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Nursing Care for Those With CJD

CJD: extending palliative care nursing knowledge/care of six CJD patients within one year in one location...

International Journal of Palliative Nursing, 2000. Vol 6. No. 3.

Clinical Practice
Creutzfeldt-Jakob disease:
extending palliative care nursing knowledge

Beth Bailey, Sanchia Aranda, Karen Quinn, Helen Kean


In a 12-month period six people died from Creutzfeldt-Jakob disease (CJD) in a Melbourne hospice. CJD is a rare neurodegenerative disease, which commonly follows an explosive course unabated into the terminal phase. For the purposes of this article the care of six patients was audited using a retrospective chart review and a focus group was conducted with nurses involved in their care. The nurses faced considerable challenges as they endeavoured to provide comfort and support for these patients. The differences in the illness trajectory of CJD in relation to the more common experiences of illness progression in advanced cancer are discussed in the context of palliative care. This review focuses on the particular care issues of six people with CJD and their families at the end of life. The personal issues experienced by the nurses who provided palliative care are also explored. The need for the future development of guidelines for families and health professionals who care for people with CJD is highlighted.

Palliative care services in Australia are predominantly focused on the care of people with cancer. Staff become expert in the care of these patients but often have limited experience in caring for people suffering from other forms of terminal illness and little access to specific information on their care needs. This article reports on a recent clinical experience of caring for six patients who died in a Melbourne hospice from Creutzfeldt-Jakob disease (CJD) during a 1-year period. It seeks to highlight the clinical care issues arising for these patients and to chart the emerging clinical expertise of the nurses in providing care for these patients.

Creutzfeldt-Jakob disease

Creutzfeldt Jakob disease is a rare degenerative disorder of the central nervous system. It is one of several fatal neurodegenerative diseases (also known as transmissible spongiform encephalopathies) occurring in human and animal species (Table 1), and is characterized by microscopic vacuoles in the brain, astrocytosis and loss of neurones (National Health and Medical Research Council (NHMRC), 1996). CJD was discovered to be infectious in 1968 and initially it was thought to be due to a viral infectious agent. However, it became apparent in the 1980s that a normal host protein, PrP, was an important component for the infectious agent. The novel mechanism by which a protein can itself become infectious and self-replicating has led to CJD and other related disorders being categorized as 'Prion' diseases (an acronym for infectious protein).


CJD as described by Trist (I998) is reported worldwide.
There are three types of CJD:

 · Sporadic: is the most common type and accounts for approximately 85% of all cases. It occurs at random, with no apparent predisposing factors

 · Inherited: accounts for approximately 10% of cases and occurs when family members are predisposed to a genetic mutation associated with the prion protein (PrP) gene

 · Acquired: accounts for about 5% of cases. Iatrogenic transmission has occurred through the use of human cadaveric tissue, for example, dural grafts and pituitary hormone therapy.

In its sporadic form CJD is mainly a disorder of older people with an estimated incidence of 0.5-1 new case per million of the population per annum. For persons younger than 30 years, the estimated incidence is less than 1 case per 200 million (Martinez Lage et al, 1994). As reported by Chipps and Paulson (1994), no distinct pattern of socioeconomic incidence prevails. In a consecutive series of 230 people with neuropathologically verified CJD, the disease was found to affect men and women with approximately equal frequency, with a peak plateau occurring between the ages of 55 and 75 years (Brown et al, 1986). Familial clustering has been noted to occur in several groups. Chapman and Korczyn (1991) and Korczyn (1991 report that in Libyan Jews who migrated to Israel, CJD occurred at a rate 30 times higher than in the rest of the world. Similar findings have been reported in areas of Slovakia and Chile (Brown et al, 1987).

