Tuesday, September 19, 2017

Colonial Williamsburg with Mast Cell Disease and Service Dog

If you just want the recap of our trip and all the fun and mischief Nick and I got into during our recent trip to Williamsburg, you can read that here. This post is going to be more about how we managed my mast cell disease and Leni while we were there. I have another post I am working on with more general travel tips and tricks, but this one will be about specifics in Williamsburg.

We chose to stay at the Govenor's Inn which is within walking distance of the historic area. I would recommend it. It is not fancy, but was VERY reasonably priced compared to the other hotels that were within walking distance. It was motel-style (exterior entrances) which I actually prefer when we are traveling with Leni. It makes things much easier when we can just open the door to take her out right before bed or right after waking up instead of having to walk through an entire hotel. Although, we did have a minor access issue with Leni at check-in.

The Govenor's Inn is pet friendly, and they have specific pet policies in place like any business that allows pets. So when we walked into the lobby, the front desk receptionist told us that we could not bring Leni into the lobby. I explained that she is not a pet but my service dog (and she was wearing her vest). She said that it was their policy that no dogs were allowed in the lobby to be compliant with their health code and in the case that any other guests were allergic to dogs. I offered to show her the ADA (American's with Disabilities Act) on my phone where it specifically states service dogs are exempt from health code requirements and cannot be denied access due to just the possibility of an allergy and that if there is someone who is truly allergic, both parties must be accommodated. She still didn't seem convinced, but didn't say anything else and let me stay with Leni.

For what it's worth, I had not planned on being in the lobby much anyway because I wasn't eating breakfast so I didn't have any need to be there. The only reason I said anything is because if I simply agreed to take Leni out of the lobby (even though it didn't matter to me), someone else with a service dog could face the same access issue I did only now the hotel could use my willingness to comply with keeping Leni out of the lobby as an example of other service dogs who were not allowed in the lobby as proof they were in the right. So I spoke up respectfully and showed what the ADA actually says, and hopefully other handlers will be able to be in their lobby with their service dogs without any issues. Even with this minor issue, though, the staff were wonderful, very friendly, and loved meeting Leni when we were out walking her. The receptionist working when we checked out did not say anything about Leni being in the lobby.

The rooms were adequate and what you would expect from a basic, motel-style hotel. Each room had a mini-fridge which we always look for because it means I can pack things that need to stay cold, we can stop by a grocery store to get safe food and drinks for me and extra snacks for Nick, and I can keep my IV fluids cold way easier than having to use a cooler and ice. There was a continental breakfast included which Nick gave about 3/5. Being so close to the historic area was great, and I did not react to anything in the room which is always a plus! (I did call ahead to request chemical free cleaning like I usually do.)

We actually ended up driving both days even though the hotel was within walking distance. We really didn't know what my energy levels would be like, so we wanted to conserve my energy as much as we could. All parking for the historic area is at the visitor center and you can either walk from there to the historic area or take the free shuttle. Plus, the first day we had to go purchase tickets at the visitor center anyway. Colonial Williamsburg actually has an incredible disability discount! When we went to buy our tickets, the lady helping us asked if Leni was a service dog and what tasks she does (both questions are not only legal but encouraged by the ADA). After I answered, she said that they give a 50% discount for guests with disabilities! She ended up giving us 50% off both tickets, not just mine. I'm not sure what the actual policy is, but it might be worth asking. I didn't know this existed, and we already had a discount from staying at a preferred hotel, but she gave us this discount because it was better. They were wonderfully educated on all sorts of disability access and very accommodating.

Governor's Palace from Duke of Gloucester Street. An example 
of the walking to get to some of the buildings.
Even the website for Colonial Williamsburg is very informative when it comes to the various accommodations they provide for all sorts of disabilities. They label which buildings are accessible and where those entrances are. In the buildings that are not accessible, all the historical period actors have an adapted presentation they can do outside the building on request. However, I will say that while it is certainly possible to use a wheelchair in the historic area, the sidewalks are brick or cobblestone or gravel...not exactly the easier surface to wheel across. Years ago my dad was in a wheelchair after ankle surgery and it was very much a workout for whoever was pushing the chair. That was one reason I wanted to walk as much as possible. Because I knew using my wheelchair would be close to impossible for me to propel myself, tough for Nick to push all day, AND could possibly cause m
ast cell issues since vibrations can give me hives and start a reaction.

More walking...the building all the way at the end is the Capitol.
BUT, the streets have lots of benches and are shaded.
Thankfully, I was able to walk both days! The historic area has benches all along the streets which was great for plenty of rest breaks as we were wandering around. Many are in shaded areas, and there are others in the sun for when I needed to warm up. Almost all of the buildings had limited seating for those who needed to sit during the presentations and all the tours we went on had plenty of designated sitting time while the guide was talking. It is a fairly large area, though, so I would recommend planning out your day. We purposefully planned our days so we didn't have to walk back and forth across the entire historic area multiple times a day. That did mean we missed a couple things we would have liked to see, but if I had passed out or had a major reaction, we could have missed more.

Oxen pulling a cart. Leni wasn't sure what to make of them.
When comes to service dog access, they were wonderful. They specifically state that service dogs are welcome throughout the historic area and in all exhibit areas with the exception of the petting area at the farm and recommendations of not having them near the shooting range without proper ear protection. There were actually a lot of different animals all over the historic area. Horses and oxen pulled carriages and carts, there were goats and sheep and chickens in multiple areas, and there were lots of people out walking their dogs. Colonial Williamsburg is open to the public, but you need a ticket to go in the buildings to watch the demonstrations or to take any tours. This meant there were plenty of locals out for a run, enjoying the fantastic weather, and of course, walking their dogs.

One challenge we had is that Leni is not used to being in work mode when we're outside. Typically when she is working we are inside. When we are just going for a walk around the neighborhood or going to the creek or in the yard, she's usually off duty. So she was a bit confused on how she was supposed to behave when we were outside most of the time, but she was still supposed to be in work mode. Her behavior was not wild or inappropriate for a service dog, but it was enough that I could tell she was a bit off because I know her so well. She was more easily distracted when we were outside, and her heel was a bit more relaxed than I like it to be when she is working. We worked through it, and she still gave me multiple alerts during our time there, so I'm not upset with how she behaved. It just gives us things to work on for next time and future outdoor excursions. I hope this can be good info for other handlers to be aware of if you are traveling to Williamsburg with a service dog.

Tired Leni Bean!
Also, something else useful to know is that there is a fantastic dog park in Williamsburg at Waller Mill Park. Typically I am NOT a fan of dog parks, but when we are traveling, it can be a nice way to let Leni run around and stretch her legs. We decided to try it in the middle of the day when most people would be at work, and there was only 1 other dog there who was very friendly and playful. The dog park was in a wooded area and fully fenced with a separate area for small dogs. Be aware, though, that you have to register yourself and your dog at the office before using the dog park. They need proof of current rabies vaccine (and proof of registration if you are local). I use an app on my phone called Vitus Vet where I keep both dog's shot records so I always have them on me. It came in very handy since I could just show copies of her vaccination records even though I didn't bring any of the actual records with us. It costs $2 to enter the park and another $2 for the dog park. The park itself has hiking trails, boat rentals, fishing, disc golf, and probably some other activities so you could go and just hike with your dog if you don't want to pay the extra $2 or don't have the shot records with you. A tired Leni is a well behaved Leni, so we were very glad to find this dog park!

And now back to Colonial Williamsburg...Overall, the biggest challenge for us in Williamsburg was probably avoiding smoke. We spent a lot of time outside either walking between buildings or on tours or watching demonstrations. We encountered more than a few smokers in the historic area. I ended up keeping my mask out of my backpack and readily available at the first sign of a smoker. Sometimes it would just be in passing, but other times we did have to leave demos or tours because of smoking nearby or a smoker also watching or on the same tour. Even though my mask helps, the purpose is to give me time to get away from the trigger. It is not a perfect seal, so it doesn't mean I can all of a sudden stand next to a smoker as long as I am wearing it. We have just accepted that as part of my mast cell disease (and are so thankful that a whiff of smoke doesn't mean instant anaphylaxis anymore!), but it can be frustrating sometimes.

The other place we had an issue with smoke was at the shuttle stop. There is no designated smoking area, so we encountered people smoking right at the curb multiple times, at the visitor center stop especially. That was not cool. It meant we had to wait far enough away downwind that it wasn't an issue, but it also meant that riding the shuttle was potentially an issue. Thankfully we were there at the beginning of September which is considered "off season" for Williamsburg. Kids were just back to school, so summer vacations were over, and it was too early in the year for field trips. That meant the shuttles were mostly empty and we could sit as far away from the smoker as possible, but at busier times, this could have been more of an issue.

