Listed – Weeks 3 & 4

We had our first dry run last Saturday (December 6th). Our transplant nurse practitioner, Tiffany, called around 1pm and said “we have lungs!”. She told us to sit tight until they received some more information. As if we were going anywhere… but we did sit tight, and around 2:15pm, Tiffany called back and said that it was only a single lung and we needed a pair, so it was a no go.

It was pretty disappointing, but at the same time, it was very encouraging that we got our first call only two weeks after being listed. It’s been a very nerve racking several weeks knowing that Anya could get “the call” at any time now. She could get the call tonight and be in surgery by tomorrow morning…it could be that fast.  Scary, but I know that the transplant is the ultimate goal.

Rick and Kim, two the best physical therapists, helping Anya walk

Rick and Kim, two of our favorite physical therapists, helping Anya walk

Anya’s been doing well.  She’s been walking at least 5 days a week.  Yesterday, she walked 5 figure eights around the floor, which equates to about 2720 feet and she only stopped twice.  She also walked two laps in the morning, so seven laps total for the day was the most that she’s walked in one day since we’ve been here.

Her recent sputum cultures didn’t grow out anything significant, except for a very minute trace of CMV, which the team was not too worried about because the sputum cultures come from the trach tube, which could be contaminated.  There has been no other positive mycobacterium abscessus culture since the July.  In terms of infection, she hasn’t produced much mucus lately, which is a good sign that the infection is under control.  She’s still on a pretty strong antibiotic called Zyvox and I think, Bactrim, to help with the abscessus.  She’ll most likely be on these two drugs even after transplant, for a good 6 months to a year.

Anya and dad

Anya and dad

Her weight has been pretty steady over the past few weeks.  Her weight went up to around 88 pounds a few weeks ago, but we think some of that was water weight.  The team has been giving her Lasix, which is a diuretic to help reduce the swelling.  Yesterday, Anya weighed 84 pounds, which puts her BMI at 16.4.   She’s been hovering around this area for a few weeks now.  We switched her tube feeding formula to a less caloric dense formula yesterday….1.2 calories per ml versus 2.0 ml in hopes of fixing her diarrhea issues.  Instead of getting 3,120 calories per day, she’s getting 2,448 calories now, with the hopes of retaining and digesting more of those calories.  We’ll see how that goes.

donation service area - texas - lifegiftOverall, things are going well.  We’re just waiting for a good set of lungs now.  I just checked the OPTN website and Anya is now at the top of the list for her blood type (B) in our donation service area (DSA), which is LifeGift Organ Donation Center.  Our DSA covers all of Houston and some of the surrounding area, so I really don’t think that it will be too much longer now.  I found this picture of our DSA:

BTW…here is our current address for anyone wanting to send christmas cards or anything…some of you have asked for it:

Anya Crum, 6441 Main Street Room 528, Houston, TX 77030

Listed – Weeks 1 & 2

Not much has happened over the past two weeks.  Our dog, Remy, ate an entire box of cookies, which put him into a food coma for 2 days.  Our other dog, Rudy, somehow got his lower canine embedded into his lower lip, which resulted in a trip to the vet.

Photo Oct 30, 8 00 12 PM

Definitely guilty.

Photo Nov 21, 4 11 22 PM

Get your hands out of my mouth!

Sleepy head.

I’m on drugs.

Anya’s mom and I spent Thanksgiving with Anya in her room.  Jocelyn and Patrick sent us Thanksgiving dinner in a box from Omaha Steaks (thanks for that!).  Becky, Lauren, Stephen, and Luke stopped by to say hi on Thanksgiving evening.

Photo Nov 27, 6 00 29 PM

Thanksgiving dinner

Photo Nov 27, 6 52 29 PM

sorry, i didn’t get Luke in the picture

Not much else has really happened since Anya got listed two weeks ago.  We’re just waiting for “the call” now.  As of November 28th, there is still only one other person with the blood type B and an LAS of greater than 50 in our donation service area, so Anya is most likely second in line right now.  She may even be first in line, depending on the size of the incoming lungs.

Anya is continuing to get stronger every day.  The occupational therapist comes in daily to do breathing and weight exercises for about 30 minutes.  The physical therapists take Anya on walks at least once a day, but usually twice a day.  Anya’s been doing great on her walks lately.  Yesterday, she walked 1,200 feet without stopping to rest.  It’s probably the best she’s done since we’ve been here at Kindred.  She walks anywhere from 900 feet to 2,500 feet daily, the length of 3 to 8.5 football fields.  Post-transplant success is directly related to physical strength pre-transplant, so that’s why every doctor has been stressing the importance of exercising daily.

Anya’s weight has been steadily increasing too.  She weighed 84 pounds yesterday.  Last week, she was 88 pounds, but we knew that something was off because she was putting on a lot of weight, quickly, so we told the doctors and they prescribed a diuretic (Lasix) to help reduce some of the water weight.  Her arms and legs were swelling a little bit.  We figure that her real weight is probably around 80-82 pounds, which is great considering where she was just a month ago.

