Chapter 7: Health promotion strategies and other clinical issues in spina bifida

A routine approach to reviewing spina bifida helps clinicians detect problems requiring early treatment and referral. In addition, information, such as dietary and other lifestyle information can help promote a sense of health and wellbeing.

Key issues for clinicians

• Attention to general health measures may help prevent significant long term complications of spina bifida.
• Obesity is a major issue in young people and adults with spina bifida and is a significant barrier to independent mobility.
• Self care, including skin care, is an important part of general health for people with spina bifida.
• Peer support organisations can provide important access to resources and support networks.

Early prevention can minimise long term problems
The early detection of changes in clinical condition and of any complications from spina bifida is essential. While monitoring of neurological and urological changes have been highlighted in earlier chapters, there are other measures that general practitioners can take to enhance the quality of life for young people and adults with spina bifida.
General practitioners often diagnose changes early, and are able to refer initial problems to specialist centres.
Some common problems of which GPs need to be aware are listed below.
Obesity in spina bifida
Nutrition plays a vital role in spina bifida in terms of general health, bowel function regularity and the prevention of obesity.
Lack of mobility is a major contributing factor to the development of obesity in spina bifida.
Neurological and orthopaedic problems can decrease mobility, resulting in weight gain. This weight gain can result in a further decrease in mobility, contributing to further weight gain. The loss of mobility may be so severe as to result in the person being confined to a wheelchair.
The GP can play an important role in monitoring weight and intervening early to prevent weight gain.
Promoting exercise, sports and activities
General practitioners can improve the general health of people with spina bifida by promoting the role of physical exercise.
In addition to helping maintain mobility and prevent obesity, sport can be a major opportunity for socialisation, formation of relationships, peer support and meeting prospective partners.
Skin care - pressure sores and neuropathic skin
Skin problems, often related to immobility, can be a continuing source of morbidity. Early treatment is the key to successful outcomes.
Altered sensation from spina bifida can result in neuropathic, atrophic skin with ulcers and chronic infection. Poor vascular supply and abnormal autonomic function are also common in spina bifida and can contribute to skin atrophy.
Lymphoedema, secondary to immobility is common and can result in skin damage and ulceration.
Care should be taken to ensure that shoes fit properly, and that wheelchairs, general aids and aids to transfer patients do not contribute to skin damage.
Patients may benefit from referral to specialist lymphoedema clinics.
Latex allergies
Allergies to latex are more common in people with spina bifida than the general population. Reactions can vary between mild reactions to severe anaphylactic shock.
Care needs to be taken to avoid the use of rubber gloves and any other latex items, such as the balloons in catheters. Some hospitals have latex free operating theatres for procedures for allergic individuals.
Clinicians need to remain alert to this possibility and to refer to specialist clinics for advice if the situation arises.
Self care and hygiene
Cognitive and physical deficits can contribute to poor self care and hygiene. A deterioration in self care may point to worsening underlying medical problems. Patients may need to be taught simple grooming techniques adapted so that they are achievable within the context of any cognitive deficits.
Social development and educational strategies
Peer support is one of the most important ways in which people with spina bifida learn to adopt healthy behaviours. Encouraging patients to join spina bifida associations can provide opportunities for peer support, which can increase self esteem and personal and social relationships (see list of associations in Chapter 9).

Chapter 8: Sexuality and reproductive issues

Managing issues of incontinence inevitably raises issues of sexuality. The complications of spina bifida that affect urinary and bowel function also affect
sexual functioning. Routine review requires regular assessment of sexual issues while taking into account any special needs. This chapter highlights some of the common issues general practitioners need to know when treating people with spina bifida.

Key issues for clinicians

• Spina bifida affects sexual function.
• Fertility is often normal in women.
• Vaginal delivery is the preferred method of delivery. A urologist should be present during a caesarean section, especially if there has been past urological surgery.
• There can be problems with erection and ejaculation in males. Ejaculation difficulties are a significant barrier to conception.
• Treatment is available for males with spina bifida.
• Assisted reproductive technologies may be of help in achieving pregnancies.

