Behavior & Eating Habits of Thal Major Child

Dear All,

My son of 2 years 9 months is a Thal Major, his hb level has been at 7.2+ for the last few months. Of late is absolutely refuses to eat anything and whenever we force him he brings it out. I am worried as he is really thin and weak compared to children of his age.
What should I do to ensure that he get all the proper nutrients he requires and his body growth is not hampered. Does this mean that we need to transfuse him, please guide me.

Regards
Ben.

7.2 Hb is very low!

Your pre-transfusion Hb goal should be above 9.5 or at least around it somewhere for him to grow normally.

Refusing to eat, spitting out are often well known symptoms that low Hb exhibits. Your child is having mood swings and is obviously not well as he lacks energy. Transfuse him ASAP.

-P.

I agree with Pratik, The appetite gets worse as the Hb drops. Today's protocol is to never allow the Hb to drop below the 9-9.5 range. There is no advantage to keeping a lower Hb and it creates many issues for the patients.

Hi Pratik & Andy,

Thanks for the advice, can you please guide me with some good doctor in Calcutta, WestBengal India.

Regards
Ben

I would also like to add that we have not transfused him even once, he has been keeping at 7.2 Hb level for the past 6 months. I really pray that I have not done any harm to him but not transfusing him all these while. I will consult the doctor and get him transfused this coming week itself. Are there any tests I can do to ensure that all is fine with him.
Moreover since this will be his first transfusion are there any specific precautions I nee d to take.

Please help.

Regards
Ben

Hi,

Are you sure that your child is not major but intermedia? Because I really doubt a thal major child would maintain Hb levels, but intermedia's can.

Sorry, I'm not aware of any docs or healthcare facilities in Bengal. But others might be knowing.

And don't worry, there was no harm done. Keeping low Hb just makes your child have mood swings and such things but long term of these may affect development and growth of his body so make sure you transfuse your child by keeping Hb around 9.5 or above it (low Hb also causes more workload for heart to pump oxygen and purify blood, so you don't want this to happen) while using chelator to chelate iron out of his body.

There is a topic somewhere here where Andy mentions all necessary pre-tranfusion and other half a yearly and yearly tests.

The most common tests that need to be done before transfusion are CBC (Complete Blood Count), SGPT/SGOT (ALT/AST - liver function tests), Serum Creatine (kidney function), that's it I guess.

Other tests such as HIV, Hepatitis should be carried out once in every six months. And yearly tests may include some mineral tests (such as calcium, zinc, vitamin e), FBS (Fasting Blood Sugar), Vitamin D. And when your child starts growing old (around 8 and above), tests such as BMD (Bone Mineral Density/Dexa Scan), etc should be done for proper management and to ensure normal growth response.

Also, one golden rule - keep ferritin as low as you can (around 500 is best). As long as the ferritin and Hb (greater than 9) is normal and within norms, your child will grow normally.

I also recommend reading below topic by Sharmin as it will give you a great insight:

http://www.thalassemiapatientsandfriends.com/index.php/topic,4669.0.html

Best,

-P.

Hi Pratik,

Thanks for your inputs. HPLC test done for him mentions that he is a Thal Major however the doctor whom we consulted had mentioned once that presently he is behaving liking an intermediate, hence we did not need to transfuse him all this while, he was keeping Hb level of 8.8 almost till Oct 2012 but after viral fever his Hb level fell down to 7.2 and he has been keeping it at that since then.
I will be doing his Hb level test and consult the doctor tomorrow, will keep it posted. Thanks again for all the information.

Regards
Ben

Andy,

What do you think? I guess the little one here is behaving as intermediate, because any major child would require transfusion, won't he?

-P.

Ben,

Do you have the results from the HPLC test? The Hb level is that of an intermedia, but the child may need transfusions, as it sounds like growth an activity are below normal.

Hi Andy
Hiw HPLC tests mention that he is a thal major. I have got his reports for Hb level yesterday and it now reads 8.0. It was 7.2 in the last reading taken in Jan 2013. The local doctor whom I consulted has said that he does not require transfusion.

His is otherwise an active child playing around most of the time, only his apetite has gone down drastically.

I was to consult some centre in India who specialize in dealing with Thalesemia, can you please guide me.

Regards
Ben

Hi Andy
Hiw HPLC tests mention that he is a thal major. I have got his reports for Hb level yesterday and it now reads 8.0. It was 7.2 in the last reading taken in Jan 2013. The local doctor whom I consulted has said that he does not require transfusion.

His is otherwise an active child playing around most of the time, only his apetite has gone down drastically.

I was to consult some centre in India who specialize in dealing with Thalesemia, can you please guide me.

Regards
Ben

No way he could be thal major I strongly believe. His Hb has increased suggesting possibility of being an intermediate.

-P.