Twenty Four - Health Update

It was a quiet week in my medical world, a rare commodity I do not often get to enjoy! I had only two appointments, a follow up with my Pulmonologist, and a follow up with my Neuro-Headache Specialist.
The appointment with my Pulm was mostly just to touch base after my UCLA appointment last week, and to go over the results of my Right Heart Catheterization. We spoke briefly about my new treatment plan involving the Cytoxan, which was decided to be done at the hospital my UCLA physician has access to instead of the university. We then went over the results of my RHC. My mean pulmonary pressure was within high normal range. According to this doctor I do not have current evidence of PH which is fabulous!

Next I saw my Neuro-Headache Specialist. This was a follow up after I saw her earlier this year for debilitating headaches as a result of my 22 day hospitalization back in December. There are few doctors I truly feel care about their patients, but she is one of the exceptions! When I am in her office I feel she sees me as a person, an individual with a life, not just a medical statistic. If this physician and my GI could just run all my care I would be one happy patient! lol Last time I saw her she prescribed a drug for my headaches called Cymbalta. This medication has completely changed my life for the better. Since beginning treatment I have not endured any more of the debilitating Occipital Neuralgia relapses. These "headaches" were unlike any pain I have ever felt, leaving me completely helpless to a primal drive due to the crippling pain that clouded my ability to think logically. The feeling of thick hot iron being filled into the back of my skull, crushing whatever person resided in my head no longer leaves me in constant fear thanks to this drug. During this appointment we talked mostly about my progressing autonomic issues, also known as Dysautonomia. With my bodies complete lack of ability to regulate blood pressure, heart rate, temperature, and heart rhythm at any position it looks like we may be battling Pure Autonomic Failure, though we wont know the exact branch of autonomic malfunction until I see my ANS specialist at the end of September. She prescribed a new medication called Midodrine that is suppose to assist the function of the autonomic nervous system since my last medication for this problem prescribed by my Electrophysiologist didn't pan out as we were hoping. After we spoke regarding all my medications and current issues we had a few minutes just to talk about life. She is the only physician that takes the time to ask me about my personal life... and she didn't even try to rush out! She legitimately wanted to talk to me, not just my disease. I feel like a normal young woman in her office, not a medical experiment like I do with many of my other doctors. It's so refreshing! She is also the only physician to ask if she could read my blog which I thought was really cool! (So if  you are reading this: Hi! Thank you for being an awesome doctor that remembers I am a human being, not just an illness!) Overall it was a great appointment and I am very excited to start this new med; we have some worries about it possibly increasing my Raynauds, which would be cause for immediate discontinuation of the drug, but only time will tell!

I don't normally talk about anything else besides my medical journey on this blog, but I thought I would share with my readers that I had a fabulous 24th Birthday! As a young woman who cant eat, and is mostly home bound celebrating can be difficult. Luckily I was feeling relatively well that day, enough to get up, put on some brand new make-up, do my hair, and head out for a day on the town! I started out with Mommy-Daughter Mani-Pedis, then headed to a local juice bar to pump "healthy stuff I would never put in my mouth" through my feeding tube with my awesome best friend who is also a Tubie, and finally got 20% off at my favorite thrift store with my Birthday coupon! Thankfully all my medical equipment behaved relatively well. I crashed hard when I got home! haha Totally worth it! I am feeling so grateful to have made it to my 24th birthday after the crazy year I have had!

How a Tubie does "Birthday Shots" ;)

