After the ATG - what happens next

[TonyBegg]

Sorry if this already answered somewhere. Karin has 95% PNH clone and severe AA. Today is 4th and last day of horse ATG and we are wondering what the next months look like. She will continue the cylosporine for up to 6 months. She also started Eculizumab last week. Her WBC are now virtually zero and she is on ant-viral, antibiotics, anti-fungal in the hospital. Will the cyclosporine keep the WBC down and will she need all the aniti-this and thats while she is on it? Presumably at some point her bone marrow reboots. We know the docs will answer some of these questions on discharge (scheduled for Sunday) but maybe your experience can help us frame the questions. Any help appreciated. Thanks!

[Amz904]

Hey TonyBegg,

I have severe AA and was treated with hATG and cyclosprine back in January 2014. Everyone reacts differently to the treatment, some will respond within weeks while for others it can take months.

In my case when I got treatment, my WBC was "less than 0.1" and my ANC was nothing. By the end of Feb was when I saw any improvement (even though it was slight) with my WBC (it was officially 0.1 with an ANC of 0.02) Over the next few months it continued to slowly rise, as of my latest labs today my WBC is 1.1 and ANC is around 0.6.

In regards to all the antibiotics/anti-viral/anti-fungal, I've been on all three since my diagnosis in December 2013, and still remain on them. My doctor wants me to continue with them until my ANC is closer to 1.0; however every doctor is different. I've heard of some patients on none except anti-fungal, others who were on all of the above but taken off at an ANC of 0.5 or even 0.2.

Good luck!

[TonyBegg]

Thank you so much Amz904. Very helpful. Am glad to see your counts are making good progress. I wish you a full and speedy recovery. Does your cyclosporine smell like skunk? Anyway, it appears that the cyclosporine does not keep the WBCs and neuts low and at least they can start to increase if the marrow has the oomph. Very useful response. Thanks again.

[Ruth Cuadra]

Originally Posted by TonyBegg

Does your cyclosporine smell like skunk?

TonyBegg, you're doing a great thing by thinking ahead about what questions you'll have for the doctors when your wife is released from the hospital! Just wanted to jump in about the smell of cyclosporine (skunk is right!), which I found pretty awful when I was taking it. If your wife is taking the liquid form, I suggest mixing it with something strongly flavored like chocolate milk or orange juice to mask the smell and the taste. If she has the capsule form, she can open the capsules and let them air out for a while (maybe 30-60 minutes) to let the worst of the smell dissipate before she brings them near her mouth. Eventually, I got used to the smell and just popped the capsules in my mouth, trying not to think too much about it.

Regards,
Ruth

[Amz904]

TonyBegg,

The cyclosporine that I would receive in the hospital had the worst smell! They always gave different brands depending on what the hospital pharmacy had in stock, so some were worse then others, and the smell would linger around for a while. Luckily, the kind I get at my local CVS pharmacy at home has little to no smell! This makes it way easier for me to handle. The type I get comes in a box and says: "cyclosporine capsules, USP [modified]" and is by Watson Pharma, Inc.

From what I understood from my doctor, the cyclosporine just helps to continue to suppress the t-cells (which is whats attacking the bone marrow in the first place) Since the ATG only stays in the system for roughly 3 months, most remain on cyclosporine for a while after that to continue to suppress the t-cells to allow the bone marrow the time to recover.

You mentioned only being on cyclosporine for up to 6 months, i'm not sure if the case is different in PNH since I'm only really familiar with AA, but doctors tend to be very cautious when it comes to tapering the dose and people tend to remain on a low dose of cyclosporine for years sometimes. A quick taper has sometimes lead to a drop in counts, requiring to raise the dose again or in some cases repeat therapy. So this is something you may want to look into!