Are you curious about what sexual problems may be present for patients with an anorectal malformation or Hirschsprung's disease? And would you like to support your patients with these possible (future) problems? This website provides practical tips to integrate the topic of sexual health into current work processes.

What sexual problems are prevalent amongst these patient groups?

Previous research has shown that having an anorectal malformation or Hirschsprung's disease can have a negative impact on psychosexual health [1]. Psychosexual development starts at an early age. It is therefore important to pay attention at an early age to the problems that may arise during this development. The table below summarises the psychosexual problems that emerged from this research [2]. The problems affecting patients with anorectal malformation or Hirschsprung's disease are largely similar and are therefore also discussed together.

A distinction can be made between physical and psychological problems. Physical problems can include sexual dysfunction caused by, for example, a narrow vagina or erectile dysfunction. Examples of psychological problems that may come into play is post-traumatic stress (disorder) possibly caused by invasive treatments (sometimes from childhood on). In addition, dependence on healthcare providers and parents when performing invasive procedures such as colonic irrigation can lead to disturbed sexuality (because the boundary between functional and emotional actions is confusing). Negative body image and shame about farting or wearing continence materials also occur. Whether, and which problems patients experience will depend on the patient, support from the environment such as parents, the severity of the condition, and what problems occur when. Here is more information for parents on the psychosexual problems that can occur during the different stages of sexual development and possible solutions and advice for dealing with these problems.

What are bottlenecks to providing support for sexual problems?

From previous research, it appears that healthcare providers experience the following challenges in supporting patient groups with their potential (future) sexual problems:

  • The patient has been out of the picture for a long time (no follow-up care after age 18 and/or does not show up to appointments).
  • The patient has no fixed point of contact and therefore does not know where to turn (especially after the age of 18).
  • It is unclear who is responsible for supporting patients with sexual problems.
  • Attention to identifying possible problems around sexuality and/or psychological problems is limited.
  • Involved healthcare providers have limited knowledge about the sexual problems affecting the patient groups and how these problems can be addressed.
  • Involved healthcare providers have limited skills with regard to discussing sexuality (inaction).
  • Involved healthcare providers do not know how to deal with the topic of sexuality (inaction).
  • Involved healthcare providers do not know to whom patients can be referred if necessary.
  • A healthcare provider’s lack of time and prioritisation of the topic during a consultation.
  • Involved healthcare providers do not see the topic of sexuality as part of the care package.

How can sexual care be incorporated into the care pathway?

It is important to properly integrate the above advice with the care pathway of patients with an anorectal malformation or Hirschsprung's disease. The care pathway is not the same for all hospitals and/or patients; it depends, among other things, on the severity of the condition and treatment method used. In addition, each hospital has set up the care pathway in its own way. A 'general' care path for patient groups has been mapped out below. In addition, the various healthcare providers involved in the pathway have been mapped and how the patient's psychosexual development can be addressed at different times. The care path can be divided into four phases:

1. Diagnosis

Anorectal malformations and Hirschsprung's disease are congenital anomalies that, in most cases, are diagnosed quite soon after birth. When informing parents about the possible impact of the diagnosis on their child, it is important to also mention the possible (future) psychosexual consequences.

2. Age 0-18 years

Treatment of both conditions should take place in a specialised hospital setting with appropriate expertise. 

Treatment of an anorectal malformation or Hirschsprung's disease may require one or more operations, dilation of the anus, flushing of the bowels and examination under anaesthesia with possible botox injections. This intensive treatment can be stressful for parents and children. It is important to make parents aware of the possible (future) psychosexual consequences of the disease, as well as how the treatment to keep defecation going, and later toilet training, can affect the attachment relationship with their children. As a healthcare provider, you can support parents and children in dealing with the psychosexual consequences of their illness. Tips for this can be found here. If you identify psychosexual problems, you can refer the patient to a mental healthcare psychologist or sexologist. A social worker can offer support when problems arise in the home situation.

The child will remain under the control of the paediatric surgeon until adulthood. Clarify to the child and parents who the treating doctor is (usually the paediatric surgeon) and who they can contact with questions. Some hospitals have a combined outpatient clinic where several doctors check the child together or separately during one appointment. This can involve the paediatric surgeon, paediatrician, urologist, (continence) nurse, neurologist, orthopaedist, physiotherapist, psychologist and a social worker to support the parents. In adolescence, girls also may encounter a gynaecologist. To give the subject of sexuality more attention, it would be good to have a sexologist involved. However, the subject can also be taken up by another healthcare provider, for example a nurse or the paediatric surgeon.

How often a patient comes for consultation depends on the hospital, the severity of the condition and/or the patient. Adolescents often have more difficulty adhering precisely to treatment and everything that comes with it, such as prescriptions from the healthcare provider. They would like to be 'normal' and thus not feel like a patient. Ways should be sought to keep patients in sight at this age. Scheduling an annual consultation helps here, but is not always sufficient. The timely handing out of a card with a link to this website and the contact details of relevant care providers can ensure that the patient still gets to the right place at a later stage. In addition, staying in touch digitally on a structural basis by offering a cyber clinic can be an option.

3. Transition 

Patients usually face long-term and intensive care from birth. To enable appropriate care even after the age of 18, a proper transfer (transition) from paediatrics to adult medicine is of great importance. If care-specific details of patients are insufficiently shared during the transfer, this can have serious consequences for both the physical and psychological health of the patient. Think of problems with bowel, kidney function or sexuality. How transition is done within different hospitals varies. Some hospitals have set up a transition clinic. In a transition clinic, knowledge is shared to avoid unnecessary procedures and admissions. To ensure that patients know who to contact (also after the transition), it is important to indicate to them (and their parents) who is the main clinician/contact person. To identify possible (future) psychosexual problems, it is important to pay attention to the young adult's psychosexual development. Identification is possible by having a questionnaire completed or by engaging in a conversation. The latter can be done by the main practitioner, but also by a trained healthcare provider and/or a sexologist. If there is no transition clinic, it is advised to draw the patient's attention to possible psychosexual problems and to indicate where they can go for questions or when these problems arise.

4. Adults

A study has shown that when patients turn 18, they often drop out of care. This can have several reasons. Many times, there are insufficient medical reasons for patients to remain under control. Sometimes no improvement in existing problems is possible. Due to the lack of counselling, possible problems around sexuality are not raised. It is therefore important to indicate when patients are discharged, how and to whom they can contact in case of any future problems. A card can be given along with contact details and a link to this website. 

[1] van den Hondel D, Sloots CEJ, Bolt JM, Wijnen RMH, de Blaauw I, and IJsselstijn H. Psychosexual well‐being after childhood surgery for anorectal malformation or Hirschsprung's disease. The Journal of Sexual Medicine 2015;12:1616–1625.

[2] Other issues not listed here may also be experienced.