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!

Monday, May 8, 2017

Bokatopia is Relocating!!!

I'm going to take a break from regular scheduled programming to share some super exciting news. In less than a month, Bokatopia will be relocating!

Sorry VA friends, not going that far! But we are preparing to move in less than a month! Nick and I have been married about 2.5 years. We initially lived in an apartment in Tallmadge. We really enjoyed it and loved the town, but after a little over a year it became hard to afford our rent due to rapidly increasing medical expenses thanks to aging off my parent's health insurance policy when I turned 26. In Feb 2016 we moved in with friends who have very graciously adopted us as their 4th and 5th children and allowed us to live in their upstairs.

We have loved living with our friends, but as my health has continued to be a challenge, we have felt the need to be close to Nick's work, church, and the doctors I see most often. A few months ago we began house hunting with my parents with the intention of finding a house that Nick and I can live in that also has space for parents when they come out for extended periods of time with their dogs. Our goal was within 10-15 minutes of Nick's work, the ER (yes, this was important for us =P), and church. After weeks of searching and dealing with the current, crazy housing market, we put in an offer on a house in Cuyahoga Falls that was accepted. The home inspection was completed a few weeks ago, and we are starting to prepare for moving!

We are very excited about this next stage in our married life! Nick is looking forward to having a much shorter commute, I'm looking forward to having all our main living space on a single level, the dogs are excited to have a yard of their own, my parents are looking forward to being able to come out easier with their dogs to visit, Nick's parents are glad we'll be closer...it's a win all around (except for our adoptive parents who we will miss seeing every day...)!

We are also very much looking forward to being close to church so on days I'm unable to drive and Nick is serving, I still have the option of coming because we are close enough for Nick to swing home to pick me up in between services. We have missed being as involved with our church family these past few months and hope that being so much closer will make it much easier to attend events even if we can't always stay the entire time.

Even in just the short time we have been married, Nick and I have been extraordinarily blessed by friends and family who have stepped up to help us out as my mast cells have been doing their best to cause as much craziness as possible in my body. God has not failed to provide for us even in times when we truly did not see how things would work out. We can both say that our married life is not anything we could have ever imagined. But we can also say that it has been and continues to be an incredible adventure that we wouldn't want to be doing with anyone else.

And now we are on to our next adventure! Please keep us in your prayers these next few weeks. Life does not stop just because we are preparing to move...I still have multiple appointments and Xolair and testing. Nick is still working full time, and we're both managing my mast cell disease on the side and in the most inconvenient moments when my body decides not to play nice...

Please keep us in your prayers these next few weeks! Moving is stressful at the best of times, and we are working around my not-so-cooperative mast cells. I am unable to be in the house while Nick is finishing up some of the small things that need taken care of before we move, I'm not able to help with the actual moving process very much, and anything that is out of our routine has the potential to trigger reactions for me. We are excited for the long-term benefits this house will provide for us, but initially it will be a challenge. Please pray that we are able to get done all the small projects easily without any complications. Pray for a smooth moving day and that things go well and we have good weather. And please pray I don't lose any essential medications in the moving process...always a good thing to pray for =)

Thank you for always being on this crazy journey of life with us through prayer and support. We are excited for this next season of life, and we are glad to share that with you!


Thursday, May 4, 2017

When Did May Happen???

Um, did someone see April? And I'm not talking about the giraffe...I think I missed it...I cannot believe it is already May...when did that happen???

May is always a busy month for our family. Mostly because my mom gets some hard core celebrations going on and the rest of us go bankrupt. Not only does my mom get to celebrate Mother's Day this month, but it also happens to be her birthday month AND anniversary month. While most years I think I can safely say I am much more prepared going into this month of motherly celebration than my brother, I may have to admit that this year he will walk away with the favorite child of the year award...I won't go into much more detail since my mom is usually the first person to read these blog posts, but I will say that even though I'm not usually one to concede defeat, I do know when I've lost a fair fight. And I suppose it will be good for Devon to get at least one year of being the favorite =P

Aside from the favorite child of the year competition and celebrating my mom, I've got some GI testing, appointments, and scheduling coming up in the next few weeks. Plus some exciting news that I will share in a later post, so stay tuned for a major Bokatopia update!

I am *hopefully* getting my feeding tubes changed sometime next week. This is not at all related to the issues I had with Sherman (J tube) a couple of weeks ago, but just normal feeding tube maintenance. This will be my first tube change since having my GJ tube split into separate G and J tubes. Despite the issue with Sherman trying to escape my intestines, I really have preferred having the 2 tubes vs. the 2-in-1 tube. My G tube drains more easily since there is not a smaller tube running down the inside and my J tube can't kink anymore, both have been incredible benefits. Actually, the best part of having the separate tubes will be realized very soon when I get them changed. Because of my anatomy, when I had the GJ tube, I had to have it changed during an endoscopy so they could get the J extension into my intestines and stitch it into place. Since me and anesthesia don't get along so well, these procedures were always a source of stress both for me being nervous for the procedure and anaphylaxis stress on my body from the medications. Having the separate tubes means tube changes can be done MUCH more easily and without having to use any sedation or anesthesia!

Ironically, despite no longer needing to be put under for tube changes, I will need anesthesia next week for a colonoscopy...so not fully escaping it yet...but hopefully this will be the last time for a while! (I know I said that last time, then I ended up having J surgery less than a month later, but this time for real!) Since our first 2 rounds of GI testing have not really given us any information we didn't already know about my cranky insides, I am having a colonoscopy next week. I've had enough endoscopies that my doctor felt that we didn't need to re-do that, so just a lower scope. It's been about 10 years since my last colonoscopy so even though we're not really sure we'll get any answers from it, we all feel that it needs done. Actually one of the reasons it has been so long since my last lower scope is because up until recently, I could not get my intestines to move without prescription motility medications. Even with those, it was a challenge at times to keep things from causing partial obstructions in my bowels. My doctors were concerned that even with the typical colonoscopy prep I wouldn't be able to get empty enough for the procedure...so we held off. Since November, however, my intestines have all of a sudden gone into hyperdrive. Not fun symptoms to have been dealing with for going on 6 months now, but at least it will be easy to get adequately cleaned out for the scope...small blessings =P

After the colonoscopy I'll be following up with my GI to determine what (if anything) came up during the scope and where to go from here. We're running out of things to test and potential causes for my intestinal issues at this point, so please be praying for discernment for my doctors to know what they are looking at and what to do and wisdom for us as we have to make treatment and testing decisions that may not be what we were hoping.

