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.

Abstract:

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...

Corticosteroids

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

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.

Statins

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.

"Hey Dude, Wait a Second There..."

Wouldn't you know it... as I'm making big plans to change up my fitness goals start seriously training for a strength competition, my body says "Hey Dude, wait a second there. I'm going to try to put a kink in your plans."

As a part of self monitoring, I perform basic pulmonary function testing twice a day.  Over the past few weeks my lung function has been slowly decreasing.  Not a whole lot, just over .2 liters. I haven't had any other symptoms of infection.  No fever or anything like that.  Usually this means that I'm building up a mucus ball in my lungs and I'll have to work at coughing it up.  Well nothing would come up.

Last week I started getting some nasal congestion so this week I got ahold of the clinic and went in for a quick exam and nasal swab.  Turns out I have a silly virus and inflammation from the virus affecting my lungs.  The virus isn't one that requires me to be admitted to the hospital for treatment (yeah), and they went back and forth on whether or not to perform a bronchoscopy and decided on not (another yeah). So right now I've just upped my Prednisone dose and will taper after a few days.  So long as my function testing results return to normal and I don't start showing signs of infection, this may be the only intervention I need this time.  That would be pretty cool

So what to do about my training plan?  Today is Day 1 of my new program.  I thought about holding off a day, or going lighter, or just doing a partial workout.  What I ended up doing was the workout as planned, just took longer in between sets to make sure I was well recovered.  The last couple of sets of kettlebell swings did seem a little heavy.  I'm not sure if that was due to it being the first time I have done deadlifts as part of my workout, or if it was due to the virus.  The deadlifts, pull up progressions and get ups all felt good and strong.

Tomorrow I'll ask my body what it wants to do.  I'll either take a rest day or move on to Day 2.  I've gotten pretty good at listening to my body, so we'll have to see what happens. Right now I'm looking forward to working on those snatches :)

A couple people have asked about my home spirometer. It is a Micro Direct Home Monitor.

I noticed that there is a Bluetooth model available. Ok all of you lung transplant teams and researchers. Here is the data collection device we've been asking for.

Potential Medical Complications of Lung Transplantation - Part 2

I wrote Part 1 of my thoughts on the study "Medical Complications of Lung Transplantation" last week. Those issues were ones that I knew about and was addressing in one way or another. As we continue down the list, I found a couple that I had not heard of.

Avascular Necrosis -

Avascular what?

"Avascular necrosis of the femoral head is a common occurrence in solid-organ transplant recipients, with an estimated prevalence of 3 to 22%. In one study of 63 patients who had undergone lung transplant, an incidence of avascular necrosis of 10% was identified."

This is one I had to look up, and talk with one of the doctors on my team about.  This is something that the team does keep an eye out for, and is another reason for why they make every effort to minimize steroid use.  This issue also seems to be population dependent and seems to occur more often in folks who were transplanted due to Cystic Fibrosis.

As a lung transplant recipient, this highlights the importance of paying attention to your body.  New and persistent pains in the hips need to be reported to your team.  My exercise routine that strengthens my hips and keep blood flowing around my joints should help with this, as should my diet that is low in inflammatory inducing foods.

Hematologic Complications -

This is another one that made me break out the ol' medical dictionary.

"Cytopenia is the most common hematologic complication of lung and other solid-organ transplants, and occurs due to bone marrow-suppressive immunosuppression medication, namely azathioprine and mycophenolate mofetil and cytoxan, as well as prophylactic antiinfectious medication such valganciclovir, acyclovir, and trimethoprim/sulfamethoxazole."

Cytopenia is basically anemia and other disorders that result in a lowered number of blood cells. I know that my team pays very close attention to these lab results and have protocols in place to address any issues that are identified. I really have not done much study on this issue at all.  If you are interested in learning more, here is a recent study that you might find interesting.

Thromboembolic Disease -

Another reason why our teams monitor us so closely during that all important first year after transplant.

