HBH

My daughter was diagnosed of HBH when she was one month old and had a transfusion at that time.  Her hg was 6.7 before transfusion.  She is 2 months and 3 weeks old now and her hg is 7.6.  We will have a check up again 3 weeks later and I am afraid she may need another transfusion soon.  HBH is supposed to be Thal Intermedia, why my daughter will need transufion when she is only one month old?  I see some of the Thal major children have transfusion a lot later than her.    I am so worried.  When I read the forum and see those babies with Thal intermediate or major, it really broke my heart.  They are so innocent and they do not even know what is going on.

Hi CLS,

Your daughter's Hb is very low for her age and it does seem likely she will soon need another transfusion. HbH patients often need regular transfusions and chelation. Their bodies make very little useful hemoglobin and in addition, the hemoglobin H damages red blood cells shortening their lifespan.

Much of the information on the internet about alpha thalassemia is outdated and inaccurate. For some good explanations of alpha thalassemia and HbH disease, go to

http://www.labtestsonline.org/understanding/conditions/thalassemia-2.html

Hemoglobin H Disease (3 affected genes). With this condition, the large decrease in the amount of alpha globin chains produced causes an excess of beta chains which then aggregate into beta4 tetramers (groups of 4 beta chains), known as Hemoglobin H. Hgb H disease can cause moderate to severe anemia and splenomegaly (enlarged spleen). The clinical picture associated with HbH disease is extremely variable, however. Some individuals are asymptomatic while others have severe anemia. Hemoglobin H disease is found most often in individuals of Southeast Asian or Mediterranean descent.

http://www.emedicine.com/ped/topic955.htm

Symptoms are consistent with a chronic hemolytic anemia and include episodes of severe pallor and anemia. Infections, fever, ingestion of oxidative compounds, or drug use may precipitate hemolytic episodes, and patients may require transfusions. Generally, HbH disease is thought to be a mild disorder. However, because of the marked variability in degree of anemia, patients may range from asymptomatic to needing periodic transfusions to having severe anemia with hepatomegaly and splenomegaly....

Medical Care:

    * General supportive care: Transfusions may be needed periodically or in periods of severe anemia, such as during parvovirus infections. Usually, patients with HbH disease live fairly normal lives and require few transfusions. Hb levels usually range from 7-10 g/dL. Transfusion therapy is reserved for patients with severe anemia (usually <7 g/dL) and symptomatic anemia.

Time will tell if her Hb levels remain as low as they have been. If they do, she may require occasional or even regular transfusions. Folic acid supplements are recommended even for infants. Early intervention to maintain Hb levels may be needed to prevent skeletal deformities.

Andy,

Thank you for your reply.

I was hopeing that she will only need occasional transfusion when she is sick or has infection.  The thing I am most scared of is regular transfusions.  I know that we will need more time and data to find out.  I felt so guilty of myself because I am a silent carrier.  I have knowledge about thal so I have asked Dr. if I have thal and they all said I have no thal. 

I really hope that my daughter will not need regular transfusion which I think will affect her life.  That's why I want to get more feedback from other hbh patients telling me that they do not need regular transfusion and still lead a normal life.  It seems to me that most of the people in this forum are of beta thal not hbh so I may not be able to get a positive response to ease my worry in this waiting period. 

The first thing I do in the morning is to check her face and hand and feet to see if they are red.  Every time when I see some red on her face, it will really make my day.  This is the little thing I pray for everyday now.

Hi CLS

Welcome to the forum and i am very sorry to hear about your daughter, but please don't feel any guilt because you didn't know and all of the parents here didn't know either, so it is just fate that we should accept. This feeling will just hinder you. Now you should be strong in order to take good care of her so thatshe can lead a normal life and remember that now a days there are more hopes for a cure than the old days .

You are right most of the people here are beta thal, but i guess that there are one or two members who has the HB H but unfortunatly they don't log daily

Anyway, i am sure that gathering a lot of information is the best thing to do right now and i am praying with you for your baby and for all our children to see this red face soon.

