Book by brandon johnson

I’M SMOKING, BUT IT’S HELPING MY LUNGS

Brandon Johnson

I’M SMOKING, BUT IT’S HELPING MY LUNGS

Brandon Johnson

Vest buckle for CF machine

I

Dedication

would like to dedicate this book to everyone who is a part of the Cystic Fibrosis community. This includes doctors, loved ones of patients, and patients themselves.

II

Nebulizer that helps with CF

acknowledgements

I

could not have created this book without the help of my mother, Carol Johnson. She gave me the idea to do my documentary on Cystic Fibrosis. I would also like to thank my sister, Tiffany Johnson, for helping me edit the text of my book.

III

A Machine that puts a salt solution into the nebulizer

The contents PREFACE ......................................................................... pg. 9 INTRODUCTON ............................................................ pg. 11 CHAPTER 1 - THE DOCTOR ........................................ pg. 13 CHAPTER 2 - THE LOVED ONE ................................. pg. 16 CHAPTER 3 - THE PATIENT ....................................... pg. 19 CONCLUSION ............................................................... pg. 21 WORKS CITED .............................................................. pg. 23 ABOUT THE AUTHOR ................................................ pg. 24

An inhaler that tends to CF symptoms

I

Preface

chose the documentary subject of Cystic Fibrosis to contact them. Instead, I found a different doctor (CF) because it is one that relates to me. Not who was able to discuss the topic. Throughout this

only does my brother have the disease, but I do as project, I learned new writing and filmmaking skills. well. I was excited to have the opportunity to educate These include making an argument on a topic and people on CF as many remain unaware of what this setting up a dual camera set-up for film. I learned topic is. Although I am passionate about this topic, I and improved more as the project progressed. I want was worried that I would not have enough research the reader to become aware of what CF is and how it material to use in my project. Another challenge I affects families. I also want them to understand that ran into was finding a doctor who would discuss this an issue that everyone can help with. the topic. Since doctors have

busy schedules, it

was hard to find someone who would have time to be interviewed. There was a specific doctor that I had in mind for interviewing, but I was not able

Nebulizer Equipment Being Sterilized 9

A vest that shakes the chest

introduction

M

ichael Johnson is in the family scuffed nebulizer sprays out what appears room,

television

watching

s h o w.

He

is

his sitting

favorite to be a thick cloud of cigarette smoke, on

his but in reality is simply spraying out a salt

favorite spot on the couch, a spot that is solution. All of this equipment is used to f a d e d f r o m h u n d r e d s o f h o u r s o f u s a g e . t e n d t o M i c h a e l’ s C y s t i c F i b r o s i s . C y s t i c This

seems

like

a

very

normal

thing, Fibrosis is a lung and digestive disease

sitting in a favorite spot on the couch, the that affects thousands of people around seat worn from use, and simply relaxing. the world. It keeps these people from M i c h a e l , h o w e v e r, h a s a v e s t a r o u n d h i s l i v i n g n o r m a l l i v e s b e c a u s e o f i n t e s t i n a l chest with two laboratory-grade tubes that damage and increased levels of mucus in f i l l t h e v e s t w i t h a i r t o s h a k e h i s b o d y, t h e l u n g s . I t ’ s s o m e t h i n g t h a t i s n o t o n l y a n d m o r e i m p o r t a n t l y t o s h a k e t h e m u c u s i m p o r t a n t t o p a t i e n t s , f a m i l y, a n d d o c t o r s , from his lungs. Shaking like going up an but to everyone because someone around incline on a roller coaster ride, bumpy you or someone you know could very well and rumbling. In his hand, a worn-out and have it.

11

“So it’s a disease that causes multiple infections in your lungs because of this thick mucus . I t blocks your airways and bacteria love mucus so you can get multiple lung infections .

