Thursday, April 19, 2018

The Next Chapter

Hi guys.  In my last post I mentioned that I was having issues with my new lungs, and asked if all y'all were interested in following along with this part of my journey.  I received some great feedback in the comments to that post, on Twitter, and off line.  The response was very encouraging so here we go.

Image from Trappancs

Before I get started, I want to mention that the issues I am going through are part of the lung transplant process for many of us. I'm not talking about my transplant rejection to be negative, quite the opposite. I hope to get some helpful information out there for folks who are on this same path.  If you are pre-transplant and reading this, hopefully I don't cause you any undue duress with my posts.

There are two basic classifications of lung transplant rejection, Acute and Chronic.   Acute rejection is the most common and can progress over a very short period of time. Acute rejection often resolves with prompt and aggressive treatment by an experienced transplant team.   Around one month after my transplant, I experienced an acute rejection.  My acute rejection was brought on by a simple Coronavirus and it took an aggressive antiviral treatment and steroid pulses to resolve the issue.  My FEV1 dropped from 77% to 61% predicted over the course of the rejection episode. That acute rejection was rough, but I recovered and my lung function continued to improve.  I have plenty of posts about the exercise and diet regimes that helped me to eventually achieve a FEV1 of 126% predicted. That's a 5 liter FEV1, I was pretty proud of myself.  It turns out that building up that extra capacity was a very good thing as it prepared me for the second type of rejection, chronic rejection.

Chronic rejection is a scary concept that folks don't really want to talk about.  There's very little information out there on the subject and even less from the patients point of view.  Chronic rejection is, very basically, a progressive fibrosis that can have an obstructive or restrictive component.  The umbrella term for chronic rejection is Chronic Lung Allograft Dysfunction (CLAD). The obstructive form of CLAD is Bronchiolits Obliterans Syndrome (BOS), and that is what I am Experiencing now.

At my peak, my spirometry results were FEV1 of 126% and FVC of 113% predicted. This was in July of 2016.  In October of 2016 my spiros started to decline. We did several different treatments to try and stop this decline.  We adjusted my medications, performed a couple steroid pulses, and in May of 2017 I underwent a Nissen Fundoplication. My decline in lung function stabilized in June of 2017.  FEV1 = 64% and FVC = 79% predicted. Lung function spirometry remained stable until this past January when they started to decline once again.  The Team decided it was time for me to undergo a Thymoglobulin course of treatment. The treatment itself wasn't that bad, I experienced the common side effect during and after the first couple of infusions.  Before starting the treatment I had to have a bronchoscopy and quite a few other tests to ensure that I had no infections or other issues.  I developed a slight fever after the bronch that delayed the treatment for a couple of days while it was resolved. It would be a bad idea to further suppress my immune system if I had an active infection so I ended up staying 11 days at my home away from home, the 10th floor at UT Southwestern Clements Hospital.  It took seven infusions of the Thymoglobulin to drive my CD3/CD4 T-Cells down to <25 cells/ul.  It's a good that we seem to be past the most dangerous part of the flu season, doing this in November would be much scarier than it is today.  I still have to be very careful about infection control and limit my exposure to the public, but the fewer viruses in the air the better.

My hospital follow up exam at the clinic was this morning and it turned out very well.  My spirometry went up a bit compared to my pre-hospital tests and lungs looked and sounded good.  Today's Spiros = FEV1 - 51%, FVC = 71%.  That is still plenty of capacity to be living an active and wonderful life.  My SpO2 (blood oxygen) readings are between 96 and 100% so I'm getting plenty of oxygen even during light exercise. I do get out of breath much easier so pacing myself when doing chores and the like is now the norm.  I am still in much better shape than I was for more than a year prior to my transplant.  These issues really are a part of the lung transplant package, and will be until researchers can grow new lungs using the patients own DNA.  Even better yet would be to find a cure for MS, IPF, COPD and the other diseases that require a lung transplant.  Until then, we do what we can do and enjoy this wonderful gift blessed to us by a special donor and donor family.

