Sailing SV Celtic Cross – Dave Kurtz

Now on to the Finals in two weeks.  Jill and I are both ready to head home tomorrow.  We’ve spent two weeks here with the goal of generating this little bag:

There’s a couple million of my stem cells in there that will be reimplanted on February 7 after they kill off my existing immune system.  I have to say that the process is pretty intriguing to an engineer like me.  Jill was less than enthused about the temporary catheters hanging out of the side of my neck.  The procedure to insert the catheters is the worst part of the process and even that is only an hour.  From that point it’s pretty much plug and play.  Book (my nurse) hooked me up to this apheresis machine.

Essentially a very sophisticated centrifuge that spins my blood into layers with the red blood cells near the top, the platelets and white blood cells near the bottom, and other components separated into layers based upon their specific gravity.  It automatically finds the stem cell layer and draws it off into the bag. Four and a half hours later they disconnect me and I go home.

In the two weeks leading up to today, I did get a pretty solid dose of chemotherapy (cyclophosphamide) followed by several days of neupogen injections designed to stimulate stem cell development.  The only side effect I had was some bone pain, but even that was pretty manageable.  I think I had a pretty easy go of it compared to some accounts I’ve read.  Anyway… on to the next phase!

The biggest problem so far has been boredom.  I’m three days into the neupogen injections and my white blood cell counts are supposed to begin dropping right about now.  From others that have gone through this I understand bone pain may show up, but no indication so far.  Jill has been ill with a stomach virus for five days now and I caught the bug a couple of days ago (Wednesday).  Wednesday was a rough day and nothing was staying down.  Yesterday was better and still improving today, but it may be another day or so before the stomach virus is behind me.  I do have some itching that I would assume is related to one of the drugs I am taking, but it is not something that requires intervention.  In the meantime, we are holed up in a hotel room for the duration.  Cabin fever is setting in…

I will say that I approached the stem cell transplant process with some trepidation.  I did not and do not have any doubt that this is the best path for me.  All other options basically slow the inevitable to a crawl, at best.  Not acceptable if there is an alternative.  The stem cell transplant is a pretty rigorous procedure to undergo, but the end result is an

autoimmune system reset and an end to scleroderma.  No guarantees here, and there is a significant relapse rate, but the reward is worth the risk.  I also learned two days ago that the treatment protocol is being modified still further by adding Rituximab to the chemotherapy treatment that kills off my existing immune system.  And I respond well to Rituximab.

I’m here for two weeks this time.  The end result is a plastic pouch with 4 to 10 million of my stem cells that will be frozen until I return at the end of January for the actual transplant process.  The “recess” is to allow me to recover, because upon return they really clobber me with chemotherapy to kill off my existing malfunctioning immune system.  To me, it’s a little bit like calling a time out to ice the field goal kicker in football.  They give me a taste of what this is all about and then let me sit and think about it for a week or two before really getting into the weeds with six straight days of serious chemotherapy.

There is no way I’m backing out of this unless I’m thrown out, of course.  It must be the engineer in me, but there is a real cool factor to all of this that really intrigues me.  I’ll the eighteenth person in the world to undergo this particular “heart friendly” protocol as a part of this clinical trial.  The really good news is that the preceding 17 people all lived!  Not the case with earlier protocols.

As a closing side note, my wife Jill has a rare autoimmune disease called Wegener’s Disease.  Back in 1988 when she was diagnosed it was uniformly fatal.  We went to see a Dr. DeRamee at Mayo Clinic as one of the experts in this almost unknown disease.  He offered us two options.  One was the standard treatment in use at the time that only offered to slow the progression.  Or we could opt for a new protocol introduced by Dr. Wegener in Germany.  Jill would be the eighteenth person in the world to be placed on this antibiotic protocol, but it showed very good promise.  We elected the new protocol.  Jill is here and thriving nearly 30 years later.  As an engineer, superstition is not my thing, but I like the confluence of our paths right now.

It seems like this has been a long time coming.  Tomorrow I enter the hospital to, basically, get a new immune system.  The plan is that it is an immune system that operates normally instead of attacking me.

This first stage is to pull a few million stem cells from my bone marrow.  The old way was to aspirate them with a long needle.  The new way is to utilize chemotherapy and other drugs to encourage the stem cells to enter the blood stream and then harvest them in a process that will take a couple of weeks.  Certainly a new adventure for me.