"Venous thromboembolism disease, which includes deep venous thrombosis and pulmonary embolism, has an increased incidence in lung transplant recipients, with a reported incidence of 8.6 to 29%. Patients that have undergone other solid-organ transplantation have a high incidence (6–8%) of venous thromboembolism as reported in kidney transplants and kidney-pancreas transplants. The time of onset from transplantation to thromboembolic event varies from each study, with two studies reporting a median time of 47 to 69 days, and another reporting a mean time of 11 months with the earliest event occurring at 3 months. The etiology for the increased incidence of thromboembolism in patients who have undergone lung transplant is unclear, but risk factors may be advanced age, diabetes, concomitant pneumonia, greater immobility, and a higher incidence of indwelling cather placement with PICCs and central venous catheters..."

My team asks us to check our vitals twice/day, every day, pretty much forever. This includes blood pressure, pulse, temperature and  a pulmonary function test. Paying attention to your body, noting changes in your vitals, and staying in close communication with your team is how issues like this are caught early on.

Gastrointestinal Complications -

Now we are getting back to a potential complication that I am actively working to minimize.

"Long-term gastrointestinal complications are common in lung transplant recipients, likely due to higher doses of immunosuppressive medications. In the immediate postoperative transplant period, ileus and colonic perforation are the most commonly encountered gastrointestinal problems, and these can be life-threatening. Long-term common complaints are of nausea, vomiting, gastroesophageal reflux disease, diarrhea, constipation, and abdominal pain. It is estimated that over 60% of patients who have undergone lung transplant have at least one gastrointestinal complaint, and while mild, it can have significant impact on the quality of life of these patients"

I am a very strong advocate of using diet to help maintain a healthy gut.   Happy gut bugs help a person to lead a healthier life.  My daily medications include two different antibiotics, one to prevent a type of pneumonia that lung transplant patients are susceptible to, and one to keep a virus that came with my new lungs under control.  I talked about how I maintain a health gut in Pre, Pro, and Anti - Biotics.

My team has prescribed a Proton Pump Inhibitor (PPI) to both prevent acid reflux (acid in my lungs = bad times) and help prevent ulcers that may be caused by my meds.  I also have an annual colonoscopy to keep an eye out for any colon issues.

So far, diet and exercise really has minimized 'tummy' issues related to my medications and the transplant.

Neurological Complications -

This is a very personal one for me. Before my transplant, I begin to notice that I couldn't trust my mental math skills. While at work, I came up with the wrong answer to simple math problems. I knew the answer was wrong, just couldn't get to the correct number.  I spoke with this with a neurologist during my pre-transplant workup, but he didn't have an answer as to why this was happening.  I had hoped to get my math back after my transplant, but nope.  Good thing we have iPhones. I know that this example is not a complication of transplant, just what has happened with me.  Post transplant issues I see are the occasional bout of tremors and an overall mental fog.  These are both side effects of my medications.  Here are some more potential issues:

One study of 100 patients who had undergone lung transplant reported an incidence of 26% of patients having a neurologic complication in the form of severe headaches, seizures, strokes, and confusion.

 The best way to protect against these issues is to pay close attention to what your body is telling you, and let you team know if you start having issues. Your team can adjust your meds as necessary to minimize most issues.

Malignancy - 

The immunosuppressant  medications that keep your immune system from attacking your lungs, also suppresses your ability to fight cancer.  This is why there are hard and fast rules requiring a transplant candidate to be cancer free for certain time frames before considering that candidate for the procedure.

Patients who have undergone solid-organ transplant are known to have a higher prevalence of malignancy than the general population. Some estimates place a three- to fourfold increase in the risk of malignancy in solid-organ transplants compared with the general population, while the relative risk of specific cancers may be increased by 100-fold. The incidence of malignancy may be even higher in patients who have undergone lung transplant than in those who have undergone other solid-organ transplants.

Skin cancer prevention has become a major part of my personal concerns.  I'm going through my third of 3 Blue Light treatments in five weeks this coming Wednesday. I see a dermatologist at least every 6 months. Each time I saw her, she had to burn-freeze-cut off more actinic keratosis and pre-cancer spots. At my last routine visit, there were too many to remove, so we are using the chemical/blue light therapy to maybe put a stop to the issue. I write more about what I do to prevent skin cancer here, but it needs to be updated  as I've become much more strict about limiting my sun exposure and wearing high SPF sunscreen.

The team keeps an eye out for the earliest signs of any type of cancer.  We have routine labs quarterly after the first year and an annual workup that includes more scans than many people receive in a lifetime.

