Parkinson’s disease, multiple sclerosis and motor neurone disease

CHAPTER 16 Parkinson’s disease, multiple sclerosis and motor neurone disease



Robin Ray, Laraine McAnally



LEARNING OBJECTIVES


When you have completed this chapter you will be able to:


• describe the symptoms of Parkinson’s disease (PD), multiple sclerosis (MS) and motor neurone disease (MND) and some of the treatments used to manage these diseases

• discuss the possible impact each disease may have on the person living with PD, MS or MND

• discuss the possible challenges faced by family and/or friends in providing in-home care for people living with PD, MS or MND

• explain the importance of assessing the individual needs and providing multidisciplinary approach to supportive care

• discuss relevant nursing strategies that would enable people and their carers to live with their disease.


KEY WORDS


degenerative


mobility


multidisciplinary team


neurological


supportive care



INTRODUCTION


The three neurological diseases featured in this chapter—Parkinson’s disease (PD), multiple sclerosis (MS) and motor neurone disease (MND)—are all progressive disorders of motor function with sensory function also affected in MS. Each disease also has specific non-motor symptoms. Study of these three neurological diseases reveals that they have some initial symptoms in common, especially those associated with movement, highlighting the need for careful diagnosis. Following a stressful and sometimes frustrating period of hypothesising and indecision, symptomatic people and their families are confronted with a devastating, often life-limiting diagnosis.


Progressive neurological diseases present unique lifestyle challenges for the person and their carers living in the community. Every case is different and each person has their own cultural and social understanding of illness, its impact and the eventual end-of-life process. People with the disease and their carers become very knowledgeable about disease management. This expertise must be recognised and valued by nurses and other members of the healthcare team (Forbes et al, 2007; Heisters, 2007). In this chapter, case studies have been provided to develop nurses’ insight into the individual manner in which each disease may affect a person’s life, their family and their relationships with the community.


Nursing people with any of these neurological diseases is very challenging, not only because of the complex and sometimes confronting symptomatology, but also the variable presentation of motor and non-motor signs in each person. A multidisciplinary approach to care is recognised as the preferred model, with nursing as a key discipline coordinating and providing care, giving advice, support and education, and monitoring the effect of medication. Effective care will only occur if nurses are prepared to work with other members of a multidisciplinary team and family carers to continually assess individual needs and collaboratively plan relevant supportive care, including timely access to community resources.



PARKINSON’S DISEASE


In 1817, after the publication of his ‘Essay on a shaking palsy’, James Parkinson gave his name to the condition we now know as Parkinson’s disease. PD is a chronic, degenerative neurological disorder, the most common of a group of conditions called the movement disorders, and the second most common neurodegenerative condition after Alzheimer’s disease. At the time of diagnosis, there is an estimated loss of around 60% of dopamine-producing cells in the substantia nigra of the mid-brain, causing a loss of dopamine in the brain (Przedborski, 2007). Dopamine is an important neurotransmitter in the basal ganglia, a collection of very specialised brain cells at the base of the brain that are responsible for the modulation of movement comprising the extra-pyramidal motor system. The basal ganglia are responsible for the modulation of movement. Recent studies have also found loss of dopaminergic neurones in other parts of the body, for instance in the gut, adding to problems with gut motility, and in the olfactory tract, causing partial loss of smell (Micieli et al, 2003).


Despite extensive research, the cause of PD remains unknown. However, there are recognised risk factors such as ageing, exposure to certain pesticides, gender (more males than females) and genetics (Przedborski, 2007). Yet, coffee consumption and smoking have been reported as protective factors (Hernan 2002). There are several genes identified for PD, but these account for a small percentage of people, and more likely to be found in young-onset PD (Vila & Przedborski, 2004). PD is sporadic in more than 90% of cases, with no known familial or genetic risk factors (Przedborski, 2007). Although PD is seen in all age groups its prevalence increases with age. Recent conservative figures estimated that 54,700 Australians (Access Economics, 2007) and 8000 New Zealanders were living with PD (Parkinson’s Society of New Zealand, 2004).


PD has varied symptomatology, but usually presents asymmetrically. The signs of PD are divided into motor and non-motor.


• Motor:
– the classical triad of (resting) tremor, rigidity (muscular stiffness) and akinesia (slowness and difficulty in initiating movement)

– flexion on the trunk, gait difficulties with shuffling and freezing and reduced arm swing

– akinesia results in difficulty turning in bed, small handwriting and a soft voice, which is difficult to understand.

• Non-motor:
– autonomic symptoms—postural hypotension, abnormal sweating

– urinary and bowel dysfunction, sexual difficulties

– cognitive decline, depression and anxiety

– sleep disturbances, fatigue

– loss of smell (anosmia)

– sensory phenomena, including pain that does not generally respond to analgesia if caused by a lack of levodopa.

