National Prion Clinic


Philosophy of Nursing at the NPC

Leddy and Pepper (2003) defined philosophy of nursing as the intellectual and affective outcomes of the professional nurses' efforts to understand the ultimate relationship among humans, environment, and health; to approach nursing as a scientific discipline; to integrate a sense of values into practice; to appreciate esthetical elements that contribute to health and well-being; and to articulate a personal belief system about human beings, environment, health and nursing. At the NPC our nursing philosophy is providing an atmosphere of comfort while delivering care to the public we serve, it is our belief that the dignity and worth of 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 Nursing service at the NPC is an advisory outreach service to multi-disciplinary health teams throughout the United Kingdom. The function and aim of the nursing team is, 

  • To demonstrate advanced assessment skills in identifying patients' physical, social and psychological needs, as a basis for planning, implementing and evaluating a collaborative programme of care and providing specialist advice that is evidence based and appropriately supports clinical outcomes in prion disease.

  • To provide information, advice and support to patients, families and other Health Care Professionals within the Trust, nationally and internationally on prion disease, by face to face contact, via the telephone advice line (including registered and generic callers), via e-mail and in writing. 

  • To be visible in the clinical area, playing an active role in the nursing care of patients with prion disease and acting as a credible role model and resource for both staff and patients/clients. 

  • To act as patient advocate, through provision of effective support and information that enables patients and their relatives to make informed choices and decisions concerning treatment - where necessary to facilitate communication and decision­ making between patients, their families and the inter-disciplinary team. 

  • To set, monitor and improve standards of care, including the development of clinical pathways in partnership with support staff, to ensure that care, treatment and service needs are met for all patients that come under the care of the NPC. 

  • To advocate a healthy lifestyle for patients and staff, providing guidance regarding wellness, disease risks and potential changes in health status in line with the Department of Health's national targets. 

  • To initiate the development of tools to improve the quality of service and to champion sound clinical leadership and develop support mechanisms for sharing good clinical practice within the clinical area, Trust wide, nationally and internationally. 

  • Contribute to the investigation of complaints, accident reports and untoward incidents within the clinical area and implement recommendations for action, in line with Trust Policies and procedures and in conjunction with individual ward or departmental managers. 

  • To champion current trends and developments in nursing education and practice­ development within the NHS and take an active lead in advancing nursing/clinical practice through the implementation of evidence based practice and research on prion disease. 

  • To formulate appropriate care plan pathways using formal referral mechanisms to the appropriate primary and secondary service to ensure effective levels of health and social care for patients with prion disease.

  • To provide information and support to clients, their partners or relatives, in relation to their known or unknown risk factors for prion disease and the impact this may have on their life.

  • To support and provide emotional, medical and social needs assessment. This will involve communicating clearly and effectively both medical and psychological information to clients in various levels of distress from varying backgrounds and belief structures



The Clinical Nurse Specialists led by the Lead Nurse will provide information, education and advice on the following health needs, which are common amongst patients with prion disease and in keeping with Roper Logan and Tierney’s evidence based model for care planning (Roper, Logan and Tierney 2000).

Maintaining a safe environment

Mobility of people with prion disease is linked to health status and quality of life. In prion disease there are large complexities of factors that influence mobility. 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 the patient’s disability. Many problems in care management also arise when patients present with cognitive impairment early in the disease process but mobility remains relatively intact. This group of patients present a particular challenge to local teams in terms of placement, as they are young patients under the age of 50 with often poor local service provision to meet their needs.

Reduced mobility involves a limitation in independent, purposeful physical movement of the body or of one or more extremities (NANDA). Reduced mobility in prion disease is related to body changes from loss of muscle mass, stiffer and less mobile joints, gait changes affecting balance can significantly compromise the mobility of patients with prion disease. Restricted movement affects the performance of most activities of daily living (ADLs) this is particularly relevant in prion disease as co-ordination is greatly reduced and often coupled with poor insight into the disability. Nursing goals are to ensure a safe environment whilst maintaining an acceptable quality of life for patients and their carer’s.


The ability to communicate at some point in the prion disease process will cease. We actively encourage patients to participate in the decision making process for the future for as long as possible. This decision making process must be started early in order to ascertain what the patient would want to happen in their care. Examples of this would include discussions around artificial feeding and end of life issues. We also employ many strategies in helping the patient communicate after their expression of speech is damaged but they are still receptively aware.

People with prion disease experience communication difficulties for a variety of different reasons. Communication difficulties resulting from prion disease can include difficulty forming whole sentences and difficulty recalling words or phrases. Communication difficulties can be particularly frustrating in the early stages of prion disease and can lead to feelings of depression and isolation.  In order to prevent a depressed mood state that results from communication difficulties it is important to be aware of the adverse effects that communication difficulties can have on a patient’s mood and employ as many strategies as possible to promote current levels of ability. Communication difficulties related to prion disease can also result in comprehension difficulties.  Such comprehension difficulties should be assessed as early as possible in order to maintain an optimum level of care. 

