Home Sweet Home

Yesterday I broke out of jail.  No, not really, I would never call the hospital jail... let me start again.  Yesterday I came home from my amazing and health-restoring vacation.  It was a wonderful two weeks, full of educating the uneducated and, well, coughing!  I coughed so much more than any other patient anyone had seen.  That's how I get better! At least it made them feel like they were making a difference.  Some of the respiratory therapists who had me were shocked that I wanted CPT (chest physical therapy) longer than 10 minutes (or 1 minute per lobe/position)!  I was shocked that no one had ever requested it or taught them differently.  So what did I do?  Educated everybody who handed me a nebulizer (except for the two wonderful ladies I met who could have written the book on being a good RT: Mary Catherine and Diana R. - you ladies rock!!)  I was even asked one of the most ridiculous questions I've ever heard out of an RT's mouth: "Have you had CF since childhood, or did you acquire it later?"  I know he didn't understand that CF is a genetic disease, because he didn't ask when I was diagnosed or even when I got sick.  He asked if I "acquired" it.  Now, I posed this issue to a lot of other CFers and many say it's common to get that question - and this alarmed me.

From a Respiratory Therapist, that question should be automatic grounds for re-enrollment in school.  How can you take care of a CF patient properly if you don't know why they are there?  The patient has lots of STICKY (more so than healthy people) mucus in the lungs.  "But Justine" you may ask, "why is it stickier than healthy peoples' mucus?"  Well I'm glad you asked, it's because of a defective mutation (actually two, one would still produce the phenotype of a healthy non-CFer) in the CFTR (Cystic fibrosis transmembrane conductance regulator) gene which causes chloride channels to trap chloride, and thus attract sodium to neutralize them both into - SALT!

Now I digress to ask you a question: What happens when you pour salt on a slug?.... Yes, it dries up.

Now back to my lungs:  If chloride is trapped inside the excretory cells (lungs, sinuses etc), the salt forms and the water, instead of being freely excreted into the airways to form mucus, is not as free to flow because it is drawn to the salt in an attempt to create a homogenous salt water concentration on both sides of the cell wall.  Result: stickier (drier) mucus.

End of science lesson.

If an RT doesn't know CF is genetic, they wouldn't know that we have sticky mucus, nor why we have persistent lung infections (bacteria thrive in moist, dark, undisturbed places).  For all they know, we just have a bad cough, and they wouldn't be able to give us the kind of treatment we need, because they weren't aware of the root problem.

Please, the next time someone in the medical field doesn't know about CF, and should, educate them! Even if it's a simplified, two sentence explanation.  It's really sad to think about how many people aren't getting the care they need!  I know I am an overly involved patient, but you can't expect anyone else to be your (or your child's) advocate.



All in all, it was a great vacation.  My PFTs yesterday morning were an FEV1(function) of 32% and an FVC(capacity) of 51%!  Great, especially considering how sick I was when I went into the hospital.  My port is healed and I was given thorough lessons on accessing and deaccessing, even doing it once myself.  After two weeks it's healed very nicely, although it's still tender.  I'm hoping that as it heals, the scar tissue will solidify it's placement.  I took the advice of many other CFers with ports and cut a hole in a sponge to put over my port when using the vest.  I tried it today, and it's still too tender for that, so I have been unhooking the shoulder velcro.

Port

This morning I was awoken out of a dead sleep at 6 to my nurse flipping on the lights, "They're ready for you in 30 minutes."  It's port time!  I had them page respiratory so I could get an albuterol neb before they took me... gotta breathe, right?  The cute resident who came and talked to me yesterday was down in pre-op. :D He answered the rest of my million questions to the best of his ability - I'm a pretty involved patient (translation: control freak), so he wasn't really prepared to deal with me.  They wheeled me into the OR, I climbed onto the table and laid down.  They gave me some versed and started to wrap me up, "like a burrito".  The last thing I remember was muttering something about having a fallback career lined up at Chipotle.  When I woke up, I had some pain and a kickass incision near my shoulder:

 

A few hours later someone came to access it so I could start receiving my IV antibiotics through my brand new port instead of the peripheral IV they had in my arm (which are very painful for me!)

So there you have it! It was fairly simple and I can't believe I hadn't done it sooner.

I've fallen in love... with a new nebulizer!

