Sunday, April 1, 2018

Celebrating Life

Happy Easter! Part of me wishes I was 10 or 11 years old again—awaking to the sight of a pink Easter basket, complete with a stuffed rabbit and a surplus of the artificial grass that urged my parents to the brink of insanity. It was always strewn about the room right as we needed to leave for the church. I still long to have one last holiday gathering at my grandmother's. My cousins and I would race for the golden egg, which concealed a small cash prize instead of candy. I usually won. After the wonder of the Easter bunny subsided, and the fun-filled egg hunt ended in victory, we would gather indoors for food and conversation.

Now there are undoubtedly differences in comparison to my previous Easter celebrations. I am no longer a child. There are no Easter egg hunts. My grandma has since passed away. I cannot indulge in my gluttonous cravings with the giant chocolate bunnies. Family gatherings are difficult, as I relocated to another state. I normally do not attend church due to my health...until I attempted a sunrise Easter church service for the first time in years.


Churches are not typically a conducive environment for patients with Mast Cell Activation Syndrome. The perfume and colognes that cause severe reactions are in abundance, as the rest of the congregation are representing their Sunday's best. However, Easter poses a special circumstance. The early sunrise services are conducted outside. That keeps the triggers from concentrating in a small area.

So, my parents and I found a local church hosting an Easter sunrise service. We set the alarm for 5 AM, dressed in our Easter attire, and hopped (pun intended) over to the church parking lot. The sermon was wonderful, but the service did not exactly align with my expectations.

I have been struggling emotionally. A number of tears have been shed over my lack of ability to go out in public. Despite being outside, the quarters were too close and I could not handle the scent triggers. I had to watch the service from the car. The whole deal was inimical to my current mental state and struck at my already fragile emotions regarding the isolation of my diagnosis.

While the preacher was preaching, I begrudgingly observed all of the people from my spot in the car. They were unaware of the physical, active presence they have been privileged. Many arrived to find no available seats. My view was mainly of those standing. I took notice of their elderly age, which only furthered my internal pity party. I knew that if I were to stand there, remaining upright for an extended period as they did would be an impossibility.

And then I snapped out of it, almost ashamed that my moment of self-absorbed egocentricism served as a distraction from worshipping our Savior. I turned a service intended for Him into something about me because a life of illness is not necessarily what I had in mind. Enduring it in isolation is even more contrasting to what I once envisioned, but the Easter service revolutionized my perspective.

I was reminded that the purpose of Easter is to celebrate life, not death. Although I am flushed, tremoring, and nauseated with a migraine as I type, this is the life I was given. I am not yet dead, contrary to how my symptoms often feel. My suffering is no greater than the pain Jesus experienced. He endured His trials on earth with grace knowing of the superior heavenly life to come. I should strive to do the same.
Jesus replied, “The hour has come for the Son of Man to be glorified. Very truly I tell you, unless a kernel of wheat falls to the ground and dies, it remains only a single seed. But if it dies, it produces many seeds. Anyone who loves their life will lose it, while anyone who hates their life in this world will keep it for eternal life. - John 12:23-25, NIV
Jesus is alive. I am alive. Because of Him, I will live.






Like this post? Read What The Resurrection Means To The Chronically Ill

Sunday, March 11, 2018

Sick Girl

“If you had the choice to have never been sick—the chance to completely remove illness from your entire past, present, and future, would you?” My answers to the hypothetical question vary.

I am the sick girl. I always have been. As my illness progressed, I was exceedingly defined by my limitations. My every move became calculated. I was the friend who could not hang out on a whim. While most girls had purses filled with cosmetics and bubble gum, mine discreetly concealed a plethora of pills and a glucose reader. I was the kid at school who attended one period a day because my body would not allow me to regularly sit in classes like my peers. By college, I was the student with an extensive list of accommodations. On Sundays, I was the member of the church congregation who could not stand during worship service. I was also the significant other viewed as a cheap date, as I could not partake in the typical dinner and a movie and if I did, I ordered from the kid’s menu from a restaurant option I previously deemed safe. There was no spontaneity. All of these descriptions of my person point to a single definition. I am the sick girl. That is what people know me as, but I can be much more.


Many claim that their illness has impacted who they are today. They are better for their illness, so they would not change it. The former does ring true. Chronic illness incites a transformation on not only the body, but the mind and soul. The latter, however, is easy to claim on the other end of hardship—when able to look back with an “I made it” grin and a sigh of relief that the health trials are of the past.  

But I can't. The previous feelings of victory I experienced after recovering from a flare were fleeting. There is no end in sight now waiting over two years for the cessation of my last “flare,” scared to finally admit to the disease progression. With my pain less under control than usual, this condition has encompassed my present and seized my future. Recent accomplishments are slim, as the majority of my day is spent in survival mode on the verge of tears. My feeding tube stoma is causing issues, my small bowel is stuck in an ileus, and the new weakness symptoms I recently developed are wreaking havoc. I have exhausted all of my options nutrition wise.  

Due to the frustration, I occasionally find myself despising those who are healthier. I hate that they live without calculating their every move and make plans without physical hindrances. I am bitter over the fact that they eat without consequence; yet, I failed TPN, the last resort means of nutrition, and am barely holding my own with J tube feedings. I am sometimes even envious of the patients with my diagnosis that have found treatments that work for them because I tried the same medications with an opposite outcome.

I have become increasingly unempathetic, as if another’s problems are petty and do not exceed my own, like their unique battles are inferior and do not cause them sorrow. I do not wish to be filled with anger, nor to seem uncaring. I do not want others to suffer either. It is during these moments that the hardship of my illness has not refined my character positively.

“If you had the choice to have never been sick—the chance to completely remove illness from your entire past, present, and future, would you?”

I would prefer it if my illness had never existed. I ache to return to a time when living did not send my body into a tailspin of symptoms and I could physically live to my fullest potential. I am not better for it. Ask me tomorrow and my answer might be different, but right now, I am just the sick girl. And I don’t want to be.    