Communication difficulties involve decreased, delayed, or absent ability to receive, process, transmit, and use a system of symbols (NANDA). The nursing staff at the NPC is able to recognise the broad spectrum of causes for communication difficulties in prion disease and work closely with local health care professionals to employ a number of strategies to improve transmission of information.

Person-centred care

Clearly these issues surrounding quality of communication with people with dementia demonstrate the importance of person-centred care. NICE (2018) guidance outlined general principles of care, including person-centred care and diversity and equality.

The guidance stressed that good communication is essential, supported by evidence-based information, to allow people with dementia to reach informed decisions about their care. It emphasised that the views of people with dementia on who should and should not be involved in their care are important and should be respected. With the person’s permission, carers and relatives should normally have the opportunity to be involved in decisions about care and treatment.

Carers and relatives should be provided with the information and support they need, and carers should also be offered an assessment of their own needs. For recommendations on diversity and equality relevant to communicating with people with dementia.

Diversity and equality in dementia care

To ensure equality in the provision of dementia care, NICE and SCIE recommend:

  • Always treat people with dementia and their carer’s with respect;

  • If there is a language barrier, offer:

  • Written information in the preferred language and/or in a format that can be easily accessed;

  • Independent interpreters;

  • Psychological interventions in the preferred language.

Identify specific needs arising from:

  • Diversity (such as sex, ethnicity, age, religion and personal care);

  • Ill health, physical and learning disabilities, sensory impairment, communication difficulties, problems with nutrition and poor oral health;

  • Identify and wherever possible accommodate preferences (such as diet, sexuality and religion), particularly in residential care.

The increasing incidence of dementia as a result of the ageing population means practitioners will need to develop and improve communication skills in caring for people with this condition, in order to have a positive effect on patients’ quality of life and well-being.


Difficulties with breathing often occurs end stage. The patient may be unable to manage his or her own saliva. This may lead to choking or noisy breathing, which is particularly distressing for their families to watch. The nursing team at the NPC are able to provide advice on symptomatic management.

People with prion disease often experience difficulty with inspiration and/or expiration that does not provide adequate ventilation

Respiratory pattern monitoring addresses the patient’s ventilatory pattern, rate, and depth. Most acute pulmonary deterioration is preceded by a change in breathing pattern. Respiratory failure can be seen with a change in respiratory rate, change in normal abdominal and thoracic patterns for inspiration and expiration, change in depth of ventilation (Vt), and respiratory alternant. Breathing pattern changes may occur in a multitude of cases.

Eating and Drinking

Swallowing problems are common in Prion disease and present themselves at varying stages of the illness. If they occur very early on in the disease process then alternative methods of delivering nutrition need to be explored. This may be something that the patient has already strongly advocated against in previous discussions. If the patients have made their wishes clear then it is often difficult for families to see death expedited through lack of nutrition. The nursing staff at the NPC offers ongoing support and management for these families. Patients and families and local teams who are keen to proceed with artificial feeding are supported through their decision making. Advice on insertion of RIGS is available and often patients will transfer to our inpatient unit to have this procedure carried out.

Patients with prion disease are at risk for entry of gastrointestinal secretions, oropharyngeal secretions, or solids or fluids into tracheobronchial passages. Both rapid and longer progression of prion disease can place patients at risk for aspiration. Cognitively impaired patients are at high risk. Aspiration is a common cause of death in comatose patients.


All aspects of personal care are affected by prion disease. End stage problems lead to double incontinence. As this is a particularly personal problem, many patients find it hard to discuss. As the majority of patients are young with the inherited forms of prion disease specialist continence specialists are in short supply. The nursing staff at the NPC, can offer advice after we have built a relationship with patients, we can discuss some of these issue’s more openly and hopefully achieve the best possible outcome for the patient. 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 accompanied by difficult or incomplete passage of stool and/or passage of excessively hard, dry stool or diarrhoea.

Constipation is a common, yet complex problem; it is especially prevalent among patients with prion disease. Too little fluid, too little fibre, inactivity or immobility, and disruption in daily routines can result in constipation. Use of medications, particularly narcotic analgesics or overuse of laxatives, can cause constipation. Overuse of enemas can cause constipation, as can ignoring the need to defecate. Psychological disorders such as stress and depression can 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. Because "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. Chronic constipation can result in the development of haemorrhoids; diverticulitis (particularly in elderly patients who have a high incidence of diverticulitis); straining at stool, which can cause sudden death; and although rare, perforation of the colon. Constipation is usually episodic. Dietary management (increasing fluid and fibre) remains the most effective treatment for constipation.

