Five Feet Apart

Out of my hiatus just to write about this movie.  We knew it would happen ;) 

I went to see Five Feet Apart today.  By myself.  I had no desire to see it with anyone, no not even Peter, sorry babe.  I needed to see it alone.  

If you aren’t sure what I am talking about, and haven’t heard of the movie, here is a brief synopsis.  It’s a movie about two teens with CF who fall in love in the hospital.  And the movie is about them taking back one foot of the requisite 6 feet that us CFers need to keep between ourselves and others with CF.  It’s the first time CF is a feature in a major motion picture.  Claire Wineland was a consultant for the movie – she was a huge presence in the CF world until her death, after transplant, last year.    

Here is a link for the trailer

It was a great movie.  I don’t think it was overly sad (some people will disagree with me, but it was what I had expected).  More tears were shed at the previews before the movie (OMG another dog’s life movie!) than for the actual feature.  It was a romantic drama that happened to have two main characters with CF.  If it isn’t your genre of movie you probably won’t like it, CF or not.  I probably still would have seen it even if it focused on Cancer or Autism or anything else because I like these types of movies.  

I should elaborate a little on the not crying part.  I think over the years I have become pretty jaded with CF.  Friends pass away and I cry for a day and then I move on.  It's how I deal.  So I think some of that rubbed off on this movie.  The next time I see it, I will most likely cry some more.  

There were some things that were all “OMG why are they talking about that or showing that” etc.  But you have to remember that not everyone knows WHAT CF is.  Many people haven’t a clue.  So there had to be some backstory to it.  There had to be a lot of explaining so people out the “outside” know what is going on.  And they explained a lot.  Not all, but a lot. 

My expectations were fairly neutral given the nature of the movie. I knew it wouldn’t be spot on, its Hollywood people.  But all things considered I think they did a bang-up job with it.  Were there inaccuracies?  Of course, like I said, it’s Hollywood.  Did they exaggerate a lot of things?  Of course, again, It’s Hollywood.  But the feel of CF I think they came close to nailing on the head. 

When you get to the point where CF has taken everything from you, your friends, your job, your freedom, you live to do your treatments, as Stella (and Claire) says.  Your life revolves around your health.  You miss out on so much being sick or being in the hospital.  They really showed this in the movie.  Maybe it was just me knowing how they felt, but I think they portrayed it well.  They dealt with survivor’s guilt, loneliness, fear before a procedure, isolation, rebellion, mortality, friendship, losing a friend with CF, missing out on life, not wanting to drag someone else into your “mess” (that one hit home the most for me and it’s still something I struggle with to this day), and of course, love. 

The movie was far from perfect as so many “sick” movies are.  They have to keep the audience’s interest.  If one wants to see a documentary on CF they can.  This surely was not one.  But no matter what people may think of this movie, it will do one thing: bring light to a disease that is not well known. For us in the CF world, it is everything.  But for those who know no one with it, it is nothing.  And this movie has the potential to change that. 

Rest in Peace Aunt Marybeth

Monday my Aunt passed away.  She had pancreatic and liver cancer.  The past two months she has been hospitalized and this was expected, but not expected.  She leaves behind 2 beautiful daughters in high school.

She was the youngest of my father's siblings, the baby.  Not even 50 years old.  She is also the first in this generation to succumb to Lynch's deadly reach.  It just goes to show that no matter how proactive you are, sometimes, it still gets you.  A sobering thought.

There are so many fond memories I have of her.  When I was 15 she took me to my aunt's farm to start horseback riding again.  My love of horses blossomed from then on.  I rode for 3 years before going to college and not having the time to continue.  On those weekly Saturday excursions, she would let me drive her little red car.  It was a stick shift.  So I learned to drive before I was 16 on the rocky driveway into the farm and sometimes even on the open roads up there.  She was a great rider.

I remember going away for the annual ski trip with the family and my brother and I hitching a ride up with her and my then uncle.  So many great memories on those trips.

I remember going to the baby shower for her first baby, my cousin.

I remember the pool at her house and the dogs and the parties we had there.  I had my high school graduation party at her house.  It was a blast!

As I got older, I lost contact with her in the sense that we didn't see each other as often as we had.  But just like the others, I saw her at all the family gatherings.

And just this past October I was blessed to be able to spend a few days with her and my other aunt in Maine.  It is a few days I will never forget.

