SJS TIPS:

 

DEALING WITH PHOTOPHOBIA

Bud Telchin

 

Nearly everyone who gets SJS/TENS has moderate to serious problems with their eyes, and something that occurs right away is photophobia, or sensitivity to light.

 

When I had SJS at age 11, I was so light sensitive, especially in the morning, that I couldn’t tolerate the light in the bathroom or in the kitchen. Sunglasses provided little benefit, and going outdoors was something to fear and avoid. It took years for me to cope better, and I want to pass on to you the benefit of my learning experiences.

 

For some people the pain is so bad that they become effectively crippled.

 

Why is this so?

 

My theory is that the nerves from the eye to the brain can handle just so much information. For normal people, bright light is translated into information to the brain, and is handled with no problem.

 

But for us with SJS, the constant irritation of the corneas (caused by rough lining inside the eye lids and inward-pointing eyelashes), pretty much fills up the circuits to the brain. When we add bright light, the circuits are overloaded, and pain, often terrible pain, results.  This is made worse if there’s wind, that dries what little tear film we have, making the corneas even more irritated.

 

So how to deal with the pain from bright light?

 

You have to do two things:

A. Short term, reduce the light reaching your eyes.

B. Longer term, reduce the irritation of the eyes.    

 

Let’s look at these.

A.      To reduce the light reaching your eyes, you can do several things:

1.      Inside the house, install dimmers on lamps in the bedroom, kitchen, bathroom. (This can be tricky, especially in the bathroom, if you have flourescent lamps. You have to replace them with incandescent.)  There are little dimmer switches that are easy to install for table lamps, but for ceiling or bathroom fixtures, better to call in an electrician.

2.      Outside the house, always wear a hat with a brim (like a baseball cap). This creates a shadow around your eyes, keeping the direct sun out. It also reduces the wind reaching your eyes, which, as we said, is often very irritating.

Next, Sunglasses: there’s good news here, with the close fitting wrap-around glasses available today.  These come in models that have foam around the inside of the frame that fits snug to the face. This blocks wind, which can be very irritating, and blocks light coming in around the sides. From my experience, blocking this light from the sides, top and bottom is far more important that how dark the lenses are. In fact, I’m quite light sensitive, but walk around with glasses that are not very dark at all, but have excellent wrap-around protection.
Two brands are:

a.       PanOptx  (http://www.panoptx.com), which offers good looking wraparound goggles with excellent side protection (for about $120) and can be made with prescription lenses.  The foam is somewhat fragile, and may have to be replaced after about 6 months. The company will replace the foam and make other repairs under warranty, and for a fee after.

b.      UV3, from The IZone company (http://www.izonestore.com/).  These are very similar to the PanOptx, with excellent side protection. They use a foam that is much more rugged than the PanOptx, and are much less expensive (at about $30). These are sold at many of the turnpike rest areas. Ask for the special models with the foam protection.

Both will provide excellent protection from sun and wind and are great looking too!. In fact, the UV3 fits so tight that I get fogging, and have to pull them down my nose just a bit. Also, be aware that in warm to hot weather, the foam can make the skin beneath sweaty, but that’s a small price to pay for the benefits of these glasses.

I’m not sure if they’re available in children’s sizes, but they fit so well, that they just might work well for children as well.

c.      For extreme situations, you can actually wear swim goggles, such as the Speedo or Aqua Sphere line (http://www.aquasphereswim.com/products/seal_tinted.html).  Drill some holes in the sides for ventilation, to avoid fogging (or ask your shoe-maker to punch some holes).

Note: it might be tempting to wear super-dark goggles, such as sun-tan-lamp goggles, but be very careful and drill ventilation holes, or the fogging can get you into real trouble. And don’t even think of driving your car with goggles that fog up.
I did this, and its really dangerous to others and stupid!

B.     To reduce the irritation of your eyes:
There are two approaches:
- protect the corneas with ‘contact’ lenses, or
- eliminate the sources of irritation with surgery.

1.      Contact Lenses: 

a.      Soft lenses provide great comfort and protection to SJS members, by covering the cornea and keeping the rough eyelids away. For this reason, such lenses are called ‘bandage’ lenses. There are a wide variety of soft lenses today, from disposable lenses you throw out each day, to lenses you can wear 24 hours a day for a week or more. The silicone hydrogel type are probably the best, since they have a high moisture content, such as the Ciba Night & Day lenses, and the Bausch & Lomb Purevision lenses.

