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1. Britirh Journal zyxwvutsr
o
J Health P&ologyzyxwvuts
(1996). 1, 95-111 zyxwvu
01996 zyxwvutsrqpo
The British PsychologicalSociety
Printed in Great Britain 95 zy
Adjustment and coping in adults with sickle
cell disease:An assessment of research evidence
Kenny Midence*
Department of Clinical Psychology, University Collexe of North Wales, Banxor,
Gwynedd LL57 ZDG, zyxwvu
UK
JamesElandet
MRC Child Pyhiatry Unit, Institute of Psychiatry, London
This paper considers the findings from psychological studies of adults with sickle
cell disease (SCD), an inherited haematological disorder which causes recurrent
episodes of acute pain. Studies are reviewed that deal with aspects of the condition’s
clinical presentation in adulthood, the risk of maladjustment and psychopathology
among affected groups, the role played by cognitive factors in mediating
psychological distress and facilitating more effective coping, and the implications
of the condition for family life and employment. Adults with SCD experience high
levels of maladjustment by comparison with other illness groups, and differences in
adjustment are predicted better by measures of social support and life stress than by
illness variables. The use of coping strategies plays an important role in pain
management and the mediation of psychosocial adjustment, with passive and
negative responses to painful episodes associated with less favourable outcomes.
Existing findings in this rapidly expanding area of research have implications for the
management of SCD and the organization of support services, and point to a need
for interventions to enhance patients’ coping skills. Future research could evaluate
specific cognitive and behavioural interventions to promote more effective coping,
and look at the effects of SCD on reproductive behaviour, family life, performance
in the workplace, and development and adjustment over the life-span.
Sickle cell disease (SCD) is an inherited blood disorder including sickle cell anaemia
(SS), SC haemoglobin disease (SC), and sickle beta thalassaemia (SPThal), in which
an abnormal form of haemoglobin polymerizes under low oxygen tension, distorting
the shape of the red cells and reducing their normal elasticity (Murayama, 1964).
Vaso-occlusion caused by sickled red cells is responsible for chronic and acute
damage to tissue and organs, and causes recurrent and unpredictable episodes of acute
pain (Davies zyxwvut
& Brozovic, 1989).
Greater medical understanding and more effective measures against infection and
other complications mean that people with SCD now survive longer than in the past.
Most reach adulthood (Leikin et al., 1989), and some live to old age (Brozovic &
Davies, 1987; Gray, Anionwu, Davies & Brozovic, 1991). This paper examines the
*Requestsfor reprints.

2. 96 zyxwvutsr
Kenv Midence and James Elander
findings of psychological research zyxwv
on adults with SCD and considers the social
and economic implications of the condition. We first outline features of the clinical
presentation of SCD that relate specifically to adulthood, going on to deal with
psychological adjustment among affected individuals, including the risk of depression
and maladjustment and the role played by strategies for coping with pain. We then
consider the implications of SCD for family life and employment. The article
concludes with a discussion of the practical and therapeutic implications of what is
known at present, and the most useful directions for future research. zyx
Clinical features relevant to adulthood
The clinical presentation of SCD changes to some extent over the life-span, with
differentcomplications arising at differentages, but the most important features of the
condition are the same in all age groups, namely unpredictable painful episodes and
restrictions imposed on activities and life-style.
Painful episodes
Many adults with SCD are symptom free for long periods, but a significant minority
experience frequent painful episodes for which hospitalization is required (Williams,
Earles zyxwvuts
& Pack, 1983),and men in their 20s report the most frequent episodes of pain.
Brozovic, Davies & Brownell (1987) found that 6 per cent of patients accounted for
over 40 per cent of acute admissions for SCD. In Platt, Thorington, Brambilla,
Milner, Rosse, Vichinsky & Kinney’s (1991)analysis of SCDpain rates among a large
sample covering the full age range, the highest rates were reported by those aged
between 20 and 29 years, with rates decreasing steadily after the age of 30. Baum,
Dunn, Maude & Serjeant (1987) found that the incidence of painful episodes among
men rose sharply to a peak at age 25 years and fell again thereafter, with little age-
related change among female patients. Age has been shown to account for a
significant part of the variance in health care use, with more frequent emergency
room visits and hospitalizations among young adult patients (Gil, Abrams, Phillips
& Williams, 1992),and younger adults have rated their pain as more severe than older
patients (Gil, Abrams, Phillips & Keefe, 1989).
