Thursday, June 22, 2017

You Know You Have Mast Cell Disease When...

Do you have Mast Cell Disease? Yes? Welcome to the club. I wish I could say it is a cool club to belong to because it most definitely is not. However, joining it does make you privy to a new way of life. That is, the life of the allergic with reactions to foods, chemical scents, and who knows what.


Life with Mast Cell Disease is challenging. It is not entirely bad though. Antihistamines, epinephrine, and a daily dose of humor inevitably saves the day. So, you know you have Mast Cell Disease when: 

Nurses stare in disbelief as you recite a list of your allergies. 

And they can never all fit on just one red allergy wristband.

You keep the local drug stores in business.  

The dose directions on the back of antihistamine boxes do not apply to you. 

Everything stinks. 

Other people would not smell sh*t if it was shoved up their nose. 

You never have to buy blush again!! Your cheeks are forever flushed a shade of pink. 

Stop, drop, and roll does not work when it is your insides that are on fire.

You give a science lesson when asked to explain your disease. 

Histamine, prostaglandins, Diphenhydramine, and Loratadine are common terms in your vocabulary. 

You are the poster child for the EpiPen ads.

Bath and Body Works is your worst nightmare. 

And you run past Yankee Candle and Hollister while shopping at the mall. 

Well. That's swell-y. 

You second as a detective, searching for what triggers have changed. 

Breakfast, lunch, and dinner consist of the same two foods. 

You can chug dye free liquid Benadryl from the bottle and not be fazed. 

Friends are jealous of your tolerance. 

Hypoallergenic is another word for sorcery. 

A successful grocery store trip is when you do not get stuck in the checkout line behind the old woman that is wearing too much perfume.

You never leave your house without a VogMask. 

Bone pain is brutal. 

Treatments work...until they don't. 

Medical professionals think you are neurotic when you request preservative and filler free medications. 

"Is it really possible to react to that?" YES!!!

You know more than your doctors, yet you do not get paid for it. 

Cro-mo-lyn, for the win. 

You can relate to babies on a new level because you drink their formula. Yum, Elecare and Neocate Jr. 

Impending doom. 

ER runs and 125 mg of Solu-Medrol. Hello, moon face. 

During hospital admissions, you bring your own "safe" supplies. 

Bedtime prayer: "Now I lay me down to sleep / I pray the Lord my next co-pay's cheap / If I should die before I wake / I'll know that high histamine treat was a big mistake" 

You learn not to take the being alive for granted.

The good moments are cherished, faith strengthened.

Struggles reveal the friends and family that truly care.

You surround yourself with a loving support system.

You learn that life with Mast Cell Disease is hard, but not impossible!

Monday, June 19, 2017

Why Writing Is Good For The Chronically Ill

My hands pound at the keyboard. My fingers clutch the pen, furiously scribbling on the lined notebook paper. The cursor foreshadows the pain to come, a post of my hardships on display for the world to see. The ink represents a script of my life, a less than glamorous rehearsal easily accessible to any curious hands. This act is characteristic of my life with chronic illness.

Writing can be troublesome for a person who is generally private. Who would want to read the public venting of my endless ailments?  Wouldn't it be humiliating if another person were to uncover the journal containing the musings of my deepest thoughts?


Before, during, and after the diagnosis, I was encouraged by many to begin a journal. I was hesitate to begin a journal in fear the embarrassment if somebody I knew found it. Steeped in my 14-year-old logic, I decided to begin a tumblr blog...because posting my thoughts on the world wide web for all to read was obviously the best recourse. As silly as it all seems now, I am glad I did. Writing is essential in managing chronic illness.

  • Writing provides an outlet to overcome emotional hardships. 
Chronic illness impacts every facet of life. It interferes with completing regular, daily tasks. It is a source of conflict in relationships. It hinders completing education. It effects self image. Controlling the mental consequences of a chronic illness is just as troublesome as living with the physical symptoms. That is not to say that those with chronic illness cannot have a fulfilled, successful life. However, chronic illness introduces a unique set of struggles in addition to the normal experiences. Conveying them aloud is not always desirable. Starting a journal, either online or handwritten, is a route to unburden thoughts and feelings. It is up to personal discretion to share when/if ready.

  • Writing is useful for symptom control.
Logs can be used to track various aspects of illness. It does not only pertain to symptoms and triggers.  The documentation can occur in a variety of ways:

  1. Symptoms / Daily Life

    Jot down symptoms as they present throughout the day. Ensure that you copy the times, along with whatever events are taking place at the onset of symptoms. When did symptoms come on - After breakfast? In the shower? Exercise? At work? Brushing teeth before bed?

    There does not necessarily have to be order to the lists - simply write everything down!
  2. Triggers

    Especially when first developing illness, one is not yet accustom to limitations. Documenting known triggers will make avoiding them second nature.

    Triggers are not easily discernible depending on the illness. The inconsistency is frustrating. Tracking symptoms as they happen, like described, is effective in establishing connections between the factors that cause and worsen symptoms.
  3. Food diary

    Food is complicated. It is necessary for survival, but tends to render an unfavorable response to chronically ill bodies. Whether GI distress or allergic reactions, food can trigger a plethora of symptoms. Log each meal, the times eaten, and how you feel before, during, and afterwards to pinpoint correlations.

    When eating is an issue, receiving the proper amount of nutrients is impossible. A food diary lends a clear picture of nutritional status - What vitamins are lacking? Is the protein, fat, carbohydrate ratio appropriate? Will current caloric intake be sufficient to maintain weight?
  4. Medication regimen

    Most are probably not as scatterbrained as myself. Regardless, writing is beneficial to track medication. Write a calendar of the medications taken daily. Include the doses and the times to be given. Keep a second record of PRN (as needed) medications. This aids in keeping the medications on a strict schedule. The log answers the frequently asked questions - Was this medication even taken? What dose? When is the next dose? How much of this medication is taken daily?

