A Swift Kick In The Gut

This week was a difficult week.  We returned to see Dr. Kanga and didn't walk out with the news that we expected.  Our adjustment to Lilly's situation had been improving.  For the first time, I actually slept the few nights before her appointment.  I didn't bite Jeremy's head off.  Nor did I take out my anxiety on Olivia.  Rather, we both felt pretty confident that Lilly was doing well and we would have a good appointment.  We have noticed that she is really filling out.  Her BMs are looking good and we finally feel like we are on the correct amount of enzymes (even though her enzymes were not approved by the FDA - don't even get me started).  She actually has cankles and fat around her wrists - hooray!  Moreover, she finally graduated from the 0-3 month clothes to 3-6 month clothes, right around her 6 month/half birthday.  We were ecstatic!  While her reflux isn't really better, I was okay in dealing with merely a "laundry problem" and enjoying an appointment that was supposed to be a little less stressful.

When we arrived at Dr. Kanga's office, I actually wasn't eat up with worry.  I thought that we were finally getting a handle on her diagnosis.  Lilly measured 25 inches long and 12 lbs. 11 oz.  Score!  That puts her at about the 3rd-4th percentile in weight and 25th percentile in height (for those interested, her head circumference is about 40th percentile - bobble head!).  I was very excited.  In talking to Diane, she was pleased with her food intake and weight gain and both she and Dr. Kanga agreed that Lilly looked good.  They prescribed us Creon because, as I mentioned before, Ultrase still hasn't been approved by the FDA.  We have another 30 day supply of Ultrase and then we have to switch to Creon, which causes me some worry, but in talking to others Creon is fairly popular and effective, so this issue seemed like a minor problem.  Then they sent us to the lab and x-ray to have her first labs drawn and her first chest x-ray - fairly typical, as it is done once a year.  I wasn't too terribly worried because to date, Lilly didn't seem to be having any respiratory problems.

Then the results of the chest x-ray came in and it all came crashing down.  Now, let me preface this by saying that in the whole scheme of things, this probably isn't that big of deal with you look at it through the CF lens.  But for us, this was a VERY big deal and VERY big kick in the gut.  Her x-ray showed some lung disease, which was explained to us at length by Dr. Kanga and Dr. Anstead (the adult CF doctor, who we also met at this visit).  In short, Lilly's x-ray showed "slightly increased markings" which they said could represent a viral respiratory infection (which is also suggested by her white blood cell count) or mucus from CF.  The problem is that the chest x-ray is just not sensitive enough to tell us exactly.  What this means to us that there are definitely some changes going on.  While Dr. Kanga has assured me that there is no evidence on the chest x-ray that these changes are chronic or permanent, he and Dr. Anstead agreed that we needed to immediately start her on breathing medications.

This is particularly true since I reported a new cough.  To be honest, I didn't think her cough was that big of a deal.  I am very sensitive to these things now and a couple of weeks ago noticed that she was coughing a little more.  It is a dry cough and very random.  I asked Ashley to watch for it and she agreed that Lilly had started coughing.  If I hadn't read in my CF treatise that all coughs should be reported, I probably never would have said anything and chalked it up to allergies.  But once I reported the cough, and we saw the result of the chest x-ray, it was determined that something had to be done.

The current trend in CF care is to remain proactive, so Dr. Kanga and Dr. Anstead prescribed Lilly Pulmozyne, which has been available for 15 years and become a mainstay for CF lung treatment, even in asymptomatic patients.  She is administered the Pulmozyne, which will thin the mucous in her lungs, once a day through a nebulizer treatment that takes approximately 10 minutes.  Moreover, they have also prescribed her Albuterol to be administered through an inhaler twice a day prior to her chest percussion therapy.

The first blow was the fact that Lilly, who seems so healthy from a pulmonary standpoint, had anything show up on her chest x-ray.  I did not expect that so soon.  I really thought that her breathing problems would manifest later down the road and frankly, I wasn't prepared to deal with it so soon.  The fact that she even has anything show up at 6 months old, particularly where she seems so healthy, seems so ominous to me.  It seems to me that in talking to other CF moms that the earlier the breathing problems start, the harder the road is for their children and that terrifies me.  I had hoped (perhaps ignorantly) that wouldn't apply to us.  I am not sure that is the case.

I know this sounds petty, but I hate the thought of adding even more treatment and particularly of using a nebulizer.  I have used a nebulizer before on Olivia when she was a baby and it was very stressful to us, primarily because she cried the entire treatment.  The thought of doing yet another treatment where I have to fight with Lilly and listen to her wail just absolutely kills me.  It is so hard to watch your child endure such treatments, even if you know logically that they do not hurt her.  But watching her wail through a chest x-ray, through the drawing of blood, through chest therapy, and now possibly through nebulizer treatments - well, it is emotionally exhausting.  It physically hurts.  But we will do what we have to do.


Jeremy and I have been so dejected since we received this news.  Sometimes this disease seems more than we can handle.  As I said before, I know this isn't the worst that we will have to face.  Moreover, I also know that people continue to tell us that it will soon be like second nature.  But right now we feel like our whole lives are consumed with CF.  It has seeped into every facet of our lives and we are so overwhelmed by all of it.  Sometimes it feels like we are drowning and we are trying so hard to not let the stress of it all get to us, but sometimes that is so difficult.  I try to stay positive, but in situations such as the most recent set-back, it is just too hard.

On a more positive note, we also saw Lilly's pediatrician this week.  She too was pleased with Lilly's weight gain and had some suggestions that coincided with those of Dr. Kanga and Anstead about her reflux.  We are now mixing rice cereal with Lilly's formula to see if that helps.  We are also considering Reglan as another possible treatment.  She remains on the Prevacid because at the very least it is helping with her malabsorption issues.  Moreover, Dr. Taylor said that Lilly is progressing beautifully as far as development goes.  She is grasping at toys, putting her hands together, cooing, and all the things that you would expect from any 6 month old baby.  No teeth yet, but they may be coming soon.  So if there is a bright side, that is it.