Last Saturday, the National Keratoconus Foundation hosted its 2018 Keratoconus Family Symposium at UC Irvine. I had the pleasure of attending and even the privilege of speaking at it.
A bit of background for dry eye readersFor those who have never heard of it: keratoconus is a rare corneal disease affecting at least 1 in 2,000 people (though one of the speakers at the event voiced his opinion that it's actually much more common but frequently goes undiagnosed). I'll borrow directly from the NKFC website for a definition:
This is obviously not a Dry Eye Thing, so a brief word about how and why I got involved is probably in order. Of course, many keratoconus patients have dry eye, so I have known many over the years just in the normal course of Dry Eye Company 'business' whether through blogging, emails and conversations, or actual retail.
Keratoconus, often abbreviated to “KC”,
But the closer connection for me with keratoconus patients is through scleral lenses, which are commonly used for correcting the vision impairments keratoconus can cause. (I too have vision not correctible with other types of lenses though for different reasons.) Actually, I first came across keratoconus way back in 2002 or so when I was doing advocacy work for people with complications from LASIK, because there is a medically induced version of keratoconus which we tended to refer to as post LASIK corneal ectasia. These days, because the Dry Eye Shop has expanded more and more into scleral lens supplies, questions that flow in through the shop are the main way I keep up a lot of communication with keratoconus patients, as well as our Facebook group, My Big Fat Scleral Lens, and it's through scleral lenses that I came to know the current director of NKFC, Mary Prudden, who was responsible for organizing this excellent event.
So, on to the actual contents of the day:
Gloria Chiu OD (USC-Roski Eye) provided an excellent framework for the presentations of the day by giving an introduction to keratoconus with an overview of its causes, onset, and progression as well as the treatments, which are pretty much limited to specialty contacts and corneal transplants, though corneal crosslinking is now being used as a preventive treatment for younger cases. If I'm not mistaken, Dr. Chiu is the only PROSE fitter west of the Mississippi. It was a joy to meet her for the first time as I've known a great many of her patients over the years.
Mindy Hutchinson MD (Vita Behavioral Health, PA) talked about the mental health impact of eye disorders. She was diagnosed at the age of 26 with keratoconus, of which she had never heard during medical school. She walked us through her own path and discussed anxiety, depression, learned helplessness and what it looks like to take back control, including education, accommodation, advocacy, and self-care.
Vivian Shibayama OD (UCLA-Jules Stein) presented on contact lens options for different stages of keratoconus, including: soft torics; corneal RGPs; topography guided RGP designs; piggybacking; hybrid lenses; and finally sclerals. She addressed some practical questions such as about refilling lenses during the day. In a second presentation, she covered scleral lenses in more detail including multifocal optics, EyePrintPro molded lenses, Hydra PEG coating, and various questions relating to solutions for both soft and rigid lenses.
Incidentally, I was curious about the current status of piggybacking - that's something I tried back around 2003 but was not able to tolerate the soft lenses. I wondered whether scleral lenses have made piggybacking obsolete but clearly they haven't - the cost and commitment factors in sclerals are such that the simpler, more cost-effective approach of piggybacking is better for some users who are still able to tolerate lenses on the cornea.
Yours truly presented on practical pointers for scleral lens users including those with dry eye, but you've heard so much from me I won't regurgitate it here!
Marjan Farid MD (UC-Irvine/Gavin Herbert) presented on corneal transplantation techniques. She discussed some current challenges such as rapid visual recovery, astigmatism management and getting patients out of contacts, and improvements in targeting different procedures to specific diseases. Femtosecond laser technology is bringing great improvements due to precisely targeted cuts and she presented on this area in great detail, particularly the zigzag method, including videos of DALK employing femtosecond zigzag cuts on the donor cornea and the receiving cornea. Questions she addressed included: "Does crosslinking ever lead to transplants?" (hopefully infrequently), "Can you get KC after the transplant?" (transplant is only 8-9mm so there is less risk of ectasia), and "How often are sclerals still required after transplant" (fit is better after, so "a lot" don't need sclerals).
Sam Garg MD (UC-Irvine, Gavin Herbert) presented on the hottest topic of the day: corneal cross-linking (CXL), which was finally FDA approved just over a year ago after having been used in Europe for many years. He started with some broader keratoconus background including risk factors (incl. Down syndrome, genetics, eye rubbing, connective tissue disorders) and also mentioned - new to me! - that the cornea is the driest tissue in the body! He explained that, like so many medical interventions, we don't know how crosslinking works, we only know that it seems to be effective at stiffening the cornea. He presented a great deal of technical background on the treatment and how it is employed. It is only approved for patients 14 years old and older. Candidates are those with keratoconus or post LASIK ectasia that is demonstrated to be progressive (though he said that with the younger ages they do not necessarily bother documenting progression first as it can be assumed to be progressive in young patients). He discussed what the procedure and healing period are like and mentioned potential complications such as haze and sterile infiltrates. There is debate about "epi-on vs. epi-off", that is, whether to perform it with or without the corneal epithelium in place. Dr. Garg addressed many audience questions, including: "When does KC historically stabilize?" (it's a spectrum), "How soon is a CXL patient able to wear contacts" (2-3 months or more), "Can it be done on younger than age 14" (off-label, yes), "Will there be any insurance coverage" (Avedro is pushing hard for this), "How much does it cost" ($5-6k with current riboflavin costs), and "How and when is patient satisfaction measured" (this question from yours truly and the answer is that there has been no formal measure of it).
Elio Spinello PhD (CSUN-Northridge) presented on various pitfalls of medical information on the internet, focusing on a variety of tests and red flags to look for when trying to determine the reliability of a given source. He emphasized the importance of having medical providers you trust and presented data on the role this plays in the extent to which people rely on what they read.
Wendy Pawling (UC-Irvine voc. rehab consultant) presented on accommodations in the workplace as well as school environments. She discussed relevant Federal and California disability laws with the aim of informing and equipping people with visual disabilities (assuming they are able to perform their essential job functions) to be able to request accommodations and to know what to expect and what an employer is, and is not, obliged to do. She discussed the interactive nature of the process and privacy limitations; for example, employers are not allowed to ask you your diagnosis, nor can they contact your doctor(s). She gave examples of accommodations she's familiar with at UCI, from equipment or software to modified work schedules.
Rachel Dungan MSSP (NKCF patient advocate) wrapped up the presentations with a powerful, moving talk including her personal story. She shared how keratoconus progressively and profoundly impacted her education and professional trajectory as well as her mental wellbeing for some years before she was diagnosed, and the many ways in which the experience as a whole has changed her. She discussed what patient advocacy and patient empowerment look like, how patients can educate themselves as well as those close to them about their needs both in visual health and emotional health. She described hopeful signs that patient-centered care is being increasingly valued.
The presentations were followed by a tour of the Eye Bank at Gavin Herbert Eye Institute as well as workshops on stress management, advocacy and scleral lens and dry eye troubleshooting. Somewhere in there we also had lunch. Aidan (assistant, who accompanied me to the event) and I sat with a family whose 17 year old son was recently diagnosed. The conversations with the parents were probably the most impactful part of the education I received that day, as they told what it's really like trying to help a young person at such an intensely vulnerable time of life come to grips with having a rare eye disease, as well as the many practical and financial considerations impacting their whole family.
Many thanks to Mary Prudder and NKCF for this wonderful event! I learned so much, and met a lot of wonderful people. It was a great day. - Also, thank you to Aidan for taking great notes for me so that I could just sit back and listen and absorb.