Thursday, December 31, 2015

The Grinch That Barfed On Christmas - Health Update

I thought I knew. I really did. I thought I knew what nausea was -- I was wrong.
Nothing I have ever been through quite compares to chemotherapy, besides perhaps almost dying of Meningitis & Guillain Barre last December. I have been on low dose chemo for years, and was working up to a full dosing of Cytoxan over the last three months, but none of it even remotely compares to the high dose I am now enduring. I can honestly say, now that I have encountered both, that there is an extensive difference in how the body responds to low, and high dose chemotherapies. While yes, they are both the same drug, one dosage provides nausea that one will never be able to comprehend until they have physically received it.
Dang. I thought I knew nausea on 25mg Methotrexate. Nope. That was a walk on a beautiful sunny beach compared to high dose Cytoxan. This is a whole other ballgame.

Gettin' Christmasy at Chemo!
Let me tell you about my holiday. Cytoxan was on the 23rd, and that night things started to get funky. Dry heaves, extremely red in the face, head pressure, sweating, hot, hot, hot, and exhaustion. The next day I was lethargic, freezing, and unable to keep my eyes open. The dry heaves increased as the second night rolled around, turning into full fledged barfing episodes all over my bed. Now, since my esophagus has zero muscle function barfing is somewhat of a challenge. It can happen, but it's not so much a projectile vomit one pictures in their head. It is more of an urp that takes way too much effort. It's noisy, and messy, and comes out in wild streams. After vomiting to the point of becoming teary eyed, and getting absolutely no sleep, I found myself lying in bed all the next day in and out of a sleep deprived consciousness. I couldn't run my tube feeds adequately, so was luckily I had the IV fluids at home. The nausea was like a ton of bricks; no medications would touch it. Holy hell it was misery. The next day of course was Christmas -- the third day. Everyone familiar with chemo will tell you that the third day after your infusion is the worst. Well, I attempted to see my family on Christmas, only to barf all over my step-dad -- twice. I then proceed to barf in the hallway as my family helped me into the bedroom to rest. (For those of you wondering, as I am sure someone will suggest it: yes, I tried medical marijuana for nausea. No it did not work.) Things continued like this until about four days post infusion. I will here by be naming Cytoxan: "The Devils Piss."

On the opposite hand, I am already starting to see some improvements in my everyday life thanks to this hell of a drug! Where once I was unable to climb a flight of stairs without stopping for rest due to exhaustion, and shortness of breath, I am now able to do so with proficiency! I noticed it the other day; I needed to run out to my car because I forgot my phone. I didn't want to be attached to a bunch of tubes and tanks for such a short trip so I unhooked my feeding tube, and left my oxygen inside. As I walked up the flight of stairs (8 steps) to return to my apartment I didn't stop, and I wasn't huffing and puffing at the top! I know that seems minuscule, but it is actually a huge deal! This means that the Cytoxan is removing some of the inflammation and other crud from my lungs, leaving me able to breathe better. From that moment I began taking my little O2 sat reader everywhere with me to see how my oxygen levels were doing at other times during the day. My oxygen stayed stable when at rest (excluding sleep of course), and stable-ish while walking short distances; only when I began to exert myself with longer trips to say: the mall, grocery store, or events did my saturation begin to decrease to the upper 80's. (Also if  I talk with too much excitement! lol) It seems the duration of time I push my body weighs into my saturation levels heavily. I noticed when I do not use my oxygen I get tired very quickly, and am not able to be out of the house quite as long. I think the low O2 upon a long day has to do with my weak diaphragm as well. My main goal with Cytoxan is to rid myself of the need for supplemental oxygen completely, and while I am no where near that goal yet, I am definitely able to start decreasing my usage which is a huge victory! So if you see me taking short trips without my O2 that is why. I will of course be smart about this opportunity, and always have a machine with me while I am away from home. I still need the assistance from the supplemental oxygen, just not constantly like I had been using it the last many months.

I also have noticed some improvement with oral intake. While my esophagus will never have muscle function like it used to, the inflammation that was once lining it has eased up incredibly! This means purees, extremely soft foods, and liquids in very small amounts/bites can be dropped down my esophagus to the stomach by gravity. Unfortunately my intestines still aren't playing very nicely and like to cause quite an uproar when I attempt any oral intake, but my gastroparesis does seem to be slightly improving at least. Due to the intestinal issues that cannot really be tamed by any medication, my digestive function, and nutritional absorption will always be very poor, leaving me tube fed indefinitely for adequate nutrition. However, that doesn't mean a bit of recreational eating (even if it comes with misery) can't be had every once and a while!
So far I have had some yogurt and it went well -- if you don't count experiencing heart burn for an hour then throwing up... Maybe next time! lol ;)

How amazing is Mount Rainier made out of lights!?

I did get to experience some fun this holiday season. I attended an awesome event called Zoo Lights with my best friend. We ran around the zoo/aquarium with tanks, tubes, and masks while every passerby looked at us like we were crazy -- or possibly patient zero. We had an absolute blast, and did just about everything, from camel rides, to the carousel, to watching sharks swim by. Even though my body is now plagued by fatigue, sore joints/muscles, and will need a good amount of rest to recoup, living like a normal person even for just a night is always worth it - I even wore my fancy wig!

Happy New Year World!


Sunday, December 20, 2015

Just Call Me Chanel MD - Health Update

This last week, I saw my Pulmonologist and performed my routine PFT (pulmonary function test). Unlike most patents, I perform this test every 2-3 months, instead of every 6, as my lungs have been relatively unstable in their values since summer. A PFT is preformed in a small glass box with what looks like a snorkel jetting out at you. You are to breathe into the snorkel using different techniques to asses your body's pulmonary abilities. My results tend to fluctuate some, as most patients do, but lately they have been taking great bounds in the wrong direction. Luckily, I stayed stable in my results this round. While we always hope for an improvement, I'll take (relatively) the same results as my previous test over tanking any day! The Cytoxan should yield better PFT results in the upcoming months if the drug is properly doing its job. So hopefully by the next test we will see some improvements!

I started home infusions this last week as well. I now have a nurse arrive to my home once a week to access my port so I can run my own IV fluids throughout the week! It is incredibly convenient, and kind of fun! I feel pretty accomplished being able to run my own medical equipment and provide myself fluids. I even get to de-access my own Port! Yup, that's right! I get to remove that giant needle from my chest all on my own! ;) It is so awesome! So far everything has gone extremely smooth, and I have met some great nurses already. My house looks like a little hospital with all my medical equipment between the central line, feeding tube, and oxygen stuff; our fridge is even full of big saline bags! lol My poor husband! So if you see me with a device sticking out of my chest, that's just my new normal. My port will be accessed 4 days a week from now on.

After a month of waiting, I finally had my appointment to have CPAP training. There was some kind of mix-up with the orders so the home health care company didn't receive them from my doctor till last week. Turns out the home health care headquarters are only about 15 minutes from my home, which is massively convenient. I arrived and was whisked back to try on masks, and trail my device. The CPAP I received is brand new on the market, and extremely quiet! It's so small it doesn't even take up half my mini nightstand. We had some trouble finding a mask since my face has some very distinct features, but eventually found a full face mask that fit well. So far sleeping with it has been a breeze, besides for the fact that it leaves big hives on my face when I awake. I think my extremely sensitive skin is very unhappy to have something suctioned to it for 6-8 hours. I will probably call the company later today to see if that is normal your first few nights, or if it's just over sensitive me. I will trial the CPAP for 30 days and then meet up with my doctor to decide if the device is working or not. Due to my weakened diaphragm we were unsure if I would need a BIPAP or not. I am having a bit of a hard time breathing out with the continuous positive pressure since I only have about 20% use of my diaphragm. The BIPAP however would breathe in and out for me. So I guess I will find out if I am switching when I see my Sleep Pulmonologist at the end of January.