Anya’s feeding tube twisted inside of her stomach last Wednesday, so the GI docs had to replace it.  This is the second time that it’s happened.  The first time, the entire tube came out, so they had to wait for the incision to heal before replacing it.  This time, the tube was still in place, so the docs just had to pull the old tube out and thread the new one back in, which was much less invasive and much less painful.  Unfortunately, Anya lost about 5 days of weight gain during that time because it happened the day before Thanksgiving and there were no doctors able to do the procedure until the following Monday.

Anya’s hemoglobin fell below the critical level (7.0) a few weeks ago.

“Hemoglobin (Hb or Hgb) is the protein in your red blood cells that carries oxygen. A low hemoglobin count is a below-average concentration of the oxygen-carrying hemoglobin proteins in your blood.”

— http://www.mayoclinic.org/symptoms/low-hemoglobin/basics/definition/sym-20050760

The low hemoglobin is most likely the result of the nutritional problems that we’ve been dealing with.  The doctors are giving Anya intravenous iron to help with the production of the red blood cells.  It’s going to take a few weeks, but hopefully it’ll help with the blood problems.  The doctor explained to us that her levels are not at a “severe” level yet because she’s been hovering around 6.8 to 7.5 depending on how they draw the blood, but he wanted to see what they could do before it got to a bad level (below 6.0).  The alternative would be to have a blood infusion, but the problem with that is that antibodies could build up causing post-transplant rejection problems, which is something the doctors want to avoid.  The doctors are keeping a close eye on it, but as of right now, it’s not a major concern.

That’s really about it for now.  Anya’s doing pretty good.  The good days seem to be way more frequent than the bad days now.  Being here at Kindred has really been the best thing for us.  I feel that Anya is the strongest that she’s been for the past 3 months.  Just waiting for a good set of lungs now…  I have a feeling that they will be coming soon…

Update

Anya’s still not listed yet.

The transplant surgeon came in last Tuesday and cleared Anya for transplant with some stipulations:

  • keep BMI above 15
  • walk daily
  • trach collar daily

Anya weighed 80.4 pounds on Friday, which put her BMI at 15.7.  She’s been walking at least 880 feet every day, except Sundays when the physical therapists are off.  As far as the trach collar, Anya’s CO2 levels went up to 85 last week (normal 35-45), so she’s been on vent support (SIMV) since Friday.  I talked to the pulmonary team about this and they told me that her physical strength (walking) is more important than weening off of the ventilator, so they are not as concerned with being on the trach collar daily.  We are still going to try to get Anya on the trach collar for about an hour or two daily, but we need to make sure her CO2 levels are lower than the 85 that we saw on Friday.  Being on the trach collar is important because it helps build up the muscles used to breathe.  The downside of the trach collar is CO2 retention.

The liver team came in last Wednesday.  They reviewed her liver biopsies from Fairfax (June 19th) and Duke (March 28th), along with the pathologist reports.  The only thing they were missing was the gradient numbers from her latest liver biopsy.  I sent them the medical records that I received from Fairfax Inova before I left and they were happy with those numbers (6-7 mm HG).  Anya has stage 3 fibrosis in certain parts of her liver (stage 4 is cirrhosis).  Cystic Fibrosis is known to cause damage in other organs, like the pancreas, liver, and intestine.  Anya’s pancreas and intestines are fine, however, her liver was the main concern and reason why Duke, UPMC, and Cleveland Clinic rejected us for a 2nd transplant.  The liver doctor over at Houston Methodist described her liver as having “spotty cirrhosis”.  Because the two liver biopsies were inconclusive for full blown cirrhosis, the doctors had to use the gradient numbers to determine if she did indeed have cirrhosis or not.  Based on Anya’s gradient numbers, the liver team believes that her liver will be fine for transplant.  The liver team fully cleared Anya for transplant on Wednesday.

So, what’s the game plan for now?  Well, the good news is that Anya is fully cleared for transplant by all teams.  Our transplant coordinator sent all of our paperwork to the insurance company on Friday to get proper approval for transplant.  Lung transplants usually cost between $500,000 to $1,000,000.  If all goes well, then we should have insurance approval by the end of the week.

Once approved, then our transplant coordinator will send our application to the United Network for Organ Sharing (UNOS) to get Anya officially listed for a lung transplant.  Once listed, UNOS will give us a Lung Allocation Score (LAS) to determine how high Anya is on the list.  The LAS ranges from 0-100, with 0 meaning that the candidate is not yet eligible for transplant, and 100 meaning that the candidate needs a transplant today.  Although Anya is not officially listed yet, the nurse practitioner told us that Anya’s score will most likely be around 60.

I’ll share some more information on median wait times, lung transplants performed, and transplant candidate numbers after we get word from the insurance company.  Just FYI, the transplant will most likely not happen immediately.  Being listed is just the first important step in the process.

<deep breath>