When I heard the news she had cancer in April, my heart broke.  But I never gave up hope that she would survive.  We are a strong family.  But sometimes, we survive by leaving this world.  She may be gone but she will never, ever be forgotten.  She is no longer in pain, no longer suffering, no longer wondering if and when she will get better.  She always had a smile on her face and love in her heart.  And until the day I join her, I will always remember that smiling face and the games she loved to play.

Rest now Aunt Marybeth.  We will always love you.

Clinic Update

I had my 3 month clinic appointment yesterday.  I can't believe I survived 3 months without a call in there somewhere.

All my numbers are the same.  I was 1.09L on October 1st and yesterday I was 1.08L.  37% and holding. I will take it.  My weight was down another 1.5lbs but I am OK with that.  I am still 116 and that isn't a bad weight to be.  I'll worry if I hit 110 or lower.

I got the all clear to star full dose Orkambi in Tuesday.  I go away this weekend so I am waiting till I get back to start.  I should be fine but hey you never know.

Still no need to start insulin.  I had a few weeks where I was running high, even my fasting numbers, but then other weeks where my fasting was normal.  One day they were 66 when I got up!  Kinda low!  So long acting is out.  And my over 200s are not consistent enough to need insulin before meals.  So its keep monitoring and see how they are in 3 months again.  Easy enough.

That is all.  Boring life here lol.  But at least there are no crazy health issues in my life right now.

I want to be a bookseller

Last weekend P and I decided to take a drive.  It was New Year’s weekend and with three days off, he was starting to get bored and I was restless having everyone at home with me.  There were a few books I wanted to pick up so we decided to take a trip to our favorite used book store up in the Ipswich area.  We meandered up and when we pulled into the parking lot we saw the building was EMPTY!  The whole thing!  No used book store and no antiques shop below.  Just a sign saying they would be back after lunch…..no forwarding address, no we moved sign.  NOTHING.  I was (still am!) devastated!!!  We loved that place.  Not to mention finding a used book store that isn’t part of the Salvation Army or in Barnes and Noble is near impossible. 

As we drove away I had the very strong desire to open my own book store.  A quaint little shop here in our town where we would sell some new, old and maybe even rare books.  We could have a small little coffee station in the store and a lounge area to read.  Free wifi of course and I could bake some bread and make some jams to sell.  During the slow hours I could relax and crochet and read.  I wouldn’t have to have the store open 10am to 9pm like retailers; a reduced hour schedule like noon to 7pm would be perfect.  Maybe only a few days a week too.  I go have book club meetings and maybe even work with the library on things. 

I thought of it all.  I got totally wrapped up in it, even telling my mom she could move up here and work it with me. 

But it isn’t possible.  At least not right now. 

But WHY?

Am I just scared and using my health as an excuse?  Or would it really be a bad idea?  I love to read.  I would love to have my own shop.  I have the perfect location in mind.  I could find funding I am sure (grants for women owners seem to be a good place to start). 

But could I do it?  And should I do?  And will I do it?

Maybe…someday….maybe…never…

How to get your 20g of fat to take with Orkambi

Tomorrow is the big day for me.  I am scheduled to start taking Orkambi.  And it got me ransacking my home looking for things that have 20g of fat in them to eat when I take the drug.  Which then got me thinking that I should compile a list of foods and beverages that someone can take or add on to things in order to reach the 20g goal.

This list is far from complete and I will gladly add on any suggestions!!!  Some of the information is taken from my house and some are taken from this website.

Junk Foods

Entenmans powdered mini donuts – 4 donuts = 12g

Oreo double stuffed – 2 cookies = 7g

Ice cream – ½ cup = 9g (varies based on flavor)

Potato chips – 1oz = 9-10g

Original Sun chips – 1oz = 6g

Cheese and crackers – 1oz = 7g

Chocolate pudding – ½ cup = 4.5g

Cheese puffs – 1oz = 10g

Beef Jerky – 1.5oz = 11g

Chocolate bar – 1.5oz = 13.5g

Chocolate chip cookies – 4 medium sized = 11g

French Fries – medium order = 24g

  

