1 Year Checkup

My 1 year checkup started on 2/16/16. I can barely believe it has been a year since surgery. With surgery, checkups and work travel I feel like I was on a plane for half the year. If you are wondering, yes I am writing this on my phone on my flight too at about 10-11pm(not sure what time zone I am in right now). This past year I have been on a total of 105 flight segments and left the country 14 times. If I continue on this pace, my passport will run out of pages later this year and need to get pages added to it.

If you have ever seen the movie “Up in the Air” with George Clooney, his comments are way too accurate about travel. He comments that you can live out of a backpack, and I can attest to that. I only carry a backpack when I travel, that can fit under the seat in front of me. Anyone using a cane can understand how difficult it is to pull a rolling bag with one hand and use a cane in the other hand. You never have a hand free to open a door, hold an escalator hand rail or hold a ticket.

I have noticed in the last 6 months that my treated eye is now my dominant eye. I see less glare and have slightly better acuity for the treated eye. These are personal observations that I notice.We did all the fun tests like normal. We also added a couple tests back from baseline testing like the Fundas photos. Thankfully the ERG was not on the list. I really hate that test.

Based on the results from the 1 year checkup, my treated eye is still at baseline. I know a lot of people wanted to see results that are improved from baseline, but remember that the goal is to not get worse. This is a success to me. It is still a little early to be definitive, because the degradation of CHM is extremely slow. I head back in another 6 months for the 1.5 year checkup. I would much rather visit Philadelphia in the August time of year than the February time of year.

I plan to be at the CRF conference in Philadelphia in June. I hope I see you there.

6 Month Checkup

It is crazy to think it has been 6 months since I had surgery. Life has been moving extremely fast for me. I feel my eye sight has improved slightly for light/dark sensitivity. I actually probably consider my treated eye my dominant eye now. In the last 3 months our daughter turned 1, now is running and climbing everywhere. Our son is about to turn 3, now he talks all the time and loves riding his bike. My work has drastically changed. In the last 3 months, I have traveled about every other week to Mexico or somewhere else. I have high hopes that travel for work will slow down greatly as a project schedule has changed, which could keep me at my desk for the next 6 months with minimal travel.

I flew to Philadelphia on Sunday Sept. 13 to have eye testing on the 14-16. I had the strangest thing happen on the flight out. The flight attendant saw me pre-board with my cane and asked if I needed assistance. I politely said no thanks. Not more than 15 minutes later, the same flight attendant quietly and slowly asked if that thing with the wires (my folded up cane) in my pocket was an explosive device. I said no. The flight attendant said oh, OK and left. It scares me that she would think that, ask me and believe my response. Then again, if she followed procedure security would have been called and I would have been dragged off the plane and taken to a special room.

I got to complete the micro perimetry test twice. The Dr. wanted to validate the good results he saw. The pupilometry machine was not working, so I was asked to come back on Friday Oct. 2. I was already flying back from Mexico on Oct. 1, so I got a flight after I got through customs from Dallas to Philadelphia. Again, I feel I am traveling too much. The pupilometry test showed good results as well.

Overall all the tests point to a minor improvement in my light sensitivity in the treated eye and no degradation of peripheral field. So far in me, this proves that this is a viable solution to stop visual field degradation. If you followed the results of the Oxford trials, the 6 month checkup is where they published results and my results are in line with the results that they had. I say minor improvement because it was quite minor. This minor of a reading  is pushing the limits of the detectable step change that the micro perimetry equipment has the capability of measuring. The scale is also a logarithmic scale which is non-linear and magnitude based. So if you are a math nerd like me, then you know that it is a base ten scale. One step is 10 times more and 2 steps is 100 times more. This is similar to a pH scale. Hope I haven’t lost you. I will just say I am extremely happy with my results and the possible improvement in light sensitivity is the best case scenario I could hope for. This trip is one of the things I will always remember for the rest of my life. It ranks up there with the Dr. giving me the phone call to be part of the trial.

