Roller coaster

I know I said it in one of my last posts, but life has seemed a lot like a roller coaster of ups and downs lately, and today was no exception.

I had my CF follow-up today. My lung function went up! Yay! But only by 4 percentage points. Boo.

The doctor decided IV meds are done and I can get the PICC line removed! Yay! But the home nursing company never got the order so it's in my arm until tomorrow. Boo.

It was a beautiful day so we went to the park with some friends! Yay! Then I locked the kids in the car. Boo.

Some more details...

My lung function is up a bit, but I dropped a total of 15 percentage points and I've only gained back a total of 5 percentage points so I'm still 10 percentage points below my "baseline" (where I started), but even my baseline was much lower than I wanted it to be. It's pretty obvious that I'm not gaining a lot of benefit from the antibiotics, so thankfully we're done with that (and I can finally sleep a FULL night!). The doctor showed me my CT scan, which was pretty neat, and again stated that there's nothing too concerning, but given the fact that my lung function is still being pretty stubborn, he'd be open to doing a bronchoscopy if I am. In the meantime he's decided to put me on an antifungal medication to treat a fungus that has been cultured in my lungs (yeah. Fungus. Gross, huh?). He said usually fungi aren't problematic, but it's our best bet at this point. Antibiotics only kill bacteria, not fungi (or even viruses, which would explain why I got a cold last week even though I was on antibiotics!), so it could be quite possible that whatever is causing this exacerbation is not one of the two bacteria that we were treating. We had initially agreed to do the bronchoscopy on Friday next week, then we both realized we wouldn't be here! So the plan is to do the antifungal medication and if I feel significantly better we'll skip the bronchoscopy for now, otherwise we'll plan for it on April 11th.

I also went ahead and did my 2-hour fasting oral glucose tolerance test while I was at the clinic today. Any woman who has had a child will probably agree with me on just how horrible that drink is! Aside from the fact that I had a mean episode of hypoglycemia on my way home (before I could get something to eat), everything went fine. My numbers were good so I'm free from worrying about CF-related diabetes (for this year, at least).

And would you believe that, even though the home nursing company spoke with my doctor and he verbally told them to remove the PICC line, they couldn't do it until he faxed over written orders? Liability reasons, of course, but they never received them, so the PICC line is still in. I told Jason it was lucky he was the one who answered that phone call or I might have asked if they'd rather the liability of me removing it myself. ;p If you actually know me, you know I would never do that; I am not a rule-breaker. And I was actually kind of frustrated because my doctor and the home nursing company have both been very helpful and on top of things through all of this, so I'm not sure where the breakdown happened.

So now the story you're really waiting for... how I locked my kids in the car. And the truth is, I really don't know! There are two things that I usually make sure of, though, and I failed on both accounts today. #1- I try to make sure never to set my keys down in the car when I'm buckling the boys. I always try to make sure they are in my back pocket or purse. #2- I never like for all the doors to be closed with me on the outside, even if I do have the keys. Today, though, I happened to set the keys down on the seat next to James as I buckled him (I think he was thrashing around and I was trying to wrangle him). Then, just as I closed James' door to head to Drew's side, Drew hit the button that closes his door (he's been panicking about bugs getting in the car now that it's spring). Then I heard an ominous "click" telling me the doors just locked. It's the "click" that confuses me because there is no lock button where Drew was. My van does have a delayed lock feature where, if I push the lock button on the clicker while doors are still open, it waits until all the doors are closed, then locks the doors. Obviously that must have been what happened, but I was unaware of the fact that the "lock" button got pressed. Of course, when I heard the click, I instinctively grabbed for my back pocket only to realize my keys were not there!! Thankfully Drew wasn't buckled yet, so we had him try to open his door. No luck. He climbed to the front seat and we guided him to hit the unlock button. No luck!! Apparently the delayed lock feature also disables the car from being unlocked from inside? I can't understand that at all, but Jason says it has happened to him. We had Drew try all the doors, and the lock button worked (you would hear the doors all lock) but when he pressed unlock, nothing happened. Thankfully my friends Frank and Amanda were with me which helped to keep me calm (and also helped me not to feel like I was crazy as we watched Drew press the unlock button with no response). Amanda was finally the one that asked if the doors had a manual lock that could be pulled up. I have no idea what it's called or how to describe it well, so this is what I mean:

Yes, we live in such a digital era that we didn't think of  a manual option at first!

