Difference

Lena,
this winter Najaf has been very resistent to cold and flu etc...there is nothing wrong without vomiting and refusal to eat.......
yes i too had that point in mind so we got Hb report from two labs one stating 4.5 and other with 5. so hb drop is for sure....

Andy,
Najaf had been ill i mean vomiting etc before the transfusion....and the condition persisted even after transfusion.....
can we say that the blood quality was not good may be blood was old enough or something like that

Abbas

hi,
Oh my Goodness, how difficult these days were....
najaf was transfuse again on 25 january and after 5 days her hb was 3.5....
at the same time she was having fever...we contacted her doctor and she said Najaf must visit her........
there she was hospitalized for 2 weeks... the main problem was fever which was uncontrolable...after every 12 hrs she had svere fever..
there were a number of possibilities i.e malaria, typhoid, Tuberclosis, but everything ruled out...
at the same time she was transfused 3 units in hospital, doctor was not able to diagonise the cause of fever....
but at the end Dr. Javeria concluded that she had "autoimune hemolytic anemia"
direct and indirect Coombs tests were positive,
at last she got rid of fever and then the problem arose of transfusion because her blood was not getting cross matched even with O negative, she was given steroids and is still taking....
even todays evening there was no cross match with O -ve.
one more thing her Fe is 5300...
i will let u know with rest of her testing later, i m in a bit hurry.....

I am so sorry to know all you have been through Please keep us updated and what is the doctor's plane to fight this autoimune H.A.   

Is she going to continue on steriods for a while??? Please make sure that she always gets the right blood??

Is she still on hydroxyurea??

manal

My son also has autoimmune hemolytic anemia. His blood type is also O-.   The first line of treatment is steroids.  2g per kg of body weight for 8 days, then half that dose for for 8 days.   See if that works.  Prednisone should be given a couple of days prior to,  during and after transfusion. 

IVIG can be used as a second line treatment if prednisone is ineffective.  If her spleen is enlarged, splenectomy may be required.  The other therapies all work better if splenectomy is done (if spleen is large). 

Lastly, ritixumab may be used in conjunction with ivig. 

If none of these methods are successful, BMT is considered as it can cure both AIHA and thalassemia. 

It is best to start with pulses of prednisone before considering any other treatment. 

Please keep me posted,

Sharmin

Manal,
doctor is fighting AIHA with steroids and she is on steroids yet... Najaf has also resumed HU.

Sharmin,
she is taking Prednisolone 0.4g/kg/day, lets c if it works...her spleen is also enlarged i.e almost double of the required size { i will let u know the exact figure later}
doctor says AIHA is not permanent, antibodies are automatically killed after completing their tenure of 15 to 20 weeks......with the help of steroids they will be suppressed soon..

Abbas

Abbas,

I am really sorry to hear what Najaf went through,it must be hard for your family too,i hope what the doctor is saying is correct and Najaf won't have to experience this again,

Best wishes,

Zaini.

Zaini Insha Allah,
lets hope so........

Abbas

hi,
sorry for being late..

during the last fortnight Najaf has been a lot finer....
she is much healthier and active...
her hb is even more than our expectation
last week; 9.5
this week: 10.2
i havent been able to understand this increase in Hb is this due to HU?
also her direct and indirect Coombs test have been negative this week. can we say that she is free of AIHA now???
najaf is also taking DFO 5 times a week....

hope all of u to be fine

Abbas

It may be too early to determine if this is all due to hydroxy, but the preliminary signs are good. It does appear that the steroid has done its job and knocked out the antibody, which would keep the Hb level higher.

Andy,

I am quiet confused, is it okay to use hydroxyurea in the presense of antibodies??

manal

Manal,

Is there any known problem using hydroxyurea when alloimmunization (antibody reaction) has taken place? I had a very hard time finding any reference to it. I did finally find one case where transfusions were stopped in a woman with sickle cell and hydroxyurea was started to boost her fetal hemoglobin level. One year later, she had not transfused and was doing much better.

http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B7W6S-4GJMPM8-2&_user=10&_coverDate=12%2F31%2F1993&_rdoc=1&_fmt=high&_orig=search&_sort=d&_docanchor=&view=c&_searchStrId=1252900609&_rerunOrigin=google&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=ad51342dec303d6e9a70e5ce2fd3e290

Summary

Alloimmunization following multiple transfusions, a frequently observed complication in sickle cell anemia patients, may make subsequent transfusion attempts extremely hazardous.

The case of a young woman with sickle-cell anemia is reported ; she was hospitalized for the treatment of extensive invalidating leg ulcers. The transfusion program that was initiated as a prerequisite to skin allografts had to be stopped, due to rapid occurrence of multiple alloimmunization.

Broad spectrum alloantibodies precluded further selection of compatible blood units.

In an attempt to get round this impossibility, a treatment with Hydroxyurea was initiated in order to boost synthesis of F-hemoglobin. After one year under this treatment, the patient's clinical status has clearly improved without requiring any new blood transfusion.

Abbas,

I am really really glad to hear this i hope Najaf will not have to face antibody issue ever again ,Good luck .

Zaini.

Thanks Andy for your efforts.

I was just wondering that since hydroxy is an immunosuppressent, so could it help in reducing the antibodies produced by the body or there is no relation

manal

Thanks Andy, Manal and Zaini sis,

thanks to all for your kind words....

Abbas

hi,

hope u all to be very fine...
Najaf's Hb is 10.5 for the last two consecutive weeks.........

today she had a little fever,, tomorrow is her first day at school 
wish her luck,,,,,,,,,,,,

Abbas