Sue Thomas and Dr Doug MacMahon discuss the skills needed by nurses to enable them to identify and address the needs of patients with Parkinson's disease.
Parkinson's disease presents a major challenge to nurses working in a variety of settings. It is becoming more prevalent and in light of future demographic changes, the demands placed on health and social agencies for these patients will continue to escalate. The authors suggest that nurses could and should identify and address the needs of patients with this chronic disease at an earlier stage both to ensure that their health is maintained and also that coping strategies are developed before problems arise. In order to do this a simple four stage clinical management scale has been developed. This identifies patient and carer needs throughout the course of the disease, and divides these into four arbitrary stages: at and around the time of diagnosis; in a stable maintenance period; in a complex phase and finally in a palliative care phase. Nursing interventions and priorities vary in these stages but with effective disease management the quality of life of patients and their carers could be improved.
Parkinson's disease
Parkinson's disease (PD) is one of a group of degenerative conditions that become increasingly common with advancing age. Parkinsonism is the term used to describe movement disorders characterised by similar symptoms to that of idiopathic Parkinson's disease. In the United Kingdom Parkinson's disease affects more than 1:1,000 of the general population rising to one per cent of the elderly population, and two per cent over the age of 80 years (Mutch, 1986). With a population of 60 million in the U.K. this means that there are an estimated 160,000 people with the disease. The disease results from degeneration and loss of neurones in the dopaminergic systems of the basal ganglia and specifically the substantia nigra in the brain. Loss of pigmentation and degeneration of these neurones brings about a loss of available dopamine, which affects the ability to produce and control movement. Normal functioning is dependent upon the balance between two chemical transmitters in the brain, dopamine and acetylcholine. A balance between the two is essential, as one is excitatory, the other inhibitory.
There has been a reawakening of interest in non-pharmacological treatment and therapy with increased use of the surgical treatments including thalamotomy, pallidotomy and sub-thalamotomy to reduce tremor and dyskinetic movement. A further recent advance in the treatment of Parkinson's disease is the license this year of an electronic brain implant (Activa Medtronic®) which can abolish tremor. An electrode implanted in the brain is connected to a small generator beneath the skin in the patient's chest. The generator sends an electrical stimulation to block the signals that cause tremor. To date around 30 patients in Britain have been fitted with this device.
Parkinson's Plus Syndromes
Up to a quarter of patients who show features of parkinsonism in the early stages later turn out not to have idiopathic Parkinson's disease. Some have other diseases collectively called the Parkinson's Plus Syndromes (PPS). With these syndromes there is not only loss of nigral cells but also of nerve cells elsewhere in the nervous system. In addition the symptoms usually respond only transiently or poorly to levodopa. The commonest of these PPS is Multiple System Atrophy (MSA) this condition can present in several ways (Quinn, 1996):
Diffuse or Cortical Lewy body disease is a new form of parkinsonism recognised in recent years. People with this condition show varying combinations of parkinsonism, visual hallucinations, memory loss and confusion. This has been cited as the second commonest cause of dementia after Alzheimer's disease.
The prevalence of this disease means that it is highly likely that all nurses will at some stage in their career encounter patients with Parkinson's disease. Nursing management presents challenges to nursing in both secondary and primary care and also in institutional settings such as residential care and nursing homes (Mutch, 1986). There has been much reference to the medical management of the disease because of its complex drug therapy and neuro-surgical techniques (Quinn, 1995; Olanow, 1994) but less attention to the care that nurses have provided care for patients with Parkinson's disease in all locations.
Increasingly the value of the Parkinson's disease nurse specialist is being recognised (Whitehouse, 1994; Maguire, 1997; Noble, 1998; Roy & Redfern, 1998). Many people with the disease and their families can cope very well providing they have enough information to manage the problems they encounter. Skilled and timely intervention by nurses and other members of the multidisciplinary team can help to minimise many of these problems. A multidisciplinary approach to the care of patients with Parkinson's disease is essential and early referral to speech, physio and occupational therapists can help patients greatly.
