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.

Monday, June 5, 2017

GI Testing Update

Now that May is over, we have finished all of the GI testing that had been scheduled and are sorting through the results. And in true mast cell fashion, things are much less straight forward than they could be.

Cute photo of Leni just because she's way more fun to talk
about than GI testing fails =)
The first test results we got back were blood work and stool samples. I think I shared the results of these a few posts ago, but basically there was nothing earth shattering that appeared with these tests. My blood work showed slightly low potassium levels which has been the case for a few months now, but nothing else too serious. The stool samples were able to rule out any bacterial infection or parasite and to confirm that I was loosing large amounts of electrolytes in my stool as well as not absorbing food properly. So no new information from these...apparently there are typically 2 main causes of chronic intestinal hyperactivity. One is due to structural issues or primary diseases such as Crohn's and the other is due to secretory issues where the intestinal activity is due to the overproduction of signals to the intestines causing them to continually be active. Like I said earlier, in true mast cell fashion, my testing falls squarely in the middle of these two mechanisms not really providing any indication of which is more likely. Some of my test results point to one mechanism and other results point toward the other...thanks, mast cells...thanks...

The next test results to come back were from a breath test that was looking for small intestine bacterial overgrowth. This tests for the activation of H2 and CH4 in the intestines after stimulating them with a high sugar drink to see if there is an accumulation of bad bacteria that may be causing chronic intestinal issues. Other than having a minor reaction to the coloring dye in the drink and getting extremely nausea before the end of the test, this was relatively okay as testing goes. During the test, the nurse told me what values we were looking for to qualify for a positive test. Despite having fairly high baseline values, it was clear that I was not going to hit the mark to have a positive SIBO test. At least we figured one test was definitive...until a couple weeks later when the test was "interpreted" and became just as ambiguous as the others...Turns out there are values that are considered positive even at baseline without requiring an increase after drinking. Both of my baseline values were just over that line...but after drinking dropped to non-diagnostic levels...so now what do we make of that??

The oh-so-wonderfully artificially flavored drink to test for SIBO
Last round of testing was the colonoscopy that I had done a couple weeks ago. We ended up adding on an endoscopy at the last minute thanks to some intestinal bleeding I had the week before the scope. At least this time it happened BEFORE the testing instead of immediately after. Both scopes were relatively unremarkable. I had some chronic irritation in my stomach that was an atypical (of course) pattern for gastritis and some acute and chronic irritation in my colon which was again, atypical for normal colitis. Thankfully, my small intestines looked relatively okay. My GI felt that the acute inflammation in my colon may have been responsible for the bleeding I had the week before the scopes, since he did not see any other areas that could have explained the bleeding. Thankfully, after stopping all oral intake and relying solely on my elemental tube feeds for a week, I have had no more episodes of bleeding so we're hoping it was just acute irritation.

I spoke to my GI on the phone about a week and a half after the scopes once the biopsy results had come back. The biopsies didn't show anything that he hadn't seen during the scopes, but we were able to rule out Crohn's (for about the 3rd time) and Celiac (#7 or 8) again and confirm there were no other infections or disease processes. Basically we have not much more info than we did before the testing, but we have more definitive answers on what is NOT going on, so I guess that is good at least. My symptoms are pointing more toward a secretory mechanism for my gut hyperactivity despite not having tests results showing that definitively. We have pretty much exhausted all the possible structural issues or primary GI diseases, so we're down to secretory problems and just mast cell driven GI issues.

Older photo, but still so familiar...
To be honest, this is not the news we were hoping for. Despite negative test results usually being a good thing, when something is very obviously wrong and the tests don't give any answers, it is very frustrating. We can't do anything to treat or manage these symptoms when we don't even know what is causing them or the underlying mechanisms. Since this flare started in November, we have been unable to try any new mast cell medications because my gut is not absorbing anything properly. The oral meds that I do take are not being absorbed and pass through undigested. Any solid food that I eat also comes out undigested even with high doses of pancreatic enzymes that I take to help my body break things down. Even my tube feeds are not being absorbed properly and we have increased them by 150% to hopefully prevent more weight loss and get as much in me as possible. Until we are able to get my GI symptoms under control, we are unable to trial any new mast cell medications orally. And that is where we are stuck...

