Tranfusion already!

Dear friends

First, thank you Andy for all your of good advices, especially on Folic acid. We take yours and others' advices very seriously. We start with folic acid now, 1mg per day. We also try to find Wheategrass, but it is not off the shelf in our country.

We have with great interest read about experiences with blood transfusions. Most of them are positive. Yet I am very very nervous because our daughter is getting the first already tomorrow. Everything happens so fast ...

Our daughter is 2.5 years old. She has remained relatively stable, with an HB of about 8.5. Sometimes higher, sometimes lower. Until April of this year is the lowest she has been 8.2 after an infection. At that time she went to 8.6 on the next test.
In April, she suddenly had a HB of 7.9. There were no infections in the weeks before. But at the time she got 4 new teeth, so we thought that was the cause. Now she has recently had 2 infections with high fever. One lasted a whole week. Her HB fell to 6.9. She increased the level into 7.4 after one week. With new infection last week she is now at 6.9 again.

Her doctors have decided that she will get a blood transfusion tomorrow, and we agree with their decision. But we are scared and sad. The reasons we agree with the decision are:

- we are not sure if she can increase the HB. And if she can do it, we believe it will take many
weeks or months before she gets built up to 8 again.
- She is now quite pale, have poor appetite and shaky (she is staggering when walking).
- It is summer and the other children are playing. Our daughter is not playing the same way as the other children, she is slow and wants to siton the lap most of the time. We do so want her to run and jump with the other
-   She is small, 10 percentil of southern Chinese children (but she is following her own curve, and are growing)
-    We spend a long time on meals
-    If this can increase her HB we hope she gets a little higher immune system so she will not be so easily sick. We do not know if it is correct.

Are these good enough reasons?

This has happened so quickly and we have so many questions that make us sleepless:
-    is it naive to hope that there may be enough with one transfusion? And that she will remain at her old levels around 8 to 8.5 again?
-   Earlier we have read that intermedias can need transfusions after infections. Is this right, that it can be needed only once?
-   Will she become dependent on transfusions?
-   Are we now in a new phase so that from now on she will need regular transfusions?
-   Should we have waited?

We have got several different and conflicting answers from the doctors since we got her diagnosis 1.5 years ago. They are really doing a great job monitoring her, but I think it is difficult for them to give consistent information. Only a few months ago, they said that we should wait as long as we can with transfusions. Previously, we learned that they think she has a mild version of Beta Thalassemia Intermedia, and that she probably will not need transfusions. She is now just 2.5 years and will receive transfusion already.
We will as all of you do the very best we can for our wonderful child, but this is really difficult.

Before I forget, I want to mention that an important key to a good immune system is vitamin D. Most people tested do test low and with living in Norway, it may be very important to make sure this is regularly supplemented. I am currently taking 5000 IU daily and it has turned my life around. I seldom get any illness, other than my constant allergies, and my annual winter depression vanished this past winter. Sunshine is the best source, but it is not available often enough in the north to keep the D levels up, except for people of Northern descent, like my Irish wife. Vitamin D can also provide a positive change in energy levels, so please investigate this. Her level can be checked with a simple blood test.

I have to agree that transfusion is called for at the moment. It will make your listless child act like a normal kid for her age. Hopefully, this will not have to become a regular thing, but the course of intermedia is very difficult to predict because other factors besides the thal mutations come into play. Co-existing alpha trait and persistence of fetal hemoglobin can moderate beta thal, and there are other poorly understood factors that can also play a role, so prediction is not necessarily possible.

Because your child is from China and living in Norway, I would highly recommend genotype matching of the blood, if at all possible. The reason for this is because she is most likely to get blood from someone of a different race, and phenotype matching alone does not take this into account. There are antibodies that are more specific to each ethnic group and matching to these antibodies is preferred whenever possible. If this is done, the chances of an antibody reaction are greatly reduced. Discuss this possibility with your doctor.

Only time will tell what the transfusion needs of your child will be. You can help by continuing a good diet with proper supplements and hopefully, within a few years, we will see more fetal hemoglobin inducing drugs. Currently, hydroxyurea is used and this may help your child, but we do expect to see new drugs introduced that raise the hemoglobin level more than hydroxyruea can.

Thank you Andy!!

We are aware of the D vitamin and have quite recently checked her level to be normal. We give her daily supplements of vitamin D as we live in this country with quite few days of sun compared to where she was born. We are also going to Spain for our holiday :-)

They have very strict programs for giving and receiving blood in our country so I am quite sure they have done the genotest (I have heard it mentioned). She was called back for an extra test yesterday, and I think it was for the genotest. We will definitely ask tomorrow morning.

We will consider hydroxyurea together with the doctors. It has been mentioned. But she is only 10,5kg so maybe it is early with this drug? Everything has happened so fast, therefore we have to learn more quite soon. In my head it is safer with blood transfusion than drugs, but I might be wrong??

Hopefully we will have some weeks and months now that we can read and learn more.

I hope everything will go well tomorrow and I am looking forward to see her with more energy. I will keep you informed.
Thanks again!

I hope the blood transfusion is going well. I hope you were able to turn it into a fun day. Poking went well?
I have been to Norway two summers ago. We went in July/AUgust and it was so very freaking cold! Some mornings it was only 3C :P
If you read this in time, make a picture! I got a friend who had a bmt and she was only able to find 1 picture from tanking up.