My go-to cancer preventative is still diet and exercise.  It is just an N-1 experiment, but I'm feeling pretty great so far and will continue on as I am.

Post-Transplant Lymphoproliferative Disorder -

Ok, honestly, I have no idea of what this is or how to avoid it.

PTLD comprises a spectrum of disorders that arise in post-transplant patients that are due to abnormal lymphoid proliferation. Histologically, PTLD ranges from benign polyclonal hyperplasia to malignant monoclonal lymphoma. Clinical presentation of PTLD in patients who have undergone lung transplant widely varies from nodal local involvement to extranodal and disseminated involvement.

The good news is:

Treatment is centered around lowering immunosuppression. Immunotherapy with rituximab, an anti-CD20 monoclonal antibody, has been used with success for PTLD in solid-organ transplants and in lung transplants.

There's a treatment, so if it happens the team will handle it. Yep, I trust my team that much.

Conclusions -

A small excerpt from the conclusions:

As the overall expected survival in patients who have undergone lung transplant has improved, and as more patients live longer, long-term medical complications that arise as a consequence of immunosuppressive therapy are seen more frequently.

That is kind of a Duh statement, but it says a lot. "...the overall expected survival in patients who have undergone lung transplant has improved, and as more patients live longer..."

We are living longer, more active, and higher quality lives.  Yes, a lung transplant is one scary, rough, hard core procedure.  And yes there may very well be complications, but life after transplant can also be wonderful, exciting and fulfilling.

I recently shared a panel with a couple other lung transplant recipients, Larry and Nancy.  I was the baby of the group.

12 years, 5 years and coming on 2 years post-transplant. Living well and enjoying life.

I didn't write these posts to scare anyone off of the idea of talking with a team about the possibility of receiving a lung transplant, and I certainly hope I haven't done so. I firmly believe that an educated patient is an empowered patient #ePatient. We have to go into this procedure with our eyes wide open, knowing the potential risks, and knowing the potential rewards are more than worth it.

I do hope this post helps to highlight the importance of observing the 'rules' as laid out by your team. Take your meds on time, all the time. Avoid infection. Take proper precautions with sun exposure. Avoid restricted foods (some affect the meds). Get active. Monitor your vital signs at least daily, twice daily is recommended. And more importantly, pay attention to what your body is telling you.  Your body will let you know when there is an issue. You will have a phone number to contact a member of your team 24/7, use it when you need to.

If this is your first time here, and if you are interested, you can read about My Lung Transplant here.

Life is good, being able to breathe is awesome, and Donors are Superheroes.

Potential Medical Complications of Lung Transplantation - Part 1

While bouncing around the ol' internet looking for information relating to some of my past lab results, I stumbled upon this interesting paper: Medical Complications of Lung Transplantation. I've read quite a few reports about the potential issues that I may see as a direct result of my transplant, or the immunosuppressant medications that I take, I've written about what I do to help prevent skin cancer, avoid infection and counteract the side effects of my meds. This report covers many of the issues all in one place. I learned a couple interesting new tidbits that I thought I'd share.

From the abstract:

As short- and long-term survival rates for lung transplantation continue to improve, and as more lung transplantations are occurring with each year, a multitude of medical complications are encountered by the clinician. This article reviews the long-term non-pulmonary noninfectious medical complications that arise beyond the postoperative period in patients who have undergone lung transplantation.

Now I have to be honest here, this could be one scary paper to read if you or a loved one are considering a lung transplant, or are already waiting on "The List". All I have to say is that an educated patient is an empowered patient, and this ePatient is very happy to have undergone the procedure. The potential of one of these issues popping up can not take away from the 22 months of making new memories with our grandchildren. If one the the issues we are going to talk about reared its ugly head tomorrow, the extended life I've enjoyed so far will be worth it.

Now to the meat of the matter.  The abstract continues:

This article reviews the development of renal failure, diabetes, cardiovascular complications of hypertension and atherosclerosis, osteoporosis and avascular necrosis, hematologic complications, thromboembolic disease, gastrointestinial complications, neurologic complications, and malignancy, including post-transplant lymphoproliferative disorder.

See, I told you it was scary.  Let's talk about these one at a time.

