Free Fallin’

35 seconds. That’s how long you are in free fall after jumping out of a plane, or at least that’s what they told me. It feels like a lot longer, a mix of adrenaline, fear, and excitement, overloading your senses and distorting all sense of time. And when the parachute opens and you can really take in where you are and how beautiful the scenery is, you are humbled by how monstrous the world is compared to our tiny being. On the way, and afterwards, I had this song in my head.

It seems like an obvious leap that I would choose this song around this experience. One of the quintessential Tom Petty songs, Free Fallin’ is a song that everyone knows and enjoys. The lyrics describe a girl from the suburbs and them falling in love, and riding the roller coaster that is love. There are highs and lows, and all of it is new and exciting. I think we can all relate to that. Tom Petty was always one of my favorites. His gift was taking a simple 3 chord progression, then writing a fantastic song that is catchy, a song one can sing at will and a story that related to them. He had humble beginnings, and always seemed to be down to earth. He was able to relay that into his lyrics, which seemed universal to all of our experiences in life. I was really sad when he died; I think his heart finally gave out from the lifestyle of booze and drugs. He had his problems, but always seemed to bring a great energy in concert. His shows were great, one big sing-a-long where the crowd’s energy would be palpable, rising to a crescendo, giving you chills. Free Fallin’, Breakdown, American Girl, and others. He will be missed.

Speaking of rising crescendos, lets get back to my Free Fallin’. I’ll take you back 10 minutes before I jumped out of a perfectly good airplane. We were standing in the parking lot, and I had my harness on, already anxious and filled with anticipation. Then I see the plane! It looks like it can barely hold 4 people, and was so light I was amazed. My nervous excitement was further building, even more than it had been the entire morning. We take off on a gravel runway, and slowly build up to 10,000 feet where we jumped. I had no concept of how high we were, it was all very overwhelming. I could feel my excitement and fear building to a boiling point. But there was a positive; I felt very secure in my harness, and was attached to my tandem jumper with 3 super solid clips that locked in. Everything was reminiscent of climbing gear, and so that made me feel more comfortable.

But then, just as I’m starting to feel comfortable, he shouts “1 minute” and they opened the door to the plane!!! Holy shit was my first reaction……a blast of cold air and then I could really see the ground below, A LONG WAY DOWN!!!! I tried to control my breathing, but it was nearly impossible. So I put my feet on the ledge as instructed and looked down. Everything was so small, and I had a moment of hesitation. The tandem jumper, doing this all day every day, wisely gave me a nudge forward rather than let me stew upon my current position. And we were off……Free Fallin’! The blast of wind was exhilarating, and we settled into nice body position quickly, and I was able to really take it in and enjoy. I screamed in delight the entire free fall. When he finally pulled the chord for the parachute, I was surprised that it was not more jarring; it didn’t even hurt! I had imagined it jerking me and causing issues with my port, but I was able to position the shoulder straps laterally so they were not directly over the hub. The slow descent afterwards was relaxing and breaktaking, taking in the landscape and the mountains in the distance. I was able to “drive” the parachute, taking little turns and helping us down to the landing spot. Nice and smooth. For the next hour or so, I felt the adrenaline pumping through me like I was hooked up to a generator, my hands and feet slightly tingly. It was an experience to remember, and an item checked off my bucket list!

Happy 4th of July everyone! Happy to be alive and healthy, and living life 🙂

A Day in the Life

This song, the last track on Sgt. Peppers Lonely Heart Club Band, by The Beatles, is a classic. The whole album from front to back is an all time favorite, as it seamlessly blends from song to son as if it is one. The climax is A Day in the Life, written primarily by John Lennon. He was inspired by the newspaper articles at the time, in 1967. Also, it seemed, this song was inspired by their experiences with LSD. They were experimenting heavily then, and had various people pulling them in all kinds of directions. They met the Dalai Lama in 1966, which then inspired many different sounds, like incorporating the sitar and other Eastern instruments. When they were recording Sgt. Pepper’s, they wanted to be more Avant-Garde and pushing the boundaries.

That became very clear at the end of A Day in the Life, as they hired a 40 piece orchestra to build this crescendo to the final note. The movement builds, highlighted by the violins, slowly increasing their pitch and volume until you think they can’t go any higher without breaking all of their strings. Then, it finally releases into an E-major chord played simultaneously on 3 pianos, stretching out endlessly in a slow fade (actually it’s about 40 seconds!) At the end of it all, it leaves you breathless, wondering how in the hell they came up with all of these innovative sounds and recording techniques that had never been tried. This is one example of The Beatles’ genius, and their everlasting legacy.

