Buzzfeed_Titler

Not OP, but my SO has Cystic Fibrosis so is usually in hospital for a few weeks at a time a few times a year.

The biggest thing nurses can do is LISTEN and treat the patient like a human. Especially if you're in for a long stay, being treated like "just another case" really takes a toll on the mind. Doctors also mess up orders way too often, so if a patient is telling you it's wrong there's a very good chance it is.

Edit: Also please don't just scan-read patient precautions. The amount of times we have had to block staff from entering the room because they aren't following contact procedures is stunning.

I read through your blog a little, interesting thoughts on Orkambi. My girlfriend has been on it for about 9 months now, and she had a lot of the same symptoms to start with (tiredness, harder coughing, she had to take a hospital stay to get over the transition onto it). A few months later, once a lot of old mucus had shifted out, she actually started feeling a lot better. It's honestly been a life changing thing for her, to the point where it's honestly now more mental than physical factors stopping her doing something. It's a amazing to watch her rediscover that she can do things almost like a normal person, all thanks to four pills a day. Just wanted to share this in a relevant discussion!

I suppose there has to be a question here. Since I'm a Brit, what do you think of the Orkambi situation in the UK? If you haven't heard, it's been provisionally denied on cost grounds, and the UK CF community is in uproar.

Oh yes, it's so great to see her so happy at being able to do things like ride a bike again, walk more than a few minutes without a fit, or even just not be tired all the time from treatments and coughing!

It seems like the main problem is that it works amazingly for some people, but not at all for others and there's no in-between. So it averages out at only a mild benefit, despite the fact that it literally saves lives. Crazy! I'm glad we don't have that problem on the diabetic side (I'm Type 1, we make quite the disease pair), at least for the essential stuff. There's issues there too, but it's not like we're being denied insulin! I think Orkambi treatment from diagnosis is the way to go with CF, to keep lungs as close to normal function as possible.

From what you wrote about it, it seems like you were on the mild dose in the trial, since you got similar symptoms to my girlfriend when she had to drop her dose for a month due to insurance issues. Would you consider trying it again in future with the full dose?

Her function was right down in the low 30s if I remember right, it's raised to low 40s now. It's worth bearing in mind that she's a "stubborn CFer" so she was still getting around quite well with it in the 30s, despite the fact that many people are bedbound at that point. Even the 40s isn't that high a number, but that's more due to scar tissue in the lungs than anything else, and the day-to-day quality of life difference is massive.

As with all drugs, they affect people differently. her doctors were actually nervous about giving it to her because of her low lung function, and I think if it were any lower she'd have had to come off it. Luckily she toughed her way through it! There's definitely a big adjustment period, it took at least two to three months for her lungs to clear out the old mucus, and even now she's only just starting to heal up some damage to the lungs themselves.

I will of course remind you that I'm not a doctor or even the CFer herself, so this is just a close outsider's take on the issue. It seems to be a crazy improvement, but only if your body can actually handle it in the beginning.

Not the person you replied to, but SO many people cannot compute what it's like to have even a mild chronic illness, let alone something more serious like yourself. I'm Type 1 Diabetic, live a relatively normal life, would even say I'm healthier than most, but it's a constant struggle that no one understands because they can't see past the "normal-looking" surface or fathom what it's like to know that you won't ever "get better."

It's infuriating, it sucks and I wish it were different. All we can do is rise to the challenge and prove that we can be more than what they think of us.