I want to thank all the Doctors, Medical Staff, the Choroideremia Research Foundation(CRF) and all the charitable donations that have made this clinical trial a reality. Please think carefully about the charities you donate to that they make a difference in people’s lives. I know I make donations to curechm.org and I hope you can see the things that they have already been able to accomplish. The journey for the CRF is far from complete and your donations would help to cure this disease. I have added a “donate to cureCHM.org” link at the top of this webpage if you would like to make a donation.

3 Month Checkup

I went to Philadelphia for my 3 month checkup on June 8-10. This was in the middle of a busy travel month for me. The prior week I was in San Diego for training and the week after I was in Mexico for work. This is why writing this post has taken longer than I would have liked.

My brother met me in Philadelphia and picked me up at the airport. It was great to see him, because we only see each other a couple times a year. Lately he has been working in middle eastern countries, so it is great that he is now back in the U.S.

The testing went well. I am still at the same baseline results prior to surgery. Remember the goal of the trial is no change for the treated eye.. The microperimetry results are the same as before surgery. I feel the results were a little better than original, but there are other factors that can effect the results. How alert I am or how much sunlight I was in earlier in the day. It is a dark adapted test, but I think my eyes never fully adjust to dark once I have been in the bright sun for that day even when I wear sunglasses and a hat.

I got to have another functional MRI on this trip. What is more fun than hanging out in an MRI machine for a couple hours? How about wearing goggles with spinning multi-color pinwheels. There was also a contrast portion of the test that I saw the lowest contrast pinwheel with my treated eye, but not with my untreated eye. The contrast portion was never tested on me prior to surgery, so we will never know if I could see that before the surgery or not. It makes me think the gene therapy was a success though.

I was interviewed by my hometown paper when I got back from the checkup. When they asked about the procedure I said they jabbed a needle in to the back of my eye. It seems my wife feels I should have been more technical and less blunt. I gave the reporter the curechm.org website, Cory’s contact information and Dr. Bennett was also interviewed for the article. I knew others would correctly portray the procedure. I just like seeing people’s reaction when I say jab a needle in my eye. You get to see how many people are not at all comfortable talking about something touching their eye. The article is at lsj.com if anyone wants to read it.

In the airport on the way home, I did get to see a guy screaming at a ticket agent and attempt to get on a plane that was already closed. Security came and took him to what i am sure is a special place in the airport. I never saw him again, but I am sure he is now on a list somewhere and will have lots of fun going through TSA “random screenings” in the future.

Please remember that you can donate to CureCHM.org using the donate link at the top of this page.

1 Month Checkup

I flew in to Philadelphia on Sunday night 3/29. A Dr. emailed me the day before I flew out and asked if I could do a test when I got in on Sunday evening. I was only out in Philadelphia for 3 days and time was limited. I said no problem, but my flight would come in at 7:45pm on Sunday night. The Dr. said that would work and would give me a ride from the airport to where we were doing the test. It was a pupilometry test (FYI my phone autocorrects pupilometry as “pupils merry” I thought it was funny). It is a very simple test, but that piece of equipment is in a different building than all the rest of the equipment. The Dr’s in this study are extremely nice that they are wiling to pick me up at the airport. It also shows how dedicated to this study that they are by working at 8pm on a Sunday night. I like it when I find other people who are workaholics like me.

On Monday night I met up with Cory MacDonald, Brett Sims(#2 in study and a link to his blog at top of this page), Chris Moen and the #4 person getting their final testing to be in the clinical trial. I also had met the #3 person back when I had my surgery. The #3 person and #4 person have not decided if they want to make it public that they have been selected, so I will only refer to them as numbers. As an engineer, I think it would be much easier to refer to all people as numbers. I have a hard time remembering names anyways. The group of us had a good time together, and got to know each other. We had a lot of fun.

We went to the city tap house near our hotel in Philadelphia. They claim to have 40 beers on tap. I say claim, because we asked for 3 beers that they were out of. We told them their sign should say “about” 40 beers on tap. How does any bar in Pennsylvania run out of Yuengling anyways? If states had an official state beer, Pennsylvania would be Yuengling. Arkansas does not sell Yuengling, so I usually try to get one while I am in states that sell it. When we left the bar, we walked back to the hotel. What could go wrong with a group of blind guys walking in the dark after drinking? There were no issues, but I am guessing we got a few odd looks on the way back.

