Seeking Some Guidance for a Thal Major with Lupus

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Offline Narendra

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Seeking Some Guidance for a Thal Major with Lupus
« on: October 15, 2008, 03:22:02 PM »
Folks,

I am seeking your input on a thal patient, I am in touch with who is going through some issues along with her health. I would like to hear what some line of treatment might be for her. Below is what I heard from her.

Her Info
16 years of age, female with thal major and lupus which causes hemolytic anemia and due to which her hb level fall very fast.

Her Treatment of Lupus
For lupus, her doctor put her on immune suppression therapy for over 2 years in 2001. But later the doctor decided that it was not a good idea as it left her absolutely vulnerable to all kinds of diseases. After that, the doctor asked her to take cyclosporine which she started taking. For sometime it looked like it was all balanced. But 2006 onwards things started getting worse for her again. Right now (Oct 2008) even with 6 transfusions a month her hb stays around 6 or 7gm per dl.

Her Iron Chelation Treatment
She is currently on Desferal and Exjade. Her ferritin levels have been way above normal since 5 years.. 10k plus. But last year she had pneumonia and got a lot of transfusions and the ferritin levels rose to 15k. She has been chelating with desferal since she was 4 years and 6 days a week for the last 5 years. Desferal dose she takes is - 12 hours 6 nights a week - 8 viles of desferal 

Was on Kelfer(L1)
Also, she was on kelfer, but due to grade 2 osteopenia and very severe all joint arthritis, had to stop it. It was so bad that she got synovial fluid replaced in her knees and elbows. Had a surgery and synovial fluid injection on her knees and elbows last year. 

Special Clinical trial with Exjade and Desferal – Due to issues with Kelfer(L1), doctor asked to try exjade instead of kelfer under a clinical trial. 8 months ago, she has started exjade(currently on 40 mg per kg), which decreased her Ferritin level a little bit, but even Exjade is messing up her counts (sgpt still continues to be high, kft and lft have all been out of range). Last april, her anc count fell to 200 and she is taking nupogen shots 4 times a week. She is also taking procrit epo 5 shots every other week.

Other Medications
She is taking hylorunic acid glucosamine sulphate and chondroitin sulphate plus iv calcium for her bones. And is also taking IV Calcium. She is taking steroids and salbutamol and epinephrine and prednisolone for asthma. She is also taking endoxan and lasix for kidneys hydrocontin and beta blocker for heart.

Compromise Quality Of Life?
Now the doctor wants her to compromise with her quality of like even more and try to take as less transfusions as possible until ferritin levels dont get better. The doctor wants to let her hb drop to 4 before her transfusion. She is about to consult with Dr Mills and Dr Black in London in next few weeks, and hopefully hear the right course of action to be taken. With college being as hectic as it can, she is not sure if she can compromise on her quality of life with low HB levels.

Has anyone gone through similar scenario's? What might help. I know I am just a 3rd party trying to get information, but I think some points might help her into what really might be useful to her. She can discuss that with the treating doctor's and may be get better treatment.

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Offline Sharmin

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Re: Seeking Some Guidance for a Thal Major with Lupus
« Reply #1 on: October 15, 2008, 03:28:34 PM »
Narendra,

I am so sorry to hear what this patient is going through, dealing with two major diseases.  In order to gain control of the diseases her hemotologist and rheumatologist need to work out a plan together.  As Andy has suggested in the past, she will require smaller, slow rate transfusions. 

I wonder if rituximab would be helpful for her, maybe she can mention that to her doctor.  I definitely think she needs to be on more desferal as well - 7 days a week and some days with 18 - 20 hours.  There are pumps available that are very small and can be worn all the time. 

I will see what other information I can get.  Andy can certainly provide you with more information.

Sharmin
Sharmin

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Offline Narendra

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Re: Seeking Some Guidance for a Thal Major with Lupus
« Reply #2 on: October 16, 2008, 05:09:35 PM »
Thanks Sharmin,

I did mention the intensive treatment with desferal to her. Also, I will mention the Rituximab treatment option and ask her to discuss it with her doctor's.  If I am not mistaken, did your son go through something similar with the antibodies attacking him?

