Monday, June 12, 2017

Can you hear me now?

Just a quick health update for those of you who are following along.

I am now 12 weeks into my treatment program with Pegasys (Interferon.).  This drug is typically used to treat cancer patients, but has been proven to have some limited success when used “off label” to treat Recurrent Respiratory Papillomatosis (RPF).   According to my research, this is usually the last treatment method that the doctors try, likely because of the cost and the side effects.

The studies show that with interferon, it can slow the growth of the virus, which in turn enables a patient to go longer in between surgeries.  I have mostly been on a six week surgery interval since late 2014.

I am pretty happy to report that on the last cycle, I was able to extend my surgery out an additional 3 weeks (9 week mark.)  We have only been able to go that long once before (July 2016.)  And to be honest, I was still able to speak audibly at that time but since we had the appointment we went ahead with the surgery.  We have planned for surgery again at the next 9 week mark, but will wait until it has been six weeks to see if my voice is deteriorating at all and if we should keep the date, or move it out.  Most patients don't report progress until 3 to 6 months into the treatment, so given I am 3 months in now, but may have noticed some improvement as early as six weeks.

The side effects are still consistent with what I noticed after the first few weeks.  My doctor at Clearview Cancer Institute said that some people get used to the symptoms over time; that has not been the case for me.  Fatigue, weakness, headaches and depression.   The fatigue plagues me mostly after lunch every day; on weekends I can nap and regain some strength.  On weekdays I fight through it but them am exhausted by the time work is over.  The headaches seem to come from no where and immediately debilitate me.  Often I have to lay down they hurt so badly.  I am still hopeful that maybe my response is just slower to the drug and the side effects will eventually go away.

I am running about 20 miles a week.  Short and (moderately) fast feels good; ie track work.  Anything that requires endurance is very difficult.  But the endorphin rush from running keeps me sane.  And the Beagles love summer walks, so they are getting their fair share!

As for my voice, I am able to speak and be heard.  It sounds a little scratchy, but has more volume and definition than I have had in almost three years; and that is what we have been after all along... some normalcy.  Being able to order coffee at the drive thru and not have the barista think I am a 90 year old woman who has smoked her whole life.  Being able to be heard in a loud room.  Regaining some confidence in all social situations when being heard means being included.

Here is the previous paragraph, spoken.


My white blood cells have been up and down, but are still very low.  My baseline was low to begin with (normal range is between 4.2 and 9.1 white blood cells per microliter) and I was at 3.4.  Since then it has dropped by half, back up to almost at my baseline, then back down to my lowest levels (1.4).  My doctor was not overly concerned because I am overall great health and as long as I can tolerate the symptoms, we want to stay the course with the current dosage plan.  I will have more tests in a few weeks.  The biggest concern about low WBC is risk of infection.  I just have to take extra precautions when being in situations were germs may be present.


The original plan was to be on this drug treatment for 6 months and then re-evalaulate.  That means that I am half way done.  We may decide to stay on it for longer if there is any regrowth on the next surgery, or we may decide to back off and see if the virus comes back.  One concern is that this drug has been known to have a rebound effect; meaning when you come off of it, the virus grows back worse than it was before starting treatment.  While that sounds bad, my condition was about the worse documented case ever, so how much worse could it get!?

So that is all for now. We will continue to fight. We are hopeful that this is the treatment that works and I will be able to have a normal life again.

You shoot me down but I won't fall, I am titanium.

Friday, April 21, 2017

Nicolas Flamel and the Philosopher's Stone

This past Thursday was my third round of Pegasys injections. Interferon is typically used to treat cancer patients, but has been proven to have some limited success when used “off label” to treat Recurrent Respiratory Papillomatosis (RPF). The hope is that with the interferon it will slow the growth of virus so that it will be longer between surgeries or that it would actually kill the virus and I would go into remission. Rachel has been able to give me the weekly injections; I hate needles and she is tough as nails to shooting me up is something she can stomach.

The side effects were mild at first, but certainly are persistent. If I were to rank them from “sucks really bad” down to “sucks not as bad,” I would say they are fatigue, weakness, depression and headaches. Side effects are supposed to be worst immediately after the injection, then slowly fade over the course of the week. Then the cycle repeats all over again. Some research shows that after a couple of months the patient gets used to side effects but that is yet to be seen.

