Category: Navigating Medical and Social Service Systems

How Are Your Eyes?

The question of “How are your eyes?” pops up from time to time at a social gathering with friends or a holiday meal with extended family.  I don’t find this question rude or intrusive, but I’m never quite sure how to answer.  Especially when the question often seems to come out of nowhere – not even closely related to the last topic of conversation.  Is the question being asked as a polite “How are you?” to which a “Fine” or “Okay” is expected.  Or is the questioner hoping for a detailed description of my last trip to the optholmologist? Did they see me accidentally dip my finger in the salsa bowl, thus prompting them to wonder how much more vision I’ve’ lost since they saw me last?

My typical response goes something like, “Well, RP is like getting older – it happens so slowly over time that you don’t notice the changes on a day to day basis.  Yes, my eyes are worse than they were 5 years ago, but I can’t exactly define how worse.” The questioner typically changes the subject as abruptly as they started it, leaving me to wonder if I’d given a clear enough answer.

A recent trip to a retina specialist at the Casey Eye Institute provides an updated answer to the “how are your eyes” question for those interested in details. Continue reading “How Are Your Eyes?” →

Do Better

My husband and I like to tease a friend of ours for his harsh response to a fast-food worker who messed up his order 3 times.  She had all sorts of excuses for why she kept messing up the order, and he was sick of listening to it, so he looked straight at her and said “Do Better”.  We thought it was a bit of an over-reaction and didn’t offer her much grace.  I know we all make mistakes, especially at work.  But after the week I’ve had, I feel like I want to shout “DO BETTER” to the world.

I hesitate to even write this post as I don’t want it to come across as a wild rant or pity party.  I doubt most people enjoy reading a rant any more than they like listening to one.  So I will try my best to explain what the last 3 days have been like for me without overstating my frustration.  I also want to apologize in advance for the amount of details I’ve included in the below timeline of events.  It’s boring to say the least, but I think the details are necessary to understand my story.

On Monday, I had an appointment at Seattle Harborview Medical Center’s Eye Institute.  My husband took the day off work in order to drive me to and from my appointment and keep me company through the long list of tests I needed. (note: We live 2 and half hours away from the eye clinic.)  It had been quite some time since I had all the tests necessary to make sure I don’t have glaucoma or cataracts (people with RP are more likely to develop those conditions), visual acuity exam, peripheral fields test, and pictures of my retina.  I needed this comprehensive list of tests in order to apply for cane training services.  When I arrived at my appointment, I handed the medical technician a 5 page form from the WA Department of Services for the Blind (DSB).  She looked over the paperwork and told me the doctor would be able to complete the forms after all the tests were finished.  I then spent the next 5 hours in Harborview’s eye clinic, most of which was spent sitting in the waiting room with elderly people. 

During my 5 hour stay, they did a basic eye exam (charts, letters, lights – something similar to what most people receive at their optometrist), received drops for the pressure tests (glaucoma, cataracts), and posed for a glamorous retina photo shoot.  I was examined by several resident doctors and observed by a young medical student as well.  Four and half hours into my appointment, I finally got the chance to meet with Dr. Chou, the ophthalmologist and retina specialist.  She explained that she was sorry for the misunderstanding, but I had been scheduled at the wrong clinic.  I should have been at the clinic across the street where they do all the testing for retinal degenerative diseases (field tests, laser scans, etc).  She was not sure why the person scheduling my appointment had not sent me there in the first place or why no one had caught this mistake earlier in the day, but assured me she would speak to her clinic manager about the issue.  She was unable to complete my paperwork for DSB because the only tests they had completed showed that my central vision was still in tact and well above what would qualify for any type of services.  I was supposed to have my forms complete for my DSB appointment the following morning, so I had to change my DSB appointment to Wednesday afternoon and go back to Harborview on Tuesday to have the correct tests.

Luckily I was able to stay at a friend’s house in the Seattle area, but my husband could not drive me to the new appointment because he had to work.  So I paid $47.50 for a taxi to take me to Harborview on Tuesday morning.  I requested a reimbursement from Harborview considering their scheduling mistake, but was told “that’s not something we do”.  Once I arrived at the correct clinic, the tests and paperwork were completed in under an hour with no time in the waiting room.  Now fast forward to my DSB appointment on Wednesday afternoon.  Just getting to the DSB office was challenging in itself because my morning sickness suddenly returned while I was in the backseat of a swerving taxi that smelled strongly of curry mixed with incense.  I somehow managed not to lose it in the cab, but I was green with nausea by the time I stumbled into the DSB office.  A kind case manager shared her crackers with me and brought me water.  I then met with an equally kind case manager who asked me all sorts of questions about the type of work I had done in the past and what type of work I would like to do in the future.  I was confused by her questions as I thought I was at this appointment to talk about my vision challenges and cane training.  When I steered the conversation away from work and towards cane training, the counselor was the one who looked confused.  She then explained to me that the DSB mainly provides job services for people with vision disabilities, and that they don’t have funding for just cane training.  She profusely apologized for the misunderstanding, but told me that the person who scheduled my appointment should have asked me if I was looking for cane training as part of an active job search.  She also indicated she had a feeling she knows who I spoke with initially and that she will follow-up on the issue.  This was all beginning to sound too familiar.

