What you should know about firing your physician

This is a topic I don't often talk about because frankly, I don't like confrontation. However, it would be unrealistic of me to share life as a patient without mentioning this often uncomfortable situation. 

Firing your physician.

I can count on one hand the number of times I have had to remove myself from a physician's care. For someone with scores of doctors and nine years of constant medical care... that is a very small number. I am one of the lucky ones, truly. A doctor-patient relationship should be a partnership, never a dictatorship. Often new patients are blissfully naive, they honestly believe a doctor can do no wrong. They hand over their autonomy and let doctors make all the final decisions. This is what the system was built on. This is what the media shows us. This is what the providers are used to. The naivete makes the jobs easier - no questions, no interjections, just a followed treatment plan utilizing the drug companies that are likely funding them or their clinic. Often, this is a win-win scenery, the patient improves, the doctor gets paid, and everyone is happy. That is... until it doesn't. Until you have been a patient long enough to lose those rose-colored glasses; long enough to have experienced the negative side of medicine. The neglect. The disrespect. The dismissal. The inflated ego which put your life at risk.

Medicine is built for patients who take orders, not for patients who want a synergistic relationship in which they can exercise their right to choose without fear. Personally, I take a very active stance in my care. I want to be well educated on my conditions, their treatments, and the medical options available to me. I may bring potential new treatments up in office and request to review their pros and cons. When prescribed a new medication I may return home and read accredited medical journals and browse clinical trials to get a better idea of a drugs potential benefits and adverse reactions. I always ensure I know what is being prescribed and why. The knowledge necessary to make my final decision will come from open communication between me and my doctors as well as my own personal research. Both parties must be actively engaged and willing to see from each other's perspective. This can only be achieved through mutual respect.

Please note that many, in fact MOST medical professionals are good, honest people who genuinely want to help others. Or at least, many of them start that way. I have many doctors on my team who I would consider family at this point. Heck, I have added some of them to my Christmas list! I know my desires for autonomy and quality of life are respected at their practice. I also know I am profoundly safe and genuinely heard while under their medical supervision. However, that is not what this blog post is about. 

I want you to know that you do not have to stay in a negative relationship with a physician. While finding a new doctor is frustrating and often exhausting, it could save your life. You deserve to be with someone you trust - someone you feel safe to bring up your concerns with. While yes, the physician holds the expertise, training, and knowledge in theory, YOU and only you are the one that must inhabit the body in question. If a medical professional disregards your symptoms, uses derogatory remarks, or acts fictitiously during your appointments you have the right to decline further treatment with that provider. Your medical care is about you, your comfort, and your quality of life. You and your doctor should be a team with the same outcome in mind. While you of course should accept and head proper medical expertise... if that expertise comes at the cost of emotional abuse or repeated physical negligence you have every right to fire your physician.

First Week of Hizentra

I mentally feel a lot better than I did during my last post. I had been really struggling with a lot of frustration over the last few weeks. Mostly with my insurance for deciding to make everything so incredibly difficult recently. Though, I think the realization that I have been fighting for nine straight years sort of crept up on me too. Nine years of literal life and death - I think my poor brain just shut off for a couple weeks there. lol Thankfully I have a wonderful therapist, palliative care team, and army of family and friends willing to hold space for me during my moments of grief.

My insurance finally approved the SCIG which began this week. This is a blood product, called Hizentra, created by thousands of plasma donors which will build back my depleted immunoglobulin. It will be infused subcutaneously at home once a week. This means four small needles will be placed into my stomach while thick fluid is pumped under the skin just above the muscle. Yes, it is about as pleasant as it sounds. The subcutaneous version of immunoglobulin has a lesser side effect profile than the intravenous version, IVIG. IVIG boasts many severe, life-threatening reactions - I had been on IVIG once in the past for a bought of GBS and did not fare well. My physicians hope I will tolerate the subq version better since it is received in lower doses more frequently as compared to one large dose a month. The subq version I am receiving is not necessarily painful, but quite uncomfortable as the fluid builds under the skin. I am left with red welts until the fluid dissipates over about 24-48 hours. The one-hour infusion feels pretty similar to rubbing stinging nettles on yourself - sort of itchy and prickly. Besides being very tired the next day, I seemed to do just fine. 

I met with my Rheumatologist to go over some blood work and to discuss treatment options moving forward. Due to this newly diagnosed immunodeficiency, we decided to hold off on any immunosuppressive treatments, as they would be contraindicated at this time. We will be reevaluating my treatment options once we get this current, "fire" under control. It is quite odd prioritizing "fire." Letting one fire burn so you can snuff out another feels counterintuitive, but in reality, it is often the only way people like me survive. I have read a few studies that showed promising improvements in MCTD patients on therapeutic levels of immunoglobulin though, so maybe this treatment will kill two birds with one stone? That would be rad... My blood work was pretty typical for someone with serve GI issues. Low vitamin D, low potassium, etc. I also showed some alarmingly low blood sugar trends, low white blood cell counts, and of course, a formidably high autoimmune panel. Like I said, typical stuff when you reside in a failing body. I'll see my doc again in a couple months to evaluate how I am doing on the SCIG and to run more blood panels. 

