Barriers to effective pain

management in sickle cell disease

Kerri Wright, Omolola Adeosun

the blockage of blood vessels. These cause

Abstract acute episodes of severe pain, leg ulcers and
avascular necrosis (Information Center for
Sickle cell disease (SCD) is a long-term condition that would benefit from a
long-term conditions approach to its care and management. SCD is growing in Sickle Cell and Thalassemic Disorders, 2000).
prevalence, affecting 10 000–12 000 people in the UK, with SCD sufferers having Painful crises are most frequent during the
an increased life expectancy from in the past. The most problematic aspect of third and fourth decades of life, with the
managing SCD is management of the pain from vaso-occlusive crises. Vaso- mortality rate increasing in those adults with
occlusive pain is the most common reason for hospital admissions in people the most frequent painful episodes (Ballas,
with SCD and accounts for large numbers of accident and emergency (A&E) 1998). SCD is a long-term condition which
attendances. A literature review was carried out to examine the management people with the disease have to learn to live
of vaso-occlusive pain in SCD. The review identified three main barriers to with and manage over many years.
effective pain management in SCD: the manifestation of vaso-occlusive pain, The most problematic aspect of managing
the sociocultural factors affecting pain assessment, and the concerns regarding SCD is the pain caused by vaso-occlusion
addiction and pseudo-addiction. Addressing these barriers will allow people with (Malach et al, 2002). Although vaso-occlusive
SCD to have their pain managed more effectively, improve their quality of life and pain itself is not directly life-threatening,
potentially reduce A&E attendances and admissions to hospital. inappropriate or inadequate treatment can
lead to unnecessary suffering and potentially
Key words: Long-term conditions n Pain management n Pseudo-addiction n Sickle fatal complications related to the disease
cell disease n Sociocultural barriers (Rees et al, 2003; De, 2005). Vaso-occlusive
pain is the most common reason for hospital

L
ong-term conditions have become
a current focus of health care. There
are estimated to be over 15 million
people in the UK living with a
long-term condition, with predictions of
this number steadily rising (Department of
Health (DH), 2007). People with long-term
conditions use a disproportionate amount of
health-care resources, with more frequent
GP consultations, hospital admissions, and
longer hospital stays.
The Government set targets to reduce
the number of emergency admissions by
5% by the end of 2008 and primary care
trusts (PCTs) are tasked to work with
acute trusts to achieve this goal (DH,
2004). Some of the strategies implemented
include appointing community matrons to
support those people with frequent hospital
admissions, getting nurses in accident and
emergency (A&E) to ensure admissions to
hospital are appropriate, and focusing on
self-management to empower patients to
Kerri Wright is Senior Lecturer in Primary Care
at the University of Greenwich, Eltham. Omolola
Adeosun is Community Nurse at Lewisham PCT
Accepted for publication: November 2008
manage their condition themselves.
At present, PCTs focus on the more
prevalent long-term conditions such as
chronic obstructive pulmonary disease
(COPD), cardiovascular disease and diabetes.
However, other long-term conditions
also have high attendance rates in A&E
and high numbers of hospital admissions.
These are rarely mentioned in literature in
relation to long-term conditions but would
benefit from the same approach to their
management. One such condition is sickle
cell disease (SCD).
Sickle cell disease
SCD is a genetic disorder which affects the
haemoglobin in red blood cells (Figure 1).
SCD affects 10 000–12 000 people in the
UK and is now the most common genetic
disorder in England and Wales, affecting up
to 1 in 300 births in urban areas (Howard
and Davies, 2007). The life expectancy of
people with SCD has improved in recent
years with a median survival age of 42 for
men and 48 for women, and many people
living into their 70s and 80s (National
Institutes of Health, 2002).
SCD is characterized by vaso-occlusive
episodes, known as crises, resulting from
admissions in people with SCD and accounts
for large numbers of A&E attendances
(Davies and Oni, 1997). The most common
reason for A&E attendance is reported to be
for pain relief. However, research indicates
that this pain is currently poorly managed,
contributing to inappropriate hospital
attendance and admissions of people with
SCD(Maxwell et al, 1999; Jacob, 2001;
Ahmad et al 2003). With the current
focus on enabling people with long-term
conditions to manage their condition in the
community, and on reducing inappropriate
hospital attendances, it is essential that pain
management for people in sickle cell crises
is further examined.
Literature review
A literature review was carried out to
examine the management of vaso-occlusive
pain in SCD. The databases searched were
CINAHL, British Nursing Index, PubMed
and Cochrane, using the terms ‘sickle cell
disease’ and ‘pain’ with ‘ethnicity’ and
‘mortality’. Literature was limited to the
past 10 years and articles related to animals,
children and adolescents were excluded.
The literature review identified several
barriers to effective pain management in

