From The Chest

A month ago, Chris, Gary and I made a 6-part series of podcasts discussing day to day issues about Cystic Fibrosis and how it affects their lives. The success of the series has brought us together to make another series and make improvements to the format.

SERIES 1

The episodes from the first series covered the following:

Part 1 – LOCKDOWN
#1 Gary’s Hair
#2 Peter’s Messages
#3 Chris’s Gym
#4 The Pub
Part 2 – THE EFFECTS OF CF
#5 Are CF & man boobs related?
#6 CF Related Diabetes


Part 3 – LIFE WITH CREON
#7 Reverse the polarity!
#8 Changes in Creon dosage
#9 Creon and snacks


Part 4 – LIFE OF P.I.E.
#10 Panic pill popping
#11 Irritating inhalers
#12 Exhilarating exercise
Part 5 – CF MYTHS
#13 Fitness levels
#14 Educating CF
#15 Laughing at CF
Part 6 – THE MIND WARP
#16 Kaftrio & brain fog
#17 Less symptoms = more time to think
#18 Bo-Peep goes on strike!
#19 A few Dame Edna’s

SERIES 2

Part 7 – KAFTRIO, DISTANCING & ISOLATION
#20 “Are you alright?”
#21 Distancing
#22 Feeling Isolated
#23 Kaftrio – A New Life

With the second series of From The Chest nearing filming, here is an experimental opening title sequence, which although won’t be used in the new series, thought it be worth sharing with out viewers to show we are planning to film another series.

FROM THE CHEST – Experimental opening title

The idea behind this title sequence is we follow the Zoom video signal as it travels through space and bounces off satellites before reaching Earth giving previews of what is to come in the episode along its way.

Building all of the effects for the animation took 2 weeks to make.

The only reason why the sequence has been shelved is simply because it doesn’t quick suit the theme of the series.

It is more of a “CF World” themed sequence than a “From The Chest” theme. Not to worry as the work gone into the 3D graphics hasn’t gone to waste.

There are other projects this will be used in.

Cystic Fibrosis Awareness on YouTube

Over the past couple of months I’ve been having Zoom chats with fellow Cystic Fibrosis friends Chris and Gary. We have been talking about our day to day experiences living with Cystic Fibrosis.

Yesterday we launched a YouTube channel, FROM THE CHEST, where we will be posting 5 minute podcasts to raise awareness how we live with our Cystic Fibrosis and promote the importance of treatment, diet, fitness and sharing our day to day lives with Cystic Fibrosis.

The main theme with these podcasts is how diverse Cystic Fibrosis is by how it affects us differently.

Our content will bring smiles, laughter and tears as we share our moments.

#1 FROM THE CHEST – LOCKDOWN

The pilot episode of FROM THE CHEST – LOCKDOWN was filmed on Sunday, 9 May 2021 and episodes 2 and 3 are in the making.

We aim to fund raise with our YouTube channel for Cystic Fibrosis. However to do this, we need over 1000 subscribers.

The channel is in its infancy as it only went live on Wednesday, 12 May 2021 and we are busy getting more films ready for uploading.

We are in the planning stages to brand this and open an online shop where you can buy merchandise relating to our channel such as mugs, coasters, etc. Again money raised will be for Cystic Fibrosis.

Please help by subscribing to our channel and watch this space for more news. We have some challenges ahead.

Cystic Fibrosis, Lockdown & Doctor Who

Cystic Fibrosis & Lockdown/Shielding
Due to my Cystic Fibrosis I am classed as a Clinically Extremely Vulnerable person, which means during the pandemic brought on by Coronavirus aka COVID-19, I’ve had to go into shielding since the lockdown first occurred in March 2020.

During the lockdown and shielding, I turned to film making and explored what films I could make using the resources I have in green screen and the edit suite software such as After Effects and Premiere Pro. I’ve already made blogs about this at the time as linked below.

Week 1: The Muppet Show
Week 2: The Muppet Show
Week 3: The Muppet Show
Jim Henson tribute

Basic green screen set used for The Muppet Show

I learned a lot about working with green screen, building 3D CGI sets, animation and choreography, which as you can see in the final video below how these come together to make a film.

The Muppet Show
Filmed in April 2020 during lockdown 1

During the making of The Muppet Show, I realised the basic set used was too small for some of the puppets used, so built a much larger set in the spare bedroom, which has since been built to a much larger scale for the more recent Doctor Who film projects.

