2D ECHOCARDIOGRAPHY MONITORING

        In a Thalassemic patient with iron overload. Iron in excess is toxic to cardiac muscles causing cardiomyopathy

Induction of Board of Trustees
June 14, 2001



  • Mr. Proceso Ramos (parent)
  • Mr. Joselito Tongco (parent)
  • Dr. Ma. Jasmin Gonzales-Ruiz (Hematology fellow in charge)
  • Mrs. Esther dela Cruz (YAKAP PRESIDENT)
  • Mrs. Eloisa Dizon (parent)
  • Mr. Ruffy Sogocio (parent)
  • Mr. Antonio Henares (patient)
  • Mr. Abel Silva (parent )
  • Atty. Tranquil Salvador III (Husband of thalassemic patient)
  • INDUCTING OFFICER: Dr. Lolita Chua President, Philippine Society of Hematology and Blood Transfusion (PSBHT)

    DR. ALLENDRY P. CAVILES JR.

            founder of the “Balikatang Thalassemia” addresses the delegates of the “First Thalassemia Workshop” in June 14, 2001 at Quezon City Sports Club, Philippines
            Parents and friends of Thalassemic patients gathered during “Thalassemia awareness day” in December 14, 1994 at Childrens Medical Center of the Philippines Conference Hall

    IRON CHELATION THERAPY

            Removal of excess ironbrought about by hemolysis and chronic blood transfusion using desferrioxamine which is administered subcutaneously anddriven by a syringe pump over 8 hours period.

    Facts

            Alpha Thalassemia

            People whose hemoglobin does not produce enough alpha protein have alpha thalassemia. It is commonly found in Africa, the Middle East, India, Southeast Asia, southern China, and occasionally the Mediterranean region.

            There are four types of alpha thalassemia that range from mild to severe in their effect on the body.

            Silent Carrier State. This condition generally causes no health problems because the lack of alpha protein is so small that the hemoglobin functions normally. It is called "silent carrier" because of how difficult it is to detect. Silent carrier state is "diagnosed" by deduction when an apparently normal individual has a child with hemoglobin H disease or alpha thalassemia trait.

            Hemoglobin Constant Spring. This is an unusual form of Silent Carrier state that is caused by a mutation of the alpha globin. It is called Constant Spring after the region of Jamaica in which it was discovered. As in silent carrier state, an individual with this condition usually experiences no related health problems.

            Alpha Thalassemia Trait or Mild Alpha Thalassemia. In this condition, the lack of alpha protein is somewhat greater. Patients with this condition have smaller red blood cells and a mild anemia, although many patients do not experience symptoms. However, physicians often mistake mild alpha thalassemia for iron deficiency anemia and prescribe iron supplements that have no effect on the anemia.

            Hemoglobin H Disease. In this condition, the lack of alpha protein is great enough to cause severe anemia and serious health problems such as an enlarged spleen, bone deformities and fatigue. It is named for the abnormal hemoglobin H (created by the remaining beta globin) that destroys red blood cells.

            Hemoglobin H-Constant Spring. This condition is more severe than hemoglobin H disease. Individuals with this condition tend to have a more severe anemia and suffer more frequently from enlargement of the spleen and viral infections.

            Homozygous Constant Spring. This condition is a variation of hemoglobin H-Constant Spring that occurs when two Constant Spring carriers pass their genes on to their child (as opposed to hemoglobin H Constant Spring, in which one parent is a Constant Spring Carrier and the other a carrier of alpha thalassemia trait). This condition is generally less severe than hemoglobin H Constant Spring and more similar to hemoglobin H disease.

            Hydrops Fetalis or Alpha Thalassemia Major. In this condition, there are no alpha genes in the individual's DNA, which causes the gamma globins produced by the fetus to form an abnormal hemoglobin called hemoglobin Barts. Most individuals with this condition die before or shortly after birth. In some extremely rare cases where the condition is discovered before birth, in utero blood transfusions have allowed the birth of children with hydrops fetalis who then require lifelong blood transfusions and medical care.