Infusions & Coloring Books - Health Update

Monday I had my Smart Pill Study. This tests consists of swallowing a large timer inside a pill. It tells the doctors the pH and temperature of my entire digestive track, along with the transit time from swallowing, to defecation of the timer. This is helpful in diagnosing exactly where my digestive track is malfunctioning. The test started out with a bang! First off, you are supposed to eat a special bar that accompanies the pill, which of course I could not. We substituted with a bolus tube feed but I ended up throwing some up and gagging to much that it was really counterproductive. Then, as you all know, my esophagus has absolutely zero peristalsis, which means it is paralyzed. Swallowing large objects is impossible as they get lodged in my throat. Well, this pill was large... beyond large! It was about the size of a fat mans upper pink, and as thick as one too! It of course settled itself into my esophagus. Urg. After some choking, gagging, and water guzzling we got it to slip into my stomach! Hallelujah! For a while we thought we would have to sedate my and push it though in an endoscopy! lol Once it was all settled in my system we just had to wait. Typically this pill takes 2-5 days to pass through a system, and someone like me with a horrible GI system could take even longer! To both the surprise of myself, and my doctors, my pill came flying out in less than 24 hours. Needless to say we were all very confused! That is not normal by any means, and in no way what we expected to happen. The results wont be in for two weeks, but I am hoping it will give my doctors some insight. I can't imagine what would cause my body to literally expel the pill like that! I guess I'll find out soon... And since I am sure you are all wondering, no, I didn't have to "retrieve" the pill. It gets flushed since all the data is wirelessly transmitted to my doctor. Thank goodness!

Wednesday morning I met with my Dermatologist for my yearly review which went swimmingly. She is one of my absolute favorite physicians... which is saying something because I have a lot! lol She is young, kind, knowledgeable, and so in-tune with her patients! She took a detailed look at my scalp as I have been having issues with sores the past year or so. Recently it has gotten to the point where I cant brush my hair without getting blood, so we decided it was time to get these pesky unknown sores taken care of. Luckily she does not believe it is anything serious, but an odd side effect of a medication, folliculitis, or possibly staph, though staph would be unlikely. We cultured them just in case and are starting a third medication since nothing else has seemed to help them heal. If this medication fails we will take a biopsy of the scalp in a few months. As a funny side note, she always comments on my "beautiful skin!" Due to the extra collagen from my illness my skin literally seems to shine, leaving no dimples or blemishes, giving it a perfect smooth appearance. I often look like I am literally made of porcelain, plus that nice "anemic-glow" I have doesn't help my case ;) lol 

Some girls accessorize with shoes, but I accessorize with tubes! ;)

Wednesday afternoon was my monthly infusion at the local university hospital. The drug I get is called Rituxan (Rituximab.) This is a drug that eradicates the immature B-Cells in my body. The purpose is to deplete my immune system of malfunctioning cells, but I can without a doubt say, this drug is not doing its job. My disease is continue to progress, and at a very fast rate. Nonetheless, my doctors are continuing my relationship with this drug until we can locate a more aggressive treatment. Rituxan in itself is a very dangerous, and high class medication used in Cancer and serious autoimmune diseases as a last ditch effort when patients respond to nothing else. Many patients have severe life threatening reactions to this medication, but the worst part is, it can happen at any point in your treatment; it doesn't always happen your first infusion. Sometimes it's the 5th, 11th, or 100th time you receive it. Each time is a lottery. One day your body decides it has had enough poison and ties to kill you. Luckily, I have not had a large reaction in my history with Rituxan, but my nurses are always reminding me of the possibility, and of how dangerous of a drug I am pumping into my blood stream.
Today I had one of my favorite nurses, Gale. She is incredible and is always willing to go the extra mile for me. We had a lengthy conversation over my five hours at the infusion center. At one point we were talking about treatment option on the horizon and she said "It is so sad that diseases like yours don't have more research. Autoimmune diseases effect the same amount, and if not more people than Cancer, yet so little is being done." Coming from a nurse who has been working in the industry for 37 years, and seen just about everything, this small statement meant so much to me. To have someone understand the torture and fear a autoimmune patient faces in a world that can only seem to grasp illness is a validating feeling. So many autoimmune diseases have no research, and flat out no treatments made available to patients. We suffer through drug after drug that do not prove us any benefit simply because no one is willing to research our terrifying, and persistent conditions. We talked about Scleroderma specifically for a while and she told me that in her 37 years she hasn't seen very many cases. It's amazing to me that a disease as debilitating, yet fascinating, can be so unknown. I cannot think of a disease much more horrifying than turning into a living statue as all your organs become paralyzed and cease to function. It is truly an awful way to die. Overall my infusion went well and seemed to fly by today. I brought a fun pass time called "The Mindfulness Coloring Book an Anti-Stress Therapy for Adults." Coloring really does clear the mind! The time I wasn't doodling I enjoyed my time chatting with Gale about life, and where I hope to go from here with treatment. We both are putting faith in the possibility that a Stem Cell Transplant is in my future.