The next possible appointment is with my neurologist. When I was in the hospital with my Sherman issues, I had an EEG come back abnormal and a brain MRI that was flagged as inconclusive. I was told to follow-up with my neurologist, so I took the disk and report for her to review and decide if she needed to see me before my scheduled appointment in July. Hopefully this is nothing and she will be able to tell it is relatively the same as my MRI from last September. If not, then I'll be seeing her a lot sooner than I expected.

I am also working on scheduling with 2 new doctors this year. I know, I thought I was done with new doctors, too...apparently appointment scheduling for a rare disease patient is never done...One has actually already been scheduled. When I saw my allergist last week at my Xolair appointment we discussed where I felt like I'm at right now and my current symptoms. Despite now going on 45+ days epi-free he agreed that we have not really solved anything, just given me more tools to manage things until we get actual answers. Unfortunately, those answers are continuing to be very slow in coming...He recommended an autonomic neurologist that he shares a few patients with as someone who might be able to piece things together. I called to schedule with him and have an appointment on October 31. Don't worry, I'll remind you when it gets closer =P At least he is right here at the Clinic.

The other appointment is with a geneticist in Florida. I did see a geneticist here in Ohio a couple years ago, but considering she had not heard of mast cell diseases or POTS, I'm not confident that the appointment accomplished anything. I was given the name of this geneticist in FL by some friends who have had similar symptoms and diagnoses who felt like he was finally able to pull all the pieces of their puzzle together. Despite having a solid mast cell diagnosis and very much trusting Dr. Afrin, there are still outstanding symptoms that just don't quite seem to fit and keep reappearing and progressing. Even Dr. Afrin encourages his patients to make sure we're not missing something else simply because we have a mast cell diagnosis, so we are doing what we can to make sure we have covered everything that could possibly explain these odd symptoms that don't quite fit in anywhere right now. This appointment will hopefully be sometime in July, and we are still deciding whether to go in person or do a video consultation.

So that's what's coming up in the next few weeks. My updates have been less frequent lately mostly because not much is going on. We're in a holding pattern while we wait for scheduled testing, follow-up appointments, and scheduling with new doctors. There's just not much to report. I am thankfully 45+ days epi-free thanks to Mort and having IV benadryl, and almost 3 weeks ER-free thanks to IV zofran. We are able to manage things at home (sometimes more effectively than others), but not really making much progress in terms of resolving symptoms and getting me feeling better.

I have some days that are more good than bad. I have a lot of days that are rough with a few hours of relief sprinkled in. I have some days I'm barely able to walk down the stairs to let the dogs out. I spend many nights in the bathroom in pain and throwing up. I often use multiple doses of IV meds to get through the days and keep me from needing epi or the ER. I am sometimes able to drive myself to appointments and other times rely on friends and family. Some days I need a wheelchair just to get out of the house, other days I'm able to chase Leni in the yard for a few minutes. It's a lot of managing symptoms, seeing doctors who can hopefully help, and praying for strength in the meantime.

And in that meantime, we're still smiling! Our motto in Bokatopia is "Smile Every Day" and we do. Some days it's because things feel good and we're thankful, other days it's through tears and much tougher, but no matter the day, it's always because we have hope in Christ that we cling to even when life is tough. Even Leni gets in on the smiling!


Monday, April 24, 2017

From the Hospital to VA in 24 Hours

Last week was not the week we had expected...

Saturday Nick and I did some fun house hunting with my parents who came to Ohio for Easter weekend. Sunday morning I woke up with major pain in my stomach close to my J tube. I didn't actually think the pain was related to my tube since running my feeds was fine and didn't cause any additional discomfort, but we later found out that was not the case. I was determined to make it to church for Easter, though...It had been almost 2 months since I had made it to church thanks to my nasty masties and I REALLY, REALLY wanted to be there on Easter. 

Well, we made it to church...but I spent almost the entire service attempting to dislocate Nick's hand as I was clenching it as hard as I could. I couldn't sit up, couldn't take deep breaths, could barely speak, and sat listening to the sermon with tears streaming down my face because the pain was intensifying. Nick brought the car around and we left church to go straight to the ER.

Up until the ER, I wasn't really focused on how bad the pain was. I knew I was hurting, but I also knew that we had always been able to get my bad pain under control with some IV meds and we'd be heading home in a few hours. After a few hours in the ER, I had been given 4 rounds of my normal pain medication (which is a 1/4 dose of a standard adult dose) and had almost no relief. I think that is when it hit me that something might actually be wrong. They did a CT scan of my abdomen and found pretty significant swelling in my intestines around my J tube. I still wasn't convinced the pain was related to my tube, but they sent the scans to the GI team at the hospital who felt that the balloon holding my tube in place may have gotten dislodged. I was still in severe pain, so we tried a new medication (at an 1/8 of a normal adult dose) that thankfully took away the pain, but also came with the side effect of making me mostly unresponsive.

Apparently my tremors and unresponsiveness (both side effects of the pain med) made the EMTs who transported me from the ER to the hospital a bit uncomfortable so when we arrived, the admitting doctor immediately ordered an EEG and some anti-convulsive meds thinking I was having a seizure. Thankfully Nick was not far behind the ambulance and they were willing to hold off and listen to him tell them that as long as my vitals were stable, they didn't need to worry. That EEG would come back a few days later, but at the time, things were relatively controlled.

