Chronicles of a CF Mama: Part I

Ten Fingers and Ten Toes

When asking an expectant parent what sex they want their baby to be, we all hear the phrase, “All I care about is that the baby is healthy.” What a true statement that is and it is what I thought I was blessed with when I gave birth to Megan Angelina Tims, a beautiful little girl, on Valentine’s Day. Ten fingers and ten toes with a great APGAR score. Everything looked great and we took her home 28 hours after she was born with a great bill of health.

We had a great 6 months living our carefree life with our three healthy children until Megan had her six months checkup.

After the Christening.

The five months after Megan was born were normal. I did notice she was always hungry and though she had the cutest chubby cheeks she seemed thin.

She was Christened in July right before I returned to work. Shortly after my mother and I took her to her sixth month checkup. They put her on the scale and she only weighed 11 pounds. I had noticed that her ribs showed but those chubby cheeks made her look healthier. After I had gone back to work she was eating less and she would only breast feed, refusing to drink from a bottle. Dr. Jo Nell Mahoney asked if we had Cystic Fibrosis in our family. I said, “No,” at the same time my Mom said, “Yes.” I had never heard this before. This is because our family did not know it is a genetic disease.

Megan’s doctor insisted that we take her to the hospital right away. She was malnourished. The baby needed testing.

The Sweat Test

That was our first day of many at St Mary’s Medical Center, our first time seeing our sweet girl cry when the nurses had to keep poking her to get an IV that worked or when getting bloodwork. To know the frustration of getting woken up a million times a night and, for me, to learn how to sleep for weeks on the Mom chair/bed. Bob Hammerton, the respiratory therapist, who we dearly love, gave Megan the sweat test, which consisted of putting little band on her arm that made her sweat as it collected the sweat. Here’s a fact we learned that day: If you have CF your sweat will have up to five times more chloride than normal sweat. Salt plays a crucial role in maintaining human health. Sodium is involved in the regulation of fluids in the body. When there is no salt in the fluids they become thick. This thick fluid, aka mucous, affects all your internal organs especially your pancreas which helps digest the fats in your food. And mostly your lungs. (We will go over that on a later day because it is a big deal). She was also tested for other issues but Megan’s sweat was very salty. Bob came in with his head hung low and confirmed it was Cystic Fibrosis. We really did not have any comprehension of what it fully meant but I could tell by the looks on the faces of the people in the room that it was not good news.

I remember being so scared and helpless. What did we need to do for our baby girl to get her healthy?

I remember being so scared and so helpless. What did we need to do for our precious baby girl to get her healthy? The first thing she needed was to gain 11 pounds before she could leave the hospital. This was our introduction to pancreatic enzymes. They take over and help her digest fat because the mucous in her body covers the pancreas and does not let her body digest fat. Her little body had not been able to do that and that is why she was so skinny. We had to start her on baby food, so we learned to open a capsule of enzymes and count out a prescribed number of little pellets and mix them in the food. (She would be known at the hospital as the green bean baby as that was her favorite). Then I had to stop breastfeeding which was hard on me mentally, mostly because she refused to drink from a bottle. I had bought every bottle in the world and could not get her to take it. In the hospital they tried the preemie bottle and she started drinking formula. It was a happy moment that I will never forget. This meant more calories as we had to double her formula for extra calories. This was the beginning of a lifetime struggle to gain and maintain weight and a constant battle to have proper nutrition. Poor nutrition equals poor health. Poor health equals poor resistance to fight infections. Especially lung infections.

Breathing Treatments

The scariest thing we learned in that first hospital stay was how CF adversely affects the lungs and is the main cause of CF deaths.

In a person with CF, the cilia cannot easily move the thick and sticky mucus out of the smaller passages. The mucus clogs the air passages, causing a chronic cough and lung infections. Repeated lung infections lead to lung damage, causing breathing problems and difficultly getting enough oxygen. This is when we learned about the importance of chest physical therapy or CPT. We also learned what a respiratory therapist is, and how this would become a big part of our life. The therapist would come in and put a mask on her little face attached to a nebulizer cup with albuterol in it to breathe in. That opened the small airways in her lungs. Then we learned how to cup our hands and hit or pat different areas of her chest wall and use percussion to help loosen and move the mucus so she can cough it out. Percussion is done for two minutes in each different position on the front, back and sides of her chest. Not an easy task on a 6-month-old. This would have to be done twice a day when healthy and four times a day when she was sick. This was a vital thing to do to keep her healthy. To say the least we were in shock at how different our life would be.

We were mourning the healthy baby we thought we had just a few days ago. What kind of a life was she going to have? What major changes would this mean for our family’s life? And learning it was a genetic disease the doctors wanted to know did Sean and Ryan have CF too? 

Genetic Defect

If we were not scared enough about Megan having CF they said the boys needed to be tested. Really? My boys could have this too? We were so frightened. Both Chris and I and were given blood tests and we were both positive for the CF gene known as a Delta F508. There are many CF genes but Delta F508 is the most common. This confirmed Megan’s diagnosis. They asked that when we brought Megan for her first doctor’s appointment after she got out to bring the boys too. I learned that when you kiss a child with CF they taste salty. Folklore from the 1500’s has a saying “Woe is the child that tastes salty from a kiss on the brow”. I must have looked like a Momma cat because I was licking the boy’s foreheads so much.

When Megan finally gained the weight, she needed, and we learned about how to do CPT we finally took Megan home to start our new norm.

That was a scary week. I had no doctors, nurses, respiratory therapist to help us. It was our job now to keep this beautiful and happy baby healthy.

The next Friday we had our first appointment at the CF clinic for her checkup and the boys were to have their sweat test. I was a nervous wreck. I felt Ryan was saltier and was convinced he had CF but to our great relief they were both negative.

The doctors told us to wait until they were older to test them to see if they are carriers. They have both been tested and Sean is not a carrier, but Ryan is. When it comes time for him to have children his wife will have to be tested. If she is a carrier having children is an important decision they will have to make.

You see both parents must be carriers of the defective gene to have a child with CF. Then you have a one in four chance of having a child with CF. One in twenty people are carriers especially if you have genes from a European country.

My CF carrier genes came from my Italian side of the family. I asked my Mom about why I had never heard of this in our family. She told me she had a cousin in her generation that passed away at 5. She remembers them tapping on his back. At that time, they did not know it was CF or that it was passed down genetically. Then her cousin had a daughter who had CF and she only lived to be 12 but my Mom did not know it was genetic. These poor children did not have the advantage of pancreatic enzymes of today. They were malnourished and had no way to fight infection.

Chris had never heard of it in his family but may have found something while doing some ancestry work—his father’s Aunt died young of Tuberculosis. It may have been CF.

I would not have changed our life before CF, nor would I change the next chapter of our lives with CF.

I am glad I did not know we were carriers. I would not trade my life, no matter how hard it can get. Megan, Ryan and Sean are my heart. Ignorance was bliss. Whenever I hear the Garth Brooks song, “The Dance” I sob. Look up the lyrics and you will understand why.

I would not have changed our life before CF, nor would I change the next chapter of our lives with CF.

–Denise Tims, CF Mama of CF Warrior Megan Tims

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