Thursday, October 24, 2019

Hooray for Farts! J Tube Surgery

On October 16th I arrived at my local hospital at 530am. You would be surprised how many people are at the hospital at this hour. I sure was! lol I was quickly whisked away to pre-op as I was the first surgery of the day. I met with the surgeon, the anesthesiologist, and many nurses. When it was time, 740am to be exact, I was wheeled into the OR. If you have ever been in an OR you know how creepy they are. Typically located down a cold, dark, seemingly forgot hallway of the hospital. The double doors open to a fully tiled floor to ceiling room covered in surgical instruments, with the centerpiece being multiple giant round lights. They make endoscopy rooms look downright cheery! 

Next thing I know, I woke up in my hospital room around 12pm. The surgery went longer than expected, and I was in much more pain than I anticipated.

Click for Image Credit 
Let me give a brief overview od exactly what placing a Witzel Tunnel Jejunostomy entails - in Chanel terms, that is. A Witzel Tunnel is a method of Jejunostomy tube placement, there are 5 or 6 different methods to place a feeding tube into the Jejunum. They all come with their own pros and cons. Needle Catheter, Roux En Y, Straight J, etc. My surgeon prefers the Witzel as he believes it reduces the possibility of dislodgement and reduces drainage from the stoma. This can be done via an open abdominal surgery or laparoscopically, either way, it is typically only performed by skilled Thoracic surgeons. Once inside the abdomen, a portion of the jejunum is located and a tube is fed through, the tube is then stitched into the intestines by creating a sort of "tunnel" with sutures. The portion of the small intestines is then tacked to the abdominal wall with more sutures. My surgeon performed this all laparoscopically. I have about a 3 inch incision above my belly button, a crescent-shaped incision below the belly button, and two incisions to the right. Well... where my belly button used to be, I don't really have one anymore. lol The tube lays to the left of all that. I also have 5 huge sutures holding the tube externally, as there is nothing holding it in internally like the G tubes. The intestines, especially after a Witzel are just too small to inflate a balloon or bumper safely.

Back to waking up. Ouch! I don't think I mentally prepared enough or this surgery. It was much more involved and much more painful than I anticipated. It also altered my anatomy to a degree I did not apprehend prior to surgery. To put it very straight forward, it was a bigger deal than I expected. Recovery was grueling. Pain control and muscle relaxers every two hours, which is tricky since I am allergic to so many opioids. We struggled to find the right combo for the first 48 hours, during which, I was literally crying out (loudly and uncontrollably) in pain... My poor roommate. Once we found the right cocktail I began to improve. Next step, attempt feeds into the tube. Unfortunately, we began feed attempts a bit too early, my intestines had not turned back on yet, we quickly realized. I became a nauseous mess. I could not stop dry heaving and throwing up. Thick, dark green bile was pouring out of my G tube. I was assessed to still have a post-surgical ileus. Bad news. We waited another day. Finally, things began to slowly work again! Wanna know how they know it's working? Farts. I farted! Finally! After almost 3 days! We began feeds which were no longer causing pain, nausea, vomiting, or producing bile backup.

Getting my feed rate up to something the surgeons were pleased with was a slow process. Finally, after a few more days inpatient, they were satisfied sending me home on 24 hour feeds. I will continue in my attempt to up my feeding rate so I can have a few hours a day off my pump. Once recovery in the hospital was complete, recovery at home began. They say it takes about 6 weeks for everything to heal up completely, so I have a ways to go! Today is 8 days since surgery and I finally left the house. Nothing exciting, just a doctor's appointment, but it was my favorite doctor, so that's something! (Hi!) My weight is down to an all-time low, 127lbs (I am 5'10"), but hopefully, now that I have a stable source of nutrition we can get that weight back.

In other news, my podcast with Enabled Warriors I recorded back in September aired this week. Please take a listen. It is hands down my favorite interview to date. The host is such an amazing woman and it felt like talking to an old friend. I have attached a few links for different listening formats below. I suggest youtube since it is visual as well. Hugs!

Spotify: (Oct. 23rd)

For more images from surgery, check out my Instagram, @thetubefedwife Have a great week!

