Thursday, September 10, 2015

1 year ago today - aHUS

I am writing this tonight, because tomorrow...I would like to spend in prayer...for our country, for my health, for my family, for my future, for our future.

1 year ago tomorrow, I entered the ER at Plano Presbyterian Hospital. 

On that day, my worst nightmare came to life. On that day, I was almost dead. On that day, my life was FOREVER changed.

I would like to share a blog that I wrote in November of last year. This was when I was finally coherent enough to put my thoughts together about the previous 2-3 months. It was when I was able to semi-write or talk about it without getting physically sick to my stomach and crying. It was when I was starting to feel a little bit better with each treatment.

At the end of the blog post from November 2014, I will share my updates of my atypical Hemolyic Uremic Syndrome as it is today. What treatment I am currently doing. How I am currently feeling. Some pictures from that time in the hospital. Plus a few other thoughts.

So, here it is. The blog post I made in November 2014.

http://everybodysgottagetaway.blogspot.com/2014/11/my-health-story-september-2014-to.html


"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 them.

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 blood work.

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 make it!

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 starts!

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 Power 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 activate.

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."

As you can see, those few months and every day since then have been a whirlwind of treatment, testing, emotions, etc.

aHUS basically rules my life. 

I go to treatment at the hemotolist's office every 14 days. I receive the max dose of Soliris possible at this time, which is 1200mg. If we dip below that, my kidney levels and platelet levels start going crazy.

This type of treatment will continue for the rest of my life. If my levels start getting crazy on this dosage, I will be required to get treatment every 7-10 days, instead of every 14 days.

I am being proactive within the aHUS Foundation, the support groups, etc. I am hoping to spread awareness of this ultra rare disease and basically just do what it takes to survive. 

Life with aHUS is incredibly hard. You see, the treatments give me migraines. I was getting them about 4-5 days a week before we increased the dosage. Now I get them about 1-2 times a week. I never even had headaches before this all started. It's been a rough road. Treatment wears me out. I am almost immediately exhausted, my blood pressure shoots up during treatment. It's not fun getting an IV in your chest. It's mentally and physically draining to go through this. 

With all of that said, I am so thankful to be here still. I am thankful that I do feel a lot better. I am thankful that I have a great team of specialists on my side. I am thankful for the family that hasn't left my side through all of this. I am thankful for the few friends that are there as well. I am thankful to be alive. I am thankful that Soliris is there to help me see good days.

The past year has been a long, hard road for me. BUT I will continue on this path of doing what needs to be done. 

Thank you to everyone that has stayed by my side. Encouraged me. Loved me. And even just listened to me complain.

I am going to share a few pictures of that hospital stay and a few others from the past year.

plasmapheresis - the coolers have donor plasma in them

nurse and plasmapheresis machine

plasmapheresis machine

plasmapheresis machine


Soliris 

me receiving plasmapheresis 

central line that was placed in my neck for plasmapheresis 

blood transfusion

plasmapheresis machine (the yellow bags above are plasma)

me receiving plasmapheresis 


getting Soliris treatment through my Power port

right after surgery to get the Power port placed




 PLEASE DO NOT SHARE MY STORY WITHOUT MY AUTHORIZATION. I DON'T MIND IF IT IS SHARED, I WOULD JUST LIKE TO TRACK IT AND BE NOTIFIED AND TAGGED IN IT. 
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