Washing and dressing

Sequencing tasks can be particularly difficult for patients with prion disease. They may lack awareness that a problem exists. They may do well in spontaneous questioning of their ability to wash and dress but evidence would suggest that the majority of patients would need some degree of assistance as their problems progress. We 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.

People with prion disease often have an impaired ability to perform or complete activities of daily living, such as feeding, dressing, bathing, toileting. The nurse may encounter the patient with a self-care deficit in the hospital or in the community. These deficits are the result of progressive deterioration that erodes the individual’s ability or willingness to perform the activities required for self care.  Careful examination of the patient’s deficit is 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. The nurse coordinates 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.

Infection minimisation

Patients with prion disease are at an increased risk for being invaded by pathogenic organisms.

Persons at risk for infection are those whose natural defence mechanisms are inadequate to protect them from the inevitable injuries and exposures that occur throughout the course of living. Infections occur when an organism (e.g., bacterium, virus, fungus, or other parasite) invades a susceptible host. Breaks in the integument, the body’s first line of defence, and/or the mucous membranes allow invasion by pathogens. If the host’s (patient’s) immune system cannot combat the invading organism adequately, an infection occurs. Open wounds, particularly pressure sores can be sites for infection; soft tissues (cells, fat, 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. Infections can be transmitted, either by contact or through airborne transmission, sexual contact, or sharing of intravenous (IV) drug paraphernalia. Being malnourished, having inadequate resources for sanitary living conditions, and lacking knowledge about disease transmission place individuals at risk for infection. Health care workers, to protect themselves and others from disease transmission, must understand how to take precautions to prevent transmission. Infections prolong healing, and can result in death if untreated. Antimicrobials are used to treat infections when susceptibility is present. Organisms may become resistant to antimicrobials, requiring multiple antimicrobial therapies. There are organisms for which no antimicrobial is effective, such as the human immunodeficiency virus (HIV).

Symptomatic pain management

Fortunately, pain is not a common feature of prion disease. However, in the early stages some patients do complain of peripheral type pains and aches. We can advise on symptomatic management once the local team has excluded any other possible source of the pain. We can provide an assurance to families that at the very end it is very unlikely that the patient can experience any pain due to the extensive damage caused by the prion disease. This is very often very comforting to them and useful for their local teams to know when providing end stage palliative medication. We advise on symptomatic management of myoclonus as these sudden jerky movements although not painful can appear so and are distressing for the family to watch.

Pain can be described as an unpleasant sensory and emotional experience arising from actual or potential tissue damage or described in terms of such damage (International Association for the Study of Pain); sudden or slow onset of any intensity from mild to severe with an anticipated or predictable end and a duration of less than 6 months.

Pain is a highly subjective state in which a variety of unpleasant sensations and a wide range of distressing factors may be experienced by the sufferer. Pain may be a symptom of injury or illness. Pain may also arise from emotional, psychological, cultural, or spiritual distress. Pain can be very difficult to explain, because it is unique to the individual; pain should be accepted as described by the sufferer. Pain assessment can be challenging, especially in patients with prion disease, where cognitive impairment and sensory-perceptual deficits are more common.

Psychiatric and Behavioural Issues

Not all patients with Prion disease will present with behavioural problems. Even within families carrying the same mutation in the inherited forms of the disease presentation of the disease can manifest itself very differently. It is important among the families who are at risk of inherited prion disease to know, that if a parent suffered and died from the illness, that their presentation should they be positive may not necessarily be the same.

Some behavioural issues are born of frustration. This tends to occur when the patient has lost the ability to carry out certain tasks but retains insight into their disability. This is difficult to treat and we can provide support for the family and local teams through this difficult time. Other behavioural difficulty are due to severe damage within the frontal lobes of the brain, which control emotion. These are easier to treat and we can provide symptomatic management advice on treatment. It is also a comfort to relatives to know, that it is unlikely that the patient has any awareness of their difficult behaviour or abusive language.

People with prion disease experience an irreversible, long-standing and/or progressive deterioration of intellect and personality characterized by decreased ability to interpret environmental stimuli, decreased capacity for intellectual thought process and manifested by disturbances of memory, orientation, and behaviour.

Chronic confusion is not limited to any one age group, gender, or clinical problem. Chronic confusion can occur in a variety of settings including the home, hospital, and long-term care facilities. While often associated with older adults with dementia, younger adults with dementia may also be affected. Chronic confusion can have a profound impact on family members and family processes as the patient requires more direct supervision and care. This care plan discusses the management of chronic confusion in any setting. It also identifies the importance of addressing the needs of the carer.

