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: http://mycfjourney.blogspot.com/2009/07/my-colon-cancer-screening-visit.html 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.  

Tuesday, May 26, 2015

CF Awareness Post Question Day 26

I took a break from CF awareness this weekend to celebrate Memorial Day and remember why we celebrate.  God bless the fallen service men and their families.

I know you like the winter months the most. Having CF is there any seasons that are better for you. Or months.

I love the snow of the winter months but my lungs do not like the cold!  I was hospitalized this past January primarily because of the awful cold weather we had making my lungs constrict so much I was requiring 3L of oxygen while exercising.  Normally I would need none for what I was doing. 

The summer isn’t too bad for my allergies but the excessive heat makes me lose more salt than I normally do and I dehydrate faster.  And the humidity makes it difficult for me to breathe. 

In spring the allergies get to me a lot. 


Fall is probably the least awful for me.  Luckily it is my favorite season.  Allergies are at bay, for me, and the really cold weather hasn’t hit full force yet.  

Friday, May 22, 2015

CF Awareness Post Question Day 21 and 22

Day 21 I asked for more questions and I got them!!!

Day 22:

What was it like for you to travel outside the US having CF??

In all honesty it was not bad at all.  I had my doctor write me some notes for all my medications and in case I needed to be seen at a local hospital.  I had researched hospitals in the cities we were visiting to see if they had CF centers and wrote them all down.  Just in case of course.  I had to pack extra medications in case we were stranded there longer.  I had to be sure I had conversion plugs since the sockets over there are different, though you have to check with the specific hotels since I think some voltage might be different.  It’s been a while lol.  I took fewer medications then too.  I didn’t do my vest so I didn’t have to travel with that which made it easier for me. I had all my medications in one bag in a carry on so that nothing would be lost.  Plus a medication bag does not count as luggage! 

As far as the trip itself, I was much healthier then and was able to do a lot of walking which we did.  Now I look back and the thought of doing all that walking makes me want to sleep for days!  We also scheduled some down time.  In Florence we were there for 6 days and we have activities planned for 4.5 of them.  So one day we had nothing planned at all and we lounged at the hotel and relaxed.  It was good for both my mom and I to rest.  We did 5 cities in three weeks which was perfect.  Two of those days were for travelling to and from Europe.  We took the train in Italy and planes from Paris to Rome and Florence (I think we took it from Florence, we may have traveled to get to the airport I cannot remember) to Prague. 


All in all, as long as you know your limitations and you can fly then it is worth the trip.  Of course this was to Europe.  I don’t know how it would be traveling to a third world country the Americas (Latin and South).  

Wednesday, May 20, 2015

CF Awareness Post Question Day 20

You have had experiences of a volume and magnitude with the health care system that few have. I'm interested in your insights.

As a nurse and a patient, I've experienced both sides of the coin and I'm aware of bad apples, general malaise and the "systemic" characteristics of a system with funding issues.

What I'm most curious about are your experiences. Are there things which healthcare workers unwittingly do or say which aren't helpful? Do you dread your interactions with the medical system? What are you overall impressions?

Being a patient that is in the hospital for extended periods of time on a lot of occasions you learn what you like and dislike.  A lot of the things I don’t like I suck up and deal because it is hospital protocol.  For instance, I use the VEST while inpatient and do not need to see a respiratory therapist for manual chest physiotherapy, though some CF patients do get this while in.  However, they still have to check on me twice a day to be sure I don’t need anything from them.  So each time they come in they listen to my lungs and ask me the same questions.   It’s very monotonous lol.  And annoying!  Especially when some of them come at 8am and you are still in bed.  But they have to do it and it’s what I get for being there for two weeks. 
As far as nursing goes, there are certain nurses that I dread getting and it’s like roulette when I am in.  Will I get her or not?  Some of the day nurses come in at 7:30 and act surprised I am still in bed.  They say things like “oh you’re still sleeping?!?!”  or “I figured you would be up by now!”  Hello it’s only 7:30 am and I have nowhere to go so why would I get up???  Others are very strict about meds etc.  Most nurses give you the day’s supply of enzymes knowing that we are able and willing to give these to ourselves when we eat.  But there are other nurses that insist you page them each time you order food or eat.  It’s annoying for us and I am sure it is annoying for them, though they do ask for it.  Sometimes nurses forget to hook me up to my IVs at the times I am supposed to.  I don’t get mad I just call.  They get busy and forget.  It happens.  I usually only have one or two “bad” nurses each stay, and considering how many I see that is good odds.  Some of the nurses I LOVE and it’s like a holiday when I get them!  A great nurse can really make your stay sooooooo much more “enjoyable.”  And on the flip side, a really shitty one can make you want to go home immediately. 

