Monday, January 30, 2017

Introducing Herman and....????

Drumroll please...Sherman!!!

Out of all the suggestions I received, I think I have settled on Herman and Sherman for my newly separated tubes. Herman will be my G tube since that is the original stoma site, and Sherman gets the distinction of being my brand new J tube. And, as promised, SHOUT OUT to Elizabeth for having suggested the chosen names!

At the risk of sounding like, yet again, a broken record, THANK YOU ALL so much for your prayers. I think I was more nervous for this surgery than I have been for any of my previous procedures. But God answered our prayers in an incredible way on Thursday and this surgery went even better than any of us could have expected.

After my pre-op appointments on Tuesday, the anesthesiologist I had spoken to actually called the doctor who would be overseeing my procedure. He had 2 days to research, and think about what our best options would be for my surgery. Before they took me back, he came to my pre-op room and we talked for maybe 20 minutes about every little detail. He knew which meds were most likely to cause mast cell reactions as well as which were usually safe for mast cell patients. He listened to all my previous issues with anesthesia and what my biggest concerns were. By the time he left the pre-op area, we felt confident that he was as prepared as possible to handle anything that might come up during the surgery.

Leni in pre-op...her head is always too heavy =)
I also got to speak briefly with the GI surgeon before the surgery. He showed me where he was planning to place the new J tube and that I would be able to have a button put in for my G. He also took a few minutes to hear about what had been going on for a few months now. I follow-up with him in a week, and he seemed very engaged and willing to help us get to the bottom of my absorption issues and relentless GI symptoms.

The surgery itself went better than anyone could have anticipated. It was scheduled for 118 minutes, so my mom got a bit of a scare when her pager went off after only 30 minutes saying the doctor was coming out to speak to her. Turns out everything went so smoothly, he was able to do everything that quickly! My anatomy that made it tough to place GJ tubes actually turned out to be beneficial for a surgical J tube. My G and J tubes are only about an inch apart and fairly high up, close to my ribs. There is so much less muscle to have to cut through in the upper abdomen vs. the lower abdomen closer to the hip bones, and I think that is partially why the recovery has not been as bad as I expected. In about 2 months or so, once the stoma has healed completely, I will be able to exchange the long J tube for a J button and be rid of long tubes forever! I am very excited for that =)

While the surgery was quick and I had no major reactions (praise God), we were very aggressive with our pre-treatment medications and continuous medications during the procedure to keep it that way. I typically get benadryl, pepcid, and solumedrol before any procedures, and this time we added in continuous benadryl and racemic epi during the actual surgery to keep my mast cells under control. They had a bit of a challenge placing the breathing tube because my vocal cords started to spasm, but they were able to control that with a muscle relaxant that we knew I tolerated well. We also found that one of my sedation "allergies" I CAN tolerate if we are aggressive with the mast cell protocol before and during. It is more of a mast cell reaction (likely to some of the ingredients and not necessarily the actual medication) vs. true allergy, so keeping my mast cells under control meant I was able to tolerate it well. This is GREAT to know going forward!

I cannot say enough good about the anesthesiologist. He was more than on top of anything that came up, and I don't think we could have done anything differently or better. He also said that for any future surgeries or procedures needing anesthesia that I may need, to call him directly and he would personally oversee me in the OR. While I will hopefully have no need for any more surgery in the near future, I feel as comfortable as I think I ever could feel knowing I have a fantastic anesthesiologist on board who knows what is going on and how to deal with things. We are so thankful for that!

In the PACU before waking up
Even recovery was so much less eventful than we are used to! Although my mom did get some weird looks from the nurses in the recovery unit when she was so unconcerned about me being completely unresponsive for 6+ hours. I think she was just happy I wasn't going in to anaphylaxis or dry heaving or wheezing! It took me a LONG time to wake up. Like I said, 6+ hours of being completely unresponsive to anything going on around me. Poor Leni was very upset when she got to see me in recovery. She kept trying to get me to pet her, but I wasn't responding at all. Although, what was really weird is that I remember almost everything that was said about me or near me in that time. After I woke up, I was able to repeat back parts of conversations that my mom had with the doctors and nurses while I was unresponsive. It was crazy. I remember almost everything that happened during those 6+ hours, but just was not able to respond or move or do anything. It was very weird, but hey, compared to some of my recoveries, we'll take it!

Just woke up in my room!
I spent Thursday night in the hospital to make sure my pain and mast cells were under control. Since I use my J tube for all my emergency meds and some of my daily meds as well, we needed to know that would work before sending me home. We were cautiously optimistic that I'd only need to stay one night, but knew it could be more. Thankfully, as smoothly as things went, I was able to come home on Friday! They gave me some pain medication in the OR before taking me to recovery, and I took some tylenol before bed, but other than that, I did not need anything for pain. I was able to start up my tube medications Thursday night, and by Friday morning was back to running my tube feeds again. I'm definitely sore, but the pain is not bad. I have 4 incisions across my abdomen and the hole they placed the J tube in. By Saturday, the swelling had already decreased significantly and I was able to vent most of the gas they had pumped in out my tubes.

Leni was very ready to snuggle as soon as I woke up.
Overall, things went better than we expected, and we are so grateful! Despite having just had abdominal surgery, I am doing well, and not dealing with many of the anesthesia issues that I have in the past. I am so glad it is over, and now I can adjust to having 2 tubes. Next up is setting up my home infusions which will start this week. I got all the supplies shipped Friday night (with only one mistake that had to be fixed...can't escape it, no matter how hard I try =P), and the nurse will come out to teach me and Nick how to access my port so we can be independent with my infusions. I have my next Xolair and surgery follow-up this week as well, so it will be a busy week. Coming up in February is a consult with nutrition, and my rescheduled functional medication and neurology appointments that I had to change for my surgery. Plus, more Xolair, appointments with my allergist, and whatever else comes up that we have to deal with.