Support for the carer

The focus of this care plan is on the supportive care rendered by family, significant others, or carers responsible for meeting the physical and/or emotional needs of the patient. With limited access to health care for many people, most diseases diagnosed and managed in the outpatient setting, and rapid hospital discharges for even the most complex health problems, the care of acute and chronic illnesses are often managed in the home environment. Today’s health care environment places high expectations on the designated carer, whether a family member or someone for hire. For many patients with prion disease, the main carer is a fragile spouse who may have his or her own health problems. Even in cultures where care of the ill is the anticipated responsibility of family members, the complexities of today’s medical regimens, the chronic nature of some disease processes, and the burdens of the carer’s own family or environmental milieu provide an overwhelming challenge. Carers have special needs for knowledge and skills in managing the required activities, access to affordable community resources, and recognition that the care they are providing is important and appreciated. Nurses can assist carers by providing the requisite education and skill training and offering support through home visits; special clinic sessions; telephone access for questions and comfort; innovative strategies such as telephone or computer support, or "chat groups"; and opportunities for respite care.

Social Services

As Service provision differs greatly in all parts of the United Kingdom, the nursing staff at the NPC provides educational support to social service teams in order to advance their knowledge on prion disease and its likely manifestation and progression in their patients. 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.


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 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.

Patients with prion disease experience time-limited disruption of sleep (natural, periodic suspension of consciousness) amount and quality. Sleep is required to provide energy for physical and mental activities. The sleep-wake cycle is complex, consisting of different stages of consciousness: rapid eye movement (REM) sleep, non-rapid eye movement (NREM) sleep, and wakefulness. As persons age the amount of time spent in REM sleep diminishes. The amount of sleep that individuals require varies with age and personal characteristics. In general the demand for sleep decreases with age. Elderly patients sleep less during the night, but may take more naps during the day to feel rested. Disruption in the individual’s usual diurnal pattern of sleep and wakefulness may be temporary or chronic. Such disruptions may result in both subjective distress and apparent impairment in functional abilities. Sleep patterns can be affected by environment, especially in hospital care units. These patients experience sleep disturbance secondary to the noisy, bright environment, and frequent monitoring and treatments. Other factors that can affect sleep patterns include temporary changes in routines such as in travelling, sharing a room with another, use of medications (especially hypnotic and anti-anxiety drugs), alcohol ingestion, night-shift rotations that change one’s circadian rhythms, acute illness, or emotional problems such as depression or anxiety. This care plan focuses on general disturbances in sleep patterns and does not address organic problems such as narcolepsy or sleep apnoea.

Dying and post mortem

Many patients appreciate the opportunity to discuss their end of life wishes with someone. When the NPC have been involved with the patient for some time, then very often we provide that opportunity. We also discuss post mortems and obtain consent as appropriate.

The nurses at the NPC Identify intellectual and emotional responses and behaviours by which individuals, families, communities work through the process of modifying self-concept based on the perception of potential loss.

Anticipatory grieving is a state in which an individual grieves before an actual loss. It may apply to individuals who have received a terminal diagnosis for themselves or a loved one. Intense mental anguish or a sense of deep sadness may be experienced by patients and their families as they face long-term illness or disability. Grief is an aspect of the human condition that touches every individual, but how an individual or a family system responds to loss and how grief is expressed varies widely. That process is strongly influenced by factors such as age, gender, and culture, as well as personal and interfamilial reserves and strengths. The nurse must recognise that anticipatory grief is real grief and that, in all likelihood, as the loss actually occurs, it will evolve into grief based on an accomplished event. The nurse will encounter the patient and family experiencing anticipatory grief in the hospital setting, but increasingly, with more hospice services provided in the community, the nurse will find patients struggling with these issues in their own homes where professional help may be limited or fragmented. This care plan discusses measures the nurse can use to help patient and family members begin the process of grieving.

End of life care

End-of-life and bereavement care delivery remains etched on the memories of bereaved relatives. The importance of delivering this final care appropriately cannot be overemphasised and is recognised by the Department of Health (2008). It said that experiences around the time of death and afterwards can influence grieving and the longer-term health of bereaved people and that suboptimal care impacts negatively on people's bereavement experiences. The DoH end-of-life care strategy (DoH, 2008) highlight the importance of end-of-life and bereavement care and the impact on relatives' experiences.

In June 2014, the Leadership Allicance for the Care of Dying People, consisting of statutory, regulatory and voluntary organisations, published One Chance to Get it Right (LACPD, 2014).  This replaced the Liverpool Care Pathway, that had been found inadequate in an independent review led by Baroness Neuberger.  One Chance to Get it Right set out a new approach to care of the dying, based on the needs and wishes of the person and those close to them.  It identified 5 Priorities for Care, as the new basis of caring for someone at the end of their life: recognising that someone is dying; communicating sensitively with them and their family; involving them in decisions; supporting them and their family and creating an individual plan of care.