A future post will include imaging and test results showing how my lungs are being affected.  If there are any lab or test results that you are particularly interested in, ask about them and I'll include them also.

Tuesday, March 20, 2018

Quick Update and a Question

It has been a good while since I've posted, and lots has happened since my last one.

I did reach the major milestone of celebrating my 3 year transplant anniversary.  Recent Scientific Registry of Transplant Recipients (SRTR) data shows that I'm a member of the 64% who have done so.  Not only have I survived, I have thrived.  Yes, there have been setbacks.  And yes, I am having some issues that I will talk about in a bit, but I have survived. I am doing much better than I was the year prior to my transplant.  I am not back on supplemental oxygen, and I am enjoying life.

Life is good, and my new lungs have been a wonderful gift from a very generous donor family.  Now part of package that comes with the gift of new lungs is the knowledge that there will be setbacks, and that new lungs don't last forever.  My lungs are on the downward slope side of the post transplant lung capacity bell curve.

The graphs above are my basic spirometry results following my lung transplant. FEV = Forced Expiatory Volume and is basically the amount of are I can forcibly expel in 1 second.  FVC is my Forced Vital Capacity and is a basic snapshot of total lung volume.  FEV1 is the data most significant to my transplant team.

We are not sure what is going on with the little dip at the tail end of the graphs.  It may be a continuation of the chronic rejection that started near mid-chart, or it may be triggered by something else entirely.  A steroid pulse over the weekend followed by a taper that I am on now seem to have stabilized things once again.  We'll see soon enough.  I've also been having issues with very low white blood cell count and other related labs so my meds are all out of kilter.  Again just part of the package.

I am a bit reluctant to talk about this new chapter in my journey as I really don't want to discourage anyone who is in the middle of making the decision about lung transplant. Thinking about it, I feel that writing about what is going on may be helpful to some, I know information like this is something that I look for, and cannot find.

So what do you think?  Are you interested in reading about my annual testing results, hearing about what's going on with the new lungs, and following along this new chapter in my journey?

Let me know, I'm really interested in your thoughts.

Monday, June 12, 2017

Monday Macros - 6/12/17

Monday Macros 

Weight = 139. Weight gain/loss this period = 0 lb. Total weight loss = 112 lbs. Macros for the week = Carb/Fat/Prot - 15/58/27%. Daily avg cals = 1,865. Body Fat = 9.6%

I tried a couple of food experiments this week.  First was adding bacon drippings for scrambling my eggs. I have some uncured Berkshire drippings in the fridge.  This worked well.  Then I tried bacon. Now this was part of one of the new egg bites at Starbucks.  Not sure if it was the bacon, or something else in the bite, but this hurt. Stomach pain and overall discomfort after the first bite with bacon.  I removed the bacon for the rest 'cause I needed the calories.  Won't be doing that again. The third test was almond butter.  I added two TBSP's into a protein shake. This resulted in one heck of a lot of nighttime gas. So back to following the  Soft Nissen Diet for awhile.

I am able to eat larger portions now, and getting my pills down is becoming easier so I am making progress.

More Monday Macros

Saturday, June 10, 2017

At Home Pulmonary Function Testing

I am very fortunate and excited to be working with a Tech company who's goal it is to design the next generation of IPF related studies and clinical trials. What really excites me is that this company is asking all the right questions, and is reaching out to patients and caregivers for guidance and help in this endeavor.

We are still in the very early stages of this process, and I will keep you updated as we go.

One issue that is part of pretty much every clinical trial involving Idiopathic Pulmonary Fibrosis (IPF), is lung function. To make studies and trials more accessible to more folk living with IPF, researchers are testing the use of home spirometers.  I am beta testing two different Bluetooth enabled devices at the moment and I'll post my thoughts on those when done.

One question that I keep seeing popping up on Twitter and in various research proposals is just how often will folks be willing to perform Pulmonary Function Testing (PFT) via a sprirometer.