The other issue with specific scent triggers in Williamsburg was in almost every single shop. Soap, candles, ink, dye, etc. Every single shop (except the candy store) was selling some sort of scented product and often multiple different products all with their own smell. We realized this pretty early on our first day there. We weren't planning on buying anything anyway, so it wasn't a big deal for us. Nick did go in a couple of the shops that looked interesting, and I just stayed outside. There was a big outdoor market that had a few scented products I think, but since it was outside I could wander around it while wearing my mask without any issues.

View from just outside the patio at Chowning's Tavern
Unfortunately, one thing we didn't get a chance to experience was how they handled food allergies in any of the historic area taverns. Nick did eat at the back patio at Chowning's Tavern for lunch one day, but I didn't get anything. The outdoor patio was a grill menu vs. the more standard historical tavern menu inside. That meant it was cheaper and faster service, but the trade off is that you didn't get any food options that were "historically accurate." The patio itself was very shaded with plenty of picnic tables behind the tavern and was a nice place to sit a take a break from all the activity for a little bit. Supposedly most of the taverns are very accommodating to food allergies, but you should be aware that some do serve peanuts with beverages.

A restaurant outside the historic area but still in Williamsburg that gets incredible reviews for being super allergy friendly is Food for Thought. We were hoping to try it one day, but sadly the nasty masties decided to veto that plan...so sadly I don't have any personal experience to share, but it has gotten multiple fantastic reviews from the food allergy community for how they handle allergies (separate kitchen, well trained staff, very willing to customize dishes, etc). There is also a Food Lion very close to the historic area and the Govenor's Inn that was great for us to pick up safe food for me and extra snacks for Nick. I was pleasantly surprised to see the variety of allergy friendly products and brands they had, so that could be an option as well, especially if you have a refrigerator in your room.

And that was how we handled Williamsburg with the nasty masties and da poodle! We had a great time, and I hope that our experience can maybe be helpful for anyone else heading down that way.

Monday, September 11, 2017

September Shenanagins

With the start of fall (at least it my mind...once September hits summer is officially OVER), Nick and I decided to take Bokatopia on the road for some adventures! After an up and down summer of medical messes and work headaches, we were both looking forward to at least a short reprieve.

Yes, this is Leni with her head
on the wheelchair...silly dog!
Elizabeth and Rover, Me and Tracker
First up was crossing the seemingly endless state of PA to the far eastern side to visit my long time best friend outside Hershey, PA. She made the trek out to Ohio earlier this year, so we had been eagerly planning a time for Nick and me to make it out to visit her. There's a lot I could say about our friendship, but I'll just say that I've known Elizabeth as long as I can remember remembering anything. From playing Little House on the Prairie and creating "musical productions" (note the use of quotation marks =P) with our stuffed dogs Rover and Tracker to figuring out this whole adulting thing complete with army deployments, nasty masties, and all sorts of the craziness that life brings. I am so thankful to have a friend I can literally say I've known as long as I can remember.

We filled our time with lots of fun adventures including the requisite stop at the hometown coffee shop, getting to see Elizabeth's "office" aka life flight helicopter (and enjoying NOT being the patient!), picnicking and attempting to teach Leni to actually catch food in her mouth (with minimal success), puzzling, and watching Harry Potter and prison documentaries (and planning our future life as felons, but shhhh). Shout out to Nick for being a great sport and putting up with lots of laughing and best friend nonsense. I caught him shaking his head at least twice =)

After Best Friend Weekend #2, we left Elizabeth and headed toward my parent's, stopping along the way to visit more friends. Or family of friends who are now also friends. Good friends of ours moved to the other side of the world (literally) at the beginning of this year, so we filled in as kids for the day and visited the family farm that was conveniently located in between Elizabeth and my parent's.

Nick is riding shotgun in the red tractor
We got a tour of their incredible farm house, got to see more
chickens than I think I even knew were possible to have in one place, and watch some of the harvesting of corn. Nick even got to ride in one of the tractors that was collecting the chopped corn. It was super cool to see an actual working farm in action that was beyond just horses. Plus, it was so fun to meet and chat with our friends' family and feel like they weren't on the other side of the world. We love you guys!

Leni spent about 10 minutes in this
position after dropping a toy down in
between the couch and the end table.
After a brief overnight with my parents, we headed off again to what was supposed to be a surprise trip to Williamsburg. I say supposed to be a surprise because I did try to plan this trip without Nick knowing. We had talked about maybe getting to Williamsburg someday and since we have an anniversary coming up I thought it would be a fun surprise. And it probably would have been if I had been able to keep my mouth shut! Far from Nick somehow stumbling across hidden information, I literally blurted it out one evening when we were sitting watching a movie...that should give you an indication of how good I am at keeping secrets...but even with the surprise ruined, it was still a fun trip.


We had so much fun! Growing up in VA, Williamsburg is a field trip destination for pretty much every kid of elementary school age...so I had been before. But I honestly didn't remember much, so it was fun to go around with Nick who had not been there before. We spent 2 days visiting most of the historic area, and we could not have asked for better weather! We got to see what the different trades would have done during the Revolutionary Era, watch how civil cases were handled in the courtroom, went on a tour that addressed how the wealthy would have interacted with the enslaved people that were considered property at that time, and got an in depth tour of the Capitol of the Colonies and the steps that were taken that led up to the Revolutionary War.

We had a great time, but one of the best parts was not even what we did. It was how we did it. If you follow me on Instagram, you may have already seen my post about this. We didn't just get to spend 2 days exploring Williamsburg, but I was able to WALK around for both days. You don't really appreciate some things until they are gone and you realize how much you have taken for granted. Walking independently is one of those things for me. July 2015 was when I first started having issues with balance and coordination and muscle weakness...for the first time. That lasted 8 months before I finally started seeing some improvement in physical therapy. We thought it was due to a bad mast cell flare combined with chronic hypoglycemia, since starting Xolair and getting my sugars under better control seemed to make a huge difference. I had 7 months of working back up to being able to run/walk 6 miles at a time.

Then in October 2016, these issues started to make a come back...only this time I was still on Xolair and my sugars had been relatively stable for a few months. Now, 10 months later, I'm still spending hours in physical therapy working hard to combat the balance and coordination issues and muscle weakness. With no explanation for what is causing these issues, it can be discouraging to keep fighting what feels like a losing battle...especially when I'm faced with losing the ability to run (something I LOVE) and walk independently (so many of us take this for granted!) for the second time. At least the first time we thought we found the reason why. Now even my doctors don't have any ideas.

But anyway, the point of this was not to share what it is like to be dealing with these issues, but to share a HUGE victory. We brought my wheelchair with us fully expecting to need it based on how I have been handling any sort of distances lately. Instead, we never took it out of the car! It wasn't perfect. I almost fell a few times, and we had to take plenty of breaks especially when we were crossing back and forth through the historic area. BUT, between Leni providing her counterbalance and forward momentum tasks, and Nick keeping me vertical when I would struggle and being a steady hand across the uneven terrain, I was able to WALK. What. A. Gift.

Being able to walk around Williamsburg was a gift we didn't expect, but it's not the only thing we are praising God for right now. I started a new medication about 3 weeks ago, and we are actually starting to see some improvement in my GI symptoms. After 10 months of no answers and no real direction, to say I was feeling discouraged would have been an understatement. Unfortunately we still don't have any explanation for why I'm having the contrasting motility issues that I have (slow motility in my stomach, hyperactive motility in my intestines), but we have at least finally found something to address the relentless symptoms that were becoming more and more of an issue with each passing month. It's not perfect, and we still have some things to sort out with the dosage and long term safety of this medication, but it's a step (see what I did there???) in the right direction.

Plus, finally making progress toward managing my GI issues better (even without knowing what is causing things) means we are even moving in the direction of being able to see if I can transition off the IV meds back onto my oral and tube meds. That's probably still weeks to months down the road, but it's on the horizon now! Before starting this medication, none of us knew if I was looking at being stuck on IV meds and fluids long term. We were kind of out of options when we made the decision for me to have a central line and IV access at home, so we all knew it was needed, but it was FAR from what any of us wanted. Obviously I don't know what the next few weeks/months look like, so I'm not saying good-bye to being accessed 24/7 yet. But I am so thankful that it is becoming more of a real possibility and not just wishful thinking.

I know I say this often, but please know that I mean it every single time: Thank you so much for all your prayers. Just like there really aren't words to explain what it means to be able to walk independently, there really isn't a way to put words to what it means to know how many people are faithfully praying for me and my family all over this country and even in some other parts of the world. I hope that as you pray with us through some of the rougher times, you can also rejoice with us during these times of improvement and tangible hope. What a blessing to know that the one thing we know will never change is God's love for us, and I see that love acted out every day through your prayers and encouragement. Thank you. I only hope that my life can be a testimony of the incredible prayer warriors lifting up me and my family and ultimately of the power and goodness of our God even in the midst of some of the tough seasons of this life. I hope that you can also be encouraged with us as God is doing what He does best in being glorified and proving to be a faithful God no matter what is going on in our lives.

