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Use of the Word “Crisis” in Sickle Cell Disease:
The Language of Sickle Cell
Todd L. Savitt, Ph.D., Department of Bioethics and Interdisciplinary Studies, The Brody School of Medicine at East Carolina
University, Greenville, NC, (Historical Perspective); Wally R. Smith, M.D., Department of Medicine, Virginia Commonwealth
University, Richmond, Virginia, (Clinician and Researcher Perspective); Carlton Haywood Jr., Ph.D., M.A., The Johns Hopkins
Berman Institute of Bioethics, Baltimore, MD (Patient Perspective); Melissa S. Creary, M.P.H., Division of Blood Disorders, National
Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA (Introduction)
What’s in a Name?
Financial disclosures: Creary and Savitt received no funding or sponsorship
in the development of this submission. Smith was supported in part by
funding from:Virginia Basic and Translational Research Program in Sickle
Cell Disease. 1U54HL090516, National Heart, Lung, and Blood Institute; Sickle
Cell Disease Clinical Research Network, National Heart, Lung, and Blood
Institute 1U10HL083732; Pain in Sickle Cell Epidemiology Study, 1 R01 HL 64122,
National Heart, Lung, and Blood Institute, NIH. Haywood was supported by
a Career Development Award from the National Heart, Lung, and Blood
Institute (#1K01HL108832-01).
Acknowledgement: The authors acknowledge the assistance of Mr. T.J.
Brown, President of the Sickle Cell Natural Wellness Group, Broward County,
Florida, in collecting and compiling the patient information from a survey
conducted on his website, http://sicklecellcantstopme.wix.com/sickle-cellnatural-wellness-group-inc#!
Abstract: Language matters. The words used to name and describe
disease phenomena are a reflection of society. The authors address the
use of the word “crisis” in SCD from sociological, historical, medical, and
patient perspectives. The term “crisis” became associated with sickle cell
disease in the mid-1920s, more than a decade after the first description of
the disease had been published. The term had been used for centuries in
conjunction with fever and as a signifier of severe pain in certain diseases
during the nineteenth century. The application of the term to this new
disease in the 1920s resulted from physicians’ observations of their patients’
urgent situations. Though commonly used by health care providers and
patients today, “crisis” may not be the appropriate term for sickle cell
patients suffering severe pain, because people endure differing amounts
of pain before stating they are “in crisis.” The result can be undertreatment
of the pain or mistrust between physicians and patients about use of
strong (narcotic) pain-relievers. Some patients believe the term is useful in
communicating the severity of their pain and the urgency of their need
for relief from it, especially when seeking care at hospital emergency
departments, while others believe “crisis” does not accurately reflect the
severity or seriousness of their situation.
Keywords: sickle cell disease n crisis n history n pain
Correspondence: Todd L. Savitt, Ph.D., Department of Bioethics and Interdisciplinary
Studies, Brody School of Medicine at East Carolina University, 600 Moye Boulevard,
Greenville, NC 27834 ([email protected])
INTRODUCTION
P
ain is the most common symptom reported by the
89,000-100,000 1, 2 Americans with sickle cell disease
(SCD). The word “crisis” has been used for decades to
describe pain in SCD. Its use however has come under scrutiny
and some question of late, as we have better understood the
epidemiology and etiologies of pain in sickle cell disease. The
purpose of this article is to consider the pros and cons of the
use of the word “crisis” to describe pain in SCD, starting with
a historical etymology of how the word came into use in SCD,
then discussing clinical, research, and patient perspectives on
the use of the term.
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The diagnosis of illness never occurs in a vacuum. Historical
and social factors are often embedded in the naming process.
This social construction of illness includes the voices of patients,
physicians, advocacy groups, government, media, insurance
companies, scientists, and the pharmaceutical industry to name
a few. It also includes an invisible context: transmissibility,
moral judgment, and stigma. All of these ingredients make for
a complex frame in which to hang what may seem like a simple
diagnosis. Disease in this way goes far beyond pathology and
genetics, and into the realm of medical sociology where people
make Janus-like assignments: normal/abnormal, victim/villain,
guilty/innocent, heroic/pitiable, good/bad.3 These assignments
dictate how individuals, family members, and society perceive
and respond to medical conditions.
