Miracle?

Hey guys!

Just thought I'd update you on Lauryns health. We went to the hospital for her bi-weekly appt and this is the great news. Her hemoglobin went UP 2 points from a 9 (2 weeks ago) to a 9.2 !!!!
the md said her heart rate was faster last time (170) indicating her heart was working a little harder. Today it was normal at 140! She weighs 13.5 lbs and he was very suprised with her colour, (she is very pink). He does not want to see us for another 3 1/2 weeks. This is the 3rd visit we thought we might have to tranx her. What a great Thanksgiving for us. We certainly have a lot to be thankful for.

Does anyone have any explanations as to why her hgb is raising??? I still want the DNA test but he insists it will not matter. Should I just wait to see if she continues to go up?
Also, we cannot find low iron cereal anywhere!!!! SO, I biught her ORGANIC whole grain cereal for now. (I have a call into her hemo.)It says there is 45% iron in it. Is this ok? Also, can i start her on peaches? It says there is  0 % iron in that. ??

Thanks guys for your input

Christine

Hi Christine,

Things sound great!  We fed my son organic cereal.  His iron levels remain at a good level with regular desferal and we avoid and fortified iron products. Organic foods don't tend to be enriched with iron.   He has a balanced diet, we limit the number of high iron meats he eats (beef and pork to once or twice a month) but he eats everything else. We absorb more iron from meat then fruits and vegetables.

We also make sure that he has enough calcium - with yogurt and milk. 

Your daughter has not been transfused so her iron levels should not be an issue at this time.  It is a good idea to keep her away from iron fortified cereals and formulas though.  I would not restrict any fruits and vegetables, but you may wish to see a dietician.

Have a great Thanksgiving,
Sharmin

Congratulations Christine. I am soooooooooooo  happy for you.  This is really good news.

  :five                                         


Christine, I don't have an explanation for why this happened, but Ahmad for example, has his hb tested every month and he had several readings. We started with 6.4 , then 7 , then 6.1 then 6.4 again and the doctor keeps saying that it is normal to go up or down,it all depends on the hemolysis rate. 

Is Lauyrn taking folic acid ??  It is very important to have it as it helps alot in the formation of blood.

There is also one important thing which is that you have to do your CBC in the same lab each time so that you can compare the readings .  Labs use different kits to give their results and changing labs everytime could lead to some variations.

Anyway, enjoy it and happy thanksgiving

Manal

That is such great news, Christine! I know one thing that really affects hemoglobin levels is hydration. If you are dehydrated at all, your blood will be more coagulated together, and your hemoglobin count will be higher, perhaps not accurately reflecting your true level.  At least that is what my doctor told me, and it sems to be true. My hemoglobin level went up when I was dehydrated with the flu, even though I hadn't had a transfusion. I'm sure there are other things that affect it too, higher or lower.
     Anyway, let's be happy for the good news. Take care, Jean
     
     

Great news!

I still don't understand why your doc is not recommending DNA test. It can solve all your queries. I guess he thinks once you know Lauryn is not Thal major by DNA, you won't be visiting him this often. 

I smell something fishy! Does he charge you every time you visit?

Take care, Peace!

I totally agree with Sajid

Manal

Well, we have great insurance, and we do not pay anything out of pocket, so i doubt that is the reason. We go to Phoenix childrens hospital, and they have a great reputation for customer service and care. I certainly hope they wouldnt exploit me like that!

His reasoning is.... She had 2 electrophoresis tests done that prove 100% fetal hemoglobin. He actually might be sparing me the cost of the DNA test?

My theory is that if her hgb keeps improving, we need to investigate further. I know its normal to fluctuate a couple of points up or down now and then.

I can see an intermedia going up and down but a major?? I dont know. Why would a major increase over a 3 week period instead of drop?? I dont understand. UNLESS she is an intermedia. All I know is I will not question it anymore, and I will take it!!!

