Wednesday, July 27, 2016

A Towering City Of Hope - Health Update

I wanted to start off this post by simply thanking everyone for their uplifting support these last few weeks. Obviously I have some difficult decisions coming up, and knowing I have an army behind me makes it a bit easier! So thank you to everyone of my readers, family member, and friends!

My body is really starting to feel the lack of immune suppression. I have been off Cytoxan about two and a half months now, and at a very low dose of Prednisone, making my body a breeding ground for inflammation and disease. I can feel myself becoming more hunched from pain, feel the growing stiffness in my joints, and feel the ocean of fatigue hit like a tidal wave. I haven't felt this poorly in a very long time. My world is caving in once again and it's surrounding me in a thick fog. I am becoming increasingly forgetful, less active, and less motivated due to the pain and shortness of breathe that comes with just about everything I do.
My Scleroderma Specialist prescribed a immunosupressive drug to hold me over until my looming Stem Cell Transplant, but unfortunately I was only able to take it a few days due to severe side effects. The drug caused unimaginable bone pain in my shoulders, and consistent diarrhea that just couldn't be lived with. I saw my Primary Care since I cannot contact my Specialist at this time due to his schedule, and she suggested that for the moment I increase my Prednisone to reduce a bit of the inflammation. A devastating, but useful suggestion. I have worked for over a year to decrease my Prednisone, and in a simple day I lost all my heard work.

I have also been working closely with my Digestive Disease specialist due to frequent episodes of aspiration. I saw her this week and she suggested I take nothing by mouth, not even water, until I can preform an aspiration assessment at her hospital. Luckily, I am scheduled for next week so the wait wont be terribly long! My mouth is so dry -- I don't know if I'll make it to next week though lol ;) I am trying to be good, but darn! not drinking water is difficult when you have Sjogrens!
This last week I also had a Stress Induced Echocardiogram which basically just takes a look at if my hearts electrical system functions correctly with physical activity. My heart actually did great, but my lungs -- not so much! After a few minutes on the treadmill my poor oxygen saturation just kept creeping lower and lower. We ended up having to stop the test at about 6 minutes because I just couldn't breathe. Overall, it seems my body is pretty much exactly where I was before Cytoxan, which was not a good place. Last year I was a wreck, and in tears at almost every appointment begging the healthcare system to do something instead of slowly watching my organs fail! Cytoxan was the magic bullet that gave me a taste of life again -- it just didn't stick. That is what is so special about a Stem Cell Transplant though, the procedure sticks!

Speaking of Stem Cell Transplants -- I owe you all an update! Ever since my Specialist uttered the words I knew it was time to start looking as my body likes to tank fast, and hard. I took to the internet this past week to review criteria and locations for the transplant. There are two trials in the country that run the HSCT on Scleroderma patients, the STAT Trial and The Assist Trial. The STAT Trial is difficult to get into as it has stricter criteria, but is nation wide and preformed at over 8 hospitals. The ASSIST is done solely in Chicago by one physician, and has a more vast criteria with more organ friendly options. Many Scleroderma patients opt to head to Chicago as it is a late stage trial, and has been preformed on hundreds of patients successfully -- the organ friendly transplant is a plus too! I actually seem to fall into the criteria for both trials, so decided to reach out to a few hospitals in the STAT and the hospital in the ASSIST.  I am currently awaiting a response from Chicago, but I did speak with the coordinating nurse who says the lead physician is looking over my intake paperwork and will have a decision on if he wants to see me for an in person consultation soon! Seattle is part of the STAT and preformed at Seattle Cancer Care Alliance.  I contacted the trial since they are so close to my home, and was shocked by the response! After a week of waiting I received an email from the head physician stating he basically thought I was too far gone. The email made it seem like it would be "charity" to even look at my medical record, but I could still send them in. If they look decent enough I could then meet the physicians in person. Well -- that is just not the compassionate response I was hoping to receive. It was actually quite the opposite. I couldn't believe they would feel it was appropriate to make such judgement before even looking at my records or meeting me in person! All I wanted was a consult. Needless to say, I will not put my life in the hands of a team that has that little bedside manner over email. It was truly disappointing.

After that response I emailed the next closest hospital, City of Hope. I received an email back in record time -- one hour! The physician was extremely kind and seemed genuinely excited that I had emailed him and shown interest in the trial! We emailed back and forth for a good few hours as I explained to him my situation. By our last email he asked if I could fly out to California and meet him in person! YOU GUYS I HAVE A CONSULT! I am so ecstatic I can hardly contain myself! His team was super fast and called me the very next morning to set up patient registration, and take my basic information for our initial appointment. They are taking care of getting my basic medical records, and I am bringing along all I have to the appointment. Their goal is take make care and treatment as easy on the patient as possible -- and apparently that starts before you  even see a physician! I think I am in love! lol Now, a consult DOESN'T mean I am accepted into the trial, it simply means the researching physician wants to talk about my case in person, and look me over to see if I qualify. I will receive a call Tuesday August 2nd with my appointment date. From our conversation it sounds like he wants it to be as soon as possible! This hospital is definitely my fist choice due to its close proximity to my state, and the obvious understanding that a patient is a person. It will cut travel costs and the length of flights drastically than if I were to go to Chicago. Unfortunately, any physician who is out of state isn't "in network" so my insurance coverage isn't wonderful but, they are contracted with the doctor! So my current need is to come up with the money for travel, and the initial appointment which wont be covered by insurance at all. I have decided to start an online fundraiser to assist with the costs of out of state consultations, and then, if he says yes to the procedure, I will immediately raise the goal of the fundraiser to match the cost of the Transplant! Link to fundraiser found here, or click the Widget on my sidebar!

