Monday, March 28, 2016

Update Victory 2016

If you remember, I chose VICTORY as my OneWord for 2016. I went in to this year not knowing what it would look like, but choosing to focus on ways that God would bring about healing and growth in my life whether physical, spiritual, or otherwise. Since I have been seeing glimpses of victory, I thought I would share this along with my regular health updates.

First VICTORY update: FOOD!

If you've found me on instagram, you saw this earlier this week. If not (Look me up! kboka27 =P), here's the big news...I ate* my first "actual meal" in a long time this past week!

* Indicates the successful ingestion of food in an amount to be considered a small meal without any adverse effects including but not limited to nausea, vomiting, abdominal pain, distension, mast cell activity, anaphylaxis, etc.

WHAT. A. BLESSING!

The past few weeks I have eaten some soft foods, fruit, and soups, but carefully, in small amounts. I've had a few bites of actual solid food, but not actually sat down with the intention of eating that as my meal...until Friday. Yay! 

I get my second Xolair injections on Tuesday (hopefully, please pray for on-time shipping!), so we are hoping that in the weeks to come I will be able to continue to add foods back into my diet. Tortilla chips, I'm looking at you! 

Second VICTORY update: WALKING!

If you remember, back in the fall I was having severe balance and coordination issues. It started in July and progressed until I was almost unable to walk unassisted on flat surfaces and had to use a wheelchair for long distances. 

Since this isn't a health update, I'm going to skip over all the medical details, those are in previous posts and I'll address that more fully in the future as well. Instead, I'm going to share photos from Easter Sunday that should speak for themselves.



Yes, that is Nick and me (and Leni and Bonk) on a HIKE! I've been in physical therapy since November working on strengthening, balance, and coordination. It has been an up and down road, and things are not yet back to my "normal," but it is such an incredible improvement from the fall.

I'm still a ways out from signing up for a race, still have not run yet, but it is so incredible to see how far I have come in just a few months. As we continue stabilizing my blood sugars, I continue physical therapy, and who knows what else we'll try or need to do, we are hoping that things will continue to improve.

So that's my VICTORY update for the first 3 months of 2016. Even being the one that this is happening to and living this in my life, just writing this post is making me pause and just sit in awe of what God has done (and is still doing) in my life just in the past few months...such a change from where I was a year ago or even just 6 months ago. After starting this year with a week in the hospital, I did not at all expect to be writing this post 3 months later. 

Life is still tough. I am not "healed" in the modern medicine view of the word. I still have mast cell disease. I still have daily symptoms and receive nutrition through a feeding tube. My time is still consumed with doctors appointments and insurance phone calls.

At the same time, though, I can 100% say that God is bringing about victory in my life. I don't know how long it will last...the Xolair may not keep working, we may discover that there is something else causing my balance/coordination issues, my mast cell disease could get 10xs worse...we just don't know. But we're choosing to focus on the victory right now, not the what-ifs.

On Easter we celebrated God's ultimate victory over death and sin, and it is because of Christ's victory that we can celebrate this victory in our lives and have hope for complete victory over pain and sickness and suffering in heaven for eternity. Thank you for being with us as we are thanking God for this victory. 

Wednesday, March 23, 2016

Quick Health Update

My last update was basically a post to tell you that I had no news yet. We've started getting some of the results of the recent testing, so I figured now was a good time for an update again.

First, the results of Sebastian (the 2-week heart monitor). We are very thankful that the 2-week monitor showed that everything was pretty much normal! There were a few premature beats and some sinus arrythmias, but apparently that is considered normal in young people (I'm normal?!?!) so my doctor is not concerned. We do not have to discuss changing any medications (praise God!) and more importantly, I do not need to see another heart specialist for any further testing, at least at this point. This was a big weight off knowing that we can at least cross something off the list of possible issues. As I'm mentioned before, while mast cell disease is systemic and can impact every body system, that doesn't mean it can't cause other disease-like processes in various regions of the body. We are very thankful that it appears that my heart is healthy and this is one system that we can check off as having ruled out any other underlying pathology. HUGE praise, so thank you for your prayers!

The next update is regarding the endocrine testing I had done a week or so ago and my appointment yesterday with my endocrinologist. The results of the cortisol stimulation test are in and, while my body apparently is not a fan of the test, my response to the stimulation was good. However, my baseline cortisol was low. My doctor thinks there may be some slight adrenal insufficiency, but it's hard to know if it is just mast cell involvement presenting that way or actual adrenal issues. For the time being, we are not treating this. Treatment for adrenal issues is something that cannot be stopped once it is started, ever. Basically, I'd be on it the rest of my life. Until we know for sure that it is actually an adrenal issue that will not reverse rather than mast cell involvement, we are going to hold off treating. Yay for a doctor who doesn't just immediately push treatment!

The next thing we discussed at length was my blood sugar. I've mentioned that I've been dealing with fairly low sugars pretty often. It was baffling us why this was happening when I've been on continuous tube feedings. Then about 2 weeks ago I started noticing a few high sugars...at weird/random times. They would go up when I hadn't eaten, I would eat and they would go down. I'd wake up high after not eating or running feeds since 6:30 the night before one day and the next I'd wake up low. The conclusion my doctor came to at my appointment: my body is weird and no one really understands it.

Only partly joking...

