Wednesday, March 15, 2017

Traveling with IV Meds, Tube Feeds, and a Service Dog...OH MY!!!

I have obviously never traveled with multiple toddlers so I can't say whether or not this is more complicated, but let me just say that it feels overwhelming to begin packing for a week away from home with all the miscellaneous medical supplies I will need along with any as needed supplies, just in case those as needed moments arise...

Despite the blizzard that has been dumping snow on the midwest and east coast, I am in the beginning stages of packing to spend just over a week in VA with my parents. On Thursday my mom is driving out to pick me up and bring me back to VA. While I am still able to drive, I've had such an increase in symptoms lately, I often don't feel safe driving due to dizziness or fatigue. Plus, it's not often I get 6 hours in a row without any for now, we've decided that it is probably not safe for me to be driving myself that far.

NEXT weekend we are celebrating one of my favorite holidays, Passover!!! My family has hosted at least one Seder (sometimes 2!) a year since I was about 4 years old. I remember falling asleep in the corner when I was little, hunting for the afikomen with our friends, practicing the Hebrew questions for weeks leading up to Seder, passing off the Hebrew to Devon as he got older, and now, helping out more in the actual planning and production of our Seders. The past few years, even Nick has been studying hard as one day he'll be stepping up to lead our own Seders! He is an honorary Jew now, after all!

Anyway, in addition to our Seder next weekend (SIDE NOTE: I should mention that Passover is not actually until April this year...thankfully we live under grace because this weekend really worked better for us to host our Seder =P), my mom also has a couple of events coming up for her travel business, Trippin' with Jamie. The second event is one of the largest she attends each year. I was there last year to help out, so we thought it would be good if I was there again this year.

So with two Trippin' with Jamie events (one being 2 days long), plus Seder prep, and the actual Seder...this week is already stacking up to be super busy for me considering I am barely leaving the house other than for appointments right now. But we're going to (attempt to) take it easy so hopefully I won't have a major least not until I get back home the following week...

So that's what's up for this week! I had 2 appointments on Monday (including Xolair), but then the rest of this week has been dedicated to resting, allowing my body to hopefully recover from the Xolair injections, and favorite...

Packing has never been one of my favorite activities. I always stress about whether I've brought the right clothes or enough of what I need (even more so after my run-in with an earthquake in Costa Rica in 2009...never heard that story?? You'll have to ask sometime =P). Add in essential medical supplies, and I'm always making lists, editing said lists, packing, repacking, double checking, etc. It's one thing to forget t-shirts (Devon, Eric, I'm looking at you!) because you can go out and get more if absolutely needed. But if I forget my medications or tube pads or needled syringes or other medical equipment, it's not quite as easy to replace. So the pre-packing planning, packing planning, and post-packing rechecking happens every time...usually multiple times =P

Plus, in addition to all my medical stuff (which includes daily meds, emergency meds, IV meds and supplies, infusion supplies, feeding tube supplies, and miscellaneous other medical necessities), I also have to pack for Leni (and Bonk since he's coming this time) AND remember "normal people" clothes =P

Anyway, all that to say, I'm headed out of snowy Ohio at the end of this week for also snowy Virginia. Praying my body cooperates, and I'm able to actually do some stuff instead of just waiting for yet another mast cell storm to pass...if you're in VA and would like to meet up, let me know!

Wednesday, March 8, 2017

When Essential Oils Become Deadly

I like to avoid confrontation. I do my best to avoid debates on facebook about politics or religion or whether that dress was blue and black or white and gold. However, sometimes, there are issues where I just cannot sit by and not speak up. You can read about my last PSA regarding the EpiPen Scandal from August of last year when the prices for this life saving medication skyrocketed with no explanation or reason. This time, as the title of this post suggests, I'm going to address the issue of essential oils.

Let me start off by saying that I am NOT anti-essential oils. My body may be (and I'll get into that shortly) but I actually think there is something to the idea that maybe there are non-pharmaceutical options for treating a wide range of ailments. I have many friends who swear by essential oils, sell them, and use them for all sorts of different things. There is so much information out about all different styles and types and brands of oils. Do they need to be diluted? Is it for diffusing or topical use or ingesting? And the all important question that I'm going to address here, can you be allergic to pure essential oils?

I know, I've just poked the sleeping bear. I'm about to tangle with a vegan over the ethics of eating meat. I'm wearing Michigan blue at Ohio State football game. I just mentioned Virginia as the area outside DC instead of specifying NORTHERN Virginia.

Regardless of which analogy you prefer, I do realize that there is a lot of debate about this issue. Can someone be allergic to pure essential oils? Before I go into WHY the answer to this question (Spoiler Alert!!) is YES, let me set the stage for why I'm writing this post now.