After further discussion with my team about hydration at home, it looks like the only way forward in the fight with Medicare for treatment coverage is to accept full cost responsibility which will generate an, "official denial." If you are scratching your head saying, "that doesn't make any sense," I too had that reaction. Essentially, my doctors cannot fight Medicare until I am officially denied payment for treatment. Once I receive the denial letter from Medicare I can then have my physicians send in all the documentation on why at-home treatment will be more beneficial in my case. It's backward I know, but it currently is the only way forwards. Hopefully, we won't have to front the cost too long and can get the denial overturned asap. At least for now, I am receiving my D5LR safely from the comfort of my own home. 

I know this was a super short update, but I just wanted to pass along that things are moving forward finally! Xoxo, Chanel

November 2020 Update

My calendar is overrun with appointments - I had to get one with bigger squares! *sigh*

A quick breakdown for those who don't want to read the whole thing:

1. Stoma dilation and PEG replacement were successful.
2. I have a new Rheum on the team - he will fill in the gaps left by my SSc Specialist.
3. My second round of eye duct plugs are going well.
4. My yearly Echocardiogram showed a Pericardial Effusion and thickening of two heart valves.
5. I was diagnosed with immunodeficiency and will be beginning IVIG or SCIG.

My GI system has continued to deteriorate over the last month. I know I say that every month - but it's true. As mentioned, we placed a new larger diameter tube to assist with gastric drainage. The surgery went smoothly and the new PEG works well. It's huge which is esthetically not pleasing, but at this point, I really don't care. I am desperate for anything that will improve my day to day life. I am having to drain my stomach contents multiple times a day, occasionally I even get woken up by the pressure and nausea in the middle of the night. Apparently, I often tell Noel, "I am nauseous," in my sleep - I am a big sleep talker.  This is not food or drink, it is just bile and air my stomach isn't passing. It is incredible what it can produce when given absolutely nothing. 

Thankfully, I am still tolerating my J tube feeds, though I am no longer absorbing all the vital minerals which they contain. My labs have been blahhh and my chronic dehydration, due to my lack of absorption, has become a serious issue. As mentioned I have been going to the infusion clinic twice a week (though it should be three) for hydration which, for now, includes a mix of calcium, potassium, dextrose, and sodium - though other minerals may need to be added in the future. It has become too difficult for my body to make the 2 hour round trip drive twice a week so I moved to the hospital's satellite location about 30 min closer to my home. Unfornutly, this satellite location is not where I feel safe, sanitary, or well taken care of - that is why I held off the switch for so long. I will continue to fight for home infusion but I have been assured that it will be impossible. Apparently, Medicare "never" approves at home hydration. Awesome...

I'm not sure if I have made this clear, but these infusions and intestinal feeds are what is keeping me alive. It is a palliative care measure - without them, I would not be able to live for more than a few weeks. It is disheartening to me that a patient can reach this point and still be denied a method of more convenient treatment all because the insurance company won't be, "reimbursed enough to make it worth their while." 

I met with a new Rheumatologist Monday. Well, actually, not new to me, but one I haven't seen in ages. His role will be to quarterback my care and to fill in the gaps left by my Scleroderma specialist who only practices here in Seattle once a month. This is actually the physician who initially diagnosed me 9 years ago. He remembered exactly who I was stating that I was the first, "incredibly severe scleroderma patient" he had seen. (This is probably the least preferable method when it comes to being remembered by a doctor haha) After looking over my chart and history he was a bit concerned - there really are no options my specialist hasn't already tried to get this disease under control. He believes another clinical trial would be my best bet. He actually mentioned another transplant, specifically an allogeneic (donor) since the autologous didn't seem to resolve the autoimmunity in my blood... but I kindly declined. I am not interested in going through another transplant and will not change my mind on that matter. For now, we will still try to move forward with the Actemera infusions which my specialist recommended back in September - though we are still battling insurance for them. He also ordered some blood panels and a follow up in one month. 

Later in the week, I met with my Ophthalmologist to trial some new tear duct plugs. This time we gave the dissolvable plugs a try since my eyes did not tolerate the more permanent ones well. So far so good. It is actually quite exciting to see how white my eyes are after just one day! Normally the whites of my eyes are always pink from irritation, even with using artificial tears 8 - 10 times a day. 

I also had my yearly Echocardiogram. Not much has changed, my pulmonary pressures and ejection fraction are still stable, though two of my valves (aortic and mitral) are starting to thicken from the Scleroderma. Their motion is still normal at this point though, which is good. The one thing of significance was a posterior Pericardial Effusion, aka fluid around the heart. This can, unfortunately, be somewhat common in autoimmune disease patients with heart and lung complications. This fluid would probably explain the chest pain and tightness I have been experiencing since last week. There isn't enough fluid to really drain so my body should naturally reabsorb it. However, we will need to keep an eye on it.

