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! ;)