On Tuesday last week I was supposed to have an appointment with my neurologist and do some testing. Please note my usage of past tense...My doctor's office is located on the second floor of a medical office building across the street from the hospital (how convenient...). Right when I walked into the building, there was a sign at the front desk announcing the presence of an essential oils fair being held in the building that day. I was a bit wary because I have reacted to diffused oils in the past, but I figured that as long as I stayed away from the fair, I would be okay. I was wearing my mask (as I always do in medical facilities), had Leni to alert me to any on coming reaction, and had my IV medications with me to keep my symptoms under control.

I got in the elevator and took it to the second floor. The doors opened, and I immediately found myself in the middle of the essential oils was being held in the lobby of the second floor with no way to avoid it. Now, I do not have a good sense of smell. We joking refer to Nick (or my mom) as my SDU, Scent Detection Unit, because I am typically unable to smell anything that could potentially cause a reaction. I just react and am left wondering what set me off. To paint a better picture of the extent of exposure I had to the essential oils, I could smell MULTIPLE different oils diffusing (and could see at least 3 diffusers actively being used). There were also many OPEN jars set out on the various tables that were likely contributing to the smell in the air. To get to my doctor's office, I was forced to walk THROUGH the fair.

Even with my mask on and doing my best to hold my breath, when I got to the front desk in the office, the nurse could immediately tell something was wrong. She got up to come around the desk to help me to an exam room and in that time Leni began alerting. This wasn't one of those reactions where I get 15-20 minutes in between her alert and my symptoms. I went from 0 to 60 in less than 3 minutes. In the time it took to get off the elevator and lie down in the exam room, my lips were swelling, I was blacking out, my face was flushed, I had hives developing on my chest, my oxygen levels had dropped into the 80s, and my throat was swelling. I tried to get my IV meds in me, but the reaction was progressing too quickly. I ended up needing to use an EpiPen which meant an ambulance ride across the street to the hospital for a bunch of IV meds, breathing treatments, and spending a few hours hanging out with a couple nurses who LOVED Leni.

So back to the question I posed earlier...can you be allergic to essential oils? Please see Exhibit A for your answer...But I'm not just going to leave it at that. I want to address a few of the specific issues regarding the "essential oil allergy."

Issue 1) It is impossible to have an allergic reaction to pure oils is because there are no proteins in the oils so there is no chance of an antigen/antibody response in the body. Any potential reaction is to a carrier oil or contamination.

There IS some truth in that argument. They are right that without a specific protein, there is no antigen/antibody response in the body. However, they have mistakenly assumed that this is the ONLY allergy pathway in the body. Even without factoring in crazy mast cell issues (which I will get to in a moment), there ARE allergens that do not cause an antigen/antibody response. Penicillin is a great example. Ironically, poison ivy and that family (which are OIL BASED) also do not have proteins in the oils that cause the reaction. So even without mast cell disease, it is possible to have an allergic reaction to substances without proteins. When you add mast cells into the mix, however, it gets even more complicated. When a mast cell is triggered, it releases dozens of chemical mediators. Each functions along a different pathway to cause all sorts of symptoms ranging from mild to life threatening. This is why treating mast cell diseases is so complicated. Each treatment targets a specific mediator, but leaves all the others free to cause issues all over the body. Maybe I'm not having an antigen/antibody reaction to the oils, but allergies don't always have to function along just that pathway.

Issue 2) There has never been a documented case of an allergic reaction to an essential oil or the antibodies found in a person after exposure indicating the possibility of an allergy.

First, I'm not sure I believe the first part of that sentence. I find it hard to believe that there has never been a case of someone reacting to an oil and going to the doctor for treatment because of that. If that truly was the case, then we can celebrate because I am now Patient Zero. I have nurses, doctors, EMTs, and ER staff that all documented my anaphylaxis to the oils this past week. The second half of the sentence actually makes me nervous because it reflect that people making these arguments don't fully understand the seriousness of this issue. The last thing on anybody's mind when a person is in anaphylaxis is to draw blood to test for the presence of the allergen...they are too focused on keeping the person alive instead of going on a witch hunt. Plus, just because it has never been documented doesn't mean it's not there. The first officially diagnosed case of MCAS was in 2007. That doesn't mean it didn't exist before then, just that no one knew what to look for or what they were looking at. I know people who have been sick for longer than 10 years and were only recently diagnosed. They had the test results for MCAS all along, but there was just no one to know to test for them. Just because there is no documentation of specific antibodies does not mean there has never been a reaction. Plus if you read the previous paragraph, you'll remember that allergies don't always have to have an antigen/antibody response anyway.

