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Sickle cell disease is a systemic multiorgan disease that most commonly presents with painful
vaso‐occlusive crises, occurring either spontaneously or due to factors such as dehydration,
infection, hypothermia and low oxygen tension. There is great interindividual variability in
the frequency and severity of crises. Pain during an acute crisis is typically severe and most
frequently reported in the back, legs, knees, arms, chest and abdomen and may last from
hours to weeks. Sickle cell crises involving abdominal organs can mimic an acute surgical
abdomen. Acute chest syndrome secondary to sickle cell disease may present with chest pain,
cough, dyspnoea and fever (Niscola et al, 2009).
Treatment of pain
Biopsychosocial assessment and multidisciplinary pain management may be required when
treating patients with frequent, painful sickle cell crises. A pain management plan in the form
of a letter, card or portfolio carried by the patient is also recommended (Rees et al, 2003).
Detailed clinical guidelines for managing acute painful crises in sickle cell disease are listed in
Rees et al (Rees et al, 2003). The implementation of clinical practice guidelines for acute pain
treatment in sickle cell crisis leads to more timely and more effective analgesia (Morrissey et al,
2009 Level III‐3).
Overall, there is only very limited evidence for analgesic interventions in acute pain crises of
sickle cell disease and meaningful meta‐analyses cannot be performed (Dunlop & Bennett, 2006
Level I).
Oxygen
Although oxygen supplementation is often prescribed during acute sickle cell crises, there was
no difference in pain duration, number of pain sites or opioid consumption in patients treated
with either air or oxygen (Robieux et al, 1992 Level II; Zipursky et al, 1992 Level II). However,
nocturnal oxygen desaturation was associated with a significantly higher rate of painful sickle
cell crises in children (Hargrave et al, 2003 Level IV).
Rehydration
There was insufficient evidence to suggest any benefit from fluid replacement therapy in
reducing pain associated with sickle cell crises (Okomo & Meremikwu, 2007 Level I).
NsNSAIDs
Single‐dose parenteral ketorolac did not reduce opioid requirements in painful vaso‐occlusive
crisis (Wright et al, 1992 Level II; Hardwick et al, 1999 Level II). CHAPTER 9
Opioids
In treating acute pain during a sickle cell crisis, IV opioid loading improved the analgesic
efficacy of subsequent oral and PCA opioid therapy (Rees et al, 2003 Level II). A continuous
IV morphine infusion shortened the duration of severe pain compared with intermittent
parenteral opioids (Robieux et al, 1992 Level II) and PCA with morphine reduced opioid dose
and related side effects (with a tendency to reduced length of hospital stay) compared with
continuous infusion (van Beers et al, 2007 Level II). The use of inpatient morphine PCA, rapidly
converted to oral CR morphine for use at home reduced the length of hospital stay by 23% and
subsequent emergency department visits and readmissions by approximately 50%, compared
with IM pethidine (Brookoff & Polomano, 1992 Level III‐3).
Although IV opioid PCA is widely accepted in the management of acute pain in sickle cell
disease, oral opioids are also effective. One trial in paediatric patients showed that oral
sustained‐release morphine for acute pain was just as effective as a continuous IV morphine
infusion (Jacobson et al, 1997 Level II). The use of oral opioids at home reduced the number of
emergency department visits and hospital admissions for sickle cell pain (Conti et al, 1996

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