Medical complications
Some of the medical complications associated with SCD are much more common in
later life, or affect adults to a greater extent than children. These include leg ulcers,
priapism and problems related to reproduction. Leg ulcerations are probably caused
when minor cuts and abrasions fail to heal because of poor blood flow in the affected
area, and affect large numbers of older children and adults with sickle cell anaemia in
the tropics (Chernoff,Shapleigh & Moore, 1954).They are one of the few visible and
unsightly complications of SCD, with the potential to increase social anxiety and
inhibition as well as causing pain and restricting activity. A study of 36 people aged
17and over who were affected by leg ulcers found that they were more likely to have
‘failed to accept SCD’than a group who also had SCD but no history of leg ulcers (29

3. Adults with sickle cell disease zyxw
97
per cent compared with 14 per cent). Sixteen of the 36 reported that they had left
school early because of their leg ulcers (Alleyne, Wint zyxw
& Serjeant, 1977).
Priapism is a persistent, abnormal and painful erection of the penis that can be
caused by sickling when the flow of blood from the corpus cavernosa is obstructed by
occlusions in the circulatory spaces and small blood vessels of the penis (Schmidt &
Flocks, 1971).Of 166cases of priapism reviewed by Ihekwaba (1980),over a quarter
were associated with SCD, and in Emond, Holman, Hayes & Serjeant’s (1980) study
of 104 SCD patients in Jamaica, 42 per cent of those interviewed reported at least one
priapic episode. Of those who were affected by priapism, 59 per cent complained of
repeated ‘stuttering’episodes lasting only a few hours and 38 per cent reported major
episodes lasting over 24 hours. Most episodes began during sleep. Mean age of onset
was earlier for stuttering than major episodes (18years compared with 21), and many
of those reporting stuttering attacks went zyxw
on to experience major episodes. Thirty-six
per cent of those affected by priapism reported at least partial impotence, and 16 per
cent reported complete impotence. Priapism associated with SCD also occurs in
middle childhood (Hamre, Harmon, Kirkpatrick, Stern & Humbert, 1991), but at
younger ages treatment is more likely to be effective (Seeler, 1973)and the condition
is less likely to be associated with sexual dysfunction. In adolescence and adulthood,
self-confidence and self-esteem may be reduced because those affected feel unable to
perform adequately, or feel that their masculinity has been compromised. This is a
sensitive problem for patients and carers, but should be attended to promptly because
early presentation and intervention have been associated with more successful
outcomes (Bertram, Webster & Carson, 1985).
Lower semen volume, sperm counts and sperm motility have been observed in
men with SCD, for reasons that are not yet clear but probably relate to sickling in the
testes. Female fertility is not affected, but pregnancy is sometimes complicated during
the final third of the term and during labour by painful episodes, infections and
problems related to anaesthesia (Tuck, 1982),and the foetus is at risk of poor growth,
prematurity, stillbirth and miscarriage (Davies, 1988). One early review of SCD
pregnancies found a maternal mortality rate of 22 per cent and a total foetal loss of 42
per cent, with spontaneous abortions accounting for 19per cent of losses (Eisenstein,
Posner & Friedman, 1965). Mortality associated with pregnancy is lower nowadays,
partly because of higher rates of elective terminations following antenatal diagnoses,
but the risk to mother and child is still higher than for the healthy population. zy
Psychological adjustment
The main issues that have been addressed by psychological research on adults with
SCD are the relative risk of psychological disturbance and the effectiveness of
different cognitive and behavioural strategies for coping with episodes of pain. In
this section we deal first with studies that compare rates of psychological disturbance,
mainly depression, among groups with SCD and those with asthma and diabetes, and
look at how well demographic and illness variables, coping styles and family factors
predict psychological adjustment. We then assessthe evidence on pain coping among
adults with SCD and those affected by other painful conditions, and consider how
coping styles and strategies are related to health care requirements and psychological

4. 98 zyxwvutsr
Kenny Midence and James Elander zyxw
adjustment in SCD. The studies reviewed in this section are summarized in Table 1.
Most were conducted in the United States, with samples drawn mainly from
populations of patients attending health care services for SCD.
Risk zyxwvuts
o
f maladjustment and depression
Damlouji, Kevess-Cohen, Charache, Georgopoulos & Folstein (1982) compared
adults with SCD to those with diabetes of the sameethnic and socio-economic group,
using the General Health Questionnaire (GHQ) to assess psychiatric symptomatology
and a semi-structured interview to assess social functioning over a range of areas.