That all happened Sunday afternoon/evening. The next 2 days were filled with severe pain, tremors, high doses (for me) of pain meds, spending hours in somewhat of a drugged sleep to keep the pain at bay, and waiting for doctors to round to figure out what to do. The GI team and GI surgeon said that the CT showed that the balloon holding my tube in place had deflated and was stuck against my intestinal wall. Plan A was for them to try to reposition it in Interventional Radiology (IR). If that didn't work, then Plan B would be surgery to try to fix the problem. Unfortunately, this got held up an extra day as they were waiting on records from the Cleveland Clinic about my J tube procedure so if surgery was needed, they could be prepared with all my medication protocols. Glad they were making sure they were ready in case surgery was needed, but in the meantime I spent another day in some of the worst pain I've ever felt.

Wednesday morning was the day of the procedure, and it was a mess from the start. As a rare disease patient, I know it is my responsibility to be on top of my care even in the hospital and my family is very capable of managing that when I'm unable to. However, even the best laid plans and preparation can go awry when there are so many pieces out of our control.

The IR team called and said they would be coming to get me about 11:30 am. We had purposefully timed my morning pain meds and mast cell meds so that they would be in full force for the procedure to hopefully minimize any potential reaction. Except even though they brought me down about when they said, I waited over 3 hrs before they got to my procedure. That meant that my pain meds and mast cell meds had time to wear off before I even got to the procedure room...

And that's where the "fun" started...I was starting to shake from pain again, so the nurses in IR called my nurse on the floor to ask what pain meds I had been on. She told them what I had been taking for the past few days, but instead of them giving me one of the meds, they gave me both at once...I am EXTREMELY sensitive to pain medications (even when in severe pain), and both at once made me completely unresponsive. The nurses in IR were not prepared for that and kind of freaked out. They decided to give me another medication to reverse the effects of the pain meds, which woke me up, but also started causing a mast cell reaction...more benadryl on board and thankfully that was controlled. Then the real fun began...

Because they had reversed the effects of the pain meds, when the surgeon started the procedure the severe pain I had been dealing with the past few days just intensified. It was pain like I have never experienced...even compared to pain after my abdominal surgeries. They did start to give me more pain medications during the procedure, but nothing really kicked in until the surgeon was finished. Unfortunately, it also took a bit longer than expected because the displacement was worse than they had thought. Initially they thought the balloon holding my J tube in place had started to get imbedded in my intestinal wall. When the surgeon started the procedure, he discovered that the balloon had actually deflated enough to slip out of my intestines and was being to adhere itself to my abdominal wall while a small part of the balloon was still in my intestines. Thankfully he was still able to fix it in IR with no surgery needed, but it was an incredibly painful 30 minutes.

The CT scan after the procedure confirmed my tube was back in place, and thankfully the second round of pain meds finally started to kick in. Once the IR team felt comfortable that I was not going unresponsive again or still in severe pain, they sent me back to my room. Despite still being in pain from the procedure itself, I could tell very soon after that the problem had been resolved. The sharp, shooting pain that came with every deep breath, any amount of light touch, and any movement was gone and replaced with intense soreness like a badly pulled muscle. I cannot put into words the relief that my entire body felt when that pain was finally gone.

Wednesday after the procedure. First time since being admitted
I was actually able to sit up without severe pain.
Despite having been taken down to IR about 11:30 for a 30 minute procedure, it was almost 4 pm by the time I got back to my room. We had discussed leaving Wednesday night assuming the procedure went well, but decided to stay an additional night for pain management. Even though the pain for the most part was gone, I was terrified of it coming back in the middle of the night and not being able to do anything about it. It ended up being a non-issue because the doctor came in not much later to tell us the EEG had revealed some abnormal activity and that I would need a brain MRI so I'd have to stay one more night anyway.

I did need one more round of pain medications Wednesday night before bed, but thankfully was able to make it through the night without needing any more. Thursday we got the all clear from the GI team and neurology gave the okay for me to be discharged as long as I followed up with my neurologist. Escape the Room: Hospital Version = Complete April 2017!

To be honest, this hospital stay was very different from many of my previous admissions. Usually I end up being admitted for uncontrolled mast cell reactions. It's a matter of using IV meds to get things back under control, then sending me home to see how long I can make it until it happens again. Nothing really gets resolved, we just get me a bit more stable for a time. This time, we left actually having resolved the reason I had been admitted! Not always the case for rare disease patients or anyone with chronic illness, but we are thankful that this was something that could be fixed, and I could actually leave the hospital feeling "better."

Friday was spent resting and PACKING. Yes, I did just say packing. Less than a day after being discharged from the hospital, Nick and I road tripped down to VA for my college alumni soccer game and visit some close friends. No, I did not play in the game =P Herman, Sherman, and Mort kinda keep me from any contact sports for now...and my mast cells are doing there part to keep me from being able to run at all, so Nick and I just watched. But it was great to catch up with some people and have time to spend with our friends who graciously allowed us (and both dogs?!?!) to stay with them this weekend.

Sometimes I think our dogs are broken...
Initially I thought it would be tough to watch everyone I used to play with and be unable to play myself. Well, the 50 degree weather with pouring rain and wind kinda helped me not miss it *too* much! But it is hard sometimes when I'm reminded of how far I am from where I used to be and what I used to be able to do. I don't struggle a lot with comparing myself to other people and feeling discouraged about not measuring up, but I do struggle a lot with comparing me now to who I used to be and feeling discouraged about all the things I can no longer do. I miss being able to play soccer and swim and run. I miss being able to get up for 6 am lifting or swim practice, have a full day of classes, more practice in the afternoon, meals, go to FCA, do homework, then go to bed just to wake up to do it all over again. I miss life before my mast cells were in control.

