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
Abstract
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).
Epidemiology
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)
Kuru
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)
Diagnosis
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).
Method
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.
Findings
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
Pyrexia
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
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.
Recommendations
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.
Conclusion
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.............