Always a whirlwind in Bokatopia! But for now, we are so thankful that surgery went so much better than we expected, and I am recovering well. We're just taking things one day at a time and doing our best to find reasons to smile every day. Thank you for being a part of our lives in prayer and with your encouragement. We are so grateful for all the prayers said for me and my family for this surgery, and we are glad to be able to share how those prayers were answered more fully than we could have hoped!

Wednesday, January 25, 2017

Surgery Details

Pre-op appointments are done. My mom is on her way back to Ohio. And we are ready for surgery! Kinda...

In case you missed it, I'm having surgery tomorrow to place a J tube in my intestines. My current feeding tube is what is called a G/J tube. I have one hole that goes into my stomach with a 2-in-1 tube to provide access to both my stomach and intestines. For a variety of reasons, this type of tube is no longer the best option for me. So tomorrow, I will be having a separate J tube placed. Going forward I will now have 2 tubes, the G will remain where my current tube is now and the J will be wherever the surgeon places it tomorrow.

I spent the better part of yesterday going to 5 different pre-op appointments to make sure everything is all set for my surgery. It was a LONG day...and not just because I had so many appointments...Of course my body likes to choose the best possible times to be so sick...I woke up yesterday morning so nauseas and barely able to stand. I honestly wasn't even sure how I was going to be able to leave the house...Obviously I made it to my appointments, but it was tough.

The day started with blood work and an appointment with an internal medicine doctor who has to sign off that I am fit for surgery. I found this to be just a matter of going through the motions because the reality is that I need this surgery. Even if my blood work comes back weird or the doctor feels there are added risks (there are...), we have to do this anyway. The doctor was very nice and listened well. We discussed her concerns with my complex history and my concerns with the surgery. She wasn't the one making any anesthesia or actual procedural decisions, but she was able to put in my chart that I absolutely cannot miss any doses of my medications and make sure there are standing orders for my emergency medications for the nurses to see when I am admitted after the surgery. Other than that it was just taking vitals, and the routine questions of whether my blood pressure is always low and if I can ever get a pulse ox reading...and of course, the doctor not realizing Leni was in the room until we got up to leave. I don't think that ever gets old =P

After the internal medicine appointment I saw anesthesia. Originally I was scheduled to just speak with a nurse anesthetist, but I had asked specifically to talk with one of the doctors since this is a huge concern for us going into surgery. This was a very productive appointment, and I think we are as prepared as we can be going in to tomorrow. We will be using a new anesthesia medication that I have not had before (which is good considering I have not done well with any so far...), and I think we have a good pre-treatment protocol set up. We are also going to be trying some continuous medications during the procedure to hopefully keep any reactions under control, so I'm hopeful that they are on top of things. Of course, the doctor I spoke to yesterday isn't the one who will actually be in the room with me, so we'll probably have to go over everything again tomorrow...but at least it's a step in the right direction and everything WAS added to my chart, so who knows, maybe we'll be surprised and the anesthesiologist will come in tomorrow super prepared.

We also made the decision NOT to do this as an outpatient surgery for a couple of reasons. The main reason is that I may need IV medications to keep any mast cell reactions under control. Since I am having J tube surgery, my intestines may need a few days of recovery before I am able to absorb my liquid medications that I need to manage my mast cell disease. We do not want to risk anaphylaxis because my intestines are not ready to absorb my medications! The main criteria to be met for discharge is that we are able to manage all my mast cell symptoms with my liquid meds through the new J tube. The other main reason is pain management. J tube surgery is reported to be a bit tougher than G tube (what I had before) so we are not sure if I will need any IV medications to keep pain under control. Pain is a mast cell trigger, so again, the goal is keeping my mast cells under control during recovery. We are hoping that I will only need to be admitted overnight, and we will be on our way home Friday, but the GI nurse said many people end up staying 2 nights...we're just going to have to wait and see!

My final appointment of the day was with the GI surgeon's nurse to discuss the specifics of the J tube placement. Of course, I couldn't make it through the whole day without any scheduling issues...For whatever reason, they scheduled my pre-op appointment at the same time that the nurse was in I waited almost 2 hours past my appointment time to see her. She was very apologetic and we got everything done we needed to, but I was beyond exhausted by the end of the appointment. Since I've had a feeding tube for 2 years now, she didn't have to go over all the tube feeding details. We just discussed where the surgeon typically tries to place J tubes, and his back up locations if my anatomy won't cooperate for his first choice. We talked about how we will manage my hypoglycemia the morning of surgery, and tried to schedule me as early as possible. I'm excited that my G tube will be exchanged for a button style tube which means no long tube hanging down! Eventually the J tube will be a button as well, but I have to have a long tube initially to allow the stoma to heal completely. In 6-8 weeks, once things have healed, we will replace the long J tube with the button. We have set up consults with the home supply company to come by on Friday to make sure I have the supplies I need for my new tubes so I can keep running my feeds with minimal interruption.

And that's it! Everything is in place, and we're as prepared as we can be. I'm definitely nervous...I don't think I'll ever go into any of these procedures without some nerves...but I'm not anxious. I know God is in control, and we are trusting that He is walking with us every step of this crazy mast cell adventure. In the past week, a couple different friends have shared these verses with me. Numbers 6: 24-26, "The Lord bless you and keep you; the Lord make His face shine on you and be gracious to you; the Lord lift up His countenance to you and give you peace." These are also the verses my parents prayed over me and my brother every night before bed when we were growing up. God has been so gracious to me and given me peace that is beyond understanding. My surgery tomorrow is no surprise to God, and I can be at peace knowing He is holding me in His arms.

I will try to post an update at least to Instagram sometime Thursday evening if I'm able or Friday (or my mom or Nick will update). I'll do a full blog post once I get home. And don't forget, voting and suggestions are still open to name my tubes! Time is running out! I'll share the names in that first blog post after surgery (and give a shout out to whoever suggested the chosen names!), so get your input in now!