Take care

Manal


PS: Haven't doctors heard of something called silent carriers before or they just take things for granted

Manal,

I felt guilty of myself because I am fully aware of the presence of thal and specifically asked husband to check and knew that he is thal minor but we did not try to figure out more as my dr. said I have no thal.  If I would have researched more and not jumped to conclusion, I would have been able to prevent this.  I do not understand why the dr. not aware of silent carrier.  When dr. told me that they suspected that my daughter has thal intermedia, I called my 2 dr. who delivered my 2 girls for me and they both said that I am not thal.  Anyway, I am blessed with an elder daughter who does not have this issue.  I did not do amnio during my pregnancy, but even if I found out my second one to have thal, I would never think of giving up and will still try my very best to take care of her.  I am praying everyday that she will not need regular transfusion and will be able to lead a normal life.  Is it true that intermedia means no regular transfusion?  Can I jump into conclusion like this?   I believed that God gave this child to me because he has trust in me and my family that we will have the strength and love to give the best care to this child.

We need to see all of them with lovely red face.

Hello,

Intermedia is a bit complicated as it is an intermediate form of Thalassemia between Major and Minor. Therefore you have to consider a cautious approach.

Although you may not require her to transfuse in the early years of life, but that might change as she grows up. It all depends on her blood counts; if her Hb drops too low then there is no option but to transfuse. however Intermedias are said to maintain their Hb around 6-7 with the help of HbF inducers such as Wheat Grass, Hydroxyurea and Resveratrol etc unlike Thal majors whose Hb keeps falling down.

Apart from that you should also keep her serum ferritin level in check as the body tends to absorb more iron from food if the Hb. levels are low. This might lead to iron overloading and would require chelation therapy lest the excess iron damages the vital organs with time.

I hope that you will be able to manage her by these HbF inducers and hopefully she will never require transfusions.

Wishing you all the best.

Take care, Peace!

§ãJ¡Ð ساجد


I wonder if HbF will help in the case of Alpha Thal as HbF also requires Alpha for the production. My daughter's case is Hbh, 3 Alpha are missing and has only 1 Alpha.  Does HbF require less Alpha than HbA?  Do you know of any inducer which can help making more alpha?  My daughter is taking 1 mg folic acid a day now.

Silent carrier alpha thal is very hard to diagnose. It doesn't show up in most electrophoresis tests so it is understandable that the doctor didn't know about it. There is no reason for you to feel guilty as only a DNA analysis would have had a chance of finding it. Family history is part of how alpha thal is diagnosed so it's important that all possibly affected family members are aware of it, now and in the future.

HbH symptoms can greatly vary. As it is studied more, researchers are seeing that there are some severe cases, where regular transfusion is required. However, most patients are able to live with infrequent or no transfusions.

Andy,

Our dr. has also said that hbh may have servere anemia but this is very rare.  The worst case senario he thought would be to have transufion every few months. 
But the other dr. has also mentioned that they are concerned on the low hg my daughter has at such an early age.  I was hoping that they do not have a lot of data for the children so small as usually this may not be detected until they get older.  I see that a lot of children are diagnosed after 15 months old.  These children may also have low hg when they were smaller like my daughter but the dr. has no data of this finding.

Do you know of any hg indcer which can help alpha type thal?

I want to share with you some good news.  We went to see Dr. yesterday and my daughter's hg is 8 now as compared to 3 weeks ago at 7.6.   I hope she will keep her hg at a higher level.
Her growth rate is very good.

That's GREAT, CLS!  I'm so happy to hear that! 

I want to share with you some good news.  We went to see Dr. yesterday and my daughter's hg is 8 now as compared to 3 weeks ago at 7.6.   I hope she will keep her hg at a higher level.
Her growth rate is very good.

Hi CLS,

That is good news indeed. I hope she continues this upward trend in her Hb and never require a transfusion. Amen.

Take care, Peace!

Wishing u the best,,keep us updated in future.

ZAINI.

That is very good news. If her Hb can stabilize in this range, the most she will need are occasional transfusions.

Thank all of you for your kindness.

I hope she can at least maintain this level if not higher.

I told dr. that there is not much information about hbh from the internet but I can get more information about beta thal intermedia.  And ask him if hbh is similar to beta thal intermedia.  He said no but did not explain the difference.  I really want to talk to someone who has experience in hbh so I can eqipped myself better to prepare the chanllenge ahead.  My heart still ache a lot when I look at my daughter.  Maybe I will feel better when she grows older and can tell me she is ok with this and that it does not affect her a lot.  Since she is still a baby, I do not know if she is feeling ok or not which make me worry all the time.  But you guys are my role model to encourage me to be brave like all of you to face this together.  Thank you so much.