Unfortunately, the reality is that we don't really understand my body. My endocrinologist thinks that what is going on is that I'm dealing with what they call "pancreatic burnout." She said it is common for anyone on continuous tube feedings for any length of time, and my mast cell disease is probably just complicating things. When I started the tube feeds, I started getting nutrition constantly for hours at a time. That causes my pancreas to respond the way it would during/after a normal meal. Except since my "meals" don't stop, neither does my pancreas. There is something of a "honeymoon" period where my body was able to compensate and keep up with my tube feeds, but after 9-10 months, the pancreas just gets burnt out. The erratic blood sugars are likely a result of the pancreas getting behind, then overcompensating, then falling back again, and I get this yo-yo of highs and lows.

The good news is that the pancreas can recover from this burnout. The not-as-good news is that this probably won't happen until Herman is removed. While that is something we hope for down the road, I'm not there yet. In the meantime, we will be trying some medications that help stabilize blood sugars to see if we can keep things from bouncing back and forth as drastically. We will need to regularly keep an eye on my sugars because my A1C (measure of long term blood sugar control) is pretty low. That indicates that over the past few months, my sugars are hanging out kind of low. Low sugars are a bit more dangerous in and of themselves than high sugars, so we are hoping that this medication will help get my sugars a bit more than they have been recently.

Last health update is about the Xolair. I am officially eligible for the next set of injections as of this week. We are working on scheduling those soon. I would say that the effects do seem to have worn off some, although, I am still better than I was before we started the injections. Hopefully this next round will continue the improvement and the side effects will be less!

So that's what's up recently. Never a dull moment in Bokatopia! Ups, downs, and everything in between. In other news, our ER counter has officially been reset as of last Sunday. Over 2 months without a date night, I think that is a new record! Everything is okay, so here's to another couple of months OUT of hospitals!

Tuesday, March 22, 2016

Leni Goes to the Airport

This weekend Leni and I had a unique opportunity thanks to our friend, Charlie Wentz, and his connections at the local small airport. Nick and I have a couple of trips planned for later this year that will require flying to get to our destinations. While Leni has been on the DC metro, a couple of boat rides, and is great in the car, she has never been on or around airplanes. Nothing much seems to phase her, but we still wanted to at least expose her to planes and airports prior to actually having to fly.

I met Charlie at Kent State a few years ago and remember him sharing about his experience as a flight instructor in the aviation program at Kent. He still has connections at the Kent State Airport, and called one of his colleagues who graciously agreed to allow me and Leni to stop by for some training.

When I got to the airport, I actually didn't know where I was supposed to go. I knew the name of the instructor who was going to show us around, but that was about it. When I walked in, though, he immediately introduced himself to me. I realized that while I didn't know who he was, I'm a bit more identifiable with Leni! He might not have known what I looked like, but knowing you're meeting up with someone with a service dog probably narrows the field considerably =P

Leni got the chance to jump into one of the 4-seater planes and sniff around a bit. We were able to practice some of her commands in the tiny space, and she was able to perform her "tuck" command (down stay, but with her legs, tail, and nose tucked into a nice ball) comfortably. The space we were working in is most likely much smaller than we will have on a commercial flight, so that gave me confidence that she will be fine if she was great in such tight spaces.

After we finished our short training session in the plane and Leni was done sniffing around, we went over to what was called the ramp. It was basically the dock for the airplanes. The planes were parked there and would be returned to their spot after the flight was over. We got to watch a bunch of planes start up, taxi, take off, land, return to the ramp, turn around, park, etc.

At first Leni was definitely a bit unsure. The small planes are very loud, especially when we were standing out on the ramp. Once the propellers started up, it was hard to hear anything else. They were much louder than a commercial airplane, as well, so knowing that she was okay with these noises also gives me confidence that she will be fine on a big plane. After a few planes came and went, she lay down, tucked her nose, and started dozing. I'd say Project Desensitize Leni to Airplanes was a success!

The only parts of the flight that are still unknown to Leni are take off and landing. We will just have to cross that bridge when we get there =) Once we are at cruising altitude, I'm pretty confident that she will be fine since she is so good in the car. Take off may be a bit unsettling for her, but hopefully with some treats and a toy, she can be redirected until things even out. I'm not too worried about landing since even if she does get anxious, the flight is just about over and we'll be off pretty quickly after.

Fun fact: Dog ear canal anatomy is different from human ear canals. I'm sure there are lots of implications for that, but the important part for this blog is that their different anatomy means dogs do not experience the ear popping sensation that humans get on planes. Their ears adapt to pressure changes differently so they don't experience that discomfort that we often get on flights.

Leni and I had a great time on Saturday, and this was a great training opportunity for Leni! Thank you so much to Charlie and the Kent State Airport for making this possible.

Friday, March 18, 2016

A Day in the Life of a Service Dog

Mom?

You moved...is it time to get up yet? Food? Outside? Maybe just cuddles?

Oh, wait, you walked away...okay, I'll just wait here and watch for you.

You're back! You look like you're getting ready to leave again, do I get to come now? Please?

Yes! Yes! Yes! What are we doing? Food time maybe? Or we could go outside and run around? Or...or you could get on your bike...yep, that's okay, too. I'll just lay down here so I can keep my eye on you.