As always, thank you for your prayers. It is such a blessing to know I am not going it alone. Not only do I have incredible family walking this road with me, but so many friends also being a part of our lives through prayer and encouragement. Thank you!

Monday, January 23, 2017

Ready, Set...GO!

So last week I posted on Wednesday how we had already been back to the ER once since I had been discharged from the hospital. I had appointments coming up last week on Thursday and Friday and this week on Monday and Tuesday. We were going to be making some decisions and hopefully trying some new treatment options.

Well...since then, I ended up back in the ER (again) this time due to anaphylaxis needing 2 doses of epi, had my GI consult on Thursday, scheduled emergency surgery for this week, rescheduled the appointments I was supposed to have on Monday and Tuesday, and still talked with my allergist about new treatment options. Yes, Thursday and Friday were a whirlwind of craziness.

Wednesday ended up being a rough day overall with my body just not wanting to play nice at all. Still not sure if it was due to Xolair, but it was just a "not happy Bob" day. I was hoping to be able to keep things under control with my meds at home, but unfortunately that was not the case. About 4 pm or so, Leni started to alert and I knew at that point I had moved beyond just troublesome symptoms to oncoming anaphylaxis. The rest of the day was spent in the ER recovering from needing 2 doses of epi, an ambulance ride in to the ER, and lots of IV meds...and getting more potassium...

On Thursday, my parents (who were in town visiting from VA) drove me downtown for my GI consult. Now, I had been told this appointment would either be fixing or replacing Herman. I went in prepared to potentially have to deal with another round of anesthesia, but also with some questions since Herman had been working alright since I had been out of the hospital. When the GI doctor came in, he took our conversation in a completely different direction that I had anticipated. Long story short, the type of tube I have is notorious for these kinking problems I have been having for the past month. He was shocked I had never had any issues until recently. He said he could replace my current tube, but it would only buy time until I would have the same issues again. The only way to really resolve the kinking issue is to have surgery to separate my G and J tubes.

For those of you new to this blog, I have a GJ feeding tube. This means I have a hole in my stomach where the tube enters my body. The larger tube is the G tube and it ends in my stomach. The J tube is smaller, runs down the inside of the G tube, and extends into the middle portion of my small intestines. Because it has to cover so much distance, there is a lot of tubing to potentially kink or get displaced. This is the issue I am having. The only way to really resolve it for good is to have a separate J tube surgically placed in my intestines. This will significantly shorten the J tube and leave very little available to kink. Because I rely on my tube for almost all my nutrition right now and also need it for emergency medications, I HAVE to have a working tube. So Thursday I will be having surgery to place a J tube in my intestines!

I am still keeping the original Herman. That stoma will be replaced with just a G tube that will end in my stomach like it does now, just without the J extension. The J tube will be done laproscopically and placed directly into a portion of my small intestines. Here is the big dilemma. I will be going from 1 tube to 2...I'm going to need another name for my new tube. I think we have decided that my G tube will remain Herman. He's been there for 2 years, and I really don't think I could call it anything else. BUT that means, I need to come up with a name for my soon-to-be J tube...and that's where you come in! I need suggestions! I am open to retiring the name Herman if someone has any really good ideas for both tubes, but I also would like some ideas for names that could pair with Herman. Right now the leading candidates are Herman and Sherman or Gassy (because I vent gas out my G tube) and Passy (my formula passes through the J tube). Voting and suggestions are open and the winner will be announced in my first blog post after surgery!

I have 5 pre-op appointments now scheduled for Tuesday this week (my other Monday/Tuesday appointments were rescheduled) to make some decisions on what anesthesia will be used, what types of tubes, and other details. As with any procedure involving anesthesia, I am nervous. It's only been a month since my last time having to be put under and that did not end well. We are running through anesthesia options faster than my mast cells degranulate which is not a fun place to be. Please be praying for wisdom and insight as we make some decisions regarding how to best keep me safe during surgery. I will likely be admitted overnight (and hopefully ONLY overnight) to make sure things are okay, so we are hoping to be home on Friday. Going forward, this will simplify tube changes significantly as I will no longer have to be put under which is fantastic in the long run, but I still have to get through surgery next week...

On top of the excitement of surgery next Thursday, we also have some coordinating to do with my insurance and home healthcare to start setting up this week. At my appointment with my allergist on Friday, we talked about how bad things are and what we need to do to get me stable. Obviously the ultimate goal would be for actual improvement, but step one is just getting me stable. Since my GI tract is still throwing a fit, oral (and even tube) medications are not a great option right now. Since I have my port, we now have the ability to do IV treatments at home. We are working on setting up some regular infusions to hopefully help stabilize my electrolytes and get me hydrated as well as having access to IV medications at home. We are hoping that this will give us the ability to keep things under better control at home and not need to be rushing to the ER nearly as frequently.

Whew...stopping to take a breath...

Not going to lie, things feel overwhelming. A lot happened in less than 48 hours...I think I'm still processing through stuff. We are very hopeful that being able to access Mort at home will help regain some control over my symptoms and get things more stable. We saw the benefit I got from IV fluids, electrolytes, and medications when I was in the hospital earlier this month, so I am looking forward to getting this set up. At the same time, though, I'm dreading it...

In my head, home IV access was always reserved for the most severe mast cell patients. That was never supposed to be me...yet here I am...What went wrong? I was doing SO WELL for 10 months of last year! What changed? Why is everything crumbling again? I thought we had moved beyond the really rough days, and I was going to be a "success story" of effective mast cell treatment. Instead, I'm finding myself in a position of needing care that I watched my doctor prescribe for some of the more severe mast cell patients. I'm looking forward to the benefit that we are hopeful I will get from the home IV access, but at the same time there is a part of me that feels like if I just hold off then maybe it means I'm not really THAT sick...Don't worry, I know there's no logic in that. We're in the process of setting everything up, but that doesn't mean my head fully accepts that this is where I'm at right now.