Creutzfeldt-Jakob disease: extending palliative care nursing knowledge

Table 1. Fatal neurodegenerative diseases

In humans:
Creutzfeldt-jakob disease (CJD)
Gerstmann-Straussler-Scheinker syndrome (GSS)
Fatal familial insomnia (FFI)

In animals:
Scrapie in sheep and goats
Bovine spongiform encephalopathy (BSE)
Feline spongiform encephalopathy in domestic cats
Transmissible mink encephalopathy
Chronic wasting disease in captive mule deer
Spongiform encephalopathy in captive exotic ungulates Adapted from: National Health and Medical Research Council (1996)

Table 2. Transmission: infectivity of various tissues

Level of infectivity in body fluid/tissue

Highest-->Brain, spinal cord, eye, dura mater, pituitary gland

High-->Cerebrospinal fluid

Medium to low-->Spleen, lung, pancreas, liver, lymph nodes, placenta

Unknown but Teeth and gingiva, Dental procedures in which blood treat as -----> and/or gingival tissue are involved should therefore infectious be regarded as a potential risk

Low--> Heart, uterus, skeletal muscle, testis, adipose and connective tissue

Probably low*-->Bone marrow, serum, red blood cells, leucocytes

Undetectable levels  Hair and skin. There is no evidence to suggest that of infectivity-->    Creutzfeldt-Jakob disease (CJD) can be spread by contact with intact skin or from hair and therefore normal infection controls should be adequate. In high risk groupst when open wounds are present maximum infection control procedures should be followed

No reported Tears, saliva, urine, sputum, faeces, body secretions presence of--> infectivity

*Authors' note: although there is no epidemiotogical evidence to support blood transfusion as a major risk factor for CJD, blood should be treated as potentially infectious, particularly in high risk patients.

+High-risk groups include people with proven CJD; people clinically suspected CJD; and all members of a family with familial CJD, Gerstmann-Straussler-Scheinker syndrome (GSS), and fatal familia insomnia (FFI)

Adapted from: National Health and Medical Research Council (1996)

Routes of transmission

The mechanism of CJD transmission is not known, except in the case of iatrogenic transmission, which has occurred through corneal grafting, dura mater grafts, the use of neurosurgical instruments and from contaminated human pituitary hormones. Guidelines produced by the NHMRC of Australia identify levels of infectivity (NHMRC, 1996) (Table 2). The guidelines state that 'it appears prudent to treat blood as potentially infectious, even though there is no epidemiological evidence that blood transfusion is a major risk factor for CJD'. There is no evidence that CJD can be transmitted through normal social contact (NHMRC, 1996). Outbreaks of bovine spongiform encephalopathy (BSE) in the UK have been linked to scrapie in sheep and are thought to arise from food contamination (NHMRC, 1996). The epidemic appears to be under control now that the source of contamination has been removed (NHMRC, 1996)


CJD is diagnosed by clinical and neuropathological examination. While a conclusive diagnosis may only be made by microscopic examination of brain tissue, Drury and Beydoun (1996) reported that a confident clinical diagnosis could be reached when certain clinical features occur. The clinical features are rapidly progressive dementia, myoclonus, and cerebellar and visual dysfunctlon in the context of periodic sharp wave complexes on electroencephalogram. Imaging techniques tend to be non-specific, but are useful in excluding other causes of subacute dementia. The cerebrospinal fluid is usually normal but a mild protein elevation may occur. Hsich et al (1996) reported that the 14-3-3 brain protein in cerebrospinal fluid is a marker for the transmissible spongiform encepbalopathies. However, this test was positive in only two of the six cases reported here just before death.

Clinical course

Most people with CJD have a comparatively rapid clinical course which terminates in death within a few months of onset. Zeidler et al (1997) reported that sporadic CJD has a mean age at onset of 65 years with only a few reported cases aged less than 30 years. The mean age at onset of acquired new variant CJD (nvCJD) (Figure 1), which is linked to BSE, is 29 years. The median duration of illness in nvCJD is long when compared with sporadic CJD (14 and 4.4 months respectively). The clinical features of CJD are outlined in Table 3.