At present medications are used to relieve some of the symptoms of PD, but they do not alter the course of the disease. Clinical trials are currently being conducted into the use of neuroprotective medications to slow the progression of PD (Ravina et al, 2003).



CASE STUDY 16.1


Dom is 68 years old. He emigrated to Australia from southern Italy when he was 23 years old and worked as a labourer in the building boom of the 60s, although his family in Italy had been farmers for many generations. His wife, Maria, is Australian born of an Italian family. They have three children.


Dom had symptoms of depression and left-hand clumsiness for two years before diagnosis. Then his voice became softer and monotonic, his face lost expression. He was often asked to repeat himself, in both English and his Italian dialect. Many of his friends told him he looked ‘grumpy’ all the time. After noticing a rest tremor of his left hand a diagnosis of PD was made. He was medicated with a levodopa replacement that enabled him to work full-time until he retired at age 60. His symptoms have worsened over time and his Parkinson’s medications have been less effective. He has become increasingly dependent on Maria. Since his balance has been affected, he has been having frequent falls.


Dom had lived with Parkinson’s disease for 12 years when he was admitted to the geriatric ward one Saturday evening, after a fall at home. He had not received any Parkinson’s medications since admission, as the doctor was concerned about his ability to swallow. He had not eaten since breakfast. An intravenous line was commenced for fluids. He became very distressed as he gradually became stiffer and stiffer until he was not able to move independently. His swallow worsened, he could not swallow his own secretions, was constantly drooling and unable to speak. He was also incontinent of urine, which embarrassed him and he became very tearful. Maria refused to go home until he had been reviewed and insisted he had his levodopa medication. A nasogastric tube was passed for medication and feeding. This was uncomfortable for him, but after a gap of over 12 hours, Dom received his dose of levodopa. He was then able to get out of bed himself. When the nurses changed his bed linen he overheard heard them complaining that he could do things when he wanted to!


Acute admissions are very difficult for people with PD. Dom reported that he had been very frightened by how helpless he felt when he had been unable to move or even speak and he thought that he might die. Don says: ‘Before PD, I was very strong man!’ He was also upset that the nurses did not understand the ‘on’ and ‘off ’ phenomena of his condition. Maria felt that no one seemed to have any knowledge of Parkinson’s disease and therefore she had little confidence in those caring for him, especially when no one was willing to ask her any relevant questions despite the fact she had been caring for Dom at home for many years.


On the day following admission a registered nurse sat down with them and took a detailed history from Dom and Maria, which went a long way to alleviate their anxieties. Issues of mobility, swallowing and diet, medication timing, continence, constipation, assistance with activities of daily living and pain were discussed and a plan of care was documented with a note to contact the speech pathologist to assess Dom’s swallow as soon as possible so that he could have his normal diet resumed. During this admission there was an opportunity for appropriate allied health assessments. The geriatrician in charge of Dom’s care had discussed nursing home placement for him, but Maria and his family would not consider this option so a family conference was initiated by the social worker. Up to this point the family had refused any external assistance but during the conference it became clear that Maria was exhausted with the 24-hour care. Maria agreed to approach the Catholic priest in their parish and in-home respite was organised for four hours, twice a week using Calabrese (Italian dialect) speaking volunteers. A daycare service, also operated by the church, offered one day per week with a pick-up and drop-off service.


Dom was discharged home with a walking frame and a referral to an out-patient exercise program to assist his gait and balance and further assess the mechanism of his falls. An occupational therapist visited the home and suggested a few modifications to assist with Dom’s care.


The introduction of respite is working well and Dom and Maria are adjusting to accepting help in Dom’s care. Maria understands that Dom is entering the palliative phase of PD, but is confident that he can be managed at home. They both realise that this will mean allowing more and more services to assist the family. Meanwhile, Dom has regular reviews at the Parkinson’s Clinic and ongoing support from the family GP. He has been booked to see the PD nurse specialist at his next visit to discuss possible use of apomorphine therapy and quality-of-life issues. Maria has been in touch with the local PD support group and they intend to attend the next group meeting.



ALTERED MOBILITY AND FATIGUE


Fatigue is a common but not well-understood symptom of PD. Yet, it does not affect all people with PD and is not always directly related to function or stage of disease (Friedman et al, 2007). Sleep disturbances, the high incidence of depression and some PD medications can result in daytime tiredness. Therefore it is important to monitor and treat, where possible, these extraneous factors. Fatigue has to be considered, as well as ‘on’ and ‘off ’ states in planning daily routine.