Did you hear that? I think someone downstairs is moving around! Is it outside time yet? No, oh, okay. I'll come back and...DINOSAUR! Yes, I think it is time to remove some more of his innards. I might even decorate the floor with said innards. Look, Mom! Do you see my decorating skills?

Nom nom nom. Okay, I better stop now or there will be no dino innards for tomorrow. Bone time! Which one? What do you think, Mom? Antler or rubber bone? So hard...maybe I'll take both, yes, I think that is the perfect solution. I'll just curl up here on the couch with both my bones and watch you finish biking.

What? Did you call me? Maybe I'll just look at you first and make sure you're actually calling me. After all, I don't want to get up from my comfy spot if you don't really need me. Oh, you mean it.

What was that? Pick up your phone? Okay, here you go. Oops, dropped it, sorry, Mom, but you should have grabbed it before I let go. What? Now you're making me pick it up AGAIN? You're the one that let go...but I'll do it for you because I am a good service dog. Do you see my tail wagging, I love helping.

Can I eat now?

BONK! AND DADDY! Yay! Mom, Mom, guess what, it's morning! Food time and play time and work time and YAY! Hi Daddy! So excited to see you this morning! Good morning Bonky! Do you want to play now? Here's Dinosaur! Or we can play with turtle! Or hedgehog! Oh wait, time to go outside! YAY!

Outside, so nice, yay, freedom! Oh wait, bathroom time. Yes, and even better than outside time, FOOD TIME! Okay, done with outside. I'm ready to come in. Upstairs, Bonk, I'll race you! I'm gonna win! Hurry up, Mom! I'm hungry!

What's that you're saying, oh, sit...what's that? I know stand-here-and-look- at-you-since-you-said-something-but-my-brain-is-functioning-too-fast-to-process-what-you-said. Is that not what you said? Oh...I'm not sure I know this sit that you speak of...can I just eat please? No, okay how about this, I know down! No that's no it either...Mom, please, I'm going to starve! Oh right, sit is this weird only my butt is down and I am still standing on my front legs...where do you humans come up with this stuff? Do we do this every food time? We do? Oh, okay, then, but hear my displeasure in my "ahroo-ing." Okay, Mom, I'm looking at you and waiting so patiently. Can I eat now, please please please? Okay? Did you just say okay?

Nom nom nom.

Mom? Mom? Where'd you go? Not in bed, not on your bike...hmm, oh good, I found you! Why do you always close the door to this room? It's like you want some privacy or something. Good thing I'm smart and can open doors. I can't be a good service dog if you won't let me join you. Poodles are water dogs, you know. If you let me join you, I can keep you safe. No, okay, I'll just lie here so I can keep my eye on you.

I have to leave now? What if something happens after you tell me to leave? Okay, if you insist, but I'm not going far, I'll be just outside the door in case you need something to trip over on your way out of the bathroom.

I see you grabbing your stuff for work, does that mean we get to go out? Does it, does it? You know I love to be with you! You're grabbing my vest! Do you see my tail wag so hard when my leash comes out of the basket? Work is my favorite! Can I bring dinosaur today? No, okay. How about hedgehog? Are you sure? Okay.

Ready to go down the stairs? I'm hear if you need to hold on. These stairs are kinda steep. Oh look, Stuey! Maybe he wants to play! Hi Stuey! Want to play chase?

Hey! What was that for? Oh right, sorry, Mom, I forgot you were holding onto my vest and when I run after Stuey it throws you off balance. Are you ready, now? I can walk good across the kitchen and out to the car. See, I know how to service dog like I'm supposed to.

Hey, my BONE! How do I always forget I have a bone in the car? This is the best ever! Look, Mom! Do you see? Do you see I have a bone here? Oh, did I just scratch your ear with my bone? So sorry, here, let me kiss it and make it better.

Where are we? Work? Yay! I like work. It means nice snoozing with intermittent petting and maybe an alert or two. I have a bone here, too, Mom, did you know that?

Do I have to go under your desk? How about I just lie in the middle of the floor here? Does this work? No, okay, I guess I'll go lie on my bed under your desk like you told me. Can I have my bone now, please? Nom nom nom.

Where are we are going? Is it time to leave already? Oh, no just going to drop some papers off for the physical therapists. That's okay, I like that too. Can I say hi to them? They like me, I know they do. I'm the office mascot now, Mom, did you know? They always ask to pet me and ask me how you are doing. They know I take care of you and that I know better than you do what your body is doing. They listen to me more than they listen to you.

Back under your desk, really, Mom? But I just got up. How about I lie over here? No, what about here? Okay, fine, I'll go back under your desk. Oh look! A bone! This is just about the best ever!

Zzzzz...I just love napping. What's that? Mom, are you okay? Let me smell you. Nope, definitely not. This is not how you are supposed to smell. But I know what to do. Here, Mom, pay attention to me again. Do you see my paw in your lap? Time to check your sugar. Now I need my treat because I did my job. Good service dogs always get rewarded right?  Nom nom nom. Here, you can give me pets too. Now my head is in your lap instead.

Good job checking your sugar. See, I told you it was low. I see you eating candy that you don't want to share with me. Why do I not get to join you in your sugar raising when it was because of me you get to eat candy? Let me check you again and make sure you are okay now. Yep, all systems go. Okay, I'll lie back down now. Oh wait, it is time to go yet? No, okay, I'll just be tucked here under your desk.

