producing small amounts of adult hemoglobin?

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Offline avasmom

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producing small amounts of adult hemoglobin?
« on: January 27, 2009, 11:36:44 PM »
at 2 months old my daughter was diagnosed with thal major , at her 4 month check up she was doing well and doesnt need a transfusion yet .but in her most recent blood work it showed that she had produced a small amount of adult hemoglobin.she was born with none.they said there is a small chance that she would be ok with her blood levels and that there was a small chance she could be considered a thal intermedia. what do u guys think the chances are that this could happen? do any of u guys have adult hemoglobin and still get transfused monthly?and o thought if u made some adult hemoglobin that u were considered thal minor . please help!

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Offline Andy Battaglia

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Re: producing small amounts of adult hemoglobin?
« Reply #1 on: January 28, 2009, 01:37:58 AM »
Most beta thal majors are capable of producing small amounts of HbA. Only when both genes are beta zero would there be none (in HbE beta zero thal, there is also no HbA). When there is enough HbA produced to survive without regular transfusions it is called thal intermedia. If a DNA analysis is done it may be able to somewhat predict the outcome based on the mutations found. Some combination of mutations are more likely to lead to intermedia but this is not definite because there may be other factors that affect the total hemoglobin level, such as how much fetal hemoglobin is produced. In borderline cases, fetal hemoglobin inducing drugs are used to try to keep the Hb level high enough to avoid transfusions. There is a small chance your child can avoid transfusions and this won't be known usually until 6-12 months because fetal Hb levels remain high until 6 months, but it is a small chance. More and more, even borderline patients are being transfused so the growth is not negatively affected by low Hb levels.
Andy

All we are saying is give thals a chance.

Re: producing small amounts of adult hemoglobin?
« Reply #2 on: January 29, 2009, 01:47:16 AM »
Our daughter had very little Adult Hemoglobin and the rest was fetal hemoglobin.  She is now being transfused, but was able to maintain a high enough Hemoglobin level until she turned 2 years 7 months (she was at a 10).  Her doctor then wanted to begin transfusion so she would not be negatively affected in growth as Andy said.  They also told us they did not want her to have frontal bossing (spelling?), or any other problems.

She gets a transfusion monthly - they do not want her hemoglobin level to drop below 10. 


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Offline Zaini

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Re: producing small amounts of adult hemoglobin?
« Reply #3 on: January 29, 2009, 03:12:22 AM »
Hb of 10 at 2 years and 7 months without any transfusion is good,did you do the DNA analysis?

Zaini.
^*^Xaini^*^

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Offline avasmom

  • mom of a beutiful baby girl with thal major
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  • Gender: Female
Re: producing small amounts of adult hemoglobin?
« Reply #4 on: January 30, 2009, 08:42:50 PM »
we did have dna testing done. me and my husband actually have the same exact genetic mutation.they said that it used to be if u made any adult hemoglobin that u were considered a minor but now its really based on every individual case.just wanted to know what u guys had to say about it cuz u guys are always so helpful. ty all
                                        avasmom=)

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Offline Sharmin

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Re: producing small amounts of adult hemoglobin?
« Reply #5 on: January 30, 2009, 08:56:20 PM »
Wishing you the best avasmom :) I hope that our little sweetie Ava is doing well.  It is wonderful to see how informed you are and what a great job you are doing.  Please let us know if there is anything else that we can help you with :)

Sharmin
Sharmin

 

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