Along with everything running through my head for that, I'm also dealing with having less than a week to mentally prepare for abdominal surgery. Especially with this surgery not even really being on the table before Thursday, it feels like things are happening so quickly. We are at peace with this decision. We do feel this is the best option for my needs right now, but that doesn't mean I'm not nervous. There are always risks with any surgery and my mast cells just add another layer of risk. Add to that the fact that the doctors who will be in the room with me may not have even heard of mast cell disease before meeting me, and I'm having to bring them up to speed in a 15 minute pre-op appointment...we do everything we can, then just have to leave it in God's hands.

Please keep me and my family in your prayers this week! Pray for my pre-op appointments tomorrow that they would accomplish exactly what we need to resolve. Pray that we will come away with answers and feeling as prepared as possible for Thursday. Pray for wisdom for the doctors during surgery that they are able to manage any issues that may arise and able to complete the surgery with few problems. Pray for safe travel for my mom as she is driving (back) out on Wednesday to be with me for the surgery. Pray for peace for Nick and our families on Thursday while I am in surgery and recovering. And please pray that in everything going on this week, we will be able to keep our eyes and hearts fixed on God. Let Him shine through us and remind us that He is carrying us through this season of life.

As always, THANK YOU for your prayers and support! As I was writing yesterday's post about it being 2 years since my official diagnosis, I was thinking about how much has happened in that time. A lot has changed...some good, some not so good. God has been constant, but so has the support of our incredible network of family and friends. We are so grateful for all the prayers and encouragement over the years and everyone walking through this crazy journey of life with mast cell disease alongside us. Thank you.

I hope you're ready for year 3!

Sunday, January 22, 2017

Two Years...or Ten...

It has been 2 years since I was diagnosed with Mast Cell Activation Syndrome. In some ways, I can't believe it has been 2 whole years of actually having a name for the battle raging in my body. And even though my chronic illness journey started 10 years ago, it has been these past 2 years that have made such a difference.

I talk a lot on this blog and Instagram about the desperate need for more funding and research for rare diseases. Obviously the ultimate goal for any rare disease patient is a cure, but even the small steps that have been made just in the 2 years since my diagnosis have been so important for my healthcare and the mast cell community as a whole.

Most of the time, "healthy" people don't understand the actual ramifications that a diagnosis means to a rare disease patient. It is so much more than just a few words on a page. It is validation. It is the acknowledgement that we are not crazy like so many doctors have told us. It is affirmation that yes, there is something wrong with our bodies. Far from being an unwelcomed label that will follow us for the rest of our lives, it is a long sought after answer to years of no answers.

I cried the night we got the call from Dr. Afrin's nurse with the offical diagnosis of mast cell disease...but not because I was upset or scared or worried (at least at the time =P). No, I cried that night out of thankfulness and joy and relief. Did I WANT something to be wrong? Of course not. But the reality was that we knew things were not right, and up until that point we had no explanation for why. The diagnosis put a name to the years of sickness and challenges. It was a moment where we could stop fighting for doctors to listen because we actually had proof that there was something wrong. There have been many, many nights since then that the tears I have cried HAVE been in fear and exhaustion and pain and discouragement...but the night I was diagnosed...we were thankful.

And we still are.

Having the diagnosis has made all the difference in the world in terms of treatment. I know, if you've been reading these blogs or following on Instagram you're probably thinking I'm crazy! I'm sicker than I've ever been. Yes, unfortunately that is my reality right now. But having an actual diagnosis has turned the tables completely.

When I see new doctors, I'm a medically complex rare disease patient. I'm NOT some random girl with all sorts of seemingly unrelated symptoms and odd complaints. My treatment protocols are now viewed as medically necessary for a patient with a severe systemic disease instead of being weird requests by some girl who is clearly just anxious and/or depressed. What used to be a fight just to be heard became an open line of communication, all because of 4 simple words...mast cell activation syndrome.

Doesn't mean there are all of a sudden amazing treatment options available (as we are unfortunately all too aware of...), but it means that (most of the time) doctors actually LISTEN. Still doesn't mean that every single doctor is now perfect and on board with the treatment I need, but it's no longer a fight to be heard. Having a diagnosis gives rare disease patients a platform to stand on. It gives us back our voice in a medical field that is SUPPOSED to be patient oriented. And being heard is often the most important piece of healthcare.

Rare Disease Day falls on the last day of February each year. This year's theme is RESEARCH. Without research the rare disease community crumbles. It is what keeps us going. It allows us to hope that one day there WILL be treatments and cures for mast cell disease and the other 7000 known rare diseases. It is because of Dr. Afrin's research that I have this diagnosis that has changed my life. It is because of him and others like him that those of us living with mast cell diseases are able to keep fighting what often feels like a losing battle. We can have hope that eventually, if we can hold on long enough, a breakthrough that could change our lives again will be found.

One of the reasons I began sharing my story of living with mast cell disease is because I don't want another girl and her family to have to go down the same road that we did. Eight years before a diagnosis is just unacceptable. I do wonder at times if we had known sooner, could we have kept things from getting as bad as they are...maybe, maybe not...there's no guarantees with mast cell disease, but I can't help but think that if we could have started treating the snowballing symptoms before they were as out of hand as they were, then maybe my body wouldn't be so worn down...maybe I wouldn't be as sick as I am. Obviously we'll never know the answer to that at least for me...but maybe me sharing what I went through means another girl and her family can get a diagnosis sooner...and maybe that will make all the difference in the world for them.

