- Email: [email protected]
Bladder and bowel management in physically disabled children
SYMPOSIUM: SPECIAL NEEDS
Bladder and bowel management in physically disabled children
carers. As a paediatrician, it is therefore essential that we support youngsters to achieve full control. Many conditions will affect the bladder and bowel, some neurological and some not. The investigation of these is discussed below, but in the absence of a specific neurological deficit there is no rush to over investigate (Table 1).
Alison Sansome
The bladder Normal bladder training can be initiated in physically disabled children at the same time as peers. Children with learning disability in addition to physical difficulties will be slower to achieve continence. Some children may never achieve reliable continence but we are often surprised at how many children with quite significant difficulty can. Children with physical disability may be hampered by poor mobility. A child with cerebral palsy may not be able to run to the potty when the need arises and being in a wheel chair will slow the undressing process. The children may also find it difficult to communicate their awareness and need. From an early age, non-communicators should be encouraged to sign or use pictorial cards to request toileting. Once on the toilet, stability may be an issue. There are some excellent high sided potties available, almost like a throne, to keep the children in the right position. Failing that, the occupational therapist will find the suitable toilet seat and foot support so that the children can use the toilet without fear of falling either in or off. Star charts and incentive can also be used to encourage progress, just as you would with the non-disabled population from the cognitive age of three and a half years.
Abstract Management of the bladder and bowels in children and young people with physical disability has advanced significantly over recent years. Its importance goes beyond achieving full continence, although that has huge importance to the individuals and families concerned. Management of the bladder now strives to achieve a functional and safe urinary tract. This will protect the kidneys from urinary tract infection and avoid the devastating long term problems of renal failure. The investigation of children known to have neurological disability starts early. Regular review of bladder function is important. Medical management is regularly enhanced by active surgical management. The use of bladder augmentation and Mitrofanoff procedures can significantly improve symptoms and the quality of life in young people. Active management of the urinary tract is coordinated with bowel management for full continence. Regular bowel movement ensures good absorption and healthy growth. There are clear NICE guidelines to treat constipation. The surgical management in children with neurological impairment is also very successful. The Antegrade Continence Enema (ACE) is well tolerated and reliable. It is important to try to actively manage the bladder and bowel in all children with disability. Even children with quite significant levels of physical disability can achieve full continence and quality of life and general health may be improved in all.
Common problems encountered Bladder instability: one of the most common problems encountered in children is bladder instability. The child is frequently wet, with little warning that it is about to happen. In these children we first have to assess fluid intake. This is often less than it should be and needs much encouragement. Children age 2e4 years are recommended to have a daily fluid intake of 800e1000 ml and age 4e8 years are recommended to have 1000e1400 ml. One way of checking the volume passing though is to look at urine output. A good void, which indicates bladder capacity, should be in the region of 30 ml per year of age plus 30 ml. The average three year old should be able to pass 120 ml each void.
Keywords ACE; bladder and bowel management; bladder augmentation; clean intermittent catheterization; Mitrofanoff; physical disability
Introduction Children with disability are just as likely to suffer the common problems associated with the bladder and bowel as their nondisabled peers. This includes enuresis, constipation and daytime wetting. In addition they may suffer bladder and bowel problems associated with their underlying condition. Sometimes the two are difficult to tease apart. Although children with complex difficulty may struggle to achieve continence, it is rare in this day and age to be unable to achieve this. When you ask wheel chair dependent youngsters about the importance of continence, it ranks on a par and often higher than, achieving ambulation. It is socially unacceptable to wet and soil. Lack of continence can be very isolating for young people. For the less able youngsters, achieving continence can also be a huge benefit for parents and
Conditions affecting the bladder and bowel Myelomeningocele (spina bifida) Caudal regression syndrome Sacrococcygeal tumour Spinal tumour Transverse myelitis Cerebral palsy Sacral agenesis Diastematomyelia
Alison Sansome BM BCh MA MRCPCH is Consultant Community Paediatrician in the Cambridge Community Services, Ida Darwin Hospital, Cambridge and Associate Specialist in Paediatric Urology at Addenbrooke’s Hospital, Cambridge, UK. Conflicts of interest: none.
PAEDIATRICS AND CHILD HEALTH 21:10
Table 1
454
Ó 2011 Elsevier Ltd. All rights reserved.
SYMPOSIUM: SPECIAL NEEDS
If the child is not drinking enough, they will have a small capacity bladder and very concentrated urine. The urine is irritant to the bladder wall and this potentiates bladder contraction. It is difficult to toilet train a child who has frequency and urgency of micturition. The first treatment must be a good drinking regimen. If good fluid intake alone is not enough, the use of an anticholinergic may help. In children who are able to take tablets, the modified release preparation is preferable. The medication must always be taken alongside a good fluid regimen. Anticholinergic medication can cause a dry mouth, dry eyes, blurred vision and facial flushing. They can also impair bladder emptying when there is an outflow obstruction or incomplete bladder emptying. An ultrasound scan, with a post micturition residual, will ensure treatment is appropriate (Table 2).