I very vividly remember how hard it was to do a full effort PFT for my doctors.  To be a useful tool, all PFT's pretty much have to be full effort.  It is different now that I have had my transplant, blowing into the tube is much easier and I'm more used to it.  My team asks that we do our tests twice per day.  I would never have been able to do that in my mid to later stages of IPF.

So my question to you is, how often would you be willing to do a PFT for research?  Some doctors are recommending daily. Would you join a trial that required daily PFT's?  Would you join a trial that required at home PFT's at all?

This poll will be open for three weeks.  Please spread the word, the more people who vote in the poll, the more data I will be able to share with researchers who are interested in this data.

Thanks in advance for your help.

Edit: I tried several times to get the poll widget to work in the post, but wasn't able to.  The poll is located at the top right hand sidebar.  Thanks again for your help.


Thursday, June 8, 2017


 Acceptance is not defeat. 

Acceptance is the bedrock anchoring the roots of resilience.

A lung transplant is not the 'cure' for any disease. One of these days, a complication of my transplant or medications is going to take my life. I accept that fact. I also understand that I would have died two years ago if not for the precious gift of my new lungs.

Accepting my likely fate is not the same as giving in, or surrendering to, it. Acceptance has allowed me to stay strong during the setbacks. Acceptance has made me resilient and strong.  

Accepting the fact that I would likely have to deal with a chronic rejection, has given me the ability to fight this issue and keep moving forward no matter what my immune system is doing to me lungs.

My lung function has been stable for over a week, a small victory worth celebrating.

Monday, June 5, 2017

Monday Macros - 6/5/17

Monday Macros 

Weight = 139. Weight loss this period = 1 lb. Total weight loss = 112 lbs. Macros for the week = Carb/Fat/Prot - 16/54/30%. Daily avg cals = 1,720. Body Fat = 9.6%

My daily calories are still a bit low, I have to work on that a bit.  I am getting good, quality calories in with very few 'empty' cals.  Total sugar was less than 20 gr/day.  That's about perfect. Total carbs were also right where I want them so I just have to work on increasing fat and protein.  Fat would be the easiest macro to boost. Just two more tablespoons would get my calorie intake where it needs to be.

Transitioning off of the Nissen Soft Diet would help get me back on track calories wise.  I miss my BBQ. 

My roof attracts debris like a magnet. I normally would have blown it off a couple times by now in the Spring.  With all that is going on, I finally got around to blowing the roof off and blowing out the gutters, yesterday. Pretty much wiped me out. But it's done and we had a gully washer yesterday afternoon so worth the time and the effort.

More Monday Macros

Saturday, June 3, 2017

My Immunosuppression Protocol

I've written about the medications I take, their side effects, and some of the steps I take to counter those effects.  I don't think I've mentioned why I take these meds.

Each transplant center utilizes its own immunosuppressent protocol. I've searched all over tor data that lists the protocol used by each center along with the SRTR/OPTN annual reports on the centers. You can compare time on the wait list, mortality on the wait list, mortality rates post transplant and lots of other data. I just haven't found a way to compare protocols along with all of this data. That would be some interesting information.

The Journal of Thoracic Disease published "Immunosuppression in Lung Transplantation" which explains the different medications and protocols used to help keep us alive.

The following is what my Team uses in my protocol. All quotes are from the above journal article. I really appreciate that this is not hidden behind a paywall.