Next week starts another parade of appointments! I see my Electrophysiologist about my malfunctioning autonomic nervous system, my Pulmonary Specialists to repeat my PFT, and my Rheumatologist to hopefully review new treatment options. Later in the month I have an appointment with my Scleroderma Specialist from UCLA; I really believe he will be able to point me in the right direction! My body is so unhappy right now and all I can do is try to keep it a float until I see him... You can do it body! Just one more month! And thank you as always to all my incredible readers for your overwhelming support and love! I read every email, and every comment! They always bring a smile to my face! 

Dwindling - Health Update & Scleroderma Conf.

Let's start with the positive.

Last weekend was the 2015 National Patient Education Conference held by The Scleroderma Foundation in Nashville Tennessee! I cannot express to you all my excitement as my Mother and I lifted off the tarmac in Seattle. Last year I had been unable to attend due to an emergency surgery so we made a pact that we would attend the conference that upcoming year! After a year of sheer anticipation the conference did not disappoint! I had the absolute pleasure of meeting some of my best friends in the entire world! As a young woman suffering a rare disease I have grown very close with many other individuals suffering this illness through social media, and to meet them in person, to hug them, spend time with them, and actually speak to them was a dream come true! I cannot even explain the immediate, lifelong connections I have sown this past weekend.
We had the once and a life time experience of partying downtown Nashville on our second day, which was actually quite comical! All of us gimpy individuals with oxygen tanks, feeding tubes, wheel chairs, and ports jetting out of our chests while dancing and singing to country music at the top of our lungs. On lookers must have been very confused! lol This was hands down one of the most memorable experiences of my life! We even got to meet the band who took pictures with us after the show!
On the last day of conference I had the great honor of speaking on the "Living Well with Scleroderma" Young Adult Panel which consisted of me, and two other patients. I adore public speaking, but never dreamed I would have the opportunity to speak at a National level! I am still in shock that it happened! I finally feel I know what my calling in life is... to yell the name of this disease from the top of every mountain!

I unfortunately got pretty sick on the last day as we winded down to the last few hours before our flight. My body just isn't used to being off a couch very often. Once the adrenaline wore off I crashed rather hard. I ended up early making it to the airport before I passed out. Luckily I had wonderful travel companions and there were doctors from the conference in the airport if I needed them ;) lol Overall, I wouldn't trade this experience for the world! It was worth the immense body pain and struggle on that last day!
Once I returned home it was back to the daily grind with an early morning appointment to see my Electrophysiologist. This doctor is a specialized Cardiologist that basically deals with the "wiring" of the heart, in very simple terms. I was referred to him for my SVT episodes, but what we decided to treat was something entirely different, and actually the reason behind my SVT! First off let me just tell you that I LOVED this doctor. He started off by being right on time, which is always a good sign, and continued his awesome streak by talking to me like a well informed individual! He could tell I knew my stuff so he didn't use any of that "baby language" as I like to call it ;) After looking over my medical records, and chatting to me about my symptoms he decided the real problem wasn't my heart at all, but my body ability to retain electrolytes. His words were "Im going to be honest, your body is screwy. Extremely screwy!" Which I whole heartedly agree with. He explained that due to my autoimmune issues, severe malabsorbtion, and my reliance on a feeding tube, my body just can't retain its electrolytes and minerals it needs to function properly. This lack of necessary nutrition and absorption is causing severe autonomic dysfunction, meaning my body can no longer regulate things like proper blood pressure, heart rhythm, and heart rate. This dysfunction is whats causing my fainting spells, low blood pressure, tackycardia, and odd heart rhythms. His protocol in this situation is fairly simple, to prescribe a medication that helps the body retain salt and electrolytes! Now, the catch is, it's a steroid and I am already on steroids... which really shouldn't be done, but we have no choice. It's safe, just not ideal. The other catch is that you have to have at least some electrolytes and sodium for the body to hold onto, which I really don't. He basically said he can't compare my baselines to the typical norm because I am truly one of a kind, so we are basically free falling here! Hopefully this pill will work, and if not our backup plan is to start daily saline infusions to keep me out of Hypotension, which is a fancy name for serious low blood pressure. In a blanket term it's called Dysautonimia. Overall it was a great appointment and we have a solid game plan! No ablation needed! I can't wait to follow up with him in a month!