Nope, my van doesn't have locks like that! After another moment or two we realized the van does have a manual lock, it was just in a different place, right above the door handle.

See it? We didn't at first.
This one is in the "unlocked" position making it easier to see.

Finally we coaxed Drew to flip that (3-year-olds don't generally understand the mechanics of cars, so it took a little explaining) and the front door unlocked, which then cued the car alarm. Then it was a scramble to try and get the other doors unlocked and opened so I could get to the keys, but without the electronic unlock button working, we couldn't get the back doors unlocked, so I had to play a game of "Twister" as I tried to climb into the back and over top of James to get to the keys (effectively knocking them UNDER the car seat, making it that much more difficult to get them). I finally got the keys in hand and the car started, ending the crazy battle. Phew! Obviously everyone was fine, minus me being a bit rattled, and Jason being a bit late to meet someone (I was trying to rush home to give him money I had accidentally left in my purse... the story only makes sense this way, right?). If this had happened in the middle of the summer, I most certainly would have been panicking! It does not take long at all for a car to get too hot for children here in the summer. But James just sucked his thumb through the whole event (he was ready to snooze and I don't think he even knew anything was going on), and Drew grinned the entire time like it was a really fun game. I used to be notorious for locking my keys in the car, but I think this may just be a first since Drew was born. Hopefully the last? (I kind of doubt it.)

Overall, today was an "up" day, in spite of the little downhills. The flowers I bought for myself may have helped with that. :)

The problem with wheat

This post is kind of long and strange because it is actually two posts in one. The first part I wrote a few weeks ago and never got around to posting (I'm guessing I wrote it right before the CF-appointment-gone-wrong). The second part is kind of an update. I thought about rewriting/changing the first part, but my opinion towards everything still stands.

Part 1:

This is where it started- where I first began suspecting that James might have an allergy to wheat. For a little summer fun, Stacey and I created some "toddler paint"- flour, water, and food coloring. The kids had a blast painting with it, or eating it in the case of James and Alyson, and it was all good fun. Until...

During bath time afterwards I noticed James' skin was red and slightly puffy under each of the patches of "paint". Neither of the other kids had this reaction. There were really only two things it could be: wheat or food coloring.

At that point James was really just starting to eat solid foods and really had mostly had only fruits and veggies. So, because of the reaction, I became hesitant to start him on any foods that had wheat. I avoided them for a while, then began slowly introducing them- toast, crackers, tortillas. We didn't always pay attention, but after a while I started noticing a pattern- when he had wheat, he suddenly got an extremely runny nose and couldn't stop sneezing. This reaction would keep him up for hours at night if he had wheat products for dinner because being a thumbsucker with a stuffy nose means you can't breathe when you try to sleep.

James had already been seen by an allergist because of a rash he had as an infant, even before starting on real foods. He had been diagnosed with an allergy to egg and sesame. So on one of our follow-up appointments with the allergist I mentioned my concerns about wheat; she brushed it off saying, "you don't want to have to avoid one MORE thing, do you?" She pointed out that his skin and blood tests only a few months earlier had shown no reaction to wheat.

Jason and I, for our own sanity (and because we love sleep), continued to avoid giving James wheat until one day I decided I was just being crazy. "The doctor said there's no reason to avoid it. Maybe I was being hypersensitive before. Let's just see what happens." We gave James some pasta for dinner and he was up for almost 5 hours before he finally fell asleep that night. "Well, it could be a cold. Or maybe he's teething," I said. "Let's give it another try in a week, after we've seen for sure that it's not a cold or teething." After a week of him being perfectly fine and going to sleep easily, we gave him some bread at dinner. He was up for only 2 hours that night with the runny nose and sneezing before we gave him a small amount of Benadryl. 15 minutes later he cleared right up and fell asleep.

I called the allergist and scheduled more blood work to test for a wheat allergy. The results came back very low- she said she wouldn't be concerned about wheat. This week we had a follow up again with the allergist about his egg and sesame allergies and I tried to explain my observations with wheat. Literally in the middle of my explaining how we had done two separate tests, the doctor interrupted me and wouldn't listen anymore. She told me it's possible that he has an intolerance to wheat, but it's not an allergy. She said "there is no test to check for an intolerance."