Sunday, March 4, 2018

Gleevec/Imatinib For Mast Cell Activation Syndrome

I began my trial of Gleevec, a drug belonging to the class of Tyrosine Kinase Inhibitors (TKIs), November of 2015...but let's back up. There are a few pertinent details that I must mention first.

I had already been inpatient at the hospital for over a month due to a Mast Cell Activation Syndrome flare up. I was stuck in a severe reaction cycle. I was unable to tolerate food and IV nutrition. Thus, my medical team contacted a specialist and then implemented the Continuous Diphenhydramine Infusion (CDI) in attempts to gain control of my symptoms.

A continuous infusion of Benadryl was not the most appealing of treatment options, nor were the massive amounts of IV steroids. The goal was to find an alternative that could take over what those medications were doing. With a lack of published studies on the CDI at the time, my medical team wanted to ensure I was not sent home on it. That is why oncology was consulted to trial Gleevec. Little did I know, I would remain inpatient at the hospital another four months.


I did document a portion of my trial well over two years ago in Gleevec and CDI Experience Update: Not My Miracle Cure. It was at the beginning of my blogging journey. Now that I am rereading, the initial post is incomplete and lacks answers to the common questions I am often asked regarding the treatment.

Dosing

The process of adjusting the Gleevec dose was lengthy. My doctors and I agreed to go very slow because of my history of medications sensitivities. A dose of 200 mg is typically the "sweet spot" for Mast Cell Activation Syndrome patients. Many do not notice any benefits prior to reaching that dose. My starting dose was a measly 25 mg once a day to test my response. I worked up to 25 mg twice daily after we determined whether or not I would have a severe, immediate reaction.

My dosing was increased in three day increments until I reached a dose of 100 mg as my total dose. Unfortunately, the oncologist decided to reinvent the wheel, so to speak, and did not abide by the plan of the specialist. The oncologist refused to increase to the dose known to be therapeutic for my condition. I spent an entire month on 100 mg with no benefits. Eventually, the dose was increased to 150 mg for one week before going up to 200 mg.

Administration

Gleevec cannot be compounded. It contains ingredients that are likely to trigger a reaction for sensitive patients —the red outer coating being one of them. The majority of patients trial Gleevec at home on an outpatient basis where taking the outer coating off is recommended to avoid a reaction to the excipients.
How to remove the coating:
1. Heat 20-30 mL of water in the microwave.
2. Submerge pill in the warm water for 5 seconds. 
3. The coating will peel away from the pill. 
4.  Take tweezers to remove the coating. 
Since I was inpatient, the hospital pharmacy was not legally allowed to have the coating removed. I was exposed to all of the ingredients in the coating.

I took Gleevec via my GJ feeding tube. The pill could not be crushed, so it was diluted in 30 mL of water, put into a syringe, and administered into the G port of my feeding tube. It is best to take the medication with food because it is harsh on the gastrointestinal tract. I did not tolerate food or tube feedings directly in my stomach. However, I ran J tube feedings before and after each dose to be safe.

Benefits

Like already mentioned, I noticed no improvements with the doses that were 100 mg or less. The 150 mg and 200 mg doses did prove to be slightly helpful! Within 36 hours of the 150 mg dose, I had a substantial decrease in POTS symptoms. My standing pulse had not been less than 150 beats per minute since July of that year and most of the time it had been hanging around 200 beats standing. With Gleevec, my standing pulse was between 100-110 and rarely exceeded 130. I was still having blackout episodes and losing vision and hearing upon standing, but my pulse was much better. There was also a reduction in the tremors in my hands, arms, and legs. My general overall "reactiveness" improved about 10-15%. Much to my disappointment, increasing to the 200 mg dose did not result in further improvement.

Side Effects

Towards the beginning of the trial, I had no side effects with the smaller doses from the drug itself. Yet, it did provoke Mast Cell Activation symptoms. The initial reactions from Gleevec did not differ from the reactions I had been experiencing to my other medications, water, and J tube feedings.

GI symptoms were a concern, but Gleevec did not cause nausea and it did not increase daily episodes of diarrhea. I did have intestinal pain correlated with my dose, especially in the evenings.

True side effects did not have a vast impact until I was a couple of weeks into the 100 mg dose. My blood counts began to drop—particularly my hemoglobin, hematocrit, and red blood cells. The counts were nearing transfusion level. Each week they would drop more.

The drug is also infamous for causing an imbalance of electrolytes. I had additional issues with low potassium. Supplementation via IV was enough to normalize the level.

Immune suppression is not ideal when relying on a central line. I had it from both Gleevec and high dose steroids (200 mg SoluCortef). While Gleevec provided modest relief, it was not decreasing the amount of Benadryl required to treat reactions. I remained very much dependent on my central line for the CDI and other IV medications. We wanted to give the Gleevec more time to work. So, my steroids had to be weaned to lessen my risk of infection. It was one or the other.

As the steroids were weaned, I gradually stopped tolerating the Gleevec. The pharmacist happened to walk in the hospital room in the middle of a reaction triggered by my evening Gleevec dose. The reactions entailed flushing, tachycardia, tremors, facial swelling, and throat swelling that was the deciding factor to stop the medication. She texted the entire medical team immediately and the Gleevec was discontinued from my treatment.

Gleevec was not my miracle cure. Had I not developed progressive reactions to the drug, it is probable that it would have to have been discontinued for its impact on my blood counts. I do not regret trying though. It was worth a shot!

Thursday, March 1, 2018

Tips For A Successful NJ Tube Placement

Prior to undergoing surgery for a surgical GJ tube placed directly into my abdomen, I became quite acquainted with the dreaded nose hose, also known as the NJ tube. An NJ tube is a plastic feeding tube inserted into the nose, down the esophagus, through the stomach, past the duodenum, to feed liquid nutrition into the second portion of the small bowel, the jejunum.

Reasons for feeding tubes vary. There are many medical conditions that require them. Regardless of the illness, the main commonality is that feeding tubes provide nutrition to those unable to eat food orally.

For a more in-depth explanation of the various types of feeding tubes, read: What Is A Feeding Tube?

So, here are my top tips for a successful NJ tube placement!!