I picked this song because of the title. I wanted to share a recent speech I gave at the National Jewish Research Conference. They asked me to give my perspective on the CD experience and how it pertains to research. Thus, A Day in the Life was perfect! I wasn’t able to upload the video, but there’s a link to audio here. Call it a mini podcast!

new-recording-16.m4a

I will start writing more frequently soon, I promise. Right now I am enjoying the summer with my family. Peace and love to all.

Chad

Here Comes the Sun

So, I’m sitting here in Mexico, poolside. It’s almost 9am, and probably already 85 degrees or so. We are going to start drinking soon, because why not? We are on vacation with some close friends, all sharing a house for a few days. All this morning, this song seems to be on a loop in my head, no matter what song is playing through the speaker. Here Comes the Sun is one of my all time favorite songs, one of George Harrison’s gems and part of the opus album that is Abbey Road. It’s such a positive song, with his acoustic melody setting the mood. He said he wrote it in Eric Clapton’s backyard on a nice spring day. They were the best of friends at the time; oddly enough Eric was madly in love with George’s wife Patty Boyd. His emotional pain was the inspiration for the album Layla and accompanying love songs. I digress, but back to the point! Here Comes the Sun just makes me happy, as it should you. George’s wonderful acoustic guitar and arrangement compliment the lyrics. The words speak of a glimmer of hope after despair:

Little darling, it’s been a long cold lonely winter
Little darling, it feels like years since it’s been here
Here comes the sun
Here comes the sun, and I say
It’s all right

As I hear this song of positivity and hope I think about my current state of mood and health. I have been in a clinical trial for some time now, relating to the CFTR modulators (see further explanation below). Look more closely at the next generation modulators since it relates to my current trial. I can’t discuss all the particulars, like the company or drug name, because the data has not been fully collected and submitted to the FDA. However, since I finished the last phase, I recently entered what’s called “open label.” That means that regardless of my double blind group for the trial, I now receive the drug until it is available on the open market. Boy, have I felt the difference right away! I started coughing relentlessly for about 5 days, lots of nastiness coming up that probably had been in there for quite a long time. Once that was over, I feel so clear! It brought to mind a few other songs: I Can See Clearly Now by Johnny Nash, Breathe by Pink Floyd, Sun is Shining by Bob Marley, among others. Suffice to say, it is a drastic improvement. My PFTs have increased quite a bit as well; the FEV1 (forced exploratory volume in 1st second) has gone from 40 to 50% in just a couple of weeks! And my overall volume capacity has improved by 0.5L, which is quite significant. I can exercise much easier, and have a tolerance for more cardio. I want to springboard off this increase, and get back to the mountains for some hiking and climbing. It has been more difficult the past couple of years, which is frustrating since we live in the foothills!

As a result of this increase, I have been able to enjoy this vacation, without CF dominating every aspect of it. That has been the trend lately, we have big plans for a good vacation, and at some point CF rears its ugly head and takes over. Whether I get sick, or have a bad coughing episode, or I am unable to participate fully in the activities we have planned, and on, and on. There have been more times like this than I care to count. But not this time, not this vacation. And that gives me more hope for the future, something I haven’t had in a long time. So a quick thanks to all the people at the National Jewish research clinic and the CF clinic who have made this possible. For continuing to push me, to make me my best so as to qualify for these trials. Because these new meds have let me see a new day ahead. One where I don’t need as many hospitalizations and have less frequent exacerbations. As the song goes, “Here comes the sun, and I say, it’s all right!”

Information about CFTR Modulators (provided by the CF Foundation)

Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that affect the production of the CFTR protein. When the CFTR protein is not made correctly, it affects the balance of salt and fluids inside and outside of the cell. This imbalance leads to thick, sticky mucus in the lungs, pancreas, and other organs.

The Cystic Fibrosis Foundation supports the development of drugs that target specific defects in the CFTR protein. As a group, these drugs are called modulators because they are intended to modulate the function of the CFTR protein so that it can serve its primary function: to create a channel for chloride (a component of salt) to flow across the cell surface.

When proper chloride flow is reestablished, mucus becomes rehydrated inside the lungs and other organs. Although modulators can’t yet completely restore proper chloride flow, they can improve the flow enough to relieve symptoms for people with CF.

There are three main types of CFTR modulators:

  1. Potentiators
  2. Correctors
  3. Amplifiers

Potentiators

The first type of CFTR modulator is called a “potentiator.” Potentiators help chloride flow through the CFTR protein channel at the cell surface. The CFTR protein is shaped like a tunnel that can be closed by a gate. Potentiators hold the gate open so chloride can flow through.