They found that 63 per cent of those with SCD showed signs of psychiatric
morbidity, compared with 50 per cent of those with diabetes. The SCD patients
also reported greater social problems than the diabetics in the areas of employment,
finance, child care and spare-time activities. In both groups, social functioning and
GHQ scores were correlated, indicating greater psychiatric symptomatology among
those with poorer social functioning, but neither of these measures was associated
with the number of physical complications recorded in patients’ medical charts. zy
In a comparison between adults with SCD, those with asthma, and healthy siblings
of both patient groups (who were all black), telephone interviews were used to obtain
ratings for 90 items covering a range of health perceptions and medical care opinions
(Bobo, Miller, Smith, Elam, Rosmarin & Lancaster, 1989). Health perception scores
for the illness groups and the siblings were below those reported for other patient
groups and the general population, probably reflectingthe low socio-economic status
of the sample. The SCD group differed significantly from the asthma group on a
number of the scales, with more adverse ratings given by the SCD group in each case.
These included health outlook, resistance/susceptibility to illness, sickness orienta-
tion, satisfaction with medical care, and humaneness of medical treatment. The
siblings reported less use of medical services and scored higher on the health
perception scales, but both illness groups had more positive attitudes about going
to their doctors than the siblings, and ratings of medical care opinions did not differ
significantly between the illness and sibling groups.
Barrett, Wisotzek, Abel, Rouleau, Platt, Pollard & Eckman (1988) assessed 89
patients with SCD using the Chronic Illness Problem Inventory, in which patients
rated the severity of 65 types of problem covering 19 areas of personal and sociallife.
Employment was the area in which the most severe problems were reported,
followed by sleeping, eating, finances, assertiveness, daily activities and body
deterioration, all of which received ratings indicative of problematic functioning.
The lowest problem severity ratings were made in the areas of physical appearance,
cognitive functioning and marital difficulty. Men tended to report greater problems
than women, but there was little relationship between problem severity and illness or
health care variables.
Thompson, Gil, Abrams & Phillips (1992) obtained measures of psychological
adjustment, stress, efficacy, coping methods, family functioning and illness severity
from 109adults with SCD. Over half (56 per cent) met criteria for poor adjustment,
with zyxwvut
40 per cent showing symptoms of depression and 32 per cent symptoms of
anxiety. Better adjustment was associated with lower levels of perceived daily stress,

5. Addts with sickle cell disease zyxw
99 zy
higher efficacy expectations about illness tasks, and less use of palliative coping and
negative or passive coping strategies, but not with the type of illness (SS vs. SC vs.
SPThal), or measures of severity (number of complications and frequency of painful
episodes). Family functioning was also related to psychological status, with higher
family support and lower family conflict and control among those with better
adjustment.
Case reports are often suggestive of a causal relationship between SCD and
depression (e.g. Morin zyxwvu
& Warin, 198l), but it is by zyx
no means clear how the link
operates in every case. In cancer patients, pain has been shown to increase
sensitivity to other physical symptoms, impair appetite, sleep and self-esteem, and
increase feelings of isolation and anger (Cleeland, 1984). Among in-patients whose
pain was inadequately treated, sleeping difficulty was the most commonly reported
problem, followed by loss of concentration, anxiety, depression and irritability
(Marks & Sachar, 1973). Findings like these imply that frequency or intensity of
pain would play an important causal role in the development of depression and
other psychological problems in people with SCD, but this is not borne out by the
studies that use information about previous painful episodes to predict psycholo-
gical status (e.g. Thompson et zyxwvu
ul., 1992). Where psychosocial information has been
used to predict pain and medical complications, the results have been more positive,
although these studies are smaller and less well controlled in other ways. For
example, half of a group of 22 people with SCD reported that severe depressive
effects such as helplessness and hopelessness, often in response to different types of
loss, mainly unemployment, preceded the onset of painful episodes (Nadel &
Portadin, 1977). Retrospective reports of this kind should be treated with caution to
the extent that scope exists for misattributions about the causes of painful episodes,
but it is possible that depression or other affective disorders could increase the
incidence and severity of painful episodes by disrupting coping behaviour and
reducing active avoidance of situations or activities that can precipitate sickling.
Leave11 & Ford (1983) collected information about life-change events during the
previous 12 months from eight men and eight women with SCD, and followed up
the sample to inquire about symptoms of SCD leading to medical treatment over the
following six months. The numbers of life-change units reported was higher among
those who were hospitalized at the time of initial assessment than those without
symptoms, and higher among those who went on to require treatment than those
who remained symptom free. Losses and separations contributed most to the
increased numbers of life changes reported by those requiring greater medical
treatment, who also reported lower levels of social support. Psychiatric disorders
were detected in half of the sample, but state-trait anxiety scores were no higher than
those reported for general medical patients and did not differ between those who
required treatment during the follow-up period and those who remained symptom
free.
