For citation purposes: Areskoug-Josefsson K. Muscular dystrophy and sexual health. OA Musculoskeletal Medicine 2013 Sep 01;1(2):17.

Review

 
Management

Muscular dystrophy and sexual health

K Areskoug-Josefsson*
 

Authors affiliations

Samrehab, Värnamo Sjukhus, Värnamo, Sweden

*Corresponding author Email: Kristina.areskoug@telia.com

Abstract

Introduction

Muscular dystrophy is a group of muscular disorders, which are hereditary with progressive muscular weakness, giving symptoms such as pain, fatigue, physical and psychological impairment. The definition of good sexual health, the individual's right to good sexual health and the relation between sexual health and general health lead to the question of how good sexual health is enabled by health professionals for persons with severe chronic disease, such as muscular dystrophy.

The aim of this review was to explore the existing literature concerning muscular dystrophy and sexual health focusing on patient information and support by health care professionals.

Materials and methods

A literature search of PubMed and PsycInfo was done for ‘muscular dystrophy and sexual’, ‘muscular dystrophy and sexuality’. The articles that were written in English and concerned humans were included. The literature review included both quantitative and qualitative studies and covered journals, research papers, conference proceedings and theses.

Results

Only five articles were found to suit the aim of this study. Through a search of referencelists from the found articles, another three articles were found. Among those articles, only three had high relevance; the other five were too old. The three articles that had high relevance had different scopes and methodology. One study compared levels of hypogonadism and erectile dysfunction (case-control study), another studied adult life with muscular dystrophy (cross-sectional study) and the third explored reproductive outcome and family planning after genetic counselling (combining interviews and questionnaires).

Discussion

The existing literature is scarce and there is a need for further research concerning sexual health and muscular dystrophy in order to explore how persons with muscular dystrophy experience their sexual health.

Conclusion

It is necessary to develop clinical advice for how health professionals working with persons who have muscular dystrophy can support the patient‘s sexual health and give relevant information to patients/partners/relatives. Despite the lack of knowledge concerning sexual health and muscular dystrophy, models for communication and openness about sexual health among health professionals could probably improve care for this group of patients.

Introduction

Sexual health is defined by the World Health Organization as: ‘a state of physical, mental and social well-being in relation to sexuality. It requires a positive and respectful approach to sexuality and sexual relationships, as well as the possibility of having pleasurable and safe sexual experiences, free of coercion, discrimination and violence’[1]. According to this definition of sexual health, it should be seen in a broad perspective where the individual’s right to good sexual health is an important part. Sexual wellbeing is an integrated part of sexual health, and sexual wellbeing is related to physical and psychological health, as well as level of sexual activity[2]. The definition of good sexual health, the individual’s right to good sexual health and the relation between sexual health and general health lead to the question of how good sexual health is enabled by health professionals, for persons with severe chronic disease, such as muscular dystrophy.

Muscular dystrophy is a group of muscular disorders, which are hereditary with progressive muscular weakness. The prevalence of muscular dystrophy varies in different parts of the world but, as an example, the incidence is one in 3500 newborn males in Western Europe[3]. Some of the muscular dystrophies are congenital, and they often have a worse prognosis than other muscular dystrophies that develop in adults[3]. Despite research efforts there is no cure for muscular dystrophy[4]. Characteristic symptoms of muscular dystrophy are loss of muscle mass with subsequent muscular weakness, not due to other disease or trauma. Muscular dystrophy leads to the breakdown of muscular cells and replacement with connective tissue or fat[3]. If the muscle cells are replaced by fat, the shape of the muscles can have normal proportions even if the muscle capacityis severely reduced. The disease can also include endocrine disorders, cataracts and intellectual impairment. It often gives symptoms such as severe pain, fatigue, and physical and psychological disability[4], which affect patients’ whole life situation, and quality of life moreover decreases with disease duration5. The most common muscular dystrophies are myotonic dystrophy, fascioscapulohumeral muscular dystrophy, Becker’s muscular dystrophy and Duchenne muscular dystrophy[3]. Becker‘s muscular dystrophyaffects boys aged 5-15 years. Duchenne muscular dystrophy also affects males, but has an earlier start, often at the age of 2-3 years. Duchenne muscular dystrophy has a worse progress,despite the progress in management of the disease, the patients rarely become older than 25 years of age[6]. It is a need to change the paradigm from thinking of Duchenne muscular dystrophy as a disease in childhood and acknowledge the adulthood of patients with Duchenne muscular dystrophy and in order to do so issues of sexual health needs to be addressed[7]. As muscular dystrophy can develop at different stages of life, it might affect sexual health in different ways. Sexual health can be affected negatively in a biological, psychological and social perspective by muscular dystrophy, for example, by a changed body image, impotence, chronic pain, fatigue, risk for depression, fear of not finding a partner and worries about not being sexually desirable[8,9,10,11,12,13]. Those with childhood onset of muscular dystrophy are at a higher risk to be single as adults than those developing the disease as adults14. However, there is limited research onhow muscular dystrophy affects sexual health. Health care professionals are often reluctant to incorporate issues concerning sexual health in the care of chronically ill patients, due to reasons such as embarrassment, lack of skills and knowledge, fear of offending the patient and beliefs that the patients are too ill[9]. Persons with disabilities often experience dissatisfaction with the support from health care professionals concerning sexual health[9,15]. It is reasonable to believe that this is true concerning health professionals working with persons with muscular dystrophy as well. Despite this, adolescents with physical disabilities often depend on professionals for sex education[16].

