As I write this I am sitting in a chair beside my daughter, age 9, who is laying in her hospital bed. She asked me yesterday, “Mom, I am going to be here more and more as my cough gets worse and I get older aren’t I?” She understands her reality without anyone telling her. Its the harsh reality for 30% of people living with cystic fibrosis. She has no delta 508 gene to qualify for Trikafta. In fact, there are over 950 rare gene mutations that can cause cystic fibrosis.
We sit here in the hospital after trying to cope with a lung infection that has turned into pneumonia. After two weeks of nonstop coughing and lethargy at home, we saw our CF team and the head doctor tried to have her admitted to our local Sick Kids but there were no beds and 20 other children admitted to no beds. We were sent home with two antibiotics to try and fight it there. After a week, it was not working, and we went to the ER and were admitted to the next available bed 14 hours later. She has missed the last four weeks of school. The last month of school is supposed to be the fun time of year filled with games and field trips; not needles, x-rays, and hospital beds. While we sit in hospital, my husband is home with our four other children including our 7-year-old son who also lives with cystic fibrosis. Between my husband and 16-year-old daughter they are managing his meds and treatments and physiotherapy while I am here in hospital doing the same for our daughter.
We have tried not to cry watching many of our CF friends receive this life saving drug and start to improve almost immediately. It is truly amazing to see the new lives that can be led. We are so happy for them but it’s hard to be left behind. It’s hard to be sitting in the hospital with a 9-year-old who is struggling to breathe without coughing while my social media feed lights up with yet another CF child who has found a way to have this medication paid for. Parties and celebrations ensue. Seeing the amazing transformation to their quality of life is truly remarkable and unimaginable a few years ago. We are truly thrilled for them but it’s so hard knowing this is not our reality. Not yet anyways. We have heard it said by everyone who has qualified not to worry, they won’t leave anyone behind. They will continue to work until a therapy or drug is found to help everyone. We trust in those statements.
While our daughter realizes that her future includes more and more hospital stays, those who have started on Trikafta have seen amazing results and don’t face the same future. The hospital nurses will grow unaccustomed to cystic fibrosis and admissions of cf patients will be a rare occurrence. The only admissions will be of those unlucky enough to be born without a delta 508 gene. Those who feel like they have waited in line for hours only to be told at the door that the room is full and they won’t be allowed to enter. Those who feel like everyone else has won the lottery except them. It’s hard to explain just how awful the feeling is. While you want to be thrilled for everyone else in line who got in, it’s extremely difficult to be left out. The only child not invited to the party.
As we watched our daughter’s oxygen levels dropping before this latest hospital admission, I can’t begin to explain how all consuming the thoughts of dread are that invade. It’s hard to function. All the stories of cf children who have passed on before rush to the forefront of your mind. I try to push the panic away, think positively and speak positively so my daughter doesn’t panic or worry. We try to remain steadfast in our hope for her future and the future of her brother. Our hope depends on the researchers, doctors, and donors. Please help us to get our children into the party.
Adelle – mother of two children living with cystic fibrosis and no delta 508 genes.