Thursday, August 24, 2017

Smashing the "Mast Cell Box" with a Hammer

One thing Dr. Afrin told us before we left my first appointment with him was that before he made any treatment recommendations, he needed lab work showing proof of a mast cell disease. His comment was that he didn't want to give me a life-changing diagnosis if he wasn't 100% sure. At the time I'm not sure I understood. In my mind it had been a long 8 years of progressing symptoms and searching for answers. I didn't think finally having a name to put to the conglomerate of systemic issues I had collected over the better part of a decade could possibly be as "life-changing" as he seemed to think. But as is the case with most things regarding mast cell disease, Dr. Afrin was right.

What happens the moment you get a diagnosis is that you get labeled. There are good things about that. Being able to have a name for the odd symptom patterns and going into first appointments with new doctors with an actually diagnosed condition and not just a mess of jumbled symptoms is not to be taken lightly, especially not in the rare disease community. But it also brings a new set of challenges.

ANY label means you begin to take on the identity of that label. I am female. I'm a wife. I'm closer to 30 than I am to 20 (although still further than Nick!). I'm (still...) a student. I am Jewish. I am a Christian. When I got married, even the name I go by changed from being associated with my parents to my husband. Labels are not necessarily bad. They not only help others understand who we are, but they also give us a way to understand ourselves better. When I got married, I stepped into a new role as a wife. I knew what that meant from seeing my parents and many couples I look up to, but it wasn't until I was there myself that I truly began understanding what it meant to be a wife.

That's kinda that same thing Dr. Afrin hints at when he waits to make a diagnosis until he has actual proof. It's one thing to be watching from the outside, feeling desperate for an explanation, and thinking that just getting that diagnosis of mast cell disease is the perfect answer. But then you get the label you've been after for so many years (even without knowing it in many cases), and all of a sudden new challenges appear.

It didn't take me too long after getting married to realize that no two marriages are the same and that my role as Nick's wife would not look like anyone else's role as their husband's wife. But that didn't keep me from trying to be what I thought was the "ideal wife." I wanted to have dinner ready for Nick when he got home from work. (Side note: Trying to cook dinner for your husband while incredibly nauseas is not a recommended activity and may result in bigger messes to clean up than just the dishes used for cooking.) I wanted to keep up with the inside cleaning so he could take care of the yard and cars. (Side note #2: Attempting to vacuum while dizzy and lightheaded will most likely end with passing out on the still un-vacuumed floor.) Every time I felt like I wasn't able to do something I thought a wife "should" do, I felt like I wasn't being a good-enough wife for Nick. It wasn't even competition with others that made me feel like I didn't measure up, it was being unable to attain unrealistic standards I set for myself.

Receiving a diagnosis, especially a rare disease diagnosis, is actually not all that different. All of a sudden we have a label and feel the need to "fit" that label. Only in many rare disease cases, there is no box to fit in or set list of absolute requirements...yet somehow we still try to create some sort of standard that we can point to and say "look, I fit in this box!" How can we not when we spend YEARS searching for answers and often being told nothing is wrong, we're just making things up, it's all in our head, etc.? There is a huge feeling of validation that comes with finally being given a diagnosis after many false alarms and false accusations over so many years. It doesn't feel like 3 simple words can possibly be any more life-changing than our symptoms already have been...but they can.

One of the reasons mast cell disease is so difficult to diagnosis is due to the systemic nature of the condition and the fact that no two patients are the same. I have friends with mast cell disease who cannot have anything by mouth, not even water. I have other friends with no food restrictions of any kind. I know some people who cannot tolerate hot climates and others who cannot tolerate cold. Some people with mast cell disease gain weight, while others fight to keep weight on. I have friends who are on oxygen 24/7 and others on tube feeds or IV nutrition. Some mast cell patients go through epipens like teachers go through pencils (shout-out to all my teacher friends heading back to school!), and others who have never had to use one. I could go on...everyone diagnosed with mast cell disease knows how diverse symptoms are.

Yet even knowing how complex mast cell disease can be, I think most of us still go through a sort of "box phase" to some degree. That's where, even knowing that no two cases of mast cell disease are the same, we somehow try to fit into a "mast cell box" created only by our own impressions of what living with mast cell disease *should* look like. Sometimes it happens immediately after being diagnosed when we finally feel validated that our symptoms really aren't all in our head like so many doctors tried to convince us of for so many years. Other times it may come later when we still feel no closer to answers than before having the diagnosis and we think that maybe it's because we're don't really have the right mast cell symptoms. Or maybe it comes when another doctor doesn't think mast cell diseases are legit and we feel like we're right back where we were before being diagnosed...and we think that maybe if we look more like we had mast cell disease, then maybe they would believe us. A lot of times, I think we have to go through varying degrees of all these situations.

What Dr. Afrin knows when he sees his mast cell patients is that the diagnosis is not the ending that many of us have been searching for over many years. It's really just the beginning of a new phase of life with a rare disease that currently has no FDA approved treatment options and has the potential to be life-threatening. A mast cell diagnosis is so far from the end of a long journey that most of us feel it should be. It's the beginning of a new set of challenges and attempting to fit into the "mast cell box" (that doesn't exist...) whether consciously or unknowingly.

I'd be lying if I said I didn't struggle at times with fitting into some "mast cell box" of my own creation. I certainly wondered if I didn't have the "right" symptoms when a local allergist completely discounted my mast cell diagnosis only a few months after being officially diagnosed by Dr. Afrin (which if you remember included actual abnormal lab results, not just speculation). When I have symptoms that don't appear to make sense (aka fit into that "mast cell box"), I question whether I'm being honest with myself and maybe things aren't really that bad. I feel like I'm on trial and trying to convince a jury of the truth when I end up in a new ER or with a new doctor while admitted and have to start from square one explaining my mast cell diagnosis and symptoms that landed me in the ER or hospital yet again.

So yes, at times, I have found myself trying to fit into some sort of imaginary mast cell box to prove to the medical world (and sometimes myself) that I'm NOT making things up, my mast cells ARE NOT playing nice, and I really do need help to be able to live normally. Why should we have to fight for that? That is why this whole "mast cell box" that doesn't exist sometimes feels like it does.

Because instead of being treated for a progressing rare disease, we've been told FOR YEARS we can't possibly have the symptoms we report, that our symptoms don't make sense and can't be real, and that we are liars. It takes time to move beyond being told by multiple doctors in often more than one state that we are (1) making things up entirely or (2) can't possibly be dealing with the symptoms we have. I had a neurologist at a world renown medical facility say to my face (with my mom in the room) that I wasn't sick enough to be in his office and to come back when I couldn't walk. Two years later I couldn't walk unassisted and I half felt like going back to him and saying that maybe if he had done something two years earlier I wouldn't have had to ever get to the point where I couldn't walk. That is why the "mast cell box" that doesn't exist is so very much real.

But even when we feel like we have no choice but to cling to that "mast cell box" during different seasons of life after being diagnosed, we learn that it doesn't have to be that way. It's been over 2.5 years since being diagnosed, and (most of the time) that mast cell box is long gone. Because I've realized that even if there was a box, it wouldn't matter. I'm not here to be the best mast cell patient I can be. I'm here to live the life that God has given me, mast cells and all. The box doesn't matter anymore because I'm not trying to figure out what it looks like to have a mast cell disease. Mast cell disease looks like me, and it's my goal to push the limits (in a good way!) of what life with mast cell disease can be.


I want to shatter any notion that being diagnosed with a rare disease has to look any sort of way to be legit. I want people to see a life blessed by God regardless of any health challenges. I don't want to go to my doctors and hear that I'm a "textbook" case of mast cell disease. I want other people to see that a life-changing diagnosis doesn't mean having to fit every point on some sort of chronic illness checklist, it just means now we know what we're up against. Life does change with a rare disease diagnosis, and I'd be lying if I said it would always be a walk in the park. But I want people to see that it doesn't have to be easy, I'm just looking to figure out what is possible.

Image result for yzma and kronk
As I was writing this post, I kept trying to find the right words to really express my true feelings about dealing with that "mast cell box." But then I realized someone already said it perfectly...In the words of Yzma from The Emperor's New Groove, " I'll put that box inside another box, and then I'll mail that box to myself, and when it arrives...I'll SMASH IT WITH A HAMMER! Or...to save on postage, I'll just POISON him!" Seriously, is that not THE BEST quote ever or what?? Okay, maybe there's nothing to poison, but it's just not complete without the ending =) Although...if anyone has any brilliant ideas on how to poison the nasty masties, I'm all ears...Let's get rid of that "mast cell box" for good.

