As a kid, we had a tiny black and white TV, like most families. I recall my Dad watching the snooker on it, but seemingly knowing the colours of the balls, to my amazement. Later, I inherited this TV. I moved out with it at 17 to my first bed-sit (a room in a crumbling, mice filled house in Cambridge). By then, to watch it at all, you needed to turn the dial ten minutes before, for it to warm up before it worked. I loved that TV and had it for years, while everyone else started on flat screens, I was still square.
Soon after, I got my first (very sexy fold out) mobile phone, and the World Wide Web and email became a thing. Since then, technology and change became synonymous to me.
As an asthmatic kid, I remember riding my bike up the hill to the doctors in the next village while having a chest infection. In addition to antibiotics (that they dished out like smarties in those days) they sent me home with a big old nebuliser to help get my asthma under control. I was thankful it was downhill on the way home as the thing weighed a fricken tonne, balanced in my wicker bike basket. Nebulisers vaporise drugs into smaller particles more easily absorbed in the lungs than dry inhalers, making treatment more effective. I had to ride the thing back a few weeks later for the next patient that needed it. Re-use was the norm.
Years later, I had a son with CF, and by 18 months old, despite daily oral antibiotics, a super nasty bug had moved into his lungs and set up camp. Since then, he has never rid the Pseudomonas nor the need for daily nebulisers to help keep it (somewhat) at bay. We came home with the same big nebuliser that I had known as a kid, but instead of asthma relieving meds, this delivered life-saving antibiotics. It came with elephant like trunking that we were to hang out the window so that vapours would be limited in the air that the rest of us would breathe in the house. Especially important when I was later pregnant with the girls.
Image: Little Isaac with long line, reaching from his ankle to groin.
Nebulising colomycin in those days meant 30 minutes or more of trying to keep Isaac sat down long enough to breathe in the drugs, Thomas the Tank Engine proved key to our success; full volume to help drown out the noise of the machine long enough for him to inhale enough medicine. Twice a day. Sometimes he cried. Sometimes he fell asleep. Sometimes we could make it a game. Sometimes we cried. Later, as more advances in treatment came along (drugs to thin his mucous, drugs to help draw water into his lungs) his daily nebulisers became two, three, four, five and then six times a day. This meant hours of mask time, with a super active kid who just wanted - quite rightly, to go out and play.
Then came the e-Flow, a much quicker nebuliser, but still tied to the mains. But soon after that came the gold that was - the iNeb. Silent, portable, small. Not only breath activated, but breath activated in the sense that it releases the drug only at the moment when the airways are at their most open, making it super effective. Each dose could be done in a matter of minutes (depending on his concentration, which varied massively). No longer a mask, but a mouth piece, meaning he could stop and start as he wanted.
The iNeb was a game changer. His/our quality of life changed beyond words. He could do treatments in the car. He could do them in bed. As long as we could sterilise the parts, he could do them camping or on the moon. All with a handheld device. The iNeb costs somewhere in the region of £3K. In the UK, we were fortunate enough for this to be gratis on the basis of our doctors prescribing a brand of colomycin from the pharma that created the iNeb - essentially the same drug, but more expensive on the basis that you get the device for free, and treatment would be more effective because of the delivery into his airways.
This kind of technology needs to be made available worldwide for CF patients now.
Image: Little Ise self portrait.
In the pandemic, things advanced rapidly. Once reliant on going into hospital at least every 8 weeks (we average on 10 CF clinic visits per year, not counting admissions, ENT clinic, six-weekly port flushes and other tests) to test his lung function (the key measure of wellness in CF) - now we had a home LF testing kit which we could hook up to our iPhone and report in data directly to clinic as often as we liked. We also began virtual clinical reviews. When he is well, these are a blessing - it’s one less trip into the hospital, saves hours, and is just all round more enjoyable for him (clinic visits to see his whole multi disciplinary team typically last 4-6 hours). Virtual clinics also help limit his risk of acquiring other bugs in a hospital environment (that said, they are not always appropriate, and occasionally, we end up going in anyway; Describing the sound, frequency and depth of a rattling cough is not always easy, and not a replacement for a stethoscope or hands wrapped around his ribs). We also have new tech to measure his O2 saturation, his weight, and his exercise tolerance.
As a little one, physio was typically chest percussion - tapping on the chest walls repeatedly, and asking him to huff and cough at intervals to try and clear the sticky mucous. Advanced technology means we now have small handheld devices that use magnets to create a fluttering force against his breath that help shake his lungs. In the UK, we do not typically use ‘the Vest’ often used in the US, as they say the clinical data shows no more positive effects over more manual forms we have here.
Image: Little Anouk with her recycled CF meds monsters.
In terms of hardware, by far our biggest advance was having his portacath inserted when he was thirteen. Before this, a huge part of CF life (heartbreak and trauma) revolved around gaining venous access to deliver the much needed antibiotics directly into his bloodstream. I cannot even try to guess how many needles, cannulas, long lines and PICC lines he had inserted over the years before his portacath. The scars on his ankles, hands, wrists and arms are reminders. I can say with horrible certainty that the failed attempts to get a line in sadly outnumber the successes. His port is now six years old and still going strong (they typically last 5-10 years, after which he will need surgery for a new one, which will need to be on the other side of his chest). His venous access when he was in ICU forced the portacath decision forward, one he had put off before, but now we could not be more thankful. Although it needs regular access and flushing even when he is well, it has no doubt reduced his needle stabbings a hundred fold.
One of the research trials I am aware of, that could potentially make a huge difference to the quality of life for people with CF is around tech enabled infection detection that we could use at home (for now, we send in sputum to the lab and wait for it to grow on a Petri dish, which delays treatment by at least 5-7 days for most bacteria, longer for others). A simple breath test that could signal infection and strain. How amazing is that?
I’ve not even mentioned the advances in testing, CT, MRI, blood screens that we have no doubt benefited from in his 18 years. What might the increased use of data and AI mean for us? Huge change for sure, this is powerful stuff. And all these hopes, when you’re waiting for a cure? Mean a lot.
Today has been a great day. All three kids in the garden tonight, playing some kind of football/basket ball mash up…. giggles galore…. the most beautiful music to my ears. For now, Isaac is stable. Going downhill for sure, but tentatively confident we can put off his next IVs until after his upcoming holiday in France with family friends. He is a hero among scientific heroism x
