What is thalassaemia?
- A genetic blood disorder whereby blood cells are unable to carry enough oxygen
When can you get it?
How do you know someone has Thalassaemia Major?
- Usually show symptoms from about 6 months: very pale, lethargic and cries a lot. And may have difficulty in feeding.
How do you know someone has Thalassaemia Minor?
What are the treatments for Thalassaemia Major?
- Regular blood transfusion (once 3 to 4 weeks)
- Iron chelation:
1) Desferrioxamine
2) Deferipone
3) Deferasirox
Is there a cure?
- Bone marrow transplant but it is complicated and difficult to get a matching donor.
How long is each blood transfusion?
Would not the patient have too much blood in the body?
Where does the old blood?
- A condition of Thalassaemia Major is that their red blood cells are insufficient and weak. Thus the red cells have short (shorter than normal) life span. By the end of the fourth week cycle their haemoglobin/ red cells count will be so low that they need to have transfusion to TOP UP.
- TOP UP means to replace the lost red cells. To do this, the plasma and white blood cells are separated and filtered out. Thus the patients are given only packed blood, namely red cells.
Do you have to pay for the blood?
Fortunately, in Sarawak blood transfusion is free. This is the result of very effective campaign and good public support to ensure that the blood bank has sufficient blood.
What is Desferrioxamine (Desferal)?
- The regular blood transfusions result in iron build up leading to iron overload, if left untreated will lead to vital organs failure (heart, liver, pancreas).
- Desferal is a drug that prevents the built up by breaking down and remove excess iron.
How often and how long the Desferal treatment each time/
- Desferal is administered daily
How it is administered?
- It is administered via a syringe attached to a battery-powered pump. It should be as slow as possible. It is infused slowly over 8 – 10 hours (usually at bedtime) beneath the skin with a pump for 5 – 6 times a week.
What is Deferiprone?
- Oral iron chelator available in tablet (Ferriprox®) or capsule (Kelfer® and GPO-L-ONE®). It is an option for patients who have problems with Desferal.
What is Deferasirox?
- The latest oral iron chelator. Should be taken once a day with an empty stomach.
When can a Thalassaemia Major start iron chelation?
- Treatment is started after when the serum feritin is above 1000 ng/ml
How prevalent is Thalassaemia in Malaysia?
- It is estimate that between 3 – 5% Malaysians are Thalassaemia Trait or carriers.
- Therefore it is important to have your blood screen to know if you are a carrier, and thus enable you to take preventive measures.
Reference: 1. Malaysian Society of Paediatric haaematology and Oncology (MASPHO); A Handbook for Nurses on the management of Thalassaemia; 2015 2. UK Thalassaemia Society from; http://www.ukts.org/home/html.
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