This was unexpected. I have been working with my hematologist since February 2020 to measure my ADAMTS-13 enzyme activity. Good ADAMTS-13 activity means no Thrombotic Thrombocytopenic Purpura (TTP). The blood is sent to another facility, such as the Mayo Clinic, and its takes about a week to get the result. For many the months, the good news was greater than 100%, but in October 2021 it dropped to 78%. I suppose this is bad news, but I am a chronic-TTP patient, so we were expecting a change at sometime. The upside is I did not have TTP. The plan now was to monitor ADAMTS-13 and get rituximab to increase its activity before TTP sets in.
On May 17, 2022, ADAMTS-13 was at 25%, so the doctor
prescribed rituximab with a dose on each Friday in June. In the third week I was
not feeling very well. I had mild symptoms – a stomach-ache, some blurred
vision, numbness in one arm. Since I was taking rituximab which will stop TTP,
I was thinking side affects. On the Thursday night, I had a miserable headache
which kept me up all night. My wife did not tell me, but she thought I had TTP.
I went for the next rituximab dose on the morning of June 17, and advised I was
not feeling too well. My doctor ordered a blood test, and my platelets were at
41, I had TTP again. Later we found my ADAMTS-13 was at 1%.
The same day I had the line put in my neck and we started
plasmapheresis. We did plasma exchange for 6 days straight and 8 in total. The
good news is the original discarded plasma was not dark, it was almost the same
colour as the new plasma. I believe this means I was not suffering all the
consequences of lack of platelets with items such as kidney issues, bruises and
petechia. I recovered from TTP#6 faster than any previous TTP episode. The
conclusion was the two does of rituximab which were provided on the first two
Fridays of June helped for quick response. I was only admitted to the hospital
for 5 days and with night passes only stayed one night in the hospital. This
helped my family have a more normal life and was much better for eating and sleeping.
When I reviewed this TTP episode with my hematologist, she
concluded we waited too long to administer rituximab. Next time, the plan will
be to administer at a higher level of ADAMTS-13 activity.
We were so close, but I did get TTP for the sixth time,
stopped working for 4 weeks and interrupted our personal life where we even missed
my nephew’s wedding.
Thanks, Bruce.
@BruceFightsTTP
