The nursing management of Huntington’s disease

Nursing people affected by Huntington’s disease offers us many challenges. It is a complex illness. People suffering from it will have a difficult mixture of profound physical disability and mental health problems. The progressive nature of the illness means that a person’s needs have to be constantly re-evaluated and assessed.

Unlike many illnesses the treatment of symptoms is often a question of trial and error, however, good symptom control is possible and the nurse’s role is to evaluate the effectiveness of any treatment. A holistic approach is essential, it is important to keep in mind the hereditary nature of the illness as this does impact on the wider family and their perception of situations.

The physical manifestations of HD cause many challenges to the management of a patient. However, it is essential to look at these objectively and problem solve. The following headings identify some of the challenges that the illness presents:

Mobility

With Huntington’s disease people can remain mobile for some time. Assessment by a Physiotherapist and Occupational Therapist can help enable this. As the disease progresses, mobility becomes more impaired. It can be difficult during the middle stages of the illness to balance the risk of falling against the desire to remain independent. It is important to remember that people with Huntington’s disease will develop a distinctive gait that to the onlooker may appear unsafe but left to their own devices they can usually mobilise reasonably safely. Ensuring the environment around them is padded and has no sharp edges, as well as providing grab rails, may maintain mobility for some time. Eventually with the progression of the illness people will become wheelchair bound. Again accurate assessment for the appropriate wheelchair and reassessment as the disease progresses is essential.

One of the most significant requirements of a person with HD as the disease progresses is an appropriate armchair. Because of the choreic movements, seating someone safely can be problematic. There are a number of different companies that produce specialist chairs – they are an expensive purchase so it is essential to trial different ones and seek the advice of an Occupational Therapist. It is also important to note that as the patient’s illness progresses reassessment is essential.

Due to the movements that are associated with the illness, as well as the likely hood of reduced mobility, and weight loss, it is essential that a risk assessment is carried out on a frequent basis and appropriate preventative measures are put in place to avoid the development of pressure sores.

Usually in the latter stages of the disease people’s involuntary movements are reduced, which enable them to be lifted, using a hoist. However, occasionally people still present with severe involuntary movement, which can create difficulties when assessing lifting, and handling problems. The involvement of the Occupational Therapist is essential in obtaining appropriate equipment.

Washing and dressing

Maintaining personal hygiene for someone with Huntington’s disease can become increasingly difficult as the illness progresses. The cognitive difficulties faced by someone with HD, coupled with the difficulty in actually performing tasks due to the disruption to voluntary movements and the presence of involuntary movements, can often make the person with HD unwilling to maintain their own hygiene needs. Sometimes they are willing to allow you to assist, other times they may refuse. It is important to avoid confrontation, as this will not solve anything. The establishment of an agreed routine at a time that the patient is at his best will achieve much better results. It is important to create the right environment, which is warm, calm and structured, as the person with HD will be feeling very vulnerable and insecure. A full explanation of what you are doing and why will prevent any misunderstandings.

Oral hygiene is an essential part of the care of someone with HD. Ensuring that the mouth is empty following meals and prior to bed as well as ensuring that it remains clean and moist will prevent the development of oral thrush and reduce risks from aspirating food which is held in the mouth. Ensuring teeth are cleaned regularly and that the person has access to a dentist regularly are also really important.

Communication

The nurse must always take responsibility for making communication with someone affected by HD as easy as possible. This can help reduce some of the frustration associated with poor communication. Even during the earlier stages of HD communication can be affected, this may present as a slowness in response or a rapid pouring out of words. Early referral to a Speech Therapist is essential, as is the regular reassessment of the individual as the disease progresses. As the speech deteriorates sadly a lot of the clues that we would normally use to detect non-verbal communication are also interfered with due to the movements associated with the illness. Communicating in a calm environment, allowing plenty of time for a response and asking simple questions that require one-word answers can help enormously. It is essential to obtain as much information about the patient as possible from family members if a patient comes into your care, once communication has deteriorated, in order to facilitate easier communication with the individual.

Elimination

Even during the earlier stages of the illness people may present as being incontinent. This can be due to the reduction in mobility, or a difficulty in removing clothes in time due to involuntary movements. Ensuring a close proximity to the toilet and appropriate clothing can help reduce the risk of incontinence. The lack of drive and motivation associated with the cognitive aspect of the illness can sometimes mean that people can respond to gentle reminders to go to the toilet. If someone becomes incontinent, it is important not to assume that it is the next stage of the illness until all the above have been investigated and also it has been established that there is no underlying cause such as a urinary tract infection or bowel infection. Once someone has become incontinent the sensitive handling of changing him or her as necessary is essential. The medication that people with HD are prescribed can often lead to the person developing constipation. This obviously needs to be monitored and addressed appropriately.

