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Prognosis in Chronic Diseases
Jennifer M. Kapo, MD, and David Casarett, MD, MA
Discussing a patient’s prognosis is a
significant challenge for many clinicians. This conversation is particularly challenging with patients who
have chronic illnesses other than
cancer, whose prognosis is especially
difficult to estimate. As a result, clinicians are often uncertain when they
should discuss end-of-life care options, and how they should help patients and their families plan for the
future. This article will review existing data to help clinicians estimate
prognosis in chronic illness. In particular, we will focus on heart failure, dementia, cancer, and chronic
obstructive pulmonary disease. Practical strategies are suggested to help
clinicians initiate and structure discussions about prognosis. (Annals of
Long-Term Care: Clinical Care and
Aging 2006;14[2]:18-23)
Drs. Kapo and Casarett are assistant
professors in the Division of Geriatric
Medicine, University of Pennsylvania
School of Medicine, Philadelphia. Dr.
Casarett is also at the Center for Health
Equity Research and Promotion at the
Philadelphia VAMC, Philadelphia, PA.
INTRODUCTION
Many older adults will die from complications of chronic diseases such as
congestive heart failure (CHF), dementia, and chronic obstructive pulmonary disease (COPD). In 2001, heart disease was the leading cause of
death in the United States, with chronic lower respiratory tract illnesses,
dementia, and renal disease ranked in the top ten, according to the Centers for Disease Control and Prevention statistics.1 Rather than dying suddenly, patients with these diseases often experience a gradual decline in
health punctuated by exacerbations of their disease.2 These patients may
live for years with multiple serious illnesses.
On one hand, this illness trajectory can help clinicians to initiate endof-life discussions because a slow period of decline with multiple intercurrent events offers numerous prompts and opportunities. However, this
extended illness course also makes prognosis very difficult to predict.
Moreover, this uncertainty in estimating prognosis can be particularly difficult to explain to patients and families.
These complexities in prognosis estimation and communication are
significant. While numerous studies have shown that patients would like
to discuss prognosis and advance care planning with their health care
providers,3 few patients have the opportunity to do so. This article will
review the answers to the following questions about discussing prognosis
and end-of-life plans with chronically ill patients:
• Why should clinicians discuss prognosis and end-of-life plans?
• How well do clinicians make and discuss prognostic judgments?
• When should clinicians discuss prognosis and end-of-life plans?
WHY SHOULD CLINICIANS DISCUSS PROGNOSIS
AND END-OF-LIFE PLANS?
There are several compelling reasons to discuss prognosis and end-of-life
care plans. For instance, patients who understand their prognosis can use
18
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— CHRONIC DISEASE —
this information to make informed health care decisions that are congruent with their preferences. Specifically, patients are able to make choices that are more
consistent with their medical circumstances.4 Patients
who are aware that they have a limited prognosis are
also better prepared to make practical decisions about
living arrangements, financial matters, and other common concerns. Prognostic information also allows
patients to seek spiritual support, if desired, and can
give patients a chance to achieve reconciliation and closure, and to say goodbye to loved ones.
There are also several ways in which discussions about
prognosis can foster better care, apart from the information that the clinician presents. For instance, these conversations may provide an opportunity for advance care
planning and anticipatory guidance. These conversations
can also open the door to related discussions about
options for palliative care, including hospice. Finally,
conversations about prognosis can give patients and families an opportunity to share their own fears about death,
and to seek reassurance from clinicians.
From the clinician’s perspective, these conversations
allow the clarification of expectations about prognosis
and illness trajectory. Most importantly, these conversations help the clinician to understand patients’ values
and preferences for care at the end of life, and to guide
them in identifying and communicating with surrogate
decision makers.
HOW WELL DO CLINICIANS MAKE AND
DISCUSS PROGNOSTIC JUDGMENTS?