Friday, August 18, 2017

Pharmacies vs. Kylene Round: ???

You know your specialty pharmacy has some issues when them actually doing their job correctly comes as a bit of a shock. I mean, they actually received an order, filled it, and shipped it without me holding their hand every step of the way??? Seriously people, this is big news! The irony is that this week I actually had time for them to screw up...of all the weeks they could actually get something right and it barely matters...go figure...

While this week my specialty pharmacy appears to have at least gotten something right, my online pharmacy has apparently decided to step up its game these past 2 weeks. So don't worry, I've still gotten my money's worth of unlimited calls...and texts...

My online pharmacy is where I fill most of my prescriptions. I get a 3 month supply each time I order, so it is great for the 2 months and 2 weeks where I don't have to talk to them. (Side note: my specialty pharmacy and online pharmacy are sister companies. In case one wasn't enough fun on its own...) Then we hit 2 weeks of fun and almost daily conversations until I finally receive all my meds and have another 10 weeks until I have to do it all over again.

How Leni feels about the pharmacy craziness...
This time started off about the same. It got a bit more confusing because on top of needing to refill 7 or 8 medications, some of the prescriptions had expired so we had to get a new script on file. Not a big deal, they just reach out to my doctors who update the order. Now, up until this past order, I was enrolled in their text alert system, meaaning I would get an automated text when my order had shipped. And that my friends, is where this story begins...

Monday of last week (so not 5 days ago, but 12 days ago), I started getting texts stating that my order had shipped. The first 5 or 6 were expected, but when it kept going I was a bit surprised. It seemed early for them to have already gotten renewed scripts for the couple of orders that had expired, but I wasn't going to complain! At least not until 57 texts later...

Yes, I am on more medications that I would like...but not 57...at that point you're taking meds to deal with side effects from meds that have other side effects from the meds that actually treat whatever condition is buried beneath those 57 medications...Anyway, after looking at the Rx # for each medication, it became pretty clear that NONE of those medications were mine (although, full disclosure, I stopped checking after 7 or 8...I suppose it's possible one of my was hidden somewhere in those 57 texts...). I thought about calling, but figured with that many texts, the pharmacy likely already knew of the issue. Plus, I really didn't feel like spending even more time on the phone with them...

Unfortunately, I ended up spending time on the phone with them Tuesday, Wednesday, and Thursday of last week as well Monday of this week. This was because those 57 texts ended up not being an isolated incident...I got another 63 texts on Tuesday, 73 on Wednesday, 84 on Thurdsay, and 72 on Friday. When I called on Tuesday I was told they removed my phone number from receiving notifications. On Wednesday I was told they would delete my phone number from their system entirely. On Thursday I was told they had no record of my number in their system anywhere. Friday I took the day off.

Sometimes you just have to hide your face and shake your
head...or, if you're Leni, rest your head on the spokes of a 
wheel...
After another 62 texts on Monday this week, I called them yet again. This time they at least admitted there was a *slight* glitch in their system. No kidding...They couldn't give me a time frame of when they expected this issue to be resolved, but said they were aware of the issue. Since calling them at this point is pretty pointless, I've just been keeping a running tally each day. This week we're up to 236 texts so far...since I've usually received them by now, I'm cautiously optimistic that maybe they finally fixed their *slight* glitch...

On top of the fun with my online pharmacy and the surprising accomplishment of my specialty pharmacy, it is only fitting that my 2 GI appointments of the past week and a half also ended with trialing a new medication which apparently could not be filled at any of the local pharmacies...Acme, Giant Eagle, Walmart, Costco, CVS, and Walgreens all said they couldn't fill the script...CVS even told me the medication didn't exist...

Thankfully I WAS able to find a place to get the script filled, and this weekend should be another fun round of how-will-this-medication-mess-with-the-nasty-masties. Actually, we're hopeful that I won't have any major issues with this new med. We know I tolerate other medications in the same class as this one, so the risk of anaphylaxis should at least be fairly low. The big questions are (1) how sleepy will this make me, and (2) will it actually work since, you know, that is kinda the whole reason I'm taking this med in the first place.

I saw 2 of my GI doctors within the past week and a half and both agree that this is probably the best option at this point. Treating my ongoing GI issues has only become trickier as I have two different forms of dysmotility in my gut with each being contraindicated for treatment for the other. We are hoping that this new medication will only impact my intestines (the intended target) since it will be going directly down Sherman (J tube) and by passing my stomach entirely. Plus, the receptors for this medication are in much higher concentration in the intestines than the stomach. So we are hopeful. Guess we'll find out this weekend!

That is if this pharmacy manages to fill this prescription properly the first time...with my track record with pharmacies...I'm not holding my breath...

Friday, July 28, 2017

We All Have the Same Fear

I am living with a rare disease. I am considered disabled. I am a woman. And I could not have survived on Obamacare.

Wait, what? Did I just say that?

Because facebook would lead you to believe that anyone with a pre-existing condition or chronic illness or is female cannot possibly think there can be any solution other than the ACA for health insurance. But for me, the opposite is true. An Obamacare policy would not have allowed me to see 2 of my much-needed doctors and would not have covered 2 essential medications. Both of the doctors and medications are a major reason I have been able to avoid constant, recurrant, life threatening anaphylaxis. I do not have the luxury of simply finding new doctors or switching to a different medication. On top of that, a policy that denied doctors and medications as well as significantly increasing my deductible and out of pocket max would have run about 40% of our monthly income...a 60% increase in cost with a significant drop in coverage.

Let me go one step further. I could not have survived on Obamacare, but the ACA saved my life.

Wait, what?

Yep, you read that right, too. Mandated coverage for pre-existing conditions, the elimination of annual and lifetime caps, and being allowed to stay on my parent's policy until 26 (and then longer with COBRA) guaranteed me the coverage that I have had the past 10 years that has kept me alive. The very policies that would have been a disaster for me had I been forced onto an Obamacare plan were the same ones that offered me the lifeline that ensured my healthcare over the past few years.

I'm sharing this with the hope that this can be an example of how nothing is ever 100% bad or good. Because I think that is being missed right now. The Republicans are operating under the premise that the ACA has to be wrong for this country because it was put into place by the Democrats when the reality is that it did make some big steps in the right direction. At the same time, the Democrats need to recognize that while there were some pieces to the ACA that were very good and should be kept, it was not perfect, and there is definitely still room for improvement.

Too bad that right now the healthcare debate is an emotionally charged argument with no actual movement in a positive direction. The Republicans keep throwing new repeal and replace plans together before the previous one has finished circulating through Congress, and the Democrats continue to rely on the voices of those who benefited the most from Obamacare to sway the emotions of the public. Neither is the right course of action. The ACA is not the savior of the healthcare system that the Democrats so desperately want people to believe. But the Republicans don't have anything better, and are just treating this issue like another political battle to win rather than recognizing that the decisions they make will have a significant impact on the lives of millions of Americans.

I know this is a heated topic. I am not going to even try to pretend that I have a solution, because I don't. And that's not my point for writing this post anyway. Like I said at the beginning, pretty much the only story going around facebook is the idea that anyone disabled or female or living with a chronic illness is fully in support of Obamacare and the ACA. I just wanted to share another perspective that has been missed either intentionally or simply because people are scared to even suggest there might be something wrong with the ACA for fear of being accused of all sorts of racism, homophobia, ableism, etc.

There is no perfect solution. There will ALWAYS be people who fall on the wrong side of whatever policy is put into place. As I shared above, it appears that my experience falls into that category for Obamacare. It is an incredibly difficult position to be in to recognize that the same legislation that increased access to healthcare also effectively ruined my access to the only doctors and treatments that are able to do anything for me.

Part of the reason this has become such a heated debate is due to the fact that people are scared. How can they not be? People who are only alive because they had access to healthcare are watching and waiting to hear how their lives may change as various policies are circulating through Congress. But just as they are terrified of losing access to healthcare, I want to remind everyone that there are those of us who fell on the wrong side of Obamacare also struggling with that SAME FEAR.

I understand that Obamacare allowed millions of Americans to have access to healthcare. I understand that it made progress in improving our badly broken healthcare system. But just because I understand doesn't mean that I don't have the very same fear of how these policies will impact me in the future...I'm just in a slightly different position where Obamacare IS the problem for me. The fear for those of us facing losing coverage under Obamacare is JUST AS VALID as the fear everyone scared of losing coverage if the ACA is repealed is feeling.