Renal Failure:

This is something that I'm sure everyone who is on '.The List', or has received a transplant, understands.  The medications that keep us from rejecting our lungs are very hard on our renal system. Our teams monitor renal system function very closely at all of our checkups, and a good part of our annual visit is focused on our kidney function.  This is why -

"Renal dysfunction is one of the most common long-term complications of lung transplant, with an incidence of 25.5% at 1 year after transplant and 37.8 at 5 years after transplant. By 6 months after transplant, 91% of lung transplant recipients undergo some degree of renal decline from their baseline pre-transplant level. Chronic renal failure with a creatinine greater than 2.5 mg/dl occurs at an incidence of 6.8% at 1 year after transplant and 11.0% at 5 years after transplant..."

One thing I do to help protect my kidneys is abstain from drinking any kind of alcohol. Alcohol is hard on the kidneys. The team did a great job of explaining this, and how our livers like to metabolize alcohol better than the meds so drinking alcohol will have an effect on the effectiveness of our medications.  I decided that if transplanted, then no more alcohol.  I also drink plenty of water. Early on my labs were off kilter and that was due to my not drinking enough water.

I also eat a very clean diet.  I figure the less crud my kidneys have to filter, the better off they will be.

These are  my Creatinine lab results from over the last year and a half or so.

Diabetes - 

Diabetes is another potential result of taking anti rejection medications that was well covered by the team prior to receiving my transplant, and is something that they monitor for at every visit.  

"The development of diabetes is a relatively common complication in patients who have undergone lung transplant, with a reported incidence of 24.3% at 1 year after transplant and 33.5% at 5 years after transplant...

...Guidelines for treatment of diabetes for solid organ transplants recommend intervention when patients have fasting glucose levels greater 126 mg/dl and hemoglobin A1C levels greater than 6.5%"

I use the same tools to reduce the risk of diabetes as pretty much anyone else would, diet and exercise. Before my transplant, the team Dietitian really tried to convince me to stop following the Primal Blueprint. It became a point of minor contention prior to the team accepting me as a transplant candidate. She really feels that we should be including whole grains and starchy foods as a major part of our diet. We came to an agreement that I could continue eating as I do so long as they couldn't identify any adverse effects of my diet following my transplant.  Well here we are, 22 months post transplant and I am doing really great.

Here are my A1C lab results -

I was on insulin for the first three months post transplant, but that was procedure and due to the very high level of steroids I was on at the time.

Cardiovascular Complications -

The risk of high blood pressure, high cholesterol, and other cardiovascular issues was also well addressed by my team while I was being evaluated for a transplant.  Monitoring for, and addressing, these issues is an important part of my post transplant care.

"The immunosuppressive medication of lung transplantation results in contributing to the development of cardiovascular comorbidities of hypertension, hyperlipidemia, diabetes, and renal disease. Hypertension is seen in 51.9% of patients who have undergone lung transplant at 1 year and in 85.6% of patients at 5 years. Hyperlipidemia is seen in 20.5% of patients who have undergone lung transplant at 1 year and in 52.2% of such patients at 5 years..."

My team addresses the high blood pressure issue by prescribing the Beta Blocker Metoprolol. I address it via diet and exercise.  Both my resting heart rate, and blood pressure, have significantly decreased over the past year, and the amount of Metroprolol I take has been reduced by a third. My average evening pulse - blood pressure for this week last year was 84 BPM - 117/78. My averages for this past week are 79 BPM - 100/61,  My heart rate is still a bit higher than I would like, but much better than the 100+ BPM it was pre-tx.

My team and I have gone round and round about the necessity of taking a statin drug to control cholesterol.  My team, of course, has won. I learned something from this study, that if my team had mentioned, would have convinced me of the importance of taking a statin.

The use of HMG Co-A reductase inhibitor medications (statins) has been shown to effectively lower cholesterol levels while demonstrating decreased acute rejection episodes and prevention of bronchiolitis obliterans syndrome when the statins were started in the within the first year of transplant. Patients on statin medication had better spirometry readings and improved mortality.

That right there is a good enough reason to take statins if you have had a lung transplant.