Similarly after known trauma, transverse myelitis or spinal tumour investigation will be prompt. The ultrasound scan is important for ascertaining the anatomy and looking for signs of reflux. It may need to be done at different times to ascertain the emptying of the bladder. Babies are not able to void to command and patience may be required. If the bladder has been seen to empty completely (on US scan) and there are no signs of upper tract dilatation, the children will be watched carefully and scanned on a regular basis. If there is incomplete emptying of the bladder and signs of upper tract dilatation, the child requires immediate antibiotic prophylaxis and further investigation. Video urodynamics will help to assess the pressure within the bladder and the competency of the bladder neck. Children with high pressure in the bladder (above 40 cm H2O during filling) or impaired emptying are moved quickly on to clean intermittent catheterization (CIC). Once they are reliably emptying the bladder on a regular basis, the antibiotic prophylaxis can stop. A child with a bladder that continually leaks with no build up of pressure and a low risk of reflux is deemed to have a ‘safe bladder’. These children do not require early clean intermittent catheterization or antibiotics, but need to be monitored carefully. In the first year, the children tend to be monitored by ultrasound scan on a 4e6 monthly basis. This is done earlier if the child develops a urinary tract infection.
Whilst talking about medication: children with disability may also suffer nocturnal enuresis. This can, on some occasions, be attributed to the fact they may find it difficult to get out of bed to go to the toilet through the night. Some children will need to be hoisted and assisted in their toileting. However the basic nocturnal enuresis management should be followed with clear NICE guidelines available. A good fluid intake during the day is essential. Children can also use the bed wetting alarm and/or take desmopressin medication according to patient choice. The newer ‘Melt’ form of desmopressin is often easier for children who may have difficulty swallowing tablets. Disabled children follow the guidelines as would their peers.
Later investigation: children with safe compliant bladders need regular US scans until toilet training is achieved. If, at that point, there is complete emptying of the bladder on micturition and there have been no urological concerns, children with stable neurology can stop having active imaging. Parents are always asked to report any urinary tract infections or changes in continence and if this happens, screening would resume. In children with ongoing neurological problems but no urological difficulties, active screening with an US scan continues but less frequently (approximately yearly). With neurological difficulty and active urological concerns, the screening remains at regular intervals. Infections should be further investigated with a DMSA renogram. Further video urodynamics may also be indicated
Specific disability With specific disability, the investigations start much earlier stage. There has been a significant change in management of children with known neurological impairment to bladder function. In the past a high proportion of children with spina bifida would go into renal failure in early adult life following repeated urinary infections in small high pressure bladders that left the kidneys scarred and dysfunctional. The emphasis is now on early clean intermittent catheterization and preventative treatment to maintain and preserve kidney and bladder function. This proactive management significantly reduces the likelihood of early renal disease and subsequent renal failure as young adults. The early neurosurgical management of myelomeningocoele also aims for optimal outcome.
Clean intermittent catheterization Clean catheterization is the urethral passage of a catheter to empty the bladder. It has replaced indwelling urethral catheters as a treatment for children with a neuropathic bladder. There are many different catheter types (Figure 1). The old simple catheters that were inserted with KY jelly are still occasionally chosen by parents or children but there is now a huge selection with pre lubricated options and compact varieties. The choice of catheter is determined by user need and preference. Regular complete emptying of the bladder is more physiological and runs a much lower risk of side effects. Indwelling catheters have a high risk of infection and stone formation. Some children need to empty the bladder twice a day, but most require regular emptying, as would be the normal frequency of void for their age. Most school age children can catheterize twoethree hourly as fits in with lessons. Regular emptying will reduce the risk of urine infections and facilitate dryness. The size of catheter
Early Investigation: a baby born with myelomeningocoele or sacrococcygeal tumour will have an early ultrasound scan.
Anticholinergic medication Oxybutynin Tablet or elixir; 2.5e5 mg, two to three times a day (to a max of 5 mg t.d.s.) Modified release tablet; 5 mg once daily (increase slowly to a max of 20 mg) Table 2
PAEDIATRICS AND CHILD HEALTH 21:10
455
Ó 2011 Elsevier Ltd. All rights reserved.
SYMPOSIUM: SPECIAL NEEDS
Figure 2 This teenager with spina bifida has a Mitrofanoff stoma in the umbilicus and ACE stoma on the anterior abdominal wall. The other surgery on the abdomen was VP shunt related.