    To be thorough, add record of symptoms. Medication logging is beneficial for both doctor and patient in determining if a medication is proving helpful, so medications not improving symptoms can be discontinued.
  5. Appointments / Specialists

    Checking in at an appointment, the receptions offers a confused stare upon typing on her computer. The appointment was really an hour ago. I've been there....I am sure you have to. Arriving to appointments at the incorrect time is quite inconvenient. To prevent this common incident, try writing a list of future appointments and the times scheduled.

    A list of each doctor and their speciality is helpful to have on hand to mention in other appointments. It is also a reference for friends and family involved in care.
In my experience, this form of writing is ideal for comparing the progression and changes in symptoms. It is resourceful to look back on symptoms that, although they may have seemed minuscule in the moment, have current significance.

  • Writing is a basis of connecting with others in similar situations. 
"Misery loves company," is the supposed saying. People relate through through the sharing of stories. Writing is one means to bring people together. Despite the initial hesitation, publicly writing about the highs and lows of chronic illness conjures the realization that nobody is alone. I met my best friend on tumblr at the beginning of my blogging journey through a mutual diagnosis. Support from those in similar situations is paramount in navigating life with chronic illness. 

While I am no longer the young, confused teenager distraught over unknown symptoms plaguing her body, remnants of her still exist in past wordy reflections. The impacts of chronic illness inevitably change throughout various stages in life. One aspect does not though, and that is the need to express those struggles. 
  






"It seemed fitting for me as well, having investigated everything carefully from the beginning, to write it out for you in consecutive order, most excellent Theophilus, so that you may know the certainty of the things you have been taught." - Luke 1:3-4

Sunday, June 11, 2017

God's Chronic Illness Index

Surgeries, medications, doctors...it is discouraging when modern medicine proves to fail. God can undoubtedly heal through scientific advances. But for reasons out of human stop of understanding, He sometimes chooses not to in this life on earth.
"He sent out his word and healed them, and delivered them from their destruction." - Psalm 107:20, ESV
Luckily for us, He has provided the best medicine. Both literally and figuratively, the Bible mentions various bodily ailments. His word is the ultimate remedy. Especially with rare diagnoses such as mine, what hospitals offer is only a poor substitute of symptom management.


The following is an scriptural index for the many symptoms and effects of chronic illness. While these verses may not actually cure physical pain, they lend encouragement amidst the discouraging trials of chronic illness. The benefits of God given scripture never fail, even when every body system is plagued with illness.


For aching bones:
"Make me to hear joy and gladness, Let the bones which You have broken rejoice." - Psalm 51:8, ESV

For chronic fatigue:
"Come to me, all who labor and are heavy laden, and I will give you rest. Take my yoke upon you, and learn from me, for I am gentle and lowly in heart, and you will find rest for your souls. For my yoke is easy, and my burden is light." - Matthew 11:28-30, ESV

For a failing heart:
"And I will give you a new heart, and a new spirit I will put within you. And I will remove the heart of stone from your flesh and give you a heart of flesh." - Ezekiel 36:26, ESV 

For muscle weakness:
"But he said to me, 'My grace is sufficient for you, for my power is made perfect in weakness.' Therefore I will boast all the more gladly of my weaknesses, so that the power of Christ may rest upon me." - 2 Corinthians 12:9, ESV

For  poor wound healing:
"He heals the brokenhearted and binds up their wounds." - Psalm 147:3, ESV 

For seizures and neurological:
“When they came to the crowd, a man came up to him and, kneeling before him, said, ‘Lord, have mercy on my son, for he is an epileptic and he suffers terribly. For often he falls into the fire, and often into the water. And I brought him to your disciples, and they could not heal him.’ And Jesus answered, ‘O faithless and twisted generation, how long am I to be with you? How long am I to bear with you? Bring him here to me.’ And Jesus rebuked the demon, and it came out of him, and the boy was healed instantly” - Matthew 17:14–18, ESV 

For mental health:
"Do not be anxious about anything, but in everything by prayer and supplication with thanksgiving let your requests be made known to God.  And the peace of God, which surpasses all understanding, will guard your hearts and your minds in Christ Jesus." - Philippians 4:6-7, ESV 

For gastrointestinal distress:
"Food is for the stomach and the stomach is for food, but God will do away with both of them." - 1 Corinthians 6:13, ESV

For shortness of breath:
"The Spirit of God has made me, and the breath of the Almighty gives me life." - Job 33:4, ESV 

For general chronic illness:
"The LORD will sustain him upon his sickbed; In his illness, You restore him to health." - Psalm 41:3, ESV

For the hope to come:
"For I consider that the sufferings of this present time are not worth comparing with the glory that is to be revealed to us." - Romans 8:18, ESV   

What are your favorite verses?


Monday, June 5, 2017

I Want To Be An Outsider

While I cannot eat, my family still has to. So, grocery shopping is inevitable.  Once a week my Mom makes a trip to the store. I always make it a point to go with her. I do not get out though. As she shops, I remain in the car.  

Before my Mast Cell Disease became severe, I was able to go inside of a store. But now that I am allergic to life, a grocery store has too many dangers that trigger life threatening reactions - airborne traces of food, chemicals down the aisles, another shopper's perfume, the list goes on. (Learning this was rather unfortunate. Thank you, Walmart, for keeping rotisserie chicken near the checkout).