Healthy(ish) Snacks

Almonds – ½ cup = 24g

Cashews – ¼ cup = 14g

Sunflower seeds – 1oz = 14g

Peanuts – 1oz = 14g

Skippy creamy Peanut butter – 3 tablespoons = 24g

Nutri-grain bar = 3g

Quaker brand Granola – 1/3 cup = 2.5g

Trail mix – 1oz = 8g

Yoplait original yogurt – 1 container = 2g (Yoplait Light has 0g)

American cheese – 1 slice = 9g

Swiss cheese – 1 slice = 8g

Provolone cheese – 1 slice = 7g

Cottage cheese – 1 cup = 10g

Cream cheese – 1oz – 5g

Colby cheese – 1.5oz = 14g

  

Fruits and Veggies

Avocado – 1 cup = 21g

Coconut – 1 medium = 133g

Meals

Spaghetti’os with meatballs – 1 can = 12g

Prepared Kraft Mac and Cheese – 1 cup = 16g

Ground beef 85% - 3oz = 13g

Chicken – 1 cup = 19g

Pork loin – 1 cup = 12g

Sausage – 2.7oz = 20g

Duck – 1/2cup = 20g

Lamb ¼” fat – 3oz = 18g

Bacon – 5 slices = 16g

Hot dog no bun – 1 = 13g

  

Drinks

2% milk – 1 cup = 5g

Whole milk – 1 cup = 8g

1% milk – 1 cup = 2.4g

  

Add on’s

Extra virgin olive oil – 1-1/2 tablespoons = 21g

Butter – 1 tablespoon = 11g

Sour cream – ½ cup = 12g

Ranch dip – ¼ cup = 8g

Follow Up Appointment

Today I had my follow up appointment at MGH after my 2 weeks in house for IV antibiotics.  Today's appointment was actually my 3 month from last appointment but I squeezed in a sick visit and hospital stay lol.

All looked good.  My FEV1 was up to 1.09L 37% so back to baseline.  My weight was down another few pounds but I lost 3 pounds in the hospital and 2 pounds visiting my family which will all come back on once I get back into my routine here at home.

I got to meet with the endo and I LOVE HER.  So much better than the one at Children's who was a bitch to me.  She was super sweet and sat with me for close to an hour going over things.  I had a 3 month log for her to review and she said right now I don't need insulin but to keep an eye on highs.  We may throw a short acting one in before certain meals if I know it will cause me to spike and crash.  She also wants me to get a dexascan (bone density) done before my next appointment.  I swore I had one 6 months after my hysterectomy but it is only showing one right before.  And at that time I was slightly below normal for my density.  So she is also having me start on an estrogen patch to help with some issues and to be sure that my bones don't get too weak.

I am scheduled to start Orkambi on October 13th and I am scared!  Check out my guest post on the CFF blog to see why!  That and the side effects can be brutal the first few weeks.  I am not looking forward to them and hoping I don't get them.  But the side effects are shortness of breath and chest tightness which is my thing.

I got my flu shot today as well.  Hello sore arm for a week lol!

I was also told that my sputum culture showed MAC (Mycobacterium avium complex).  Right now I am not worried.  One positive does not mean I have MAC in there.  Especially since I feel good and I responded to the IV antibiotics, which would have done nothing for a MAC flare up.  So I gave another culture today and we will see what it grows.  Most likely it is a false positive which is very common for it.  But if I grow it again then we will have to reexamine what to do.  Since I feel good it could just be a monitoring thing.  Which would be way better than 12+ months of IV antibiotics!!!

Other than that things are going well.  I see transplant clinic tomorrow and they can tell me to come back in 6 months again.  I may see if I can do yearly.  Seems silly when I feel good to be there twice a year.  But who knows.....

Hospital Time!

I had a sick visit clinic appointment today.  Recently I have been more short of breath and have been having some lung pain.  And since I am supposed to be starting the new drug Orkambi soon, I wanted to be sure I was in tip top shape.  The first few weeks on it people can be more short of breath and tight and since that is normal for me, I don't want to make it any worse.

So Wednesday I will be going inpatient at MGH for the first time, for two weeks.  Then when I get out I can start Orkambi and see if it works on me!

Also, I did a 24 hour urine collection last month to see how my kidneys are functioning.  Well the place we used screwed everything up so I need to do it again.  Clinic tried so hard to understand the results with no luck.  And for some reason the place put down I only had 500 mLs of urine which is nothing when I know I had at least 2100 mLs since I looked before I dropped it off.  So I get to do that again tomorrow so I can bring it with me when I am admitted and then MGH can handle it.  He also mentioned that I might need to see a renal doctor after we get the results.  But we are going to give Tobra another try which I like because my lungs always respond very well to it.