Eventually

there ’ s damage to your

lungs which can cause respiratory failure and eventually death ” - Dr. Mary Helmers 12

1 - THE DOCTOR

W

hat is Cystic Fibrosis specifically? Doctor Mary

Helmers says: “Cystic Fibrosis is a genetic disease

which you get from both your parents … majority of patients have pancreatic disease but there’s a portion of patients that don’t. It can also affect liver and reproductive organs. So it’s a disease that causes multiple infections in your lungs because of this thick mucus. It blocks your airways and bacteria love mucus so you can get multiple lung infections. Eventually there’s damage to your lungs which can cause respiratory failure and eventually death.”

Doctor Helmers is a pediatric nurse coordinator for the Cystic Fibrosis center at Stanford Children’s Hospital. She sees Cystic Fibrosis patients, checks up on their health, and prescribes medications when necessary. Some of the things she checks for in Cystic Fibrosis patients are: “vitals, your height, and weight because that’s a key sense to see how you’re doing nutritionally. … The nurse will go over

Miscellaneous CF equipment all the medications that you’re on … The RT (respiratory therapist) will check … how well you’re lung function is doing and … if there’s any social issues that are needed or if you’re having problems with bills or finances or any insurance change or no insurance or parents lost their jobs or just family concerns the social worker will also speak with you.” She determines what kind of medicines they need by “what their symptoms are, the progression of their disease, or what they have [and] the extent of their disease.”

These medications include respiratory medications and nebulizer (a tube like inhaler) medicines. Recently, there 13

have been new advancements in the development of are used to help treat infections. They may not be used for drugs that treat Cystic Fibrosis. These include new triple inhalation but we do. So there’s studies that are looking combination drugs made by Vertex and a drug called into making those medicines being used for nebulization.

RPL554 that can “significantly improve lung function in Then the target besides looking for all these new better patients with Cystic Fibrosis, according to top-line data antibiotics to treat all of the new different bacteria that seem from a Phase 2a clinical trial.” When asked about the triple to be out there these days, the biggest change has been for combo medicine, Helmers states: “I will say this new triple these modulator therapies looking how they can change combination will be something that we will be looking at to the defective gene or make it function more normally. … see in time. But I think it’s too new right now to see in our I think more the focus is on that. There’s a lot of research new patient population because we haven’t switched over. being done on the types of bacteria that patients cough up.

If patients have done well on the Orkambi or the Ivacaftor Some of our docs are looking at that and seeing the types of [Other more common drugs] we haven’t changed them things that can be done to help limit or lessen the bacteria over at this time.” When asked about the RPL554, Helmers load in patients with CF as well.” As a result of all the had no comment on it as it was one she had not heard of different symptoms and medications of Cystic Fibrosis, it before. In addition to research on new potential treatments, can be a very overwhelming disease. So what would it be other research is on the way that can help change the future like for a loved one taking care of a patient? of Cystic Fibrosis. Helmers states that: “research was done on testing new antibiotics and therapies. Not that we’re not checking for new antibiotics because I know there’s certain medicines that are given through an IV, meaning intravenous going into your blood that are antibiotics that 14

Dr. Mary Helmers

“As a parent , you usually want your children to be just the best at everything they can be and you ’ re concerned about just how well they ’ re doing in all aspects of their life and now it ’ s focused a lot on his health , and

I

want to

instead be more focused on how happy he is rather than oh , is he going to get into the best college or things like that or is he the best at this sport . that anymore . happy .”

I

don ’ t really care about

I just want him to be healthy and - Carol Johnson 15

2 - THE LOVED ONE

C

ystic Fibrosis can be a tough disease for one to have.

It’s also difficult to be a loved one of a patient. This is

true for Carol Johnson, a parent of the CF patient Michael Johnson, mentioned earlier. Before the diagnosis, Carol was a mom dealing with the stress of raising four kids.