This morning I met with my Immunologist to go over the results of the vaccine we introduced to assess my humeral immune memory response. Unfortunately, my body did not "absorb' the external antibodies. I actually showed a whooping 0% percent repletion. This is bad news. In laymen's terms, my immune system no longer creates its own immunoglobulin or responds to the external introduction of protective antibodies, such as vaccines. This is categorized as an Immune Deficiency Disease. There are over 400 types of Immunodeficiencies - my physician believes my subset is a secondary immunodeficiency due to so many years of immunosuppressive treatments. It is a rare side effect but can happen. So, once insurance decides what they will and won't cover, I will be beginning either subcutaneous immunoglobulin (SCIG) or Intravenous Immunoglobulin (IVIG). These interventions should help bolster up my immune system against foreign contagions. Maybe I will finally get a break from my near-constant sinus infections! Whoop!

I will have to meet with my Scleroderma Specialist again this month and, due to this new diagnosis, will likely be unable to continue with the path of traditional immunosuppression for autoimmune disease management. Long story short, it is a confusing and transitionary time.

Xoxoxo, Chanel

October 2020 Update

I just realized I haven't updated since my care team changed everything up last month. Whoops! Honestly, it's been a long haul attempting to get insurance pre-auths pushed through. My insurance is stellarly crappy. It's almost impressive how much they deny... While we couldn't get them to approve at home hydration or even hydration at the clinic near my home, we were able to get it approved at a hospital about 45 minutes from my home. It equates to a lot of time spent on the road, but the nurses there are SO worth it. It is a cancer center, so they have the capability to run not only my lactated Ringers and Zofran, but my steroids and (soon to be) Actemra infusions as well! Plus they are all fabulous with port access. Speaking of ports, my port surgery went super smooth. The surgeon was incredible - as she looked over my old scar she said, "A man placed that previous port, didn't he?" I was sort of shocked how she would know that and asked how. She expressed that the device was placed "in the boob," and that only a man would do that. hehe I had a good laugh. Classic. 

I have noticed a serious downtick in symptoms since I began the bi-weekly hydration infusions. It is amazing what keeping a body hydrated can do! My blood pressure finally reaches above 85/53 on most days and I'm actually producing urine again! While more than twice a week would be ideal, my insurance simply won't approve hydration outside of the hospital clinic - and my body just cannot handle the long drive every day.

A had a wee emergency this month. My G-Tube, the tube that vents air and bile out of my paralyzed stomach, stopped working. The two-way valve became a one-way plug. Yikes. My poor belly distended to what seemed like a late-term pregnancy. I couldn't stop vomiting acid and burping. The pain was intense from the severe bloat. Unfortunately, my insurance also doesn't allow me to keep a backup tube on hand. *Sigh* So I had to venture to the ER to get a replacement tube asap! By the time I got there I was totally incapacitated by nausea and they got me in swiftly. Once we deflated the balloon the tube nearly shot out of me! Think of a whoopie cushion - lots of noise and mess came erupting out of my sad little guts. INSTANT RELIEF. We quickly placed the other tube - more bile and air escaped noisily. I think I quite startled the doctor. lol She kept saying, "wow, you must feel so much better! I have never seen anything like that! You must feel like a whole different person!" Ahh, the joys of digestive tract paralysis. My stomach literally can't handle air. AIR. You ever just think, wtf is life sometimes?!

Since that blip. I have been awaiting an endoscopy to place my preferred brand of tube, as well as explore some areas of chronic pain around the stoma sites internally. Unfortunately, low profile tubes aren't really an option for me anymore with the necessity of near-constant drainage, so I am back to long, clunky tubes. Bleh. 

Bloodwork, I almost forgot! I've been in close contact with my new Immunologist over the past month. Unfortunately, the extra blood work that was taken during our new patient appointment did show some concerning abnormalities - again. I have literally zero of the twenty-three antibodies that protect against conditions like pneumonia. I thought that was very random, but he expressed that with my total lack of Immunoglobulin, he was not surprised. So, he introduced a new medication via vaccine that should take about 4 weeks to replete the missing antibodies. In 4 weeks I'll do another blood test to see if my body was able to, "absorb" this introduction of external antibodies. If it does, we should be good to continue with my new immunosuppressive treatment, if it doesn't, I will likely have to begin IVIG after all. So, still a waiting game there but, I am so grateful to have such a knowledgeable new doc on my team!

Pebbles and Boulders

I'll get right to it since I am sure that's why you are all here. I do not have Cancer, Hallelujah! However, before you congratulate me, I did not only receive good news this week. Honestly, that was the only good news I received...