Now, please don't mistake this post as support for whatever policies the Republicans decided to submit for a vote this week. Despite my experience with Obamacare, I also cannot support attempts to repeal the ACA simply to win a political victory. As I said earlier, I DO recognize that there were positive steps made, even for those of us who would have lost coverage under Obamacare.
The ACA is not perfect and needs work. But some replacement plan drafted in a matter of weeks cannot begin to address the complex issues facing our healthcare system.

This post is not intended to be a political post. I am not on either side of this debate...I was unwillingly thrust into the middle. My purpose is simply to share my story. The media has been saturated lately with people who have various disabilities speaking out in support of Obamacare and stories of children who will be left without essential coverage should the ACA be repealed. My purpose is to draw attention to those of us who fell on the wrong side of Obamacare...but still can't support the AHCA or BCRA or whatever is the latest proposal.

The unfortunate reality is that there will always be someone on the "wrong side" of whatever happens. Our voices are no less valid because we are the minority. Our fears are just as valid as the fears of millions facing losing coverage. They are scared because they can see what could be their reality should the ACA be repealed. Some of us are scared because we are already living with that reality under the current healthcare system and are desperate for a lifeline.

There is no and will never be a perfect solution. My experience with Obamacare and the ACA was both potentially devastating and equally life saving. Whatever legislation comes next could be similar. Of course I hope that whenever the dust finally settles, I end up benefiting from whatever legislation is put into place. We ALL do. Let's all come together and at least agree on that. And it is scary to be facing an unknown future being determined by people who are far removed from the front lines of these healthcare issues. And for those of us on the front lines, we can feel helpless to do anything in a debate that is effectively determining the future of our disease progression or the extent of disability or quality of life.

This blog has never been for anything more than sharing my story and experiences with mast cell disease. This is another piece of that story whether I like it or not. Healthcare has already been a huge part of my life, and while I've definitely had my share of going 10 rounds with my insurance company, the fact that I have the coverage that I do is what has kept me alive. This is an incredibly sensitive topic, and I know that. I am not claiming to have any answers or solutions. I honestly hoped I could let things play out and not feel the need to post anything...but then I realized that I couldn't do that. In the midst of an increasingly polarizing debate, I have found myself in a unique position with one foot on each side of this issue. That is why I ultimately chose to post this. Because I use this blog to share my life with mast cell disease, and this has been and will continue to be a huge part of my story. 

Thursday, July 20, 2017

Redemption Through Chemistry

Ready for some deep thoughts born out of many nights spent on the bathroom floor? Before you laugh, I'm actually serious...these are a compilation of thoughts I've had over the past week between the hours of midnight and 4 am...

If you didn't already know, I majored in chemistry in college (and Spanish, too, but that's not relevant for this post. Maybe my next round of bathroom thoughts by Kylene will be in Spanish...). I really enjoyed my undergraduate chemistry program. So much so, that I went on to get a MS in chemistry. The fact that I am no longer in chemistry should be an indication of how much I enjoyed THAT program.

Image may contain: one or more peopleApparently once you get to Masters level chemistry, the point is to prepare you to go into a chemistry career...they're not quite as tolerant to minor (and only 2 major!) explosions, experiments just for the purpose of changing the color of the chemicals, melting gummy bears in a fiery blaze, and miscellaneous other mischief...I'm pretty sure my advisor was relieved when I graduated! And I know the budget was...there was a reason my lab was referred to as the Glass Graveyard.

All that aside, I really did enjoy my undergraduate chemistry program beyond just playing with fire and having access to all sorts of dangerous chemicals. It was fascinating to me how 2 completely opposing chemicals could come together and create something that had no resemblance to either. Things that individually would be toxic or dangerous come together to create something not only safe, but essential for life. To me, there was no greater proof of a Creator than to just look at the intricate science behind these chemical relationships that are the foundation of our entire world.

It is one of those relationships that gave me the aha moment that prompted this post. One of my secondary diagnoses is POTS which stands for postural orthostatic tachycardia syndrome. Basically it means that my brain and my heart don't communicate properly to regulate my heart rate, blood pressure, and all sorts of other things that are considered "automatic" functions of the body. There are medications that can be used to help the autonomic nervous system (ANS) function better, but almost all are not recommended, if not actually dangerous, for someone with mast cell disease. The main reason being that the medications can interfere with the effectiveness of Epipens. So that means, no medications for me to address the POTS. Instead, there are other, non-medication options that can help. One is a high fluid, high salt died. The increased fluids act as a buffer to give the ANS more time to react to needed changes in blood pressure and heart rate, and the increased salt helps the body hold onto those fluids instead of simply passing them through.


Simple salt. Sodium chloride. NaCl.

Not just important for those of us living with POTS, but an essential compound used in many different ways all over the world. Sodium chloride is one of the best examples of this coming together of complete unrelated elements to create something bearing no resemblance to either. And it's crazy when you think about the chemistry behind it.

Sodium is an essential element for humans which means we need to get it through diet; our body cannot make sodium on its own. The balance between sodium and other elements in our body is important in maintaining many functions, one being fluid balance that I mentioned above. At room temperature (so the temperature it was in the Boom Room where all the chemicals were kept =P), sodium is a soft, silvery-gray metal block. It is the consistency of butter and is cut just like you would cut a stick of butter. At room temperature, it is extremely reactive with water and can be explosive under the right conditions. Small amounts sliced off a block of sodium into a sink cause small, sparking explosions when the slivers hit the water. (I will neither confirm nor deny whether I had any first hand experience with any reactions of this sort...)

No automatic alt text available.And now, the second half of salt, chloride. Or, technically, chlorine in its elemental state. Unlike sodium, chlorine is not stable as a solid, but as a gas. It is most known for first being used as chemical warfare during World War I. This gas is very toxic, and there is no treatment for chlorine gas poisoning. It is not nearly as much fun to play with as sodium...

Two very different elements, each individually dangerous and having the ability to cause damage and destruction upon exposure. Yet bring them together and we get a compound that is essential for life. Is that crazy or what?!?!

And if you think about it, that's exactly what God does with us. We all have those moments in our lives where we felt ourselves crumbling under the weight of whatever was going on. We feel broken down, destroyed, damaged beyond repair...And we think there is nothing that could bring about good out of the pain and hurt. Yet looking back, often we get a glimpse of how God took some of our darkest moments to bring about light we never could have imagined.

God takes the destruction, damage, and pain the enemy intended to use to destroy us and brings together moments that individually make no sense beyond the pain, but together show us evidence of God's ability to redeem even the times in our lives that we feel beyond repair.

We could not survive on earth without sodium chloride, yet each element on its own has the ability to kill. Often there are experiences or circumstances in our lives that threaten to crush us. Just like how sodium and chlorine come together to create something we cannot live without, God is able to take moments of darkness and hurt and redeem them to something that bears witness to who He is, and NOT to the events that threatened to destroy us.

Monday, July 17, 2017

I'm NOT sick...I'm just...

I know my posts have become a bit more infrequent the past few months. It's partially a reflection on the fact that I really don't have many updates to share. We're still trying to get to the bottom of the relentless GI issues that began last November...

Month 9. I think this is my longest flare to date. At what point does it stop being just a flare and move to just being how things are now? Hopefully longer than 9 months because I'm not quite ready to wave the white flag yet.

I had an appointment with my neurologist this past week. I like my neurologist, I really do. But this time it was a discouraging appointment because I had been holding on to some hope that maybe she could help with some of the increasing neurological symptoms I've been having. Instead, she basically told me what I've been hearing from everyone for the past 9 months...these "new" symptoms are just fall out from my cranky GI tract. Unfortunately that means nothing much is probably going to improve in this area until we make progress with getting my intestines to start absorbing again...everything is just coming back around to GI...

Years ago when we were first starting down this road of on going medical issues, I remember being desperate for a team of doctors who could help. We probably spent every other appointment dealing with doctors who either had no idea what was going on or didn't care to figure anything out or doctors who thought I was making things up or making myself sick. I have been blessed to always have at least one doctor who believed I wasn't just starving myself or making things up, but that hasn't always meant we've been able to do anything about my symptoms.

Somehow I always imagined that once I got diagnosed and finally had a team of doctors who knew what I was dealing with and had the resources to do the right tests and ability to prescribe the necessary treatments that it would be smooth sailing. I kinda of knew I might not be 100% healthy or get all the way back to where I had been, but I had no reason to doubt that what I considered my worst symptoms at the time wouldn't improve. I just needed that team of doctors who cared.

Yet now I'm facing a different reality. I have that team of doctors. I am very thankful for all my doctors. I truly believe that they care and want to help me any way they can. So what went wrong? Why is it that now that I have that team of doctors I used to only be able to dream about that my symptoms are worse than I ever could have imagined?


