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. 

Update on surgery

Just a quick update.  The Dr just stopped by talk about next steps.  They are still reviewing the lungs.  The donor was in some type of high impact accident and they are concerned that the lungs maybe damanaged.  They will visually inspect the organs in the next 20 min or so and that will determine if the transplant is a go or dry run.

At UCSF

Thank you all for the great comments and notes today.   I will try to provide timely updates as we go through the next 48 hours to keep everyone informed.

We arrived at UCSF this morning and where in a room by 8.  The team is great.  We just finished with the pre op review by the transplant nurse.  The UCSF team has gone to the donor hospital to look at the lungs.  (The donor is in the bay area but we can not be told where or any details about the donor, why they are donating etc.)  There is a 1 in 4 chance that this will be a "dry run". If so we will go home this afternoon and wait for another call.

Going on the assumption that the lungs will be a go, we are scheduled for 1 pm surgery.  The surgery typically last 8-12 hours. It can go longer and is never shorter.  As the new lungs are en route, the will make a clam-shell incision from one armpit down below the breasts, cut through the chest bone and then back up to the other armpit.  When the replace the lungs they will do the left one first then the right.

After the transplant they will wire the bone back together and then suture 2 to 3 layers of skin, gluing the final outside layer.  All suture will naturally disintegrate.

Laura will wake up ventilated and restrained. The natural instinct will be for her wake up a pull out the tubes.  Not the best idea. Along with the ventilating tube she will have multiple chest tubes inserted as well to assist with drainage.

The next 5 days will be spent in ICU and then another 7-10 days in a room.  Obviously this can and is extended often based on circumstances.  

The next step is to meet with our surgeon, Dr. Jasleen Kukreja, she is the best in there is so we could not be in better hands.

As we learn more throughout the morning, I will let you know.  

XOXO

The Call

Good morning everyone.  We received the call this morning from UCSF and are now going through the admission process.  We will keep you all posted as we learn more.  

A better July

Hello friends,

After a tough June we finished the month ending IV antibiotics and entered July with hope. After IV there is usually a window of better lung function and easier days.  This was the case and was a great advantage as Laura started Pulmonary Rehab.  

Laura entered John Muir's Pulmonary Rehab program with some hesitation and skepticism but with an amazing team the experience and results have been good.  The rehab is 3 days a week and ranges from 3-4 hours.  It is not easy but the effort has increased Laura's strength and endurance, both needed for the transplant.  Thank you to the team at John Muir for all your encouragement and hard work.

We also had a check-in with the UCSF team.  Laura's oxygen flow has increased and thus her transplant number increased as well.  Again, there are number of factors that go into the number but the increase in oxygen to 6 liters per min has elevated her score to 47.3.   The doctors say we are in the zone and should be ready for a call at any moment.  We are ready and waiting.

The John Muir team recommended a new treatment call the Vibralung Acoustical Percussor.  This is a crazy acoustical inhaler that splices sputum from the lungs.  It is house/techno meets health.  Here is a view  If you have not guessed Laura is below the average age in ALL rehab classes.

Toward the end of the month we also met with the team at Kaiser for the normal checkup. The results were ok, lung function is still at 26%.  No decrease is great news!  We did learn that Laura qualified for Orkambi.  Orkambi is a combination of Kalydeco (which is a miracle drug that can make CF disappear in younger patients) and lumacaftor.  The goal of both is to make the CF gene disappear.  Pretty amazing work by the bio-geniuses.  We do not have any expectations that Orkambi will show drastic improvements but hope for a slight increase in lung capacity. 

As we enter into August we hope for the call soon and look forward to celebrating Everett's 8th birthday.

I can't say enough about the effect of your thoughts, prayers, emails, txts and calls.  It is really great to hear from all of you and it makes a difference.  We have been taken completely by surprise on the reach of our story.  There are a number of people that are one degree away and share a very similar CF journey.  The blog and video have exceeded 8,000 people in reach in 35+ countries.  Knowing that you have that many people in our corner makes us feel very blessed.

For our local support network, you are amazing. We just can't say enough.

Enjoy the last weeks of summer and hope to post something soon.

Love

The Z's

The Summer Cold

Well we thought we were making good progress and then the house caught a summer cold. For what ever reason the cold this season packed a punch.  After a day or two we knew that this was not the cold that was going to go away and made the call to the doctors.  After a few conversations, they put Laura back on IV antibiotics and increased oxygen to 4 lpm (liters per minute). This round will be a 3 week track of IV Meropenem and oral Cipro.

After the cold went into her chest, the coughing attacks were just brutal.  Not only are they hard deep coughs they last for what seems like forever. 10,15,30 min attacks that sap whatever energy and reserves Laura has.  

This week has been one of the hardest ever.  It is a battle of wills.  Laura's mental strength vs. her bodies consistent attacks on itself.  The constant fight takes its toll as at the time it seems like it will not get better.  It is a 24/7 fight that slowly beats you down and makes you wonder if the body will make it to a transplant.

Today is day five of this round of antibiotics.  We used to see a dramatic effect of IV within a day or two.  Over the last year as the disease has progressed the results are delayed and diminished.  Hopefully as the cold subsides the IV will take effect and in another week or so we will be back on track.