In the leisure time waiting, I have the opportunity to reflect on the banalities of this isolated life. My eyes observingly divert to the car window. I see families with multiple children in baseball uniforms piling into a mini van. Guffaws of laughter are evident from their facial expressions. There is also a woman chattering away on her cell phone, nonchalantly pushing a buggy into the store. I notice a group of friends posing for a selfie after finishing a meal at a restaurant in the same shopping complex as the grocery store.  

It is easy to hate random strangers for living their lives and taking simple tasks, like shopping, for granted. They are not dictated by their body's jurisdiction to surrender to its every whim.  However, I try to remember that we all have their burdens to bear. They can just more easily hide their struggles behind daily life.  

These bitter feelings are not very becoming, but the emotions are real and relatively new because I have not always had to experience this degree of isolation. As previously mentioned, exposures to the world used to not provoke such intense, dangerous symptoms. When I did go through flares, maybe I could not eat, but I could at least be in the vicinity of food cooking. And providing there were no chemicals sprayed directly in my face, I could tolerate shopping malls, family get togethers, and grocery stores.  

There is an entirely different world out there that I cannot truly live in due to illness. Trapped in the confines of my house and car,  I am from a different world, one that revolves around symptoms, medications, and preventing reactions. It is the opposite of the saying, "on the outside looking in." I am on the inside looking out when all I wish is to be an outsider, like the healthy strangers parading the parking lot, and to not know the conditions of the lesser world I am currently in. Recognition only makes it worse, as I am aware of the life I am missing.  

Thankfully, I do have friends in similar situations  that can relate. We can pretend to forget the former world together.


Monday, May 29, 2017

All About The Continuous IV Diphenhydramine (Benadryl) Infusion - VIDEO

Intravenous Diphenhydramine (Benadryl) is rather common, right? IV Benadryl administered continuously, literally 24/7 is not. The idea prompts looks of confusion from those unfamiliar with Mast Cell Disease. The Continuous Diphenhydramine Infusion is used in the treatment of severe Mast Cell Diseases to keep patients out of a constant anaphylactoid state. 

I do write responses to questions, like what the Continuous Diphenhydramine Infusion is, its role in Mast Cell Disease, and how my body responds to it. However, if reading  information is overwhelming, this one is for you. The video discusses all of that and more. I go in depth about my Mast Cell Disease reactions on versus off of the continuous pump. I also demonstrate connecting to the infusion nightly. 


Any information not mentioned in the video is likely in one of my previous blog posts, Diphenhydra-what? - The Continuous Benadryl Infusion or 150,000+ mg of Benadryl: Continuous Diphenhydramine Infusion Update

I hope this is helpful! For academic sources, as well as patient resources, check out the IV Therapies For Mast Cell Disease + Related Conditions Resource List


Saturday, May 27, 2017

Fibromyalgia, Irritable Bowel Syndrome, and Chronic Fatigue Are Not Real?

Do you have a chronic illness? 

Did it take years to receive a diagnosis?

Did it take even longer to receive the correct diagnosis? 

If you answered "yes" to the first question, surely the same response applies to the questions proceeding it. 
  

Again, if you said "yes" to any of the above, it is likely you saw a medical professional. You probably underwent various inconclusive tests, results tossed aside in an apathetic manner. If you are lucky, the doctor may care enough to nonchalantly pawn symptoms off with a vague term, such as Fibromyalgia (Fibro),  Irritable Bowel Syndrome (IBS), or Chronic Fatigue Syndrome (CFS) without further studies. These terms conceal copious controversy.  

What is Fibromyalgia? 
"Fibromyalgia is a syndrome and common disorder characterized by widespread pain, diffuse tenderness, and a number of other symptoms. Although Fibromyalgia is often considered an arthritis related condition, it is not arthritis because it does not cause inflammation or damage to the joints, muscles, and other tissues. The causes of Fibromyalgia are unknown." [ x ]
In summary, Fibromyalgia causes widespread, chronic pain. The cause is UNKNOWN.

What is Chronic Fatigue Syndrome?
"Chronic Fatigue Syndrome is a condition where you have long term, disabling tiredness (fatigue) and other symptoms not caused by any other known medical condition. The cause is not known." [ x ]
To conclude, Chronic Fatigue Syndrome causes debilitating tiredness (Although, the word "tired" does not do the debilitating symptom justice). The cause is UNKNOWN. 

What is Irritable Bowel Syndrome? 
"Irritable Bowel Syndrome is a common disorder that impacts the large intestine (colon)... [and] causes cramping, pain, gas, diarrhea, and constipation. IBS, unlike ulcerative colitis and Crohn's disease, which are forms of inflammatory bowel disease (IBD) — doesn't cause changes in bowel tissue. It's not known exactly what causes IBS." [ x
Irritable Bowel Syndrome (IBS), not to be confused with Inflammatory Bowel Diseases (IBD), causes significant, chronic gastrointestinal upset. The cause is UNKNOWN.

Do you notice a commonality among these diagnoses?

The similarities lie in the mystery concealed in the terms. They are catch all terms describing symptoms of an UNKNOWN origin. They are also deemed not real by a large portion of the general public. And in a sense - they are just that, not real.

Let me elaborate to avoid offense.

I had a diagnosis of all three.

The symptoms of Fibromyalgia, Irritable Bowel Syndrome, and Chronic Fatigue Syndrome are disabling. They are undoubtedly true, not imaginary. However, it is rare for a body to succumb to severe fatigue, gastrointestinal complaints (life-altering for some), and/or chronic pain just because. The above diagnoses are frequently symptoms of a larger, underlying health condition. It is imperative and potentially life saving to identify the cause(s).