The past 6 months have been a holding pattern of hoping we'll get a breakthrough with my gut issues so we can start trying new mast cell medications. At this point, I have tried many of the oral mast cell medications already. There are a few left to try, but doing so with my intestines not absorbing would be pointless. We won't know if it's the medication actually not working or my body not even absorbing it if we add anything new in right now. We don't want to start any IV treatment options because they are VERY difficult to get off of once they are started. So we've been waiting for all this GI testing and hoping we would get some answers. But now that we're on the other side of all this testing with not much to go on, we're looking at the reality that my symptoms could be entirely mast cell driven and only presenting in the gut but not actually an intestinal problem.

With that being the case, we are running out of options. We may be forced to try some IV therapies to get my body more stable. Once my GI tract has calmed down, we can add in some oral mast cell medications, and then hope I am able to transition back off of the IV treatment. Not our first choice by any stretch, but at what point do we no longer have a choice? Obviously we want to get me more medically stable, but at what cost? When do we decide to risk potentially being stuck on IV medications long term in an attempt to prevent long term damage from relentless symptoms? I think these questions are some of the most difficult to answer when living with a chronic, rare disease.

I'm heading out to MN again next week to follow-up with my mast cell specialist and this will be one of the big things we will be discussing. I see my GI again a few weeks later and my allergist also before the end of June, so hopefully we'll have a plan of action in the coming weeks. Maybe it's not what we want or hope, but even still, I am so incredibly thankful to have a team of doctors willing to help figure things out. It still wasn't that long ago when I was still fighting to be heard in the medical community that something was actually wrong. Two and a half years after being diagnosed with mast cell disease and I am so thankful to have a local allergist, mast cell specialist, neurologist, endocrinologist, GI surgeon, and 2 other GI doctors all working to help me figure things out. Too bad that sometimes there just isn't anything anyone can do...

Tuesday, May 30, 2017

Moving with a Rare Disease

Since we are now moved in to our new house (?!?!?!), I thought I would share a bit of what it was like to be house hunting with mast cell disease and some of the additional considerations we had to make as well as some of the extra moving-related checklist items that come up when moving to a new area with a rare disease. If you missed my previous post about the relocation of Bokatopia, you can read about it here.

The first thing I should mention is that I did not actually go in most of the houses we looked at. Out of the dozens of homes we saw, I think I actually entered 3 houses. And when I did enter, I did it with my mask on. We chose to do things this way mostly because I am incredibly sensitive to scents. During the house hunting process, possible scents include paint/stain from updates done to the home, mold if that is an issue, air freshener is often used when the home is still being lived in to mask any potential smells, and/or candles again used to make the house smell fresh. All of which have the potential to cause a major reaction. If Nick went through the house and could immediately tell it wasn't the right home for us, there was no need for me to risk being exposed to a trigger. When we found a house we thought was a good possibility, I would go in with my mask on so I could see it AND so if I did start reacting, we would know before we actually bought the house. At some point we had to risk a reaction to see whether or not I could actually live in the house! Thankfully, we were able to successfully complete the house hunting process with no reactions!

As we began our search, there were a few things we specifically looked for in a house because of my mast cell disease. The most important thing was to have full living space on the first level. We looked at many ranch homes because of this need. Because of my passing out and balance/coordination issues, stairs can be a hazard on bad days (and some good days too...=P). We needed a house that had a bedroom, full bathroom, kitchen, living space, and exterior door on the main level. Everything else could be upstairs, basement, or same level but we needed me to be able to stay on a single level if needed. Another must-have for us was a fenced yard for the dogs that could be accessed from the main level. On rough days, I am not always able to get around easily. Having a way to let the dogs out even when I can't walk them was a must. While we initially were focusing mostly on ranch homes like I said earlier, we ended up with a colonial that had a bedroom addition added to the first floor which works great for our needs. There is a yard that is partially fenced that we will be finishing to give Bonk and Leni space to run around like the crazy dogs they are.

I jokingly mentioned in the first post that our search criteria was 10 minutes to Nick's work, church, and the ER. While in passing it's fun to laugh about, that WAS a factor in our decision process. In addition, we also knew there were certain ERs and hospital systems that we needed to avoid even if they were within our 10 minute radius for Nick's work and church. We purposefully avoided a few areas that might have otherwise been fine, except we knew the EMT's first choice of ER (which would be the default if I was unable to communicate or unstable medically) was one that was not able to handle my mast cell disease. Thankfully, we were able to find a house not only within 7 minutes of an ER in the right hospital system, but it happens to be one that we have been to many times (and is one of our favorites!!). They know me (and Leni), are familiar with my mast cell disease, and know I have specific protocols. This ER also admits to our preferred hospital where the majority of my doctors have privleges and is less than 5 minutes from Nick's work for when my mast cells decide not to play nice during the workday.

