Little Ari

Thanks Andy,  so far Ari looks fine. He is a little pale but he's eating, smiling and awake all day.  He wont even nap!!! always wants to look aroundl.   I want to take him back this week regardless. I was thinking on Tues....do u think I should even attempt to if he is still okay?

What is beta zero? 

Carisma,

I feel that unless there is a noticeable change in Ari's looks or behavior, that they will not transfuse until a second blood test at least two weeks after the first, confirms the low Hb, because he is at the age when Hb level may not be totally accurate because of the nadir period. What remains to be seen is how much HbF he will still be making. He looks good in his FB pics.

Dore,

Beta zero is when both beta globin genes are deleted and no normal adult hemoglobin (HbA) will be made at all. The only hemoglobin will be fetal hemoglobin, which normally disappears for the most part by six months of age.

Just an update...Ari seems to be doing okay these past few days.  He is definitely very pale but he is overall very alert so it is so confusing!  Yesterday he was so alert and awake all day (God bless him) playing and smiling.  THe only thing is that today he was a little more tired and did not eat as well.  I get confused because i would think he WOULD be tired after being so active the day before...right? 

I don't know...i am going to watch him tomorrow and see how he does. 

One more question just to clarify something.  I'm sorry if this is repetative info but i am so confused.

I've been reading back on posts on genotyping.  I requested to have this testing done before the transfusion started and the doctor told me this was called the RBC phenotype test.  THe other links i've been reading here keep referring to genotyping to get the most accurate match and prevent antibodies in the future.  I believe some areas state that it is genotyping that cannot be done after the first transfusion. I know Sharni mentioned that the RBC phenotype is the correct test but what is genotyping then?  Do they automatically do this before every transfusion?  I want to make sure I am asking for the right things before the first transfusion if there is something else I need to ask for. 

I don't think i mentioned that my doctor here did not have much information on RBC phenotyping!!!??  They were actually looking through a book to see what they needed to get from Ari.  THe blood bank was also not aware of the testing!!!  Luckily my hemotologist in Orlando contacted the one from Atlanta and they contacted our blood bank and sent them anonymous results of the tests so that the blood bank was aware of what to look for.

This has scared me and now i know i just have to be on top of my game in order to get the best treatment from Ari. 

Also, are there any links to the transfusion process for babies on the thalpal site?  I have been searching but haven't found it. 

Thank so much - love, C

Dear Carisma,

I am glad to hear that little Ari is doing well.  I have been meaning to communicate with you all week, but it has been the busiest week ever!  Lil A just finished a late hockey game tonight and I finally got the little monkey to go to bed. 

The best type of testing to have done is genotype testing - so be sure that it is done soon.  The reason that phenotype testing should be done right away is that it becomes impossible to do it once the patient is transfused.  It is useful to have the results of both tests so that the best crossmatch is always found.   Of the tests - genotype testing is the most sophisticated and best test to prevent formation of antibodies. 

Even though the hg is dropping - babies seem to adjust to the drop in hg.  Lil A was very active even at a hg of 4.4 when he was a baby.  He played and played at the doctors office the day that he was diagnosed - even with such a low hg.  I think that the hg drops slow enough for their bodies to accommodate. 

You may notice a decrease in appetite and a tendency to spit up more milk when the hg is very low.  For these reasons, I hope that the hospital will decide to transfuse him soon.  I know that they are hoping for his hg to recover - as it does in non thal babies - but that is not the case with beta zero babies.  I just hope that Ari's hg does not drop too low.  Having really low hg will cause his spleen to grow and interfere with his growth during the next month.  I don't like the idea of a baby having so little oxygen and blood.  Please let me know what the hospital says.

You are right in that you have to stay on top of everything, we had to learn so much on our own to have lil A treated.  There wasn't enough information here so we accumulated our information at comprehensive thal centers.  Dr. Vichinsky and the Oakland hospital have done wonders for us.  I hope that lil Ari will also be followed by one of the larger centers who can guide your local doctors with Ari's care.  If you are followed by the large centers everything will go very smoothly for you - and you will always have the best information. 

I will give you a call as soon as things slow down for us here.  Perhaps tomorrow morning. 

Take good care:)  Hugs to little Ari ((hug))

Sharmin

Hi Carisma,

When you refer to Atlanta are you referring to Children’s Healthcare of Atlanta? That is the Center of Excellence in Atlanta. If you are, you should have them guide your local hospital in this process. They can tell them exactly what should be tested in the phenotype testing. I hate to confuse matters even further, but I would like to see all children genotype tested before the first transfusion and the transfused blood be genotype matched, especially when the blood being given is from a different ethnic group than the recipient. If both phenotype and genotype are done from the beginning, it does minimize the chances of antibody reactions. However, not all hospitals are willing to follow the genotype matching (including some of the Centers of Excellence). This is frustrating but because you are Indian and your child is likely to receive blood from a non-Indian, the genotype does become more important and if at all possible. It has been shown that there are antibodies more unique to specific ethnic groups and these are not always caught by phenotyping alone. Regardless if this can be done, thorough phenotyping must be done before the first transfusion, so only the baby's own antibodies are present. With each transfusion, new foreign antibodies can be introduced that cannot be told apart from the native antibodies, so knowing exactly what the child's antibodies are is essential.