It’s December 29, 2016 and I am back in Chicago for more testing and appointments.  Getting to the actual stem cell transplant is a little like getting a security clearance.  I need to be approved at multiple stages and by multiple doctors in order to continue on.  Today is Dr. Ben Freed, my cardiologist here at Northwestern.  After getting an x-ray and twenty test tubes of blood drawn this morning (required two venipunctures) I met with Dr. Freed to have my heart checked once more to ensure it is healthy enough to go through this.  It is, and I’m a go for next week.

After spending an inordinate amount of time online learning more about both lung transplant and stem cell transplant, I found that a Dr. Richard Burt in Chicago was having a great deal of success with autologous non-myeloablative hematopoetic stem cell transplant for patients with scleroderma, along with other autoimmune diseases such as lupus and multiple sclerosis.  Moreover, he was adamant that the limiting factor to a successful transplant depended on the condition of the heart, not the lungs.  Stem cell transplant overall experiences about a 10-12% mortality rate during treatment, making it a rather risky undertaking for those electing to go through with it.  Dr. Burt found that those that died were dying of heart failure.  They were not suffocating as a result of poor lung function.  Moreover, he found that the heart failure is related to the radiation treatments (myeloablative) patients were subjected to along with the chemotherapy.  By eliminating the full body radiation from the treatment protocol (non-myeloablative) Dr. Burt’s treatment related mortality rate dropped to 5-6%, basically half of what everyone else was experiencing.

Based on this information, I decided to apply for hematopoetic stem cell transplant (or HSCT as it is more commonly known) with Dr. Burt at Northwestern.  On October 24-27 I was in Chicago undergoing a week of testing to determine if I was a viable candidate for HSCT.  Easily some of the most comprehensive testing I have been through.  A two hour CT scan that generated over 5,000 images of my torso.  Pity the radiologist that had to review those files!  A right and left heart catheterization that was pretty miserable to undergo, but it yielded some new information on my heart.  Even though the right heart catheterization at U. of M. ten months earlier had found nothing wrong with my heart, by this time scleroderma had begun hardening my heart, or more specifically hardening the pericardium surrounding my heart.  Not good news.  This is the condition at the root of the 10-12% mortality rate among HSCT patients.

Not to be deterred, Dr. Burt had developed what he refers to as the “heart friendly” protocol to allow HSCT to go forward for patients with heart involvement.  Basically less cytoxan chemotherapy and more fludarabine, another chemo drug that is easier on the heart.  I would be approved for HSCT based upon using the new heart friendly protocol.  All that was left was to convince my health insurance to pay for the $175,000 procedure that was so new that only 15 patients have gone through it as a part of a clinical trial.  No problem there, right?!?  Northwestern has a nurse coordinator for the HSCT program, Carol Burns, who knows insurance companies.  Carol worked her magic and in a little over a week I was good to go.  Onward…

So…. lung function still declining, albeit more slowly.  Need to look at what other options are out there to ensure I live past 2017!  As I see it, there are four other paths that others with systemic scleroderma follow with varying degrees of success.

• An antibiotic protocol (AP) that I do not know a whole lot about just yet.
• Stem cell transplant.  My rheumatologist, Dr. Khanna, recommends against this.
• Accupuncture along with Chinese herbal therapy.  They have found some success in putting autoimmune diseases into complete remission using this, although I do not know of any scleroderma patients that have gone this route.
• A full lung transplant (the nuclear option).  This will work, but I can likely see my ultimate demise on the horizon because average life expectancy following a lung transplant is about five years.  Plus, many lung transplant centers will not perform a lung transplant unless scleroderma is already in remission, which leaves me out right now.

After sorting these out, I settled on a path forward.  First, I need to learn more about stem cell transplant.  After much research, I found that one of the world’s foremost authorities on hematopoetic stem cell transplant for systemic sclerosis (diffuse scleroderma) patients is Dr. Richard Burt at Northwestern University Medical Center in Chicago.  I initiated a contact with them to see if I could be evaluated for their program.  At the same time I met with Dr. William Lynch, a thoracic surgeon at the University of Michigan lung transplant center to begin the process of being accepted for a lung transplant.  I figured that if I ended up going the transplant route, I could try the accupuncture/herbal therapy while I was waiting for a transplant and if herbal therapy happened to work I could skip the transplant.  On October 10, 2016 I had an initial meeting with the lung transplant team and Dr. Lynch to learn more about the process.  Frankly it was grim, primarily because it was uncertain if I could get approved and if I was approved my life expectancy following transplant was not very long.  However, if no other options exist, five years is better than no years so I decided to go ahead with the preliminary testing required for lung transplant.  At least I had a direction…