Some eye doctors discourage use of soft lenses because of concern about infection. If you get trained properly in handling and cleaning the lenses, the risk of infection is very small, and the benefit of protecting the corneas far exceeds this risk.

b.      The second type of ‘bandage’ lens is the scleral lens. This is a larger lens that covers not only the cornea, but rests on the white of the eye itself (called the sclera). This provides great relief to many SJS members who had lived in pain for years.  The lens is made of semi-rigid plastic and is quite a bit harder to fit, but the results can be wonderful. Start with the Boston Foundation for Sight at http://www.bostonsight.org/lens2.htm   
It might take a week or more to get a good fit, and they can be expensive, but we’re playing for keeps here, so keep expense in perspective.

2.      Eye surgery to reduce irritation of the cornea:

SJS ravages all the skin of the body, causing tremendous blistering and ‘burning’ of the skin. It also attacks all the mucous membranes inside the body, including lungs, mouth, genitalia, and definitely the eye lids and surface of the eye.

Except for the eyes, and in some cases the lungs, most of this damage heals over time. For the eyes, the damage gets progressively worse, leading to photophobia, debilitating pain, and in too many cases, ulceration and eventually blindness.
 
Within weeks of the attack, the normally soft, moist lining of the eyelids begins to toughen, shrink and turn into a very rough, abrasive form of tissue, called ‘keratin’. This rough, ‘keratinized’  tissue is what irritates the cornea as well as the white of the eye.

Surgeons have developed several procedures to reconstruct the eye lids, with the goal of making them close to the way they were before the SJS attack:
- Mucous membrane Grafts
- Turning out of the lids
- Permanent removal of eye-lashes
 

a.      Mucous Membrane Grafts:
  Eye surgeons developed the reconstructive procedure called ‘mucous membrane grafts’ to take soft mucous membrane tissue from the inside of the mouth or elsewhere and graft it into the lower and/or upper lids. This requires several days in the hospital, and one to several weeks wearing a bandage. There is considerable trauma (black and blue, swelling etc.) following the surgery, as with a ‘nose job’, but the results are often tremendously worthwhile.

Many patients achieve great comfort immediately, or following a second or third procedure. Its not perfect, however, as some of the grafted tissue shrinks, or doesn’t take long term. You probably want to do only one eye at a time.

b.      Turning Out of the Lids:
After the SJS attack, the lining of the lids shrinks as it becomes harder. This shrinkage pulls the lid margin ‘inward’, and suddenly, beautiful eye lashes that had been pointing where eyelashes normally point, are now pointing inward, at the cornea! 

This condition is called entropion.

Short term, the lashes can be plucked out by the eye doctor. But if left untreated, the lashes can cause serious irritation and damage to the corneas.

Fortunately, reconstructive surgery of the eyelid can be performed to reverse the damage of SJS, and turn the lids back outward. This is done by making hidden incisions along the lid margins, either inside the lid, or outside, and reinforcing or manipulating the many muscles there.  The skilled surgeon has many choices of how to do this, so ask what would be done for you, and what the likely outcomes are!

There can be trauma following this surgery too, and it is often done at the same time as a mucous membrane graft. 

c.      Permanent Removal of the Eye Lashes:

If you don’t opt for surgery, or even after surgery, there seem to be millions of eye lashes, any one of which can be irritating. There are several procedures for permanently removing them:

1)       Electrolysis: inserting a fine needle into the lash’s follicle and burning it out. This is done under a local anesthetic.
Pro’s: gets rid of the ‘bad guys’.
Con’s: not easy for the doctor to find the follicle; if not a direct hit, the eyelash can grow back; the local anesthetic can be very painful; many visits may be necessary; the burning leaves some scar tissue, which can be irritating in itself.

2)      Laser Treatment:
Relatively new approach; burns out large number of follicles in one session.
Cons: may leave large areas of scar tissue.
Ask your doctor about outcomes.

3)      Freezing:
This procedure covers the entire lid with liquid nitrogen, freezing and killing everything in sight, so to speak. This effectively gets rid of lashes, but destroys much of what good mucous membrane tissue you had left.  Ask your doctor if this is still a viable choice.

3.      So What to Do?
Try the soft bandage lens as soon after the SJS attack as you can. Don’t wait for your doctor to ‘see how things develop’. If you’re comfortable with the soft lenses, and there are no complications, you may have solved your photophobia problem.
If the soft lens doesn’t work out, the next least-traumatic approach is to try the scleral lenses.