National Prion Clinic


Patient care

At the National Prion Clinic (NPC) our nursing philosophy is to provide an atmosphere of comfort while delivering care to the public we serve. It is our belief that the dignity and worth of the individual must be respected and preserved. We further believe that the nursing care must be consistent with and specifically designed to meet the needs of the patient, family members and the community in which the patient lives. The following information explains how the nurses and medical staff at the NPC can help, with difficulties that patients and their families may experience, due to prion disease. We work closely with colleagues from local teams and those from the National CJD Research & Surveillance Unit based in Edinburgh.
Bladder and Bowel Function

Problems with urinary incontinence can be particularly difficult for patients to discuss.  The nurses at the NPC aim to discuss these problems openly and hope to achieve the best possible outcome for the patient through advice and support.  We can advise on management of frequency and incontinence including drug treatment e.g. tolterodine, for the former, and use of pads and catheters for the latter.  We can also advise on symptomatic management of recurring urinary infection and dehydration as well as skin care.

In prion disease there can be a decrease or increase in normal frequency of defecation.  This can be accompanied by difficult or incomplete passage of stool and/or passage of excessively hard, dry stool or diarrhoea.  The "normal" patterns of bowel elimination vary so widely from individual to individual.  Some people believe they are constipated if a day passes without a bowel movement; for others, every third or fourth day is normal. 

Constipation has a variety of causes, such as, too little fluid, too little fibre, inactivity or immobility, stress and depression.  Use of medications, particularly narcotic analgesics or overuse of laxatives and enemas can also cause constipation.  Because privacy is an issue for most, being away from home, hospitalised, or otherwise being deprived of adequate privacy can result in constipation.  

Dietary management (increasing fluid and fibre) remains the most effective treatment for constipation.


The ability to communicate verbally at some point in the prion disease process will cease.  At the NPC, we actively encourage patients to participate in the decision making process regarding their future care, for as long as possible.  This decision making process must be started early in order to ascertain patient wishes around care.  Examples of this would include discussions around artificial feeding and end of life issues.  With the person’s permission, carers and relatives normally have the opportunity to be involved in decisions about care and treatment.  We also assess carers and relatives needs and provide information and support as required.

If you want someone to make decisions about your finances you can set up a power of attorney. Information can be found at www.gov.uk/power-of-attorney

People with prion disease experience problems with communication for a variety of different reasons.  Problems can include difficulty forming whole sentences, difficulty recalling words or phrases and difficulty with understanding.  It is important to assess communication problems as early as possible in order to maintain an optimum level of care. 

Communication difficulties can be particularly frustrating in the early stages of prion disease and can lead to feelings of depression and isolation.  To promote current levels of ability many strategies can be used.  For example, communication aids, allowing extra time to communicate and empathetic understanding.  

Even if a patient cannot speak they may still be able to communicate their wishes by nodding/shaking of their head or another gesture.  If this is not possible, and the patient is not moving, they may still enjoy hearing a loved one’s voice or favourite music.

Disorders of Movement

All patients with prion disease will eventually have difficulties with their mobility.  In prion disease, this presents particular problems as it is usually accompanied by a cognitive impairment or lack of insight into their disability.  Reduced mobility in prion disease has many causes including damage to parts of the brain that control movements and walking, loss of muscle mass, stiffer muscles and less mobile joints and problems with balance.  Jerky movements of the limbs and trunk (myoclonus) frequently occur and many patients are extremely unsteady with poor coordination of limb movements (ataxia).  Nursing goals are to ensure a safe environment whilst maintaining an acceptable quality of life for patients and their carers. 

Medications such as baclofen, dantrolene and diazepam are sometimes effective in reducing certain types of stiffness (spasticity) but are less effective when the muscles are rigid.  Anti Parkinson drugs e.g. sinemet or ropinirole can sometimes help rigidity.  Local injections of botox are useful in those patients where rigidity or spasticity is confined to a few muscles.  Physiotherapy can also help.  Keppra (levetiracetam) and clonazepam can be used to reduce myoclonus.  Both of these drugs are antiepileptic agents and are therefore useful in the occasional patient with seizures.  Ataxia is extremely difficult to treat and there are in most cases no effective medications. 

Eating and Drinking

Swallowing problems are common in prion disease and present at varying stages of the illness.  If swallowing difficulties occur very early on in the disease process then alternative methods of delivering nutrition may be raised.  This may be something about which the patient has already expressed a clear view in previous discussions.  The nurses at the NPC offer information, ongoing support and management to families when faced with these difficult decisions.  Speech and language therapists may also be involved.  Increased salivation is the result of swallowing difficulty and can be helped by drugs that reduce the amount of saliva e.g. atropine drops or hyoscine patches. 

