#cystic

Uh

uhm

For two years, I have been severely limited by oxygen which I sometimes ‘purposely’ forget, so I can have some humanly mobility.

It amazes me that the worse your lungs digress, the heavier those tanks and machines become.

First I was prescribed a heavy tank on wheels, that I used until they brought over a block-like concentrator and a equally heavy condenser. Those two machines were my furnace during the whole day and was very noisy!

With these two machines, came two tanks that gave me 8 hours of O2 on 2lpm or 4 hours of O2 on 4lpm. This meant: no outdoor overnight camping, no long trips and no staying away from the house for over 7 hours...it wasn’t life! It was a restraint.

I begged them for another tank, and reluctantly they gave me one extra for a total of 12 hours. Those tanks held me over for short trips, but when my lungs slipped a little further down, my obligated 2 liters went up to 6 liters and I refused to use that much, so I leveled at 4 liters and 6 hours of oxygen.

I brought my case to my pulmonologist and she agreed that I needed a battery operated concentrator and I was given the Simply Go which was a 12 pound brick. And carrying the extra battery, cords and packaging...it was well over 20 pounds of weight on my back as I could not work or even do daily tasks without just leaving it somewhere on the deck as I jump roped with the hose...tangling up, knocking things over and being on a short 6ft leash.

The hours weren’t any better either! With two batteries at 4lpm, it only tasked me 4 hours. I could squeeze 8 hours out of 2 liters, but once more I was limited by the weight, size and time.

So when my family surprised me with the purchase (over 3500$) for the Inogen 5; I was skeptical.

When it arrived, it was only a small device, about the size of a cereal box, and weighing just under 5 pounds. It was remarkably light and it came with two extra batteries. A carry case and backpack.

I plugged in one of the large batteries and tested it out. One battery at 3lpm seemed to last me 11 hours. Two batteries, 22 hours...I still have yet used the smaller 5.5 hour battery that I call ‘The brick’ as they are about the size and weight of a brick.

I tested to see if I could use it around the house and found daily tasks like laundry, dishes, vacuuming and walking outside was tolerable. Only issue, I have no shoulders due to muscle wasting from CF, so it is a struggle with the strap and my bra straps...lol!

One feature it has is a loud warning alarm which first sounds at 3% battery (only once, which is a downside as I sometimes miss that first alarm) and a final steady beep at 1% for about 1 minute until the machine turns off. Another ‘neat’ feature is the breath detect light that would flash green each time I breathed.

One of the most beneficial features that is amazing is the battery hour counter. You can read the time you have left and that takes away the guessing game or playing ‘beat the clock’! I use it to see when I need to go fetch the other battery if I am going out on a walk or just lingering around.

I few downsides to the Inogen 5 model is that it only has one mode, pulse. The smaller Inogen 5 model has steady pulse in its functions, but the battery life isn’t any good. And considering I use pulse while awake, it works to it’s beneficial. It goes from 1-4lpm...so it covers my oxygen needs (however, 4lpm greatly reduces the battery life).

Now the real question...could it be possible? 🧜‍♀️

It seems a fun fantasy that would make a riveting novel! Even when I was young, I too thought I was born on the wrong side of the ocean as I seem to be drowning on air and breathing my own bodily fluids...and of course having scaly eczema does not help!

     As mentioned in my last post, cystic fibrosis is rarely featured in movies, TV shoes and literature that isn’t a medical school lesson. Some of the TV programs pull at the deepest emotions and some of the TV programs make you wonder what the writers were thinking. Some of these programs I’ve never seen and will not give comment about my viewpoint as a CF patient.

                                Foreverland | Film | 2011

     In this Canadian film, cynical 21-year-old cystic fibrosis patient, Will (Max Thieriot), has given up on trying to find a job or relationship because of his obsession with the idea that many with CF don’t live to their 30s. He goes on a road trip to Mexico and rediscovers his thirst for life in the process. The film uses salt as a thematic symbol — a clear connection to cystic fibrosis patients’ salty skin. It also shines a light on the daily life of a CFer: treatments, the feeling of ‘drowning’ in mucus, uncontrollable coughing fits, and an overprotective mom who worries about the sound of his ‘soupy cough.’