The weirdest part about a side effect is the psychological aspect. Do you think you have side effects because you are supposed to have side effects, or are they all in your head? Do I really feel lousy or is it just my subconscious?

Other than the side effects, I also have a decreased white blood cell count. This was expected. The normal range is between 4.2 and 9.1 white blood cells per microliter (mcL). My baseline tests before I started taking the injections showed I was at 3.7, or slightly below normal. But in the few weeks since taking the injections, my WBC has dropped by half, down to 1.8. Low WBC counts significantly increase your risk of developing an infection so I need to be very careful with trying to remain healthy.

As an engineer, I know that you need three data points to create a trend line. So just having a baseline and the single point since means that the trend is inconclusive until we take another sample this week. It is very well possible that 1.8 is the new baseline and it will not drop any further. Dr. Schreeder at Clearview Cancer Institute agrees, and said that there is no need to adjust my dosage until we see the third data point.

Just like Nicolas Flamel and the Philosopher's Stone from Harry Potter, we are hopeful that this treatment will be the magic elixir that we have been looking for.

I will go to see my physician at UAB on Tuesday for a scope in clinic to determine the regrowth rate of the virus. We don’t expect to see any results from the interferon injections for 3-4 months, but since this virus is an unknown entity, it could go to sleep at anytime for no apparent reason.
So that is all for now. We will continue to fight. We are hopeful that this is the treatment that works and I will be able to have a normal life again.

You shoot me down but I won't fall, I am titanium.


The regrowth on the right is much worse at 5 weeks, than the left after 6 weeks.

Friday, March 31, 2017

The Battle Wages On

For almost 2 1/2 years now I have been battling an incurable virus attacking my vocal cords. I am the 1 in 100,000 people who have this rare condition and in reality, it’s more like 1 in a million who have this and it doesn’t go away in the first two years. It’s even a mouthful to say. Recurrent Respiratory Papillomatosis (RPF)

The typical treatment is a surgical procedure where an Otolaryngologist performs a micro direct larangoscopy. This is performed under anesthesia and a CO2 laser and a coblader are used to physically remove the virus from the vocal cords. The surgeries always come in pairs, as they typically will work on one vocal fold at a time, then allow healing, before operating on the other fold. The nature of the virus is such that as soon as the procedure is complete, it begins growing back. In an aggressive case, the virus will return fully in two months and obstruct the folds from vibrating correctly as lesions allow air to pass through freely. This means that the patient loses the ability to speak with any audible tone. In my case, this happens more frequently, which means that I require surgery about every six weeks.

Dr. Richard K. McHugh is my doctor at the UAB department of otolaryngology and is a specialist in dealing with this virus. He has been a Godsend in my treatment and his bedside manner is amazing. When your doctor sends you personal emails and you can tell that he is physically upset to see that our measures are not killing this virus, you know you have found a great doctor.

Dr. McHugh has been able to try more advance techniques and try additional experimental drugs such as cidofovir and avastin. To obtain avastin, he had to petition the medical board for justification since it is not labeled for RPF and it costs about $100,000 per year. So during the surgical treatments, I receive injections of these two drugs directly into my vocal cords to help fight off the virus.

It’s hard to say if they are working since the virus keeps coming back. Maybe without them, it would be much worse.

So recently we tried a 14 week period where I did not over exert physically. I had been training as an ultra marathoner non stop for 12 years and even though the research showed that exercise did not impact the virus, we wanted to see if not running would help. The theory was that my immune system was so suppressed that it could not fight off the virus. I hoped it would work, but admit that I was skeptical. I have always been very healthy and before contracting this virus, I had never taken a sick day at work which lasted for 16+ years.

At the end of the trial, we discovered that exercise was not fueling the virus. It was actually a little worse on my last surgery which was disappointing.

So what is next? Maybe our last hope.

After a significant amount of work, Dr. McHugh was able to get me approved to begin taking Pegasys, which is an interferon used for cancer treatments. This is “off label” which means that insurance typically only covers drugs that are labeled to address specific problems. Since interferon is not labeled for RPF, we had to go through a lot of justification to be able to receive it. The cost is about $40,000 per year for treatment.