Inspiration of Doctor Who & Cystic Fibrosis storylines
In 1998 I wrote a story called Genesis of a Time Lord, which used Doctor Who to tell the story of what it is like to live with Cystic Fibrosis. In 2003 I took an extract from the novel and made a film. Here is a link to a blog about the making of the film and includes a download of the original novel.

The Screenplay of 2003

Considering the resources, edit suite and lack of actors, I feel I did a good job of the film, which sees Wythenshawe Hospital and the Cystic Fibrosis clinic used as a backdrop.  The receptionist, Pauline Jacklin, and Professor Webb in the film aren’t actors, but actually work in the Cystic Fibrosis unit and played themselves.  Professor Webb has looked after me since I was 16 years old and I’ve known Pauline Jacklin for at least 20 years.  Pauline since retired in December 2020.

Master of Deception came about as a suggestion by a fellow Cystic Fibrosis, friend Chris Strand, who played himself in the episode. He watched Genesis of a Time Lord and suggested I did a remake with the resources I now have.

Although I thought this was a brilliant idea, I knew keeping faithful to the novel version of Genesis of a Time Lord still wouldn’t be possible. Mainly due to the number of actors, costumes, props and sets required. The scale of the production and budget would be similar to the BBC.

However the idea behind Master of Deception was to use the Genesis film as an explanation why Earth is often invaded by Daleks, Cybermen and other Doctor Who aliens whilst telling the story of how Cystic Fibrosis life would have been affected had the Cystic Fibrosis gene had not been discovered in 1989.

How film making has helped with lockdown/shielding
Filming has helped keep my mind working. Plus there’s a lot of physical work involved too.  Making the console changed how I make films vastly and the episodes COVID, Master of Death and Planet Unknown were tests to see what I could and couldn’t do with the resources I now have. I’ve learned a lot from these and learned how to work with green screen and the console to give the films a professional finish.

Future Doctor Who episodes
I’ve written episodes 5, 6 and 7 with the script for episode 5 almost ready to start filming.

Episode 5, Master of Dimensions, will look at how the Cystic Fibrosis community interact with each other. In reality interactions usually take place on social media such as Facebook. In Doctor Who, the story looks at how the Cystic Fibrosis community interact with each other through dreams.

Episode 6, Evolution of the Silurians, will look at the basic science behind the making of vaccines and treatments, not just for CF, but for general illnesses. This episode will feature a new look Silurian.

The Silurian design as seen in 1970 and 2010
The Silurian Hybrid

The costume I have (Silurian Hybrid) was designed and made by Robin, who is also writing the script. The Silurian Hybrid will feature in the episode and become a new companion for the Doctor.

Episode 7, title TBC, will explore a behind the scenes of how the TARDIS scenes are made, which sees the Doctor visiting Peter.  This will more or less be a documentary drama with some comical interactions between the Doctor and Peter.

Inspiration for the Doctor Who episodes
When writing and filming these recent Doctor Who episodes, fellow Doctor Who fan Robin, who lives in the Isle of Man, has inspired how the history of Doctor Who can be used to make new episodes whilst fellow CFer Chris, who lives in Manchester, has inspired how the workings of CF and related issues such as diabetes can be used to make CF-related storylines.

Doctor Who – Master of Deception

The moment a lot of people have been waiting for, an episode of Doctor Who that tells the true historic story about the discovery of the Cystic Fibrosis gene in 1989 and the revolutionary treatment made as a result, which includes the most recent miracle drug, Kaftrio, which is currently the closest to a cure for Cystic Fibrosis.

Doctor Who 4 – Master of Deception

Synopsis
Whilst the Doctor is in a video call with his recent travelling companion, Chris, who has Cystic Fibrosis, the video call is cut off when the console alarms with a time paradox alert. Not all is as it seems. When Chris later shares a friend’s home-made film with the Doctor, the film reveals why the Cystic Fibrosis community were targets of a deadly attack by the Doctor’s enemies.

Fun Facts

  • The video blog in the pre-title is real and was made by Chris Strand. His channel is ChrisVSCysticFibrosis
  • Robin Burchill reprises his role as the voice of the Master. His channel is RobinGBurchill
  • The working title for this was Doctor Who – M508D.
  • M508D is a common mutation of the Cystic Fibrosis gene.
  • As stated in the episode, the Cystic Fibrosis gene, known as CFTR, was discovered by Lap-Chee Tsui, which opened doors for better treatments that has improved the quality of life and life expectancy of Cystic Fibrosis people, which includes the most recent drug, Kaftrio.
  • Kaftrio is currently the closest to a cure for Cystic Fibrosis.
  • The film Chris shares with the Doctor is Genesis of a Time Lord, a film made 2003, which tells the story how Time Lords came up with Cystic Fibrosis as a myth that allowed Time Lord refugees to live safely on Earth when a Time War broke out on their home planet Gallifrey.