This certainly is not how I imagined life would look when we started down this long road of medical issues 10 years ago, but when does life ever turn out exactly as we plan? I might not get a say in what my life with mast cell disease is going to look like in the future, but I do get to choose HOW I'm going to live with it. The mast cell diagnosis changed my life and my family's life, no two ways about it. We pray and hope for research breakthroughs and medical advances that may give temporary healing in this life. We do what we can as a part of the rare disease community to raise awareness and support the doctors that sacrifice so much of their own lives for what are often thankless endeavors. We live as best we can with what we have, and remember that life can still be beautiful even in the midst of sorrow.

Just because life has changed, doesn't mean that it's all bad or that we have to give in to the despair that can be so easy to fall into if we place our focus on the things of this world. We're choosing joy, finding reasons to smile every day, and remembering that ultimately our hope is in Christ and not anything in this world.

Two years down, a lifetime to go.

Wednesday, January 18, 2017

Keep On Keeping On

January is not slowing down one bit. I clearly was mistaken when I thought that whirlwind of a week in December was going to be an isolated week of craziness and that things would slow down once it was over. You'd think by now I'd know better...

I've been out of the hospital a week now and only wished I was back in 2 or 3 times. Yeah, you read that right. My dislike for being in the hospital is often only outranked by my hatred for my mast cells...and while I did have fantastic nurses and good doctors this go round, the fact that I actually wished I was back in gives you a glimpse of how rough things have been.

To put things a bit more in context, let me explain a bit.

As I'm sure you've picked up on over the past few weeks/months as things have been falling apart again, my GI tract is one of the biggest offenders at the moment. This takes many different angles, but the important one here is my lack of absorption. When my GI tract gets as inflamed and irritated as it is right now, my oral meds come out undigested, my liquid meds likely run straight through me, and my tube feeds are barely able to get me any nutrition as they rush out faster than they went in. This means that not only am I dealing with GI pain, nausea, vomiting, and the like, but I'm also dealing with the fall out of not absorbing any of my mast cell medications. Not a good combination...

That is part of the reason I ended up in the hospital last week. (The other part being severely low potassium and severe dehydration.) While I was admitted, I got IV medications around the clock to keep my mast cells under control with other IV meds available as needed for breakthrough symptoms (nausea, pain, etc.). The second night I was home, I was having some of the worst GI pain I've had in a long time. I was curled up on the floor outside the bathroom asking Nick through tears if it was "bad that I wish I was still in the hospital?"

And unfortunately, that's still where we're sitting. We did end up having to go back in to the ER once since I've been discharged (for GI issues), so that can give you an indication of how much better things are right now. (Please note the sarcasm that sentence should have been read with.) The next 2 weeks I have a few appointments coming up that we are praying will give us some answers. I see my allergist and get my first official Xolair of 2017 this week. I have some big follow-up questions to discuss with him at that appointment, so please be praying that he will listen and be willing to consider options that are not what he may be familiar with.

Because of how rare mast cell diseases are and the fact that I have gotten significantly worse in a very short amount of time, we are in a bit of uncharted territory for both me and my doctors. My allergist is typically very willing to listen and recommend different options, but like most doctors, prefers to stay in his comfort zone. I am already out of his comfort zone. Now some of the treatment options that I very desperately need are also outside that comfort zone of what he is used to and familiar with prescribing. Please pray that he will be receptive to my needs and consider the recommendations of both Dr. Afrin and the doctors from when I was in the hospital last week.

Tomorrow I have an emergency consult with one of the GI surgeons at the Cleveland Clinic to resolve the Herman issue. The appointment was scheduled such that we will either leave with Herman fixed and working or I will leave with Herman V. I am very nervous. As I have said many times on here, anesthesia and I do NOT get along. I am mentally preparing myself for the possibility that I will have to be put under on Thursday, but the not knowing is difficult. Please be praying for wisdom for us as we will have to make some big decisions very quickly. Pray that the doctor will be confident in what we need to do and willing to listen to the issues I have been having. Pray for safety during any procedures that may need to be done.

Next week I will be seeing my functional medicine doctor and neurologist, and a few weeks after that, I am scheduled to see another GI doctor at main campus. I am hopeful that with all these appointments, some progress will be made toward getting me back to my baseline. At the same time, I often feel like I am just shuffled between doctors because no one seems to know what to do and we get even fewer answers despite having more specialists. I feel like I'm starting a collection...I used to collect key chains, now I collect doctors! Haha, so fun...

But what can you do, right? We keep on keeping on. ALL of us. We all have stuff going on in our lives. I'm not the only person struggling right now. Your struggle may not be with your mast cells, but that doesn't make it any less difficult or exhausting. I think we get caught up in the trap of our culture where if our perceived challenges aren't as great as someone else's appear to be then ours are not true. Even an 8 oz. glass of water will eventually be too heavy for the strongest man in the world if he holds it out long enough. We are all subject to the brokenness of this world and our lives here on earth will forever be a mess because of that.

If right now, you're going through a tough time, too, then take a moment and smile. Because I'm cheering for you. God is cheering for you. He wants to walk with you through whatever it is you're going through. He knows. He gets it. He stepped out of heaven and came here just so when He whispers, "I know. I understand." He could mean it. Smile because your hope is not in this world. It's in the hope of heaven that we have because of Jesus. Maybe you're smiling through tears...I know I have plenty of times, especially in these past few weeks...but there is beauty in trials, because we know that ultimately, we're going to make it out okay.

Life is tough right now. But I'm doing all I can do...and that's hold onto God and keep on keeping on.

Thursday, January 12, 2017

January Whirlwind

January...time for resolutions and starting off the new year right...I'm not sure my mast cells got the message...

In the first 10 days of 2017, I have not just hit my deductible for the year, but also my out-of-pocket maximum. Yay?!?! Thanks mast cells...thanks...

To be fair, the ER trip on New Year's Day was NOT my mast cells fault. It was because Herman IV has been causing issues for the entire 3 weeks he had been in. Although if we're REALLY getting down to the nitty gritty, I wouldn't even have Herman if it weren't for my mast cells, so I guess my mast cells can't get out of this one either.