The pattern of findings in this area points to life stress and psychosocial adversity z
as the most likely precursors of maladjustment and psychopathology among adults
with SCD. This is a similar picture to that observed among patients with chronic
fatigue syndrome, where both positive and negative aspects of social support were
related to measures of anxiety and depression (Ray, 1992). People with chronic

6. 100 zyxwvutsr
Kenny Midence and James Elander zyxw
illnesses have often been shown to have higher levels of depression than the general
population (Friedland & McColl, zyxwv
1992), and the overlap between symptoms of
depression and those of many chronic illnesses and disabling conditions may lead to
an overestimation of depression among those groups (Frank et al., 1992). It is also
worth noting that, whereas clinical reactive depression is characterized by mood
states and behaviours which persist over changes in circumstances, many of the
apparently psychopathological problems observed in SCD do not require psychiatric
care and can be countered effectively by more general improvements in the advice,
support and information provided to the individual. In Collins’ (1986)analysis of
psychosocialproblems among 386 patients with SCD (not all of whom were adults),
the most common were treatment compliance difficulties, family or relationship
problems, poor relationships with health professionals, mood disturbance and
financial difficulties. Many of these were successfully addressed by providing better
counselling or emotional support, or by improving the liaison between medical,
social service and counselling services. Others responded to simple behavioural
methods such as instruction, reinforcement, or the use of strategies to monitor
specific problems. More closely integrated programmes of care for medical, social
and psychological problems have also been shown to reduce the need for medical
treatment (Vichinski, 1991;Vichinski, Johnson & Lubin, 1982).Interventions of this
kind could be said to address problems in adjustment more directly, without looking
for causes in the person’s medical condition and without pathologizing patients’
distress.
Coping withpain
A more recent trend has been to look at pain coping styles and strategies in SCD
patients and those suffering from other painful conditions, and to relate these aspects
of coping to medical requirements for pain management and psychological adjust-
ment. Gil, Williams, Keefe & Beckham (1990)investigated associations between
negative thoughts in relation to pain, severity of pain and psychological distress in
groups of patients with SCD, with rheumatoid arthritis, and those attending a pain
management programme with chronic back pain or headache. Higher scores on two
dimensions of negative thoughts (negative self-statements and negative social
cognitions) as well as the overall frequency, number and pervasiveness of negative
thoughts were significantly related to higher pain ratings and to most categories of
psychological symptoms. However, these findings were produced by combining data
from all three patient groups. There was little relationship between measures of
negative thoughts and demographic variables, but the groups were not matched for
ethnicity and over half the total sample was white. Group comparisons for negative
thoughts showed that the chronic pain patients differedin a number of ways from the
other two groups, scoring higher for negative self-statements and negative social
cognitions, lower for control over negative thoughts, and reporting more negative
thoughts generally, but there were no significant differences between the SCD and
arthritis groups. These effects, all showing greater negative thinking among those
attending the pain programme, could be because this group experienced more
constant daily pain rather than intermittent, episodic pain associated with SCD and

7. Adults with sickle cell disease zyxw
101
arthritis, or because these patients were attending the programme after other pain
control interventions had failed.
In a smaller study with groups matched for ethnicity, Anderson zyx
& Rehm zy
(1984)
examined relationships between measures of coping, perceived intensity and quality
of pain, and responses of family members in patients affected by sickle cell anaemia,
rheumatoid arthritis and low back pain. There were no significant differences in
reported pain or pain coping methods between the three groups, and only modest
relationships between measures of pain and pain coping. Pain coping was more
closely related to demographic factors, with those who used more coping strategies
and those who reported greater support from family members coming from larger
families. However, there were differences in the ways that reported pain and
responses of family members were related in the three groups. The families ofz
SCD and arthritis patients were perceived as showing less concern to patients whose
pain was more severe, whereas the families of patients with low back pain were
perceived as more solicitous to patients reporting more severe pain.
Other studies have focused exclusively on patients with SCD to look at the extent
to which various styles and strategies for coping with pain are associated with
psychological adjustment. Gil et al. (1989) looked at relationships between coping
styles and adjustment in 79 adults, using structured interviews and self-report
questionnaires to assess coping strategies used and distress experienced. This study
took care to identify independent relationships among variables, and coping
strategies accounted for significant portions of the variance in adjustment, control-
ling for demographic factors and illness severity. Coping strategies were not related
directly to pain frequency or duration, but appeared to mediate psychological
adjustment and consequences of pain. People with coping styles characterized by
negative thinking and passive adherence reported more severe painful episodes, more
frequent hospitalizations, higher levels of distress, and lower levels of activity during
episodes, and those who reported more coping attempts also reported more activity
during painful episodes. The findings emphasized the importance of irrational
cognitions (e.g. catastrophizing) in exacerbating suffering,and suggested that passive
adherence to medical recommendations is an ineffective means of coping.
