Cysterhood
One woman’s story of living with cystic fibrosis.
Introduction
Cysterhood
One woman’s story of living with cystic fibrosis.
Introduction
Cystic fibrosis is something you occasionally hear about on the news, but most people have no idea what it is. Or even if you have a vague idea of what it is, its probably only a drop in the bucket. There are so many facets to this disease that I could probably write an entire book on the medical aspects alone. Instead I want to focus on the personal aspects. I have no intention of being shy about it either. No topic is sacred for me, including but not limited to; sex, bodily fluids, bodily functions, hospitals, relationships, family, and death. I plan to blow the lid off of this disease and let you into my life.
I suppose I can’t get into the nitty gritty without giving you a basic lesson on CF. I’ll try not to make it too technical.
Cystic fibrosis (CF) is the most common genetic disease among caucasians, but does occur in other ethnic groups. Basically, the body of a person with CF makes thick sticky mucus…..everywhere. Think about all the mucus membranes in the body. There’s your sinuses, eyes, mouth, lungs, digestive system, for the ladies there’s the vagina. I told you I wasn’t going to be shy.
The main trouble spots for CF patients are the digestive system, the sinuses, and the lungs. I’ll get into more detail about exactly what happens as we go along, but I will say that the main concern with CF is the lungs. All of that sticky mucus I mentioned sits in our lungs and makes a lovely breeding ground for bacteria. That means chronic lung infections.
Imagine the worst chest cold or flu you’ve ever had. That’s how I feel. ALL. THE. TIME. Those infections damage the lungs and create scar tissue. That scar tissue doesn’t function and eventually will cause respiratory failure. Many CF patients receive double lung transplants because of this. The ones who choose not to get new lungs, or the ones that don’t make it until lungs are available will literally suffocate to death.
Chapter 1: Childhood
I was born on September 28th 1981. I was not part of my parents plans for the future. See, my brother, Keith, was born a year and eight days before me. It was immediately clear that something was very wrong with him. Mom had to have an emergency c-section because every time she’d try to push my brother out, he’d go right back in. When they got him out his stomach was so bloated that it looked he was the pregnant one.
Keith was rushed to a couple of different hospitals before getting to Stanford Children’s Hospital. He had somewhere in the neighborhood of 16 hours of surgery before he was 24 hours old. When they first opened him up they discovered that he was literally full of shit.
Hey had meconium ileus. In English that means his first bowel movement had gotten stuck and created a blockage in his large intestine. Then everything else backed up behind the blockage until his intestine ruptured from the pressure. Then the, uh, feces had leaked out into his abdominal cavity.
Keith’s first year of life was spent going in and out of the hospital, having various procedures and surgeries. When he was about 3 months old he was tested for cystic fibrosis because meconium ileus is very common in babies with CF. The test was positive. My parents were told that the disease is genetic and that any future children they had would have a 25% chance of having it as well. At that time my parents decided not to risk it. They were not going to have any more children. Little did they know that I was already on the way!
Fortunately I did not have meconium ileus when I was born. Obviously this was a huge relief to my parents. In those days babies weren’t tested for CF right away. Genetic testing wasn’t available yet so they did what’s called a sweat chloride test. Results were more accurate with babies that were at least 3 months old. My parents talked them into doing mine when I was about 6 weeks old.
A sweat chloride test measures the amount of salt in a person’s sweat. People with CF sweat out a lot of salt. Our cells aren’t able to take it in, so it comes out through the sweat glands. One way they used to test for CF was to lick a kid to see if he was salty. Mom says my number was astronomical, there was no doubt that I too had CF.
From the start my parents decided that CF wouldn’t run our lives. I did all the things any normal kid does. Sleep overs, bike riding, roller blading, volleyball, public school. I did it all. At that time CF didn’t interfere with my life too much. In the morning there was a nebulizer (neb) treatment. Liquid medication was put into a little cup which was hooked up to a small air compressor with a hose. The compressed air vaporized the medication so I could breathe it in.
The identity of the disease.
When a person grows up with a disease, especially one like CF that requires a lot of work, their identity and their disease become intertwined. This phenomenon becomes more apparent as the disease progresses and life becomes harder. For example, there is only one room in my house that does NOT have some sort of medical device or equipment in it. That’s my step son’s. Even my oxygen machine lives in my step daughter’s room because she’s only here on the weekends.
There isn’t a day that goes by that CF doesn’t come up in conversation somehow. It could be with my boyfriend, a family member, friend, on facebook, but it happens every single day. I go to sleep and wake up with CF literally in my face since I sleep with oxygen. My mornings are often spent coughing up nasty green slime that’s pooled up in my lungs during the night.
More and more of my time and energy every year is being taken up by taking care of myself. That makes it very hard to separate me as a person and me as a diseased person. I know that CF has played a huge role in who I am today and the things that I value. Even if I could give up CF I don’t think I could give up what’s its taught me about what’s important in life.
I have even made major life decisions because of CF. The only career I ever wanted was one in medicine. However, a CF patient working in a hospital or medical clinic is a very bad idea, too much exposure to viruses and bacteria. On the other hand, if I didn’t have CF maybe I wouldn’t have wanted to be a doctor! How is that for a paradox? Since I couldn’t go into medicine I never went to college. I didn’t see the point of getting an education and spending all that time working for something I didn’t really want.
I was also forced to make the choice to not have children of my own. From the time I was a little girl I knew I wanted kids. I remember getting a doll one Christmas and I loved that doll so much. That was the first time I recall wanting kids. I don’t think I was even in first grade at that point.
At this point my CF is actually pretty stable. My lung functions have been around 50% consistently for about 6 years. The thing is, it takes a lot more work to maintain 50% than it did just a few years ago. In my early 20s I didn’t really do anything as far as maintainance for my CF. I took my enzymes to digest my food and took oral antibiotics when I got sick, but that was about it. Now my regimine is much more involved.
Not only is there CF but a couple of years ago I was diagnosed with CF related diabetes (CFRD). Since the pancrease already has a hard time doing its job because of CF its very common for scar tissue to build up and slow or stop the production of insulin.