Friday, October 4, 2019

October 2019 Update

Remember when my tube broke in June a month after it was placed? The J portion clogged, flipped, and tied itself in a little knot inside my stomach. Well, that happened again in July, and August, and September. We tried three different types of GJ tubes to no avail. Just an over a week ago we tried a special, low profile GJ tube that is supposed to have "anti-kink/flip" technology. We really thought this one would work! It lasted 7 days. SEVEN DAYS before it flipped back into my stomach. 
Let me give a brief explanation of the most common types of feeding tubes real quick:

  • Gastrostomy Feeding (G-tube or peg tube) G-Tubes are surgically inserted with an endoscope. These are a permanent option for patients who can tolerate feeds directly into the stomach, typically placed to bypass the upper digestive tract. The G -Tube is placed directly into the stomach.
  • Gastrostomy-Jejunostomy or GJ-tube (Transgastric-Jejunal or TJ tube) The GJ-Tube is surgically inserted via endoscopy or IR into the stomach but has an attachment that bypasses the stomach down into the jejunum. It is one twube with two ports, giving access to two organs. This allows patients who require decompression of the stomach simultaneously with Jejunal feeds to have only one stoma instead of two separate tube sites.
  • Jejunostomy (or J-tube A laparoscopically placed tube placed directly into the jejunum for feeding. This bypasses the upper digestive tract and the stomach for patients who cannot tolerate feeds directly into the stomach.

Each time these tubes flip, I go days without nutrition. I cannot feed into my stomach due to my gastroparesis. When my the J portion of my GJ flips into the stomach it is essentially like sewing a human's mouth closed. We must wait for a surgical or IR team to be available for replacement. In the meantime, I simply must wait. I typically get very sick during these times. My body shuts down without nutrition. I lose weight, my blood sugar tanks, I become severely dehydrated, my intestines slow to a near halt, etc. After my last tube failed, I was down to 129lbs and ended up in the ER with a blood sugar of 64. 

After four failed GJ tube attempts and weeks of accumulated malnutrition, my doc finally sat me down and had, "the talk." It has been decided that for my health and safety we must place two separate tube sites. This means, instead of one stoma which houses a GJ tube, we will make an incision directly into my intestines during laparoscopic surgery to place a separate tube site just for Jejunual feeding. I will keep the current GJ site, but it will be converted into a G simply used to decompress stomach bile. Two tubes, two stomas - double the drainage, pain, and work. Urg. This will completely remove the issues we've seen in the previous GJ tubes, but does require a more difficult, invasive surgery. I have met with a surgeon at my local hospital who I feel very confident in. He will be placing the J tube via the Witzel Tunnel method to reduce drainage and skin degradation. This surgery will require multiple incisions, reorganizations of my lower GI tract, and a few days inpatient. 

For now, I am stuck with an NJ tube, a long catheter that comes out of my nose and ends in my jejunum for feeding until surgery can be booked. Let's hope it's not too far out as NJ tubes are miserable and cause a myriad of sinus and throat issues. 

I met with my Pulmonologist and Rheumatologist in the past couple of weeks. The bad news is that my lungs are definitely affected by this relapse. I've lost about 10% of my FEV and FVC since my last test in May. This would definitely correlate with the dip in oxygen saturation with physical activity. The good news though, is that the CT scan did not show new or increasing fibrosis in the lungs. MCTD can cause lung and heart damage in many forms, so we are running some other tests to find the cause of this PFT deterioration. 

I started Rituxan in September, and have now successfully completed my first round which involved two infusions. We really won't know for another month or so if the drug seems to be helping not. The infusions went smoothly and my body tolerated them well - I was simply exhausted for a couple days after. With all the recent GI complications and such, I did have to stop working completely. Even part-time was just too much on my body and mind. For now, I am simply focusing on my health and trying to rest up before the big surgery ahead of me. 

Many people inquired about a GoFundMe or other crowdsourced fundraisers during this time. As of now, I am not be starting any fundraisers, however, I do have an Amazon list where I periodically update needed medical supplies my insurance doesn't cover. If you currently see a low amount of items, or nothing, on the list, it is because everything has been fulfilled. I appreciate everyone's contributions so, so much! They ease a great amount of the financial burden on my family and supply me directly with necessary medical equipment.

Sunday, September 1, 2019

I went Skydiving. Also, my health is crap.

Early morning phone calls from the head of your medical team are never a good sign. It what something like this, "It is imperative, vital, that you begin treatment immediately. I am... worried about you." Not many things shake me to my core, this is one of them. I have spent the last two months deteriorating, but also, living. I made my skydive on Aug 28th, to the dismay of pretty much my entire team. Luckily, my specialist understands that happiness is as much of a "treatment" as anything else and signed my waiver when none of my other physicians would. I am incredibly grateful to have him. The experience will stay with me forever. P.s Sorry for the title. I was too tired to be punny.

