KeraClub is a community event organised by the Save Sight Institute (SSI) to link keratoconus (KC) patients with researchers and clinicians. The event is a collaboration between the SSI, the Sydney Eye Hospital (SEH), and Keratoconus Australia.
The event, held in SEH’s Claffy Theatre on October 24, was opened by accomplished violist and violinist Ms Michelle Urquhart. One of the event’s organisers, she has already clocked up nearly two decades of living with KC.
Thanks to her positive outlook, as well as the experienced presenters on the night, the tone of the whole evening was quite upbeat. This was despite the bulk of the audience having, in some cases, first-hand experience of living with KC for several decades.
Almost 60 people were in attendance, and a significant number of others viewed the event via a livestream.
Real experiences
Long-term KC patient Ms Joy Alleyne gave her experience of coping with KC and sought, successfully, to demonstrate that the disease need not hinder them from living a normal and lengthy life.
It would seem that, provided corneal grafts are not part of the equation, most activities can be undertaken unhindered. If eyes have been grafted the consensus was that contact sports should be deleted from the social calendar, but most other activities were still possible.
Her care started with spectacles in the 1970s, followed by CLs for the next 15 years. She noted that the wearing of CLs deterred knuckle rubbing, a well-documented, induced behaviour in KC patients. CLs gave her an unrestricted field-of-view, better eye-hand co-ordination, better depth perception, and greater confidence. Coloured CLs also featured in her life.
She advised CL-corrected KC patients to insert their CLs first thing in the morning (ditto for those using spectacles) so that they adjust to their correction from the beginning of each day.
In parting, she advised the audience to follow the instructions of the professionals consulted, don’t let KC define who you are, get on with life, and only do what is necessary.
Melbourne-based Ms Ana Sandoval has much less experience with KC but underwent corneal cross-linking (CXL) in 2017 and 2019. She still experiences some hazing of vision as a result of the procedure in one of her eyes. Her initial correction was a pair of GP corneal CLs which were followed by piggyback CLs, but the latter was replaced with scleral CLs following problems of pain and allergy.
However, about 8 hours CL wear per day is her limit and eye redness continues to be a problem. Surprisingly, she has not found any other KC patients in her area.
Professor Stephanie Watson
Professor Watson is well known in Australian ophthalmology circles and has a key role in SSI’s Keratoconus Registry. She is also head of the Corneal Research group at SSI.
She reported that SEH was now ranked 6th in the world among eye research institutions. She gave the prevalence of KC as being as common as 1 in 600, but figures are dependent on ethnicity, geography, and other factors, not all of which are known or understood. She believes that KC affects a patient’s quality of life more than AMD.
One of the motivating factors behind the KC Registry is that relatively little modern information is available on the disease despite it being known for millennia and documented for more than 300 years. Even simple issues, such as the natural course and history of the disease over a lifetime, are poorly documented.
Much of what is available is old and involves techniques and designs that can now be avoided.
Although the Registry was commenced to better understand the methods and outcomes of CXL, it has now morphed into a comprehensive, Australia-wide database of KC.
Currently, the Registry holds data on 22,000 professional visits involving 2,200 eyes of 4,000 patients and is growing at a rate of about 10% per month. An optometry module is now also part of the Registry.
Of particular interest are key indicators such as Ks, KMax (cases whose KMax > 55 D have a greater risk of progression), the faster rate of corneal changes in the young, and changes following CXL. Participation in the Registry also constitutes a RANZCO-approved clinical audit.
Known downsides to CXL include scarring, hazing, slow healing, persistent epithelial defects, MK, and corneal infiltrates. Watson stated that the Keratoconus Outcomes Research Questionnaire (KORQ) was the best way to assess quality of life.
Dr Jim Kokkinakis
Dr Kokkinakis’ practice is firmly centred on CLs, especially difficult or non-routine cases such as KC. He acknowledged that every KC case was different, but with appropriate management KC patients can do just about anything.
He dismissed several KC myths he finds common. They include: life is over, you’ll go blind, rigid CLs will stop KC progression, you will not get a drivers licence, and Intacs (and other stromal rings) will make CL fitting easier.
He often finds spectacles to be an adequate correction at first, but CLs are usually required eventually. His ‘contract’ with KC patients is simple; he undertakes to fit the CLs properly and they promise not to rub their eyes and sit in his chair as required.
Furthermore, he also forbids: swimming, showering, or sleeping in CLs, the use of tap water at any stage of CL care, climbing Mt Everest (the partial pressure of oxygen in the atmosphere at high altitudes is inadequate for safe CL wear), and not be tempted to wear CL camping or overnight in the bush or when hygiene is poor.
He also advised, as do most surgeons, that surgery only be done when all other options have been explored and exhausted. A graft’s expected life cycle remains on average around 15 to 20 years. Longer life is possible, but the graft’s condition is usually sub-optimal in the longer-term. Artificial corneas seem to still be quite some time off, but research is ongoing.
In terms of careers, aviation, with the probable exception of passenger planes, is possible but defence and police careers were given a “maybe”.
Panel Discussion
From the panel discussion that wrapped-up the evening’s programme, it was learned that KC is only about 10% genetic and the causes are complex. It can occur in children as young as 8, CL fitting can be a difficult process and some Pasifika races have KC is an many as 1:50 of their population. For example, in New Zealand KC males are more common than KC females and seem to have a higher allergy rate. Additionally, KC is seldom a symmetrical disease.
Often the eye adjacent to the dominant hand has more advanced KC because of more ‘effective’ eye rubbing. Rubbing harder is worse, but a motivating factor is that it decreases heart rate and is more relaxing as a result. The use of preservative-free saline to fill the bowl of scleral CLs was also mentioned.
Current data suggests that grafts have a 90% survival rate at 20 years, but up to 20% of cases start a graft-rejection process that requires pharmacological suppression. Signs of pending rejection include: redness, soreness, and blurry vision.
Other associations with KC include: sleep apnoea, especially if the patient is >30 years of age and the KC is of recent onset, those with a connective tissue disorder, Down syndrome, Ehlers-Danlos syndrome, and a heart-valve prolapse. Diabetes has no KC connection.
While grafts can be lifesaving, surgery can only follow a comprehensive risk vs. benefits analysis and, ultimately, the choice to be operated on is up to the patient in consultation with their ophthalmologist.
Urquhart ,who hails from the NSW Central Coast, is endeavouring to form a local cell of Keratoconus Australia closer to her home, but so far has identified few cases locally. Anyone interested can contact her through the SSI Keratoconus Registry or Keratoconus Australia keratoconus.org.au.
By Lewis Williams