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 PAIN MANAGEMENT

SCD, which are preventing people from management knowledge for SCD, for Lorig, 2000). This relationship allows trust
receiving the appropriate care and treatment patients the pain is familiar and known. to develop through a shared understanding
for their pain. Addressing these barriers People with SCD are therefore likely to be of the long-term condition and the impact
would allow people with SCD to have their extremely knowledgeable about their own that it has on the individual’s life (Luker et
pain managed more effectively, improve pain and the most effective management al, 2000). Through this relationship patients
their quality of life and potentially reduce strategies for it. Therefore, to effectively are encouraged to participate in their care
A&E attendances and admissions to hospital. manage vaso-occlusive pain, health-care and management and become empowered
The three main barriers identified are: professionals firstly need to complete a to manage their lives with the condition
n The manifestation of vaso-occlusive pain comprehensive assessment of the person (Kralik et al, 2004). If this relationship
n The sociocultural factors affecting pain with SCD, one which includes listening to is compromised by sociocultural barriers
assessment and valuing the experiences and expertise of then it can affect the whole management
n The concerns regarding addiction and the individual with SCD (DH, 2001). of a person’s SCD, not just their pain
pseudo-addiction. management.
Each of these barriers will be discussed Sociocultural factors Sociocultural barriers can result in
in turn in relation to the management of There are sociocultural factors that impede patients from black and minority ethnic
vaso-occlusive pain and SCD as a long-term the effective assessment of people with SCD. groups receiving inadequate care and
condition. A patient’s experience of pain is subjective. being dissatisfied with health-care services.
This means effective assessment of pain is Reports have highlighted the disparity in
Manifestation of vaso-occlusive reliant on communication between patients perceptions of care received and increased
pain and health-care professionals (Todd et al, alienation that black and minority ethnic
One of the difficulties in managing vaso- 2006). Where there are sociocultural barriers groups experience when seeking treatment
occlusive pain appears to be the diversity of (e.g. of class, race, ethnicity, language), the for racially neutral conditions in the UK
ways that it manifests in individuals, making communication and assessment of pain can health-care system (DH, 1993; 2008). This
it difficult for health-care professionals to be compromised. In addition, the concepts alienation is expected to be compounded
develop experience in managing it. Pain of participation in care and shared decision- further for patients with SCD due to its
episodes vary greatly in frequency and making can also be affected by race and status as a ‘black disease’ in the UK (DH,
severity. Some patients rarely have painful ethnicity: patients from different ethinic 1993; Maxwell et al, 1999).
crises, while others spend the greater part of backgrounds to their physicians are less A study by Maxwell et al (1999), using
a given year in hospital receiving analgesics. willing to communicate their pain impacting interviews and focus groups of patients with
Also, the pattern of pain varies over time, negatively on their care (Green et al, 2003). SCD, compared the experiences of people
so that a person who has had a particularly Long-term condition management relies frequently attending hospital with those
severe year may later have a prolonged on a good relationship between the patient managing their pain at home. It found a
period characterized by only minor pain. and the health-care professional (Holman and range of interrelated themes of mistrust by
The mode of onset of vaso-occlusive pain
varies between patients, some of whom
develop agonizingly severe pain in as little
as 15 minutes, while in other instances
the pain gradually escalates over hours or
even days (Information Center for Sickle
Cell and Thalassemic Disorders, 2000). The
location and the type of pain also varies
considerably. Vaso-occlusive pain can occur
in the extremities, thorax, abdomen, and
back, and can be described as throbbing,
sharp or gnawing (Ballas and Delengowski,
1993).
Vaso-occlusive pain is unique to the
individual. Despite the variations between
different patients in location, severity, length
and description of vaso-occlusive pain, for
each individual person, the quality of the
crisis pain is usually similar from one crisis
to another. Vaso-occlusive pain tends to
recur at the same site for a particular person,
and patients can usually recognize the pain
as being typical of their SCD. Although for
the population as a whole, vaso-occlusive
pain varies, and for health-care professionals
it can be difficult to develop generic pain Figure 1. Red blood cells in sickle cell disease. (Eye of Science/Science Photo Library)

