I am getting more and more excited. I read Gunnar Esiason’s blog last night and my mind is trying to take it in – or encompass or even hold the possibility.

He writes:

The first few weeks of the study were some of the most powerful of my life. My lung function seemingly skyrocketed overnight, I suddenly felt like I had more energy than an Olympic athlete in the prime of his career, and the nagging cough that we all know vanished.

“Did your PFT’s go up?

YES! My PFT’s basically exploded.”

Read his full blog, it’s pretty fucking cool. http://www.gunnaresiason.com/my-time-in-the-triple-combo-study/

Gunnar Esiason, if you don’t know but might have seen or heard of his name, is the son of the NFL quarterback Boomer Esiason. He was on the triple combo study, now known as Trikafta. I discovered Gunnar’s podcast through the CF channels of the world, and through it, I thus discovered Josh Llewellyn-Jones as well.

I am definitely going to email Children’s probably soon to work on getting the drug. I know they are being inundated with patients right now, and the patients that are the sickest are a priority.

It is all very exciting and I feel extremely fortunate to be living and able to think and breathe at this moment in time.

On that note – I feel good overall being on Symdeko, the new drug. My lung wheezing has seemed to resolve. I think it was allergies.

Do my lungs feel any different or can I tell any difference?

They feel good but is it from the drug or the fact of many increased treatments, I do not know. I will say I usually start to get a little bit cruddy about 3-4 weeks after I stop IVs. It has been 7 weeks and I still sound pretty clear.

The drug could be helping unknowingly inside, making its way through the channels while I type away on this computer. Who knows? I have been doing more treatments so that is difficult to know as well.

I am pretty positive that my weight gain of 4 pounds in a week and a half is from the medication. I have never gained weight so fast in my life. The previous 7 pounds in a month was a really good number for me, so this was shocking.

I want to give a low-down on the meds. See the ingredients below:

First-generation, Orkambi: Lumacaftor/Ivacaftor

Second-generation, Symdeko: Tezacaftor/Ivacaftor

Third-generation, Trikafta: Tezacaftor/Ivacaftor/Elexacaftor

See how each one builds on the other. I was on Orkambi for 4 years and now Symdeko. And how they build onto one another is why it is important to keep going on Symdeko.

I want to give a short explanation of the CFTR protein dysfunctionality, and why such a wide range of patient wellness. I don’t think I ever have.

In short, the protein (the CFTR protein) in my cells have 1,480 amino acids. Fortunately, my cells make the amino acids, some mutations do not. It is to form a 3-D shape that fits perfectly to transport chloride at the cell’s surface.

And let’s allow the CF Foundation to pick it up from here:

When a mutation causes an amino acid to be deleted or an incorrect amino acid to be added, the CFTR protein cannot form its correct 3-D shape and function properly. These mutations are considered to be protein processing mutations.

The most common CF mutation, F508del, is primarily considered to be a processing mutation. The F508del mutation removes a single amino acid from the CFTR protein. Without this building block, the CFTR protein cannot stay in the correct 3-D shape. The cell recognizes that the protein isn’t the right shape and disposes of it.

The drug combination lumacaftor/ivacaftor (Orkambi^®) works by enabling CFTR protein with an F508del mutation to fold in a more correct shape, and then activates the protein to allow more chloride to pass through. Although this drug combination is not a perfect fix, it helps the mutant CFTR protein to move some chloride. This movement of chloride reduces the symptoms of CF.

CF is a difficult condition because under the term “cystic fibrosis” there are many different types of mutations with this protein. For example, Class I mutations, the CFTR protein isn’t even created.

I am in Class II, the most common F508del. I am thankful that my protein is at least created. It is mutated and gets lost but at least it is created.

I have this thought or hope or just forceful thinking, that perhaps some of my proteins fold properly and make it to the cell surface. I don’t know if there is a proper exchange of salt and water, of course. I think maybe . . . because how could I still be here? There are the treatments but my cells don’t work right.

Just to say, the different classes make it understandable why some patients are so ill no matter what they do and seem to spend so much time in the hospital, while others spend less.

