Thursday, May 30, 2019

My Last Days: Behind the Scenes

Over the past two years I have been in contact with the wonderful team that produces the revolutionary documentary: My Last Days. A beautiful series aimed at sharing the stories of individuals facing, or that have faced, terminal conditions. It generates positive conversation surrounding hard topics and uplifts the community by expanding their understanding of joy and purpose. In early 2017 Justin Baldoni, the visionary of this production, reached out via an Instagram DM (I was shocked to say the least lol) asking if I would be interested in participating in their show. I found the thought intriguing and terrifying.

I have openly shared my health struggles for years in hopes of conveying an accurate portrayal of daily life with disease, as well as to generate positive, relatable content for patients enduring similar struggles. To my benefit, I found sharing to be an incredible decompression emotionally; no longer solely carrying the yoke of my health issues, instead, adding the support and wisdom of other patients to ease the heavy burden. However, the thought of purposely sharing my journey on a multi national television station was daunting. Would my story be portrayed correctly? Would my message be depicted authentically? After many video chats, phone calls, and text messages between myself, Justin, and the director, I decided to participate in hopes of garnering awareness for my rare condition and to broaden the understanding that joy can be found even while enduring difficult situations.

To watch my full episode click here: My Last Days Season 4 Episode 2 
Unfortunately, The CW limits their content by country. To Watch outside the United States you will need to use a VPN!

...Before we begin, let's get a few things out of the way. Noel and I have been married for just under 8 years. Yes, we really did get married at 19. (Happiest day of my life!) We both are currently 27. Yes, I am really tall. 5'10" Yes, I have lost a TON of weight since filming. lol Unfortunately, it is medically weight loss. Booooo! Remember in the episode when we explicitly talk about, "the door is closed, but at any moment that could be our life again?" Well, that door is open. Wide open. I recently had a relapse of my conditions. The beautiful valley of remission I experienced post transplant is far behind me. Only time will tell how fast things will hit the fan this time around. If you remember, it took a couple years for my lungs and heart to really crap out post diagnosis. True to my initial experience with this horrible disease, it has taken my digestive system first. I am back on a feeding tube, a Gastrostomy-Jejunostomy (GJ) to be exact. I currently run continuous feeds into my Jejunum since my esophagus and stomach no longer function enough to sustain human life. Enemas are also a staple due to severe pelvic floor disfunction. I am undergoing a wide array of medical testing to asses what other organs have been effected by this relapse.

Filming began in March of 2018, and ended in August 2018. My episodes director, Farhoud, was a passionate, kind individual who truly wanted to share my quirky and tenacious personality with the world. While we only filmed a total of about four days, they captured me, my outlook, and my personality magnificently. Because the show is produced as a documentary, the filming schedule mostly consisted of following my family and I to activities and conducting personal interviews. Including one trip to LA in Aug 2018 to film the round table discussion. The crew honestly became family. We ate meals tougher, cried together, and laughed together. Their dedication to generating an authentic story which showcased the genuine disposition of their subject was insanely amazing.

I figured I would take this post to expand on a few scenes from my episode. Remember, we filmed for 4 straight 12 hour days, and only produced an 20 minute episode. That's a lot of unused conversation!

My Diagnosis(s):

When interviewing me to explain my health conditions, an hour long conversation was cut to five or so minutes. Please be aware the five minutes aired on television don't even begin to scratch the surface of my journey with a debilitating, life limiting, horrific disease. My history is quite vast, just ask the docs who can tell my chart by the sheer thickness and number of volumes! lol Due to the final cut, the explanation of my condition was not quite portrayed in full. I have an overarching autoimmune condition called Mixed Connective Tissue Disease. This condition encompasses three autoimmune diseases with a specific antibody (Anti-U1 RNP) that creates a different line of progression than if you had the three diseases individually. The diseases include: Systemic Sclerosis (Scleroderma), Lupus (Systemic Lupus Erythematosus), and a Polymyositis. These conditions also cause multiple comorbidities (other diseases) because of the damage they cause to my body. For example, Systemic Sclerosis (Scleroderma), which results in widespread connective tissue and vascular damage can cause Pulmonary Fibrosis, Pulmonary Arterial Hypertension, Gastroparesis, and heart disease, among others. Lupus, can cause conditions such as arthritis, Vasculitis, and neurologic damage, among others. For me, Systemic Sclerosis has been the most damaging of the three encompassing diseases, wreaking havoc on my internal organs. The treatment for these diseases consisted of heavy immunosuppression which resulted in a severely weakened immune system. Due to my severely weakened immune system, when I become sick with a common cold I get very, very sick. My immune system does not know to handle the remedial task of fighting off foreign invaders and causes severe immune reactions, such as my run in with Guillain Barre in 2014. I don't talk about that particular incident much, as it was the single most horrific few months of my life. It was also the first time doctors told my family, "you need to say your goodbyes." Friends and loved ones literally drove across the state to hold my hand one last time. Needless to say, it was an emotionally scaring event myself, nor my family care to re-live. 