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Offline Sharmin

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Re: Seeking Some Guidance for a Thal Major with Lupus
« Reply #3 on: October 16, 2008, 11:01:52 PM »
Hi Narendra,

Yes, my son did have antibodies for a couple of years, they were causing hemolysis and an increased tx requirement.  Thankfully the problem seems to be resolved at least for the time being, hopefully for good.  His iron levels were 2880 in June because he had been transfused quite a bit in the last 2 years.  Combination Exjade/desferal chelation therapy has reduced it to 1320 so things are much better.  During the summer he was on desferal 7 nights a week, 12 hours a night.  Now he is on desferal 5 nights a week, 12 hours a night as well as Exjade once a day - and we hope to continue to reduce desferal until he can be on Exjade alone. 

I really hope that the thal patient you describe gets this treatment soon because both lupus and the oxidative stress from the iron overdose put her at a high risk for organ damage.  Perhaps she should be admitted to the hospital a few times a month for 24hr IV desferal - as well as around the clock desferal via pump infusion.  She really needs to get the iron levels down, because the chelation must manage the iron already stored in her body as well as the iron coming in from her frequent transfusions.  I really believe that the rituximab treatment will help with the autoimmune hemolysis.  It may help with the lupus too (but I am not sure because I don't know if B cells also make the antibodies that cause lupus).   They should give her gamma globulin (i think it would be 4mg per kg of body weight) followed by a course of rituximab - which usually consists of 4 treatments 1 week apart each.  This can be followed by monthly IVIG treatments, and I think her transfusion requirement will decrease within a couple of months once the ritux treatment is complete. 

Best of luck, please keep us posted on this patient.

Sharmin
« Last Edit: October 16, 2008, 11:19:32 PM by sharmin »
Sharmin

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Offline Andy Battaglia

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Re: Seeking Some Guidance for a Thal Major with Lupus
« Reply #4 on: October 17, 2008, 04:09:13 PM »
One note on rituxan and lupus. Trials of rituxan in lupus patients have been somewhat disappointing and rituxan has not yet been approved for lupus patients.
See http://www.fiercebiotech.com/press-releases/genentech-and-biogen-idec-phase-ii-iii-study-rituxan-fails-meet-endpoints

Phase 3 trials are continuing at this time. http://www.gene.com/gene/pipeline/status/immunology/rituxan/
Andy

All we are saying is give thals a chance.

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Offline Sharmin

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Re: Seeking Some Guidance for a Thal Major with Lupus
« Reply #5 on: October 22, 2008, 04:44:41 AM »
Narendra,

Perhaps the patient can look into registering for a bone marrow registry:

http://www.blood.co.uk/pages/marrow_info.html

Sharmin
Sharmin

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Offline Dori

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Re: Seeking Some Guidance for a Thal Major with Lupus
« Reply #6 on: October 23, 2008, 08:59:33 AM »
My highest doses of Desferal was btw 5x24hours a week...
I feel so sad for her, I can't imagine so many problems. I thought mine were worse, but is much worser. Though I know these probs of chelation therapy.
Narenda, I'm Pear from the other forum....;)

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Offline Zaini

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Re: Seeking Some Guidance for a Thal Major with Lupus
« Reply #7 on: October 23, 2008, 10:49:05 AM »
Hi Dore,

Welcome on the forum  :hugfriend i wanted to ask you if you transfuse regularly?

Take care,

Zaini.
^*^Xaini^*^

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Offline Dori

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Re: Seeking Some Guidance for a Thal Major with Lupus
« Reply #8 on: October 24, 2008, 01:50:26 PM »
yes, I do

While I was a young child I'd every 6 weeks a transfusion
While I went to highschool every 4 weeks
Last year every 2 weeks
At the moment every 3 weeks :)

 

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