In July 2017, The National Institute of Health and Care Excellence (NICE) published revised guidelines for Parkinson’s disease in adults.1 Clinicians will be trying to adhere to these, but what are the implications of these guidelines for commissioners and providers of services for people with Parkinson’s disease? Parkinson’s disease is a progressive degenerative disorder, and one of the more common neurological conditions, with an annual incidence in the UK of 15–20 per 100,000 and a prevalence of 1 in 625 of the adult population.2
Patients with the condition become progressively disabled by a combination of movement problems (mainly slowness and difficulty initiating movement), and non-motor symptoms such as mental disturbances, autonomic disturbance (for example, involving sweating, salivation, swallowing and digestion, sexual function, micturition and blood pressure control), cognitive failure and pain. Ultimately the condition becomes dominated by symptoms that respond poorly to medical management, and palliative care is required.
Management of Parkinson’s disease is therefore complex and can be resource intensive. Secondary care services are usually involved early, either Neurology or Care of the Elderly, depending on age, co-morbidity of the patient and local provision. Without effective care systems, the burden of care on family and carers can be intolerable, and the use of emergency care disproportionate. Patients and their carers need effective means of communication with the clinical team so as to address promptly the problems associated with Parkinsons’ as they arise. As management of the condition improves and people with the condition live longer, the requirement for support and liaison between multiple clinical disciplines gets ever more important.
Why do we need a NICE guideline on Parkinson’s?
NICE publishes evidence-based recommendations for health and care in England (not Wales or Scotland, although they may also be used there). The express aim of the Institute is to prevent ill health, to promote and protect good health, to improve the quality of care and services and to adapt and provide health and social care services. The guidelines are widely used to define ‘minimum standards of care’ in the UK, so that patients and carers using the National Health Service (NHS) know what they are entitled to receive from healthcare providers. Commissioners and Trusts are expected to adhere to NICE guidelines and to assure the process through regular audit. If this does not happen, then providers would be open to censure, for example by the Health Service Ombudsman in the event of a complaint, and may lose their eligibility to bid for provision of specialised services.
NICE publishes quality standards in the form of statements that are designed for commissioners and providers to identify gaps in service provision and areas for improvement, to facilitate measurement of quality of care and demonstration of high quality care, with the aim to facilitate commissioning of high quality services. Quality statements are reviewed annually, and those for Parkinson’s were last published in February 2018 (Box 1).
The quality standards emphasise a responsive point of contact (usually considered to be a specialist nurse), provision of information about impulse control disorders (a common treatment associated issue), involvement of multidisciplinary professional services and treatment on time for in-patients and availability of clozapine for those who develop hallucinosis. Each of these aspects provides organisational challenges for providers. Clinical teams would normally be expected to audit against these standards regularly, and commissioners might be expected to receive assurance that this is undertaken.
What do the guidelines say?
In the 2017 guidelines, great emphasis was placed on empowerment and provision of information in ways that are accessible to patients and carers. Since there has been a trade-off between the time available in clinic, especially outside of London where there is a shortage of neurologists,4 and the provision of comprehensive information; in practice, a nurse specialist service is required to bridge this information gap.
The diagnosis is clinical (that is, depends on history and examination rather than a laboratory tests) and can be difficult because the features can mimic those of other neurodegenerative disorders and those of ageing. The guideline recommends prompt referral of the untreated patient to a specialist.5 This recommendation is designed to facilitate accurate diagnosis, because treatment disguises the characteristic clinical symptoms of the condition (slowness, stiffness and tremor worse on one side of the body).
While this is ideal where patients can be seen promptly, there is a trade-off between referral untreated and the ability of the local neurology service to see patients and assess them in a timely manner. Many neurology and elderly care services in the UK can only guarantee that the patients are seen and treated within the government stipulated 18-week pathway from referral to treatment. The stipulation to refer untreated can therefore result in a patient experiencing avoidable discomfort for many weeks while waiting for an appointment. As a result, this recommendation is sometimes relaxed in practice.
The guidelines recognise that diagnosis is uncertain and requires clinical acumen. There is a limited role for the radiological investigation123 I-FP-CIT SPECT (known as DaTScan®) in the differentiation of Parkinson’s from essential tremor. This test should be made available to the specialist, but its use is uncommon in most centres.
