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

Update on Starting Extracorporeal Photopheresis

In my last post I mentioned that my Team wanted me to start Extracorporeal Photopheresis (ECP). It turns out that the Centers for Medicare and Medial Services (Medicare) will only cover this treatment for a lung transplant patient as part of a clinical trial.

CMS covers extracorporeal photopheresis (ECP) for the treatment of bronchiolitis obliterans syndrome (BOS) following lung allograft transplantation only when ECP is provided under a clinical research study...

There is one clinical trial that is reported as to not being accepting new applicants.   From what I understand, the trial has been extended and that UT Southwestern should become a study center come August.  This is the first time that Medicare has denied a procedure.  I've gotten used to Part D trying to deny my medications, but procedures is a new one for me.  I understand that most private insurances do cover this procedure with peer to peer review, but that does not work with Medicare.

Good news is that this trial is a research study and not a randomized clinical trial. The procedure works and an RCT would be unethical.

Thankfully I am relatively stable following my Thymoglobulin treatment, and I have a decent amount of lung capacity remaining, so this isn't as large an issue as it is for others in my situation.

Image found here. 

Recruiting for a Patient Advisory Board

In my latest Monday Macro post I mentioned that I am working on a very exciting project, and promised a follow up post with more details.

I've been approached by WEGO Health Experts to help put together a Patient Advisory Board to help a company design clinical trials for the IPF community.  We are looking for a small, yet diverse, team of IPF patients and caregivers to help design trials that better support the participants.

We have an opportunity to really help improve the way clinical trials are conducted and get us closer to a cure for Idiopathic Pulmonary Fibrosis.

There is not travel involved with being a part of this board.  We will conduct three 90 minute conference calls over the next 6 months and we will be compensated for our time.

If you might be interested in participating as a member of this board, shoot me an email.

We are looking for folks who have a genuine interest in helping to shape the direction and application of current and future trials and are hoping to assemble this team fairly quickly.

Looking forward to hearing from you.

University Health Network, Toronto, Study on Pre/Post Lung Transplant Physical Rehabilitation

I am always on the lookout for solid information on post-transplant fitness and physical rehabilitation.  Yes, it has been almost 22 months since my transplant, but I still consider myself as "under construction". I am fitter and in better physical shape than I was in the previous decades pre-tx, but until I stop feeling 'it' in my chest, there is restoration to be done.

One of the best sources for information on how the transplant has affected my muscles is CAN-RESTORE, the Canadian Network for Rehabilitation and Exercise for Solid OrganTransplant Optimal Recovery.  I spoke about them in a previous article here. 

I follow a couple of the doctors that are involved with CAN-RESTORE, and they have published, from the,Toronto Lung Transplant Program, University Health Network, another informative study on physical rehab for lung transplant patients. What is way cool, is that once again this paper is published under Open Access.  I really appreciate their willingness to share this data with everyone.

Physical rehabilitation for lung transplant candidates and recipients: An evidence-informed clinical approach

From the abstract:

Physical rehabilitation of lung transplant candidates and recipients plays an important in optimizing physical function prior to transplant and facilitating recovery of function post-transplant. As medical and surgical inter­ventions in lung transplantation have evolved over time, there has been a demographic shift of individuals undergoing lung transplantation including older individuals, those with multiple co-morbidites, and and candidates with respiratory failure requiring bridging to transplantation. These changes have an impact on the rehabilitation needs of lung transplant candidates and recipients. This review provides a practical approach to rehabilitation based on research and clinical practice at our transplant centre. It focuses on functional assessment and exercise prescription...

I am not going to review the entire study, but do highly recommend you head on over and read it if you are at all interested in pre/post lung transplant rehab and physical fitness.

I will pull this one paragraph from the section discussing long term rehab:

The 6MWT is reassessed regularly post-transplant, to monitor changes in exercise capacity and exertional oxygen saturation, which may change over time. Although the majority of exercise training programs occur in the first three to four months following transplant, longer-term exercise training may provide additional benefits to exercise capacity and the management of long-term co-morbidities of hypertension, hyperlipidemia and diabetes are prevalent at one, three and five years post-transplant. A randomized trial found that lung transplant recipients who underwent rehabilitation in the first three months following transplant had higher physical activity levels, improved fitness and lower 24-h blood pressure one year post-transplant compared to recipients who did not participate in rehabilitation. Daily physical activity has been reported to be significantly reduced one year following transplantation as compared to healthy controls...

"Daily physical activity has been reported to be significantly reduced one year following transplantation..." This highlights the importance of making a habit of doing some sort of daily activity early on after a transplant. The article gives some great ideas on how we might accomplish that goal.

Medical and surgical advances continue to improve the availability of lung transplantation. Exercise training provides an essential role in optimizing functional capacity and fitness pre-transplant, as well as improving outcomes and quality of life post-transplant. Physiotherapists and clinical exercise specialists working with lung transplant candidates and recipients require expertise in general exercise training principles and specialized knowledge of pre- and post-transplant complications, oxygen titration, side effects of medications and a sound understanding of how to modify exercise programs during episodic illnesses/ exacerbations and/or change in lung function pre- and post-transplant...