(Gross alert! Skip paragraph if you don't like bodily fluids! lol) Friday I woke up in some discomfort, feeling a bit nauseous and bloated. I noticed my feeding tube had become looser than normal so I checked the ml amount that typical filled my anchoring balloon. Where there should have been 15ml, there was only 1ml. I quickly filled the balloon back up only to have water, and puss gush out of my tract that holds the tube in place. Figuring the balloon had ruptured in my sleep I called the doc who wanted me to come in and be seen. Typically I would have just swapped the tube out myself but, since there was a good amount of puss, and pain, I was asked to not do the procedure myself until I was evaluated. Once I arrived to the physicians office they sent me straight down to the Emergency Department. Figuring I had an abscess, we ran CT scans, and x-rays attempting to find the origin of the infection now oozing out of my stoma. Unable to locate anything highly abnormal after a long 12 hours in the ED, we decided to remove the current tube and place a fresh one. I went to pull out the old tube and 'who only knows what' came rushing out! Yuck! Luckily, this event pretty much explained the entire situation. When my balloon ruptured the night before all that liquid, and the now free floating stomach acid, had moved into my tract, leaving it caught between my stomach, and abdomen wall. Yes -- it is as painful as it sounds. In turn the tract become aggravated and pussy, mimicking the look of an infection. It was a rough night filled with pain killers, nausea meds, and bodily fluids that no one should see escape a hole in their body. lol Once the new tube was in place, and the extra nasty fluids had been drained, I immediately started to improve. I will be a bit sore for a while but, no major injury, or infection ensued. As a funny side note, all the physicians in the ED were surprised when they found out I remove, and place my own tubes. (Probably a little relieved as well! lol) The look on their faces were priceless when I yanked that popped balloon out, then handed it to the one standing closest to me; he particularly looked really grossed out actually! haha I don't blame them though, they weren't GI doctors, so they probably don't see that everyday! Just call me Chanel MD ;)

Today I did something I haven't done in over 5 years, performed. Way back before illness overthrew my life, I was a classical vocal performer; music was my world. I had an almost full ride scholarship to a wonderful college, took part in multiple vocal competitions (which I normally won), and performed on stages for hundreds of people. There was no rush that quite matched you and a microphone staring out into a dark venue knowing everyone attending was listening to you. Well, Scleroderma stole that voice from me, the one that opened doors to my world. Thick fibrosis now fills my vocal chords and lungs, and my diaphragm has but a measly function; it is simply no body for a singer. Due to this change in my voice I was too ashamed to sing over the last many years. I wanted people to remember me as my old voice: the one that brought tears to their eyes, not laughter. The last few weeks I have been mulling over in my mind the joy that performing gave me, the rush and excitement it brought to my life. I missed hearing the last note of my favorite aria echo in a performance hall. I started to think about all the posts I have written about accepting ones new limitations when you find yourself stricken by illness; all the posts I wrote about doing what makes you happy, and realized -- I wasn't taking my own advice. No, my voice is not the award winning Soprano it used to be, but performing still brings that thrill to my heart, so why not? This is me, this is who I am now, and I must accept that. Today I sang "Oh Holy Night" for my church's Christmas program, and it was the most fun I have had in years! I may not have been on pitch at all times, and I may have cracked on every high note, but that excitement of performing was still there! :)

Tuesday I have my third round of Cytoxan so, if you don't hear from me for a little bit, that's why! I hope everyone has a fantastic holiday! Merry Christmas and a Happy New Year! ...Though honestly the new year is never very exciting for chronic illness patients as our insurance deductible normally restarts! lol As a little gift to my readers, here is the most epic Santa picture ever taken! ;) Feeding tubes, central lines, oxygen tank, bald head, and all!

My best friend and I may have frightened Santa! 

Saturday, December 12, 2015

Ten Awesome Perks Of Medical Hair Loss

Lets face it, when it comes to medical hair loss there are a lot of negatives. An individual's hair is a focal point for society, and is often one of their most beloved physical traits. Losing that familiar identity can be a rough transition, especially when it's forced upon you by an illness you never asked for. I recently found myself bald while receiving chemotherapy for an autoimmune disease called Systemic Sclerosis. (Yes, chemotherapy is used in other illness besides cancer, read about it here!) Once a woman who spent hours primping her features to perfection, I now found myself with a very conspicuous, very shiny bald head. I have decided however, that there are actually some pretty awesome perks to rocking the bald look!

1. Inclement weather doesn't bother me in the least!

Pouring rains, heavy winds? Bring it on! I no longer have to hide a perfectly executed Pinterest inspired hair-do under an umbrella or hood. No longer do I worry about the wind tangling my curly locks in my favorite statement necklace. My bald head fears no act of mother nature -- well besides snow, that can get pretty cold!

2. I can try on as many shirts at Target as I want!

Worried about messing up that perfect top knot? Not me! I can pull as many t-shirts, dresses, and sweaters over my slick head as I please -- with no consequences. I don't worry about catching my hair in that adorably buttoned top, or ruining my pony tail on a built in necklace collar. Target has an unlimited dressing room item amount, and I am about to go for the record!

3. I can sleep in another half an hour every morning!

I used to take about half hour every morning to play with my hair. Maybe try a new style from my favorite magazine, or push in a few bobby pins. I would straighten my bangs, curl the rest, or add in a cute headband for the day. Creating a flawless look to match my style was something I truly enjoyed -- but you know what I enjoy even more? SLEEP! No hair means no prep. I hit that snooze button with gusto every morning! Take that chemo!

4. Fancy events are so much less of a hassle!

Worried about how to achieve that detailed 50's hairstyle with your new tea length party dress? Nope! I just slap on my fancy outfit and make sure my red lipstick and cat eye liner are on point! I don't have to deal with the tricky correlation of hair and outfits any longer. Saving time, money, and possibly the outfit, if I forgot to put it on after I started in on my luscious locks. Don't you just hate when you have to wiggle your cute top over your already done up 'do' just to get make-up and hair product all over it!?

5. My sink drains have never run smoother!

This should probably be number one. (I am sure my landlords like me a lot more as a bald woman than they did when I had hair!) No more snaking out the tub drain before every shower, and no more sinks backing up when I try to wash my face at night. Drano is no longer on my shopping list!

6. I save tons of money on hair care products!

Shampoo, conditioner, gel, hairspray? No thanks! Not to mention the hairdryers, hair straighteners, curling irons, etc! I cannot even begin to tell you how much money I have spent on those babies, too much if you ask me. Not to mention all the money I save on salon hair cuts!

7. I never have bad hair days!

Please -- I woke up like this!

8. My showers last about two minutes!

Body washed? Done. Need I say more?

9. I never have to worry about bad hair cuts!

Remember leaving the salon in tears over those bangs that were too short, or the pixie that was suppose to be below your ears, not above them? Yes, we all do. (I am sure each of you reading cringed at your computer remembering your last botched hair style.) Yikes. No longer am I susceptible to a stylist who: "does this kind of cut all the time honey!"

10. I still look flawless when I work out!

Okay, lets be honest, I don't work out! (I mean who has energy for that on chemo!?) However, if I did hit the gym I wouldn't ever have to worry about that cute guy on the treadmill seeing me sweating like a pig. I don't shed a moment of worry over dripping, wet hair from perspiration. My bald head looks even better when it's glistening from physical exertion!

The author, Chanel White, rocking her bald head!

Monday, December 7, 2015

Cytoxan Round Two vs. The Hulk - Health Upodate

Round 2 of 12 
Two weeks ago I received my second round of Cytoxan. (Wow, two weeks since an update!? Oops!) The infusion went well, and the dosage was upped by an extra 250mg. This round brought more nausea, and some extra fatigue, which my team was expecting since we are increasing the dose each time. There was one issue during the infusion, and that was a mix up with the medication (Mesna) that is used to protect my bladder from the harsh side effects of chemotherapy. Apparently the orders expressed that I was to pick up the dosing of Mesna, in oral form at my local pharmacy, instead of receiving it intravenously during my infusion like last time. (Which is still unclear if that was actually the case of just what the hospital told me.) This of course turned out to be inaccurate, but I had no way of knowing since I am unfamiliar with the medication. I quickly called my physician's office once I was informed by my pharmacy that no one carries the drug outside of the hospital. They were extremely surprised that the hospital did not provide me with the Mesna. After some phone tag with different hospitals, and my physician's office, it was decided that there was nothing they could do (which was NOT okay) since the time window to receive the drug to reach efficiency had passed. So basically, due to a screw up, my bladder was left unprotected from this round. I am absolutely following up on this oversight with my physician's office and the hospital where I received my Cytoxan. I will not let something like this happen again.The few days following Cytoxan were filled with nausea, a constant dosing of Zofran, and resting on the couch watching movies. Thankfully the effects only last about 4 days till I begin to feel human again.

