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!

Dreams

This is a beautiful song by the Allman Brothers. Written by Gregg Allman, it was the first song he had prepared when he joined his brother’s band. At the time, he was out in California trying to make it as a solo artist, after literally shooting himself in the foot to avoid the draft. When Gregg joined the band, everything clicked. They spent night after night refining their sound, usually with the help of mushrooms. They adopted the mushroom into their logo and all had corresponding tattoos. Of course, that was the late 60s, during the free love movement and war protests, when psychedelics were at their height. Music at that time was dominated by bands like The Beatles, The Byrds, The Rolling Stones . While there was a blues influence to some bands of the day, the Allman brothers took this genre and put it at the forefront of there music. Along with Gregg’s soulful vocals, his brother Duane was a master of the slide guitar. Duane had made a name for himself by playing with Wilson Pickett and Otis Redding, but they needed to create their own sound. The two complimented each other very well along with Dickey Betts on guitar; then the rhythm section of dual drums and bass provided the thriving backbone. They were just making a real name for themselves, after the Fillmore East recordings became a thing of legend. Tragically, Duane died of a motorcycle crash shortly afterwards, and their bassist died the same way a year later. Through all this, The Allman Brothers powered on and made music as a band until Gregg’s death last year. As a band they made an undeniable mark on music and influenced countless people, including me.

At the time I discovered them, I was coming out of a heavy metal phase and looking to find myself a little better. While I embraced the emotion of heavy metal, I didn’t always feel like I connected as well as I should. My father and I had been talking about music and some of his influences growing up. He had mentioned them, although they weren’t one of his favorites. I think I was excited that they had two drummers and wanted to see what that sounded like. So we went out to the store, and I picked up a few CDs. I got Live at the Fillmore East and was immediately enthralled. Gregg’s voice boomed through the speakers, full of hurt and despair, with the guitars accenting different phrases along the way. They built their songs to crescendos, the emotions just pouring out so much it was impossible not to feel. Dreams is a slow emotional journey, a simple chord structure played on Gregg’s organ with blistering slide guitars on top. The lyrics talk about getting away to solve your problems with a little solitude, fearful that your problems may overcome you and prevent you from reaching new heights in life. As the chorus goes:

‘Cause I’ve a hunger for the dreams I’ll never see, yeah, baby.
Ah, help me baby, or, or this will surely be the end of me, yeah.

I really connected with this; how all of your pain and anguish can be expressed into music but without rage. At the time, I was having trouble coming to terms with CF. I wasn’t sure who to talk to about anything, since almost no one I knew had any inkling of what I dealt with. Being a teenager was hard enough, without the added strain of a chronic illness. I was angry and confused, insecure about all the daily intricacies that come with the territory. It caused me to be more of an introvert, which I still am today. I have a hard time expressing my emotions, doubts, or fears. Sometimes I need solitude in order to come to terms with these feelings and cope. It’s as true today as it was back then.

It’s easy to let CF overpower you; it can be overwhelming. Figuring out how to deal with this illness while trying to figure out who you are as a person is a real challenge. Everyone needs a creative outlet of some kind, whether it’s art, theater, music, or something else. Music was always that for me, and the blues created a whole new path to coping with my illness. To this day, I will blast the blues in my car when I’m having a bad day. I will sing along to my favorites, like Buddy Guy, Freddie King, Eric Clapton, and Stevie Ray Vaughn. I connect with them on a visceral level, even though our experiences are totally different. The emotions feel the same, and relate to my life and struggles. This musical companion of sorts is with me whenever I need, like a comfortable blanket. And I have the Allman Brothers and my father to thank for that.

Smells Like Teen Spirit

My life has swung full circle. I am now a father, having recently crossed over into an old age (40). My son just just turned 13 and is a full blown teenager. As I look at him now, many things that come to mind. First, I am amazed at how fast he grows; both Mari and I realized a while ago we would soon be looking up at him! Next, I think about how much of us I see in him. Even though I am not his biological father, I see so many traits that remind me of myself. No matter how different he is from me in some aspects, in others I can see my influence. That can blow your mind sometimes, how you can raise a human being and always see parts of yourself in them. He is entering an age where he will hopefully find his true self.