Behavioural difficulties

People with Huntington’s disease are often misunderstood. Their often loud and difficult to understand speech, coupled with the involuntary movements, can present a frightening picture to someone who is not familiar with the illness. The person affected by HD can become angry and frustrated by the demands placed upon them. It is difficult as a caregiver not to take this personally but you must always remember it is the illness not the person that is creating the difficulty. An important part of the care that you offer is to look carefully at the individual behaviour and try to establish a cause.

Some of the possible trigger factors include:

 

  • Communication – not being understood
  • Communication – not understanding
  • Frustration at deteriorating ability
  • Chronic/acute pain
  • Hunger
  • Over tiredness/ over sedation
  • Over stimulation – noise
  • Perceived threat
  • Learnt behaviour
  • Pre – morbid personality
  • Over heating – personal and environmental
  • Miss-interpretation by other people
  • Inconsistent limit setting
  • Verbal and none verbal presentation of other people

Factors that relate to the cognitive aspect of HD that influence behaviour include:

 

  • Slower thinking
  • Rigidity of thinking
  • Difficulty in organising actions
  • Need for routine and consistency
  • Neurological lack of self awareness
  • Getting “stuck”
  • Poor impulse control
  • Poor judgement
  • Difficulty in waiting
  • Mistaking the movement disorder for misbehaviour

 

It is essential to create strategies to assist you in the management of some of the difficulties that you are presented with. It is also important that you work closely with your colleagues so you have an opportunity to discuss your concerns and frustrations. This will enable to continue working with the individual who is affected by the illness.

Some suggested strategies for the management of challenging behaviour include:

  • Work towards creating a calm, stress free environment
  • Allow extra time for comprehension, response and completion of tasks
  • Use the need for routine and consistency to your own advantage
  • Encourage independence – offer assistance but don’t take over
  • Avoid confrontation by distracting attention
  • De-escalation techniques
  • Encourage choices and alternatives
  • Allow considered risk taking
  • Avoid power struggles – back off!
  • Be specific when giving time limits – keep your promises
  • Positive re-enforcement – avoid criticism and praise achievements
  • Invest your time
  • Be self aware – verbal and none verbal communication
  • Know your limits and take care of yourself
  • Relaxation techniques
  • Diversional activities
  • Medication

It is much easier to see and manage the movement disorder than it is to see how difficulty in thinking can affect behaviour. These symptoms of the illness present us with a challenge but by understanding how the illness affects the brain, it is a challenge we can often overcome.

Recreation and relationships

It is important to remember the need for continued stimulation. People with Huntington’s disease still have hobbies and interests and it is essential that they can pursue these. It is the Nurse’s role to facilitate this. The involvement of an Activity Co-ordinator can help assist with this. It is important to plan activities around the individual’s interests and to take into consideration their shorter concentration span and the tendency to fatigue.

It is important to recognise that people with Huntington’s disease will still have sexual needs and creating an environment that will enable them to express those needs is important.

Nursing someone with HD involves the care of the whole family. Family caregivers are often exhausted by the time a person goes into full time care and they may be left feeling guilty that they have not maintained that person at home. It is also essential to remember that often the family members are themselves at risk of developing the illness and seeing their loved one progressing through the illness is a mirror image of what could happen to them. That presents us with a real challenge to ensure the best possible care for both the individual affected by HD and their family.

End of life issues

The progressive nature of Huntington’s disease and the communication and cognitive impairment that people face later on in the illness mean that it is essential that some consideration early in the illness is given to what the person affected by Huntington’s disease wishes when it comes to some of the more difficult decisions. Clearly this is a very sensitive area that should be approached with care but if a patients wishes are recorded when they are able to express them it makes the decision making easier when the need to do so becomes necessary.

Areas that need considering include:

  • Care at home or as an inpatient
  • Gastrostomy feeding
  • Life sustaining emergency procedures
  • Use of antibiotics to treat infections
  • Guardianship, advanced directives
  • Power of attorney
  • Brain donation for research

Conclusion

Nursing people with Huntington’s disease presents us with many challenges. The complexity and diversity of the illness and the way it affects individuals is reflected in the diversity of symptoms. It is essential that we look beyond the illness to the person themselves and offer the best quality of care.

Cath Stanley – Chief Executive, Huntington’s Disease Association

For more information please visit the HDA website http://hda.org.uk/