February 19, 2023
One of the hardest parts of living with cystic fibrosis is the fact that most symptoms are often “invisible” to others. Whether it is friends, family, those at school or people we see and connect with out in public, we often hear, “She doesn’t look sick…” Olivia may only be 5, but we are seeing more and more just how much this disease sits in the background and how we can sometimes “forget” that her body is constantly fighting a battle.
The least “invisible” part of living with cystic fibrosis, is the complications with the respiratory system. Behind the scenes, mucus builds up in the nasal, sinuses and lungs and can cause chronic cough/congestion and sometimes lung infections. A person with CF is more susceptible to harboring bacteria in their lungs which make it extremely dangerous. Making sure they are not in contact with anyone who has any kind of bug, big or small, is so important to keep them germ free.
Another thing people with CF have to look out for is anywhere in the environment where water is stagnant. Harmful bacteria can grow and thrive in those environments and can be deadly for people with CF. Pseudomonas is one of the most common harmful bacteria and you can almost find it anywhere in nature. And as we can’t see bacteria, people with CF have to be careful to keep their hands clean and stay away from places where they can potentially be in contact with them.
Mucus also builds up in all other areas of the body which can cause symptoms that are more “invisible” to anyone that is not living with the disease. For instance, Olivia has a really rough time with her digestive system. Her journey started all the way back from birth when she was born with meconium ileus, a blockage caused by the sticky mucus in her intestines. According to Seattle Children’s, about 1 in 5 babies with CF are born with this type of blockage.
She also has chronic constipation, and we have to constantly be on top of her daily MiraLAX drink and sometimes have to do flushes to clean out her system. CF can also cause malabsorption and can be hard to keep a healthy weight. Olivia was on a supplement drink until she was 3 for this but was fortunate enough to be able to get off of it. Other people with CF can sometimes have a gastrostomy tube (G-tube) to help consume more calories on a daily basis.
These are the symptoms we’ve had experience with Olivia’s short life, but CF affects so many other parts of the body that can go unnoticed and make understanding the disease as someone on the outside very difficult.
This has been hard to come to terms with as we’ve gone through managing Olivia’s CF. The past couple years, she has struggled with anxiety and anger.
Each case is different but most people with CF have on average 4 visits with their care team a year, yearly x-rays and blood work. When she turned 4, she started having procedural anxiety and it was to even get her temperature taken. We have taken steps to help her become more comfortable at the doctor’s, but it is a long road and will continue to be a challenge as her disease continues to progress.
Unfortunately, her procedural anxiety started flowing over into her daily life and started causing some separation anxiety with her not wanting to go anywhere, even around the house, without me or her dad and having a hard time leaving when we did have to drop her off somewhere. She also has developed some control issues where she can sometimes be defiant because she doesn’t want to lose the sense of control in a situation. The times she struggles with control, she can sometimes lash out in anger or become extremely emotional and break down.
Most of her life is out of her control, she has so many things she has to do on a daily basis for her disease and it is unlike everyone else she is around, including me and her dad. Being a bit of a control freak myself, I can only imagine what that must feel like.
People living with CF have pretty intense daily treatment plans that consume a large part of their day. Right now, Olivia is on her “preventative / healthy” plan which consists of around 1.5 hours of breathing treatments (usually using her nebulizer and high frequency chest therapy vest), and daily medications she takes throughout the day that take in total about 30-45 minutes to prepare.
When she is on her “sick” plan we bump up to at least 4 breathing treatments a day totaling close to 3 hours; AND that doesn’t take into account the frequency and how hard it is to get anything done in between. Needless to say, the amount of time spent managing this disease on a daily basis is incredibly hard to understand unless you are living in the same household. Even then, sometimes I forget how much time we’ve spent and how much “easier” life could be if we didn’t have to stay on top of it.
Caring for a child with a chronic illness is challenging and when that illness is “invisible” to most of the world, it makes it that much more. On one hand, I’m grateful that she doesn’t have anything visible that will make her stand out in the crowd as different but on the other, people not knowing the dangers that come with her disease can sometimes be just as detrimental.
We need a cure for EVERYONE living with this disease! We won’t stop until CF stands for Cure Found!