Living with Parkinson's disease
MacMahon and Thomas (1998) have identified a paradigm of disease management in Parkinson's disease, which illustrates the care requirements to consider in order that symptoms are eased and controlled, basic needs for daily living activities met and complications prevented. Management is divided into four specific areas; early care around the time of diagnosis, maintenance therapy, complex care and palliative care. With skilled medical and nursing intervention in these stages, individuals can be helped either to maintain independence with anticipatory care or regain lost independence when a crisis occurs.
The 'maintenance stage' usually follows, when symptoms are well controlled, usually with relatively simple drug treatments, and problems are easily overcome. Attention to health promotion is important, and sensible programmes of diet, exercise, and lifestyle are advisable, with an occasional review to watch out for complications. Patients who pass from the diagnostic to complex very quickly, without several years of stable maintenance, may have one of the variants (Parkinson's plus, MSA etc.). In order to improve care the first UK specific guidelines on the management of Parkinson's disease have been produced (Bhatia et al., 1998). These guidelines contain a treatment decision tree to aid decision making for prescribing. Drug treatment in PD is normally started when symptoms cause significant difficulty in maintaining independence, employment or social activities, because long-term drug therapy can provoke complications. Many drugs need to be given at specific times for maximum effect and drug interactions are common, therefore, nurses should familiarise themselves with common drugs and side effects (Appendix 1) and should be aware of the importance of timing of medications and compliance.
Maintaining a safe environment: A significant proportion of Parkinson's disease patients may have their safety affected because of balance problems causing a propensity to fall. Other contributory factors may include motor fluctuations, postural hypotension and confusion. For patients with mobility problems referral to the physiotherapist is indicated and can help patients to maintain mobility. It is essential nurses are aware of mobility problems so that steps can be taken to maintain the patients safety.
Motor fluctuations: specific problems encountered in Parkinson's disease after a few years of medication may initially be 'end of dose fading' that gradually become more intense as patients move from smooth control of their symptoms to more noticeable periods when their parkinsonian symptoms reappear. This may be complicated by dyskinesias (abnormal writhing movements of the limbs). Eventually motor control can become totally erratic with total loss of predictability and the characteristic 'on off' phenomenon where the patient may suddenly switch from an animated state to total immobility. This obviously has implications for safety.
Confusion, hallucinations and dementia: Agitation, anxiety and depression can spontaneously arise as a side effect of anti-parkinsonian medication. Hallucinations of a bizarre visual nature can sometimes accompany them. In later disease cognitive impairment becomes increasingly common with dementia found in up to 25 per cent of Parkinson's disease patients. These problems can all affect patient safety. Drug effects and depression should always be excluded before assuming the patient is demented.
Communication: Parkinson's disease patients may experience a variety of speech difficulties, any of which can affect their ability to communicate. Research has shown that many people with Parkinson's disease do not see a speech and language therapist even though they have speech difficulties (Oxtoby, 1982). Many are unaware that communication therapy offers an open referral system and that in many areas visits can be organised at home. Patients need to be able to communicate to their carers and friends otherwise they may lose the will to communicate and socialise. Communication is not just verbal: facial and body language form an important part of expression. The muscle rigidity present in this disease gives rise to a reduction in body and hand gestures and the 'mask like' expression which also deprives the patient of meaningful communication. This lack of body language can be misinterpreted by some as a lack of understanding or disinterest (Pentland, 1987) and nurses should be aware of this when experiencing difficulty communicating with patients.
Carers: Many carers have feelings of isolation and despair in their caring role. The Parkinson's Disease Society can offer valuable support here by the provision of local branch support for both patients and carers. The Society also has regional welfare coordinators and the national headquarters produces a large range of educational material for patients, carers and professionals alike.
Pressure Sores: Many patients with PD have difficulty with movement and mobility. In the complex and palliative care phases of disease management these patients may be at risk of pressure sore formation. This will be the case particularly when spending long periods of time sitting in a chair or lying in bed. For such patients it is advisable to use a validated pressure sore risk assessment score so that the situation can be monitored and timely advice given to prevent the risk of ulceration.
Working and playing: One serious problem patient's face is that of isolation and loneliness. Many patients lose their social contacts either because of mobility problems or their embarrassment because of tremor, loss of facial expression, or other features of the disease. It is important to try and encourage patients to maintain existing social contacts and develop new ones. Day centres and luncheon clubs are a useful source of social activity. The Parkinson's Disease Society also arranges holidays for members.