Currently there is no specific treatment to eradicate the infectious agent or to slow the progression of CJD. Medical management is directed towards provision of supportive care and symptomatic treatment.

Review process

Between April 1998 and May 1999, six people died from CJD in a Melbourne hospice. These cases, which represented an unusual cluster of CJD, were reviewed to International Journal of Palliative Nursing, 2000, Vol 6, No 3 examine the care issues for each person and his/her family as part of the unit's quality assurance programme. Thc families of the patients were not involved in this review; however, a study of their experiences is planned. Thc central goal was the enhancement of care for people with CJD and their families.

Figure 1. Coloured magnetic resonance imaging (MRI) scan of the brain of a 17-year-old male with new variant CJD. The two red areas in the centre are the diseased thalamus (Photograph: Simon Fraser/Royal Victoria Infirmary. Newcastle Upon Tyne/Science Photo Library).


 The review was divided into four discrete activities:
 · A literature review to identify previous analysis of the terminal care of people with CJD and to describe the pathophysiology and clinical features
 · A retrospective chart audit of the six cases to describe the clinical course of their illness and to identify clinical care needs
 · Accessing an internet chat-group for people with personal experience of people with CJD and their families was used as a proxy for not including the families of the patients in this review and also to provide information on family issues
 · A focus group was undertaken with the relevant nursing staff to discuss their experience of providing care and support for these patients and their families.

Four key care issues were identified:

1. Physical issues in caring for the person with CJD
2. Psychosocial issues in caring for the person with CJD
3. Issues in caring for the family
4. The personal issues experienced by the nurses caring for these people and their families.


Literature review It was apparent on reviewing the literature that most articles on CJD focused on the clinical and disease aspects and not the people experiencing it. Specifically, articles related to research and opinion on sporadic, inherited, and acquired CJD; incidence, duration and presenting symptoms; and transmission and infection issues (Brown et al, 1984, 1986, 1987; Fradkin et al, 1991; Tateishi and Kitamoto, 1993; Martinez Lage er al, I994; Zeidler et al, 1997). Two single case reports were located in nursing journals, both addressed issues surrounding the nursing care of people with CJD (Brazell, 1997; Rotkoff et al, 1997). On reviewing the palliative care literature there was no information on the illness trajectory care issues or the experience of people and their families.

Internet contact

An invitation was placed on the Internet web message centre inviting responses from people with personal experience of thc Issues regarding CJD. it was explained that the information would be used to help professionals improve the care provided for these patients. Twenty responses were received from around the world. The detailed and often distressing personal accounts received correlated closely with the presentation and issues arising from the audit and the focus group. In two cases, reported via the Internet, the family member with CJD was admitted to a psychiatric hospital. LD (internet, 1998) told of the family experience when her father was transferred to a psychiatric hospital after 1 month in hospital. On arrival, she noticed the diagnosis of CJD on a form. Staff at the psychiatric hospital gave little explanation except to say that CJD had to be confirmed at postmortem. The family members felt helpless as they

Creutzfeldt-Jakob disease: extending palliative care nursing knowledge observed their father suffering from pain and other symptoms, and ultimately his death in the psychiatric hospital. Another Internet respondent (DM) described the experiences of his family members as they accompanied their mother during a 6-month period from the occurrence of the first symptom to death from CJD (Internet, 1998). The observations of DM and other respondents are included in the discussion.

