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I was diagnosed with AA in November 2012 and at the time had a 16 month old and another daughter who had just turned 3. Due to the risks associated with a BMT (and with no sibling match) I opted for horseATG and had this in November 2012 and responded very well, my counts recovered to completely normal within about 3 months!! I remained on cyclosporine and my doctors implemented a slow taper. My life pretty much returned to normal and I acted like I had beaten AA throwing myself back into work etc etc. By January 2015 I was down to taking 100mg of cyclosporine per day but unfortunately I had been noticing a downward trend with my platelets. My doctors were hopeful that the counts would not all fall and that things would stabilise. Unfortunately by March/April 2015 all 3 blood lines were falling. My doctors decided to increase cyclosporine and it did not work all it did was cost us time really as we had to give the increased cyclosporine about 6 weeks before we could determine whether it worked but honestly I think we had to give it a go. So in May of 2015 I decided to opt for a second round of ATG this time rabbit. For me, with 2 children aged just 4 and 5 at the time, I found the thought of a BMT still too risky (I am currently 39 so still not considered old for AA but not necessarily young). I met with the BMT team and they were very frank with me. They suggested that I would respond to the second round of ATG but that my response may not be as good as it was the first time round but the very fact that I responded well the first time was a good indicator that I would respond well again so with mortality risks of about 20 to 30% with a BMT we all agreed that I should try the ATG again. So how did I respond to the second round ...... very very very slowly. It was tough because I had the first round of ATG to compare it to so while my counts improved quickly post the sound round of ATG such that I did not require transfusions ..... they really only responded to a level that just had me above the level where transfusions are needed. So I read and I read about response times ..... most papers suggest if you don't see a response in 3 to 6 months then you are unlikely to see a response .... but some do suggest you give it longer. I then stumbled upon someone who had not responded until 9 months post and this gave me hope as this person was actually requiring transfusions for the full 9 months and then suddenly started to respond. So in many ways I was a lucky person as my levels were "safe" in the sense that I did not need transfusions they just weren't getting any higher. Around 8 months post completing the second round of ATG I started to see a more positive trend with my counts and right now (almost 12 months to the date that I started rabbit ATG) my platelets are nearly normal!!! my haemoglobin is OK, it is around 11 but my WBC is still low at around 2 .... so yes that does worry me.
SO what about PNH ..... well like your wife I started this journey with a very small PNH clone .... less than 1% ...... and my doctors were like it is so small that if we tested everyone in the population we could find that a large percentage of people have a clone of a similar size so I tried not to let the PNH concern me. They told me it was a "positive sign" as typically people with a PNH clone respond well to IST ..... and I guess i have proven that to be the case ..... however the question you are probably wanting to really know the answer to is has my PNH clone increased in size and unfortunately the answer is YES. Post my first round of ATG my PNH clone increased in size (to about 3.5%) but then it reduced in size when things were going well and around the time I relapsed it started increasing in size again so I was not too concerned as the doctors still considered it very small. BUT post my second round of ATG my PNH has really worried me ... so having been less than 1% when this all kicked off it increased to around 15%.... we were checking it every month post ATG and every month we were seeing a real increase. It has not been checked for about 6 months now so I am hoping that given I have started to respond that it is also decreasing in size. I have read a number of papers and the majority of papers I have read suggest that in about 1 in 8 cases ATG can cause PNH to increase in size but typically the increase is for it to only double in size ..... but clearly in my case it more than doubled. These papers however do suggest that in the majority of cases where the PNH does increase in size that with time the increase does slow and then reverse but potentially not back to the levels it was..... so I agree that you should have some reservations about the way the PNH clone may respond but whether this is reason enough to avoid a second round of IST I don't know. Clearly in my case I decided the risks associated with un-related BMT were too high. Whether I have made the right decision I can only hope that I have. I just continue to tell myself that for every year I remain alive and reasonably healthy is another year that modern medicine has to learn more about the disease and improve the means in which they treat the disease. I have tried to surround myself with positive stories and it does seem that there are a number of people out there who simply needed a second round of IST to beat this "bloody" disease and I hope that I am one of those people who has now beaten this disease. I do however believe that I will be taking cyclosporine for a very long time but hopefully the dose will be able to be reduced over time.
I wish you and your wife all the very best and if you have any questions please let me know.
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