Last week I had two appointments, one with my Autonomic Nervous System Specialist (Neurologist), and one was with my Gastroenterologist. The appointment with my ANS Physician was to review the results of my Tilt Table Test. In one of my last posts I had expressed that the results showed I had a form of Dysautonomia called POTS. (Postural Orthostatic Tachycardia Syndrome) While this information was accurate, my specialist informed me that my results actually indicated that I have a specific form of POTS known as Hyperadrenergic POTS. This is an uncommon form in which the body does not correctly regulate blood norepinephrine levels, leaving them extremely high. Norepinephrine is also known as "the stress hormone;" it is the bods y'"fight" response hallmarked by a thudding heart beat, tachycardia, heavy breathing, increased sweating, and a state of anxiety. Essentially with too high levels of this hormone, you are an adrenaline pumped mess at all times, leaving your body in tremors until you lose consciousness from sheer overload. It is pretty miserable. I imagine it's what the Hulk feels like! lol ;) Good news is, it is highly treatable with medications that block norepinephrine. My doctor placed me on a drug called Propranolol which has already successfully lowered my resting heart rate (yes I said resting!) from 110bpm to 78bpm! I cannot tell you how good it feels to not have my heart exploding out of my chest! The other useful treatment is the use of salt tabs and saline, which we began when we first suspected POTS many months ago. My Saline infusions will be upped from once a week, to twice a week since we now have a firm diagnosis. Even more exciting though is that these saline infusions will now be at home! Due to some confusion between the ordering physician and the local hospital I receive my fluids at I was able to secure a prescription for home infusions instead of trekking to the hospital twice a week on top of all my other appointments. This means a nurse will come to my house twice a week to access my port and run fluids from the comfort of my own couch. Awesome right!?

So many tubes! Mmmm Saline!
The appointment with my GI was basically just to check in, reevaluate my medications, and of course get a big hug because she is my favorite! My GI has always been my most bothersome system however; with the progression of my other organs, the GI tract has been relatively quiet for a change. My GERD and SIBO are both well under control on my current medications which is seriously a miracle! Finally, after a two years of messing with medications my system seems to have found a sweet spot. This doesn't mean that my organs are magically cured and I can eat meals again; it just means that we finally have found the right cocktail of meds to prevent me from barfing while simultaneously crapping my pants. Score! 
In all seriousness though, I am very happy with my digestive tract right now. It has been so nice to me lately I have even been able to have some small holiday snacks like Hot Cocoa without a violent reaction! (Give it sometimes it doesn't go well, but I have had a few successful cups!)  Hopefully this will continue through the Christmas season!


Saturday, November 28, 2015

Chemotherapy Isn't Only For Cancer Patients

When you hear the word chemotherapy what immediately comes to mind? Cancer. Bald. Nausea. Vomiting. Infusions. Sterility. Bone Pain? Upon hearing that word, most people jump to the same conclusions, with the same general consensus on how chemo works, who receives it, and what side effects it has on the body. The truth is chemotherapy isn't only for cancer patients, and affects each patient differently depending on how it is used. 

Chemotherapy is a widely used class of drugs to treat many different disorders including, but not limited to: cancers, blood disorders, and a plethora of autoimmune diseases. Similarly it can be administered in many different forms: intravenously, topically, injected, or given by pill. Each chemotherapy affects patients individually depending on the of type of chemo and the dose given. While many cause major hair loss, sterility, and nausea, others do not. This does not mean however, that the drug being received is not a chemotherapy agent. 
All chemotherapies work homogeneously, to eliminate the growth of harmful cells in the body. Some do this by directly damaging DNA, interfering with DNA building, while others simply alter the DNA in a way we do not quite yet understand. The different types and dosages of chemo given are decided by your physician who can determine what the patient will benefit from most.

Lately there has been stir in the media about a young singer named Selena Gomez battling an autoimmune disease called lupus. Selena stated in a recent article that she was "going through chemotherapy." This statement was met with backlash from every corner of the internet with comments such as: "Chemo is only for cancer," and "You cant be on chemo if you still have hair!" Upon reading these comments I was baffled by what little knowledge the general public had on the effects and uses of chemotherapy! Chemotherapy agents are widely used in the autoimmune disease world in both low doses: to help control an over active immune system, and in high doses: to completely eradicate the immune system when a patient presents serious organ involvement. Even in patients like Selena, who don't necessarily have any visible symptoms, chemotherapy still takes its toll on the body, and is a highly dangerous drug. At low doses the patient may experience nausea, malaise, exhaustion, minor hair loss, mouth sores, and possible damage to ones reproductive system. They may receive their allotted chemotherapy dosing through weekly pill or injection -- though you would never know it.

As an autoimmune disease patient myself suffering from systemic sclerosis (scleroderma), lupus, and vasculitis, I have undergone both low, and high dose chemotherapy. I received weekly injectable low dose chemotherapy for two years, and a weekly pill form for another year after that. At that low dosing I had significant hair thinning, (including my head, eyelashes, and eyebrows) nausea and fatigue a few days following the injection, and damage to my fertility. Most people around me had no idea I was getting weekly chemotherapy, but I was. No matter how low the dosing, the class of drug and what it does for the body doesn't change, simply the side effects are lessened. Chemotherapy is chemotherapy no matter how you receive it, or how much.

Since I now have serious organ involvement with a fatal prognosis I have been moved to the higher intravenous dosing of chemo, one that the society acknowledges due to its obvious side effects. I have lost all of my hair, become sterile, and spent long nights in nauseated misery in the attempt to abolish my malfunctioning immune cells. I must follow this regimen every 28 days for the next year, then be switched back to the low dose weekly chemo for the rest of my life. Chemotherapy never ends for autoimmune patients like myself. 

No, I do not have cancer, but I am going through chemotherapy. 
For some reason the general population cannot seem to comprehend this. They believe chemo is somehow not "real chemo" if it isn't attached to a cancer diagnosis. This is absolutely inaccurate. Our IV bags are brought into the room in the same yellow Hazardous Material Bag, labeled with the same large block letters that read "CHEMOTHERAPY" in all caps, by a nurse who must wear protective gloves when hanging the drug -- just as a cancer patient. 
Again, the fact of the matter is chemotherapy is chemotherapy. Please do not demean patients going through such an aggressive, and life altering treatments if you truly have no knowledge on the situation. The disease you are receiving the treatment for makes no difference to the actual drug, and certainly doesn't make chemotherapy any less "real" -- just ask any patient receiving it. Yes, in the lower doses you will receive far less of the harsh side effects, much less than the typical cancer patient, but that doesn't mean you aren't receiving chemo. You are simply obtaining a dosage that the world is not familiar with, such as Selena received. 

Every patient receiving chemotherapy has a heavy load on their shoulders whether it be for cancer, lupus, scleroderma, MS, PNH, or any other illness. We are all warriors just trying to win back our bodies

Thursday, November 19, 2015

The "Hopefully Soon To Be Rested" Tube Fed Wife

I just have a short update for the blog today! I saw my Sleep Specialist today and he had some interesting revelations for me. What I interpreted from the Sleep Study results as an overall data sum of the entire night was actually an average data sums per hour of the study. Meaning when I expressed in my last post that I awake due to Sleep Apnea 24 times a night, I actually wake 24 times an hour! Whoa! 

