Thursday, December 5, 2019

ECP Trial News



Back in July I mentioned that I had been accepted into the observational arm of a clinical trial involving Extracorporeal Photopheresis (ECP).  This is a Medicare study to show ECP works well enough that the procedure can be covered by Medicare. I was a bit disappointed that I wasn't getting the treatment, but happy to be contributing to the trial.

I am very happy to report that I was transferred to the treatment arm of the trial and finished up my third cycle of the treatment. It feels pretty awesome to be receiving a treatment that may slow my chronic rejection.

A quick recap and explanation about my rejection.  There are two kinds of lung transplant rejection, acute and chronic.  Here is a quick description of lung transplant rejection.

Acute rejection often comes on quickly, and can be usually be treated.  I had a pretty severe acute rejection about a month after my transplant. The rejection was triggered by a Coronavirus and took some pretty intensive in-hospital treatment to recover from.  I have an awesome Team, they took care of that issue, and the myriad of others that followed.

A chronic rejection may come on slower, and has far fewer treatment options. My chronic rejection was triggered by getting stomach contents in my lungs. We treated for acute rejection and performed a Nissen Fundoplication. The rejection slowed, but continued.  Once diagnosed with chronic rejection I went into the hospital for a Thymogobulin Treatment. The Thymogobulin reduced my T-Cell count from over 1600, to less than 30 cells/ul.  The Thymo did a nice job of stabilizing my rejection.

My highest post transplant spirometry included an FEV1 of over 5.8L.  FEV1 is the amount of air I can forcibly exhale in one second. My current FEV1 is around 2.0L. This puts me in Class 3 rejection.  Specifically Class 3 BOS.

From the rejection link above:
Over time, you may develop slowly worsening, chronic rejection called chronic lung allograft dysfunction (CLAD). A common form of CLAD is called bronchiolitis obliterans syndrome (BOS)...
So I'm rejecting, it's progressing (slowly), and I'm in a clinical trial of a treatment that has been shown to slow the progression of the rejection.

Extracorporeal Photopheresis is a procedure that involves removing 1.5 L of blood, separating the white blood cells from the red, treating the white blood cells with a medication then hitting them with UV light.  The whole batch is returned with a bit of saline to keep things flowing smoothly.

Here is a good explanation of the procedure and how it helps with chronic rejection.

This shows the results of an earlier trial of ECP, and why we have hope that this is going to help keep me healthy.




I've had 6 treatments in these first 3 cycles.  Only noticeable side effects are lightheadedness during the procedure, very sensitive to light after the procedure (increasing with each procedure), and tiredness for the rest of the day.

Now it's up to me to keep myself healthy during the current Cold/Flu/RSV season.

My ECP Team

Wednesday, October 9, 2019

Checking In

I sure am slacking on writing posts.  No particular reason other than not sure what to write about that's new information that might be of interest.

I am kind of excited that it's coming up on a full year since I've been in the hospital. Excited and a little superstitious about mentioning it.  About this time last year the upper lobe of my right lung collapsed several times and we never did figure out why. There is permanent damage to that part of my lung, but it has not progressed.

I went to my once every 3 month check in with the Team on Monday, and everything is stable. X-ray results indicate "Postsurgical changes of bilateral lung transplant. No cardiomegaly. No effusion, pneumothorax or focal consolidation. Stable right apical pleural-parenchymal thickening. No acute osseous findings." and all of my labs look good. The medications have been rough on my kidneys and my Creatinine levels run high and I remain well within my normal band, 1.58 mg/dl this time.


We were fortunate enough to be gifted tickets to one of the last Ranger's games at the Ballpark in Arlington. Wearing SPF 100 and brought my own shade. We were also able to carry in several liters of cold water and had a great time. That's my best friend next to us. When so many became distant when I was really sick, Saul stood with me. I really appreciate that guy.

If there is anything you would like me to write about, or have any questions, write them down in the comments. I'd be more than happy to answer.

Tuesday, July 9, 2019

ECP Trial Update

Yesterday I mentioned that my Team had requested that I be evaluated for participation in a clinical trial using Extracorporeal Photopheresis to help slow the progression of chronic rejection.  A lot has happened since then.