Once we moved passed the hunting phase and have actually been preparing to move, there are a few things I have on my to-do list for just before moving or immediately after. The most important of these is visiting the local EMS. Any time I move to a new area, I make sure I know where the closest 2 EMS station are located. The reason I look for the closest 2 stations is because if something were to happen on a day when all the teams have already been dispatched from the closest location, they often call the next closest for assistance. I try to schedule a time to speak with whoever is in charge of the squads so I can bring them up to speed on my mast cell disease and other relevant info. I don't focus too much on the specifics of my treatment protocols, because you never know who is on call, what they'll remember weeks later, etc. What I do focus on is making sure they are aware that I have a rare disease. Usually they can flag an address in their system. For us, we make sure they know the code to the side door so they can come in if I'm unable to get to the door. I make sure they are aware I have all my medical information and protocols hanging next to the door. I bring them up to speed on service dog laws if they are not familiar and request they bring Leni along in the ambulance if at all possible. Thankfully it won't be an issue here, but in other places I've lived I have made sure they know my preferred hospital system and which ER I want to be taken to. Details like this (which are also spelled out on the medical information sheets located by the door) can be incredibly important to discuss ahead of time because in the moment, especially if I'm unable to communicate, things get missed.

I also speak with the power/utilities company and water if that is separate. Because I rely on a feeding tube for the majority of my nutrition, do IV fluids by a pump at home to maintain my electrolytes and hydration, and have medications that need a temperature controlled environment, power outages can be quite serious. A few hours without power is not a huge issue, but if it is a widespread outage, sometimes restoration can take days. That is when things can be more of an issue. All power/utility companies have something of a priority list for when widespread outages occur. This list typically includes people who rely on medical devices that need charged or actually plugged in to be used. The neighborhoods and areas where people with this type of equipment live can be prioritized to help ensure they are not without medical equipment for too long. Again, for us it's not an immediate crisis if the power were to go out, but with an extended outage things could become an issue. Worst case scenario, we stay with my in-laws for a few days, but often by simply alerting the local power/utility company, this can be a relatively simple issue to resolve.

Another miscellaneous thing that I have to do any time we move to a new house is re-train Leni. No, she doesn't immediately forget all her training just because we moved, but she does get conditioned to where my phone usually sits, where I keep my EpiPens, how to open the fridge, etc. When we move, all her familiarity with those tasks is gone. My phone will have a new spot it is most likely to be in, my EpiPens will find a new home, the fridge is a different style for her to open...things like this that are important tasks will need some proofing and possible re-training to keep her sharp and quick in her tasking.

Then of course, there are the small, but oh-so-important things like making sure I have a clear path from the bed to the bathroom in the event of cranky mast cells in the middle of the night (not that this EVER happens...=P). Making sure we have space allocated in the refrigerator for my IV fluids and Leni-accessible water bottles. Finding an outdoor water-tight bin for medical deliveries in the rain to protect my supplies. Figuring out what storage arrangement will work in the new house for all my medical supplies to keep them easily accessible when I need them but not a mess in the middle of everything. There's always something!

We are very excited (and a bit overwhelmed =P) to be moved! Thank you for keeping us in your prayers these past few weeks! We are excited for this big step in our "adulting" abilities and next season of life for Nick and me.


Thursday, May 18, 2017

And a Big THANK YOU Goes to...

You know those moments when someone goes above and beyond to make you feel comfortable or welcome? And you just want to thank them but somehow words can't possibly communicate how appreciative you are for what they said or did for you? Well, I'm going to attempt to put one of these moments into words...but I hope that regardless of how well my words communicate one of these moments, that my gratitude truly comes across.

One of the things about living with mast cell disease is that every time I leave the house, I risk being exposed to a trigger that could causes a major reaction. (Now technically I can still go into anaphylaxis just sitting at home, but not focusing on that right now =P) Sometimes we decide to risk that with some careful consideration, other days it's not an option. There are some places that are "safer" than others, and it is always nice to know that we have those and be able to relax at least a bit.