I am a well-educated, intelligent, assertive, middle-class, fluent English speaker who cannot effectively navigate our health and government services.  I grimace at the thought of what elderly, uneducated, mentally disabled, immigrant, limited English-speaking individuals go through to obtain the proper health care and government services they need.  I know there are plenty of sick people and disenfranchised individuals that have to trudge through this confusion on a daily basis, and I honestly cannot comprehend how they do it.

Now here’s the rant I promised not to indulge in.  Considering the current economy where so many people are out of work and the job market has grown more competitive, I am surprised that Harborview and the DSB cannot find more competent people to answer their phones and schedule appointments.  I know that I have a rare eye disease that not many people are familiar with, even within the medical field, but all these individuals had to do was spend a few extra minutes on the phone with me to avoid a whole mess of confusion that cost me time and money.  Looking back, it seems that the main goal of these receptionists was to get me off the phone as soon as possible rather than responding to my actual requests.  It would have taken very little effort for them to “do better”.

My mom always encouraged me to see the positive in any situation, so here goes.  The Harborview ophthalmologist invited me to participate in a stem cell research project she is working on following my pregnancy.  Although she cannot promise any grand results, it is a rare opportunity for me to see how my actual stem cells react to various trial drugs. (they use my cells in a petri dish, so it is non-invasive) In addition, I learned a lot about DSB services that could benefit me in the future when I return to work.  Meanwhile, I am looking into other options for cane training in my state.  I’ve decided that I will ask to speak to multiple individuals before scheduling my next appointment to make sure I am being told the correct information over the phone.  Then hopefully I can avoid telling anyone to “do better”.

Return to Fishman: Part 2

If you’re wondering what these two ultrasound-looking photos are, they are actually photos of my retinas taken at my most recent appointment. Like I said in Part 1, I learned a lot at this appointment, and the photos were just part of this wealth of information.

While I knew that I had a form of RP known as Lebers, I hadn’t realized that a 1995 blood test revealed the specific RP gene that Jenelle and I have; it is called the CRB1 Gene. This gene, unfortunately, is not the specific gene in clinical trials for gene therapy right now (the treatment that Dr. Fishman feels is the most promising within the near future).

Another gene, the RPE65 gene, is currently in human trials, and from what I gathered from Dr. Fishman, this is mostly due to the fact that it is one of the more common RP genes and that patients with RPE65 tend to have their central nuclear still intact, a necessary requirement for gene therapy

This is a new term I learned today, and they actually took pictures of my retinas to see if my central nuclears were still viable, meaning that the cells are alive and could therefore be receptive to gene therapy. This is, of course, all hypothetical considering the CRB1 gene is not currently in clinical trials. I naively asked whether it would be the next gene in trials, not realizing that there are many other RP genes that have also been identified that would be in the running. He said that research studies are more likely to take place with the genes that affect the most amount of people first. When asked how common the CRB1 gene is, he said it’s right in the middle. So there may be some waiting. He said that the results for the RPE65 gene are promising so far, however, and that once the initial FDA-approval process is over with, the following trials will be able to move at a much faster pace than the first one. Of course, even if the CRB1 gene begins human trials in the near future, Dr. Fishman pointed out that there are still many risks to weigh in whether Jenelle and I would want to volunteer to be among some of the first groups for treatment, especially considering they have to detach your retina during surgery, which could potentially decrease vision even further.

Dr. Fishman himself is involved more directly in some pharmacological studies that do similar work as gene therapy but are taken as medication rather than surgery. Some patients have had improved fields from this drug study, though the patient he was specifically testing did not have improved fields (but improved his acuity by 17 letters, which is substantial).

These are pictures of my retinas. The dark area in the periphery is scarring, or as Dr. Fishman put it, “charcoal ashes from the fire that is the disease”. The small white area in the center shows my central nuclear, which is still intact, though it is a very thin layer. The center of everyone’s retina is thin, but is extremely thin in patients with RP.