But I am thankful for the memories. Even though some days they are more painful, I am thankful for the time I did have to do those things. In high school and college we always joked I was busy enough for 3 people. I was always a go-go-go type of person who liked to be rushing around and did well with little to no "free" time. Maybe those years of craziness were God's gift to me knowing that in a few years I'd be unable to do many of those things. Maybe He gave me the strength and energy to be able to even the crazy amount of activity I did for those years so that I'd have all those memories to sustain me when things would get tough.

Despite doing very little all weekend, by Sunday night, I was exhausted. I am glad we decided to go ahead with our trip to VA that we had been planning for a few months now, but especially after the week we had in the hospital last week, I was wiped. I know some people probably think we're crazy for taking a road trip less than 24 hrs after getting out of the hospital, but the reality is that if we waited for the perfect conditions and ideal health, we might be waiting forever and watching life pass us by. Living with a chronic illness means that I often choose to push myself knowing I may pay for it later so that we can have some moments of victory over my mast cells. Sometimes I may miscalculate and end up suffering major repercussions for those choices. But sometimes we're able to walk the fine line that lies between pushing things too far and not allowing my mast cells to completely dictate how I live. Nick and I managed to find that fine line this weekend and have a good weekend with each other and friends. And we won't even really know if I really do crash from this trip because I'm getting Xolair today so that will mask any crashing symptoms! Small blessings... =)


This past week was NOT the week we expected. We are thankful it is over and that my J tube was able to be fixed without surgery. We are so very thankful that we were able to make the trip down to VA this weekend and see one of my best friends and her husband. We might not have expected last week, but let's be honest, our whole life is nothing we could have anticipated. We're still finding reasons to smile every day and praying that our lives reflect God's goodness even in the tough moments. Ain't no mast cells gonna stop us!

Wednesday, April 12, 2017

Third Time's a Charm, Right???

Tomorrow will be Take 3 on attempting to complete some GI testing...Both of the first two attempts had to be rescheduled due to an inability to keep any liquids in my body which is necessary for this test. Round 2 even came with blood sugars in the 40s...just cause, you know, puking bile and stomach acid at 8 am wasn't enough fun to begin the day with...


So, Breath Test vs. Kylene's Nasty Masties Round 3 will go down (hopefully) tomorrow afternoon. And I really, really need my body to cooperate tomorrow. This breath test cannot be done within 6 weeks of having had a colonoscopy. After tomorrow, the next availability for this breath test is not until May and, you guessed it, AFTER the colonoscopy that was also scheduled after my GI appointment last week. So I'm going in prepared with a double dose of zofran and some benadryl (cause you know, nausea probably isn't enough excitement for my mast cells to throw at me, there's always the potential for some allergic reaction to what I have to drink, too...) and ready for tomorrow afternoon to be the best. day. ever. (If you haven't caught on to the amount of sarcasm usually used in these posts, please insert sarcasm and re-read that last sentence =P)

Anyway, puking episodes delaying this breath test is not the only fun that's been happening since my GI appointment last week. I am cautiously optimistic that the appointment went well and this doctor may actually be able to help us figure some stuff out. Unfortunately I have learned that only time will tell when it comes to determining whether a doctor will truly be helpful, but I am encouraged and we are hoping that he will be able to at least get us moving in the right direction.

The best part of the appointment for me was the very end when we were getting up to leave. Leni had been tucked under his desk the entire appointment, but had been so quiet that he hadn't realized she was there. He spent 45 minutes with her head less than 6 inches from his feet...I'm just glad she didn't try to rest her head on his foot! He actually asked me, "Has she been here this whole time?" I wasn't sure how to reply to that other than to smile and nod. I wish my quick, witty comebacks that come so easily when I'm writing here would come to mind in the moment... =P No...she vaporized into the room silently right about when we started talking about my Creon dosage...


Anyway, moving past the case of the silent service dog, the appointment did go well. At least as well a first appointment can go. Like I said, we'll really know how well it went in the follow-up from the testing, but for now we're cautiously hopeful. I was referred to this doctor by my GI surgeon and motility specialist (also both at the Clinic) because both of them thought I might have some form of pancreatic insufficiency going on and this doctor's main specialty is in pancreatic issues.

He spent about 45 minutes with me going over all my recent history in depth and listening to my progression of symptoms and how my mast cell disease was impacted by these GI issues. He felt that my symptoms and lab work are not consistent with chronic pancreatitis (which I've been diagnosed with about 6 times) which my mast cell doctors agree. He said that the scarring found on my pancreas during an endoscopic ultrasound is also not consistent with chronic pancreatitis, although it could be an atypical presentation. He also felt that it was not actual scarring but clusters of tissue which he said could be indicative of mast cell infiltration of my pancreas. This was what my specialist had guessed when we mentioned these pancreas issues to him, so it was interesting to get another point of view on this. Unfortunately, there's no easy way to look into this further...the only option would be a biopsy which is not something we feel is needed right now.

We also discussed some of my other issues with absorption and blood sugar fluctuations. The reality is that these symptoms could be entirely mast cell driven with no GI mechanism behind them. If that is the case, it makes figuring out the next steps a bit more challenging as we may have to look into treatment options that bypass my intestines and hope we can get things under control then pray I can transition back onto oral/liquid meds and stay stable. But before we go that route, we still have some more GI testing to do to make sure we aren't missing anything.

Ironically, one benefit of having nonstop absorption issues is that I am able to give a stool sample at the drop of a hat! Small blessings, right?? =P He ordered a few stool tests to check for parasites, malabsorption, and a few other potential causes that we don't want to miss simply because we thought it was too simple to test for. Those results have all come back and I am parasite, h pylori, and c diff free, so that is good news. (See Liz, 8 years later and still no real Herman =P) The stool samples did confirm that my body is not absorbing electrolytes properly and is dumping them out of my body at fairly high levels. Obviously that is not good, but didn't really give us any additional information that we didn't already know, just confirmed what we already knew. We're adding in some extra blood work to see what my overall electrolyte levels are to determine if we need to adjust what I am getting in the fluids I run at home twice a week.