I received a call from the ECP team at UTSW early this morning and was sitting in an exam room by 10:00.  There was a physical exam checking vitals and to see if I had good veins (I do), then I met with the team and we went over the trial. We talked about the goals of the trial, how photopheresis worked, and the potential negative effects of the procedure. I filled out a quality of life survey (these have really improved over the past few years and applied to participate in the trial.  There are two arms to the part of the trial I qualified for. One arm receives the treatment, and the other is the observational arm.

 I got a call this afternoon and... I've been accepted into the trial as part of the observation arm. Not quite what I was hoping for, but I am still a part of this important trial. Also, if my rate of decline increases I could be transitioned to the Treatment Arm. So treatment is still a possibility for me.

Monday, July 8, 2019

How Are You?

My new lungs are pretty awesome. They've been thru a lot, and my immune system is doing it's very best to reject them, but they just keep on keeping on. This image is of my lung function over the past 4 1/2 years, from transplant to now.


Lots of stories in those dots. My early major acute rejection followed by a nice long era of improvement. Then we have the beginning of my chronic rejection, likely triggered by stomach contents getting into my lungs. A Nissen Fundoplication and lots of hard work by my team got that mostly stabilized. The chronic rejection continued to eat away at my lung function, so we decided to try a Thymoglobulin Treatment. That treatment got me stabilized for a nice period, until the upper lobe of my right lung collapsed. The collapsed lung seems to have triggered the progression of my chronic rejection again, Chronic rejection isn't something you can cure, it is just something that you can slow down or even pause for a bit if you are lucky.  I have been lucky.

I'm writing this post to answer the question "How are you doing" when asked by friends and family. When things are normal, I usually say "I'm doing well" or "I'm fine", and I mean it,  I feel that i am doing well, as a matter of fact, better than well.  I'm still alive and I'm feeling better that I did before my transplant. If I talk about my rejection, or how my meds are messing with the rest of my body, people feel sad or sorry for me. I want my friends and family to be happy that I'm still here and that I am doing well,  The rest of this stuff is just part of the package of accepting a lung transplant.  If you look at the numbers, about 40% of the people who were transplanted about the same time as I was have passed, and I'm still here.  Next weekend we are going to watch our granddaughter compete in a national Taekwondo tournament in Denver, then another granddaughter is going to stay a week with us.  Life is awesome.

UT Southwestern has been accepted to participate in a clinical trial involving using Extracorporeal Photopheresis to help slow the progression of one type of chronic rejection. My Team submitted me to be a part of this trial today. They tried to get this treatment for me awhile back, but Medicare would not cover it.  This trial to prove to Medicare that this treatment is helpful to lung transplant patients. If accepted, I'll be sure to post a lot about my experience.

So how am I doing?  I'm doing great :)  Yeah, my kidneys are having issues with all the meds (Stage 3 CKD) and my bones aren't what they used to be (Osteopenia on the verge of Osteoporosis). Lung function is trending down, but plenty of room to go before I start having 'real' issues :)  All stuff my Team and I are working on. 

How are you?


Thursday, May 2, 2019

I'm Stable and the Dallas Stars are Rocking It.

Last November while in the hospital with my collapsed lung, I was watching the Dallas Stars and hoping that they would make go at it this post season - and that I would be here to see it. The Stars have won their first round in the playoffs, are playing very well in the second, and yes I'm still here.


As a matter of fact I'm doing quite well. I almost don't want to talk about it so I don't jinx things. This is the first time I've made it to May in a new year without having to be admitted to the hospital at least once.  I'm very happy about this accomplishment.