As far as other results from this visit, one major disappointment from Dr. Fishman moving offices is that a lot of records did not make it over yet, including my previous visual fields tests. I had been anxious to compare the results of today’s test to previous tests, but such is life Hopefully they will eventually find all of their files.

I did feel like my fields were pretty narrow during the test, but I think I am always surprised by how much I really can’t see when I take the fields test. Marty, my favorite technician who was doing my test, was particularly astonished that in my left eye– the one that can’t even see the giant E on the chart at 20/400, could still see a tiny pen-light in the center of the field test “All-be-darn” was his exact quote. Again, since they don’t have my previous records at the moment, I have no idea whether this is something I could see during my previous tests 3 years ago. While I did a fields test at my local eye doctor in Naperville this past fall, the test was not nearly as thorough, and the technician got really confused while doing the test because she had never done a test on someone with such low vision before, so I can’t use that one to compare to either.

Marty also said that he finds it fascinating how I am able to even walk from the waiting room to the exam room without any problem. He remarked on how the human brain compensates for the missing pieces of vision and fills vision in to make things look like a whole picture when there should really be no way to see the whole picture at all. He said with the vision I have, even walking around should be nearly impossible, but because my brain has filled in these pieces, I’m able to (even if it’s not always very well!) While I have had many of these same thoughts before, I was never sure if they had any actual scientific basis to them before hearing a professional say them.

As I said in Part 1, Dr. Fishman’s care for his patients was particularly evident to me during this visit I’m not sure whether it’s the new office environment or whether maybe I’m just better at asking specific questions now as an adult, but I feel like he took such care explaining extremely complicated scientific information in simple terms that I could understand.

I definitely plan on recording my next visit so that I can actually listen to it once I get home since there is way too much information to even begin to remember, much less reiterate to my family and friends later. Until then, I hope my modest attempt to convey pieces of info from the visit is helpful. Thanks for reading!

Return to Fishman: Part 1

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So I visited my childhood opthamologist and world-reknown RP researcher, Dr. Gerald Fishman last week. I have to say it was one of the most eye-opening visits I’ve ever had with him (and this is both pun and literal– you can always coulnt on your eyelids being pried wide open during an exam).

Back in the fall when I wrote, “Trip to Opthamologist” I was pretty darn honest about my memories of my visits with Dr. Fishman growing up, and I have to say that as I sat in his office, I kept cringing imagining if he were to ever see what I wrote “Fishbreath” isn’t the most flattering descriptor for a respected doctor.  (not that a famous researcher would ever be browsing through doublevisionblog!)

Dr. Fishman is now at the Lighthouse for the Blind in Chicago (rumor has it that it was a political upset that led to the switch from UIC Eye and Ear Infirmirary to the Lighthouse). I found it interesting that it wasn’t just the doctor himself who moved practices– his sidekick technician, Marty, who has been with him since 1996, was there to razz me with his usual goofy comments, so it felt like a little reunion of sorts, as I had not been to an appointment for a few years.

He also still had a Fellow working under him, Dr. Collingsby, and I kind of wonder if some of these younger doctors have been the ones teaching their mentor how to improve his patient-communication skills just as he has been teaching them about retinas, as I notice Dr. Fishman has become more personable over the years.  Or maybe it’s me getting older.  Or the new building he is in.

Dr. Collingsly examined me first, and though there were the usual bright lights shone stingingly in my retinas, he had clearly brushed his teeth and carried the aroma of soap, which beats many other potential smells.  He did still emulate Dr. Fishman’s audible, scientific terminology during the exam….. “asteroids in the center vitreous…..look up please……scarring in the outer……all the way to your right please….white lipids…..” which always cracks me up because I have no idea what any of it means, and most of it sounds like they’re looking through a telescope at the solar system, not my retina.

While I think part of the audible descriptors are for the sake of educating the doctors-in-training, the fact that Fishman mutters these terms to himself even when there are no other doctors present tells me that he is simply engrossed in what he does (or he says thing aloud to help remember them in order to write them on my chart maybe?)

True side story today: As Dr. Fishman has my left eye pried completely open with a bright light shining directly on my retina, he says something about looking at a beautiful universe of greens and blues and pigment mumbo jumbo and then mumbles “nothing to do with the RP but I can’t stop looking at these” and then chastises himself, saying “come on Fishman, back to work!” And I don’t think this was crazy-talk or senility or anything. I think he is so enamored with the human retina and all its details that he really, really loves what he does.

