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Level III‐3; Friedman et al, 1986 Level III‐3). However in children, the incidence of acute sickle
chest syndrome, and plasma levels of morphine and M6G, was significantly higher with oral
morphine compared with IV infusion (Kopecky et al, 2004 Level II).
Care must be taken when using opioids in the treatment of pain in sickle cell disease. In a
review of 35 patients who died in hospital following an exacerbation of sickle cell disease,
9 of the 35 patients received excessive opioids and ‘overdose’ directly contributed to death in
5 out of the 35 (NCEPOD, 2008). In two‐thirds of patients, there were inadequate observations
of sedation and respiratory rates after opioid administration and IM pethidine administration
was prevalent.
Inhaled nitrous oxide
Inhaled N 2O in 50% oxygen used for limited periods may provide analgesia for acute sickle cell
pain in the primary care setting (Rees et al, 2003).
Inhaled nitric oxide
Nitric oxide (NO) deficiency or defective NO‐dependent mechanisms may underlie many of the
processes leading to vaso‐occlusion. Inhaled NO may be of benefit in painful acute vaso‐
occlusive crises in children; however, further studies are required (Weiner et al, 2003 Level II).
Corticosteroids
Parenteral corticosteroids appear to reduce the duration of analgesia requirements and length
of hospital stay, without major side effects, during sickle cell crises (Dunlop & Bennett, 2006
Level I). In children, a short course of high‐dose IV methylprednisolone decreased the duration
of severe pain associated with acute sickle cell crises but patients who received
methylprednisolone had more rebound attacks after therapy was discontinued (Griffin et al,
1994 Level II).
Epidural analgesia
In severe crises, where pain is unresponsive to other measures, epidural analgesia has been
used effectively (Yaster et al, 1994 Level IV).
Prevention of painful sickle cell crises
Hydroxyurea increases fetal haemoglobin levels, thereby reducing the frequency of acute
CHAPTER 9 crises, blood transfusions and life‐threatening complications (including acute chest syndrome)
in adults with severe disease who are homozygous for the sickle cell gene (Davies &
Olujohungbe, 2001 Level I).
Niprisan (an antisickling agent), zinc and piracetam (which prevent red blood cell dehydration)
may reduce the incidence of painful sickle cell crises (Wambebe et al, 2001, Level II; Riddington &
De Franceschi, 2002 Level II). The evidence for pircetam, however, is insufficient to support its
use (Al Hajeri et al, 2007 Level I).
Haemophilia
Deficiency of Factor VIII (haemophilia A) and deficiency of Factor IX (haemophilia B) are
inherited disorders of coagulation characterised by spontaneous and post‐traumatic
haemorrhages, the frequency and severity of which are proportional to the degree of clotting
factor deficiency. Bleeding into joints and muscle is common, although other sites such as
abdominal organs may also be involved. In haemophilic arthropathy the most frequent sites of
pain are the ankle joints (45%), knee joints (39%), spine (14%) and elbow joints (7%) (Wallny et
al, 2001 Level IV). Haemophilia patients may also have pain syndromes associated with human
immunodeficiency virus /acquired immunodeficiency syndrome (HIV/AIDS) (see Section 9.6.8).
Recurrent acute pain may have a significant adverse impact on mood, mobility and quality of

258 Acute Pain Management: Scientific Evidence