If the meaning of an illness derives from what are its
prominent features—such as hallmark symptoms, how it is
contracted or transmitted, and/or the vulnerability of specific
individuals or groups to it—this understanding is also bound to
its name, which can convey subtle and not so subtle ideas about
stated notions of good and bad.4 Collectively, these notions
produce our biased assumptions about who has the disease,
how they should be treated, how much attention they should get
from the public and private sectors, and the general meaning of
disease.5 These notions also have downstream implications for
the provision of benefits, availability of pharmaceutical drugs,
unemployment certification, and other legal claims.
In the case of SCD, the social factors that impact the
perception of the disease (race, ancestral disease origin,
education levels, poverty levels) begin with the diagnosis. In
1923, Sydenstricker and colleagues wrote that the sickle cell
disease diagnosis is made “without difficulty—the race, the
symptoms of anemia, with a history of rheumatic pains and
abdominal crises, the scleral discoloration, the absence of
splenomegaly, the presence or history of leg ulcer, all suggest
the condition.” There is a specific social context in which
this diagnosis was made. By 1923, medical researchers had
begun to associate sickle cell with the black race. For decades
after, though scientific racism was debunked, notions of
inferiority regarding African Americans remained, along with
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USE OF WORD “CRISIS” IN SICKLE CELL DISEASE
assumptions that certain clinical experiences were inherent to
the black race.
In the present day, many assumptions about SCD are still
made in the medical community. Sickle cell disease and its
related painful crises are often associated with urgency,
unpredictability, bad behavior, narcotic use and abuse. These
crises are also entrenched in power dynamics and certain
racial identities that translate into implications for access
to care, awareness of the disease, and legitimacy of the
patient population and patients’ experiences. The historical,
pathological, and personal narratives that follow will illuminate
the importance of the word “crisis” and its relationships with
the sickle cell population and outside worlds.
HISTORICAL PERSPECTIVE
Crisis. What does it mean to say that someone is “in crisis” or is
“having a crisis”? Our usual understanding is that that person
is experiencing “an emotionally stressful event or a traumatic
change” in his or her life.6 “Crisis” is not now a medical word,
though for centuries it was used as one. The Oxford English
Dictionary’s (OED) first definition of the word is medical:
The point in the progress of the disease when an
important development or change takes place which
is decisive of recovery or death; the turning point of a
disease for better or worse; also applied to any marked
or sudden variation occurring in the progress of a
disease and to the phenomena accompanying it.7
Despite the wording of this definition, diseases do not
experience crises. Only human beings can have or be in a
crisis. The classical medical “crisis” described above, where a
patient has a fever that either breaks and resolves or progresses
and ends in death, refers to a human event, not an event of
the disease. The crisis is the result of the way the disease
affects a person. Human observers label an event in the life of
the patient with the disease a “crisis”—a critical point in the
patient’s experience with a particular illness.8
Early use of “crisis” in English (1400s) referred to that
turning point in a fever.9 The word originated from the Greek
krisis, meaning “discrimination, decision.” The first use of
“crisis” in English to mean a “decisive moment” is identified
in the OED as occurring in 1627. This new use was a logical
extension from the medical meaning to, as one scholar stated,
a “figurative” one which continues to this day.4 According to
the OED, “crisis” is now used to describe:
A vitally important or decisive stage in the progress of
anything; a turning-point; also a state of affairs in which
a decisive change for better or worse is imminent; now
applied especially to times of difficulty, insecurity, and
suspense in politics or commerce.7
24 VOL. 106, NO 1, SUMMER 2014
The word thus came to be associated with events outside of
existential human ones, for example the Cuban Missile Crisis
or “the world is in crisis.”
How does this discussion of etymology relate to SCD?
We refer to the acute pain episodes associated with SCD as
“crises.” Why do we use that term? Certainly the image “sickle
cell crisis” evokes is of a person suffering sudden-onset,
unrelenting, excruciating pain that feels like it must be relieved
immediately. Such a situation would appear to others as a crisis
in the sufferer’s life.
The first description of SCD was published in 1910,10, 11
the second, third, and fourth in 1911,12, 13 1915,14 and 1922,15
respectively. Based on the medical literature (which is the only
source available because none of those early physicians or
patients is alive today), physicians began using the word “crisis”
in reference to SCD pain episodes after the fourth case appeared
in print in 1922. By that time, Drs. Virgil P. Sydenstricker at
the University of Georgia Medical Department (now called the
Medical College of Georgia) and John G. Huck at Johns Hopkins
University and Hospital were studying numbers of SCD
patients. They and others recognized what came to be known as
sickle cell anemia (SCA) as a disease entity with its own unique
characteristics and applied certain words to describe those
characteristics. “Crisis” became one such descriptor.