All the testing went well over the two days. Overall the tests showed that my vision is back to the same as the baseline tests, prior to the surgery. This means that I have pretty much fully recovered from the surgery. There is still a small amount of the suture in the eye that has not dissolved, but should be dissolved in the next couple weeks. Thankfully There was not an ERG test at the 1 month checkup. I am really hoping i don’t have to do that test again anytime soon. The redness is reducing and I no longer look like I have a blood shot eye.

I also have some good news at work. I was recently promoted to a corporate position as the Regional Automation Manager. This means I will be traveling more often now. I would like to Thank Cory MacDonald for convincing me to use an ID cane starting a little over a year ago. Places like airports and convention centers were really starting to stress me out when I would not use and ID cane. I am now much more comfortable in areas with groups of people. The interesting part will be people I work with do not know I use an ID cane when I travel. It is not something that comes up in conversation. Next week I will be traveling with some people I work with to Mexico, so we will see what their reaction is. One person I work with actually saw me at the airport when I traveled for the 1 month checkup trip and was very confused.

Surgery 2-24-15

I flew to Philadelphia on 3/19/15, nearly a week before the surgery for the pre-surgery appointments and a few last minute tests. This was nice to relax for a few days. I also got the chance to go out to Gino’s and get an original Philly Cheese Steak. They have a good sandwich. I just wish the weather wasn’t 20 degrees and snowing, since they have no indoor seating. I pretty much stayed in the hotel most of the time, to ensure I did not get sick before surgery.

Day 0 – surgery day 2/24/15. Arrived at 6:30am. Blood work, check in, eye lash trimming (quite funny they cut them off), “X” stamp above my right eye (also funny and I have a picture with the stamp on my head). I was asked if I wanted a drug referred to as giggle juice, my answer was “Yes Please”. No idea what the real name of it was but I took it. The Dr’s tell me the surgery went well, obviously I don’t remember. After the surgery I am told I kept asking my mom if she called and told my wife I was awake, and also if I checked my work email. I would like to point out that I did ask about my wife before work, that is important that I make that point. I walked out of the hospital about 3:00pm with a stylish eye patch. The rest of the day I pretty much laid in bed and listened to the TV. I did not open my other eye barely ever that day, as every time I moved it the other eye moved and it didn’t feel too good. I think the only time I got up was to use the bathroom and eat a bowl of soup.

Day 1 – follow up appointment and blood work.
I got the eye patch removed. I am told that a high percentage of young to middle aged men get light headed when they open an eye they can’t see out of. I am definitely one who falls into that group. For anyone who doesn’t know me that is nothing new for me. I have a scar on the back of my head to prove it from passing out in the past (but that is a whole other story). It was completely blurry in my right eye. The only thing I noticed was bright light. There is an air bubble in the eye from surgery that your blood has to work it out.

Day 2 – I sat in the hotel most of the day. In the afternoon I went back to the office for a follow up video that is for UPENN and the CRF.

Day 3 – follow up appointment and blood work. If you are thinking they have been taking my blood, you are correct. In the previous 10 days I have had my blood drawn 5 times, and 3 pricks in my hands for IVs. With all the blood draws every mark had slight bruising. At this point, if you saw me walking on the street you would think I was a drug user due to marks in my hands/arms and a blood shot eye. At the check up appointment, everything looked good and the Dr. cleared me to go home. Due to the bubble in my eye, I was only allowed to travel by car home and not allowed to fly. My parents graciously drove me back from Philadelphia to Fayetteville Arkansas. We stayed the night at my brother’s house outside of D.C. area. It worked out well, as he had just gotten home that day from being out of country.

Day 4 – riding in a car all day from D.C. area to NW Tennessee to see my grandma and aunt. This was a long day of riding in a car. I could start to see clearly the right passenger mirror in the car, but most everything else was blurry. I had a lot of time to look around in the passenger seat.

Day 5 – last leg of traveling home. We made it home about 3:30pm. I was very happy to see my wife and kids. I would recommend not taking a 21 hour car ride just a few days after this eye surgery. If I had to do it again, I would do it the same way because I wanted to see my family and it was all worth it.