Guest Blog on the CFF

Guest blog post up on the Cystic Fibrosis Foundation's new blog!!!

My Decision to Stop Working

I will be doing another one in a few weeks about Orkambi.

Check it out, I was super excited to do it :)

Game Changer

I have discussed this before, but now that reality is happening.  Orkambi has been approved and is available to those with DDF508 mutations.  ME.

Vertex' drug has been approved and my life could change.  Dramatically or barely noticeably.

So as I sit here thinking about what could happen I find myself floating in a sea of endless emotions.  I am excited I may be able to go back to work.  Excited I may be able to earn money again, and not just collect SSDI.  I may get the chance to grow old with my husband.  To see my niece and nephews graduate and get married and have babies of their own.  To watch my own step kids do these things.  I may get to experience all the things that old people experience.  The good and the bad.

So why am I feeling this barrage of emotions?  And why are there some not so happy ones mixed in there?

Because I have lived almost 35 years with this disease.  I have come to know my body, my progression, my health.  I know when I am a little out of sorts or a lot.  I know what to expect of it, most of the time.  I am pretty vanilla.

I have also lived my whole life expecting to die at any point.  That my life has a early expiration date that is gaining momentum as I age.  I have put aside thoughts of ever having to bury family members and never having to bury a husband.  Never seeing anyone get old and never having to worry about it for myself.

But this medication could change all that.  I don't know what to expect.  I do know to expect the unexpected.  I know I have to think about retirement, real retirement.  I have to think about old people diseases and aging.  I have to think about watching my parents age.  All these scary things that most people don't even consider because they are a part of a long life.  But I never thought about a long life.  Hell I thought I would be dead by 26 but here I am almost 35 and alive and kicking.

So what do I do now?  The answer is complicated.  I know I have to sit back and relax and see if I even get any help from Orkambi when I can get it.  And then I can start to think about the rest.  About growing old....with my husband.

Maybe I will be able to sit on the porch on rocking chairs and watch our grand-kids play in the yard...maybe, just maybe....

My Kidneys

My Kidneys....

I am not quite sure what is up with them.  Or if it is even them causing me issues.

Back in March I had a BAD UTI.  So bad I was peeing purple....yes PURPLE.  I should have taken a picture it was so weird!!!  Even the nurse and doctor commented they had never seen pee that color.  Fabulous.  I got an Rx for Bactrim and within 36 hours my pee was normal again.  I was on a study for colon cancer and contacted my study coordinator to let her know.  I thought this was my first UTI.  Turns out it was my NINTH.  I never knew about the other ones because they were discovered when I was inpatient and they cleared up with the IV antibiotics I was given for my lung infections.

So no one ever told me about them.  I had a feeling something was going on because each admission I would have to do 2-3 collections since the first and sometimes second would have microscopic blood in it.  But no one ever told me they were UTIs.  For 3 freaking years I have been having them!!!

Fast forward to my MGH appointment in June.  We had discussed doing a 24 hour urine collection to get an idea of how my kidneys are functioning especially since I am no longer taking IV Tobra.  So Monday I start the collection.  I had been feeling like I was getting a UTI since it felt a little funny to pee but I just ignored it.  Monday night, more blood in my urine.  So I am freaking out that I will have to re-do the dam collection again (I had done it on Thursday not realizing the place I had to drop it off was closed Friday!).  I called my CF clinic Tuesday and she said not a big deal it actually helps because then they can see what is going on better.  And then I called my PCP to go in and give a sample and get more Bactrim.

Now this is 2 UTIs in 5 months that have visible blood in them.  My thinking is that I am usually getting them but I go inpatient for IVs before the visible blood starts.  But now I am going on 6 months without an admission so my body isn't getting the drugs to combat them.  So I pee blood.  March's I have no idea what that was all about.

Hopefully the 24 hour collection will reveal something about what is up with these infections.  They are not fun at all.  And I cannot figure out a cause for them.  I just hope it isn't anything serious.  My mind is obviously thinking my kidneys are failing or I have UT cancer since that is part of Lynch.  But my fingers are crossed its something silly that can be fixed easily.