Figure 1 There are many different types and design of catheter and choice will depend on patient preference.
been used to create the tract for introduction of the catheter into the bladder. Originally, the procedure was performed using the appendix; now in addition to the appendix, segments can be taken from the ureter, bowel or fallopian tube in girls. The conduit is catheterized regularly to empty the bladder. Continence of the conduit is based on a valve effect between the pressures of the bladder pressing the conduit against the abdominal wall. In some patients, with good compliance in CIC, surgeons may also tighten the bladder neck to improve the chance of complete continence
will increase as a child grows. Most small children will start with size 6 or 8 catheter, progressing up to size 10 and then 12 in teenagers. This reflects the diameter of the catheter. Boys will use a longer length catheter for urethral catheterization. Complete continence will depend on a number of factors, not just bladder emptying. The capacity and compliance of the bladder will dictate the likelihood of continence and the competency of the bladder neck is critical. Bladder augmentation Children who have small high pressure bladders are at high risk of that risks sending back flow of urine to the kidneys (vesicoureteric reflux) with recurrent risk of UTI. This is confirmed on US scan on urodynamic studies. These children need to be monitored closely. In some, introduction of an anticholinergic may improve compliance. Most will require surgical bladder augmentation. This treatment improves the bladder capacity and will often improve continence. The augmentation may be taken from the ureter but most often from the bowel. Children need to use clean intermittent catheterization after bladder augmentation as the bladder will not empty completely. Bowel segments in the augmentation tend to produce mucus which needs to be cleared. These children benefit from bladder washouts done via a catheter and oral vitamin C. There is a small long term risk of neoplasia in the bowel segment if it used for bladder augmentation.
Injection therapy: there has been a recent advance in the endoscopic management of bladder instability and incontinence. Botulinum toxin can be injected into multiple sites internally in the bladder wall. The temporary paralysis of the muscle allows increased bladder capacity and improves compliance. The improvement in continence lasts upwards of 6e9 months but repeated injections may be needed on a regular basis. An incompetent or leaky bladder neck may be made slightly more effective by injection of an artificial polymer at the orifice. This can improve the resistance at the sphincter and aid continence. Longer term studies are required to evaluate both of these. It is sometimes difficult to achieve full continence if there is significant impairment to the bladder neck function. In the older boy, with good hand function, there may be consideration of an artificial sphincter. This is not undertaken before some of the pubertal changes and growth has occurred. There needs to be a guarantee that the sphincter will be released regularly each day so the youngster needs both intellectual competency and manual dexterity to do this. Whilst awaiting this, some youngsters may use a sheath catheter draining into a leg bag to achieve dryness. The sheath catheters can also be used to ensure dryness in young men who have significant difficulty with mobility. A young man with cerebral palsy who needs hoisting for toileting may benefit from this when out and about.
Mitrofanoff Some youngsters struggle with CIC. Many children with complex disability who are wheel chair bound find the physical practicalities difficult. They may not be able to catheterize urethrally due to poor dexterity or the inability to balance independently in the sitting position. These children may benefit from a ‘more accessible’ route of catheterization. The Mitrofanoff procedure is an excellent alternative continence mechanism for patients with incontinence due to a variety of different pathologies including neuropathic bladder with or without spina bifida, posterior urethral valves or following spinal or pelvic tumours (Figure 2). The Mitrofanoff procedure involves construction of vesicostomy to the abdominal wall, typically to the umbilicus, that allows for catheterization of the bladder. Many conduits have
PAEDIATRICS AND CHILD HEALTH 21:10
The bowel The management of the bowels although separated in the text, in practice goes hand in hand with the bladder. The issues of getting
456
Ó 2011 Elsevier Ltd. All rights reserved.