There was always a lot going on and it never seemed to

Connectors to the CF vest

stop. The stress was amplified once her youngest son, became a teenager. When Michael was diagnosed, he had Brandon, realized there was a nasal polyp, a giant sack a chronic cough and he would cough pretty much every filled with fluid, in his nose. Confused, Carol took Brandon night. I would hear him coughing.” From there, Carol’s to the doctors, who were completely dumbfounded. They life changed drastically. It was hard for her to emotionally were referred to another doctor, who suggested it could handle the fact that Michael had a chronic illness that be Cystic Fibrosis. Carol had all four of her children tested could not be cured. She had to learn how to adjust to this and it was determined that Brandon and Michael had CF. new way of life, getting her sons medicine, and helping Even before Brandon discovered the nasal polyp, Michael them stay healthy. On top of that, the financial costs were was already showing signs of Cystic Fibrosis. Carol recalls: high. According to The National Center for Biotechnology “When Michael was a baby, he ended up with bronchiolitis Information, the average cost for treating Cystic Fibrosis a couple of times which is an inflammation of his bronchial is $15,571. For Carol, she says that, “for our circumstance, tubes. Now knowing that he has CF, it was probably one the cost of having a child with Cystic Fibrosis is about of the reasons why he ended up with bronchiolitis … He $2,000 a year I would say on average. We are lucky though continued to have coughs when he would get colds when because we have very good health insurance. If we did not

16

he was in elementary school. His cough got worse when he have good health insurance, we would end up spending so much money on Cystic Fibrosis.” $2,000 is much cheaper

than $15,000. However, both amounts are still a lot of are showing great promise in helping people with Cystic money and would be steep costs for anyone to adjust to. Fibrosis. Hopefully by the year 2020, they will approve Although the diagnosis has brought about these issues these two medications. That might help Michael a lot with and many more, some good has come out of it. Carol says, his Cystic Fibrosis and maybe help his lungs out a lot too. I “I do have to say that I think with Michael having Cystic know that there’s also being some research done right now Fibrosis that it’s made our family stronger because we have on animals that would actually change the mutation that to help each other more and help each other get through causes Cystic Fibrosis so that these animals no longer have the difficult times which Cystic Fibrosis is notorious for Cystic Fibrosis. I heard it’s working well on the animals, but bringing a lot of difficult times.” She also mentions another I also heard that it’s very difficult to get these medications aspect that has strengthened her relationship with her to work in humans. I’m hoping, though, that the scientists son. She expounds: “As a parent, you usually want your will be able to figure out how to get these medications to children to be just the best at everything they can be and work in humans because then, they can actually change you’re concerned about just how well they’re doing in all the mutation that causes Cystic Fibrosis and make it into a aspects of their life and now it’s focused a lot on his health, healthy gene which means that Michael would no longer and I want to instead be more focused on how happy he have Cystic Fibrosis.” Cystic Fibrosis affects the family of is rather than oh, is he going to get into the best college or patients financially and emotionally. However, it also has things like that or is he the best at this sport. I don’t really the power to strengthen relationships. Not only that, but care about that anymore. I just want him to be healthy there is hope for the future of medications. and happy.” Not only can family bonds be strengthened, but also the potential relief from the sickness itself. When Carol was asked whether she was hopeful about future CF research she said: “I am very hopeful for the future of CF medications. Right now they have two drugs that they are in trial phase 3, that’s the last stage of the trials before patients can start getting the medications, and they

Carol Johnson 17

“I think that looking back on Cystic Fibrosis one hundred years from now , they ’ ll be like , people used to have this problem , and it ’ s kind of like we don ’ t have this anymore .”

