Thursday, July 8, 2010

Starting Ben's CF treatments

This week has been such a whirlwind of activity, and now, a moment of quiet to reflect. My two big boys are tucked into their beds, and Benjamin is sleeping on his Daddy's chest while he watches TV.

This week, through a series of three different tests, Benjamin's diagnosis of Cystic Fibrosis was made official. Wednesday he had the sweat test, the standard way to diagnose the disease because people with CF have more sodium chloride in their sweat.

I took him to the hospital, where they attached electrodes to his arms that would send a small electric current into him to cause him to sweat in that area. This had to stay in place for five minutes on each arm. It was hard to see him hooked up to those electrodes, as I tried not to imagine him being hooked up to medical equipment in the future, suffering complications of CF. To make matters worse, I had to hold his arm out straight the whole time, and he cried and cried because he didn't like the feeling of confinement.

After the time was up they put a gauze strip on his arms to collect the sweat, and wrapped them both in plastic for ten minutes. During that time I got to wrap him up in a blanket while he nursed, so the bad part was over.

We received the positive results a couple hours later when he went to the regular ped. for his 2 week appointment. A level greater than 60 is considered a positive result, and his was a 94 on one arm and 106 on the other. After receiving the genetic results, this didn't come as a big surprise, but it was deflating to get that final diagnosis.

Yesterday Mark, Benjamin and I had our first meeting with the Cystic Fibrosis team of specialists that he will see throughout his life. We spent the morning receiving a lot of information, and came away feeling encouraged. To start with, we are treating the two main symptoms of CF: digestive and lung problems. Benjamin has been doing pretty well over all, but he had been nursing very frequently, and then having frequent large stools to the point where I was feeling depleted, and wondering if he was really digesting all the calories he was taking in. So, they started him on a digestive enzyme called Creon. He takes a little bit of it with a tiny amount of applesauce before each feeding. With CF, the pacreas often becomes insufficient because of secretions being too thick, and that is what they think was happening with Ben. They are testing a stool sample to be sure, but wanted him to start the enzymes right away so he is able to absorb what he eats.

We found out his weight to height ratio is only in the 22nd percentile, and they like to see CF kids at the 50th or above. I was told that sometimes kids come in for the first time, and they are below the chart. If I hadn't been nursing him frequently on demand, they thought he would be doing much worse. Finding this out was the saddest part of the appointment, because it was the first time we had to realize he was already suffering some of the effects of the disease. Since starting the enzymes yesterday, I feel better because he has been lasting longer between feedings, and seems more satisfied. Before, anytime someone would hold him against them, he would automatically start bobbing his head like a little woodpecker trying to find a nipple to latch onto!

Relating to digestion, he also has to have a liquid multi-vitamin every day, and 1/8th tsp salt. So far I haven't succeeded with the salt. I'm supposed to give it to him little by little over the course of the day by dipping my finger in and putting a little on his tongue. The first time I tried it today, he gagged and spit it back up and then refused to nurse. I'll try again tomorrow, starting with a little less on my finger.

So far his lungs sound clear, but we've started chest therapy as a preventative measure. For 20 minutes twice a day we have to pat him in different spots on his chest and back to loosen up mucous.

So, that's the gist of it so far. Today was the first day doing his treatments, and aside from the salt it has gone fine. Three of his feedings were still only two hours apart, and they prefer I stretch him to 2 1/2 or 3, but it's still an improvement from before.

It's definitely overwhelming, and right now I'd have to say we're pretty much in survival mode. Going on very little sleep, and just doing the next thing that needs done at the moment as the day progresses (while many things are left undone.) I'm sure we'll get down to a good routine soon, and I'll start breathing much easier when he begins to grow and hopefully put on a good amount of baby fat. That's our number one prayer request right now, along with just getting into a routine where I can manage Benjamin's needs while balancing it with the needs of my other two children. I really want to have quality time with them and provide them an enriching environment, but I know that will come with time.

I'll close for now because this post has taken longer than expected and it's gotten late. What began as peace and quiet lasted only a moment, and was interrupted by my three year old getting out of bed several times, Benjamin crying with a bad stomach ache, and my husband's announcement that a Pull-up went through the laundry and all the clothes are covered with particles. Hopefully I can get this little guy to bed, so I can rest up for another eventful day! Oh-and I also had the bright idea of signing the boys up for early morning swimming lessons for two weeks, so that's the first thing on the agenda for tomorrow.
If anyone wants to know more about CF, the best source of accurate info is


  1. Oh, I was praying so hard that it would be a mistake, that Ben does not have CF! How overwhelmed you must feel. At the same time, we know that God is in control of Ben's situation and He will be with you, just like the scriptures on your blog. It sounds like Ben has a great team of professionals taking care of him. I will continue to pray for you all, as you adjust to this diagnosis and arrange your schedule to include all you need to do. I never dreamed that when I donated to cystis fibrosis research, I would someday "meet" someone whose child would benefit. (((HUGS)))

  2. Jess,

    Just want to say you are in my close thoughts and prayers, as is your little man. I can only imagine how difficult this new journey will be, but God's grace is sufficient. You are a true testament to that.


  3. I remember those early days... soon enough all the CF stuff will be routine. 22nd percentile is great! My daughter started out below the chart, and is now about 5th percentile. 50th percentile for everyone is a great ideal, but as our ped said, this is the real world! As long as he's growing well, that's the most important thing.

    What kind of vitamins are you using? We started out with AquADEKs and they stained horribly when she spat up. We're now using Vitamax and they don't really stain. We always gave the salt in a bottle of pumped milk. I tried but I couldn't get her to take it any other way. :(

    I wish you luck as you begin your CF journey! It's important to remember that right now, our kids are NOT sick! They require some extra work to keep healthy, but they aren't sick unless they get an infection. You will be rewarded for all the hard work you do to keep Ben healthy when you see him thriving!

  4. I'm glad that he seems to be responding to the enzyme already. That is good! I pray that he continues to grow and thrive. I'm sure you guys worry about him and what is ahead. Praying God's guidance for you.