My cholesterol labs -

Osteoporosis -

I've talked a lot about post-transplant bone density, and minimizing this risk is a major focus of my little N-1 experiment on post-tx general health. Here's what the report states the issue:

"Osteoporosis is a complication of lung transplantation that can cause significant negative impact on quality of life and on morbidity due to related fractures. Solid organ transplantation has long been established to be associated with a high incidence of osteopenia and osteoporosis. The use of corticosteroids and other immunosuppressive medications are believed to have the largest impact of causing osteoporosis, although other factors are implicated as well..."

 If you are interested in a more in depth explanation of  what I'm doing for my bones, you can click the link above.  Basically it involved diet, exercise and supplements.

There is more on this list of potential complications of lung transplant.  This is just Part 1 of a two part series.  If you have read this far, you can see that there are actions that we as empowered and educated patients can take to help ourselves to "Live Long and Prosper".  I don't know where that come from, but what the heck.

Please remember that I am an educated patient, not a medical authority. If my thoughts give you any ideas about things you can do with your own diet or exercise program, please talk about it with your own team before making any changes.  Each situation is different and we all have our own unique to us medicine cocktail that our teams have dialed in for us.

If you have received a lung transplant, what do you do to help minimize the risk of these complications?

Medical Complications of Lung Transplantation - Part 2

Scammers are Scum

Folks with rare, progressive, terminal diseases are often desperate to find some glimmer of hope for a cure.  I looked to clinical drug trials for hope, and lifestyle changes to improve my quality of life.  My IPF had progressed too far to be accepted into most trials, and I was not the perfect patient for the one trial close by, in Oklahoma, that would take people with more advanced disease. My final option was the lung transplant, and I am very fortunate to have been placed on the list and offered new lungs.

There is a seedy underbelly of humanity (and I do use that term loosely here) that will seek to profit off of desperation. These people and organizations are scum of the earth. You will find them posting in internet communities that are dedicated to supporting the patients and caregivers of disease.  They are also easily found when searching for information on rare diseases.  If they happen to get ahold of a patients email address, they fill the in-box with seductive promises of health and well being.  These miracle cures can cost hundreds, even thousands, of dollars a month. This is money that most cannot afford to be spending, especially spending on false hope. Often the patient can offset the cost of their 'treatment' by referring others to the scammer. Kind of like multi-level marketing for snake oil.

I recently had one of these scammers post a comment here on the blog. I will not be party to promoting disinformation like that.  I did allow this one comment to stay as I wanted to address the issue.  All future comments like this will be deleted without comment or explanation.

Here is the comment and my response:

Joan DavidsJuly 30, 2016 at 7:10 PM(MUST READ: HOW I GOT CURED FROM PULMONARY FIBROSIS)
I was diagnosed of Pulonary fibrosis in March 2014, my doctor told me it has no permanent cure, i was given medication to ease the situation, this affected me so badly as i constantly go short of breath, this continued till a friend of mine Karen told me about Ejiro Herbal Clinic were she bought herbal medicine that totally cured her mother of pulmonary firbosis, I contacted this herbal clinic via their email and purchasd the pulomnary fibrosis herbal remedy, i received the herbal medicine within 7 days and when i started usage my lungs gradually got better until i even forgot i had pulmonary fibrosis, i went back to my familly doctor to test for idiopathic pulmonary fibrosis my doctor confirmed my lungs tissue were 98% repaired. Contact this herbal clinic via their email - deleted

 JRJuly 30, 2016 at 8:57 PMJoan, I have never understood the depravity of individuals who prey upon people with terminal illnesses. Your miricle clinic supposedly cures everything from ALS to COPD to emphysema to Parkinson's and now IPF. During my tenure as an IPF patient I was inundated by scum like you who sought to profit off my presumed desperation. I have seen fiends and acquaintances spend money they could not afford on the false hope provided by reprobates such as yourself. They died taking these snake oils, and left their families worse off for it.
I really hope there is a hell, one with a special place for people like you.
Don't bother replying, or commenting on other threads. Comments promoting snake oils will be deleted w/o comment from now on

I am a big fan of people making money.  Heck, I like making money when I can and Lord knows I could really use a bit more of it. But people like Joan here don't just disgust me, they really piss me off.  These are much worse that the Nigerian scammers who prey upon an individuals greed, they go after the hopeless.

