Hi Everyone,
As you will see, I am new to this forum and looking for some direction on where in the forum I might best fit in! Looking for any information, other families like ours and any suggestions! Below is our story so far!
My husband was diagnosed with Pure Red Cell Aplasia near the end of the school year in 2009 (he was a teacher in Florida) at age 42. We thought he had heat exhaustion from mowing the lawn (it gets in the high 90s in May) and was just struggling to recover. Turned out to be the start of a very rough few years with very frequent transfusions of packed red cells. We celebrated our first wedding anniversary with a visit to the Mayo Clinic in Jacksonville, FL in June of 2009 (yup, we had just gotten married in 2008!
).
After the first 2 initial hospitalizations, within a week of each other, where he needed 3 units of PRCs, we knew it was not going to be an easy time. Our local hemotologist did the first initial bone marrow biopsy with no real answers in the results. By that time we had an appt out at the Mayo Clinic, who after reviewing the case diagnosed Brian with Prca and as he was receiving PRC transfusions every 7 to 10 days, iron overload.
Initial therapy included Prednisone (horrible stuff, I'm sure most would agree!) of 60 mg and Exjade. Brian continued to need transfusions every 7 to 10 days. A 2nd bone marrow biopsy
in October 2009 showed a small colony of CLL clone cells. As Prednisone produced no relief, we moved on to taking cyclosporine at the start of 2010 and tried to get off the Prednisone (like I said, horrible stuff, Brian suffered severe rashes and reactions, resulting in it taking 6 months to get off the prednisone).
Cyclosporine produced no results, Brian continued to need PRCs every 7 to 10 days. Brian had continued to be on Exjade throughout, with ferritin levels off the chart and then getting into the 3000 to 4000 levels. By this time we were a 10 months in, veins collapsing, Exjade dosage way up and not helping, and moved into the next step of the rituxan/cytuxan chemo combination. We had spent countless hours in the hospital (do we get a frequent visitor card?), and we continued to laugh and say it was like being at Cheers - they all new our name at our small town hospital! Each step of the way we worked with Mayo in Jacksonville and our local doctors to administer treatments. We had the first port installed before chemo and also switched to the Desferal infusion 5 nights/week 12 hrs.
A 3rd bone marrow biopsy in the summer of 2010 (post chemo) no longer showed CLL clone cells.
Our family struggled to understand what was going on and really did not grasp what PRCA was and all the various complications that we would then run into. My mom and our friends locally have been our life line in helping us get Brian to treatments, doctor appts and taking the 4 hour trip from our home to the Mayo Clinic. Luckily my job has been incredibly flexible and we have fantastic insurance.
2010 turned out to be a really difficult year for Brian as we battled frequent transfusions, treatments, port installations, port removals - one for a blood clot and another for a major infection, on to a PICC line to administer both Desferal and IV antibiotics and finally the removal of his spleen in December 2010. Our scariest moment was having to go to an infectious disease doctor, while on immuno suppressants
. I have lost count of the number of transfusions and times we spent multiple days in the hospital.
In Dec 2010 it appeared the bone marrow was functioning again and the spleen was harboring the red cells. Throughout the prior year and a half we noticed that after any type of procedure (port install/removals) we got a bit of a breather from transfusions. With the spleen removal Brian was able to go 6 months w/out a transfusion.
2011 started out very well for Brian, recovering from the surgery, all ports/PICC lines removed and levels starting to stabalize. February was a great month for us! By the time March/April came around, we were heading back towards transfusions. Starting in May, Brian has been transfusion dependant again - about every 3 to 4 weeks right now (still an improvement over 2009/2010 at weekly) and is on Exjade again.
He just had his 4th bone marrow biopsy last week at Mayo, with confirmation so far that the PRCA is still present. We are pending further results to confirm if there is any CLL this time as well. We are submitting our case for a "paper" review to Dana Farber, but so far based on the discussions with the doctor there, things are as would be expected
.
We are pending the final results from the recent biopsy, but it sounds like they want us to start with the Prednisone again, along with switching to Desferal infusions. We are not looking forward to a repeat of last year if he needs to have a port installed again. Since last year's treatments he now has the additional complication of an adhesive allergy, along with things like chloraprep etc.
So that is where we are at today, we should hear more from our doctor in this up comming week. As we start down the path to the treatments again, I started to look for others that are facing similar situations. It has been tough with so many of our friends and family not understanding PRCA and that Brian is tired all the time and that this is a chronic issue for him. The doctors believe he has chronic idiopathic PRCA, but hope that with the spleen being gone (and further reducing his immune system) in combination with treatment again, we might see more relief between transfusions.
I started to read the thread in reference to the iron overload and will go through that in more detail as one area that will be helpful. But also wanted to introduce us to the forum for any other suggested topics that might be helpful! Thanks for reading (if you made it this far!) and look forward to chatting with you all!