Before I say a word – I was trying to find a quote I remembered I learned from school.  I did not find that quote, but happen to read a blip from one of my papers – it is all-so-fitting:

“I like the idea that if someone is writing about one’s life, can one really write accurately about their life while they are living it?  How close is living, reading, and writing? Are they, or are they separate identities? Writing gives meaning to life, although writing one’s life is not an accurate assumption.

When one writes, one walks closely between and among autobiography, memoir, and fact.  Can there be facts in a memoir or an autobiography? Isn’t it based on perception and not fact?

Then we fall into Sterne’s, Tristram Shandy, challenging the reader throughout this entire funny and silly text that very question. 

Focus, I will.

I have been delaying, or trying to find the words to express my experience at the MN CF clinic accurately.

Disclosure – I made a short trip home to the CF clinic.  I did not, could not tell everyone because I did not have the time to visit and see everyone I would have liked.  Next time – I promise.   I am planning on returning more frequently after this visit.

Note – there is a lot of info here – I learned a lot; walk away with tons, and am so thankful I went. 

Returning to the clinic, it feels like the missing puzzle pieces have been found.  I have been doing this a long time and one would think I have this down, but the truth is – it is all too much. Actually it is fucking-all-too-much for any one person to do.  The ever-pressing constant.

Thus why there is a team of folks to fill in the gaps, answer the questions I do not know to ask, because technology changes lightning speed then at the same time, some things never change.  The tug and the pull and finding the equilibrium.  How to make sense, put together, tear apart, and make sense of all the pieces.

My new doctor at the CF clinic, Dr. Billings – so kind, so thorough, so receptive, engaging.  She spent around two hours with me, just going through data, history and present.

Thankfully, MGH sent my records.  I brought copies of a fair amount of my records, but not all. The nurse working with Dr. Billings made a Table of Contents of my records with page numbers, categories:  itemized, outlined, highlighted, and flagged. Then all my records were uploaded into their system:  medications, allergies, pharmacy, problem list, etc.

Let me jog my memory – I did take notes.

Oh – let me say, their intake sheets were outstanding.  Three sheets of intake info.  They didn’t leave one thing out.  They brought up things I don’t particularly think about.

At this point I was starting my third week of Orkambi – Dr. Billings takes notes furiously.  She points out that the biggest concern is liver damage.  I have made it over the allergy, side-affect hump overall, but per the studies some people develop liver damage long-term. Sometimes the liver damage is reversible, sometimes not.  The liver in some cases lacerate.  The signs and symptoms – acute abdomen pain and yellowing of eyes mostly.

In the past when my liver enzymes have been elevated, just one or two times, I have taken milk thistle which is an herb that is an anti-inflammatory and anti-oxidant.  When I take it consistently, my liver enzymes seem to remain normal. Chronically, CF patients develop liver problems from all the medications and possible blockages in the ducts connecting the liver and pancreas. Statically, five percent of patients pass from liver failure.

It is all a gamble.  Life is a gamble.  Throw the cards in the air, maybe confetti – who knows.

I am going to break for a moment –

Yesterday, I literally sprinted up the stairs at work and my lungs had a normal response time and reaction. I then saw the shuttle at the door, I sprinted again – coughed once on the shuttle.

It feels like time is reversing.

This is what I used to be able to do not all that long ago.  I do not have words for this –

Onward – I mentioned to Dr. Billings that in the last couple years I have had difficulty while doing my PFTs, specifically my trachea closing, flat-lining the results.  No air passing through.  When this happened, the respiratory therapist said, “This usually happens in children.” Meaning – I should know what the hell I am doing by this point.  I do – but I believe I was trying too hard.  I have had to retrain myself to open up my trachea while blowing out.

Dr. Billings said, “Actually that is often times due to vocal cord damage.  It happens frequently in patients with CF.  Overuse after so many years.  You think it is your trachea, lungs – but it is your vocal cords.  Does it happen when you aren’t doing your PFTs?”

I said, “No. I don’t think so.”

She continued, “Let me know if it does because we can help you with that. She should have never said that to you.”

Thank you.  That stupid bitch.  Yeah, I said it.  I wanted to smack her.  You try sitting in this chair for all these flipping years and give it all you got.

And now we move upward –

Dr. Billings mentioned that I believe her name was Nancy, the nurse, organized my notes, and that they couldn’t find the result of this environmental allergy that people can become resistant to overtime. They tested me for it in June but I still do not have the results.

Dr. Billings looked at me like this is unacceptable.  “We test it right here in the hospital and it is back in 2 days.  It looks as if it was sent to Northwestern lab and it is probably still sitting there. You need to get the results because if your body is building an allergy to it it is a completely different standard of care how we treat it.”

She mentioned that I am on albuterol in my neb.  I said, “Actually, no I am not.  She said, “It is listed right here.”  Then before I said it she said “This is probably from your hospital med list.”  I said, “I do not take it.  I haven’t been on it in years, and I am pretty sure they didn’t give it to me in the hospital either” (I put my own neb together).

