Living with familial exudative vitreoretinopathy (FEVR)

Post date: 
Monday, 19 February 2018
Photo of Urmilla looking out of a window at a view over London

There are over 200 recognised eye conditions. Some conditions are much rarer than others, many people have more than one condition, and for some, sight loss is only one aspect of another condition altogether. NB Online spoke with Urmila Sharma-Valand about familial exudative vitreoretinopathy (FEVR).

 
1. What are your eye condition, and how does it affect your sight? 
 
I have a rare genetic condition called FEVR which has caused progressive vision loss. It affects my retina, the light-sensitive tissue that lines the back of the eye. I also have chronic panuveitis.
 
I am registered blind. I have blurry peripheral vision in my right eye and can make out dark shapes and silhouettes, but have no vision in my left. I can read size 24-plus fonts on my iPad when it’s about five inches close to my face. I may see a car when it’s very close but won’t be able to see the colour, who is inside, or where the doors are. 
 
At night I can’t see anything because everything is black to me. I have similar problems when it is too bright, when after a little while my eyes start to hurt and I have excruciating pain in my head.
 
2. Are any of your conditions congenital, and are they related? 
 
FEVR is congenital. Uveitis is quite common after operations. I guess one problem leads to another if I may say…
 
3. How long did it take to get the diagnosis? 
 

It all started when I bumped my head so hard that I lost control of myself. I thought I was being silly so got up and carried on. A few weeks later I saw tadpole-like floaters as well as curtains on the side of my eye. I went to the emergency eye clinic and the consultant said I had a detached retina, which needed operating on immediately! 

My whole world fell apart because what followed after was internal inflammation, more surgeries and so on. During one visit to the hospital I was referred to the genetics department where they found out I had FEVR. 
 
4. Have you had any treatments for your conditions?
 
There is no cure for FEVR, however many medical professions are now aware of it. At present, I am going through a treatment for panuveitis which includes a daily dose of medication, three different types of eye drops as well as regular blood tests. 
 
5. Was it difficult to find out information about your conditions?  
 
There isn’t much information about FEVR, even my consultant had not heard of it before. My family helped me search and we eventually spotted an American website which is outdated but does have some very useful information.
 
6. How do your conditions affect you day-to-day?
 
Driving is out of question and the inflammation in my eyes has affected my ability to complete daily chores. However my rehab officer has helped me to adapt in my kitchen. I love cooking and I find it very therapeutic. I do get severe headaches on a regular basis and because of this I try to avoid big functions like weddings and concerts.
 

I try not to let it stop me. I’m currently a community research volunteer on the Leicester Ageing Together programme aimed at reducing social isolation and loneliness amongst older people in Leicester. I get to meet lovely people in the community who are so talented and skilled but lack confidence. It's like giving something back to the community and is very rewarding!

7. Do you know if the conditions affects different people in different ways? 
 
It can be hereditary and affects people in different ways; some people present no symptoms whatsoever their entire lives, whilst others can be affected from birth or progressively through their lives. 
 
8. What would you like eye health professionals to know about your conditions that may be helpful when treating other patients?
 

All professions are evolving and the eye health profession is not exceptions. They need to act in a "human" way because all visually impaired people go through emotional turmoil. The journey from sighted to blind is very difficult to accept and adjust to. The visually impaired should be included and not labelled. I don’t want to sit in a corner because people think I am unable to do things. 

9. Is there any technology that helps you day to day?
 
My iPad is my companion and is a window to the world. I use it for navigation, to read, and of course Siri is a useful tool. I also have an iMac with a very large screen. I use social media to connect with like-minded people and for support.
 
10. What's the strangest question you've been asked about your conditions?  
 
People in my community have always seen me as a sighted professional, so they find it difficult to accept my lifestyle now. They ask questions like, “How did you hurt your leg?” and, “Why have you got the walking stick?” 
 
In other words I’d like to say that we need to educate the community about sight loss. 
 
Tags NB Online