- Michael Johnson 18

3 - The Patient

S

o far, we have seen how both CF doctors and parents

of CF patients feel concerning the disease. That raises

the question, what is it like as a patient? As mentioned before, Michael Johnson is the son of Carol Johnson and has Cystic Fibrosis. While Carol had emotional changes in her life after the diagnosis, Michael had physical changes in addition to the emotional changes as a result of being a

CF salt solutions

CF patient. As mentioned earlier, Michael has to undergo had to take different antibiotics and different side effects a treatment involving the vest machine everyday to keep of those medications have been very very severe and the mucus loose in his lungs. He says: “The idea is that then I have to take pills whenever I eat too and I can’t it loosens up the mucus that’s in my lungs and gets it so just eat whatever I want to eat whenever I want to eat.” it moves upward and keeps infection from staying in my Michael’s vest is like his life with CF. He is limited and lungs because with Cystic Fibrosis, the mucus is very, strapped down, but continues the treatment because he very thick and that’s basically the main problem with has no other choice. The struggles of dealing with the everything is that the mucus is too thick and so it makes a treatments for CF are universal for all patients suffering perfect bacterial breeding ground in my lungs. So on a daily from the disease. A CF patient named Kate Smith said that basis my life changed from the moment I was diagnosed the hardest thing about having CF is the exhaustion of because I had to start doing these lungs treatments all “rigorous exercise and physiotherapy.” Michael was asked the time which I hadn’t been doing. I had to make sure whether he would agree with this statement. He said: “The I exercised all of the time to keep my lung function up hardest part about having Cystic Fibrosis I think really too and those were like the main immediate things that depends on the day because sometimes I’m like, o.k., this changed. Since then a lot of things have changed so I’ve isn’t bad. I can do this. But then other times I think a lot of 19

times it is the fatigue and just kind of feeling not well all those struggles seem unbearable. Despite the hardships the time. That wears on you long term, you know wears Michael has had when dealing with CF, he says: “I am on your mind. You’re like, man, I thought this was going hopeful for the new medications that are coming out with to get better. But then if it doesn’t… that is hard.” All of Cystic Fibrosis and I anticipate that I’ll actually be able to these are common struggles and obstacles that CF patients use some in my lifetime, and I won’t have to do all of this must handle. Treatments are the only way to get better, stuff every day and maybe I won’t have to take the pills so dealing with the side-effects is a daily occurrence. The when I eat and maybe I’ll just feel well. I’m very hopeful National Center for Biotechnology Information lists a very overall and I think that looking back on Cystic Fibrosis uncommon way of treatment, however. What is this form one hundred years from now, they’ll be like, people used of treatment? Acupuncture. The idea is that it reduces pain to have this problem, and it’s kind of like we don’t have in patients and makes them feel stronger and healthier. this anymore. So I think that for especially my kids and When Michael was asked whether he would take this posterity down the line they won’t have to deal with this.” unconventional route, he said: “I’m totally up for trying new or alternative methods because I realize that some things will work for some people and other things won’t.

Just because the Westernized community which very much values procedures that work for everyone like when their doing the drugs they want to make sure that it works for everyone. I think some of the alternative methods may work for some bodies and some people and won’t work for other people which is why it’s not widely accepted. I’m willing to try different things if it means feeling better and getting better.” Whether it is these alternative treatments or common, standardized ones, treatments are a common struggle that patients like Michael deal with, even if at times 20

Michael Johnson

Conclusion U ltimately, Cystic Fibrosis is important not to just people like Michael, Carol, or Mary Helmers, but to everyone else as well. Thousands of people are affected by this disease. Families must try to pay for the cost that Cystic Fibrosis brings.

They also carry the emotional burdens of having a child with Cystic Fibrosis. If everyone is made aware of this issue, we can make a difference and help families that have numerous financial and emotional burdens. We can provide money that is utilized to research Cystic Fibrosis so doctors can find more effective treatments, and one day potentially even a cure. If this cure can be discovered, that means seventy thousand patients will be able to breathe normally again. Seventy thousand patients will be able to eat what they want, when they want.

Seventy thousand patients will not need to worry about constantly being sick.

Seventy thousand patients could pursue any career without worrying about their Cystic Fibrosis getting in the way. Seventy thousand

families will not have to pour their earnings into their Cystic Fibrosis patients. Seventy thousand doctors would not have to decide whether a patient needs a new set of lungs. All of the money poured into Cystic Fibrosis research could be used in other medical, or even governmental capacities.

