The KeraClub – an annual event for people with keratoconus, their families, friends, and carers including health care workers – has become an important event in many people’s calendars.
KeraClub 2021 – the sixth annual community event for patients with keratoconus – was co-hosted by the Save Sight Institute and Keratoconus Australia as a webinar. Understanding patient perspectives and the latest research in keratoconus management methods, including contact lenses and surgery, were the highlights of the session.
The 2021 event attracted registrations from 319 people from around the world, with the majority from Australia (Figure 1).
KeraClub 2021 was chaired by renowned violinist and violist, Michelle Pritchard, who has a living experience of keratoconus having had three corneal transplants and graft rejection episodes.
In her opening address Ms Pritchard said, “This is always an exciting part of the year. It is when we all get together to learn the latest research (in keratoconus) and share the lived experiences of this disease and support each other. Some of us have just been diagnosed, and some of us have had it for a long time. We often have parents of patients as well”.
IMPACT OF COVID-19
Professor Stephanie Watson, head of the Corneal Unit at the Sydney Eye Hospital; head of the Corneal Research Group at Save Sight Institute; and the Chair of Ophthalmic Research Institute of Australia, presented on managing keratoconus in the COVID-19 world and innovation in keratoconus management. COVID-19 has been found in corneas but is unlikely to be transmitted with a corneal transplant. The cornea has no lymphatics or blood vessels normally, and the betadine used for sterilisation when harvesting cornea during corneal transplantation inactivates or kills the COVID-19 virus.1 COVID-19 virus may cause mild transient conjunctivitis, but this is generally when the person already has systemic COVID-19 and is probably not associated with keratoconus.2
The Save Sight Keratoconus Registry data have shown that a lower number of keratoconus patients are attending clinics and a lower number of corneal cross-linkings have been performed since the COVID-19 outbreak. This is a great concern as, if patients with keratoconus are not diagnosed and reviewed, their keratoconus may progress to a point when glasses or contact lenses can no longer correct their vision.3 Similarly, delay in treatment of red-eye, particularly with contact lenses, may lead to scars in the cornea and serious consequences such as reduced sight. Therefore, keratoconus patients with red eyes, particularly contact lens wearers, should seek early treatment.
“Rarely, cases of corneal graft rejection have been reported following COVID-19 vaccination, and most importantly, the most cases of rejection can be treated avoiding graft failure.4 It is therefore important to get a COVID-19 vaccine and seek treatment if you get a red eye afterwards,” said Professor Watson.
INNOVATIONS IN TREATMENT
Professor Watson also discussed innovations in treatments that are under development, such as eye drops to induce corneal crosslinking. These drops are currently being evaluated in clinical trials and are not available in the market; more evidence is required to demonstrate if they actually work. She also discussed innovations in the surgical management of keratoconus. Historically, up to 20% of keratoconus cases required corneal transplantation.7 Now, with better fitting contact lenses and the availability of corneal cross-linking, there has been less need for surgery. There are also new surgical treatments available, such as intracorneal ring segments, partial corneal transplants (deep anterior lamellar keratoplasty, DALK), laser procedures sometimes combined with cross-linking, and intraocular lenses. These surgical procedures may be necessary when contact lenses are not working and quality of life is affected. The type of surgery is determined by various factors, including vision in each eye, quality of life impacts, corneal thickness and shape, and the risks of adverse events.
Professor Watson highlighted updates from the Save Sight Keratoconus Registry (SSKR). The SSKR is a web-based international database of patients with keratoconus.6-8 Clinicians can enter their patients’ data to monitor their outcomes and compare to benchmarks. Analysis of the data can improve keratoconus management, determine who needs treatment and when, decide which treatment to have, and find out associations between patient factors and clinical outcomes to improve clinical care. The registry has been crucial to understand the outcomes of new therapies and provide answers to the solutions to transform lives into the future.6-8
Associate Professor Mark Roth OAM talked about dry eye, allergy and contact lens tips. He shared clinician perspectives and clinical advice on managing dry eye and allergy in keratoconus patients, which was well received by the participants.
PATIENT SUPPORT AND ADVOCACY
A/Prof Roth’s talk was followed by a talk from Larry Kornhauser, OAM, the inaugural and current president of Keratoconus Australia, who has lived with keratoconus from a very young age and has undertaken corneal transplants.