Of course I was mad. I was interrupted in the middle of something that is very important to me. I have a right to be listened to, and I'm not making this up. But Jason brought up a good point- "what do you want her to say?" Do I want him to be allergic to wheat? No. Do I want her to tell me to avoid wheat? I already do. Do I want her to tell me to give him wheat? She already has, and I just don't agree. Do I want her to give me an EpiPen for it? We already have one.

But here's the thing- in my research I have found that a food intolerance causes stomach pain, gas, bloating... an intolerance is when a body can't digest a food. A food allergy is an immune system response so congestion and sneezing can be common symptoms.

I guess I just want to know that I'm not crazy and making this up. Or I want to delve into some of the other things it could possibly be that might often be paired with wheat. The problem is there isn't much in common between pasta, bread, tortilla, and crackers besides wheat itself.

I haven't fully explored the possibility of food dye being an issue, but we just don't eat a lot of products that contain food dye.

Part 2:

At our appointment in which the doctor wouldn't listen to me, we scheduled James for an "egg challenge". It had been a year since the original blood tests revealing egg and sesame allergy had been done, so she wanted us to have it tested again and was fairly convinced that his egg allergy level would be low enough for us to have him come in to the office and be monitored while trying egg. Unfortunately the egg allergy level increased drastically from "moderate" to "very high" (side note: the sesame decreased only slightly from "high" to "moderate") and the nurse informed us that the doctor did NOT recommend doing an egg challenge anymore, though said she would be willing to do a "baked egg challenge". Many people who are allergic to egg can eat it in baked goods (cookies, cakes, muffins...) because the length of the cooking time helps to break down the egg protein that triggers the allergy.

I agreed to do a baked egg challenge though finding a baked good with egg but without wheat wasn't easy without delving into gluten-free baking. I finally found a recipe for gluten-free muffins that I actually had all the ingredients for, so I baked up a batch (they were NOT good, in my opinion) and we headed to the doctor.

The baked egg challenge didn't go well. James ate 2 and a half mini muffins. After each half muffin we waited ten minutes, checked him for reactions, then gave him another piece. He was fine after the first two pieces. After the third piece he had a small red patch under chin/neck that the doctor checked out; she wasn't concerned so we continued with the 4th and final piece of muffin. Apparently at this point James was done eating. He put a few pieces of the muffin in his mouth then proceeded to spit it out all over his bare belly. Within a few minute a small red patch appeared on his belly. The nurse checked on him and said she'd get the doctor to take a look. By the time the doctor came in the one small patch had turned into 5 small patches of hives. A shot of epinephrine later, the doctor said baked egg is a no-go, though obviously he would be fine if he were to get a small amount since he had no airway problems nor any vomiting. Part of me thought "but he was fine until it touched his skin" but then the other part of me realized "if it does that on the outside, what on earth is it doing on the inside??"

Since we were going to be there for a long period of time (and since I was initially under the impression that they were going to do the skin test with egg) I basically demanded that they do the skin test again for wheat. This was the result:

After 2-3 minutes

After 10 minutes

The H on the left is "histamine" and is intended to have a reaction. The N is "negative" meaning it's the control (and shouldn't react). I think it's there just to show the body's natural reaction to being pricked. The W on the right is wheat. The basic conclusion is that he didn't react to wheat. There is a small amount of redness but no bump, and considering it was smaller than the control, the nurse really didn't consider it to be anything abnormal. She did note that it was "a little red", but again, nothing that she was overly concerned about. [Side note: would I be crazy to wonder if she got the control and wheat needles mixed up? I guess either way it's more or less the same reaction.]

So we're left with a very low (but not negative) blood reaction and an almost negative skin reaction. Am I crazy? Am I making this up?? Avoiding wheat is SOOOOO difficult so it would be so great to be able to feed it to him, but I feel like we've done pretty fair trials. I've been told by reliable friends (those that study medicine) and have read that neither skin tests nor blood tests are completely reliable and our pediatrician says that observation is sometimes the only evidence. I guess it's just hard not to want a definitive answer.