My Transplant:

While the variance was not addressed in the episode, you will notice the clinical trial I underwent was called by two names: Bone Marrow Transplant (BMT) and Stem Cell Transplant (SCT). Think of it like dog breeds. I could say, this is a dog, or, this is a Poodle. They are both dogs, simply, one denotes its breed. Similarly, I can say I had a BMT, or be more specific and explain the type by expressing, I had an SCT. These are interchangeable names for an overarching procedure in which a patient has their old immune system completely eradicated via copious amounts of chemotherapy with a coinciding new, immature immune system transplanted in. This is an incredibly grueling, long, and exhausting process. A Stem Cell Transplant is a subset of Bone Marrow Transplant where the hematopoietic cells are harvested peripherally instead of through direct bone marrow aspiration. This is the most commonly used method of harvest in modern medicine. My particular BMT was an Autologous Hematopoietic Stem Cell Transplant (HSCT). For more information on why this was considered a clinical trial and how it is a beneficial, yet dangerous proceeded for a treatment resistant autoimmune patient, please follow the link here: Autoimmune and HSCT

My Memory:

As a result of the detrimental levels of chemotherapy I endured over the years, I experience distressing levels of "Chemo Brain." This condition happens as a result of brain cell death from the chemotherapeutic drugs which can affect a patient long after treatment has halted. Chemo Brain can be transient or permanent - passive or aggressive. These neurologic side effects of high dose chemotherapy are common in patients, however, mine seemed to be a thicker more aggressive fog than was clinically expected. I found myself experiencing bouts of delirium level confusion, long and short term memory loss, even stuttering and dyslexia. Symptoms persisted well past my first year post HSCT. I went from someone very poised with an impeccable memory, to someone who had trouble with everyday cognitive tasks. While I consider this a minimal symptom of my treatment, and therefor my condition, my talented director found it a beneficial topic to explore. While I seem graceful and well spoken in my episode, the team had to cut out hundreds of, "what was I saying? What was I talking about? Etc." Honestly, I am amazed they made it all so fluid! (Seriously, they are so incredibly talented!) For example, the one scene where I say, "I can't think of the word," seems very stark. In reality, this was after me throwing out a bunch of words and stumbling for a solid 15 minutes on how it felt to forget. The word I so desperately fished for: frustration. 

The Sandwich:

Yes. I ate a Subway sandwich immediately after being released from the isolation unit on the oncology floor. I. Do. Not. Recommend. This. It was a dumb decision, one that could have cost me my life. Upon regaining my ability to swallow after being tube fed for almost three solid years all I wanted, all I craved, was a generic sub sandwich. You guys. I LOVE sandwiches. I straight up dreamt about them while pumping unflavored nutritional formula straight into my digestive tract for years on end. So, immediately after receiving the hour long discharge lecture from my physicians, being handed the 'life post-HSCT novel' on diet precautions, human interaction precautions, and other insanely strict precautions to memorize, I broke EVERY (I mean EVERY) rule and went straight to Subway. Located in the hospital lobby! lol Thankful, I was okay - but some patients aren't so lucky.