I feel like trash. A dumpster on fire filled with hot garbage. This week started out with a nice big infusion of Lactated Ringers, D5, and a Zofran push. My GI tract has been doing extremely poorly the past two weeks and I have become dehydrated to a level that makes everyday life extremely difficult. My blood pressure, which is already naturally low, plummeted, along with my blood sugar. When the nurse started my IV she uttered, "wow, your blood is almost black. You must be really dehydrated! You look like you need some sleep too, honey." I love it when the nurses are honest. Actually, I love it when anyone is honest. Please, don't tell me I look great if I don't, it is extremely devalidating and confusing for me! lol

Midweek I saw the Immunologist. He was incredible; very thorough and clearly interested in my vastly complicated case. He reassured me that my recent labs do not seem to indicate Cancer but instead damage to my bone marrow caused by years and years of immune suppression. Unfortunately, when you're immune system is trying to kill you, you must suppress it. Immunosuppressive medication, while it suppresses your disease, also suppresses vital immune function. A double-edged sword. Without these vital functions, you are more susceptible to infection and Cancers. The drugs I have primarily been taking the past 5-6 years specifically depletes B cells, which are where Immunoglobulin is created. He believes this is why my Immunoglobulin was so severely reduced. Unfortunately, there is nothing that can be done to raise my IgA and IgM as there is no medical treatment to do so. The only Immunoglobulin you can raise is IgG via IVIG. However, my IgG is still 50ish points above the level the immunologist requires to begin IgG replacement therapy. The best way to increase your IgA and IgM would be to halt all immune suppression - and that is clearly not an option for me. We will have to keep a very close eye on these blood counts in the future as the IgG will likely continue to decline - meaning I will eventually require IVIG infusions. He decided to run a gambet of additional blood tests to assess the rest of my immune functions as well. These tests will give us a better idea if my bone marrow is permanently altered, or if perhaps it still contains the humoral memory to reproduce on its own. I will see him again in 4 weeks for the results. 

Next, I saw my Rheumatologist. He was very reassured by the Immunologist's findings and mentioned he truly through I had blood cancer, which is why he called me in such a panic. He also expressed feeling a bit guilty for the destruction of my bone marrow - but we both acknowledged without these years of immune suppression I would already be dead. I knew treatment for this disease had risks, but I also knew not treating it was the bigger risk - so I of course told him he should not feel any blame! Dead vs dealing with side effects seems like a pretty easy choice to me. It was clear that we needed to switch treatment to give my B cells a break from constant depletion. So, instead of continuing Rituxan, he has decided to switch to a drug called Actemra. This immunosuppressive drug works differently and should not deplete my Immunoglobulin as severely as Rituxan. Actemra is an IL6-Cytokine inhibitor. The IL6-Cytokines mediate and modulate immunity, inflammation, and hematopoiesis. They aid cell to cell communication in immune responses, infection, and trauma. For autoimmune patients, IL6-Cytokines go into hyperdrive and disrupt the balance in the immune system. Rituxan has been a miracle drug for me over the past year, improving my lung function and general quality of life tremendously. I am honestly terrified to discontinue it. However, I know my poor B cells need the brake. Trialing different drugs to see what works best for your particular immune dysregulation is something patients like myself become very accustomed to - though it never gets easier or less frightening. While Actemera will come with its own potential side effects, it seems to be the best option at this time. It will be a once a month infusion. 

On top of all this, I currently have an extremely unhealthy amount of inflammation in my body. (I have little sausage fingers again.) After physical evaluation, my doc said about 60% of my muscles and joints are in a state of heightened sensitivity. Plus, I have a raging sinus infection - likely a result of the depleted IgA which normally protects your mucosa from infections. He decided to do a 1000mg Solu-Medrol infusion to immediately calm the inflammation while I was in office. (If you have never had high dose IV steroids just know, it's hell. Everything, even water tastes like metal and you feel like a mentally unstable chipmunk on Adderall for a few days.) He actually wrote orders for one 1000mg Solu-Medrol infusion a month for the next six months to get ahead of this general autoimmune flare before it completely derails my progress over the last year. We are also going to run fluids three times a week for the next month to combat the chronic dehydration. If you're keeping count, that's 14 infusions a month - a number my already damaged veins could not possibly handle. The decision was made to place a port. I had my last port removed back in 2017 and it served me well. While I am devastated to receive another, I know it will save me from so much pain and vascular damage over the next 6 months to a year while my body recovers from this flare. Surgery to place the port, a central line which gives vascular access to the heart, will be Monday. 

Later this week I saw the Oncologist/Hematologist. She agreed with the Immunologist's general impression of my blood work. That my abnormal results are likely due to years of immunosuppression and not Cancer. She was, however, interested to receive the results of the further immune assessments the Immunologist ordered. She assured me if she saw anything off she would order a bone marrow biopsy asap. Honestly, she was my fav physician of the week. She was incredibly knowledgeable and took a whole hour to go over my case in office. Most of my physicians are so overwhelmed by their specialty it's not often I get a doc who genuinely tries to acknowledge my body as a whole instead of separate pieces in a larger cog. She was deeply concerned at the lack of follow up post my Autologous Stem Cell transplant and made a list of doctors and tests that a post-transplant patient like myself should be monitored for. So helpful!