Table 3. Clinical features of Creutzfeldt-Jakob disease

Initial presentation may include some of the following symptoms:
Rapid progressive dementia, including memory loss, disorientation
Impairment in judgment
Lack of coordination
Visual changes (may progress to cortical blindness)
Speech, swallowing disturbances
Behavioural changes
Emotional lability including high anxiety
Ataxic gait
Dizziness, headaches
Involuntary muscle spasms, myoclonus (may be unilateral)

As the illness progresses the aforementioned symptoms may be more pronounced, especially the following:
Mental deterioration
Extreme difficulty in communicating and swallowing
Weakness in all extremities, inability to ambulate
Bladder disturbance, incontinence or retention
Bowel disturbance usually causing constipation
Muscle spasms may progress to generalized myoclonus
Sensory disturbance, hypersensitivity to loud noise, bright lights, movement, and to touch
Pyramidal signs including spasticity, hyperrefiexia and abnormal posturing Seizures, focal and/or major

Terminal phase is usually marked by the following:
Diminished level of consciousness progressing to coma
Severe myodonus, seizures
Changes in respiratory function which may include loud grunting
Abnormal posture which may progress to decorticate and decerebrate postures
Death is usually due to infectious and respiratory complications

Retrospective chart audit

In the cluster of six people reviewed in this audit no risk factors for CJD were identified; the clinical presentation of each person was sporadic CJD. Comparative data from the audit indicated that the mean age of the group was 61 years and the duration of the illness was 5.5 months. A profile of the six patients is provided in Table 4. Rapid deterioration in clinical condition, movement through multiple care settings and late diagnosis were common to all and occurred within a short period of time. These patients moved through an average of 4 care settings before admission to the hospice. Case D experienced 5 care settings in 3 months. This results in a confusing and stressful experience for the person and his/her family. Another important consideration is the short duration from clinical diagnosis to hospice admission; the mean was 9 days (range was 1-21 days). These statistics highlight the difference between the illness trajectory in CJD and the more common progression in advanced cancer and terminal non-cancer illnesses. Brown (1997) described CJD as 'a very demanding disease, rather like Alzheimer's disease in fast forward'. The distressing symptoms and rapid deterioration make CJD a particularly devastating disease for all those involved including the health professionals.

Physical care

Pyrexia: was a common symptom in these patients with wide fluctuations in temperature occurring even in the absence of diagnosed infection. While most patients developed pneumonia in the late terminal stage, high temperatures and profuse sweating were evident early in the admission. Pyrexia, may be due to the effects of the disease on the central nervous system. Nursing assessment was based on temperature measurement, observation and touch. The nurses noted that pyrexia caused or exacerbated agitation and restlessness in these patients. Several measures were adopted to provide comfort. Paracetamol suppositories were not always effective in controlling temperature. Environmental measures such as tepid sponging and the use of fans reduced the temperature and enhanced patient comfort.

Dysphagia: was evident in all six cases, five being severely affected. Management of dysphagia necessitated careful assessment of the swallowing reflex to reduce the risk of aspiration. In the case of impaired swallowing, use of thickened fluids in conjunction with appropriate positioning proved effective. One person, receiving nutrition via a gastrostomy, tolerated these feeds until reflux and aspiration became a problem. Subcutaneous fluids were given in three cases to provide comfort when the symptoms of dehydration were assessed to exacerbate discomfort. Regular mouth care was an important comfort measure but was frequently difficult because of myoclonic jerking. Maintaining nutrition and hydration was important to all the families, especially when they were unwilling to accept that their relative was dying. O'Connor (1999) emphasizes that the reactions of family and

Creutzfeldt-dakob disease: extending palliative care nursing knowledge friends need to be monitored closely to ensure that the process of dying is understood. Offering reassurance that an inability to eat or lack of interest in food is part of the dying process was difficult in this situation because the rapid deterioration meant several families were unable to accept that their relative was dying.

Myoclonic jerking: was a significant issue in all cases. It occurred spontaneously and sometimes in response to stimulation. Pyramidal signs of spasticity and hyperreflexia were evident to varying degrees in the majority of cases. Extrapyramidal signs such as seizures, both focal and major seizures, were problematic in some cases.

