Wilful blindness, a state we sometimes succumb to in matters of medical emergency. Sensing something was awry in his left eye, our Trust Chairman adopted a “blokeish” bravado and ignored the obvious while delaying the inevitable. They say procrastination is the thief of time but it can also steal your sight, as Dr Child warns.

By Dr Anne Child & Victoria Hilton

Q: I should have known my left eye was not right! My father had suffered with detached retinas in the mid 1960’s – “it’s the Marfan, stupid”, but did I go straight to an eye hospital? No, I went into typical bloke mode – that is, it will be fine and waited a bit. Eventually I need to visit an eye clinic on a Friday, receiving a diagnosis of Retinal Detachment and referral to the Vitreoretinal clinic at Moorfields the following day. Thus, very early on a Saturday last March, I am in the main entrance to Moorfields City Road. The seats soon fill up and then it’s join the steady stream to our clinic. I suspect for an eye hospital there is a daily occurrence of detached retinas that need immediate attention. A 61-year-old, I am on the list for surgery for that day and I am lucky there are no children or more serious injuries to go ahead of me. It’s a fascinating procedure completed on me without a full anaesthetic. I have an awareness of what is going on. Once complete, I recover with tea and a sandwich, head down on my lap and a taxi home that afternoon. Back in the day it would have been a week or more in Hospital.

Once again, a first-class service from the NHS my thanks to all. But I should have presented earlier if you have any problems you must not delay the extent of any sight loss is at stake.

At the Marfan conference last Saturday whilst enjoying a fascinating presentation by Mr Robert Henderson, some questions occurred to me for Doctor Child's Casebook:

Why are we with Marfan syndrome so vulnerable?

Apart from regular eye examinations with an optician is there anything else we can do to reduce the risk? 

Finally, any dietary advice other than a daily beer and scotch egg?

A: Procrastination is NEVER the stance to adopt when symptoms present! Every second is precious with the eye, as you well know! It is sometimes human instinct to filter into our consciousness what makes us feel good but resist what is damaging to our ego! Please abandon the default stance of blokeish bravado and seek help.

In Marfan syndrome the risk of retinal detachment is significantly increased. It occurs because the light sensitive tissue peels off from the back of the eye. The connective tissue is weakened in Marfan syndrome and the tissue has come away easily.

For our wider public, symptoms that may indicate a retinal detachment are:

  • Bright flashes of light, especially in peripheral vision
  • Translucent specks of various shapes (floaters) in the eye
  • Blurred vision
  • Shadow or blindness in a part of the visual field of one eye

To answer your second question, you must undergo comprehensive eye exams with regular ophthalmologic follow up, particularly paying attention to the status of the lens, refractive errors, and retinal health. You will have heard during Mr Henderson’s presentation that there is a risk of lens slippage in Marfan syndrome. Known formally as ectopia lentis, the crystalline lens slips out of place because the zonular fibres that hold it in situ are weak.  To prevent it happening again avoid sports such as squash involving possible direct blows to the eye.

In terms of your diet – an all-inclusive diet, plus a one a day multivitamin- multimineral tablet will make sure you have all you need to make fibrillin. Other than that, whatever food makes you happy!

Marfan Trust, a CIO registered as a charity in England in Wales with charity number 1198847 at: c/o 24 Oakfield Lane, Keston, Kent, BR2 6BY. Contact us at [email protected] or by phone on + 44 (0)333 011 5256
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