Sleep Apnea is a disorder where you stop breathing due to a collapsed airway while initiating, or maintaining sleep for any amount of time. This often send an alarm to the brain to awake, even if you don't remember waking. It highly disrupts your sleeping patterns and causes extreme restlessness. Essentially, I wake up about every 3 minutes resulting in less than 10% of the night in an actually REM sleep. REM sleep is the cycle that causes the complete paralysis of muscles, the suppression of brain impulses, and vivid dreams. This is the phase of sleep that results in a rested feeling if properly attained each night. You can see how this could cause some major issues; sleep is an essential part of the bodies healing, and emotional coping process which I cannot seem to successfully participate in due to this disorder. My Sleep Doc has decided to fit me for a CPAP (Continuous Positive Airway Pressure) device that will constantly initiate air pressure through my nose and mouth to keep my airway open. We will trial this machine for 30 days and record the data to see if I need to alter the air pressure being received, or if I need to move to a BIPAP with would initiate inhalation and exhalation. 

Next week is round two of Cytoxan... The day before Thanksgiving actually! Even though I cannot eat I do still truly enjoy the holidays, even if they are centered around food. I just really like hanging with family and friends! It's okay though, this treatment is so important and I can always celebrate the Holidays another day. The week after that I will be back to the constant appointment grind! lol Finally seeing my Autonomic Specialists about my recent POTS diagnosis the first week of December! Wow, December so soon! Funny side note, turns out I was wearing a mask the wrong week after chemo. I thought I was most susceptible to germs the week of chemo, turns out its the week after! Oops... I think I'll just wear my cool new VogMask the first two weeks after treatment!


Happy almost Thanksgiving! Eat some Pumpkin Pie for me! ;)

Sunday, November 15, 2015

POTS -- But Not The Kind I Keep In My Cubbard

There has been a pretty big transition in my life since I last posted. Unfortunately due to previous chemo damage, malnutrition wreaking havoc, and prior thinning from illness in general, my hair did not make it through my first round of Cytoxan. After stepping our of the shower last Friday I found myself with a large bald spot to the front right quadrant of my scalp. I decided to take this situation and make it one of empowerment, heading to the salon with my best friend and husband to shave my head. I had no idea what my head would look like under my hair, besides for the existence of an odd rash I have been battling due to my connective tissue disease. Thankfully as I sat in that chair watching my hair be sheered off a perfectly round noggin smiled back at me! lol I am actually kind of in love with my new do! -- Who says you need hair to be a confident, beautiful woman?!

My bald head, and a new friend!
Not much medically has been transpiring in my world as of late. I have been trying my best to stay away from the hospital for fear of germs since I chemotherapy has left me with a white blood cell count of 3... My poor little body! So far so good though, no colds, or even a sniffle has come from me! I have of course been continuing to get my weekly saline infusions however; gotta keep those fluids flowing! I did receive some test results from a few procedures I had preformed last month, the Sleep Study, and the Autonomic Nervous System Testing. While I have not reviewed the results with my physicians, I did get to read over the impressions from the interpreting physician.
The sleep study showed I have Moderate Sleep Apnea with my oxygen levels dipping into the high eighties. It also revealed that I wake an average of 24 times a night do to this condition, giving me barley even one hour in deep REM sleep. This would explain why I am exhausted constantly, and never feel rested upon waking. I see my Sleep Specialist this week to talk about sleep aid devices.
My ANS Testing unfortunately didn't have the greatest data due to my inability to preform a few of the tests adequately. Many of the breathing maneuvers that can indicate internal autonomic involvement were not done to a satisfactory enough level to produce data, this is because my lungs were to weak to execute them. The good news is that the tilt table test alone was able to give us a firm diagnosis of my dysautonomia subset: Postural Orthostatic Tachycardia Syndrome. What my physician thought was originally stand alone Autonomic Neuropathy due to my connective tissue disease, turned out to be a whooping case of POTS as well. POTS is a condition where postural changes, such as supine to upright, cause an abnormally large increase in heart rate (at least 30bpm.) Upon standing my vascular system has to work too hard to maintain blood pressure, and blood flow to the brain due to my faulty autonomic system which doesn't tell my vessels to constrict correctly. Patients end up having very low blood levels, or more specifically a decrease in the volume of blood plasma with positional changes causing: tachycardia, low blood pressure, and fainting among other symptoms. I have been prescribed quite a few different medications in an attempt to assist this condition, though the most helpful so far has been high doses of sodium; 6 Grams a day! This helps keep my plasma levels higher which wards off my fainting spells and severely low BP. I meet with my specialist the first week of December to discus the results in detail, and hopefully get a few more answers on how the POTS ties in with the Autonomic Neuropathy.

On set with the film crew!
On the calendar for me this upcoming week will be an appointment with my Rheumatologist to review my first chemotherapy labs, a consult with my sleep specialist regarding sleep aid devices, and of course some weekly fluids at the infusion center. Then, the week following I will have my second round of Cytoxan.
In other news, some wonderful awareness opportunities have been in the works lately!  I had the pleasure of filming a short documentary for a wonderful organization called Faith Counts this past week. The video will be in production over the next month and I cannot wait to share the finished product with you all! I also had the honor of speaking to class of nursing students at a college in my area. I was invited to share my personal Scleroderma experience with the future faces of patient care and couldn't have been more excited! It looks like there will be a few more opportunities to share my experience in this manner and I am really looking forward to it! I love speaking to medical students because I get to use all the acquired medical lingo from my many years spent as a professional patient -- and I don't even have to explain the acronyms! haha I cant wait for my next college speaking gig in January!

Wednesday, November 4, 2015

Living With A Terminal Illness vs. Dying

After rolling out of bed a disheveled mess I got ready to begin my daily medical rituals; I started my tube feeds, took a couple fist fulls of medication, did a few breathing treatments, rubbed myself down in Biofreeze, and then turned on my laptop. (The laptop obviously ins't medical, it just takes my mind of how badly I hurt in the morning.) It was going to be a good day! I mean how could it not? The first things I see when my computer whirs to life is my face on the front of one of favorite websites; XOJane. Next to me my little oxygen concentrator started beeping, alerting me that I had waited to long to take a breathe. (How embarrassing. I had literally forgot to breath out of excitement.) I took a nice deep breath through my cannulas, and got ready to read my article!
The title read:Why I still Want to Looking Beautiful, Even Though I'm Dying

...Wait a minute, what!? 

Dying?! What did they mean dying?! I know I am severely ill, fatally ill in fact, but "dying"? I mean, if we are being honest aren't we all technically dying? It's just common courtesy not to announce it right? From the second we are born our timer has begun towards our imminent deaths. Death is the one thing humans have yet to find a means of escape from. It awaits each of us at an unknown place and time. I do not classify myself as "dying" anymore than you would classify yourself as "dying." Now don't get me wrong, my death is in the carpool lane, while your death is stuck in rush hour traffic, but still, I wouldn't call myself dying. Yes, I have a terminal illness (Progressive Systemic Sclerosis) that will take my life long before I want to leave this earth. One that leaves me strapped to constant hospital stays, appointments, procedures, and treatments. One that leaves me in pain more often than not. One that forces me to tote an oxygen tank, a feeding tube, and a central line for survival. While yes, I reside in a body that is slowly failing, leaving my with a reduced time to live -- dying is still not the word I would use to describe myself.

December 2014
The reasoning behind my thoughts? (I mean, I am sure you are wondering what I consider dying if this inst it!) The truth is, I have faced death before; it was December of 2014. I was in every sense of the term dying, lying paralyzed from head to toe in a hospital bed while a nurse suctioned my spit so I didn't drown in it. There was little hope for a recovery after a sudden onset of an illness called Guillain Barre. Doctors were a constant ebb and flow out of my room on the Neurology Critical Care floor during a month long admission that nearly took my life. Family and friends had driven across the state to say their goodbyes which I have little, to no memory of. The bright lights of the OR, blood, pain, tubes, and a hopelessness mixed with shear terror as I faded in and out of consciousness are my only memories spanning over that hell of a 24 days.
There are plenty of mental scars (and a few physical ones too) from that month of "dying" that I encountered. Dying changes you as a person, changes your perception, and leaves a deep wound that can never be healed. I attended therapy for severe mental trauma to overcome the reality that I had in fact almost lost my life. It had placed me in a dark bottomless pit that I still now tremble to recall. The memories of Acute Rehabilitation where I relearning how to sit up, walk on my own, use my hands, and get dressed by myself at 23 years old haunts me still. The memories of medical procedures gone wrong (as in: botched spinal taps), and treatments that only hindered (as in: gave me Meningitis) instead of helped filled my dreams for months. Yes, I had in every way, shape, and form been "dying" last December. I remember what dying felt like. I remember the feeling of emptiness it brought to the center of my chest, the way it filled everything with blackness. The fear. I had never before in my life felt like I had lost a battle, but I knew that I had lost this one. There was no fight left in me, my body was too weak to continue, and my mind followed. While I lay paralyzed in that hospital bed, it was as if my ability to fight, to even think about continuing had been paralyzed as well. You can tell when it's the end, as your mind starts to slip, you can feel it closing in... and I was thoroughly surrounded. Somehow, someway I recovered. My body pulled through, and so did my mind... mostly. A piece of me was definitely lost during that admission, something I cannot quite put a finger on. Being that close to death, to actually be dying is unlike anything I have ever experienced, so dying is not an adjective I take lightly.