Financial difficulties: Living with a chronic disease can be expensive, additional money is frequently needed for heating, food, additional washing etc. Many people are unaware that they are entitled to benefits, others may not apply because the complex application forms are 'off putting'. A further aspect of the holistic care needed is that of ensuring patients are in receipt of the benefits they are entitled to. In the final palliative phase of the disease, relief of symptoms and distress in patients and carers become priorities.
Progressive drug withdrawal is often appropriate alongside the employment of other palliative care techniques and agents used in the management of other terminal conditions. As with many chronic diseases, slow deterioration occurs before death and the difficulty in identifying a terminal phase of the illness may mean that some patients are never recognised as being terminally ill. Thus the necessary support and counselling required coming to terms with the illness for both patient and carer may never occur. Hence the need for a 'palliative care' stage in which nursing has a major role in support of both patient and carers. The Carers Recognition and Services Act, 1995 has brought increasing recognition of the central essential role of informal carers and their needs. Carers need to be provided with timely access to accurate information about the disease, information on welfare and personal income support, domiciliary care, carer support schemes, day care and respite care. Without this information coping strategies may not be adequately developed.
Parkinson's disease is a complex progressive neurological disorder, the symptoms of which are variable throughout the course of the illness. The overall goal of care in patients is to improve prognosis and reduce the impact of the disease on both patients and their care givers with skilled medical and nursing intervention; to maintain functional ability and retain independence. At differing stages of the disease, care requirements will change, thus the authors have suggested an arbitrary staging process in the form of a simple clinical scale to indicate patient care requirements.
Medical treatment with drugs and surgical techniques is effective in Parkinson's disease, but there is also a major role for nurses in helping the patient come to terms with the disease, provision of information and care management. It is suggested that nurses familiarise themselves with this scale in order that the problems that may result as a consequence of the disease can be identified and addressed. Nurses should note that care of the Parkinson's disease patient is multidisciplinary. One professional cannot hope to meet all the needs of a single patient therefore it is essential that effective communication lines between care professionals are established for the good of the patient. The scale can assist in the recognition of these needs, and prompt review and reassessment of the needs as the disease progresses. Providing timely and appropriate care for patients can do much to enhance quality of life, and the paradigm can provide an aide-memoire to facilitate this process.
Glossary
Bradykinesia slowness of voluntary body movement.
Dyskinesia an involuntary or abnormal movement affecting any part of the body. Caused by some anti-parkinsonian drug therapies. Normally described as chorea or athetoid writhing movements.
Festination walking with a pattern of accelerating short rapid shuffling steps, often associated with freezing and difficulty in initiation.
Freezing this symptom is quite common, which causes the person affected to stop suddenly while walking and being unable to move forward for several seconds or minutes. It makes people feel their feet are frozen to the ground and can often happen in doorways and at thresholds between rooms.
On-Off Phenomenon this term is used to describe abrupt and unpredictable changes in mobility of some people with long standing Parkinson's who are on levodopa. In the 'on' state they are able to move. In the 'off' state they can be virtually immobile. They can switch from one state to the other within a brief period of time, minutes or even seconds.
Parkinsonism the term used to describe movement disorders characterized by similar symptoms to those of idiopathic Parkinson's.
'Pin rolling' a term used to describe the type of tremor between the thumb and index finger of people with Parkinson's. It derives from the method that apothecaries used to make round pills.
Rigidity the name given to the special type of stiffness which is one of the main symptoms of Parkinson's. The muscle tend to pull against each other instead of working smoothly together. This is due to a failure of reciprocal relaxation of the antagonist muscles. There are two types:
Levodopa has been used for the treatment of Parkinson's disease since the 1960's and is still the mainstay of modern treament. Levodopa is converted in the brain to dopamine. It is always given in combination with a peripheral dopa decarboxylase inhibitor to reduce side effects. Madopar and Sinemet are the two main ranges available and come as preparations with differing duration of action (dispersible is the quickest, CR is the slowest). Most patients can tolerate these drugs and experience considerable improvement for several years especially where slowness and stiffness of movement are concerned. Special care is needed in older patients to reduce postural hypotension and confusion. Long-term drug therapy can result in reduced effectiveness with periods where the drugs do not work with sudden switches from on to off when the patient may be immobile. Involuntary movements (dyskinesias and dystonias) also occur increasingly frequently as the disease progresses.