Similar results were produced by the only prospective study of its kind, which
looked at the relationships between coping strategies at baseline, and measures of
pain frequency and duration, health care use and activity reduction over a nine-month
follow-up period among 89 SCD patients (Gil et ul., 1992). Negative thinking and
passive coping were predictive of poorer outcomes, with greater activity reductions
and longer and more frequent hospitalizations among those who engaged in negative
thinking and passively relied on rest. Pain coping strategies were relatively stable
between baseline and follow-up, suggesting that, without intervention, patients
prone to negative thinking and passive responses to pain may be at greater risk for
continued adjustment difficulties.
This series of studies seems to show that negative and passive responses to pain
play a greater role than positive aspects of coping in relationships with pain
management and psychological adjustment. This is in line with findings from
other patient groups (e.g. Keefe, Brown, Wallston & Caldwell, 1989), and suggests
that interventions designed to modify patients’ methods for coping with pain should

8. zyxwvutsrq
1
. Psychologicalstudies of adults with SCD
Sample
Size zyxwvutsrqpo
& type Age Measures Findings zyxwv
stment and &pression
del & 22 SCD 16-35 Open ended interviews about Painful episodes seemed to be more
tadin,1977 life-events and emotional common following life-eventsand
states prior to painful during zyxwvu
periods of depression.
mlouji 30 SCD
f., 1982 30 Diabetes
vell & 16 zyxwvutsrqp
S C D
d, 1983
lins,1986 386 SCD
rett et af., 89 SCD
88
15-56
episodes.
Social functioning schedule
(SFS); General Health
Questionnaire (GHQ).
Greater employment, financial, child-
care and spare-time activity
problems in SCD group. SFS and
GHQ scores positively correlated
in both groups. No relationship
between functioning and physical
complications.
19-53 Schedulefor recent events Those reporting fewer life-events were
less likely to attend hospital over the
following six months. State-trait
anxiety was not related to
symptomatology.
were treatment compliance,
relationship impairment, impair-
ment of relationship with health
care providers, mood disturbance
and financial difficulties.
and assertivenesswere the most
severely affected problem areas.
Little relationshipbetween
problem severity and illness and
health care variables. Men tended
to report greaterproblemsthan women.
(SRE); State-trait anxiety
inventory (STAI).
< 5-50 Assessment of psychosocial Most common psychosocial problems
problems and interventions
by review ofzyxwvut
case records.
18-67 Chronic illness problem Employment, sleep, eating, zyxwv
hnances
inventory (CIPI).
d zyx
0
N
k
P
Q
2
s
P
a
P
a.

9. le zyxwvutsrqpo
1. continued
bo zyxwvutsrqponml
et zyxwvutsrqponmlkj
af., zyxwvutsrqponmlkj
89
ompson
al., 1992 zyxwvutsrqp
g witbpain
derson zyxwvutsrqpon
&
hm, 1984
l et af.,
89
48 SCD
60 Asthma
50 Healthy
siblings
109 SCD
20 SCD
20 Rheumatoid
arthritis (RA),
20 lower back
p;un ( L W
79 SCD
SCD 16-48 Telephone interview to
Asthma 17-56 collect ratings of health
Sibs 18-35 perceptions and
satisfaction with
medical services.
18-68 Measures of stress and
efficacy; Ways of coping
questionnaire; Family
environment scale;
Symptom checklist
(SCL-90-R).
Mean 35 Self-control schedule:
Spouse response
questionnaire; McGill
pain questionnaire;
Maudsley zyxwvut
personality
inventory.
Mean 30.8 Structured pain interview;
(SD 9.0) Coping strategies
questionnaire (CSQ);
Symptom checklist
(SCL-90-R).
SCD group had lower scores for health
outlook, resistance/susceptibility to
illness and sickness orientation, and
rated medical services lower for
humaneness and satisfaction than the
asthma group.
Poorer adjustment was associated with
greaterstress,lowereAicacy expectations,
negativecoping strategies,and family
functioning characterized by high
support and low conflict and control. b
k
??
L,
6
2
%
P
L,
No differences in scores between groups,
but pain intensity and spouse responses
were related positively in LBP group
and negatively in SCD and RA groups.
Pain coping strategies were related to
Negative thinking and passive adherence
9
demographic factors. m
a
3
were associated with more pain
episodes, more activity reduction,
greater psychological distress, and
greater health care requirements.