A few days later, I had the wonderful first time experience of calling 911 for myself when I went into anaphylaxis while I was home alone. While I do appreciate my mast cells helping me experience new, no I do not appreciate more new experiences, at least of the mast cell variety...I am experienced out when it comes to my mast cells...On the bright side, I only needed 1 EpiPen this time, and most of the EMS squad knows me by now so they know what to do. Small blessings, right?

Then just 2 days later Nick and I took what we thought would be a relatively straight forward date night for some fluids and IV meds...Instead, I got admitted due to extremely low potassium...and that's where things have been this past week.

Thankfully after 6 days and A LOT of IV potassium, my potassium was back within the normal range. I've also got IV meds around the clock to manage my mast cell symptoms which also helped me feel better as I'm not absorbing my oral meds well at home. Unfortunately, when we were in the ER, guess who decided to clog yet again? Since I was conveniently already being admitted, GI was called to see if we could figure out what has been going on. I had a CT scan over the weekend that didn't give too much info, so I had a gastrograph on Tuesday. That also didn't give us any useful information, so for now, the Herman issue is still up in the air.

THEN, (oh yes, don't stop reading now, the plot is thickening) we found out the some cultures that were collected had started growing gram+ bacteria. So I appeared to have an infection even though I was asymptomatic...for an infection at least, I still had all the wonderful mast cell symptoms to enjoy. Thankfully, the doctor was willing to wait to start any antibiotics until we knew exactly what was growing since I have such bad reactions to medications. That ended up being a huge blessing because the next day, it turned out that the cultures were contaminated and I didn't actually have an infection. So glad we didn't just start antibiotics immediately! Again, small blessings =)

By Wednesday, we had gotten most of the issues I had been admitted for under control so we started talking discharge. Then my morning blood work came back showing low many times does that make it?? Way too I ended up getting another 2 rounds of IV potassium. Plus my blood sugars started crashing...even after a few cartons of apple juice, my sugar still wouldn't come up, so I ended up needing IV dextrose. My sugar crashed again in the evening while we were waiting for the GI doctor to come around, and 4 cartons of apple juice wouldn't make it budge, so more IV dextrose it was.

The GI doctor finally came by around 8 pm. Unfortunately the reality is that there are no easy answers right now. Because my GI symptoms are mast cell related and not a primary GI disorder, there isn't much this doctor felt that he could do to help. The problem is that right now, because I'm not absorbing my meds, we're not actually able to tell if any of the new treatments we're trying for my mast cell disease are working since a lot of my meds and passing through undigested. I'm stuck in between GI not knowing what to do and my allergist offering suggestions that only work if they get's a vicious circle and we're not entirely sure what the answer is.

He did suggest a few options from a GI perspective to maybe deal with some of the ongoing tube problems I've been having, so we have some things to think about going forward. He also gave me a prescription for some medications that may help with some of the absorption issues so that could be good if it works. Thankfully, even though it was after 9 pm by the time he left my room, I was still able to be discharged! I am so glad to be home and hope to stay here for a good while!

In other news, my Xolair was reapproved for 2017!!! We are so thankful that not only was it reapproved, but that it happened in time for me to hopefully not miss a dose. Although when I was on the phone talking with the insurance rep about co-insurance until I've hit my deductible and out-of-pocket maximum, he looked at all the claims and then said it shouldn't be an issue. I guess if I'm going to have to meet all the insurance maximums, doing it within the first week of the year makes the rest of the year smooth sailing...small blessings, remember??

I have an appointment with my allergist on Monday and will also be getting my first official Xolair for 2017. I also have appointments with my functional medicine doctor and neurologist coming up before the end of the month. In February I am scheduled to see a new GI doctor since the doctor who changed my tube in December left a week later to run the GI department at the Cleveland Clinic campus in Abu Daubi...yes, you read that right...I couldn't make this up if I tried! Never a dull moment in Bokatopia!

Welcome to 2017...

Wednesday, January 4, 2017

My Year of VICTORY

The last few years, I've done a year end review and also written a post about what I learned from my OneWord for that year. This year, both are so closely intertwined that I decided to just write one post to cover both.

If you're not familiar with the OneWord Challenge, it is where instead of making a long list of New Year's Resolutions, you choose a single word to focus on for the year. I've done this for a number of years now, and if you're curious, you can go back to January of the previous few years and read about the words I chose. Last year (2016), my OneWord was VICTORY.

I don't choose these words lightly. I typically spend time at the end of the previous year praying and asking God what is something He wants me to focus on for the upcoming year. I remember last time when I really felt like God was putting VICTORY on my heart...I was in the middle of a very rough patch, and I really didn't understand how I could pick a word like that when I was struggling so much...

Then the year started off with me being admitted to the hospital...and I REALLY didn't understand how I was supposed to focus on victory when my health was worse than it had ever been. And now that I'm back to feeling so rough, it can be hard to focus on how 2016 was a year of VICTORY.

But it was...oh, it SO was...

I went from lying in a hospital bed in January to running by April. I was struggling to tolerate my tube feeds during that admission at the beginning of the year, yet in September and October I was able to eat real food while I was in Disney and Yosemite. From being mostly home bound to traveling all over the country. Yes, this year was absolutely a year of VICTORY.

But even more than the physical victories that I saw throughout this year, I've learned that victory is not always what we expect.

Sometimes VICTORY is big and exciting. It's finally getting Xolair approved after months of fighting with insurance. It's eating my first solid food meal in over a year. It's taking that first run on my own. It's taking our first flight in years to a family wedding. It's being able to enjoy Disney. It's taking our first vacation as a couple to the other side of the country. It's regaining enough balance and coordination to hike around Yosemite. Yes, sometimes we see VICTORY in these huge moments that stand out, change the course of our year, and are remembered long after they are over.