If you've been reading along, you know that I am having issues.  I am rejecting my lungs, but the rejection has been stabilized. The official name is Chronic Lung Allograft Dysfunction (CLAD). My type of rejection is primarily obstructive (Bronchiolits Obliterans Syndrome - BOS), and I am classified as BOS 3. There are also some restrictive components with my rejection. but I am primarily BOS. Chronic rejection is a bad thought for those of us living with new lungs.  There isn't any "cure" for chronic rejection and the articles you find during a web search don't offer much in the way of hope.  Pretty much like web searches for IPF.  But just because there is not cure does not mean there is nothing for us to do.  My Team has worked hard to get me stabilized a couple of times now, and I am enjoying my current stability.  My latest X-Rays indicated:
1. Stable right upper lobe volume loss with underlying bronchiectasis.
2. Stable mild right middle lobe volume loss. No acute consolidation. 
The volume loss indicated in the X-Ray results is because the upper lobe of my right lung has collapsed three times that I'm aware of, the last was on Christmas Day while we were visiting family.  I was cooking a rib roast when it happened.  Dinner turned out awesome because I didn't let anyone know until we got back to Texas. Seriously, there wasn't much anyone could do other than what I had already been doing so it wasn't an emergency.  I did visit the Team when we got back and things were as expected. Later, at my January annual visit we found that the middle lobe was starting to also have issues. So I'm happy to see that both lobes are stable from January.  Stable is good.

Keeping these lung healthy does come at a cost, but well worth it.  The medicine that is keeping me alive is kinda rough on the rest of my body.  My list of Current Health Issues listed on MyChart is becoming a bit of a long read.  I'd list the major issues, but really don't want to come across as complaining.  I really don't feel negative about any of this.  I am still alive and life is wonderful.  Yesterday we enjoyed our granddaughters orchestra concert, and before that we watch her win the sparring portions of two Taekwondo tournaments. Both times she beat boys older than her.  She's on top of the world.  This Summer another granddaughter is coming to stay with us for awhile, we get to share with her some of the really awesome stuff that the DFW Metroplex has to offer.  So yes, the issues that come with a lung transplant have been worth it to me and my family.

If you are interested in some of the medication related issues that I'm experiencing, just ask.  It would be a good topic for a future post.

Friday, February 1, 2019

Fourth Annual Post-Transplant Exam

On January 1st I celebrated the fourth anniversary of my lung transplant.  Four years of new life thanks to the wonderful gift from my donor family.

With each anniversary, comes my annual post-transplant exam. The annual exam is basically a very thorough physical. The test/exams for the day included labs (14 vials), a CT of the chest, ultrasounds of my heart and abdomen, a bone density test, chest x-ray, a 6 minute walk test, arterial blood gas, a couple pulmonary function tests, and a physical exam.

The lab results were routine.  My white blood cell count is a little high, 7.09x10(9)L. We prefer it to be a point or two lower and will adjust my meds a bit to get it down.  My Creatinine, an indicator of kidney function, is high but not higher than what has become normal for me. I average about 1.4 mg/dl creatinine which indicates how hard the meds are on my kidneys. Other than that my labs were all good.  Immune system properly suppressed, cholesterol and A1C are normal. and liver function is good.

My bone density exam showed the the meds continue to demineralize my bones.  I've lost between 17 - 19% of the bone mineral density in my hips since my transplant. I'll be seeing my Mineral Metabolism Doc next month.

Now we get to the part of the exam that shows the condition of my lungs and progression of my chronic rejection. The X-rays, CT of my chest, and full Pulmonary Function Tests offer a solid picture of where I stand at the moment. You have to look at all three together to get the full picture.

From my X-ray:
Lungs and pleura: Stable right upper lobe volume loss with underlying bronchiectasis noted. Mild right middle lobe volume loss seen. No acute consolidation.
From the CT:
IMPRESSION:
1. Complete right upper lobe collapse with mild bronchiectasis as before. Stable mild bronchiectasis and volume loss of the middle lobe. No endobronchial mass identified.
2. Air trapping on expiratory phase images is suggestive of small airway disease such as bronchiolitis obliterans.
3. Near complete resolution of previously noted groundglass opacities at the lung bases with few residual groundglass and reticular opacities at the left costophrenic recess.
And from my PFT's:
Forced expiratory flows are severely reduced with a moderate reduction in the forced vital capacity.
No bronchodilator response
Lung volumes reveal a normal total lung capacity with incomplete exhalation of the vital capacity and trapping of a large residual volume
Diffusion capacity is moderately reduced
Sever obstructive ventilatory defect with air trapping and impaired diffusion. COPE/Emphysema. Vascular disease, Bronchiolitis cannot be excluded
The most interesting part of these results relate to my collapsed lung. I have been having issues with the upper lobe of my right lung collapsing.  We've been able to get it reinflated a couple of times, but it appears that it is going to be collapsed more often than not.  What is new in these results is volume loss in my middle lobe.  That's not at all what I was expecting, and a bit disheartening.  I'm not quite as stable as I had hoped.