I would be lying if I said my health hasn't taken a huge hit since July. It's odd. I had this idea of what my second time around with this disease would be like. I thought my progression would move just as slow, if not slower, than the first time around. I figured I would have plenty of time to keep doing the things I love, like working. I also figured I would treat more naturally this time around. Using homeopathic remedies and life style adjustments, like cleaner everyday products, detoxifications, and supplements, instead of prednisone and immune suppression. I figured it would be easier because I knew what to expect. That I would somehow handle it better. Unfortunately, my body has made it very apparent, that any idea I had was wrong. I have declined faster than my doctors or I expected.

My lungs have recently been showing signs of disease. I've noticed shortness of breathe with physical exertion and increasing sleep issues... which of course makes everyday life extremely challenging. Sleep for someone like me is vital. My team is desperately trying to get me scheduled for a sleep study to get my Bipap prescription and levels renewed, but clinics in my area are booked out for months. (Apparently everyone truly is Sleepless in Seattle lol) The level of exhaustion during the day has reached a point where I am simply not functioning. I can't focus or stay awake, the bags under my eyes have their own bags, and driving to work has honestly become unsafe. I've had to completely cut out aerobic exercise and have a very difficult time with stairs right now. My muscles ache and I feel myself struggling to get a breath, often resulting in headaches if I push myself too far. My pulmonologist, who is booked clean into mid September, decided I couldn't wait to be evaluated and sent me to my PCP for a six minute walk study. I began to desaturate once we picked up our walking pace - with O2 levels hovering just below 90. They both concluded that my current lung issues cannot wait until mid September and gave me a referral to see one of their mutual associates who can see me sooner. They also scheduled me for PFTs at two different clinics in hopes that one will have a cancellation for an earlier appointment, as well as an HRCT to get a view of exactly what's happening inside my lungs. For the time being I am to, "avoid physical activity above a brisk walk..." Um what? I have stairs into my apartment, I have stairs at work, I frequently go to the gym, and I enjoying being out in nature! My life is pretty much always more involved than a brisk walk.

On the GI side of things, I've been dealing with a plethora of stoma issues. I had to have a large abscess drained on the left side of my stoma recently. Abscesses can be extremely dangerous and must be monitored closely. Thankfully, after the infection was drained the wound has almost completely healed. As abscesses have a high rate of reoccurrence, I am under carful watch. My SIBO has been absolutely intolerable. It took my insurance company nearly 6 weeks to finally approve the necessary medication! Thankfully I've been doing much better since we started the constant dosing of Xifaxan. I am still struggling to gain any weight and am unable to reach above 139lbs, even with 2,000 calories a day of formula. The idea of, "recreational eating" has been almost a complete bust. At this point my effort is in simply drinking more than only water without feeling horribly sick... My team had hoped many issues such as brain fog and hair loss were related to nutritional issues, however, that was deemed inaccurate. My latest blood panels seemed to be within reasonable nutritional levels for someone on tube feeds, yet my fog and hair loss continues. They now believe it is all simply related to this progressive flare I am experiencing.

Pain. Where to even begin. Exhaustion and pain are a never ending cycle. Being in constant pain is absolutely exhausting, that exhaustion leads to more pain, which leads to further exhaustion, etc. Every muscle in my body aches, every nerve is on fire, and every inch of me is screaming. I am struggling to leave my bed every morning, and when I do, I find myself back in it in a few hours. The feeling you get after being up for 48 straight hours, that delirious level of fatigue, is currently my baseline.

After the phone call from my doctor, and the last month of existing in my body, I have realized two things:
  1. I cannot continue like this.
  2. If I want to survive I need to make some very tough choices. 
Everything within me is pushing against these choices. My feet are digging into the proverbially ground, desperately trying to defy what has to be done - but my legs are too weak to hold out. I've spent more time in the last month crying than I have in a very long time. I feel like I've lost again. Lost my autonomy. No matter how desperately I want to, "get out and live a normal life," I cannot force my body to do so. I cannot pretend to continue on like I am not sick. Like I don't need doctors or treatment. I cannot wake up and go to work every day. I can hardly get out of bed let alone get ready, get in a car, sit in traffic, and work an 8 hour shift managing multiple employees. So, to address number one and number two, I will cutting my hours back from 40 hours a week to 18. I am hoping this will give my body enough time to rest and recuperate between work days. I will also be starting treatment; Rituxan. I have done Rituxan in the past many times and feel it is the, "easiest of all the evils" I have experienced, however, it is also highly effective. This is the absolute last thing I want to do, but I know that continuing to work a full time job, as well as neglecting treatment, will only destroy my body faster.

