Hayley Quaintance

Daughter of Rebecca and Shaun Quaintance, Fort Wayne, IN.

When was Hayley diagnosed with CF and what were her symptoms?

Hayley was diagnosed with CF when she was 3 months old. She had severe reflux and was not gaining weight. Then she started aspirating her formula into her lungs and actually stopped breathing on us. We were admitted to the hospital and Hayley ended up having a surgery called a Nissen Fundoplication, which is a stomach wrap, so now she cannot vomit and basically nothing can come up. We recovered from that surgery and went home from the hospital only to go back the next day because Hayley was still having trouble breathing. Then they did the sweat test and it came back positive for CF. That was the worst day of our lives and I still remember it as if it were yesterday.

What CF-related medications and therapies does Hayley take?

Hayley does her vest therapy three times per day for 30 minutes each time. The vest basically shakes her to try and loosen all of the thick mucous. She also does several nebulized medications with her breathing machine. She takes Xopenex three times per day and Pulmozyme once per day. Hayley recently started taking Tobi, which is an inhaled antibiotic. So this adds another breathing treatment she must do two times per day. She also has to take enzymes before all meals containing any fat to help her digest her fats properly. Right now she takes five pills before each meal, but the number of pills changes frequently based on her weight and how much fat is included in each meal or snack. Hayley takes a special vitamin, Vitamax, that has several different vitamins that people with CF have trouble absorbing. In addition to that, she takes Nasonex nose spray and Zyrtec for allergies. We also have to increase her vest therapy and breathing treatments to every four hours when she is sick. Hayley usually spends at least three hours per day doing her maintenance treatments, just to keep her healthy.

Hayley is only 4 years old and already has been hospitalized six times due to her CF. She has been on IV antibiotics five times. Her hospital stays normally last about 5 days until they can get the IV medication levels right and then we are able to go home and finish the IV medications at home. This required my husband and I to learn how to give the IV medications.

How does CF affect the routines of Hayley and your family?

Hayley normally does about 2-3 hours of treatments per day for her CF, which causes her to miss out on a lot. We have to stop what we are doing a lot to do her treatments, so no long car trips, no long days at the zoo, ect. It is also very hard on our son, Nathan, who is 7 years old. He does not have CF, but is a carrier of the CF gene. Hayley gets a lot of extra attention due to the CF and all of her treatments and when she is hospitalized one of us is always at the hospital with her, so that means that Nathan is without one of us and gets shuffled around to relatives homes a lot during the hospitalizations. I am sure the schedule of treatments and medications will become even more difficult as she gets older and starts school. My mother also retired early so that she can stay at home and take care of Hayley as it would not be a good idea for Hayley to be in daycare with all the germs and illness. My husband and I both have to work to continue to have good insurance coverage for Hayley to pay for all her medications.

During the winter months, we stay in the house a lot to avoid all the cold and flu bugs that are going around. We also have to avoid sick people because even a cold could turn into a serious lung infection for Hayley, so this sometimes mean missing out on family parties and events if someone else that will be there has a cold or is sick.

What are Hayley’s interests and hobbies?

Hayley is your typical four year old. She loves to play with her dolls and play games on the computer. She loves to dance. She has been taking ballet for the last year and loves it! She started preschool at a local church this year and attends two mornings per week. Hayley loves school and wishes she could go everyday. She really is such a trooper and does well with all the treatments, medications, and medical appointments because it is all she has known in her short life. We hope to continue to encourage her to be all she can be and get her involved in as much physical activity as we can, because the physical activity and exercise is so important to her lung function.

What will the future bring?

We hope the future will bring a cure for CF in Hayley’s lifetime and right now we strive to keep her as healthy as possible so she will be able to benefit from any future medications, treatments, or a cure. My wish for Hayley is that her life will not be shortened by CF and that she will be able to live a long and happy life. That she will find a spouse that will love her and support her in her fight against CF and that some day she will be healthy enough and able to have a child or children of her own. It is very difficult for females with CF to get pregnant and most male CF patients are sterile.