Lung transplantation can be a life-saving procedure for those with end-stage lung diseases. Unfortunately, long term graft and patient survival are limited by both acute and chronic allograft rejection, with a median survival of just over 6 years (1). Immunosuppressive regimens are employed to reduce the rate of rejection, and while protocols vary from center to center, conventional maintenance therapy consists of triple drug therapy with a calcineurin inhibitor (cyclosporine or tacrolimus), antiproliferative agent [azathioprine (AZA), mycophenolate, sirolimus (srl), everolimus (evl)], and corticosteroids (CS). Roughly 50% of lung transplant centers also utilize induction therapy, with polyclonal antibody preparations [equine or rabbit anti-thymocyte globulin (ATG)], interleukin 2 receptor antagonists (IL2RAs) (daclizumab or basiliximab), or alemtuzumab (2). While these agents are used to prevent acute and chronic rejection, they are not without adverse effects, including drug-specific toxicities, as well as opportunistic infections and malignancy. This review will summarize these agents and the data surrounding their use in lung transplantation, as well as additional common and novel therapies in lung transplantation.
I am going to focus on maintenance immunosuppression and novel therapies that I am taking.

Maintenance Immunosuppression:
Maintenance immunosuppression is lifelong immunosuppressive therapy that is given to prevent both acute and chronic rejection. The goal is to not only to prevent and minimize immune-mediated injury to the allograft but also to minimize adverse effects associated with the medications used. Conventional maintenance immunosuppressive regimens consist of triple drug therapy with a calcineurin inhibitor, antiproliferative agent, and CS.  Note: CS = Corticosteroids
Calcineurin Inhibitors 

The Calicneurin Inhibitor that I take is Prograf.  My highest dose was 4 mg twice/day.  Current dose is 1.5 mg twice/day.
A second calcineurin inhibitor, tacrolimus(previously known as FK506) (Prograf©) became available for use in 1997. It is 10-100 times more potent than cyclosporine. Tacrolimus binds to intracellular FKBP12, forming a complex that prevents transcription of cytokines, including interleukin 2, and ultimately prevents T lymphocyte activation and proliferation... 
...most centers utilize trough concentrations for therapeutic drug monitoring (31,32). Target ranges vary according to center-specific protocols and practices, and take into account patient characteristics, such as time post-transplant and rejection and infection history. Generally, target trough concentrations range from 5-15 ng/mL.
My current Tacrolimus goal is 10 - 12 ng/mL.

Anti-Proliferative Agents

For this leg of my therapy, I take CellCept (Mycophenolate). Current dose = 1750 mg twice/day. This is the highest dose I've been prescribed.
Mycophenolate is the most frequently used antiproliferative agent used according to the most recent ISHLT Registry report (2). Mycophenolate mofetil and mycophenolate sodium are converted to the active metabolite, mycophenolic acid (MPA), which inhibits inosine monophosphate dehydrogenase (IMPDH), the enzyme responsible for T and B lymphocyte production. Inhibiting this enzyme results in decreased T and B lymphocyte proliferation. Because lymphocytes lack the ability to utilize salvage pathways for nucleotide synthesis and thus rely on the IMPDH pathway, mycophenolate is selective for T and B lymphocyte proliferation inhibition (47). Mycophenolate undergoes rapid absorption and conversion to MPA. MPA is metabolized hepatically into mycophenolic acid glucuronide (MPAG). MPAG is excreted via bile into the intestines, where it is converted back to the active metabolite, MPA, resulting in a second peak concentration in the plasma. Doses range from 1-1.5 g IV or oral twice daily. Therapeutic drug monitoring is available for mycophenolate, with AUC being the optimal parameter for measuring treatment response...

This is pretty much common in all transplant centers.  I take prednisone, 10 mg/day with the occasional pulse of high dose IV Methylprednisolone. 
CS have been used in solid organ transplant since the very beginning and have not only remained a corner stone of both induction and maintenance immunosuppression but they are also used to treat acute cellular rejection (ACR) as well. The most commonly used CS in solid organ transplant are methylprednisolone and prednisone. CS are known to have antiinflammatory properties and exert their effects in a variety of ways, including inhibiting the NFkB pathway, preventing T cell proliferation, decreasing macrophage activation, inhibitingcytokine production and altering lymphocyte migration (67). According to the most recent ISHLT registry report, CS continue to be used by almost all transplant centers, at one and five years post-transplant. Initial doses range from 500-1,000 mg given intraoperatively, and are gradually tapered over weeks to months to 5-10 mg per day for maintenance...
Novel Approaches


