The Starting Gun

Today is December 12, 2012,  the thirty-third anniversary of Holly's diagnosis. In 1979, it was the day before her seven month birthday. Every year since then I have grieved as I remembered the devastation of learning that Holly had cystic fibrosis. This year, however, I have decided to focus on the positive ramifications of that diagnosis.

There's no denying that learning my daughter had a chronic and fatal disease was unspeakably painful, the news was also, in a way, a relief. Now we had a name for the cause of the awful problems she'd experienced, and, with that name, came the knowledge of how to help her.

For seven months, my baby girl had struggled to gain weight and suffered with excruciating stomach cramps. She could only sleep at night if I lay on my back on the sofa with a hot towel between our tummies to ease the pain.

Holly was a beautiful baby from the time she was born. At the nursery window in the hospital, her daddy's friend had to make a way for him through the group of people crowded around in front of her bassinet to admire this lovely little girl. However, some days her healthy pink color would change abruptly to a deathly gray, which would snatch my breath away.

Most babies have a sweet scent that adults love. I noticed very early on that Holly didn't smell sweet at all even immediately after a bath. For some odd reason, that little detail made me very sad. Perhaps, it forced me to face the fact that something really was wrong with my precious baby girl. I also observed that she tasted salty when I kissed her, and, when she cried, her tears left tracks of salt behind on her cheeks. Unfortunately, I was unaware of the significance of that one fact, so I told family members but never mentioned it to the pediatrician.

At seven months old, she had only recently begun to eat more than rice cereal. However, because her body didn't produce the enzymes necessary to produce her food, no matter how much she ate, her little tummy never felt full. The amount of food she consumed was astonishing considering her age. No matter how much I fed her, she always cried for more. It had gotten to the point that her plate of food equaled mine, and she still didn't gain weight. When I fed her eight ounces of formula, she would scream for more. But, I quickly learned that if I gave her more, she threw it up, because it was too much for her little tummy. She was malnourished and weak.

Finally, on Dec. 12, my big sister called and forced herself to overcome her fears of offending me by telling me that I must call Holly's pediatrician and insist on a sweat test. God's perfect timing made me open to what she had to say; I had read an article the day before mentioning that the taste of salt on babies often indicated a disease I knew little about called cystic fibrosis. She instructed me to hang up from talking to her and immediately call my doctor. As we talked, he started in with his usual arguments until I interrupted him, saying, "But she tastes salty." He responded, "Oh, my god. I'll call Children's Medical Center immediately to set up a sweat test." I wrote about that day in my previous blog post.

Though the diagnosis was devastating, the good news was: now we knew there were treatments to help her. The lung disease, which had not caused problems yet, could be slowed. What a relief we experienced when we learned that, by using pre-digested formula and artificial enzymes to digest her food, the awful stomach cramps would stop immediately! She would gain weight!

Our guilt and frustration over our inability to help our daughter were instantly relieved with the knowledge that we could help her. Yes, we were forced to face the long-term realities of CF, but part of the reality was that the treatments available were going to make her better.

And they did. At that time, when children were diagnosed with CF, they were immediately admitted to the hospital. Actually, we were told that the whole family was being admitted in order to learn about CF and how to care for her. The nurse brought Holly's first bottle of pre-digested formula and told me that it smelled and tasted awful. I smelled it and decided to take her word on the taste. She said that Holly probably would refuse it until she figured out she wasn't getting anything else. She was wrong. I took Holly in my arms, offered her the bottle and, instead of refusing it, she sucked it down, hardly breathing, because she was so hungry. When she finished the bottle and I set it down on the counter, she smiled! The nurse was as incredulous as I. Holly smiled at the bottle, because, for the first time her seven-month-old life, her tummy was full. My heart burst with happiness and relief.

In the first month after her diagnosis, Holly gained two pounds. Two pounds! She gained energy. Our nights on the sofa with the hot towel ended. Now, we could put her down to sleep in her crib with a full tummy.

Of course, we struggled with the realities that the average life expectancy was only 18 and the problems that would inevitably be part of her life. We had no way of knowing that, at 33, she would be married with an amazing daughter and still doing well.

So with that perspective, today on the thirty-third anniversary of her diagnosis, I've decided to celebrate. As Holly put it, "Instead of  a death knell it was a starting gun." What could I possibly add to that?