SYMPOSIUM: SPECIAL NEEDS
to the toilet and staying on it remain the same. A poor fluid intake will also negatively affect them and the instigation of a good ‘daily drinking regimen’ is, sometimes, all the treatment required. Constipation is a common difficulty. The initial preference for treatment has been the simplest, a combination of lactulose and senna. This should be considered in bowel with no neurological impairment. For children with neuropathic impairment, it would be more acceptable to start with a stimulant laxative e.g. movicol paediatric. The dose for all medication can be very variable and needs to be titrated against response. The important factor is that it is given regularly (Table 3). Anal plugs Many children, despite significant problems, develop a good routine with minimal intervention. They are, however, prone to accidents. These are especially noted at times of physical activity and swimming or hydrotherapy. The embarrassing episodes can be minimized by the use of anal plugs. The plugs only come in two sizes, medium and large. Children with a neuropathic bowel will all need the large size. Antegrade Continence Enema (ACE) Children with a neuropathic bowel may continue to struggle with constipation and faecal incontinence despite intensive
Laxative medication. Recommended doses in accordance with NICE guidelines Oral Macrogols Polyethylene glycol 3350þelectrolytes e paediatric formula C Child under 1 year e 1/2 to 1 sachet daily C Child 1e6 years e 1 sachet daily C Child 6e12 years e 2 sachets daily Osmotic laxatives Lactulose C Child 1 monthe1 year e 2.5 ml twice daily C Child 1e5 years e 2.5 to 10 ml twice daily C Child 5e18 years e 5 to 20 ml twice daily Stimulant laxatives Sodium picosulphate C Child 1 monthe4 years e 2.5 to 10 mg elixir once daily C Child 4e18 years e 2.5 to 20 mg once a day Bisacodyl C Child 2e18 years e 5 to 10 mg once daily Senna syrup (7.5 mg/5 ml) C Child 1 monthe4 years e 2.5 to 10 ml once daily C Child 4e18 years e 2.5 to 20 ml once daily Docusate sodium C Child 6 monthse2 years e 12.5 mg three times a day C Child 2e12 years e 12.5 to 25 mg three times a day C Child 12e18 years e up to 500 mg daily in divided doses Suppository Glycerin suppository Bisacodyl C Child 4e18 years e 5 to 20 mg
Figure 3 ACE stoma showing catheter during daily washout procedure in a teenager with caudal regression syndrome. a ACE stoma site on the abdomen of a teenage girl with caudal regression syndrome. b The catheter is inserted into the stoma ready for the bowel washout. c Flush through of the bowel continues until completely empty.
management with medication. With impaired bowel control, it is sometime easier to manage children when they are slightly constipated. If the stool is normal or soft in consistency, they may have no control at all. However there is a major health implication for these children with chronic constipation with
Table 3
PAEDIATRICS AND CHILD HEALTH 21:10
457
Ó 2011 Elsevier Ltd. All rights reserved.
SYMPOSIUM: SPECIAL NEEDS
outcome desired by the children and young people we look after, as doctors we must also aim to achieve excellent quality of healthcare. The preservation of the renal tract is critical and this can be achieved with intervention to give a compliant safe bladder that empties completely. This will help prevent infections in the kidneys and avoid devastating long term renal failure. The active management of the bowels can also significantly improve the health and quality of life for the youngsters in addition to achieving reliable continence. Children with physical disability should always be encouraged and supported to aspire to complete continence with the confidence and good health it brings. A
poor growth and impaired absorption of food. The children also suffer significant pain and discomfort on a daily basis and the behaviour around toileting may deteriorate as a result. These children may require surgical intervention in the form of an Antegrade Continence Enema stoma. This provides a way of emptying the bowel once a day at a convenient time which will reduce the constipation and soiling episodes (Figure 3). Classically the ACE procedure used the appendix to connect the large bowel to a stoma site fashioned on the abdominal wall. The procedure is often done at the same time as a Mitrofanoff procedure. There needs to be coordination and separation of the stoma sites. Both procedures use the same type of tissues for conduit formation. The children are in hospital for about 10 days for the joint procedure. The enema is given through the ACE stoma initially once a day. There is variation in the enema solutions. It may be a simple flush of saline or may contain a mild stimulant laxative (often bisacodyl) with a flush of saline. The child sits on the toilet for the duration of the enema and the large bowel is literally flushed clean. The enema may take as little as 30 min or as long as 2 h. Most children achieve full continence with this enema procedure, but it does require commitment and compliance to do reliably. The ACE stoma is usually not performed until the children can fully cooperate with the procedure. This may be difficult for parents who wish their children to be continent before starting school. There may be complications with stenosis of the tract but generally the ACE is well tolerated and a long term effective solution. The children can take part in all normal activities, including swimming, following this procedure.
Practice points C
C
C
C
C C
Child Protection
C
As with all children, always think of child abuse in cases of unexplained continence issues. This is important when there has been a sudden change in symptoms. Children with physical disability may be very dependent on carers and can be at higher risk of physical, sexual and emotional abuse.
C
Conclusion
Acknowledgements
The appropriate management of the bladder and bowels of children with physical disability can be very rewarding. We have a much clearer overview of the importance of active intervention of both. Although reliable continence is the
PAEDIATRICS AND CHILD HEALTH 21:10
Active management of the bladder and bowels in physical disability is both for continence and protection of the renal tract. All children with physical disability should be encouraged to achieve full continence. Children may suffer constipation and enuresis just like their peers. Neurological impairment requires early screening and monitoring. Prophylactic antibiotics may be indicated. Clean intermittent catheterization may need to start early. Surgical interventions such as the Mitrofanoff or ACE are well tolerated and can make a significant improvement to quality of life. Child protection. Be aware of unexplained continence issues.
With thanks to Alice and Rachel for assistance. Thanks also to Dr Elaine Lewis and Mr. Martyn Williams for advice.
