frustrated

Umair and Zaini, thank you so much for your wishes.

Yesterday, before his transfusion his ferritin was 1249 - which is really good.  I hope that the additional transfusion yesterday did not increase it too much.  We are going to keep chelating him everyday as much as possible in order to get it down further.   I hope that the SQUID reveals that his iron levels are low.  At the end of the day, even if his transfusion requirement remains a little high - meaning that he needs to transfuse every three weeks - the important thing is to keep the iron levels down.  I hope that we have gained control over the iron now.  Of course I will post the result from our hotel as soon as we get the results! 

His hemoglobin today was 146 so I am hopeful that this transfusion will last him for a while. 

Andy,
Before yesterday's tx his doctor did some tests to determine whether he is hemolyzing excessively,  below are the results:
hemoglobin Monday was 108, hemoglobin on Wednesday morning was 103
Albumin 34 (normal range)
direct bilirubin 6 (normal range)
total bilirubin 26 (high normal is 19)
ferritin 1249

Do these results tell us anything?  It seems to me that the hg drop was small - and the bilirubin and albumin levels were not significantly elevated suggesting that there wasn't any serious hemolysis going on.  Do you agree?  Thank you,

Sharmin

Sharmin,

Do you have previous bilirubin test results for comparison?

Andy,

I don't have previous bilirubin results at this time.  If the hospital has not thinned his chart then I will be able to look it up on Tuesday.  His eyes have been red since the 2nd tx, but his hg the day after his tx was 146.  He will have another cbc on Tuesday before he gets his ivig.  He will also have an echo that morning - and I am praying that the results will be good.  This time he has been transfused before doing this test.  When he was tested last spring his hg was very low. 

I am praying that too much hemolysis is not taking place.  Having so many tests planned in the next week has me nervous, especially because of the recent drop in hg.  I hope that everything goes well, I don't know how much more my nerves can take. 

In this week we will have an echo, SQUID and T2* - along with numerous blood tests.  Our last visit opened our eyes to how many things needed to change in respect to his transfusions and chelation.  He was transfusing too much and his iron levels were too high.  I am now hoping that these changes will yield positive results. 

Do these results (hg, albumin, bilirubin) suggest anything? 

Thank you Andy,

Sharmin

Sharmin,

The only result that raises any question is the bilirubin, but if it is not significantly different from previous tests, it would be of no concern. It is a bit high but that is fairly normal in thals.

Sharmin,

I hope everything will be ok,please be strong,i wish you the very best.

Zaini.

Thank you Andy and Zaini 

I think the blood test on Tuesday will tell a lot too.  His eyes don't appear yellow - which is something that his doctor told me to watch out for.  He also told us to watch for a change in his urine color (if it is brown or darker than what it usually is with desferal) as that would be a sign of hemolysis. 

The medical staff in Oakland is amazing and it is much easier getting information from them than it is getting it here.  The records that they keep are unbelievable.  They have asked for a bilirubin each month, maybe it will be easier for me to get his bilirubin levels from them. 

Sharmin

No yellow in the eyes is very good so I doubt there is anything unusual going on with hemolysis. I am really looking forward to hearing the results of his upcoming tests. I considered asking about combination desferal and Exjade at the NYC symposium, but thought that perhaps we should wait to see more results of this small trial, and also didn't know if I would be announcing something that shouldn't yet be announced.

Andy,

Personally, I think that the combination has helped us get the iron levels down faster than using one chelator alone.  Using Exjade 7X a week has allowed us to get away with 5X a week of desferal.  If we had desferal alone he would need to be on it much more in order to get his levels down.  The last ferritin (1249) suggests that the levels are coming down.  His appearance (as you have seen) also suggests that the levels are down. 

Sharmin

Oh Sharmin My love and thoughts are with you both.
I do understand your fustration.

Andy,

His total bilirubin in October was 14 (measured during the time that he was able to last 5 weeks without tx).  January 5th his bilirubin was 21 and Wednesday February 4th his bilirubin was 26.  Normal bilirubin range is (0 - 19umol).  The direct bilirubin value is normally 5 this time it was 6 - but it was within the normal range (0 - 7umol)

Does that sound high? 

Thank you Andy,

Sharmin

Andy,

The hemoglobin today is 124.  It dropped from 146 to 124 in 5 days.  I hope that any further drop will be slow so that he can last 3 to 4 weeks before needing another tx.

How does this sound to you as far as hemolysis?

Sharmin

Sharmin,

The hemoglobin level of 146 may not have been quite accurate as it would have meant a 4 point jump after his transfusion, which doesn't seem right since you should only see about a one point jump for every unit of transfused blood. Because the blood volume is increased right after a transfusion, the immediate measurement may not be totally accurate. The state of hydration of the patient for each test is also a factor. His current Hb is in line with what I would expect at this point after the transfusion.

His bilirubin level may be related to his recent illness so again, I wouldn't make much out of it yet. If he has a sudden drop in Hb then I would be more prone to believe that it is the result of hemolysis.

Thank you Andy. 

Yes, that bilirubin was from Wednesday morning when he was still recovering from his cold.  I am hoping that it won't be as high now.  We leave for Oakland tomorrow afternoon, the echo was done today and I am hoping that the results are all good.  I will also ask Oakland about phosphate deficiency and what they have noticed. 

Thanks again Andy,

Sharmin

Hi Sharmin ,
i dont know much abt and wat can i say abt is to wish you allll the best . believe in one thing everything will fine and our admireable su[re lil champ  will b fine . take cares
May God Bless you with the better than the best and more than your aspectations , just keep praying and asking for more ...


Best Regards
Take Care
Umair