30/11/2019
Pathophysiology of SCD RBCs -hard, sticky, sickle shaped
Vaso-occlusion
Haemolysis
S/S ofhypoxia due to anaemia, reduced circulationand pulmonary complications of SCD
Anaemia
Cholecystitisusuallydue toCholelithiasis (haemolysis causes increasedbilirubin which causesgallstones
Impaired circulation to major organs
Lung-acutechest syndrome Pulmonary infarcts
Cerebral infarcts Splenicatrophy
Abdominal pain
Jaundice (due to increased bilirubin)and abnormalLFTs
Nauseaand vomiting
Feverdue to inflammatory process
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Cholecystitis in relation SCD
Physiological process • Haemolysis
• Studies show that up to 50% of children with SCD develop gall stones, often referred to as pigment stones and should be investigated with a screening ultrasound. • Studies show that haemolysis comes with several complications such as cholecystitis, cuteanous leg ulceration, priaprism and pulmonary hypertension. (Rees, et. al. 2010) • Cholecystectomy is the most frequent surgical procedure performed in SCD patients with the preference of laparoscopic surgery as it reduces the patients hospital stay without exposing the patient to SCD complications. This technique is recommended over open cholecystectomy.
• Micro-vascular circulation occlusion
• Ischemia or infarction of tissues supplied by occluded vessels. This is the dominant cause of morbidity and mortality.
• Vaso-occlusive crisis is a painful infarctive crisis which presents with fever, tachycardia and leucocytosis. These are commonly precipitated by infection, fever, pregnancy, cold, dehydration, stress and surgery.
(Schnall & Benz, 2002)
• As the presentation is acute and with further investigations my patient and parents were informed of the need for surgery.
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Case study Master George Foreman is a 17years old teenager of Afro-Caribbean origin, presenting to A&E with recurrent pain in the right upper abdomen, episodes of jaundice, low fever of 37.6ºC, bloating, nausea and vomiting. Surgical history: Circumcision Medical history : SCD diagnosed at 9 months, none functioning spleen, asthma He attends haematology clinic as part of his ongoing care. Frequent admissions to hospital following infections and vaso-occlusive crisis associated with SCD. Weight 58kg, No known drug allergies Medication: ibuprofen, oxycontin, oxynorm, lansoprazole Diagnosis: Cholecystitis
The SCD patient in surgery
Primary aim for these patient group is to manage triggers of sickle cell related crisis
• Hypoxia • Acidosis
• Hypothermia • Hypovolemia
Anasthestetic agents and surgery can induce the above in a surgical patient and hence should be managed but extra considerations should be in place for patients with sickle cell disease. (Firth & Head, 2004)
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