No second transfusion ? Please advise Andy

Hi Andy,
My son was due for his second transfusion today. As I posted earlier he was transfused the first time on 18 Nov. because he had tested positive for RSV  and they thought he needed more than 7.7 Hb to recover well. However the doctor also explained that he would recomend regular transfusions as my son wasnt growing well and there was no need to take risk involving bone expansion , slow growth, etc. as Iron over load is much easier to control and not a cause for so much fear any more. So the decision was to transfuse him regularly keeping his pretransfusion Hb around 10.
However, even though his Hb today was 9.1, another doctor said they transfused last time because of his RSV infection and they will not tranfuse today and will continue in this manner until and unless his Hb drops below 7. I pointed out that he had grown so well this month ( weight increased 0.6 kg and height 2 cm) and that last time the doctor had recomended regular transfusions. He said the previous doctor didnt have the new Hb electrophoresis results which point out that he is probably intermedia and doesnt need regular transfusions. He pointed out that nucleated RBCs are 4.2, RETIC% 2.2  which means bones arent under stress and that there is no organomegaly or bone expansion visible /palpable. He recomended only observation ( next appointment in two weeks) and intervention with blood transfusion if Hb is less than 7 or there is bone expansion  or long term growth retardation( a few months) as previous slow growth could be b/c my son was sick. The electrophoresis report says HB A2 1.9 , Hb F >40 and Hb Ao 15.7.
What is Hb Ao?
Th report also says there is a major predominant peak witha retention time consistent with Hb F and several smaller peaks with very short retention time unmeasurable by the instrument which may represent unstable Hb. What does this mean?
I am so confused  about what is right for my child now. What do you think?
Zahra

Hi Zahra,

 HbA0 is the same as HbA, normal adult hemoglobin. The unstable hemoglobin seen may not have any significance.

http://www.dhss.mo.gov/Lab/Newborn/Hemoglobinopathies.html

Unidentified Hemoglobin Variants

In the course of screening all newborns for the presence of the common abnormal hemoglobins, various other hemoglobin variants are uncovered, most of which are by and large unidentified.  There are over 800 hemoglobin variants described in the literature at present.  The vast majority of these have little known clinical ramifications and end up being merely incidental findings.  Some are fetal hemoglobin variants that fade away with the fetal hemoglobin by six months of age and become undetectable.   

I agree that the child is most likely intermedia, but if growth is being severely stunted by low Hb, transfusions should be started. The typical transfusion frequency at this stage is every 4-6 weeks depending on how well the Hb level is maintained. In the past, intermedias were not transfused as readily as today and as you've seen, doctors differ on how to proceed. There is no real danger in waiting for a week or two and seeing how he does, but if his Hb continues to drop and he doesn't grow and has noticeably lower energy levels, I would recommend transfusing.

Hi Andy,
Thankyou for your reply. I guess we will wait and see if the doctors idea that he wasnt growing because of the RSV is right or wrong. All I can say at the moment is that his appetite is getting more and more diminished as his Hb falls. His activity otherwise seems unaffected. Just a lack of interest in food. Will let you know what happens next visit on the 30th.Thanks once again for all your help.
Zahra

Hi,
My son had his appointment today. His Hb was 8.6. Its been 6 weeks since his transfusion. They are going for the no transfusion unless Hb less than 7 option. How long does transfused blood stay in the body? The good news is he gained 3 cm and half a kg. .
Zahra

Congratulations Zahra for this gain in weight and height

As for how long the blood stays in the body, .. when an intermedia is transfused for the first time, mointering his blood level will telll you how long it will take him to drop below 7. Cause in intermedias every case is really special and independant. You can find some indermeias who just need to tansfuse 3 or 4 times annually and other who need more frequent transfusions and they are still not dependant on transfusions. This variations is the outcome of different mutations and other factors that control ther body, like proper supplementations, nutritions, .....

Normally the blood you transfuse will disappear from the system in weeks but the abiity of the bone marrow to produce this little good HB of intermedias is the one we mointer to see how long it will be maintained provided that there is no infections and the body is in the normal conditions

I hope i can express myself clearly, please don't hesitate to ask

manal

Hi everybody,
Just wanted to add an update. My son was ill again a week after the last post. His Hb dropped from 8.6 to 7.7 in this time. Part of this would be the last remnants of the blood transfusion he had 7 weeks before this. He was now in the 7-8 range he has been on his own. They didnt transfuse him this time but said to give him some time to recover . At 8 weeks post transfusion ( 8 months age) he was still at 7.7 but had gained some weight . He is now 9 kg. So now they have given an appointment after 3 weeks to monitor again.
The doctor told me that Thalassemia intermedias have a normal life span though they do need transfusions when ill . Also that they do need chelation but much later at around twnty years of age. Besides bone expansion and spleenomegaly he mentioned that gall stones must be looked out for. What other problems do intermedias have? They arent sure yet but say that all indications are that he will be intermedia.
I wish I knew what intermedias feel /are going through. Its going to be a long time till my son can speak up and tell me anything. Any other information about intermedia and its prognosis would be welcome.
Zahra

Zehra,
A  Message for your son ,.. may ALLAH Bless him with the best of the health ..