Day 8 – I went back to work. Using only 1 eye to work on a computer was quite difficult. Great way to cause yourself to get headaches.

Day 10 – I was able to read the clock on my wall in my office, if I squinted. I was very happy about this. Feel bad for all the people who came in my office that day, because I told everyone. There was also a sales rep. who came in that asked about the surgery. When I told him about the needle to the eye, he about jumped up out of his chair. It was pretty funny.

Day 12 – I could read the TV guide channel just about as good as my other eye. I would say this is the day I felt I was fully back to normal using both eyes. There was still some blurry parts of my vision, mainly when I look down. Ended up installing and modifying cabinets in my garage, so I would say I was doing well.

Day 13 – I could clearly read my phone. Headed back to Philadelphia for my 14 day follow up appointment. I actually wrote the majority of this on my phone as i was flying to Philadelphia.

Day 14 – Back in Philadelphia for a follow up appointment and blood work. This was a very good follow up appointment. I was able to read just about the same as before the surgery on an eye chart. I think I missed a letter or two on the smallest line, but only 2 weeks out from surgery that is pretty good.

I am very thankful for everyone who helped make this surgery possible. This was a huge effort put forth by many people.

Thank you to the CRF for the years of fundraising and awareness that you have created to get this to clinical trials. Also to everyone who has helped with fundraisers.

Thank you to the Dr’s who worked for years, some even decades, on making this a reality. There are many Dr’s who have worked very hard and have been very helpful through the surgery. They truly care about the work they do and for the well being of their patients.

Thank you to my wife, who has taken care of our children with all the trips I have made back and forth to Philadelphia. It has been a little difficult for our son to understand why dad has been gone so much lately, but I know he will understand later on.

Thank you to my parents who stayed with me out in Philadelphia for a week, drove me back to Arkansas and are helping my wife watch my children on my day 14 appointment trip.

Thank you to my mother-in-law who came to Arkansas to help watch my children for the two weeks I was away for the surgery.

Thank you to my wife’s friend Katie who came to Arkansas to help watch my children from one of the trips I had earlier this year.

Final Testing before Surgery

My final testing was on Tues. 2/10 through Thurs. 2/12.

2/10 – Goldman field, Humphrys field (30degree, 10 degree and horizontal line tests), OCT, color test, reading test, contrast reading and several others tests.

2/11 – repeat the attribute tests (Humphrys field (30degree, 10 degree and horizontal line tests), reading test, contrast reading), cannon high resolution photos and a few others.

2/12 – finish cannon high resolution photos, ERG and a few other tests.

There may be people wondering why I duplicated half the tests. The truth is, the doctors have to prove that I am repetitive for all tests that I interact with the results (click a button). I have to be validated that I get the same results every time I take a test. This is the only way to prove the outcome results after the procedure. If they didn’t test me a minimum of twice then all results of the whole trial could come into question by the committee who will review all the results.

Out of all the testing, the only one I despise is the ERG test, and this was the 4th time in my life I have done it. The first time I did this test I was about 8, and it is a mildly traumatic experience to an 8 year old. Honestly who thought up the concept of putting giant contacts in your eyes with a wire sticking out of them? Then said, this is not crazy enough lets go ahead and dark adapt their eyes first then flash a bright strobe light at the patient.  I understand the good data that comes of the ERG test and why it is a good test, but whoever thought up this test though I would like to say some choice words to them.

On the same trip to final testing I volunteered to take part in being filmed for the study and the CRF. I have to say this is a first for me that someone wanted to film me. Maybe they were unaware that I am an engineer and lack many social norms in comparison to the average person. Seriously, there are people close to me who refer to me as an emotionless robot. I am not offended by that, as it is a fairly accurate representation because the decisions I make are calculated. For those who watch the TV show The Big Bang Theory, I would fit in well with those guys.

It is just under 1 month until my birthday (turning 34 on March 18th). I have decided for my birthday I do not want any presents. Please instead make a donation to curechm.org. As an incentive to donate, I will match $1 for every $1 donated up to $1,000. (Maybe I should have asked my wife about this before I posted. Oh well, I’m sure she will be fine with it) I have added a direct Paypal link at the top of this page to Donate to CureCHM.org for anyone who is interested.

