In the Process of Processing

I met with the pancreatic surgeon yesterday and my CF doctor today.  There is a lot of information to talk about.  But I need some time to process it all.  Once I do I will post an update.

Tobi Podhaler

I started the pod haler on Friday.  So far it hasn't been awful to use.  I only have a 7 day trial so I won't get the full 28 day dosing, but we are doing it to see how my lungs react to it.  I have a horrible reaction to inhaled antibiotics.  Severe bronchi spasms.  Hate them.  So far nothing serious like that but its only been 3 doses.  The first night, Friday night, I had quite a few suffocation dreams and P said I was moaning a lot in my sleep.  So last night I upped my O2 from 1.5L to 2.5L and it seems to have worked.

Saturday morning I also woke up with a sore throat.  But that could be from the inhaled meds.  Or so I thought.  Today I woke up with a left eye that won't stop watering, a nose on constant drip, and sneezing up a storm.  Looks like a cold.  Fabulous.

I have clinic on January 28th as my follow up from the 3 weeks of IVs (if you want to call it that) and to discuss how the pod haler worked for me.  Or didn't.  I also have an appointment with a pancreatic surgeon on the 28th.  My GI doc at Dana Farber agreed that I should meet with one to discuss the possibility of removing that precancerous cyst from my pancreas.  She doesn't think I need to right away, and that monitoring it will be sufficient, but it dawned on me on Friday that BWH will NOT transplant me with a precancerous cyst.  So if something were to happen to me before it was removed, and my lungs took a dive, I would not be able to be listed until it was removed.  So why wait?  I need this bad boy removed asap.  That is something I will discuss with the surgeon on the 28th.

So until the 28th my lovely blog readers...

One More Week

One more week to go on Ceftaz...yaaaaaay *sarcasm* I can deaccess on Monday.   I'll be down there anyway for a derm appointment so I am going to go in after to do PFTs.

My numbers are up some 1.05L, up from .85L.  33, almost 34%.  Still not up to my usual 1.11-1.13L but its an improvement.  I don't expect much more since its only Ceftaz I'm on and 2 orals.  Which really sucks because next infection will drop me low again.

She wants me to get a hearing test next time I come in since I haven't ever had one.  Glad.  I don't think my hearing is horrible but I know certain pitches I can't hear.  Whether its age or TOBRA related who knows.

That's all for now.

Home from the Hospital

I am home from the hospital.  I actually got out on Monday but I have been so busy finishing up my paper, that I turned in Wednesday, and appointments, that I haven't been able to update.

I was admitted last Wednesday night and for some reason on Thursday afternoon I spiked a fever.  102.3 at its highest.  No flu, no blood infection.  Nothing out of the ordinary except that fever.  Tylenol brought it down and by Friday night I was back to normal.

The doctors started me on Q36 for the Tobra this time since we always end up at that point anyway.  Seemed to work well.  Then I came home.  I had 3 doses total when the nurse drew my labs Wednesday morning and I was told yesterday to stop taking it.  Kidneys are not happy.  WTF!?  So now I am on oral Bactrim, oral Cipro and IV Ceftaz, aka. cat piss.  Hoping my FEV1 came up a bit so that I can stop the IV Ceftaz before Christmas.  I have clinic on Tuesday.  My body never responds to orals.  This is just wonderful.

Yesterday, Thursday, I had my follow up appointment to my colonoscopy.  It was a little disconcerting.  My colon polyp was adenoma as usual.  That wasn't concerning.  But, like I mentioned before they were finally able to biopsy that pesky cyst on my pancreas.  Turns out it isn't CF related at all.  Its a precancerous cyst related to my Lynch Syndrome.  Like the adenoma in my colon, if left untreated it has the potential to turn to a cancerous tumor.  "Biopsy of your pancreatic cyst revealed benign cells and CEA level 825 with amylase less than 3.  This may be consistent with a mucincous type of precancerous pancreatic cancer."  Fabulous.  We didn't' discuss removing the cyst.  Honestly I was a bit in shock that it WAS something that I forgot to ask about surgery.  She wants to monitor it every 6 months with MRI/MRCP's again, and if it grows, I can talk to a pancreatic surgeon then.  I am going to email her about just having it removed.  I don't want that shit growing.  On the bright side, I don't need mammograms just yet...

That is all for me.  School is over and I am doing lots of crocheting and sleeping.  I will post an update next week after my clinic appointment.

It's That Time Again

Yup it is time for IVs!!!  This way I will feel fabulous (or as fabulous as someone with my lung function can lol) for Christmas and visiting my family.