I see you putting you things away, does that mean we are leaving soon? Nope, looks we're going back to see the physical therapists. Uh oh, I see where this is going...Mom, wait...are you sure? Down stay here? You KNOW this part of the day makes me have to work harder. Yes, I'll lie right here, but don't think I'm taking my eyes off you for a seconds. Balancing on one foot and throwing a ball against a trampoline is NOT an approved activity. Do you remember you used me for balancing coming down the stairs this morning? Now you're TRYING to stay on one foot? Sometimes I do not understand you humans?

Uh oh, do you need me Mom? I see you doing hoppy/jumpy things down a bunch of boxes on the floor. I know you put me in a down stay, but this is seriously not how this service dog thing is supposed to work. How can I help you every time I see you almost fall over when you keep telling me to stay? I think you forget that I am here to keep you safe. Here, I'll just lie with my nose and front paws in the middle of this ladder thingy. Then you have to jump over me every time! That way I get a close look at you and can make sure you're okay.

What's that? You just told you're physical therapist that you're doing okay. Didn't your mom teach you not to lie? Here, maybe this will help. I'll come over and grab your leg with my paw. No more hoppy/jumping until you check your sugar again, Mom. HA, see, you can't ignore me now! Now your physical therapist says no more PT until you check your sugar. I told you they listen to me more than you. Guess what, guess what, guess what, Mom? It's gonna be low...

TOLD YOU! Here, let me place my head in your lap for rewarding pets. Only after I get my yummy snack rewards, of course. Maybe you'll share your candy this time? No, okay...although somehow that doesn't seem fair...but I love you anyway! Do you see my tail wagging again? That's because I know I am a good service dog and taking care of you.

Down-stay AGAIN? Did you miss what just happened? Down-stays and amazing service dogs do not go together. I must be right next to you. Oh look, you're trying to stand on that squishy half-ball thing, can I join you? That looks like fun! We have one of these at home, Mom! At home I like to put dinosaur on top while I am removing his innards. It is much easier that way. Plus then he can still see the TV with me. Aren't I so thoughtful? Oh, I can't join you this time...okay, I'll just hang out over here. But don't think I'm snoozing, oh no, I got my eye on you. I'll keep you safe, don't you worry. Although, sometimes you make this a bit challenging...


Time to go? Yay! I like work, but I like new adventures even more. Hold on, I have something I need to do before we leave. SSSTTTRRREEETTTCCCHHH. Okay, I'm ready now. Oh, did I say I was ready? I meant almost ready. One more good stretch. Okay, Mom, now we can go.

What's that? Take what? I see you pointing to my leash, but I know it is attached to my neck so I am not sure why I would need to take it. Okay, okay, I hear you...This seems pointless...I'm picking up something that is already on me...sometimes I don't understand you, Mom. But I still love to work for you! Do you see my tail wagging so happily because I get to do things for you? Okay, let's go now.

Are we actually leaving, Mom? Because sometimes you do a fake-out and we walk down the hall like we are leaving but we actually turn the other way to the bathroom. You don't seem to want your privacy from me in this bathroom. Those stalls are cramped. But yay! We are turning toward the outside!

Look, Mom! The wind is blowing! Do you see my ears flapping? Do you? I just love sunny days and breezes! I love rain and puddles too, but you don't let me play outside when there are puddles. I don't know why, splashing is just so fun. I try to get you to join in, but you don't seem to be as eager to go splooshing with me.

Hey, Mom, LOOK! I have a bone in here! Did you know I had a bone in the car? This is practically the best day ever! I know I just had a bone to chew all day at work, but THIS bone here, now this one is special.

So, are we going anywhere fun today? I'm up for exploring if you are! No, okay, home is good too. Home means Bonky and Stuey and dinosaur and hedgehog. Yep, home definitely sounds good.

LOOK, IT'S STUEY!! He's in the yard. He must have been waiting for me to get home! Can I play with him, Mom, please? Quick, quick, turn the car off, open my door! Hi Stuey! Oh wait, I can't play yet, Mom says no playing until my vest comes off. I'm listening, Mom! Kind of...can I play, please, please? I did good today, can I play, please?

Yes yes! I heard the snap on my vest. It's coming off! Just a minute Stuey, just a minute! Okay, vest is off, just waiting for my release words. Come on, Mom, come on...Yes, yes, yes, I will most certainly "take a break!" Thanks Mom! Let's go Stuey!

BONKY! Yay! Are you here to play, too? Let's run! Or let's chase a ball! Or let's sniff around! Oh, so nice to be free!

Wait, Mom, where are you going? Why are you going inside without me? I like to play, but I can't do my job if I'm not near you. Come, Stuey, we can play inside. That way I can keep my eye on my Mom at the same time.

Whew! What fun! Thanks, Mom. I just love playing and working and snuggling with you. So please can I come snuggle right now. I see you watching House on TV. You're just sitting on the couch looking lonely with no dog on top of you. That looks like a very sad place to be. Here is my head in your lap. Can I come up? I will snuggle so close to you because I love you.

Oh yes, very nice. Thank you for letting me come up with you. But wait, is that dinosaur I see all alone on the floor? I think he needs to come up on the couch too. I'll go get him. Don't worry, Mom, I'll be right back. See, I'm here just like I promised. And look, dinosaur is not lonely anymore. Can I bring him up too? I can?!?! Oh thank you! Don't worry, I won't remove any of his insides right now. I save that for during your biking in the morning.