The air trapping noted in these findings is indicative of my chronic rejection. The air trapping combined with the >50% loss in lung volume from my highest results shows the progress of the rejection.

The good news about these results is the 'Near complete resolution of previously noted groundglass opacities..." This indicates that we have addressed the unidentified infection that was previously an issue.

So the end result of this exam is that we will be adjusting my meds a bit to drop the WBC, and I'll be using Acapella airway clearing device along with my incentive spirometer each day to potentially help with the collapsing lung.

Update: My goal after this visit is to stay out of the clinic and hospital until my next quarterly exam :)

Since every blog post needs an image, here is the box we sit in for our full pulmonary function tests.


Image found here

Update: I've increased my CellCept to 1500 mg twice/day and I'm a line item topic of discussion for the Team's weekly meeting next week. They are going to attempt to refer me to Extracorporeal Photopheresis again, but it is not likely it will be approved by Medicare, again.

Friday, December 7, 2018

Chest Muscle vs Body Fat as an Indicator of ICU Survival

If you have been reading along with me for awhile, you know that losing weight has been a large part of my journey.  When I first met my Transplant Team, I was denied the opportunity for a transplant because I had too much body fat.  At my heaviest I weighed 289 lbs. At the bottom of my weight loss journey I weighed in the mid 140's.  I feel my best in the mid 150's, but my Team wanted me to put on a bit more weight to give me an energy reserve for those times when I was sick. I'm currently in the low 170's and this makes my Team (and my Mom) happy. I would prefer to be lighter.  I bring this up because the results of a recent study indicate that body fat may not provide any additional chance of surviving a visit to the ICU.  From PulmonologyAdvisor:




The above article suggests that the amount of muscle in your chest is a much better indicator of survival than amount of body fat when you visit the ICU.
Larger PMA at admission was associated with significantly better outcomes, including higher rates of 6-month survival ..., lower rates of hospital mortality ..., and more ICU-free days (.... In contrast, SAT was not significantly associated with any of the measured outcomes.
PMA = Pectoralis Muscle Area.  SAT = Subcutaneous Adipose Tissue.

What this says to me as a patient who spends a lot of time in the hospital, and some of that time will be in the ICU, is that I should maintain my body fat and muscle mass pretty much the same as a healthy individual.

It is also a bit of a concern for me as during my last visit the Doctor mentioned that the muscles in the right side of my chest are shrinking.  Back to the kettlebells I go.

Something to talk with my Team about during our next visit.

12/9/18 - Muscle mass and core strength are also shown to help with lung transplant recovery.

Friday, November 30, 2018

Post-Hospital Update

Thursday was my two week post-hospital followup visit with my Team.  The reason I was in the hospital is that the upper lobe of my right lung had collapsed for the second time. The first tine it collapsed, it re-inflated during a bronchoscopy. The second time it collapsed we couldn't get the issue resolved during my hospital stay and I came home with the very good possibility that this was my new normal.

My home therepy included a 60mg prednisone taper, three weeks of oral antibiotics and using an incentive spirometer.

During the two weeks following my hospitalization my home pulmonary function testing (PFT) results improved to my pre-collapse capacity and I feel much better.  I was looking forward to seeing my X-rays and wasn't too surprised to find that my lung has done a good job of recovering.  It's not fully inflated, and you can tell that there are issues, but it is much better than when I left the hospital.  We still don't have a good explanation for why it collapses, or why it has re-inflated.  Our working theory at the moment is that during a transition off of an antifungal mediation my immunosuppressant medication levels got out of balance for a few days resulting in a rejection episode.

Visually, the tissue in that area is more white than pink indicating to the Team reduced blood flow to that lobe. My next annual series of testing is in January. The VQ scan should have some interesting results.

My current plan is to monitor the issue.  I continue to perform daily PFT's (as should all lung transplant recipients) and will be having chest X-rays every 2 weeks or so for awhile.

On a side. and likely related note, the Doc mentioned that my chest muscles on the right side are shrinking.  He's not sure why atrophy is only on that side, more interesting stuff.