I blew some really shitty numbers today.  FEV1 of 28%, .85L ha!  Last time, Sept 17, I blew 1.11L, 36%!!!  Holy drop batman!!!  I haven't seen numbers like that since I did the Vertex study in Jan 2011.  I was glad to see them so low because then she wouldn't suggest Cipro and Prednisone first.  She did get an Xray done to make sure I didn't have a collapsed lung or anything like that.  And I don't think I do or I would have heard back by now. 

BCH and BWH made some changes and all CF patients up to age 35 have to be admitted to BCH unless they are listed for transplant or already transplanted.  Luckily for me, I am technically listed for transplant even though I am inactive on the list.  Seems weird to say that.  But that means I can stay at BWH and not get used to another new hospital.  Yayyyyyyy!!!  

So it looks like tomorrow night I will be admitted and then I can be home on Monday, just in time to finish up my grad class.  Lots of editing of my paper and crocheting will get done!  Plus I starting getting a new magazine so I have 2 of them to bring with me.  I won't be bored that is for sure!

Time again for IVs I think

I had to bump my clinic appointment up to this Tuesday from December 17th.  Its only a 2 week bump but with Thanksgiving there was no way I could get in any earlier.

Anyway, my lungs have been really horrible lately.  I am super SOB, super tight, and in a good amount of pain 24/7.  Since my doctor took me off of maintenance Motrin a few months back I deal with minor pain constantly.  But nothing I can't handle.  However, the last few weeks I have been popping it like candy again.  I try really hard to avoid taking it but the pain gets so bad and I get so uncomfortable I have to take it.

I am also back up to 4 treatments a day and sometimes 5.  I barely make it 4 hours before I am sucking back the meds.  Sometimes, particularly at night, I don't even wait that long.  I just can't breathe at all.  I could barely make it up the flight of stairs, going super slow, at the library today and that was only 1.5 hours after my morning treatment, when I am supposed to be in my "best shape."

And the junkiness.  Can't forget the increase in the wonderful mucus.  Not much, but for me it is.

I really hope she just goes straight to IVs and does not want to try Cipro and prednisone first.  I go to PA right after Christmas and I don't want to feel like shit when I am there.  I always do and now I have a chance to be in better shape BEFORE I go down.  I don't know how it will all work anyway since we are no longer inpatient at Brigham and Women's but instead admitted to Children's.  This should be fun... I'm also worried she will want to start IVs but only at home and not admit me since we are in Flu season.  I like starting my course inpatient.  Its nice to have those few days to relax and not do household work, or cook.

The good news is if I do go inpatient I can finish up my final paper and some crochet projects that I have.  My last day of class is December 11th and then I am off for almost 6 whole weeks.  Being inpatient will give me some time to finishing editing my 38 page paper (ha its supposed to be 20ish opps).  Seriously I am really looking forward to a few days to rest.  I don't do that at home, that's for sure.

My appointment is on Tuesday and if I am lucky I will go in on Wednesday or Thursday.  This way too, if I have to do 3 weeks I will be done by Christmas, just ha!

Nothing out of the ordinary

I realize its been almost 2 months and I have no excuse, only an apology.  Just really busy.  I guess that is a good thing right?  Lots of things coming up this month though.  I have an ENT appointment on the 15th and my annual colonoscopy/endoscopy on the 22nd.  I will keep you all posted on the results of those.

Other than that...just a bit more junky than normal.  Coughing a good amount and actually bringing junk up.  I don't see my doc until December 17th so crossing my fingers I last that long.  I have that sample of the TOBI podhaler sitting in my drawer but I am not really excited to try it.  We shall see...

Fabulouso

Today's clinic went a little better than I had thought it would.  They just updated their PFT cart so now there isn't a flexible tube, but instead a rigid stand.  They also changed the predicted values to the standard.  So before I was switching to my calculations, for this blog, I now don't have to do that.  My "new" predicted is 3.06L which is only slightly below what I was using at 3.10L.  The difference is really insignificant (1/2%).   I was expecting to see my % go down because of the new predicted values....

So now on the results of said clinic.  My FEV1 went up slightly instead!  I was at 1.03L in July, exactly 2 months ago, and this time I was 1.11L.  My % stayed the same at 36%, but now it is a true 36%.  I shall take that 3% increase (my calculations were 33% for 1.03L last time - are you as confused as me?!?!).  However, I am still lower than I was right before I was admitted back in June when I was at 1.13L.  But that is a small difference.

My weight was down 5 lbs, but my appetite has been sluggish and with the exercise it is to be expected.  She was not worried about it because I feel FABULOUS.