Patients, families and local teams who are keen to proceed with artificial feeding are supported through their decision making.  Advice on insertion of Radiologically-Inserted Gastronomy(RIG) is available and on occasion patients will transfer to our inpatient unit to have this procedure carried out.

Infection Minimisation

Open wounds, particularly pressure sores, can be sites for infection.  Soft tissues (cells, fat and muscle) and organs (kidneys, lungs) can also be sites for infection either after trauma, invasive procedures, or by invasion of pathogens carried through the bloodstream or lymphatic system.  Being malnourished, having inadequate resources for sanitary living conditions, and a lack of knowledge about disease transmission place individuals at risk for infection.  We can advise how to minimise transmission of infection.

Pain Management

Fortunately pain, apart from headache, is not a common feature of prion disease, although leg pain is a characteristic feature of vCJD and certain hereditary forms of prion disease. Gabapentin, pregabalin and amitriptyline are all useful for neuropathic pain of this type. 

Psychiatric and Behavioural Issues

People with prion disease experiencean irreversible and/or progressive deterioration of intellect and personality.  This is characterized by decreased ability to interpret environmental stimuli, decreased capacity for intellectual thought process and manifested by disturbances of memory, orientation, and behaviour.  Some behavioural issues are borne of frustration.  This tends to occur when the patient has lost the ability to carry out certain tasks but retains insight into their disability.  Other behavioural difficulties are due to severe damage within the frontal lobes of the brain, which control emotion, personality and behaviour.  Visual hallucinations, often of frightening quality, are common and can lead to aggressive behaviour as they are real to the patient. 

Many of these symptoms can be difficult to treat.  These changes can have a profound impact on family members and family processes as the patient requires more direct supervision and care.  We can provide support for the family and local teams and advise on treating individual symptoms.  It is often a comfort for relatives to know, that it is unlikely that the patient has any persistent memories of their difficult behaviour or abusive language. 

Medications are often useful adjuncts to this support.  Aggression and agitation are helped by atypical antipsychotic drugs such as quetiapine, risperidone or olanzapine.  Diazepam can also sometimes help.  Visual hallucinations respond well to drugs used in Alzheimer’s disease e.g. donepezil, galantamine and rivastigmine. 


Many patients experience trouble with their sleeping patterns in prion disease.  These problems range from sleeping all the time to not sleeping at all. The prion disease damages the thalamic areas of the brain responsible for controlling sleeping cycles.  This can very often be difficult to treat.  We provide advice on symptomatic management for example, temazepam, clonazepam, diazepam, zopiclone, and monitor progress of this.  We also provide educational sessions to carers who look after these patients.  This has particular relevance to patients who don’t sleep, as a night duty may mean following the patient around their house or care home for a full 12 hour shift without sitting down.  Therefore care providers need to consider this when placing patients or discharging them to their homes.

Social Services

Service provision differs greatly in all parts of the United Kingdom.  The nurses at the NPC provide educational support to social service teams in order to advance their knowledge on prion disease and its likely manifestation and progression.  We also attend multi-disciplinary case conferences in order to assist the local team in clinical assessments of their patients.  We provide supporting documentation to support health needs assessments.

DHSC document

Support for the Carer

Nurses at the NPC can assist carers by providing the necessary education and skill training.  We can offer support through home visits; special clinic sessions; telephone access for questions and comfort, e-mail contact or "chat groups"; and opportunities for respite care.

Terminal Care

The NPC offer patients and families the opportunity to discuss end of life wishes.  The nursing team at the NPC are able to provide advice on difficulties that arise such as management of breathing, swallowing and hydration.  These symptoms can be particularly distressing for the family.  Input from palliative care will aim to ensure care provided will lead to a peaceful end to life. 

We also discuss post mortem examination with the relatives and obtain consent.  This is discussed further on the post mortem information sheet.

Washing and Dressing

The majority of patients will need some degree of assistance to wash and dress as their illness progresses.  The nurses at the NPC can advise local teams on the likely progression of the disease and offer regular nurse led clinical assessments to assist with assessment of needs.  The medical team at the prion clinic will also see the patients regularly and immediately should the local team require further support.

Prion disease can erode the individual’s ability or willingness to perform the activities required for self care.  Careful examination of the patient’s problems are required in order to be certain that the patient is not failing at self-care because of a lack in material resources or a problem with arranging the environment to suit the patient’s physical limitations.  We can help coordinate services to maximize the independence of the patient and to ensure that the environment that the patient lives in, is safe and supportive of his or her special needs.