I am miserable. I can only hope that giving myself more time to rest and beginning treatment will improve my circumstances soon. Thank you to everyone who has stuck with me on this crazy teeter-totter ride! For more info and photos regarding my skydive, please check out my instagram @thetubefedwife

Thursday, July 18, 2019

Caterpillar Soup and Butterfly Wings

Relapse. Approximately 22 percent of patients relapse post HSCT. Those healthy harvested stem cells "re-activate," once again mistakingly attacking the body. Relapse is officially categorized as the need for DMARDS, aka immunesupressive therapy. This week, my team confirmed what we have all know for the past few months, I fall into that 22 percent. I have relapsed post HSCT.

Unfortunately, this goes well beyond my GI tract, though it was certainly the first system to be severely impacted. Even with my J feeds and gastric decompression I simply am not getting adequate nutrition. I continue to lose weight, be severely dehydrated, and don't seem to absorb much of the vital nutrients found in my formula. Words like TPN and gastric pacemaker have been thrown around recently. My hair is falling out. My finger tips are deteriorating. My joints are inflamed... And the pain. My gosh I forgot how much pain I used to be in pre-transplant. My body feels like it's been used as a battering ram.

My team would like me to begin immediate Rituxan infusions, though Actemra is also being considered. Honestly, I don't want to do either. I don't want to do any treatment, period. I struggled through so many grueling treatments over the last 7 years. Half the time the side effects from treatment were just as bad, if not worse, than my actual disease. I blindly gave up my autonomy in hopes of prolonging my life. I gave up my job. My schooling. My dreams. My ability to have children. Everything. There were weeks at a time where I felt less than human. Not to mention all the times I nearly died trying to stay alive.

I now understand that those years were my chrysalis. They molded and shaped me - but also, much like a caterpillar, completely disintegrated me in the process. I am not interested in another metamorphoses. Especially since I now know what to expect inside that cocoon. I already worked to become a butterfly! I no longer want to be a robot whose sole mission is to survive, to preserve life at any and all cost. I want to be a whole person, not a MRN number read off by a nurse at the infusion center for the thousandths time. I want to skydive! I want to sing opera!

This time around, instead of doing whatever it takes to have more time I simply want my time to BE MORE! This isn't giving up the fight, this is fighting to fully live! I want to use those butterfly wings I worked so hard for to FLY - not be disintegrated into caterpillar soup (again) in hopes of better wings. 

Luckily, I have an amazing team. They get it. They don't necessarily like it, but they understand. They even signed off on my skydiving waiver... very, very hesitantly, while explaining what a horrible idea it was. lol (I jump on Aug 28th!) We decided to come to an agreement. I get two months free of treatment, free of surgeries, free of new medications, free of pressure. They want me to take these 60 days to really think about my decision. To truly decide what route I want to take - as this is not a decision to be taken lightly. It was explained to me in detail that I will decline within this time frame, but my concerns and thought process were also validated. If I decide to move forward with treatment, a gastric pacemaker and port would be placed and Rituxan and steroids would be begun in mid September - though they are all really hoping I change my mind and begin much sooner than that. Truth be told, right now I don't know. What I do know is that I need an undisclosed amount of time to clear my head, talk with family members, meet with my therapist, and do some serious prayer work. Life is about so much more than always trying to get to a better place. Sometimes it is just about enjoying the ride - even if it is down a bumpy, unpaved gravel path.

Tuesday, June 25, 2019

Please, Listen To Your Patients

Medical devices are finicky things. Humans are finicky things. Egos are finicky things. While no one person is at fault for the events that happened to me over the past week, one person could have easily stopped it. Coordination of care was so utterly absent that I was put in a very dangerous situation. To fully grasp what occurred let's break it down by day.