Here is this kind of cool link. My MN doc showed me this:

https://www.cff.org/What-is-CF/Genetics/Know-Your-CFTR-Mutations-Infographic.pdf

This is all super exciting stuff happening – and I think I am in shock and almost in disbelief while knowing this is all true. There is documentation to support.

On that note, this week I decided I needed more rest. The ebbs and flows as we all know. I dialed it back on my treadmill, yet I did yoga instead of the treadmill. Good, bad or the other – I do not know, but my body wasn’t giving it to me.

I am also craving berries and have for a while. I did one smoothie this week a bit on the fly. I need to get a few things to reintroduce smoothies back in again. I’m craving something.

I have also had to dial back my fat intake. My body is not craving that or needing it – so that is actually good. I am happy with my weight, and it is good to have a little extra padding.

I added back in my ginseng and milk thistle. I have been tired and ginseng gives me energy and milk thistle helps cleanse the liver. The cell correctors, in general, can tax the liver. I am not sure how this one is processing in my liver. I will get my liver enzymes checked in a couple months.

The ginseng and milk thistle both can thin the blood a little, they think and maybe it could throw off my INR. But it isn’t really “known,” and you know what – I think I am only on this for another 3 weeks. Maybe it’s good I start these while I am being monitored cause then perhaps I will actually know.

I also wanted to share something kind of interesting I read this week in school. Oh before that, I decided which professor is going to be my final project advisor. He is going to make me work, he will push me. It is like you need that person, in tennis per se’, a partner that is slightly better than you – because then you improve. Your partner can’t be at the same level or lower, because you will never grow.

He is also extremely intelligent and curious, his mind just goes, zips. We also have a Midwest connection, and come to find out he actually went to the University of Minnesota for his PhD. And I can’t believe he is younger than me by a couple years, but he is.

This little bit below is what I learned at school. It probably makes this blog too long, but I want to share it. Let’s call this is the long format blog.

Places in the country where we produce paper – the tool, object to create literacy, are actually places in this country where illiteracy is the highest – and even generational illiteracy.

“Papermaking is a dirty business” – there are chemicals involved that are not safe. Papermaking mills usually are established in areas of low population; less than 100,000 people and as low as 2,500 people while the main industry in that area is papermaking.

Did you ever read A Civil Action – a phenomenal book.  Remember Erin Brockovich – it is along these lines. Higher rates of cancer in and around paper mills by dumping toxins in the water.

The main goal for these plants is to produce – there have been a couple huge waves of paper demand but a huge influx between 1934-1974; we have population growth and consumption of reading material – the need to read. The US population doubled between the years 1920-1970, and the consumption of books and newspapers increased by a factor of seven.

Because of resources and optimal areas for the papermaking plants to be created are by rivers, the south (South Carolina, Tennessee, and some other parts) have been a perfect targeted area.

As early as 1909 – the first lawsuit was filed for environmental reasons against one of the largest papermaking plants, Champion mill in Tennessee. During those years, many residents worked at Champion while they could have cared less if they were literate or had an education. They just needed workers in the mills. Some only got 3-months of schooling a year. They pushed for productivity and wealth.

Things changed post-WWII – when they needed more skilled workers. They could attract skilled workers, but the families did not want to live in those areas. The schools performed terribly as there was little investment in the schools. There is extensive documentation of literacy rates in every country and for every year, while there is a concrete relation to low taxation, low per-pupil spending on education, and thus high adult illiteracy.

The mills eventually had to invest in the schools through taxation to get skilled workers to stay. The schools then began to perform at a higher level. With their better education, people demanded better environmental policies.

Eventually, the mills had to comply but got away with terrible regulations for years. One of the major rivers in Tennessee was brown and it had to be cleaned up. There are still environmental issues with mills documented as late as the 1990s.

In this case, to demonstrate – communities need industry. Communities actually need many industries. The industries create growth, well-being, education, and wealth. They also need industries that contribute to their communities and taxation.

Corporations aren’t really willing to give communities what they need – they aren’t really known for that. They want the workers to put in the hours but forget about well-being. The only way is to have that accountability.

Still today, areas around paper mills – the means to create literacy, have the highest rates of illiteracy.

Things that make you go hmm . . . I just thought it was interesting.

Much love.

Reference: “Reading Material” – Peter Mortensen. 2001.