I have been very fortunate as regards my health. Over nearly 65 years I’ve spent one night in a hospital and usually saw a doctor once a year for a physical, if that often.
However, in 2015 I noticed something was going on.  When I walked up a flight of stairs I had to catch my breath before I could talk to my wife. I was supposed to be training for my eighth Ironman race and my training was flatlining when it should be peaking! What was this all about? I had an aerobic engine better than guys half my age.  I used to run flights of stairs as a workout.

What happened was diffuse scleroderma, an autoimmune disease that hardens your skin and can affect any number of internal organs.  It was considered incurable and the options going forward are not very good.  I was diagnosed on December 23, 1015, only a few months after noting that something was wrong.  In that respect, I was again fortunate.  The average time to diagnosis is 3 to 4 years after the first signs of the disease.

What follows here is a rather detailed log of what has transpired over the past year and a half to provide a permanent record for reference.  I’m not out of the woods by any measure, but at least I have an opportunity to improve my outcome.  The history may help.

1. April-May 2015  My triathlon training program began flattening out at a time when it should be improving significantly.
2. August 2015  I noted that I was fatigued and really did not have the energy I usually had.
3. September 2015  Walking up one flight of stairs left me out of breath to the point that I could not talk to my wife without waiting to first catch my breath.
4. October 8, 2015  I woke up to find my hands swollen so much that I could not close them.
5. October 14, 2014  I went into U. of M. Emergency for suspected heart trouble (fatigue, swollen hands, shortness of breath).  I ended up being admitted for three days while undergoing a battery of tests (X-rays, CT scan, Echocardiogram, etc.).  The diagnosis was pneumonitis which was reasonable except that my hands were still swollen.
6. October 22, 2015  First pulmonary function test.
7. October 24-28, 2015  In bed and ill with severe cough and very tired.
8. November 9, 2015  Saw Dr. Kiningham (PCP) with a sore throat and cold; coughing, chills, fever, and hands that are still swollen.  Basically confirmed that it was pneumonitis and ignored the swollen hands.
9. November 16, 2015  CT scan showed slightly swollen lymph nodes in my lungs.
10. December 14, 2015  Bronchoscopy with lavage and biopsy.
11. December 23, 2015  Dr. Khanna diagnosed me with diffuse scleroderma based upon swollen hands, blood tests, and bronchoscopy.  Started Prilosec for reflux.
12. December 30, 2015  Lung biopsy confirmed diagnosis of interstitial lung disease.
13. January 7, 2015  Right heart catheterization showed no sign of heart involvement and a normal PAH (i.e. none).
14. January 8 to March 16, 2016  Began Cellcept orally at 3,000 mg per day.  No positive effect.  Lung function is still declining.
15. April 20 to September 7, 2016  Six doses of Cytoxan (1,430 mL per dose).
16. August 8 & 22, 2016  Two doses of Rituximab.  Cytoxan was not looking effective so Rituximab was added.
17. October 11, 2016  Appointments with Dr. Khanna and Dr. Flaherty.  Dr. Khanna he is somewhat encouraged that Rituximab is having a positive effect.  The problem is that my PFT today shows a continued decline in my DLCO (27%) and I am running out of numbers on the low end.  My meeting with Dr. Flaherty was to discuss a lung transplant.  Because it takes months to go through the approval process it was decided to begin testing to determine if I am a candidate for lung transplant.  Also discussed stem cell transplant with Dr. Khanna.  He said I am not a candidate because my lung function is so low.  All in all not a good day today.

A new year and a new project for me.  2016 was, from my perspective, a steady ride downhill as regards my health and my prospects for a long life going forward. Although it is by no means certain, I seem to have leveled out somewhat or at least slowed the decline over the past few weeks.

A new year always brings new hope for a better future. This certainly applies to 2017 for me. I am more than ready to put 2016 in the rearview mirror and look forward to what I believe is a path forward in stopping this disease, if not reversing some of the damage it has done.  This blog is intended in part to document the journey.  Being ever optimistic, I look forward to a much brighter 2017. I wish nothing less for each of you.