What you experienced in the joy-filled, beautiful 20 minute documentary created and produced by the talented crew of My Last Days is simply a glimpse; a tiny fragment of compacted stories from a grueling battle. Remember that the final product is an orchestration of my life. It does not cover the still and constant struggle while working a full time job to afford reliable health insurance. It also does not reflect my current medical situation, which includes the reintroduction of a surgically placed feeding tube due to relapse. It does, however, exquisitely and unequivocally share the joy and reverence I feel for this beautiful mess I call life. I could not ask for a better portrayal of  such a miraculous chapter of my life!
Thank you to Justin, Farhoud, Ahmed, Krista, Sarah, Cat, Drew, Scott, Jamey, Josh, and everyone else who made this possible and for your dedication to create an authentic and uplifting glimpse into Noel and I's bonus years! I will never be able to adequately express my gratitude for the gift you have given myself and my family. I also want to take this time to acknowledge the other cast members. Travis, who is in chronic rejection from his second double lung transplant and is now waiting on a third - and of course the inspiring cast members who have passed away since filming began; Jessica, Shannon, and David. Thank you for spending your precious limited time sharing your messages of hope and tenacity with the world - and thank you to their families for their willingness to share their loved ones with us all. I am so humbled to be a member of the My Last Days family.

My Last Days Season 4 aired May 22nd 2019, May 29th 2019, and will wrap up June 5th 2019. My episode is featured the second night, May 29th at 8pm. If you missed an episode, don't worry! Simply go to The CW's website or the Google play store / the App store and download The CW app. (It is free!) Choose My Last Days and enjoy! Each episode will be on the app/website the morning after it airs. The episodes in order are Jessica & Travis, Chanel & Shannon, Dave & Discussion.

For all previous seasons and episodes, click here: The CW Shows- My Last Days

Friday, May 24, 2019

Transitioning Back to Feeds

TMI Warning. This post is gunna be a bit graphic.

After returning home from surgery I noticed my stoma (medical hole that the tube resides in) became very aggravated. It was red, raised, and becoming more painful instead of less. Suddenly, it started oozing thick green puss. It was like a faucet of goop. I would soak the gauze pads made to last a day in 2-3 hours. Over the course of two days the discharge became do bad it would leak down my stomach. I started to become nauseated, flushed, and was completely unable to touch the tube without immense pain. We headed straight to the ER where I was diagnosed with a heavily infected stoma. So puss, thick green puss is what was oozing out of me. Gross. I was placed on round the clock liquid antibiotics through my tube. The next few days were absolute agony. Luckily, after day four of the antibiotics, things began to look up! The discharge came to a near complete halt, the pain subsided, and the stoma looked about one hundred shades less red. Hoping I wont need a second round of antibiotics, but am preparing for that just in case. My docs all agreed the infection was likely a result of being hospitalized right after the tube was placed for my seizures. Urg. Isn't it sad that the hospital is actually less safe than ones home post surgery? So weird. 

I saw my GI doctor for surgical follow up Thursday who was very pleased the infection seemed to be letting up. She explained to me that she spoke to the surgeon who placed the tube and he expressed, "I placed the tube in a very painful, thick part of the stomach. Since it is a GJ tube we wanted the G as close to the bottom of the stomach as possible so the J line would have less a possibility of flipping back into the stomach." So, this explains why I was in so much more pain compared to my first feeding tube surgery in 2014. I was a bit worried about a large slit in the stoma, which no one can really explain. Stomas should be completely round and fit snuggly to the tube. Mine however, is round on one side and completely slit like an arrow on the other. This causes the stoma to be a bit too big. The theories are, I ripped the area while having my seizures post surgery. Another, Urg. We stitched it up and are hoping it will heal correctly. 

The tube I have currently is different than my first tube back in 2014. This tube is a Gastrostomy-Jejunostomy, or GJ tube. This means I have access to both my stomach and Jejunum (small intestines). The tube is placed in the stomach but has an internal attachment that bypasses the stomach and goes deep into the Jejunum. Since my stomach is functioning so poorly, I cannot longer run feeds into it - the G part of this tube is mainly for medication and venting. The J is where I run the formula that feeds me. As the intestines don't expand like the stomach feeds must be run very slowly - I am on continuous feeds to receive enough calories. 

My GI doc is so cute, I came into her office and she goes, "You got a new backpack! Where is your Vera Bradley?!" She remembered the old backpack I used to wear when I had my G tube. lol I just love her! Luckily, right now I can still drink - very slowly. As you know, my esophagus backs up if I try to put too much into it at once. She always recommends her patients occasionally try what she calls, "recreational eating." This essentially means, attempting small bits of food/drink to see what our bodies can/can't handle. With me, that's tricky since my esophagus sucks so hard - so it will really only be soft, non thick foods. Many patients can't "recreationally eat" at all, but some can. It's considered recreational because it is simply that, just for fun - it isn't enough to sustain human life. She said to be smart about it. If something gets lodged or I get super nauseas/bloated or vomit, obviously stop! lol So, hopefully in the future there might be a few things my body can handle for fun - while my tube sustains my nutrition. 