Remain calm and ask questions. 

Surrounded by equipment and supplies, walking into the interventional radiology room to have a NJ tube placed is kind of intimidating. The NJ tube was my "first-step," so to speak, in the medical world. I had scant experience with tubes and lines before then. I quickly learned that an immense part of remaining calm is knowing what to expect. Have someone explain the procedure as it is happening. Do not hesitate to ask questions. It is your body and you deserve the clarification. 

Here are the basics that I do elaborate on in the further sections of the post: 

  • Unless there are extenuating circumstances, anesthesia is not given. And if there is, it is typically only twilight drugs. 
  • First, the length of tube to be inserted is measured and marked for a stopping point.
  • A guide wire is put into the tube for placement, giving it the rigidity to guide the tube into the small bowel.   
  • The NJ is then inserted into the nose, down the throat, past the stomach, and into the small bowel. 
  • Placement is checked via x-ray/fluoroscopy with contrast (or 60 cc of air if allergic to contrast) and the tube is flushed with water to assure tube function.    
  • The tube is secured to the cheek or nose with tape. 

Request water for insertion. 

An NJ tube is placed using fluoroscopy to ensure the tube is eventually situated in the correct section of the GI tract, the jejunum. That does involve laying flat on a metal table with an x-ray above the abdomen area, but the beginning of the placement is more comfortable done sitting upright. 

While inserting the tube, the radiologist will likely tell you to swallow. The act of swallowing lessens the chance of the tube going into the trachea. If tolerated, drink a few small sips of water. It helps decrease the gaggy feeling. Repeating the swallowing motion sitting up is helpful just until the tube is in the stomach. 


Think about food

Once the tube is past the nasal cavity and into the stomach, it has to be threaded into the small bowel. The opening of the stomach (the pylorus) has to open for the tube to advance to the intestine. That can take some time, especially in patients who are prone to dismotility. One great way to speed up the process is to think about food. 

I know what you are probably thinking: "I cannot eat. I need a feeding tube for nutrition. Yet, this girl is telling me to think about food. How torturous!" 

Thoughts of food cue the body to prepare for digestion, causing the pylorus to open. I have had 4 NJ tubes. Placement time was cut in half when the radiologist made that suggestion. From that point on, I always dreamed of an apple cinnamon muffin for each consecutive placement. Yum! 


Lubrication or sterile water 

Lubrication jelly is typically offered for placements. It is not as painful if the tube is well lubricated because it has to be inserted into the sensitive nasal cavity. However, there are certain situations where jelly may not be an option, like in the case of allergies. Mast Cell Disease causes allergic reactions to many triggers. Lubrication jelly happens to be one of mine. I was not able to lubricate with the jelly for all of my NJ tube placements. The radiologist used sterile water instead—running it along the outside of the tube as it is advanced.


Adjust tube location and rotate tape. 

Immediately following insertion, nurses generally try to tape the tube to the front of the nose. Perhaps I was vain, but I wanted my NJ tube to look as inconspicuous as possible. Transforming my nose into a smaller version of an elephant trunk did not suit those desires, nor was it comfortable. I asked the nurse to relocate the position of the tube to the side of my cheek with clear Tegaderm. 

Unfortunately, my skin could not hold up with the adhesive. Rotating tapes prevented building up a sensitivity to any one products. I found paper tape to be the easiest on my sensitive skin. There were occasions where I was also successful with:

  • Tender Grips 
  • Duoderm 
  • 3M Micorpore 
  • Covidien 

Remember that the gag reflex does dissipate. 

Since the tube does go down the back of the throat, it does hit the gag reflex. During placement, I asked them to stop advancing the tube until I was able to adjust to the sensation enough to continue. Afterwards, the tube felt like I was choking on a giant spaghetti noodle. It did cause me to dry heave while swallowing. I spit my saliva into a cup for a few hours until the feeling passed.


Expect soreness in the nasal cavity and throat. 

The nasal cavity and throat are not accustom to a foreign object residing in such sensitive mucosa areas. There will likely be pain. 

Personally, my nose was not very sore initially. It built up over the duration of a couple of months. Using a saline spray in the evenings soothed nose pain caused from the friction and  dryness from the tube. 

Contrary to my nose, my throat did hurt extremely bad. The first placement had caused the worst sore throat I had ever had. It was comparable to swallowing needles. I gave the pain a week to dissipate prior to pulling the tube out at home. Within a few days after it was pulled, the threat pain subsided. The tube was replaced with a smaller pediatric size and I had no pain the second go. Swishing with 'Magic Mouthwash' or a combination of Maalox and Benadryl gives at least a little relief. I give the recipe in this video: Spooky Feeding Tube Experiences (NG/NJ/GJ).

Despite the brief physical discomfort of the procedure and the overwhelming emotions of adjusting to life with a feeding tube, I am immensely grateful. I learned love my NJ tube. Symptom wise, I felt better even 24 hours later. The plastic contraption was a symbol of healing by giving my body nutrition it needs to thrive. 



Monday, February 19, 2018

Where Then Is My Hope?

I remember it vividly. I was barely a teenager when I stepped into the autonomic clinic. My medical journey had just begun. A copy of Stephen Chbosky’s The Perks of Being a Wallflower laid in my hands, an attempted distraction from the boredom of the waiting room. I counted on a million ‘if only’s— if only I encountered the right specialists, if only I found the magic medication, if only I underwent the appropriate testing, if only the doctors found the underlying diagnosis, if only…if only.  

And thus, I flew from my home state of Florida to overcast Ohio for an appointment with Dr. Tom and Gisela Chelimsky. He is a neurologist, she a gastroenterologist. They join forces on scheduled days for their autonomic clinic. Although previously diagnosed with POTS by the Mayo Clinic in Minnesota, the Chelimskys confirmed that diagnosis, added neurogenic bladder and fibromyalgia to the list, and aided in symptom management. All of the traveling since becoming ill had to lead to answers. If only I encountered the right specialists.