People with PD have different levels of motor function depending on whether they are in the ‘off ’ or ‘on’ states. The ‘off ’ state describes the patient when the signs and symptoms of PD are most obvious, while the ‘on’ state describes the best the person can achieve with PD medications. The same patient who requires full care in feeding, showering and transferring may be able to manage their care independently or with assistance in the ‘on’ state, after their medications take effect. Difficulty with gait, posture and balance means that mobility is often compromised, and showering, toileting and ambulating require careful assessment, so that measures are put in place to accommodate the individual mobility limitations and the person can achieve a measure of independence within safety limits. As the disease progresses, balance often deteriorates and falls are common. Walking aids may be required after review from the physiotherapist. To enable the person to realise their daily goals, their routine needs to be planned around the ‘on’ time.


Medications are commenced when disability affects quality-of-life or function. Dopamine replacement, in the form of levodopa combined with a dopa decarboxylase inhibitor to prevent the peripheral side-effects of levodopa (such as Sinemet, Madopar or Kinson), remains the first-line treatment for PD. At the onset of PD this medication often returns the person close to a normal baseline, but with disease progression many people find that the ‘on’ time shortens, leading to motor fluctuations complicated by chorea-like involuntary movements called ‘dyskinesias’. It is important to differentiate tremor from dyskinesia to ensure that correct treatment options are considered. Other PD drug therapies include synthetic dopamine agonists (pramipexole, available in New Zealand but not on PBS in Australia; pergolide; bromocriptine; cabergoline; and apomorphine), which act on the dopamine receptor sites with a similar but weaker action, except for apomorphine. Apomorphine, a by-product of morphine without opiate activity, is an injectable dopamine agonist that has a comparable effect to levodopa. It can be used as an injection to rescue the person from ‘off ’ states or as an infusion for management of symptomatology. Postural hypotension is common with this group of medications and they are more likely to cause hallucinations in susceptible patients. Obsessional behaviours such as hypersexuality, excessive eating and excessive gambling are recognised as side-effects of levodopa and dopamine agonist therapy in susceptible patients (Avanzi et al, 2006).


The COMT inhibitor (entacapone) prevents the rapid uptake of levodopa, thus allowing a longer effect time. A combination drug (levodopa/carbidopa/entacapone) is also available. Anti-cholinergic drugs (benzhexol, benztropin, biperidin) are rarely used because of their many side-effects and limited benefit. Amantidine has a mild benefit in motor symptoms and is more useful in controlling dyskinesias. Lastly, a MAO type B inhibitor (selegeline) was originally marketed as a neuroprotective agent in PD, but subsequent studies have not supported this, although it has a mild anti-Parkinson effect.


It is crucial that PD medications, in particular levodopa, are given on time, as delay may cause much discomfort as well as a reduction in the level of function. Dietary protein can interfere in the absorption of medications. Thus medications should be given at least 30 minutes before food, wherever possible. Timing of the effect of medication is important and can be measured with PD diaries, where motor fluctuations and dyskinesias, which are a side-effect of long-term dopamine replacement, can be documented, as well as ‘on’ and ‘off ’ states.


PD medications should not be stopped or drastically reduced abruptly, as this can lead to a rare but life-threatening condition called neuroleptic malignant syndrome (Ward, 2005). This syndrome is associated with worsening of Parkinsonian signs, altered mental state, hyperpyrexia, tachycardia, raised serum CK, renal insufficiency and a high mortality. Also there are many medications, including certain anti-emetics and phenothiazine medications, that may cause acute and severe exacerbation of PD and should be avoided.


It is accepted that people with PD have difficulty in motor sequencing. The use of external cues is very helpful to foster independence, especially with mobility, although swallowing or speech problems also respond to cues. Cues can be given by the nurse or carer or, where possible, instigated by the patient. Some useful cues, which initiate recovery from freezing of gait, include counting aloud, stepping over a laser line, humming a march tune or pretending to climb a stair. Getting out of bed can be broken down into motor segments, and cues can be used to get the patient through the entire sequence of movements.



BODY IMAGE


Many changes occur with PD that can lead to an altered body image. A stooped posture, slowness in movement and gait disturbance ages the person. Drooling, reduced facial expression, unblinking eyes, slowness in thought (bradyphrenia) and voice disturbances not only lead to a loss of personal dignity, but also interfere with communication and the person may appear of low intellect. Social isolation for both the person with PD and the carer can ensue. People with PD may resent their wishes, feelings and opinions being interpreted and reported by others and may often feel shunned by previous friends as the condition progresses (Habermann, 2000).