Mentally I am in a much better place than I was two weeks ago. I feel like I am done wallowing. Hallelujah! With this infection almost behind I am starting to feel like me again. I would say I went from coping at about a D+ to a B+ in the last ten or so days. It is for sure a weird and frustrating transition. I ordered all the supplies I need to feel comfortable and confident, organized all my supplies, got down my morning, afternoon, and evening routine, and finally feel less burdened by this device. While it makes life a thousand times trickier, I know it is what is going to keep me alive. Frankly, last month I was starving to death - now I am slowly recovering and receiving the desperately needed nutrition I was devoid of. My blood work is still all out of wack, but that will all recover as my body begins to transition to feeds. Good news, I am back to work - with tube in tow!

Wednesday, May 15, 2019

Surgery and Seizures

Sunday evening we received a call around 8pm for my gastrointestinal surgeon. They had a slot open up Monday morning and asked if I could make it. Noel and I quickly made arrangements and rushed over to the hospital bright and early for placement of my gastro-jejunal (GJ) feeding tube. Due to the recent decline of motility throughout my digestive tract a feeding tube once again become necessary to receive proper nutrition. The placement of a tube is typically a quick procedure, patients either go home immediately post procedure or stay overnight if they are unfamiliar with tube feedings. I was scheduled to head home post surgery since I have vast experience with feeding tubes. However, things did not go as planned. The anesthesia team decided to add an inhaled anesthetic, Sevoflurane, as compared to only the IV anesthetic. Unfortunately, this drug in rare cases induces seizures. While coming out from GA I began to seize, Versed was given to stop the seizure but I began to seize again. Another higher dose of Versed was given and the seizure activity finally ended. I truly don't remember any of this, though I do vaguely remember hearing my name being yelled repeatedly while looking up at bright lights. Due to these seizures I was kept for observation and testing. A brain MRI, EEG, and full Neurological work up was performed. Luckily, the test concluded that I do not have any sort of chronic epilepsy, but did absolutely have two seizures induced by the anesthetic agent. I just wish my body would behave normally...

Pain from tube placement is horrific, especially after you wildly convulse post surgery. Since I have adverse reaction to most opioids, finding a cocktail to keep me comfortable was a serious struggle. I had two nights of wildly uncontrollable pain that left me howling and bawling... And let's not even start with the number of blown IVs I had in 48 hours. My veins are garbage. Thankfully I had incredible nurses who were so caring and sweet. Three days later, after being cleared by three hospitalist, the GI team, and Neurology, I was finally aloud home! Hopefully life with this tube goes smoother than the placement itself. It's been a hell of a few days.

All of this sort of doesn't feel real - It's like I am in a nightmare. I keep looking down at this tube thinking, "how the hell am I back here?!" It's so defeating to have come so far just to be dragged back. I know I will eventually adapt and conquer tubie life again, but right now it just feels like a kick to the teeth. Ya know? So please excuse me while I wallow and adjust for a bit. It's hard, frustrating, and incredibly overwhelming. Honestly, I am just angry. Angry at my disease for once again taking something that brings me so much joy: food.

A very wise young warrior who has experienced multiple relapses of her rare blood cancer told me recently, "Growth isn't always constant. Relapses happen. You are not back at square one. Your growth prior to relapse isn't erased. Take your time - don't be hard on yourself. You're going to get through this." Thank you Ali.

Friday, May 3, 2019

Gastrointestinal Tract Relapse

Swallowing has, unfortunately, only gotten more difficult since I started experiencing dysphagia again. At first, swallowing solids was accompanies by a slow, full feeling in my chest. Almost like I took too big a bite every time I ate. It honestly could be quite painful. After a few weeks, that feeling turned into a chocking, stuck sensation. Solid food began not going down and regurgitating back up. Eventually I was forced to move to liquids only. However, as of two or so weeks ago, even liquids began to cause a choking, stuck sensation. The only way I could comfortably drink my nutrition was at about 12oz an hour. Weight started dropping off my body quickly. I lost almost 15 pounds in less than three weeks, leaving me malnourished and exhausted. The truth is, I have been struggling for longer than I have let on. Denial is real. So real...