Wednesday the 19th: 

  1. I saw my GI bright and early for my 6 week surgical check in. The site was looking okay. No more infection, though some nice granulated tissue has begun to settle in. And by nice I mean ugly and hurty. The slit on the one side of my stoma finally healed up, though it's made one side of my stoma way deeper than the other so my tube doesn't sit flat. Honestly, whatever, as long as I am being fed. 
  2. I saw my sclero specialist around 3pm regarding all my current symptoms. Severe hand/finger  swelling, joint inflammation and pain, hair loss, excessive rashes, worsening nail fold capillaries, new muscle weakness, and of course, my failing GI tract. He did my skin score and looked over my recent PfTs. The good news is that my skin score is still stable, as well as my PFTs. Yesssss. However, the bad news, autoimmunity is wreaking havoc everywhere else and causing some serious wide spread inflammation. He does not believe it is the sclero, but another one of my autoimmune conditions encompassed in my MCTD. Perhaps Lupus as the symptoms more closely match... well, besides the GI stuff. He would like me to trial some anti-inflammatory medications to help reduce the crazy inflammation in my body currently. If that doesn't work, we talked about a new immunosuppressive infusion. 
  3. By 4pm that evening and the J portion of my GJ tube had clogged. Totally unusable. I spent 7 hours in the ER trying to get it unclogged. We used Coke, meat tenderizer, hot water, even a special system called Clog Zapper. Unfortunately, turns out it wasn't a clog. The J tube had dislodged and somehow wrapped itself in a perfect figure eight knot inside me. Needless to say, my soul source of nutrition being unusable was bad, very bad. 

Thursday the 20th:

  1. I frantically called my GI the second they opened and asked to get a solve asap. I suggested we just place a new J through my G via endoscopy My GI decided she wanted to place a new tube with a larger french to prevent this from happening again. I told her I was pretty sure my stoma was too young but tried to trust her judgement. 
  2.  I arrived at the IR appointment only to be told, "No way! The stoma has to be at least 6 weeks!" *Insert sarcastic "awesome"* They decided to place an NJ until I reached six weeks, which would be... the next week.
  3. After two and half grueling hours under x-ray, four NJ tube attempts, six different guidwires, both nostrils, over six dedicated techs and physicians, and more numbing gel than I can fathom, they called it quits. The dislodged J portion of my tube had become so wound up inside me that they could not get a NJ tube in the appropriate position. It was literal torture. 
  4. I was given two options, make another appointment to place a picc line and start TPN for a approximately three days until they could schedule a tube replacement - or simply go without nutrition and get an a daily infusion at the infusion center of lactated ringers to at least stay hydrated. Honestly, by the time we got a picc placement scheduled and a company to ship out TPN it would already be Monday, so I opted for the LR infusions everyday. 

Friday the 21st - Sunday the 23rd

  1. Received Lactated Ringers at the infusion center, drank what I could and nibbled on a slice of watermelon. Threw up that slice of watermelon. Lost 7lbs. 

Monday the 24th

  1. Monday we arrived at the hospital for what was supposed to be a tube change. This is where they barbarically yank out your bumper tube and replace it with a balloon tube. Now, if you've been keeping tract I've been telling my doc, "Hey, I don't believe my stoma is mature enough. Why don't we just place a new J through my G via endoscopy?" 
  2. We get my IV started, completed pre-op and arrive to the IR surgery room. The doc takes one look at my stoma, "Woah, there is no way I am doing this! This stoma isn't mature! I'll do irreparable damage to your abdomen and you will end up on an OR table with a giant whole in your stomach!" 
  3. At this point I lost my damn mind. I cried and I advocated for better patient care and coordination. I expressed ALLLLL my frustration - in a civilized but brutally honest fashion. I expressed how these last two procedures could have been entirely avoided if my physicians would have just LISTENED to me. (Crazy huh, that a patient thats been battling a disease for 8 years might know what they are talking about?!) The awesome nurse in the room was like "Hell yeah, you're right!" The IR doc ended up siding with me as well and telling me what BS this whole situation was. He personally called the head of GI at the hospital and made me an appointment for.... drumroll please.... a new J through my G via endoscopy. What I had been saying all along!
  4. It only took two unnecessary procedures, five days of starvation, thousands of insurance dollars, and multiple physicians to finally decide I was right. WTF.

Tuesday the 25th 

  1. Zip on up to the hospital, talk with anesthesia about my special precaution; aspiration and seizures. Get wheeled back to the procedure room. Voila! A new J tube was placed through my G via endoscopy. All in all, an hour long procedure plus a well deserved propofol nap. Problem solved! 