This breath test that I'm going to do tomorrow (hear that mast cells?!?!) was supposed to be last Thursday. It will indicate whether I have a bacterial overgrowth in my small intestines. The complication with it is that I have to drink (and keep down) a mixture of disgusting tasting stuff that will activate any possible bacteria that may have accumulated in my small intestines. I can avoid having to taste it by putting it directly into my stomach through Herman (G tube), but since it has to go in my stomach, there is still the possibility that I can throw it up. And that would be why I will be going in to Round 3 armed with a double dose of zofran and some benadryl.

We also scheduled a colonoscopy for the beginning of May. It's been almost 10 years since my last colonoscopy and he felt that it was time to re-do it based on the symptoms I'm currently having. He will be checking for specific inflammation patterns that could potentially indicate a bowel disease that could be treated independent of my mast cell disease and taking biopsies for anything that may be happening on a microscopic level. Can't say I'm looking forward to having to deal with anesthesia again...I was hoping that my J tube surgery was going to have been the last time for a while.

BUT the good news is that things will hopefully go as smoothly as they did for my J tube. When I had that surgery, the head of the anesthesiology department had overseen my case and stayed in the room with me the entire procedure. He had researched mast cell diseases was able to keep things under control and prevent any severe reactions; it was about the best I had ever tolerated having to be put under. He had given me his card and said he would either personally oversee any future procedures or make sure whoever was on my case was up to speed on how to handle things. When I emailed him he said he would be happy to make sure things go smoothly again and that if he was not able to personally be on my case (since it's not in an OR) he would talk with whoever was assigned to me. So we are hoping that things will go well on that front!

Other than that, we're back to any chronic illness patient's favorite thing to do...hurry up and wait...We already have the results of the stool samples, and I'll have the results of the breath test tomorrow when it's finished. I go back to see my allergist in 2 weeks for an actual appointment (not to be confused with my twice monthly quick appointments when I get my Xolair), so I'm hoping we'll have at least something to discuss from the testing.

In other news, I am now officially 32 DAYS epi free!!! Despite still feeling incredibly sick most days and not having much resolution of symptoms as a whole, this is HUGE! Anaphylaxis is incredibly taxing on the body. It is literally the body going into shut down mode from shock. The only way to reverse that is to give an equally shocking injection (epi) to jump start the body and follow that with IV medications to stabilize things. It feels about as jarring as it sounds...so while I do hope that my epipen usage soon matches my actual symptoms, in the meantime, I am so very thankful to be a month free from anaphylaxis.

So that's the update from my GI appointment last week and what we're hoping to sort out in the next few weeks. Lots of testing, lots of waiting, and of course, lots of nasty masties...nothing to do but keep moving forward, finding reasons to smile, and continuing to work on Leni's ninja skills to keep surprising unsuspecting doctors!

Thursday, April 6, 2017

Remembering it ALL

This past week, Nick and I went to a class our church was offering based on Tim Keller's book, Walking with God Through Pain and Suffering. The class is a multi-week course, but the church opened up couple of sessions to anyone who wanted to hear some personal testimonies from the lead pastor and another member of the church who is pretty much a modern day Paul.

This week, the lead pastor shared his story about losing his brother in a motorcycle accident and how that impacted him, his family, and his relationship with God. Even though our experiences are so vastly different, I was reminded that it is through our seasons of struggle and pain that our understanding of God's character and purpose grows, and through that, our relationship with God (eventually...sometimes we don't see that right away...).

But what I really took away from his talk was a comment he made at the very beginning of his testimony. He made the comment that there is "something about pain that wire memories so we don't forget" anything about those moments in our lives. For him, it was his last day with his brother and the hours/days after hearing the news of his brother's death. I'm sure many other people have similar stories of recalling specific details from some of the darkest moments of their lives.

My initial thought was how weird it must be to remember so vividly certain moments in life when most of us can barely remember what we did a week ago! But as he kept speaking, I realized that even though my pain is not the same that he experienced, I do have some of those moments of vivid memories. I can remember specific details about certain days, weeks, and seasons with incredible clarity (while still barely remembering the previous week...). And in realizing that, I also realized that I have been given a gift.

My pain is not that unexpected, acute shock that he spoke about. It is a chronic, building pain that grows over time and continually pushes the boundaries of how much worse can things get. It is on going with no end in sight. Living with a chronic illness means that I have learned to live with the echo of pain always in the background, but also facing the reality that there are moments where that pain explodes from the background back to center stage. It is in many of those moments of pain that increase beyond my normal baseline where I experience moments like our pastor spoke about that cement memories into my mind with incredible clarity.

And here's the gift...those moments of increased pain may have been what triggered my brain to retain those snapshots in time, but in doing so, also meant that I retained other details about those same moments...things I might not have remembered otherwise...

I remember the day I had to use my first epipen and how terrifying it was to feel my throat closing and being unable to swallow...but I also remember friends from church stepping up to help, nurses helping to keep me calm and actually stabbing me with the first epipen, others calling Nick and an ambulance and making sure the EMTs had my medical info, and the many texts I received that night and the next day from people asking how I was doing and letting me know they were praying for me.

I remember heading in to my first endoscopy and tube change at a new hospital and being terrified of going in anaphylaxis from the anesthesia...but I also remember doing MadLibs with Nick in the pre-op area that made me laugh so much I was sore the next day (and not from the procedure =P).

I remember being admitted 2 days before Christmas and spending all of Christmas Eve in the hospital dealing with doctors who didn't understand mast cell disease, my body not responding properly, and a clogged feeding tube...but I also remember somehow fitting me, Nick, and Leni in my hospital bed to watch the Christmas Eve service online and just spending time together in the midst of the crazy holiday season.