My doctors all scrambled to run the proper tests and get me in their office as quickly as possible due to the fast and progressive nature of my GI distress. The first scheduled test was an Esophagram. This test is performed by drinking barium while taking a x-ray of the esophagus to asses motility. The esophagus is a tube made of smooth muscle which moves food from your mouth to your stomach. It should work in one cohesive movement to quickly and efficiently move a bolus down into the next organ. The transit of barium from the mouth to the stomach, in a normal patient, should take around 10 seconds. We began my assessment with the thinnest barium. Unfortunately, we didn't get any further than that. My esophagus was completely devoid of the waves to move a bolus through. The swallowed barium, a few fluid ounces, all convened in my esophagus, backing up almost the entire length of the organ. While gravity should typically move a bolus, even in an organ mostly devoid of peristalsis, mine simply didn't. (For a video please visit my Instagram or Facebook.) My physicians and the radiologist assured me my LES was wide open and I didn't have a blockage or stricture. It just simply... stops. A process that should be almost instantaneous took many minutes - after it eventually passed, it still left a trail of barium upon a final check in nearly 8 plus minutes later. The test had to be prematurely finished as I ended up regurgitating most of the barium onto the test table due to an overflow of fluid. Typically, patients are required to drink one to two 12oz cans, plus attempt a few other barium consistencies. However, I never made it past a few ounces of the thinnest liquid - and the barium that eventually passed filled up my entire stomach after a few swallows. The exact words in the final impress were, "severe esophageal dysmotility with absence of primary, secondary and tertiary waves. There is marked distention throughout the esophagus. Exam was terminated prematurely as patient experienced vomiting. Unable to ingest normal volume of contrast."

Unfortunately, this confirms that my dysmotility is absolutely back... with a vengeance. To make a long story short, after further evaluation by my Digestive Disease Specialist, my gastric emptying study was quite delayed (Gastroparesis) as well as my small bowel transit time. So, not only is the esophagus not functioning, but the stomach and bowels too. While they may not be as severely inhibited as the esophagus, they are inhibited enough that I am not digesting or absorbing nutrients - even simple nutrients like meal replacement shakes. The protein drinks I am forcing down my throat currently feel like bricks in my stomach. They sit for hours causing nausea, fullness, and discomfort. I can stomach maybe 1- 2 a day, leaving my body clinging to nothing but 500 or so calories of sustenance - calories I am not even absorbing correctly.

I was also referred for an emergency Upper Endoscopy to look for anything the other the external tests could have missed. Everything looked normal, well, normal for me. Again, they found no strictures or blockages - so no esophageal dilation.  I did still have stomach contents that never emptied from who knows when though. Unfortunately, this caused aspiration during the procedure. Luckily they, "got it under control quickly," or at least thats what they told my Dad. I awoke with a horrible wet cough, sill using an oxygen mask, needing to be monitored for hours longer than expected.
I require General Anesthesia when undergoing invasive procedures. I. Hate This. It is dangerous and seems so unnecessary to me, but but my team simply won't use twilight because it never sedates me enough. They told me I, "get too combative due to my Visceral Hypersensitivity." Urg. Any form of aspiration can be extremely dangerous - luckily they went into it prepared as my GI warned them about my completely useless LES. I am extremely lucky to have a team so familiar with my case. I am praying that this doesn't turn into aspiration pneumonia - I have been prescribed antibiotics to hopefully hold that possibility off. That is literally one of my biggest nightmares. As you know, I lost three dear friends to aspiration pneumonia because of Scleroderma. I love you Ashley, Alyssa, and Shelby. You have never, ever left my daily thoughts. I miss you all every single day.

They decided to place a NJ tube during the procedure. I wont lie, I broke down when I saw it. I honestly tried to rip it out while still high from the GA. I am devastated. This "nose hose" is a temporary solution for desperately needed nutrition until we can surgically place a G/J tube to bypass not only by esophagus but my stomach and a portion of my small bowel. We will be utilizing a peptide formula with hydrolyzed protein for ease of digestion. I honestly cannot wrap my head around this right now. I worked so hard. I fought so hard. Only to be back... I might need some time to process this outside of social media. Surgery will happen as soon as they can get a IR and Anesthesia team to coordinate - I have been told likely next week.

Due to this rapid and aggressive decline we will be testing my other organs this month, such as the lungs, to see if this relapse is localized to the GI tract or more widespread.