Ethnic: Mediterranean, African-American, middle east and Asian
-thalassemia o major (homozygote) (Cooley’s anemia) severe, need treatment o intermediate (hetro) o minor (hetro) o manifest after 1st year of life, when Hb F disappears. -thalassemia o 4 genes for the -chain synthesis o 1 gene deletion silent carrier. o 2 genes deletion mild microcytic anemia. o 3 genes deletion severe hemolytic anemia. o 4 genes deletion hydrps fetalis GI absorption of iron and with multi-Tx iron overload hemochromatosis . treatment : o Tx and folate in mild form o Multi Tx and iron chelating agent in the severe form o Splenectomy o BM Tx Anesthesia consideration: A prominent maxilla due to extra-medullary hemopiosis difficult airway B PE, pulmonary HTN, and restrictive lung disease. CV CHF, Arrhythmia due to iron toxicity , Pericarditis and pericardia effusion. CNS spinal cord compression due to the size of the vertebral bodies GI-Endo iron overload damage to Pancreas DM Thyroid and parathyroids hypo Hepatic failure Hema anemia and it’s severity , coagulopathy MSK cortical thinning easy # (position)