Experiencing visual changes such as blurry or distorted vision can be an unsettling experience for anyone. While everyone processes health changes differently, patients with special needs, particularly Down syndrome, may have difficulty communicating with caregivers and could be less likely to report early changes in vision. For conditions like keratoconus, early detection allows patients the greatest number of treatment options, including the chance to halt the progression of the disease.

Some conditions have been associated with a higher prevalence of keratoconus. For example, 5-15% of people with Down syndrome also have keratoconus, an often-difficult combination that can impact an individual’s ability to function at their highest level. To halt the progression of the disease as soon as possible, it is important for caregivers of special needs children to schedule regular eye exams beginning in infancy and to screen for keratoconus during the teen and early adult years.

While keratoconus can be a challenging condition, it is manageable with the proper steps and preventative care. Now, a minimally invasive and FDA-approved treatment, called corneal cross-linking, provides patients with another option instead of relying solely on corrective lenses or receiving a more invasive corneal transplant.

We recently sat down with Dr. Erin Stahl, a Pediatric Ophthalmologist at Children’s Mercy Hospital Kansas City, to discuss the unique considerations for families with children who have special needs and keratoconus, specifically how to determine the best treatment options and recovery process.

How can I best prepare my family member with special needs to receive necessary eye tests to determine if they are living with keratoconus?

As a pediatric ophthalmologist, I am very comfortable working with pediatric patients of all abilities and levels of fear surrounding medical visits. There are a few necessary steps in achieving a good eye exam. The first is using one of many techniques to check vision. This ranges from following a lighted toy to checking vision on an eye chart. The next important step is to dilate the pupil with eye drops. After the pupils have been dilated (which takes about 30 minutes so bring something to entertain your loved one) we use special lights and the “red reflex” from the reflection on the back of the eye to determine glasses prescription, and to get a very good view of any corneal irregularities including early and late keratoconus.

Cedric, an individual with special needs from  Hoover, AL, is living with keratoconus. Read his KC Journey here. (Photo by Sydney Cromwell)

If your loved one has done well with these tests which are done with flashlights from about a foot away from the patient, we can move to more measurements. Using a microscope called a slit-lamp we can get a close-up view of the cornea and look for scarring and subtle corneal changes, as well as signs of allergy. If your loved one is comfortable with all of these tests, then we try to obtain measurements of the cornea including a topographic map (corneal topography) and corneal thickness measurements. These tests use machines that do not touch the patient and do not hurt.

In some cases, we get more information from each visit as the patient has less fear with subsequent exams.

You can best prepare your loved one for these tests by telling them that they will get eye drops which will make their vision blurry. You can tell them to expect a number of different flashlights but nothing painful. Many young patients worry that they will get shots or have painful tests at the doctor. As a pediatric ophthalmologist, I have many distraction toys – lighted toys, movies, moving animals and stickers. Many think a trip to the eye doctor is pretty fun!

My loved one has Down syndrome and keratoconus. What do we need to take into consideration when looking into treatment options?

Pediatric ophthalmologists are very experienced in working with all children and adolescents.  There are also adult eye doctors who work often with these age groups. Children and adolescents with special needs should be cared for by someone who is comfortable examining young patients and have the tools in their office to make the exam fun and easy.

One of the most limiting factors in choosing a treatment option is the availability for different levels of sedation during the treatment. Most patients with special needs will need sedation for corneal cross-linking treatment. The procedure takes about an hour, so sedation can be needed to ensure the patient is calm and has no pain or discomfort during the procedure. Anesthesia for the procedure can range from topical numbing drops to general anesthesia. Pediatric anesthesia is safest when done by pediatric anesthesiologists in a monitored setting. These decisions are made with your family depending on the needs of your loved one.

What is the best way to help my loved one with special needs prepare for and recover from the cross-linking procedure?  

Corneal cross-linking, approved for patients 14-65 years of age, can cause post-operative light sensitivity and discomfort. There are many steps we can take to reduce these symptoms. In more cooperative patients, a contact lens will be placed and removed in clinic 3 days after surgery. This lens acts like a bandage and reduces pain. In more fearful patients, a dissolvable contact lens can be placed on the eye and does not need to be removed after surgery.

Gel ice packs can be applied to the eyelid after the procedure to reduce pain and can be very effective in reducing pain if your loved one will allow placement. Tylenol and ibuprofen can also be helpful to reduce discomfort.

As the eye heals after the procedure, it is important that your loved one does not rub their eye.  We tape a clear shield over the eye and most patients leave it in place. Glasses and sunglasses can take the place of the shield if your loved one prefers. We ask that the shield stay on for 3 days after surgery. It is not typical for a patient to rub the eye after surgery because it hurts more when touched.

Prepare a semi-dark room for your loved one to rest for 2-3 days after surgery. TVs and iPads may be too bright to tolerate during healing so have music, read-aloud stories, or other activities available that do not require bright lights. Plan for your loved one to stay indoors for the first few days to avoid direct sunlight. After this healing period your loved one should not have persistent eye pain and typically go back to their regular activities within 1 week.

Follow #KCExperts on our social channels, Facebook, Twitter and Instagram for weekly updates.*Dr. Stahl is a paid consultant of Avedro, Inc.