The drug ivacaftor (Kalydeco®) is a potentiator. This drug can help patients with gating and conduction mutations in CFTR. It also works on residual function and splice mutations where an insufficient amount of normal protein is present. In all of these mutations, some CFTR protein reaches the surface of the cell. However, either not enough protein reaches the cell surface, or the protein does not allow enough chloride to flow through. By holding the gate on the CFTR protein open, potentiators allow more chloride to flow through and reduce the symptoms of CF.

Correctors

The next type of CFTR modulator is called a “corrector.” Correctors help the CFTR protein to form the right 3-D shape so that it is able to move — or traffic — to the cell surface.

Almost half of people with CF have two copies of the F508del mutation, which prevents the CFTR protein from forming the right shape. The corrector drugs lumacaftor and tezacaftor help the CFTR protein to form the right shape, traffic to the cell surface, and stay there longer. But, even with lumacaftor and tezacaftor, only about a third of the CFTR protein reaches the cell surface, so by itself it can’t reduce the symptoms of CF.

Additionally, the proteins that do reach the cell surface do not open sufficiently to allow chloride to pass out of the cell. But, if a corrector is used in combination with a potentiator — such as ivacaftor — to hold the gate on the CFTR protein open, enough chloride can then flow to reduce the symptoms of CF. The combinations of lumacaftor/ivacaftor (Orkambi®) and tezacaftor/ivacaftor (Symdeko®) are therefore used to treat people with two copies of the F508del mutation. (Tezacaftor/ivacaftor also can be used to treat people with a single copy of one of 26 specified mutations –regardless of their other mutation.)

Amplifiers

The last type of CFTR modulator is called an “amplifier.” Amplifiers increase the amount of CFTR protein that the cell makes. Many CFTR mutations produce insufficient CFTR protein. If the cell made more CFTR protein, potentiators and correctors would be able to allow even more chloride to flow across the cell membrane. Amplifiers, which are being developed and tested, are not yet available.

Next-Generation Modulators

Ivacaftor and lumacaftor are sometimes called “first-generation modulators” because they were the first modulators approved to treat people with CF. Tezacaftor, approved in Feruary 2018, is also considered a first-generation modulator.

“Next-generation” modulators are new and potentially more effective CFTR modulators. The next-generation CFTR correctors currently in clinical trials target different problems caused by the F508del mutation to further improve CFTR folding and increase the amount of CFTR trafficked to the cell surface. The goal of next-generation therapies, which will likely be part of a triple combination therapy, is to develop treatments that benefit more people, including individuals with a single F508del mutation.

Individually, each of the three drugs (which will likely first be composed of ivacaftor, tezacaftor, and a next-generation modulator) in a triple combination therapy addresses a different aspect of the defective CFTR protein. When used in combination, the results may be better than using one or two of the drugs alone. Already, several next-generation modulators in development have the potential to be significantly more effective than current FDA-approved modulators.

Next-generation modulators may also benefit more people with CF than first-generation modulators. The first-generation drug combination lumacaftor/ivacaftor can only help people with two copies of the F508del mutation, or about 50 percent of the people with CF. But, early clinical trial results suggest that several of the next-generation modulators could benefit people with CF who have only one copy of the F508del mutation. Because nearly 90 percent of the CF population has one or two copies of the F508del mutation, next-generation modulators would enable a much larger number of people with CF to have a modulator treatment.

Don’t Stand So Close to Me (Five Feet Apart)

I chose this song more for its ironic title than the content. It’s a song by the Police, released in 1980, apparently about a female student and a school teacher who have a forbidden sexual chemistry. While I like the song I don’t particularly like the content and background of the lyrics. The topic has been sung about before, like in Van Halen’s Hot for Teacher. The fact that Van Halen and the Police were awesome bands that doesn’t make it okay. We have all heard in the news about inappropriate relationships between female or male teachers and their students, and the consequences that resulted. I won’t glorify that any further, but will just say I liked the title of the song as it relates to the topic I really wanted to talk about, Five Feet Apart.

This is a book, written by Rachael Lippincott and collaborated on by many, about 2 teenagers with CF (Will and Stella). They are polar opposites: Stella is neurotic about her treatments and even developed an app to remind her to do and take everything on time. Will is more of a rebel, rarely doing his therapies and stating as soon as he turns 18 he will leave the hospital to live out his days on his own terms. He has a ticking clock since he has colonized a deadly bacteria, B. Cepacia (see explanation below). The main difficulty with this bacteria is its resistance to most or all antibiotics.

