New Member - Wife of PRCA patient, Florida

[Reneesctmiller]

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!

[Neil Cuadra]

Welcome, Renee. Please know that Brian isn't alone. We've met a number of PRCA patients and their spouses at Marrowforums and at conferences sponsored by the Aplastic Anemia & MDS International Foundation. Some of them have been fighting pure red cell aplasia for many years. You'll find many stories here about transfusions, PICC lines, hospitalizations, iron overload, and other experiences you've shared, including families who don't quite understand what's happening to you.

Among active forum members I know that triumphe64 and Lbrown often talk about their PRCA. Look for their posts as you read threads of interest.

Given Brian's allergic reaction to prednisone last time, do they have suggestions on how to avoid a similar reaction if it makes sense for him to take it again?

[triumphe64]

Renee,

I'm the only active PRCA poster on this board. It sounds like you at least are seeing the better doctors. I'm going to write you a note off line. Usually, they have never even seen a case or very few cases.

The people at http://www.aamds.org/ can be of great help. Talk to Leigh Clark there. I went three years of total chaos, but not as bad as yours, before I found them. They directed me to Dr. Maciejewski at Cleveland Clinic. He has the most PRCA patients (23 at last count). Otherwise, you just won't find any. I am greatly improved, but there are no quick fixes.

I'm a peer support person for PRCA for the AAMDS people. There is one other lady doing that. There are a group of four of us on facebook. We all have different stories.

[triumphe64]

Renee,

You are blocked from emails or private messages on this board, but if you contact AAMDS as I said, they can help you get some direction.

[Reneesctmiller]

Thank you! I'll update my profile so I may receive private message - sorry, so new to this! I would greatly appreciate any info! I just signed Brian and I up for the conference in Tampa, FL in hopes of connecting with others. Any information would be great! Thank you!

[triumphe64]

I'll reply later in the day, I hope.

The conferences are great, but you will probably not find any PRCA patients in Tampa. You WILL find people who are undergoing similar experiences. That, by itself, will be helpful. I'm not sure what they do in the one day conferences, but they used to have separate groups for patients, for spouses, and for kids. Go to the sessions for AA because it is the most similar to PRCA.

You are on the right track. It took me three years to realize just how rare this is and how little doctors knew. They like to say it's "not very common". That's doctor talk for "I've never seen this before".

[Lbrown]

My official diagnosis is PRCA.

Deb

[Reneesctmiller]

Thanks all! I appreciate the messages. Neil, we are in discussion with the doctors on the next steps, we saw absolutely no results last year with prednisone, but did have complications, so we are hesitant to go that path again. The dr. wants to wait to see all the final results of the biopsy before we decide what's next - we should know some time this week. He had discussed going straight to cyclosporine again, or some combination.

As triumphe64 notes, we do get the comment of not very common a lot! Our doctor at Mayo has seen PRCA before and has been referring back to the main campus in MN, but we realized last year how rare it was when our local hemotologist basically said he could not do anything other than follow the treatment plan Mayo layed out - he has never seen a case like this and basically told us at that point how rare it was.

I am looking forward to the conference and picking up some informaiton in regards to the iron overload - already saw a few things that might be helpful!

I'll keep a look out then for both Deb and triumphe64 post for additional insights! thank you all!

[Reneesctmiller]

So since my last post we have heard back from the doctor at Mayo with the results of the recent BMB and all the blood work/CT scat etc.

Brian's bone marrow results show no RBC production, confirms it is PRCA, flow cymetry normal, no genetic anomoly, no CLL in the bone marrow.

Brian does have 2 lymph nodes that are enlarged, the doctor is concerned that there is CLL in the lymph nodes, although unable to biopsy due to their location.

He is recommending that Brian take cytoxan (daily), with rituxan and decadron every 3 weeks for 18 weeks.

I have a lot of questions for the doctor, so am making a list for our next call with the doctor, but also looking at a 2nd opinion up at Cleveland with Dr. M that triumphe64 is seeing. If CLL is the underlying cause, want to treat that, but w/out it being found in the bone marrow and still in bone marrow failure, concerned as to what is going on and the longer treatment time recommended.

Anyone else on this type of combo? Brian is starting his wheatgrass as well to help with the iron levels, he is currently on Exjade, so hoping this will help as well!

[Blood Vic]

Hi How are you doing? Any updates? I am just on Prednisone again. Getting a quick response but in the past no long term effect.