September 2015
Currently I am "living," and while my version of living has some extensive differences than the average individual, I still consider myself to be very much alive. I partake in shenanigans with my best friend (Hi Makanda!) every Friday night. I head to the craft store on Saturdays and buy 30 dollars worth of art supplies to make something I probably could have bought off Etsy for 7 dollars, and I go out on date nights to awkwardly watch my husband eat a cheeseburger since I cannot. (Which I find oddly satisfying!) If I was truly dying none of these things would be possible, living wouldn't be possible. I know this from experience. I no longer feel the pull of death like I did during December, no longer feel surrounded, and crushed my imminent demise. So no, I am not currently dying, because I know what dying actually feels like -- and this is definitely not it. I can still do things that bring me joy, still take part in activities that bring me happiness; when I was dying in December there was nothing but darkness and fear. I had completely lost my ability to do anything but lay hopelessly and wait for death.

I feel fortunate to live while my body slowly deteriorates, to not be bound to death like I was in December even though I am riddled with failing organs, and a fatal prognosis. Someday again I will be "dying," lying in a hospital bed with only days left ahead of me -- and truthfully yes, if you want to define "dying" as losing a battle to illness then I am in every sense of the term dying... but personally I like to call residing in this defective body, riddled by disease: successfully, and enthusiastically living.

Sunday, November 1, 2015

Cytoxan Round One - Health Update

After months of anticipation, preparation, insurance appeals, and tug-a-wars between my physicians I found myself wandering the hospital halls on my way to my first round of chemotherapy. From the moment we arrived at the infusion center I knew I was right where I belonged! The nurse walked us into a large private room which was unlike any infusion center I had seen at the local hospital I typical attend. Everyone on staff was extremely attentive and invested in their patients, you could just tell! The procedure was thoroughly explained to me before we began, and all the meds were described in detail, including a full list of side effects. Right off the bat we had a fabulous Port access which always sets a great mood in my opinion! ;) The infusion started out with some saline, followed by Mesna, a medication that protects your bladder from the harsh Cytoxan. (Without pre medicating with Mesna you will find yourself peeing blood, or ending up with bladder cancer.) About an 1-2 hours into the Saline and Mesna we started the Cytoxan drip which actually only last about an hour. The bag was brought in by a gowned and gloved nurse in a bright yellow bag that read CHEMOTHERAPY in all caps. It was actually very intimidating! She carefully hooked up the medication and we began! Everything went extremely smoothly and I had no reactions. After the bag was empty we ran more saline and another batch of Mesna. The entire encounter only lasted about 4 hours.

During the infusion I made sure to suck on some lemon drops which I heard can help nausea during chemo! I also kept myself busy with a nice big coloring book, and the company of my Mother and best friend! The side effects of the Cytoxan really didn't hit me until about 6 hours after the infusion. That night I suddenly become completely exhausted, as in 'could barley make it from the couch to the bed' exhausted. A small headache had begun, and a bit of a spinning sensation began to take hold. I quickly dosed up on some Zofran which is an anti-nausea med. Luckily this kept my vomiting at bay! There were definitely some nights where I woke up dry heaving over the side of my bed, but no serious vomiting took place. For the most part the few days after chemo were mostly filled with sleepiness, nausea, a slight headache, and some hives/itchiness. I laid on the couch and binged watch Netflix for a full 72 hours! haha Once I hit the fourth day I started feeling like myself again which was great!

To keep in mind: the dosing of Cytoxan I received was my "initial dosing," meaning a smaller than normal amount to simply introduce my body to this drug. I did not received a full fledged dosing such as the ones I will be receiving from this point on. As of my next infusion we will be moving up with the toxicity by increasing my dose, tripling what I had this week by my third infusion! As I am sure you have guessed each infusion will be harder, and more toxic, meaning each infusion I will get sicker, and handle it with less and less grace. While this round may have been "easier" than I imagined, the next few, especially the third and fourth dosing, will be extremely unpleasant. I am very lucky that my physician thought to work me up to the complete dosing since my body is so sensitive! I really believe this will help my body adjust so much better to this harsh poison. Unfortunately since my initial dosing was the 27th of October my next rounds will fall the the last week of every month, meaning the day before Thanksgiving and even Christmas Eve... I did not plan that well! haha

Since the infusion things have been pretty quiet. I have been taking it very easy, and being sure to wear a mask if I leave the house. I will have to head up to the hospital at the end of this week to take some blood panels and see "officially" how my body reacted to the medication. In the mean time I will continue to take it easy and probably stay at home for a few more days. One thing with Cytoxan is that you have to be filled with a ton of fluids for the first few days! They ask you to drink at least 3 liters of water a day to make sure you are flushing the chemicals out of your bladder adequately. Luckily this isn't too hard for me since I can pump water though my feeding tube all day! (Tubie win!) The only problem is that you have to pee constantly, and since you have a dangerous chemical passing through your bladder you are not aloud to hold it! So if I have to pee everyone better clear the way! lol

Speaking of my feeding tube, the balloon that holds it into my stomach popped this morning! The tube was just kind of falling out of my stoma which was causing some pain. Luckily I had an extra tube at home as I had recently scheduled an appointment to see my doc this upcoming week to change out the tube. Unfortunately it wasn't going to last that long so I had a choice to make: 1. Wait hours in the ER for a doctor to do it OR 2. Do it myself at home. (Yes, people do this all the time.)
I decided to go ahead and just do it at home in the end which was quite an adventure! I created a sterile area, put on some gloves and pulled the tube out of my stomach. Since the balloon was deflated it just kind of slid right out of the hole. It was a pretty odd thing to do to yourself I am not going to lie! lol Next came placing the new tube which was even weirder! I lubed up the tube (with KY because I didn't have any surgical lube haha), placed it into my stoma, filled the balloon and TADA! My new tube was in place. I was seriously so proud of myself! I'm not sure why it freaked me out so much in theory because it was actually super easy and painless! So now I have a shiny new tube and don't have to drive three cities away to see my doc for 15 minutes! Woop!

I even got to enjoy my Halloween! Mask, oxygen, feeding tube, tons of equipment and all. I was the "Diet" Kool-Aid Man. OH YEAH! Halloween is one of my favorite holidays because I absolutely adore making costumes! It can be tricky having medical devices though because everyone just assumes they are part of a costume. It makes for some really awkward conversations having to explaining that you are actually just really ill! lol We stopped by a Krispy Kreme Doughnut shop on Halloween because we passed one and Noel thought it looked yummy. The host asked us what type of free doughnut we wanted, and Noel and I were both like "Wow, everyone gets a free doughnut on Halloween!?" And the poor guy behind the counter goes "Yeah, anyone in a costume gets a free doughnut!" ....I was not wearing my costume. Like I said, awkward! lol But hey, I'll still take the free doughnut! ;)

Sunday, October 25, 2015

Lupron Side Effects - Health Update

What a week! Things did not go quite as planned after my injections last Wednesday afternoon. The Lupron, which was the injection to induce a chemically induced menopause like state to protect my ovaries during chemotherapy, unfortunately did not agree with my body. I found myself in the Emergency Department of my local hospital for some pretty extensive blood loss by last Sunday.