I think we're going to give it a go again. It would make life so much easier if he could eat wheat. My plan is to give him cream of wheat for breakfast one day. Cream of wheat because it's only wheat and it would take away any chance of a "coincidence" reaction from another ingredient. Breakfast because we won't have to lose sleep over it.

I'll keep you posted.

Divide and conquer

Another quick update:

Jason is out of town. Boo. But my mom is here. Yay! Although "here" is kind of not true. After a rather exhausting day yesterday (with two kids who were both whiny and altogether unable to be pleased), and because James seems to be suffering from a cold, we decided our best approach was to "divide and conquer". She took James to the lake house last night and will stay until tomorrow and I have Drew here. We've planned a couple easy dinner meals for me here and I have lots of leftovers for lunches. I bought the movie Frozen (although we hadn't even seen it) to keep us entertained while I rest. Drew's pretty easy on his own (and he slept until 8:30 this morning so no complaints there!) and, as I said before, I think he can really use some extra attention these days, so it's kind of a bonus both ways.

I will actually say I'm kind of glad Jason got a chance to get away from all of this. He's been so amazingly supportive and has done so much to keep it all together here that he's just as run down and exhausted as I am. Yes, he's away on a business trip, so he's working plenty, but I'm hoping the free time after work will be good for him. I made him promise me that he would do something fun while he was away.... as long as that something fun wasn't a college girl on spring break. ;) Kidding. He is in Tampa, though.

Because I know many of you are sending up prayers, I figure it's only fair to keep you updated: I got the chest CT scan results and nothing looks so abnormal that they want to go ahead with a bronchoscopy. My lungs definitely look like CF lungs, and there are some questionable "cloudy patches", but we'll see how I do at my follow up and go from there as far as possible treatment options. The doctor did say that my lungs look quite clear so I'm doing a good job with airway clearance, so that's good news.

Thank you for all the prayers and notes of encouragement sent my way. I have been saying my own prayers of thanks for the wonderful people in my life. I am so appreciative of the offers of help from so many of you, but I am just as thankful for those who are thinking about us and praying for us.

Frustration and fear

Due to the fact that people with CF are not supposed to be in close physical contact with each other (because of the risk of cross-contamination of the many bacteria that inhabit our lungs), there is a pretty strong online support community. I will admit that I do not participate in this community because I honestly find it more depressing than helpful. A visit to a person's Facebook page or blog leaves no doubt that they have CF, that they hate it, and that it's all they want to talk about. I do not hide the fact that I have CF but... I don't define myself by it and I don't let it consume me. Perhaps I would be wise to focus on it more because, honestly, until recently, I've never made a life decision with even a second thought to the fact that I have CF, and maybe there are some things I should have thought through more carefully (going to Costa Rica without meds?). Part of that is because I've been blessed with very good health, but I think it's also due to the way my parents raised us as if we were normal and could do anything that anyone else could do. But, either way, I simply don't like to spend time whining about CF and feeling sorry for myself.

So, in spite of my recent posts and at the risk of my blog turning into a CF gripe fest that I hate, and despite the fear that someone stumbling upon my blog would think that CF consumes my blog and my life, I go ahead with the following post because I find writing therapeutic ... 

As I said above, I try to keep a positive attitude towards my health. I don't think it benefits anyone to ask "why me?". But I also don't think I'd be blamed for being a bit down lately. The truth is I am frustrated and scared.

I'm frustrated that I can't be there for my family in the way I want to be. I set high standards for myself, and I recognize that, but it's very frustrating to not have the energy to do the things I want. Recently Drew, who has always had nothing but great reports from his teachers, has been having some "off days" at school and I hate the fact that it's because of me. I know that's a lot of blame to put on myself, but it's not a secret that kids often struggle when their parents are ill. Between the lack of energy, and the time being put into getting better, plus the fact that Jason has to pick up the slack on everything else around the house, there's not as much attention to go around for the kids. James, too, has been quite clingy and fussy lately. I'm happy to sit and cuddle with James, but it's not so easy with Drew. Because he LOVES to talk and be listened to, I decided Drew could really use some one-on-one time so I took him on a date this week. Unfortunately it ended up being on the day of my follow-up CF appointment that went all wrong. They wanted me to do a chest CT scan that day and I refused because I wasn't going to miss my date with my son. But even still, I had to deal with pharmacy calls for the new IV antibiotics on our way to lunch. Once we got there I put my phone on silent and decided I could handle the voice mails when our lunch was over. Drew was just a little over the moon at having lunch at the airport where we watched planes taking off and we even saw a really looooong train go by!