Next I was off to my Pulmonologist for a  pulmonary function test (PFT). We have been following my pulmonary and diaphragmatic function very closely lately. A typical patient has this test done every six months to a year, whereas I am having it preformed every 4-6 weeks. There are three very important factors we look at on my PFT, the Diffusion Capacity (DLCO) which expresses the lungs ability to push oxygen through the air sacs of the lungs into the blood, the Forced Expritory Volume (FEV1) which expresses the amount of air your lungs can force out, and the Respiratory Muscle Function (PI/PE). This values give my doctors a look at my overall respiratory systems functioning ability, and also a good idea of how my disease is progressing. Unfortunately during this PFT we saw a large decrease in my respiratory functions. My DLCO dropped by almost 20% leaving me now in the low 60s, my FEV1 dropped 11% leaving me in the low 80s, and my PE/PI stayed within my typical range thankfully, however it only functions at a measly 20%. These numbers make the obvious statement that my disease is progressing and the current treatment regimen I am on is not adequately slowing this disease. The DLCO especially is a huge red flag as any number in the 60s means there is an evident obstruction, or possible Pulmonary Hypertension. We will be re-preforming the tests in two weeks to verify the results, and scheduling a right heart catheterization.

While I convoy mostly strength and resilience and this blog I want you all to know that having a disease that you know will take your life much before your time is truly devastating. I have my days when I break down, become distraught, cry, and feel emotional about my circumstances. Constantly being left in limbo about your life is such a difficult existence. I loath relying on medical professionals for my every need; I was once an independent individual with reigns to my world, but declining health has stolen that from me. While I feel I have prepared myself for every blow, and truly accepted the destructive nature of this illness, sometimes it is still hard to embrace. Each day is another hurtle, another tempest, another period of time spent trying to pick yourself up again. No matter how adjusted to bad news you feel you are, sometimes it just still hurts.  I have said before that life with this illness is like a vase being repeatedly smashed. I know that my beautiful, perfect vase will be destroyed and I have prepared myself. I know that no matter how hard I try to frantically place those broken pieces back together I will never be able to place them exactly the way they were before; it would be a lost cause. I feel with this knowledge the blows become easier to take, each smash doesn't leave me lost. I have learned that even with my shattered pieces I cans till construct something beautiful, though it may be different, it is still functional and pleasing to me. Yes.... I know my vase will be mashed and I am prepared... but that doesn't mean it doesn't hurt watching those pieces splinter. It doesn't mean that glue I used to reconstruct it was dry of tears, or that the tape I used wasn't wet from sobbing. I am a positive person, and a strong individual, but I am here to let you know that accepting and embracing every emotion is never a bad thing, even if that emotion is pain, fear, hurt, or the acknowledgment of an unstable future.

This upcoming week I have many appointments and procedures as usual. I start with a Smart Pill Capsule Study to time my digestive function on Monday morning, plus a Rituxan Infusion, and a Dermatology appointment later in the week. Before I sign off on this post I wanted share a wonderful article one of my beautiful Scleroderma Sisters, and fellow Chronic Illness Blogger wrote after a conversation we had last month. I am honored to work with this individual; the Scleroderma community is so blessed by her knowledge and willingness to share.
To visit our Feature: You Won't Believe The Story Behind These Two Faces
To visit her Blog: Comfortable In My Thick Skin

Breathe Easy, Not Ugly!

When I was first prescribed supplemental oxygen my home health care sent me some very large, very ugly oxygen cannulas. At the age of 23 I was pretty disappointed to wear these very obvious cannulas in public; while yes adequate oxygen flow is the most important factor here, receiving adequate oxygen while looking fabulous isn't a bad thing! I took to Google to view photos of different makes and models but came up empty handed, especially for patients of a young age. All I seem to find were staged hospital stock photos, or 90 year olds learning how to use an oxygen tank for the first time. Neither of these situations were helpful in my case, so I decided to purchase multiple brands of cannulas to find out which one looked the best on a young persons face. All of my cannulas were purchased off VitalityMedical.com as they have the best prices on single oxygen cannulas, plus a very large selection!
After purchasing many cannulas and finding which were the most aesthetically pleasing, I decided I couldn't be the only person in their twenties who has faced this dilemma! Hence this video was born! It turns out there are many makes, models, and brands of cannulas that all look, feel, and sit differently on a the users face. I reviewed just a few of my favorites to show you all what the different types of cannulas look like. Please remember this video reviews the cannulas on an aesthetic basis only, and not on functionality, quality, of efficiency. 

The cannulas I review in order are the:
Salter Labs Nasal Cannula Micro
Salter Labs Nasal O2 Cannula
WestMed Comfort Soft Plus Adult Cannula
AirLife Nasal Oxygen Cannula
Hudson RCI Nasal Cannula with Star Lumen
AirLife Mini Cushion Nasal Cannula
(All cannulas were non-flared tips)

This smaller window will give you the best quality video.