Essentially this drug was suppose to slowly transition your ovaries to a dormant state, normally the patient experiences things like hot flashes, minor cramping, and the loss of ones menstrual cycle. Side effects also include mood changes, night sweats, insomnia, diarrhea, and other typical consequences of menopause. Well, my body decided to take this transition very poorly and attempt to eject my entire uterine wall in 24 hours; you can imagine why this would be a serious problem. Neither my prescribing physician, or the ER doctors had ever heard of any patient having this effect from the drug, so they were quite surprised. They called a OBGYN who basically said it is an extremely rare side effect that happens to about .001% of patients... So of course I would get it! lol I am always that patient that receives the crazy side effects no one has ever heard of. Needless to say we will not be continuing the injection of this drug every month which will result in a 99% chance that I will become completely sterile from my Cytoxan treatment. Honestly, I am not feeling super upset about this. My husband and I have discussed the prospect of children greatly over the last many months, and also with my physicians. Pregnancy in a patient like myself would be an incredibly great risk, most likely resulting in my death, or in an innocent life riddled by birth defects. Right now pregnancy is the last things on our minds; our main goal is to have as many years together as possible. While yes, we both dearly wanted biological children, we have accepted the fact that it is not in the cards for our family. As long as it hold each other I know we will be just fine.
After the eventful weekend I was put on a high dose of the female hormone called Progesterone to counter act the side effects of the Lupron which have helped my body revert almost completely back to normal... Minus the continuing hot flashes. Hopefully the remainder of the month will continue smoothly as the rest of this drug leaves my system, unfortunately it resides in the patients body for approximately thirty days.

Thursday morning before my weekly saline infusion I saw my Primary Rheumatologist who was less than happy to hear I was unwilling to proceed next month with my scheduled dosing of Lupron. Even after everything that happened as a side effect of this drug she still believed I should attempt another injection to save my fertility. Unfortunately she is not accepting of my husbands and I opinion, or my other physicians warnings about pregnancy in a patient like myself. Honestly, she is still very frustrated with my decision to accept the Cytoxan prescription given to me by my world rewound Scleroderma Specialist from UCLA. While I understand her hesitation, truthfully, if I have a the worlds top specialist in my disease telling me to do something, why would I do the opposite? I immensely trust his judgment, and while I respect her professional opinion just as greatly, I truly believe he will lead me down the more aggressive, and victorious path. Our appointment was filled with a touch of animosity, which was a tad bit unbecoming, but something I am willing to deal with if it means I get to attack this disease head on with my UCLA specialist. She will still be my Primary Rheum, and is willing to keep an eye on me during my infusions, even transfer the infusions to her hospital if I would like, but all this was a bit spat out. At one point she even expressed that one of my other doctors would be "dissatisfied" with my decision. What she doesnt understands is that this wasn't "my" decision, it was a thoroughly thought out treatment plan by another physician that was prescribed while she wasn't even my doctor. I feel sometimes physicians forgot that it's not about "whose idea it was" or "what physicians they do or do not like," it's about giving the patient the best chance at living. Now, don't get me wrong, I know she cares exceedingly about my well being, I just don't think she grasps that this is my fight not hers... And I am ready to wage war. 

Cytoxan begins Tuesday. I will apologize now for the lack of updates that will ensue next week during my chemotherapy stupor. I have been told I will be quite physically ill for the first few days, and pretty miserable for the two weeks following. I will try to update everyone as soon as I can after next week. I also want to take this opportunity to thank everyone tremendously who assisted in the "Chemo Clean" of our small apartment. I am always so exceptionally humbled by the generosity of our family, friends, and neighbors. Your service will make an enormous difference in my health during treatment. Thank you, thank you, thank you! Please also excuse the back-up in email and comment responses. I hope to reply to everyone's well wishes and kind words soon! Hugs!

Friday, October 16, 2015

The Torture Table - Health Update

The weeks are slowly counting down, and all my pre-chemotherapy ventures are underway! My first round of Cytoxan will be October 27th, with another round following each month for the next 6-12 months. I am mentally preparing for this new chapter in my life, but also physically preparing. This week I had some preliminary vaccines, one is was the drug Lupron which will put me into a chemically induced menopause (yay for hot flashes! lol) in hopes to preserve my fertility, and the second was a Prevnar injection, conceivably to prevent me from contracting Pneumonia with my weakened immune system. I am also beginning small endeavors such as finding comfy chemo clothes, locating friends and family to thoroughly "chemo clean" my apartment before infusion day, and shopping around for some pretty head scarves! For more information on how this treatment is used in a Rheumatic disease like Scleroderma, Lupus, and Vasculitis (all three of which I have) visit this link: Cytoxan and Rheumatic Diseases, and for more information on the drug in general (as it is typically used for many, many types of Cancers) visit this link: ChemoCare: Cytoxan

Wednesday I had my second round of Autonomic Nervous System Testing. As a quick recap, I recently became a patient of a renowned Autonomic expert who has taken great interest in my case. He referred me to redo the autonomic testing I had done a couple years ago so he could have updated data, which will lead to a more accurate diagnosis. This particular set of testing is actually the least favorite out of the incredible list of procedures I have preformed. It is not invasive, and it is not particularly painful but, it takes a massive toll on the body of someone with Dysautonomia. The test last around three hours and includes being strapped to a table the entire duration with multiple blood pressure cuffs down your arm, all the way to the tip of your finger. There are three distinct portions of the test: 

  • 1. The Quantitative Sudomotor Axon Reflex Test (Qsart), which evaluates the nerves that regulate your sweat glands in different areas of the body. This portion is actually slightly painful as the test feels much like you are being rubbed with stinging nettles down the entire left side of your body for 5 minutes straight. 
  • 2. Breathing Tests composed of Valsalva Maneuvers to measure how your heart rate and blood pressure respond to forceful exhaling. I am always bad at this portion since my diaphragm is so weak; while patients breathe out to typically get a small dial to reach 40mm, I was unable to get it to reach even 15mm... so we may not have enough data to accurately diagnose anything from this portion. 
  • 3. The Tilt Table Tests (or as Dysautonomia patients call it: The Torture Table Test) which monitors the blood pressure and heart rates response to change in positions. While strapped to the table you are lifted from lying flat, directly to standing. While this would not cause any issue for someone whose autonomic nervous system responds correctly, compensating for this positional change by constricting vessels and redirecting blood flow, a Dysautonomia patients response does not work leaving the brain void of blood and their heart racing uncontrollably. Typically patients with Dysautonomia become severely unstable during this procedure with a heart rate ranging anywhere from 150-200bpm, and lose consciousnesses almost immediately upon being positioned upright. 

The Torture Table in all its glory.

You can see why I loath this test whole wholeheartedly. During my performance of the Tilt Table I blacked out, then attempted to compose myself with just enough time to not choke on my own vomit. It is truly a miserably three hours that I hope to never have to endure again... though that's what I said last time! Let's hope my doc has enough data for that to really be my last go! Results will arrive in December. As a comical side not, while preforming the test special electrode type stimulators are put on your feet. For some reason they just wouldn't stay on my so the tech had to re-adjust them multiple times. Finally in frustration she said, "Has anyone ever told you your feet feel very hard? Like extremely bony almost?" I tried not to laugh ;) Why yes, yes they have! Apparently these particular electrodes do not like hard Scleroderma feet! lol We ended up having to bring in another tech to hold the stimulator to my foot while the original tech ran the test. Oh the life of a Scleroderma patient!