I will be sure to keep everyone up to date as the next few weeks unfold. Be sure to follow my Instagram and Facebook for photos and updates as well! I love to document my health journey so I am sure you all will get some fun pictures of California! Hugs!

Wednesday, July 13, 2016

The Future Of My Treatment -- Health Update

I know it's been a really ling time since I posted an update. Things have been a bit rocky and I just haven't been able to write about it all until I felt I had some sort of outcome for my readers, and family. I cannot thank everyone enough for being so invested in my care; it means the world to me to have such an amazing support group from all over the world. I got some big news this week... which I know none of you were hoping to hear but, it is the reality of aggressive autoimmune disease.
As you know I was pulled off Cytoxan last month due to severe side effects. It had damaged my bone marrow enough that I was not adequately producing WBC's leaving me Nutropenic more often than not. I am actually still quite low in my ANC, WBCs, and platelets almost two months after the initial decline. I also became very ill after my last few infusions, to the point where I was hospitalized after each round. Of course this couldn't continue so we halted the medication; after missing only one round of this life saving drug my body has started to fall apart.

I was scheduled for a Pulmonary Function Test last week which showed a 25% decrease in my DLCO (the ability to transfer oxygen into my blood stream). This is an indicator that my lung disease, Pulmonary Fibrosis, is active once again. My joints and skin have been on the decline as well, with swelling and tightness now being a daily occurrence. Unfortunately, my GI tract has taken a huge blow as well. I have begun to choke on food again as it becomes lodged in my esophagus, as well as apirating a good portion of my liquids into my lungs.

I met with my Scleroderma Specialist from UCLA today who was surprised to see such a decrease in health over the short span of a month I have been off chemotherapy. He expressed his worry that my illness is continuing to progress and that it was time to start thinking about the future of my treatment. With the recent developments he wants to start talking Stem Cell Transplants, formally known as Bone Marrow Transplants. This procedure is a long, complicated, and expensive treatment where you receive extremely high dose chemotherapy over a few day period, then receiving life saving healthy stem cells that will graft to your bone marrow -- creating a new immune system. While the treatment is considered experimental in autoimmune disease, it has already proven to be the most useful, life saving treatment available; however, the victory of remission from your disease is only if you live through the transplant. Many weak autoimmune patients will pass away during the process due to organ rupture, and septic shock. You can see why agreeing to this procedure is an extremely difficult decision.

Since The HSCT is technically an experimental clinical trial it is not preformed everywhere. You must fit specific criteria, at specific hospitals,written by specific physicians. You have to be just the right amount of sick - essentially dying, but not too far gone. It's a very delicate balance. Luckily, there is a trial here in Seattle, but it's run by a physician who I do not trust. The only hope for a Seattle transplant where I would feel comfortable is to get another physician involved which is unlikely. I could however, have the transplant preformed in California, it would just add travel to my already non existent funds.
Did I mention the experimental transplants can be over 100,000 dollars? Yeah...
So, the game plan for now is to try one last line of defense, an immunosuppresant called Tacrolimus, and for my physician to speak to a few of the transplant teams here in Seattle until I see him again in September. The drug is nothing special, and is actually quite dangerous for the kidney, but there really inst any drug stronger than Cytoxan -- and I have already failed the rest, so that's pretty much my only option. If my lung function continues to drop we will start the process of consulting with transplant teams in the area. We already know I qualify the basic criteria, it will just be getting a doctor who is willing to accept my case.

I know many of you are wondering how I am taking this decision emotionally. To be honest, I am not going to pay it much thought until I see my physician again in September. Why worry about a decision that isn't 100% set in stone? I have always figured a HSTC would be in my future however, so I have already given it a lot of thought, and accepted it as a part of my treatment plan. Yes, I am obviously worried about the mortality rate, but I have also seen this treatment work miracles -- and I am more than ready for my miracle! My decision will come down to two very important factors: location and fundraising. I will keep you all updated as the situation unfolds. I have a few more tests this week and next to hopefully get a good idea at where else the Cytoxan has worn off.

Don't forget to follow along on Facebook for images you wont find here on my blog! I created a Facebook page last month and completely forgot to announce it formally. It pretty much just has stuff I wouldn't post here -- such as daily photos. Head over and take a look! As an unrelated side note -- who is attending the Scleroderma National Conference in New Orleans this year?! If you would like to meet me, I will be speaking on Saturday and would love to connect with my fellow patients/readers!