Hey, Mom. You're smelling off again. I know my whole front half is in your lap right now, but let me sit up and put just my paw in your lap. Are you paying attention? You're body is about to start not playing nice again. This time it's not your sugar. I see you checking that right now, but you'll see that it's fine right now. No, this time it's your nasty masties as you and Daddy call them. Better go take your sleepy meds that make you write songs for Daddy and fall alseep super early. Do you need me to get them for you? Because I know where they are. I can "go get meds" if you want, then you don't even have to get up. Stay, Mom.

Here you go. It took some time, but now I can "go get meds" like a good service dog. Aren't you so proud of me? Look at my tail wagging! Oh, now you need water. Okay, this one is a bit harder. I know I have to "open fridge" and "take it." But then I get confused. I think I bring the water to you, now, right? Oops, looks like I left the door open on the fridge again. Sorry =( But I still brought you water to take your meds with. And now I'm going to snuggle up with you again. Are you feeling better? No? I'm sorry, Mom. Here, I'll share dinosaur, he always makes me feel better.

Look, Mom, Daddy is home! Are you feeling better yet? Yes! Looks like your meds are working! Did I do a good job? Daddy, daddy, daddy! Guess what we did today? Actually, I don't really remember, but I know that I love you and I'm super excited to see you, and LOOK, I brought you dinosaur! I'm feeling very sharing to share him with both Mom and you all in one day.

Is it time for dinner? Bonk seems very excited and that usually happens at dinner time. I like dinner too, can we eat now? Oh yay, food! You're going to make me sit again aren't you...are you sure I have to? Maybe just down? Okay, okay, look, I'm sitting now. Please can I eat now? Do you see my puppy dog eyes asking so politely? I can? I can! Oh, thank you! Thank you! Nom nom nom.

I'm done now. So what's next? Oh is it time to go downstairs for your evening food? Can we come? Stuey is down there, you know. He might be eagerly waiting for me to play with. I should be polite because you taught me to be a good friend. I think now might be a good time to make sure Stuey is not lonely. He does not have a Bonky like I do to keep him company.

Yay! Downstairs time is my favorite! Stuey, where are you! Do you want to play? I do! Bonk might too, but not as much as I do! We can play here in the kitchen or in the living room or under the table. Wherever is fine, but not too far from my Mom. I have a job to do you know. I can play lots, but I have to keep my eye on her.

Okay, I think I'm tired now. Mom, can I rest my head in your lap? Sometimes it just feels so heavy. Thank you for giving me pets while I recover enough to hold my own head again. I think I'll just lie down right here. Look, Mom! There is even a service dog head rest here. Whoever designed this house must have known I would live here. I can lie anywhere along this step and have an automatic headrest. Yes, good plan.

I see you and Daddy getting ready to go upstairs, is it bed-snuggle time? That's my favorite! Okay, let's go! Bonky, I'll race you up the stairs again! Watch Mom! Watch Dad! Look at how far I can jump! I can jump all the way from the doorway onto your bed! Here, I'll go ahead and choose my snuggle time spot while you guys have to go change clothes. Wouldn't it be nice to be a dog right now?

Wait, Mom, I can't see you anymore...I'd get up, but I saw you walk into that privacy room where you always close the door. Maybe I'll just watch from here. I'll see when you come out. Plus, if I get up now I'll lose my snuggle spot.

MOVE?? That is definitely not fair. Mom, didn't you see, I was on the bed first. Why are you making me move? Besides, I had the foot of the bed last night, too! I don't think I can remember the last time you had the foot of the bed...that doesn't seem fair...But okay, I'll move, because I love you. Can we compromise? I'll let you have the pillows, but then I can come snuggle with you? I do love to snuggle you know.


I CAN! Oh Mom, thank you! This is about the best ever! I'll just flop myself right here. Oh, did I bump Herman? I'm so sorry, Mom! I didn't mean to, but you know, he is a bit intrusive on our snuggling...But here, what if I put my head here? Yes, good. Oh I love you so much! And Daddy, I can reach you with my nose, look. I love you too! And Bonky, don't worry, I didn't forget you. You're my bestest buddy, too.

SIGH, Another day over. It was good, wasn't it Mom? We did lots, and I kept you safe and...why did you stop petting? Don't you know that you can't have snuggle time without pets? Please? Thank you, Mom. I just love having a job.

Oh look, hedgehog! I missed him ALL day. Can he come up, Mom? Please? Yes! Thank you Thank you Thank you! Don't you move, I'll be right back. He is after all, looking so lonely. Hedgehog definitely needs some bed-snuggle time, too.


Mom, psst, Mom, open your eyes. Dad is telling me I have to get off the bed now. Do I have to? Can't I sleep with you tonight? Just this once? No? Okay...but I'm taking hedgehog with me. At least I have a nice blanket bed here right by you. Me and hedgehog, we'll just be right over here. If you need me at all tonight, you let me know. I see your eyes closing again.

Oh no! Dinsoaur! Mom, mom, I left dinosaur all alone in the other room. Can I go get him? He might be lonely. Here, I'll stick my nose on the bed and smush it into your back. Do you know I'm here? Please, Mom? Can I go get dinosaur? No...but...no...okay, guess I'll just lie back down and...HEDGEHOG! Mom, look! I'm sure dinosaur will be fine, and LOOK hedgehog is here. Yes, life is good. I see your eyes closing again, Mom. I love you! Good night.