Thursday morning I saw my Scleroderma Specialist form UCLA; I feel extremely lucky to be in his very competent care. He has made such a vast impact in my treatment plan, and hopefully the longevity of my life. During the appointment we went over some recent procedure results, did a physical exam, spoke about my upcoming Cytoxan Infusions, got some baseline labs drawn, and lastly made the decision to prescribe my fist sleeping medication. It has been months since I had a restful night; I have both trouble initiating and maintaining sleep, for this reason he prescribed Trazodone. This is commonly known as an antidepressant, but has been found in small doses to help insomnia greatly. Hopefully this drug will help get the permanent bags under my eyes to recede! lol I never really know the full outcome of his appointments until his visit summery arrives in the mail, which typical takes about a week. This particular physician does things a bit differently than my others, so I will have more to update about this appointment in upcoming blog posts.
That afternoon I headed off to my Primary Care Physician to get my Lupron and Prevnar vaccines. I must say, I was not expecting the Lupron to be as bad as it was! When the nurse pulled out the "kit" containing the administration device it honestly looked quite similar to a spinal tap! The needle is extremely long in length since it is an intramuscular shot, meaning the gel (yes it is more a gel than liquid! Ouch.) inside the syringe must reach deep muscle tissue, not just the top layer of fat like most injection. Unfortunately this particular vaccine also contains a TON of fluid, meaning the duration of injection time is long, long, long! And since it is more of a gel than liquidy it cramps the muscles horribly! Dang. That was one un-fun shot! I'll be sitting on one butt cheek for the next week! haha Thankfully I only have to get this done once a month! Next came the Prevnar (Pnemonia Vaccine) which was a walk in the park, just your typical shoulder shot. For some reason we were in the pediatric room so I got some pretty sweet pink band aids and a Batman sticker! haha ;)

Friday morning has rolled around and I am feeling pretty beat. My body feels as if someone had popped all my joints out of socket while I slept, and beat the rest of my with a bat. Urg. I am guessing my poor body is just overloaded with foreign substances after yesterday and is fighting back. Boy am I miserable today. Absolutely ever inch of me is wreathing in pain. I did have my weekly saline infusion this morning which seemed to take the edge off the lovely migraine I woke up with at least. Hopefully tomorrow will be a better day!

A very exhausted Chanel.

Friday, October 9, 2015

Houston, We have Liftoff! - Health Update

Term: Spoonie
Definition: A person living with chronic illness.
Origin: The Spoon Theory written by Christine Miserandino
About: The spoon theory is an analogy used by patients to describe their living experience when their illness results in a reduced amount of energy, compared to that of a healthy individual. Spoons are an unit of measurement used to track how much energy a person has in a day. In the event someone runs out of spoons they are unable to continue with their day. 
Example: "I am running low on spoons so we will have to reschedule."
               "I have to save spoons for tomorrow so I should stay in today."
               "She is a spoonie like us, suffering from disease or disability!"

I am sure some of my readers, and even family members have heard me mention my "spoons." I wanted to give you all the definition and origin behind my odd chronic illness lingo. The link is in pink above for you to read the Spoon Theory in its entirety. I adore this analogy, and so does my husband lol It is a tangible way for him to express to me when I have overdone it and am too stubborn to slow down! ;) It's a term you will hear often in our home as we even have a spoonie cat! Our little Apocalypse (yes that's his name) has multiple chronic illness so we often tell him "you ran out of spoons!" when he is tired and grumpy. Our week started out with our poor spoonie kitty at the vet actually! He was battling his mamma for most appointments of the week! (Three in total... so he didn't quite beat me this time haha) He is doing well and on the mend after some infections took a toll on his tiny little immune suppressed body. Here is a picture of him! I realized I have never posted a picture of my darling little Abyssinian kitty. 

He helps me write all my posts.

I had a total of four appointments this week, nothing to out of the ordinary, though I did get to catch up with my Rheumatologist after getting the devastating news that she did not want me to move forward with the chemotherapy I had been prescribed by my UCLA Scleroderma Specialist. I patiently awaited my appointment with her which was of course at the very end of the week! (Talk about being on pins and needles!) If you didn't catch my last post, this one may not make a lick of sense so feel free to go back and read "New Doctors & Diagnosis - Health Update'" to catch up on the Cytoxan fiasco! In a nut shell I was prescribed an aggressive chemotherapy in August which my insurance STILL hadn't approved by October. October came along and my Primary Rheumy (not my world renowned specialist that prescribed it) decides she wants to use another drug instead! Flash forward to the day after I receive that news: my insurance approval comes in the mail! I was approved for my Cytoxan Infusions to start immediately! (Houston, we have liftoff!) You can see the dilemma, and sheer disappointment. Right when I FINALLY received the great news that my new treatment was approved, I also get hit with the fact that my main Rheum may not agree with it! To save you the anticipation I had a meeting with her yesterday, a long two hour appointment where both myself, my husband, and her discussed my treatment options, and the reasons she has hesitancy to use this drug. We hashed out everyone opinions, and the other options that she was interested in: Actemra (an RA drug used to fight inflammation) and Cellcept (a chemotherapy in pill form used to suppress the immune system). I have previously been on Cellcept (twice actually) both times having a poor experience; while Actemra didn't seem to have any promising case studies when it come to lung fibrosis. While Cytoxan is very dangerous, it has incredible benefits for the lungs, skin, and even the GI tract! You cans see why I would want to experiment with Cytoxan even though it is a highly toxic drug. Its effects improve more organ function than the rest, it's the one that I have yet to try, and it is the strongest drug used to combat autoimmune disease. I feel confident that after the year of heightened progression I have endured that it is time to undergo this treatment and give my disease a real run for its money! 
The only draw back is that this is also the same drug used when a patient undergoes a Stem Cell Transplant. If you have reached toxicity to this drug you may lose your eligibility to preform the transplant, which my doctors absolutely believe is in my future. My compromise is that I will undergo the Cytoxan, which my UCLA doc prescribed, but for six month instead of a year, then reevaluate. That way I still have the potential for a transplant since I will have not reached toxicity by that time.
My Primary Rheum was pleased with this compromise and expressed that while she is in no way against using the Cytoxan, she will just hate to see me go through all the horrendous side effects of chemotherapy when there 'could possibly' be other options (though there is no guarantee those other options will work, and could set me back to where I am too sick to complete Cytoxan in the future). Hair loss, nausea, vomiting, bone pain, sterility, heightened infection risk, and sheer exhaustion will be the next six months of my life, if not more. Luckily since I am young there is a drug that can potentially protect my ovaries from the drug effects, though it's not a guarantee. It will put me into a state of induced menopause while I undergo treatment in the hope that one day I will be fertile. The drug is a once monthly butt injection. (Goodie... showing my bum to nurses is not my idea of a good time.) So if all goes as planned next week when I see my UCLA Scleroderma Specialist on the 15th we will be starting Cytoxan just before Halloween!

My other appointments this week consisted of a Pulmonary Function Test, a meeting with my Pulmonologist, and of course my weekly Saline infusion. My PFT stayed more of the same so nothing new to really report there. My Saline infusion however was a different story! I have something called an implanted Port (click for info/photos). This is a device embedded under the skin above the breast for patients who have difficult IV access, or need constant IV access. It is accessed by a hollow one inch needle pushed through the skin into the Ports reservoir. This is actually about ten times less painful than starting an IV I promise! As long as you can get passed the fact that a giant needle is coming at your heart area, you're good! lol ;) Well my port is a bit jumpy since I don't have much... um... "fat" in that area of my body. (small boob joke!) So if not anchored (held in place) properly my port will slide to the side when accessed resulting in a giant needle being plummeted into my chest cavity instead of the device. This hurts. A LOT! Well as you can guess the reason I am telling you this is because this happened. Twice. I was bloody and bruised and not a happy camper! I kept explaining to the nurse that she really had to grab it hard, but I could tell she was hesitant to hurt me from grabbing too tight! After the second miss we called down an Oncology nurse that was very familiar with ports to access me. She got it in one painless swoop! Woohoo! Needless to say I will be wearing high necked shirts for a while until my breast looks less beaten!

Notice the Spoon necklace! ;)

In other super awesome news I started writing for a new media outlet! XOJane is an online magazine for woman to be their "unabashed selves!" It's an outlet I have been following with posts written by woman from around the world and all different walks of life for a while, so when the opportunity arose to write for them I couldn't have been more excited! I was recently their cover feature in an article they titled: Why I Still Want To Look Beautiful, Even Though I Am Dying.
I had to giggle when I saw the edits they had made, especially the title. Not what I would have chosen, but hey, that's the life of a freelance writer! haha I'm just so honored to be part of the writing team! Next week I have a full day of Autonomic Testing, a Saline infusion, and an appointment with my Sclero Specialist from UCLA. I'll be sure to update everyone next Friday! 