Conditions under the ‘Dysautonomia’ umbrella, like POTS, rarely occur alone. The same applies to my other diagnoses of Mast Cell Activation Syndrome, Gastroparesis, etc. Each is caused by an underlying cause. During my appointment, the Chelimskys suspected that they knew mine—Mitochondrial Disease. I was told not to research the condition online. There is a wide spectrum in terms of patient severity, and a search surfaces the most petrifying. I left the appointment under the direction to take Coq10 and carnitine. The supplements are included in the ‘Mito cocktail.’ (The Coq10 helped with episodes of weakness and the carnitine did nothing). If only I found the magic medication.

Well, I did Google Mitochondrial Disease. I cried all night in the hotel bathroom. The thought of Mitochondrial Disease was eventually dismissed. Two years later, after it was mentioned by a second neurologist, the possibility resurfaced. I was sent to see Dr. Shoffner in Atlanta, Georgia. The neurogeneticist conducted a series of blood draws, urine collections, a muscle biopsy, a skin punch, and a lumbar puncture as part of the genetic workup. If only I underwent the appropriate testing.

A couple months passed and I received an email informing me that the remainder of my tests were not in; however, the lumbar puncture revealed unexpected results. My methylfolate levels in my cerebral spinal fluid were extremely low. It was already impacting my neurotransmitters and I was positive for folate receptor antibodies. I was diagnosed with Cerebral Folate Deficiency. I did not align with the typical presentation, but I was prescribed a medication called Leucovorin as treatment. To an extent, I was relieved to have been given answers. I had finally found helpful doctors. I had testing. I found a diagnosis to be treated. The ‘if only’s’ I hoped for seemed to be transpiring.

I was never able to make it to my follow up appointment, as I ended up inpatient and was unable to travel. Following my lengthy admission, the doctor had gone strictly to research and was no longer seeing patients. I was to have my pediatric doctors in Florida handle the situation. They were stumped. Cerebral Folate Deficiency is normally caused by Mitochondrial Disease, but my muscle biopsy was inconclusive with non-specific findings: atrophy, amino acid and acylcarnitine abnormalities, and 2 heterozygous mutations that are “likely not disease causing.” In the interim, I started rejecting oral medications due to reactions from my Mast Cell Activation Syndrome (MCAS). Unaware of the brain damage consequences of untreated Cerebral Folate Deficiency, the Leucovorin was discontinued. I had not noticed benefits anyways. Apparently, it takes up to 9 months of treatment to notice improvements. That tidbit was unknown at the time.

Now that I have moved and switched care to adult doctors, I am restarting treatment for Cerebral Folate Deficiency. Instead of the tablet, I will infuse Leucovorin via a central line to bypass reactions in the GI tract. Current doctors are just as perplexed—if there is no obvious confirmation of Mitochondrial Disease evidenced through the muscle biopsy, what is causing the Cerebral Folate Deficiency that is common amongst the Mito patient population?  

My records were sent to a local geneticist specializing in Mitochondrial Diseases and metabolic disorders. I have my appointment on April 4th.  With this health regression, I will try anything to help me feel better. I want answers more than ever because maybe, just maybe, the mast cell disease will improve if only we find the main underlying diagnosis.  

The ‘if only’s’ returned much quicker than they had left. The tests only prompted additional questions, the specialists more doctors, and the medications new symptoms while not preventing progression.

I am not that same 14-year-old girl reading in the waiting room of the autonomic clinic . I am sicker than I ever thought possible at age 21. Out of sheer desperation, I placed every last inkling of hope into modern medicine. It is not like God cannot use those things as a vessel to do His work. He certainty can. However, they must be recognized as such. No doctor, no medical test, no medicine, and no diagnosis can heal me. I cannot put my faith there. God alone can fulfill my ‘if only’s.’







"Where then is my hope—who can see any hope for me?" - Job 17:15, NIV

Friday, February 9, 2018

What Doctors Do Not Tell You About Your Feeding Tube

I am a strong advocate for portraying the positives of any situation. There is no use in focusing on the negatives that cannot be changed. Despite this, I realize that maintaining an incessant happy happy joy joy demeanor is unrealistic.

As evidenced by my last post, Feeding Tubes Are a Good Thing and there are endless reasons why.


I had my first feeding tube placed at 16 years of age. Prior to that though, the prospect of a tube was threatened by my doctors on numerous occasions. Yet, they failed to offer explanations, good or bad, about the device that supposedly saves lives. I was left with, “Okay, you are starving. Here is a feeding tube. Now you won’t die.” I had to form my own conclusions and a feeding tube was the worst possible outcome. The prospect was petrifying.

All of my energy went into avoiding it. Stepping on the scale was a tear inducing ordeal. As the number on the scale decreased, I knew I could not continue in a state of malnutrition. And then I was tubed. Initially, resorting to tube feeding seemed as if I had given up. It was like I had lost the fight, succumbing to hell that meant living with a feeding tube.

Shortly after my GJ feeding tube surgery, I realized how incorrect I was. My life had changed, but it was not for the worst as the doctors previously implied. It was for the better. My quality of life had drastically improved. I was less nauseated, had energy, and my fainting and blackouts lessened. I became thrilled about life again because my feeding tube opened a world of new possibilities. I anticipated the future with excitement rather than nervous dread of the unknown.

So, I try to raise feeding tube awareness centered around the good—the positive outcomes not often spoken about. Still, an important facet of awareness is authenticity. Just as medical professionals fail to mention the positives of a feeding tube, they do not even begin to cover the challenges. It would be wrong of me to pretend that they do not exist and to claim that I did not believe in a plethora of the feeding tube misconceptions.

 I would tolerate the feedings and my symptoms would disappear.  

Food and I were obviously not working out. A feeding tube meant I did not have to rely on consuming food for survival. Problem solved, right? Such an assumption was a bit misled. In my case, the issue was not food, but my GI tract. Placing a feeding tube does not heal my faulty GI tract. It is simply a tool that makes the intake of nutrition a little easier. There is no guarantee that formula will be tolerated without symptoms.

Complications would not happen to me.

Infection, buried bumpers, tube migration, stoma burns, granulation tissue…complications are real. No tubie is immune to them.  