Keratoconus Australia (www.keratoconus. org.au), was established in 2000, and is managed by volunteers; some of them with keratoconus and others the parents of children with keratoconus. The organisation has been a platform for supporting thousands of patients with keratoconus and their carers, and is heavily involved in research, advocacy and governance in areas related to keratoconus care.
The Keratoconus Australia website has information about keratoconus, with answers to common questions. The organisation also provides people with useful bulletins, corneal transplantation and cross-linking brochures, and public information seminars. Additionally, it operates a helpline and support for patients and their families.
“People with keratoconus spend half of their lives trying to explain things to their families and friends – even the basic things like why they need rigid contact lenses, not the soft lenses or glasses to see,” said Mr Kornhauser.
People with keratoconus are often misdiagnosed, misinformed, mistreated, and living with a belief that little or nothing can be done to restore their failing vision. Mr Kornhauser stressed that while most problems experienced by keratoconus patients can be solved, it is often incredibly difficult to locate the right specialist. Recognising that competent care can be life changing, Keratoconus Australia has compiled a list of keratoconus experts.
Corneal cross-linking has remained a focus area of Keratoconus Australia. The organisation has also supported the establishment of the Save Sight Keratoconus Registry, the world first registry to track outcomes of keratoconus treatments including cross-linking. The registry uses the Keratoconus Outcomes Research Questionnaire (KORQ), a quality-of-life survey designed specifically for patients with keratoconus. Early data contributed to having corneal crosslinking supported by Medicare in 2018, making this crucial and costly procedure affordable and accessible to thousands of Australians.
Patient Chloe Davies was invited to share her account of her lived experience with keratoconus and multiple procedures. She was diagnosed with keratoconus at age 10 and has had three corneal transplants and a corneal cross-linking. “I was 10 when I was diagnosed, but it could have been there beforehand. I could have had it from a really young age and not noticed a vision difference. If you grow up with that vision, you don’t know that something is quite wrong. It was at 10 that it progressed very rapidly” she said.
Ms Davies shared all the inconveniences and emotional impacts of living with keratoconus. She also shared her coping strategies, “I have learnt to live with my keratoconus. I understand what my vision is; I have changed my lifestyle to suit it. I have a large screen; I take a lot of breaks. I don’t drive at night because I don’t feel that safe, and I just don’t have vision good enough for it. I am able to manage it otherwise, quite well.”
Several participants said they could relate Ms Davies’ experience to their own. “It’s always good to hear the experience of others living with keratoconus. Over the years, I have had the same kind of struggles with contact lenses,” one patient participant said.
Ms Davies’ talk was followed by Dr Himal Kandel, who presented recent research out of the Save Sight Keratoconus Registry. Dr Kandel is the inaugural Kornhauser Research Associate at the Save Sight Institute, The University of Sydney. Four recent publications from the registry are briefly described below:
The Optometry Module
This paper6 was published in Clinical and Experimental Optometry, the official journal of Optometry Australia, the New Zealand Association of Optometrists, the Hong Kong Society of Professional Optometrists, and the Singapore Optometric Association.
The paper describes and highlights the advantages of the Save Sight Keratoconus Registry for optometrists, as a new tool to help manage patients with keratoconus. The optometry module in the Save Sight Keratoconus Registry is the only one in the world dedicated to optometrists.
Why is this important?
With optometrists playing a critical role in managing keratoconus, the Registry’s real-world data can help improve their clinical practice and patient care.
When is Keratoconus Likely to Progress?
This registry study7 discovered that steeper Kmax and young age were the most clinically useful baseline predictors of keratoconus progression. This paper was published in the prestigious British Journal of Ophthalmology, the British Medical Journal’s flagship ophthalmology journal.
Why is this important?
The findings of this study will assist clinicians in decision making regarding when to undertake corneal cross-linking. Closer follow-up and a lower threshold for cross-linking should be adopted in younger patients and those with steeper corneas.
Which Cross-linking Protocol is the Best?
