Life’s a peach and then you…well everybody dies but we try to prevent the inevitable for as long as possible.
Lorcán, like his mother, loves his food. So often when we think about food we’re not thinking about the good that it does for our bodies. Rather we’re thinking about what it’s going to look like when it’s sitting on our hips, or tum, or double chin! I know I do and have done, and with my wedding coming up it’s on my mind more than usual.
But what if your body wasn’t able to digest fat? I’ve done every crash diet under the sun (oh god, flashbacks…the egg diet, don’t do it folks…the smell alone), I used to think that not being able to digest fat would be a dream come true, all the chocolate you want! Now I know that it’s really, really not.
Many people with CF have pancreatic insufficiency. They still make the enzymes needed to digest food that we all make, but the mucus caused by the faulty gene means that the enzymes can’t get out of the pancreas to where they need to go. Because of this a lot of people with CF need to take enzymes every time they eat food that contains fat and/or protein. Every single time, just imagine that. Most of the people with Lorcán’s mutations (double DF508, the most common mutation- affecting around 40% of people with CF) will need to take these enzymes and they’ll still need to eat a diet high in fat and protein.
The enzymes they take do a really good job but because fat is so crucial (embrace it! Love it! Easier said than done!) people with CF can still end up underweight and with deficiencies in vitamins and minerals (lots of which are fat soluble) as well as many other issues like osteoporosis.
The first precision drug out there (and available now), that deals with the problem rather than just the symptoms of CF is called Kalydeco (Ivacaftor). It works on mutations that affect around 4-5% of people with CF. It works incredibly well, so much so that sometimes people don’t need to even take enzymes anymore. Orkambi (Lumacaftor/Ivacaftor) doesn’t work quite as well but it works enough so that people can begin to absorb more of that fat (love it people!) and protein. As lung function is linked to BMI this is a huge deal. Many people taking Orkambi have reported an almost immediate weight gain.
Orkambi isn’t perfect but it’s still really, really good. New combinations of drugs that correct the problems caused by Lorcán’s mutations are being developed and trialled, and this will happen for the other mutations too. This is all really fab and I’ve got great big piles of hope about it that I carry around in my pockets and plait through my hair.
But right now there’s Orkambi.
Right now people are dying.
We need Orkambi right now.
Click on the link to see what you can do to help make this happen:
Life’s a peach…so go out and eat it.