But sometimes, VICTORY looks a little different. Sometimes it's the quietness of regular life. It's the days, weeks, and months lived without our lives revolving around ER trips and hospitals. It's Nick coming home after a day of work to a home cooked meal. It's sleeping through the night. It's being able to go to Bible study a couple of weeks in a row. It's getting "stuck" in our routines because we have the luxury of normality.

This year has been a year of so much VICTORY. We had the exciting moments and the quiet ones. We've had adventures that would never have been possible in 2015. We've smiled through buckets of tears and laughed until I couldn't breathe (literally...). We celebrated some of the best health that I've had in a few years. And we are SO GRATEFUL for every moment of VICTORY, both big and small.

But even in the midst of a year of overwhelming VICTORY,  we've also had some tough, tough moments. I remember crying when not even a week into 2016, I was admitted for uncontrolled mast cell issues with no answers. I had no idea what this year was going to look like and was so discouraged. I definitely did not feel very victorious going in to day 4 in the hospital with no direction.

These past two months has also challenged this year of VICTORY as I have had a drastic increase in symptoms with no known cause. Just in the last 2 weeks of the year, I dealt with more anaphylaxis, procedures, and new devices in my body than I would have ever anticipated. Even after all the VICTORY of the past 9 months, suddenly I'm not feeling so victorious...

But what an incredible reflection of our VICTORY in Christ. It's not always linear and certainly not always smooth. Christ had to die before He saw His victory over death, why should we expect to achieve victory in our own lives without a struggle?

This year I used more EpiPens than I ever have in a year. I've had 2 tube changes, been admitted 3 times, and had surgery for a central line. I regained the ability to run and eat only to lose both AGAIN just a few short months later. In some ways, this was one of the toughest years I've ever had with my mast cell disease.

But at the same time, I also had months of better health than I ever could have dreamed of the same time last year. I traveled to MN twice, spent time with family in WI, flew to TX for a wedding, enjoyed Disney with my mom in FL, took an actual vacation with Nick to CA. I spent months being able to actually EAT. I was able to run 6+ miles again. Right now, as I'm struggling so much just like I was at the beginning of 2016, even I can't forget how much VICTORY I had last year.

So even though this year didn't finish the way I would have liked, I am so thankful for every moment of victory I had. And I'm also thankful for the tough moments, tears, and frustration. Without those moments, victory would not have felt nearly as victorious. Even though I felt like I would crumble under the weight of the struggle at times, I am thankful.

And now we're in yet another new year and my year of victory is over. If I choose to focus on how the year ended, then it would be easy to get discouraged and feel like this was just another year of life with mast cell disease and the fallout that brings. But instead, if I choose to focus on what God put on my heart as my theme for this year, I can finish 2016 KNOWING I had a year of VICTORY. Maybe sometimes that victory looked different than *I* might have chosen, but it was also full of victorious moments that were beyond anything *I* could have imagined.

As I have said many times, not just this year, but throughout this crazy ongoing season of life, thank you for praying for me and being a part of this year of victory! I know that there are many moments, often during the tough times, that I can feel the weight of the world but also the power of so many prayers being offered for me. It is partially due to those prayers and the support of so many friends and family that as I wrap up another OneWord Challenge and reflect on the year, I can say with complete confidence, that yes, 2016 was absolute a year of VICTORY!

Monday, January 2, 2017

Christmas Whirlwind

When will I learn that life with mast cell disease doesn't ever work the way we think it will??? I apparently naively thought that once my crazy week of tube change, trip to MN, Xolair, and port surgery was over that things might calm mast cells had one thing to say to that...NOT GONNA HAPPEN!!!!

I had planned to spend the rest of the week at home recovering from surgery, and I did for the most part. Until Friday at least...

Since this particular flare started, I've having episodes of massive GI emptying that is leaving me seriously dehydrated with electrolyte abnormalities and the inability to remain vertical for any length of time. As you may know, I pass out often. Rule #1 in our family is Don't Pass Out! When I'm having these episodes, however, my tolerance for the upright position decreases to about 60 seconds...barely enough time to get from the bed to the bathroom.

When this happens, we are ending up in the ER for fluids, often electrolytes, and making sure I'm not having any indication of a bad mast cell reaction since my oral and liquid meds are not being absorbed during these episodes (or at all, but that's another issue...). And that is where this whirlwind of 24 hours started.

On Friday the 23rd, I was having one of the roughest days I've had in a while. My standing tolerance was only about 45 seconds and even sitting up was causing symptoms. When Nick got home from work, we headed out for a Boka style date night.

The best part about this date night was that it was our first chance to test out Mort! I am so thankful to be able to share that he worked perfectly! I actually expected it to hurt more getting accessed because I was still sore from surgery, but it wasn't bad at all. Plus, once the needle was in, that was it! Blood return was great for collecting labs, and we didn't have to worry about how much blood the nurse could get before it stopped flowing, especially with how dehydrated I was. I got hooked up to fluids and was given my meds all through Mort and there was no worry I would blow the IV or anything. I can definitely say that having Mort was great!

When my blood work came back, my potassium was low (again). We had just been in the ER the previous Sunday and my potassium had been low. On Friday it was considerably lower, even after having it treated on Sunday...and this time it was so low that it was going to take about 8 hours of potassium infusions to get it back to normal. When my nurse told us that, we knew I was going to be admitted. The ER doctor said if they ran potassium all night, hopefully by morning it would be back to normal, and I could be discharged. Sounds simple, right??

Nick and I figured that since this was a simple straight-forward issue, being admitted to the tiny, local hospital would be okay even though we avoid it for any of my normal issues. If there was a lesson to be learned in all this craziness, it is that with mast cell disease, NOTHING is ever simple. There were some extenuating circumstances that made things worse than they might have been on a regular day, but the bottom line is that this hospital is just not set up for complex cases, and even though it should have been a straight-forward fix, mast cell disease adds another level of complexity even to something as easy as raising potassium levels.