This study8 compared the efficacy and safety of two common corneal cross-linking protocols, standard (long) and accelerated (short). They found that both procedures improved visual and corneal-shape outcomes. However, the findings suggested that the shorter method may be better for clinician and patient time, convenience, and comfort. To date, this study is the largest of its kind that has investigated this research question. The paper was published in the Cornea Journal, the official journal of the Cornea Society.
Why is this important?
The findings from this study have led clinicians to adopt short corneal crosslinking with higher confidence, and provided data on the risks vs. benefits for patient counselling.
What Can Go Wrong with Cross-linking?
Serious corneal infection is rare but possible after corneal cross-linking. This study9 looked at the largest cohort of patients with serious corneal infection after crosslinking. The common organisms isolated were presented.
Why is this important?
The paper informs clinicians on what is the most likely cause of infection after cross-linking and on how to prevent and manage serious infection after corneal cross-linking in keratoconus patients.
The talks were followed by a panel discussion on topics including recent advances in contact lenses and surgeries, adverse events, treatment decisions and research priorities. Recordings are available on the YouTube channel of the Save Sight Institute.
KeraClub 2021 received excellent feedback from participants who commented on aspects they found most useful and opportunities for future improvement, some of which follow:
“Absolutely everything was ever so useful. I really appreciate that it was free for anyone to attend. Having it available online for rural Australians was excellent.”
“Simple language, personal experiences and latest updates on treatment/care for keratoconus made the event so successful.”
“Information from different points of view (experts and patients) has made the KeraClub a unique event.”
“The webinar was excellent with a range of speakers. It was beyond brilliant – well-presented and conducted. Thank you so, so much.”
To remain informed about the research by Corneal Research Group at Save Sight Institute, and events including future KeraClubs, visit eepurl.com/dvSQtz.
Dr Himal Kandel is the inaugural Kornhauser Research Associate at the Save Sight Institute, The University of Sydney. Professor Stephanie Watson is an ophthalmologist and head of the Corneal Unit at the Sydney Eye Hospital; head of the Corneal Research Group at Save Sight Institute; and the Chair of Ophthalmic Research Institute of Australia.
- Anderson DE, Sivalingam V, Kang AEZ, et al. Povidone-iodine demonstrates rapid in vitro virucidal activity against SARS-CoV-2, the virus causing COVID-19 disease. Infectious diseases and therapy. 2020;9(3):669-675.
- Bal S, Chodosh J, Venkateswaran N. Impact of SARS-CoV-2 on Ocular Surface Pathology and Treatment Practices: a Review. Current Ophthalmology Reports. 2021;9(3):77-82.
- Romano V, Vinciguerra R, Arbabi EM, et al. Progression of keratoconus in patients while awaiting corneal cross-linking: a prospective clinical study. Journal of Refractive Surgery. 2018;34(3):177-180.
- Moshirfar M, West WB, Marx DP. Face mask-associated ocular irritation and dryness. Ophthalmology and therapy. 2020;9(3):397-400.
- Phylactou M, Li J-PO, Larkin DF. Characteristics of endothelial corneal transplant rejection following immunisation with SARS-CoV-2 messenger RNA vaccine. British Journal of Ophthalmology. 2021;105(7):893-896.
- Jhanji V, Sharma N, Vajpayee RB. Management of keratoconus: current scenario. British Journal of Ophthalmology. 2011;95(8):1044-1050.
- Kandel H, Downie LE, Watson SL. The Save Sight Keratoconus Registry–Optometry Module: an opportunity to use real-world data to advance eye care. Clinical and Experimental Optometry. 2021; doi.org/10.1080/08164622.2021.1924626
- Ferdi A, Nguyen V, Kandel H, et al. Predictors of progression in untreated keratoconus: a Save Sight Keratoconus Registry study. British Journal of Ophthalmology. 2021;doi: 10.1136/bjophthalmol-2020-317547.
- Kandel H, Nguyen V, Ferdi A, et al. Comparative efficacy and safety of standard versus accelerated corneal crosslinking for keratoconus: 1-year outcomes from the Save Sight Keratoconus Registry study. Cornea. 2021;40;12:1581-1589
- Khoo P, Cabrera-Aguas M, Watson SL. Microbial Keratitis After Corneal Collagen Cross-Linking for Corneal Ectasia. The Asia-Pacific Journal of Ophthalmology. 2021;10(4):355-359.