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'