The first written record applying that word to SCA
appeared in a paper Sydenstricker delivered in November
1923 at the Section on Pediatrics of the Southern Medical
Association’s 17th annual meeting in Washington, DC,
and subsequently published in the March 1924 issue of
the Southern Medical Journal.16 (pp178,180,183) Referring to the
diagnosis of SCA, Sydenstricker stated: “This [diagnosis] is
made without difficulty. The race, the symptoms of anemia,
with a history of rheumatic pains and abdominal crises [italics
added], the scleral discoloration, the absence of splenomegaly,
the presence or history of leg ulcer, all suggest the condition”
(p180). In response to others’ comments during the discussion
of this paper at the meeting, he used the word again: “We feel
… that the gastric crises [italics added] with severe pain are
due to infarcts of the spleen” (p183).
In another paper Sydenstricker delivered, this one to the
Section on Practice of Medicine at the 75th annual session of
the American Medical Association in Chicago in June 1924, he
compared SCA to familial hemolytic icterus: “The jaundice in
both is due to increased red cell destruction; the type of anemia
is similar, and the evidences of erythropoietic activity seen
in the blood are much alike. Both diseases exhibit hemolytic
crises [italics added] with increase in jaundice and in pigment
excretion, with diminution in the red count and hemoglobin,
and with abdominal pain referable to the spleen.”17 (p12) He
repeated this observation in a comment made after a paper on
SCA delivered by his colleagues WA Mulherin and RW Houseal
at a meeting of the AMA Section on Diseases of Children in
1924 (perhaps the same AMA meeting as above—in June in
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USE OF WORD “CRISIS” IN SICKLE CELL DISEASE
Chicago): “We have seen [sickle cell] cases pass from the latent
into the active state, and vice versa. In the first instance [latent
to active], the development of ‘activity’ is associated with
‘crises’ similar to those seen in congenital hemolytic icterus.”18
(p87)
In 1927, only one author of an article on SCD used the term
“crisis” with regard to a severe pain episode:
The clinical picture of meniscocytic anemia
presents varying degrees of anemia, palpitation,
weakness, dyspnea, muscular and arthritic pains
without evidence of inflammation, a greenishyellow discoloration of the sclerae, abdominal crises
[italics added], ulcers of the legs, and a tendency to
remissions and exacerbations.19 (p913)
The term appeared in a French medical-journal article the
following year20 (p678) and then occasionally in sickle cell articles
over the next few years, usually in reference to abdominal pain.19
(p913), 21 (p50), 22 (p375), 23 (p814), 24 (p374)
By the early 1930s, the term seems
to have become part of the lexicon of those treating SCD patients.
Indicative of physicians’ acceptance and common use of “crisis”
in sickle cell disease are two articles that include the term in their
titles. The authors of the 1931 Journal of the American Medical
Association article “The Pathology of Sickle-Cell Anemia:
Report of a Case with Death During an ‘Abdominal Crisis,’”
remarked that “crises of abdominal pain are not uncommon in
this disease” and that a patient of theirs “was suffering from the
usual ‘abdominal crisis.’”25 (p1624) The following year, an entire
article in another prominent medical journal discussed the topic:
“Abdominal Crises in Sickle-Cell Anemia.”26 The word “crisis”
was becoming an integral part of the language of SCA.
One of the hallmarks of the disease from the very first case
onward was pain—epigastric pain, gastric pain, muscle pain,
joint pain. For example, in that first recorded case Herrick
described one of Walter Clement Noel’s episodes as “one of his
’bilious’ attacks, in which he had had quite severe epigastric
pain.”10 (p520) Often authors added the modifier “severe” to
the description.12 (p490) 27 (p1192) Just as often they used the word
“attack” or other terms such as: critical attack, episode of severe
pain, severe pain(s), paroxysms of prostration, paroxysmal
crises, periods of distress, paroxysmal abdominal pain, critical
episode. 26 (p390), 27 (p1191), 28 (p604), 29 (p497), 30 (p768), 31 (p1175) Both “severe”
and “attack” moved the description of SCA symptoms from
strictly clinical, medical-journal-like language to the language
of feeling—language that gave readers some insight—even
if they did not realize it—into what people with SCA were
experiencing. The word “crisis” added an even more human
dimension to the description. It gave the reader a sense of the
immediacy of the patient’s situation.