Yes FABULOUS.  I feel great.  There were no issues to report.  No extra coughing.  No fevers.  No chest pain (besides my regular which hasn't been as bad as I had expected given my no Motrin usage now).  Nothing, nadda, zilch, zero.  I told her my O2 has been higher with rest - usually I am around 93-94% on room air at rest, sometimes 95% if I sit long enough - but lately it has been as high as 97-98%!  And my exercise tolerance has improved dramatically too.  I have started doing rolling hills on the treadmill and strength training twice a week.  Once with the trainer and once on my own.  I don't push myself as hard as she does, but its easier to lift heavier than you can with a spotter.

I was told to come back in three months (something I haven't done since moving up here) and call if there are any issues in the mean time.

I could not have been happier with today.

Surviving

When you have CF you spend your life waiting for the ball to drop on your health.  You live life as best as you can: you go to college, you get a job in your field, you stop when you need to and in between you fit in hospital stays, IVs and therapies daily.

But what happens when you hit your 30s, you can't work because your health is too precarious, and you have been evaluated for a double lung transplant, and then you learn that you MIGHT get a new drug that could push that expiration date to over 60?!

It sounds so exciting and amazing and just plan AWESOME.

But it also sounds scary and emotional and weird.

WHY???

Well that's because you spent your whole life up until this point thinking that you would never ever see 40.  That making it to almost 33 is a miracle in itself.  And that the thought of living to 60+ is just impossible.  I know to someone who hasn't had to deal with this it probably sounds ridiculous.  But let me explain.

I don't remember when I became fully aware of CF and its consequences.  I always knew my life was going to be shorter but I don't think I ever truly THOUGHT about it.  Not until 15 or so years ago anyway.  Then I began to think about my life expectancy.  I went to college right after high school and I got a job in my field right after college in the fall of 2001.  I worked in my field for 8 years, almost to the month (November 2001 to December 2009).  I participated in the 401k offered by all my employers but I don't have one anymore.  I took the penalties when I moved up to Boston and took that money to survive on until I was approved for SSDI. When we would have meetings in work with the 401k company I would tell them I was not going to live to retirement age so what were my options?  Just get it anyway.

I have never thought about burying my parents or living without them.  I have always assumed I would be buried first.  I have never thought about old age and spending 30 years with my husband.  I always assumed I would be dead by 40.  I never expected to see my niece and nephew graduate high school, college, or get married.  I haven't worried much about things because I was expecting to be dead by 40.

Now I am faced with the possibility of ALL of those things.  I might have to bury my parents.  I might spend the next 30 years with Peter.  I might see my niece and nephew grow up and get married.  I might make it to retirement age and I might not be prepared.

When you expect to die early you live your life completely different than someone who expects to retire some day. And not just in "living" life, but in preparing for old age.  Why prepare if there is no need for it?  I wanted to keep my 401k in a 401k when I "retired" in 2009 so that my family wouldn't have to be burdened with my funeral costs.  I was told by them not to worry about it, to take the money to survive on then.  You think differently.

How does one cope with this new prospect of life?  Especially for someone who likes to be prepared, how do you cope with the possibility of NOT being prepared to retire?  Kalydeco, when it comes out for DDF508 mutations, will change my life forever.  I will live to retirement age.  I will grow old with Peter.  I will be able to go back to work.  But its going to be like starting out at 35.  Those 14 years where I was working, and then not working, will be like they didn't exist.  I will have to start from scratch.  And let me tell you how scary that thought is.

I don't want anyone reading this to think I am being ungrateful.  Believe me, I am thoroughly ecstatic that I will get to do all that I wanted to do again without needing new lungs.  I won't have to worry about the threat of cancer being exacerbated tenfold because of immuno-suppressant medications. I might get the chance to be as close to normal as possible.  And that is amazing and exciting to me.

But I still have to deal with things I never thought I would have to.  I guess it makes me human and normal to now think about things that my husband and brother and friends think about.  To worry about retiring and having money to do it.  To worry if I will spend the rest of my life working somewhere I hate to get a good retirement, or if I will do something I love for 30 years.  The possibilities are endless, and though they are scary and exciting, they are inevitable.

I hear there are survivors groups for people with CF living over the age of 40.  Maybe we need more of these, for those who will be changing their life's outlook completely in just a few short years...or less...

PRIDE

Pride

It's a greedy little bastard that keeps us from doing things we KNOW are right.

Pride

It's one of my many faults.

Pride

It's something I WILL overcome.

Why can we not admit when we do something that is wrong, and we are called out on it, and we know that we need to change our ways?  How hard is it to modify your attitude slightly so you can do this tiny little thing that you know needs to be done to make someone happy?

Apparently it is very hard ha!  But I WILL change it.  I am working on it.  I am trying my hardest to not let my pride get the better of me.  I have to do this, not just for me, but for those around me.