Hello, new member

Hello everyone,

First of all, I'm Dutch so English is not my primairy language. Hope you understand my posting.... :-)

Here's another HBH-patient. Well, it's not me but our daughter.
She's now almost 8 years old (in 2 months) and we adopted her when she was 10 months old.
She's originally from Southern-China, an area where thalassemia is known.
We live in the Netherlands (Europe) and there are not much people/children with HBH in our country. The children who have Thal. are thal. minor or beta thal.
The first medical reports from China (age 0-6 months) didn't indicate that she was a
"thallasemie-girl", but after we got home she was diagnosed with an anaemia that was first treated with iron.
The anaemia didn't get better, hb didn't get above 6. MCV, MCH and RDW were also not in a normal range.
So we have insisted to examine for thallasemia. With a DNA-test it was confirmed that she has HBH, at that time she was 1,5 years old.

Now, she's almost 8 and did get a transfusion in December 2004 (age 5) and 2006 (age 7). Both after having an infection.
She didn't recovered fast enough and her body (blood) couldn't handle it anymore.
Her "normal" Hb is between 5.3 and 5.6. She got the transfusion when she had an Hb of 4.8.
Normally she's stable at her normal Hb (see above). Sure, she doesn't have that much energy that children of her age have. But her friends keep that in mind and try to keep up with her. For her it's not (yet?) a problem. There are no other fysical problems, her spleen is a little bit to big but the hemotologist said not to worry.
She goes every 6 months for her check-up to the hemotologist. The last time she didn't like that anymore (my blood must be normal, I don't want to go...)
And takes folic acid 0,5 mg every day.

It is notable, however, that when she's in colder water (swimmingpool, swimming-course) her energy falls down. Because of that it took her 2,5 years to get her swimming-diploma. She gets that cold that she almost can't move.
She doesn't have any problems with heat.

Well, that's our story. Hope to see more "HBH- patients" here.

Christine (Netherlands)

PS: I did post this message also at the intermedia section, but that's maybe not the correct section for introduction....

Hello YuMei,

Welcome aboard,I am sorry i don't know much about HBH,But i think there are some patients here,And i am sure Andy would be helpful as always.

Take care,

ZAINI.

hi,
welcome on board to the site,thanks for sharing your story with us.
Best wishes 

Hello YuMei,

You will find a lot of information on Thalassemia through the net.

One of member on this forum - Zadkhi has Hemoglobin H and you can read her descriptive post at http://www.thalassemiapatientsandfriends.com/index.php?topic=531.msg3991#msg3991

You should try to get in touch with a doctor who specilized in Thalassemia and knows about Hemoglobin H. I was able to locate a medical center in the Netherland, where you might be able to get some help.

From:- http://www.lumc.nl/4080/patientcare/patientcare_COUN.html#Clinical%20Genetics%20Outpatient%20Clinic

If after reading this information you still have questions about heredity analysis, you can contact us at the phone number or e-mail address listed below. More information can also be found in the dutch brochure  “What’s hereditary?”

Clinical Genetics Outpatient Clinic, 5th floor LUMC
zone K5-R
telefone number : 071-5268033

From, what I can understand your daughter can be more affected by viral infections which may be due to increased hemolysis. From:- http://www.thalassemia.com/alpha_thal_3.html

Individuals who have Hgb H generally have a persistent stable state of anemia, which may be accentuated by increased hemolysis during viral infections and by exposure to oxidant medications,
chemicals and foods such as sulfa drugs, benzene, and fava beans ... Therapy for individuals who have Hgb H disease includes folate, avoidance of oxidant drugs and foods, genetic counseling education and frequent medical care.

Also, you should make sure - the type of food she eats and get her the best management of HbH
From:- http://www.med.cmu.ac.th/dept/pediatrics/06-interest-cases/ic-52/case52-page-1.HTM

What are the managements for this patient?
1. Immediate management:
a. Assessment of cardiovascular status
b. Oxygen supplement
c. Intravenous fluid to maintain adequate urine output
d. PRC transfusion
e. Supportive/symptomatic treatment for URI
f. Patient and family education

2. Long-term management:
a.  Patient and family education about Hb H disease: genetic basis, natural course, plan of treatment and complications
b.  Folic acid supplement 

Here is one more links related to HbH - for your reference
http://www.chw.org/display/PPF/DocID/21862/router.asp

Hello!  :D

Zadkhi is me and I'm the other Hemoglobin H patient here!

From what you've been describing to me, I need to ask if you know if your daughter has the classic Hemoglobin H (deletional) or the non-deletional Hemoglobin H Constant Spring.  They are very similar, but the Constant Spring seems to cause more severe symptoms.

Narendra's post is a good start in learning more about HGH and if you click on the link she gave you, you can find some more information I've collected.