Or it should have. Physicians, however, had been applying
“crisis” to other medical conditions since the latter part of the
nineteenth century, which may help explain how easily they
adapted the term to SCA. In addition to the traditional use
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION
of “crisis” to describe a point in the natural history of fevers,
late 19th and early 20th century physicians applied the word
to pain and hematological events associated with, among
other conditions, “lardaceous disease,”32 gastric sarcoma,33
skin diseases,34 angio-neurotic edema,35 pernicious anemia,36
familial hemolytic icterus (as Sydenstricker mentioned in his
1924 paper at the AMA Section on Diseases of Children),17
(p12)
and, most prominently and regularly, tertiary syphilis.37,
38
Syphilitic crises identified in a 1903 medical dictionary
included bronchial, clitoral, genital, nephralgic, pain, urethral,
and vesical.39 A syphilis textbook of 1920 added nephritic,
laryngeal, cardiac, intestinal, rectal, and gastric crises to the
list.40 The word was thus familiar to readers of medical journals
and, presumably to physicians in their everyday practice.
Sickle-cell authors in this early period occasionally used
the term “crisis” to describe not just the severe abdominal pain
attacks, but also other aspects of the phenomena associated with
the disease. Sydenstricker, in his 1923 paper at the Southern
Medical Association meeting, used “crisis” to describe both
a blood phenomenon (“normoblastic crisis”) and a pain
episode (“abdominal” or “gastric” crisis),11 (pp178,180,183)], and at
the 1924 AMA meeting to describe another blood occurrence
(“hemolytic crisis”).17 (p12) Two articles on sickle cell appeared
in 1927 that mentioned febrile crisis41 (p236) and erythroblastic
crisis.42 (p932) And in a discussion of a paper presented at the
1932 annual meeting of the Section on Pediatrics of the AMA,
Thomas B Cooley of Detroit, well-known for his clinical
research on sickle cell anemia and thalassemia, mentioned
several kinds of “crises” he had encountered in SCA patients,
including hemolytic, abdominal, joint, and splenic.43 (p905)
Most, if not all, physicians writing about sickle cell
anemia between 1910 and 1932 appear to have been white.
Some of these physician-authors, practicing in both northern
and southern cities, seemed, by their choice of words when
describing patients in crisis, to recognize and appreciate that
these African Americans were truly suffering:
Eight days before [admission to the hospital], while doing
housework, severe pain in both legs developed which
forced her to bed. The pain continued unremittingly....
On the last admission she was fairly well nourished,
anemic, and apparently in great pain.25 (p1672)
During her stay in the hospital the child was drowsy
and moderately prostrated, with an anxious expression,
marked dyspnea and periods of restlessness.44 (p1000)
It may then develop that the pains [experienced during
abdominal crises in sickle cell anemia] . . . are nothing
more than the so-called toxic pains which occur in the
course of many infectious diseases. They are however
peculiarly severe in sickle-cell anemia.26 (p391)
VOL. 106, NO 1, SUMMER 2014 25
USE OF WORD “CRISIS” IN SICKLE CELL DISEASE
The patient was an anemic, undernourished black girl,
aged 4 years, very restless, tossing about, with her legs
drawn up.26 (p385)
On many occasions during the patient’s long stay at the
hospital he was seized with severe abdominal pain, at
times localized in the upper quadrants and at other times
in the lower quadrants.... The agony was so excruciating
that the patient assumed all kinds of bizarre positions, at
times extreme opisthotonos.26 (p384)
This acknowledgement of patients-of-color as human
beings capable of feeling pain and experiencing suffering
merits mention because of the generally negative attitudes of
whites who, during this era, often saw African Americans as
lesser members of the human family.
Additionally, these white physicians caring for almost
exclusively black sickle-cell patients provided their African
American charges with strong pain medicines and, at least in
published articles up through 1932, made no disparaging racial
remarks, overt or covert, about drug-seeking. For example:
From the time he was admitted to [United States Veterans’]
Hospital No. 88 he was not entirely free of pain either in
muscles or back, limbs, joints, or gall bladder region.
