Pediatric Hematology Unit H

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Pediatric Hematology Unit HEamek Medical
Center Afula, Israel

• Koren A. MD, Levin C. MD
• Grinberg B. RN, Mary M.SW

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Chronic hematological patients

• Coagulation disorders
• Bleeding, Thrombotic diseases
• Thrombocytopenia
• ITP, Fanconis Aplastic Anemia
• Neutropenia
• Kostmmans, Fanconis Aplastic Anemia
• Chronic Anemia
• Sickle Cell Disease, Congenital
  Spherocytosis, Thalassemia

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ßThalassemia is a genetic disease, that starts
merely as hemolytic anemia but becomes a chronic
disease with multisystem involvement impairing
organ and tissue function.
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ßThalassemia Prevention

• Autosomal recessive disease
• Prevention of the disease is possible
• through genetic counseling

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ßThalassemia Treatment

• BMT is the only definitive cure, limited by HLA
  matching donor and associated complications
• In best conditions still associated with 5
  mortality and 10 morbidity
• Conservative treatment Blood transfusions and
  Iron chelation, Fetal Hemoglobin inducers

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ßThalassemia Treatment

• Regular Blood
• transfusions
• every 2-4 weeks for life
• Transfusions begin from
• the first year of life
• (Pre-transfusion Hb level
• of 9.5g/dl)

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ßThalassemia Treatment

• Iron Chelation therapy for life
• Access to new drugs improve quality of life

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ßThalassemia Treatment

• Iron Chelation therapy for life
• Access to new drugs improve quality of life

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ßThalassemia Complications

• Most of the complications are
• related to Iron overload
• Can be prevented by
• adequate iron chelation therapy

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ßThalassemia Complications

• Cardiac disease
• cardiomyopathy, arrhythmia
• Continues to be the most
• frequent cause of death
• Infection high susceptibility due to excessive
  Iron availability and splenectomy

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ßThalassemia Complications

• Liver disease related
• to iron overload and/or
• HCV infection
• Thrombotic disorders
• Osteoporosis
• Pulmonary hypertension

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ßThalassemia Complications

• Endocrine Abnormalities
• delayed growth and puberty,
• fertility problems,
• hypothyroidism,
• hypoparathyroidism, diabetes
• Bone deformities due to bone marrow hyperplasia

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Living longer, living better

• 68 of patients live beyond age 35
• Quality of life improved dramatically

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ßThalassemia Coping

• Intensive and demanding treatment
• Growth retardation, sexual immaturity
• Facial deformities
• Congenital nature of the disease
• Fear of death
• Social acceptance
• Cultural and family expectations

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Medical care
Psychologist
Transfusion unit
Hematologist team
Cardiologist
Nutritionist
Endocrinologist
Thalassemic patient
Genetic counseling
Occupational therapist
Gynecologist/ fertility
Inpatient unit adult/ pediatric
Osteoporosis team
Blood bank and Laboratory facilities
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Psychosocial support
Education
Economic support
Self help groups
Occupation
Thalassemic patient
Social worker
Caregivers support
Activities/ sport
Family support
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Our patients

• 64 patients with ß-thalassemia major and/or
  intermedia
• Age range 1year to 42 years old
• 37 patients receiving regular blood transfusions
• 35 patients receiving chelation therapy

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Patients social characteristics
Adult not working or studying College/ University High School School Pre -school Amount
11 1 8 8 5 33 Arab Muslim
1 1 - 1 3 Arab Christian
1 1 Jew
12 2 9 9 5 37 Total
No Adult patients working
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Thanks to the staff for all the hard work!