As if that's not enough to be frustrated about, I find myself incredibly frustrated by the fact that I feel like I'm not in control. I've always been able to achieve what I set my mind to do. From high grades in school, to having two natural births, to running a half marathon, to cloth diapering... once I set my mind to something, I am usually successful. I have set my mind to get healthier and so far I've failed. No, I don't really label it as a failure, but the perfectionist and control freak in me has a really hard time dealing with the fact that I am not fully in control of this situation. In the past when I've had dips in lung function I get a little more serious about doing my meds and exercising and I bounce right back. Now it feels like my effort is getting me nowhere and that's frustrating. I have been doing all my medicines religiously. I've agreed to do this exhausting and frustrating IV thing. I had a sinus surgery done. I've been researching other things I can do to improve my health (best diet, supplements that are beneficial, etc.). I've been trying so very hard to get out and exercise. I have never put this much effort into something and not gotten the results I expected.

And that's what leads to the fear. Cystic fibrosis is scary- it's a degenerative disease with no cure. Not having control over it terrifies me. I touched on it briefly above, but here's a fact you may not know about cystic fibrosis: people with CF are susceptible to bacterial infections that "normal people" are not susceptible to. Research has found that these bacteria, once in the lungs of a person with CF (lungs that are lined with thicker, stickier mucus making it easy to catch these bacteria in the first place), behave differently and form almost impenetrable shells making them incredibly difficult, if not impossible, to kill. Cystic fibrosis treatments focus on managing the colonies of these bacteria in our lungs so we are constantly on various oral and inhaled antibiotics, and occasionally require IV antibiotics. Pseudomonas aeuruginosa is one of the most common bacteria, and one that I myself culture (ie. grows from sputum cultures I give... even though that's just as cryptic, I won't give any more details than that*). Recently I've also been culturing MRSA. While neither of these are as scary as burkholderia cepacia, they certainly wreak enough of their own havoc. I've cultured pseudomonas for so long (since high school? Maybe even earlier?) that I've never thought much about it, but it's finally getting my attention and it's scary. [Note: this is my non-scientist understanding of very complicated sciencey things].

So I'm frustrated and I'm scared, but I am thankful that I have doctors who don't just look at this as "typical CF." Yes, we are expected to lose lung function as we age, but my doctors know this isn't typical for me and are trying to get to the bottom of it. Some of the possibilities are just as scary (CF-related diabetes), but I'm just hopeful it's something I can do something about so I can feel in control again. 

And in the meantime, I can't forget that I have an amazing support group. I have my amazing husband who works so hard and does so much it's really too much to even describe. I have my sister and as much as it sucks that she has to go through this too, it's nice to have each other when CF can be such a lonely disease. I have amazing parents that taught me that I am normal and to fully live my life, and who also come to my side at the drop of a hat (my mom is cutting a trip to Canada short in order to fly here on Monday to help out. She went to visit my grandma for her birthday and she'll be leaving ON her birthday. :( My Grandma is pretty awesome and understanding too!). I have so many friends that, in spite of everything they have going on, are happy to help out when called on. And I have an amazing God. Others might ask the "why me?" questions or even play the blame game; instead, I truly believe God works in His own mysterious ways that we may never understand on this side of heaven. Right now I believe He's trying to get my attention and I fully trust His plan for me. He is my rock and comfort in these difficult times and I relax in the knowledge that He loves and cares for me.

Proverbs 3:5-8 Trust in the Lord with all your heart and lean not on your own understanding; in all your ways submit to him, and he will make your paths straight. Do not be wise in your own eyes; fear the Lord and shun evil. This will bring health to your body and nourishment to your bones.

*Anything that I "won't give any more details" about is only as protection for those reading. As CF has a lot to do with "gross things", like mucus, I worry I'll gross people out. But I am never offended when people ask questions and I'm very open if I know you want to know more. So, please feel free to ask questions if you're ever curious!