Right Heart Catheterization Procedure - Health Update

Friday morning was my Right Heart Catheterization procedure at the local University hospital. Bright and early my Mom drove me down town and we checked into the Interventional Cardiology Radiology Unit. The techs got my central line accessed so we could run some blood work prior to the procedure, and also to give me a bit of medication for my nerves! I must admit I was a tad bit nervous for this procedure as I have never had a procedure preformed involving the heart. It was my understanding that I would be under "twilight sedation" which involves the patient being able to breath on their own but also totally asleep; it turns out their version of "twilight sedation" meant totally awake with some anti-anxiety and pain medication! The doctors however assured me that the procedure would be painless, and be over very quickly. They expressed to me the risks (which includes puncturing a lung, yikes!), I signed the consent form, and then they wheeled me back into the cath lab which is much like an operating room; big bright lights, a large screen, walls of supplies, and surgical techs covered in fancy sterile gowns/masks. The first item of business we took care of before I even transferred to the operating table was what music I wanted to listen to over the loud speakers! I love when they let me choose the tunes! (I chose the Shins in case you were curious.) I laid down on the small table in the middle of the room with my head tilted to one side. The tech cleaned my neck and covered my surrounding body parts, including my face, with a sterile sheet, then it was time to begin! The doctor started by injecting a numbing medication called Lidocaine into my neck. After each shot in the neck they ask you if you can feel a poke; it took about a whole bottle and six injections but my neck was finally good an numb! The procedure official began once the doctor made the incision into my jugular vein, inserting a small sheath. This was 100% painless, I didn't even feel pressure! Once the sheath was in place they were able to slip the catheter through and pass it all the way into the heart. Once it reached my heart it gave quite a few flutters; it was a very odd feeling, but again totally painless. The catheter was then passed into the pulmonary arteries, and finally pulled back out with a short hold of my breath. My neck was patched up and I was done, the whole procedure only lasted about 20 minutes!

Headed into the procedure!

The RHC was honestly one of the least painless procedures I have ever endured! The best part was getting to watch the catheter slip in and out of my heart on the large monitor the doctor was using to guide him. I love to see my insides on the big screen! lol ;) The worst part of the whole ordeal was that I was allergic to the dressing they used to keep the gauze on my neck afterwards! I guess I should start bringing hypoallergenic dressings to my procedures and surgeries... It looks a bit like a vampire attacked my neck!
The operating doctor expressed to me that my pulmonary pressures were on the very high side of normal. Pulmonary Hypertension is the number one cause of death among Scleroderma patients so hopefully the results are as close to normal as possible!

Thank you for all your well wishes as my impending chemotherapy draws nearer! Right now it's still in the preliminary phases with scheduling, battling insurance companies, and prior authorizations. I hope to start my first round in September, but it really just depends on how fast my insurance will approve the treatment. This week I only have two appointments to attend which is amazing! So much free time to enjoy the last week of summer, and my birthday on Friday! Both the appointments are follow ups so there wont be anything too crazy happening, I love low key weeks!

Cytoxan: A Bittersweet Victory - Health Update

First let me start off by letting all my readers know that I am free of the hospital, and the lovely kidney infection that brought me there! After a few days of IV antibiotics my pain has subsided and the infection is no longer present in my body. Resting at home is so much more satisfying than in a stuffy hospital! I am so sorry to have left you all hanging after that last post, wondering if I had ever made it home! I have found myself extremely busy and unable to write at all this week. I am so happy to bring you my update today however! I finally feel I am on a productive treatment plan that is sufficient for the degree of progression that is manifesting itself in my current test results. Some very big, very aggressive changes will be made over the next month. This entire next year will honestly be a bit of a transition for me if all goes as planned!