Nursing assessment was based on frequent observation and evaluation of the efficacy of pharmacological interventions. Patient management aimed to:

   · Minimize touching, turning and movement

   · Promote a calm, quiet approach and create a quiet environment
   · Relax the muscles using benzodiazepines, e.g. clonazepam. Nurses commented on the difficulty in achieving therapeutic levels of clonazepam to control myoclonus without oversedating tbe patients. DM, an Internet (1998) respondent, described the struggle his family members experienced in caring for their mother:

'The myoclonus came and went sporadically, interrupting sleep and sometimes bringing on episodes of anxiousness and terror. Finding the right dose of haloperidol and clonazepam was difficult and basically just trial and error. When the initial dose of haloperidol left our mother sleeping through the day we felt bad to have robbed her of even that one day's experience. At the same time, we wanted to avoid the terror attacks and for the rest of her illness struggled with the balance between keeping the attacks and myoclonus in check, while trying to keep her as awake and aware as possible.'

Sensory disturbance: hypersensitivity to touch and environmental noise. There was evidence of heightened sensitivity in all cases but this varied greatly in intensity between individuals. Nurses based their assessment on their own and the families' observations. Reactions occurred to touch, loud noise and loud music, producing immediate signs of distress and agitation. Management included:

Careful planning to reduce the need for touching the person· Controlling environmental activity and noise, which entailed explaining the situation to all personnel who accessed the areas where these patients were being nursed· The use of sort lighting.

Table 4. Comparative data of the six people with Creutzfeldt-Jakob disease (CJD) admitted to a hospice in Melbourne from April 1998 to May 1999

 Case Case Case Case Case Case
 A B C D E F        Mean
 (a) M M E F F M
 (b) 65 57 59 67 63 59 61
 (c)  Australia Italy   Indian  Bosnia Singapore Italy
       born in
       East Africa
 (d)     15 7 28 11 22 3 14
 (e) 5 2 9 3 9 5 5.5
 (f) 3 4 6 5 3 4 4
 (g) 14 5 8 7 I 21 9

 (h)     Post    Post     CSF     CSF     Brain   Post
         Mortem  Mortem  14-3-3  14-3-3  biopsy   Mortem
                         5 week  result  1 month
                         before  obtained before
                         death   after    death

CSF=cerebrospinal fluid; *protein test sensitive
(a)= Sex
(b)= Age in years
(c)= Country of birth
(d)= Length of stay in hospice (days)
(e)= Duration of illness from first symptom to death (months)
(f)= Care settings from first symptom to admission
(g)= Duration from clinical diagnosis to hospice admission (days)
(h)= Mode of confirmation of CJD

Creutzfeldt-Jakob disease: extending palliative care nursing knowledge

'Certainly, the potential for pain exists for people with CJD given the presence of spasticity, hyperreflexia, and bladder and bowel disturbance. The issue of pain was a particular concern for nurses who were experienced in pain assessment in the context of cancer...'

The families were all aware of the need to provide a quiet, restful environment. For case A, playing soft, familiar, classical music produced an obvious calming effect. The family of case C found that massage promoted relaxation in their mother in the early stages of the disease, but that it caused agitation when sensory disturbance increased later in the disease process. Several Internet respondents commented on the calming effect of soft familiar music. Nurses learned to approach the person in a quiet, calm manner and gently explain every action before starting to touch or perform care. Nurses commented on the 'startle response' which was evident in many patients. This response is referenced in the literature and may be due to heightened sensitivity (sensory disturbance) or to the startle response if the person is cortically blind. It is important to ascertain if cortical blindness is present by testing whether the person can track movement with his/her eyes. In addition, the person may be more aware of activity on one side of the body than the other as illustrated by IS (Internet, 1998) who said:

'I wish my family and I knew enough to sit on my father's left side
during visits. It was almost too late when we realized he responded more to us when we sat on his left side.'

