Anesthetic management of a patient with sickle β⁺ thalassemia

Abstract

Sickle cell disease is a congenital condition and its most common clinical manifestation is anemia due to chronic hemolysis. Persistent and accelerated hemolysis associated with multiple transfusions is a recognized risk factor for the development of cholelithiasis. The occurrence of gallstones is one of the most important manifestations of sickle cell disease in the digestive tract. Most gallstones are pigmented and characteristically occur at younger ages and the prevalence of cholelithiasis increases progressively with age, affecting 50% of young adults. Cholecystectomy is the most common surgical procedure performed in sickle cell disease patients. Anesthesia in this population of patients for major surgeries deserves special attention due to various complications particularly silent infarctions of end organs are common. We are reporting a 14-year-old girl diagnosed with sickle cell anemia and β+ thalassemia with cholelithiasis went for cholecystectomy under general anesthesia. Although the patient has both β+ thalassemia and sickle cell disease component, the latter is of more concern for anesthesia.

Sonographic Assessment of Some Abdominal Organs in Children with Sickle Cell Disease in Ilorin, Nigeria

“…This is similar to previous studies carried out by Bharati et al . ;[ 16 ] Balci et al . ;[ 17 ] Luntsi et al .…”

“…;[ 13 ] Bharati et al . ;[ 16 ] Luntsi et al .,[ 14 ] also reported splenomegaly as the most common splenic abnormality. Causes of splenomegaly include acute splenic sequestration, infectious or granulomatous disease, malignancy, and other hematologic diseases.…”

ANAESTHETIC MANAGEMENT OF a PATIENT WITH SICKLE Β+ THALASSEMIA WITH CHOLELITHIASIS

“…Anesthetists have to demonstrate a judicious planning of anesthesia and analgesia and meticulous execution of the strategies devised in all cases of Hemoglobinopathies. [24][25] In conclusion, maintenance of adequate hydration preoperatively, avoidance of acidosis, hypoxia, hypovolemia, hypothermia reduced the perioperative sickle-induced complications. Good intraoperative and postoperative pain relief, oxygen therapy, incentive spirometry sand regular ABG monitoring played an important role in successful management of the case.…”

Dexmedetomidine as sedative and analgesic in a patient of sickle cell disease for total hip replacement

“…The basis of anaesthetic management has traditionally been avoiding the factors known to increase erythrocyte sickling and precipitate the vicious cycle of vaso-occlusive episodes (VOC), such as volume depletion, hypoxemia, infection, acidosis, hypothermia, hyperthermia, decrease in 2, 3 DPG. [1] Sickled cells have increased adherence to endothelium leading to micro-vascular infarction and pain, the basis of VOC. [2] The risk intrinsic to the type of surgery should be considered.…”

“…The main goals of the anaesthetic management in this case were adequate analgesia, early mobilization to prevent stasis and deep vein thrombosis, oxygenation to avoid hypoxic episodes,[1] adequate hydration and thermoregulation.…”