Thank you.

Waiting for the long list of people to see me at the University of Minnesota CF clinic, Anne walks into the room, with a mission – as she always has.

“How are you?” Anne says.

“Good overall.”

“What the hell has happened?  You left 10 or so years ago with 100% lung function and now you are at 55%?”

She dives right in.

I have known Anne all my life.  Since “yay” high. I remember her when I was five, six, seven years old. She was one of the respiratory therapists present at CF camp when I as fourteen.

She is militant. Voice surrounds and eats up a room. A bullhorn that announces then captures all its prey, but with love and kindness.

I go in – explain the stress, allergies, the new and old baseline. How since 2011, I can’t seem to go a whole year without another hospitalization; hoping Orkambi and this clinic’s guidance will make a difference this year.

“How long are you on IV antibiotics when you are being treated?”

“Two-three weeks, depending how I am feeling,” I say.

“We do a minimum of 4-6 weeks, all the way up to 3-months if need be.”

“Anne, that is intense.”

“We are intense.”

She continues, “You don’t get a new baseline. Your old baseline is your new baseline.  Some patients will call us up and say, “I can’t do this anymore” – and that is their decision.  But, if we don’t get to your old baseline, we get you awfully close to it.

You shouldn’t be at 55%.  But, you are here now.  We have you.  You will live to 80.”

Note: MN CF patients historically over the last 3o years live 10 years longer than other patients nationwide. 

Anne has a look of anger, disbelief, and frustration as she looks not at me but across the room.

“We have patients come in for 3-4 days, get their levels and then there are out. Patients heal better, thrive better at home. They get better rest and just do overall much better at home.”

I am sitting, listening, eyes open –

“You can work you know.”

“Yeah, I know  . . . the schedule.”

“We work with you.  We get you over that 1-2 week hump and then we get you there. We work with your schedule.”

“So, patients come back every  . . . couple weeks to do a PFT to see where they are at?”

“Yes. If they have not returned to their old baseline, there baseline, then we keep treating up to 12 weeks.”

“I know there have been gaps . . .  I just didn’t know what they were.”

“You are here. We won’t let you go down, remain the same or go up.”

Philosophical and protocol difference/indifference:  When one’s lungs are injured, the lungs have this defined window of opportunity for healing – the 12 weeks. During this time, the inflammation goes down, the tissues heal and repair, and with hope, little scarring. The continuation of IV antibiotics are to eliminate any possibility of new bacteria inhibiting this healing process, because after 12 weeks the lungs have healed as much as they can – for the most part. Nothing is definitive especially with lung tissue. This is when one may lose lung capacity and may or may not ever get it back.

In MA, they treat for a minimum of 2 weeks and if you “feel” better you may stop. I believe many patients do 3 weeks. I am unsure what their protocol is for longer IV treatments, if any. I always feel better and am quite tired of IV antibiotics and their schedule after 2 weeks, so I stop.

The problem is, and because I was never sick as an adult in MN, I would not have known by any possible imagination the protocol of MN vs.MA, and to note – “feeling” isn’t a true marker of recovery or standard of care. Every day I don’t feel like doing my treatment but it all adds up during the course of a lifetime, so it doesn’t matter what I feel today or tomorrow, it must be done. It is the same philosophy but even more so with antibiotics. My doctor in MA practices the philosophy “wait and see” what your new baseline may be.  I may return to my old baseline, I may not. A toss of a coin. No real science.

I want to point out, I like my team in MA. They treat with a different philosophy, protocol, perhaps data, and experience. The result being is that it is not sufficient, and this is my life.

I say to Anne, “I had no idea. I thought this was standard protocol.”

“Why would you know?”

“I never was sick, because you, everyone kept me healthy. And that is why I am here.”

She repeats in the time that I am with her at least 4 times: “You are here now. We will not let you go down. You can live until your 80 -”

“Really?” I have heard 50, 60 but never 80.  It may not happen and that is Okay.  The point – I have never heard anyone say I can live to 80.

“With all the drugs, the medications in the pipeline – yes.”

She continues,”Who is your RT (respiratory therapist) in Boston?”

“I don’t know.”

“Who is your RT?”

“I. Don’t. Know.”

“Who. Is. Your. RT.?”

“If I have one, I don’t know who they are and I don’t know their name.”

“Where are you going?”She turns to the computer and starts clicking.

“Mass General Hospital.”

She brings them up – “How many patients?”

“I think 70-100 patients. They were rambling off some stats when folks started the new drug.”

“Well that is why. You need at least 150 patients for a RT. We have 600.”

I find out through this I have been contaminating my neb cups by rinsing them out between treatments.

“You are an educated woman, “Anne erectly sits starting into my eyes.

“I know – I thought rinsing between would be better than have old medicine sit in the cups and grow bugs.”

“No. The tap water is contaminating them. What you do is, do one neb in the morning – DON’T TOUCH IT, then do the next one at night. Take that neb, put it in a big pot to collect at the end of the week, and boil them or put them in the dishwasher. Nobody has time to do that daily, it is ridiculous.” Back in the day, Dr. Warwick had this insane process of washing neb cups daily, wash your air compressor tube daily, and hang them someplace in your house and mark them Sunday through Saturday? It was nuts. We never did it.

“I do that – regularly.  I have my pot. I didn’t think the tap water wasn’t perhaps the best, but better than the alternative.” I am wrong again. I don’t have a microscope at home – why I need everyone to fill in the gaps.

I order all new neb cups. I have some new ones, but I need all new ones ASAP. Anne contacts the MN service they use, going to check to see if they will fill for me in MA.

I found out that I cultured one environmental bacteria that usually comes and goes and luckily it has. If it did not, this is from last November to February, I would had needed to be treated with high-dose antibiotics. No one in MA told me.

I cultured another environmental bacteria in May that should be fleeting. I do not know yet. My doc will investigate. These may be normal for the east coast. I do not know and again how would I? No one has brought them to my attention.

What to say?

Without a doubt I need to consistently book my MN CF appointment every 6 months.

When I get sick again – issues will arise. Egos will bunt. I will follow Minnesota’s protocol, not MA’s.

I signed some more HIPPA forms for my doctor in MN can see my results more readily, and I set up and account to communicate with my doctor easier.

I am slightly angry – but not with burning anger, more like yesterday’s ashes.

How will this work long-term?  Will see.

Take away –

I have known losing almost half my lung capacity in 5 years is not right. I have not shared  this with many. Worrying does no good, but only creates more anxiety. I can’t have that. I have had this underlying anxiety for quite some time, but I find there is no point in raising that level. During these IV episodes I can only do as much as I can do, and that is to get better. Then, pursue, tackle bigger objectives when healthier. I am thankful that I am healthier to tackle these bigger issues and that I am not in survival mode.

Although Anne is startling, her reassurance of me being here now and the fact she believes whole-heartedly with all the science and know-how I will live until 80 – is a pretty nice fucking feeling.

I left with this soothing feeling of thankfulness and gratitude. It washes over me like warm, healing, and almost magical water.

Thank you.

Thank you.

Thank you.