Knowledge is power, and if everyone is aware of this issue and how solving it could help not just the patients, families, and doctors, but all people, we can work towards a better world. We can use funding for other projects and for other uses. We could have a happier, healthier world.

Part of a CF nebulizer 21

Nebulizers that help with CF

WORKS CITED

“About Cystic Fibrosis.” Cystic Fibrosis Foundation, www.cff.org/What-is-CF/About-Cystic-Fibrosis/.

Stendahl, Max. “Vertex Unveils Promising Data on New Cystic Fibrosis Drugs as Sales Top $2B.” Boston Business Journal, 31 Jan. 2018, www.bizjournals.com/boston/news/2018/01/31/vertex-unveils-promising-data-on-new-cystic. html.

Melão, Alice. “Vertex’s VX-659 Triple Combo To Be Tested in Phase 3 Trial in Patients with 2 Copies of F508del Mutation.” Cystic Fibrosis News Today, 2 Mar. 2018, cysticfibrosisnewstoday.com/2018/03/02/vx-659-triple-combo-inphase-3-trial-for-cf-patients-with-two-f508del-mutations/.

Melão, Alice. “Verona Therapy Improves CF Patients’ Lung Function, Phase 2a Trial Shows.” Cystic Fibrosis News Today, 7 Mar. 2018, cysticfibrosisnewstoday.com/2018/03/07/phase-2a-cystic-fibrosis-trial-rpl554-improves-lung-function/.

Van, K, et al. “Understanding the Costs of Care for Cystic Fibrosis: an Analysis by Age and Health State.” Value in Health : the Journal of the International Society for Pharmacoeconomics and Outcomes Research., U.S. National Library of Medicine, www.ncbi.nlm.nih.gov/pubmed/23538187.

Lin, Y C, et al. “Acupuncture Pain Management for Patients with Cystic Fibrosis: a Pilot Study.” The National Center for Biotechnology Information, U.S. National Library of Medicine, www.ncbi.nlm.nih.gov/pubmed/15844843.

Smith, Kate. “Living with Cystic Fibrosis: My Life Expectancy Is 31. I’m 29. So That’s Two Years of Parties, Extreme Sports and Romance...” Daily Mail Online, Associated Newspapers, 23 Feb. 2008, www.dailymail.co.uk/femail/article-517754/ Living-cystic-fibrosis-My-life-expectancy-31-Im-29-So-thats-years-parties-extreme-sports-romance-.html.

Johnson, Michael. Personal Interview. 5 March 2018.

Johnson, Carol. Personal Interview. 15 March 2018.

Helmers, Mary. Personal Interview. 19 March 2018.

Johnson, Carol. Personal Interview. 29 March 2018.

23

About the Author B

randon Johnson is a student film-maker who resides in Los Altos Hills, California. As a result of having Cystic Fibrosis his whole life, Brandon thought the perfect topic for this documentary project would be on his brother

who has the disease as well. Outside of filmmaking, he enjoys eating, watching television, and watching movies.

Also, he is currently trying to make his room into the ultimate “Star Wars” room. It’s clear from this statement that Brandon is a huge fan of “Star Wars” and is extremely excited for the next film to come out. He loves theme parks like Disneyland, California Adventure, Universal Studios, and Great America as well. Brandon is also a big dog lover and has a Miniature Dachshund named Titan. Brandon hopes to watch the Nathan’s Famous Hot Dog Eating Contest in person and go to Walt Disney World, as a result of his Disneyland obsession. Brandon’s biggest hope is that he will be able to attend college after he graduates from high school. His dreams for the future are making a “Star Wars” film and making a musical film that is set in Disneyland. Brandon hopes that Cystic Fibrosis patients

24

who read this book will feel hopeful and inspired to keep on making great strides.

I’M SMOKING, BUT IT’S HELPING MY LUNGS

Brandon Johnson