I had the big appointment with my Scleroderma Specialist from UCLA today and it was everything I had hoped for! My specialist took one look at my tests results over the past few months and could immediately point out the obvious advancement of my illness. He was very concerned by the sudden drop in lung functions, and by the recent Vasculitis diagnosis which he believes could be contributing to this recent drop in health status. He was also unhappy with the continuing downward succession of my GI tract, and my failing autonomic nervous system. One of the first new treatment options we decided on was to start weekly Saline Infusions to assist my body absorb electrolytes it desperately needs. This will be an intermediate treatment (that could be subject to change) just to keep me afloat until next month when I see my new Dysautonomia Specialist.
After reviewing my records together we did a physical examination where he also observed the development of further skin tightening. Scleroderma patients do what is called a Rodnan Skin Score which asses the thickening of the skin. Within the three months since I had seen this physician last my score jumped from a 6 to a 10, with tightening now in areas of the body such as the chest where it hadn't previously been. We spoke about the limited options Scleroderma patients have in regards to treatments that will slow, and sometimes even halt progression of disease. Each major intervention that is used to fight progression of this illness I had already been on, leaving one option open to us: Cytoxan. Cytoxan is a harsh chemotherapy drug used as the very last ditch effort in severe autoimmune disease. It is a Nitrogen Mustard class of Alkylating agent which directly damages the DNA. This drug is the typical "chemo" you think of when you hear the word. It sterilizes you, causes you to vomit, and of course makes your hair fall out. I will be starting once a month treatments that will last a year in duration. The doses will start low to prepare my body before we hit it hard on the third/fourth month of treatment. By December, if my body can handle it, I should be up to the full dosing with all the wonderful accompanying side effects. I have basically been told the next year of my life will be hell, but the long term benefits for my disease will be (hopefully) unimaginable! I am going to be honest, there are many opportunities for problems to arise during this treatment besides just the typical chemo side effects. This drug can actually itself cause Cancer due to the harsh damaging effects it has on the body, especially to the bladder and bone marrow. There is also the real possibility of becoming severely leukopenic which can be very dangerous if not taken care of immediately. My blood work will have to be carefully managed through out the next year. While this treatment will be cruel and jarring to my already weak body, it could save my life... Chemo is a bittersweet victory. At this point I am welcoming the opportunity to move forward with this option and be treated for the aggressive disease I have. This physician is not afraid to pull out the big guns and fight for my life... which I greatly appreciate!

This Friday is my Right Heart Catheterization. This is a high risk procedure, but one that is entirely necessary with my recent decline in DLCO. Originally we thought the cardiologist would go through my groin up to the heart, but recently I found out we will be going through the jugular instead. I am not often nervous for procedures, but I must admit I get a bit fluttery when I think of this one! I will more than likely not be up to posting until next week as this procedure will have me down and out for a few days. Hopefully all will go well!

After I escaped from the hospital this weekend my husband drove us up to the North Cascades to visit Lake Diablo. The water is such a surreal blue! I don't often get moments as incredibly serene as this, honestly I don't get many moments where I leave the house! lol So I just thought I would share it with you all! Thank you so much for your continued support and love! I wish I could have my impending chemotherapy infusions here... The outdoors are the best medicine!

The Impatient Inpatient - Health Update

I type to you today from my local hospital. I currently find myself inpatient on a heavy load of IV antibiotics for a severe kidney infection. Last night I was rushed to the ER after my fever spiked. I was shivery, drenched in sweat, and in an unbelievable amount of lower back pain. I was admitted fairly quickly after my blood work and urinalysis was run. I will more than likely be in the hospital a few days since my complex medical history makes doctors quite nervous. They want to triple check that I have no other underlying infection before I am discharged. They took quite a few blood cultures to check for infections of a more systemic nature which take about three days to receive results. Luckily I have a lovely private room with a beautiful view of the sunset, and a nurse who is keeping my pain very well managed!

IV Antibiotics, Fluids, and Tube Feeds

With medical complexities such as mine you end up seeing many. many physicians. On my teams first line of defense I have five doctors that I see monthly, on the second line of defense I have another four doctors I see at least every three - six months, and on my third line of defense I have over ten that I see periodically for issues as they arise. It makes for a very crowded kitchen, and as you all know too many cooks don't make a pleasant meal. There will always be issues that arise between physicians, their separate clinics, and even sometimes their separate ideas for how my care should be managed. This can often lead to much unneeded confusion for the patient, leaving us feeling like a rag doll being pulled in different directions. Amidst all the disarray, there is one constant that we as recipients of healthcare need to remember; our care is exactly that, OUR care. What many patients don't realize is that you hire a medical practitioner, and you alone have the option to find a new one if your current is unsatisfactory; you are never stuck with a provider. I have had to learn his lesson over, and over again! While many times it did FEEL like I was stuck, I always eventually found another doctor who cared about my wellbeing and understood my complex case - and if they didn't, was willing to learn! A clinician that you are comfortable with is a huge part of your healthcare, and well being. Never settle.
There will always be other doctors; though the transition from one provider to another is horribly time consuming and difficult, it is always worth it in the end. This week I wanted to share with my readers about a very special physician on my team, one who has made such a positive impact on my life.