So, it doesn't seem to be Cancer at this time. Of course, I'm THRILLED and incredibly relieved! However, please remember chronic conditions are just as devastating, just as expensive, just as deadly, and just as intensely strenuous. So please don't be quick to congratulate me on all of this mess being, "only a medication side effect." There was no good outcome in this situation, unfortunately. I am now emotionally processing major changes to my treatment plan, 14 infusions a month, upcoming surgery, and an extremely physically tasking flare. Moral of the story, the next few months are going to be full of a lot of adaptation and changes for me. Plus a lot of roid induced crying sessions hehe ;) I know many of you reading this who have followed my journey for years are thinking, "This is nothing! Chanel has dealt with so much worse!" While that is technically an accurate thought process, I am still human. I still feel disappointed. I still feel angst when changes arise. I still don't enjoy being cut into and having medical devices placed into me. Having a chronic illness never gets easier. It never gets less exhausting. Over time each little set back begins to feel so heavy. Pebbles don't weigh much alone but added up can be heavier than a boulder. 

I am still pretty jittery and emotionally fragile from the massive steroid dose, so please excuse this very frank, jumbled, and extremely angsty blog post. It is hard to keep my thoughts coherent and pleasant when I haven't slept in almost 48 hours... 

Xoxoxo, Chanel

Unresolved Worry

Hi. I am honestly not sure how to start this post. I debated whether I should even make this post. I do not like to spend time worry about things I cannot change, and well, worrying about results I don't even have yet seems to be the absolute epitome of that. Yet here I am, worrying. 

Remember when I said my doc took 9 or so vials of blood during my last Rituxan treatment? Well, last week I got a call from my physician... from his cell phone. It is never a good sign when a doc calls you after hours from their personal line. I am pretty good at reading people and there was obvious concern from the first hello. The call went something like this, 'How are you doing?' 'Your blood results showed some concerning results.' 'You are nearly devoid of a vital protein that play an essential part in the body's immune system.' 'I am sending an urgent referral to oncology. My team will call you to set up your appointments.' As you can imagine, my brain sort of shut off at Oncology. Within minutes his team called me expressing they were, 'calling every hospital in the area to find the soonest appointment available.' That, 'my physician was personally calling an Oncologist, Hematologist, and Immunologist to go over my case.' By the next morning, I suddenly had an onslaught of appointments scheduled for the coming week. 

From what I understand, my white blood cell count and ANC have been chronically low for quite some time. Low even for someone on immunosuppressive treatments for an autoimmune disease. This alerted my team to run specific blood work on my immune system, specifically, antibodies known as Immunoglobulin. Immunoglobulin A, G, and M are proteins that your immune cells (B cells) make to fight off bacteria, viruses, and foreign invaders. Even while on immunosuppression these antibodies are to be kept at specific levels, mine, however, are nearly nonexistent. Especially my IgA and IgM, with my IgM being almost undetectable in my blood. Apparently, this phenomenon can be attributed to a few things: rare medication side effects, specific blood disorders, and Cancers. I am sure there are more, but these three seem to be of most concern to my team currently. 

That's all I know for now. From what my physician has expressed, it is likely that, at the bare minimum, I will need to begin Intrevanious Immunoglobulin therapy (IVIG) and possibly stop my current immunosuppressive treatment, Rituxan, which would then have to be replaced with another therapy. However, I won't have any solid answers or treatment plans until I see the Oncologist, Immunologist, and Hematologist. We are hoping they can give us an explanation as to what is causing this sudden and aggressive immunoglobulin deficiency. I will of course keep everyone updated here on the blog - Keep in mind though that things may still be unclear even after I see the new physicians next week - as I am sure there will be more tests to run before they can truly solidify any diagnoses.

Sorry this is such an unresolved post. I really don't have any information besides, something is wrong and my team thinks an oncologist may have the answers. Ooof. Here is to hoping it's just a rare medication side effect! 

Summer 2020 Update

I just wanted to pop in and thank everyone for their patience with me as I transition my social platforms. It's been incredibly therapeutic to cut down on my social presence. I am currently in a pretty decent place mentally after starting with a new therapist. (That will be its own post soon.) If this Covid quarantine has taught me anything it's that being cooped up is extremely difficult if your mental health isn't in order. I've spent much of quarantine, "Marie Condo-ing" my brain. Getting rid and letting go of the things I no longer need to carry with me while learning to neatly and peacefully compartmentalize the things I must carry with me. It hasn't been easy, but I am getting there. 

Health updates are far and few between due to quarantine. I see most of my physicians through telehealth right now. As I'm sure many of you have experienced, telehealth is sort of a huge waste of time. Since our physicians can't see or experience our symptoms in person, it's basically just a huge waste of a copay. lol I have had a few tests and procedures in person, however. I'll run through everything in bullet points to make this as easy and quick as possible. 