I was able to receive 4 hours worth of potassium in the ER before being admitted along with a bag of fluids and a couple rounds of IV medications. It was about 1:30 in the morning before I got taken to my room, then the nurse had to run down her whole admission checklist. I just wanted to get the next round of potassium going so I could be discharged early-ish the next day (or technically the same day now), but my nurse said the admitting doctor hadn't ordered anything yet so she had to wait for the orders.

It was close to 3 am before Nick and I were finally able to get some sleep, only to be woken about 5 am for bloodwork. At that point, my nurse told us the doctor wasn't going to order anything until he saw the morning labs and came by to see me during his rounds. In addition, the pharmacy didn't carry half my meds (big surprise...) so Nick had to run home to bring my meds from home for the pharmacy to approve before I could take anything. Plus, the doctor refused to even have benadryl or pepcid as PRN orders (in case of a mast cell reaction) until he came to see me. Thankfully it had only been a few hours since my last ER dose, so we figured I'd be okay for a bit until things got straightened out.

The doctor came around about 10 am, and I'll be honest, I was very frustrated by this point. NOTHING had been given to me since the ER, and it was Christmas Eve...if they weren't going to do anything I did NOT need to be sitting in the hospital. Thankfully, the doctor ended up being pretty good and there had been some miscommunication from the ER so he didn't realize how low my potassium had been. Sure enough, when he looked at my morning labs, my potassium was still very low. He was going to order 2 more rounds of IV potassium, but we asked if we could try one IV and one down Herman so that we could leave sooner since each IV dose takes 2 hours. Plus we asked that if my potassium was still low if I could be discharged with a prescription for more down my tube at home since that is what the ER had done last week. He wasn't thrilled with the idea, but agreed probably because it was Christmas Eve.

And that's where things started getting fun...when the nurse came in to hang the IV potassium and give me the liquid for my tube, we discovered that Herman was clogged...very badly...Both the nurse and I worked to get fluid down and it was NOT happening. Eventually, she had to go page the doctor (who had gone home by now) and tell the resident. The resident put in a consult with GI, and said that I had no choice but to do the second IV dose at this point. We knew he was right, but it was still frustrating because it meant another 2 hours before I could be discharged.

Meanwhile, it's now almost noon and the pharmacy had still not yet approved my medications. The benefit I had gotten from the IV meds in the ER was all but gone, and I was starting to have more and more symptoms. Compounding my reactivity was the fact that we were pushing large amounts of Coke and Ginger Ale down Herman trying to unclog him, both of which make me sick. We asked the nurse multiple times for both my meds and to have IV meds ordered (since I couldn't do any of my liquids down my tube) but the resident said I couldn't take anything until he came to see me...

It finally got to the point where we explained to the nurse that I was in the beginning stages of a mast cell reaction and if I didn't get benadryl and pepcid, I could very well end up in anaphylaxis. To my nurse's credit, she went back to the resident and basically told him I needed the meds NOW and couldn't wait. Seriously, nurses have kept me safe so many times when doctors can't be bothered! I was able to get the IV meds I needed and about half an hour later, the rest of my oral meds, so we were able to avoid a severe reaction.

My 2nd dose of IV potassium finished about 3:30 pm, and then we had to wait 2 more hours before they could draw the labs. In the meantime, we kept trying to get Herman to unclog. We had moments of thinking we had a breakthrough only to have the next syringe be just as tough as before. Plus we kept asking to see the resident who still hadn't been in to see me by 5:30 pm when the nurse came for the blood work.

It was almost 6:30 pm before we saw the resident. My blood work still hadn't come back, so we were discussing what had happened so far. He asked about my tube and said he had put in for a consult with GI. When we said we hadn't seen anyone from GI, he said that just meant he must have gone home already and would come by during his rounds on Monday...yes, Monday. There was no way I was going to spend Christmas in the hospital to wait for a GI doctor who had no idea of anything to do with my care...if I was going to be without nutrition either way, I didn't see any reason to be sitting in the hospital when nothing was going to be done on Christmas. Unfortunately, the resident didn't seem to agree...He said we could discuss it again when my blood work came back.

And then my blood work came back...Remember how the ER said it was going to take about 8 hours of infusions to get my potassium back to normal? Well, after all that, my potassium had only come up half as far as it should have...I thought we were for sure spending Christmas in the hospital...But the resident surprised us!

By this point, we could sometimes get a little bit down my tube if we held the syringe a certain way and pushed really hard (and ignored all the liquid that oozed out onto our hands and the bed...). The resident said that if we could get another 40 meqs of liquid potassium down Herman, he would discharge me with a prescription for more potassium and would be satisfied that my tube would be okay until Monday. Challenge accepted!

I figured we were pretty much home free because I was determined to make my tube work whether or not it wanted to. While my nurse went to get the potassium to put down Herman, the nurse tech came in to take another round of vitals. And she was wearing a scented lotion...I immediately started to react, Leni came over and alerted, and Nick went to find the nurse to get more IV benadryl. Thankfully we were able to keep my reaction under control which was good because I thought that had just sealed my fate to spend Christmas in the hospital.

Once we got my reaction under control, my nurse gave me the potassium that I had to get down Herman to convince the resident I could go home. To be completely honest, I think more liquid ended up on my hands and the bed than down my tube, but it was enough to convince him! He was probably getting just as frustrated having me in the hospital as I was being there! We finally got discharged about 9 pm on Christmas about a Christmas miracle!

As soon as we left, we headed home to quickly pack for our trip to VA to visit my family. We spent the night and a few hours Christmas morning with Nick's family, then drove to VA to spend this week with my parents. And that is how we spend our holidays in Bokatopia! Talk about a whirlwind!