At one point I asked him the round-about question I always end up asking. And even though I know he can’t give me a clear answer, I can never stop myself from asking it in some form. So today I worded it, “So I think I remember you telling me that most patients you’ve seen who maintain usable central vision like I do are able to hang onto that bit of central vision for quite awhile”. And he said that’s true of people with 20/40 or better, and since I’m at 20/50, it’s really hard to tell. But what really got me today was that he kind of touched my upper arm and said genuinely, “Because each RP case is so different and there’s not a typical path for anyone, I cannot predict the outcome that you deserve to know.” He said I deserved to know what will happen with my vision. It isn’t possible, of course, but he said I deserved to know. And since I didn’t tape record him, I don’t even remember the exact quote– I think he said it better than what I quoted, but for the first time I felt his sadness in not being able to really help his patients– or even give them an accurate prognosis. How frustrating as a doctor.  Not to mention as a researcher who has devoted their career to finding a cure.

So I’m looking at this doctor– this researcher with the bow tie who speaks in scientific terms with fiery breath way too close to my face.  I stare at this small man who is frail and balding– the same one I dreaded seeing annually as a child– and he no longer appears as the villain. As a kid, your mind interprets events in crazy ways, and these difficult visits filled with bad news, poking and prodding definitely painted the doctor as the antagonist.

And I guess at this visit I grew up and saw how this “antagonist” has devoted his life to RP research well beyond the age most doctors retire (Marty-the-sidekick said he doesn’t know the definition of retirement!  Marty also would not tell me Dr. Fishman’s age, and I thought it would be weird to ask the doctor himself!).

Before leaving, Dr. Fishman asked, “have I answered all your questions?” and after saying yes and walking out of the office, I noticed he had been answering my questions for over an hour (on top of the 2 previous hours of tests)– an insane amount of time to spend with a patient these days.

And that’s the main reason I will make the trip to the city to see him each year instead of simply going to my local eye doctor.  I think any retina specialist can check for cataracts or fluid leaking in my retinas (two risks with RP), but I go to this particular specialist for the education. Yes, I could look online, but when you’re looking online, you really have to piece together a lot of information that may or may not be accurate.

For an update on what the appointment actually found vision-wise, please read part 2!

Trip to the Opthamologist

So I went to the Opthamologist a couple days ago.  It was my first visit in about three years, and it was just with a local Opthamologist near my house as opposed to the specialist I have always gone to downtown Chicago.  I received a letter from my specialist about a year or so ago, saying that he was retiring, and since he was the main reason I went to that clinic, I opted to just get a referral from my FP.  For some reason, I thought that going to a new office would lessen my anxiety, since I have never liked going to the eye doctor, but I found myself a nervous wreck the morning of my appointment.Growing up, I always dreaded the day-long visit to our Opthamologist in Chicago.  His name was Dr. Fishman, a world-reknowned RP researcher and specialist, and he was the epitome of a stereotypical nerdy professor, complete with bow-tie and medical terminology that went way over my head in conversation.  Since his office at UIC was a teaching hospital, I could always count on a couple “trainees” standing over me in the dark as the brightest-possible light from his nifty head-lamp shone directly in my pried-wide-open eyes.  “Hold it there….steady…” I remember him saying, breath in my face (in hearing about the visits growing up, my husband affectionately nick-named him “Fishbreath”, as he could have used a good breath mint).

“See these islands and pairing here with the red pigments….” he’d begin, and for about 20 minutes in that dark room I was not Joy– a living, breathing being with hopes, dreams, fears, and feelings– I was a lab rat demonstrating the atrophy and rapid decline of millions of cells in the retina.  And since I knew, even on some odd level as a child, that this doctor was doing this to hopefully find a cure one day, I’d succumb for the most part, wincing here and there when it became too uncomfortable, but mostly I’d will myself to think about “happy” things– plans for later in the day (as a child, the trips always involved stopping at Ed Debevics for lunch afterwards, so that gave me something to look forward to!)

The remaining parts of those visits usually entailed a standard eye exam, such as reading charts, dilating eyes, glaucoma test, etc.  And, of course, the dreaded visual fields test, which didn’t hurt at all, but always caused anxiety since I knew that its results would tell me how much periphereal vision I had lost since the previous visit.  I remember trying to fake the technician out as a pre-teen.  I was only supposed to “beep” the little buzzer when I saw the light, but I’d beep it randomly, hoping that it would “up” my scores and that they’d tell me I had more vision than I did.  Looking back, I wonder why I would want them to report a higher number since it obviously wouldn’t change the fact that my narrowing fields still affected my daily life.  Plus, if I couldn’t fake them out as well the following visit, it would make my decline look even worse (yeah– I was not the most logical thinker as a 12-year-old).  After awhile, the technician was on to my games, however, and asked me to state where the light was each time I hit the buzzer. Busted.