There is some information about transfusions in our Documents section and also within the Standard of Care Guidelines for Thalassemia which are attached as a pdf file to the post at http://www.thalassemiapatientsandfriends.com/index.php?topic=1762.msg14639#msg14639
All parents and patients should familiarize themselves with these guidelines, and make sure your local center is also aware of them. It makes a huge difference in quality of life when these guidelines are followed.

Goodness....I called them to ask about the genotype  but they r closed. What if they don't do the genotype before the first transfusion...I am going to go on Friday 

Carisma,

I hope that they can do the genotype testing soon - but it takes several weeks to get the results.  Ideally, they would have drawn the blood for genotype and phenotype matching at birth so that they had a enough time to get results.  

Although it is best to have the genotype information before any blood is given, even extensive phenotype matching will provide a good match until the genotype information is available.  It took Lil A two years to produce antibodies against foreign blood, and it is usually multiple transfusions that cause the problem.  Try to get the matching done before the transfusion, but if you cannot then just try to get it as soon as possible.  Sometimes it can take up to a month to get the results of genotype matching - see if they will do it for you quicker.  

This must seem very overwhelming to you - I hope that things get ironed out soon.

Here are a few things to expect during the first transfusion.  Baby will not like getting the IV at all - babies veins are tiny and it may take several attempts to find a vein.  Babies become very distressed, scream and sometimes hold their breath when they are upset.  Just try to remain calm, the baby will feed off of you and learn to be calm.  Once the IV is in and he is taped up you can hold him and get him busy doing something else - so he can quickly forget the poke.  Try to smile and be playful so that he is not scared that something bad is happening - even as he grows this will be important in order to keep a positive attitude about thalassemia for him and for you.  

IV's sometimes don't work in the hands, at times they need to poke feet (which babies hate!) - and I have seen some babies get it in the head (Lil A has never had a poke on his head but another little girl at our hospital does get it in her head).  

If he is well hydrated and is playing a lot before the poke his blood vessels will open up and the poke will go easier than if is dry or sleeping.

The nurses will start the IV, and probably give him saline before starting the blood very slowly.  Ari will have a bracelet (lil A likes to wear it on his ankle rather than his wrist because it causes less attention if he has to wear it to school).  Blood is tested the day before the transfusion, Ari will be given the bracelet with his cross match number on it - when you go in for a transfusion the nurses will check the unit against the number on his braclet.  Two nurses should be there to check the number and sign - at this time you can also compare that the blood type on the bag matches his blood type and number on his bracelet.  

His vitals - blood pressure, temperature, heart rate etc will be checked before the transfusion, and then every 15 minutes during the first half hour - then every hour until the transfusion is complete.  If you notice any hives, temperature change or sudden change in behavior you should let the nurses know.  

It is a good idea to use Ari's own clothing and blankets because hospital blankets and PJs sometimes give babies rashes - so it is hard to know if the rash is due to the linens or the blood.  Lil A's skin would get irritated due to the linens so I started using his own and the irritation stopped.  

Ari won't like the blood pressure cough on his thigh tightening up, the poking and prodding and perhaps the cold fluid going into his vein - but by holding him and playing with him a lot you can keep him distracted.  Soon he will forget about the transfusion and get busy playing.  Lil A actually liked transfusion day a lot as a child - by the time he was 18 months he learned to drag his IV machine around and play with all the toys.  He enjoyed having a special day with mom and dad at the hospital watching movies and having treats that he couldn't have other days. The nurses spoiled him too because he was a repeat patient that they got to know well.   Be sure to bring your favorite coffee and a good book for yourself so that you can enjoy your time there as well - it will help you to form a more positive attitude about the experience which will then trickle down to Ari.  He may nap for some of the time that he is there so you'll need something to do during that time.  

It is nice to have visitors - grandparents - uncles & aunts - however - no one should look sad or act as they feel sorry for him.  The should act like there's a party going on in his room and play with him.  

Once the transfusion is done, the IV will be removed - vitals will be checked.  They may keep him in for another hour to watch for reactions or fever.  Some centers do a post transfusion hg (our center does not do it because they think that the extra poke is not worth upsetting the child) but other centers do.  For the first few transfusions it may be a good idea to do the post transfusion blood test so that they can determine a good pattern for Ari's transfusions.  

I hope that this gives you an idea about what transfusions are like and how to deal with them,  I will keep posting more as things occur to me:).

Sharmin

Wow, Sharmin I am amazed with your instructions and all the little things you seem to care for when Lil A gets transfused. You are a role model for all parents, if I may say so! It's very important to lay down the lines like that and I am sure Carisma will benefit from everything you have written.

Lena.

Thank you for your kind words Lena.  We received some very good guidance when our son first started transfusing and it proved to be very helpful.  Since the antibody and the treatments he has had related to the antibody - we have not been able to manage the emotional part as well.  I hope that we can get things back on track soon.  I appreciate your encouragement and all of the advise that you have given me - it will make a big difference. 