My Gastroenterologist from day one has always put the patient first. She truly cares for an individual and is willing to go the extra mile. She has always let me take a strong lead with my health, and gives my opinion and ideas much consideration before we ultimately make a decision together. I cannot tell you how many times this woman has saved me from crisis. She is what every doctor should be; compassionate, attentive, and real. When she walks into the room I don't feel like a statistic or number, she sees me, she treats me, she understand me as an individual. We dig deep for new therapies, and work together in my healthcare. She has never shied away from calling me on the phone, or emailing me when I need her. Today was no different. We had an awesome appointment with a detailed explanation of my test results, and a solid game plan to move forward with. When I expressed my concerns she replied with empathy and reception.
I visited her Monday to go over the results of my Smart Pill Study, the test that was to track the transit time of my GI system so we could trace exactly where my problem areas were. The results showed strong abnormalities as we predicted. The pill slowed through my stomach, but slowed considerably more when it reached my small bowl. The small bowl is the most important organ in the entire digestive track, it is responsible for the absorption of nutrients and minerals, 90% of all digestion takes place here. The pill showed that my small bowl is severely delayed, more than likely caused by fibrosis from Scleroderma. Due to this delay my organ produces to much bacteria and leaves my body unable to absorb essential nutrients and minerals such as iron, phosphate, calcium, magnesium, and many others. Once the pill made its way out of my small intestine and into the colon it had quickened transit time. It seemed to shoot right through the colon much faster than a typical patient. This heightened transit in the colon is caused by Gastrocolic Reflex, a reaction caused by a malfunctioning autonomic nervous system. This reflex triggers the colon to increase its motility and expel any byproducts of digestion left in the body very suddenly. This is actually quite uncomfortable and causes a lot of cramping, sweating, lightheaded, and tachycardia. This reaction is triggered by simply putting contents in the stomach... for those of you that catch the drift, that means EVERY TIME I hook up my tube feeds, or have to do a bolus water flush I have to immediately poop. It's quite inconvenient, especially when you have a weakened sphincter due to fibrosis. While none of these tests results are "good," we at least know what we are battling and can locate the proper therapies and treatments to combat these issues. Much of my digestive issues, while they stem from Scleroderma, also are in part from my severe Dysautonomia. My awesome GI doc was already way ahead of me and has referred me to a Autonomic Nervous System physician to treat my failing body.
On the good news boat my recent blood panel showed that the use of chronic antibiotics is finally helping to keep some of that SIBO at bay! We saw a large increase in my electrolyte panel which means I am finally absorbing bits of nutrients here and there! Yipe!

As a random side note I received a call from my Pulmonologist today expressing the sudden interest in scheduling an immediate Right Heart Catheterization. A RHC is a procedure in which a small incision is made in your groin, a long catheter is then fished all the way up into your heart where it then passes into the pulmonary arteries to test pressures. This test detects the presence of Pulmonary Hypertension. And no, you are not asleep for the procedure.
The procedure is schedule for next Friday!

Before I sign off on my update this week I want to give my readers a brief over view of the Autonomic Nervous System, and why it is so harmful when it malfunctions. The ANS is responsible for the regulation of our most principal involuntary body functions; it controls internal organ activity. It manages your breathing, heart rate, blood pressure, vessel dilation, digestion, body temperature, production of bodily fluids, the balance of electrolytes, and many other vital functions of the human body. As you can probably guess any one of these crucial functions malfunctioning could cause great awry in the body. When the ANS malfunctions a general term of Dysaytonomia is given, but there are many subsets of Dyautonomia that generate different responses from the body. They symptoms can range from a daily nuisance to life threatening; though most cases of Dysautonomia fall into the category of severely limiting ones quality of life. Patients are in extreme discomfort, and home bound do to their malfunctioning nervous system. There are unfortunately not many, if any treatments. The main goal of physicians is to address symptoms, and try to make the patient more comfortable; many patients due to severe damage of body systems however are bound to walking aids, feeding tubes, constant IV fluids or urinary catheters.