Thursday, March 17, 2016

A Week With No Doctors?!?!

Can you believe I actually have an entire week with NO doctor appointments scheduled??

Yeah, me neither =P Okay, full disclosure...

I do have a physical therapy session on Thursday and return Sebastian (my heart monitor) on Friday, but actually seeing doctors...NOPE!

What am I going to do with all my free time??

Oh wait, I have an insurance company where the right hand doesn't even know the left hand exists, and if they do ever get to the point of acknowledging the others existence, they apparently don't speak the same language...

So my week has been spent trying to sort out these ongoing issues...I can't be the ONLY person with these problems...

Is it bad that I have a standing appointment in my phone to call my insurance company twice a week because I know SOMETHING will be a mess that we'll have to resolve??

I suppose I shouldn't complain too much, though...after all, they did suggest that I switch my medications to the 90-day mail order so I can save money. How thoughtful, right?

I just got notification that my medications finally shipped...5 weeks after we tried to get this started...a week and a half after I ran out of some of my meds...1 week after we had to go and buy glucose test strips out of pocket to make sure I wasn't having dangerously low blood sugars...

This has been a weird time for me recently. We are thrilled that the Xolair has been making a difference in my symptoms. At the same time, though, I'm having new symptoms that we don't understand. We're not sure if they are completely new or connected to the Xolair or another medication I recently started...or a result of the Xolair wearing off...so much fun! Plus I'm finishing up my 2 week heart monitor and will be finding out the results in another week.

It like I'm finally starting to feel better and now it's my doctors trying to convince us there really still is something wrong instead of us trying to get doctors to listen to how bad things have gotten. Is that a nice switch? Not really sure =P

Anyway, not much to share, just another chance to let you know what's going on in Bokatopia and say thank you (yet again) for your prayers. My next Xolair injections are coming up in the next couple of weeks plus lots of follow-up appointments with my endocrinologist, cardiologist, and my allergist. I also have another GI appointment at the beginning of April (can you believe we're already talking about April?!?).

UPDATE:
I had someone ask me recently how Leni has been doing. I thought about doing a separate post completely, but then I decided to just put this at the end as a reward for anyone who actually reads all the way to the end!

She is doing great! She is a big reason I've only needed epi once since 2015 and we've been able to avoid the ER since January. We are so thankful for her! She is able to catch my reactions about 10-15 minutes before my symptoms start so lower doses of my emergency medications can be used to control my reaction. This means I not always in a drugged state trying to recover from a severe reaction and hoping to not need epi. That's good news! She is getting more consistent with her alerts. She hasn't been wrong yet, if she alerts it has either been to a sugar or mast cell reaction. BUT she is not quite as consistent as we would like. She is great if I ask her to "check-it" but in distracting environments or when she is "off-duty" in her mind, she is not as focused on what my body is doing.

We went to Columbus to meet up with her trainer and visit with their training group a couple weeks ago and got some good tips on training activities to get her alerting more spontaneous and less having to always cue her. But we love her! She and I are growing in our partnership every day, and I am finding myself relying on her more and more to be in tune with my body and to help keep me safe. Like I've mentioned before, service dogs are considered medical equipment by the ADA, and she really is becoming just as important to my healthcare as other pieces of medical equipment such as my nebulizer or glucose meter.

Hope those of you that read all the way to the end of the post enjoyed that little Leni update! PLUS, today is Bonk's birthday so leave a comment and wish him a happy 5TH BIRTHDAY!!! He's getting so big =)




Thursday, March 10, 2016

Xolair, Heart Monitor, GI doctors, Cortisol, Anaphylaxis, Oh My!

It has been just over 2 weeks since my Xolair injections, I've been on the heart monitor almost a week, saw a new GI doctor last week, and I just finished some endocrine testing so I figured now was a good time for an update.

So, up first, Xolair...what's the scoop now 2 weeks later? I am so thrilled to say that it is still going well! Things are not perfect, I do still experience symptoms, especially during the times of day that histamine activity is highest, but it is greatly improved. My "histamine hangovers" are significantly decreased, and it takes more of a trigger to set me off. All good news! Even better news is that Xolair has officially been approved by my insurance company through December 2016! PLUS, since I have already met my deductible and out-out-pocket max for this year (hospital stays are good for something...), all I will have to pay is my regular co-pay. Such an answer to prayer!

The heart monitor was put on last Friday afternoon and has been named Sebastian. That was a huge answer to unanswered prayer as the timing was totally a God-thing. It was originally supposed to go on after work on Tuesday. However, the time of the appointment meant I would have had to leave work early. I figured I might as well call and see if there were any other appointments so it wouldn't impact my work schedule. Long story short, I was told there was nothing, I kept the appointment on Tuesday, then 10 minutes later I got a call saying I could go on Friday. What made the scheduler call back after we had hung up, I have no idea (actually I do, but you know what I mean =P). We are doing this monitor because we discovered my heart rhythm has been a bit abnormal and my doctor is hoping to catch it in the act. However, because I rarely have what we think are cardiac symptoms, we weren't exactly sure if this would give the information we need. In God's perfect timing, though, Friday night at community group, I started experiencing the same chest pain I had in the hospital when the ECGs in question were taken. This is a pain I only get every so often, so to have actually caught it on the heart monitor is nothing short of God. We are very thankful to now be able to identify whether or not this pain is associated with any specific heart abnormalities or just another mast cell symptom. Since then I have had no other cardiac symptoms. I have to wear the monitor until next Friday and then will follow up with my doctor for the results.

