He will awake (late).... groggy. His sinus infections (for which he has already had surgery for twice) can fill his head with a pressure that never leaves him. Just getting out of bed begins to shift the sticky mucous in his chest, and the coughing begins.
With the coughing fits come retching, and often, some pretty spectacular vomiting. The mucous he coughs up may be blood stained (from inflammation in his poorly lungs).
His digestive system (also clogged with mucous and reliant on synthetic enzymes to help break down all fats and proteins) causes more pain, nausea. Breakfast may be a delightful cocktail of anti-nausea drugs and pain killers (if he can keep them down). Curling up in a ball helps until it eases, but treatments need to be done. Many toilet visits.
Walking, talking, laughing, anything can bring on the coughing fits. Despite the nausea, he must swallow down the dozens of pills he needs, many of which are simply drugs to offset the side effects of other drugs he takes. Inhalers and nasal sprays are taken.
Then nebulisers; the first is hypertonic saline, the ‘salty neb’ - this helps draw water into his lungs to loosen the mucous (and causes yet more coughing). This is interspersed with breathing techniques through a physio device (to help him shift the mucous and cough it up). Can you imagine coughing tons, but having to do treatments that actively bring about more coughing? When your head, throat and ribs ache already? Rounds of deep breaths and huffs, followed by a ton of coughing (and more retching, and possibly more throwing up). Sputum sometimes patterned with thick plugs of mucous. The darker the mucous; the more evil the infection. But better out than in.
After that, he needs to rain antibiotics on his now clearer lungs, with a second nebuliser. Trying his best to breathe this down into the deep crevices of his poor angry lungs. A third nebuliser is an enzyme that works to break down and thin the mucous over the day, making it easier to clear later. This will all take an hour or more, and it might be hours before he can eat anything.
During the day, exercise will bring on more coughing, and fatigue may mean he won’t want to, but exercise is so important, to help keep his lungs clear, that he must try. As the day goes on, the cough will likely settle some. With any luck, he may manage a chuckle without fear of it bringing on another coughing fit. The audible sound from his chest is like breathing through treacle, it rattles, crackles. He will feel exhausted all day; breathing like this consumes a huge amount of energy, and his weight plummets with each exacerbation.
The day may also be filled with IV antibiotics. In his case, roughly every 8-12 weeks; Ceftazadine and Tobi, with a side of NAC to help protect his poor and battered liver and kidneys. And dozens of Creon capsules with everything he eats.
Come evening, he needs to repeat the same morning routine; physio, nebs, meds. 365 days a year. There is no holiday from CF. And the nights can be the worst, endless coughing, needing to do physio in the night to help clear his lungs. Exhausted is not a strong enough word for this. We become wordless, numbly doing anything we can to help him.
When we can, we manage this all at home, rather than in hospital (I am trained to give him IV drugs myself, not all families are able). Some years are worse than others, with many admissions. Some admissions requiring oxygen supplementation, surgery, HDU, ICU. And there is never a time that we are not awaiting test results of some kind; lung function, sputum results from the lab, chest X-rays, CT or MRI scans, blood sugar tests, ultrasounds, bloods for infection markers, liver and kidney health, care for his surgically implanted intravenous device in his chest (portacath). Appointments not only with his core CF team, but with ENT, Gastro and Immunology...
What I describe above is what a typical ‘CF sucks day’ can be like for Isaac. We are very, very fortunate that inbetween exacerbations, he is currently able to bounce back to a level of lung function that means he can still play football, gain back the weight, run and swim, so it is not always like this, for us. The relentless treatment regime keeps him well enough that he can lead a near normal life, but only until the infections become rife again in his lungs, that he becomes more symptomatic and we climb back on the IV train (for two to three weeks at a time), hoping each and everytime that we can manage this at home and not need to be admitted to hospital (more so now than ever). But also knowing that each and every exacerbation can chip away at his lung function that bit more, maybe permanently, and that is scary. Every new infection could be the one he doesn’t bounce back from.
For many others with CF, they get no bounce back, no respite. This is their everyday. Unable to walk up the stairs without O2, or laugh without the coughing taking over...
I am not telling you this for sympathy, I am writing about this in anger, as right now, there is a drug, manufactured and sitting in big pharma warehouses that is proving to be very, very effective at improving the health, lung function and quality of life for the 90% of people with CF that this will work for (not Isaac unfortunately, due to his rare mutation) and yet it is STILL not getting to those who so badly need it in the UK. Because of DELAYED DECISIONS, and ultimately, COST.
This drug is amazing, and the delay must end.
“Trikafta changed my life. Since I started taking it in November 2019 my Pulmonary Function Tests have improved by more than 40%. I can exercise at the gym 5 times weekly, do daily activities with more energy and efficiency than ever before, and I’m about to finish a university degree. I have hope for the future, for the first time I dream about what it’s like to live to be middle aged, I can breathe like I have never been able to in my life. Every cystic fibrosis patient deserves to know what it feels like to take a deep breath.”
To support the campaign to get this drug into the hands of people who need it NOW, please click
here. Alternatively, copy and edit the below text and send it to your local MP. Every day people are living through and dying of end-stage cystic fibrosis. I have no doubt that this drug will be approved, they will agree a deal, it will be licensed, it will get to patients, but every day they drag their heels, it saves money and lives are lost in the process.
Thanks so much for reading x
Letter for MP’s:
Dear X
I am a constituent of yours who is a parent of a child affected by cystic fibrosis (CF). I’m writing to share my experience of cystic fibrosis and the desperate need for urgent access to life-saving drugs. A new CF treatment, the triple combination therapy, could treat up to 90% of those living with the condition – including 40% who currently do not have the eligible mutations for the existing drugs, Orkambi and Kalydeco and Symkevi.
This drug, known as Trikafta in the US, has already been licensed by the Food and Drug Administration and shown to make a marked difference to the lives of those with CF.
Currently, the drug is being assessed by the European Medicines Agency (EMA), where it needs to receive a licence before it is appraised in the UK. The NICE appraisal process had begun alongside this, but disappointingly there has been a delay, and it has been pushed back to January 2021. Appraisal bodies in the devolved nations have yet to announce a date for their appraisal of the drug.
CF is a genetic life-threatening condition that affects over 10,500 people in the UK. The median age at death is just 31 years old.
My own son, Isaac, is unfortunately in the 10% of people with CF who these drugs will not work for, due to his rare, severe mutation, but these amazing new drugs give us much hope for a better future for him. We have already lost too many friends from this cruel disease, please support us in this fight to give him, and his friends a chance of a future.
The newer triple combination therapy has the potential to be even more effective for those who can’t benefit from existing drugs. While conventional CF treatments treat the symptoms of the condition, these new drugs target the underlying cause. It is not a cure, but it has been shown to significantly reduce decline in lung function – the leading cause of death for someone with CF.
The pandemic has caused disruption to usual CF care, whichrisks irreparable lung damage. This makes access to this drug even more important at this time.
We have already seen a lengthy campaign for Orkambi and Symkevi and we cannot afford to see this repeated when it comes to the triple therapy. The charity Cystic Fibrosis Trust is urgently calling on all parties to work together to make sure that the triple therapy is made available in the shortest possible time to those who need it, and I ask you to lend your support to this. For more information please contactpublicaffairsteam@cysticfibrosis.org.uk for a full briefing.
Yours sincerely, Dah dah dah...
