Professor Joy Ho

speaker

23 appearances 1 recordings 1 series first heard Aug 2016 last heard Aug 2016

Professor Joy Ho’s voice in public audio — every appearance, attributed to the second.

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Health Report · Blood · 22 Aug 2016 podcast

So thalassemia is a genetic disorder which affects globin, which is a crucial protein forming hemoglobin in red blood cells.

The form of thalassemia which particularly impacts life expectancy in terms of becoming transfusion dependent like Peter and Arthur.

Is beta thalassemia major?

These patients become anemic.

And they do not have enough red blood to survive.

So patients with beta thalassemia have to be transfused to stay alive.

So I'm pleased to say I wasn't yet in clinical practice then.

Uh however, it is necessary to try to remove the iron

that comes with the transfusion because the main cause of mortality is the accumulation of iron

in major organs and they are the heart, the liver and the hormonal glands, the endocrine glands.

But the main cause of mortality is in fact heart failure.

In the old days, the life expectancy was around thirty years and patients sometimes die in their twenties and as you've heard from Peter and Arthur, they are two of the older patients we have

So they will tell you that when they grew up it was almost unbelievable that they could be still here.

out.

The drug called desveroxamine revolutionized the treatment of thalassemia in that it was possible to chelate the iron.

So this is a drug

that binds the iron and then the iron is got rid of from the body through the urine.

Because of a very short half life, it doesn't last so to speak.

So when you take it by mouth it can't get into the bloodstream.

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