The aim of this review was to explore the existing literature concerning muscular dystrophy and sexual health focusing on patient information and support by health care professionals.

Materials and methods

A literature search of PubMed and PsycInfo was done for ‘muscular dystrophy and sexual’, ‘muscular dystrophy and sexuality’. The articles that were written in English and concerned humans were included. The literature review included both quantitative and qualitative studies and covered journals, research papers, conference proceedings and theses. The bibliographies of relevant publications were checked to identify additional studies. The search was carried out in June 2013.

The identified studies were assessed using the models for critical assessment of studies recommended by the Swedish Council on Technology Assessment in Health Care. The author made an initial screening to rate the relevance of the articles included. Potentially relevant articles were reviewed independently by the author and thereafter assessed for quality and relevance. To achieve high relevance, the study should be published in the last 15 years and relate to patients with muscular dystrophy and their life, including sexual health.

Results

The literature search of PubMed and Psycinfo for ‘muscular dystrophy and sexual/sexuality/intimate relationships’ gaveeight articles where the abstracts seemed to be covering the aim of this study (20130615). After reading the full text, only five articles were found to suit the aim of this study. Searches for ‘Myotonic dystrophyand sexual/sexuality’, muscular dystrophy,‘Duchenne muscular dystrophy and sexual/sexuality’, ‘Fascioscapulohumeral muscular dystrophy and sexual/sexuality’ and ‘Becker’s muscular dystrophy and sexual/sexuality’ gave no further articles. The found articles were from 1977 to 2011 and were limited to refereed journal articles in English. Through a search of referencelists from the found articles, another three articles were found. Among those articles, only three had high relevance; the other five were too old. Three of the five older articles were also limited in relevance since they were not specific to muscular dystrophy but described population studies that had included one or more patients with muscular dystrophy. Both of the remaining older articles focused on male reproductive sexual health. The three articles that had high relevance had different scopes and methodology. One study compared levels of hypogonadism and erectile dysfunction (case-control study[17]), another studied adult life with muscular dystrophy (cross-sectional study[18])and the third explored reproductive outcome and family planning after genetic counselling (combining interviews and questionnaires[19]). Due to the small amount of studies included and the diverse methods used, it was not relevant to perform a uniform quality test.

This is a very small number of research articles for a disease that develops among infants, children and adults, men as well as women. Due to the small amount of research material, an additional search of the internet was made using Google to find networks and associations for persons with muscular dystrophy. This search gave online sites for patients and relatives, which were mainly addressed to relatives, with very limited information about sexual health for persons with muscular dystrophy.

Discussion

There is a very limited research on sexual health in persons with muscular dystrophy, both concerning symptoms that might decrease sexual health as well as solutions for improving sexual health. One reason might be the shortened lifespan in some of the muscular dystrophies. However, the lifespan has increased for boys with Duchenne muscular dystrophy, and the quality of life of persons with Duchenne muscular dystrophy is good in all areas except sexual health[18]. In this study the persons with Duchenne muscular dystrophy were described as being ‘in desperate need of a lovelife’. The conclusion of the study was that it is important to give persons with Duchenne muscular dystrophy the possibility to develop from boy to man with competence in all areas of life, including sexuality.

