The Journey to CF (Part 3)

by Sarah | Mar 17, 2009 | All About Kennedy, Cystic Fibrosis

(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.

The Journey to CF (Part 2)

by Sarah | Mar 16, 2009 | All About Kennedy, Cystic Fibrosis

(Part 1 is here)

(Sorry for the delay…my host has been really cruddy the past couple of days and posting this second part has been difficult because it has been SO SLOW.)

So after two months of barky coughing, we finally got Angel into the pulmonologist.  She did a thorough exam, listened to our story and ordered x-rays and a bronchoscopy. 

The following Friday we reported to the PICU at 5:00AM.  Angel was prepped for her ‘minor surgery’ and her nose was sprayed with a numbing agent.  We were then shooed from the room and into the PICU waiting room where families slept and silence reigned. 

Hushed undertones of inane conversation crossed the table between us, our hands clenched together belying our internal fears.  The lights started flickering on, the sequestered families started waking to decide who would go steal a shower first.

Still we sat quiet, our forced conversation disappeared as we watched the faces that coule be ours depending on what happened face a new day.  The strained lines, the forced normality of conversation that had been ours.

The nurse arrived finally with a smile and told us we could go back and see her – that she was awake and feeling ornery.  We leapt to our feets and rushed back to the room, scooping our precious Angel into our arms and holding her close and tight. 

The pulmonologist had her wonderfully calm and warm smile, letting us hold her for a few minutes before she started in on the results.

She showed us images from the camera and told us that Angel’s Adenoids, Aretnoids and larynx were all swollen.  Then she showed us this barriers of mucous that lined the path to her lungs.  She said that this could mean many things, including Cystic Fibrosis – but she believed it was still the mucous of baby asthma.

She was so reassuring as she sent us off with a schedule card for blood tests and “just as a precaution” a sweat test. 

We went into the Outpatient Center of the hospital, and the blood work was horrifyingly terrible as it always is when they stick needles into a child’s arm…and then it was time for the sweat test.  The tech was so wonderfully sweet and kind as she cleaned our tiny babie’s arms and put on the medicine – followed closely by two electrodes.

We sat for five minutes as the machine buzzed, sending waves of electricity through Angel’s skinny little arm.  Then her arm was wrapped up in layers of wrap and we were sent out to play with a little timer set for 20 minutes.  By the time we got back to the room, our nerves were raw.  Even though we’d been assured that the pulm truly thought it was baby asthma…the thought of the words ‘Cystic Fibrosis’ sent chills down our spine.

The super-nice tech frowned as she peeled off the wrap to reveal the litmus paper on the arm, “Oh my, this little one doesn’t sweat much, does she?”  She pondered whether there would be enough sweat to even test…and carried it out of the room.  We sat silently, looking up in surprise when she returned and handed us a call-back card.  Apparently we had to wait until 4:30 for the results.

I headed home, and Archie to work.  Our nerves were wrapped up tight, but we suffered silently until 4:30.  I called the number with shaky hands, a deep seated dread filling me.  I punched all the numbers and the computers automated voice said the words I’d been longing to hear “Your test results are normal.” 

I heard nothing else, hanging up and calling Archie in relief.  We cheered our escape from such a fate, cried happy tears and went back to our days with lots of planned hugs for Angel.  We’d lived through our short journey to answers…our relief was immense….

And short lived.

An hour later Archie had come by and was playing with the kids – and the phone rang.  I was surprised to hear the pulmonologist on the other end of the line, “I know the automated system told you to call in, but I didn’t want you to have to wait.”

My heart stopped beating for a full ten seconds as I stammered, “Uh…no…the…the computer said the results were fine.”

“I’m so sorry, but it was wrong.  We’ll have to look into why it told you that.”

I met Archie’s eyes across the room, I knew tears were starting to fall, and that I wasn’t breathing.

“I wanted to call you personally because I didn’t want you to panic.”

Too late.

“But the test results showed elevated sweat chloride levels…”  The rest of her comments were a blur until she said, “Cystic Fibrosis.”

What had been a short journey into answers was about to get a lot longer – and impossibly more frightening.

The journey to CF (Part 1)

by Sarah | Mar 9, 2009 | All About Kennedy, Cystic Fibrosis

Two years ago right after Valentines Day was a rough time for our family.  Archie and I had separated – he was living with a friend.  I was just starting to experience life as a single mother to three children. It was for sure known that Riley was special needs – she’d been seeing a specialist for months for her FTT.  He’d said the words “Russell-Silver Syndrome”, and we were beginning to look into autism.  We had just recently started Angel in Early Intervention because of her muscle tone – although we didn’t know the depth of that yet.

The day after Valentines I posted on the mommy forums I visited asking when it was appropriate to call a doctor for a cough.  Angel had developed a cough, very light and dry – but it was becoming more barky.  My first two kids hadn’t ever had a cough, so I didn’t know what to do.

A few days later I took her in.  The ped said it wasn’t in her chest, and not to worry.  Three days later the cough was worse.  I took her back in and we started with regular breathing treatments (every 4 hours) using albuterol.

For a month we went on that way.  Breathing treatments, doctor’s appointments.  We tested for Whooping Cough – we treated for asthma…we had no idea what was making our previously happy baby so miserable and coughy…

After a month of coughing without explanation our pediatrician referred us to a pulmonologist.  If anyone could find the cause – it would be her.

To be continued….