Younger patients reported more severe
pain.

10. 0 zy
zyxwvutsrqpon
1.zyxwvutsrq
continued P
Sample
Size zyxwvutsrqpo
& type Age Measures Findings zyxwv
zyxwvutsrqpon
ef ai., 64 SCD Mean 44 Pain rating scale; Inventory Negative thoughts were related to pain
0 71 Chronic pain of negative thoughts in ratings, catastrophizingand
(CP) response to pain (INTRP); psychologicaldistress. Negative
50 Rheumatoid
arthritis (RA)
ef ai., 89 SCD
2
18-68
Symptom checklist
(SCL-90-R); Coping
strategies questionnaire groups.
thought &ores were higher among the
CP group than the SCD and RA
F
Q
questionnaire(CSQ) at
g$
and activity reductions. Coping a
baseline and %month
follow-up; Structured strategies were relatively stable over 2
s
pain interview.
visits and hospitalizations. zyxw
4
(CSQ).
Coping strategies Baselinepain coping strategiespredicted
numberanddurationo
fhospitalizations,
time. Younger patients had more ER

11. Adults zyxwv
wiib sickle ceff diseasezyxw
105
concentrate not only on promoting positive coping skills but also on reducing
maladaptive and ineffective coping behaviours. zyxw
Social implications
Sickle cell disease has the potential to limit or disrupt a wide range of activities
because exercise, cold exposure and other factors can precipitate sickling, but family
life and employment are the most significantareas of sociallife in SCD, because of the
hereditary nature of the condition and because financial status plays an important role
in coping with physical disability and most of those affected by SCD belong to ethnic
groups who are already at a significant socio-economic disadvantage.
Farnib issaes
The heritability of SCD raises important issues for adult sufferers who want to be
parents. They may wish to consider the genetic status of prospective partners in order
to minimize the risk to their offspring, and have the option of terminating
pregnancies in which SCD is diagnosed prenatally. Contraceptive choices may be
limited for women, because oestrogen increases the risk 'of stroke in women with
SCD (Haynes zyxwvut
& Dunn, 1967),and intra-uterine devices can precipitate infections and
painful episodes. Barrier methods and progesterone preparations present no addi-
tional risk, however, and depo-provera (an injectable progesterone) has been shown
to reduce painful episodes (De Ceular, Gruber, Hayes & Serjeant, 1982).
In a study of families affected by thalassaemia (another inherited haematological
disorder), many couples discovered the risk to their children only by producing a sick
child, and this knowledge caused them virtually to stop reproducing (Modell, Ward
& Fainveather, 1980).Very little is known about the effectsof SCD on reproductive
behaviour zyxwvu
or parental relationships, although early antenatal diagnosis has been
shown to be overwhelmingly popular among women with SCD (Jones, Shickle,
Goldstein & Serjeant, 1988). In Black & Laws' (1986) survey of people with SCD in
north London, all 10 of the adults over the age of 25 were parents, and several had
more than one child, but many of the women reported reluctance among male
partners to have a blood test to ascertain whether they carried the trait. A survey of 79
adults with SCD around San Francisco showed that the proportion of married people
was far below that for the general population of the area. Males were much more
likely to be single by comparison with the general population and women were more
likely to be separated or divorced (Broome & Monroe, 1979).An inherited illnesscan
cause various difficultiesfor adult relationships, including the possibility that testing
could reveal previously undisclosed paternity of children, but small sample descrip-
tive data are difficult to evaluate without comparable information from healthy
individuals of the same ethnic and social groups.
The main aim of genetic counselling is to allow people to make informed decisions
about their health-related behaviour based on all the available information and
options (Emery & Pullen, 1984). An important aspect of SCD counselling relates to
heredity, or risk in reproduction, but this is not its sole focus. Satcher (1976) has
described the importance of dealing with self-concept and social relationships in

12. 106 zyxwvutsr
Keny Midence and James Elander zyxw
counselling for SCD, which should also address personal physical and emotional
health, and any aspects of life that are potentially affected by SCD.
Mothers with SCD may require additional support because they have to spend time
at hospital or are periodically unable to cope with looking after their children at
home. Both formal and informal assistance with child-care would be expected to
make a difference to the lives of women with SCD who have children. Most adults
with SCD in Europe and North America are members of ethnic minorities, and the
distinct cultural patterns of these communities need to be considered in any research
on family life and social support in relation to SCD. Membership of the black
community, with its traditionally high levels of cooperation and sharing (Aschen-
brenner, 1975), could be an asset in coping with SCD, but the existence of an
extended network of informal support should not be taken for granted. Surveys show
that many black adults in Britain live alone or have limited contact with their families
(Atkin zyxwvuts
& Rollings, 1992), and older black people may be at particular risk of social
isolation (Fenton, 1987).