What It's Actually Like To Be Sick

In the onslaught of popular media can you see past the facade of beautiful celebrities with their perfect hair, make-up, and unscathed bodies during a hospital admission? Or do you look at those depictions of disease and believe it is an accurate portrayal? Lets take a moment to step back from the illusions we are fed by social demand and familiarize ourselves with the true situations serious illness sufferers face.

In the real world hospital admissions are not quickly staged over by the seasons changing outside a window, or time lapsed by a fade to black -- they linger, and are mind-numbingly monotonous. You are vividly aware of every week, day, hour, minute and second spent stuck in that uncomfortable bed. Much of your stay is spent counting ceiling tiles, stalking your friends on Facebook or simply sleeping because you're in too much agony to do anything else. There are not always gaggles of doting visitors in your room either; the reality is many people are too busy to drive miles out of their way for a visit to your hospital in another town. After the first few weeks you are no longer an interesting commodity anyway, but a tired post on social media that people scroll right by. Your room is not quiet -- loud beeps break through every hour as your many pumps finish a set of fluids, nurses enter in to take vitals every 15 minutes even during the night and you probably have a roommate that listens to the television much too loud. A private room is a hot commodity you are more than likely not going to get, sometimes you don't even get a window. And you definitely can't leave your room, let alone your bed, without an alarm going off alerting staff that you are not where you are supposed to be. You are more than likely a disheveled, greasy mess; you sometimes don't get to shower for many days at a time, and sponges baths really aren't everything they are cut out to be. You wear the same clothes over and over, or have surrendered to the typical hospital garments because you probably didn't expect to get admitted when you entered that ER. Your hair is matted and sweaty, you probably have remnants of smudged, old makeup caked into your skin, and you most definitely possess large bags under your eyes, bruised arms from blown IVs and an overall exhausted appearance from the loss of sleep. The hospital is neither a perfect establishment where everything goes according to plan, nor run by perfect people exempt from making mistakes. The wrong medications get sent up to your room which delays your treatment, and the pump that was ordered for your tube feeds doesn't correspond with the bags ordered so you have to wait another hour to get your nutrition. You may have just had an allergic reaction to the new medication you received and are now back at square one waiting for the doctor to prescribe another. With the way your luck is going your IV may have just blown for the eighth time since arrival and now you have to wait for IV therapy as well. You are miserable, lonely, in pain and wanting nothing more than to escape. Hospitals are in no way a peaceful place of healing like they are illustrated to be on television -- and doctors are not always the caring, compassionate individuals you see represented. The reality is a far cry from the impression popular media depicts.

A photo from a 22 day hospitalization back in December 2014

The days not spent withering in a hospital bed are not consumed by enjoying your "loads of free time" due to your inability to work a traditional nine to five job as believed by the general population. Your body is much too tired, exhausted and in pain to leave your house most days. You spend the majority of time on your couch watching Netflix and taking intermittent naps while hooked up to your home IV hydration, supplemental oxygen or continuous tube feeds -- with the occasional trip 10 feet down the hall to throw up in the bathroom (but let's be honest, sometimes you don't make it to the bathroom). You cuddle with your cat and stay wrapped in your blanket shivering even though it's 90 degrees outside because your body can't regulate your vitals correctly. You spend the remaining hours of the day navigating the complicated backstage workings of the patient health care industry, functioning as our own personal secretary: making phone calls, writing emails, completing loads of paperwork, and sending faxes. Insurance companies, billing departments, research facilities, state disability programs, physicians, RNs and care coordinators are just a few of the people you deal with on a daily basis. Chronic illness is a full time job with no pay, no vacation and no weekends. Every day is a sick day -- but you still have to work. This notion of "free time" doesn't actually exist for the chronically ill. When not exhausted on the couch you're in a waiting room at one of your eight appointments a week, getting tubes and needles shoved places they don't belong, or sitting in an infusion room chair getting your chemo, iron, or saline infusions. The rare occasions when you do decide to leave your humble abode for a coveted "non-illness-related activity" -- it is anything but sunshine and rainbows. First off, putting on real pants can be a serious challenge when most of your days are spent in cozy pajamas. Then there is the fact that you must pack like you're going off to war even for the shortest of tips... because anything could happen at Target! You'll need a backpack full of the chronic illness necessities: a mini pharmacy, extra formula, emergency supplies in case your feeding tube or Port gets yanked, an extra oxygen tank if you want to stay out for an extended period of time, warm clothes in case the temperature drops, a list of all your current medications and physicians information, etc. When you finally get to your destination your so exhausted from all the prep you just endured to simply leave the house that you decide to bag the day out anyhow. Maybe you will try again tomorrow.