A Breath Actuated Nebulizer... how cool is that?  There is no waste, whatsoever, and the treatment time is fast - faster than any neb I've ever had!  When you breathe in, the neb cup with the medicine pushes down on the air supply, nebulizing the medication, and when you breathe out, it lets up.  SO cool!  It's called the AeroEclipse and I'm going to get a script for them when I leave.  I have been doing research on nebs tonight and was wondering if anyone had any experience with using this neb with Tobi?

 

Also, I'm set for getting my port a cath in the morning, so I'll make sure to update, hopefully with a picture of it!

Tune up time

Went to the doctor yesterday, the PFTs were WAY down.  I think they are at my lowest, FEV1(function) at 23% and FVC(capacity) 32%.  I'm getting admitted to the hospital today.  While I'm here I'll be getting a port a cath so I won't have to get any more PICC lines (yay!).  I'll update more once I get checked in and settled. xoxo

Cupcake Disaster

Disclaimer: No one was harmed in the making of these baked "goods", but our egos were slightly damaged.

Several weeks back I was in Barnes & Noble collecting as many books as I could carry and I picked up this little book of cupcake recipes - a purely impulsive purchase.

Anyway, when I got home, as I flipped through all (okay there were only 26) the recipes, I had fantasies of becoming the cupcake lady, baking delicious, decorative treats for everyone I know, going into business and acquiring fame and fortune, all because of a bargain book I grabbed on a whim. Famous and important people would be BBMing me their orders and twittering about my amazing cupcakes......

...sorry, I'm back.

Reality: Today I had the idea I would bake some cupcakes (maybe 12 to start) and take them with me tonight when I headed out.  I grabbed my book and passed my dad's study on the way downstairs, "I'm going to make cupcakes."

"Do you want some help?"

"Yeah, could you come show me how to turn on the oven?" (I'm serious about this part, we had a conversation yesterday about the fact that I didn't know how to operate the folks' new fancy stove, which we have had longer than the 10 months I have been back living with them.)

"No, but I'll show you how."

As the oven preheats, we're taking turns with the electric mixer trying to get the god damn eggs and sugar to become fluffy.  We already quarreled about the size of the eggs, the recipe called for large and we had jumbo.  Dad (chemist that he is) said the egg to sugar ratio was too high.  I said it was fine and continued mixing.

Cut to 45 minutes later, we're still cleaning up glass shards and jam residue from the floor.  Dad had one of those dropsy days... when no amount of moisturizer can cure the dry, slippery finger tips.  After sweeping, vacuuming, 409ing and swiffer wet mopping, we get back to the business of these damn cupcakes, which we have, by now, lost all motivation in baking.

After combing all the ingredients and looking at all the muffin tins we have to fill with this piddly amount of batter, we go ahead and continue following the directions, suspending disbelief for the time being. "They'll fluff up in the oven"...

Rationing batter is hard enough as it is, but doing so when you have to save half to fill on top of a dollop of jam is just ridiculous.  We split the batter in two and got to filling.  I'm at 18 and out of batter, yet each cup that has batter doesn't even have enough to cover the bottom of the freaking cup.  Just follow the f*cking directions.  Dollop of jam, done... top half of batter, also done.  We even had enough to make 4 more whole "cupcakes" bringing the grand total to 22, two shy of the recipe's indication.

Popped those suckers in the oven for 15 minutes and hoped for the best.

I took this opportunity to satisfy Zoe's incessant need to go outside and sit motionless in the yard.  I wrestled her into her walking jacket and we headed out on the leash (more about this genius idea another time).  We make the rounds, she eats some grass and then sits under a tree for 5 minutes.  I finally started walking up to her to bring her in and spotted half of a robin's egg nearby.  I picked it up (yes, birds are disgusting and I washed my hands thoroughly afterward) and was going to carry it inside to show Dad (what else was I going to do, leave it there and mention it to no one?)  I was so excited that I stepped on Zoe's leash as I went down to pick her up and it jerked my arms as I came back up.... *crunch* Stupid egg - it was so fragile! I brought the cat back in and threw the remnants of the egg on the outdoor table.

Time to check the cupcakes!

