Dry-Run 2

Not the best sequel but confirms we are close.  We received another call from UCSF Tuesday night explaining that there was a possible pair of lungs and that we should be at the hospital the following morning at 7.   These calls are obviously with mixed emotion, excitement, fear and concern but mostly with optimism.

When we told Everett his comments where pretty stellar, "Yes, this is the best day of my life.  My mom will get new lungs and I don't have to go to school!"  He proceed to pack his bad for 5 days in the city.  He actually packed pretty well.  Not as hard if you wardrobe consists of sport shorts, mismatched socks and t-shirts.  He did pack a toothbrush.

So yesterday we spent 14 hours at UCSF.  The team their is awesome.  A few remembered us and greeted us with open arms and did everything possible to make Laura comfortable.  The second time around was very similar to the first and seemed a bit easier knowing that it really a waiting game.  Knowing that this could be dry-run we did not have the emotional attachment to the process as we did in the first time.  

Early on the doctor did say that she did not think it would work and we both thought that this would not be the time.  At the end of the day the lungs were not in the optimal condition the doctor wanted and she did pass.  They did go on to someone else who has different circumstances.

People have asked what happens to the lungs if it is a no for us.  Typically there are a number of potential candidates in the waiting.  Based on their profile and needs the lungs would be provided to the best match.  Since Laura is younger and relatively healthy compared to other older candidates they want younger, stronger lungs for her. The lungs yesterday did save someone land were put to great use.

 Thank you all again for the great support throughout this process.  We read about people with little to no family and friends navigating this process without a network. That scenario is unimaginable to us because you are all here, thank you.

Hope the end of the trilogy finishes strong.

Cheers, 

The Zs

Still watching the phone

Hello all,

It has been 6+ weeks since the “dry-run” and we continue to wait for the next call. As we wait, Laura continues the cycle of appoints and tests.  Her last check up came in relatively the same.  Her lung capacity was at 25% just a bit below the previous test and all other numbers including her weight were good.

Shortly before the ‘dry-run” Laura was approved to try a new treatment “drug” named ORKAMBI.  CF is the result of mutations in the CFTR gene.  Those mutations can result in missing CFTR proteins or create proteins that do not work correctly.  These defects reduce the amount of chloride ions that can pass into or out of cells.  This creates an imbalance of salt and water and eventually leads to the symptoms of CF. (Better graphic to explain). ORKAMBI is intended to create a better chloride ion transfer and thus reduce the imbalance.

Since the treatment is so new, it was unknown how it would affect Laura.  Thus far it has made a bit of a difference.  She has not had to rush back onto IV antibiotics as quickly as she normally would after her last treatment.  She is also having fewer coughing attacks mid-day.  Both of these results are good.  The longer she can go in between antibiotics the better, allowing her body time to recover and gain strength.

Obviously we have no idea how long these results will be maintained but will enjoy the positive results in the short term.

Hopefully more exciting news to follow soon.

Thank you again for the support.  Emails, posts, letters and prayers are all appreciated.