Issue 3) Any potential reaction to an essential oil is not an allergy but a detox reaction. To help the body eliminate the toxins, the oil should continue to used to allow the body to rid itself of those toxins and eventually the person will be able to tolerate the oil with no issues.

In my mind, this is the worst of the 3 arguments because it minimizes how serious this issue is. Allergies are NOT something to play around with. Making the assumption that any reaction is just a detox reaction puts people's lives in danger. Continually exposing a person to their allergen has the potential to cause more and more severe reactions with each exposure. I'm not going to claim I know the science behind the detox theory or whether there is some truth to it. What I will say is that what I experienced was NOT a detox reaction. My throat was closing, my oxygen levels were dropping, I was losing consciousness...that is NOT something to be messed with. Now, most people who have reactions to essential oils don't have full blown anaphylaxis, but that doesn't mean their reactions should be minimized. Allergies can get worse with repeated exposure. To assume any reaction is just the body detoxing puts people in danger because it minimizes the reality that these oils can and do cause severe, life threatening situations for some people. Encouraging someone who has had what appears to be an allergic reaction to an essential oil to continue using it to allow their body to "detox" puts them in a position to potentially have increasingly severe reactions. Again, I don't know if there is some truth to the detox theory, and I AM open to the idea that there may be some facts behind it. HOWEVER, what I experienced last week was NOT a detox reaction and to continue exposing myself to the oils could put me (and anyone else who may have had an allergic reaction to these oils) in a very dangerous situation.

Okay, I'm stepping down off my soapbox now. Again, my purpose in writing this was not to start a debate or condemn the use of essential oils. It is to share an often overlooked, but very important perspective. For many people, essential oils are a great alternative or addition to standard pharmaceutical interventions. I'm not in any way trying to take away from that. If you use essential oils, please know that I am not trying to discourage anyone to discontinue using them. HOWEVER, I do want people to know that there is another side to this issue that needs to be shared. Please be aware and understand that there ARE people who are unable to use them in any form. We are not detoxing. Our bodies do not just need time to adjust. We are having serious reactions that can turn into life threatening situations.

Back to the essential oils fair in the medical building lobby. I don't know how many people walked through that building last week, but I'm pretty sure I was the only person to leave in an ambulance due to the essential oils fair. I recognize that I am just one person, and that there were many people who were probably very interested in trying the oils and talking with the representatives about the different uses. However, I don't think it is unreasonable to expect a MEDICAL facility to be a safe place for me to enter. When they put the essential oils in the lobby, they made it impossible for someone to avoid the fair. I am not asking for them to never host another essential oils (or any scented products) fair. All I am asking is for them to consider those of us who are unable to tolerate these substances. Holding the fair in a conference or meeting room off to the side with plenty of signs directing people to the fair would be a way to still offer these products for those interested, but also to allow those of us who cannot be around the oils to have the option to avoid them.

I did contact the building manager to express my concerns, and I received a reply earlier this week. Not sure if anything will actually be done, but the manager did say that someone would be in touch with me after they reviewed the situation to discuss things further. Again, I'm not trying to condemn essential oils or demand that they never be offered in any medical setting. But I don't think it is unreasonable to ask these medical facilities that I spend considerable amounts of time in, to take a few steps to ensure that I am able to even just enter these buildings without fear of anaphylaxis. Medical facilities wouldn't even dream of offering a peanut butter convention for fear of a severe reaction...these oils are just another allergen (granted, a bit more rare, but still, no less serious). I don't think it is wrong to expect a medical facility that is filled with doctors who took an oath to "do no harm" to make a few accommodations to ensure the safety of all patients...both those that swear by essential oils and those of us unable to be in the same room as them.

Wednesday, March 1, 2017

Rare Disease Day 2017

Happy Rare Disease Day 2017!!!

In case you didn't know, Rare Disease Day falls on the last day of February each year. That means that every 4 years, a day celebrating rare diseases is celebrated on the rarest day of the year, Leap Day! Sadly, this year is not a leap year, but that doesn't make today any less important (just slightly less cool =P).

Some of you may have never heard of Rare Disease Day, some of you may have only heard about it from reading my blogs over the years, some of you may be personally impacted by a rare disease as well, whatever the reason, THANK YOU for taking the time to read this post and being a part of raising awareness for the almost 7000 identified rare diseases!

In the USA, a disease is considered to be a rare disease when fewer than 200,000 people have been diagnosed with it. Mast cell diseases as a whole currently fall under that criteria...for now...There are multiple forms of mast cell disease, and while some are definitely very rare, others are still under diagnosed. That means that as more doctors and hospitals are educated in what to look for and how to recognize some of these mast cell conditions, not all mast cell diseases will continue to be in the rare category. 