Shortness of breath: was a common symptom thought not to have resulted from pneumonia, in some cases, it appeared to be exacerbated by the neurological complication of muscle spasms in the neck and face. In the late terminal stage, when coma occurred, decorticate and decerebrate postures were observed in several cases. Shortness of breath was associated with colour changes, indicating varying degrees of cyanosis. In some cases, shortness of breath increased when the person was moved or turned, indicating the need for careful positioning and frequent observation. Nurses commented on the difficulty in achieving a comfortable position for the person. Small regular doses of morphine were helpful in relieving shortness of breath. LP (Internet, 1998) explained:

'My father was given morphine near the end, this slowed his breathing down, took care of the physical pain and helped with the anxiety.'

The use of morphine for comfort requires careful introduction when family members are having difficulty in accepting that their relative is dying.

Incontinence: was universal and caused agitation, restlessness and distress. In the majority of cases, the sensory, disturbance and reaction to wet bedclothes and to being touched appeared to be the cause of the patient's agitation. Management was directed towards the use of catheters and condom drainage to prevent wet bedclothes and reduce the need for frequent bed changes, touching and turning the patient.

Constipation: occurred because of reduced fluid intake and impaired mobility combined with the effects of neurological disturbance on the bowel. A daily bowel chart helped to monitor constipation, occasionally supplemented by physical examination including rectal examination. Most patients could not swallow laxatives, even early in their admission. Complexity surrounded bowel management decisions because both rectal examination and rectal intervention had the potential to exacerbate myoclonus and agitation. A constant balance was required between over-intervention and the distress associated with unrelieved constipation.

Pain: assessment was a difficult issue. Understanding the underlying disease process and the potential causes of pain was important for accurate patient assessment and management. Certainly, the potential for pain exists for people with CJD given the presence of spasticity, hyperreflexia, and bladder and bowel disturbance. The issue of pain was a particular concern for nurses who were experienced in pain assessment in the context of cancer but assessment of pain in CJD patients was made more complicated by communication impairment. In some cases a small, trial dose of morphine was used to assess whether symptoms of restlessness and agitation were due to pain, however, the results were not always conclusive. Two families did not want morphine used because of their concerns that morphine would hasten death. This reaction was not unexpected given the public debate about euthanasia including misinformation about the uses of morphine, the lack of precise pain assessment data, the patient's rapid deterioration and concerns over hastening death when relatives continued to hope for the person's survival.

Infection control: CJD is not considered to be highly contagious, with most risk identified in relation to brain matter and CSF

Creutzfeldt-Jakob disease: extending palliative care nursing knowledge

'The families had all experienced an intense period of uncertainty and multiple investigations associated with efforts to determine the diagnosis. These families were shocked, anxious and upset by the rapid deterioration in the patients condition.'

(NHMRC, 1996). While there is no evidence to support blood contamination, all situations of blood handling should be managed using universal precautions. Confusion exists over infection protocols as illustrated in the description of care by Brazell (1997) who mistakenly describes universal precautions as wearing gloves, gowns and face shields in all situations, rather than in association with high-risk activities such as during an episode of bleeding. Rotkoff and colleagues (1997) describe the use of full precautions (gowns, gloves, masks and goggles) when caring for the person with CJD who was bleeding rectally and had some bloodstained emesis. The use of full precautions continued until death and well beyond cessation of bleeding. LP (1998), an Internet respondent, stated:

'When CJD was considered a possible diagnosis, we had to wear gowns and gloves whenever we visited my dad.'

Such a reactive protocol sits uncomfortably with palliative care principles of providing empathetic and quality care. The nurses in this audit followed universal precaution guidelines and used full precautions only in relation to CSF and the removal of staples
 following brain biopsy.

Psychosocial issues

While people with CJD are often considered to be unaware of what is happening to and around them because of associated dementia, the nurses looking after these six patients felt that five of the patients demonstrated varying levels of awareness. Assessment was based on observed responses to requests, environmental stimuli and family interactions with the person. Specific examples included: attempts to respond to requests in a manner that indicated a level of understanding; appropriate tears and laughter in response to personal family stories; and successful instruction of two families in the use of blink communication. Nursing care centered on:

·Always assuming that the person could hear and understand what was being said to him/her· Frequent observation and quick response to perceived requests and needs· Ensuring quiet surroundings· Promoting comfort measures including the presence of someone to sit with the person· Judicious use of sedation to reduce anxiety and agitation following careful discussion with the family.

