Sigh.

Anya wanted me to write an update.  Where do I start?

Well, we got married!  July 23, 2016.

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It was hands down the best six hours of my life!  Our life.

Months and months of planning, from the DJ’s playlist to the kid’s shoes to the color of the hydrangeas…we got it done.  And when I say “we”, I really mean Anya, and of course our awesome wedding planner, Heather.

People kept telling me that the day would be a blur, but I didn’t believe it.  Sure enough, it was a blur.

Also, I really thought that I was going to be nervous, but when the time came, my nerves went away because everything felt so right.  I married my best friend.  My partner in crime.

I couldn’t have dreamt of a better day, but I knew deep down inside that Anya wasn’t feeling too great.  She was having trouble breathing the days leading up to the wedding.  It was on my mind all day.  We both knew that she should’ve been in the hospital 3 days prior.  Instead we decided to wait until after the wedding to take her to the hospital.

After the hoopla died down around 11pm, Anya’s parents took us back to our house.  We were supposed to spend the night in the hotel, but Anya just wasn’t feeling up to it.  On the car ride home, Anya was dry heaving…partly from exhaustion, but mainly from the lack of oxygen over the past few days.

Before we went to bed, I asked Anya if she wanted to go to the ER that night or wait until the morning.  She said “morning”.  By 2:30am, she woke me up and told me to call the ambulance because she had a splitting headache and she wasn’t feeling right.  She hadn’t slept all night.

The ambulance promptly came and whisked us away to the ER at Inova Fairfax hospital, our home away from home.  By that time, Anya was hypoxic — her lungs weren’t processing enough oxygen causing a toxic buildup of too much CO2.  When we got to the ER, Anya was pretty much out of it.  It was a scary night.

The ER team decided to put her on bipap to help her remove the unwanted CO2 and replace it with oxygen.  It kinda worked, but not fast enough, so the transplant team made the decision to intubate her the following morning.

It took about 24 hours, but being intubated and on the ventilator helped rid her body of the bad CO2.   This all happened on the day after the wedding.

By the third day post wedding day, she looked a lot better.  She was awake and alert, unable to talk, but physically, she looked a lot less distressed.

Fast forward 7.5 weeks, Anya has been in the ICU this entire time.  We joked that this is exactly how we envisioned our honeymoon to be!  She’s currently fighting a mycobacterium infection, pseudomonas, and chronic rejection.  She’s been on a ventilator this entire time.  She’s completely awake and alert, eating and drinking (past few days), and walking but it’s just that her lungs aren’t doing great again.  Yeah, it sucks.  Bad.

Walking

We’re still hopeful that she can get off the ventilator at some point in the near future.  She’s been walking around the ICU floor almost daily.  She got up to a mile last week.  Eighteen laps with her ventilator in tow.  I’ve never seen anyone with so much heart.  Physically, she is pretty strong, but every time they take her off the ventilator, her CO2 levels elevate.  Her lungs just aren’t strong enough right now.

I’m not quite sure where we go from here.   What I do know is that Anya needs some support right now….Thanks for reading…

Sup?

 

 

 

“Oxygen is Overrated”

I went to my niece and nephew’s swim meet this morning (Go Sully!), and I saw this sign hanging by the bleachers.

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Typically, I let Mike take the reigns when it comes to writing the blog posts, but this sign really struck a nerve today. These kids, swimming their little hearts (and lungs) out have no idea how lucky they are!

Officially, it’s been 368 days since we arrived back to Virginia from our time in Houston. Although so much has happened over the last year, some things still remain the same. Unfortunately, airway stenosis has continued along with receiving several bronchoscopies over the last year – both in the ER and the bronch suite – that have attempted to open up and expand my airways through the use of flexible and rigid bronchoscopes. Typically, through the use of balloon dilation and cryotherapy (a form of thermal tissue ablation, i.e., the application of repetitive freeze/thaw cycles that cause tissue damage and destruction), my airways still only remained patent for two weeks, on average. As Mike mentioned in his last post, Inova Fairfax hospital has an amazingly talented and tenacious interventional pulmonologist who decided that bi-weekly bronchs, which sometimes can take all day and require anesthesia, are no way to live a life. In February, he made the decision to try a semi-permanent fix by placing stents in the openings to both lungs. In his plan, Dr. Mahajan would place small stents in either lung; after six weeks, those stents would be replaced by larger stents; in another six weeks, the left side’s stent would be removed, and the right side’s stent would be replaced with a silicone stent that will remain within the airway for six months to a year. As with every procedure, there are some risks, and the risks associated with the stent placements have been significant.