458
Ó 2011 Elsevier Ltd. All rights reserved.
Bladder and bowel management in physically disabled children
carers. As a paediatrician, it is therefore essential that we support youngsters to achieve full control. Many conditions will affect the bladder and bowel, some neurological and some not. The investigation of these is discussed below, but in the absence of a specific neurological deficit there is no rush to over investigate (Table 1).
Alison Sansome
The bladder Normal bladder training can be initiated in physically disabled children at the same time as peers. Children with learning disability in addition to physical difficulties will be slower to achieve continence. Some children may never achieve reliable continence but we are often surprised at how many children with quite significant difficulty can. Children with physical disability may be hampered by poor mobility. A child with cerebral palsy may not be able to run to the potty when the need arises and being in a wheel chair will slow the undressing process. The children may also find it difficult to communicate their awareness and need. From an early age, non-communicators should be encouraged to sign or use pictorial cards to request toileting. Once on the toilet, stability may be an issue. There are some excellent high sided potties available, almost like a throne, to keep the children in the right position. Failing that, the occupational therapist will find the suitable toilet seat and foot support so that the children can use the toilet without fear of falling either in or off. Star charts and incentive can also be used to encourage progress, just as you would with the non-disabled population from the cognitive age of three and a half years.
Abstract Management of the bladder and bowels in children and young people with physical disability has advanced significantly over recent years. Its importance goes beyond achieving full continence, although that has huge importance to the individuals and families concerned. Management of the bladder now strives to achieve a functional and safe urinary tract. This will protect the kidneys from urinary tract infection and avoid the devastating long term problems of renal failure. The investigation of children known to have neurological disability starts early. Regular review of bladder function is important. Medical management is regularly enhanced by active surgical management. The use of bladder augmentation and Mitrofanoff procedures can significantly improve symptoms and the quality of life in young people. Active management of the urinary tract is coordinated with bowel management for full continence. Regular bowel movement ensures good absorption and healthy growth. There are clear NICE guidelines to treat constipation. The surgical management in children with neurological impairment is also very successful. The Antegrade Continence Enema (ACE) is well tolerated and reliable. It is important to try to actively manage the bladder and bowel in all children with disability. Even children with quite significant levels of physical disability can achieve full continence and quality of life and general health may be improved in all.
Common problems encountered Bladder instability: one of the most common problems encountered in children is bladder instability. The child is frequently wet, with little warning that it is about to happen. In these children we first have to assess fluid intake. This is often less than it should be and needs much encouragement. Children age 2e4 years are recommended to have a daily fluid intake of 800e1000 ml and age 4e8 years are recommended to have 1000e1400 ml. One way of checking the volume passing though is to look at urine output. A good void, which indicates bladder capacity, should be in the region of 30 ml per year of age plus 30 ml. The average three year old should be able to pass 120 ml each void.
Keywords ACE; bladder and bowel management; bladder augmentation; clean intermittent catheterization; Mitrofanoff; physical disability
Introduction Children with disability are just as likely to suffer the common problems associated with the bladder and bowel as their nondisabled peers. This includes enuresis, constipation and daytime wetting. In addition they may suffer bladder and bowel problems associated with their underlying condition. Sometimes the two are difficult to tease apart. Although children with complex difficulty may struggle to achieve continence, it is rare in this day and age to be unable to achieve this. When you ask wheel chair dependent youngsters about the importance of continence, it ranks on a par and often higher than, achieving ambulation. It is socially unacceptable to wet and soil. Lack of continence can be very isolating for young people. For the less able youngsters, achieving continence can also be a huge benefit for parents and
Conditions affecting the bladder and bowel Myelomeningocele (spina bifida) Caudal regression syndrome Sacrococcygeal tumour Spinal tumour Transverse myelitis Cerebral palsy Sacral agenesis Diastematomyelia
Alison Sansome BM BCh MA MRCPCH is Consultant Community Paediatrician in the Cambridge Community Services, Ida Darwin Hospital, Cambridge and Associate Specialist in Paediatric Urology at Addenbrooke’s Hospital, Cambridge, UK. Conflicts of interest: none.
PAEDIATRICS AND CHILD HEALTH 21:10
Table 1
454
Ó 2011 Elsevier Ltd. All rights reserved.
SYMPOSIUM: SPECIAL NEEDS
If the child is not drinking enough, they will have a small capacity bladder and very concentrated urine. The urine is irritant to the bladder wall and this potentiates bladder contraction. It is difficult to toilet train a child who has frequency and urgency of micturition. The first treatment must be a good drinking regimen. If good fluid intake alone is not enough, the use of an anticholinergic may help. In children who are able to take tablets, the modified release preparation is preferable. The medication must always be taken alongside a good fluid regimen. Anticholinergic medication can cause a dry mouth, dry eyes, blurred vision and facial flushing. They can also impair bladder emptying when there is an outflow obstruction or incomplete bladder emptying. An ultrasound scan, with a post micturition residual, will ensure treatment is appropriate (Table 2).