I would really like to see more studies on post-transplant physical rehabilitation and fitness.  We have the technology to acquire and track all kinds of data.  Studies that look for any correlation between average activity levels and all cause mortality, lung function over time, and quality of life, would be a good place to start.  Changes in bone density, A1C levels, resting heart rates, blood pressure... would all be interesting data points to monitor in future studies involving post-tx activity levels.

Pro tip: The references cited (and linked to) in this study will keep you reading, and learning, for a good while.

Some Good News on the IPF Front

The Pulmonary Fibrosis News recently reported on a study out of Japan that found "Adding Recomodulin (thrombomodulin) to the standard treatment given for an acute exacerbation of lung fibrosis more than doubled patients’ chances to survive..."

An Acute Exacerbation of  Idiopathic Pulmonary Fibrosis (AE-IPF) is about the greatest fear of a person living with the disease.  Just as recently as March of last year, the Journal of Thoracic Disease published that "To date, no randomized, controlled trials specifically directed at the treatment of AE-IPF have been reported".  Using the current recommended management approach for the treatment of an acute exacerbation of IPF, The survival rate is very low. Mortality rate can be as high at 85%.  A treatment that can cut this mortality rate in half is a significant step forward and helps to provide hope for IPF patients and their families.

The study: Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study 

The PF News report on the study: Anticoagulant Seen to Improve Survival in Lung Fibrosis Patients During Flares

More on Acute Exacerbation's of IPF (now slightly outdated): Acute exacerbation of idiopathic pulmonary fibrosis—a review of current and novel pharmacotherapies  

Some Great News About Pirfenidone for the IPF Patient

When I was living with Idiopathic Pulmonary Fibrosis (IPF), there was no effective treatment for the disease.  As a matter of fact, one of the routinely prescribed drug cocktails turned out to do much more harm than good.  In October of 2014, two drugs were approved by the FDA for the treatment of IPF.  Pirfenidone and Nintedanib were shown to slow the rate of progression of IPF in patients who were at a mild to moderate stage of the disease.  By the time these drugs were available to the public, I was passed the mild/moderate stage and could not get on the medicines.

These are not cures, and they do not work for everyone.  They slow the progression of the fibrosis for many patients.  That is pretty awesome in a disease where a patient can often expect a 3 to 5 year life expectancy after diagnosis.   These drugs offer hope, and trust me, hope is a precious commodity when you have an expiration date.

Some very good news for patients who did not see posative results with Pirfenidone has just been published in Thorax (one of the world's leading respiratory medicine journals).  As with a lot of these studies, the title is quite long. "Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis".

So what did they find that is so awesome?  From the paper:

What is the bottom line? 

In patients with idiopathic pulmonary fibrosis who experienced a ≥10% absolute decline in FVC during the first 6 months of treatment, continued treatment with pirfenidone reduced the risk of a second ≥10% decline in FVC or death compared with placebo. 

Why read on? 

Our findings provide the first available evidence to suggest that continued treatment with pirfenidone may confer a benefit to patients with idiopathic pulmonary fibrosis who exhibit evidence of meaningful disease progression during treatment.

Not a cure by any means, but a life extender for many.  Slowing the progression increases the odds of living until they do find a cure, or more likely, a patient is matched up with a good set of lungs and successfully transplanted.

We do need a cure, and there are promising trials and studies going on right now.   Pirfenidone and Nintedanib are an important step in the right direction, I am excited to see what's next.

Anti-Rejection Meds and Daylight Saving Time

Daylight Saving Time, I do believe Arizona has it right.

With the exception of our coffee pot, pretty much all of our clocks shift automatically for DST, so no worries about being late to anything tomorrow, but it is still a bit of a pain in the rear.

The first few months post-transplant are spent getting lots and lots of lab work done.  The Transplant Team is shooting for a specific concentration of one anti-rejection medication, and a very specific blood chemistry to ensure that the immune system is properly suppressed by the other.  This helps ensure the body does not reject the new lungs.  For lung transplant recipients, this is a lifelong  balance.  My body will never accept my new lungs without the help of my anti-rejection meds.  I take my anti-rejection medications at 9:00 AM and 9:00 PM.  It is important that the meds be taken as close to 12 hours apart as possible.  Labs to check medication levels and immune system function are taken at 8:00 in the morning, 11 hours after the last dose.  Being off by an hour in either taking the medications or getting the labs will give the Team faulty data.  It is important to stay on schedule.

Right now my blood chemistry and medication dose is all out of whack due to the infection that had me in the ICU recently.  My immune system ended up way too suppressed and allowed a simple infection to just go wild.  I guess I should write a post about that episode soon.  Well anyway, the team gave me meds to help my body produce white blood cells and they reduced my anti-rejection drugs.  The lower dose of both of my main anti-rejection meds is kind of scary.  Another med that keeps me from catching a virus that came with the new lungs has also been reduced.

My next visit with the Team is Tuesday morning.  When the time changes I adjust my med schedule a bit each day until I'm at the new 9 and 9.  Tomorrow's morning dose will be at 9:15 then I'll be back on schedule tomorrow evening and everything should be balanced by my labs on Tuesday morning.

The rest of my meds are not nearly so time sensitive so shifting an hour here or there does not have much of an effect on my day.