Direct Agonists include bromocriptine, pergolide, lisuride, ropinirole, cabergoline and pramipexole. Pergolide is currently the most commonly prescribed non-levodopa treatment. Direct agonists do not need to be converted in the brain to dopamine but have a higher rate of side effects. Domperidone is sometimes required to counteract any nausea that may initially result from therapy.
Apomorphine is also a direct agonist, but it is currently given either by subcutaneous injection or syringe driver for treatment of severe sudden fluctuations in symptoms which cannot be controlled by adjustments in medication. In selected patients who experience motor fluctuations apomorphine can provide a means of rescue within 515 minutes. Effects usually last for up to one hour following a single subcutaneous dose. Side effects of nausea and hypotension can be availed with oral domperidone, which can be tailed off after several weeks when tolerance usually develops. The syringe driver is used when all other treatments have been exhausted.
Selegiline previously research had suggested that selegiline may slow down the progression of PD but its safety is still questioned following a report which suggested higher mortality in patients who were taking 10mg of selegiline in combination with levodopa rather than levodopa alone (Lees, 1995). A recently launched preparation (Zelapar) melts in the mouth and contains only 1.25mg selegiline and produces fewer metabolites and is well received by patients.
Anticholinergics (benzhexol, benztropine, and kemadrine) have been available for many years but are often poorly tolerated. Side effects include dry mouth, hallucinations, confusion, blurred vision and constipation. These drugs are best avoided particularly in the older person.
COMT (Catechol-o-methyltransferase) Inhibitors have recently become available (Entacapone). This new drug will reduce the breakdown of levodopa and is used to improve the duration and quality of response. A specialist usually initiates treatment, since side effects may occur in some patients; notably diarrhoea, dyskinesia and confusion. Levodopa doses are usually reduced on their initiation.
References
Mutch, W.J., Dingwall-Fordyce, I., Downie, A.W., Paterson, J.G., Roy, S.K. (1986) 'Parkinson's disease in a Scottish City'. Br Med J. 292: 5346.
Ellenberg, J.H., Koller, W.C., Langston, J.W. (1995). Etiology of Parkinson's Disease. Marcel Dekker.
MacMahon, D.G. (1996) 'Parkinson's Disease.' Primary Health Care Journal.
Quinn, N. (1996) Multiple System Atrophy. Parkinson's Disease Society Information Leaflet.
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Quinn, N. (1995) Drug Treatment of Parkinson's Disease. Br Med J. 310: 5759.
Olanow, C.W., Marsden, C.D., Lang, A.E., Goetz, C.G. (1994). Neurology; 44 (suppl 1: S1720).
Whitehouse, C. (1994) 'A new source of support: the nurse practitioner in Parkinson's Disease and dystonia.' Professional Nurse. 9: 44751.
Maguire, R. (1997) 'Parkinson's Disease.' Professional Nurse. 133; 1: 3337.
Noble, C. (1998) 'Parkinson's disease and the role of nurse specialists.' Nursing Standard. 12; 22: 323.
Roy, S., Redfern, L. (1998) 'Parkinson's Disease.' Nursing Standard. 12; 22: 4956.
MacMahon, D.G., Thomas, S. (1998) 'Practical Approach to Quality of Life in Parkinson's Disease.' Journal of Neurology. 245 [Supp]1; 1: S1922.
Oxtoby, M., (1982) 'Parkinson's Disease Patients and their Social Needs.' Parkinson's Disease Society. London.
Norton, C. (1997) 'Your bladder and bowels in Parkinson's disease.' Parkinson's Disease Society. London.
Pentland, B., Pitcairn, T.K., Gray, J.M. (1987) 'The effects of reduced expression in Parkinson's disease on impression formation by health professionals.' Clinical Rehabilitation.
A Guide for nurses working in primary health care: 'Parkinson's Aware in Primary Care' can be obtained from:
Parkinson's Disease Society
215 Vauxhall Bridge Road
London SW1V 1EJ
Enclose a self addressed A4 envelope with 32p postage.