So starting this week, I received my first injection. It is a subcutaneous injection that will be administered once a week. My wife Rachel can actually give me the shots at home, but I have to go to Clearview Cancer Institute every other week to have blood drawn and my vital signs taken.

The drug has favorable results, albeit on a small sample size. The problem with a rare disease is that there aren’t that many people to be able to take a clinical trial. Overall, 70% (46 of 60) of patients had a response (complete response, 35%; partial response, 40%). Partial responses were observed at 3 and 4 months. For the 22 patients that had a complete response, 18 (86%) showed a partial response by 4-6 months of treatment. Out of the 35% of patients that had a complete response, 75% were free of disease for as long as 6.5 years.

So while these look like solid results, I will say that surgical procedures, and use of cidofovir and avastin were supposed to have worked too. So we are hopeful, but skeptical at the same time.

The side effects of interferon are scary. I am not going to lie. Flu, fatigue, chills, fever, liver damage, low white blood cell counts and depression. Of course everyone responds differently to drugs. And typically those who receive this drug are already sick and have cancer, whereas I am in excellent health. The side effects are supposed to be worse in the first few hours, and then lessen up over the week until you receive another injection and then the side effects start all over again. People apparent;y “get used to” the side effects over time.

I felt fine immediately after the injection and even rode my bike a little. But after six hours when we went to bed, I developed the worse case of the chills I have ever had. It was even worse than the time I had hypothermia and had to call for an ambulance while on a trail run on the Pacific Crest Trail. I had on fuzzy pants, a sweatshirt, and about six blankets and still was shivering so violently that it was shaking the bed. Fortunately it only lasted about an hour and eventually I fell asleep. I don’t think that I am out of the woods just yet on symptoms; we will see how things progress in the next couple of days and then on receiving the next treatment next week. Rachel was very caring for me and made me feel as comfortable as possible as she could tell that I was in a lot of pain. She is amazing.

The hope is that with the interferon it will slow the growth of virus so that it will be longer between surgeries or that it would actually kill the virus and I would go into remission. The downside of this drug is that once you stop taking it, the virus can tend to come back worse than it was originally. For now we will be on it for about 6 months, and hope to see some positive results in the 3-4 month range.

I will say that it was very surreal to be at CCI. The mostly perform experimental treatments for cancer, so like me, many people there are on their last hope. Sitting in a chemotherapy treatment chair; being surrounded by people who have cancer; it was eye opening as to the struggles that some people are facing that is so much worse than me.

So that is all for now. We will continue to fight. We are hopeful that this is the treatment that works and I will be able to have a normal life again.

You shoot me down but I won't fall, I am titanium.

Sunday, January 1, 2017

Don’t call it a comeback

I admit that this is long over due, but I finally have decided that I can no longer fight my health issues and continue to push my body beyond its limits with running.

I have been living with an incurable virus attacking my vocal cords for well over two years. I am the 1 in 100,000 people who have this rare condition. It is not going away with our current treatment and surgery routine. My doctors want to take an even more aggressive approach and I am ALL IN on the treatment. The side effects are a bit frightening, but should be manageable.

I would like to be able to have a normal life again. This means being able to talk, be in loud places and be heard, and not be afraid or self conscious to be in social environments. I can't keep having surgery every six weeks and losing my voice in between. My livelihood is dependent upon my ability to speak and I want that normalcy back again.

So it’s time to develop a new strategy, which includes many lifestyle changes and reduction of stressors on my body. I have fought as hard as I could and by no means am I giving up, I just realize that something has to change. As such, I am temporarily stepping away from competitive running. Running fast or far is just something that I do and while it has become a part of me, it is not who I am.

I don't like ever being selfish, but my health and my family need to come first right now and I need to focus 100% of my efforts on those things. My wife Rachel has been my strength when I was weak, and my voice when I couldn't speak. It is with her support that we tackle this next step in our lives together. She truly is an angel to me.

I don't know how long I will be away from competitive running, or if I will ever be able to to return. But rest assured that if I do, it will be the greatest comeback of all time.