The post I didn't want to write

I've had it in my head for over a week that today's post was going to be titled "I'm free!" because today was the day I was finally getting the PICC line removed. I even planned to take a picture of my unencumbered arm.

Instead, this sits curled by my side...

Here's the roller coaster of my last month:
February 4th: I had a regularly scheduled CF appointment and wasn't expecting much. Instead I found out my lung function had dropped a drastic 15%. The decision was made to start a two-week course of IV antibiotics in response to the drop and in preparation for my upcoming sinus surgery.
February 7th: I had the pre-op for my surgery with my ENT doctor, then with the anesthesiologist at the hospital, then ran to another hospital to have my PICC line inserted.
February 8th: I began IV meds.
February 12th: The day of my scheduled sinus surgery we were buried in snow and ice. The surgery was rescheduled. Because the surgery was rescheduled the IVs were extended from 2 weeks to 3 weeks to ensure I would still be on antibiotics after the surgery.
February 24th: I had my sinus surgery (which went well and was successful)
February 26th: My sister and her boyfriend Robin arrived (yay!)
February 27th: My parents arrived (also yay!) and I had a follow-up CF appointment. Even though it was only 3 days after my surgery, my lung function was up 13%! That means I was only 2% below my original lung function! My 3 weeks of IVs were set to end on March 1st, but we decided to keep the IVs going until March 3rd to make sure I had a full week of IVs after the surgery, then, as long as I was feeling well, the PICC would be removed on March 4th!
March 4th: I am feeling achey. Like, flu achey. Or mastitis achey. Either way, it's a bad sign. I had no other symptoms and felt all the meds were kind of interfering with my system so we decided to stop the meds but leave the gosh darn PICC line in (I still can't shower without help!!) for another week until I could have another follow-up with my CF doctor.
March 13th: I had another follow-up CF appointment. My lung function has dropped 11% again only a little over a week after stopping IVs. So I'm pretty much at the same number I was when we started this mess. The decision was made to re-start IVs with a different drug this time. Only problem? This one is every 6 hours (instead of every 8) and runs for an hour. And I'm supposed to sleep when? I'm also doing a chest CT scan and based on those results a possible bronchoscopy (going into my lungs with a camera) to see if we can figure out what's going on. I would go into more detail about why it's being done, but it's all rather gross and boring.

*EDIT: Please note, I use the term "percent" when referring to my lung function ups and downs but I really should say "percentage points". What I  mean with the above numbers is that, if I had started with 100% lung function, it dropped to 85%. Then went up to 98%. Then back down to 86%. If that makes sense. I tend to not like to share the actual numbers for various reasons.*

This timeline doesn't include the almost daily battles I've had with pharmacies and insurance to get my routine medications. We were forced to switch insurance in January so I've been trying to get all new prescriptions sent to all new pharmacies and just keep hitting walls. Knowing the switch was coming I had ordered a 3 month supply of all of my meds in December so I haven't had to deal with all of this until now. Yes, I should have started working on it sooner... but who can blame me for avoiding all of this??

The timeline also doesn't include the fact that I've had multiple allergic reactions (skin only!) to the dressings and antiseptics used on my skin and have wanted to sever my arm off because of itchiness for 4 of the last 5 weeks (I had one week in there where everything was just right and I wasn't itchy. I think I've now finally pinpointed the exact causes of the problem). Nor does it include all the follow-up ENT visits to vacuum out my sinuses (with no sedation or numbing!), or the fact that I spent an extra 30 minutes at my ENT visit today trying to get a torrent of a nosebleed to stop (which the doctor was completely surprised by and tried his best to get it to stop which seemed to only make it worse and worse).

A bloody massacre?

As I just commented on my aunt's blog, I'm actually quite thankful that I chose to participate in a photo challenge called "100 happy days". I post a picture every day to Facebook of something that makes me happy. On some of my worst days I've still been able to find something that makes me smile. Sometimes it's quite small (donuts!). Other days it's something big (all my family in one place!). But altogether it's helped me focus on the fact that even when it seems like everything is going wrong, there is still so much to be thankful for.