Discussion Forums > Thalassemia Major
Little Ari
Sharmin:
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
Andy Battaglia:
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.
Cari:
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
Sharmin:
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
Lena:
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.
Navigation
[0] Message Index
[#] Next page
[*] Previous page
Go to full version