I was originally diagnosed with Mast Cell Activation Syndrome a little over 2 years ago. This is the most common form of mast cell disease, and will possibly lose its rare label within the next 5-10 years as awareness spreads throughout the medical community. Hopefully as that happens, research will continue to offer better treatment options as well! 

Now, I say "originally diagnosed" because in the 2 years since being given a name for my symptoms, some questions have been raised by a few of the experts in the mast cell community as to whether my diagnosis is complete. Some of my test results point toward a slightly different form of mast cell disease (ironically one that falls into an even grayer area than mast cell diseases in general...). At this point the only way to get a definitive answer is to do some invasive testing. Since treatment protocols are the same whether I have MCAS or this other form of mast cell disease, and the testing is likely to set off a reaction on its own, we have made the decision not to find out at this time. If things change and there are different treatment protocols based on the type of mast cell disease, then we will revisit whether I need this additional testing. For now, though, we are continuing to treat my mast cell disease as best as we can.

And that's why today is so important to me and my family. Because today is a day that we can celebrate hope and continue raising awareness for these rare diseases. This year's theme is RESEARCH. Without research, we have no hope. Let those words sink in for a moment. For a rare disease patient, the ONLY thing that provides any potential for answers for our conditions is research. 

When I was diagnosed by Dr. Afrin 2 years ago, he gave us a list of possible treatment options to try one at a time. Instead of being listed in order of most likely to help to least likely, they were listed in order of cost...start with the most inexpensive options and progress to the ones that cost an arm and a leg and first born child. There is currently no indication as to which treatment options will be better for specific way of knowing until something is tried whether or not it will help, make no difference, or cause anaphylaxis...I've had my share of all 3 of these results...

It can be terrifying to live with mast cell disease. Anaphylaxis is always a very real possibility and can come from known external triggers (smoke, perfumes, scented cleaners, etc.), known internal triggers (digestion, hypoglycemia, pain, etc.) or from absolutely nothing. Even when our reactions don't end up in full anaphylaxis, we still experience chronic symptoms. Mast cell diseases are systemic which means that they can and are everywhere in the body and have the potential to cause issues everywhere as well. Every day is lived trying to find the balance between keeping severe reactions at bay and not being so drugged that we're unable to do anything. 

This is why today is SO IMPORTANT for rare disease patients. I've said many times before that there's no research without funding, no funding without awareness, and no awareness if we as patients aren't willing to speak up. This is my contribution to Rare Disease Day and me doing what I can to raise awareness for mast cell diseases. This is why I share my story on this blog and why I post on Instagram. This is why I've decided to be open and share some of the messy details of living with mast cell disease. Because if I'm not willing to speak up and raise awareness, how can I expect any progress to be made?

And progress IS being made! Rare Disease Day is about more than just sharing the gory details and messy aspects of life with a rare disease to raise awareness. It's also to celebrate the huge steps that have already been taken in making progress toward better treatment options and cures. As I mentioned in December, Dr. Afrin has been doing genetic research looking at possible common mutations between MCAS patients. The first round of testing showed promising results, and he was provided funding to double the number of subjects for the study! There are multiple research projects at NIH focusing on two different forms of mast cell disease and the connection between them and other chronic illnesses that are often diagnosed along with MCAS. In October, ICD-10 codes (aka, insurance billing codes) were released for almost all forms of mast cell disease! This is HUGE because it means that treatments and medications can now be approved based on actual diagnoses instead of just symptom codes which increases the likelihood of insurance approval instead of having to fight for coverage.

This year is the 10 year anniversary of the first officially diagnosed case of MCAS. In those 10 years, a lot has changed in the mast cell community. I am incredibly thankful to be under the care of one of the top doctors in the country for mast cell diseases. I am excited to see how much progress has been made since the first case of MCAS was diagnosed, and I am hopeful that in the next 10 years, we'll be able to see countless more breakthroughs that will make a huge difference in the lives of so many mast cell patients. And I have that hope because of days like today. Even just one day a year focused on raising awareness and funding for rare disease research reminds us as rare disease patients that there ARE doctors and researchers who have dedicated their lives to save ours.

Even in the short couple of years since I have been diagnosed, I have made many friends also living with mast cell disease or another rare disease. Some are only alive today because of doctors willing to take a chance on new research. Others are desperately holding onto hope that a breakthrough will come soon as they are dealing with snowballing symptoms in uncharted territory with doctors who don't know what to do. Research is our lifeline. It's why we continue going to doctors when there are so few answers. It's why I keep traveling to MN every six months. Because we know that as we do our part to raise awareness and funding comes in, someday, one day, there will be more answers than questions. And maybe, hopefully, prayerfully a CURE!