Thalassemia Patients and Friends
Discussion Forums => Thalassemia Major => Topic started by: Keep Smiling on November 04, 2009, 03:15:20 PM
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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
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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.
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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
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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.
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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.
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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
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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.
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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?
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:welcome2
Sharmin
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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.
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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.
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What age was she when this test was done?
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this test has been done on october 26, 2009. she is 4 years and 1 month old
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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
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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.
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Hi,
This case is similar to my daughter's,the difference is she was diagnosed at age three and was put on transfusions right away.
Thal intermedia is bit tricky to understand,your niece ,as Andy said is thal intermedia,i'll advice you to transfuse her if her hb goes lower then what it currently is.
Basically there can be two approaches with intermedia,either you can try medicines like Hydrea,to raise her hb with out transfusions,but hydrea does not work for every patient,for some it does and their hb goes up by one or two grams,and for some patients it does not work at all,and as it has some side effects,so you have to constantly monitor the patient with blood tests.
Second way is to start transfusing regularly,to maintain hb on higher levels so that child can grow normally without any growth delays,this is what i personally prefer,but when you transfuse a child regularly,iron starts to over load in the body,and you have to use chelators to get it out,and with chelators you have to monitor patient with blood tests,and keeping iron low is very crucial,because if not chelated,it can cause damage to the organs.
I have tried to make it as simple as i can,but thats not it,there are so many factors related with intermedia,i'll advise you to read through our Thalassemia intermedia section as much as you can , or atleast this much ,
From http://www.thalassemiapatientsandfriends.com/index.php?topic=1575.0
Thalassemia Intermedia
Technically, thalassemia intermedia refers to a patient, who is able to maintain a hemoglobin level in the 6-8.5 range without taking blood transfusions. Most cases of beta thalassemia intermedia are the result of two defective beta hemoglobin genes, although some rarer cases are the result of the seldom seen dominant thalassemia gene and some are cases of thal beta zero. Usually, intermedia is the result of at least one of the gene mutations being of a "milder" variety, that enables the patient to still produce some useful hemoglobin. Many intermedia patients do not require transfusions when younger, but eventually need to begin regular transfusions. Some doctors will reclassify a patient as major once regular transfusions begin. In recent years it has been noted that starting a program to induce fetal hemoglobin in intermedias when young can help them avoid transfusions later in life. Intermedia can also occur when two beta thal genes are present along with alpha thalassemia trait. The reduced production of alpha hemoglobin is a better match with the reduced production of beta hemoglobin and the result is intermedia rather than major, as the alpha thal acts to moderate the beta thal.
The decision to transfuse in intermedia is based on a combination of hemoglobin levels and bone development. If the low blood supply severely impacts bone growth in children, either transfusion or fetal hemoglobin inducing drugs are recommended. Chief among these drugs are hydroxyurea and butyrate. Current research into drugs related to butyrate hold great promise for the future for thal intermedias and majors. The goal of these drugs is to turn the gene back on that regulates the production of the gamma hemoglobin that combines with alpha hemoglobin to produce the fetal hemoglobin that the body uses until about six months of age. In addition to the drugs, many patients have some degree of success in raising fetal hemoglobin levels by using natural products like wheatgrass and resveratrol, which is derived from red grapes. Both substances are known to increase fetal hemoglobin levels in many patients, enabling them to maintain Hb levels that do not require transfusion. If fetal hemoglobin production cannot be induced by these drugs or supplements, and the Hb level of the patient drops below 7, blood transfusions will most likely be needed.
Intermedia patients require regular medical treatment and observation. Some common problems are enlarged spleen and excess absorption of iron in the gut, slow or inadequate bone development, decreased stature, endocrine system problems and a low immune system. Some of these problems can be alleviated with transfusions but transfusions bring a whole new set of problems and are avoided whenever possible. Often, removal of the spleen is done to eliminate the filtering out of red blood cells by the spleen. The spleen sees the irregular red blood cells found in thalassemia as being defective, and removes them. With splenectomy, this can be reduced but it doesn't work for all patients and some patients eventually return to the same low blood state that they had before the splenectomy. The decision to transfuse in thalassemia intermedia should never be taken lightly and all other avenues should be explored first. However, there may come a time when the quality of life issues outweigh the problems related to transfusion, and the patient will find better health with transfusions.
Intermedias often find themselves with a condition of iron overload even though they don't transfuse. Simply put, because the body is somewhat starved for blood, the bone marrow becomes overactive trying to produce red blood cells (many of which have no useful function) and a mechanism in the gut causes too much iron to be absorbed from food in order to supply iron to these new red cells. Because iron overload is not a normal condition, the body does not use these iron stores to feed the new red cells, but rather uses the normal system of absorbing iron from food. After some years, this can create a dangerous iron overload condition that can damage the organs and destroy the health. Once this occurs, iron chelating drugs are used to reduce this iron load. To limit the amount of iron absorbed, intermedias should drink tea with meals and avoid citrus and vitamin C with meals. Tea inhibits the absorption of iron from food and vitamin C increases it. Foods containing C can still be used by intermedias in moderation but should be taken as snacks between meals. The natural supplement IP6 (phytic acid) which is a member of the B complex family, is a natural iron chelator and can help reduce iron concentrations in the cells and also act as a powerful antioxidant which can help prevent some of the damage that iron does at the cellular level. For maximum effectiveness, take IP6 on an empty stomach and take it with Inositol, a related B vitamin. Raising the Hb level will also help reduce the amount of iron absorbed in the gut, so fetal hemoglobin inducers should also be considered.
It should be remembered that thalassemia intermedia is a classification and genetically, there is little difference between intermedias and majors. Intermedia is basically a more moderate form of major, and much of what applies to majors also applies to intermedias. Intermedia may not be apparent as early as major is but will normally be discovered in the first few years of life, possibly after a "failure to thrive" medical diagnosis.
I hope it will help you understand pros and cons of transfusing and not transfusing,personally i would go with the approach of regular transfusions and strict chelation so that child can grow properly.With out any stress on organs.
Zaini.
P.S i am sorry if it seems like i am rambling but as Andy said i have flu right now and its a bit hard for me to concentrate with watery eyes.
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Hi
What about your niece's growth?? Is she having a normal weight or and height for her age?? Has she been examined by an endocrinologist? Growth and bone deformity are things to consider in deciding whether to transfuse or not
Manal
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she is having almost normal weight and height, but she has not been examined by endocrinologist why to be checked by endocrinologist?
she is not having any bone deformation
but there is a problem that she is a bit bit weak physically may be thats because she gets severe fever after every 4, 5 months right from her birth,
thanks for the help to you all
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her weight is 15 kg at an age of 4 years and 1 month i dont know its normal or not
what u say????????????
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This is one of the things the endocrinologist tells you. For example the doctor will examine her height according to her parents height. Will examine the function of her glands and make sure that everything is functioning normal through some blood tests. The most important part is to check too that the rate of growth is constant too.
manal