After IVs in August, October, January and March, Isaac has had the longest gap between courses in a year. I think it’s been ten whole weeks - which we are bloody thankful for. I’ve said before, the hope is to have planned IVs four times a year, but the last year has been difficult - hopefully this longer stretch is a sign of things to come. We had hoped to eek out this good run, so IVs were closer to our summer holiday, meaning we could be more confident of him being well while we are away, but as always, if he needs it, bring it on!
Most of his annual review test results are in now. Concerns over CFRD (CF related diabetes), which he will have a repeat test for once he is well enough, and liver disease (again, CF related). But the bloods were mostly reassuring, infection markers down from over a thousand when we were last in, to under 200. No sign of the NTM since the end of last year, but chronic Pseaudomonas infection repeatedly show heavy growth.
Tomorrow I had hoped to join the protest for access to the new CF drug Orkambi, for those people it works for, this can be a breakthrough treatment. Vertex (the pharma) and NHS England need to make negotiations their priority, and end this! It’s been almost a year since I last attended a protest for this drug in London, and although talks have moved forward, this drug is still not freely available to those who so badly need it. Read more here. However, this also clashes with wear yellow day to mark the end of national CF week, so instead I’m running a cake sale at school, following a non-school uniform day, raising money for the CF Trust. Although we’re always doing some kind of fundraising, we’ve never done any through school, as Isaac doesn’t like the attention. But now he’s at Secondary school, he was happy for us to go ahead at the girls Primary school - Anouk and Rosa are so excited.
I also attended an information evening for CF, run by the Trust this week. Here are some of the highlights:
- Cambridge is home to the first CF innovation hub. This will soon be on site at Addenbrookes, when the building is complete, and so we hope, gives us an increased chance of access to cutting edge clinical trials.
- The hub is focusing on infection and inflammation, the two biggest problems for lung health in CF. As well as drug discovery, they are looking at using smart tech in CF care, and regenerative therapies (its long been thought that once the lungs are damaged, there is little you can do to bring back that lung function, but stem cell regeneration may change that) and precision drugs.
- Our own Consultant presented a new iPhone app, that we have been involved in trials for, which gives kids a really cool game which responds to their breathing during physio. It can show not only how many breaths they have done, but how deeply, how long, and gives them an incentive to complete the physio (physio can take Isaac up to an hour every morning and every night, mostly due to chronic procrastination). The app even allows kids to play online against other kids with CF (who they could never meet, due to cross infection) which adds a social interaction they may benefit from. The app looks great, and would be especially good for younger kids in creating good habits, and in turn, help prevent much head banging for parents.
- The NHS is in a staffing crisis; in some areas up to one third of positions are not filled. With the fast growing CF population (CF used to be a disease of childhood only, but now, just over half of those with CF are now adults) this is a massive concern.
- Dr Charlie Howarth presented the new Papworth hospital building, now on site at Addenbrookes in Cambridge. The old Papworth hospital has a Cf population which has grown from 130 to 330 in 15 years. The new building offers many ensuite clinic rooms, theatres, nuclear medicine, 42 critical care beds, 25 room dedicated CF unit, all with ensuite, fridge and exercise machine, lifts which are segregated by what bugs you are infected with, high tech UV cleaning (kills all the bacteria in a room in 15 minutes), and 15 air changes per hour - all of which are designed to limit cross infection risks. Amazing!
There was more too, but I have cakes to bake and kids to cuddle.
Have a great day x
Late walks by the lake, in the lake and summer fun.