Just like with the Nigerian scam emails, there are hints in the comments and emails that this comment is just meant to swindle money from the sick.  I really don't want to point out the obvious as it may help scammers in the future, but if you know your disease, you can find the errors in the message.  It is very obvious that this "Joan" has not been seen for pulmonary fibrosis.

No, a Japanese silkworm enzyme or a Himalayan fungus is not going to cure your fibrosis. No, the drug companies are not suppressing a natural cure so they can sell their high dollar medications.  Before putting your family into debt searching for the miracle cure, remember that quackery is alive and well on the internet. Do your due diligence and do background checks on the treatments offered.

There are quite a few very interesting clinical trials recruiting IPF patients that are worth looking into. One is a Study of Autologous Mesenchymal Stem Cells to Treat Idiopathic Pulmonary Fibrosis. This is not the same as the Stem Cell clinics that seem to be popping up all over the place. I would approach those with a great deal of caution.

Don't get me wrong.  There are supplements and lifestyle changes that can improve quality of life for many people. The difference is these are not going to empty your bank account and you don't need to get them from a special lab or clinic.

We have a long way to go in improving the diagnosing and treatment of Idiopathic Pulmonary Fibrosis and clinical trials really are our best hope for the future of treatment.  Right now we have two treatments that slow the progression of disease for many people. Please, look into these before sending your money off to some grifter who is just trying to separate you from your money.

Edit 5/7/18 - You would not believe how many spam comments this post has generated.  Many of them begin with "Glory be to..." the go on to proclaim how the commenter has been cured of AIDS, IPF, Lung Cancer, Herpes and a plethora of other ailments.  These folks obviously didn't read the post, just searched some keywords.  This is both irritating and disheartening.  It really pisses me off that so many people are out to prey upon the desperation of the chronically ill, and saddening that it is profitable for them.

Scams are everywhere.  Last time I was in the hospital I received a call from "Blue Cross Blue Shield" about my account.  I don't have BCBX but they had the last 4 digits of an old plan.  They were trying to "upgrade" my policy.  When I mentioned that I no longer had BCBX, they said, "Oh, I see you have UCH now".  Heavy sigh.  They were obviously a scam from the first sentence out of their mouths, but I kept talking for a bit until I couldn't take any more.  I'm sure it was just a coincidence that I was in the hospital when they called, but they are still scum.

Be careful out there.

Edit 10/11/18 - The scam spam keeps on coming.  One of the latest I've had to delete stated they could cure testicular and ovarian cancer, and... promote penis enlargement.

This kind of crud is going mainstream.  One local radio station has an advertiser that makes like they are sharing testimonials from people who have been helped with everything from cancer to MS to Alzheimer's.  This particular company is selling a very expensive subscription supplement program and is rated D1 by the BBB.

If it sounds to good to be true, it is. 

Anti-Rejection Medications and Their Side Effects

I am a member of the steering committee for the PF Warriors of North Texas pulmonary fibrosis support group.  During today's conference call, several people mentioned that  they wished someone had taken more time explaining the post-transplant medications and their side effects before they underwent the lung transplant procedure.  Sounds like a good subject for a blog post.

I am writing this post from the patient's perspective, and am describing the side effects that I experience.  Each patient is different and may handle these medications differently.  A transplant team works very closely with their patients to minimize the side effects of these life preserving drugs.  Following a lung transplant, a patient will spend a lot of time with the transplant team dialing in the most effective drug cocktail to protect the new lungs with the least amount of side effects.  Working, and communicating with, your transplant team is the best way to ensure that your particular cocktail is the most effective for you.  Oh... one last thing... your meds are pretty much always changing.  The changes are not as drastic, or nearly as often as you progress, but they will change.

My medications:

Post Transplant Bone Density

Immunosuppressive anti-rejection medications are a permanent part life after a lung transplant.  We take our meds on a fairly strict schedule and submit to routine labs to check blood levels for the meds, the status of our immune system, and check our liver and kidney function.

These medications are so important that my daily routine is basically centered around my med schedule.  Noncompliance with taking immunosuppressents is a major cause of transplant rejection.  One month post transplant I underwent an acute rejection event due to a virus, that is something that I really do not want to experience again.