I am fully aware there are many wonderful doctors who listen to their patients. I often praise and acknowledge them on my social platforms. I owe many of them my life! However, there are also some really poor doctors who do not. Over the past week I had to deal with this first hand - it is simply the reality of health care. Please do not de-validate my 8 years of living with this disease. Do not let your ego get in the way of my care. Do not think you know my body better than I do. No, I do not have an MD, and yes, you often do make the right decisions, but you are still human. You can make mistakes. That MD doesn't doesn't mean you have the right to not have to listen to my concerns. So yes, I will hold you accountable when you place me in situations like this. Why? Because many patients aren't like me, they think you're a god who could never do wrong. They don't know they have to advocate for themselves because they believe you can, "do no harm." So I will be loud. I will share the good the bad, and the downright ugly. Please, listen to your patients, they may just say something useful.

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.

Saturday, April 13, 2019

April 2019 Update

During times of discouraging health set backs I like to remind myself just how far I have come. Two and a half years ago I was literally dying and relying on Social Security Disability benefits for income. Now, I am a mostly functioning young professional directing the entire client relations department of a multinational company. Those are big, BIG strides... but it's still difficult to not get discouraged when I experience set backs. Recovery, especially from clinical trials, is not a short, sturdy stair case to a perfectly safe balcony. Recovery from a clinical trial is more like a steep sheet of ice to a rickety old deck, you make a bit of progress, slip back down, make a bit of progress, slip back down, etc. Sometimes, when you finally get to the top, the deck collapses out from under you. It's frustrating and messy. Did I mentioned frustrating?! But no matter what, your only option is to keep crawling the slippery ice while praying that old deck will hold up as long as possible.

Lately, I feel like I have been clawing my way up that slippery ice only to find the rickety deck swaying from side to side while nails silently slip out of their holes. I'm thrilled that I have made it to the top, but I have no idea when the nail will slip that was holding the last few boards together.

As many of you know, I reached my two year post HSCT mark back in January, however, my blood work is still horribly awry. My WBC is still well below normal and my IgA, IgM, and B cells are completely non-existent. This are all solid indicators that my bone marrow never fully recovered post transplant, especially at two years out. There is really nothing to be done besides being overly cautious of germs - and to be put on antibiotics at the first sign of any sort of sicknesses. (Which is a whole can of worms in itself.) The last thing I want to do is is live, "over cautiously," avoiding crowds, public places, pools, certain foods, etc. So, the truth is, I am not. I am just living. I am fully aware of the implications BUT I didn't fight this hard to live only to sit at my house basking in hand sanitizer. Please understand, I am not disrespecting my bonus life, nor am I completely throwing all caution to the wind. I am being smart. I am being safe. I am simply enjoy this second chance to the full extent possible.

I saw my Scleroderma specialist last month who noted swelling of the hands and increased GI distress. The good news is my skin score is still stable, but the bad news, I am clearly aspirating and backsliding in regards to GI progress. Wet, ripping coughs accompanying each meal. Tightness in the chest. Food and phlegm coming up hours, sometimes days latter. Recently, after a particularly bad bought of aspiration I ended up with a nasty chest infection requiring antibiotics. My biggest fear is aspiration pneumonia, which has taken the life of three of my best friends.

I was given an emergency referral to my Digestive Disease Specialist who was wonderful as always. After a rectal exam no one enjoyed, she noted the months of Pelvic floor therapy I did was a total failure. The muscles in my pelvic floor are shot. Being unable to defecate due to a lack of muscle tone causes extreme constipation, which eventually leads to horrible diarrhea and leakage. (Cute I know.) There is literally nothing they can do. My dyssynergia and loss of muscle tone is just too great. So... home enemas every three days for me! As for my upper GI tract, my esophagus has been desperately holding on to food. Take for example, burping up a piece of egg I ate 3 hours ago that never made it to my stomach while driving to work. Ew. Likely, I have a stricture in my esophagus causing the retention of food - plus the severe dysphagia which doesn't help. I am scheduled for an Upper Endoscopy in May to dilate my esophagus. We are also working on scheduling another swallow study to asses my pharyngeal muscles which are likely contributing to the aspiration along with the strictures. While at my GI we noticed my resting oxygen sats were down which is never a good sign. So I have been referred to my Pulmonologist next month as well. I will be so unbelievably bummed if my GI tract has begun ruining my lungs - they were finally stabilizing in the high 70s! I am losing weight, nauseas, and a literal burp factory.

....Urg. Insert all the curse words.