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Facts about the Disease |
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| What is Fanconi Anemia? |
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| Fanconi anemia was first described by a Swiss pediatrician Dr.Fanconi and
published his clinical observations, that mostly it affects children. These
children suffered severe life threatening aplastic anemia and their blood
countings will go down from 12 to 3 hb and the platelets count will be 15000
against the normal countings i.e. 4,00,000. Their blood systems could not
successfully combat infection. In addition, as a result of anemia, they were
chronically fatigued. Because their platelet counts were low, they suffered
spontaneous bleeding.
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| What is the Prognosis for an FA Patient? |
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No one is certain how long any given individual with FA will survive. This
illness is unpredictable. According to cases reported to the international
Fanconi Anemia Registry, the average life expectancy is approximately 22 years.
But life expectancy for any one individual can be quite different from any
“average”. |
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What are bone marrow transplantation?
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Bone marrow transplantation(BMT) and Umbilical cord blood stem cells are
procedures that restore stem cells that have been destroyed by high doses of
chemotherapy and /or radiation therapy. |
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What are bone marrow and stem cells?
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| Bone marrow is the soft, sponge-like material found inside bones. It contains
immature cells called stem cells that produce blood cells. There are three
types of blood cells : white blood cells which fight infection; red blood
cells, which carry oxygen to and remove waste products from organs and tissues
and platelets, which enable the blood to clot.
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| Research has established that
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| 1. |
FA is one of severe deadly inherited anemias. |
| 2. |
FA patients experience a high incidence of leukemia(18% - 20%) |
| 3. |
FA patients have a much higher incidence of cancer than the general population. |
| 4. |
FA patients may have a variety of noticeable birth defects, ranging from minor
to serious. These defects may affect every major system of the body. |
| 5. |
FA will have more common birth effect or features. |
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a. Short stature |
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b. Anomalies of the thumb and arm |
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c. Kidney(renal) problems |
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d. Skin discoloration |
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e. Small head or eyes |
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f. Mental retardation |
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g. Low birth weight and failure to thrive |
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h. Abnormalities of the gastrointestinal tract |
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i. Heart defects |
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Recent Development in Bone marrow Transplantation by using Stem Cells:
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The Stem Cells Bank will collect, test, preserve and make available stem cells
for recipients, who require transplantation. The cord blood was derived from
the umbilical cord, discarded as waste. It contained stem cells capable of
developing into any other cell type. The cord blood in the bank would be
available for those in need of stem cell transplantation after HLA typing was
done. When a match was detected, the required unit of frozen stem cells would
be transported to the hospital where the transplantation was scheduled. The
cost for the stem cell in Singapore could be as high as Rs.11 lakhs.
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Avinash
C, 9 year old student of Petit seminaire, Pondicherry