________________________________________________________________

                      Red Band Society | TV Show | 2014

     In this now-cancelled show, patients with various medical conditions have adventures in the ward of a fancy L.A. hospital called Ocean Park. A rebellious character named Dash Hosney (Bradley Brian, Jr.) has cystic fibrosis and is in need of a lung transplant. Despite being in end-stage disease, his first scene features him in a hazy closet sharing a marijuana joint with a friend without ever coughing. He also runs and skateboards easily without need for oxygen. Not all end-stage CF presents itself in the same way, but his high energy is a head scratcher at times. At other points, the disease does show itself, such as when he coughs up blood during an argument. I’ve watched this series and found that the writing for cystic fibrosis is terrible.

     For example; Dash was listed as a lung transplant patient which has to honor five requirements to qualify for a transplant: 65 or younger. (1)No smoking or substance abuse in 6 months. (2)BMI less then 30%. (3)No prednisone for 6 months. (4)Have a social support system. (5)FEV1 lower then 30% and/or on oxygen. The only one he passes was the BMI and no prednisone. His history of smoking marijuana and drinking alcohol violates number 1. We never see his parents or friends which violates number 4. His ability to be physically active without oxygen and his energy is confusing for a terminal patient needing a lung transplant. The only time we see some symptoms of CF is in the last two episodes when his condition matches his symptoms.

________________________________________________________________

                          Bates Motel | TV Show | 2013–2017

     A re-imagining of Alfred Hitchcock’s 1960 classic, Psycho, Bates Motel features a character with cystic fibrosis named Emma Decody. She’s 17 years old, on the waiting list for a lung transplant, and on supplemental oxygen. The show, taking place in present day, casually mentions her life expectancy is 27 years old, while the actual reported average life expectancy is 37. Despite her end-stage disease, Emma climbs up a mountain without much trouble then easily sprints through a forest without a single cough at the end of episode two. While hiding from the men chasing them, through the mountain, protagonist Norman whispers to Emma, “Don’t even breathe.” Then he remembers her cystic fibrosis: “Sorry.”

________________________________________________________________

                           Ghosts | Graphic Novel | 2016

     This story is about middle-schooler Catrina’s relationship with her little sister Maya, who has cystic fibrosis. The girls and their parents move to Northern California’s coast in hopes that the salty sea air will help Maya’s lungs. Maya has advanced disease and so the topic of death is thematically explored — Maya has an obsession with ghosts. Basic information about cystic fibrosis symptoms are nailed, as is the effect of salty ocean air on lungs and the common feelings of resentment the disease can produce.

________________________________________________________________

         No One Dies in the Garden of Syn | Book | 2016

     Synthis (Syn) Wade is a teenager with cystic fibrosis who is pushed into a pond that portals her to a new world where illness and death do not exist. Most of the book has Syn cured of her disease, but the basics of cystic fibrosis are still covered for background purposes. In the normal world, she has morning treatment routines, coughs because of thick mucus, and has very little energy. This is the first book in an ongoing trilogy, so there is room for the disease to be explored more in-depth.

________________________________________________________________

  Everyone Dies in the Garden of Syn | Book | 2018

     When Synthia (Syn) Wade discovered a secret world where illness and death did not exist, for the first time in her life she didn’t have to worry about her cystic fibrosis. However, a dark truth and a dangerous foe were waiting in the shadows.

     Syn must now return to the Garden to save a loved one held captive by a madman and the odds are against her. The secret world is overrun by the terrifying Creepers. Her one-time allies have turned their backs on her. And worst of all, the healing powers of the Garden are no more. This time Synthia fears fighting alone, without respite from her life-threatening illness. The last time Syn visited The Garden, death was held at bay. Now Everyone Dies in the Garden of Syn.

________________________________________________________________

                                            Teeth | Book | 2013

     A boy named Rudy is forced to move with his family to a remote island in an attempt to save his 5-year-old brother Dylan, who has cystic fibrosis. The fish at the island have magical healing properties if eaten, but Dylan must eat them for the rest of his life if he is to remain cured. Rudy enters a strange, violent friendship that forces him to choose between his happiness and his brother. The book describes common CF symptoms as well as its potential for lethality, though much of the story takes place with Dylan being cured of these symptoms because of the magical fish. It’s fantasy, if you haven’t guessed.

________________________________________________________________

                                         ER | TV Show| 1997

ER Episode (season 3, episode 14) Who Apply Now?