As early as in 1989, another study acknowledged the importance of including intimate/sexual relationships in the care of persons with Duchenne muscular dystrophy[20] and, in a study concerning reproductive sexual health, both emotional and sexual function are brought up as factors influencing family planning decisions, as well as considerations about the heredity of muscular dystrophy19. The recommendations in earlier research werecounselling and support groups in order to improve sexual relations for this group of patients[20]. For the other major muscular dystrophy diseases, there was no published research concerning sexuality or sexual health, showing a need for future research in the field. There is, today, no description of how common sexual health problems are in persons with muscular dystrophy or what problems they experience as major problems.Despite the lack of descriptions of the way in which sexual health might be affected by different muscular dystrophies, there are symptoms of the diseases that might affect sexual health (Table 1) and the impact on sexual health is probably diverse, including physical, psychological and social issues.

Table 1

Symptoms of muscular dystrophy with possible negative effects on sexual health

The age at onset of the disease is also important, since persons developing muscular dystrophy as adults might have developed their sexual identity and their sexual problems might therefore have a very different character from those people whose disease emerges when they areadolescents, children or infants. When becoming ill as an adult, there might be a need to change a sexual role, from active to more passive, in order to perform sexual activities. For persons who develop the disease early in life, the issue is more about developing a sexual identity and finding a way to achieve a satisfying love life as adults.Here the support of relatives is important, especially since many persons with muscular dystrophy often need a great deal of assistance by their relatives in their daily living. A majority of adolescents report dissatisfaction with their body image, and it is likely that adolescents with physical disabilities are in this group[21]. The support of relatives isessential for children and youths in order to develop a good sexual health, since parents play an important role in a person’s development towards independence in all areas of life, including sexuality[22,23]. Areas of importance are, for example, that the family acknowledges sex as something positive, supports sexual feelings, gives possibilities for healthy sexual play with peers (flirting) and confirms that sexuality is not associated with guilt and shame.

It is necessary to see sexual health from a gender perspective, since there are differences in how sexual health problems among adults with muscular dystrophyare experienced; women experience the physical weakness as the greatest hindrance in their sexual life and erectile dysfunction is the most common difficulty for men[17,24]. Neither has received much attention in clinical practice or research. There are also differences in perceived sexual roles that might increase the burden of muscular dystrophy, such as that ‘men should be muscular and strong’.

There is research on physical and psychological disabilities in general being associated to sexual health, but this is unfortunately, probably, not sufficient for persons with muscular dystrophy due to their complex range of symptoms,including physical and psychological disability, pain and fatigue. However, specific therapy suggestions concerning single symptoms might be useful for the individual patient, for example exercise therapy to improve mobility and thereby indirectly improve sexual health or interventions directly improving sexual health such as medical treatment of erectile dysfunction. Health professionals often hesitate to communicate about sexual health with their patients, but it is still essential that they have good skills in this field[9,25]. Knowledge and openness are the basis for good communication between health professional and patient about sexual health issues, even if health professionals can experience barriers in approaching the subject. Common barriers arethe sensitivity of the subject; the health professionals being unsure as to how to bring up the subject, being unsure of how they can support persons with muscular dystrophy who experience sexual health problems and believing that someone else in the health care team is responsible for discussing sexual health with patients[26,27,28,29]. The patients themselves might be reluctant to initiate communication about sexual health problems, especially if they believe that the health professionals are unavailable to offer any support or if they believe that they shouldn't be sexually active due to their disability[29,30,31]. If the patient has cognitive impairment, this might increase the barrier towards communicating about sexual health and a way to overcome this barrier is to see the individual behind the disability and to acknowledge the individual’s right to a sexual life[32,33]. However, there is a greater acceptance of discussing sexual health among health professionals than among family care givers, which can limit communication for patients where family care givers are present in the clinical meeting[34]. The heredity of muscular dystrophies might increase the difficulties of encouraging and discussing sexual health with this group of patients. If sexual activity is only seen from a reproductive perspective, heredity might be a difficult issue to approach, both for the patient and health professionals. However, sexual health deserves a broader perspective including desire, joy and satisfaction. It is therefore important to explore how caregivers and relatives give support and how their attitudes are towards supporting sexual health in persons with muscular dystrophy.Even if the perspective is to improve sexual health in order to ensure sexual pleasure and satisfaction, there is probably a lack of support in this field as well. There has been a change of paradigm in sexual therapy, from a focus on sexual function to a focus on sexual satisfaction, which should be acknowledged by health professionals[2]. Unfortunately, this change seems to be delayed for persons with disabilities, where the main focus still is on reproduction and sexual function[35]. The individual’s choice might be a problem of how support concerning sexual health is ensured for persons in need of advice.For example, it should be the patient’s choice how they wish to use their reduced muscular capacity, but it is doubtful whether patients gain understanding from health care professionals if they choose to use their energy on sexual activities instead of other activities of daily living. It should be acceptable to choose to get assistance with dressing in order to save energy for sexual life. The same choice should be given concerning medication; if there are side effects that might affect the sexual heath of the patients, they should be informed in order to be able to make their own choice of what is most important to them as individuals. It would be of interest to further study how health professionals address this issue. A keyword in improving sexual health and making an informed decision is information. However, considering the scarce research publications, information is probably lacking for this group of patients. It should be natural that an individual is allowed to make his or her own decisions in this field, instead of somebody else making the decision from the perspectiveof ahealth care professional or a relative.Even with the limited research available concerning sexual health in persons with muscular dystrophy, the existing information should be presented to the patients and their eventual partners. An example of information could be explaining the relation between pain and sexual function and/or sexual desire.