On July 15 all the above symptoms became markedly
exaggerated, morphia gr. 1/6 gave no relief.29 (p498)
The patient was in the hospital six and one-half weeks
until her death. During this time the pains in the legs
were practically continuous and excruciating, requiring
frequent injections of pantopium hydrochloricum daily
with only partial relief.25 (p1672)
In the population generally, many individuals present
hypochlorhydric states of a like degree but certainly do
not have pains which are only relieved by morphin as
was true of our first 2 [sickle cell] patients.26 (p389)
The patient died 5 months after admission to the hospital.
During this time she had frequent episodes of severe
lower and upper abdominal pain which were relieved
with difficulty only after repeated administrations of
morphin.26 (p385)
In fact, the authors of one article even commented matter-offactly about a patient who might have been addicted to opiates:
During the remission phases this patient asked for
no medication. This seemed to controvert a thought
26 VOL. 106, NO 1, SUMMER 2014
entertained by some that he was experiencing some
morphin withdrawal effects.26 (p389)
Why, then, did physicians apply the word “crisis” to certain
pain episodes of patients with SCA? Did they see these people
as undergoing a crisis – an existential experience? Based on the
few descriptions available from the time period, patients were
suffering excruciating pain that they urgently wanted relieved.
Patients seemed to see these episodes as crises in their lives—
crises where their very existence seemed to be at stake.
CLINICIAN AND RESEARCHER
PERSPECTIVE
The clinician’s central question around use of the term “crisis”
is whether continued use, or change in pain nomenclature
in SCD, would improve SCD diagnosis and treatment. In
contrast, the researcher’s central questions around use of terms
to describe pain in SCD relate to validity and meaning. Further,
there are some questions around use of pain nomenclature in
SCD that concern both clinicians and researchers. The answers
to these questions are only partially known. Below are the
central questions, and some evidence that relates to each set
of questions.
Will use of the word crisis, or change in pain
nomenclature of SCD, improve SCD diagnosis and
treatment?
Many studies have documented mistrust or misunderstanding
between patients and physicians about SCD pain,45, 46, 47 or
claims of undertreatment of SCD patients’ reports of pain48, 49.
Consequences of undertreated pain may be as far-reaching as
loss of employment and productivity by patients and caregivers,
and even strained relationships between patients and their family
members and friends. Perhaps continued use of the word crisis
will bring attention to the need for acute and prompt treatment
of pain in SCD that presents for medical attention.
Are the term “crisis” or other terms to describe pain
in SCD valid, reliable, and meaningful? What are the
measurable subjective correlates of crisis in SCD?
How much of SCD pain managed at home qualifies
as a crisis? Might crises at home be undertreated
because they are unmeasured? Is the number of
crises predictive of short-term or long-term prognosis?
If not “crisis,” what terms could be substituted to
describe pain in SCD, other than “pain”? (How) should
we score the severity of a crisis, apart from scoring
pain severity?
Like all pain, pain in SCD is a subjective symptom, with biological,
affective, and social components, each deserving of measurement.
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USE OF WORD “CRISIS” IN SICKLE CELL DISEASE
The term “crisis,” as used by clinicians and researchers, is
meaningful, but is highly prone to misclassification.
The word “crisis” traditionally has been associated with
urgent health care utilization. In clinicians’ parlance, we say,
“The patient came to the hospital for a crisis,” or, “The patient
was admitted, but this time not for a crisis.” We almost never
say, “The patient came to the clinic for a crisis,” or, “The patient
went to work in a crisis.” We use the word “crisis” to connote
disabling pain, interfering with function, and requiring acute
emergent health care attention.
