Iron Chelation Practice in Kuwait Adekunle Adekile
Following the Policy of the National Regulation 3.3 , page 17, on CME disclosures, dated 5 November 2009, and on behalf of the Provider , - Collage S.p.A.- n. 309 I ( Adekunle Adekile ) HERE DECLARE DISCLOSURE OF RELEVANT FINANCIAL RELATIONSHIPS YES , -over the past two years – I do have a personal financial relationship with a commercial interest and control over educational content related to the products and/or services of the commercial interest(s). *if yes please provide information below. - …………………………………………… - ………………………………………….... - NO , have no relevant personal financial relationship in the medical/health field. DISCLOSURE OF PROMOTIONAL TALKS NO , I have not presented any promotional talks for any pharmaceutical companies within the past 12 months YES, I have presented promotional talks for one or more pharmaceutical companies within the past 12 months *if yes please provide information below. - ……………………………………………. - ……………………………………………. I understand that continuing education accreditation guidelines prohibit me from accepting any reimbursement (financial, gifts or in-kind exchange) for this presentation from any source other than the accredited CME provider ( Collage S.p.A.) 15-16 September, 2017 Adekunle Adekile
Kuwait • Land area – 6,880 sq. miles; population ~4.5million • 6 governorates
Thalassemia in Kuwait • No current registry • Estimated ~500 TDT both pediatric and adult • Cared for in different hospitals • Most pediatric patients are seen in the Department of Hematology and Oncology in Sabah Hospital • My unit sees pediatric patients in 3 hospitals • Diagnosis based on family history, clinical presentation, HPLC and molecular studies
Patients analyzed • Total 39 • Males 22 • Females 17 • Mean Age 7.7 ± 3.7 (range 1 – 16 yrs) • Age at diagnosis 1.3 ± 0.9 (range 0.5 – 4 yrs)
Details of Chelation • Of the 39 in the study, 32 (82.1%) were on exjade • 27 (84.4%) had serum ferritin <2,000 • Duration of chelation 4.8 ± 3.0 years (Max 11 y) • Currently, 26 (81.3%) have serum ferritin <2,000 • Combined therapy with deferrioxamine in 13 (40.6%) • All drugs are free for thalassemia patients
Serum Ferritin Levels
Liver Iron • Liver T2* obtained in 10 patients • 1 was normal • 8 had mild to moderate IOL • 1 had severe IOL
Exjade Tolerance • Very well tolerated • 2 cases of GI effects – abdominal discomfort and nausea • 2 cases of generalized rash • 1 could not tolerate and switched to desferal
Age an and lab laboratory ry par arameters s of of patie tients with ith NTDT in in Kuwait it Age Hb MCV MCH T. Bil Ferritin Retics Group (yrs) (g/dl) (fl) (pg) (mmol/l) (ng/l) (%) β TI (n=20) 12.5 ± 8.2 9.6 ± 1.2 67.0 ± 9.0 21.7 ± 3.1 34.5 ± 16.9 247.3 ± 1458.5** 2.5 ± 0.9 (2.5 – 36) (7.4 – 11.4) (51.6 – 87.0) (15.8 – 27.0) (14.0 – 80.0) (4.5 – 1463.0) (1.0 – 3.7) HbH (n=18) 12.2 ± 5.2 9.5 ± 1.2 57.7 ± 4.8 17.7 ± 1.4 19.8 ± 8.5 74.6 ± 61.8 2.2 ± 0.9 (6.0 – 24) (8.0 – 12.0) (47.6 – 65.0) (14.7 – 20.0) (10.2 – 37.0) (14.0 – 212.4) 0.5 – 4.0) Eβthal (3) 14.3 ± 17.9* 8.7 ± 0.8 64.8 ± 7.6 20.7 ± 2.4 69.0 ± 7.1 358.2 ± 532.2 3.0 ± 1.5 (3.0 – 35) (7.6 – 8.9) (56.0 – 69.5) (18.0 – 22.6) (64.0 – 74.0) (46.0 – 972.7) 1.9 – 4.0) All (n=41) 12.5 ± 7.7 9.4 ± 1.1 62.7 ± 8.5 19.9 ± 3.1 21.8 ± 18.0 1458.5 ± 281.1 2.4 ± 0.9 (2.5 – 36) (7.4 – 12.0) (47.6 – 87.0) (14.7 – 27.0) (10.2 – 80.0) (4.5 – 1463.0) (0.5 – 4.0) *Median 5.0 years **Median 171.7 ng/l
β -Thalassemia intermedia patients on Exjade Age (years) Ferritin (ng/ml) T2* (ms) Morbidity 22 267 3 Hypothyroidism 36 1,437 n.a. None 17 222 6 None
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