It is clear that clinicians are uncomfortable making estimates about prognosis. In a survey of primary care
physicians, the majority expressed that it was stressful
and difficult to make prognosis estimations.5 Furthermore, 80% felt that patients and families wanted too
much certainty with the prediction, and that this
adversely affected clinicians’ comfort in communicating prognosis.6
Available data indicate that this lack of comfort is justified and that clinicians are not accurate in predicting
prognosis. Several studies have found that physicians
have a tendency to overestimate life expectancy and are
19
reluctant to provide information about prognosis to their
patients. In one study, 343 experienced physicians were
asked to give survival estimates for 468 terminally ill
patients at the time of hospice referral.7 Although 20%
gave accurate estimates within ± 33%, on average the
clinicians overestimated prognosis by a factor of 5.3.7
Not only do physicians overestimate prognosis, but
they also consciously communicate overly optimistic
prognoses to their patients. In another study, 258
physicians referred 326 patients to hospice.8 The physicians were asked to give survival estimates for the
patients (called the formulated prognosis). They were
then asked what prognosis they would communicate to
the patient and family (called the communicated prognosis). The physicians reported that they consciously
overstated the prognosis by a factor of 1.2. When the
actual survival was compared to the communicated
prognosis, the investigators found that physicians had
overstated prognosis by a factor of 3.5.8
WHEN SHOULD CLINICIANS DISCUSS
PROGNOSIS AND END-OF-LIFE PLANS?
Ideally, clinicians should discuss these issues routinely
with all of their patients. However, such blanket statements provide little useful guidance to clinicians.
Instead, it is useful to consider reasonable triggers for
conversations about prognosis.
One helpful strategy to use as a clinician attempting
to determine prognosis is to ask yourself: “Would you
be surprised if [the patient] died within the next year?”9
If the answer is “No,” then this should prompt the
provider to begin to discuss end-of-life care with the
patient. These are patients who are likely to have a limited prognosis, and for whom prognostic information
is likely to be particularly relevant. Although the answer
to this question offers a useful guide in identifying
patients with a limited prognosis, it does not provide
specific prognostic information and does not help to
structure a discussion with a patient.
Models have been developed to aid in prognostication. However, even the best prognostic models perform imperfectly. For instance, the Study to Understand Prognosis and Preferences for Outcomes and
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Risks of Treatment (SUPPORT) developed prognostic
models and supplied patients’ prognosis estimates to
their physicians.10 The model—which combined variables from the Acute Physiology, Age, and Chronic
Health Evaluation (APACHE II), with disease-specific
variables such as low ejection fraction in CHF and a
low forced expiratory volume in 1 second (FEV1) in
COPD—was only able to identify 50% of the patients
who died within 6 months, and performed no better
than physician estimates. However, physicians who
used the model made more accurate predictions, which
suggests that using models in combination with clinical acumen will increase accuracy in prognostication.10
In another study, researchers examined the data from
the SUPPORT study to determine whether criteria
developed by the National Hospice and Palliative Care
Organization (NHPCO)11 can distinguish patients with
non-cancer diagnoses who have a prognosis of 6 months
or less, and are therefore hospice-eligible. Although the
NHPCO criteria identified over half of the patients who
died within 6 months, the criteria failed to identify a significant number of patients who died soon after hospital
discharge.12 Therefore, when presenting prognosis estimates to a patient and his or her family, it is important
to state that although it is known that large groups
behave a certain way, every individual’s illness course is
different. There is no perfect predictor of prognosis estimation in chronic disease, and the studies cited below are
imperfect. Nevertheless, there are a variety of disease-specific signs and symptoms that can help to identify
patients who have a limited prognosis.
Cancer
Compared to the illness trajectories of organ failure
syndromes, such as renal failure and congestive heart
failure, the illness trajectory of cancer has been fairly
well described. Cancer survival can be considered by
using two major categories: performance (functional)
status, and signs and symptoms. Performance status is
a measure of a patient’s functional status.
Two commonly used scales are the Karnofsky Performance Status Scale13 and the Eastern Cooperative Oncology Group Performance Status (ECOG PS).14 Both
scales have been validated for the evaluation of survival in
20
patients with cancer. A Karnofsky score of < 50 indicates
that a patient requires considerable assistance and frequent medical care, and has a survival that is likely to be
less than 6 months.13 Similarly, an ECOG PS score of >
2 identifies a patient who spends more than 50% of waking hours in bed, and has a similar prognostic outlook.14
Although investigators have improved on these models by
incorporating additional data,15 they continue to offer
substantial predictive value by themselves.