Again the emphasis of the guideline is on informed choice between treatment options, with no clear winner as to the best initial treatment to offer to patients. Each option has risks and benefits, and these must be explained in detail to the patient starting treatment. NICE is clear that surgery for Parkinson’s should not be offered as initial or early treatment.
As the condition progresses, adverse effects of therapy generally emerge in the form of fluctuation in response, involuntary fidgety movements (dyskinesia) and psychotic ideation. Eventually, treatment-resistant symptoms dominate the clinical picture.6 Treatment of more advanced disease can be clinically challenging, and the guideline recognises that there are many treatment options to be considered. These include:
- Apomorphine, a drug in solution given by intermittent subcutaneous bolus injection or infusion under the skin using a small wearable pump
- Deep brain stimulation using implanted electrodes and a battery driver – surgery is usually provided in the regional specialist neurosurgical centre
- Clozapine for patients who cannot tolerate dopaminergic drug treatments because they develop psychotic symptoms such as hallucinations and paranoia.
NICE’s detailed economic analysis examined a further option for advanced Parkinson’s, that of levodopa–carbidopa intestinal gel (Duodopa®). This involves infusing levodopa directly into the gut jejunum so as to ensure constant delivery of the drug via the bloodstream to the brain. However, it was so far from being cost-effective that NICE determined it should not be offered.
At present there is a conflict between the NHSE commissioning policy on Duodopa7 (which is that it should be offered in particular circumstances, and was drawn up before NICE’s evaluation was published) and this recommendation from NICE in the current Parkinson’s guideline.
Treatment of complications of Parkinson’s
There are some specific problems that require expert management in people with Parkinson’s, and some drugs are used which are not commonly otherwise prescribed in primary care, and which therefore may challenge clinical systems which rely on prescribing these in primary care:
- Excessive sleepiness is common in those with Parkinson’s and may represent a risk for driving. If this symptom is troublesome, the guideline recommends consideration of the drug modafinil, which then requires regular monitoring by a specialist.
- Rapid eye movement sleep behaviour disorder is characterised by abnormal movements during sleep, often associated with vivid dreaming, which may be violent. NICE recommends consideration of clonazepam or melatonin for this condition.
- Orthostatic hypertension (drop of blood pressure on standing) is a common problem in Parkinson’s, worsened by medication. It may require adjustment of medication and the use of the unusual hypertensive agent, midodrine.
- Drooling may require treatment with the anticholinergic drugs, but the compounds commonly used for this problem (for example, when associated with cerebral palsy) such as hyoscine or atropine may readily provoke confusion and hallucinations in those with Parkinson’s. Glycopyrrolate, a drug that does not readily cross the blood–brain barrier, is specifically recommended by NICE, because it is less likely to precipitate hallucinations, but the drug is not well known or readily available in primary care in the UK.
- Use of the dopaminergic drugs bromocriptine or cabergoline is relatively unusual in the UK, but some clinicians see that these drugs have specific benefits for patients with advanced disease. These drugs can potentially cause fibrotic reactions in the lung, peritoneum, pleura and heart valves, and patients on these drugs therefore need regular blood tests and echocardiography.
- Dementia is common as the disease advances. NICE’s recommendations of cholinesterase inhibitors for this problem is in line with accepted practice in Alzheimer’s disease and other dementias,8 and nowadays poses no particular problems in primary or secondary care, though the support for carers of patients with dementia in the community may be very challenging.
- Palliative care for patients with end-stage disease is recommended. Palliative care teams may be resistant to accepting such referrals as they may be commissioned principally for the management of malignant disease.9
General practitioners may feel that prescribing these drugs is beyond their competence or remit in primary care. It may be that local secondary care services have to draw up shared care agreements with primary care to clarify the prescribing roles and responsibilities for these drugs.
Allied professional services
Although the guideline falls short of recommending that nurse specialist services should be provided, recommendation 1.7.1 states that people with Parkinson’s should have access to clinical monitoring, a continuing point of contact for support, home visits and reliable information for themselves and carers. In practice, because of the shortage of Consultant Neurologists and Geriatricians in England, it is difficult to imagine how this could be effectively achieved without a Parkinson’s nursing service.
