VKC: Relief and Hope

Most patients with VKC have mild symptoms such as red eye and itchiness; however, we see patients who have not responded to simple treatments by their primary care practioners, or general ophthalmologists. We often find patients presenting to the eye clinic with disabling symptoms and potentially sight-threatening sequelae.Distressingly, most VKC patients are young children or adolescents; the impact of this orphan disease therefore extends into educational and developmental issues. This situation is exacerbated by the diagnostic challenges associated with VKC. Firstly, it’s relatively uncommon, so GPs and pediatricians don’t always think of it when they see a patient. Secondly, its symptoms – red, irritated eyes and a watery discharge – can be confused with blepharoconjunctivitis or even infective conjunctivitis. Indeed, the more subtle features of VKC, such as a papillary rather than a follicular presentation, or itchiness as opposed to foreign body sensation, are easy to miss – particularly given that VKC patients often have a generalized atopic background, including hay fever and asthma. Consequently, many VKC patients end up misdiagnosed with blepharitis.

Orphan diseases get mistreated

Unfortunately, a correct diagnosis of VKC does not mean that the patient’s troubles are over. For example, many clinicians respond to corneal staining with a knee-jerk prescription of topical lubricants – and that’s misguided, because it doesn’t treat the underlying condition. But even if clinicians wish to address the cause of the symptoms, the options are limited: until recently, there have been no approved drugs for long-term management of this chronic allergic condition. That constraint has often forced clinicians to opt for topical steroids – with their long-term side-effects – or off-license formulations of topical calcineurin inhibitors (ciclosporin and tacrolimus). We even had doctors prescribing formulations indicated for veterinary use – which underlined both the poverty of alternatives for VKC management and the pressing need for better therapies. That said, I’m happy to say that the situation is improving – the approval of Verkazia (see Box) for long-term use in severe VKC is most welcome.

Space for more

Verkazia is great news for VKC patients and their clinicians. Even so, it’s just one drug – we need more options for VKC treatment. And this means more companies investing in therapies for children with severe external eye disease. For example, patients with the most severe forms of VKC may need surgery or systemic immunosuppression – topical treatment just won’t work. Other approaches for external eye disease could include immune desensitization. So, when it comes to treating VKC, there are still many gaps in the market! But I’m hopeful for the future; several groups are working on VKC, and it’s particularly encouraging that there is commercial interest in this field.

I’d like to make a couple of points for those who may be reluctant to prescribe a ciclosporin-based drug like Verkazia. It’s true that ciclosporin is a potent anti-inflammatory agent, and I can understand people being wary of applying it in the eye. But the fact is that topical administration of ciclosporin results in negligible systemic absorption – the risk of complications is minimal. Also, remember that topical ciclosporin preparations have been used in the eye for over 30 years now, and have proved to be efficacious, well-tolerated and very safe in this context. So, although Verkazia has only recently come onto the market, it is supported by a significant history of topical ciclosporin use in ocular conditions. Hopefully, clinicians will find that reassuring. And, after all, Verkazia has been through the regulatory process – if that doesn’t give clinicians the confidence to prescribe it, what will?

The holistic approach

Not all VKC patients are the same. A combination of a twice topical mast cell stabilers and oral antihistamines can be used for patients with mild VKC either during the allergy season, or throughout the year.  Nevertheless, where patients have sight-threatening features from the outset, or more than two flare-ups in a year, I’d always prescribe a topical calcineurin inhibitor as a steroid sparing agent. But whatever therapeutic approach we choose, we must remember the broader needs of the patient. Most VKC patients are children – the administration regime of VKC drugs therefore must accommodate the specific needs of this population. In particular, we should think carefully about the drop regime; my approach is to minimize the number of agents and administration frequency. Also, I may start the children on strong topical steroids – especially where they present with a severe flare-up – just to show them that, yes, their condition can be managed with treatment. Once VKC is under control, it can be maintained thus with a drop regime – ideally no more than three times per day, maybe four times daily on the weekend. We have to ensure the patient’s family knows what the treatment requires, and make sure that the treatment schedule can fit around family life.

And that raises a very important point: patient education. Most obviously, given that VKC is a chronic problem, it is critical that patients adhere to the drug regime. That can be difficult when parents are working, children are at school, and so on. It’s vital that clinicians have that discussion with patients and families, because they don’t always appreciate that VKC treatment must continue for years. And there are other, more fundamental, educational needs: patients’ families often think VKC is just ‘itchy red eyes’, and don’t understand the importance of actively managing it. That can turn into a real issue if they have lost faith in the medical system – for example, if they have been prescribed lubricants for months without seeing any improvement.

Escape tunnel

Finally, children and their families need counseling, education and above all support to enable them to fully participate in – and enjoy – normal life. A very important part of this is to persuade them that there is light at the end of the tunnel. Most children grow out of the condition, and if they stay on the treatment, they should escape the VKC years without too much trouble. So we must encourage them to trust the clinician on this treatment journey. We must ensure they understand that it will last several years; above all, we must give them hope! And the clinician’s hope, of course, is that newer and more effective drugs will help our VKC patients escape the worst aspects of their condition until it finally resolves.