I don’t dread seeing my doctors at all.  I know that they are they to make me heathier.  The hospital itself I am not a fan of but I am hoping to remedy part of that by switching centers.  Then I can be seen at a different hospital.  My current hospital, I prefer to do a full two weeks in and they prefer me to do it at home (by they I mean the people in charge of the hospital….they tried to move the CF patients to an entirely different hospital because we cost too much money but instead they came to a compromise with the CF clinic where anyone under 35 does inpatient at Children’s and anyone over 35 or over 18 and seen by the transplant team stays in patient at Brigham and women’s).  But financially I cannot do my stays at home and it is a fight each time I go in for me to stay.  That results in my feeling like an unwanted house guest.  So this new hospital is perfectly fine with you being there the two weeks and won’t make me feel like I am unwanted (so I have been told!!!). 


I’ve learned to juggle my life around the healthcare system.  I know that if I have an appointment at 11am I should not schedule anything else before 2pm or later depending on the doctor.  I know that if I am being admitted I will most likely sit in the waiting room for at least an hour and I should bring stuff to occupy my time.  I know that most doctors run late and it isn’t always their fault (sometimes patients ask A LOT of questions and I would rather them answer the questions than make someone feel rushed.  I might be that person one day and would hate it!).  I know there are a lot of faults with the system that need to be changed but they might never get done.  I can’t avoid it so I need to learn to deal with it.

Tuesday, May 19, 2015

CF Awareness Post Question Day 19

Here's a question: are there any dietary restrictions?

There are no real restrictions, but some individuals can tolerate certain foods better than others, like the healthy population.  People with CF need a higher calorie and higher fat intake than the average person.  We burn more calories so we need to take in more calories.  This is for those who are pancreatic insufficient, meaning the pancreas does not break down food like it is supposed to.  Some people notice that dairy products make them produce more mucus so they avoid milk and ice cream. 

Growing up I was told to eat eat eat, anything and everything.  My mom would put butter on everything.  We would search the food store looking for high fat foods only ever finding low and no fat.  You would be surprised how hard it is to find good foods that are high in fat and calories (by good I mean healthy and not chips, snacks and junk foods).  To this day it is hard to control my eating because of how I grew up and needed to grow up.  I never had to have self-control with food, because there was no need for it.  So if I felt like eating a container of ice cream I was allowed (I don’t think I ever did lol).  This is an area in the CF world that is slowly changing.  Stressing not only high calorie and high fat foods but also healthy foods.  Especially if growing old is an option now.  If you think you won’t live to see 30 you aren’t so concerned with heart disease etc.  But if you are going to live to be 80 you need to worry about those things! 

Gaining weight is an issue.  We struggle every single day to maintain our weight let alone put weight on.  I have been fortunate enough as I aged (thanks menopause!) to not have to worry so much about my weight.  But up until I was 30 I struggled for every ounce I gained.  By the time I lived on my own, I had a calorie regiment that would blow your mind!  I repeatedly heard the “you must have a hollow leg” comments or “where do you put all that food?”  Breakfast was light as I have never been much of a breakfast person.  But all day long I would snack and eat like it was my job, even while working, because it WAS my job.  I would eat snacks almost every hour if not more.  I brought two bags to work, one for food and one for work stuff.  Yes an actual bag dedicated solely to food and a drawer filled with snacks and cheese dip (I loved cheese dip on pretzels!).  I spent small fortunes on food and supplements.  I tried all types of high calorie milkshakes to eat throughout the day, coupled with snacks and dips and meals.  I would come home and eat more and then eat dinner and then have a snack before bed.  And even with all of this, I was lucky to be 107 pounds.  When I graduated high school I weighed 104 pounds.  By the time I was 25 I had only gained 3 pounds in 8 eight years.  For reference, since weight has stopped being a big concern for me, right before my pancreatic surgery last July I was 132 pounds.  I was a normal weight for someone my size.  Now I am 114-117 pounds depending on the day, time, etc. 

Many times I would stop eating because it was not fun for me.  It was a chore; it was a treatment for me. It was just like all the medications I was taking, except that I had to do it continuously. (this has changed now that I stopped worrying about my weight, I love food again!)  Sometimes I would dip below 100 pounds and I would stop getting my period.  I took many pregnancy tests thinking I must be pregnant, only to realize I was fighting something off and my weight had plummeted.  I was lucky again in the fact that my weight never got dangerously low where I needed to get overnight tube feedings via a g-tube.  But I know many people, as adults and children, who have gotten tube feedings in order to just maintain their weight.  It is a real struggle. 


And it is a struggle that many healthy people think is great.  Hearing “oh you are so skinny you must love it,” or “but at least you are thin,” are NOT helpful.  Twigs as legs and arms, they are not pleasant.  A barreled chest (common in CFers thanks to lung disease – http://www.mayoclinic.org/diseases-conditions/copd/expert-answers/barrel-chest/faq-20058419 ) makes us feel abnormal and weird.  Especially when you have skinny arms and legs, you feel like E.T.!!!  This country, and many others, thinks that weight issues are only a problem on the other side of the spectrum…those who are obese and overweight.  But super thin is a weight problem too.  