Many comments from the Internet respondents referred to the unwillingness of health professionals to accept that people with CJD were aware of their surroundings, despite strong family beliefs otherwise and this caused considerable distress.

Family issues

Family distress was high in all six cases. In every situation the person had moved through many care settings (mean=4) across a mean illness duration of 5.5 months. The six families appeared confused and disbelieving of the diagnosis, which was often made just before hospice admission. The families had all experienced an intense period of uncertainty and multiple investigations associated with efforts to determine the diagnosis. These families were shocked, anxious and upset by the rapid deterioration in the patient's condition. All families had accessed the Internet to obtain information about this rare, terminal illness. The widely circulated conspiracy theories about a medical cover up of the extent and cause of CJD added to their confusion and distress. The conflicting information families obtained through the Internet and across thc multiple care settings they encountered hindered the hospice nurses' efforts to develop helping trusting relationsbips with each family. Added to this were their fears of inheriting CJD, given thc absence of an identifiable cause in all six patients. Management was based on listening to the families, which enabled the nurses to gradually develop an understanding of each family's experience. Misinformation and false hopes made the opportunities for helpful information exchange difficult. The nurses observed that the families were often too distressed to take in much information. Nurses facilitated family involvement in the patient's care. A caring relationship had to be established in a very short time with these families (the mean length of stay was 14 days; range was 3-28 days).

In several cases families were desperate for help and put pressure on staff for treatment to prevent their relative from dying. The family's struggle to accept the diagnosis occurred during the patient's rapid deterioration. Pollard et al (1999) argue that each person and each family will experience awareness about the changing nature and progression of a disease at different times and in different ways. New realizations and readjustments occur with time. The art of good palliative care is to support

Creutzfeldt-Jakob disease: extending palliative care nursing knowledge the clinical and psychosocial needs of the person and family throughout these periods of uncertainty, and change.

Nurses' experience

The nurses were challenged by their limited knowledge of the CJD disease trajectory in providing support to these patients and their families.
Some of the comments from the nurses are listed in Box l.

Box I. Nurses' comments from the focus group Feelings of helplessness

'It felt like working in the dark, not knowing what to expect. Unlike the cancer experience there is a lack of a clear understanding of the trajectory of the illness in CJD; so much uncertainty. The majority of people admitted for palliative care (people with cancer) and their families have received relatively consistent explanations from health professionals over time.'

'The deterioration was so rapid, we treated issues as they arose, we were basically reacting and unable to anticipate what problems might arise and adopt preventive measures. The families were aware of our lack of knowledge and experience in this area. We were unable to do all the family education that is a normal part of our role.'

'It was so difficult to achieve a comfortable position for these people. We trialed so many different positions and didn't reach the appearance of comfort that we achieve every day with other people in the palliative care setting. Our trust and confidence in our skills were certainly challenged.

'We tried to meet the families' special needs including cultural practices. In one case, we had to assist the family, daily, with ceremonial washing of their ill member. It was incredibly difficult because of the severe myoclonic jerking. The practice was so stressful for all concerned we questioned what we were achieving.'

Feelings of stress, sadness and grief

'Observing the rapid physical changes in these people with CJD was far more stressful than observing the changes in people dying with cancer. All these people had been vibrant, healthy individuals a matter of weeks or a few months before their admission to the hospice. When we looked at the family's photos of their member we were so sad and shocked; the changes were unbelievable.'

