New from Malaysia

Hi!  I'm Noraini from Malaysia, working as Physician at one of the hospitals in Malaysia.. I know i have thal trait ever since i conceived my firstborn.. my husband went for screening (just using FBC) was told to be normal.. so i never had worry whenever i got pregnant..

So i have 1 girl & 4 boys, ranging from 16 months to 8 yrs.. a few weeks ago i noticed that 2 of my sons aged 6 yrs 2 months & 5 years 2 months have got a spleen.. so i took them to hospital to have some tests.. the 6 yr old had Hb 5.8, with MCH 18.2 & 5 yr old had Hb 6.8 MCH 18.. Both full blood pictures suggestive of thalassaemia intermedia... obviously after knowing this i sent my husband for check up last week and it turned out his MCH was 26.8, which was just <27... so the whole family went for Hb Electophoresis last week & i am still waiting for results to come back this week...  My daughter who is 8 has  a Hb 13 with MCH 24... my 2 younger sons, one is 3 yr & 2 months had HB 9.8, with MCH 18.2... and the 16 months had Hb 11.5 with MCH 18... as for me i always have Hb abaout 10 with MCH 19..

I was very sad for my kids.... i was not expecting this news especially when part of my training did involve looking after thalassaemic patients...  so my first boy had the first transfusion last week as he did look rather tired... the second boy was still ok.. obviously i am worried about their spleen though... i hope it wont get bigger too soon..

I have to say all my paedatric colleagues have been so supportive.. we do access to daycare & my hospital is one of the good hospitals for thalassaemia in children... i found this website & found it very resourceful & gave me the innerstrength to carry on as normal... my 2 boys are very active... i must say that they dont get unwell that often... if i had not examined their tummy, i wouldnt have noticed it that they were actually pale...

Hope to get more input... in my case the possibilities for them to be Hb E/beta thal is high as we are from South East Asia

hi again everyone.. I have received the preliminary results for all of my family members

Husband: E thalassaemia trait
Myself: beta thalassaemia trait
Daughter: E thalassaemia trait
1st son: E beta thalassaemia (intermedia) Hb 5.8, transfused last week (does have spleen, about 4cm below left costal margin)
2nd son: E beta thalassaemia (intermedia) Hb 6.7, not transfused ( dose have spleen, 11 cm on ultrasound scan)
3rd son: beta thalassaemia trait
4th son: beta thalassaemia trait

Next appointment for the 2 of them will be on 12 March 2013..

I hope I can get others to share their experience dealings with children with thalassaemia intermedia/major... though i am within the medical fraternity but it is different when one has to deal with their own children...  at present, i am giving them folic acid, vitamins A, B, D, E, inc, magnesium, spirulina, wheatgrass drinks... will keep all of you updated with their performance

Thank you for the reports, as they explain things clearly. HbE beta thalassemia can have a wide range of phenotypes or physical manifestations. Some only have mild anemia, while others need regular monthly transfusions, but most fall somewhere in between. Research in recent years has shown that transfusions in HbE beta thals should not be based solely on Hb level, but instead on the physical health of each patient, as there is an adaptive mechanism in HbE beta thals that allows them to live with an Hb that would force others to require transfusions. Transfusions should be based on the physical condition of the patient, and many will need an occasional but not regular transfusions. Much depends on honest observation of the patients.

Your supplement program is a good step. Combine this with a healthy, nutrient rich diet that avoids junk foods and empty calories as much as possible. Encourage fresh fruits and vegetables to make sure they getting all the trace elements. also. Mild to moderate exercise should also be encouraged.

thank you andy for your comprehensive explanation..

i have started reading in details also about thalassaemia... i just knew things on the surface before...

will keep you updated once we go for the review on the 12th march

Welcome to the forum, Dr Noraini.

I'm from Sabah, where we had the most thal in the country. We will look forward on your updates.


Gracia

Salam kenal Dr. Noraini. Saya Berlian dari negara tetangga, Indonesia. Saya penderita beta thalassemia minor :-) .

hi Andy,

Just to get your opinion.. as i said before 2 sons of mine have E Beta Thalassaemia..

ist son, he is 6 yrs & 3 months.. in Feb his Hb dropped to 5.6, he did have a spleen of about 4 cm below the costal margin..his liver is normal.. he was transfused since he was very lethargic & did have a bit of frontal bossing.. following transfusion, he became very active & with all the supplements I am giving, 3 weeks later his Hb is 8.9g/dL.. so no transfusion was ordered last week..

my second son who is 5 yrs & 2 months, very active & noted to have a spleen about 5 cm below the left costal margin... his initial Hb was 6.7g/dL and last week when we went for the check up, it picked up to 7.1g/dL... he is a very active boy.. a bit small.. but i am not happy with the size of the spleen... what do you think if i were to ask them to organise regular transfusion just to shrink the size of the spleen?

the reason i am asking this is that they were talking about putting them into thal major transfusion regime if they fail to maintain Hb>7g/dL... my understanding is that if they need support for normal growth, you may introduce blood transfusion but after the growth spurt, you may consider stopping 7 look at the trend... waht say you

With HbE beta thal, the decision to transfuse should be based on the physical condition of the patients. The important questions are is normal growth and development slowed by the condition and is the skeleton developing normally? If the children are growing normally and have normal energy levels, transfusions are not necessarily required, as those with HbE beta thal most often do adjust to hemoglobin levels lower than what is typically the level where transfusions would begin. The spleen size is not at a dangerous size.

Hi Noraini...... Welcoming u here 

Don't wory.. I only have 2 children and both of them thal major. But thru this site, I found out lots of useful info
on how, what and where to get proper treatment for my children.

Do visit us at 'JJ Medical journey' @ FB and I'll share u lots of things related Thal .. 

Best regards;

Joyce
Sabah

I was diagnosed with hemoglobin E/beta thalassemia.  Not sure what this all really means but, I am very active individual in my 40s. I exercise regularly and I play paintball as sport with lots of running involve.  when I was younger in my teens and twenty, I was much more active. I used to exercise a lot and played various sports. I began to noticed that my spleen began to grow big during my college years. Never gave it a second thought until now. My doctor told me that I have iron overload...so, I guess I got to do something about it.

So, my point here is this: Don't worry too much about it. As long as your kids are not showing signs and symptoms of the manifestation of this disease process, let them be kids and let them grow up happy. If you "overly obsessed" about their disease and they have no symptoms, you are making it worse for them psychologically. Just them them grow normally and observe them unobtrusively as possible.
Hope that helps.
paints from NorCal.

Oh and another thing: I wish I had known this when I was younger. I also noticed that sweet sugary drinks like sodas or sweetened coffee/tea are not good for my spleen. My spleen feels the pain whenever I drink something sweet. It feels very tender and uncomfortable. Now, I drink dark bitter tea to help built up tolerant to spleen discomfort and help makes it non tender and "normal". No more sodas for me, just plain water and occasional beers.   

paints from NorCal

Welcome! My mother in law is thal intermedia, my husband is a carrier, and two of my 3 children (the girls) age 5y3m are at least carriers. This site is a wonderful (thank you Lisa, Andy, and friends) source of information and support.    Wonderful that so many people post. As you say with your son you didn't see the paleness, sometimes it's good to hear from others  related to their experiences as you may not see something if you aren't looking for it.