1.  June 2020
1. After many rounds of Iron infusions, my Anemia seems to have been curbed again. I am feeling much better and am no longer the color of my white walls. Iron Deficiency Anemia always kicks my butt. I am so grateful for a physician who listens to my concerns and acknowledges how well I know my own body. I can always tell when I am in a bout of anemia - and she is always quick to order the tests and proper infusions. 
2.    July 2020
1. I had a quick J tube change since my balloon popped in June. The balloons only last about 6-9 months so it was to be expected. I decided to move forward without sedation again since I wanted to be in and out as quick as possible. Thankfully, it was much less painful and traumatic than my first J tube exchange. While it was still unbelievably painful, my tract was more mature which meant the procedure was quicker and easier on my body. 
2. I had my biyearly PFTs. It seems the Rituxan is finally showing improvement in my lungs! My PFTs were some of the best I have ever blown! This is incredible news as we truly didn't have high hopes for the Rituxan - since it wasn't a very useful drug for me pre-transplant. Isn't it funny how my new immune systems seem to take so much better to Rituxan than my old one?! 
3. August 2020
1. I had my two Rituxan infusions. Nothing to note. They went smoothly. Thankfully the infusion center nurse is absolutely stellar and extremely easy to chat with. 
2. My blood work has been showing some abnormalities we were hoping would dissipate with time. My white blood cells are still incredibly low - even for someone on Rituxan. As in, I have been Neutropenic for months... We took 9 vials of blood during my last infusion as my team wanted to run quite a few tests to determine exactly what is causing this abnormality in my bone marrow. Hopefully, we will have answers soon!

Outside of health updates, I currently spend most of my time down by the river. Being outdoors and soaking in some much-needed Vitamin D is so good for my soul. (While not so good for my skin due to my medication list! lol) Thankfully I have found many beautiful secluded spots to maintain physical distancing from others. I have updated my Amazon wishlist with a few items that continue to keep my daily care as stress-free as possible. https://www.amazon.com/hz/wishlist/ls/33N4NWO6A1DJF?ref_=wl_share I am so grateful to everyone who has donated items to ensure my home care is financially accessible for my family! 

I hope everyone is still hanging in there during this incredibly weird time! Xoxoxoxo 

Changes For My Social Platforms

It dawned on me that I’ve been documenting my life via a blog for nearly nine years. In 2015, after four years, I left the simple health updates of CaringBridge behind and started a more in-depth look at me as a whole. My thoughts, experiences, creativity, and ramblings we’re given room to grow on a more customizable blogging platform. I found my mind needed more room to unpack its reflections as my life became more and more consumed by illness. I named this blog, “A day in the life of the Tube Fed Wife,” having no idea that nickname would stick with me so many years later. This extension of my innermost thoughts, fears, and often dark reality, lead to more attention than I anticipated.

What began as my journal, a place where I could unburden myself from the thoughts constantly swirling in my mind, over time, became a community. A beautiful community of caring individuals who wanted nothing more than to support me and my family. I found tremendous strength and friendship through their kind messages and comments. Eventually, this incidental community where millions of individuals tuned in to read, watch, and listen to my musings, became a sort of online feast of my most intimate, fragile moments. While everything I shared was authentic, it was just that, EVERYTHING… and holding nothing back from millions of people leaves little to nothing left for you. My emotional decompression, my diary, slowly spiraled into a platform I felt governed by - instead of the other way around.

Bred by my unhealthy internalized expectations to keep this accidental community afloat, I widened my content to other social platforms that would be more convenient and resourceful. As each new social platform was added my blog became utilized less and less. Every day I feared letting that community down by not posting an update, image, or video. I become a moderator, content manager, and social curator, instead of a chronically ill individual writing for mental alleviation and comfort. My posts were no longer geared towards generalized reflections of my life with disease, but instead, reflections of what I felt the community desired the most within my battle. Even while my health spiraled I clung desperately to this shackled virtual reality. The one where I could make others happy. The one where I wasn't a burden. The one that, while gruesome, still looked curated and tasteful. The one where I was more than just a shout into the void of humanity. My online presence began to eclipse the true reason I started to blog in the first place. I found my first thought of each morning being worry over what content I would publish that afternoon, what interviews I needed to prep for that week, or what comments and messages I had yet to reply to. My method of stress relief morphed into an unhealthy, full-time job.

I do not regret the light I was able to shed on living with this unknown disease for so many years. And I certainly don’t regret experiencing the beautiful friendships, expressions of selflessness, support for my family, or incredible opportunities that sharing provided me. In fact, I am eternally humbled by those encounters. Those years were some of the best of my life, even at my lowest health. I am grateful, however, that my consciousness has grown enough over the last year to acknowledge the unhealthy side of sharing. The vortex that can suck you in and slowly begin to consume you.

Does choosing to share less mean I’m suddenly not sick? Does it mean that I a suddenly not struggling? Does it mean that I no longer want anything to do with the community that carried me through the hardest times in my life? Absolutely not! It means that I have grown. It means I can now see the harmful consequences of losing yourself. It means that I am moving back to basics. It means I am removing self-imposed pressures. In so doing I have deactivated my public social media accounts. So please do not worry if you can no longer find me on Facebook, Youtube, or Twitter!

I love each and every one of you who have supported my journey. I am so grateful you are in my life. This change is such a weight lifted off my shoulders. A weight of self-imposed commitment and expectation I have been trying to dismantle for years. I was simply never stong enough up until this point. I feel the lightest I have in ages!

I will still be around on my private Instagram @TheTubeFedWife and of course here on the blog with health updates.

