What's the difference?

I have had several people talk to me about my health lately and have said "I can't believe Crohn's gave you aHUS"....or "I can't believe you had to get your thyroid removed due to aHUS." 

The above are not the case AT ALL. So I wanted to take a few minutes to tell you the differences in these and my health history with the 3 of them. 

Let me first start this by saying, ALL THREE DISEASES ARE DIFFERENT!

Crohn's Disease - diagnosed 2012. 

https://www.nlm.nih.gov/medlineplus/crohnsdisease.html

Crohn's disease causes inflammation of the digestive system. It is one of a group of diseases called inflammatory bowel disease. Crohn's can affect any area from the mouth to the anus. It often affects the lower part of the small intestine called the ileum.

When I was diagnosed with Crohn's Disease, I had been ignoring it for a very long time. It wasn't until I had bleeding for more than a week before I contacted a GI and ended up at the ER. 

Because of this, I had a lot of scar tissue in the small intestine, which caused narrowing in the intestine. I got MANY obstructions.

In 2013, I underwent a lower right colectomy. This basically means they removed about 18 inches of my small intestine, my ileocecal valve, my appendix, and a few inches of my large intestine. 

I do still have some issues with my Crohn's, but for the most part....I follow the diet I know works for me and I keep up with my symptoms and see my GI regularly.

This is something that has no cure. 

Grave's Disease (autoimmune hyper thyroidism) - diagnosed 2013.

https://www.nlm.nih.gov/medlineplus/ency/article/000358.htm

Graves disease is the most common cause of hyperthyroidism. It is due to an abnormal immune system response that causes the thyroid gland to produce too much thyroid hormone. Graves disease is most common in women over age 20. But the disorder can occur at any age and can affect men as well.

After having crazy moments (literally), high heart rates, hot flashes, hair loss, drastic weight loss, major swelling on my neck; I went and got checked out. After multiple blood tests, it was confirmed that I had Grave's Disease. 

For 2 years, I tried the medications. I failed the medications. My Grave's was not treatable with medication. So June 2015, I had a total thyroidectomy. Since, I am on medication because I now have no thyroid to produce the hormones needed.

atypical Hemolytic Uremic Syndrome (aHUS) - diagnosed October 2014.

http://www.ncbi.nlm.nih.gov/books/NBK1367/

Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome of hemolysis, thrombocytopenia, and renal insufficiency. Genetic mutations in the alternate pathway of complement are well recognized as the cause in more than 60% of patients affected by this thrombotic microangiopathy.

September 2014, I was on a medication for Crohn's (a low dose chemo drug) and had liver failure from it. Because of this, it triggered the genetic mutation I didn't know I had, called aHUS. This put me in kidney failure. Caused my platelets to crash. Blood counts dropped, etc. 

I currently get treatment for aHUS every 10 days via IV. This is something that has no cure. 


So, please take note that while there are some similarities in my diseases, they are not causing each other. They are all very different and are treated very differently. 

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 6^th, 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. 

http://www.nhlbi.nih.gov/health/health-topics/topics/ttp/

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 12^th, 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.

http://ghr.nlm.nih.gov/condition/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. 

Why I chose a total thyroidectomy over RAI treatment

This topic is something that I have been thinking about posting for a while, but have kept it to myself. Mainly, because its a hot topic for me and it really gets under my skin.

I want to preface this with a little background about me. Most of you that have known me or read my blog for any amount of time, know that my health is at best....not good.

• I have severe Crohn's disease (diagnosed May 2012)
• I have/had aggressive Grave's disease (autoimmune HYPERthryroidism) that wasn't responsive to medications. (diagnosed February 2014)
• I have a very ultra rare disease called atypical Hemolytic Uremic Syndrome. (diagnosed September 2014)

I've been through multiple surgeries/procedures/etc for the last 3+ years due to these illnesses.

I have my people that will advocate for me. But I have also learned to be my OWN advocate.

In doing so, I have read anything/everything that I am able to get my hands/eyes on in relation to my diseases. I pride myself in learning about them and being able to educate others on them when needed.

And trust me, with an ultra rare disease (and the others as well) I have had to educate even regular doctors and specialists on my disease.

I don't claim to be in the medical field or be an expert on it all. I don't claim to know EVERYTHING there is to know. But if I don't know I will research the hell out of it and ask questions to the appropriate channels.

So, this brings me to my topic: why I choose a total thyroidectomy surgery (total removal of the thyroid) over RAI, or radioactive iodine treatment.

I researched each of these very closely. I reviewed the pros and cons of each. I asked around for personal opinions. I seeked medical advise.

One thing I will tell you is that "most" of my doctors suggested RAI treatment so that I wouldn't have to go through another round of anesthesia and another surgery.

I got 2nd opinions. I did my research. I learned the ins and outs of both.

When I was in the testing phases of the thyroid disease. I had to do an uptake scan. I thought to myself, this will be no big deal. I will go in, swallow a pill, go back later for a series of scans. I've done a million (that's an exaggeration, I've really only done 8,427 ha!) other tests in my health history and this one appeared to be easy.

And it was. That is until I got to the radiation room at the hospital to get this tiny pill. Then I became a little terrified.

I was taken into a room for radioactive materials. Ok, no big deal. X-ray room. No. I sat in this room with my husband. Finally a radiologist appears. He is in full gear. Hazmat outfit, gowned head to toe with gloves and shoe covers and a mask. I start to panic. He goes into the back room to get this "special pill". He comes out with a metal tube. He opens it. pulls out another metal tube. Opens it. Pulls out a medicine bottle. opens that and pours the pill in my hand and says...."here, take a drink and swallow the pill. Then dispose of the cup in the radioactive container before you leave."
um what the F? but I obliged and did it. I was seriously scared of what was going to happen with this pill.