Think about the mindset of a tween/early teenager. One is easily influenced at that time by both your peers and pop culture, still unsure of the person they are to become. It’s like a fresh ball of clay that has started to form but hasn’t taken a real shape. Hormones infiltrate and take over the brain, causing times where there seems to be little or no function. I look at Jackson at those times and wonder what (if anything) is going on in that head? There seems to be very little, mostly because he is tuning us out and thinking that he already knows what we are trying to tell him. However, at other times he can be very sensitive and focused. The wild swings between the two are mind-boggling! There are many more life benchmarks ahead, and Mari and I are both bracing for and trying to figure out how to best manage them as we go.

I recall when I was his age, during the early nineties. Pop culture was dominated by the likes of Milli Vanilli, Boys II Men, and R&B groups of the like, along with shows like Saved by the Bell and Full House. I wasn’t huge on this genre, but was also easily influenced and tried to get on board. I had my Z Cavaricci pants and short sleeve button downs. I rocked a mullet, which should never come back into style. I tried some of the dance moves like the running man and danced to NKOTB. Yeah, I was trying to be that kid.

But I knew that wasn’t who I really was. Then, suddenly and miraculously, Nirvana bust onto the scene in 1991 and instantly turned the music industry on its head. The music was raw, with plenty of angst and anger, highlighted by heavily distorted guitar and pulse-pounding drumbeats. Kurt Cobain screamed more than sang, voice cracking with emotion as he belted his dark and moody lyrics. It was a stark contrast from everything else at the time. Smells Like Teen Spirit hit the charts and spawned a new music genre, grunge. After the release, many other Seattle-based bands came to the forefront in their wake, like Pearl Jam, Soundgarden, Alice In Chains, and Stone Temple Pilots. That grunge sound really struck a chord with me. The songwriting was more melancholy and real, unlike the boy bands and bubblegum pop that was on the radio.

I was going through a lot at that time, both with my health and my family. My parents were unhappy, seemingly always fighting. They would split up shortly afterwards, which meant we weren’t moving to San Diego to all live together (another story for another day). At the same time I was trying to come to grips with my CF and its progression and its result of regular hospitalizations or tune-ups. While I was hospitalized many times earlier in life, they were primarily related to GI issues and not respiratory in nature. But I was starting to colonize strands of bacteria in my lungs, causing infections to consistently build up in my lungs. I tried to downplay it in my mind, but it affected me. I had to miss large chunks of school, and I was no longer able to successfully hide my CF from other.

Being a tween and early teenager is inherently about your insecurities and attempting to overcome them. This was nearly impossible for me, as I was reminded many times a day in class. Most days I would have productive coughs that made me sounds like I was a chain smoker. It would get the usual looks of disbelief or dread from my classmates, like I was going to infect everyone around me. I would attempt to hide this, either by excusing myself to the bathroom multiple times a day (which probably caused its own suspicions), or trying to “swallow” my coughs (not effective, just swallowing air and causing GI issues). I would also try to “breathe” through it, which took most of my concentration and probably affected my attention in class and probably sounded like a Lamaze class! Anyway I tried, it was nearly impossible to hide. As these issues were more apparent, I shied away from a lot of interactions, especially with girls. I was unable to let anyone get close to me for some time, affecting my growth into a young man I’m sure.

Now I know what you may be thinking. Everyone has issues at that age! And to any given teenager, at the time, those are the most difficult things they could imagine. That is true, and I realized later on that everyone had their own crosses to bear. But that doesn’t help one during that age, with so many changes in your physical, emotional, and mental self. So I try to think about this when looking at my son. That he is dealing with issues different from what I did, but no less challenging or embarrassing in his mind. And even if some of these issues seem trivial and not important, I need to think about it from his perspective. To go back to what it was like to be his age. To the era of grunge. And listening to Nirvana and Smells Like Teen Spirit helps me get there.

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