Xoxo, Chanel

Rituxan and Iron Infusions

February and March have been sort of a dumpster fire. Technically, they have been for the whole world though. Like me, I am sure many of my readers are in quarantine. But, I get ahead of myself. Let's start at the beginning of February.

Noel and I made it to our mini tropical vacay before everything hit the fan - which we are so grateful for. We got to spend 2 full days on the north side of Oahu in ignorant bliss. My health actually cooperated, which never happens. It was a major blessing we both desperately needed. The sun, the sand, the ocean. I spent wayyyy more time in the water than I should have, but since I have my G & J tubes I cannot swim anywhere besides the ocean due to my high infection risk. Even then, I am supposed to keep it short. Yeah... I spent most days just lounging in the crystal clear waters of the North Shore lol Check out my Instagram for some photos! I unfortunately now have horribly painful granulated tissue around my G tube, funny enough though, the excess of saltwater seemed to have cleared up the infection pretty well! Totally worth it.

After getting back from Hawaii my fatigue really started to increase. I can't explain it, but I just felt sicker. I was pale and exhausted all the time. I mean, I am normally pale and exhausted all the time, but this was a new level. I had experienced similar symptoms about five years ago so we had my doc run some blood tests, low and behold, I had a lovely case of Iron Deficiency Anemia. So, we scheduled three weeks of Iron infusions to get my numbers back to normal. Unfortunately, this all went down the same week I was supposed to start my next Rituxan cycle. I received two rounds every five months. This resulted in a double whammy of back to back Rituxan / Iron infusions. Not pleasant. Neither infusion makes you feel good, in fact, they both make you feel pretty bad for about 48 hours. Needless to say, it was a very unpleasant three weeks.

About two weeks into these infusions is when Seattle became the epicenter of the Covid-19 pandemic. Everything shut down and the world became a very scary place for everyone, but especially for those who are extremely vulnerable to viral contagions. I am currently in full lockdown mode with no end in sight, unfortunately. My thoughts are with all the infected, and all the medical professionals on the front lines. We are so grateful for the sacrifices you have made to keep our loved ones alive.

Thankfully I am doing well in lockdown. I have had so many caring individuals reach out to check on me during this time. Actually, it seems the Rituxan and Iron have finally put some pep back in my step! (Just in time to be stuck at home! lol) I wake up with a bit of energy now and feel much less fatigue and weakness in my day to day routine. In other good news, it seems the Rituxan is finally benefitting other areas of my body, not just the lungs. I've noticed I can stomach a bit more which has been unbelievably exciting! I am not anywhere near eating regular meals, but, I am doing very well with smoothies and can even occasionally stomach some soft foods. It's really hit or miss, but something is better than nothing!

I hope everyone is staying safe, practicing good hand washing, and exercising their social distancing! Hugs! Elbow bump! hehe

January 56th 2020

I don't know about you all, but I feel like we're on the 56th day of January. lol I kid, but seriously. It has been a hell of a month that has dragged, and dragged, and dragged, and dragged... You get the picture.

After my J tube exchange my body just sort of revolted. My G tube stoma has been struggling with an infected abscess since the 7th. We've trialed multiple antibiotics. None have seemed to assist the infection for longer than a few days post the course. The last antibiotic we trialed, Amoxicillin, sent me into full-blown anaphylaxis. I have never had a reaction to this antibiotic in the past. Once my lips and tongue started to swell I had to administer an EpiPen and call the paramedics. They arrived quickly, which was a godsend, as the Epi wasn't calming the reaction as much as it should have. IV Benadryl was administered in the ambulance, as well as steroids and more IV Benadryl once at the hospital. I have never had an anaphylactic reaction outside of a hospital setting, so this was quite frightening. I was home alone and the reaction came out of nowhere. After 5 hours of monitoring, I was sent home to rest and recoup. I had a bit of a rebound reaction the next morning, but nothing serious enough to warrant medical attention. There is no doubt that EpiPens save lives, but wow, they really make you feel like absolute garbage for days on end. I'll take absolute trash over suffocating on my own tongue any day though.

I saw my Sclerodma specialist for a long-overdue visit.  We decided to move my antibody therapy up to every five months instead of every six. This means my next Rituxan infusion will be at the end of February instead of March. My skin is continuing to soften which is wonderful and my joints have definitely been less inflamed. He had me set up an appointment to get Punctal plugs since my eyes are really struggling. These are small silicone plugs that stop your tear production from draining too quickly, giving the eye more moisture. It is a common severe dry eye treatment. Well, my body hated them, unfortunately. I felt like they helped increase my eye moisture tremendously, but my eyes become very swollen and red. It looked like I had pink eye - so they had to be removed.

I'll be changing out my G tube this week to see if that helps with the chronic infection. My doctors have suggested we may need do some more invasive debridement of the abscess, which would involve moving the stoma, aka do a new surgery for that tube. I honestly can't even fathom that possibility right now. My GI issues make me feel like I am constantly treading water. It is exhausting. I am sorry I don't have a more positive update. It's just been a rough couple weeks with one thing going wrong after another.