Thank you all for the wishes, prayers, emails, comments...etc. We warmly and gladly accept them all and they do fuel us.  

Love The Zs'

Dr. Apt and One Republic

Well we are almost done with our 3rd week on the list.  The ring of the phone no longer makes our hearts stop and we are getting back into our normal routine.  Laura did have a Dr. Apt and her lung capacity is now 26%.   Not a surprise but always a bit unnerving to hear.  Go for a walk and breathe though a straw, challenging to say the least.

All candidates have to be very aware of their weight.  A patient may lose 15% of their body weight during/post surgery, so they have to maintain the proper weight prior.  Laura has always had a tough go at this and the only way it has been possible is via tube feeds.  It is hard and has side effects but she is diligent and the effects are great. She is now up to ... (like I would say) The doctors are happy :).  

No big tests in the coming weeks, so back to the wait.  Many of you have asked if you could share the link to the documentary, please do.  We have learned that many co-workers and friends of friends have relatives or friends that have CF.  Laura and Amy wanted to make the documentary show parents and young adults with CF that a  full life with CF is possible. Share away!

Now onto pop music.  Obviously a logical transition.

As I no longer wait for Friday Night Videos or watch videos for that matter, I missed One Republic's latest.  Laura asked if I had heard the song I Lived.  By title I was not aware of the song.  After hearing the track, I was aware of the song but it was usually heard in a car of kids in route to some practice. I had not listen to the lyrics or seen the video.

Laura suggested that we watch the video, so Everett, Laura and I did that.  I have to give One Republic full props for the theme of the video.  I do not want to say too much more, so the link below is to their video.  AWESOME!

I Lived - One Republic

If anyone knows the band, we would love to know the back story.

Thank you again for all of the support, well wishes, prayers and thoughts. It is amazing how lucky we our to have you all.

Cheers,

Breathe in Life - The Documentary

Breath in Life one mother's journey

As many of you are aware over the past 10 years Laura and Amy worked on a documentary about CF.  Throughout the years the focused changed and the film became a short story geared to our son Everett.  The film debuted at the California Independent Film Festival in 2014 and was nominated for Best Documentary Short.  

We hope you enjoy the film.

We Made The List!

Hello everyone.

Given all that is in the works we wanted to create a blog that would allow us to communicate throughout the transplant process with our friends and family.  We also wanted to allow others that may be in similar situations to understand our journey and hopefully that will make their journey easier and less scary.  

So that said…We Made The List!

Laura was officially placed on the list for a double lung transplant last week.  It was the culmination of 5 months of rigorous testing.  She had to endure every test you can image. The reason for these tests is to make sure that she has no other diseases that could possible affect her recovery after the surgery.  She was cleared and that was a major hurdle.

Now that she is on the list we hurry up and wait.  We have our phones ready 24/7 and answer every call waiting for UCSF to tell us that they have a matching organ en route.  Throughout this process we must stay within a 2 hour commute of the hospital and be ready to get there ASAP.

Lungs are given to people on the list based on their Lung Allocation Score (LAS), along with matches for lung cavity size, blood type, and antibodies. Laura scored high on the list as an outpatient (someone not hospitalized while waiting). Which means we could receive a call at anytime.  Based on her score a reasonable expectation for a transplant is within the next 3 - 6 months.

Once we do receive the call the adventure begins.  We recently learned that about 20% of the time the call is a “dry run”.  This means that between the time they harvested the organ and prior to the transplant something happened to the organ and it is no longer considered good enough to transplant.  We can only image the emotional rollercoaster this puts you on but much better to be safe.  This experience happened to one gentlemen 5 times.  So you may see a post that says we are on the way and then a few hours later hear something different.

When the transplant does happen, Laura will be in surgery for 8-12 hours.   (If you are truly interesting in the surgery, Youtube has some interesting videos.)   Once the surgery is complete, she will be in ICU for a week and then the hospital for a second week.  Hospitalization could be extended based on complication but 2-4 weeks is standard.

Within 24 hours of the surgery they will have Laura up and walking and that starts rehab. Pretty incredible.  Once she is ready to leave the hospital we will need to live in the city for the next 6-12 weeks.  This is where we need some help.  If anyone knows of a rental unit (1 or 2 br, kitchenette –in law type place) please let us know.  We really can’t use an extra room as the environment has to be as controlled as possible.  We are speaking with the hotels, looking at AirBnB….etc. but it is difficult since we do not know what the timing is.  If you have suggestions just send me an email.  The reason for moving to the city is to be close to the hospital as there are daily visits to assess her recovery, watch for rejection, infection and learn about all of the new meds.

As you can image, emotions are everywhere.  It is time for this to happen but it is one of the most complex surgeries to be done and that is scary. UCSF and our team is #1 is the U.S. we could not be in better hands.  We also have all of you as an amazing support group that has helped us get this far. Thank you thank you thank you.  Our appreciation cannot be expressed enough.

So now we can all wait together.  As soon as we hear, we will let you know.  As things change and events happen we will post them to this blog as well.   For those of you who have asked about the movie it will be posted here within the week.  Feel free to share.

Cheers