There are new recommendations on physiotherapy, occupational therapy and speech and language therapy that should be offered to those who require it (that is, those with motor or balance problems, those with difficulties with activities of daily living and those with communication, swallowing or saliva problems respectively). These services must therefore be provided. Referral to a dietician is a ‘consider’ recommendation (that is, it is not considered mandatory to offer such a service).
Impulse control disorder
Compulsive and obsessive disorders are increasingly recognised as a major challenge of treating Parkinson’s, especially in advancing illness.10 These include pathological gambling, excessive shopping, eating or hypersexuality. Mild degrees of impulse control disorder are very common in people with Parkinson’s but, even when severe, the condition may remain hidden from the family and carers as well as from the clinician involved. Patients with pathological gambling may lose hundreds of thousands of pounds, and there is the potential for expensive litigation if patients are not appropriately warned and the warnings recorded.
Those with hypersexuality may acquire a forensic record. Patients may appear indifferent to the problems impulse control disorder creates around them. Prevention of problems is better than cure in this situation. NICE emphasises the importance of providing information to alert patients, family and carers about impulse control disorder when starting and reviewing medication, and recommends that dopaminergic therapy is reduced if this develops. The disorder does not respond readily to drug treatment. NICE stipulates that cognitive behavioural therapy should be offered if drug reduction does not help.
Hallucinations, predominantly visual, paranoia and agitation can be troublesome adverse effects of medication in Parkinson’s, especially in advanced disease. These symptoms are driven predominantly by dopaminergic drug treatment, that is, by l-dopa and the dopaminergic agonist drugs (ropinirole, pramipexole, rotigotine and bromocriptine) that are the mainstay of Parkinson’s treatment. Dopaminergic drug reduction does not always facilitate a reasonable balance between good mobility and psychotic symptoms, and in some there is an uncomfortable trade off between mobility and psychotic symptoms.
Some patients require antipsychotic drugs. Unfortunately most antipsychotic drugs are contraindicated in Parkinson’s because they greatly worsen mobility. NICE therefore recommends only two antipsychotic drugs for this indication – quetiapine and clozapine. The former is only modestly effective, but reasonably well tolerated.11 The latter, while more effective, is more difficult for patients to tolerate and poses significant risks to health, being associated with a high incidence of serious bone marrow suppression and heart rhythm abnormalities, either of which can be fatal. The mitigation of risk of clozapine treatment therefore requires the provision of a regular monitoring service, with weekly blood samples forwarded to the monitoring service. The drug is usually dispensed weekly or fortnightly only on receipt of a satisfactory blood sample. The establishment of such monitoring requires close collaboration between the pharmacy, nurse specialist and primary care.
The challenges of commissioning and providing services for Parkinson’s
The 2017 NICE guidance now underlines the multi-disciplinary nature of care in Parkinson’s. Physiotherapy, occupational therapy, speech and language therapy, dietician, pharmacist, and neurosurgeon are now all involved. The old model of a Consultant Neurologist or Geriatrician sitting in a clinic seeing a list of patients who have waited for many months for the privilege of being seen once and discharged promptly to the care of a GP no longer works.
Patients and their carers now are entitled to, and expect, a comprehensive and responsive quality service provided from diagnosis to death. The management of chronic neurological diseases has become a major challenge to the NHS and commissioners and providers of neurological services can find that there are conflicts between primary and secondary care as to who takes responsibility for service provision and continuing care. Neurology services in secondary care may not be designed to provide continuing care, and primary care services, already overwhelmed by demand, may struggle with the complexities of neurological treatment and support.
In this context, it is useful to start with the NICE quality statements on Parkinson’s disease when designing services (Box 1). As Consultant staff in the NHS are poorly accessible because of the scarcity of that resource and workload demands, adherence to NICE guidance usually requires a ringfenced nursing as well as a medical workforce. Most specialist centres recognise the fundamental value of a Parkinsons’ nurse specialist resource in order to provide an adequate and responsive service to patients.