This is the backdrop of them “meeting” in the hospital. I say that in quotes because the isolation and infection control measures for us cystics in the hospital are extreme, to say the least. CF patients are isolated in their own rooms, and all medical personnel must wear full disposable gowns and sanitize in and out of every room. I am always astounded by the amount of gowns that are used just for me everyday, probably upwards of 50! Then if a CFer wants to leave their room, they must wear a mask and stay 6 feet apart from other patients. During the story, Will and Stella end up liking each other, and Stella decides to “steal” one foot of distance thereby making them Five Feet Apart. I won’t spoil the story or the ending, so you will have to read the book or go see the movie.

There has been an ongoing debate in the CF community about this book/movie, with wildly different points of view. Some believe that the CF isn’t accurately portrayed in the movie, and that is just a backdrop to the romantic storyline (which is also unrealistic). Others believe that while the CF and hospital depiction may not be fully accurate, it is important to show the mindset of a CF patient in the hospital. Also it could help raise awareness and publicity not only for the illness but how patients and their loved ones cope with this debilitating disease. I land somewhere in the middle; truth be told I only read the book and haven’t seen the movie. I think the book probably more accurately portrayed the CF hospital environment and the mindset of a patient, as books can offer a better imagery than a movie. However, I believe the movie is a good thing overall. So let’s discuss from both sides.

There are a few things that might turn people in the CF community off to this film. As seen in some of the trailers, both of the characters do not wear masks at all times, and are given free reign over the hospital to wander as they please. This may or may not be accurate depending on the institution, although the mask rule is not a variable and CF patients must wear them when around other CFers! I have been in hospitals where I was able to roam, and some when they wouldn’t allow me to leave the room let alone the floor. So it’s hard to judge that aspect. But, the romantic element to the movie is probably exaggerated and fantasized. Like anyone in the hospital is in the mood for love; the whole reason you are in the hospital in the first place…….is because you are sick!! And not just sick, but on oxygen, hooked up to IVs, fatigued, fighting with your body. Some people are waiting for lung transplants, or even too sick to be considered for one. I know when I’ve been at my lowest, I wasn’t thinking about hooking up with other people who are inpatient for exacerbations. There is too much else going on: treatments, exhaustion, effects of strong antibiotics, etc. Safe to say, it’s not a pretty picture. I’m just saying, the hospital romance seems very Hollywood. I’m sure that other elements of the hospital experience aren’t accurate, even though they consulted Claire Wineland. She was a CF activist and advocate who sadly died recently from complications of a lung transplant.

That brings me to the other side of this arguement, and the biggest positive of this movie: awareness and publicity. Any awareness and publicity for CF is a good thing in my view, as society as a whole does not know much about this disease. Even people who might know the basics, or know someone who has CF, they probably are unaware about the hospital experience, the daily difficulties and complications that come with this disease. Seeing it on the big screen, all that comes into play both physically and emotionally, is hard to relay to people. While this film may be slightly glorified, it’s not as dark as other movies centered around CF. When I was little, there was a film Alex, written by Frank Deford about his daughter Alex and her life. She died in 1980, and her short life was riddled with hospitalizations and complications. It was a very tough movie to see, so tough that I wasn’t allowed to watch it when I was young. Then there is A Fault in Our Stars, a more recent film with a similar pretext to Five Feet Apart. Two teenagers, one with B. Cepacia, fall in love online and get married despite all the warnings against. They both died shortly after. And………that’s about it. There haven’t been a lot of movies centered around CF, because it is so hard to depict given the subject matter. Five Feet Apart may not be the most real or depressing movie about CF, but it will show people some of the difficulties and hardships people struggle with their entire lives. They might tell their friends, or try to learn more about it. If more people know about CF, then more money can be raised for research, which can lead to a cure. CF can someday stand for Cure Found.

So go see the film. Bring your friends. Read the book. Learn more about this disease.

Watch the movie trailer below:

https://www.youtube.com/watch?v=24YrEAGF32M&vidve=5727&autoplay=1

Information about B. Cepacia from the CF foundation:

Burkholderia bacteria are often resistant to many antibiotics, which makes them difficult to treat once they infect the lungs. However, some species may be successfully treated with combinations of antibiotics.

Basic infection prevention and control practices reduce the risk of getting or spreading B. cepacia. These bacteria pose very little medical risks to healthy people. However, some people who have a serious illness such as cancer or acquired immunodeficiency syndrome (AIDS) may be at risk of an infection from these bacteria.

How B. cepacia complex species affect people with CF varies. Researchers do not yet know why some people with CF are more likely to get B. cepacia than others.

In many people with CF, infection with B. cepacia may not worsen lung disease. In up to one-third of people infected with B. cepacia, the rate of lung function decline appears to be only slightly faster. However, for a smaller number of people, B. cepacia can cause a rapid decline in lung function and health. This can lead to more severe lung disease and maybe death.

