“Ethan has overcome everything that has happened. He has remained positive. – Natalie Kranig

Ethan Kranig did not earn the nickname “Super E” for nothing. At nine years old, Ethan continues to prove time and again that he is a fighter in facing the many challenges of his ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome. Ethan’s positive attitude radiates to everyone around him, even those who barely know him.

Ethan’s symptoms of EEC include lack of sweat glands, ectrodactyly, cleft lip, little hair, vision difficulties, tooth and nail abnormalities, minute ear canals and a flaccid bladder.

He has undergone numerous surgeries. But his most recent one was particularly exciting for him and his family. This past April, Ethan received his first pair of rigid gas permeable scleral lenses know as PROSE (Prosthetic Replacement of the Occular Surface Ecosystem).

Ethan (bottom left) recently attended the NFED Family Conference with his mom, Natalie (top, center) and his family.

Poor Eyesight

For years, Ethan has had a high sensitivity to light. His mother, Natalie, did not realize how poor Ethan’s eyesight had become until she visited Dr. Akpek at the Wilmer Eye Institute of Johns Hopkins Medicine in Baltimore, Md. A pediatric ophthalmologist at the Mayo Clinic in Minnesota recommended Dr. Akpek to Natalie after telling her that Ethan was legally blind. He had no vision in his right eye and partial vision in his left eye.

Dr. Akpek concluded that the chronic dry eye caused by Ethan’s EEC had broken down the limbal stem cells in Ethan’s cornea. The sclera then compensated for the limbal-cell-breakdown by over-producing conjunctival cells and invasive corneal blood vessels (known as vascularization) to continue to protect the cornea of the eye. This over-production by the sclera causes cell build-up on the surface of the eye which can lead to full or partial blindness.

On April 21st, Ethan arrived at the Wilmer Eye Center for the first step in addressing his lack of eyesight. He went through a surgery to cauterize or burn the blood vessels of the sclera to stop and reverse the vascularization of his corneas. The surgery lasted only 30 minutes. Ethan rested his eyes the rest of the weekend and began preparing to receive his PROSE lenses.

PROSE Lenses

Ethan’s surgery with Dr. Akpek went well, but his work was not finished. Ethan was going to start using PROSE lenses to combat his chronic dry eye.

Marc Steingesser

Natalie first heard of PROSE lenses after National Foundation for Ectodermal Dysplasias (NFED) Family Liaison Marc Steingesser reached out to her on the NFED Facebook page. Five years earlier, Marc, who is also affected by EEC, had learned about PROSE lenses from Dr. Richard Lewis during an NFED Family Conference, and was fitted for PROSE lenses at the Wilmer Eye Institute by Dr. Hessen. He forwarded Dr. Hessen’s information to Natalie and assisted the family throughout the process. Marc had recorded his process of fitting and using his PROSE lenses. His video is available on the NFED website.

PROSE lenses are like contacts, but they are harder and cover more of the eye surface. They are custom made for each individual to accomplish a high quality fit, but the quality comes with a hefty price tag. The fittings and first pair of lenses cost approximately $15,000. A “Team Ethan” Facebook page and YouCaring fundraising page that the Kranigs set up have helped cover part of the cost of Ethan’s treatment.

To use the lenses, they are first filled with a sterile saline. The lenses do not actually touch the surface of the eye. The solution is placed in the lenses before putting them in, so the eye surface stays moisturized throughout the day. Ethan needed to practice many times before getting the hang of using the lenses. The lenses are much bigger than a conventional contact lens. He also had to be sure and keep most of the solution in the lens while he inserted them.  The first time he tried, it took two hours to correctly place the lenses in his eyes. After several days of training and effort, his persistence paid off. Now he can put in his PROSE lenses in less than a minute!

Natalie highly recommends PROSE lenses to anyone affected by EEC and chronic dry eye. She greatly appreciates the work of Dr. Akpek and Dr. Hessen.

Natalie extends her thanks to Marc for his support.

“He was amazing!” Natalie said. “He gave us major encouragement on a gloomy day.”

Without Marc’s help, Natalie would have never even known about the possibility of PROSE lenses.

In turn, Marc enjoyed getting to know the Kranig family. He was happy to use his experience with PROSE lenses as an opportunity to help others through the process. Marc admits that the process is not an easy one. He is truly proud of Ethan’s success with PROSE lenses. As long as the initial fitting process takes, the feeling of relief that comes from finally placing the lenses in both eyes correctly can move anyone to tears.

“(PROSE lenses) are remarkable,” said Marc. “They are life-changing.”

Marc praises the use of PROSE lenses for ectodermal dysplasia patients with dry eye. For him, it is worth the time to consider PROSE lenses.

“Imagine that, all your life, you’ve lived with something that is uncomfortable and painful and one day, that gets taken away,” Marc said.

The success of PROSE lenses is not limited by age. Both Ethan and Marc share in the benefits of PROSE lenses. Marc sees Ethan as a pioneer that will help future PROSE lens patients and research.

Ethan and Buster

Natalie continues to be amazed by the way Ethan has handled his EEC. Ethan has had support from many along the way, but his attention has been focused on one particular furry friend. Ethan’s dog, Buster, recently joined the family and has helped Ethan stay positive and active.

Natalie reminds all parents of children with ectodermal dysplasias, “Although, the road seems endless, it is worth it.” She has found that the children she knows who are affected by ectodermal dysplasia have “the most perfect souls.” They deserve credit for their bravery and amazing attitudes!

“Ethan’s happiness is my triumph as a mother. – Natalie

Natalie’s goals are to see Ethan thriving and succeeding. She is willing to do whatever it takes to keep him smiling!

 

(Ethan was recently featured in the news when the New Jersey police granted a wish for him. Watch his story.)

 

One comment on “PROSE Lenses Give Super E the Ability to See”

  1. 1
    Nancy Bundza on September 21, 2017

    What a inspiring story about a young boy with courage, dreams of being a police officer and love of life. Super E has jumped every hurdle in spite of all his obstacles and still has a smile on his face. Ethan has annually gone to the NFED conference to see all his friends , their family and learned something new every year. May God bless him always and continue to give him strength and courage. Along with the others that are affected.

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