Sickle cell disease has become the most common genetic condition in some European countries (Roberts & De Montalembert 2007). The disease is characterised by the painful sickle cell crisis often experienced by sufferers. This article sets out to examine the pathophysiology of this disease, alongside the factors that perioperative practitioners need to be aware of in order to reduce the risk of triggering a sickle cell crisis.
AdamS., JonassaintJ., KrugerH.2008Surgical and obstetric outcomes in adults with sickle cell diseaseAmerican Journal of Medicine121916–21
2.
AkerJ.1999Sickle cell disease: implications for perioperative careJournal of PeriAnesthesia Nursing14(4) 221–27
3.
BuckJ., CasbardA., LlewelynC.2005Preoperative transfusion in sickle cell disease: a survey of practice in EnglandEuropean Journal of Haematology7514–21
4.
BuckJ., DaviesS.2005Surgery in sickle cell diseaseHematology/Oncology Clinics of North America19(5) 897–902
5.
BurgerL., FitzpatrickJ.2009Prevention of inadvertent perioperative hypothermiaBritish Journal of Nursing18(8) 1114–19
6.
CopeA., DarbyshireP.2013Sickle cell disease, update on managementPaediatrics and Child Health23(11) 480–85
7.
FirthP.G.2005Anaesthesia for peculiar cells — a century of sickle cell diseaseBritish Journal of Anaesthesia95(3) 287–99
8.
FirthP.G., HeadA.2004Sickle cell disease and anesthesiaAnesthesiology101766–85
9.
GongL., ParikhS., RosenthalP., GreenhouseB.2013Biochemical and immunological mechanisms by which sickle cell trait protects against malariaMalaria Journal12317
10.
GyamfiY.A., SankarankuttyM., MarwaS.1993Use of a tourniquet in patients with sickle cell diseaseCanadian Journal of Anaesthesia4024–27
11.
HowardJ., MalfroyM., LlewelynC.2013The transfusion alternatives preoperatively in sickle cell disease (TAPS) study: a randomised, controlled, multicentre clinical trialLancet381930–38
12.
KanterJ., Kruse-JarresR.2013Management of sickle cell disease from childhood through adulthoodBlood Reviews27279–287
KatoG.2014No NO means yes to sickle red cell adhesionBlood123(12) 1780–82
15.
LaddL.A., KamP.C., WilliamsD.B., WrightA.W., SmithM.T., MatherL.E.2005Ventilatory responses of healthy subjects to intravenous combinations of morphine and oxycodone under imposed hypercapnic and hypoxaemic conditionsBritish Journal of Clinical Pharmacology59(5) 524–535
16.
MalanjumL., FisherB.2008Procedures under tourniquetAnaesthesia and Intensive Care Medicine10(1) 14–17
17.
McCanceK., HuetherS.2006Pathophysiology: The biological basis for disease in adults and childrenMissouri, Elsevier Mosby
18.
MercadanteS., BrueraE.2006Opioid switching: a systematic and critical reviewCancer Treatment Reviews32(4) 304–315
19.
NaikR., StreiffM., HaywoodC.2013Venous thromboembolism in adults with sickle cell disease: a serious and under-recognized complicationThe American Journal of Medicine126(5) 443–49
National Health Service2011The NHS sickle cell and thalassaemia screening program 2010/2011 Annual report Available from: sct.screening.nhs.uk/getdata. php?id=11558 [Accessed March 2015]
22.
National Institute for Health and Care Excellence2008Inadvertent perioperative hypothermia: The management of inadvertent perioperative hypothermia in adults CG65 [online] Available from: http://guidance.nice.org.uk/CG65 [Accessed March 2015]
National Institute for Health and Care Excellence2012Sickle cell acute painful episode: management of an acute painful sickle cell episode in hospital CG143 Available from: www.nice.org.uk/guidance/CG143 [Accessed March 2015]
25.
O'MearaM., AllfordM.2010Anaesthesia for patients with sickle cell and other haemoglobinopathiesAnaesthesia and Intensive Care Medicine11(6) 242–43
26.
O'MearaM., DavieG.2013Anaesthesia for patients with sickle cell disease (and other haemoglobinopathies)Anaesthesia and Intensive Care Medicine14(2) 54–56
27.
Redding-LallingerR., KnollC.2006Sickle cell disease: pathophysiology and treatmentCurrent Problems in Pediatric and Adolescent Health Care36(10) 346–76
RobertsI., De MontalembertM.2007Sickle cell disease as a paradigm of immigration hematology: new challenges for hematologists in EuropeHaematologica/Hematology Journal92(7) 865–70
Scottish Paediatric and Adult Haemoglobinopathy Managed Clinical Network2012Management guidelines for adult patients with sickle cell disease Available from: www.spah.scot.nhs.uk/Documents/Adult%20Guidelines%20v2.pdf [Accessed March 2015]
32.
SerjeantG.R.1992Surgery and anesthesia in: SCDOxford, Medical Publications
33.
SteinbergeM.2012Sickle cell disease and other hemoglobinopathies In: GoldmanL., Schafter (eds) Goldman's Cecil MedicinePhiladelphia, Elsvier Saunders
34.
TaylorS.M., ParobekC.M., FairhurstR.M.2012Haemoglobinopathies and the clinical epidemiology of malaria: a systematic review and meta-analysisLancet Infectious Disease12457–68
35.
Sickle Cell Society2008Standards for the clinical care of adults with sickle cell disease in the UK Available from: sct.screening.nhs.uk/getdata.php?id=10991 [Accessed March 2015]