Saturday, October 3, 2015

New Doctors & Diagnosis' - Health Update

With all the fun I've been having "opinion" blogging recently I realize I haven't done a health update in about two weeks! If you missed it, I recently wrote a few pieces for The Huffington Post and The Mighty! My favorite of which was a feature on The HuffPost Healthy Living Blog called "An Open Letter To The Person Staring At My Medical Devices" This article was a great way to educate individuals on respectful conduct when it comes to meeting patients like me out in public... and guess what? Staring is actually okay... I just have one request! Follow the link above (in pink) to take a read. 

The last couple weeks have been an amazing step forward in my treatment! I started my weekly Saline Infusions which add some extra fluid and salt to my body as it cant seem to retain them currently. This issue could be a by product of a few different causes: 1. Autonomic Dysfunction 2. Malabsorbtion or 3. An Endocrine Malfunction. Currently we haven't pinpointed exactly why I am not retaining the salt my body needs to function, but upping the dose is a good step forward no matter the cause! So every Thursday I drive myself down to the local hospital to be infused with sweet saline! Being well hydrated does the body a world of difference!



I also had my long awaited appointment with Washington State's top Dysautonomia Specialist! This doctor deals with malfunctions of the Autonomic Nervous System, which regulates all the bodily functions not consciously directed, such as breathing, heart rate/beat, blood pressure, temperature regulation, papillary changes, dilation of blood vessels, urinary/deification regulation, the digestive process, and much more! Over the years since I have started showing symptoms of this complex systems malfunction I have received a plethora of diagnosis all falling under the "Dysautonomia" umbrella; Postural Orthostatic Tachycardia Syndrome (POTS), Pure Autonomic Failure, Vasovagal Syncope, and Orthostatic Hypotension, were just a few that many forms my physicians expressed were possible. After a few months seeing each physician they would express their uncertainty in diagnosis and send me to another doctor. The ANS is complicated, and elaborate in its working, composed of two main nervous systems; the Sympathetic Nervous System, and Parasympathetic Nervous System. Each system is responsible for basically the exact opposite function, one induces the bodies "fight or flight" response, and the other induces the "relax" response. Each separate form of Dysautonomia affects the patient differently, though treatments are very often all the same. Receiving the correct form of diagnostic form is helpful however, as some forms can be more aggressive and require extensive treatment. After going over my symptoms, and doing a few in office exams over the period of two hours this amazing physician was able to express a form of Dysautonomia that matched every one of my symptoms; Autonomic Neuropathy

According to The Mayo Clinic: "Autonomic Neuropathy occurs when the nerves that control involuntary bodily functions are damaged. This may affect blood pressure, temperature control, digestion, and bladder function.The nerve damage interferes with the messages sent between the brain and other organs. Signs and symptoms of Autonomic Neuropathy vary based on the nerves affected. 

  • Dizziness and fainting when standing caused by a sudden drop in blood pressure.
  • Urinary problems, such as difficulty starting urination, incontinence, difficulty sensing a full bladder and inability to completely empty the bladder.
  • Difficulty digesting food, such as feeling full after a few bites of food, loss of appetite, diarrhea, constipation, abdominal bloating, nausea, vomiting, difficulty swallowing and heartburn.
  • Sweating abnormalities, such as sweating too much or too little, which affects the ability to regulate body temperature.
  • Sluggish pupil reaction, making it difficult to adjust from light to dark."
These are just a few of the most clinically found symptoms of Autonomic Neuropathy, though it is certainly not limited to this list. Essentially it is the total failure of the nervous systems that control your body's functions. This dysfunction can be caused by many different sources: Diabetes, infectious diseases such as Lyme, direct injuries to the nerves, or in my case an underlying autoimmune disease that has caused irreparable nerve damage. There is a particular form of Autonomic Neuropathy called "Autoimmune Autonomic Ganglionopathy" that is actually caused by the bodies immune system releasing an antibody that directly attacks the ganglion receptor responsible for these systems. This is extremely rare and is characterized by the same symptoms as regular AN . The diagnostic criteria is a specific antibody found in the blood. Though currently my specialist believes that is not the case for me, he is interested in running the blood work since I have severe Aidie's Pupil (pupils fixed in dilation), a typical attribute of that particular form. He has also requested that I preform the classic Autonomic Function Testing (list of procedures found in pink link) within the next month, along with some biopsies of my feet to asses the extent of damage done to my nervous system. Once we have fully evaluated the situation and have a firm understanding of my case we will be starting a new medication that helps control these systems called Mestinon.
And if you are wondering, yes, having pupils that are constantly dilated is really uncomfortable! Bright lights bother me extremely, I cant focus on things close up for long periods of time, and I have near constant headaches because of this. It's honestly a wonder I can blog or use my phone! haha I take lots of breaks to re-affix my eyes. 

Last, and definitely most exciting, was the appointment with my new (but actually old) Rheumatologist! Back in August 2014 I received the devastating news that my favorite Rheumy, and really the only one who has ever made any progress with my case, was leaving the practice I attended. After a year away she has returned and I couldn't be happier! It's been a hell of a year full of absolutely horrible physicians that treated me with not one ounce of respect, or cared enough to appropriately treat my aggressive disease (besides my UCLA Doc). When I received the news she was returning I waited patiently for months to covet her first appointment! I called every week in September to secure a spot asap! lol I am sure the scheduling desk hates me ;) But I am happy to report my dedication payed off and I was the first patient she saw back on her first day in clinic! 
Withing the first second that office door opened we were both all smiles! It was so refreshing to see this familiar face! We met for about an hour which consisted of reviewing the past year since she has been gone. That was quite a whirlwind of emotion, especially when I had to recall all the events of my month long hospitalization in December that nearly took my life. I think she was surprised to see how far I have progressed within such a short time frame, but was still very grateful that my skin has continued to stay 'relatively' stable-ish! She too believed it was time to change my treatments and move onto a drug that will help stabilize my organs, though she was a bit on the fence about the use of Cytoxan (the chemotherapy my Scleroderma Specialist prescribed, which STILL has not been approved by my insurance yet! Urg.) She wants me to try a drug that I have already been on called Cellcept once more before we pull out the harsh Cytoxan treatments. While I understand her hesitancy to give this dangerous drug, I have a few rebuttals: 1. I have already been on Cellcept and I believe in moving forward, not backwards 2. Cellcept takes three months to make any clinical difference in the body (which is three months too long if you ask me) 3. I would much rather hit this disease hard, then move to trying drugs over again once my immune system is reset and more willing to accept them. Overall, my organs are showing heightened progressing from month to month, and at this rate in three months I will be in rough shape. I am not willing to risk that I may be too weak to preform Cytoxan at that point; I want to undergo treatment now while I am still relatively strong. I explained all this to her and she definitely took my opinion into consideration. We both agreed that she would need to get herself thoroughly caught up on my case reports before we agreed to any treatment alterations, and that she would need to speak to a few of my other physicians before we made any set in stone commitments. I will follow up with her next week after I undergo yet another Pulmonary Function Tests, which will also give her the opportunity to contact the physicians who have been treating me in her absence. 
My only worry is that she will have to speak to the care provider that treated me so horribly back in August since they work in the same clinic. I can almost guarantee he will disagree with anything I say solely because I am a "depressed and moody" young woman in his opinion. I did explain the situation that occurred with him, and she acknowledged my frustration which was reassuring. Hopefully next Thursday I will have a solid game plan for the next month or so; I am so tired of playing the waiting game! I feel it is all I do these days... but at least it's only one week this time! 

I am so grateful to have a physician back in my life that I feel truly cares for me emotionally, and physically. I am not simply a number on a chart to her, one that can be swept under the rug till the next appointment. I can tell that she dedicates her time in, and out of the office to her patients, and that is why I love her so much as a care provider! True dedication to sustaining individuals lives. 
One of the funny things she mentioned in office today was, "I still haven't met anyone that loves their feeding tube as much as you!" It made me smile! I really do appreciate the device that supports my life. The day I received my feeding tube was the day I received my life back! I was a 5'10 122lb sack of skin and bones that constantly looked like I was on deaths door step; too miserable to even get off the coach to throw up. Now look at me! I may still be a medical mess, but I am at least a somewhat functioning medical mess... one who even leaves the house occasionally! ;)