British Journal of Nursing, 2009, Vol 18, No 3 159

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 health-care professionals of patients with
SCD, stigmatization and neglect when
referring to hospital care. These experiences
led some people to manage their condition
at home rather than negotiating health-care
services. Pain management was frequently
found to be inappropriate when arriving at
A&E. Most people in the study attending
A&E only did so when they had tried
all other avenues to manage their pain at
home, but felt their crisis was worsening
and required opioids. Such expertise,
derived from a lifetime of experiencing
vaso-occlusive pain, seemed to be largely
ignored by A&E staff, who were perceived
to be unsympathetic to their pain. The study
concluded that there was a pervasive mis-
trust of patients with SCD which resulted
in staff exerting excessive control over their
pain management regimen.
Addiction and pseudo-addiction
One of the difficulties in managing vaso-
occlusive pain in SCD effectively is the
reluctance of health-care professionals to
administer and prescribe opioids (Pack-
Mabien et al, 2001). This reluctance is
borne from concern that opioids are being
requested not to manage the vaso-occlusive
pain, but due to opioid addiction (Todd et
al, 2006).
A study by Elander et al (2004) attempted
to examine this issue by using a substance
misuse assessment with patients with SCD
to ascertain whether their pain symptoms
were as a result of vaso-occlusive episodes
or arose from addiction. The study carried
out 51 in-depth interviews with patients
with SCD and compared them to the
Diagnostic and Statistical Manual for Mental
Disorders (DSM-IV). The authors argued that
individuals with tolerance or withdrawal or
both would meet the DSM-IV criteria
for psychological dependence. Their study
revealed that pain-related symptoms arose
from attempts by people with SCD to
manage their vaso-occlusive pain, not from
substance addiction. However, attempts to
manage this pain could also lead to behaviour
that is similar to symptoms suggestive of
analgesic dependence, thus making patients
vulnerable to misconceptions of substance
abuse by health-care professionals. The
authors indicate that despite the concerns,
there is very little evidence of substance
dependence in SCD and suggest that more
information for health-care professionals
regarding pain-related symptoms would
provide better insight into ways that coping
160
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with pain could give rise to perceptions of
dependence.
Inappropriately managed pain can result
in the patient displaying pseudo-addictive
behaviour in an attempt to manage their
pain and receive appropriate analgesia. This
behaviour is an iatrogenic syndrome resulting
from poorly treated pain (Weissman and
Haddox, 1989).Without adequate knowledge
of pain management and how this can
manifest, pseudo-addictive behaviour, such
as groaning or other physical behaviour
where the patient is trying to demonstrate
that they are in pain, can be misconstrued
as behaviour associated with substance
addiction. Such behaviour could be, for
example, aggression, conflict and arguments
about analgesia.
Inadequate assessment and treatment of
vaso-occlusive pain for people with SCD
can thus lead to people, out of desperation
to have their pain managed, becoming
aggressive, displaying overt physical signs of
pain and getting into conflicts with health-
care professionals regarding their care and
treatment. It is important to remember that
this behaviour, although similar to behaviour
found in substance dependence, is actually a