My "Fibromyalgia" symptoms are from my genetic condition, Ehlers Danlos Syndrome.

My severe "Irritable Bowel Syndrome (IBS)" is actually a form of Inflammatory Bowel Disease (IBD) known as Mastocytic Enterocolitis, stemming from Mast Cell Disease and wreaking havoc on my GI organs.

My "Chronic Fatigue Syndrome" transformed into Dysautonomia, the extreme tiredness an effect of my body compensating for my faulty nervous system.

The diagnosis process is arduous, but I encourage all to pursue answers for their UNKNOWN illness(es). Seek doctors that care, crave knowledge, are persistent in their curiosities, and do not flee from the patients who are medical enigmas (like me).


Friday, May 19, 2017

This Is What Ehlers Danlos Syndrome Is Like

May is Ehlers Danlos awareness month. In honor of awareness month, I created a video [shown at end of post] depicting a day in the life of someone with Ehlers Danlos Syndrome and its comorbid conditions. The extent of symptoms varies patient by patient. This is how I used to present prior to my secondary Mast Cell Disease becoming severe. I tried to add a humorous twist to the video. Enjoy!


You awake in the morning, rubbing the sleep out of your eyes. Last night was spent tossing and turning, so you are not well rested. You wonder why you expected to feel otherwise when you have Ehlers Danlos Syndrome.

In attempts to make it out of bed, you roll over, but your actions are interrupted when your hip cries a startling "pop." It is another dislocation. A searing pain courses through your skeleton, while you contort your body to adjust the joint back into position.

Since the joint is back in place, the injury requires bracing. You eye the brace from the corner of the room. You are dreading the arduous journey, as you have not yet managed to make it out of bed. Hobbling, you grab the brace and get dressed for the day. You completely bypass the bathroom. A shower is just not in the cards this morning.
Ehlers-Danlos syndrome is a group of disorders that affect the connective tissues that support the skin, bones, blood vessels, and many other organs and tissues. Defects in connective tissues cause the signs and symptoms of Ehlers-Danlos syndrome, which vary from mildly loose joints to life-threatening complications. [ x ] 
Instead, you trek down the hall. Your heart is pounding like a subwoofer, a bass echoing in your ears, and stars dance across your vision. It is just Dysautonomia flaring. The lax vessels formed from faulty collagen are unable to adequately pump blood to the brain. Mid-backout, you surrender to the floor to avoid completely losing consciousness.

Now you head towards the kitchen. It is time to prepare for AM medications and intravenous infusion set up. You are sort of sick to your stomach. Regardless, you need to eat breakfast. Taking pills on an empty stomach is never a practical idea. You realize you can depend on tube feedings, but you prefer not to rely on the formula if you can help it. You choke down a bland piece of toast.

The day has barely started, but you are already exhausted. You grab your bag to head out the door...and pop!!! You partially dislocate your finger. In the interim, you grow even more queasy, proceeding to up chuck in the grass near the front steps of your house. There goes that piece of toast. You also notice remnants of last night's dinner. Darn, Gastroparesis, a paralyzed stomach.
"Each type of Ehlers-Danlos Syndrome is defined as a distinct problem in making or using one of the types of collagen. Collagen is what the body uses to provide strength and elasticity to tissue; normal collagen is a strong protein that allows tissue to be stretched but not beyond its limit, and then safely returns that tissue to normal." [ x ] 
You have a gut feeling something is not right, and it is not just the rotten food from days prior. It is probably not safe to drive, so you need a ride to run your errands. You do not want to inconvenience the same person you called three different occasions last week. You realize you can be a burden; however, rarely do others admit it.

The professor drones on. You finally made it to class! Concentration is difficult. Brainfog is no joke. Your hands shakily scribble your essay. Maybe it is the hypoglycemia. After all, you absorbed little to none of your meals. You got a C on that paper and wonder if you could have gotten a higher grade if you had not had to sacrifice studying for that doctor's appointment.

There are errands to attend to, but you have no energy. Fatigue is evident, despite the 2 PM cat nap. You totally skip lunch. Why bother, since symptoms indicate you will probably puke that up too. You are aware of each inhalation. It is difficult to breathe. Your lungs crave the air that your body is inadequate at giving them.
"An analogy: If one builds a house with bad materials, with cheap nails or only half the wood required, problems will arise. Some problems are more likely to show up than others, but because the bad or missing materials are everywhere and not necessarily visible, one can be surprised where some problems occur." [ x ]
Pressure builds at the base of your skull, right where it attaches to the neck. The tissues are weak and are unable to support your head. An intense ache accompanies the pressure and the room is spinning with nauseating vertigo. You joke that you are bionic woman with your silly neck brace, but are thankful for the reprieve.

That evening, you meet with your family. You enter into your grandma's house when a sense of doom hits you. It is a mast cell reaction to your aunt's perfume. Your skin turns flushed, red, and blotchy. Your limbs tremor and jerk. Parts of your face begin to swell, with your throat quickly following suit. A cousin stabs you with an EpiPen. Mom administers emergency Benadryl before you have the chance to drop into a seizing fit.

