Hbh Disease (Deletional 2 globin Alpha SEA and 1 Deletional alpha 3,7 kb)

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Hello all forum's member...
i am new in this forum and this is my first post.
This forum is very complete all about thalassemia but sometimes I am still confused so please help...

i live in indonesia and i have 2 YO child.
The First time he diagnosed with alpha thalassemia ( HbH Disease ) is when he was 13 Month. At that time, he has fever, diarrhea , cold and flu for 10 days. So I decide to do blood examination worrying about high incidence of dengue fever in Indonesia.

He is very active child,  No Splenomegali, Body weight= 11.2 KG, Height  = 85 cm, still can not talk ( 10 words), sweat easier than others.

The latest haemoglobin level 8.8 g/dl ( May 18).

DNA Analysis : Double Heterozygot Deletional 2 gen globin  Alpha type SEA and One Deletional Globin alpha type 3,7 kb ( -- SEA / α-3,7kb).

Hb Electrophoresis / Hb Analysis /  HPLC:
HbA2       :  < 1.7%
HbF         :  < 1%
Other Fraction   : NEgatives

 Here is the history of blood exam in my child.



Until Now He never been get any transfusion.
We give supplementation folic acid 2.5mg + Vit E 100mg daily

My Question is:
1. Do you think transfusion is required ?   
 some of the local pediatric haematologic  suggest to do blood transfusion base on the Hb level…no matter the type of thalassemia…but some also suggest to monitor the growth with CDC chart growth before transfusing...but I postpone it because no symptomps...
2. are we in the correct  management?
2. What is the blood examination should we monitored in my childrdren?

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Offline Andy Battaglia

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  • Will thal rule you or will you rule thal?
Blood transfusion in this case should be based on growth and development. If the child is severely lacking in development, then transfusion might be considered, but with the knowledge that this opens the door to iron overload, along with other issues. Continue to monitor the Hb, but do not panic if one reading comes in low. Alpha thals will normally have a drop in Hb when ill, and for most alpha thals, this is the only time they will transfuse. In general, the rule is transfusions start if there are two consecutive Hb readings below 7.

I would like to know if the Hb has risen since you started folate. I would also suggest B-12 tablets that dissolve under the tongue be used.
Andy

All we are saying is give thals a chance.

Thank you andy for replying my email...

as a parents i am so worry...and until now i still read and learn about this condition....
i still have some question...

1. How can we know many of side effects are happening / in progress in my child? do we need to do another laboratory check other than we ever checked? so that in case if there are in progress side effects , we can do early prevention and intervention...

2. in my 2 YO child.  He can talk only around 10 Words. late develoment for his age, But he understand any command / instruction. Do you think this condition corelates with this thal intermedia?

3. do you think it is important to do HLA test? as a preparation if maybe someday thal intermedia become major...

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Offline Andy Battaglia

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  • Gender: Male
  • Will thal rule you or will you rule thal?
The main issues with HbH are low Hb when sick, and that can require transfusion if the Hb drops below 7 for two readings, and a lowered immune system.

The minimal speech at this age may be just slow development due to low Hb, but if the child's learning stays behind, there may be something else delaying development.

I don't think the HLA test is necessary. It can be done, but it won't change anything.

Most essential is that the child takes L-methylfolate and vitamin B-12 daily. This will help the Hb more than anything else. Vitamin D and zinc will both also help the immune system and will keep the child healthier. Establish healthy eating habits at an early age.
Andy

All we are saying is give thals a chance.

 

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