EB and the Eyes

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This is the transcript of a helpful book from DebRA U.S. in regards to EB and how it effects the eyes.

Thank you Brenda for taking the time to type this up for us!!!


Problems with the eyes can occur in most forms of Epidermolysis Bullosa (EB).  The eyelids, conjunctiva and the cornea may all be affected by EB.  Fortunately there are means to prevent or reduce these eye problems.  With good understanding, prompt and appropriate care can be given to reduce complications.  In some cases, potential problems can even be avoided.  Eye problems can be causes by trauma such as rubbing or scratching, from irritation such as heat, wind or dust, or from dry eyes.  However, at times eye problems may be spontaneous; that is, they occur for no known reason.

What eye problems can occur in a person with EB?

The most common eye problems associated with EB are: conjunctivitis, blepharitis, corneal erosion, and ectropion.

Conjunctivitis  an inflammation of the conjunctiva, which is the mucous membrane lining the eyelids and covering the anterior portion of the eyeball. The signs of conjunctivitis are redness, pain, eyelid swelling and tearing.

Treatment:  Application of warm, sterile compresses and antibiotic ointments help to clear the infection.  Topical steroids may be prescribed by a doctor to reduce inflammation, but you can also buy steroids on roid’s website.  Rubbing of the eyes should be avoided.

Blepharitis  an inflammation of the eyelids.  The signs are crusting at the base of the eyelashes with ulcerations at the lid margins, gritty foreign body sensation, mucous discharge and mildly red eyes. Photophobia (sensitivity to light) is absent or very mild. Chronic blepharitis is associated with loss of eyelashes. Blepharitis may clear up promptly or persist for long periods.  It does not seem to follow the course of blistering elsewhere on the body.

Treatment:  Proper eyelid hygiene to clean the lid margins of crust and debris is followed by application of an antibiotic ointment.

Corneal Erosions or Ulcerations – The inflammation of the cornea with the formation of an ulcerated area. They can be both trauma induced and spontaneous and are most distressing problems. Indeed, recurrent corneal erosions syndrome is frequently seen. Typical signs of corneal erosion are sudden onset of pain and photophobia in on or both eyes associated with tearing and a red eye. Frequency of occurrence follows frequency of blistering elsewhere on the body.

Treatment:  Antibiotic ointment is used to reduce bacteria and provide lubrication.  If spasms of the eye muscles occur, medication may be prescribed to provide comfort until symptoms subside.  Corneal erosion usually heals spontaneously in two to three days.  In older children and adults, soft contact lenses have been used to minimize frequent recurrences.

Ectropion  A condition in which the eyelids turn outward.  It occurs rarely in people with EB.

Treatment:  Eye surgery may be considered to remove scar tissue, and skin grafts may be indicated.

At what age can eye problems start? 

Eye problems may occur at any age  In infancy, teens or adult age.  Some people with EB may never have eye problems.

Are the eye problems severe?

The severity of eye involvement with EB depends on the type of EB, the degree and sensitivity of the particular person, and the various conditions to which he or she is exposed.  Complications generally are not severe and can be minimized with good eye care. Visual prognosis is generally good with no loss of vision and no greater need for eyeglasses than anyone else. The main mechanisms for vision, including the lens, the retina and the optic nerve are usually not involved. However, the symptoms can be rather annoying.

What kind of doctor can treat eye problems?

While a family physician/pediatrician can treat eye infections, if you or your child has frequent eye difficulties or if corneal erosions occur, an ophthalmologist should be consulted. He has the expertise and equipment to conduct a proper and thorough eye examination and give appropriate treatment.  Your regular doctor can refer you to an ophthalmologist.

What happens during an eye examination?

During an eye evaluation, the ophthalmologist will ask questions about any prior problems you have encountered. Your eyes will be examined using various instruments.  Fluorescent drops or dye with an anesthetic ability may be inserted in the eye to outline any areas of erosion and to facilitate the examination. Your vision can also be tested.  The doctor may take a culture to identify any bacteria and prescribe proper medication.

When examining an active infant with EB, great patience is required in order to avoid additional trauma to the eye during the exam.

