(Part 1 is here)(Part 2 is here)

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“Cystic Fibrosis.”

The pulmonologist – one of the top in her field – had said it.  In relation to our tiny little baby girl.  I’d never known what it was like to nearly faint – until that moment.  I managed to hold it together, even though Archie was watching me with fear and concern.

I clutched the phone tightly as Dr. A explained that she had left instructions to get us in the following week so that she could explain to us better what CF meant. She reiterated again to not panic and emphatically said “Do NOT research online. Many sites will give you the worst case stories in an effort to raise money.”  She explained that Angel’s numbers were in the mid range for a very low amount of sweat – so we couldn’t be 100% sure yet. 

We would rerun the sweat test. We would have a blood draw for genetic screening. I didn’t want to listen anymore, but I had to so I could repeat it all for Archie.

We went back to the hospital a few days later, still numb and confused.  We met with the pulmonologist.

She said that Angel’s first test had had very little sweat on it.  But that “safe” numbers for CF were 0-40. Mid line (in the CF range but very low) were 40-60, and surefire was 60-100.  That Angel’s numbers had been 62.  That 100 was usually the results she saw with a positive result. 

She explained with reassuring tones that CF wasn’t the death sentence it once was – that many people today live long lives with CF.  That every case is different – that she just started treating a 45 year old woman who only learned she had it because her daughter was diagnosed.

All the reassurances couldn’t stop the sickening beat my heart had pulsed since she’d first said Cystic Fibrosis.  She answered all of our questions through my numb haze I can’t remember any of it…but told us to call any time we had any questions at all.

We went for the tests and were told that it didn’t seem that she’d sweat enough for the test to work, but they’d try to run it anyway.

It didn’t work – Angel does not sweat well.

It took two months and five more sweat tests to make the child sweat enough to run a second test. 

In the mean time the x-rays came back clean.  The genetic screen (which only screens the 80 most common genetic indicators of CF – there’s like 190+) came back clean.  Her weight continued to be low, her cough faded away into the night.  But still we waited and wrung our hands.

Archie moved back in and we worried together after we put her in bed each night.  Our family repaired itself, but our baby still hovered in the unknown.

Finally after 7 sweat tests we got enough sweat to test again.  Again the numbers came back elevated – but still on the low side. Dr. A consulted with another doctor and then said that they had to call it…it was Cystic Fibrosis.

In June 2 years ago we went into the hospital for a day long marathon of tests.  More x-rays and blood work.  A lung function test (which is FUN for a baby that reacts poorly – or not at all – to knock out drugs). A sputum culture – a day in the CF clinic.

Since then we’ve been blessed with the good fortune of Angel being relatively healthy.  After a year of CF we were told that her lungs were ‘starting to look more CF’.  But we’ve still had a peaceful time.

At the time of her diagnosis, the pulmonologist said “At her age you are more likely to have problems from her other issues like the hypotonia than you are from the CF.”  So far she’s been correct.  We only hope that it remains that way for a LONG time.