I was recently asked to submit a patient impact statement to the Canadian Agency for Drugs and Technologies in Health (CADTH) for the Common Drug Review process for ORKAMBI, a drug that treats patients with the particular gene mutation of Cystic Fibrosis that I have. This drug has now been approved by Health Canada so the next step is waiting for the individual provincial coverage to be put in place as the drug is very expensive. I was excited when I was asked to be a part of this process, even if it was just in writing, so I thought I’d share what I wrote.
My name is Cassandra Grisdale. I am 22 years old and from Melfort, Saskatchewan. I was diagnosed with Cystic Fibrosis (CF) at 6 and a half months old. There is nothing in my life that goes untouched by this disease.
CF affects many parts of the physical body. It causes the lungs to produce thick mucus which becomes a breeding ground for bacteria that causes lung infections. This, in turn, diminishes lung function and causes patients to develop a persistent cough and shortness of breath. CF also affects the digestive system. My body cannot digest food properly and because of this, I only weighed 29 lbs in kindergarten.
At the age of 5, I underwent surgery to have a gastrostomy tube inserted into my stomach so that I could be tube fed overnight in the hopes that I would be able to gain weight. Thankfully, I started putting on weight immediately, but I had to deal with the fact that I was the only one of my friends and classmates that had a “button” sticking out of her belly. As an adult, there are certain types of clothing I cannot wear because the button would be visible to others. Additionally, I had problems with the sizing of the button for my adult stomach so it was quite embarrassing when the button would leak while I was hanging out with friends or at work because my shirt would become inexplicably wet. Recently, due to issues with acid reflux, I was switched to a new style of button that has to be changed in the radiology department at the hospital every 6 months. This has added yet another recurring medical appointment to an already long list of appointments that I have to schedule other things in my life around.
As a kid, I was hospitalized to receive intravenous antibiotics at least once a year. This continued until my early teens. I was usually hospitalized for a minimum of two weeks so I became quite familiar with the pediatric ward and the nurses who worked there. I spent several holidays in the hospital including Halloween when I was 7 and Christmas when I was 12.
Constant hospitalizations make it difficult to have a normal childhood. You learn things other children don’t have to. Like how to ignore the child crying constantly from the room down the hall, or how to stop the IV pump from beeping when the nurse hasn’t had the time to come and start your next dose of drugs yet. You are forced to grow up quickly so that you can keep up with the discussions the doctors are having with your parents about your health and so that you can have some control or say in what is being done to you. You learn how to question the doctors and negotiate your way out of needles and treatments from your hospital bed. Having CF also makes you tough. You learn to put up with pain when parents and doctors convince you that it’s for the good of your health. You have to learn how to fight a losing battle against the CF, a battle you wish you had more control of.
Growing up, I spent a lot of my life trying to show everyone that I was tough and that I could handle the CF because I didn’t want their worry or their pity. I have to live my life knowing that it’s, most likely, going to be shorter than yours. Shorter than my parents’ lives. Shorter than my younger brother’s life. No one should have to live like that.
Now that I’m an adult living with CF, the realities of the disease are catching up to me. My health is worse than it’s ever been before. I am being hospitalized more frequently and am sometimes treated with IV antibiotics at home with the help of a home care nurse. I haven’t been able to complete any sort of post-secondary education because my poor health keeps getting in the way. I’m having to wear oxygen more and more which is a struggle for me because I don’t want the people around me or the strangers I pass during the day looking at me as a sickly person. I’m not a sickly person. Hell, I went to Mexico the day after I finished a course of IV antibiotics!
However, not having enough breath to do the things I want to do on a daily basis is incredibly frustrating. I don’t want to have to drag oxygen with me everywhere I go. I want to have enough breath to run up the stairs again. Enough breath to hike down to the dock and go fishing with my dad. Enough breath to clean the house. I am a bright 22 year old and I am in the process of applying for financial assistance because I am not healthy enough to work full-time to support myself. I want to have a life where I don’t have to constantly rely on my parents or live in their basement.
In January, my parents and I will be attending the physio program at the lung transplant clinic in Edmonton, AB. This program is long and requires my parents and I to find accommodations and cover the cost of living in Edmonton for 6 weeks. It disrupts their work schedules and our lives in general and it won’t be cheap.
I’m not ready for a lung transplant yet. I’ve been told by several doctors that it’s like exchanging one disease for another. There are countless complications that can arise before, during, and after transplant. I’m not ready to sign up for that yet. I’m not ready to give up on my own lungs yet. I still want to find a way to make them work for me. Maybe ORKAMBI can help me do that. Maybe it can help me avoid having to take on all the complications that can arise from a major surgery like transplant. Once I have someone else’s lungs in my body, these new CF drugs are of no use to me. That scares me so I’m holding out hope that ORKAMBI will, at the very least, give me a few more years with my own lungs before I have to consider transplant.
CF is slowly stealing my life from me. I have dreams. I want to travel and see the world. I want to work at a job I love and be able to support myself and buy a house. I want to get married and not break my husband’s heart when the CF stops mine. I only get one shot at my life. There are no second chances. It’s go big or go home. I’m not ready to go home yet. I still want all my dreams to come true. And, if ORKAMBI is approved, it could be a stepping stone for future medications that will allow my dreams, and the dreams of other CF patients, to come true while our own lungs are still in our chests.