I was diagnosed with PRCA June 2009. I am 60 and healthy except for weekly blood transfusions and now iron overload. On Exjade. Have been treated with antibodies, Rituxin, Prednisone, Cyclosporine & Cyclophosphamide. Prednisone has had the only ibut just sporatically and hasn't functioned/helped over a longer term. 50 - 100mg/day

I just finished cyclophosphamide and they are looking at possible spleen removal or trying tymoglobulin. Any experience with this? Any new treatments?

[triumphe64]

Vic,
I am on 50mg BID cyclo, 2 1/2 mg every other day Prednizone and 200 Mg Danazol. When the prednisone went to zero, my counts dropped. I hopes this helps. Feel free to email me.

[Blood Vic]

Hi

Thanks so much !! I would like to connect by Email ... not sure how.

I will check out your success as I have been all over the map with different treatments. I have three Hemotologists working on my case but they are stumped.

Look forward to keeping in touch.

Thanks,

Terry

[triumphe64]

I sent an email to you.
FYI - If you click on the name the options come up.

[Blood Vic]

Thanks for the tips. Is your Hemo level fairly
stable? What level? How often do you typically expect transfusions?

I am typically getting 2 pints per week. How did you come up with the drug mix? Is there a doctor that came up with this? I haven't heard of the danazol ... whats its purpose?

Any guesses on the cause of your condition? I have worked in an office
and never been exposed to any special chemicals or bad environment that I
know of.

[Reneesctmiller]

Hi Vic,

Welcome! Currently my husband continues to get 2 units PRBC every 3 weeks or so right now. Over the summer we made the decision to switch from Mayo to the Cleveland Clinic (thanks to Ron !) Last year my husband did have his spleen removed, it was hoarding the red cells, and after that surgery we had almost 6 months transfusion free! Unfortunately at the start of the summer he bacame transfusion dependant again. After visiting Dr. M at the Cleveland Clinic we decided to follow a few "off" label treatments to cross a few things off the list.

First stop, needed a new port installed, but before that could happen we ended up with a bit of a lengthy hospital stay due to a strange infection. So we were a bit delayed in getting this next round of treatment started. He is now doing IVIG to see if that might help at all (so far no change, but not too surprised, they knew it was a long shot). Next up will be prednisone again sometime in the new year with a combo of Cytoxin.

He is taking Exjade again, iron is off the charts once more. We found it very helpful going up to Cleveland - well worth the trip. Gave us a lot more hope in a time we were starting to give up. Also, went to the conference in Tampa, that helped even more to give my husband a lot of hope in seeing all the survivors.

So while we are still searching for the combo that might provide some relief from the transfusions, Dr. M at the Cleveland Clinic and his team have been fantastic in serving up options and always there to help. Feel free to email me anytime - happy to give any info or support!

[Blood Vic]

I have PRCA ... I typically get 2 pints per week on 3000-3500mg per day Exjade to control Iron at 1500. trying to maintain my hemo at 80 (8.0).

I have had periodic positive reactions to prednisone at 50mg/day but no other drug and only temporary ... for a week or two.

I am going to try a new cocktail now. Did Dr. M set you up with something?

Only small reaction to IVIG and no reaction to cyclosporine 6 months. Cyclophosphamide 3 months or Rituxin 3 weeks. The last two were taken with prednisone.

Please keep me posted and I will do the same.

Good luck,

Terry
Victoria, BC Canada

[triumphe64]

Renee - I'm glad to see Dr. M has been of help.. I knew the Tampa visit would help also.

Terry - There was a recent posting on here about PNH. Based on our talk, you don't show the classic symptoms, but you lose blood so quickly, you might have them test you, if they haven't already.

[gina66]

I have PRCA official dx since 12/2011. Mine is definitely due to Parvo Virus infection that was not detected. I have been taking Cyclosporine 200 mg/day and prednisone 20mg/day for 2 mos. now but no progress. Mr. Triumphe helped a lot with info. I am making an appt with Dr. M at Cleveland Clinic. I am also trying to get appt. with NIH. Mayo is a good clinic but I think Dr Neal Young has done a lot of researched already on these illnesses. I was suggesting to my local Dr. to see if I can get more IVIG tx due to the cause of my illness is virus infection. IVIG can boost immune system and may or may not help because currently my Parvo virus test by PCR is negative. IVIG is expensive and so not considered due to negative Parvo virus test result. I have been tempted to stop all the meds and try to boost my immune system by food, supplements, shark oil liver but I do not know if it is the right decision to go. I am just saying based on the source of my illness is due to virus infection, normally they want you to rest, proper nutrition, supplements to boost immune system. Right now with my medications, my immune system is suppressed. After finding out that I have no more Parvovirus infection and I was not taking any meds, my Hemoglobin went up for few weeks. I had PET scan last October 2012, negative, that may mean I have no LGL.

