Iron Chelation Practice in Kuwait Adekunle Adekile Following the - - PowerPoint PPT Presentation

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.

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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
• f 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

• f patie

tients with ith NTDT in in Kuwait it

Group Age (yrs) Hb (g/dl) MCV (fl) MCH (pg)

• T. Bil

(mmol/l) Ferritin (ng/l) Retics (%) βTI (n=20) 12.5 ± 8.2 (2.5 – 36) 9.6 ± 1.2 (7.4 – 11.4) 67.0 ± 9.0 (51.6 – 87.0) 21.7 ± 3.1 (15.8 – 27.0) 34.5 ± 16.9 (14.0 – 80.0) 247.3 ± 1458.5** (4.5 – 1463.0) 2.5 ± 0.9 (1.0 – 3.7) HbH (n=18) 12.2 ± 5.2 (6.0 – 24) 9.5 ± 1.2 (8.0 – 12.0) 57.7 ± 4.8 (47.6 – 65.0) 17.7 ± 1.4 (14.7 – 20.0) 19.8 ± 8.5 (10.2 – 37.0) 74.6 ± 61.8 (14.0 – 212.4) 2.2 ± 0.9 0.5 – 4.0) Eβthal (3) 14.3 ± 17.9* (3.0 – 35) 8.7 ± 0.8 (7.6 – 8.9) 64.8 ± 7.6 (56.0 – 69.5) 20.7 ± 2.4 (18.0 – 22.6) 69.0 ± 7.1 (64.0 – 74.0) 358.2 ± 532.2 (46.0 – 972.7) 3.0 ± 1.5 1.9 – 4.0) All (n=41) 12.5 ± 7.7 (2.5 – 36) 9.4 ± 1.1 (7.4 – 12.0) 62.7 ± 8.5 (47.6 – 87.0) 19.9 ± 3.1 (14.7 – 27.0) 21.8 ± 18.0 (10.2 – 80.0) 1458.5 ± 281.1 (4.5 – 1463.0) 2.4 ± 0.9 (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