A lot has gone on. You give yourself a few hours to recover. Not showering again is not an option. The heat from the shower dilates your blood vessels, exacerbating your Dysautonomia again. You hope you do not faint again. After reaching for the soap, you wash your face that is covered in itchy hives. Oh no, please no rebound reaction.
"It is much the same thing with Ehlers-Danlos Syndrome and collagen. The collagen with which a person with Ehlers-Danlos Syndrome is built is not structured the way it should be, or only part of it is produced. With badly built collagen, the tissues that rely on it can be pulled beyond normal limits and thus be damaged." [ x ]
Once evening medications are over, you connect to your feeding tube in a Benadryl stupor because eating was obviously not a success. Your prayers are pleas for a restful repose. Unfortunately, there will be no soothing lullabies, only formidable tales where your symptoms are incorporated into nightmares. Even in the wildest dreams your brain can conjure, you are never healthy. You close your eyes, knowing tomorrow you will do it all over. Because this is what having Ehlers Danlos Syndrome is like.


Having Ehlers Danlos Syndrome is not all doom and gloom, like this post may suggest. Happiness still exists in a life with chronic illness. However, especially during awareness month, those with Ehlers Danlos Syndrome and its co-morbid conditions often undermine their struggles. We do not want to be thought of as always depressing or always crying poor pitiful me. Hopefully this accurately depicts a day in the life of someone with Ehlers Danlos Syndrome.


Thursday, May 18, 2017

Little Victories With Chronic Illness

For the first time since June 2015, I wore sneakers. Why, you ask? Because I am finally able to walk up the tiny hill in the backyard!


Last May commenced a huge flare. It is assumed it was provoked by an unfavorable response to a rarer treatment for Mast Cell Disease, the Continuous Famotadine Infusion. I began that treatment earlier; however, my body did not reject it until months later. The cause was not discerned immediately. It was a perplexing situation, as the medication was helping while simultaneously triggering my mast cells.

In the midst of a flare, my pediatric doctors would jokingly remark that I looked like I was drug through the streets. This was like that, but tenfold. I never knew it was possible for my health to get as bad as it did. Undoubtedly, the medication setback resulted in extensive damage. It has taken months and months to return to my baseline. Recovery has been painstakingly slow.   Lately, the days have been more tolerable. They have included a couple of good hours. I have definitely been taking advantage of them with my recent goals.

  • Sunlight  
The first of my goals is to receive sun exposure. The amount is limited, as I only stay outside for 10 minutes. Heat exacerbates POTS, causing the blood to pool to my lower extremities before I faint. Temperature, combined with UV rays, can also cause mast cell degranulation. To avoid reactions, I keep outside adventures very brief. Regardless, seeing the natural light of day is healing. The extra Vitamin D is a plus too!

  • Movement  
Next, my goal is to ‘exercise’ to gain muscle back. I got down to 81 pounds. Fat was not in excess, so I know my muscles were wasting.   Overexertion is futile, especially with my cardiovascular symptoms. The 48-hour holter monitor from last week caught episodes of Ventricular Tachycardia. An old stress test where my mast cells were triggered from exercise revealed a partial blockage of the artery, which can lead to a heart attack if I react bad enough. I should be careful. Remaining sedentary is not ideal either though!

So, walking up the hill in the backyard is one way to work on these goals. I feel as if I am finally able to make some progress. And for whatever crazy reason, after not being able to walk for most of last year, the triumph over this simple task is greater than the sense of accomplishment from an intense gym workout during my (much) healthier spells. Holding on to moments of success makes any setbacks with chronic illness easier to handle!


Tuesday, May 16, 2017

Intravenous Hydration For POTS/Dysautonomia

Dysautonomia is a term defining conditions impacting the autonomic nervous system. The autonomic nervous system controls every bodily function occurring "automatically." A subset of Dysautonomia is Postural Orthostatic Tachycardia Syndrome (POTS), effecting a body's ability to regulate upon postural positional changes. Because of POTS, blood is unable to reach the brain when transitioning from laying to sitting or sitting to standing.


Read STOP POTS? to learn about Postural Orthostatic Tachycardia Syndrome.

POTS primarily manifests with a multitude of cardiac symptoms:

  • Tachycardia (high heart rate)
  • Bradycardia (low heart rate)
  • High blood pressure
  • Low blood pressure
  • Blackouts (pre syncope)
  • Fainting (syncope)
  • Blood pooling
  • Fatigue
  • Dizziness
  • Lightheadness
  • Excessive sweating
  • Shortness of breath
  • Headaches

The majority of POTS patients suffer from a high heart rate and low blood pressure. Since it is a dysfunction of the nervous system, symptoms can range from one extreme to another, similar to constantly flipping a hypothetical on/off switch in the body.

Sodium and fluid regulate the unstable pulse and blood pressure. To combat the debilitating symptoms, it is recommended that a POTS patient consumes 3 to 10 grams of salt and over 2 liters of fluid daily. [ x ] [ x ] That intake is difficult. In severe cases, the amounts needed to control symptoms are impossible without the support of IV saline therapy through a central line. That is why some resort to IV saline therapy! 


"How much saline do you run?"

For the treatment of POTS/Dysautonomia alone, 1 to 2 liters is typically infused at a time.

"How frequently do you infuse the fluids?"

The frequency of administering saline is dependent on the medical situation. If the patient is physically able to obtain adequate hydration normally, liters of saline do not necessarily have to be infused daily. The supplemental hydration for POTS/Dysautonomia treatment can be spread out to 3 to 4 times a week.

"What symptoms does it improve?"

Saline infusions help fight the miscellaneous symptoms stemming from POTS cardiac involvement, low blood volume, and dehydration. It helps with the following the most:

  • Dizziness
  • Lightheadedness
  • Presyncope/Syncope (fainting)
  • Tachycardia
  • Fatigue

"How is this different than drinking fluids orally/via feeding tube?"

Receiving intravenous saline is superior to simply drinking fluids in improving symptoms. 

The following can influence the success of IV saline.

