One Year follow up

We are leaving for our trip tomorrow.  Our treatment in Oakland began one year ago.  This is also when we started the combination therapy.  I believe that it has been very good for him and during this one year we did not experience any adverse effects, other than occasional nausea.  I hope that others will soon be able to use combination therapy such as this in the near future.  Personally, I think that exjade/L1 combination would be great.

Lil A's ferritin levels have been below 1000 recently, we will see what the SQUID test reveals.  If his levels are low enough perhaps we can stop using desferal!  He would be thrilled about that! 

I will post updates from our visit and anything new that I learn as soon a possible.

much love to all of you and thank you all for your support throughout this time.  Andy, I cannot thank you enough for encouraging us to go to Oakland. 

Sharmin

Sharmin,

Have a safe trip  i am sure every report will be fine ,much love to Lil A.

Zaini.

Thank you for your support Zaini   
I will be thinking of you and lil Z when I am there. 

Have you heard anything about having ferriscan or T2* there for lil Z?

Sharmin

Good luck Sharmin, i am sure that the doctors will be amazed with his reports

manal

Sharmin and Everyone,

The recent tragic passing of young Thoa is a harsh reminder that patients need to be treated by doctors who fully understand how to treat thalassemia. I am so glad that little A is able to be seen at Oakland and I want to encourage everyone to talk to their doctors about the comprehensive approach to treating thal. With a well planned treatment program and annual assessments, the lives of thals can be greatly improved and even saved. This close supervision usually has the benefit of improving patient compliance with the program. Competent treatment should be a basic right for all thals. 

The Standards of care guidelines can be found in the Documents section at
http://www.thalassemiapatientsandfriends.com/index.php?topic=1762.msg14639#msg14639

Sharmin, have a good trip. I think little A is doing well and this will be shown by his annual assessment.

Sharmin,

Ferriscan or T2* isn't available in our country,so right now we just have to depend on Echo,ultrasound or MRI  Hope thses technologies will be available soon,there is a great number of thals in Pakistan who can benefit from these technologies.

Zaini.

Sharmin ,
Good luck with Lil-A's annual check-up .... wish that everything will be fine  ....

lots of Best Regards
Umair

Thank you all for your wishes and support.  We have had our one year follow up.  Things look good at this time.  The SQUID results revealed that lil A's iron level is 943 - which is an amazing result.  The clinic was very pleased that the combination therapy - with very good compliance  has been very successful.  We have decided to discontinue desferal, at least for the time being (perhaps permanently) and to give lil A the maximum dose of exjade (30mg/kg).   We arrived at home last night, so we are waiting to arrange exjade here so until then we will continue with desferal. 

The docs are happy with lil A's progress and they feel that he is very healthy at this time.  The presence of the antibody is an issue and they do not think that continuing with rituximab is a good idea.  Another child was given ritux with steroid treatment - and that child overcame antibodies completely.  In our case that didn't happen.  Even though the antibody problem became less significant it did not diminish.  The docs in Oakland say that we need to come up with a better solution because giving more ritux can cause damage to the B cells.  Transplant was given as an option.  Of course we are very afraid of transplant and have come home with a lot to think about. 

In the meantime, I am glad that lil A is healthy at this time, and we are hopeful that the antibodies do not pose a big problem.  We are also happy to be free of desferal - for a period of time, maybe permanently. 

Any and all progress that we have made, we owe to Andy and this group.  Lil A would not be in the great health he is in, and he would not be receiving this wonderful care if it weren't for Andy and this group.  Thank you from the bottom of our hearts Andy and everyone else here. 

I will post more details from our visit soon. 

Thanks again everyone,

Sharmin

Sharmin,

I am extremely happy for Lil A, i wish and i hope that every thal in this world would be as healthy as him,you are really lucky that you had the chance to visit such a wonderful thal center.

What is going on with Lil Z's Sgpt levels has got me thinking that may be if her doctors were willing to try desferal with Asunra,it would have lowered her ferritin much much faster and we might have been able to avoid this situation  ..

Zaini.

Dear Zaini, dont think in if and do NOT blame yourself. Everythink possible is crossed for you. Hugs from me and the cat to your daughter and you.
Your daughter is not the only one. Exjade is the one to blame.

i am glad to hear that Sharmin!!!!! Im bit confused about the anti body thhing. I pray they find an easier treatment than transplant.

Have à good new week,

Dore

Thanks Dore  for your kind words,i am praying that Lil Z's SGPT will be down by the end of this week.

Zaini.

Zaini,

I hope that lil Z's SGPT levels will be good soon.  The dfo/exjade study will come to an end soon and the data should be published soon.  The results have been very positive in compliant patients, therefore I think that more doctors will be encouraged to prescribe to combination in the near future.  I think that lil A was able to try this combination because things were spiraling out of control for us a year ago as his iron levels approached 3000.  With his high tx needs and the beginning of iron overload, the docs felt that enrolling him on this trial may be a way of removing iron as well as keeping of with the excessive iron that was coming in.  I am happy about the iron at this time, however I am afraid of the antibody that may still lurk as we have run out of options to deal with it.  With the antibody comes fatigue, multiple transfusions and increased iron.  It is very scary.  My hope is that the antibody is not going to come back this time. 

I hope that the results of this trial will help many people and that lil Z's doc will prescribe combination therapy.  Within a short time lil Z's iron levels will come down and then she can continue with exjade alone. 

It would not hurt to take exjade away completely for a few days because she is not overloaded.  Once the SGPT issue is reversed then she can resume - I think that this may be the best way to make things return to normal.  Way had to do this with lil A when he was 6 years old (we took away desferal for 1 month) and the toxicity was completely reversed and then we resumed desferal.  In lil Z's case a few days should be enough.

Best of luck Zaini, always thinking of you and lil Z:)

Sharmin

Sharmin,

I think concentrating on building little A's immune system will be of some value in countering the antibody problem. Continue to do everything you have been doing. I have seen this with other patients and often it does go into remission. The supplements play a key role in maintaining a healthy immune system, and you should note that other illnesses can open the door to the antibody by weakening the immune system, so keep him as healthy as possible and always, always, always, reinforce the idea that he should wash his hands after contact with anything public. I would also suggest using a neti pot to keep his nasal passages clean. This really does cut down on the number of colds one gets and even helps with allergies. Keeping him healthy will help his immune system function at an optimum level.

I'm heading out tonight to go back home, so I will not be online for awhile. I'll check in late tonight.

Ok so may be i was wrong  i thought cutting off chelation totally wasn't good,so i kept giving her 400 mg,since she just got transfused on saturday and that was confusing to me that there would be more free iron in the blood which can cause damage  to tell you the truth i am still unable to decide whats best  .

Zaini.

Thanks Andy,

I will focus on the supplements.  I hope that you enjoyed your vacation, have a safe trip home.

Zaini,

Usually it is not a good idea to stop chelation completely, but in certain cases it is important.  I think that it is a good idea to give the liver a complete break before resuming exjade - and chances are that her levels will be good in the future.  While she is off of the exjade increase her IP6 - give it to her at least 3 times a day.  Also give her more fresh fruit throughout the day - blueberries, rasberries, cranberries - if possible.  Antioxidants will bind the free iron and protect her from damage while she is off of the exjade.  Tea will also be a good idea during this time.  I think she should be fine for a few days without chelation.  In this case it might be a very good idea.  If her SGPT does not return to normal after taking her off for a few days, perhaps you can try L1 for a month and then return to exjade. We are lucky that we have lot of options these days, switching for a short time can be very helpful. 

Andy, do you agree?

Sharmin