Thursday, October 1, 2015

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.....

Monday, August 24, 2015

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.

Saturday, August 15, 2015

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 :)

Monday, August 3, 2015

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....

Wednesday, July 29, 2015

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.

Tuesday, July 28, 2015

One Year Post- Whipple

Today marks my one year since having the whipple done on my pancreas.

Last night I re-read all my previous blog posts prior to surgery.  I re-read how scared I was of not bouncing back.  How worried I was that it was cancer.  How necessary the surgery was if I were to ever have a transplant. I re-lived all those emotions last night.  It was quite the roller coaster even on this side of the ending.

Not much has changed in a year, besides my weight and the long scar I now have on my torso.  I am at my ideal weight (Mine not anyone else's) at 116 lbs.  But I had put on quite a few pounds before surgery to be sure I wouldn't fall too low.  I was 132 lbs going into the operating room.  Way more than I ever wanted to be or want to be again.

My health is doing great.  I still do not require insulin although my OGTT I had done recently still put me in the abnormal range.  So I am monitoring sugars for my appointment in October.  But I am not diabetic like we thought might happen.

I did not have any serious complications after surgery which to me is a miracle in itself! The stats for complications were ridiculous!  Besides some lingering nausea into the new year, I was complication and side effect free.  I have my appetite back and I can eat large meals again, which only took about 9 months to sort out.  My bowels have returned to normal too.

It is almost like surgery didn't happen.

Here are some photos to prove that it in fact did happen:

Getting changed into my gowns.  

Putting the IV in.  I can't look when it is inserted or I pass out!  

after the epidural.  Those things are awful but man do they take the pain away after!

post surgery when I was still out cold.  You can see my collection tubes on the left with liquid in them.  They were more annoying than anything.  I had to carry them every time I got up after I was mobile.  And they were sore.  And pulling them so I could go home was HORRIBLE.  I almost broke my mom's hand when they pulled them.  Fuckers were like 4 feet long (I have no idea how long they actually were but let me tell you they felt like they were a mile long)!  

1 week after surgery.  Scar looks pretty good here!  I was still bloated from everything.  You can see the vampire bite on the side of my abdomen.  That is where the two tubes were.  

You can really see the difference in my face with the weight I lost.  This was 11 days post surgery.  I had a really good day and felt great this day!  I remember it well.  It was a rare feeling those first few weeks.

This is my 6 weeks comparison photo I posted on Facebook.  

And this is me today.  My scar looks awesome. I am not embarrassed by it at all.  In fact I love showing it off.  Its something to be proud of.  I can say I survived the whipple.  Not that I wear skimpy clothes but I have a bikini I bought that I love to wear!!!  

Some previous Whipple posts:

Monday, July 27, 2015

I saw Dr H!!!

I was in Philly July 16-20 so I made a date with Dr. H to visit with him.  My mom came with me.  It was SO FREAKING AWESOME to see him again!!!  He hasn't changed much, only his wardrobe is more up to date.  I did miss the tweed jackets!

We chatted for about an hour.  Just about advancements in CF medicine, my health and life in general.

It was like seeing an old relative that was super beloved.  I am so thankful I got to see him.

Tuesday, June 16, 2015

Feeling the need to be more creative

I think I want to get back into painting.  Although between the crochet and sewing and baking and research for the Wakefield 4th of July parade I am pretty much out of free time.  But after the 4th I can finish up the sewing projects I am working on and maybe I can add some painting into the mix.  I need to get back into drawing and that sort of art.  I miss it!  Maybe I can take another class again this fall like I did a few years ago.  I really enjoyed it.

Tuesday, June 2, 2015

First Appointment at MGH

Today was a very long day.  But it was worth it.  I really like the clinic at MGH and I am going to stay there.

I had a full PFT appointment at 8:30 am so I had to be up and out the door by 6:30.  It has been raining for 3 days and I knew traffic would be a mess.  It was.  PFTs went well, I have not changed since April which is good.  They did a post as well and I shot up 3% from 37% to 40%.  It doesn't seem like much but it is a 9% increase which is huge!  I got a little panicky in the booth when they blocked the air for the test.  But I only had to stop once.  The woman was very nice and didn't get annoyed thankfully!!!  I never had that happen before.  Good old anxiety haha!!!

At 10:30 I had my clinic visit.  I saw the nurse and we went over everything.  It was probably close to an hour we chatted.  I had to give a history and medications etc since I am new.  I talked about some of the issues I have and things I would like to focus on.  We are going to do another OGTT to see how my sugars are.  I am going to monitor and log my sugars so when I see the Endo I have a good idea where I am at.  I scheduled an appointment with her for September.  I am making an appointment with the PT to do an exercise tolerance test and to set up an exercise plan.  I am going to see a gynoendocrinologist who focuses on hormones etc after menopause.  I never knew someone like that existed and I think it will be great to meet with them.  We are going to do an overnight O2 study to see how my numbers are when I sleep.  I haven't been using O2 when I sleep the past month or so and I have been feeling OK.  But I want to be sure I am not hurting myself by doing this.  I got a prescription for Ativan which I loooooove.  So thankful they gave me one for my anxiety.