Azithromycin is one of three routine antibiotics that I take.  My dose is 250 mg 3 times/week.
Azithromycin is a macrolide antibiotic with anti-inflammatory and immunomodulatory effects (89). These effects, in conjunction with the beneficial effects of maintenance azithromycin seen in cystic fibrosis patients led to pilot studies of azithromycin in lung transplant recipients with BOS (90-93). In 5 of 6 patients, thrice-weekly azithromycin for 13 weeks demonstrated an average 17% improvement in FEV1 (92) and an average 18% improvement in FEV1 after 12 weeks of therapy in 8 others (93). A retrospective analysis of 20 lung transplant recipients also demonstrated an improvement in FEV1 after 12 weeks of azithromycin therapy (average 110 mL from baseline) (94). However, not all patients respond to azithromycin therapy (95-97). Evidence suggests airway neutrophilia and elevated interleukin-8 bronchoalveolar (BAL) concentration may be predictors of response (95,97,98). Furthermore, studies have indicated that early initiation of azithromycin, e.g., BOS 0-p, may have more of an impact on preventing disease progression and may improve survival (97,99,100). In a randomized, placebo-controlled trial of 83 lung transplant recipients, there was a significant reduction in the incidence of BOS at 2-year in those who received azithromycin prophylactically compared to those who did not (12.5% vs. 44.2%, P=0.0017) (101). There was also a significant difference in BOS-free survival (HR 0.27, P=0.020), although overall survival was similar between groups. Collectively these data suggest early initiation of azithromycin in lung transplant recipients may prevent the incidence of BOS and prolong BOS-free survival, and may improve or stabilize pulmonary function after the onset of BOS, particularly in those with neutrophil- and IL-8-predominant BAL.

Being prescribed statins threw me for a loop. I really did not want to take statins and resisted when my Team first prescribed them. Then I learned how beneficial they could be for the lung tx recipient. I take 20 mg of Pravastatin daily.
Statins, 3-hydroxy-3-methylglutaryl coenzyme Areductase inhibitors, have been shown to have properties which may have a potential beneficial impact on lung allograft function post-transplant. They have been shown to reduce the gamma interferon induced expression of major histocompatibility molecules on cells, increase the number of CD4+CD25+ T regs, inhibit growth factor expression in lung fibroblasts and inhibit the development of obliterative airway disease in animal models (105-108).  
 These abovementioned immunomodulatory and anti-fibroproliferative properties have potential benefit for lung transplant recipients. However, clinical evidence in lung transplant recipients is limited to retrospective single center studies only. Johnson and colleagues showed improved 6-year survival in statin group compared to controls, 91% vs. 54%...
These are the immunosuppresents that I take. The list of meds/supplements that I take to counter the side effects of these medications is longer yet.  I've talked about many of them in the past.

Upping my CellCept Dose, Some Information I Found Interesting

In my last Clinic Update, I mentioned that the team increased my immunosuppressent medications.  I now take 1750 mg of CellCept and 1.5 mg of Prograf twice/day.  That's the highest dose of CellCept I've been on.

We also agreed to reduce my Protonix (a PPI) from 40 to 20 mg/day.

Since I'm on this high a dose of CellCept, I spent some time last night going back over dosing recommendations and side effects of the med.

One of the side effects that I knew about, and the reason I take the Protonix, is the damage that the med can do to my digestive system:
Because CellCept has been associated with an increased incidence of digestive system adverse events, including infrequent cases of gastrointestinal tract ulceration, hemorrhage, and perforation,
One thing I learned last night, that I don't remember reading when I studied CellCept in the past, is that PPI's  have a negative effect on the efficiency of CellCept:
Proton Pump Inhibitors (PPIs) Coadministration of PPIs (e.g., lansoprazole, pantoprazole) in single doses to healthy volunteers and multiple doses to transplant patients receiving CellCept has been reported to reduce the exposure to mycophenolic acid (MPA). An approximate reduction of 30 to 70% in the Cmax and 25% to 35% in the AUC of MPA has been observed, possibly due to a decrease in MPA solubility at an increased gastric pH. The clinical impact of reduced MPA exposure on organ rejection has not been established in transplant patients receiving PPIs and CellCept. Because clinical relevance has not been established, PPIsshould be used with caution when coadministered to transplant patients being treated with CellCept
Basically MPA is the active metabolite that does the work of suppressing my immune system.