     Dr. Doug Ross treats Jad Houston (Chad Lindberg), a 17-year-old cystic fibrosis patient who wants to die, but isn't old enough to sign a DNR.

ER Episode (season 3, episode 16) Faith

      Jad Houston (Chad Lindberg) turns 18 and requests that Doug take him off the respirator which has been keeping him alive.

________________________________________________________________

                         Grey’s Anatomy | TV Show| 2011

(Season 7, Episode 16) Not Responsible

      Ricky was in the hospital for a lung transplant to treat his cystic fibrosis. When Altman found out that he was dating another person with cystic fibrosis, she said she wouldn't do the transplant unless they ended the relationship, so they agreed to break it off.

________________________________________________________________

                                      House | TV Show| 2009

Episodes #514 "The Greater Good"

________________________________________________________________

                                     NY Med | TV Show| 2012

Season 1 Episode 6 

     A woman with cystic fibrosis needs new lungs; an ER nurse makes a startling personal decision after hearing an elderly couple's story about their 65-year marriage; surgical residents share tales of hazing by senior surgeons.

________________________________________________________________

                                    Lifebreath | Movie | 1997

     Having atypical cystic fibrosis is a nightmare! With just plain CF, there is no indecision or second guesses. You have a dedicated care regiment, organized therapy and policies put into place to help you during times of flareups and infections. But with aCF, there is no help...no dedication or policies to keep you healthy and safe while in the hospital. This typically leads to a shortening of life expectancy for aCF patients; just because the rules have not changed.

     All my life, I was diagnosed with asthma. Whoever, when my asthma became unmanageable and I was not responding to typical treatments, a study was done that found out that I don’t have asthma. So you’d think they’d correct their diagnosis...

     Wrong.

     For years, physicians stood by and watched my health decline. They tried drug after drug, therapy after therapy with no resolve. When they ran out of ideas or caved in; it was your fault that they failed and either dismissed you from their care or just ignored you.

     I had one pulmonologist back in 2014 who loaded me up with drugs. His philosophy was that if he could not control the symptoms, then he’d just overdose your medication. At that time, I was on Advair, Qvar, Asmanex, Spiriva, Theocron, Prednisone Regiment, Breo, Incruse, Albuterol, DuoNeb, Singular.

     It wasn’t until 2016, when I got a new pulmonologist, did he realize that this might not be just asthma. With digestive complications, metabolic issues, lung disease and a long history; he agreed that the disease sounded like cystic fibrosis.

     Without the aid of a positive genetic test and sweat test; my access to many programs was nonexistent. To correct the weight loss and muscle loss we added creon to my regiment and that stopped the muscle loss and balanced my minerals and vitamins. With the constant lung infections, we decided to add CPT to remove the mucus and in a year, he cut infection rate in half. Pulmonozyme and hypertonic saline was added to the regiment.

     For the first time in my medical history, I was actually improving.

     When my healthcare was noticed by the CF Center at Multicare, they were very hesitant with my care as they did not know much about atypical cystic fibrosis; however, the symptoms I was having matched that of a CF patient and I was only responding to CF-style treatment. In November of 2018, I was suppose to go to the CF Center for a work-up, but it was delayed after hospitalization.

     I was told over-and-over by pulmonologist that I did not have CF, yet they could not say what was causing these symptoms. The hospital doctors said that everything looked like CF and when they tried to treat it only as asthma, my health rapidly failed. With all that I’ve been told, I expected to be notified that my new CF doctor was dropping me from his care.

     That call never came, however, only 24 hours before my CF clinic visit, they canceled it. However, it was canceled due to my hospitalization and rescheduled one month out.

     Is this typical of a CF center? Typically, a pulmonologist wants to see you after a hospitalization, so I was surprised to be delayed. I checked my diagnosis codes and still saw that I was covered by the CF Clinic and seen for non-classic cystic fibrosis. A day after my canceled appointment, there was an appointment for the CF Nutrition service scheduled.

     I decided that I was no longer being followed by the MultiCare CF Clinic and was glad that I did not drop my CHI Pulmonologist. About a week later, the MultiCare CF Clinic called to reschedule a new appointment that will be my first CF Clinical appointment. They asked me to mask up and expect a long meeting with the doctor, nutritionist, social work and nursing.

      Where exactly this is going? I don’t know!