I would never feel hungry.

I was under the impression that I would never want food due to how ill it makes me. Despite having a feeding tube, I do get hungry and I do crave regular food. Thankfully, I will not exhibit physical signs of hunger when receiving sufficient calories through my feeds. If circumstances require an increase in calories, like if sick with a virus, hormonal influences, etc., then my stomach will start growling and I will feel hungry.

The tube stoma does not hurt after the initial surgery.

My feeding tube site, the stoma, is always super sore. There are various explanations for stoma pain. After the initial surgery, the pain in my abdomen was comparable to working out and doing lots of sit-ups. Within a full month that muscle soreness pain completely dissipated. However, it was replaced with a new pain.


The body attempts to close the new wound during the healing process, but it can never fully heal because the tube is holding the stoma open. That results in a buildup of tissue around the stoma called granulation tissue.   With the tube present in the GI tract, the body’s natural instinct kicks in. The stomach tries to digest the tube. Although the tube is held in the stoma by the internal bumper, digestion causes movement of the tube—a feeling similar to a spasm and like the tube is sucking in and out.

Healing, movement from digestion, and daily activity all contribute to granulation tissue. Granulation is perfectly normal, and some have it worse than others, but it hurts! The tissue contains nerve endings. As the tube moves, the granulation tissue bleeds and the pain increases.

By the end of the day, my abdomen is really sore!  

Eating for pleasure would be easier.

A feeding tube definitely does not prevent a patient from consuming food orally, nor does it mean that they should not try! Before I Mast Cell Activation became a factor, there were moments when I could eat ice cream, apple sauce, or drink and it would all literally pour out of my stoma onto the floor.   Leaking varies. My stoma tends to leak excessively. Adjusting tube sizes is helpful, but does not totally eliminate the leakage. It causes oral eating to be difficult. Aside from causing symptoms related to my condition, the leaking adds to the pain because the food particles irritate the stoma and causes burns from stomach acid around the site. I have a very high pain tolerance. I have endured sepsis and multiple surgeries without pain med one, but there is something about burns around my feeding tube stoma that makes me cry like a baby.

I wouldn’t mind if I were connected to a pump the majority of the day.   

The amount of time I am connected to run J tube feeds has differed depending on my health. It is rarely less than 12 hours. Typically I run feeds for 16-18 hours. Tubing, a feeding pump, and a backpack is my best friend for the duration I am on feeds. I usually accept that fate with a smile. Inevitably the tubing starts to tangle, air gets in the line, or I am restricted from an activity…and then feeling “tethered” to tubes and lines is incredibly frustrating.  


There is no doubt that my health has declined since I was first tubed. I was exceedingly more functional then. And because of that, I now occasionally feel that the pain and inconveniences of a feeding tube do not warrant the benefits. But no matter how many melodramatic just pull out my tubes and let me die moments I must endure, it is a small price to pay for being alive.






Feeding Tube Awareness Week 2018

Tuesday, February 6, 2018

A Feeding Tube Is A Good Thing

"Hello, my name is ______ and I have a feeding tube." Such forthright statements make for some pretty awkward introductions.

There is an overall stigma that feeding tubes are "gross," "weird," or "only for babies and old people." To be clear—no, those with feeding tubes do not constantly smell like vomit and are not gross. It seems weird because the concept is unfamiliar. And it is true that babies and geriatrics have medical conditions requiring alternative feeding methods, but feeding tubes are not isolated to one patient population. So, hiding the fact that I have a feeding tube fuels that stigma rather than clarifying common misconceptions.


Pouring formula into a bag to later prime the feeding pump is just as normal to a tubie as cooking a spaghetti dinner is to the able bodied. It is a daily part of life and not given a second thought...until it is, which is when it is important to explain tube feeding to others.

As tubies, we are accustomed to life with a feeding tube and all that it entails. We tend to forget that other people may not know how to respond. Whether with family members, friends, strangers, and acquaintances—there are inevitably situations where the topic arises. There are a few thoughts that tubies should keep in mind!

Be honest.    

When it comes to tube feeding, I abide by honesty is the best policy. It is a balance between giving enough information without seeming completely overwhelming. An immediate confrontation spewing random facts and tidbits about my feeding tube and why I need it is obviously inappropriate. However, if the student in the next seat over offers a snack or if a coworker proposes the prospect of meeting for lunch, it might be worth briefly mentioning.

Methodically plan responses. 

Explanations change as the relationships grow and change. Initially, keeping it short and sweet is sufficient. An acquaintance probably does not need elaborate in-depth details, but a close friend, family member, or potential partner does. Later, staggering tube comments and responses based on the role that person plays in my life is helpful.

For example, I told my now fiancĂ© about my feeding tube in the friendship stage prior to beginning the relationship. I happened to slip subtleties about my illness and the possibility of a tube in conversation. I felt he must be aware of what he was getting into.

They stress too.   

Sharing the part of life that revolves around formula cans, feed and flushes, and unexpected tube feeding catastrophes can be difficult. Regardless, it provides the ideal opportunity for others to ask questions for awareness. Similar to how we stress over when and who we should tell about our feeding tubes, they too have worries: Am I allowed to eat around a tubie? What questions are rude to ask? Is this insensitive?

Spread awareness. 

A feeding tube, while perceived as negative, is a good thing. It gives life, where without it, there would be none. Why not share the good news? You would be surprised to learn that most are okay with the idea of feeding tubes and are genuinely curious.






Feeding Tube Awareness Week 2018: February 5-9th. 

Wednesday, January 31, 2018

Not If, But When

A repulsive, disbelieving gaze diverted to the lumens dangling from the upper portion of my arm. I never truly apprehended its meaning as the scratchy, repetitive noise of the portable IV pump resonated in my ears. It was a reminder that this was not supposed to happen. Central lines were for the patients who were really super sick, the ones who had exhausted all of their options. That could not be me, could it?

But it was. I was airlifted via Angel Med Flight to a hospital out of state due to uncontrollable, severe reactions caused from God knows what. The first of my countless PICC lines was placed for TPN and high dose intravenous steroids. Oh, and Benadryl as needed. Eventually, we learned my reactions were a manifestation of Mast Cell Activation Syndrome.


