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.