This is crucial to provide counselling, to advise on treatment, liaise between primary and secondary care, signpost to other teams where required, co-ordinate care and address problems and crises. They may also have an important role in care of the patient undergoing surgery for Parkinson’s. Objective demonstration of the value of Parkinson’s specialist nursing services is difficult and much of the published research is largely anecdotal, but it has been plausibly suggested that nurse specialist services reduce unplanned admissions and length of stay, as well as facilitating adherence to NICE guidance.
Nurse specialist services have to be well designed and managed. An effective nursing service must be resilient (that is, not dependent on a single individual), geographically comprehensive (that is, accepting patients from defined localities without geographical gaps) and financially sound. It can be sited in primary or secondary care, and each model has advantages and disadvantages. Services can be cost-effective in secondary care if funded partly or largely on tariff income from nurse-led clinics, a model that may allow some flexibility for services to grow with patient demand.
In primary care, service provision less dependent on clinic income and therefore more flexible to provide services in the patients’ homes, but often reliant on funding from a ‘block contract’ that may result in services that become unstable if they cannot respond to increasing service demands. It has been estimated that Parkinson’s nurses are cost-saving in the UK, but the model was sensitive to the salary and additional costs of other health professionals’ time.12 Commissioners have to be alert to the changing demands on integrated services and good communication between providers and commissioners is mandatory to optimise provision.
Care of patients admitted to hospital with Parkinson’s is now frequently recognised as suboptimal. A recent campaign ‘Get it on time’ by the support charity Parkinson’s UK emphasised the need to get medication on time to avoid fluctuation in clinical state, which may result in delayed discharge or avoidable morbidity, for example from falls or confusion. This is recognised in NICE guidance and in the quality standard for Parkinson’s.
The problem of ongoing monitoring of patients with chronic neurological disease is nowhere better illustrated than with Parkinson’s disease. Patients are faced with progressive disability and require incremental support, and the condition is characterised by fluctuation and crisis that can be averted only by prompt attention.
Some drugs used in the condition, particularly clozapine, require the establishment of fail-safe and accessible monitoring systems which usually require close collaboration between multiple agencies in primary and secondary care, including medical, nursing and pharmacy. While psychiatric units may be familiar with the use and monitoring of clozapine in patients in the community (as it is a drug often used for refractory psychosis), psychiatrists are rarely familiar with the particular challenges of treating patients with Parkinson’s who are very much more sensitive to the adverse effects of antipsychotic medication than most patients with schizophrenia.
Reliance on extending existing commissioned services for clozapine that exist in the mental health services may therefore be misplaced, as such services may not have the necessary expertise to manage the clinical challenges.
The provision of a clinical service for patients with Parkinson’s diseae compliant with NICE guidelines is a major challenge for any organisation, requiring integration of services across primary and secondary care and also across nursing, therapy and medical disciplines. Patients require information and support from the earliest stages to the last day of palliative care, and there are particular problems for families and carers. Emergency care and duration of hospitalisation can likely be minimised by excellent care from a well-led team. The challenges for leadership and management of the teams are considerable.
Box 1: NICE quality statements February 2018
- Statement 1: Adults with Parkinson’s disease have a point of contact with specialist services.
- Statement 2: Adults with Parkinson’s disease taking dopaminergic therapy are given information about the risk of impulse control disorders, when starting treatment and at least annually.
- Statement 3: Adults with Parkinson’s disease are referred to physiotherapy, occupational therapy or speech and language therapy if they have problems with balance, motor function, activities of daily living, communication, swallowing or saliva.
- Statement 4: Adults with Parkinson’s disease who are in hospital or a care home should take levodopa within 30 minutes of their individually prescribed administration time.
- Statement 5: Services for adults with Parkinson’s disease provide access to clozapine and patient monitoring for treating hallucinations and delusions.
National Institute for Health and Care Excellence. Parkinson’s disease in adults. NICE Guideline [NG71]. July 2017. www.nice.org.uk/guidance/ng71
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12 National Institute for Health and Clinical Excellence; National Collaborating Centre for Chronic Conditions (UK). Parkinson’s Disease; National Clinical Guideline for Diagnosis and Management in Primary and Secondary Care. NICE Clinical Guidelines No 35. London: Royal College of Physicians (UK); 2006.