Total gut rest on TPN and IV medications lessened the severity and calmed the reactions down. I was later sent home to begin real life with a central line. Suddenly, the horror stories replayed in my mind—sepsis deaths, site infections, blood clots and DVTs. My doctor was less than comforting. He offered no consolations to ease my mind, only risks to stress the seriousness of the situation. While the line was and is 100% necessary to save my life, it could take it just as quickly. He said, "It's not if, but when."

In the beginning, I was meticulous with my line. I knew I had to be. However, I was not over the top. I also trusted other medical professionals with line care. Although I was previously familiar with central line dangers, I never fully understood before my two battles with sepsis. (The first was from a skin bacteria and the second after the clave fell off of my bi-fuse extension). My entire perspective changed. I was no longer meticulous with my own line care, but absolutely anal. And my trusting nature with medical professionals disappeared, as I witnessed some of their mistakes with sterile protocol.

Since then, central lines have continued to be a critical asset to my treatment. I do not tolerate any oral medications and am reliant on continuous infusions. I must live knowing the impending risks of central lines and unconventional treatments like the Continuous Diphenhydramine Infusion, or not live at all. But is life with that kind of overwhelming anxiety considered living?

I recognize the fact that I can do everything correctly and sepsis can still occur. So, when the nurse leaves the flush uncapped a little too long, I kindly ask him to do it again, because it is hard living with the premise, "It's not if, but when."



Saturday, January 20, 2018

Hospital Admissions With Mast Cell Disease Part 3: Hospital Checklist

Wallet: check. IV medications: check. Change of clothes: check. Tube feeding supplies: double check. I often joke that I bring everything, including the kitchen sink, just to go five minutes down the road to the nearest store. Going to the hospital is no different. In fact, it requires even more planning!


A recent pre-planned hospital admission to begin TPN really got me thinking. Mast Cell Disease presents unique circumstances. I previously wrote a series of blog posts about hospital admissions with Mast Cell Disease. The first post addressed the never ending battle of confronting medical professionals that are not knowledgeable about the condition and its manifestations.

Read Hospital Admissions With Mast Cell Disease: Part 1 

The hospital can easily become a dangerous environment because Mast Cell Disease causes reactions to commonly used chemical cleaners, fragrances on hospital staff, and airborne and ingested foods. So, the second post elaborates on how to navigate those added triggers in an inpatient hospital setting—like alternatives to overcoming scent-laden doctors and nurses, as well as prepping allergy friendly meals with the hospital room as a kitchen when their menu offered is not safe.

Read Hospital Admissions With Mast Cell Disease: Part 2

With trigger exposure significantly increased, the necessary items for a hospital admission are not the straightforward PJs and snacks. It is important to have a hospital checklist to prepare for any potential situation that may arise while out of a home environment.

Medical Supplies

  • Medications (Scheduled and PRN) / Pumps, Pump Batteries, Syringes, Bags to Administer 

Some patients, like myself, are brand specific on medications and bags/syringes in which they are administered. Other medications are not easily obtained because they must be compounded to remove the fillers and preservatives. It is imperative to have ALL medications on hand because there is no guarantee that the hospital will have safe versions of specific medications. The hospital can "check in" home medications for the nurse to administer.

Home health infusion companies are not allowed to deliver if the patient is admitted. Instead, the company should be able to send a currier to the hospital pharmacy with the medications and supplies.  That way, the hospital can prepare medications and supplies identical to the way they are prepared at home.

  • Feeding Tube Formula, Pump, Pump Charger, Bags, and Extensions (if applicable) 

While in the hospital, I am usually on tube feedings or supplementing with tube feedings.  The hospital does not always have my special elemental formula in stock. I bring a few cans of the formula to use until the nutritionists are able to order it. 

Extra extensions for tube feeding and venting are also nice to have. Inevitably I will drop my extension for my mickey GJ button on the hospital floor. As a result, it takes many hours and five nurses to hunt down a replacement extension. 

Hospitals tend to prefer patients use Kangaroo feeding pumps. The pump I typically use from home is the Infinity. I always pack the pump and its compatible bags to use through the entire admitting process—whether it is in the ER or in between Kangaroo pump failures. (Trust me, those do happen)! 

  • Cooler 

Perhaps it seems crazy to tote a cooler through the hospital. It's not like a trip to the beach, requiring an artillery of refreshments, right? The cooler is beneficial to store safe food and water, as well as home IV medications that must be kept refrigerated.

  • Vogmask with Carbon Filter

The hospital is brimming with germs and scent triggers. The yellow masks typically provided are not suitable for a sensitive patient with Mast Cell Disease. They do a poor job at keeping out the strong cleaning chemicals and perfumes and colognes on staff. I prefer the Vogmask with the double carbon filter to ensure my safety because triggers are even encountered when cooped up in the room. 

  • NO Scent Warning Sign

The Vogmask is not entirely effective for scent triggers. I put a hand-made allergy warning sign on the outside of the hospital room door. Examples are in this post. It lessens trigger exposure by warning those wearing scented products not to enter. Unfortunately, there are always some who believe a medical degree exempts the warning from applying to them. So, the mask is backup! 

  • Central Line Dressing Kit / Alcohol + Betadine 

Again, I am very brand specific due to wacky mast cell allergies. If the admission is longer than a week, I pack a dressing change kit and my safe brand of alcohol or betadine in case the particular dressing kit is not available at the hospital. 

  • Tubie Pads / 2x2 Split Gauze

I go through gauze and tube pads like they are going out of style. Psh, I wish. When I forget to bring my own, I am stuck with the bulky 4x4 gauze at the hospital. I quickly learned not to forget! 


Self-Care

  • Unscented Soap/Shampoo

I do not tolerate the soaps offered by the hospital, despite it usually being delicate Baby Shampoo. 


Washing my hair in a puke bucket when unable to get out of bed is less than glamorous. Inevitably, it makes a mess! A friend made me aware of the hair washing trays and/or basins during my most recent admission. Hair washing is simplified with the tray, as a bed or chair can be moved to the sink. 