result of poor pain management and is an
indicator that this person needs their pain
management approach reviewed urgently.
Importance of nurse–patient
relationships
The under-treatment of pain by health-
care professionals is not solely due to
their concerns about addiction, but is also
related to more fundamental issues of trust,
control and patient involvement (Shapiro
et al, 1997; Maxwell et al, 1999). Effective
pain management depends on a trusting
relationship between the patient and health-
care professional. The breakdown of that trust
may influence patients’ ability to manage their
pain. Failure to involve patients with SCD in
making decisions about their care undermines
self-reliance and self-knowledge, reducing
the capacity for self-management. Repeated
experiences of controlling behaviour and
neglect by health-care professionals can erode
a patient’s trust. This can lead to considerable
anxiety about receiving adequate treatment
for pain. Subsequent ineffective treatment in
A&E can exacerbate this anxiety and lead to
behaviour which a health-care professional
could interpret as opioid addiction, rather
than the desperation to receive the treatment
the patient knows works to relieve the pain.
This can be further exacerbated when, as
frequently happens, people are not given
enough analgesic medication on discharge
or cannot obtain the required opioids when
they experience sickle cell crises and are thus
forced to attend A&E again to gain opioid
analgesia.
Long-term conditions require care and
treatment to be proactive. Many long-term
conditions have periods of acute episodes
which require intensive treatment. Planning
for these episodes can ensure that the right
treatment is available to ensure care can
be continued at home without needing
to use acute services such as A&E. For
example, ensuring that people with COPD
have a supply of antibiotics available and
know when to begin this treatment is now
a common strategy to empower people
with COPD to manage their condition and
prevent unnecessary admissions for chest
infections. People with SCD would benefit
from similar strategies. This could include
an individual care plan which details their
response to vaso-occlusive crises and the
treatment known to be effective for them.
In addition, it could also include strategies
where the individual can obtain the required
analgesic easily without the need to attend
A&E.
Conclusion
In order to reduce hospital admissions and
A&E attendances, and improve quality
of life for people with SCD, it needs
to be viewed as a long-term condition.
Health-care professionals need to work
with and respect the expertise of people
with SCD. Unfortunately there is still a
huge sociocultural barrier which needs
acknowledging by health-care professionals.
This barrier impacts on all aspects of care
and treatment given. If patients’ narratives
are accepted and listened to, and their
knowledge and expertise are believed, it
will be much easier to ensure that the most
appropriate care is put in place. Failure to do
this results in ineffective pain management,
leaving SCD sufferers frustrated and in
severe pain, and contributes to feelings of
mistrust and suspicion between some health-
care professionals and people with SCD.
People with SCD need individualized care
plans and strategies which can empower
them to obtain appropriate analgesia and
manage their pain themselves at home. Unless
these issues are addressed, people with SCD
will continue to be forced to attend A&E
and be admitted to hospital, often
unnecessarily. BJN
British Journal of Nursing, 2009, Vol 18, No 3