Well.. they're certainly small.  But good things come in small packages, right? I was too excited to try one to wait for them to cool... or to bother dusting with powdered sugar.  I managed to get it out of the paper in almost one piece, but the jam was adhered to the bottom, which was, uh, chewy.  Not a word I want to describe a cupcake I'm about to consume.  It was terrible.  The jam had dried up and the cake portion was so dry I nearly choked.  Dad said it was like corn bread, with a little jam on top (that is after you scraped it off the paper, if you even bothered).  It was so little, I couldn't even call it a muffin... it was more of a dry, puffy cookie in a big paper bowl.  I should have been suspicious when the book was marked down to $6.

So needless to say, I'm not bringing delightful cupcakes to anyone tonight.

I can breathe!

Good news: I'm on the mend!  I woke up this morning and could actually breathe.  I'm still pretty wheezy, with a lot of congestion, but it's moving and I can clear some, which is more than I can say about yesterday.  I feel like I've coughed for days straight, my lungs and throat ache.  I had energy and an appetite, too.  Dad brought me breakfast from McDonald's this morning and I ate first thing after I got out of bed, which may have been a mistake because I didn't get around to doing a vest treatment until 3pm.  I mostly puttered around on the internet and watched my parents figure out their new Topsy Turvy planters from inside.  I had a friend suggest I go out in the sun and get some Vitamin D.  I would love that but there are several reasons I don't go out in the sun:

1) I'm fair skinned

2) I'm on a medication that makes me even more sensitive to the sun than I already am

3) As I plan to become a post-transplant patient someday, I know that I will be immuno-suppressed from then on.  My body will be less able to fight off cancers, including those caused by sun damage.  All of my past sun exposure is working against me, so I've got to be careful.  I'm so glad Aveeno has come out with some high SPF sunblocks!

Therefore, I stayed inside most of the day.  Before dinner I got a spurt of energy and decided to get out of the house and make a run to Walgreen's, as I had 8 prescriptions to pick up.  While I was out I filled up my car with gas and ran it through the car wash.... so now I'm expecting it'll rain tonight or tomorrow.

One more shameless promotion (or demotion): my Blackberry somehow reset, and while I still have phone numbers, all of my pictures and ringer settings are gone, so I keep jumping every time my phone makes an unexpected noise.

Nike!

Ok I'm done.

Ill and puny

When I was in lower school and early childhood, kids who were sick didn't go to "the nurse", they went to the "Ill and Puny", a room with a cot by the principal's office.  I can't actually remember if there was ever a sign that said that, but that's what my dad and I called it.  So, today, this is my status.  Thursday I started getting tighter and more congested in my chest, lots of unproductive coughing, sore throat, labored breathing.  Since then it's gotten steadily worse.  That being the case, I haven't left home since Thursday morning.  I finally mustered the energy for a shower tonight after a meager dinner.  It felt good, since I've just felt fuzzy from sleeping so much, but it took a lot out of me.  So much so, that my dad offered to dry my hair - and I let him.  He hasn't done that for a good 15 years!  I'm hoping I feel better tomorrow; I don't want to go in the hospital. :(

I am le tired... signing off now.

Appointment update

Today I had a clinic appointment, more out of habit than necessity.  Last week I started with a tickle in my throat, then a sore throat, then nasal congestion (and loss of smell). Today I could smell (and taste) but I was very congested in my chest, so I'm glad I had the appointment pre-booked.  My PFTs were down a little, FEV1 down to 28% (function) and FVC at 42% (capacity).  Not time for antibiotics yet, but I'm going to keep an eye on it.

Also, the headaches have returned.  The last time they hung around, I had an MRI done and began the sinus rinses...I had an ABG (arterial blood gas) drawn today to check my CO2 levels.  If my body isn't getting rid of enough carbon dioxide, it will build up in my blood, which can be lethal. I'll hopefully hear something tomorrow.

That's about it for me tonight - I'm ready for bed.

Don't Drink the Water

Someone brought to my attention today, a notice that Wichita Water Utilities customers received (beginning 3/25). I'll sum it up with some direct quotes:


Our water system recently violated a drinking water standard...We routinely monitor our water for turbidity (cloudiness).  This tells us whether we are effectively filtering the water supply...If you have a severely compromised immune system, have an infant, are pregnant, or are elderly you may be at increased risk and should seek advice form your health care provider about drinking this water... This is not an emergency... turbidity can interfere with disinfection and provide a medium for microbial growth. Turbidity may indicate the presence of disease causing organisms... Steps have been taken... Turbidity measurements have returned to normal and we expect to be in compliance in April.


Um. WHAT? You mean the water I have been using to brush my teeth, cook, clean my food and body isn't clean?