It was not the visual fields test that worried me a few mornings ago, however.  Going to a new office, I suddenly began to think about some of the “once-in-awhile” tests that I’d have to undergo as a child– the ERGs, the photos of my eyes, and the placement of hard contacts in my eyes (can’t remember why they did that, but it hurt!)  These more invasive procedures did not occur at every visit, but they were definitely tests that went on during those preliminary visits when they were first trying to diagnose my sister and I when we were kids.  And since this was a first visit at a new office, I wondered whether they’d need to do similar tests.

I really didn’t need to worry.  In fact, by the end of this visit, I was the one practically begging for more tests.  Even though this was an appt in a major metropolitan area with a retina specialist from a major medical group, it was probably the most unthorough, basic exam I’ve ever had.  While I’m not sure that’s necessarily a good thing, it definitely put me at ease.  I was mostly just confused about the technology while I was there.  That, and the knowledge of the technician.  He seemed to know nothing about RP, and when I asked whether I was going to have a visual fields test, he looked confused and went to ask another technician.  I overheard him talking to two technicians in the hallway, asking whether he should put orders in for a visual fields test.  I heard one of the technicians ask, “Retinitis Pigmentosa?  Isn’t that night blindness?” and another technician chime in, “Just do a family history on her.  I’ve only seen the doctor order one of those fields test one time.”  And this technician wasn’t joking; there wasn’t even a technician who knew how to do the field test on staff that day, so I had to schedule it for a future date.  And the funniest part was that the technician decided to do his own “makeshift” fields test on me when he returned, having me just stare at his nose and tell him when I saw his finger.  I had trouble concentrating on the “test” because I kept analyzing his oddly-shaped nose in my head, trying to figure out why it was so much shinier than the rest of his face.

So when another technician came to take me to get photos of my eyes, I was expecting the same rudimentary-type scenario, especialy since I remembered having my eyes held open with Q-tips as a child while photographs were taken.  To my relief, there was an amazing machine that took pictures easily without any need for pried-open eyes or Q-tips.  She kept apologizing while taking the pictures, saying “I’m sorry this is so uncomfortable” because she spent a lot of time readjusting my head to get just the right angle.  I tried to explain to her that this was pretty much a spa treatment compared to my UIC appointments.

When looking at the Opthamologist’s name before he entered the office, I was expecting a man with a thick accent to walk in the room, and i imagined that he would throw around medical jargon like Dr. Fishman did.  But within 1 minute of meeting him, I could tell that he was completely different from my lifelong specialist.  He had a full set of hair, wore snazzy clothes and cologne, and was about as personable as they come.  I have to admit that I was very skeptical of this younger hot shot, especially after having to explain RP to his technician, but I soon realized that he’s a retina specialist for a reason. He spoke very highly of Dr. Fishman (who, by the way, is now volunteering at the Lighthouse for the Blind and cares very much for his patients despite my lab-rat comment).  He was up-to-date on all the latest research, and he was definitely the most hopeful-sounding eye doctor I’ve ever spoken to.  At one point, he looked right at me and said, “With all the break-throughs in stem cell therapy, there will very likely be a cure in your lifetime that will not only stop degeneration but will most likely reverse it as well.”  Wow, I think my parents, sister and I would have flipped over if we had ever heard Dr. Fishman say that 20 years ago.  I remember my dad trying to press Dr. Fishman for some hope, asking very specific questions about when and if a cure would be found, and Dr. Fishman was always careful with his wording and would say “It’s possible”, but usually with nervous hesitation.  I think he tried hard not to be misleading, but he definitely didn’t leave us with a feeling of hope.  I usually had to get that from my parents after eye appts.  I remember my dad always saying, “Science is moving ahead every day, and I truly believe that there will be a cure in you girls’ lifetime”, and I remember I loved hearing him say those words, and growing up I believed them.  But there defintiely was something different about hearing a doctor say it that gives me hope as an adult.

While it gives me hope for the future, my sister and I are getting a little tired of waiting for western medicine to come up with a cure.  We’ve begun to look at other routes, specifically eastern medicine, and we are equally intrigued by what we have found.  In fact, my sister’s next post will talk about our upcoming treatment that we will be undergoing in just 3 weeks!  Stay tuned…..