Three days following the first stent placement, I was diagnosed with pneumonia, and since the initial placement, I’ve been infected with Aspergillus (mold) and pseudomonas (bacteria) and hospitalized three times. Although both types of mold and bacteria are readily available in the environment, through the soil, water, and plants, and typically do not cause infection in otherwise healthy people, they do cause complications in immune compromised patients. We were informed, prior to the stent placement, that the chance for infection was increased because secretions are difficult to clear when a stent is in place.

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I was given strict instructions for airway clearance, which I had/have been doing since my second transplant, including daily nebulizers to help open the airways, keep them opened, and thin secretions. Though continuing my nebulizing regimen on a twice-daily basis has caused no great burden, it hasn’t seemed to help in my fight against infection. As of right now, I am infected with Aspergillus and am taking inhaled Amphotericin to fight it. I suppose nebulizing Amphotericin is better than the alternative, which is IV antibiotics. At this point, I’m not sure my battered stomach can handle any more!

With all of that said, living life is pretty sweet, even though I often need to drag my oxygen tank along with me for the ride. In between hospital stays, transplant appointments, bronchoscopies, and various other doctors’ appointments, I do manage to find some time for those things I enjoy. I spent spring break in Florida with my sister, Jocelyn, and my niece and nephew, which was fabulously fun (minus the third degree burns I received on my feet (thank goodness that’s all that was exposed on the beach)), and I’ve spent the last few months busily planning our upcoming wedding! I can’t believe that I will be Mrs. Michael Huie in three weeks! What a lucky girl I am! And to all those kids who take oxygen for granted, I am eternally envious!

Happy 1-year Lungversary to Anya!

I can’t believe that it was exactly one year ago today yesterday that Anya got The Call for new lungs and was transplanted!  It really does seem like a long, long time ago.  I guess mainly because we’ve done a lot since then.

I’m happy to say that things are still going pretty well since transplant.  We’ve been home for 6 months now.  During that time, Anya’s had quite a few doctor’s appointments with her new (old) transplant team at Inova Fairfax.  Every doctor on the team wanted to get an assessment and see how she was doing since the last time they saw her before her 2nd transplant.

When we left Virginia to go to Houston last August, Anya was in the Inova Fairfax ICU fighting off an infection.  At that time, I’m not sure if any of the doctors thought that they’d ever see her again because she was in pretty bad shape, but SURPRISE….

The entire transplant team at Inova Fairfax played such a huge part in getting Anya over to Houston Methodist for an evaluation.  After visiting so many different transplant centers over the past few years, it made us realize how lucky we were to have such a wonderful team in our own backyard.  We can’t thank them enough.

So, how’s Anya doing?  The main problem that we’ve been dealing with post-transplant is her airways narrowing.  Bronchial stenosis is the official term for what she’s dealing with.  Due to the trauma and lack of blood supply to the new lungs during the transplant process, the airways sometimes get kinda flimsy in certain areas due to the prolonged healing period.  It doesn’t happen often.  I think it happens in about 15% of transplant patients, but one of the treatments for bronchial stenosis is a procedure called a balloon dilation (or dilatation) where the doctor puts a rigid bronchoscope into the airways and dilates the airways back up to their normal size.

Anya has had a bronchoscope, on average, once every 2-3 weeks since transplant.  We counted and she’s had roughly 30 bronchoscopies this year alone.  The narrowing of her airways causes several problems, like the inability to clear mucus from the lungs, which leads to infection and reduced lung function.

Luckily, the Inova Fairfax transplant team works with an awesome pulmonary interventionalist that deals only with these lung related issues.  He joined the transplant team some time after we left for Houston.

For the past 6 months, she’s been getting balloon dilations from this doctor, but her airways would eventually close again a few weeks later.  One thing that the doctor noted was that she continued to have an abnormal amount of mucus inside of her lungs, even though the cultures showed very little fungal and bacterial growth.

In early November, after one of these routine bronchs, Anya developed pneumonia and required a 6 day hospital stay.  It was rather scary going back into the hospital again and going on oxygen for about two weeks.  Especially after her lung function dropped down to about 41%. But after another bronch 2 weeks later, things started getting better.  So over the past month and a half, after her hospital stay, the team has been more aggressive with the treatment of this lingering infection (staph and aspergillus…two common bacterial and fungal infections in CF patients post transplant).

As of this past Monday, it looks like the infection is finally clearing up and her airways are maintaining a semi-normal size.  The bronch doctor told us that we don’t have to do another bronch for 6-8 weeks, which is a record.  Anya’s lung function is currently at 61%.  Her highest lung function post 2nd double lung transplant was 72%, so we’re getting back to a nice number.  And no more appointments until the end of January!  Woohoo!

Have a great new years everyone!

A picture says a thousand words….so here you go: