Monday, September 7, 2009

The Monster Within

I was born July 13, 1981. I came a month later than scheduled. Weighed 7 lbs 11 oz. I was a petite baby for my family. Like most parents, my mother was concerned about whether or not I had the correct number of fingers and toes. I had deep black eyes that would change to a chocolate brown in the following months. And I would eventually have a head full of brown curls. My mother described me as a cherub baby. I was named Solana, the Spanish word for sunshine.

Little did my family know, I was battling a monster. A monster that was inside me. A monster that I would be battling for the rest of my life.

My mother was a new parent. Since she had no comparison, it took her a while to realize that something was wrong with me. Though, I must say, she is one of the main reasons I am still alive today.

According to my mother, I was constantly starving. She could not supply me with enough milk to keep me satisfied. She probably felt pretty helpless, being awake all night listening to my cries of hunger. When I was about two or three months old, my mother went against conventional standards of the time. She started giving me formula and rice cereal at a very young age. She would make the formula, add the rice cereal to thicken it up. Then she would have to make a larger hole in the nipple so I could suck it out. According to my mother, I really liked the concoction she made me. At the time, this was considered taboo to start a child on formula and cereal at so young an age. My mother didn't know it then, but she had just taken the first of many steps necessary for my survival.

See, the main underlying problem in Cystic Fibrosis patients is that our bodies don't hang on to salt like they should. So a Cystic Fibrosis patient needs to consume a high level of salt to replace what is constantly lost. Breast milk supplies little to no salt to infants, whereas, formula does supply salt. So by switching me to formula, my mother had unknowingly ensured that I would receive some of the salt I was losing.

Because I kept getting sick, my mother became what most doctors consider an overly paranoid parent. The type of parent that brings her children in every time there's a sniffle or a sneeze. My mother knew something was wrong with me, but she just could not figure out what. She constantly took me to the doctor. It wasn't for a sniffle or a sneeze. She would take me in because my cold would turn into pneumonia or my food seemed to pass right through me. But the doctor kept sending me back home. He would tell my mother that children get sick, that my GI system wasn't fully developed yet, or that she wasn't feeding me the proper foods. I would guess that it was a hard time for my mother, knowing that something was wrong with her child, yet feeling helpless as to where to go for help.

Finally, when I was just over three years old, my body could no longer keep up the fight on its own. I had gotten sick again. I was extremely dehydrated, hallucinating, had broken out in a rash, had cradle cap (a sign of malnutrition). My mother too me to the doctor once again and once again he chose to send me home, but this time he threatened to turn my mother in for child negligence. Outside the doctor's office my body went into failure. I was rushed to the nearest emergency room which was Ridout Memorial Hospital (which is now part of the Fremont-Ridout Health Group).

The doctor who sent me home was at the hospital. He told my mother, "You just killed your daughter." Now that is the last thing a parent needs to hear during a crisis like that. My mother was already devastated, fearful, and feeling helpless at that time. Guilt was not needed to be added to the list. When it was found out what had made me so sick, that doctor was properly disciplined.

I was in such bad shape at the time that the hospital staff could not find a vein that would take an IV. They had to do what is called a "cut down" in my ankle. A "cut down" is when they cut into the skin to find a vein that is hiding. I still have a scar on my right ankle from it. I was in renal failure by then and was a very critical case.

When I had stabilized enough for transportation, I was airlifted to the UC Davis Medical Center in Sacramento, California. I have no memory of that helicopter ride, but I know that the crew (doctor, nurse, and respiratory therapist) worked very hard to keep me alive during that flight.
I was in a coma for about a week. Once I had been stabilized and awakened, doctors decided to find out what had made me so sick. They had several theories ranging from vitamin B deficiency to being allergic to sunlight. My family history was looked into for clues as to what was wrong with me. Lab tests were ordered to test each theory. I became a human pin cushion. The tests continued to come back negative, and the doctors were forced to consider other possibilities.

Finally, an intern suspected Cystic Fibrosis. She actually came in to ICU after hours to sit down and speak with my mother. She was the first medical professional to genuinely listen to my mother's concerns about my health. Since Cystic Fibrosis is genetic and there was no known family history of the disease, it wasn't originally tested for. But to be thorough, Cystic Fibrosis was added to the list of conditions to test for.

Testing for Cystic Fibrosis didn't require me to become a human pin cushion again. All that was needed was a sweat test (also known as a sodium chloride test). This type of test entailed my arms being wrapped and electrodes and sensors being placed on my skin. My arm was made to sweat and the sodium chloride levels were measured. I had a higher than norm level of sodium chloride in my sweat, meaning I had tested positive for Cystic Fibrosis.

In CF patients, cells are unable to properly bring chloride in. This extra chloride is expelled from the body by combining it with sodium and releasing it through sweat. This causes the sweat to to be extra salty.

My sweat is so salty that I actually have a thin layer of powdery salt on my skin whenever it's hot out. I'm literally a walking salt lick for the animals on the family ranch.

Chloride is actually needed for proper cellular function. When chloride is prevented from going into the cell, organs begin to have problems. Thick, sticky mucous develops in the lungs, making it a breeding ground for bacteria which leads to multiple lung infections. This mucous also blocks enzymes from getting to the intestines, causing malabsorption (lack of absorption of nutrients), which was why I was starving. And in most male with CF, the vas defrens don't develop, making them infertile.

So the monster inside me finally had a name. Doctors gave my mother a regime for keeping me healthy. They also told my mother that I wouldn't live past the age of seven. They didn't recognize a fighting spirit when they saw one.