Turns out. It's a very tiny dose of radioactive iodine. Blah. I went back the next day for the scans. My thyroid absorbed 93% of the radioactive iodine. This isn't good. This indicates high hyper-thyroid disease.

Then I started wondering, what happens to the remaining percentage of the radioactive iodine that my thyroid didn't absorb. What effects could this possibly have on me?

So I was back to doing my research. 

When you are given RAI treatment ultimately, it absorbs high doses of it in the thyroid and this kills the thyroid. You are quarantined from children and pets for a great amount of time due to the radiation. You can't use the same bathroom as others for a while. You must flush twice in the restroom, etc. 
Once your thyroid is dead,  you are considered hypo thyroid and will take meds for that to level you out.

So, again, I questioned what would happen to the RAI that my thyroid didn't absorb? It has to go somewhere right? 

I started asking questions to my specialists and I learned a lot. Most people with RAI, their body absorbs it and they are fine. Some expel it and are fine. 

Others have future risks. Like cancer later in life due to RAI,  which can include bladder, breast, central nervous system, colon and rectum, digestive tract, stomach, pancreas, kidneys, lungs, melonoma of the skin.  

This is where I stopped. I have Crohn's disease which carries a higher risk of cancer to the colon/digestive tract. I also have family history to stomach and pancreatic cancer.

Do you see where I am coming from when I say that RAI Treatment was not an option for me? 

To me, this was a no brainer due to my health history and potential health issues in the future. 

I went in, had my total thyroid removed. Spent the night at the hospital, had the drained pulled the next day and went home. 1 week later I was back at work and starting the thyroid replacement medications.

Yes, I had ANOTHER surgery. Yes, I lost a very important gland that our body needs. Yes, recovery (mainly just the voice issues) haven't been a blast. Yes, any surgery is risky. 

I get it. 

Does this make me deserve to be questioned about my decision for MY health? Or does this justify me literally being told that I am stupid for choosing a total thyroidectomy over RAI treatment?

If you have thyroid issues and surgery/RAI treatment have been mentioned, I highly recommend that you do your research. You ask questions. You do what is best for YOU.

I am adding a few links to this that have been very beneficial for me during this process. Please read them.
Join the FB groups and visit the website for Stop the Thyroid Madness. Ask questions to patients, specialists. 

I am always open to answer any questions that I can. 

But please remember, I am not a medical professional. I will answer questions to the best of my knowledge and research. If I don't know the answer, I will refer you to a place or person that does.

Please also note, the above only pertains to my situation and health history. What you choose for yourself is a very personal opinion and one I will not ever judge you for. Please give me that same respect.

http://www.stopthethyroidmadness.com/rai/

http://www.ncbi.nlm.nih.gov/pubmed/19281429

What she said was exactly what I needed to hear today.

Every time it is time for an infusion, I get a "this f*cking sucks! why is this my life?" attitude. I hate going, I hate the side effects, I hate getting an IV in my chest, I hate the unknown of the disease, I hate the unknown of the drug. I hate everything about this disease and what comes with it.

Now, I know you are thinking, this is going to be a "woe is me" post. You're wrong. :)

Yes, I have crappy stuff that happens with atypical HUS. Yes, I have a non-curable, extremely rare disease. Yes, I was in multi organ failure 8 months ago. Yes, I will have to get treatment at max every 14 days to keep me alive, for the rest of my life. Yes, it sucks. 

I won't lie about those parts.

But what I would like to talk about today is the support groups, the people you meet (in person and via the internet). The people that help you see that you are not alone.

Now, I do have other diseases and I have friends with the same. I'm on support groups for those 2 as well. They are so very helpful and understanding. And I've made some amazing friendships through them.

However, this time, I am discussing the people I have met since being diagnosed with atypical hemolytic uremic syndrome. 

Like I said above, I am normally pretty irritated and down when infusion time comes around.

I get my infusions at an oncologist/hematologist's office. They have an infusion room in the back that has about 8-10 recliners where people are getting pumped full of IV drugs. Some people that have crazy, serious side effects. Some are people that are dying. Some are people that are very, very sad. There are the angry people. There are the VERY happy people.

The happy people are who I try to always sit near. Since getting treatment anywhere from every 7 to 14 days for the last 7 months, I have gotten to know a lot of the "regulars" there. Most of the people have cancer and are there for chemotherapy that will make them very ill.

I have seen people have bad reactions to the drugs, people vomit, etc. This type of place isn't for the light hearted. 

But today, I noticed something different. I started talking to another regular there. We have met many times and always have a few laughs together. She is completely bald. And has had cancer for THREE years. She's been through almost every type of chemo there is. 

She is positive. She makes jokes about her cancer, her treatment, her side effects, the fact that she is bald and she makes people laugh. She more than once has stood up and walked over to someone else (while she is hooked up to an IV and pushing an IV pole), just to give them a high five; rub their back when the nausea comes on; just to tell them they will be ok.

Today, after one of the above happened, she was talking another patient through treatment. was telling her just to breath. To picture happy thoughts. To pray, even if outloud. I've seen her pray over them.

Once she got back to her seat, I asked her, "how do you do this?" "how do you not bust out crying with these people?" "how do you stay strong when you are going through so much?"

Her answer was shocking to me, "you learn more in these type of settings than you will learn from most doctors. You learn that there are others to lean on when you need it. Those that get what you are going through. Those that you pick up while they are down, are the ones that will pick you up when you need it. You have to try to stay positive or these diseases will kill you VERY quickly. The power of prayer and the power of positiveness is the only thing that has gotten me through this for so long. So now I get my treatment and I support others."

I got teary eyed and just said "thank you". What she said was exactly what I needed to hear today. I simply could not do these treatments without my support group. 

Hope this helps others too.