  • Sodium Content
A high sodium content is necessary in combination with fluids to benefit symptoms, such as increasing blood pressure and decreasing the heart rate. Since regular water lacks the sodium saline has, it is less effective. 

"Why not drink Gatorade and/or electrolyte drinks?"

Gatorade, Powerade, Pedialyte, and other electrolyte drinks can be helpful. However, they contain large amounts of sugars, dyes, and additional ingredients. The additives are not ideal in excess, like a POTS patient requires. The healthier homemade options still contain sugars to taste, which are still less palatable in large quantities.

  • Gastrointestinal Distress
While POTS encompasses the cardiac symptoms of Dysautonomia, the majority of patients have symptoms in other bodily systems. Whether caused directly from the Dysauotnomia or from another primary or secondary health condition, the gastrointestinal tract is often effected. Nausea, vomiting, acid reflux, and diarrhea are common symptoms. Stressing the body by ingesting a high volume of water exacerbates the GI complaints.


A Mast Cell Disease Perspective 

Some patients with POTS/Dysautonomia have a Mast Cell Disease. The unstable mast cells in patients with Mast Cell Disease are known to result in secondary Dysautonomia. So, the involvement in the autonomic nervous system is actually a symptom of the Mast Cell Disease! The above information regarding saline therapy for POTS still applies.

It is important not to confuse the symptoms of POTS with other cardiac symptoms of Mast Cell Disease. If experiencing such symptoms laying down, it is likely not POTS in that moment because POTS is triggered from POSTURAL changes.

However, Mast Cell Disease presents unique circumstance that may change the response to IV hydration therapies.

"Can IV hydration help MCAD symptoms?"

Aside from POTS, intravenous hydration is helpful in other ways. The extra fluid is beneficial during reactions and daily baseline symptoms.

  • Third Spacing
Third spacing can be a symptom of Mast Cell Disease. It is characterized as the improper shift of fluid in the body, contributing to dehydration and angiodema. The body is unable to carry out normal function when oral fluids are displaced. IV hydration is proven to counteract third spacing.

  •   Low Blood Pressure
POTS is not the only condition that causes low blood pressure. Mast Cell Disease patients also have low blood pressure unrelated to postural changes. The low pressures are related to allergic reactions. Saline infusions temporarily increase the pressure, even if the fluid treatment is reserved for acute situations.

  • Dehydration
Mast Cell Disease patients tend to vomit and have diarrhea frequently. This inevitably leads to dehydration. Saline infusions replace what is lost. Electrolytes can be added to the fluids, which resolves imbalances.

"How is this different than drinking fluids orally/via feeding tube with Mast Cell Disease?"

  • Anaphylaxis / Reactions 
Many patients with POTS/Dysautonomia have a Mast Cell Disease. Mast Cell Diseases cause easily triggered, severe allergic reactions that can potentially lead to anaphylaxis. Triggers vary. In extreme cases, water is a trigger. That is obviously not ideal if needing to drink extra water to treat POTS/Dysautonomia. 

"How is water a trigger?"

The actual water is rarely the trigger of reactions, but secondary factors instead. 

First is temperature. Both cold and heat causes mast cells to degranulate. Ingesting water straight from the fridge is cold. In extremely sensitive individuals, even drinking water at room temperature is enough to induce symptoms. Warming the water to body temperature reduces the possibility of reactions to the water temperature. 

Next, excipients in the water is the most frequent cause of reactions. The vessels holding the water is a reaction risk. The plastics forming the bottles leech into the water, thus contaminating it with chemicals. In the normal, healthy population this is not a huge deal. The companies simply avoid the use of BPA and most feel comfortable purchasing the product, but there are plastics other than BPA that are harmful to people with chemicals sensitivities. Drinking water that was stored in glass rather than plastic offers less chance of contamination. 

However, purchasing the brands of water that come in glass, like VOSS, present another issue. The fancier waters have a variety of minerals added that are not confuse to those with a Mast Cell Disease. Such minerals and traces of chlorine and chemicals are found in some store bought brands and tap water. Distilling water through a filter removes the contaminants, decreasing reactions. 


Mast Cell Disease Reactions To IV Hydration Therapy

Replacing PO hydration with intravenous hydration does not remove the prospect of reactions. While it is supposed to help, sometimes receiving intravenous saline actually causes reactions. This is due to a number of reasons.

How can saline cause a reaction?

  • Rate
When mast cells are already triggered, shocking the body with bolus fluids can causes further degranulation. Reducing the rate that the saline is normally ran at ensures that an already compromised body is not overloaded.

The rate I usually infused my hydration is 200 mL/hr. In the case of a reactive day, I choose to run the saline on my backup pump set at 80 mL/hr.

  • Temperature
Just as water temperature can be a trigger when drinking it orally or administered via a feeding tube, it is important to keep intravenous saline at body temperature too. Prior to infusing, I use body heat to warm my fluid back if it is cold. I wrap it in a towel and "hug" the bag until desired temperature.

  • Excipients in the IV bag
The materials that make up the IV bags can leech into the intravenous fluids. Be cautious of the types of plastics in the supplies. Try to find bags without latex and DEHP.

  • Preservatives 
Not all intravenous saline is pure. Preservatives are added to prolong stability. A reactions to the "saline" might only be a sensitivity to inactive ingredients. 


Read Excipient Recipient: Mast Cell Reactions To Excipients to learn more about the added ingredients in medical supplies and medications that can provoke reactions.

The idea of beginning IV treatments is conceivably scary, but the positives are worth it. The therapy definitely makes life with POTS/Dysautonomia and Mast Cell Disease easier.

Don't forget to check out the IV Therapies For mast Cell Disease + Related Conditions Resource List!