I remember endless days of being unable to eat, throwing up, pain, and more...but I also remember Nick willingly bringing me (also seemingly endless) slurpees each night when I couldn't tolerate anything else.

I remember a day where I had not left the house in over a week, had been throwing up multiple times, passed out twice, and was physically, mentally, and spiritually exhausted...but I also remember Nick coming home with a Venus Fly Trap that we started feeding flies and spiders to. (It lived 2 weeks, I think =P)

I remember when I was struggling to walk because of balance issues, loss of coordination, and muscle weakness and felt scared because we had no answers and so limited in my independence and ability to do things...but I also remember Nick pushing me around the Toledo Zoo in a double stroller (they were out of wheelchairs =P) so we could enjoy one of my favorite things (zoos!) and spend time with friends.

There are so many other moments that have been cemented into my memory because of the pain of that day/week/season. It goes beyond just vague recollections and hazy mental images...these are clear moments of specific details that echo in my mind as reminders of some of my toughest moments. Yet these memories are so much more than sad and painful...they are also full of joy, encouragement, laughter, and smiles.

Without the pain, these memories of joy would not be as clear. The toughest moments that have left their mark on my life like scars that never fully disappear, have also allowed me to remember things that otherwise would have faded into the background and been forgotten. It is BECAUSE of my pain that I have the GIFT of these other memories. For me, my pain and hope, sadness and joy, tears and laughter are so closely woven together that I can't have one without the other. And I wouldn't change that for anything.


Monday, April 3, 2017

Big Appointment This Week

I've been home from VA a full week, and I think it has rained every day...at least that's a decent representation of how my body's been feeling, too...

Other than my Xolair last Monday, I have not left the house. It's been a rough week... I knew it would be. I pushed myself to be able to do things with family and friends when I was in VA. Sometimes I'll make a decision to do that extra activity or eat some food knowing I'll pay for it later. I made a few more of those choices that week than maybe I "should" have... I'm not sorry I made any of those decisions, but that doesn't make the symptoms I have been dealing with now any less severe...

Leni working hard at a Trippin' with Jamie conference in VA
Anyway, for the past few months (pretty much all 2017), we've been waiting for an appointment that is now this week! Since this recent flare started, I've been having major issues with absorption. Meaning my medications are not being absorbed, any food I do eat is coming out undigested, and my tube feeds have been running through me. That is the main reason I now have IV meds to use when my intestines are just not playing nice. At least those IV meds have gotten the constant anaphylaxis under control, but it hasn't solved the problem, just slapped on a temporary band-aid.

And that's what we are hoping and praying this appointment will be able to help with. I'm seeing a pancreas specialist in the GI department at the Cleveland Clinic on Tuesday. I currently have a GI and GI surgeon at the Clinic and both have referred me to this new GI to hopefully get some insight on all these absorption issues. My allergist and neurologist are also both waiting to hear what this doctor has to say too, because we can't try any new medications until we get my absorption sorted out.

Lazy day snuggles on my parent's couch
So you could say there's a lot riding on this appointment...I hope this doctor is ready! But seriously, there is A LOT riding on this appointment...and I'm nervous. I've had more than my share of doctors who can't or won't do anything to help. And I don't know what we'll do if that happens with this one, too... All my current doctors have deferred to this appointment. We can't try any new oral meds until we get the absorption figured out, so we've been waiting months for this appointment to get here. When I saw my allergist and neurologist earlier this year, they both were just asking what I needed from them to GET to this appointment because there's nothing more for us to do until we get some answers about my pancreas.

It's great to have doctors so willing to help, but also frustrating (and the unfortunate reality of rare disease patients) when there's nothing they can do. As my allergist said, until we can get my body absorbing my meds, we can't even try any new meds for my mast cell disease because we won't know if they just don't work for me or if they're not working because I'm not absorbing them. Even though I do have IV access, we really don't want me getting dependent on IV meds long term. If we were to start any IV treatments, then I'd be stuck relying on those, so we're holding off on that unless absolutely necessary and we have no other options. So that means these past few weeks/months have been a matter of just getting through the days as best as possible and keeping me as stable as possible so I can get to this appointment...

And now that the appointment is here, we have to pray and hope that he's willing to listen and can do something to at least get us moving in the right direction...NO PRESSURE, right??

Let's do this!
So that's what's up this week! Big appointment that will either give us a new direction to go in and potentially new angle for treatment options OR will be a complete waste of time and I'll leave wondering "now what?"...Please be praying that second possibility is NOT the case.

In other news, I am now 26 WHOLE DAYS EPI FREE!!!! That, my friends, is big news on it's own! Since October, I've been going into anaphylaxis every 4-5 days and needing at least one (often more) dose of epi multiple times a week. When we finally got the IV medication situation sorted out, I made it 15 days before the unfortunate run-in with the essential oils fair. After we reset the counter yet again, I am now SO CLOSE to a full month without epi!! That's a reason to celebrate! Although we'll be keeping it super low-key because we know strong emotions can be a mast cell trigger and we don't want to rock the boat when I'm so close to A MONTH!!!

I know my posts have been less frequent lately, and that's mostly because there hasn't been a lot going on (at least that is worth sharing online =P). Like I mentioned above, the past few months have been a holding period waiting for this appointment this week. Now that it's here, hopefully (prayerfully) we will be able to start moving in the right direction to get things under better control and get me back to where I was last summer.