Next up: GI doctor update. We have been in the process of trying to get a good network of doctors locally that I can call in an emergency. I absolutely LOVE my GI in Pittsburgh. He has been fantastic in working with me and managing my care. However, we recently decided that it was probably better for both of us (for multiple reasons) if I look for a local GI to manage my care. I had one GI appointment last week and have another coming up in 2 more weeks. Hopefully one of these will pan out and I will have a local GI contact for any issues. Last week's appointment was good. We covered some of our major concerns, made some decisions regarding what testing needs done in the near future, are trying a new medication, and have a plan going forward. At one point, I had the doctor, the head of the department, and the nurse practitioner all in my exam room discussing my case! It was a good appointment, and we'll see how the next GI appointment goes before we actually schedule any procedures.

The last bit of news is the Cortisol Stimulation Test that I had done yesterday morning. I got a call from my endocrinologist last week saying some of my recent blood work had come back abnormal and we needed to do more testing. For this test, they drew baseline blood work, injected me with some synthetic cortisol, then drew more blood work at 30 minutes and 1 hr. Pretty straight foward, but it ended up a bit more eventful than we hoped...I ended up going into anaphylaxis just before the 1 hr blood draw. We thought we had avoided any reactions since I was okay after the initial injection, but in true Kylene fashion, my body threw us a curve ball. Thankfully, Leni alerted to my reaction just before things got really bad, so my nurse was able to page the doctor and emergency meds before things got even worse. Both the doctor and meds had already arrived by the time I was starting to have trouble swallowing and my coughing turned into actual wheezing. The doctor was fantastic and had actually heard of mast cell diseases! She took charge and got all the meds I typically need ordered without me having to say anything. After a round of epi, benadryl, pepcid, a breathing treatment, oxygen, and a couple doses of something else (I forget) to control my tremors, I was doing better. Thankfully we were still able to get the final 1 hr blood work, so at least it wasn't a complete waste! Small blessings =) I won't know the results of this test until my follow-up appointment in a week and a half or my doctor calls.


Long update and most of it is just waiting to hear back from doctors. It has been such a blessing these past 2 weeks to have some break in my symptoms (despite the anaphylaxis yesterday). At this point we are able to say that the Xolair is definitely having a positive impact for me! Hopefully I will continue to improve with every additional injection. It has been so nice to rest in peace and joy for a time rather than discouragement and frustration. At the same time, there is still a lot up in the air with the heart monitor testing and endocrine testing going on. We may have some major decisions to make in the near future regarding medication issues and treatment plan going forward depending on the results of this testing. Please continue to lift us up in prayer, but this time with thankfulness as well as supplication!

Thursday, March 3, 2016

It's Not Good-Bye, It's See You Later...But It Shouldn't Have To Be

One of the toughest things about dealing with a rare disease is that sometimes the happy endings we hope and pray for just don't happen. I do my best to be honest about what life looks like while dealing with a chronic illness, but sometimes there's nothing that you can say or do to prepare...

A friend of mine just lost her battle with a form of mitochondrial disease. She was 24 and was "officially" diagnosed in 2013. She loved horses and riding and was the kind of person who always had something encouraging to say no matter what she was dealing with in her life. She had a faith that I always admired and was mature beyond her years.

My heart breaks for so many living with these rare diseases that have so few answers. Please say a prayer for her family as they begin to live life without her and peace knowing that she is fully healed and in the presence of Jesus.

Our symptoms started around the same time, and we have had many similar procedures and experiences. I actually emailed her multiple times when I had a new procedure coming up to see what her experience had been. No family should have to be going through what her family is dealing with today and in the weeks, months, and years to come. 

It seems cruel that she is gone less than 48 hours after Rare Disease Day. No one was a bigger advocate for mitochondrial diseases and dysautonomia than she was. She made such a difference in the rare disease community to raise awareness for so many living with these chronic illnesses. But awareness can only go so far. Without taking the next step, THIS IS THE REALITY for so many families impacted by these rare diseases.

Please, take a few minutes and remember those of us who are living with our own rare diseases. While my mast cell disease may not have the same prognosis that her mitochondrial disease did, that does not make it any less in need of treatment options and ultimately a CURE. Just because a disease isn't terminal doesn't mean life with it is easy or without its own pain. I've seen the helplessness on my family's face and heard the desperation in their voices as we hunt for answers that may or may not be there. It is such a helpless position to be in to have doctors giving no answers, seeing so many people suffering from these rare diseases in so many ways, and being able to do so little to make a difference.

As I've mentioned, I am a part of the Running for Rare team that raises awareness for rare diseases and funding for the Undiagnosed Diseases Program at NIH. This program is a last resort for many rare disease patients that seem to collect doctors like some people collect coins. The funding that goes toward this program leads to a better understanding of the mechanisms behind these rare diseases and eventually treatments, and in some cases, even cures. I am currently in the process of applying to this program despite having a mast cell diagnosis as I continue to have odd symptoms that my specialists do not understand. I can't do much, but I can speak out...for my friend and so many with similar stories, for all those living with rare diseases that have no cure, for my family.

