Tuesday, May 7, 2013

CF Awareness Month & Our Story

For as long as I can remember, my brother Robert has been sick. 

Throughout our childhood when he was fighting a "cold or allergies" worse than usual (because it was a continuous battle) or his "asthma" had flared up again our entire lives would stop to revolve around him.  My parents tried to maintain a regular schedule and routine for the family but when Robert was especially not feeling well, everything about his personality and demeanor changed. He wasn't the artistic little boy who loved sketching or working out puzzles anymore. He was a screaming, angry, heathen of a child. Despite talking to doctors and behaviorists, the only thing my parents could find to control these outbursts was keeping him medicated at the slightest sign that his "sinuses" were going to "act up" again.

As Robert got older his respiratory issues got worse. When he would have a bad case of "allergies" or a "cold" you could tell he didn't feel good by his physical appearance. His face would literally change, becoming very swollen across the cheek bones and around the eyes. 
Our pediatrician was doing his best to treat his "asthma" and get him relief, but as he entered his preteen years it was clearly not working anymore.

When Robert was in 7th grade, he was having one of his "episodes". He was just so, so sick. An already skinny boy (at 5 foot 8 inches he only weighed 95 pounds!), he was visibly loosing weight. His face was distorted with sinus blockages and the cough that we'd all grown accustomed to because he's had it since he was an infant was growing more frequent and awful sounding.

He wasn't acting himself at all, but rather than the tantrums of his childhood; he was shutting off from everyone. He was in and out of the doctor on an almost weekly basis. New antibiotics or breathing treatments, my Dad was on the phone with the doctors office every. single. day.

At one point that spring it got so bad that Robert couldn't lay down to sleep at all. My Dad was staying up with him practically all night because his coughing had gotten so bad that he couldn't really sleep even if he could lay down. What little sleep he did get was upright in a recliner. My very frustrated father was explaining this to the doctor who was beyond sick of hearing from him so frequently. It was then that the pediatrician finally recognized how severe Roberts illness was.
We already had an appointment with a Childrens Pulmonologist (lung doctor) at Cooks Children in Fort Worth, but the earliest they could get us in was over two months away. Now that we had our doctor advocating for us, he got Roberts appointment moved up to the next week.

Just as luck would have it, in the short time between finally getting the doctor to understand how ill Robert was and finally making it to the pulmonologist; Robert was finally doing better! My parents joked that the doctor would probably not do anything because it looked like he had just had a bad cold and was finally turning the corner, but they kept the appointment anyway.

It was a routine appointment with tests and exams. Mom, Dad and Robert took their time coming home and enjoyed the afternoon in Fort Worth with Robert finally feeling better. The rest of us kiddos were hanging out with my Grandma, it was the first day of Spring Break. As they got back into town, the three of them were going to run home for something and come back and as a family we were going to go out for dinner at our favorite Tex Mex place.

Sitting at Grandma's house, I knew it was taking too long for them to get from our house back to hers. I thought it was odd, but then again - my family never runs on time.
When they got there, the atmosphere was immediately one of seriousness.

Robert went off to the play room to entertain himself and Mom and Dad sat down to talk to the rest of us. When they got home, there was a message on the answering machine from the Pulmonologist himself. He said that he needed to talk to them and that he would wait at the office for their call and gave them a back-door number to call in on after hours. In later days, Mom recalled that she knew something was seriously wrong; that they had finally found out what was wrong with her son if the doctor was waiting on them like this.

Robert has Cystic Fibrosis, a genetic condition affecting his pulmonary system and digestive systems in turn affecting his endocrine system. Being a genetic condition, he has had it since birth; but when he was born, it wasn't like today where the test is administered in the hospital before sending the infant home.  As bad as his respiratory problems were at the time, throughout his life he would have been considered a "healthy" CF patient (even without proper medical care). No one even thought to look for CF because if it isn't caught very early on in life, it usually isn't diagnosed.  Robert didn't display the normal symptoms. Other than his constant cough, his respiratory problems would wane with vigilant treatment from over-the-counter drugs. His primary constant symptom was the fact that his digestive system clearly didn't work the way yours and mine does (trust me, you don't want to know...).

The diagnosis came as a huge shock to my family.
When my parents were kids, children with CF didn't live to be teenagers.
Today we have treatments and amazing medical studies that are making great strides towards allowing those with CF to live a longer, fuller life. As of today, there is no cure. The average life span of a CF patient is now into their 30's or 40's depending on the severity of the case.

We are very fortunate that Robert responds very well to his CF treatments and is considered "healthy" for a CF diagnosis. Each day comes with lots of medications, treatments and challenges.
I love my brother dearly and while I'm heartbroken that he is sick, it is a huge relief to know what's wrong and what we can do about it.

I shared some of his story today because May is Cystic Fibrosis Awareness Month.
For us, knowing that Robert has CF answered so many unanswered questions.

He couldn't gain weight because he doesn't digest fat and proteins.
(This is also the source of the horrible digestion problems)
His awful "dandruff" during the summer isn't skin - it's flakes of salt (I'm sure he's thrilled I shared that!)

The behavior problems associated with him not feeling well was as a result of his brain not getting enough oxygen - it affected his entire personality and mood.
When he was admitted to the hospital the day after his doctors appointment for his first two week stay, he was at 60% lung function. After being put on the appropriate meds to assist in digestion, he gained over 10 pounds in a week and was over 100 pounds for the first time in his life!

CF isn't very common, and you may or may not know someone with it - but I hope that you learned a little something from our story.

1 comment:

  1. A friend of mine has CF, and it's heartbreaking to see how challenging her daily life can be, but she has an awesome outlook. I feel like so many people are clueless about the disease, not really at their fault, but just because it's a disease that isn't discussed enough. Thanks for sharing.


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