[triumphe64]

gina,

The last time I had heard from Terry, he had somehow recovered completely and was only worried about his high ferritin levels from all his transfusions. He was doing so well they were going to have him "donate" blood rather than taking meds. I guess miracles happen. I'll see if I can contact him now that I have seen his name.

I'm glad to see the parvo is gone. My next appointment with Dr. M is in March.

[Blood Vic]

Hi Gina,

Jan 2, 2013

Latest update ... in remission for 1 year & 1.5 months now. The exjade stopped reducing my iron. I am letting blood now to bring my iron down. I think my immune system was depleated and traumatised and this put me into remission. I feel prednisone is the key but it needs alot of help and over long time periods was not very useful. The cyclophosphmide by IV may have weakened my immune system. 8 pints of new blood ... maybe there was something special in that but getting down to 50 hemo(Canadian scale 60 is critical) and a 100mg dosage of prednisone ... was on antibiotics for a tooth infection? Some drugs boost the effect of other drugs. This is risky mixing drugs but I found alcohol boosted the affect of the prednisone. Sometimes prednisone had a huge but short lived effect and sometimes it didn't have any affect. I had been on it for a number of times over the 2.5 years. I reduced my usage to 100 or 50mg dosages over 5 days to avoid the effects of coming off it which were rough at times. Over longer periods month or two I would be 50 or 30mg/day.

My PRCA had gone into remission after 2.5 years. Not sure why. I was on 5mg/day of Prednisone & Exjade. No blood transfusions since mid November.

Just enjoying the break from 2 pints per week. One week in November I had a big crash & received 8 pints in an eight day period. Its been smooth sailing since then.

My story ...

No bones broken. Alott happened. For three months prior Aug 15, Sept 15 & Oct 15 I received cyclophosphmide by IV. With each treatment I took 5 days of prednisone... 100mg for 2 days & 50mg for 3 days. After three months there was no response. ( I had previously been on prednisone 30mg for 6 weeks in June to mid July with no response).

At the end of October I had a root canal done. The tooth had died & there was some infection but no pain. I was on erythromycin for 10 days. (in sept I had been on penicillin V for 10days for a tooth ache.

I had blood transfusions to get my blood up to 80 (wanted 100) but couldn't get it higher prior to a vacation.

I went to Maui for 1 week. I got back Nov 3 & my hemoglobin was at 50?? Critical. I went into emergency & got 3 pints of blood ... 2 more mid week & 3 more weeks end. My reticulocite count ranged from 0 - 5 over the last 6 months and I was getting 2 pints of blood per week. I was extremely healthy prior to getting PRCA idiopathic.

Nov 15 I took 100mg prednisone for 2 days ... My retic went from 0 to 50 & was over 100 in 4 days. I was on 50mg prednisone for 3 days & dropped 5 mg per every 3 days down to 5mg per day. My retic stabilized at 50 & my Hemo rose to 140 ( low normal).

Ps I will be 62 in April 2013.

Ps 2 ... My theory is eating lots of salads, less red meat, beer & coffee and drinking lots of water has helped the prednisone (and combination of other events) to effectively restart my immune system. Lots of questions & theories? Was there a bacteria, infection or cancer lurking that was killed off by all the treatments? Or was it alot to do with getting 8 pints of blood in one week? Or partying on prednisone for an extra kick? I am feeling or healthy and enjoying Gkids.

Ps 3 ... I am suspicious that mine was caused by an undetected parvo.

I hope this helps,

Terry

[gina66]

Triumphe64 and Terry,

Thanks for responses. I was reading Dr. Neal Young's article about Parvovirus, quote " relapses of anemia may require repeated administration of immune globulin". I hope to get an appointment with Dr. Neal Young. Would they provide the treatment there or they would make recommendations of tx to my Doctors?

[triumphe64]

I've never been there. Maybe someone will read this who knows that answer.

[Sally C]

Hi Gina 66,
My husband has been a patient at NIH in Bethesda where Dr. Young is since March, 2009. Unless I'm mistaken they only take patients who qualify to be part of a clinical trial. If accepted, any treatment done there is free to the patient. Along the way they have given his hematologist instructions on what they needed for their research along with regular visits to Bethesda.
I think I'm correct in saying that patients don't just make appointments - they have to be screened to see if they qualify.
Our hematologist was the one who convinced them to take Don as his case was so unusual she had not been able to get a firm diagnosis for 6 months.
Good luck!
Sally