Everything is now a go for the next step, which is surgery. Surgery should take place in the near future. Wish me luck! I am anxiously awaiting surgery. Never did I think I would be anxiously awaiting someone to stick a needle in my eye, but I am.

Consent Review

The first step to become part of the clinical trials is to review and sign the consent form. Thursday Jan. 15 was when we had scheduled to review the consent form. I flew in Wed. late night getting to my hotel room about 1am. We started the review of the consent form at 8:30am. There were several Dr’s and staff in the room for the review. It probably looked bad that I was yawning during the review, because I had only slept about 5 hours the night before. I made it through the 16 page in depth review.

There is one potential side effects that states: “It is possible that if the treatment does work, you may be disturbed and uncomfortable having more vision”. My face was probably quite funny when we reviewed that and I asked “Isn’t that what we are shooting for here?” I think we all got a good laugh at that. A Dr. said that they have to cover all the potential outcomes as possible side effects. There is another part that is funny about “you should not father a baby for a minimum of 4 months after gene therapy.” I laughed at this and said I already have my 2 children and no plans for more. Dr. Bennett said that in the LCA trial they had the same note in the consent form which was really funny seeing that there were some boys who were 8 years old in that study. Can you imagine having to tell an 8 year old boy that that they can’t father a child for 4 months?  A friend asked me later why that comment would be in the consent form. My response was so I don’t create my own genetically modified superhero X-men team and take over the world (during the day of course, as none of us can see at night).

Risk analysis is a major function of what I do. I analyze potential outcomes vs possible benefit. For me, this clinical trial makes sense when I look at the outcomes in 10 years. To simplify the analysis, I am considering my current vision as OK and ten years from now my vision will be poor without participating in the trial. I chose 10 years, because I feel this is a realistic potential for this to be a viable approved procedure if all goes well (this could be slow or fast depending on the time for each phase of the study and approval time is unknown). I have two options(participate in trial or not) and there are two possible outcomes(success or not). Below are the 4 possible outcomes for my eyes in 10 years:

Participate in Trial
Yes No
Trial Successful Yes 1 eye OK from trail, 1 eye Poor, but can get surgery to not get worse Both eyes poor, but can get surgery to not get worse
No Both eyes poor, small chance of trial eye poor sooner Both eyes poor

The other reason I signed the consent form is for my daughter, as she is now a carrier. For many years, I considered not having any children because I did not want to pass on CHM. I have come to realize that children having joy and happiness in life have nothing to do with any obstacles that are in their lives. There will always be obstacles in your life to overcome whether it’s genetic, random chance or self induced. Being there for your children when they need you and being the best parent you can be creates a lifestyle for your children to have the ability to overcome any obstacle. To quote Albert Einstein “We cannot solve our problems with the same level of thinking we were at when we created them.”

A cane is now with me when I travel in airports, as people tend to seem lost and not paying attention to where they are walking. This is quite comical, as everyone is at an airport for a purpose to go somewhere and there are enough signs to be able to figure out where you need to go. With a cane people apologize to running into me. Without a cane people get mad at me for hitting them. People also attempt to contain small children when I am walking near by, which I truly appreciate since they move fast and are below my field of view.

My flight home was uneventful, but it reminded me of the last time I flew. I had just scanned my ticket to board when the gate agent asked me if I needed any assistance. No I did not need any help, I told her. She was extremely polite and asked again “Sir, are you sure you don’t need any help?” I told her I have tunnel vision, so I am good in a tunnel. At this point the woman behind me had been listening and started laughing extremely hard. She quickly contained herself and profusely apologized for laughing at my comment. I told the lady behind me that it is no problem and was meant to be a joke. I was glad someone laughed at it, because at this point everyone was looking at me at the gate area. The gate agent with a straight face told me that was not funny and I shouldn’t be making jokes about a serious situation. Not sure what everyone else thinks, but if you can’t have fun and make jokes about yourself then you are living life too seriously.