These life saving medications come with some pretty heavy duty side effects.  Half the pills we take are taken to counteract the effects of the anti-rejection meds.

Eating clean and following the Primal Blueprint are tools that I use to help protect my body from unnecessary inflammation and to help maintain kidney and liver health.  I use daily exercise and walking to help counter the effects of muscle weakness and wasting.  I also take various prescribed and recommended vitamins and supplements to help keep my body generally healthy.

Bone and joint deterioration is one of the many side effects of these medications.  I think the Prednisone is probably the largest culprit in this area, but the immunosuppressents are also contributors as are the proton pump inhibitors (PPI's) that many of us are on as prophylactics preventing micro aspirations and GERD.

From an article on Bone Mass and Solid Organ Transplantation:

To quote Elizabeth Shane, a recognized leader in this field, "Immunosuppression insults an already compromised skeleton.

To counter the bone loss side effect of the meds, my Transplant Team prescribes Citrical to be taken twice a day.

I have a long term n-1 experiment that is a bit more detailed than that.  I need to minimize, or even reverse any bone loss, and definitely prevent joint deterioration.  After surviving the procedure and getting to the point in my recovery where I began to believe that I really could live a healthy and mostly normal life, I began studying how to maintain my bones.

The Transplant Team had the supplements covered with the Citrical, so my little n-1 experiment has to do with diet and exercise.

On the diet side of the equation, I eat fermented milk products like kefir, yogurt and aged cheeses.  I also eat plenty of leafy green veggies, the occasional can of bone in sardines, and lots of home made bone broth.

On the exercise side I slowly progressed from Pulmonary Rehab to light strength exercises with resistance bands, to a beginner's body weight routine, to my current Simple and Sinister kettlebell program.  It is very important to note that each progression was approved, and carefully monitored by, my Team.  I may have pushed things a bit, but I pushed them with my Team's approval. Trust me, if you attempt something that your chest is not ready for after a lung transplant, your chest will let you know.

The recommended exercises for increasing bone density are ones that create stress on the bones.  High impact exercises like jumping and sprinting, and heavy load bearing exercises like deadlifts and squats seem to be the most mentioned.  Both of these were pretty much off limits for most of the first year post-transplant.  I was just approved for jumping this past visit to the clinic.  My exercise plan was not optimal, but was the best under the circumstances.

How what are the results after the first year?  Mixed.

From my first post-transplant annual bone scan:

Percent change in BMD from baseline study: 

Lumbar spine: 1.7%
Left total hip: -9.8%*
Right total hip: -8.6%*

I have to admit that initially I was a bit dissapointed, I was expecting better results.  After looking into it, these are not that bad.  As a matter of fact the lumbar spine numbers are pretty great.  One study found half of the patients in the study lost 8% Bone Mineral Density (BMD) in the first year post transplant.  So actually gaining density is awesome.

It does appear that diet and kettlebells have helped my spine.  So I believe that I am on the right track.  I just have to make some adjustments to my plan.

So what changes to make?

We discussed using a medication to help restore bone density, but decided to go another year with my n-1 experiment including my proposed adjustments.

Historically my Vit D levels have been low.  That's reasonable seeing as how I'm supposed to refrain from getting much sunlight.

To increase my Vit D, which is necessary for calcium absorption in the gut and is needed for bone growth, the Team prescribed a cycle of once/week 50,000 units of Vit D3 for eight weeks followed by a daily dose of 1000 units D3.

I do not plan on making any changes to my diet other than maybe drinking more mineral water.

As for changes to my exercise plan, I will start jumping once my tooth extraction site is healed.  One of our granddaughters is excited that the Team has finally ok'd my jumping on the trampoline with her.  I will also begin doing plyometric jumps daily.  I generally do the Simple and Sinister routine five days a week.  On the other two days I've started doing kettlebell deadlifts and military presses.  I plan on doing heavier deadlifts once I can acquire a (very) inexpensive bar and weight set.

This exercise plan supports my goal of competing in the Tactical Strength Challenge next Spring.

We won't know the results of this little n-1 experiment until my next annual bone density test come January.  I am confident that I will see improvement, and I know I will be fitter for the attempt.

For more on bone health, see 8 Primal Rules for Building Better Bones