Ahmad W, Atkin K, Anionwu E (2003) Evaluating
Service Support to Families Caring for a Child with a
Sickle Cell Disorder of Thalasaemia. Department of
Health, London. Available at: http://tiny.cc/lWaiY
(accessed 5 Jan 2009)
Ballas SK (1998) Sickle cell disease: clinical management.
Baillieres Clin Haematol 11(1): 185–214
Ballas S, Delengowski A (1993) Pain measurement in
hospitalized adults with sickle cell painful episodes.
Ann Clin Lab Sci 23(5): 358–61
Davies SC, Oni L (1997) Management of patients with
sickle cell disease. BMJ 315(7109): 656–60
De D (2005) Sickle cell anaemia 1: background, causes
and incidence in the UK. Br J Nurs 14(8): 447–50
Department of Health (1993) Sickle Cell, Thalassaemia
and Other Haemoglobinopathies: Report of a Working
Party of the Standing Medical Advisory Committee. DH,
London
Department of Health (2001) The Expert Patient: A
New Approach to Chronic Disease Management for the
21st Century. DH, London. Available at: http://tiny.
cc/6XVQE (accessed 6 Jan 2009)
Department of Health (2004) Improving Chronic Disease
Management. DH, London. Available at: http://
tinyurl.com/bt8e24 (accessed 3 Feb 2009)
Department of Health (2007) Disease Management. DH,
London. Available at: http://tinyurl.com/bv9bw6
(accessed 4 Feb 2009)
Department of Health (2008) Report and Analysis of
the Experience of Patients in Black and Minority Ethnic
Groups. DH, London. Available at: http://tinyurl.
com/5n4xcc (accessed 8 Jan 2009)
Elander J, Lusher J, Bevan D, Telfer P, Burton B
(2004) Understanding the causes of problematic
pain management in sickle cell disease: evidence
that pseudoaddiction plays a more important role
than genuine analgesic dependence. J Pain Symptom
Manage 27(2): 156–69
Green CR, Anderson KO, Baker TA et al (2003) The
unequal burden of pain: confronting racial and
ethnic disparities in pain. Pain Med 4(3): 277–94
Holman H, Lorig K (2000) Patients as partners
in managing chronic disease. Partnership is a
prerequisite for effective and efficient health care.
BMJ 320(7234): 526–7
Howard J, Davies SC (2007) Haemoglobinopathies.
British Journal of Nursing.Downloaded from magonlinelibrary.com by 130.088.090.140 on January 19, 2015. For personal use only. No other uses without permission. . All rights reserved.
PAIN MANAGEMENT
KEY POINTS
n Sickle cell disease (SCD) is a long-term condition that would benefit from
a long-term conditions approach to its care and management.
n Vaso-occlusive pain is the most common reason for hospital admissions
in people with SCD and accounts for large numbers of accident and emergency attendances.
n The barriers to effective pain management in SCD are the manifestation
of vaso-occlusive pain, the sociocultural factors affecting pain assessment
and the concerns regarding addiction and pseudo-addiction.
n People with SCD need individualized care plans and strategies which can empower them to
obtain appropriate analgesia and manage their pain themselves at home.
Paediatrics and Child Health 17(8): 311–16 opioids and physicians fail. Gen Hospl Psychiatry
Jacob E (2001) Pain management in sickle cell disease. 24(6): 442–7
Pain Manag Nurs 2(4): 121–31 National Institutes of Health (2002) The Management of
Kralik D, Koch T, Price K, Howard N (2004) Chronic Sickle Cell Disease. NIH, Bethesda, MD. Available at:
illness self management: taking action to create order. http://tinyurl.com/ls14 (accessed 8 Jan 2009)
J Clin Nurs 13(2): 259–67 Pack-Mabien A, Labbe E, Herbert D, Haynes JR (2001)
Luker K, Austin L, Caress A, Hallett C (2000) The Nurses’ attitudes and practices in sickle cell pain
importance of ‘knowing the patient’: community management. Appl Nurs Res 14(4):187–92
nurses’ constructions of quality in providing palliative Rees DC, Olujohungbe AD, Parker NE et al (2003)
care. J Adv Nurs 31(4): 775–82 Guidelines for the management of the acute painful
Information Center for Sickle Cell and Thalassaemic crisis in sickle cell disease. Br J Haematol 120(5):
Disorders (2000) Management of patients with sickle 744–52
cell disease: an overview. Available at: http://tiny. Shapiro B, Benjamin M, Payne R, Heidrich G (1997)
cc/Cru5O (accessed 5 Jan 2009) Sickle cell-related pain: perceptions of medical
Maxwell K, Streetly A, Bevan D (1999) Experiences practioners. J Pain Symptom Manage 14(3): 168-174
of hospital care and treatment seeking for pain from Todd KH, Green C, Bonham VL Jr, Haywood C Jr, Ivy
sickle cell disease: qualitative study. BMJ 318(7198): E (2006) Sickle cell related pain: crisis and conflict.
1585–90 J Pain 7(7): 453–8
Malach S, Levin T, Saravay S, Martin R, Rao S, Weissman D, Haddox J (1989) Opioid pseudoaddiction
Horowitz D (2002) Sickle cell disease – when – an iatrogenic syndrome. Pain 36(3): 363–6