Clinicians involved in the management of spina bifida need to create an open, nonjudgmental environment that is favourable to the discussion of issues of sexuality. Dealing with issues of incontinence control inevitably raises questions of sexuality and the clinician needs to be prepared to deal with such important issues.
Self esteem issues of spina bifida impact upon sexuality
The issues of body image and self esteem that arise in spina bifida profoundly influence a person’s sexuality, but even people with profound disabilities are capable of active sex lives. Creating a safe environment for discussing and dealing with such issues helps to foster a sense of personal wellbeing and to promote healthy sexuality.
Creating an open environment for discussing sexuality
General practitioners can greatly improve the quality of life of their patients with spina bifida by providing a supportive environment for the discussion of sexuality.
Providing permission to discuss sexual concerns helps to normalise sex and helps to overcome fears and isolation that patients may have about sex. This is especially important for young people who are in the process of becoming independent from parents and carers, and who might not otherwise have the opportunity to discuss sexual matters.
Taking the time to listen to sexual concerns validates the fact that the clinician is prepared to help deal with sexual issues. While the GP may not have the expertise or resources to deal with complicated issues of sexual function in spina bifida, demonstrating a commitment to listen enables the GP to detect any concerns early, and then refer the issues to a sexual counselling clinic if necessary.
Providing simple suggestions may be all that is necessary in some cases to assist some sexual problems. As feelings of isolation are common in sexual health problems, minor concerns can often become major sources of anxiety. Patients may just require simple information about sexuality, such as what menstruation or a nocturnal emission is, to reassure and normalise their sexual concerns.
Many patients will require intensive therapy for specific sexual problems, and this can be arranged through specialist spina bifida clinics for adults or young people.
Privacy concerns and young people
Some young people may not want to discuss sexual health issues when accompanied by their parents. Clinicians may need to indicate to a young person that they are prepared to discuss sexual health problems in the absence of their parents, although considerable diplomacy and tact may be required with the parents when dealing with this sensitive issue.
As long as clinicians are aware that privacy may be an issue, then strategies for achieving this are likely to evolve.
Effect of spina bifida lesions on sexual function
The effect of spina bifida lesions on sexual function varies widely between patients, and often the best way to assess function is through a neurological opinion. This can give a prognosis of the person’s anatomical and physiological sexual function, which can provide the basis for developing management strategies during subsequent sexual counselling.
Males may have normal sexual function, but are commonly affected to some degree. Satisfactory erections are often possible, but without ejaculation. Other types of sexual dysfunction are also possible. In some cases, circumcision may be considered appropriate.
In males and females, issues of altered genital sensation can affect arousal patterns and sexual function.
Orthopaedic problems, for example, with lower limbs can affect the ability to use certain sexual positions.
All of these physiological and anatomical issues and others need to be taken into account when counselling on sexuality issues.
Safe sex
Safe sex education needs to take place in the early teen years, as precocious puberty is very common in people with spina bifida.

Safe sex education needs to be adapted to the special physical and cognitive needs of the individual with spina bifida.
For example, the use of some types of contraception, such as condoms, requires good hand-eye coordination. Problems with coordination and manual dexterity need to be taken into account when recommending specific types of interventions.
Similarly, the cognitive effects of spina bifida (see Chapter 2) need to be considered when educating about safe sex.
Sexuality, conception and pregnancy issues
Issues for women
Although women with spina bifida often report altered vaginal sensation, normal sexual response is often possible.
Spina bifida generally does not affect fertility in women, and contraception and preconception counselling is therefore paramount to decrease the risk of unplanned pregnancies and neural tube defects.
The course of pregnancy in spina bifida is similar to that in women without spina bifida except for:
o an increased risk of urinary tract infection
o a risk of pressure sores
o an increased rate of lower pelvic pain.
The current recommendation is that pregnant women with spina bifida be encouraged to deliver vaginally as women who have vaginal deliveries have fewer complications, faster recovery times and shorter hospital stays.
Women delivered by caesarean section have a higher rate of complications, and surgery is often complicated if there has been past urological surgery for spina bifida. The presence of pelvic scarring often makes identification of ureters and other structures difficult.

