Hi Joyce,
I don't know how much your doctor explained to you, but there is a complete absence of HbA, the normal type of hemoglobin. There is also a low amount of the secondary hemoglobin, HbA2, which indicates that the delta gene is also affected. The delta and beta genes are very close to each other on the same chromosome and the delta gene normally produces only a small amount of hemoglobin. However, in spite of having no normal hemoglobin, your son has an Hb of over 10 at this point in time. This is completely due to a very high level of fetal hemoglobin (HbF). This hemoglobin is what is used by the fetus and usually drops to very low percentages by age 6 months. The fact that there is enough of this HbF being produced at 15 months to have a hemoglobin in the thal minor range shows that fetal hemoglobin is still being produced and at a high rate.
Comment:
Thalassaemic full blood picture features with high Hb F.
Homozygous Delta Beta Thalassaemia. However, normaly patient will present with mild thalassaemia intermedia to severe thalassaemia major.
This boy also has thalassemia. Because there is a complete absence of HbA, there is no doubt that this is beta zero thalassemia, which under normal circumstances would result in transfusions at a very young age. But, there is an observation in this condition where the delta is also affected that there is normally a higher rate of HbF found than in beta zero thalassemia. This is possibly a result of increased gamma globin, which replaces beta globin in HbF, which is known to occur in the delta/beta zero thalassemia patients, and/or it may be the result of a condition that seems to have some connection to beta/delta thal. This condition is known as hereditary persistence of fetal hemoglobin (HPFH). In non-thals, this is also found with no real consequence, but when it is found in thals, it is a blessing, because it results in a higher production of HbF throughout life. Your son has a very high HbF level for a child his age. As the doctor told you, it may take some months before the full picture is clear, but I would have to say that there is an unusual high production of HbF at this age and this is most likely due to one of the two factors I mentioned, or both, and that this will result in a less severe, intermedia condition. He may be able to avoid transfusions, but if eventually does need them, it may be less frequent than in a major.
I know this isn't what you hoped to hear, but I do want you to understand that even though he does have thal, it is very likely not severe. There is also a good chance that his hemoglobin level can be increased through fetal hemoglobin inducing supplements like wheatgrass and resveratrol and possibly by the drug, hydroxyurea, if the Hb drops below 8. A good diet and vitamins without iron can also be beneficial.
Right now, you wait and see how things progress and if he can maintain a hemoglobin level high enough to avoid transfusion. I would highly suggest he be put on a baby vitamin without iron and also takes folic acid. These should also be taken by the infant. Do you have the test results for the youngest child also? I would like to see the results of his electrophoresis and any comments, if these are available, so I can compare them to the older boy's results. I especially would like to know if there is any mention of delta in the younger boy's results. His HbF will be high because of his young age, so that won't tell us much at this point, but it would be helpful to see any comments. Is the younger boy transfusing yet?