'Observing the shock, strain and stress in all the families made us very sad. All the staff felt significant sadness and grief; in each case it was a heart wrenching experience. We worried about these families, wondering how they were coping in their bereavement, many of them were at risk of complicated bereavement outcomes'

The potential for nurses caring for people with CJD at the-end-of-life to experience stress should be recognized and support offered through opportunities for education, debriefing and counseling. Wilkes (1999) reports that nurses must reflect on their practice to become aware of their own stress and what precipitates it, to acknowledge their feelings and talk about them and even to cry. However, nurses also need to reflect on their positive experiences in order to enhance their self-esteem. This review illuminated the nurses' evolving skills in caring for the patient and family within the context of CJD.

This review, while highlighting evolving expertise in the care of people with CJD and their families, also exposed some important gaps in car. Experience suggests that there is a need to develop specific approaches to the care of these patients in the following areas:

Continuity of care: the complexity of care needs associated with each of these six patients suffering from CJD required frequent observation and assessment by a team of nurses 24 hours a day. This complexity was increased by the distress of families already subjected to multiple care settings and conflicting information. The need for consistency in assessment and intervention suggests that a model of care that enhances continuity of care should be adopted. Consideration should be given to the allocation of a primary nurse to communicate with the family to overcome the problems associated with receiving inconsistent information and to enhance observation of the subtle factors exacerbating patient discomfort. However, the primary nurse would require significant support given the physical care needs of the patient and the high levels of family distress.

Family support: the first contact with family members in the palilative care setting should provide adequate time for them to express their feelings of anguish and anger, along with their frustrations and hopes. The families in this review desperately searched for information about CJD and often found conflicting and unhelpful material which added to their confusion and distress. Some of the internet families were distressed on receiving palliative care information that focused on cancer. There is a clearly identified need for appropriate information to be made available for families experiencing CJD. The Internet respondents and Melbourne families experienced significant uncertainty, stress and confusion and had encountered a large number of health professionals in a short time. This review has highlighted the importance at providing a mechanism to help families to work

Creutzfeldt-Jakob disease: extending palliative care nursing knowledge through their experience, including the early involvement of allied disciplines such as pastoral care and social work.

Bereavement support: many of these families were assessed by staff as being at risk of developing complicated grief reactions because of their traumatic experience. Kissane (1998) stated that 'where we identify some risk, the provision of early support through telephone, practice or home visits invokes a preventative approach aimed at reducing further morbidity'. Early involvement of psychological support staff is therefore imperative, given that late involvement of bereavement staff not involved during the patient's illness is often experienced as intrusive (Kissant, 1998). Early involvement also provides support for the nursing staff in meeting the complex needs of these patients and their families.

Clinical care pathways: The common care trajectory of these six patients suggests that a care pathway could be developed to assist nursing, medical and allied health staff to plan care appropriately. The clinical pathway for these patients appears to be very different from the pathways of people dying from cancer or end stage organ failure. In CJD, the person is in a rapidly deteriorating phase until death. This is unlike the more common chronic, remitting illness trajectory seen in the palliative care setting with movement occurring between the deteriorating and stable phases where most people experience a plateau in their illness before the disease progresses to the next phase.


This review of caring for six people with CJD and their families,
 including the observations of palliative care nurses, highlights the complexity of symptom management in the person with CJD and the intense support required by the family. The detailed personal experiences of the internet respondents correlated closely with the issues identified from the audit and the nurses' focus group.
 The literature on CJD focuses on the disease process and clinical
 progression not on the people involved. This review offers new insights into the clinical care of people with CJD and their families. There is a need to develop clinical guidelines for health professionals and families who are involved in caring for people with CJD. Health
 professionals, who understand the rapid trajectory of CJD illness,
 will be better able to provide successful palliation and skilled care
 for the person and support for the family.

 The authors would like to thank all those involved in this project, but
 in particular the internet respondents who contributed their personal
 experiences to our learning. The help Mrs Kerry Seipolt, Nursing Unit
 Manager, Caritas Christi Hospice in requesting this audit is gratefully
 acknowledged. The administrative support of Ms Linda Devilee is also acknowledged.............

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