This use of the word “crisis” influenced the way SCD pain
was characterized in research studies.50 Pain was thought
to be mainly acute, and mainly due to vaso-occlusion and
its inflammatory and ischemic consequences. “Crisis” was
almost synonymous with SCD pain, which was almost
synonymous with formal Emergency Department and
hospital utilization (Figure 1). Pain was therefore thought to
be easily observed. A landmark study used the term “pain”
to describe health care utilization due to pain in SCD, and
found pain rates to be infrequent. It also found pain between
and within subjects to be quite variable and unpredictable,
suggesting that the intensity of the underlying pain stimulus
was also variable and unpredictable. This research showed
that the number of crises per year was predictive of mortality,
and that it was lower in patients with higher fetal hemoglobin
levels, or lower total Hb levels.51
But this use of the term “crisis” also implies that any disabling
pain will by necessity result in a visit for a physician’s acute
attention. This use therefore creates a measurement problem,
in that crisis may be conflated with pain, vaso-occlusion, and
utilization. This may result in an under-measurement of pain,
especially if it doesn’t result in emergent health care utilization,
yet it is deemed by patients sufficiently severe or disabling to
be called a crisis. Health care workers’ and some researchers’
definition of a crisis ignores crisis pain treated at home.
In fact, new results show that severe SCD pain, pain severe
enough to be called a crisis, may occur far more frequently
than what was once thought,52, 53, 54 and that the substantial
majority of SCD pain, even crisis pain, is managed at home.55
It reveals that pain in SCD, and crisis as defined by patients
if not physicians, has been vastly under-measured and often
misclassified in previous research studies. Figure 2, based on
data from the Pain in Sickle Cell Epidemiology Study (PiSCES),
shows that, opposite to what was predicted by previous studies,
the frequency of pain for most adult patients is high, and only a
small minority of adults has very infrequent pain.
Further, based on PiSCES, patients experience SCD pain
on 55% (95% CI, 51% to 61%) of days, but experience crises on
15% (CI, 13% to 19%) of days, and only utilize care for pain
on 4% (CI, 3% to 5%) of days. PiSCES also suggested patients
must experience a certain intensity of SCD pain before they
call it a crisis, and that crises can be graded in severity. Patients
experienced an average pain severity of 4.2 ± 2 (0-9 scale)
during SCD pain that wasn’t described as a crisis, vs. 5.5 ±
2.1 during crises that didn’t result in utilization, vs. 6.5 ± 2.3
during crises that resulted in utilization.56
Are crises objectively verifiable with biomedical
correlates? For example, are there objectively
measureable correlates of vaso-occlusion that are
present during a crisis and not outside of a crisis?
Crises are only partly objectively verifiable with
biomedical correlates. The pathophysiology of SCD results
from erythrocytic vaso-occlusion,57, 58 due in part to
autosomal recessive hemoglobin alteration, hemoglobin S.
Hemoglobin S demonstrates impaired oxygen binding, and
deoxy-hemoglobin S polymerizes inside the erythrocyte.
Polymerization of deoxy-hemoglobin S results in,
classically, sickle-shaped erythrocytes.59 But also, SCD
vaso-occlusion is associated with adhesions expressed on the
erythrocyte membrane.60, 61 The altered erythrocytes occlude
the microcirculation.
Although crises seem dramatic and acute, vaso-occlusion
seems chronic: it results in chronic, ubiquitous hemolysis,
ischemia, infarction, hemolytic anemia, and ultimately
organ damage.62 Endothelial function in SCD is impaired both
during and after crises.63 The increased expression of vascular
adhesions on sickle erythrocytes seems to be stable over time
within a given patient, but differences in expression between
children are correlated with differences in crisis frequency.54
In contrast to the constancy of expressed vascular adhesions,
evidence shows that microvascular hemodynamic forces
within patients constantly change, mediating widely variant
adherence of sickle cells to the vascular endothelium.64 This
could account for sudden painful episodes within patients.
Home-managed and hospital-managed sickle cell disease pain
may thus be at opposite extremes of a varying continuum
Figure 1. Conflation of vaso-occlusion, pain, and health care utilization in sickle cell disease.
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION
VOL. 106, NO 1, SUMMER 2014 27
USE OF WORD “CRISIS” IN SICKLE CELL DISEASE
Figure 2. Histogram of percentage of SCD pain days in Pain in Sickle
Cell Epidemiology Study
of pain frequency and severity caused by correspondingly
varying but chronic underlying vaso-occlusion.
Researchers have organized evidence supporting a theory of
distinct phases (e.g., evolving, inflammatory, resolving) of a crisis
that results in hospitalization, complete with objective serum or
clinical correlates.65, 66, 67 68 But this theory is not yet completely
validated, nor have researchers produced therapies successful at
aborting a crisis by aborting underlying vaso-occlusion.69
PATIENT PERSPECTIVE
Like any sub-group in society, the community of persons with
SCD is not a monolithic one. Rather, we are a heterogeneous
bunch. While the vast majority of us might share the experience
of SCD pain in at least some of its many varied forms, there
will be great variation among us in the ways that we experience
that pain, the ways that we cope with that pain, and the ways in
which we talk about our pain.
