Cystic Fibrosis: A Personal Story - Part One: The Discovery

The following is an interview and a first in a series about a friend of mine, who has lived with cystic fibrosis for many years and is currently one of the longest living people in the US with Cystic Fibrosis.

When did you first learn that you had Cystic Fibrosis (CF)?

It was the summer before I entered 9^th grade, when I was diagnosed with Cystic Fibrosis (CF). A lot of things were happening in our lives at the time. My mother left our household of two children, a dad and a dog, right before school started for 8^thgrade year.

This was a scandal in and of itself; a mother leaving her children! That year, school had been rough on my brother; he kept having pneumonia and getting sent home from school. Since dad was newly charged with mine and my brother's health, he took us to the new town doctor. Before that, medical care was provided by the town nurse.

What was the first sign?

My brother was really struggling to breathe and the doctor said that my brother had asthma. He gave him some inhalers.

At the same time, I was also seen by the doctor and I will never forget the doctor looking into my nose and saying that he saw polyps. He told me he had never seen them in a child before. He was so impressed he went and got his new partner to come look too.

The asthma and allergy medicine that my brother and I started taking really did not do anything for us. My dad insisted that something had to be done for my brother so that he would not get sick all the time.

The doctor said he would send my brother to Hartford for a Cystic Fibrosis sweat test. This was around May of 1978.

The test came back positive. I had never heard of this disease but when I read about it I knew that I had it too. I had every single symptom: the cough, the sinus problems, the digestive problems, the funny fingernails called clubbing, the very thin body, the very pale skin, and I was short for my age.

At the time the life expectancy of a child with CF was about 12.

How did you get diagnosed with CF so late in life?

All the signs of Cystic Fibrosis were there but they had all been explained over the years. The cough and sneezing was explained as allergies. And it did seem that everyone in my family sneezed and coughed more than "normal", so it was ok and never questioned.

The digestive problems for me started right at birth. I had Rectal Prolapse as a baby and continued until I was a young child. The doctor had correctly predicted that I would outgrow it, however, back then this was not always recognized as a symptom of CF. I had constant stomach aches but my parents discounted that because I had had the problem since birth.

My parents' marital circumstances also likely played a roll in our late diagnosis. They had not been talking to each other in a cooperative manner for probably 5 years. Who wants to think about their children having something seriously wrong with them?

Are there other symptoms and signs of Cysctic Fibrosis (CF)?

Club Nails:

My grandmother and the kids on the playground knew my fingernails were different. They were kind of curved. My grandmother used to rub them and say how pretty they were but the kids at school called me mushroom fingers. Other kids said I had Fred Flintstone toenails. These funny shaped nails are caused by a lack of oxygen and should have alerted a doctor to a problem but it was missed.

Insatiable Appetites:

Rod, (name changed) my brother, and I always had very, very, good appetites. We would get up at dawn and fix ourselves our own breakfast of cereal. When adults in the house would get up we would eat with them too. If we were really lucky somebody might make a third breakfast on a Saturday or Sunday morning.

We had an Italian grandmother who loved to have us come because we could eat forever. As soon as school got out I needed to go home and eat. It seemed like 4:30 was an endless wait for supper. That good appetite was seen as really marvelous, healthy even.

Pale Skin:

Both my brother and I had blonde, blonde hair. Pale skin went right along with blonde hair so being really pale did not seem odd to my family. My mother was pretty short so the fact that we were small on the growth chart seemed ok as well. At the time, everything seemed ok but it was not ok.

After we were diagnosed

We were both a little old to just be finding out. My mother had a very hard time with the diagnosis. What mother wants to look in the mirror and think about the fact that she left her husband and children, and then find out a few months later that they are sick with a potentially fatal illness.

People often think that God doles out one hardship per family. Well, I don't believe God has a quota on such things. Our family had a lot of problems and then we were diagnosed with CF too.

Fortunately my grandparents were very supportive of my father. Unfortunately they had moved down south shortly before our diagnosis so they could offer only so much help.

How did the Doctors miss diagnosing Cystic Fibrosis?

A lot of times people think that doctors know everything. They wonder how things get missed. Doctors are just people. They have bad hair days, they are preoccupied with their family, they may have had a really busy weekend as a sports warrior and feel kind of tired.

My parents together thought that they had normal, fairly healthy children. The doctor that did our school physicals never got any feedback from my mother saying anything was wrong with us. That town nurse was really the one that put up the alarm flag that something was wrong with my brother.

Hurray for her attention! Everyone has to participate in health care so that the best possible outcomes have a chance to be possible. Everyone has to articulate for themselves and their dependents their symptoms and what is going on with their health.

Sources:
Personal story of "Eleanor", name changed to protect her identity

Dear readers,

Thank you for allowing me to introduce you to my friend, via the first of a series of interviews. She has asked me to change her name for privacy purposes, and so I have dubbed her "Eleanor" in honor of another courageous and spirited lady, in history, Eleanor Roosevelt.

I recently got a chance to see my friend from my youth, and she told me she's been reading my "stuff". She was extremely enthusiastic and supportive and so I asked her if she would agree to an interview about her life with CF, and she did.

I find her to be inspirational, inquisitive, full of life and love, and one of the most tenacious and spirited people I know - in fact she is a kindred spirit.

***
My friend named "Eleanor" is also making history living with cystic fibrosis in the United States. She was diagnosed over 30 years ago and is now 45.

Did you know that in April 1933, Amelia Earhart and Eleanor Roosevelt stole away from a White House dinner, commandeered an airplane and took off on a glorious adventure? The story is told in 'Amelia and EleanorGo for a Ride', written by Pam Munoz Ryan and Brian Selznick (Ill).

Well, I'm just the driver in this case and we're nowhere close to the White house, but hopefully you enjoyed the ride. Look for more of her story, a story about tenacity, being inquisitive, and being a survivor.