To give you a small idea of what its like to live with Dysautonomia imagine this scenario: Do a head stand for 10 minutes, immediately get up and spin in circles for ten minutes, now drink a large slushy and receive a tremendous brain freeze, then run until your heart rate is 150, all while having food poisoning on a 115 degree day in Arizona... Oh and your blood pressure is 70/40. Couldn't stay conscious huh? Neither could we... But all we did was stand up from the couch. Dysautonomia is miserable.

Dealing With The Desolation Of Progressive Illness

I have had so many individuals question how I deal with the undeviating desolation of a progressive, and fatal illness. When bad news seems to be a constant in my life how have I not fallen into a deep, unfathomable depression. I have thought long and hard on this topic and found that the answer may have even surprised myself slightly. What I had attributed to "always staying positive" during the turmoil that is my daily existence, turned out to be almost the exact opposite. I found that I had the ability to keep my bubbly and lightheaded demeanor simply by not anticipating anything positive at all. In a nutshell, I always expect the worst and keep my expectations low. While that sounds like a heartbreaking way to live, the truth is when battling a disease like Systemic Scleroderma there truly isn't many other options. Before jumping to the conclusion that I am a pessimistic, morose individual, let me explain my reasoning:

When navigating the devastation of a degenerative illness you become familiar with phrases your loved ones, or even complete strangers may send your way. For me the terms I most received were idioms such as "Dream. Believe. Achieve.," "Always Stay Positive," and "The Best Is Yet To Come." While these expressions come with good intent, I found none of them helpful in my situation; I could not simply believe my illness away. No matter how hard I dreamed of a functioning body, no matter how positive I stayed I achieved nothing but further progression of my disease. The company around me encouraged me to stay positive and have confidence that I would receive good news from my next round of testing, so each time I was presented with poor results I was devastated. Each deficient medical result was another blow that took weeks to recover from. With this forced sunny disposition on an illness that is anything but, life seemed so bleak; constantly receiving information about the deterioration of my health when I was hoping for the exact opposite took a serious toll on my mental stability over time. I could no longer live like that -- It was time for a change.

I had, had enough of this "Dream. Believe, Achieve," perspective, it was unrealistic, and frankly counterproductive of an illness that a skilled physician had already expressed would take my life. Why would I stay positive for suitable test results when I knew they were not going to come? Why would I put myself through the torture of constant let downs? Why would I submit myself to the torment of persistent disappointment? Instead, I decided from that moment on I would prepare myself for the reality of my illness, and acknowledge the desolation it would wreak in my life. I told myself then and there that I was no longer going to hide from my reality but embrace the life style that had been thrust upon me. No longer would I cower in fear of unpleasant results or the progression of my fatal illness. I would meet these results, and new challenges head on!

While may people around me depicted this mindset as "pessimistic" I utterly disagreed. I believed wholeheartedly that my view was one of acceptance, realism, and endurance; what I liked to call "a realistic optimistic perspective." With this new outlook in place each of those negative test results, or new symptoms that developed did not knock me back for quite as long. I felt composed, calm, and prepared; I knew these results were coming and I was not surprised. I still felt the tinge of disappointment, but no longer the devastation and horrendous sadness that used to accompany my results. While yes, some days tears still flowed down my cheeks, my recovery was easier. With the full acceptance of my illness I was able to find more joy in my life and celebrate the small victories that manifested themselves; no longer in a constant state of mourning over my deteriorating health, happiness become easier to find.

I am in no way stating patients should halt their belief in miracles, or that they should abandon their hope. I myself believe strongly in the power of miracles, however, I will not put my life on hold for something that may not manifest itself in my lifetime. I plan on focusing on the now, and being ready for combat at any moment. I will fight this disease with every fiber of my being, and to do that I must be prepared; I will not be caught underestimating my enemy. I would much rather be prepared for the wreckage than live in fear of it.

I am sure this post will raise questions about my faith. If you are interested in my testimony of the Lord Jesus Christ and His hand in my life please click this link to a previous post: A Note To My Readers and read #2. I have found that many individuals state blindly, "you can be healed if you just ask." Please read through that post thoroughly before you comment on this posts, or any others with responses such as that. I have a strong testimony in my Savior and his healing powers but, His will is what governs my life, not my own.