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=1575.0Thalassemia 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.