Emplyment
The level of employment-related problems experienced by people with SCD will
depend zyxwvu
on the severity of their symptoms and the extent to which they can be
managed, the nature of their work, and the attitudes of their employer. The type of
work which affected individuals choose to undertake or are able to obtain is a critical
factor, for although the ability to maintain regular attendance at work may be an
important consideration in employing a person with SCD, there is no reason why, in
a suitable occupation, individuals with SCD should not be expected to perform as
well as anyone during long periods of good health. People with SCD should avoid
work which is physically strenuous, exposes them to extremes of temperature or low
oxygen concentration, or which cannot be interrupted to take fluids. Many perform
highly skilled work, but the majority report great difficulty in finding and keeping
suitable jobs, and find that SCD places them at a significant disadvantage in the
workplace.
In Black & Laws’ (1986) survey of people with SCD in Newham, London, nearly
half of those aged over 16 were unemployed. Those who did work were in a variety
of occupations, but many reported great difficulty in getting and keeping work, and
felt they were restricted in the types of job they could consider. Employment was the
main source of income for only 36 per cent of families in Collins’(1986) study of SCD
patients in Chicago, with 95 per cent of those who were working concentrated in low-
skilled occupations and 85 per cent reporting that employers were inflexible about
absences from work due to painful episodes. Only 34 per cent of a group of adults
with SCD from the patient population around San Francisco were employed at the
time of interview, although a wide range of occupations was listed for those who
were working (Broome & Monroe, 1979).
Franklin & Atkin (1986) interviewed 13 people with SCD in the West Midlands
about their experiencesat work, and canvassed 11 major employers in the area about
their experiences with staff who were affected by SCD. All but one of the people
interviewed were considered fit for work, yet only one of the sample had been in

13. Adults with sickle cell diseasezyxw
107 zy
employment for more than three years. Of the remainder, two had never had a job,
and seven were unemployed at the time of the study, having lost jobs because of
repeated brief absences from work or (in two cases) leg ulcers. Of the employers, only
one (a company involved in commercial diving) tested applicants for sickle cell, but
another firm who did not test (and whose business was not judged by the authors to
be hazardous for staff with SCD) considered applicants with SCD to be unsuitable for
employment. Three companies reported that they had never considered SCD in staff
selection or had any problems related to the condition, and the remaining six claimed
to deal with each case individually, taking account of previous attendance records,
medical history and advice from the Occupational Health Service, their main concern
being the prospect of ‘regular and efficient attendance at work’. The employers’
responses revealed levels of awareness about SCD and sickle trait that were much
lower than had been indicated by insurance companies and the armed forces, and even
those who claimed to deal with applicants affected by SCD zyx
on a case-by-case basis
tended to confuse trait carrier and illness status, whereas trait carriers are symptom
free under all but the most extreme conditions. Generalizations about the way that
company policies and practices affect the working lives of people with SCD on a
wider scaleare not possible from such a smallsample, and more evidence is needed on
the types of problems encountered in the workplace by people with SCD, and how
these relate both to company policies and to attitudes and knowledge about SCD on
the part of individual employers and managers. zyxw
Conclusions
Levels of psychopathology and maladjustment among adults with SCD are generally
high, exceeding those for comparison groups with asthma and diabetes. Adjustment
has been more closely related to life stress and social support than to medical
complications and health care needs. Finance and work are areas in which some of the
most severe problems have been reported, and this is reflected in the high levels of
unemployment among samples of adults with SCD. Various styles and strategies for
coping with SCD pain have been associated with measures of pain frequency and
intensity, and use of strategies has been shown to predict psychological adjustment
over and above demographic factors and measures of illness severity. Passive
strategies and negative thinking have been associated with lower activity levels
and greater treatment requirements, and seem to play a more important role in
relationships with overall adjustment than positive aspects of coping. There are few
differences in styles of coping between adults with SCD and those with arthritis, but
patients with chronic back pain or headache have been shown to display more
negative thinking about their pain and to have families who were more solicitous
during painful episodes.
In a previous review of findings on psychological functioning among children and
adolescents with SCD, outcomes were highly variable and several studies found no
differences between groups with SCD and healthy controls (Midence, Fuggle &
Davies, 1993). In adults, no such negative findings have been reported, and the
pattern is one of more consistently raised levels of maladjustment. SCD may have
greater potential for causing distress and anxiety in adults than children because of

14. 108 zyxwvutsrq
Kenny Midence and James Elander zyxw
increased personal responsibility for coping, which in adverse social and economic
circumstances could precipitate disturbance by placing the individual under stress.