I remember going in to appointments feeling like I had to fight for what I needed, either because the doctor really didn't want to have to deal with my odd symptoms or because they didn't think there was anything actually going on. At my neurology appointment this past week, my doctor spent about 5 minutes trying to convince me that some things really are more of an issue than I'm willing to admit. That was a weird turn around...

In my mind, I'm not sick. I'm just me. Okay, so I happen to be living in a body that is allergic to the world, have practically a pharmacy in my house (and in my backpack), 2 feeding tubes, a central line, spend more time on the phone with my insurance and pharmacies than my family, but SICK? No way. Maybe I'll concede that I'm living with a chronic illness, but me actually chronically ill?? Nope, that's not right. I'm just...not sure...I can't really call myself healthy either I suppose...

So if I can't quite call myself healthy, and I'm most definitely NOT sick, what does that leave as options??? As someone with a science background, I thought I'd look at it from a purely quantitative perspective. I'm just...performing systemic functional analysis of my insides (with occasional spot checks of the outside as well)...

SPOILER ALERT: I think something's wrong...

Thursday, June 29, 2017

It Takes Effort to Mess Things Up This Bad...

I grew up with a mom who is all about numbers. She taught Algebra 2 for years in our homeschool co-op, loves data and spreadsheets and graphs, and was a pro at on-the-go calculations of what percentage (and estimated foot falls) we had left of our 17 mile long run when we just passed the 4.5 mile mark. So while, I may not quite be the numbers expert that she is, I can make a decent effort.

So when I say that the probability of a pharmacy getting as much wrong as they did in a single week is just not statistically possible, you're looking at them actually having to put in effort to make as many screw ups as they did...

Leni's response to the hours I spent on the phone trying to sort 
this out...and yes, for those of you wondering, that is a 
Norwex dryer ball...also doubling as a service dog toy...
No, I'm not actually saying I think my specialty pharmacy messed things up on purpose...I AM saying that the odds of all this happening at once at not working in their favor. But you add to the equation how many times I've had similar mix-ups, and all of a sudden either they need some major quality control reviews or someone has some deep rooted bias against black fluffy poodles or large stuffed hedgehogs or slurpee consumption or some other randomly held belief. I'll leave you to decide which is more likely =)

At this point, you're probably wondering what the heck happened....I'm so glad you asked! Pull up a chair, sit back, relax, and get ready for some not-so-happily-ever-after story time. (Spoiler alert: It does end on a good note, so I guess it is happily ever after...until next time at least...)

So, some background. Because I have Mort (my port), I am able to give myself IV medications at home. I have 2 prescriptions that I get filled each month, these are ondansetron (aka zofran) and diphenhydramine (aka benadryl). To be able to give myself these IV meds, I also need other supplies one of which comes in the form of another prescription for saline flushes (basically 10 mL syringes pre-filled with sodium chloride that I use to flush my line before and after I use either of my meds). THEN, I have one additional prescription with this specialty pharmacy. My Xolair that I receive every other week is the last prescription I have on file here, but I do not receive this at home. This one ships directly to my doctor's office once a month with 2 doses at a time. Four prescriptions, one that I've been ordering for a year and a half, the others for going on 4 months now, NOTHING brand new. Ok, following so far?

This whole mess started two weeks ago. If you remember (and I barely do so it's okay if you don't), I was in MN the weekend before to see Dr. Afrin for my 6 month follow-up. My mom and I got back to Ohio on Tuesday and we had friends come in Wednesday to stay overnight, so Thursday was the first day I was able to call my pharmacy to refill my meds.

I called and we set up the order. For whatever reason they set it up as 2 separate orders in the system so I had one order with the zofran on it and a second order with the benadryl and flushes. I was told that they didn't have a full order of the zofran in stock, but they would send a partial order then send the rest once it came in. That was fine, but I made sure they understood that I needed these meds (however much they had) because I was going to run out over the weekend and these are a huge reason I have been able to stay out of the ER. The lady I spoke to assured me that the benadryl order would ship in full, no issues (remember the flushes are on this order...this will be important in a minute) but the zofran would be a partial order. Again, that was fine, so she submitted the order.


At this point I also asked about my Xolair because I was due for it in just a couple of days but my doctor did not have the next doses yet. She pulled up that order and said they were still waiting on one more authorization, but as soon as that came in, it would be good to go. I explained how important it was that I did not miss a dose, so she agreed to mark it as a STAT order which would hopefully speed things along. And that was the end of Day 1...

Friday comes and I get a phone call about 10 am to let me know none of my medications had shipped like I was told they would. This time, the reason was that the flushes were out of stock, so the entire order had been held up. Since it does not appear to be possible to talk to the same representative ever, I re-explained the whole situation to this new person. Apparently in less than 24 hrs, they all of a sudden had a surplus of zofran (while I was also told they had not received any shipments of it) so she would set up a special delivery for Saturday with the zofran and benadryl. She told me the flushes were out of stock (which no one had mentioned the day before), but they would send those as soon as they came in. That was not unusual because they don't always stock the pre-filled syringes so I expected that. Typically I would get a call the next day, and they would ship only a day or two behind the meds.

I, again, also asked about the Xolair and was told they were still waiting on something from my doctor, but as soon as they received it, the order would be good to go. I can only get my Xolair on Monday or Tuesday, so I had a *bit* of wiggle room, but not much. As long as they got what they needed on Monday, my doctor would have it on Tuesday and I could still get my injection in the afternoon, so I didn't push it.

Saturday, the zofran and benadryl arrived with no issues. I give my pharmacy maybe half a point here since it was delayed for no apparent reason.

Monday morning I called my doctor's office to see about scheduling my Xolair and see if they had sent what the pharmacy needed to ship. They said they had already re-sent everything twice the previous week, but had not yet received anything. I called my pharmacy and left a message for whoever I was supposed to talk to, but heard nothing. By the end of the day with no call back, they sealed the deal on me missing my Xolair this week.

Tuesday morning and I'm again on the phone with my pharmacy. Not only did my Xolair not get shipped in time, but I still hadn't heard about the flushes. Now, having my meds was great, but I cannot use my IV meds without having saline flushes. At this point, I'm getting to the point where I may not be able to use my IV meds if we don't get this sorted out in time.

I spent almost 2 hours on the phone on Tuesday. It appeared the pre-filled syringes were now in stock (yet no one called like they said they would), so the order was set up. I confirmed, double, and triple checked that what I was being sent were the pre-filled syringes and NOT the vials. I cannot use the vials for multiple reasons, so even though it is technically the same medication, they are useless for my needs. The person setting up the order confirmed multiple times that yes, he was sending out the pre-filled syringes and I would have them the next day.


Wednesday comes, the order arrives, and I had a partial order of the vials...they couldn't even get the wrong order right! BACK on the phone again...at this point, I have only a few flushes left until I would be unable to give myself IV meds. The person I spoke to on the phone listened to the problem, then came back and said since they had already sent out the vials and billed my insurance for them, they couldn't send me anything else because it would be double charging my insurance which would mean they would likely deny coverage for the second order...

While I may have had to give up a lot to my mast cell disease, one of the things that I have gained is the ability to know how insurance works and how to work around some of these issues. I calmly and politely (at least I was trying, okay?) told him that (1) I had called yesterday and double and triple checked this order was correct and was assured that it was (and that he was welcome to go listen to that recording if needed) and because of that (2) he needed to figure out how he was going to resolve this because it was not my fault that they screwed up, but it would be on them if I ended up in the ER because I didn't have the meds that I need since it was their mistake in the first place.

Please understand that it is never my intention to be rude or "that patient" when I call to resolve these issues. I wish everything could be resolved with a simple please and thank you, but that is not reality unfortunately. Had I not pushed this issue, I would not have received the meds that I needed and very likely could have ended up in the ER. I will readily admit my mistakes if something I did caused the error in the orders, but this was not my mistake and I should not have to suffer the consequences of them messing up. As a pharmacy, they need to understand that they are speaking with patients who rely on the medications they dispense to stay alive and function. Mess ups in our orders are not just inconveniences most of the time. These mistakes are the difference between ER trips and managing symptoms at home. So while I do my best to be polite and firm in these situations, I will push until things get resolved.

After going back and forth with the representative I was talking with and the pharmacy and his manager, I was finally told that they were going to need to call me back the next morning. I made sure to get a direct call back number and the name of the person I was speaking with before hanging up. Thursday morning, I did receive the promised call. He said they would be shipping out the pre-filled syringes to arrive on Friday and that they would deal with amending the billing with my insurance. I thanked him and told him that I was not upset with him personally and I did appreciate the work he put in to get this order correct. I do understand that the people on the phone are not the ones (usually) dealing with the actual medications, and I did appreciate him sorting everything out (eventually), but again, sometimes you have to be firm and push to get things fixed. I am willing to do what is needed to get these issues resolved, but I will also thank the people who do get things sorted out.