Among the different species of B. cepacia complex, some may be more harmful than others (for example, B. cenocepacia and B. dolosa). The Cystic Fibrosis Foundation is working with researchers to learn more about the B. cepacia complex and help identify new treatments.

It is not always known how people with CF become infected with B. cepacia complex. Research has shown that people with CF can get B. cepacia from others who are infected with these bacteria. The germs spread either by direct contact, such as kissing, or indirectly from touching objects with the germs, such as doorknobs. This is known as cross-infection and can happen in social settings like events, gatherings or meetings.

In some cases, shared infection was not found in the lungs of a person with CF until two years after being exposed to someone else who was infected with the germ.

For many people with CF, infection with B. cepacia complex cannot be traced back to exposure to another infected person. In these cases, infection may have occurred by exposure to Burkholderia in the natural environment.

For the Love of Money

I was listening through a funk mix the other day, and this gem came on, courtesy of The O’Jays. Written by the songwriting pair Gamble and Huff who was largely credited with creating the Philadelphia sound of the 60s and 70s, it is a song that anyone can enjoy. Whether you’re a funkateer or not, or are part of different generations (millennials, Gen Xers, baby boomers, etc.), anybody listening to this song starts bobbing their collective heads to the rhythm. Starting with a hi-hat and funky guitar, then slowly added with a great baseline, and the melodic voices of the O’Jays that was very reminiscent of the Temptations. Everyone knows the words to this timeless classic; they hold true as much today as they did in 1973.

For the love of money
People will steal from their mother
For the love of money
People will rob their own brother

For a small piece of paper it carries a lot of weight

Call it lean, mean, mean green

Almighty dollar

While I was listening, I related this to a current and very important matter: drug prices. This is an issue that affects not only the CF community, but American society in general. Anyone with Diabetes who may need insulin, to someone with and auto-immune disease such as Rheumatoid Arthritis or Crohn’s disease, to a person with cancer. These people are clinging to hopes of any new medications that may be available, that in turn may increase not only their life expectancy but also quality of life. Mari and I were watching Hasan Minhaj’s new show on Netflix, Patriot Act, while he was discussing this very topic. America has the highest drug prices of any developed country, causing certain people to prioritize whether or not they can get their needed medication due to overwhelming increase in costs. People are traveling to other countries and smuggling back their meds, or spacing out their required daily medications and not taking them as prescribed because they cannot afford the refills. That is an incredulous statement for the richest country in the world, don’t you think?

For me, a person who with CF, I can relate to these struggles. While we were not poor growing up, I can only imagine the sacrifices my parents made in order for me to receive the proper care and all the newest treatments available. I know I probably didn’t appreciate it at the time, but I sure do now as an adult. There have been more than a couple times that I have not been able to afford my medications, due to a variety of reasons. Whether it was a poor insurance policy that only covered a minor percentage of my medications (most CF meds are top tier without any generic substitute), or not being able to shell out the hundreds of dollars for nutritional supplements or vitamins/minerals that are not covered on any insurance, or going to collections for the rising number of monthly payments to hospitals and clinics, the list goes on. All the while, I make a good living….this fact makes it more difficult to swallow. How can people with chronic illness afford be expected to succeed in this system when they can’t even afford what they need to survive?

This discussion becomes even more relevant to the CF community in the age of CFTR modulators. It’s a new class of drug that has been undergoing trials over the past several years. In short, it helps regulate the sodium chloride pump and prevents the buildup of sticky mucus congregating in the lungs. It’s important to point out that these drugs are not cures, but can significantly impact lives of those with CF. There are different classes of modulators, dependent on the specific genetic mutation of the person with CF.

Orkambi is one such drug, one that targets those with CF who have 2 forms of the most common genetic mutation (F508Delta). It is now on the market, at the low, low cost of approximately $100,000 per year!! Sounds like a bargain, right?! Not so much say the insurance companies. Certain companies, both private or state/federally regulated, have determined that the cost does not justify the possible benefit of this medication. In turn they are denying coverage for these meds. Imagine this……..someone who has lived their whole life just getting by until a possible life-altering medication comes by, and when it finally does it isn’t covered. What would you do? Most people, myself included, could not even fathom shelling out many thousands of dollars per month in order to afford the drug. This is another example of CF literally beating the life out of you…….even when there is hope it comes with heavy strings. Are you supposed to go into bankruptcy just to live? What kind of life is that? How is the government going to help these people?? Oh yeah……..they’re not! They are just going to try to reduce people’s access to the medical care and aide they need by reducing the qualifications for open market insurance and Medicaid expansion. It’s exhausting and infuriating!!