However, methodological differences may also have contributed to the contrasting
patterns of findings. The studies of children used measures covering aspects of
personality, intelligence and behavioural observations, whereas those of adults
employed a narrower range of constructs, mainly psychopathological criteria,
which would make consistent findings more likely. The adult samples may also
have been more homogeneous than those of children, with self-selection of more
seriously affected individuals through continued attendance at clinics.
A focus on more severely affected adults would not limit the value of the studies,
sincethis group would be the target for any interventions based on the findings, but it
would mean that generalizations about the prevalence of psychological disturbance
associated with SCD should be treated with caution in the absence of epidemiological
data. It is likely that many of those not included in clinicalsamplescope well with the
condition, or experiencefew significantproblems. Other methodological points to be
considered include the selection of comparison groups, the measurement of pain-
related constructs, and the limitations of cross-sectional analysis. In the studies of
adjustment, the comparisons were with ethnically matched groups of asthmatic or
diabetic patients, and seemed to show worse outcomes among those with SCD. In
those of pain coping, they were with groups suffering from arthritis or chronic back
pain or headache. Arthritic patients seemed broadly comparable to those with SCD
but the groups with head or back pain reported less positive approaches to pain
coping and depended to a greater extent on the support of family members. These
conditions may produce different patterns of pain that are less easy to cope with, or
the effects may be due to factors that were not taken into account, such as ethnicity or
the effectiveness of available pain management in each case. Even where ethnicity is
matched, the potential for cultural influenceson how pain is perceived and expressed
raises the possibility that standard instruments for the measurement of pain-related
constructs may not always be appropriate. One advantage of clinical samples is that
self-reported information about painful episodes can be supplemented by records of
hospital attendances and admissions.
Life stress and family support have both been associated with poorer
psychological adjustment, but correlational designs leave the door open to
alternative interpretations in each case and there have been no large-scale
prospective studies in this area. There is stronger evidence that the use of
active strategies, or the avoidance of passive, negative responses to pain, plays a
causal role in more effective pain management, but the extent to which cognitive
or behavioural pain coping interventions could contribute to more effective
treatment regimens would depend on how far spontaneous styles and strategies
can be altered as well as on how they predict different outcomes, and evaluations
of particular interventions or training programmes would be needed to assess
properly the practical and clinical efficacy of such methods. Various cognitive-
behavioural strategies for the management of chronic pain have been shown to
be effective (Benjamin, 1989; Linton, 1986), but there is much less evidence of
this kind from groups with SCD.
One aspect of pain management that has not been considered in great detail is

15. A
d
& with sickle cell diseasezyxw
109
problem drug use. This is a difficult concept to apply with a group who do have
intermittent legitimate requirements for drugs like pethidine or morphine. However,
in the most severely affected cases behavioural interventions will of necessity run
alongside pharmacological ones, so that interactions between the two need to be
considered. It is important also because the assumption is often made that people with
chronic painful conditions are at increased risk of drug dependence (e.g. Maruta,
Swanson zyxwvuts
8c Finlayson, 1979)whereas the limited evidence availabledoes not indicate
higher levels of problem drug use among patients with SCD than might be expected
(Midence 8c Elander, 1994, chap. 3).
Other gaps in the psychological literature zyxw
on SCD are the effectsof the discovery of
SCD within a family on reproductive behaviour and family relationships, and the
transition from childhood to adulthood. There have been no longitudinal studies of
adjustment over the life-span of people with SCD, so that it is not yet possible to
assess the extent to which experiences in childhood, such as the apparently increased
difficulty experienced by boys and adolescents (Hurtig, Koepke zyx
& Park, 1989), is
reflected in the adjustment of adults.
The need clearly exists for psychological interventions to enhance the psycholo-
gical adjustment of patients and increase their ability to cope with SCD. From what is
known at present, the most useful perspectives for psychological interventions would
be those with an emphasis on individual stress management and maximization of
social support, along with the reduction of negative, passive styles of coping. Closer
coordination and integration of services for medical, social and psychological aspects
of care and support in SCD have been shown to reduce physical health care
requirements (Vichinski, 1991;Vichinski et al., 1982),but would also allow potential
problems in psychological adjustment to be identified and addressed at the earliest
possible stage. zyxwvut
Acknowledgement
We are grateful for a referee’s comments on a previous draft of this paper.
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