I don't know anything about water treatment or related legal guidelines, so this may be a normal, acceptable occurrence - but to me, as a customer and as someone who is at high risk for infections, I'm alarmed.  There are no other options - I can't shower with bottled water, nor can I avoid washing dishes, clothes, food, etc. in the water the city is supposed to be treating to meet certain health standards.


I don't know why they even bothered to tell us this happenED. Why would they alarm the public about something that we can't do anything about, and isn't an issue any longer, unless it were something very serious.  I know, some of you probably don't think it's a big deal.  After all, people years ago didn't have the luxury of chemically-treated, government-regulated city water, they drank the well water, organisms and all.  Some people may argue that it's good to get a little dirty water every now and then to boost the immune system... which is a load of crap. Bacteria is bacteria, and when you're infected with E. coli or C. diff, then tell me how healthy you feel.  


I'm probably over-reacting - but I definitely won't be drinking the water.








UPDATE 5/3:
Now that Boston's water main break has happened and they were without clean water for a couple days, I feel like my freak-out is a little unwarranted.  *sheepish look*

Daily Reminders

Even on days when I'm feeling (relatively) well there are always little (and sometimes big) reminders of my illness.

For starters, I wake up and cough. And I don't mean clear my throat... I mean COUGH - braced and hunching over until I'm red in the face, gasping for breath cough.  This happens whenever I wake up, so if I stir in the night, it happens.  I'll be up for 5 or 10 minutes just coughing so I can be clear enough to lay back down.  Another 10 minutes to fall asleep, if I'm lucky.

When I get up in the morning, the first thing I do (after going to the bathroom and grabbing a soda) are my breathing treatments.  This consists of aerosolized medications and a vest treatment - the vest shakes my chest to help loosen the congestion so I can cough it up. This takes anywhere from an hour to two hours -  and can be exhausting if I'm really congested.

Then I can start my day.

Get food (Need those calories to maintain my weight - coughing burns a LOT of calories).  Did I mention I wear oxygen? I didn't.  Ok - I wear oxygen.  I don't notice it so much when I'm sitting in one place, but when I begin to move around the house, the tubing leash I have trailing me can get caught, stepped on or run out.  Making food in the kitchen is a hassle, because it reaches just to the refrigerator, but not the stove, cutting board, dishwasher or sink... convenient when I don't want to clear my dishes, but not so much when I'm alone.  Off comes the oxygen.

After eating, I pop pills and shoot up.  No, seriously.  I take about 15 different pills every day... many of them multiple times a day.  I won't list them all, so you'll have to just trust me on this.  I also have CF related Diabetes for which I take insulin shots whenever I eat.  These two things are probably the easiest part of my health maintenance, and I'm really looking forward to a day when that's ALL I'll have to do.

One difficult part about CF is that because I need so many calories, I'm often eating a large volume in one sitting, which makes it difficult to breathe.  With the stomach pressing against the diaphragm and lungs, I end up having to cough and clear after meals.  Which is okay if I plan to be near a trash can or toilet. Yeah, sometimes I cough until I barf.  Although that has happened a lot less since I had my gall bladder (which would swell after eating) removed.

Before I get myself ready to leave the house, I have to make sure I fill up my portable oxygen device an hour before I plan to need it.  It has to build up pressure to be able to function properly, and if I'm unable to use it, my blood oxygen saturation will drop and that causes the heart to work too hard (and the last thing I need is a heart transplant, too!).

When I leave the house, it's usually time to give another good cough.  I often purposely cough when I'm driving places so I won't have to when I get where I'm going.  One of the hardest things for me to deal with is coughing in public.  I hate to gross people out or worry them.  People think I'm choking and get really alarmed when I have a coughing fit.  I find myself giving the dorky thumbs-up just to get them to leave me alone until I catch my breath.  I hate having to justify it afterward, "Oh yeah, that's normal for me.  And it's not contagious."

When I'm home during the day, a lot of times I take a nap, which for me symbolizes starting my day over: I get up, cough, and do a breathing treatment.  The added exertion from coughing and struggling to oxygenate my blood often wears me out in just a few hours out of the house and I need naps!

Before bed, it's the same routine as the morning: breathing treatments, vest and bedtime snack (food).

I share this, hoping that more people will understand when someone says, "I have Cystic Fibrosis".  It's not just a label, it's a lifestyle.