Noel and I are supposed to leave for our first vacation together in years Monday; I am just so worried my health will prevent it. Honestly, it was probably pretty ballsy of us to even attempt a vacation in the first place, but dammit, I just want to sit on a beach so bad! All I want is three days of blissful normalcy like any other vacationing couple. I know that's not our reality, but I can dream, right? Needless to say, if you've made it this far, you maybe can tell I am feeling a bit angsty. lol I've been talking to my therapist and PCP for a while about these overwhelming feelings and we decided to up my antidepressants. I really don't talk much about mental health treatment on this blog. I feel like that should change. Honestly, it is a huge part of life with a condition like this.

So, hi, my name is Chanel and I take mental health medication. I cannot handle my reality, this reality, without a bit of pharmaceutical help. Depression, anxiety, PTSD, they don't care if you have the most wonderful support system in the world, they will still grab you by the ankles and drag you into their bottomless pit.

New Year 2020 Update

Happy New Year!

The last couple of months have been spent adjusting to life with my new J tube. Recovery was long and painful honesty. I would say about 7-8 weeks out from surgery I was finally able to resume my normal activities without pain. The stoma is healing really nicely and I have had no 'feeding tube' emergencies since this tube was placed. I did have a few minor clogs because my formula is so darn thick, but nothin a little at home Clog Zapper couldn't handle. The most difficult part of the last couple of months has been the large stitches holding in my tube. The way my surgeon placed my J tube, the stitches are the only thing holding it in.

About 8 weeks in my stitches started to become extremely aggravated. They were weeping and starting to tear through my skin. They all created massive hypertrophic scars. I couldn't take it anymore, plus was very worried about infection. I cut out the remaining stitched. My tube immediately fell 6 inches out of my body - luckily I have about 45cm of tubing inside me! Yikes. I taped the heck out of it and went into the clinic the next morning. The skin was too degraded to place stitched back in the same area, so we had to tilt the tube up a bit so the new stitches wouldn't bother the old healing sites. This was quickly done in office. Unfortunately, my body has already rejected those as well -much faster than the initial stitches. About 2 weeks post that placement the new stitches began to weep, tear, and become hypertrophic. I called my surgeon's team and explained that external stitches are not an acceptable long term solution for me. Thankfully, this failed set of second set of stitches finally changed his mind on the "stitches are a long term solution," idea. Hallelujah! My surgeon and my GI are working together to decide which type of tube they want to place and when. It will still be a J tube, but will have an internal bumper that way stitches won't be necessary. Don't ask me why this wasn't done in the first place, I was asleep and have no idea! lol This will be a quick tube swap done in IR. I am hoping this swap will improve my range of motion since the stitches right now severely hinder that. I have no idea when this will all happen since the holidays really fudge up communications between my doctors.

I met with my pulmonologist a couple weeks ago to see how the Rituxan is affecting my lungs. Great news - we are FINALLY starting to see improvement! My spirometry has improved! It's a small percentage, but definitely the right direction. I am thrilled! MY DLCO hasn't changed much, but stable is a good change in my mind! There was also nothing new on my CT. So, as of now, I can say the Rituxan has stabilized my lungs to a totally livable function which does not require the use of oxygen at rest OR activity!

GI symptom wise, things are about the same.  I am able to tolerate a little more orally now that I am finally receiving viable nutrition. It is amazing what proper nutrition can do for the body, isn't it? While the only progress I've maid is non-dairy smoothies, it's definitely progress. I cannot enjoy them entirely without symptoms, but at least I am not projectile vomiting every time I try one now. Success! My smoothie attempts aren't always successful... the other day I was violently ill in a thrift store bathroom because I attempted a new ingredient that morning. Embarrassing. lol I am so excited to enjoy even the tiniest bit of oral intake.

Other than that, I have been getting lots of rest, reading lots of books, and spending time with family.  I want to thank everyone who has assisted with my Amazon wishlist. I am humbled by the outpouring of support my family has received. My life is immeasurably easier because of those items. Actually, the generous individual who sent me a bunch of Clog Zapper actually saved me multiple trips to the ER! Thank you, from the bottom of my heart.

The beautiful images are courtesy of Body-Love Photography by Natasha Komoda. She is a local Seattle photographer celebrating the human body. My session with her was transformative and therapeutic, to say the least. For more of these powerful images feel free to check out my Instagram. To learn more about Natasha's mission, and to help her fund sessions for other low-income individuals, check out her website at: https://www.natashakomoda.com/bodylove

P.S. Wow! I turned three today! January 3rd 2017 I received my beautiful new stem cells. I am so happy to leave the 'terrible twos' behind! hehe This grueling treatment was supposed to be my miracle; it was supposed to keep my disease from progressing for much longer than two and a half years. I would be lying if I said I wasn't a little bit bitter... but the truth is I DID get my miracle. I am HERE! I am still alive. If I hadn't have had the transplant, I wouldn't be. This extra time is a miracle no matter how it has unfolded. So, while my nearly symptom-free years were fleeting, they existed - and for that I am grateful.

Hugs!