So, before I finish that piece of the story, back to the Xolair. After my very nice, but firm insistence that they figure out how they were going to fix their mistake, I got transferred to the right department to figure out what was going on with the Xolair. I was told the same thing again, they were waiting on documentation from my doctor. I told them that I had spoken with my doctor's office and that the paperwork had been resent TWICE already. She dug a little deeper in my file and discovered that, yes, my doctor had already sent the proper authorization...and she could even see that they had resent it two days later. Just out of innocent curiosity, I asked for the dates on the faxes from my doctor...okay, maybe not so innocent, but they had seriously exhausted the limits of my patience this week...who's been praying for patience for me recently?? PLEASE STOP! =)

You may have already figured out where I'm going with this, but yes, the faxes had happened with plenty of time to have shipped the Xolair in time for me to have gotten my injection this week...Somehow they got lost in the system and had I not kept checking back who knows if it would have even gotten sorted out for next week. This was too far removed from when things had happened for me to even begin to figure out what had really happened, so I just asked for it to be shipped as soon as possible. I did get the confirmation email that it had shipped to my doctor's office, and when I called to schedule Xolair for the next week, the nurses confirmed they had the vials. I give them a quarter of a point for this...if I end up in the ER because I had to go an extra week in between injections, I'm taking that quarter point back =)

Thankfully, the happy ending I promised happened about 1 pm on Friday when the pre-filled syringes showed up (more than a week after I started this process). (Although I won't mention that the UPS man left a cardboard box labeled 'medical supplies' sitting in the pouring rain...) So after hours on the phone and a week of confusion and mishaps, I do have all 3 of the meds that I need at home, my doctor has the Xolair. Now I can breathe a sigh of relief that I have 3 whole weeks until I have to do this all over again...

Thursday, June 15, 2017

Last Trip to MN!!! Kind of =)

We just got back from my latest trip out to MN to see my mast cell specialist, Dr. Afrin (actually his PA this time). If you've been reading this blog for any length of time, then you know (1) Dr. Afrin is pretty much amazing as a doctor and researcher for the mast cell community, and (2) I go to MN every 6 months to follow-up with him (or his PA). Except not anymore...maybe...

Could I be any less clear? Probably =)

Leni just resting that heavy SD brain she's got =)
Anyway, the big headline news in the mast cell community of the past week is that Dr. Afrin is leaving the University of Minnesota where he has been for the past 3+ years. At first glance, I think pretty much everyone in the mast cell community was in disbelief after hearing the news. How could such an incredible researcher and advocate for his many rare disease patients be leaving when so many of us (and our doctors) rely on him for his vast understanding of mast cell mechanisms and disease processes?

The answer...he couldn't...So he's starting his own mast cell institute! 

There is already a Center of Excellence for Mastocytosis and Mast Cell Activation Disorders in Boston where there are a couple other mast cell specialists that many east coast patients see. But other than that cluster, the mast cell knowledge is very much sparsely spread out across the country. In addition, beyond the specialists in Boston, Dr. Afrin, and a couple of others, there are very few doctors who truly understand the complexity of mast cell diseases and have the experience and knowledge to effectively treat them. 

It is very exciting news that Dr. Afrin is heading off on his own to start another center for mast cell research and treatment! We hope that as he gets set up with his new institute, more doctors will be brought on that are familiar with mast cell diseases, can learn from Dr. Afrin, and become additional resources for the many patients who wait years to see some of these top specialists. We are hopeful that with this new institute, new research can be done, additional treatment options explored, and maybe some breakthroughs will finally happen for those of us living with these complex, rare diseases that right now are understood by so few!

We are hopeful that this could be an incredible crossroads for mast cell research and be one more step in the right direction toward better diagnostics, more effective treatment options, and someday, one day, a cure!

In the meantime, though, there's plenty to keep all of us busy...while this is exciting news and a chance for renewed hope, it doesn't solve any of our current challenges in living with mast cell disease. My gut is still not absorbing, my neurological symptoms have not disappeared, and I'm still fighting my mast cells every day. So in the meantime, we have some new options to consider and decide what the best plan of action is at the moment. 

Like I mentioned in my previous post about the end of my GI testing (you can read that here), we are at a tough point. With my GI tract not wanting to play nice, we are forced to get a bit creative in terms of treatment. Unfortunately, until we get my GI symptoms under control, trialing any new oral medications is just not an option. Which puts us in a tough spot of having to decide what to do to get me more stable. While I am using IV meds on a daily basis through Mort (my port), the meds I am using currently are only rescue medications and not regular everyday management medications (despite me needing them daily right now...). We have been doing everything we can to sort out my GI issues, because we do not want to start any long term IV medications for everyday management. While I am living with a needle in my chest 24/7 right now, this is not something we hope to have to deal with long term. Thankfully, after my appointment on Monday, we came up with a few more options to try!

As we have exhausted all possible explanations for my GI issues (although there is potentially an update here as well!?!?), we are realizing that my symptoms appear to be mast cell driven. Meaning nothing will change until we can get my mast cell more stabilized and we can't keep waiting and hoping things will just resolve on their own. And since those very symptoms make adding new oral meds not a good option, we are faced with having to get a bit creative.

We'll be significantly increasing my dosage for a couple of the oral meds that I am on to see if by adding in higher amounts of the meds, we can increase even the small amount of absorption I am currently having. We will be trying to switch formulations for a few of the meds to see if that may impact how well I absorb things. We are also changing some of my meds from just as needed to regularly scheduled use. The meds are doing this with are localized medications but have also been reported to have a systemic benefit. Meaning that even though one may be targeting mast cells in the lungs and the other in the eyes, some patients have still seen improvement in other areas when taking these meds. Right now any options that bypass the gut are high on our list of things to try, so we'll be seeing if I can get a systemic benefit from some of these meds, too.

We also discussed (at length...) my Xolair situation. It's a bit tricky. If you read my previous post, then you saw I both passed out in my doctor's office AND went into anaphylaxis (warranting 2 separate ER trips) within 8 hours of my injection last week. This is not the first time I have had a severe reaction or anaphylaxis within 24 hours of getting Xolair which is one of the black box warnings for these injections. HOWEVER, I've also managed to have an anaphylactic reaction to absolutely nothing while sitting in my room at home without having had anything to eat or drink or done anything that could have possibly triggered a reaction...so it's not really clear cut if I have started reacting to the Xolair...We know that when I started it over a year ago, it made a HUGE difference for me. I was able to begin eating again without reacting severely to digestion and that benefit has continued. Even though my gut is not playing nice in many ways, I am still able to eat some solid food on good days without risking anaphylaxis. I could possibly lose this again if we stop Xolair. We just don't know. And unfortunately, no really does. Dr. Afrin's protocol is usually to stop Xolair after the first anaphylactic reaction, but yet again, I seem to have found a gray area in an already not well understood rare disease.

We have decided to give it a few more months. We are going to be a bit more aggressive with pre-treating prior to the injection AND for the first 24-48 hours after I get it with the hope that being a bit more pro-active in preventing issues may help avoid any potential reactions to the injections. We'll be adding in a few new meds during that 48 hour window and...just see what happens...Always said I'd love to donate my body to research, but somehow I didn't think that meant while I was still alive...

To be honest, I'm very torn about this. There is a part of me that wants to run in the opposite direction of any potential anaphylaxis trigger...who wouldn't want to do everything possible to avoid that?? But at the same time, I know how much benefit we have seen from the Xolair...that we KNOW is from the Xolair...I desperately don't want to lose what has been one of the most effective treatments for me! Since we're not 100% sure the reactions are directly caused by the injections...we keep going, keep trying, and I keep pushing the fear from my hands into God's and trusting Him since I definitely don't have any control over this...

Photo on the art museum in Minneapolis. Somehow
I don't think it's the same type of POTS... =)
Please keep us in your prayers for these next few weeks as we attempt to put together a plan for the next few months. Our trip to MN was good to get some new suggestions and discuss options which I will be discussing more with my my local allergist and GI at my appointments later this month. Like I alluded to earlier in this post, we also have a new potential direction to head with my GI issues that we are praying may be the breakthrough we have been looking for. We still need to go ahead with this new treatment plan for mast cell issues since any testing and treatment would be a potentially lengthy process, but we have another small glimmer of hope that we are pursuing for one more chance that there is something we might actually be able to address in my GI tract. Please pray for ease of scheduling and discernment for my doctors as they review my testing and history and help decide on the best plan of action for me and my nasty masties. And as always, pray for smiles, joy, and laughter and that we are able to glorify God no matter what my mast cells may be doing. 