Monday, May 18, 2015

CF Awareness Post Question Day 18

I know the Cystic Fibrosis foundation as well as other organizations have funded research and development programs specially aimed at identifying innovative new therapies for CF. As a patient do you believe these programs have added value? Have you been approached to enter a clinical trial for a new therapy?

I definitely believe they do!  They have a drug out right now that “fixes” the genetic issue of CF.  It reopens the channels across the cell membranes leading to almost normal function.  This drug is only for specific mutations but they are working on more for others.  This drug can and has helped people live much more normal lives – healthier, able to keep on weight, less to no lung infections.  For some, this drug could be the “cure” that people want.  But that depends on your definition of cure!!!

I have participated in many trials.  Not so much lately because they usually have a 40% FEV1 cutoff and I miss that mark.  But in the past I have been involved in a bunch.  And actually right now I am in one just where they collect your mucus to examine it once a year, only requirement is culturing Pseudomonas.  Even these little studies help the larger picture.  I feel that it is my duty as a person with CF to participate in trials that I feel comfortable participating in.  There is a small pool of people that can participate so we need all the volunteers we can get to do so.  Foundations like the CFF can do research and studies galore in the lab, but if there is no one to test these on then they become moot.  I don’t do these trials so much for me, I don’t know how much of them will actual help me in the long run (I did an inhaled Cipro study 7-8 years ago and had great results but since then my lungs have decided to not like inhaled antibiotics so that med will never help me out), but more for future generations. 


Even though this is not a CF specific study, I am currently enrolled in a colon cancer research study.  Its 6 months of taking a mediation daily to see if it helps ward off colon cancer.  Anytime I get asked to do a study I participate (unless it’s a procedure that might put my lungs at risk).  The current one I am on I am able to be taken off of if my lung health declines and I need to stop.  I am not allowed to take certain types of antibiotics, and of course those are the ones that I usually try in oral form before doing a different kind of IV antibiotic.  I also like doing studies because I am such a hodgepodge of diseases that they can see the results on someone like me.  I don’t mind being a guinea pig at all!!!

Saturday, May 16, 2015

CF Awareness Post Question Day 16

Do people with CF always get diabetes?

Not always.  Because our pancreas is a mess, many CFers develop CF related diabetes (CFRD) which is different than Type 1 or Type 2 diabetes.  I don’t know the ins and outs of any of the diabetes so I can’t really explain them.  Similarly, many, if not all, post-transplant patients develop diabetes because of the need to take prednisone constantly to avoid rejecting the new lungs. 


In my case, a couple of years ago I was given an OGTT (oral glucose tolerance test) which came back as positive for CFRD.  So I received my fun little test kit to test my sugars.  Since then I have remained in the normal range when doing finger sticks for the most part.  I only ever require insulin shots when I am on a prednisone burst/taper.  I actually tend to have more lows than highs and insulin could make this worse.  Ironically, and I have no actual data to back this up just me, the prevalence of lows has decreased significantly since I had that large cyst removed from my pancreas in July.  My A1C, which is the marker for your sugar levels over the past couple of months, is only slightly higher than normal at 6.3, normal being 4.5-6.  But my A1C has gone up to 6.8.  It fluctuates.  At some point I will need to use insulin, whether it is before or after I am transplanted, I won’t know. 

Friday, May 15, 2015

CF Awareness Post Question Day 15

What's the one biggest misconception you get when people hear you have CF?

A lot of people in the non-medical world have never heard of CF before so they have no real misconceptions.  Most of the time they are a clean slate.  Which is of course good and bad! 

Occasionally someone will have a friend whose friend’s child has it so they have heard of it.  Then the misconception is that I am an adult and alive.  Sounds crazy, but CF is portrayed as a childhood disease so people assume it’s a childhood disease and there are no adults with it.  But in 2013 49.7% of people with CF were over the age of 18 (the CF annual report comes out the following year, so we don’t have 2014’s yet).  CF is NOT a childhood disease anymore.  The stats are almost 50/50 thanks to advancements in treatments.  Even in the medical field I have run into nurses who are surprised I am alive and still have my original lungs!  I always give the benefit of the doubt, especially if they are in a field where they may not see CF at all or all that often, and take the chance to educate them on how adults are alive and well living with CF.  But this is something that adults are working with the CFF on.  We want to be portrayed too.  But kids bring in donations and donations are needed to make those kids in to adults. 


Another is that I must be really healthy if I am still alive.  Not true.  The body can endure a lot, CF is a good example of this.  Just because I am 34 and have my original lungs does not mean I am healthy.  Remember, 35% lung function is not good.  It’s not horrible, but it’s not good lol.  For where I am in my disease progression I am healthy, but compared to the average Joe, I am not :)