Are you interested in IV therapy? Do you have a Mast Cell Disease? Join the Facebook group IV Therapies For Mast Cell Disease + Related Conditions

Monday, May 8, 2017

Empty Sky

The weather is beautiful. Gazing upwards, there is not a cloud in the sky. It still seems so empty though, aside from the stray birds soaring in an arrow formation. A melancholy mood rolls in with the breeze. Despite the surrounding beauty, the sun’s rays cast light on everything that is wrong. It illuminates life's questions, like why and how come. Unlike the creatures of the sky, I cannot take flight from the misfortunes I have no desire to face.


In the recent weeks, there have been multiple losses in the chronic illness community. The first was really close to home. The next was more personally devastating, as it was a friend with a common diagnosis. Both have been an eye opener for everybody involved. The situations could easily be any of us.

Life is short. These events not only denote its fragility, but they also cause questions to surface. Why did my friends die? Why were they sick? Why am I sick? Why do people have half of the problems that they do? How come life is not fair? What have we done to deserve this?

I then came across John chapter 9. Jesus eventually healed the eyesight of a blind man. New believers were definitely roused from the trials and tribulations. Regarding the man, the disciples question Jesus:
As he went along, he saw a man blind from birth. 2 His disciples asked him, “Rabbi, who sinned, this man or his parents, that he was born blind?” John 9:1-2
Their inquiries are tremendously similar to my own. The answer is one frequently ignored.
“Neither this man nor his parents sinned,” said Jesus, “but this happened so that the works of God might be displayed in him. As long as it is day, we must do the works of him who sent me. Night is coming, when no one can work. While I am in the world, I am the light of the world.” John 9:3-5
And I realized something. This is not about me. It is not entirely about the precious lives lost either. It is about Him. Life always works out the way it should, whether understood now or later. The highs and the lows of life inevitably serve a greater purpose. The sky is no longer empty.

Rest in peace to my fellow warriors. xx


Friday, May 5, 2017

Excipient Recipient: Mast Cell Reactions To Excipients

Patients with Mast Cell Disease and its related conditions are obviously on the severe end of the spectrum if considering the addition of intravenous infusions to their treatment regimen. It becomes disheartening when the last resort options prove to be a failure. Treatments for the disease are not always effective. They are obscure and the risks are significant. What gives? Shouldn't the benefits be just as immense?

Severe forms of the disease present the perplexing phenomena of reacting to excipients in the medications and the ingredients that compose the infusion supplies. Excipients are "usually inert substances." They are added to medications, saline, or supplies to provide stability and substance.


Frequently, the therapies are not a total fail. There is great potential in receiving treatments, but reactions to the excipients prevent desired improvements.

Excipients are a topic of discussion in the Facebook group that I moderate, IV Therapies For Mast Cell Disease + Related Conditions. This post is meant to serve as clarification.

For medical references outside of patient experience, Pharmacological treatment options for mast cell activation disease has valuable information regarding excipients.

Medications

Oral / PO Form

Before resorting to, or in combination with IV therapies, miscellaneous medications by mouth or through feeding tube are attempted.

Histamine (H1 and H2) blockers are a must in the treatment of Mast Cell Disease. The non-drowsy, second generation antihistamines are preferred. These medications are commonly purchased over the counter at a local drug store because they are intended to use in the treatment of allergies, but many are prescription too.

Along with the active drug ingredient, medications in PO form contain a plethora of inactive excipient ingredients. They include dyes, fillers, and preservatives.

Reasons for excipients/inactive ingredients: 

  • Aesthetic 
Whether a bright red liquid suspicion or a florescent pink pill, medication manufactures like to believe that dyes make their products look visibly appealing. Excipients are added to change the appearance of the medication.

  • Preservation
Preservative ingredients increase the shelf life of medications. It is inconvenient to replace a medication every few days. Preservatives allow a medications to last months to over a year!

  • Substance 
Medications under a certain dose require filler ingredients that provide enough powder to physically scoop the medication into a capsule or to form it into a pill.

Medications frequently do not work like they should in sensitive patients because of varying reactions to excipients. Just as someone with a Mast Cell Disease would not ingest a reaction inducing packaged cookie that is laden with preservatives or consume a hot dog soaked in nitrates, one would want to avoid the "bad stuff" in medications also.

Cetirizine (Zyrtec) is an OTC (over the counter) example.
10 mg of pure Cetirizine (Zyrtec) is too minuscule to be a stand alone ingredient. Various forms of cellulose gives it bulk to form a pill. Yellow iron oxide colors the pill. Silicon dioxide prevents clumping. 
The above ingredients is approved safe for consumption in the normal population, but it is not conducive to chemically sensitive patients with Mast Cell Disease.  
There is a recourse to the toxic medications at the pharmacy or purchased in a store. Special compounding pharmacies have the ability to compound medications, omitting the unnecessary ingredient in medications and substituting them with safer options.

Compounding Ideas

Compounding medications is still trial and error, but it is superior to the alternative.

  • Sterile water
Compounding with sterile water in a liquid solution is preferable if the drug is available in a liquid form. Medications compounded in sterile water typically require refrigeration. Depending on the active ingredient, the solution does not remain stable longer than a couple of weeks.

  • Pure powder 
There is no need for additional ingredients, aside from the pure powder, if the medication is equal to or exceeding 50 milligrams. The pure powder must be placed in a vessel of some sort.

Capsule: The pure drug can be put into a safe, vegetable capsule. If the tolerance of a capsule is unknown, try dumping the powder out of the capsule and into a glass of water to drink.