  • Hair Dryer, Hair Clips / Ties 

  • Toothbrush with Safe Toothpaste

  • Unscented Hand Soap

Nurses and other hospital staff are required to wash their hands and use hand sanitizer upon entering and exiting the room. Depending on the hospital, the hand sanitizer and soap are both scented and occasionally cause reactions. I pack safe hand soap and place it by the sink for all to use. 

  • Safe Cleaners

The products hospital janitors clean with cause me to react. They obviously clean my hospital room prior to my arrival, but any cleaning thereafter must be completed with safe products. I bring large alcohol wipes to clean surfaces if they do not agree to using water only.


Entertainment

  • Card Games
  • Laptop
  • iPod
  • Chargers for electronics 
  • Book / Kindle
  • Clip on Book Light
  • Headphones


Documentation 

  • Specialist Plan 

The average hospitalist is not very informed of Mast Cell Disease. And that is not a bad thing if they are open minded and willing to learn. Still, it is not wise to demand treatments in a confrontational manner. Having a written treatment plan from a physician that specializes in the disease is a great teaching tool and any medical professional is more apt to follow instruction from a fellow doctor than a patient.  

  • Contact Information

With a complicated condition, the more people on our side, the better. A written plan from the specialist is helpful, but a hospitalist might have further questions. Give them the contact information to the offices of other doctors on your medical team.


  • Medical Records 
The majority of admissions are to fix emergent situations and not for investigative purposes. Yet, it is convenient to have a binder full of medical records to reference.


Other

  • PJs / Comfortable clothes 
  • Socks
  • Sheets, Pillows, Blanket

A good night's sleep does not consist of your head sinking a foot into the pillow. While the hospital bed comes compete with linens, I bring linens form home that I know are washed in a familiar detergent to place on top of what is already on the hospital bed. 

  • Slippers
  • Rubber Flip Flops 

Showers in the hospital are what I call grody. The flip flops decrease the "ew" factor just a smidge. 

  • Positive Attitude 

Days in the hospital run together. Looking out of the small window, people as small as ants are participating in the hustle and bustle of life—oblivious to the isolated patient watching from seven stories up. 

Feeling sick makes it difficult to remain optimistic, but a positive mindset going into an admission really is essential. Try to keep from going stir crazy. I made it eight months. You will be home before you know it! 




Monday, January 8, 2018

What A Chronically Ill Patient Wish Medical Professionals Knew

I just got discharged from a four day hospital stay. Before then, it had been awhile since the hustle and bustle of the hospital world collided with my own. This most recent admission surfaced a bit of emotion. Confrontations with nurses and doctors became overwhelming. While some medical professionals can drone on forever, losing themselves in science-y musings of the human body, it reminded me of the valuable information I wish they knew when handling their chronically ill patients.


#1 When I complain about pain, it is significant. 

I do not moan about my many ailments to be a drama queen. In fact, I prefer not to break out the melodramatics. I keep most complaints to myself. When I do mention my symptoms, they are serious.

I have an exceedingly high pain tolerance. For example, due to severe allergies from my diagnosis, I suffered through sepsis without any pain medication. Therefore, I might not seem like I am in pain when in all actuality, I am. Perhaps I am engaging in conversation, happily knitting, or completing homework—none of which disregard the pain I claim to be in. Conversely, this applies to the opposite, too. It is incorrect to assume I am pain free judging solely by external circumstances.


#2 I do want to be compliant.  

Medical professionals are often sought as a wealth of knowledge in their field. They are paid to offer their advice and suggestions each appointment and most do their job in the best interest of their patients. However, my chronic illness is not typical. Its ensuing health problems are not always obvious and are not guaranteed to be treated in the same manner as the average case. Years of medical mistakes have resulted in a lack of trust. Try not be frustrated if I am not immediately game for every treatment or procedure you mention.


#3 Being sick is stressful, but stress is not the cause of my symptoms. 

Pills, appointments, uncomfortable symptoms. Managing day to day life while chronically ill is difficult, especially considering the health implications that may result. It gets frustrating. As a psychology major, I know how mental health can vastly correlate with physical health. That's why I have learned helpful coping mechanisms. Dismissing my symptoms as a consequence of stress or anxiety is not okay. Stress and anxiety are two common repercussions that are secondary to long term chronic illness, but they do not cause my genetic conditions.


#4 Accommodating my quirky medical needs is appreciated.  

One of my diagnoses, Mast Cell Activation Syndrome, causes life threatening allergic reactions to perfumes, lotions, and scented products. Even in a medical environment, controlling all triggers is impossible. Every now and again a strong laundry detergent or hairspray will get to me. Whether it is changing into hospital scrubs or wearing a gown, it makes my family and I abundantly happy that you are willing to go out of their way to keep me safe!


#5 Appointments are just a snapshot of my day. 

Appointments are booked for a 15 to 20 minute time slot. That duration is probably the most put together and functioning I will be in a day's span. A smidge of makeup and nice clothes can conceal a lot! You do not see the remaining 23 hours of my day: fainting upon waking, puking up my lunch, or the nights spent in agonizing bone pain. Take that into account before sending me away without a concise plan, the discouraged expression clearly evident as I exit the office building.


#6 Without experiencing chronic illness firsthand, you do not know what I am feeling. 

I would not wish chronic illness on my worst enemy. It is not easy to convey what my body is feeling. An attempt to empathize does provide comfort, but forget the comparisons. They are usually inaccurate. True understanding of a patient's point of view only comes from those who have endured a chronic illness themselves. To say you 'get it' is almost belittling.


#8 Patients and doctors are both real people. 

Medical professionals have a career to be admired. Although your intervention in my care is necessary, you are more than prescription writers and procedure performers. That is why I ask the sonographer about her weekend or the cardiologist about his family. My desire is to be treated as a real person, so I treat you as such. I do not want to be viewed as the next number in an assembly line of problems to be fixed.


#7 I cannot do this without you. 