As always, THANK YOU for your prayers. In my absence from this blog, please know that I am very much aware of many of you who have been faithfully lifting up me and my family in your prayers. We are so very grateful and thankful for that! Please be praying that this appointment with the pancreas specialist is productive and that he will have the insight that we need to move in a good direction. Please be praying for strength and encouragement for me as I am struggling with some increasing symptoms and feeling more isolated and unable to manage simple daily tasks some days. Please pray for strength and joy for Nick as he is working full-time, serving me so selflessly, and acting as the main point person for our house hunt. Please pray that even in the midst of this season of life, our lives would be a light to the doctors and others that we meet and that our struggle is being used in a way to bring encouragement and Christ to others who are also hurting.

Wednesday, March 15, 2017

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

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

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

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

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


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

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

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

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

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

Wednesday, March 8, 2017

When Essential Oils Become Deadly

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

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

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

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

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

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

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


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

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

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

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

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

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

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

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

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

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

Wednesday, March 1, 2017

Rare Disease Day 2017

Happy Rare Disease Day 2017!!!

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


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

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

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

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

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

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

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


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

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


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

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


Tuesday, February 21, 2017

Four Weeks in Coming

If you live with a chronic illness, than my guess is that you've had AT LEAST one incident of insurance company frustration or pharmacy confusion or inconvenient doctor absences. If you're me, then you've likely experienced all 3...at once...right when we had major stuff to resolve...seriously, if dealing with being sick every day wasn't a full time job itself, then spending hours on the phone certainly is.

Since my recent flare started back in November, we've been desperately trying to get things back under control and me back to where I was in September and October when I went to Disney and Yosemite. Despite having no idea what sent me into this downward spiral, we still had to try SOMETHING as I was rapidly losing all the progress I made from Feb-Oct of last year.

First up was seeing what we could do about my GI tract. Since initially, the first symptoms to reappear were GI issues, that was where we went. After my admission just before Thanksgiving, we knew something had to give and fast. In December we changed out Herman III to Herman IV to see if that was the answer (spoiler alert: it wasn't). During another admission in January, the GI there said that he felt I needed access to IV medication since it was clear that my gut was just not absorbing my oral meds at that point. A week later, I spoke with my allergist and he agreed. And so began a month long process of trying to get said medications approved...more on this later...

In addition to needing IV meds, we also realized that my time with the GJ tubes was rapidly coming to an end. For multiple reasons, it was time to separate my G and J tubes, and at the end of January, I had surgery to change Herman to a G button and to create a new stoma for Sherman, my new J tube. I am happy to report that this surgery DID resolve all the tube issues I had previously been having. It did nothing to improve my absorption, but I do now have a referral to a pancreas specialist who I will see in April. Hopefully we'll be able to get to the bottom of some of these issues, but in the mean time, the IV meds because even more important to just get me to that appointment.

Before I get back to the long, drawn out, complicated, frustrating, confusing, month-long process of figuring out how to actually get the IV meds that my doctor had prescribed, here's a little background ...since this flare started, I had not gone more than 5 days without being in the ER or hospital (until this past week!!! Read on to find out the length of my current streak!). When I was having all the tube issues, if I started to have a bad mast cell reaction I would go to take my emergency medications. Half the time my tube would be kinked, clogged, or for all intents and purposes just not working. This meant I had no way to stop the snowballing reaction and ended up needing my epipens way to frequently.

After just 6 weeks of relentless mast cells, on going reactions, and way too many ambulance rides, my peripheral veins were shot. One particularly bad reaction I ended up needing 3 doses of epi simply because they couldn't get IV access in time to prevent the rebounding reactions. Five days before Christmas I had surgery for Mort the port to give us central access for the majority of the ER trips and hospital stays so we could salvage what was left of my peripheral veins for these actual anaphylaxis emergencies and the EMTs. Having Mort meant that the option of IV meds that the GI doctor had recommended while inpatient was an actual possibility. The only challenge was just figuring out how it would actually work...

And so began the fore-mentioned month of frustration, confusion, and me realizing that I appeared to know more about the inner workings of my health insurance company than the actual employees did. At my appointment in January I got a prescription for not just IV meds, but also at home IV fluids and electrolytes. Since he was in the same hospital system as the infusion pharmacy we were going to use, it *should* have been a simple matter of putting the orders in their online system. And for the fluids and electrolytes it more or less was. We had a minor hold up with them not wanting to approve home infusions but wanting to have me come into an infusion center. The other issue was them not wanting to teach Nick and me how to access Mort ourselves which we felt was important for my overall care and independence. We got that straightened out *relatively* easily. The main hold up was the IV meds...

First time accessing my own port!
Now, let me explain that the IV meds my doctor has prescribed are NOT protected substances. One can be bought by 10 year olds OTC (benadryl) and the other is given like candy to patients going through chemo and other conditions likely to cause nausea (zofran). They are not narcotics, they are not controlled, and even my doctor commented that he watched someone try to OD on one of them and only ended up sleeping for hours and a serious cotton mouth. Yet when we tried to get these things approved, it was way more complicated than we would have imagined. The infusion company providing the fluids and electrolytes said they only provide benadryl as a pre-treatment option before antibiotics or other infusions known to cause potential reactions. They could not provide it as a stand alone order...even with my doctor's prescription...

So that meant we had to figure out another way to get the IV meds. I already get my Xolair from a specialty pharmacy through my insurance company, so we thought that might be our next option. Depending on who I spoke to, I was told that we could get the meds there and that I couldn't. Talk about confusing...it got even more confusing when my case manager with my insurance company was told different information than I was...

It took two weeks, but we finally figured out that yes, the specialty pharmacy was in fact the place to send the scripts. The next hurdle was figuring out whether or not the meds would be covered by my policy. Again, mixed messages...first I was told it would be covered as a Tier 2 medication, then my case manager was told that they wouldn't be covered at all. The next morning I called the pharmacy to start the appeal process and instead of being told how to go about filing the appeal, I was told that the meds had not only been approved, but were covered at 100% and had already shipped!