Similarly after known trauma, transverse myelitis or spinal tumour investigation will be prompt. The ultrasound scan is important for ascertaining the anatomy and looking for signs of reflux. It may need to be done at different times to ascertain the emptying of the bladder. Babies are not able to void to command and patience may be required. If the bladder has been seen to empty completely (on US scan) and there are no signs of upper tract dilatation, the children will be watched carefully and scanned on a regular basis. If there is incomplete emptying of the bladder and signs of upper tract dilatation, the child requires immediate antibiotic prophylaxis and further investigation. Video urodynamics will help to assess the pressure within the bladder and the competency of the bladder neck. Children with high pressure in the bladder (above 40 cm H2O during filling) or impaired emptying are moved quickly on to clean intermittent catheterization (CIC). Once they are reliably emptying the bladder on a regular basis, the antibiotic prophylaxis can stop. A child with a bladder that continually leaks with no build up of pressure and a low risk of reflux is deemed to have a ‘safe bladder’. These children do not require early clean intermittent catheterization or antibiotics, but need to be monitored carefully. In the first year, the children tend to be monitored by ultrasound scan on a 4e6 monthly basis. This is done earlier if the child develops a urinary tract infection.
Whilst talking about medication: children with disability may also suffer nocturnal enuresis. This can, on some occasions, be attributed to the fact they may find it difficult to get out of bed to go to the toilet through the night. Some children will need to be hoisted and assisted in their toileting. However the basic nocturnal enuresis management should be followed with clear NICE guidelines available. A good fluid intake during the day is essential. Children can also use the bed wetting alarm and/or take desmopressin medication according to patient choice. The newer ‘Melt’ form of desmopressin is often easier for children who may have difficulty swallowing tablets. Disabled children follow the guidelines as would their peers.
Later investigation: children with safe compliant bladders need regular US scans until toilet training is achieved. If, at that point, there is complete emptying of the bladder on micturition and there have been no urological concerns, children with stable neurology can stop having active imaging. Parents are always asked to report any urinary tract infections or changes in continence and if this happens, screening would resume. In children with ongoing neurological problems but no urological difficulties, active screening with an US scan continues but less frequently (approximately yearly). With neurological difficulty and active urological concerns, the screening remains at regular intervals. Infections should be further investigated with a DMSA renogram. Further video urodynamics may also be indicated
Specific disability With specific disability, the investigations start much earlier stage. There has been a significant change in management of children with known neurological impairment to bladder function. In the past a high proportion of children with spina bifida would go into renal failure in early adult life following repeated urinary infections in small high pressure bladders that left the kidneys scarred and dysfunctional. The emphasis is now on early clean intermittent catheterization and preventative treatment to maintain and preserve kidney and bladder function. This proactive management significantly reduces the likelihood of early renal disease and subsequent renal failure as young adults. The early neurosurgical management of myelomeningocoele also aims for optimal outcome.
Clean intermittent catheterization Clean catheterization is the urethral passage of a catheter to empty the bladder. It has replaced indwelling urethral catheters as a treatment for children with a neuropathic bladder. There are many different catheter types (Figure 1). The old simple catheters that were inserted with KY jelly are still occasionally chosen by parents or children but there is now a huge selection with pre lubricated options and compact varieties. The choice of catheter is determined by user need and preference. Regular complete emptying of the bladder is more physiological and runs a much lower risk of side effects. Indwelling catheters have a high risk of infection and stone formation. Some children need to empty the bladder twice a day, but most require regular emptying, as would be the normal frequency of void for their age. Most school age children can catheterize twoethree hourly as fits in with lessons. Regular emptying will reduce the risk of urine infections and facilitate dryness. The size of catheter
Early Investigation: a baby born with myelomeningocoele or sacrococcygeal tumour will have an early ultrasound scan.
Anticholinergic medication Oxybutynin Tablet or elixir; 2.5e5 mg, two to three times a day (to a max of 5 mg t.d.s.) Modified release tablet; 5 mg once daily (increase slowly to a max of 20 mg) Table 2
PAEDIATRICS AND CHILD HEALTH 21:10
455
Ó 2011 Elsevier Ltd. All rights reserved.
SYMPOSIUM: SPECIAL NEEDS
Figure 2 This teenager with spina bifida has a Mitrofanoff stoma in the umbilicus and ACE stoma on the anterior abdominal wall. The other surgery on the abdomen was VP shunt related.
