John Henderson is a 42-year-old man with a moderate/mild learning disability and Prader-Willi Syndrome.  He lives alone in a flat with 3 hours of support per day from a care worker. He has a two cats of which is very fond, and has a passion for country and western music. Except when challenged about his diet, or given a direct order he doesn’t agree with, John is a very likeable, agreeable individual.

John has a history of challenging behaviours. John can have significant mood swings. John is resistant to instruction and many forms of authority. He can manifest mood swings into heightened states of anger or happiness.  He can be poorly compliant with instructions and is obsessional about eating.

He has type 2 diabetes that is poorly controlled because of his lack of dietary compliance. (Type 2 diabetes mellitus has been reported in about 25% of adults with Prader-Willi Syndrome; Butler et al., 2002). On average John has a consumption of at least 7000 calories per day.

John arms and face show minor injury due to obsessional picking characteristic with Prader-Willi. This is particularly manifest when John is upset. There has been a frequent history of infected sores as a result of picking existing sores. Additionally his extremities are showing thin shiny skin and oedema.

Two days ago John presented at his GP with his support worker saying he did not feel right. His BP was measured at 170/118. There was a diagnosis of uncontrolled hypertension. He was given a prescription of amlodipine 10 mg per day. It was discovered at this appointment that he had stopped taking his metformin.

As the diabetic nurse specialist within the practice you are meeting with him.

• To assess his understanding of his type 2 diabetes and concordance with his diet and medication
• To assess his understanding of his hypertension and concordance with the new medication
• To assess his health, and to consider if further intervention is necessary

Points to think about

John is a man with many complex needs. The nurse needs to think with which professionals to develop and coordinate care:

• His support worker, and any other significant others
• The community LD nurse
• The GP
• Psychologist
• Cardiologist and cardiac support team in primary and secondary care areas
• Endocrinologist and diabetes support team in primary and secondary care areas

The importance is to engage with a person-centred approach. The first steps must be:

• Take time and effort to develop and maintain the therapeutic relation
• Give extra time for appointments
• Identify clearly what he knows and experiences with his own health.
• Ask John what he wants.
• Address the hypertension as the clinical priority:
  
  • How does it make John feel?
  • Does John understand when and how to take medication? If John is unable to tell time, how else might this be done?
  • Work with John to identify lifestyle factors that may influence hypertension management: explain why he is feeling the way he is, recognise potential areas for change and create a collaborative management plan to assist in reaching his goals, which may prevent secondary complications. This is an important consideration: is there something that interests John that he is limited in engaging with because of his health, e.g. going to a concert, or something involving his cats. This may be a way to address his smoking. It is not realistic to consider his diet can easily be resolved.
  • Consider motivational interviewing
• Be aware that John is resistant to authority, and it is vital that he is able to engage and trust you.

The overwhelming consideration within this is John’s diet and the impact this has on his health and quality of life. The issue of capacity is central in this. For most people with Prader-Willi Syndrome, they do not have the ability to make decisions about when they have eaten enough. The Mental Capacity Act (2005) establishes the principle that an unwise choice in itself is not indicative of a loss of capacity, and that we have right to make unwise choices. Should the same general principle of respect for an adult’s autonomy apply to John?

The argument against respecting autonomy is that people with Prader-Willi Syndrome lack the capacity to make choices about food. If there is a lack of capacity, the process then becomes supporting that person into an environment where access to food is supervised permitting a healthier lifestyle.

Please read: Goldstone, A. P., Holland, A. J., Hauffa, B. F., Hokken-Koelega, A. C., & Tauber, M. (2008, November 1). Recommendations for the diagnosis and management of Prader-Willi Syndrome. Journal of Clinical Endocrinology & Metabolism, 93(11), 4183–4197.

https://academic.oup.com/jcem/article/93/11/4183/2627225. This discussion gives recommendations on the diagnosis and management of Prader-Willi across the lifespan. This gives an effective understanding of Prader-Willi Syndrome. The complex genetics, aetiology, multiple phenotypes, and evolving natural history of Prader-Willi Syndrome means that a multidisciplinary professional, parental, societal, and environmental approach to the management is required with many challenges to reducing morbidity and mortality and improving quality of life.

Reference

Butler, J. V., Whittingdon, J. E., Holland, A. J., Boer, H., Clarke, D., & Webb, T. (2002). Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi Syndrome: A population-based study. Developmental Medicine and Child Neurology, 44(4): 248–255.