It all comes down to money: how much the drug companies can make, and how many politicians they can have in their pocket to influence their profit margins. Like the song says, “For a small piece of paper, it carries a lot of weight.” Truth!

Thinking Out Loud

I wanted to write a post to my wife, Mari, for Valentine’s Day. Both of us think that Valentine’s Day is more of a “Hallmark Holiday,” and thus we don’t really celebrate it. However, I thought that this would be a nice gift, a way to express how much she means to me.

Our life is rather complicated, more than most people our age. Largely, that has to do with CF rearing its ugly head more and more as time goes on. Add to that both of our careers, our teenage son, and managing our everyday life and it all adds up to…….A LOT!! Sometimes, we both feel like our head is going to explode, and we both just want to run far away from it all. But we don’t (obviously); I’d like to think that has to do with the deep love and respect we have for each other. I know for myself that she has made me a better man, husband, father, son, and person with chronic illness. So this song expresses a deep love that will last through thick and thin; this just so happens to be one of her favorites!

Thinking Out Loud, by Ed Sheeran, is a love song about growing old together and letting the love grow with age, like a fine wine. It has become one of the most successful singles of all time, having been streamed over a half a BILLION times and was number one on the charts for over a year. Sheeran sings this song as if he is older than he really is, like an old soul that has experienced this deep and long lasting love. In truth, he is only in his 20’s and probably has not. But that’s the power of music; being able to channel something that you may or may not have done or felt in life into something that resonates with the masses. In turn, the listener can interpret the song and relate that into their own life. This song is pretty transparent in its meaning of everlasting love, and has thus become a staple on the wedding circuit. Here are a few lyrics:

When your legs don’t work like they used to before
And I can’t sweep you off of your feet
Will your mouth still remember the taste of my love
Will your eyes still smile from your cheeks
And darling I will be loving you ’til we’re 70
And baby my heart could still fall as hard at 23

Cause honey your soul can never grow old, it’s evergreen
Baby your smile’s forever in my mind and memory

So this song is for you Mari. Not only are you incredibly beautiful, but you’re smart, funny, and possess an inner strength that is unmatched. You are my wife and partner, have had to navigate waters not many people can understand. Being a spouse of someone with CF means sitting by and watching the person you love go through immeasurable obstacles and life-threatening infections. All the while, that person has to manage the entirety of the life built together, as the hospitalizations grow in frequency and intensity, the ability to work and support becomes less and less, and the world you built together gets flipped upside down over and over and over……….. It’s exhausting, and the spouse often gets overlooked for all of the sacrifices made. Even if a person knew some of the hardships of CF they could not be ready for all it entails as the progression takes hold.

Mari, you are the love of my life, and my rock through all this crazy and maddening ride we call the CF train. I cannot imagine my life without you by my side, and these words cannot fully express how much you mean to me. I hope that we grow old enough to make Ed Sheeran’s words come true.

Happy Valentine’s Day!!

Fly Away

So I’ve been gone from this blog for a bit, since I was released the hospital. I just needed some time away from it all. CF encompasses so much of my daily life, especially leading up to and during any hospitalizations. When I get out, and I’m feeling more at my baseline, I try to not think about my illness for a while. I was hoping for a little more of a “vacation” from CF, but it wasn’t cooperating. It only lasted a week. I developed another cough and my day to day difficulties crept back in well before I was ready for them. Now, I’m back to increasing my therapies to reduce my cough, trying to prevent another downslide and keep myself out of the hospital. And this is my life, every freakin day of it. I’m just tired of the grind.

As my symptoms started to creep back in, I was trying to figure out how to express my frustrations and thoughts about wanting to escape CF. I was in my car driving, and this song came on, Fly Away by Lenny Kravitz. It’s a song about escaping the difficulties of the day, even for a while, and getting away with the people you love. While that is not steeped in reality for most, who cannot just charter a flight to a great getaway on a whim, it is more the thought that works for me. Trying to escape my CF, even for a little while in my mind, to help make the bad or frustrating days a little easier. I learned early on in life that even 10 minutes of meditation, sitting alone and focusing on nothing but breathing can make a big difference. Ironic, it seems, as breathing is the cause of most of my difficulties! Even though it is difficult to do, I feel a little more balanced when I’m able to. So….I’m listening to this song as I’m feeling really frustrated about my cough and symptoms. Not only is the song a great message, but Lenny in general is a great person of positivity with a great story to tell.

