optimally supported.
At the same time it is common to forget that the surgical solution is a central, but not the sole fac- tor in the treatment of congenital deformities.
2. “What can self-help groups do?” In many cases the surgeon has no idea about the sequelae of the malformation or the work of self-help groups and is afraid of the unknown. Both are simply a sign of ignorance.
3. “Only problematic cases are in self-help groups.”
Sometimes the surgeon fears that parents are neg- atively influenced by individual hard cases in self- help groups. Even if this were true, it would be especially desirable to increase the number of suc- cessful and satisfied patients in self-help groups.
After all, a physician should not underestimate the capacity of the majority of his families.
4. “Self-help groups send my patients to another doc- tor.” This statement assumes that self-help groups would direct patient currents. It is possible that this may occur through negative experience in in- dividual cases, if certain discontented parents ask around for advice by other parents. However, this is always a subjective experience and never the of- ficial view of a self-help group. A self-help group or its members would never recommend a single physician, because this is too great a responsibil- ity. The surgeon may think that parents have to take the decision by themselves as it is the life of their children. This impression emerges when the surgeon does not perceive the sorrow of the fam- ily and therefore the appropriate supportive work of self-help groups. The power of self-help groups is overestimated; only the quality of the treatment is decisive.
Wrong personal estimation and lack of knowledge about self-help groups by the surgeon directly af- fect the welfare of patients. Out of this consideration about supposed prejudices, the lack of information and cooperation with concerned families can itself be appraised as a massive quality deficit. Our central wish as a self-help organisation is not competition, but synergy for the welfare of the affected children.
37.4.1.1 Benefit for the Surgeon
Self-help groups are not only advantageous for the
patient and their families, but also for the surgeon. It
is really disappointing for a family when they finally
discover after years of loneliness and troubles that
there are others with similar problems. Then the par-
ents question themselves: Didn’t my surgeon know about the existence of these groups, which means that he was not at the state of the art? Or what was the reason? If surgeons are open to further support possi- bilities, they show that they are not narrow minded. A multidisciplinary approach proves that there is noth- ing to be hidden.
We have found that even between surgeons work- ing in different places there may exist some hostility, which is not good for patients. Some knowledge is not shared. For instance, although we know the conti- nence results for individual centres, every time it has been evaluated slightly differently. A scientific com- parison to determine the best treatment is impossible without a common basis for discussion. A parent or- ganisation may provide a natural exchange forum.
The number of treated patients is not generally a sign of the quality of a surgeon. Sadly, some families must suffer very experimental surgery with disas- trous results. Even the best surgery with insufficient follow-up, could lead to catastrophic outcomes, with pseudo-incontinence [18,23]. Feedback from other cases through the parent organisations could improve the desire for a thorough follow-up, which would in- crease the patient compliance and overall experience of the physician.
Another advantage for the surgeon is to learn specific solutions for a rare disease. For instance, new treatment forms have been introduced by par- ent organisations. The enhanced bowel management programme, including not only medication but also methods like systematic colonic flushing, were first introduced into Germany by parents! We could not find any physician who would introduce it to us, so it was necessary to invite experts from other countries.
Happily, nowadays a few centres already offer this more holistic follow-up.
We are grateful to surgeons if they give patient-fo- cussed advice at our congresses or in our newsletters.
Of course this can work in some way as advertise- ment, but we prefer local experts. By contrast, it is a major misbelief of some cooperative physicians to assume that a self-help group will and can control pa- tients and will support only one doctor. Contentment, quality and exhaustive information are the main fac- tors that influence patients.
Just recently, during the XIIth Colorectal Club meeting in Dublin [26], specialised paediatric sur- geons emphasised that the benefit for both parties can be improved through intensified communication between patients and physicians in these self-help or- ganisations. In this context it should not be forgotten that some of the self-help organisations were estab-
lished by committed surgeons. Perhaps similar people can be identified for those countries without support groups?
37.4.1.2 Benefit for the Patient
It is reassuring when patients and parents come into contact with other families and no longer feel alone in the world with their problem. Children and adults with the same handicap can share intimate concerns, which could never be shared with uninvolved people in the same way. Since ARM is a rare condition, in- dividual solutions are often necessary and only in such groups do concerned parents or patients meet so many different individuals!
The accumulation of different experiences brings a family’s subjective one-sidedness into a more global perspective. To share often very simple tips and quirks with associates cannot be overestimated. It can be facilitating little things like dragging the catheter or modifying the daily routine. In the hurry of a hospital these things cannot be clarified. This information and education results in better aftercare for faecal conti- nence and QOL, with finally a better outcome and greater degree of contentment.
A parent and patient organisation is a reservoir of lively information regarding all issues that could oc- cur in the life of a child who is born with an imper- forate anus.
37.5 Conclusion
Living with an ARM means for patients and their families not only bearing the hardships of one to three operations, but also the situation of patients with ARM is not satisfying concerning the diagno- sis, the treatment and the aftercare. Based on the re- sults of our surveys, study of the literature and our experience gathered from many consulting sessions, we conclude that the care of ARM should be in the hands of experienced specialists. Today there is a lack of information regarding the diagnosis and prognosis for patients. A wrong diagnosis can result in incorrect surgery. Therefore patients should have the possibility of a second opinion and the possibility to share expe- riences. Furthermore, a wrong prognosis can awaken false hope and this could result in bad, inappropriate or no aftercare and, at worst, in unnecessary opera- tions.
There is a need for standard guidelines and their
monitoring, which is also required for aftercare. The
situation of faecal continence after ARM has to be considered in a very dedicated way. Patients with ARM and their families need support! They need qualified help to be continent or to reach social con- tinence. Surgery has to look at the long-term conse- quences, if it is to be successful. Collaboration of all concerned disciplines is mandatory.
The impact of ARM on affected families is compa- rable to that of other chronic conditions. The entire family needs support to cope with the situation with the help of social workers, psychologists and similar specialists. Surgeons should supply the basic informa- tion about the physical problems. ARM require cau- tious handling, since they concern several highly inti- mate areas. This creates trust and prevents additional psychological stress on the patient.
We believe that surgeons should work together with self-help organisations and they should give patients and parents the opportunity to contact other fellow sufferers so that they do not feel alone with their dis- ability. The authority and power of the treating phy- sician has to be applied in a responsible way for the sake of our children, as it decides the child’s and the family’s quality of life – today and in the future.
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