I started Lauryn on cereal and peaches and she loooovvveeesss it!!!!! :pop :pop

Love to all,
Christine

There is absolutely a logical reason for increase in HB level. Lauryn is still under one and she is well capable to produce Fetal Hemoglobin, the body tendency to produce Fetal Hemoglobin increases when the Adult Chains fails to work. The doctor will not transfuse her until her Fetal chains stop working. This is exactly similar to my daughter situation where she kept on going without transfusion for nine months until comes a point where she gave up. Actually come to think about it, Lauryns heart rate is telling that her body is working extra hard to compensate for falling HB levels. Well hang in there and enjoy Thanksgiving.

Regards

hi canadian,

the dr says her heart rate has improved and is at a normal pace right now. he said it is not working hard. it was before at 170 and now its at 140.

Christine, that is, of course, fabulous news!  :hug

I agree with what Jean said and the bit about fetal hemoglobin.

Since Lauryn is still so young, it's a bit difficult to predict just 'stable' her hemoglobin is at this moment because her fetal Hg is still fluctuating.  When her 'adult' Hg levels start showing themselves, you will have a better indicator.

At this point though, that's very heartening to hear that her levels have increased and that she has a healthy appetite for the cereal and peaches!  I adore peaches myself.   

As for the DNA test... without looking at the DNA itself, how would you know exactly what sort of thalassemia or anemia she has and what sorts of deletions or abnormalities there are?   

But I suppose electrophoresis combined with looking at the blood through an electron microscope would be enough to make a fairly specific diagnosis in most cases.

I'm guessing that under the electron microscope, thalassemia looks a certain way - I know with HgH Disease, there are particular indicators combined with electrophoresis results that more or less point to HgH with little room for anything else.

100% fetal hemoglobin usually indicates either sickle cell anemia or beta thalassemia major and since sickle cell also has a very particular look to it, the conclusion is that the 100% fetal Hg is indicative of beta thal major.

 

Hi Zadhki!

Yes I am estatic for the obivious.... but also b/c her dr.s were almost positive at the rate she was dropping she would've needed a tx 2 months ago. She goes in  around 3 week intervals.

at birth: 16
8 days:13.3
3 weeks:10.3
1 1/2 months:9.9
2 1/2 months:9
3 months:9.2

So you see, it looked like she was dropping .3 decimals every 3 weeks or so. I think knowing her exact mutations is essential in her diagnosis. But if you think about it, if shes going to exude all of the thal major symptoms, then treat her like a major? Of course Only when her hgb is low enough to tx.

Can you explain how her heart rate improved that much from jumping just .2 decimals?

Andy, Can you shed some light on this whole situation??? What do you think this is an indicator of?


Thanks

Christine

Hi Christine,

Every thal patient is unique and presents differently.  In the first 2 months my son's hemoglobin was perfect (due to fetal hemoglobin) but at 3 months his hemoglobin had fallen to 44.  Only his pediatrician will know for sure, and I can only hope that it is true - maybe your daughter's thalassemia will be less severe.  I've also heard that there are some people who (for some reason) produce fetal hemoglobin their whole lives and live very normal lives.  This is however very rare, and I can't be sure what is going on with your daughter.  Let's hope that her thalassemia is less severe and that she will need fewer transfusions and therefore less problems with iron.  Could it be possible that she may have alpha trait as well which may make her  thal less severe?  For all of these reasons I personally would recommend a DNA test so that she gets the best treatment possible and so that you are not left guessing.  It can be emotionally draining trying to figure this out blood test to blood test.  These emotional highs and lows can be tough on you, especially if she were to maintain her hg for a while only for her fetal hemoglobin to taper off, yielding a lower hemoglobin sometime in the future and dissappointing you.  Of course, this may not be the case - but getting a dna test would take the guess work out of this and prepare you for what to expect.  To me it sounds like your child's thal is less severe, in my personal experience, although my son had a great apetite and was very alert at 3 months - he began to look quite pale, had a slightly enlarged spleen and very low hemoglobin.  Take this as a good sign and get that DNA test!  All the best!  and happy Thanksgiving.  Sharmin :hug