Let’s face it: Lenny Kravitz is really cool. He so cool, he’s a bad mutha……..Shut yo mouth! (Couldn’t help a shaft reference there). Kravitz grew up in New York to a white Jewish TV producer and African American actress mother, Roxie Roker (She was on the Jeffersons). Growing up in this world, he was exposed to more culture than most people his age. Immediately he was drawn to music, influenced heavily by funk, jazz, blues, etc. He started in the mid 80s with his debut Let Love Rule, but didn’t make a huge commercial mark until Are You Gonna Go My Way. He had long dreads and sung about love and peace. It didn’t hurt he married Lisa Bonet, who was super popular at the time as a star of The Cosby Show (when it was on top of the world well before everyone knew about Mr. Cosby). He was totally cool; guys wanted to be him and girls (I assume) wanted to be with him. By the time he came out with his 5th album, on which Fly Away was a track, his stardom had continued to rise. Yet he continued to be philanthropic, and to spread his message of peace and love, which he continues today.

Back to the story at hand. I think about trying to get away from things. Not that my life is bad, by any means. I feel extremely lucky and fortunate, having found the love of my life, Mari. We have built a great life for us and our son in Chicago and now in Denver, and I have made it to 40 years old. I have a wonderful extended family and great, supportive friends. I am a medical professional and a CF advocate. Most days I am happy to take all that on; but on other days I don’t want to be that person. I don’t want CF to rule my life, I don’t want to take care of others; I just want to go away with my family somewhere far away. To where I’m healthy and can run around and play without coughing fits. To where I don’t have to worry about getting a minor illness that could catapult me into the hospital before I know it. To where I can just be, living a regular existence without the constant roller coaster that is CF.

But I know that’s not reality, so I have to get it in small doses. Whether that’s not being involved in CF and advocacy for a few days to a week, or not being in constant communication to everyone about the status of my health, or even just taking a day where I don’t do anything! (Although even those days I have to do my treatments, so I can’t even escape then!! UGH!). Sometimes, we all need a break. To get away, or fly away in this case. Hopefully, the people around you will see and allow for this. Because CF or chronic illness or just life is hard, and we all need a break sometimes.

I promise not to take too many breaks from this blog, but if I do you will know why 🙂

Wiser Time

This is a beautiful ballad by one of my all time favorite bands, The Black Crowes. They burst on to the scene in 1989 at a time when there weren’t many mainstream rock bands. Their first hit was a remake of Otis Redding’s Hard to Handle. They were brash and in your face, fronted by the brothers Robinson (Chris on lead vocals and Rich on backup vocals and guitar). The Robinsons grew up in Georgia, influenced heavily by The Allman Brothers, Derek and the Dominoes, Lynyrd Skynyrd, and others. Chris had such a raw power to his voice, you swore he was older and bigger than he was. To look at him then, he was a beanpole that looked like a strong wind could blow him over. Nevertheless, he could belt out anything he wished, from soul, to a great ballad, to a great rock tune. The band as well embraced their newfound fame, bringing back the excess and debauchery that dominated the 70’s rock bands. Their best album in my mind was their sophomore effort, The Southern Harmony and Musical Companion. This album kicks some serious ass from start to finish, with highlights such as Remedy and My Morning Song just to name a few. During the recording of their third album, Amorica, (which Wiser Time was one of the results), their drug excess led them to a bit of a spiral. The brothers were always fighting and the band broke up a few times. However, they still managed to crank out some great songs.

Wiser Time is my favorite track on Amorica. It has a Southern rock feel, with slide guitar and dual percussion accenting the great harmonies of the brothers, reminiscent of The Allman Brothers. Chris and Rich wrote this song about traveling on the road and all the adventures and personal growth that come along with it. As the title seems to imply, one gets wiser and older when you step out of your comfort zone and push forward into the unknown.

No time left now for shame, horizon behind me, no more pain
Windswept stars blink and smile, another song, another mile
You read the line every time, ask me about crime in my mind
Ask me why another road song, funny but I bet you never left home

As I read these lyrics again, I think about how I came into my own. I had just finished college, where one obviously becomes more of an adult and needs to care for themselves. For the first time, I had been in charge of my health and daily care of CF. There were ups and downs, admittedly I had times where my daily routines were ignored. That may have been in part due to my parents. They were always there, within a few hours; the safety net there in place. Whether I was at San Diego State or Iowa, my parents at that time still helped me with insurance and the like, carrying the majority of the heavy weight that is CF on my behalf. While that was comforting, I knew I needed to grow and figure it out completely on my own.

So, after college, I decided to take a road trip. I packed my Corolla and traveled for approx 2 months and over 6,000 miles, driving solo through the western part of the country while I looked for my next place to land. I would stay in hotels or camp, having my medical supplies at my side. While on the road, I had countless hours to myself to think and reflect, on everything from life and death, my family and friends, and my CF. It was in Boulder, CO that I saw possibility for a different and healthier lifestyle. I hiked in the mountains and did some rock climbing, enamored by the environment and atmosphere. I already had a few buddies that had settled there, and decided then to join them permanently.

