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Sickle cell disease

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Introduction The case study that follows shows the peri operative care that would be undertaken for someone who has underlying medical conditions which impede on the surgical intervention that is taking place, the module that I, as a student have just undertaken has involved underlying medical conditions that effect our care of the patient, the pathology of disease and disorders and how this may change our peri operative care of the patient, with this in mind the case study I have chosen to write about is a patient who is a sufferer of sickle cell disease. The benefits of the last module has enforced the importance of firstly, understanding the underlying condition so as to adapt the care for the said patient with this in mind I will first look at what is sickle cell and the biology of sickle cell and then what implication this has on perioperative care What is Sickle Cell Disease? Sickle cell disease comprises of a group of inherited blood disorders that alter a persons haemoglobin, causing chronic haemolytical anaemia and producing acute and chronic pain a a result of reoccurring episodes of vascular occlusion. The most prevalent sickle cell pathology is sickle cell anaemia; this occurs when a person inherits a valine substitution in place of the glutamic acid, from two parents, the substitution occurs in the sixth position of the beta globulin chain of haemoglobin. The alteration in the beta globulin chain is the cause of the haemoglobin molecule to deform creating a sickle shape. ...read more.


as cited in Frith and Head(2004 p771-2) states "The clinical picture of sickle cell disease is one of evolving organ damage punctuated by intermittent periods of severe pain and pulmonary complications. The severity and progression of the disease is remarkably varied, with some patients experiencing a relatively indolent course and others suffering early organ dysfunction and death. Pulmonary and neurological disease are the leading causes of morbidity and mortality." According to Firth and Head (2004 p772) "Many deaths are not attributable to overt chronic organ failure but occur during an acute episode of pain, respiratory compromise, stroke, or a combination of these events." Perioperative Even though sickle cell trait does not have a marked increase of morbidity or mortality in the perioperative setting. Atlas SA (1974) and Heller et al(1979) cited in Firth and Head (2004 p773), patients with sickle cell disease are known to have high incidences of perioperative problems, these can be sickle cell disease specific and can include pain crisis and acute respiratory syndrome, occurring with high frequency in the perioperative period there may also be a risk of erythrocyte alloimmunization and transfusion reaction complications, or there may be non-specific these can include fever, infection, bleeding, thrombosis, embolism and death from other causes than sickle cell disease. In patients with sickle cell disease the main risk we have to be careful of is vaso-occlusive crisis a precipitating event leads to a sickling cycle as blood flow becomes more sluggish and coaguable leading to hypoxia in the tissue. ...read more.


Tobin and Butterworth (2004) Management of a patient with sickle cell disease during the Intraoperative phase is a challenge to the anaesthetic team, as reversal of the sickling process to date has been difficult, with the anaesthetist focusing on prevention. Dix HM (2001) Most recommendations with regarding the measures to protect the patient from potential vaso-occlusive crisis are generally accepted with little controversy. Tobin and Butterworth (2004) Recommendations that are readily accepted are to keep the operative area warm, the patient hydrated and well oxygenated as well as paying meticulous attention to positioning in order to avoid circulatory stasis. Dix (2001) Throughout this assignment I have been looking at the importance of how underlying medical conditions affect the peri-operative care of the patient, with this in mind I feel that recognising these factors would greatly improve the care I would give the patient. Reference List Bassett C (1996) Caring for surgical patients with sickle cell disease Nursing Standard May 10(33) 38-39 Davey A and Ince CS (2004) Fundamentals of Operating Practice 2nd London:2004 Dix HM (2001) New Advances in the treatment of sickle cell disease: Focus on perioperative significance AANA Journal August 69(4) 281-6 Firth PG, Head A (2004) Sickle Cell Disease and Anaesthesia. Anaestesiology Sep 101(3) 766-85 Hammer et al (2003) Perioperative care for patients with sickle cell who are undergoing total hip replacement as treatment for osteonecrosis. Orthopaedic Nursing Nov/Dec 22(6): 384-97 King (1998) Brigdens Operating Department Practice 1st Bath:Churchill Livingstone Midence K, Elander J, (1994) Sickle Cell Disease a Psychosocial Approach. 1st. ...read more.

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