plz guide me

dear my niece najaf zahra has got beta thalassemia
and i dont know at what stage she is
she is 4 years old
plz guide me

Hello and Welcome to thalpal.

The prognosis of thalassemia has changed leaps and bounds and your niece has medical advances on her side. There is nothing that can stop her from growing as a healthy person, if she complies to known treatment for Iron Overload.

Is najaf on transfusion? if she does take transfusion, does she take medicine for chelation (to remove iron from the body)? There are many members today who are in their 40's and 50's living a normal life and there have been many advances for the treatment and cure for thalassemia.

From : http://www.thalassemia.com/prognosis.html

The life expectancy of children who have used Desferal since a very young age remains to be seen, though researchers and physicians expect to see long, productive lives.

In affluent countries, a child who is born with thalassemia major (either beta thalassemia or alpha thalassemia) will face a lifetime of monthly blood transfusions and nightly infusions of desferoxamine. Over the past several decades with the progress of medical technology,this once universally fatal disease has been converted to a chronic illness.

Do ask questions and there are many members who can share their experience with you.

she does not take blood regularly so far in 4 years she has got blood only 3 times once in 6 month age and second time in september 09 and third time in october
she has been very recently diagnosed
doctor is giving her only folic acid
plz help me

If she does not take regular blood transfusion, she seems to be a thal (intermedia) and needs to be properly managed. It seems like her body is making some hemoglobin and you might want to find out ways for her HB to increase (Possibly with the use of Hydroxy urea or similar medication). Talk with the doctor in regards to it.

Do you know what hemoglobin levels is she maintaining? It is important for a growing child to maintain a proper hemoglobin level so that she can grow up normally. Could you tell us where she resides so that we can point you to some good doctor's around (if we know the location).

Also, drinking tea with food with help her reduce Iron Absorption. Iron is absorbed from food and intermedia's run into Iron overload even if they are not transfused regularly.

Another link I just saw would be helpful for your niece.

http://www.cooleysanemia.org/updates/TI3.pdf

In children with thalassemia intermedia, there are many decisions to be made by the health care team and the family in terms of transfusion. Frequently, your child’s doctor will only give transfusions if the hemoglobin falls below a certain value on several occasions. Other times the team will decide to give chronic transfusion for a certain time period and then re-evaluate. These decisions depend on the well–being of your child and how he or she feels. It is hard to predict in what category your child will fall; therefore, the child will require frequent check-ups with your doctor

So, as mentioned earlier the doctor treating her, would need to evaluate her on a regular basis.

Again from the same link above

Are there treatments that can increase my hemoglobin so that I don’t have to get transfused?
Some experiments with chemotherapy classes of drugs, such as hydroxyurea, have increased the amount of fetal hemoglobin in a patient, possibly increasing the baseline hemoglobin one to two grams or reducing transfusion requirements. Some patients have been prescribed drugs such as Procrit or Aranesp, that stimulate the creation of red blood cells; however, red blood cells created in this manner still contain defective hemoglobin. As a result, the patient simply makes more “defective” red blood cells, which do not alleviate the anemia.

Other medical therapies are being explored which attempt to increase the hemoglobin via means other than transfusion. At the current time, however, transfusion remains the most effective way of raising or maintaining hemoglobin levels for most patients.

thanks for reply
she has a hb level between 7.5 and 7.9 before transfusion
once in september she had hb level at 7.2 then she got transfused and raised to 11.2
she is having only folic acid

Hi,

Your niece seems like an intermedia,can you tell me from which city in Pakistan you are and from where your niece is getting her treatment?

Zaini.

i m basically from toba tek singh near faisalabad
she has been diagonised last week then we got her to children hospital lahore where the doctor didnt do any tests and advised her to take folic acid for 6 weeks and then for next check up
wht to do now?

 

Sharmin

Doctor didn't do any testing? to check thal status,there is a test called HB Electrophoresis,was it ever done on your niece? If yes then when and what was the result?

Zaini.

previously a doctor in multan took some tests and i think electrophreseis too
i m stating some of her results if u could understand
Hb A2 2%
Hb F 55.6%
Hb A 42.34
global chain variant = N/C
possible genotype B+/B+
VDLC/ TLC=18400
Poly 37%
lympho 56%
retics 18.8%
platelets 140000
normoblasts 16

below is the quotation by the testing lab

" severe hypocloromia, microsytosis. moderate macrocytosis, anisocytosis, poikilacytosis, many spherocytes, pessay cells, target cells and normoblasts.

What age was she when this test was done?

this test has been done on october 26, 2009. she is 4 years and 1 month old

hi everyone
my niece had a hb level of 11.2 on september 15
on october 26 it dropped to 7.6
is it too dangerous>
she is hoped to be thal intermedia

It definitely looks like intermedia. Majors have very little, if any HbA. If her Hb drops much more she will have to be transfused. Much depends on how well the child does without taking transfusions. If the child is not healthy, regular transfusions may be needed. I think Zaini may be able to give you some guidance on this, as it looks similar to her daughter's intermedia. However, be patient. Zaini has the flu and I don't know how much energy she'll have right now.