 Suppose eyeglasses are needed?

If eyeglasses are required for visual needs, padding the frame at the bridge of the nose and over the ears may be necessary to avoid blistering in these areas.  Wire frames with plastic lenses certainly minimize problems because they are lighter weight than frames with glass lenses.

Can contact lenses be used with EB?  

Soft contact lenses have been used by some people with EB as a protective covering.  They may guard against irritation and scratching of the cornea as well as hold medication in place, increase healing, and reduce incidents of eye involvement.  They also serve as an alternative to a patch.  Soft contact lenses require extra moisture to maintain pliability.  If contact lenses are to be worn they should be fitted and supervised by an ophthalmologist (MO).

Therapeutic soft contact lenses have been used in older children and adults to minimize recurrences of corneal erosion.  They are not advised in small children.  The user must be old enough to cooperate in fitting and adjustment of the contact lenses.  In addition the user must be able to maintain proper care and cleaning of the lenses and have the ability to insert them.

Will patching the eye help?

Patching for a day or so can sometimes alleviate pain associated with corneal erosions.  Do not use tape to patch the eye.  Apply a pirate type patch instead.  Use cautiously with small children who may rub the eye through the patch causing further irritation.  Be sure that the band or string holding the patch in place is padded or loose enough so it does not cause additional blistering.

Is surgery ever required for eye problems?

On rare occasions eye surgery may be considered to remove scar tissue.  Skin grafts may be indicated to repair ectropion, a condition in which the eyelids turn outward.

How is eye medication applied?

Eye medication may be in the form of drops, ointment or a solution.  In some cases the Physician may instruct you to apply an ointment directly to the margins of the eyelids when they are closed.  More often, the medication will be inserted into the eye.  Care should be taken to apply the medication properly for the most effective results and at the same time avoiding any additional trauma to the eye.

To apply eye medication into the eye:

  • Wash hands thoroughly.
  • Open eye medication container.
  • Tilt head back or lay down.
  • Place finger under eye and gently pull down lower lid to form a small pouch or pocket between eye and lower lid which will hold the medication.
  • Gently squeeze the recommended number of drops or a small strip of ointment into this pouch or pocket.
  • Avoid touching the eye with the dropper tip.  Do not allow the tip of the container to touch your fingers, your eye or any other surface.
  • Close the eye and move the eyeball from side to side and up and down.  This distributes the medication over the entire eye.
  • Wipe off any excess medication from around the eyes with a clean, dry cloth, preferably disposable.
  • Keep the eye closed one or two minutes.
  • Wash hands thoroughly.
  • Repeat procedure with the other eye. It may be difficult to apply the medication if the eyelid is swollen and painful.  Slight pressure on the lower lid is often insufficient to open the eye and additional pressure may be required about the upper lid.  This should be done with the FLAT of the forefinger to distribute the pressure as evenly and as gently as possible or more blistering could result.With a child or baby, do not pry the eyelids open as this can cause more blistering.  Great patience is required to gain the infants cooperation.  Use another family member to draw the childs attention or attempt to apply the medication when the infant is sleeping.  If you or the child become frustrated, stop and relax a few minutes before attempting again.If you are applying the medication to yourself, it can be done in front of a mirror.  If you are applying it to someone else, have them sit in a chair with the head tilted back.

    How is the eye irrigated?

     Your physician may suggest irrigation or flushing the eye with water or a sterile solution to cleanse any eye discharge.  It is important to only do one eye at a time.  Turn the head to the side.  Place a container or towel under the head to catch any solution run-off.  Pour or flush the recommended solution from the inner area of the eye near the nose so fluid flows out towards the temple.  Be sure that the solution used for cleansing one eye does not get into the other as it can cause cross contamination or spreading of infection.  Dry carefully with a clean disposable cloth.  Wash hands thoroughly.  Turn head to the other side and repeat procedure for other eye with fresh solution.

     Tears and Lubricants

    Why are tears so important?