Figure 1 There are many different types and design of catheter and choice will depend on patient preference.
been used to create the tract for introduction of the catheter into the bladder. Originally, the procedure was performed using the appendix; now in addition to the appendix, segments can be taken from the ureter, bowel or fallopian tube in girls. The conduit is catheterized regularly to empty the bladder. Continence of the conduit is based on a valve effect between the pressures of the bladder pressing the conduit against the abdominal wall. In some patients, with good compliance in CIC, surgeons may also tighten the bladder neck to improve the chance of complete continence
will increase as a child grows. Most small children will start with size 6 or 8 catheter, progressing up to size 10 and then 12 in teenagers. This reflects the diameter of the catheter. Boys will use a longer length catheter for urethral catheterization. Complete continence will depend on a number of factors, not just bladder emptying. The capacity and compliance of the bladder will dictate the likelihood of continence and the competency of the bladder neck is critical. Bladder augmentation Children who have small high pressure bladders are at high risk of that risks sending back flow of urine to the kidneys (vesicoureteric reflux) with recurrent risk of UTI. This is confirmed on US scan on urodynamic studies. These children need to be monitored closely. In some, introduction of an anticholinergic may improve compliance. Most will require surgical bladder augmentation. This treatment improves the bladder capacity and will often improve continence. The augmentation may be taken from the ureter but most often from the bowel. Children need to use clean intermittent catheterization after bladder augmentation as the bladder will not empty completely. Bowel segments in the augmentation tend to produce mucus which needs to be cleared. These children benefit from bladder washouts done via a catheter and oral vitamin C. There is a small long term risk of neoplasia in the bowel segment if it used for bladder augmentation.
Injection therapy: there has been a recent advance in the endoscopic management of bladder instability and incontinence. Botulinum toxin can be injected into multiple sites internally in the bladder wall. The temporary paralysis of the muscle allows increased bladder capacity and improves compliance. The improvement in continence lasts upwards of 6e9 months but repeated injections may be needed on a regular basis. An incompetent or leaky bladder neck may be made slightly more effective by injection of an artificial polymer at the orifice. This can improve the resistance at the sphincter and aid continence. Longer term studies are required to evaluate both of these. It is sometimes difficult to achieve full continence if there is significant impairment to the bladder neck function. In the older boy, with good hand function, there may be consideration of an artificial sphincter. This is not undertaken before some of the pubertal changes and growth has occurred. There needs to be a guarantee that the sphincter will be released regularly each day so the youngster needs both intellectual competency and manual dexterity to do this. Whilst awaiting this, some youngsters may use a sheath catheter draining into a leg bag to achieve dryness. The sheath catheters can also be used to ensure dryness in young men who have significant difficulty with mobility. A young man with cerebral palsy who needs hoisting for toileting may benefit from this when out and about.
Mitrofanoff Some youngsters struggle with CIC. Many children with complex disability who are wheel chair bound find the physical practicalities difficult. They may not be able to catheterize urethrally due to poor dexterity or the inability to balance independently in the sitting position. These children may benefit from a ‘more accessible’ route of catheterization. The Mitrofanoff procedure is an excellent alternative continence mechanism for patients with incontinence due to a variety of different pathologies including neuropathic bladder with or without spina bifida, posterior urethral valves or following spinal or pelvic tumours (Figure 2). The Mitrofanoff procedure involves construction of vesicostomy to the abdominal wall, typically to the umbilicus, that allows for catheterization of the bladder. Many conduits have
PAEDIATRICS AND CHILD HEALTH 21:10
The bowel The management of the bowels although separated in the text, in practice goes hand in hand with the bladder. The issues of getting
456
Ó 2011 Elsevier Ltd. All rights reserved.