Parchment: An unsafe capsule can contaminate the pure drug powder in extremely sensitive individuals. To avoid reactions, the pure drug powder can be put into parchment paper until the patient is ready to mix the powder into a glass of water to administer.

  • Sugars or Flours
For smaller milligram doses, the pharmacist can substitute excipients with safe food ingredients. Sugars or flour provides a powder substance to combine with the medication, ensuring there is enough substance to make a pill or to put into a capsule.

Examples of sugar/flour fillers are: cane sugar, palm sugar, all purpose flour, rice flour, oat flour, chickpea flour, spelt flour, buckwheat flour, coconut flour, almond flour, sorghum flour.

  • Avicel
The sole ingredient in Avicel is microcrystalline cellulose. For some patients, it is a safe compounding option.

  • Elemental Formula
Elemental formula is a nutritional, powdered formula designed for patients with severe food allergies. Recently, I was made aware that Neocate and/or Elecare can be used in compounding. 

The options are endless with a willing, understanding compounding pharmacist. Tolerating a medication may be the difference of altering one ingredient. For example:
"patients who react to wood-based microcrystalline cellulose might tolerate cotton-based microcrystalline cellulose without any difficulty at all, or vice versa." [ x ]
Intravenous / IV 

Intravenous medications do not necessarily contain the same fillers as their PO counterparts. They do have excipients deriving from preservatives and other contaminates though.

As previously expressed, very rarely is the patient reacting to the active ingredient in their medications. The reactions are likely from excipients. Do not rule a medication as ineffective until it is obtained in a completely preservative free form.

Benadryl (Diphenhydramine)

Mast Cell Disease patients frequently experience this predicament when seeking intravenous Benadryl (Diphenhydramine). The severely afflicted might notice that the Benadryl (Diphenhydramine) is not relieving symptoms. In all actuality, they become significantly worse after administering the medication until the active ingredient partially quells the reaction.

The described situation is distressing. Trialling various brands of intravenous Benadryl (Diphenhydramine) can help determine the cause of reactions. Not every IV Benadryl (Diphenhydramine) is the same, preservative free or not,  as they each undergo various processes in manufacturing. [ x ]

  • Westward
Benadryl (Diphenhydramine) produced by Westward is not preservative free. 

From personal experience, Westward Benadryl causes adverse reactions. My neurological symptoms increase with minuscule amounts of the medication, with strange head sensations accompanied by flashing lights in vision, tachycardia, and flushing. 

  • APP / Fresinus 
APP, which has now been bought out by Fresinus, manufactures preservative free intravenous Benadryl (Diphenhydramine). This is the brand I do best with.  


Intravenous medications undergo a rigorous analysis to ensure the product meets specifications for impurities.
Analysis for APP/Fresenius Diphenhydramine (Benadryl)
  • Hospira
Hospira is the "cleanest" brand of Benadryl (Diphenhydramine) in terms of excipients. 

The majority of patients do use this brand. However, there are always exceptions to the rule. I am one of them. Upon administering Hospira, I immediately began flushing, tachycardia, "locked-in syndrome" where I lost the ability to speak, and respiratory symptoms. A dose of APP Benadryl (Diphenhydramine) eventually settled the reaction. 

Reactions to excipients do not have to entail a life threatening reaction. They can consist of minor symptoms. Take the Continuous diphenhydramine infusion and imatinib for KIT-D816V-negative mast cell activation syndrome: a case report for example:
"She also had nausea and vomiting, probably due to the excipients (preservatives) in the CDI preparation. The diphenhydramine most likely helped control the dysfunctional mast cells, whereas some excipient simultaneously aggravated the dysfunctional mast cells." 
While impurities are minimal and deemed safe for humans, these subtle differences between the brands of intravenous Diphenhydramine (Benadryl) can cause reactions in a patient with a Mast Cell Disease. Just like no two brands of Diphenhydramine (Benadryl) are the same, no two patients are the same either.

Supplies

Various medical supplies are used in care: IV bags, syringes, IV tubings, oxygen tubings, enteral feeding bags, and more. As if excipients in medications are not difficult enough to manage, reactions are also caused by specific infusion and enteral (tube feeding) supplies because of how they are made,  thus contaminating the infusion.

Plastics

Pinpointing the exact trigger in the medical supplies is an arduous task. It could be ANYTHING. However, in my case the culprit is usually a plastic that leeches into the infusion.

  • DEHP
DEHP is a type of plastic. It is not supposed to be found in products in amounts harmful to humans, but mast cells are finicky. They do not care about that. Eliminating DEHP was a huge game changer. I no longer felt like I was reacting to my IV therapies, like the continuous Benadryl infusion, once I switched to products that did not contained DEHP.

Read Scientific Facts on Diethylhexyl phthalate by GreenFacts.

B. Braun makes products free of DEHP. They have (2) IV bags that are DEHP and latex free: L8000 and E8000. The latter is what I use. The post, Contaminates Be Gone, Thank You B.Braun discusses their products.

  • Latex 
Latex is a common allergen, so it is not as prevalent in medical supplies as it once was. There is the rare occasion it is in intravenous bags, syringes, and tubings. It is advisable to avoid if possible, as it does have allergenic properties. 

Lubricants

Another probable excipient is the lubrication in syringes. The black, rubber stoppers in syringes are lubricated for easier gliding when drawing up water or medications.

The lubrication is especially a problem in enteral supplies, like feeding tubes. Coviden syringes are better tolerated than when I flush my feeding tube with BD syringes. Thoroughly rinsing with hot water helps lessen the amount of lubricant on the syringe.

Mast Cell Disease is tricky. There is no concise answer as to what treatment is optimal. Eliminating triggering excipients is a good starting point though.