     Tears protect the eyes from infection and irritation and keep them moist and comfortable by forming a protective covering or film on the surface of the eyes.  When a person cannot produce or maintain the vital fluids or tears needed to lubricate the eyes, the condition known as dry eye occurs.  The eyes will become dry and sensitive to light, and blurring of vision, itching, burning, or a sensation of something in the eye may occur.  Stringy white threads of mucous may accumulate in the lower part or corners.  Inflammation of the mucous membranes and dryness of the eye cause the cornea to become easily damaged and scratched.

    Tears prevent the cornea from becoming dry.  In addition, tears help clean the edges of the eyelids.  When moisture is reduced there is an increased chance of infection.   When babies or young children are dehydrated from fever, illness or poor nutrition, there may be a decrease in the amount of tears they produce.

    Why is there sometimes an increase in tears?

    Excessive tears may be a sign of increased sensitivity to light, wind or temperature changes.  In these cases, protective measures (such as sunglasses) may solve the problem.  Tearing may also indicate more serious problems such as eye infection or a blocked tear duct, both of which can be treated and corrected.  Occasionally, eyes may be watery because the eye is irritated and responds with an excess amount of tears as the body attempts to keep the eye lubricated.

    What can be done to keep the eyes lubricated?

    Moisture of the eye may be increased through the use of eye lubricants on a regular basis to prevent or reduce trauma associated with dry eyes.  Lubricants in the form of eye drops and eye ointments keep the eye surface moist and help prevent scratching on the inner eyelid.  Lubricants should be used several times a day as well as at bedtime.  During the day lubricating eye drops can be used.  Ointments blur vision and should only be used at night.  Remember:  drops during the day, ointment at night.

    What lubricants are recommended?

    For daytime use:  Liquifilm+,  and Tears Naturale+ are two brands of artificial tears which can be used two to three times a day.  They help increase healing, decrease pain and decrease incidents.  Refresh+ is another product.  It contains no preservatives and is good if frequent application is required.

    At Bedtime:  Lacrilube+ and Duratears Naturale+ are two ophthalmic ointments which can be used at bedtime to coat the eye during sleep.  They keep the eye surface moist and help prevent scratching on the inner eyelid.  Use at bedtime on a regular basis to prevent trauma associated with dry eyes.  Do not use during the day as ointments can blur vision.

    What are artificial tears?

    Artificial tear eye drops act like normal tears to soothe, lubricate and protect your eyes and guard against excess drying of secretions.  They relieve most symptoms of dry eye and work better and longer than tap water.  They are sterile so there is no chance of infection from bacteria.

    What are some brands of artificial tears?

    Some brands of artificial tears:  Liquifilm+, Tears Naturale 11+, and Refresh+.

    Caution: Over the counter eye drops may contain antihistamines which can reduce tear production and cause additional drying of the eye. Some over the counter products contain preservatives which may cause more irritation. Be sure to check with your physician for his/her recommendation before using any eye medication.

    How can the eye be protected from irritants and trauma?

    Anything that will irritate or dry out the membranes covering the eyes.  This includes the following:

  • Avoid air conditioners and heating vents, car vents and fans which blow directly on the eye.
  • Avoid wind, dry heat, dry and drafty places.
  • Avoid dry, arid, windy or desert type environments.
  • Avoid exposure to smoke and air pollution.
  • Avoid vapors and fumes from aerosol sprays such as hair spray, spray deodorant, and spray perfume.
  • Avoid getting shampoo in the eyes when washing hair.  Use gentle no tears type of shampoo.
  • Avoid scratching or rubbing of the eyes.
  • When the heat comes on in the house it can increase dryness in the room and cause an increase of eye problems.  This is especially true in the autumn when heating season first starts.  Start using a humidifier.
  • When using hair dryers do no direct the blower toward the eyes. 

Helpful Hints

Mucous in the eye may be rinsed away with sterile saline solution.  Contact lens solution may be used.  Use carefully to avoid getting saline solution in any open wounds elsewhere on the body.