Thanks Dori! Well, it can be really cold in this country but right now it is 27 c so the summers are usually nice 

It went very well yesterday. We had a nice day at the hospital, and she accepted everything without problems. It was pretty much waiting, and the transfusion took 3 hours since it was the first time. The blood was genotestet. Poking was ok, and pichures was taken    I am so relieved that everything went well. I was very nervous before and struggled with a severe headache.

Today has been incredible. We have a “new child” in the house. She runs and laughs and sings. She is far more physical than usual when she plays. She has red cheeks and red lips. We are glad that we decided to transfer now, and hope that it will last a while.

We have not checked her level of HB, we'll check tomorrow or Thursday.

We are relieved that everything went well and that we finally could do something for her. It has been hard to see her lack of interest in playing and so on. It was at this time when she came down under 7 we really noticed how slow she was. But seeing her today – we know that it is a big difference from when she was 8+ as well. Making us think…. when is it right to start treatement?

Thanks for listening and for your good advices!

Dear Friends,
I will start to say that I am so pleased that I found this site. My husband and I are in here almost daily. We read your stories and try to learn as much as possible about Thalassemia in order to help our daughter to get the right treatments. Today we are meeting our doctor again, and we have several questions we will like to discuss, based on information we have found here.  As we learn and experience more, maybe we will be able to give advice in the future as well.

An update on our daughter:
She was transfused the 28th of June. After one week her HB was measured 11,2. Two weeks later it was measured to 11,1. After 5 weeks it fell to 9,5 (she had 3 days of fever in between)  and then it was stable for another two weeks.  In the middle of September she dropped to 8,5. Now her hb is 7,6. She has not been sick during the period she has dropped from 9,5 to 7,6.

The doctors have told us they will transfuse if she dropp’s down to 7, we agree and it will probably be done during this week. As parents it we feel it is really important to take the right decisions, but how can we know what is right? Here quality of life seems to be much better when her hb is above 9. At the time she dropped down to 8,5 and then 8,0 she was really sad for some days. Now she is not as sad, but she is slow, eats less, sleaps less and isn’t that interested in playing with the other kids. But she is such a brave and good girl.
We do not know for sure what the future will bring, and how she will drop this time. We discussed with our doctors when she was around 8,5 if this is a ok level to live with. We do not think so, because we have now seen how she is when she is above 10. But on the other hand, what are the consequences of starting regular transfusions – compared to not do it. (I know we do not have a choice this time, so this is just IF she will be stable around 8,5).
We are reading about the different possible cures that might be possible in the future. Do you think it will affect the results in negative way if she has been regularly transfused? We think that a regular transfusion (if she needs it) will give her a higher life quality, but we are so afraid to do something wrong.

Does anyone have thoughts or knowledge that you will like to share? We will be thankful.

Hi,

Actually it depends on case to case basis,some kids do well with low hb,their growth isn't effected,but some can't cope with low hb and they need to get transfused regularly,

If you start transfusing her regularly,you'll be able to keep her hb levels up,which in turn will boost her energy levels and will help her grow properly,and she'll be able to live a fairly normal life with hopefully no growth issues or bone deformations,but then you'll have to deal with iron chelation and possible reaction during blood transfusions.And diseases can be transfered through blood,although screening is much better now a days but it still happens sometimes.

If you don't transfuse her regularly,the risks that come with blood transfusions will be eliminated,but her growth can be effected in the long term,and iron does overload,although slowly in intermedias,because when body is on chronic low hb it tries to absorb as much iron as possible through the gut,and serum ferritin is not a reliable way of measuring iron overload.

Just my two cents,you as a parent along with your doctors will have to decide whats best for your daughter .

Good luck.

Zaini.

Thank you Zaini for sharing your experience and for your kind words! She got her blood transfusion today, and everything went well. Right now she is tired but happy, with a new glow in her face. Hopefully it will last some weeks – or months 

How is BabyNorth doing nowadays?

Dear friends

Happy New Year!

Thank’s Dory for asking how we are doing!

Our Christmas has been really good. Our daughter is turning 3 years next week. She is a happy girl with lots of energy, good appetite and sleeping habits. It is really fun to have a 3 year old in the house  :cheerand she is such a sweet, smart and loving girl.

9. December she had her third transfusion; this was 9 weeks after her second. Her hb was at that time 9, and had been stable for around 1 ½ weeks. She is not feeling well when her hb is lower than 9,5 and knowing that it would fall further we decided together with her doctors to give her transfusion. We wished for her to have a good Christmas and birthday celebration – as she has been looking forward to this for a long time. We have not checked her hb since the 22. December, and at that time it was 10,9 (two weeks after transfusion).

Her ferritin was checked in December (before transfusion) and was 370.

As for now we think it is really good for her to receive transfusions when she falls down to hb 9. There is such a big difference in her quality of life having a higher hb than 9. When she is lower she is tired, sad and has poor appetite. As she gets older it will be easier to talk to her and find what is best for her, until then we and her doctors takes the decisions based on how we consider to be the best for her.

We do not know how often she will need transfusions; I will keep you posted on how long this 3rd will last…

Until now she has not needed iron chelation, and her/our situation will maybe look very different when we have to start this. But right now we think that receiving blood is best for her, and we hope that removing iron will be manageable (??!!). Our doctors are telling us that they always start with the iron chelation that requires pump, because this is the safest drug as it has been on the market for a long time. Is this right??

As for being parents it is easier for us now. We are not as scared as we were the first year. Thanks to you all!!!

I will just add one comment to my post above: Our daughter has been below 7 two times....