Thursday, August 19, 2010

Postpartum Clinic Visit

Today was my first CF clinic visit since having Lillian. And I got good news - my FEV1 is holding steady at 111%! My last sputum culture still showed normal flora. I still can't believe I cultured PA for seven years and now I have not cultured it for three years. The last time I cultured anything was Staph back in May of 2009.

I hogged a lot of my doctor's time today to discuss a few topics:

The first thing we talked about was the possibility of Lillian having CF. Greg had a genetic test for the 97 most common CF mutations, and it came back negative. However, there are thousands of rare mutations, so there is still a chance Greg could be a carrier, and therefore, there is a chance that Lillian could have CF. Please note that I was aware of all this when we started TTC and I had talked it over with my CF doctor - he said at that time what we had done was enough. However, I wanted to be more informed so I mentioned that I've seen some talk on the CF forums about the need to have genetic sequencing done in order to completely rule out CF. I asked my doctor his opinion on this. Here is what he said:

Texas automatically tests for CF in all newborns by analyzing the blood for a by-product of the pancreas (IRT). Even people who are pancreatic sufficient, (like me), will show abnormal test results. If the levels are abnormal, a sweat test is performed. After that a genetic test is done. So, with Greg passing the 97-panel test and Lillian passing the IRT test, the odds of her having CF are very, very small. Doing full genetic sequencing is unnecessary.

He further noted that the state of California is automatically performing full genetic sequencing on ALL newborns, and they are finding all kinds of new mutations in children that have zero symptoms. There is debate in the CF community if these people really have CF and about "what to do" with these cases.

Please note that I am NOT making any judgments about what other people choose to do regarding genetic testing. I am just sharing the information I received.

The other subject I brought up was a new school of thought I heard about cases like mine: Instead of "mild CF" perhaps I should consider myself having "late onset CF." I mean, I DO have CF - I have sinusitis, I get lung infections, I cough up junk. My lungs are probably getting damaged, just more slowly than the typical CFer. But someday, (without proper treatment and new drugs), my lungs are going to be really bad. I will need to spend time in "Club Med." I may need a lung transplant. Eventually (barring something else) CF will probably kill me. When? I have no idea and neither do my doctors. But I'd like it to be later rather than sooner. Actually, I'd like it to be never because death by CF sucks. I don't even want to be hospitalized all the time in 20 years.

So I told me doctor, "You know, you are so used to looking at really bad x-rays. Sometimes, I feel like, when you look at mine, you think 'she looks great' and send me on my way. But I want to know what is going on. Please tell me if you see mucus plugs, or a small amount of lung damage. It will motivate me to take care of myself." He told me that chest x-rays don't really give much information regarding small airway damage, it just tells him if something major is going on. And he confirmed that people can be experiencing lung damage even when their PFTs are holding steady. So he is sending me to have a chest CT scan done. Based on those results, we will decide if I should add more airway clearance and/or Pulmozyme to my routine.

The last thing we talked about was my GERD. I mentioned that I am sick of waking up and needing my inhaler. So I am going to see a gastro. Hopefully we will be able to get this under control.

So, that's that. Greg stayed home with Lillian so I could go with out having to worry about her fussiness. She did pretty well for him - she slept most of the time, had a happy period, and then she got hungry and refused a bottle, so there was about a half hour of crying until I got home. I really hope she will take a bottle soon because I'd like to be able to go on a date with my husband!


Colleen said...

I too have "mild" CF. We actually have a very similar case of CF. I have been consumed recently with the idea that I will be facing "delayed" CF and my fate is no different from what the statistics tell us. It's scary. The unknown is horrible. The best I can do is take good care of my self and see what the future holds.


Lindsay said...

Glad it went so well!

Alicea said...

So glad you are doing so well and you are so persistent with your doctors. Kyle just had his first chest x-ray done and they said everything looked great. What scares me is the unknown and what they can't see on those. I certainly don't want any false hope when it comes to his health.

Is the IRT the newborn screen? Kyle's came back with false negatives twice and it wasn't until the genetic testing that they discovered he had CF and of course, his numbers were high on the sweat test, too (in the 80's, I think).

bbjoys said...

Good luck with whatever you can find out. Good for you for being so proactive. :)

cindy baldwin said...

Hi there! I've been following your blog for a little while... I too have CF and my husband and I are hoping to try to get pregnant very soon. I wondered if I could e-mail you and ask you a few questions about your experience with fertility treatments?

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