After your official diagnosis, it was hard. I think the Internet was actually more of a hindrance than a resource, or at least in that first week. I wanted to look up the the worse case scenarios. I wanted to prepare myself for what our journey may look like. Your daddy seemed more at ease. Not that he didn't care, but had more faith in the advancements that were always being made in the medical field. I think he also knew that we, together, were the best parents for you.
When we traveled to Children's Mercy after your diagnosis they were very surprised that you had gained weight. Most babies with your mutations, struggle with weight gain and have to start the enzymes immediately. Many infants even have surgery within the first 72 hours of birth simply due to the pancreas and bowel already being blocked with thick mucous. They had done a little bit of testing to determine that even though you were gaining weight, you were severely pancreatic insufficient and that since our plan from the 'get go' was to stay pro-active then we needed to start you on the enzymes.
We were instructed to take a small amount of applesauce and open a capsule and mix the enzymes with the sauce. I remember thinking that this was going against so much that I knew as a mother. I never even introduced solids to your sisters until about 6 months of age, and here you were still a tiny little thing, not even 4 weeks old, eating from a spoon.
I could see a big change within 24 hours. The enzymes were actually helping. Prior to the prescription we were changing your diaper as many as 20 times a day and we knew the enzymes were doing their job; they were allowing you to absorb the nutrients you needed from your food. You see, that is usually the first stages of CF as an infant. You have difficulty absorbing the nutrients that you need form the fats in your diet. Many individuals with CF have faulty or malfunctioning protein channels. Your protein channels were just fine; however, the number of channels you had were insufficient. Your channels were not allowing the chloride ions to escape from their cells. This chloride ( which easily comes from table salt - NaCl and is naturally in our bodies) is needed to attract the polar water molecule to allow for hydration to occur. The process of hydration is what is needed to thin the mucous in our bodies. Most individuals can easily sneeze and cough to break up the loose mucous when an infection sets in, but CF patients develop a thickening of their mucous which first tends to block the pancreas and interrupt digestion and then slowly works its way into to the lungs where it settles. While others can easily remove the mucous by blowing their noses, we will have to go through a series of "treatments" to help thin and loosen the mucous so it does not serve as a petri-dish for any foreign bacteria that finds its way into your system.
When others had heard of your diagnosis, I had several individuals ask me if you tasted salty. It took me awhile to test and see, but I would lick my lips, give you a big kiss on your forehead and then I could taste for myself......you tasted like a little salt lick. You see, since your cells were not releasing the chloride ions inside of your body, they were leaving with the cells through your pores. That is why everyday for the rest of your life you will have to have salt added to your diet. Right now as a 3 week old, we are adding table salt to your bottles.
I feel that I am starting to get this blog caught up to date. There is some information that I have not included, but as I reflect in future blogs, I believe the details will make themselves known. There is one more thing I need to add. We had traveled to Children's Mercy again on June 25, 2012 for two reasons. 1) was so you could have the sweat chloride test and 2) both your sisters Arya and Tara were to be tested to see if they possibly had CF or if they were carriers like your father and I. Even though we knew you had CF through genetic testing and had already started treatment, the sweat chloride test would inform us as to the rate at which your body was losing your chloride ions. I received a phone call on July 2 stating that your numbers were 90 and 120. As I did further investigation I found out that for your age these numbers were astounding. Under 30 millimoles/L = not likely CF, 30- 60 millimoles/L = borderline CF, and 60-80 millimoles/L = CF. When I asked the doctor what it meant, she had stated that as you got older and participated in sports we would have to really increase the amount electrolytes you consume. Dr. Bear also mentioned that you would be most likely adding salt to the standard glass of water that you would be drinking. As for the results of your sisters' blood tests, we are still waiting. It has been 13 days and I expect we will here something tomorrow.
Much Love Today, Tomorrow and Always,
|Gettig the elctrodes placed on your arms for the sweat chloride test.|
|Arya and Tara after they had their blood taken |
for their genetic tests.