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.