So... we've increased my dose of CellCept, and reduced my PPI.  My labs next week are going to be interesting.

I'm glad we are doing this at this time of year, less viruses spreading in the wild so safer for me to be out and about.

The above information was taken from the CellCept prescribing literature.

Thursday, June 1, 2017

Clinic Update - 6/1/17

Today's clinic visit included the usual labs, chest x-ray and spirometry. I also had a CT scan and Ventilation Perfusion scans.

On a positive note, my lung function was statistically the same as last week.  This is the first time that it hasn't fallen in consecutive visits.  Now, it was only a week between PFT's, and I have been working on conditioning this week, but no notable loss is great news.  I'll take it.

The x-rays looked good.  The results of the scans were not in yet, but I could see changes in my VQ scans.  Surprised the tech that I could knowledgeably talk about what was showing on the screen.  I've spent a lot of time looking at my past VQ scans recently.

When I sat down with the Team, we performed a trend analysis on several data set and found some interesting information.  We looked at immune system function, white blood cell count, medication dose history, PFT results,  tacrolimus levels in my blood, time switching from brand name to generic meds and other lab results.  Other than PFT's and other indications of rejection, the individual labs and tests were all in a standard and very acceptable range.  But when we overlapped the charts, we found that at the same time as my problems started, trends in immune system activity and medication levels were also moving.  My tacrolimus (Prograf) levels are in the correct range for individuals two years post transplant, but, obviously too low for me.  Same thing with my immune system.  It is high normal for someone at my stage post transplant, but too active for me.  So we are increasing my Cellcept and Prograf daily doses.

More meds = more side effects = more work on my part to counter the side effects.

I think I remember this line from Old Yeller, but others attribute it to John Wayne:

"A man's got it to do what a man's got it to do."

I should have the results from the scans on Monday or Tuesday, then back to clinic in two weeks.  Hopefully we'll see some improvement in lung function.

On a side note.  A couple of the docs on my Team went to the ATS annual meetings recently.  It's fun to see them re-energized.  They have one heck of a workload and I'm really impressed with their empathy and knowledge of us individual patients.

Edit to add PFT results for the past year. Everybody likes pictures :)

Monday, May 29, 2017

Monday Macros - 5/29/17

Monday Macros 

Weight = 140. Weight loss this period = 1 lb. Total weight loss = 111 lbs. Macros for the week = Carb/Fat/Prot - 24/48/28%. Daily avg cals = 1,704. Body Fat = 9.8%

Macros look much better now that I can eat some foods. Carbs are still a bit high at 105 gr/day, but necessary to get in the cals. 

The Nissen Soft Diet is much better than a liquid diet, it just takes awhile to eat smaller portions of food.  I do get punished it I try to eat too fast or don't chew quite long enough.  If the procedure helps to stop the decline in my lung function, it will be worth it.

Yesterday I did two sets of 10 kettlebell deadlifts, three sets of 5 hike passes, and three two handed power swings with a 20 kg kettlebell. Wiped me out. I was going to do halos, presses and maybe some snatches today, but my body insisted on waiting a bit. It's a heck of a hill I've decided to climb.

On a very positive note, my body does remember the movements and my form was correct.  Also, the surgical locations on my lower stomach did not hurt too much after the kettlebell practice or even today.  Just enough to let me know to be careful.  

My lungs did not enjoy this, especially my right lung which has been irritated for awhile.  Hopefully they'll get over it.

More Monday Macros