Best REgards
Take Care
Umair

Hi Zahra

Sorry to hear about your son illness, i hope that he will recover soon

Also that they do need chelation but much later at around twnty years of age. Besides bone expansion and spleenomegaly he mentioned that gall stones must be looked out for

*Determining when to chelate does not go with age but rather with the level of ferritn that should be monitered after approximatly 10 transfusions. Usually chelation starts when ferritin levels cross the 1000

* Starting the age of 10 years or when it is possible for your child to sit still for a while, it is advisable to do ferriscan( this is an MRI that measures the liver iron concentration ) . This is really important to do as well as T2* ( measures iron overload in the heart) because in intermedias ferritn is not a true reflection of iron overload in the body ( which is not the same case as thal majors ). These tests are too early to do for your son but i just want you to know.

*It is advised that splenctomy would be avoided as much as possible

*Usually intermedias are more prone to thrombphilia and Pulmonary hyper tension(PHT). One of the supplements that helps maintain the heart vessels is the L-carnitine that i hope your son will be taking. It has proved too that it can increase the intervals between transfusion in addition to more benefits. you can read more about it in these links

http://www.thalassemiapatientsandfriends.com/index.php?topic=2495.0


http://www.thalassemiapatientsandfriends.com/index.php?topic=2240.45


* One more thing you should be taking in consideration is Osteoproseis, generally speaking and whether thal intermedia or major, there are other factors in the body that may lead to Osteoproseis. So having a good supplement that has calcium, vitamin D, magnesium and zinc, is very important for having healthy bones. I am not saying that this must happen or even at an early age but i just want you to know that monitering the bone growth as well as giving good supplementation is extremly important. As i do believe that we as parents should know all possible complication to try to give as much good supplementation in order to prevent these complication as much as possible. This is my aim from the post to let you know more than to let you get down , hope you can understand my point

manal

 

Hi Zahra,
I don't think that we spoke before, I'm Kathleen Olivia's mom. Livi is almost 5 yrs old and May of 2008 she started to receive regular transfusions. It was only going to be for 6 months to see if she grows and develops better on tx than off. Well her Dr and I decided to keep her on a regular tx schedule. She used to run a hb of 8-8.6 but then she started to drop for no reason. I am at peace with our decision to keep her tx she is growing like a weed and her mood is so much better and she eats better too. She grew an inch in 4 weeks! She has started Exjade and so far so good ( we are into our 2nd month of Exjade). I used to be so afraid of tx, Im not anymore. It's what Olivia needs. Take care.....Kathleen

PS..Hi Manal how are you and your family? Hope to talk to you soon!

Hi Kathleen

I am happy to hear from you, it has been a while since we talked   hope you and all the family are well.
I am really so happy to know of Oliva's progress and how confident you are now. Please keep us updated and good luck my friend in everything

manal

Hi Kathleen,

That's a really good news  I am so happy for Olivia,My daughter has also started exjade a few days back,i am glad that Olivia is doing well with exjade ,give her a big kiss for me  .

Zaini.

P.S We haven't heard from Christine for a while,how is Lauryn doing?

Hi All,
I know I would like to be on more but life is so busy. I have my brother-in-law and his girlfriend and their 4 kids living with us for 3 months now. They were in a bad situation so we asked them to come live with us and I've started to watch my son's teacher's 2 children a 3 yr old and a 7 month old. So I'm busy. Lauryn is doing very well, last month her hb got very low 7.6 because when she went for her tx her hb was a 12.8 so her md said to come back in three weeks which ended up being too long (it was 6 weeks total between tx) and she also was very sick with 104 fever. Now she is doing better. She is so cute and she knows it. I really am so happy for Olivia she is so different. Right now her ferritin in 1019. Last month it was 965, thats when we started Exjade 350 mg. I know it can go up as the iron is being removed from the organs so at this point we are not worried. Im glad we were able to get 4 yrs without tx her, for now Im so glad she is because we see the benefit of the tx. I just pray that she remains healthy and has a long healthy life and that all Thals see a cure soon. xoxo Kathleen

Dear Nice friend ,
Thanx for your duas.
 
Zahra

Dear Manal,
Thank you so much for all this information. Do you know at what age these supplements can be started . My doctor hasnt advised anything but folic acid. I asked about Vit E for infants and he didnt seem to have any idea. What brands do you use? Are they suitable for infants? my son is 8 months old now and has started to eat some solids now. I dont know what scans are available /used here yet but I plan to find out. Also how do you monitor bone growth. Osteoporosis is scary.
Zahra

Dear Kathleen,
Thank you for your post. I'm glad Olivia is doing well on transfusions/ exjade. I hope she continues to grow well and thrive .
My son is eight months old .The docs think he may be intermedia because he has some adult Hb but its not defnite yet. He was transfused once when sick and turned a lovely pink colour. He seemed stronger and more active and was so hungry you couldnt feed him enough. But now the doctor thinks that for now he doesnt need regular transfusions . Its so hard to be sure which is better for him. I'm glad you are staisfied with the course taken. I,m amazed at your being able to take on so much. Wow.
Zahra