Unfortunately, because they are IV medications, they had to be shipped to my doctor's office unless there was additional authorization from my doctor to ship directly to my house. We figured this was just something minor to resolve until I called the office to pick up the meds...only 1 vial had shipped...and that vial was only a single dose...of course this happened on a Friday, too...


Monday morning, I thankfully had an appointment scheduled with my doctor in addition to my standing Xolair injection. By the time I left, we *hoped* we had finally gotten everything resolved. I gave the specialty pharmacy until Wednesday to process the new scripts. When I called they said that yes, they saw the new scripts, but it was still showing that they had to ship to my doctor. Another call made to my doctor's office...side note, the nurses can now recognize my voice before I even say my name...

They called me Thursday morning saying they spoke to the pharmacy again and confirmed that there was authorization on file for it to ship to my house. Back to the pharmacy...I was told, yet again, that I needed additional authorization to ship to my house...we went back and forth a couple of times before they finally found it buried in my file. Then I was told it would be 3-5 business days of processing. I very nicely pleaded with them to expedite things. I explained how long this process had been and how badly I needed these meds. The lady I was speaking was very helpful, and 30 minutes later the 3-5 days worth of processing was done. She told me they would ship out that afternoon and I would have them the next day!

When nothing had arrived by 4 pm the following day, I figured that yet again, they had shipped to my doctor's office and I'd have to wait until Tuesday (why does this ALWAYS happen on the weekends???). At 5:30 pm I got a call from the pharmacy...the meds had not shipped out on Thursday like they had originally told me because they discovered they were out of one of the meds. They DID ship out on Friday and would arrive before noon on Saturday. At this point I wasn't believing anything until I actually had the meds in my hands...


Thankfully, I am very happy to be able to report that the meds did, in fact, arrive Saturday morning around 9 am. Since then, I have already had the opportunity to use both the benadryl and zofran in situations that otherwise might have escalated to anaphylaxis and uncontrolled vomiting. I wish I was not in a place where I need IV medications at home, but right now, I'm so very thankful to have this option.

A MONTH after this process started, it has finally been resolved...I understand there have to be policies and procedures in place, but I'm not so sure they are working in the best interests of the patients...it should NOT have been this difficult! How many epipens could have been avoided in that time? How many ambulance rides would not have been needed? I could write another whole post on the insurance issues that arose just with this issue (and every issue I've had could be its own blog altogether...), but I'll spare you the gory details. =P

Instead I'll end with sharing that I now have a new record!! I'm going on 9 days without an ER trip or ambulance ride. That is largely due to having these IV meds, and we are hopeful that in having benadryl and zofran as options, this streak will continue to increase!

Friday, February 17, 2017

Mast Cell Humor for Valentine's Day

In case you missed it, Valentine's Day was this past week. I am so thankful to have married a husband who is equally as uninterested in this holiday as I am. We did absolutely nothing to celebrate. And it was wonderful!

That evening, after a very, non-romantic, but no less love-filled dinner (aka, Nick ate and I had some broth), we were catching up on the new season of 24. I started texting a mast cell friend who had posted a funny "mast cell valentine" on Instagram with some of my own mast cell humor. That snowballed into some very bad (or amazing!!) mast cell jokes that are only appropriate to share with loved ones who understand the unique life that is lived with mast cell disease.

Our brilliance in coming up with these "pick-up lines" or endearing poetry or rewritten Taylor Swift and Carrie Underwood songs, was too incredible to keep to ourselves. We *might* have just discovered our new career path...So consider yourselves warned! Haylee and I hope you enjoy some of our mast cell humor courtesy of Valentine's Day:

My love for you flows as freely as my GI tract during a reaction.

Flushing is red,
My lips are turning blue.
Please stab me with my epi,
So I don't leave you!

I decided to save money on buying a card, so instead I wrote a message on my skin. #dermatographia

Only you know the way to my heart...through my port and catheter.

You're more attractive than a lifetime supply of Benadryl!

All I need is you! Or at least your steady hand to stab me with epi and give me IV meds.

*Goes out to dinner* This food is to die for! No literally, my throat is swelling and I can't swallow...

The memories of you are stronger than my brain fog.

I'm crazier for you than I get during mast cell reactions!

When I think of you, my heart races like I just stood up.

Being in the same room as you takes my breath away...seriously, you must be wearing some perfume...

You make my adrenaline pump for than epinephrine.

I fall for you more than I fall from reactions.

Losing you would hurt more than losing my bowels.

Missing you like I miss my life before mast cell disease.

Your love makes me weak at the knees...or maybe that's just my neurological issues...

Life with you is as perfect as finding a temperature that is neither too hot nor too cold.

My love for you swells more than my body in anaphylaxis.

Your love is electrifying...oh wait, that's just my neuropathy.

I'd give up all my medications for one more day with you. Although without my meds I wouldn't have one more day to spend...

You make me loopier than a double dose of IV Benadryl.

You're so hot you give me a heat rash.

I love you so much I'd stay up thinking about you even if I didn't have painsomnia.

Sung to the tune of You Belong with Me by Taylor Swift:
Mast cells try to kill us, we fight back with epipens.
Flushing, rashes, hives, and more; so much for a good weekend...
Dreaming 'bout the day when we wake up and find
That mast cell disease was just a nightmare this whole time!

Sung to the tune of Before He Cheats by Carrie Underwood:
I stabbed my epi into the side of my quadriceps muscle in my outer thigh.
Kept myself from needing a code blue.
You injected my meds into my central line,
I'm conscious again and we bought more time.
Maybe next time I'll think twice before I eat...

Hope your day (and love life =P) has been enriched by our incredible brilliance and potential new careers in mast cell humor greeting cards! We certainly got some good laughs out of all our texting. Yes, life with mast cell disease is often very tough and difficult. But it has also brought me friends I wouldn't have met otherwise and moments of laughing so hard I wake up the next day sore at my less than 3 week old incisions from abdominal surgery. Don't forget to find reasons to smile and laugh in every day!