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.

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!

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.

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,  also I was approved for my Cytoxan Infusions to start immediately! (Houston, we have liftoff!) 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.

 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! 

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! 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.
 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!