I have shared this story on two different websites now, so I thought I would share it here. It is on Beautiful Beyond The Pain and on the Atypical HUS website.
I would like to share my story and to keep record so that I can reference it in the future if need be.
Please ask any questions you may have. This has been a VERY scary time for myself and my family.
My health story September 2014 to current
Early in September, I started not feeling
well. I was tired all the time, dizzy, getting headaches, brain fog, dark
urine, and overall not feeling well. I kept telling myself that I needed to go
have blood work done, but I kept putting it off.
Sunday, Sept 6th, I knew something
wasn’t right. So off to the ER we went. After waiting forever to see a doctor.
I had blood work drawn, got one bag of IV saline and was released with
“dehydration” and told my lab work was normal. I asked for a copy of the lab
work on the way out. Once I was in the car, I looked over the blood work. It
was far from normal. So I figured I would check with my GI and make an appt
soon. This was at a small ER that I thought I could trust, but not the one my
specialists were at. Just closer to home.
Monday and Tuesday of that week, I was
getting worse and assumed that I had a stomach virus because I was now vomiting
as well as now having bloody noses and worse headaches than before.
I worked through it on Monday and Tuesday.
Wednesday, there was no way I could even get out of bed. So I called in sick to
work on Wednesday and Thursday. Thursday, Ben made me go to the ER were my
specialists are. He knew something was bad wrong, but we weren’t sure what.
We get to the ER, I am projectile vomiting in
the room. Blood pressure and heart rate shooting through the roof. Nose
bleeding and the headaches were so bad that I could barely breathe through
The ER doctor recognizes me from my many
visits due to Crohn’s and immediately knows something isn’t right. I am yellow.
My skin, my eyes, etc. She orders lab work and heart monitors and does it on a
stat. She also asks to see the lab work from the other ER. This is where the
problem comes in. The previous ER only ordered simple, basic lab work even
though they were advised I was on chemotherapy medicine. I needed the extensive
Back to Plano Presby. The doctor is pacing
and waiting for the lab work. She is already working on admitting me, as she
knows something is bad wrong. This is about where I black out and don’t
remember anything for 7 days.
The next 7 days, I relive through what Ben,
my mom, and other family has shared. It was bad. I was in acute liver and
kidney failure. My platelets were low. My blood counts were all low. Extreme
migraines. Something bad was wrong. Something very bad. I was near death many
times during these 7-10 days. I started receiving blood transfusions. We were
later told by multiple doctors, that had Ben not gotten me to the hospital when
he did, I would have been dead within 24 hours.
Fast forward to 7 days later (Sept 18), I
have a bone marrow biopsy, kidney biopsy, and draining of the lining of the
right lung due to fluid. When the kidney biopsy comes back on day 8, it’s not
good. There are clots in my kidneys. It is immediately determined that I must
be treated for TTP, or Thrombotic thrombocytopenic purpura.
This is a rare blood disorder that causes
small blood clots in various organs in the body. The testing is very extensive
and requires 14-21 days for confirmation. We didn’t have time for that. If TTP
isn’t treated immediately, it will kill you. That night, Sept 19, the OR team
was called back in and into my room to do an emergency central line in the
jugular vein of my neck. I was given a local (lidocaine) and surgery was done
while I was awake. It was a bit traumatizing.
The next morning, Saturday Sept 20 at about
4am, I started treatment for TTP. The treatment for TTP is plasmaphoresis, or
plasma exchange. Basically, this is where your blood is pulled out of your body,
filtered, and your plasma is spun out as it is bad. As this is done, they spin
donor plasma that is clean back into your body. So out with your bad plasma and
in with new donor plasma. This transfusion process takes 4-6 hours to complete
and is done through the central line in the neck.
I did this process every day for 5 days. My
platelets started coming back up, I was still getting blood transfusions, but
things were starting to look up. My liver and kidney functions were improving.
I was feeling a million times better.
Sept 25, it was determined by all specialists
that I was ok to go home and to check in for blood work several times a week
with all specialists. I was doing ok. I was on new meds. I was relaxing at
home. And checking in with my doctors for multiple appointments. I was going to
This is where things take a spin!!! Nothing
is normal with my body. Just when they think things are figured out, my body
does a tail spin and puts all the specialists in shock again.
Friday, October 10, I was feeling weird
again. I had gotten my lab work back that day and my platelets had totally
crashed. So the renal doctor suggested I meet her at the hospital for direct
admit, just in case.
When I got to the ER, the ER doctor was
waiting for me at the door. He had already been educated on TTP and was told to
get me in a room ASAP. So that he did. I was admitted. My renal doctor finally
comes in and tells me that they had just received the final results for the TTP
tests done in September. The kicker? My tests came back negative for TTP. We
were back to square one.
I was in the hospital until Sunday, October
12th, then released to go home and rest. Not do anything except go
to specialist appointments.
Hold onto your pants! This is where the spin
Wednesday, October 15, I go to an appointment
with my mom at the hematologist/oncology office. I had a bad feeling about the
appointment. You see, Dr Le and I had not seen eye to eye many times and we had
rubbed each other the wrong way more than once. She is very abrasive and we all
know that I don’t do abrasive well.
This appointment, she came in the room and I
could immediately tell she was different.
She sat down and explained that she had been
racking her brain since I was in the hospital originally as she wasn’t
comfortable with the TTP diagnosis, but she had not been able to pinpoint what
was wrong with me. In a sleepless night, it hit her. I needed to be tested for
Atypical HUS, or Atypical Hemolytic Uremic Syndrome.
So, she got together with her specialists
buddies at a conference that Tuesday before my appt and presented my case to
them. She wanted to know what they thought. It was a unanimous vote between
them that I more than likely have aHUS.
Atypical HUS is scary stuff. It’s not for the
light hearted to read, for sure. My hematologist had never diagnosed it before,
nor had her mentors. There are only 300ish cases in the USA with the diagnosis.
Most people with aHUS go undiagnosed because it is so rare and they die from
end stage renal disease. I got blood
drawn for the genetic testing for aHUS on Thursday, October 16.
Because I have been in/out of hospitals, doctor’s
offices, etc…..I have no veins left. They are all blown and not useful. It was
taking 3-10 tries to get a vein for an IV or even a blood draw.
On Thursday, October 23, I underwent surgery
to have a medi port installed in my chest. This will give them one shot access
for IVs and blood draws.
On Friday, October 24, the genetic testing
was returned. I tested positive for Atypical HUS. We needed to get the ball
rolling on treatment, ASAP.
Atypical HUS is a genetic mutation. I have
had this my whole life. Currently we believe that the trigger factor that
brought it out was the chemotherapy drug that I was taking for another illness
I have called Crohn’s Disease. My body just couldn’t handle the medication and
this triggered the aHUS to come to surface.
There is no cure for aHUS. There is currently
only one treatment for it. It is IV infusions once a week for 5-6 weeks, and
then every other week indefinitely. This drug is called Soliris. It’s pretty
new to aHUS, so the side effects aren’t well known.
On Monday, October 27, I got vaccinated for
Meningitis, Pneumonia, and Tetanus. I also started very strong antibiotics to
protect me so that I could start the treatment before the vaccines fully
Wednesday, October 29, I started Soliris
treatment for aHUS.
While I would like to be very hopeful (and I
am trying to be), the rare-ness of this disease frightens the hell out of me.
The new drug is scary. Not knowing side effects are scary. Not knowing what my
future holds for this disease is scary.
This is my story to present date.
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