I do not have the resources to control my medical care. Despite my greatest efforts, I cannot prescribe my own medications, order tests, and operate in surgery. I cannot diagnose, organize hospital admissions, and schedule follow ups. These things are imperative to the chronically ill. You have the ability to change our lives. For the good or for the bad. Please don't abuse that power.



Tuesday, January 2, 2018

Letters To Myself

I wrote two letters to myself at the end of last year. They were both packed away in my stocking with the remainder of the Christmas decorations. The odd hiding place was intentional to ensure I found the letters exactly a year later. Their contents reflected the best moments, worst moments, adventures, and accomplishments of 2016.

Now I reread both letters. To My Future Self follows the best moments, worst moments, adventures, and accomplishments of 2016. From My Future Self summarizes expectations of 2017 that I had at the end of 2016, and is written as if my hopes had actually come to fruition.


Although 2016 is old news, and the focus of this post is 2017, my words then prevail. And in another year, my words now will hold true too.

The worst of 2017...

My health sort of plateaued in 2017. There was some semblance of unstable stability with my Mast Cell Disease diagnosis. Symptoms were on an even keel, providing I abided by strict trigger avoidance. I remained on the Continuous Diphenhydramine Infusion, SoluCortef, and small doses of intravenous Pepcid. I did tube feeds only with minimal amounts of water by mouth. I could not be in a public settings for more than half an hour. A slight alteration in just one of those things sent my body into a downwards spiral.

After not enduring the same dramatic health fluctuations of the previous year, I had time to focus on how different my life is. I fell into the trap of comparison, comparing my life to that of what it used to be and to the lives of peers my own age. Resultantly, 2017 was one of the most emotionally difficult.

I found it difficult to accept that my life was stagnant. Yet, "living to the fullest" in the way I yearned for was an impossibility. It caused to weeks to months of life threatening flares whenever I tried. I did have physical gains, but they were not enough for me to function outside of my rigid limitations. And it was not like fear was a mental restraint holding me back. It was a proven reality. I lost a close friend to a shared diagnosis and the loss made that reality significantly more real.

I distanced myself from others solely because of envy. Because they have the life I want. The life I can't have. I got angry at God for not allowing it to be.

The best times of 2017...

Feeling moderately better did not prompt the sudden influx of adventures I desperately yearned for. I was forced to confront the unproductive emotions I had struggled with.

I continued my education online. A year into my bachelors degree at a Christian college, having courses based on a Christian perspective was refreshing. I was able to take classes like Jesus In Matthew's Gospel that were not part of my major, but they were helpful in reconciling my relationship with God and releasing pent up anger. It really got me seeking God's will as opposed to my own.

November 2017
I met multiple best friends. We had connected years ago on social media through various chronic illness tags, but we had not ever met in person until this year. I am extremely blessed to have supportive friends. Their occasional company provides a precious sense of normalcy. I will never stop being thankful for those who are understanding.

Of course the highlight of the year was getting engaged to the love of my life, which I wrote a full post about in November! The center stone in the ring is from the wedding set my biological father had given to my mom before his passing. My fiancé and I experienced our first holidays as an engaged couple. It was so so special.

Read The Blessing of Chronic Illness In a Relationship.

December 2017
Winter is my absolute favorite. I got to see it snowing at home twice! Sledding down the hill in the backyard was a blast. My family, friends, and I made a snowman larger than me, complete with a top hat and a cute carrot nose.

Mom and I had endless Netflix binges, an inevitable consequence of spending sick days on the couch. Let's be real, "sick days" were a constant. The positive from such unfortunate circumstances is that we had extra quality time together that would not have occurred had I had the life of the typical twenty year old college student.

January 2017
Accomplishments of 2017...

I sent in applications for miscellaneous scholarships to contribute to my college tuition. Thus far, I have not had to pay aside from book materials. The financial protection shows that God never fails to provide.

My current medical team was tremendously helpful in managing my care outpatient—the total opposite of my health plan prior to moving out of state. I went a full year without a hospital admission!!! That is saying something for the girl who once spent 8 months out of a year in the hospital.

December 2017
Hospital or not, steroids were a necessary evil. I was on 150-200 mg of SoluCortef for daily maintenance to control my disease. Weaning in the typical manner was often unsuccessful. Decreasing the dose quickly or by multiple milligrams caused anaphylaxis, extreme bone and nerve pain, pancreatitis, and cardiac issues and arrhythmias.

Throughout the year, I discovered an appropriate method to wean. I made it to a total of 50 mg daily by weaning 0.5 to 1 mg per every two weeks. I am adrenal insufficient from long term steroid use, but I have come so far in my wean!

December 2017
Slowly upping my walking distance, considering I was mostly wheelchair bound before, is a HUGE accomplishment. I went from staggering to the bathroom and back to walking around the neighborhood. I also increased my exposures. While never without repercussions, I went in to the office supply store, Old Navy, Catos, Books a Million, and Barnes and Noble. My shopping excursions lasted all of fifteen minutes. I cherished that brief freedom nonetheless.

Predictions...

Going back to the letter From My Future Self, my predictions for 2017 were definitely wrong. I did not get the cure I wanted. I did not even find a medication to help, let alone a treatment that would allow me to stop the experimental Continuous Diphenhydramine Infusion that I have relied on for over two years. Every trial was a fail. I lost people I loved. I doubted my faith. Regardless, I came out stronger on the other side of the obstacles faced.

October 2017
My reflections are a reminder to keep persevering. Going into 2018, the start of the New Year has not treated me kindly. Worsening abdominal pain led me to the emergency room. I was diagnosed with acute pancreatitis with a lipase of nearly 800 and an elevated white blood cell count that is higher than when I was septic! By all means I should be admitted, but with the flu running rampant, I was discharged under the condition that I triple my home IV hydration, hold feeds, and get labs rechecked this morning. If there are no improvements, I will be admitted to discuss alternative nutrition options like TPN.

Still, nothing very good and nothing very bad lasts for very long. Whether a blissful moment, a steady mundane existence, or a precarious health crisis, I must learn to appreciate all seasons of life.