Wednesday, June 7, 2017

God Uses Our Weaknesses, Not Our Strengths

One of the themes that always seems to appear in the Bible is that God doesn't use people's strengths. He uses their weaknesses so that He is glorified when things turn out incredible moments. At first glance, this seems backwards...If God created us, why doesn't He use the very strengths He has given us?

Because if He only used our strengths, then it would be so easy to think we are doing everything in our own power. But when instead, He uses the very areas He knows are a challenge, then we have no choice but to rely on Him for the strength we are lacking. And in that, God is glorified. 

In my mind, sunsets, sunrises, and the beauty of nature are 
some of the best displays of God's glory.
I had a very rough day yesterday. It started with my regular Xolair injection at 8 am. At 8:30 am, I passed out as I was getting ready to leave my doctor's office. That prompted ER trip #1 for the day. After spending a few hours in the ER, I was able to come home about noon. Only a couple hours later, I was starting to have symptoms of an on coming mast cell reaction. Not long after, Leni alerted which meant this was potentially a severe one. A double dose of IV rescue meds wasn't able to control my reaction, so when my throat began swelling and I couldn't swallow, my anaphylaxis and epipen free streak was broken. Ambulance called, ER trip #2, and the comment that prompted this blog post.

One of the nurses in ER #2 was training another nurse, who made the comment that got me thinking. It was when she heard that I can go into anaphylaxis from unknown triggers and have used over 25 epipens just this calendar year. She made a comment that it must be terrifying to live with mast cell disease and never knowing when I could possibly have an anaphylactic reaction to nothing. I just kinda laughed it off like I usually do and said that you just learn to live with it, but it got me reflecting a bit.

If I stop to think about it, yes, it is terrifying. Throat swelling, not being able to swallow, passing out, etc. can all be signs that the body is in shock from whatever triggered the allergic reaction. Anaphylaxis is life threatening. Every day I live with this reality and knowing that absolutely nothing could send my body into a severe reaction. Even with as many times I've gone into anaphylaxis, it never gets easier. You just don't get used to choking on your own saliva because you can't swallow or realizing every breath is getting more difficult because your throat is swelling or the feeling of the world fading out when you're about to pass out and wondering if the epi will kick in in time to keep you conscious. It is terrifying every. single. time. 

Yet I don't live in fear. Which doesn't make sense unless you read the first few paragraphs of how God doesn't use our strengths but our weaknesses. But I'll be honest, I almost couldn't believe it myself when I realized that I really am not stuck in fear constantly. With anaphylaxis involved, I certainly feel like I should have every reason to be!

Something that not a lot of people know is that I struggled with anxiety when I was younger. And not just the getting nervous for an exam anxious or worrying that everything has been turned off before leaving on vacation anxious. Paralyzing fear that would stop me in my tracks, left me in tears almost every morning, and consumed my thoughts almost every moment of every day. Most of that fear was not actually based on anything that was a legit reason to be anxious, but during that time, it certainly felt real. And now I'm faced with a reality that is a very real and legit reason to be scared, and I'm not...how does that work??

I wish I had some WOW moment where all of a sudden I was no longer anxious. It certainly would have been so much easier! But there was no aha moment where all of a sudden God took my fear and replaced it with peace. It was a years long process of challenging me, shaping me, teaching me to trust, and God not letting go when I tried to push away. And through those years, I found myself in positions where I truly had a reason to be afraid, but every time, God met me in my struggle and replaced that fear with peace. And in those moments where I was literally facing my worst case scenarios, I learned that God is always true to His Word to never leave us to walk alone.

In 2009, I was studying abroad in Costa Rica when my school group got stranded on a mountain after a 6.1 earthquake hit with the epicenter only a mile from where we were. Not only did I have a severe asthma attack from the smoke from fires that were lit for warmth, but we were on the top of a mountain with no access to medical assistance for close to 24 hrs. I was eventually airlifted out by helicopter and taken to a mobile Red Cross relief center somewhere outside the immediate earthquake damage zone. And that's when the reality hit...I was alone, in a foreign country, I had to work to understand people and communicate when I had full mental capacity let alone after a long night of struggling to breath and running a fever, I had no identification or money, had no idea where I had been taken, and had no way of contacting my professor, other students, or my host family...oh, and it was only my 3rd day in the country...Yet in the midst of the chaos and confusion, I had an incredible peace knowing that God was in control. Worrying or panicking was not going to change anything about my circumstances, but trusting God would keep my eyes, ears, and heart open for Him to move. Plus it was an incredible reminder of God's sovereignty when we later found out that had we not been delayed earlier in the day, we very likely would have been further down the trail with another group of tourists when the earthquake hit. That group along with their guide were killed by falling rocks during the earthquake. 

And now with my on going medical issues and challenges, I feel like if I ever had the right to be fearful I certainly do now! Yet again, while I absolutely have moments of fear and get nervous or worry about upcoming appointments or procedures, I am able to live free from the gripping control fear used to have on my life. It's not because I try to convince myself that things aren't as rough as they are, and it's not because I've accepted the fear as inevitable and just accepted that this is how things will be for the rest of my life. It's because I have learned that I really don't have control of anything in my life...including my mast cells...Giving up control of something we never really had control over seems like a no brainer, but I'm willing to be that we are all holding onto things we feel we might have control over...even though we actually don't. 

That was something that God began teaching me in Costa Rica and has continued to mold and shape my life through that new mindset. As I continue living with a condition that in the world's perspective gives me every right to be fearful and anxious, God has granted me glimpses of how He is being glorified as I am learning to trust Him more fully.

Who would have picked Moses, a man with a speech impediment, to be the spokesperson for an entire nation? Who would have chosen Ehud, a disabled man with only one working arm, to conquer an enemy ruler? Who would have chosen Esther, a Jewish girl with no influence, to save the Jewish people from being killed? Who would have chosen Peter, a man who despite having walked this earth with Jesus denied him 3 times, to be such an outspoken advocate for Jesus being the Messiah that he would stand up to powerful rulers and eventually become a martyr? Who would have chosen Mary, a teenager from a poor family who was already engaged, to be the mother of Jesus in a culture where she very easily could have been killed? Who would have chosen Gideon, a fearful man who tried to stay away from the action, to lead an army of only 300 men against an army of 10,000?

God did. And now I have a front row seat to see how God is taking the fearful, anxious teenager that I was and becoming my strength in the midst of this life with a chronic, rare disease causing repeated episodes of anaphylaxis and on going medical challenges for the very same reason. Because when He picks those who can't, it only highlights all the more that He can.

No one that God chose to use in the Bible came through their challenges unchanged. I am being changed every day and continuing to learn new and deeper ways to trust God who faithfully proves Himself trustworthy over and over. I am not that anxious and fearful high school student spending every morning in tears due to paralyzing fear and completely consumed by my thoughts and what ifs. I am a daughter of God who is learning to let go of the control I like to pretend that I have and to trust in the only One I know never changes and truly does have control over everything.

I share my story on this blog for a couple of reasons. One is to keep family and friends up date on what's going on and ways you can be praying for us. Another is to help spread awareness for mast cell disease and all the fun that can bring along with it. But it's also to share God's role in all this. I may be writing these blog posts, but God is the One writing my story. As He uses me, very much weak in the struggles I had with fear and anxiety, to walk the unknown road of life with a rare disease, He is being glorified. Every procedure I leave in His hands, every tough treatment decision we pray for wisdom in making, every Epipen and ambulance ride and hospital admission, God is glorified in our smiles, our hope, and our trust in Him. THAT is why I share my story. 

We smile every day because God is good, even when our circumstances aren't. We have hope because we know that one day I WILL be healed if not in this life, then in heaven. And we trust God because despite our attempts to convince ourselves otherwise, we really don't have control over anything. But God does. And every moment we can lean into Him when life gets tough, He is glorified. 

I never thought I would be free from anxiety and fear. I spent many nights crying out to God in desperation feeling as though I was crumbling under the weight of my fear. And now, 10 years later, I live with the reality of an unpredictable rare disease, repeated bouts of life threatening anaphylaxis, tough decisions with no right answer but many wrong ones, and an unknown long term prognosis. I can stand here (technically sitting since standing too often results with passing out...) and say in complete honesty that I do not live in fear, even with having every reason to do so, and it is because I am learning to give up control of things I never had control of and learning to lean into God who is in control of everything. And in that God is glorified. 

God doesn't choose the strong because they have no need for strength. They already have it. God chooses the weak, because we have no choice but to rely on Him. As we are made strong in our weaknesses, we are able to do and be things we never thought were possible. And as the world looks on and watches, they don't see our strength on display. They see our weaknesses being held up by God.