I couldn't find an image of an X-ray similar to mine, so here's one of me trying on a silly Christmas jacket.


12/5/18 - X-ray results from my visit: 

1. Right upper lobe bronchiectasis and partial collapse, with probable improvement in aeration since 11/13/2018. 

 2. Prior densities in the left lower lung field have also improved.

"Improve..." used twice in one reading.

Thursday, November 22, 2018

Happy Thanksgiving!

Happy Thanksgiving my friends.  I hope this note finds you warm, happy and feeling well loved.


There is so very much to be thankful for. My Sweetie who shares this chapter of our story, my family for supporting and loving us as they do, for the Lord for blessing us with all of our gifts, and for my donor family for this precious gift of life.

I am also thankful for you guys, for checking in and being a part of our journey.  Thank you all so very much and have a wonderful Thanksgiving and Holiday Season.

Wednesday, November 14, 2018

My Latest Hospital Stay

Hey all, I know it has been awhile since my last post.  Things have been in a bit of flux and I wanted to wait until I got a bit of stability before posting.  That's not going to happen, so I might as well post away.

I had mentioned that my Team wanted me to have Extracorporeal Photopheresis (ECP and that UTSW was trying to be a part of a Clinical Study that included ECP for post lung transplant patients in chronic rejection.  Well that's not going to happen.  We didn't become a part of the study and ECP is not covered by Medicare for this application.

Events over the past few weeks may make that point moot anyway.

In late October I had some chest pain on the right side.  It kind of felt like pneumonia but only on the right side.  My daily Pulmonary Function Test (PFT's) showed my lung function had fallen off a bit. Went in to see the Team and found that the upper lobe of my right lung had collapsed.  We left the clinic and headed to the hospital for a bronchoscopy.   This was bronch #19 for me, and the roughest of the lot.  It was also the hardest my doctor remembers doing.  The docs who perform the bronch's are the Team doctors, so we know each other well and can have very honest discussions about what's going on. Getting six biopsies from the upper lobe caused a good deal of bleeding and I woke during the procedure gagging and coughing. On a happy note, the procedure, or the coughing, opened up my lung and things looked good after.

I was scheduled for a CT the 2nd week of November and a couple of days before the test my chest felt off again.  Not as bad as before, but off.  The CT showed that my that lobe had collapsed (Atelectasis) again, so I was off to the hospital on Saturday.  Now one really nice thing about being a part of this team is the fact that when you head into the hospital, whether it is to the ER or a routine admittance, they are waiting for you when you get there. So I checked in and whisked up to the 10th floor, our home away from home.

To make a long story shorter. We tried Intermittent Positive Pressure Breathing (IPPB), BiPAP, CPAP,  an incentive spirometer and the Acapella (flutter) device. We also did IV antibiotics and another bronchoscopy. And once again, I'm unique. The team hasn't seen a post transplant lung collapse like this without a physical cause.  We also couldn't get it re-inflated. So I'm back in the comfort of my own home, on a 60 mg Prednisone taper and three weeks of oral antibiotics.  I'm also using the incentive spirometer and the Acapella.

I'm feeling good, and my Pulmonary Function Tests (PFT's) are a bit better than prior to the hospital stay.

Just another paragraph in this chapter of my story.  An interesting one to be sure, and one that hopefully helps my team with future patients.  This paragraph has to include my CT (with contrast) from the hospital:

IMPRESSION: 
1. Redemonstration of complete collapse of the right upper lobe, no definitive endobronchial lesion identified. 
2. Grossly unchanged multifocal groundglass opacities in the bilateral lower lobes. Findings may represent infection/aspiration. 
Chronic allograft lung disease is also on the differential.
My next annual VQ scan will be interesting.

Edit: I forgot to include the leading 'best guess' as to what happened.  Back in April I underwent a Thymoglobulin treatment that dropped my T cells down to < 25 cells/ul. That is very low. To help prevent a serous fungal infection, I took Vfend, a potent anti fungal for 6 months.  Vfend has a large effect on how my body metabolizes Prograf, a major anti rejection drug.  When transitioning off of Vfend, the Prograf level in my body dropped low, this may have triggered a rejection event. Imaging does indicate that my chronic rejection is progressing.