Side note – while compliling my records for this trip, I read I had pneumonia this last hospitalization.  Never knew. Patients always the last to know. 

Continuing, “You need to be on albuterol. It helps open up your little airways for the medicine can get in there.  Can I recommend it to you?”

I said, “Can you just put a script in?  You are a doctor and it shouldn’t matter that you are here and my insurance is in Boston.”

The nurse uploaded my mail order pharmacy as well, and Dr. Billings wrote a script and I received it yesterday.

We talked about Tobi and to continue it for a year, “Even though it may seem you don’t need it. It is a prophaligic drug.  We don’t want to change factors while Orkambi is taking affect and we can reassess in a year – sound good?” Perfect.

I admitted that I often times do not do my whole nebs while on Tobi because I do not have two hours every day to do them.  She said, “Many CFers end up shortening or cutting out there nebs a lot of the time while on Tobi.”  It is not optimal, but she understood.

I suggested about the air compressor that delivers Tobi much quicker and more concentrated.  She asked when was the last time I got a new air compressor.  If it has been in the last 3 years because insurance should cover it.  She is going to have Ann, the long-time, well-known, respiratory therapist, call me.  

I asked which settings were best for my vest.  This has changed over time with new updates to my vest, so I am not sure.  I have looked in my manual, but it doesn’t say directly.  In Boston, I have asked and they say which ever setting I can handle, feel comfortable with while still breathing comfortably. Which is fine, but wishy-washy.  In MN, you start out at 100% pressure (everything you start at 100%, not work up to it), and if you cannot handle it the optimal settings 80-100%.   Ann would go over all that and more, as when to replace my neb cups as well.  Ann is a force.  You do what she says.  A mother with a mother’s voice.

Oh – and the amount of enzymes that I take keeps coming up.  It is technically too many for my body weight.  The problem is I have tried to cut back, but then my gut starts to hurt, I mean really hurt.  I have had an obstruction in the past and the pain always resides in that area and I am so afraid of getting another obstruction.  I have been close 3-4 times over the years.

My homework is to count them daily for a month and find my average.  If the dose is still too high, she brought up switching the brand of enzymes altogether. The situation is the getting the correct ph. in my stomach.

All my meds, including my enzymes, create a lot of acid.  I take acid pills to help equalize the acid.  If my ph. level is too high, my enzymes cannot do their job and that is probably why I am taking more at times to actually digest the food.  Too much acid, take more enzymes.  The enzymes create acid – take more enzymes. Stick a needle in my eye.

Apparently, there is an enzyme that has a bicarbonate in it, so when it opens up, the bicarbonate helps neutralize the acidity.  I did not know this.  This has never been brought up to me.

Overtime – as with everything, scarring, damage takes place. Taking too many enzymes can create fibrosis in my large colon.  What that means – probably means it won’t work well.  So, the enzymes are kind of a big deal.

Back in the day, we counted all the enzymes.  Many, many small Tupperware bowls of enzymes.  It is a lot work and completely annoying.  I kind of have an idea how I will do this, hoping it won’t be too annoying.

There were a couple other points I came away with, but those are the big hitters.

Following my instincts – I followed them when I left MN and now with a couple friends mentioning it in my ear, it was a great idea to return.

I was asked a few times if I would ever consider moving back to Minnesota.  The weather is just so harsh.  When I left Minnesota, I kept on developing one bad lung infection after the other during the last 1-2 years I was there.  I would get better then hammered again.  I could not shake it.  The condensed pollution in some areas combined with the extreme cold, then heat and humidity were the isolating factors.  The weather wasn’t changing fast enough – so I had to change.

Minnesota in so many ways is such a fabulous state – driving there is a dream.  The signage, the space, the lights, the smoothness of the roads, the shoulder that is actually used as a shoulder; the non-aggressiveness. One of my favorite things this trip.

The people are so nice.  I almost forget how nice people can be.  Right away you know you are in Minnesota when the shuttle driver talks with you, is kind, shakes your hand, and remembers your name as I ran into him when I returned my car.  Then, the hand waves, the working together, the just overall non-aggressiveness, less resistance.  Taking it down a notch and calming the fuck down – so nice.  Sometimes I think I am too Midwestern to live in Massachusetts, but I love the ocean.  Visiting Minnesota more often may be a good solution.

I left making an annual appointment, but honestly would like to return in 6 months.  That is my goal.

So – one leaves it up to the all-loving Universe.

It washes, and recedes,
Warms to rise, cools to release,
Blooms, then awaits for the next sunrise,
Breathing-in, and giving breath,
Is held, and will always hold.

Work Cited:

Sterne, Lawrence. The Life and Opinions of Tristram Shandy, Gentleman. New York. Penguin Classics. First published 1759-67, 1967, 1978, 1997, 2003.