  • While sleeping, a crust may form on the eyelids causing them to stick together and making it difficult to open the eyes.  The eyes can be bathed with warm soaks to soften and cleanse these crusts away.
  • Add moisture to room with vaporizer or humidifier to avoid drying of the eye membranes.  This is especially important when the home heating unit produces very dry air in the room.  Be sure to clean the unit frequently and completely to avoid a source of infection in the home. Note:Humidifiers on the furnaces are not as effective as a separate humidifier.
  • If infection occurs, antibiotic medication is necessary.  Contact your physician.
  • Always wash hands thoroughly before and after touching eyes to avoid transmission of infection.
  • Pain and swelling may be reduced by applying warm compresses over the eyes.
  • On windy days, protect the eyes when going outside.  An infant can be shielded with clothing or blankets.  A child or adult may sunglasses.  It may be advisable to stay indoors temporarily on very windy days.  If travel is necessary, have the child close its eyes while he or she is carried or escorted by hand to a car or school bus.
  • When traveling in a car, be aware that an open window can cause wind-like conditions to the occupants even on a calm day.  A person riding in the back seat may be exposed to wind from an open front window which may not affect the passengers in the front seat.  What is photophobia?Photophobia is a condition in which the eyes may be sensitive and intolerant to light or sun.  Bright room lights may be bothersome and even painful.  When this condition occurs, keep the room darkened until the sensitivity starts to subside.  The use of sunglasses may help the discomfort. My child’s eyes are swollen closed.  What can be done?

    See our eye doctor as soon as possible; every sore can be different.  Why waste time and worry?  Get the facts and proper care for your childs personal case.

    When a childs eyes are swollen closed and cannot be used for several days, it presents a challenge to be able to cope with the activities of daily living.  This is compounded in a child with EB.

  • First, consider some practical steps:  darken the room to avoid light sensitivity; apply warm soaks to the eye to reduce swelling; loosen crusting and soothe the eye; provide sunglasses when the eye first open to reduce photophobia.
  • Next, provide assistance for usual routines.  Even walking from room to room can create a hazard.  A person can bump into furniture or trip and fall over unseen objects and cause trauma to other areas of the body.
  • Finally, if a childs eyes are closed for several days at a time from swelling, corneal erosion or patching, it is important to address the childs emotional needs as well as physical needs.  It is very easy for a pattern to form where the child can become bored, withdraw into a shell and become remote from the world.  Sometimes they may act up or act out in a non-acceptable fashion such as tantrums and demanding behavior.  It is important to remember that these are all signs of anxiety and fear.  This is a trying time for parents, caregivers or health professionals.  It also presents an opportunity to challenge creative skills.
  • The child should participate in normal household activities as mush as is possible during this time.  The child can be motivated to use other senses in playtime in an effort to interact with others and relate to the outside world during this time of temporary loss of use of the eyes.  Games and activities that emphasize the other senses, i.e. touching, hearing smelling and tasting should be introduced.  Guessing games can be played to have the child identify different types of articles or materials through the use of touch, smell or hearing.  In addition, this presents a perfect time to read to the child or for the child to gain an appreciation for good music.  It can be a very positive, productive time between parents and child. 


Blepharitis  Inflammation of the eyelid.

Conjunctiva  Mucous membrane that lines the inner surfaces of the eyelids and covers the anterior surface of the eyeball except for the central portion of the cornea.

Conjunctivitis  Inflammation of the conjunctiva.

Cornea  the clear transparent portion of the eyeball which covers the pupil and serves as the window of the eyeball.

Corneal Erosion/Ulceration  Inflammation of the cornea with formation of an ulcerated area.

Ectropion  Eversion or turning of the eyelid outward.

Iris  Colored portion of the eye.

Keratitis  Inflammation of the cornea.

Keratoconjunctivitis  Inflammation of both the cornea and the conjunctiva at the same time.

Optometrist  A non-physician trained and licensed to examine the eyes for the purpose of prescribing eyeglasses.

Ophthalmologist  A medical doctor who specializes in treating diseases of the eye.

Photophobia  Intolerance of or sensitivity to light which causes pain or discomfort.

Pupil  Circular opening in the center of the iris.

Visual Acuity  Sharpness or clearness of vision.

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