I'm going to share one of those places with you and hope that you will help me truly thank the owner by frequenting this incredible local business. And since many of you know and love this place already, that shouldn't be too difficult =)

Corner Cup Coffeehouse in Stow...THANK YOU for how welcoming and accommodating you have been for Leni and me since we first walked in almost a year ago I think. Heather does not know I am writing this post, but she truly has gone above and beyond in not just accommodating my allergies but making me feel welcomed. And not just Heather, but her entire staff which is really even more reflection back on how Heather is setting the environment for everyone working at Corner Cup.

The first day I went to Corner Cup, I ordered a drink made with almond milk, only to have to ask it to be remade because it was mixed with a spoon that had just been used in a milk based drink. Not only was my drink remade quickly (and with a smile), but they asked about my allergies so they would be aware in the future. I appreciated their questions, but figured I would have to repeat myself again every time I ordered a drink. Far from that being the case, the next time I entered Corner Cup, even with different staff behind the counter, they knew what to do to prevent cross contamination. Granted, having a service dog makes me a bit easier to remember, but this was still beyond the job description of a coffee shop barista.

Months later, I walked in just after the pumpkin spice latte had returned for the fall season. That used to be one of my favorite drinks before I developed all my allergies, and I thought I would at least ask if the seasonal syrup was safe. The staff not only knew what I was asking immediately, but they had already read the bottles and had an answer for me. One of the baristas had made drinks for me many times and said she read the bottle when it had arrived because she was curious to know if there was milk in the flavoring. While I was disappointed to find out the syrup was not safe, I was very impressed with the staff again going above and beyond. These little things seem so insignificant to many people, but to someone with severe allergies and extreme dietary limitations, these minor details make a huge impact.

Just this past week, I had another experience at Corner Cup that prompted this post. My mom was in town, and we had decided to make a stop to get drinks before running some errands. Not long after sitting down, my mom heard a couple other customers talking about essential oils. If you remember, a few months ago, I went into anaphylaxis in a medical office building thanks to some diffused oils (you can read that post here). Heather had read my blog post and shared that made her aware of how airborne fumes can impact not just me, but many people with varying degrees of sensitivity. Not long after, a lady walked in with a bag of essential oils prepared to do a demo for the ladies we had heard talking about them. Unfortunately, this meant that my mom and I had to leave immediately as my throat began itching, I was developing hives, and my lips were tingling.

Leni and I got to the car so I could take my IV meds, but my mom hung back to speak with Heather and the lady with the oils for a few minutes. Now, please understand that I was in no way upset at all. I know that whenever I go out in public, I risk being exposed to a major trigger and I am prepared for those occurrences. It was a bummer that we had to leave in a hurry, but that's just my reality right now. My mom made sure both Corner Cup and the ladies with the oils understood that we were not upset but that we could not stay if they were going to do an essential oils demo. We left to run our errands and didn't give it another thought. At least not until the next evening...

The next day, I received a message from Heather that prompted this post to share how much I appreciated both her message and efforts to keep Corner Cup a safe place for me to visit.

Heather's efforts to find a way to allow people to meet at her coffee shop to discuss and trial essential oils while still trying to keep it a safe place for me to visit goes above and beyond anything a small business owner should be doing. In her message, she explained her new protocol for Corner Cup regarding essential oils and how she is going to manage these events in the future. She took the time to apologize for us having to leave so quickly the day before (which was not at all something she needed to apologize for) and shared how she was going to ensure there were no impromptu essential oils meet ups but planned in advance to allow people (mostly me) who were sensitive to avoid those times.

Remember that I am ONE CUSTOMER. Now I know there may be other people who are sensitive to essential oils or have allergies, but still. Heather and her staff have gone above and beyond to make me feel welcomed and safe at Corner Cup which, again, is a testament to the quality of the staff who work there. A few days later, Nick and I stopped by to pick up some drinks on our way to his parents. I stayed in the car and Nick went in to order. Even without me in the store, they recognized Nick and made sure to make my drink in a way that avoided any possible cross contamination.

If I haven't said it enough in this post, I'll say it again. THANK YOU Heather and the entire Corner Cup staff. You have truly gone above and beyond, and while it might not seem like much to you, it means a lot to me and my family. Thank you.

Leni says THANK YOU to everyone at Corner Cup!