After the nurse, I met with my new CF doctor.  He was great.  Very personable and open and I felt very comfortable with him.  We talked about everything, my history, action plan etc.  One thing he wants to do is have a 24 hour urine collection done on me to see how my kidneys are actually functioning.  Since IV Tobra has been off the table for a while but my kidney functions have always looked OK, he wants to see if there is a reason why they stopped the Tobra.  I was never really given a reason just that my numbers were off and we tried different doses and times to fix it but it didn't work.  So he wants to see if maybe there is some kidney damage that isn't showing up on my blood tests.  His main concern is that I am treated by the same family of antibiotics instead of getting two families in there.  So if we can add Tobra back in that would be good.

Overall I was there for 3.5 hours and I feel like I got so much accomplished.  I felt very comfortable there, not like I was out of place at all.  At BCH you get shoved in a room and I always feel like I am a caged animal.  AT MGH clinic I was put in a room but I was able to leave to use the restroom and when I left I didn't feel like I was just another person waiting to check out.  I chatted with the woman at the desk and it was nice.  I felt like they cared and like I wasn't just another patient on the list.  I missed that from my old clinic in PA.  I am so glad to have it back.

So a shout out to my 3 great Cysters to answered my numerous questions over the last few months and helped me get set up to move!!!  You ladies rock!!!

Now to send a good bye letter to BCH...

Wednesday, May 27, 2015

CF Awareness Post Question Day 27

How does having Lynch Syndrome affect your CF? And vice versa?

For those who are not sure what Lynch (HNPCC – Hereditary Nonpolyposis Colorectal Cancer) is I will tell you.  It is a genetic predisposition to certain types of cancers.  They include colon, anus, pancreas, brain, sebaceous glands (skin), uterus, ovaries, bile duct, urinary tract, stomach and small bowel.  Basically the digestive organs and some reproductive organs. (I have a blog post about it here: I apologize for the text, I have changed my blog layout since it was written!).  My family carries the hMLH1 mutation, but there are four total.  A strong family history means you should probably get tested for the gene.  This quote from the print out I have will help calm anyone out there that is thinking OMG my great granddaddy had colon cancer, and that was it, in my family! “Persons at risk for HNPCC usually have a family history of two successive generations of colon cancer or at least once generation with cancer and one with polyps.” So if only one person in your family has had colon cancer, passed the age of 50, and no one had has polyps, chances are slim you have the gene. NOT saying you DON’T, just saying chances are slim. And remember I am NOT a genetics counselor nor am I a doctor!  If you are concerned that you may have this in your family, talk to your PCP and find a genetics team to discuss your family history with.  They will tell you if you need to be tested or not.  My family history is every generation has had colon cancer and colon polyps on my dad’s mom’s side.  This includes my dad having colon cancer when I was 26 years old, and only a few months old.  So I grew up with knowledge of both CF and Lynch Syndrome (didn’t know the name of LS just that colon cancer was hereditary).

Lynch affects my CF in a lot of ways.  I need to have yearly testing right now to be sure I am not growing any cancerous cells in my body.  Yearly I get colonoscopies, thyroid ultrasound, upper endoscopy and a skin checkup.  Having CF complicates my colonoscopies and upper endoscopies.  A prep for someone without CF only takes the day before the procedure.  If the procedure is on Thursday they stop eating Tuesday night, clean out Wednesday and are nice and clean for Thursday.  Not for me.  I need to stop eating Saturday night and eat clear liquids/foods on Sunday.  Then Monday, Tuesday and Wednesday I have to clean out.  And even with this I am not always “perfect” but enough to get good results. 

Lynch has also played a significant role in my evaluation for a double lung transplant.  The team at BWH was not too sure what to do with me.  They had many meetings and in the end decided I would be an OK candidate.  Under the strict terms that once I am transplanted, those yearly tests become six month tests.  There is a much higher risk for cancer post-transplant because of the immuno-suppressants so my risk will be even greater than that.  As it stands now with my yearly colonoscopies, there are always 3+ adenomas polyps removed (those are the ones that if left to fester can turn into cancer).  My whipple done last July was done only because the transplant team did not want to take the risk that I had precancerous cysts on my pancreas.  They removed me from the list until it was figured out, and it could only be figured out by doing the whipple.  So I had to have this huge surgery to be sure.  I was lucky and have had no real complications so I am fine with having had it done now and thankful they were only CF cysts.  I also had a full hysterectomy in 2011 because of Lynch.  Many years of reproductive issues coupled with my desire to not pass my genes on to children led me to have all my reproductive organs removed.