We cannot do this alone! Please consider supporting my friend, me, my R4R teammate, Sarah, as she trains for the Boston Marathon on April 18, and the entire rare disease community. We need awareness, we need funding, we need treatments, we need CURES. 

Wednesday, March 2, 2016

Xolair Update

It has been just over a week since I got the first round of Xolair injections, so I figured now was a good time for an update.

We expected that I would have some side effects from the injections. That is completely normal and a common issue with antibody/immunotherapy treatments. I knew it was coming, but it still hit me harder than we had anticipated.

Tuesday last week was ROUGH. I felt like I had the flu...body aches, fever, nausea, bad vomiting, shakes, headache/migraine, swelling, etc. It was all my normal symptoms amplified, plus flu-like symptoms for fun! Yeah, not so much. Wednesday was a bit better, and Thursday was slightly better than Wednesday.

Then I woke up on Friday.

And I. felt. good.

Not just the, well-I-still-have-a-bunch-of-symptoms-but-it's-improved-from yesterday-so-I'll-just-say-I-feel-good, but actually truly good. THAT was a weird feeling!

After a mix up at the lab, I had to get more blood work on Friday morning. We were worried because Friday-Saturday was the women's retreat with our church that I had been looking forward to and hoping I could go on for weeks. Blood work typically knocks me down for a day or two, so I was afraid I was going to miss yet another event I wanted to do. Spoiler alert: that didn't happen! I went and had my blood work done. And that's it. I felt okay...I barely know what to do with myself when I'm not having symptoms...

I decided that no symptoms was a good reason to hide in a closet when Nick got home and attack him with a nerf gun (don't worry, I made sure he was armed, too =P). Anyway, I was able to go on the women's retreat, and it was such a GIFT. I forget how precious time with Godly women is when it has been mostly absent from my life for a while. By 11 pm Friday, I was still feeling good (although fading quickly, just normal tired, still no symptoms). Saturday was a bit rougher, only because like everyone on the retreat, I stayed up way too late and was starting to fall asleep by the afternoon sessions. But even despite the headache and exhaustion, I was thankful. What a gift to have been able to go on the retreat and FEEL GOOD!

At that point we were hoping it was an effect of the Xolair and not just a couple day fluke. It's now Wednesday, and while I did have a bit of a hiccup yesterday, overall the past 5 days have been the best I have had in a while. Things aren't perfect. Xolair is not a cure, just (what appears to be) an effective treatment. It helps calm down my mast cells so they are less reactive, but I can still have reactions. Yesterday I had a bit of a flare up in the afternoon/evening, but that is to be expected. Things are not all of a sudden perfect; ups and downs will still be the norm for me. Hopefully, the difference is that with the Xolair, now my ups will be higher and more frequent and the downs will be fewer and less severe.

We don't know how long this will stick around, but it does appear to be the Xolair working, so hopefully it will last until I get another injection next month. If not, we are still thankful for as much time as we get with me feeling better. It is nice to take a break from the discouragement and frustration and rest in a time of hope and peace for a bit. Even if (in the words of Marlin from Finding Nemo) "good feeling's gone" in another couple of days, we are thankful for whatever break we get.

So in case you're one of those "readers" who just skips to the end, here's the cliff notes version: Xolair appears to be working and we are SO THANKFUL!

But wait, there's more!

Unfortunately, the second part of the update is not quite as good, and we still very much need prayers. When I was in the hospital back in January, I had a few episodes of chest pain and had a couple ECGs done amid all my other symptoms. At the time I was told the ECGs were normal...turns out, not so much...

I saw a cardiologist on Monday and discussed the results of my abnormal ECGs. He also did one in his office yesterday for comparison which was still abnormal. Basically I now appear to have Long QT Syndrome. That means that the ventricles of my heart take their sweet time to contract and the atria want to start the next beat before the ventricles have actually contracted. This can result in arrythmias and may be the source of my passing out with no warning (not to be confused with my "normal" passing out that I DO get warning for). I will be wearing a 2-week heart monitor to hopefully give my doctor the information he needs to determine whether this is being caused by a medication issue or something else. Treatment will depend on what the underlying cause is. Please be praying that the 2-week monitor provides exactly what my doctor needs to make a more firm diagnosis. Also, please pray for wisdom and discernment for my doctors right now. Some of the medications that may be involved are ones that I rely on daily/multiple times a day to control my mast cell symptoms. Things may get tricky, and we will desperately need wisdom to make the best choice for my health and treatment.

That being said, we are choosing joy for the moment. Yes, the cardiology appointment was not what we were expecting, but when does life ever turn out how we planned? There is nothing to be gained by worrying about what COULD be. Worry accomplishes nothing for the future, it only steals joy from the present. Until we know more, we are choosing to still live in the hope and rest that has come with the Xolair injections for now.

As always, thank you for joining us on this crazy journey through life. Thank you to everyone who read my Rare Disease Day blog and shared. Thank you to everyone who has offered up a prayer on our behalf. Thank you to everyone who doesn't believe in God but still has come alongside us during this season of life. Thank you for carrying us when the weight of life is too much. And now, THANK YOU for rejoicing with us when we are resting in hope and peace!