SYMPOSIUM: SPECIAL NEEDS
to the toilet and staying on it remain the same. A poor fluid intake will also negatively affect them and the instigation of a good ‘daily drinking regimen’ is, sometimes, all the treatment required. Constipation is a common difficulty. The initial preference for treatment has been the simplest, a combination of lactulose and senna. This should be considered in bowel with no neurological impairment. For children with neuropathic impairment, it would be more acceptable to start with a stimulant laxative e.g. movicol paediatric. The dose for all medication can be very variable and needs to be titrated against response. The important factor is that it is given regularly (Table 3). Anal plugs Many children, despite significant problems, develop a good routine with minimal intervention. They are, however, prone to accidents. These are especially noted at times of physical activity and swimming or hydrotherapy. The embarrassing episodes can be minimized by the use of anal plugs. The plugs only come in two sizes, medium and large. Children with a neuropathic bowel will all need the large size. Antegrade Continence Enema (ACE) Children with a neuropathic bowel may continue to struggle with constipation and faecal incontinence despite intensive
Laxative medication. Recommended doses in accordance with NICE guidelines Oral Macrogols Polyethylene glycol 3350þelectrolytes e paediatric formula C Child under 1 year e 1/2 to 1 sachet daily C Child 1e6 years e 1 sachet daily C Child 6e12 years e 2 sachets daily Osmotic laxatives Lactulose C Child 1 monthe1 year e 2.5 ml twice daily C Child 1e5 years e 2.5 to 10 ml twice daily C Child 5e18 years e 5 to 20 ml twice daily Stimulant laxatives Sodium picosulphate C Child 1 monthe4 years e 2.5 to 10 mg elixir once daily C Child 4e18 years e 2.5 to 20 mg once a day Bisacodyl C Child 2e18 years e 5 to 10 mg once daily Senna syrup (7.5 mg/5 ml) C Child 1 monthe4 years e 2.5 to 10 ml once daily C Child 4e18 years e 2.5 to 20 ml once daily Docusate sodium C Child 6 monthse2 years e 12.5 mg three times a day C Child 2e12 years e 12.5 to 25 mg three times a day C Child 12e18 years e up to 500 mg daily in divided doses Suppository Glycerin suppository Bisacodyl C Child 4e18 years e 5 to 20 mg
Figure 3 ACE stoma showing catheter during daily washout procedure in a teenager with caudal regression syndrome. a ACE stoma site on the abdomen of a teenage girl with caudal regression syndrome. b The catheter is inserted into the stoma ready for the bowel washout. c Flush through of the bowel continues until completely empty.
management with medication. With impaired bowel control, it is sometime easier to manage children when they are slightly constipated. If the stool is normal or soft in consistency, they may have no control at all. However there is a major health implication for these children with chronic constipation with
Table 3
PAEDIATRICS AND CHILD HEALTH 21:10
457
Ó 2011 Elsevier Ltd. All rights reserved.
SYMPOSIUM: SPECIAL NEEDS
outcome desired by the children and young people we look after, as doctors we must also aim to achieve excellent quality of healthcare. The preservation of the renal tract is critical and this can be achieved with intervention to give a compliant safe bladder that empties completely. This will help prevent infections in the kidneys and avoid devastating long term renal failure. The active management of the bowels can also significantly improve the health and quality of life for the youngsters in addition to achieving reliable continence. Children with physical disability should always be encouraged and supported to aspire to complete continence with the confidence and good health it brings. A
poor growth and impaired absorption of food. The children also suffer significant pain and discomfort on a daily basis and the behaviour around toileting may deteriorate as a result. These children may require surgical intervention in the form of an Antegrade Continence Enema stoma. This provides a way of emptying the bowel once a day at a convenient time which will reduce the constipation and soiling episodes (Figure 3). Classically the ACE procedure used the appendix to connect the large bowel to a stoma site fashioned on the abdominal wall. The procedure is often done at the same time as a Mitrofanoff procedure. There needs to be coordination and separation of the stoma sites. Both procedures use the same type of tissues for conduit formation. The children are in hospital for about 10 days for the joint procedure. The enema is given through the ACE stoma initially once a day. There is variation in the enema solutions. It may be a simple flush of saline or may contain a mild stimulant laxative (often bisacodyl) with a flush of saline. The child sits on the toilet for the duration of the enema and the large bowel is literally flushed clean. The enema may take as little as 30 min or as long as 2 h. Most children achieve full continence with this enema procedure, but it does require commitment and compliance to do reliably. The ACE stoma is usually not performed until the children can fully cooperate with the procedure. This may be difficult for parents who wish their children to be continent before starting school. There may be complications with stenosis of the tract but generally the ACE is well tolerated and a long term effective solution. The children can take part in all normal activities, including swimming, following this procedure.
Practice points C
C
C
C
C C
Child Protection
C
As with all children, always think of child abuse in cases of unexplained continence issues. This is important when there has been a sudden change in symptoms. Children with physical disability may be very dependent on carers and can be at higher risk of physical, sexual and emotional abuse.
C
Conclusion
Acknowledgements
The appropriate management of the bladder and bowels of children with physical disability can be very rewarding. We have a much clearer overview of the importance of active intervention of both. Although reliable continence is the
PAEDIATRICS AND CHILD HEALTH 21:10
Active management of the bladder and bowels in physical disability is both for continence and protection of the renal tract. All children with physical disability should be encouraged to achieve full continence. Children may suffer constipation and enuresis just like their peers. Neurological impairment requires early screening and monitoring. Prophylactic antibiotics may be indicated. Clean intermittent catheterization may need to start early. Surgical interventions such as the Mitrofanoff or ACE are well tolerated and can make a significant improvement to quality of life. Child protection. Be aware of unexplained continence issues.
With thanks to Alice and Rachel for assistance. Thanks also to Dr Elaine Lewis and Mr. Martyn Williams for advice.
458
Ó 2011 Elsevier Ltd. All rights reserved.