There are many complementary therapies available for people with PD to treat non-motor symptoms and improve body image and quality of life. Natural therapies, herbs and over-the-counter medications have been used for constipation, urinary frequency and urgency, postural hypotension, sexual difficulties, depression, anxiety and dementia. However, preparations should be checked by the pharmacist to ensure they do not interfere with PD medications or aggravate PD symptoms.



QUALITY OF LIFE


Quality of life means different things to different individuals and is adversely affected in PD for a variety of reasons. Motor symptoms have an effect on areas such as role change, independence, working, driving and physical comfort, yet studies show non-motor symptoms as more troublesome (Heath, 2004). Pain and sexual limitations are non-motor symptoms that have a negative impact on quality of life (Mott et al, 2004, 2005). The incidence of depression in up to 50% of cases is much higher than in other chronic neurological conditions (Pirtosek, 2007; Ravina et al, 2007). Psychiatric complications can reduce quality of life in patients and family and require careful monitoring and expert intervention (Raymond, 2006).


Social interaction diminishes as the disease progresses, not only because of body image changes but also because of communication difficulties. Communication is a basic human need and nurses need to be aware that establishing an effective means of communication is paramount to developing a rapport with people with PD. When faced with people who have soft monotonic voices, slurring of speech and reduced facial expression, it is important to listen to content rather than delivery of speech to gauge needs, mood and cognitive function. Using cueing to get people to speak loudly and key words is helpful and enables the personal expression of needs. For those who are unable to speak, communication boards are useful.



FAMILY AND CARERS


As with the majority of chronic conditions, Parkinson’s disease is a family affair. The dynamics, relationships and traditional roles in the family may change and resentment, guilt and grieving can become major problems if changes are not addressed. Social support is important and counselling outside the family is often helpful to resolve these issues.


A well-informed family is more able to assist the person with PD to make decisions about their care and solve many of the day-to-day quality-of-life issues. While direct caregiving may be exhausting in later stages of PD (see Case study 16.1), the family also carry the burden of the condition vicariously, as observers of the toll that PD is taking on their loved one (Habermann, 2000). Carers need support and time-out to avoid carer burn-out. Since this condition mainly affects the elderly, the carer’s health needs should also be closely monitored (Hirst, 2004).



MULTIPLE SCLEROSIS


Multiple sclerosis (MS) is a demyelinating disease affecting the nerve fibres in the white matter of the brain, the spinal cord and the optic nerve (Porth, 2005). Myelin forms around the axon as an insulator that interprets and conducts the message along the fibre tract. A process of acute inflammation causes demyelination to occur. In some instances the body remyelinates the axon and function returns. In other situations the myelin is lost and scarring or sclerosis occurs, slowing or impairing the transmission of the nerve impulse (De Souza & Bates, 2004). The size and distribution of the sclerotic plaques dictate the locations and types of symptoms experienced.


Geographical variations in incidence have led to several hypotheses about causal factors, including climatic influences, environmental factors and ethnically based genetic differences (Wallin & Kurtzke, 2003). Some researchers support the notion that demyelination is precipitated by an abnormal immune response of unknown origin, perhaps triggered by a virus in genetically susceptible people (Noseworthy, 1999).


Diagnosis is established through magnetic resonance imaging (MRI), evoked potential testing (measurement of sensory, visual and auditory nerve conduction) and examination of cerebrospinal fluid (Palace, 2001). This combination of testing is required because abnormalities in one test, for example the presence of lesions on MRI, are inconclusive and could be the result of other disease processes (Frankel, 2007).


Various presentations of MS are classified into four categories.


• Relapsing remitting MS: This is the most common form, characterised by episodic attacks of neurological symptoms, commonly sensory disturbance that may not be accompanied by clinical signs. Symptoms are usually experienced in one or more limbs, with optical, cerebellar and vestibular disturbances and accompanying fatigue. Between attacks the person recovers partial or complete neurological function (Palace, 2001). Arona’s condition (see Case study 16.2) exemplifies one of 16 forms of relapsing remitting MS.

• Primary progressive MS: About 10% of older people with MS live with primary progressive MS. This form begins with vague symptoms that develop into gait deficits, sometimes confused with the ageing process (Palace, 2001). Neurological damage continues to occur without remyelination, resulting in a steady increase in the level of disability. Antibodies that attack central nervous system antigens are thought to prevent remyelination in this form of MS (Pender, 2004).

• Secondary progressive MS: This begins in a similar manner to relapsing remitting MS, but develops a more constant progressive path with only minor occasional remissions (Frankel, 2007).

• Progressive relapsing MS: In this form the disease progression is continuous, with acute attacks occurring at intervals, accompanied by minor recovery (Frankel, 2007).

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Mar 13, 2017 | Posted by in NURSING | Comments Off on Parkinson’s disease, multiple sclerosis and motor neurone disease

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