To improve sexual health in persons with muscular dystrophy, research concerning other chronic diseases and sexual health might be useful in clinical practice, since research concerning muscular dystrophy and sexual health is scarce. Decreasing symptoms of chronic disease, such as pain and fatigue,and increasing functional ability have been described to improve sexual health in persons with other chronic muscular diseases.Partner communication, cognitive therapy and physiotherapy may also be beneficial[36,37]. Treatment of erectile dysfunction can improve quality of life and self-esteem and can be treated with medication or cognitive behavioural therapy[39].

A basis for improving sexual health for persons with muscular dystrophy could be to use the Recognition Model[26]. The basis of this model is to acknowledge the person with a disability as a sexual person with sexual needs and the right to good sexual health. This is assured by all health care professionals taking their part in improving sexual health for the patient, based on their professional knowledge. As an example, the physiotherapist can be supportive in reducing pain and increasing physical function and the occupational therapist can assist in planning daily activities.

Another useful model is the PLISSIT (permission, limited information, specific suggestions, intensive therapy), a step-wise model for communication concerning sexual health[39]. The PLISSIT can be used by different professionals to determine the appropriate level of intervention[40].

Conclusion

There is a need for further research concerning sexual health and muscular dystrophy in order to:

• Explore how persons with muscular dystrophy experience their sexual health andthe importance of sexual health in their lives, in order to improve care for this group of patients.

• Explore the type of support needed from health care professionals in order to improve sexual health for persons with muscular dystrophy.

Relevance to clinical practice

It is necessary to develop clinical advice for how health professionals working with personswith muscular dystrophycan support the patient‘s sexual health and give relevant information to patients/partners/relatives. Despite the lack of knowledge concerning sexual health and muscular dystrophy, models for communication and openness about sexual health among health professionals could probably be useful and improve care for this group of patients.

Authors contribution

KAJ was responsible for the study design, data collection and analysis, and contributed to the manuscript preparation.

Competing interests

None declared.

Conflict of interests

None declared.

A.M.E

All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.

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Symptoms of muscular dystrophy with possible negative effects on sexual health

Type of muscular dystrophy Symptom Possible negative effects on sexual health
Myotonic dystrophy Difficulties closing the mouth Difficulties in kissing and oral sex
  Delayed muscular relaxation, feeling of muscular stiffness Difficulties with bodily positions during sexual activities
  Testicular atrophy Negative body image
  Pregnancy complications Affect sexual and reproductive functions
  Unclear speech Difficulties communicating with potential partners
Mental symptoms, such as apathy and inactivity Difficulties finding a partner and performing sexual activities
Fascioscapulohumeral muscular dystrophy Difficulties with mouth movements Difficulties in kissing, performing oral sex
Difficulties lifting the arms Difficulties with hugging, caressing
  Hearing impairment Difficulties communicating with partner/potential partner
Becker’s muscular dystrophy and Duchenne muscular dystrophy Decreased lower limb muscles Functional disability, negative body image