Charishma,  I hope that things are going well with little Ari.  I have been thinking of you and hoping that all is well.  Please let us know if we can help with anything at all. 

Sharmin

Hope everyone had a great thanksgiving  Little Ari had a lot of family madness these past two days...I don't think anyone can get enough of him.  Poor boy is exhausted but he is doing good.  I spoke with his doctor on Wednesday and he told me to bring him in to the emergency room if I thought i needed to.  He said he would transfuse if his level had dropped but it was best to wait until monday.  Not much help for me...

While i had him on the phone I asked about genotyping...he said that there is no such thing as an RBC Genotype test and that we had done the phenotyping and that was all we needed.  

Now, I dont know what more to do.  I sent him all the info about both tests that I gathered from this site 2 weeks ago...

Andy, the center is the one referred to me by Eileen while I was pregnant.  I had actually visited dr. boudreaux there in June.  When I spoke with her last week RBC Phenotyping was the only test she mentioned that I need to have done and she did guide my doctor through this.  Luckily my doctor responded to her.  How do you think I should go about getting the genotype testing done?  

I am afraid at this point that the transfusion will be done before the genotyping will be done.  

Sharmin, I am sorry i missed your call!  We celebrate thanksgiving on wed with one fam and on thursday with the other so it's been a busy few days.

Also, regarding lena's comment - I am blessed to have such great support from everyone on this site - especially sharmin.  My son has the same type of thalessemia as Little A.  Her guidance has kept me stronger than I would have been through all of this if I was by myself and for this I am so greatful.  Seeing Sharmin and her family has kept my husband and I so positive about Little Ari's future.  

I know that Sharmin went through the beginning with Little A alone - I CANNOT imagine that.  I am so so so blessed.  

Hi Carsima,

The purpose of DNA genotype is to supplement what the phenotype matching can do. With genotype matching, you can make sure the blood is closer to your own racial or ethnic group, as these groups have characteristics more unique to that group. When the donated blood does not come from the same racial group as the recipient, it does increase the possibility of picking up one of the more rare alloantibodies that are not elimintaed with phenotype matching. However, because this happens when the recipient is living where there own racial group is not native, it becomes harder to find blood that is from your own racial group, so it is often not practical to try to find genotype matched blood. When there is a case like little A where the alloantibody has resulted in an autoantibody reaction, it is extremely helpful if genotype matching can be done to avoid introducing anymore of the more rare alloantibodies to the blood.

The general consensus now is that genotype matching, although an important part of the blood matching process, is not completely practical because of the difficulty in finding blood that totally matches the genotype. There is some hope that in the future, those at risk for alloantibody reactions will be able to be identified so that genotype matching is also done, but this is still just in theory.

If your doctors are interested in learning more about genotype matching, please show them the link to this article, which was published this year.
http://bloodjournal.hematologylibrary.org/cgi/content/full/114/2/248   I'll print the conclusion here.

DNA-based blood group typing provides a valuable adjunct not an alternative to traditional methods of pretransfusion testing. Traditional methods for ABO and D typing are likely to continue, and methods for antibody detection and identification will still be required. Theoretically, it is possible to match patient and donor blood group genotypes electronically with the use of gene chip technology. However, this assumes DNA-based methods are totally robust, which they are not because novel mutations arise continually. It also assumes the blood available for transfusion at any given time will match all patients' blood group phenotypes for all possible polymorphic antigens capable of stimulating clinically significant antibodies, which is unrealistic. Tandem application of DNA-based methodology and existing methods will however provide improvements in the provision of extensively blood group–phenotyped red cells for patients with alloantibodies.

And thank you. Your words about how much difference this group can make to new parents means a tremendous amount to me. As a parent, I can really appreciate what that means and it tells me that we are really fulfilling one of the major goals of this group, and that is to help parents get through that period when they first learn of their child's thalassemia. One reason I have chosen parents as moderators is because they have lived through it and are in good position to help new parents go through the same experience. I am very happy with the ongoing efforts of our moderators and our members, who all do so much to help others, even though there is no reason they have to help anyone. But to me, it's all part of being good humans.

Carisma,

I hope that Ari is doing well.  Please keep us posted on his hg and what the doctors are deciding to do. 

Little A had his genotype testing done November 2008.  If you contact the Oakland clinic (I have sent you their phone number) - they can send you the information needed for genotype testing. 

Please let me know how it goes,

Sharmin

Hello All,

Ari's hg is still at 6.  They still do not want to transfuse and have the hope that he is going through the nadir.  I have told them that is unlikely as he is a beta 0 and explained the info sharmin gave me but the doctor says that his vitals are fine so it should be fine.  I don't really know how to handle the situation and make them change their minds on this issue.  I will continue to watch Ari and I am schedueled to go back in a week and a half.

As far as genotyping, I have somewhat good news.  My doctor will review the information Andy has sent.  He also knows dr. V from the oakland center and has told me he will contact him to get info.  I will also contact him tomorrow.