Before 10 years ago, 40-year-old Cystic Fibrosis Advocate Jillian McNulty generally had her cystic fibrosis in check… so much that coworkers at the radio station for whom she worked for 12 years, didn’t even know she had CF.
“I would have to explain sometimes because I look healthy and people think I’m grand. I look fine most days and people say things like ‘You run and walk; there’s nothing wrong with you.’ And then they get a big lecture from me.”
But then, something happened 10 years ago that would change her personal and professional trajectory, and the trajectory of CF in Ireland.
“My first hospital admission when I was 31. It was then that I had seen firsthand, the bad conditions for a patient with cystic fibrosis. To say it was culture shock is an understatement. There were five to six people in the same ward. Most were older patients with some type of dementia and didn’t really understand what was going on. Other patients had flus, colds, and pneumonia. Not to mention the smell of a six-bed ward was horrendous, I will never forget it. There were patients dying the room straight across from me and at night you couldn’t sleep because there were confused elderly patients shouting about.”
The horrors didn’t just stop at the overcrowding.
“At times I shared a shower and toilet with up to 40 other patients… it made me sicker every time I went into the hospital. It turned into this vicious cycle. I would sign myself out of the hospital and I’d have to go home. Then, I’d get sicker and have to go back.”
After her scarring experience in the hospital, Jillian knew she had to take action so that neither she nor anyone in the CF community had to endure the potential dangers of that situation again.
“Cystic fibrosis patients were in these conditions 30 to 40 years before me, so it was clear things had to change. So I came out with my CF on national radio to spread awareness around the conditions, and it all kicked off from there. I, along with tons of other people working for this cause, continued to campaign for private en-suite rooms. By 2012, there was a dedicated isolated single-care unit for CF patients. It was well worth all the fighting and hassle. Things are getting better in Ireland. It has changed. ”
Jillian then made fighting for the CF community her life’s mission. After helping in the campaign for Kalydeco, a CF drug for which she isn’t eligible, she found a new drug to be a CF campaign focus: Orkambi.
Orkambi has been approved by the United States FDA since July 2015. It is considered a breakthrough therapy for those suffering from cystic fibrosis. So why are Jillian and the Irish CF Community working so hard to get access to Orkambi if it’s already approved? Well the Irish government has to pay for CF treatment because they have a “free healthcare” system, with CF patients on priority. Simply put: Orkambi has been deemed too expensive for the Irish Government to invest in. But, just as Jillian campaigned for better hospital conditions based on her experiences there, she will do the same for Orkambi. Jillian is one of the few “fortunate” CF patients in Ireland to have access to the drug.
“At the beginning of 2012 I was training for a marathon and was at 59% lung function. Then, November of 2012 to July of 2013, over a period of seven to nine months, I had lost 20% of my lung function. I don’t know what happened! It was a very sharp decline in my health. I spent nine months in the hospital and was on six to seven weeks of IV antibiotoics. I was getting very sick, very quickly.
My research nurse was in the hospital with me and asked if I wanted to be a part of a clinical trial. I said ‘absolutely I do!’ So I was in touch with my research nurse the whole time I was hospitalized, to bug the hell out of her. I really wanted to get in the clinical trial, though she warned me that I may not pass the testing, because to be eligible, your lung function had to be at 40% to 90%. On the day I did the eligibility testing, my lung function was 41%. I literally scraped by and I got the call later that I was selected. Needless to say I was crying I was so happy. I went forward and started Orkambi August of 2013 and haven’t looked back since.”
So what exactly happened with Jillian and Orkambi to make her so passionate about it?
“Within a couple of days of the trial, I saw small changes. Usually, when I ran or sweated, salt crystals would form under my arms or on my face, and when I’d wipe my face I would expect a gritty texture under my hands. Well, I was out running and wiped my face and I was like ‘hang on a second, there’s no grit!’ And I know this sound weird, but I was just licking myself and I didn’t taste salty at all. So I told my mom and she licked me and she was like ‘Oh my gosh! You’re not salty!’ Additionally, my body temperature had changed.
But within six weeks of starting the trial, I had gotten quite sick and ended up in the hospital. We almost stopped participating because we just didn’t know if I was on the drug or the placebo. But once we got over the initial bump, the Orkambi was working deep down in my lungs, clearing the massive infection there. Before Orkambi, I would go on average three to four weeks out of hospital and then back in for six to seven weeks, most of the time. About five or six months after I started taking Orkambi, I started getting three months out of the hospital… and then six months out of hospital, then a full year out, with no infection, no hospitalization and my life completely changed.”
“Every time I talk about it, I feel like crying because even now it still seems like I can’t actually believe that it’s happened this quick. It’s changed my life SO much.”
And since Jillian has been on Orkambi, her lung function baseline is up nine percent. If Orkambi has done all of this for one patient, effectively giving her, her life back, why is not accessible for every eligible patient in Ireland?
As Jillian explains it, “It’s the cost of Orkambi. The government and health system is saying it’s too much. At first, Vertex went in with a bid at €159,000 per patient per year. But now it’s reduced. And our government still says there is no way they’re going pay that much. But take me for example: In the last three years, I have had maybe twelve weeks in the hospital. Without Orkambi, I would have been in for 25 months. So really, we saved them tons on hospital bills.”
While that original quote seems steep, the way Jillian frames it makes business sense to me. But as Jillian says, money isn’t always the point when dealing with human lives.
“It really shouldn’t come down to the price. It should also come down to quality of life for people with CF.”
Just hearing that gave me chills. All I can think about during this conversation with Jillian is how unfair it is that someone is born with CF at all, yet in a time where effective treatment is available, in a place where they don’t want to pay for it.
I’m speculating, but it seems that certain parties are deferring the decision to pay for or (not pay for) Orkambi because they know that human lives are at stake. It’s as if they don’t want to be a part of making a potentially controversial decision because it would inevitably be equated to the Bystander Effect. This diffusion of responsibility indicates that they believe their inaction isn’t as damning to patients as just blocking Orkambi access all together. And now, the decision lies in the House.
As Jillian explains, “So far, there have been two official no’s in January and June… and we are waiting now on the third one. They keep passing the buck, said ‘No we are not making the decision,’ and now it’s a political decision.”
When I started writing this article, I thought “I don’t want to piss anyone off, let alone Orkambi or the Irish Government.” But then again, maybe I should want to ruffle a few feathers to incite action. And despite what seems to be constant adversity, Jillian and many others in and out of the CF community are doing everything they can to push Orkambi’s availability in Ireland.
“Obviously I have been very involved in the campaign for Orkambi, and spoke in the House. I am also going to speak again before my next protest to try and get them on board. I organized a protest in December and we protested outside the government building. We had support from senators and TDs. I have also organized a second protest to put pressure on them and to let them know we aren’t going away.”
