It will have to be the 4th time

Well another dry run.   

The circumstances for today were a bit different from the past.  The donor was a younger person who was on life-support but still had brain function.  In most cases the donor has no brain function and once taken off life support they can harvest the organs.  In this case, once the donor was taken off life support they needed to wait for the patient to die. The patient has not past and the doctors are concerned that the lungs will go through too much stress and thus call it off.

We will go home this evening and wait again for the phone to ring.

Thank you all for the support.  You are all amazing!!!

Love the Zs

3rd Time may be a charm

Hello all. As we came home from a sunny baseball practice we arrived to Laura on the phone with UCSF. Unlike past visits this was super stat and we were told to come to the hospital ASAP.

10 minutes ago Laura went into surgery. There is still a chance that this ends up a dry run but we are now farther along than ever before.

Thank you all for the support thus far. We could not have made it with out you. I will continue to keep you all up to speed as we know more.

Happy Holidays to all.   

Cutting to the chase, we are back on the list!

After a month on the new heart medication, Laura went back for another round of tests.  The goal was for her heart to increase in strength and the new medication should have promoted that change.   The results of the tests were flat. No increase in function but no decrease either.  Given the results the next step was to increase the dosage and wait another month and then test again.  

Last week Laura meet with the head of Cardiovascular at UCSF for a number of tests. His thoughts are that the heart is strong enough to endure a lung transplant and that it will thrive will more oxygen from new lungs. Great news.

They are going to make some changes to current meds and look at how they can manage Laura's diabetes, believing that that too could help her heart. 

So now we wait again.  We can't thank you all enough for your thoughts, prayers and well wishes.  The little things that are left at the house, help with Everett and in general positive support is unbelievable.  Many in our situation do not have this support and it is hard to imagine going through this alone.

We hope you and your families have a wonderful Christmas/Holiday and glorious New Year celebrations. Wishing you all the best in 2016!  

Love the Z's

Another bump in the road

Time for sharing :)

While at the hospital for Dry-Run 2 the doctors had some concern about a previous echocardiogram.  The results showed some deterioration of Laura's heart performance.  After speaking to the doctors at UCSF and while we where there, Laura completed another normal echocardiogram as well as a thoracic echocardiogram. Given the size of Laura's lungs and their condition it was difficult to determine any definitive results from those test.  The next step, more tests.

Last week we went back to the hospital and Laura had a MUGA scan. A MUGA scan is a multigated acquisition scan that creates video and still images of the ventricles to check whether they are pumping blood properly. She also had a left and right heart catheterization procedure to view inside the heart.  The results of theses test were conclusive.  

The good news is she has no blockages and the heart is clean. She has however lost some function of the heart and is classified as mild to moderate degradation of function.  This resulted in her being de-activated from the transplant list. A tough mental blow.  After 2 dry runs we thought we were close and this felt like a huge step backward.  

The next step is to start a set of beta blockers, Carvedilol and Losartan to help improve the pumping function of the heart.  Normally they re-test 2-3 months after starting the drugs, but in Laura's case they will do another echo in 1 month, to try to get her back on the transplant list as soon as possible. 

Beyond this Laura has felt pretty poor, coughing for the first 4-5 hours of each day and will go back on IV antibiotics tomorrow.  With the ORKAMBI it has been almost 5 months since she was last on IV, so her body has had sometime to recover and hopefully the results will be longer lasting and help control the sputum production.  

The typical route for IV is 3 weeks and then she generally feels better for a period after. Since this is the first time while on ORKAMBI it will be interesting to see how well it works and if the benefits are longer lasting.

As always thank you for all of the notes and positive thoughts.  Hope you are all well.

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.

A dry run

Well we just found out that this was a dry run.  The lungs were too damaged due to the impact of the accident.  The fact that we did receive the call is an of itself good and means we should receive another call soon.

We all just went through a crazy roller coaster and your thoughts, comments and prayers made a huge difference.

Thank you all for the thoughts and support.