Hi Christine,

As Canadian Family said, it is normal for the HB to stay at acceptable levels at this point. Many thals don't need transfusions before the 6-9 month period. I agree that transfusion should only be started when the Hb has dropped (the accepted level may vary from place to place) to the 6-8 range and shows no sign of leveling off. As has been stated here before, something as simple as the hydration level can affect Hb readings. Even though there has been some slight variation in the readings it isn't a large change and indicates that the HB has temporarily reached this range. I've seen nothing in her case that suggests she will need early transfusion. Many factors affect the heartbeat, excitement and sodium or hydration level can also be a factor,  and the higher beat may not have even been Hb related. I doubt the slight change in Hb is why the heartbeat slowed. And as others have suggested, the later manifestation of symptoms may indicate that Lauryn's thal is not on the severe end of major. Since the major distinction of intermedia from major is an ability to maintain a low Hb level, but high enough to sustain a minimum of health, it may some months before you know for sure. Please do ask the doctors if they can tell for sure if it is major or intermedia at this point.

I think all we can tell now is that things are going normally. Her Hb is high enough to sustain her health and growth at the moment but signs of thal symptoms often only begin to first show around six months. Most people I have talked to first found out about their child's thal from the symptoms. You are lucky to be in one of the 42 US states that check for hemoglobin disorders at birth. I know it's not lucky to find out your child is thal but the earlier it is known, the earlier treatments will be started, resulting in normal growth. I cannot not emphasize enough what a difference this makes for the child, especially in terms of skeletal development. Even delaying treatment until age two can result in deformities. When you talk to parents in countries where diagnosis usually comes after some damage has already been done and where care is basic at best, you will start to realize that your daughter is indeed very fortunate to have been born in a county where thal is found early and treated as soon as necessary.

Hi Zadhki!

Yes I am estatic for the obivious.... but also b/c her dr.s were almost positive at the rate she was dropping she would've needed a tx 2 months ago. She goes in  around 3 week intervals.

at birth: 16
8 days:13.3
3 weeks:10.3
1 1/2 months:9.9
2 1/2 months:9
3 months:9.2

So you see, it looked like she was dropping .3 decimals every 3 weeks or so. I think knowing her exact mutations is essential in her diagnosis. But if you think about it, if shes going to exude all of the thal major symptoms, then treat her like a major? Of course Only when her hgb is low enough to tx.

Can you explain how her heart rate improved that much from jumping just .2 decimals?

Andy, Can you shed some light on this whole situation??? What do you think this is an indicator of?


Thanks

Christine

Hi Christine! 

Something that I have never quite understood about the types of thalassemia is exactly where the symptoms come from. 

Do they come from the low(er) hemoglobin alone?  Are they a result of a certain deleted or dysfunctional gene - eg: two people have a Hg level of 8 and have different symptoms - is it because one has Intermedia and the other one has HgH or HgH Constant Spring?

If the symptoms are only a matter of Hg levels, then I can see how doctors might think additional DNA typing would be unnecessary because all they have to do to predict the course of a thal's thalassemia would be to compare it to those who share the same Hg levels and the same general type of thal.

To me, it is a foolish way to try and predict and treat thal because as we all know, even in each division of thal, there are variants, and where one can make an educated guess based off of something similar, it isn't fair to compare a granny smith apple to a fuji apple and say that they're exactly alike.

Now exactly whether the different gene deletions affect HOW the symptoms manifest and WHAT they are versus basing symptoms off of Hg levels... I really have no idea.

Bleh.  I think I just confused myself.  I guess this is something I should research more on, but YES, I definitely think that genetic testing is useful in telling exactly what sort of mutations or deletions you have going on. 



Anyhow, about the heartrate.

Heartrate can be affected by a lot of things - stress, illness, overexertion, etc.  Considering that a lower Hg level requires more work from the bone marrow and whatnot, I wouldn't be surprised if it also affected the heartrate as well?  Now that the Hg levels bumped up a bit, her body doesn't have to do -quite- as much work?

 

I can definitely tell you that when I was more anemic, I definitely had variations in my pulse and for the longest time as a child, I had a very rapid heartbeat.

Even now at 24, my heartrate is at 80-something and that is normal for me and my Hg levels are 7-8.