Blogging about life in Minnesota, raising our six kids with Down syndrome while battling Breast Cancer.

Be the kind of woman that when your feet hit the floor in the morning the devil says, "Oh shit! She's up!"

Sunday, October 04, 2015

Two words I hate: Cricopharyngeal Achalasia

I need you to go back on my blog several years. Six years, to be exact. You'll want to read this post, particularly the last two paragraphs. Then come back here.

Key word: Cricopharyngeal Achalasia = CA

I'm not sure what I thought six years ago when we sat in the office of Dr. Nurko at Boston Children's hospital. I remember him telling me, "Well, she's doing really well right now..." and we discussed plan of care.

About a year ago I noticed that Angela was having more episodes of food getting stuck in her esophagus, and just some subtle changes to how she was swallowing. Things like tucking her chin, which I knew she was doing to protect her airway. I started getting my mind prepared for the fact we would need to head back to Boston soon.

Then in April Angela had a very bad case of aspiration pneumonia. Actually her lungs didn't look too horrible on x-ray but she was having a tough time managing her airway. She had to be on oxygen for several days and her blood pressure kept tanking. Getting to Boston was discussed, but the doctors at St. Paul Childrens Hospital had never seen a patient with CA before. Gee, surprised surprise! NOT! Nobody has seen it. There are currently only three kids in the entire USA right now who are living with CA.  Angela is the oldest. Two are treated by Dr. Nurko in Boston. There was another person with DS who had the disease, but at the time we saw Nurko 6 years ago she had already passed away from complications of the disease. If I remember correctly she was 14 or 15 years old.

Let me tell you a tiny bit about CA.

There are three types:
1): acquired CA: Usually happens to elderly stroke victims and is not progressive. Most people with acquired CA die from complications from either the stroke that caused it or aspiration pneumonia. Acquired CA is not progressive.

B: congenital CA: It is not uncommon for newborns to have CA. It is not progressive, but the newborns need to have a myotomy (cut) in the cricoid muscle so they can swallow safely.

C: CA of unknown etiology. There is no known cause, and it is a progressive disease process. It is EXTREMELY RARE. It is NOT related to Esophageal Achalasia which is significantly more common. Again, it is a progressive disease.  This is where the last two paragraphs of that other post become important.

Now back to Angela's pneumonia back in April. She had one really bad bout and got over it. Summer marched on and we put it behind us. In the meantime I started working on getting Angela back out to Boston. One round of aspiration pneumonia in a person with Down syndrome isn't completely shocking. It happens and you move on.

On the August 30th Angela started coughing, which is not unusual for her. By morning her cough had changed a tiny bit. Around noon there was a shift. Her cough was sounding wet. I called the pediatrician's office who said they could get her in at 3:00. At 1:00 I put her in the car and headed for the peds office anyway. Its only a few minutes away. On the way there she said her chest hurt, and thats my indicator that we're headed for trouble. Her chest hurts because of how she's breathing. As always they put us right into a room and took her blood pressure right away. She was low, but not so bad that that she was going to crash on us. We did a chest xray and the tech showed it to me. Oh man...yuck...her lower right lobe was a mess. The doctor got all Angela's paperwork ready for me when I said, "Nope. I want her transported this time. Last time she tanked on me in the parking lot." Looking at how Angela was in that moment the doctor thought transport was a bit overkill but followed my lead anyway. Two young male paramedics walked in and Angela declared, "Oh, you're smok'in hot! I'm riding with you guys!" The paremedics seemed a bit confused that this very with it young lady was going to ambulance. I was following the ambulance when we came to a yellow light about four miles from the hospital. The ambulance made it through, but I did not. As the cross traffic started to move in front of me, the ambulance turned on its lights and sirens. CRAP! CRAP CRAP!!
I got to the hospital just a couple minutes after them. Angela's blood pressure had bottomed out in the ambulance.

Ok...that's about enough for this post, huh? I'll continue with another post so you can rest your weary eyes.



1 comment:

Cat Bond said...

Hello,

I was diagnosed with CA this year. I noticed I had difficulty swallowing around April. No pain, but there seemed to be something in the way when I swallowed. It is on the right side of my throat. My doctor sent me for a test and the medical technician and the doctor told me I had a classic case of CA. He did not know much about it but said he does not see it people my age (I am 54) - it is a condition he sees in the elderly. He did not know what causes it or if it is serious or if it gets worse. He wrote it out on a piece of paper so I could look it up on the internet. That is how I came to read your blog. I hope your daughter is ok.

Angie