Is there an increased rate of birth abnormalities?
Potential parents will want to know about the possibility of birth defects, especially in view of a mother affected with spina bifida. Apart from the increased risk of neural tube defects, the question of whether there is an increased risk of birth defects is unknown.
Newer fertility techniques such as fertility drugs and in vitro fertilisation techniques are sometimes used in people with spina bifida in conjunction with specialist spina bifida clinics.
Issues for males
Less is known about fertility in men with spina bifida than in women. Undescended testes are more common in men with spina bifida and this can contribute to poor fertility. In addition, repeated catheterisation may result in scarring and past epididymoorchitis infections from repeated urethral instrumentation can also decrease fertility.
However, men with spina bifida can still father children, although they may require the use of assisted reproductive technologies.
The major difficulties associated with conception in men with spina bifida are associated with achieving erection and ejaculation.
Difficulty in achieving an erection is a common problem for men with spina bifida, and while this may be treatable in some men, achieving ejaculation is more difficult.
The lower the level of the lesion, the more likely it is that the male will be able to achieve an erection.
Lack of erections can be treated by using physical techniques, such as vacuum pumps, pharmaceuticals such as sildenafil or prostaglandin injections such as Caverject, and with surgical techniques such as penile prostheses. Issues of cost of treatments become important for patients, especially if they are on a low income or a pension.
Ejaculation in many men with spina bifida does not usually occur during sex. Ejaculation using vibroejaculation and electroejaculation techniques often has low sperm counts, resulting in a reduced conception rate.

Assisted reproductive technologies
Many of the issues surrounding conception in spina bifida are due to mechanical and anatomical difficulties in conceiving, rather than a lack of fertility. Access to, and use of, assisted reproductive technologies may be of benefit to parents with spina bifida.
Preconception counselling
Preconception genetic counselling to decrease the risk of neural tube abnormalities in offspring (see Chapter 2) is generally well accepted by patients and families prior to the young person becoming sexually active.
This can provide a good opportunity for the clinician to demonstrate and emphasise that they are prepared to discuss sexual health issues at any time.
Specialist spina bifida clinics can refer at-risk patients to clinicians experienced in managing pregnancy in women with spina bifida.
Psychological and social issues
Young people with spina bifida and independence
Sexuality is an area where young people with spina bifida often take their first steps toward independence from their parents, family and carers.
Family relationships can be disrupted when a young person begins to become sexually active. Close relationships form between parents, carers and children from many years of intimate contact that is required for the successful management of spina bifida. This relationship is often tested at this time of increasing independence as new boundaries become established.
These issues are further complicated because even when adulthood is achieved, some level of dependence may need to remain. Disharmony, disputes and tense relationships are common, and the GP is in an ideal situation to provide support through this time.
Ending isolation - peer support and finding partners
Young people and adults often feel isolated when affected by spina bifida. Not only do they feel personally isolated due to their disability, but they may have real issues of physical isolation due to difficulty in independent transport which prevents meeting others and prospective friends and partners.
The increasing move towards incorporating disability care into mainstream organisations has increased this isolation for many. In the past, when people with spina bifida may have been educated together, mutual support and sharing of coping strategies was common.
Peer support - spina bifida associations
Many people with spina bifida find that peer support organisations - such as spina bifida associations - are one of the best ways of overcoming this isolation. These organisations facilitate contact between members through regular newsletters and events, thereby providing opportunities for socialising.
Spina bifida associations are more than just social groups- they create a forum where affected people can offer each other mutual support, identify important common issues which then become the basis for community education and political movements. Some patients become very active in these organisations and find participation a very fulfilling part of their lives.

Further resources
Specialist spina bifida clinics can refer to appropriate sexual counselling clinics if the need arises.
Family planning clinics manage contraception issues for people with disabilities, and are often located close to families.