Genesis of a Time Lord can be watched in full here:

Genesis of a Time Lord

The full story of Genesis of a Time Lord can also be downloaded for reading here:

Doctor Who – M508D

During lockdown and shielding, I’ve been rather busy building a TARDIS set and making TARDISodes.

Episodes so far are:

  1. COVID
  2. Master of Death
  3. Planet Unknown
  4. M508D (working title) *

* This film is currently being written and will star Chris Strand as himself, who plays a vital role in this CF related storyline.

The episode will revisit the film, Genesis of a Time Lord, made in 2003 that starred Prof Webb and Pauline, who work at the Manchester Adult Cystic Fibrosis Centre, Wythenshawe Hospital.

Genesis of a Time Lord told the story of how Cystic Fibrosis was a cover to allow Time Lords to live on Earth during a Time War. However there’s more to this than meets the eye.

The Cystic Fibrosis community will be, I hope, totally amazed by this latest episode.

Add Cystic Fibrosis to the exempt from prescription charges list

Please click here to sign the petition

People with certain medical conditions are exempt from paying prescription charges. However, the list of exempt conditions does not include cystic fibrosis. This is because the exempt list was drawn up over 40 years ago, in 1968, when most children with cystic fibrosis did not live until adulthood.

Fortunately, the outlook for people with cystic fibrosis has improved markedly since then and most people with cystic fibrosis live until adulthood, although still with a reduced life expectancy compared with the population as a whole.

However, the list of exempt conditions has not been properly revised since 1968 and therefore does not take account of this.

Many people with cystic fibrosis have to pay for all of their prescriptions unless they develop diabetes, a complication of cystic fibrosis that affects over 30% of people with cystic fibrosis and which is included on the exempt list.

The increasing ageing cystic fibrosis population over the years is as a result of improved treatments. This strongly demonstrates the exempt list is over 40 years out of date and cystic fibrosis should now be included.

A time for the Cystic Fibrosis community to celebrate…

Today I’ve had news that NHS England has reached a deal with Orkambi manufacturers Vertex Pharmaceuticals after months of talks.
 
This was brought to my attention through the Cystic Fibrosis (CF) clinic I attend after it was announced on BBC News (see link).
 
https://www.bbc.co.uk/news/health-50144742
 
CF is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections.
 
The effects of CF reduce the quality of life of patients as they age and whilst over the years the quality of life and average life expectancy has improved due to advancing treatments, the research into new treatments will always be ongoing that will eventually see CF being cured.
 
Orkambi is currently the closest to a cure for CF. Though until now has been offered to patients on compassionate grounds. Either as a result of a drop in lung function or as a result of volunteering to take part in an Orkambi related drug study.
 
I’ve been taking Orkambi since January 2017 on compassionate grounds after taking part in a drug study.
 
Within a week commencing the drug, I found I was able to breathe easier and the first month saw a vast improvement with my endurance. Now my endurance is back to how it was 5 years ago.
 
You can say today’s news has been a huge welcome and something worth celebrating about.

Life with Cystic Fibrosis

Originally published on Tuesday, 28 February 2017

When I was young (I’m a 1980’s child), management of CF was primitive compared to how it is now.

Diet & Treatment
From what I remember, Creon wasn’t on the market until around 1985. However, there was another tablet that aided digestion, which wasn’t as powerful as Creon. As a result, fatty foods such as cake, crisps, biscuits, etc were foods to avoid in the CF diet. So the introduction of Creon came as a shock to me when I was allowed these types of food and although I am able to have these foods, I don’t really eat them due to not having much of a sweet tooth.

Over the years treatment has improved vastly, aiding a better quality of life and CF people living longer too.

Not only has treatment improved over the years, but also equipment used to administer them. With advances in technology, nebuliser equipment has become more compact and treatment taken through these have become quicker to take too.

Medical advances seem to be happening fast at the moment with all these promising drug trials.

OK not all trials work out as we hope, lessons from them are learnt for the next drug trial, so all is not lost.

Exercise
It was once believed exercise was bad for the lungs of CF patients based on the coughing fits caused as a result.

It was later realised coughing during exercise was down to the airways opening up as a result of deep breathes, which in turn improved the lung function.

Mix and match
Before segregation came into force in 1991, CF patients were allowed to mix no matter the strain or bugs they had. Little was known about cross-infection.