Large studies of patients with cancer who have died
have identified easily-recognizable symptoms that predict a survival of 30 days or less. These symptoms
include anorexia, dysphagia, confusion, and dyspnea.16
It is important to note, however, that symptoms like
anorexia may be very common in patients with
advanced cancer at all stages.17,18 Therefore, although
they may be associated with mortality, these common
symptoms offer limited specificity. Hypercalcemia,
when found in cancers other than multiple myeloma or
breast cancer, is also a predictor of end-stage disease.
Malignant pericardial effusions and carcinomatous
meningitis, although less common than the signs listed
above, also indicate that the disease is in its last stages.19
Taken together, these data provide substantial information to clinicians. Performance status should be used
as a routine screening tool for all patients with cancer.
The clinician should be alert for the presence of endstage findings, such as carcinomatous meningitis and
pericardial effusions.
Chronic Obstructive Pulmonary Disease
Patients who die from COPD often experience an
unrelenting decline in pulmonary function over several years. Declines in the FEV1 have been shown to be
inversely associated with survival in hospitalized
patients. An FEV1 of < 1.0 or a decline in the FEV1 of
* 40 cc in 1 year predicts prognosis of less than 1 year
in the majority of patients.20
Other characteristics may be helpful as well. In a retrospective study of elderly patients with COPD with
an FEV1 of < 1.0 who were admitted to an intensive
care unit, 30% died while in the hospital, and almost
60% died within the next year.21 Studies of ambulatory patients have found that the FEV1 is less predictive
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of mortality than a poor functional status, as measured
by the Manchester Respiratory Activities of Daily Living scale and peak VO2.22,23 An elevated carbon dioxide (CO2) level, cardiovascular comorbidities, as well as
steroid and oxygen dependency, also predict poor survival.24 A recent study found that a multidimensional
index of four patient factors, including body mass
index, FEV, dyspnea, and exercise capacity, was more
accurate a prognostication than FEV alone.25
Congestive Heart Failure
Like other chronic diseases such as renal and hepatic failure, patients with CHF experience an illness trajectory that is marked with exacerbations resulting in
hospitalization followed by either stabilization or death.
In addition, many patients with CHF die suddenly of
arrhythmias. This makes prognostication particularly
challenging. One common approach is to consider the
patient’s New York Heart Association (NYHA) classification, which is a scale ranging from 1-4 describing the
presence or absence of symptoms with varying degrees
of exertion. A score of 4 (NYHA IV) describes a patient
who develops symptoms with minimal exertion and at
rest. In several large studies, these patients had an average 4-year survival of less then 35%.26 Functional status, as measured by oxygen consumption during exercise testing, is also used to identify patients with poor
prognosis; however, this is not currently widely available outside of research.2,27
Physiologic measures may be equally useful. An
ejection fraction of < 30% has been found to be predictive of poor survival in some, but not all, studies.
Other predictors of poor prognosis in heart failure
include tachycardia, a serum sodium of < 134 and a
creatinine of > 1.8.28 Brain natriuretic peptide has been
identified as a potentially useful serum marker for
prognosis in CHF; however, further study is needed to
identify useful thresholds for prognostication.29 Additionally, the use of implantable ventricular devices may
considerably improve the prognosis of some patients.30
Although physiologic measures have been identified
as useful guides, the clearest indicator of limited prognosis is the NYHA IV classification, identifying a
patient who has symptoms of heart failure at rest.
21
Future research is needed to identify clinically accurate
prognostic indicators in CHF.