While there would be many subsequent bumps in the road, that experience was invaluable. Living completely independent of my family was absolutely needed for my evolution at the time. Obtaining my own insurance (and having to deal with all the intricacies therein), and figuring out how to support myself through the good days and bad. Managing my increasing daily and long term medications, I supported myself physically and financially all while handling hospitalizations and exacerbations alone. While not easy, I felt I had to accomplish this myself. I think back to Wiser Time:

On a good day, it’s not every day,
We can part the sea
And on a bad day, it’s not every day,
Glory beyond our reach

I take from these lyrics that the bad days are not as bad as they may seem, while the good days and the accomplishments achieved will propel you forward to new heights.

I think about that experience and time in my life, as I walk these halls of the hospital filled with fellow CF patients. I feel very fortunate of my path, forcing myself to grow into a more independent person battling a disease that can be very hard to manage. Many cystics may not feel comfortable going out on their own, but I think it was crucial to my overall development. And I encourage all people with CF or other chronic illness to try.

Remember, it is better to try and fail then never to have tried at all. If you fail, or if things become too difficult, the people closest to you will be there to pick you up. But you will become stronger and better for persevering. And you will be wiser for the road travelled.

Bouncin’ Round the Room

So, here I am, back in the hospital, yet again. I prepared for this one, taking time off work. It’s more of a tune-up or an “oil change” so to speak. I’ve had to accept that I need more frequent hospitalizations to maintain any kind of lung function and normal everyday life. As I sit here looking at these strangely familiar walls of my room, I often think of this song.

It’s a Phish song, written by Tom Marshall, who writes a majority of their lyrics. The origin of the song is about his experience in a bouncy house where he was inadvertently knocked unconscious. Sounds a little dark I know. However, the music is written in a major key, highlighted by a great drumbeat as always from Joh Fishman. When they play this song in concert, the mood is light and the crowd is always into it. The song features all four members singing different lyrics and melodies in a call and response theme. (If you’re not familiar with this, just think of row, row, row your boat, gently down the stream…). It’s a crowd favorite, since everyone can pick their favorite part to sing. The call and response continues to repeat, building up to a crescendo. The energy in the crowd is palpable.

I remember when I first was exposed to Phish. I had heard my friends talking about them, probably Sophomore year of high school, 1994 or so. They were raving about their unique sound, mostly gushing about how great they were live. Mostly, they raved about Trey Anastasio and his guitar solos, and how he was as good as Jerry Garcia (Trey is amazing and all, but I don’t know about that). So I went out and bought an album, A Live One, because I thought that would be the best way for me to hear and evaluate them for what they have always been, a jam band that is best heard live. The first track on that album just happened to be Bouncin’ Round the Room. I liked this song a lot, as well as a few others. Soon I was hooked, and would wind up seeing them more than a couple dozen times in concert over the next several years.

Now, back to the present. As I sit in this hospital bed, yet again, I have a feeling of deja vu. I have been here in these rooms, that all look and feel the same, more times than I care to count. This is the life of a cystic, the constant exacerbations and hospitalizations. This stems primarily from the consistent buildup of bacterial lung infections that will never cease. Just in the past 12 months, I have probably spent 2 of them in here, constantly hooked up to IVs, doing nebulizers, and having the sputum literally beaten out of me. It can be exhausting and demoralizing at times. You sit here and try to keep busy, just trying to keep your mind occupied. It can be really hard to sleep, even though that’s what you need the most. Sometimes, you are just goofy and slap happy. That’s when I hear the lyrics to this song:

Then before and now once more, I’m bouncing round the room
That time then and once again, I’m bouncing round the room
That time then and once again, I’m bouncing round the room
That time then and once again, I’m bouncing round the room

I hear it like it’s being looped over and over again. While I always had enjoyed the song in concert, I don’t always love it while here in the hospital, because of how I feel in those moments. While it can be easy to get into that negative headspace due to the environment and circumstances, it can often be much harder to get out. So I try to transition from hearing those lyrics to thinking about many of the great moments I can remember from those Phish concerts. Some of them are a little more cloudy than others, but still I can remember being part of the energy and community which kept people coming back to their shows over and over again. That time then and once again, I was there in the crowd. And can revisit those times whenever I need a boost of positivity. These are some of the tricks I use to help me through yet another hospitalization. If any other cystics are reading this, I hope you find your own way to remain positive and keep out of the negative headspace that often comes from being in here. Keep calm and carry on!

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