Looking at how segregation works now, people new to CF and use to the segregation rules will not believe that there was a time when CF patients went on coach trips together. One trip seeing the patients camping at Sherwood Forest, with 6 patients of different bugs and strains sharing a tent.

Lessons learnt about CF for new parents of children with CF
From my own experience, one thing I’ve said to parents new to CF is to encourage exercise with their CF child from an early age. Find something the child will enjoy and would take up as a hobby.

I am lucky to have a mild form of CF and have coped with it really well.

However, not all has been rosy with CF. There are times when I get upset, stressed or anxious about things. In particular when I’m not well.

However once treatment for a chest infection shows signs of working, the psychological strain this has on me is lifted.

CF will have a psychological effect on not just patients, but also their parents, siblings and partners. This side of CF happens to everyone at some level.

CF people do try to remain positive and tend to use a lot of humour as a coping mechanism, which for me works.

Every day will be a learning curve for anyone, whether it be for a CF person, relative or even the CF team at the specialist clinic.

One thing for sure, despite the effects CF can have, the treatment of today means we get to live a better quality of life and live longer and longer.

Two years ago a man well known at my hospital passed away at the age of 75, which for me is the oldest known patient.

If he can live so long especially when at the time he was born little was known about CF, then there is hope for us all.

Cystic Fibrosis, Orkambi & Warrington Borough Transport

Originally published on Monday, 20 February 2017

On my way home from Pure gym, I was delighted to see Warrington Borough Transport (WBT) display a poster raising awareness about Cystic Fibrosis (CF) on the bus stop on Manchester Road opposite the Esso garage.

Had I not been driving, or the roads were busy at the time of travelling, I would have pulled over to take a photo of this as it really made my day to see such publicity in aid of a life illness that I and many others suffer from.

I thought it would be nice to give WBT an insight into what I’ve been through with the new exciting breakthroughs in regards to better treatments for CF, and also update the Warrington Guardian with how things have been since I did the QBW251 trial in October 2015, a drug that was to be the closest to a cure for CF.

Although the outcome of the QBW251 drug trial concluded with the drug not being as effective as predicted in lab tests, there have been other similar medicines that have made it onto the market and used by CF people who meet criteria due to high costs for the drug.

One drug, which was on the news in recent months, is called Orkambi. This is a pink tablet whereby 2 are taken twice a day with food.

Orkambi is on the market in America but is yet to come on the market in the UK due to high costs whereby for 1 CF person, costs mount up to £100k per year.

However, all is not lost.

There have been continual drug trials happening over the last couple of years to find a drug that either work in the same way as Orkambi or will work alongside to make Orkambi more effective.

I am currently doing a drug trial whereby I have to take Orkambi, which is being tested alongside a trial drug that’s inhaled to see the effects these combined drugs have on CF.

At present, I am on week 4 of the trial whereby I am taking Orkambi before I start the trial drug next Tuesday (28 February 2017).

The drug trial program is as followed:

  • Month 1 – take Orkambi only
  • Month 2 – take Orkambi and trial drug
  • Month 3 – take Orkambi only
  • Month 4 – take Orkambi, trial drug and Hypertonic Saline (a nebulised salt solution that helps clear the lungs)

At the end of the trial, as a reward for my volunteered help, I will be allowed to continue Orkambi.

The question you’ve probably been asking throughout this email is, “Have I noticed any effects of Orkambi?”

The simple answer is, yes.

“What effects have I experienced?”

I’ve been able to breath better and when I cough, the feeling I have in my lungs is as though nothing is there.

My coughing that occurs in the morning after a nights sleep has reduced dramatically to almost non-existent.

When I’m at the gym I am able to work that little bit harder on the exercise bike and my heart rate at its peak has dropped from 150 beats per minute to 120.

I am yet to know how Orkambi has affected my lung function. At the beginning of the trial, my lung function was 44%, so it will be interesting to see how this changes over the coming months.

Had it not been for the likes of Warrington Borough Transport and many others who raise awareness of CF, the donations raised for the CF Trust would be almost non-existent and vital drug trials, an example described above, will not happen.

It may have been one poster on the side of a bus stop that I have seen on my travels through Warrington, but if I have spotted the poster, there’s a chance other commuters and bus users will have seen this too.

I am pretty sure if every single person donated £1 after seeing the poster, this would help fund further investigations into vital treatments leading to a possible cure for CF.

So thanks again Warrington Borough Transport for your contribution in raising awareness of CF, a life-threatening illness.