As an adult living with SCD, the word “crisis” has been a
part of the lexicon I use to describe some of my experiences
with the disease for as long as I can remember. Like most,
if not all, persons with the disease, I was initially taught to
use the word by the medical profession. However, the varied
experiences that I have had throughout my life with the disease
have caused me to develop my own particular ways of using
the term. What I refer to as my “typical sickle cell crisis” may
be a little bit different from the next person’s with SCD, whose
use of the term may differ still from the next person with SCD,
and so on. For example, I frequently make distinctions in my
life between the impact of “minor crises” or “little crises” as
opposed to the effects of “severe crises” or “really bad crises”.
For me, those distinctions hinge on such factors as the severity
of the pain…whether or not I had to seek help from a medical
professional…and the extent to which I was forced to miss
school or work. The fact that I use the term “crisis” in this way,
though, doesn’t necessarily say anything about what is meant
when another person with SCD uses the term.
28 VOL. 106, NO 1, SUMMER 2014
Although the specifics of how persons with SCD use the
term “crisis” may differ, there is some evidence to suggest
that there is widespread agreement among us that the term
has a role in describing some of our experiences with pain.
An online forum for persons with SCD recently conducted a
poll to determine the extent to which persons with the disease
thought that use of the term crisis was appropriate to describe
sickle cell pain.70 Twenty-one of 24 respondents agreed that
use of the term “crisis” was appropriate. However, a close
examination of the responses for and against use of the term
reveals an interesting trend. Examples of statements in favor of
the use of the term “crisis” include:
I think it’s totally appropriate. Literally when I’m in
pain it’s a “crisis”. I need help, support and attention
immediately.
I think [it is] appropriate but at the same time why is it
hard for nurses to take it seriously? They think “what’s so
critical and why do they need help so bad?”
Crisis implies that immediate help is required therefore
I do feel it is the right word. The problem is that the
medical community does not take the word literally.
It is clear from each of the statements quoted above that the
respondents believed the term “crisis” adequately expressed
a desired sense of urgency and immediacy in efforts to get
help for their sickle cell pain. This is in contrast to the few
statements provided from persons who disagreed with the
appropriateness of the term “crisis”:
I don’t like the terminology crisis because it honestly
doesn’t express how severe or serious the event is. I don’t
know what would be a better word to describe it.
Crisis is not appropriate. We use terms such as incapacity,
inability to mobilize, and severe disability. They probably
get more attention.
It appears, then, that disagreement among persons with
SCD regarding the appropriateness of the term “crisis” to
describe our pain hinges in large part on the extent to which
we believe that the term adequately expresses the severity of
our pain and the urgency of response that we feel should be
provided to us accordingly when we do decide to seek medical
professional help for our pain.
What role should the term “crisis” have as a descriptor for
part of the experience of living with SCD? From the patient’s
perspective, I believe that the term only holds instrumental
value. Yes, many of us living with SCD, especially those of
us who are adults, have an affinity for the term. It has been an
integral part of our language since childhood, and it holds real
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION
USE OF WORD “CRISIS” IN SICKLE CELL DISEASE
meaning for us. However, if tomorrow the medical and public
health professions came out with a joint statement saying that
they were going to eliminate the term “crisis” from the SCD
lexicon in favor of some new terminology, I suspect that most,
if not all, persons with SCD would gladly go along with the
change…assuming, of course, that the reason for this shift in
language is because the medical and public health communities
assure us that this shift in language will lead to fewer disputes
between patients and healthcare providers about the legitimacy
of our pain experience, an increase in the urgency with which
medical professionals respond to us when we present for acute
care, and an overall improvement in the quality of the SCD
pain management experience.
Unfortunately, it is the continued lack of those desired
quality-of-care outcomes that constitutes the most significant
“crisis” faced by the sickle cell community.
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70. Survey conducted and compiled by Mr. T.J. Brown, President
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http://sicklecellcantstopme.wix.com/sickle-cellnatural-wellness-group-inc#!
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