Nursing Home
Residents of nursing homes represent a heterogeneous, frail population of patients whose multiple illnesses pose a particular challenge to clinicians attempting
to estimate prognosis. Several retrospective studies are
helpful in providing signs and symptoms that identify
patients who have poor prognoses. These include a drop
in serum albumin by * 1.0 mg/dL in 1 year,31 new agitation in a patient without dementia,32 and new decubitus ulcers.33 Decline in functional status is also an important predictor of poor prognosis, particularly the decline
of two or more activities of daily living (ADLs) in 1
year.34 Two recent studies used data from the Minimum
Data Set (MDS) to create mortality prediction models.35,36 One of the prediction models—which consisted
of patient factors, including functional status, weight
loss, age over 88 years, low body mass index (BMI), swallowing dysfunction, male sex, and the presence of CHF
and/or dyspnea—predicted 1-year mortality.
Four predictors may be used to identify nursing
home residents who may die in the next 12 months:
decline in albumin by > 1.0 mg/dL in 1 year, the development of new agitation, the development of a new
decubitus ulcer, and decline of two ADLs in 1 year.
Dementia
The most widely studied guidelines for prognosis in
dementia focus on functional decline. The Functional
Assessment Staging Tool (FAST) is a staging system
that describes the progression of functional decline in
patients with dementia in a series of seven stages. In validation studies of the FAST staging system, patients at
stage 7 (language limited to several words and dependent in all ADLs) have a prognosis of less than 1 year.
This is generally the functional level at which patients
become eligible for hospice. Earlier in the illness course,
loss of ability to ambulate further identifies a population with poor prognosis.37
Hospitalization of patients with dementia may also
predict poor prognosis. In a study of patients with
dementia who were admitted to the hospital with a
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diagnosis of a hip fracture or pneumonia, over half died
within the 6 months postdischarge.38 A recently published study of nursing home residents with advanced
dementia used data routinely collected in the MDS to
create a mortality prediction model.35 The presence of
cancer or CHF, dependence in ADLs, age over 83
years, and male sex were important predictors of 6month mortality.35 Although acute illnesses such as hip
fractures and pneumonia predict poor prognosis,
assessment of performance status remains the most
accurate tool used to predict the prognosis of individuals with dementia.
LAST DAYS OF LIFE: SYNDROME OF
IMMINENT DEATH
It is impossible to definitively determine when a patient
is actively dying; however, there are signs and symptoms that indicate that a patient may be imminently
dying. Once the signs and symptoms of imminent
death are observed, the family can be notified so that
they can prepare for the impending loss. There are
obvious practical benefits to knowing when a patient’s
death may be imminent (ie, allows travel from a distance), as well as psychological benefits, including a
chance for closure and an opportunity to say goodbye.
Signs that suggest imminent death include:
decreased responsiveness with environment and
decreased consciousness; decreased food and water
intake, with resultant diminished urinary output; significant changes in skin color and temperature (including cooling of the extremities and the appearance of
livedo reticularis); and dwindling pulse and blood pressure. Dying patients often develop swallowing dysfunction resulting in retained secretions and “rattles” in the
chest, relaxation of facial muscles, and breathing
changes, including Cheyne-Stokes respiration and periods of apnea.39-43 In one study examining the most
common signs and symptoms during the last 48 hours
of life per the family’s recall, the most frequently reported were noisy breathing, urinary incontinence and
retention, and pain and restlessness.40
Clinicians can use these known signs and symptoms
to predict imminent death and identify symptoms in
22
need of palliation. In addition, many families benefit
from having some basic understanding of what to
expect in the last hours of their loved one’s life. In fact,
many hospices and long-term care facilities routinely
provide this information to families.
SUMMARY
Many patients and their families value conversations
about prognosis and end-of-life care. Both clinicians and
patients clearly benefit from timely and effective communication. However, few conversations occur before
the patient is in the end stages of disease. Asking ourselves the question, “Would I be surprised if this patient
died within the next year?” can prompt providers to initiate these conversations about prognosis and treatment
goals. In addition to disease-specific characteristics (ie,
decline in FEV, COPD), performance (functional) status has proven to be an accurate guide for predicting
prognosis, particularly for patients with congestive heart
failure, cancer, or dementia. Use of known prognosis
predictors may help health care providers to improve
their prognostication skills and decrease the level of discomfort clinicians experience when faced with discussing
prognoses with their patients. ✧
The authors report no relevant financial relationships.
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