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Ethan KranigLittle American with EEC syndrome
Country:
 USA |
Content:
- A Young American With EEC Syndrome
- Diagnosis and Genetics
- Medical Challenges
- Surgeries and Treatments
- Development and Support
- Concerns and Hope
A Young American With EEC Syndrome
BirthEthan Cranig, a nine-year-old boy from Prescott, Wisconsin, was born with EEC syndrome, a rare disorder that affects one out of 10 million people. The condition causes clefts in various body parts, ectrodactyly of the limbs, and signs of ectodermal dysplasia.
Diagnosis and Genetics
EEC syndrome is diagnosed through genetic testing or a blood test. It is usually inherited from parents, but Ethan's case was different. Neither his father nor mother carried the mutated gene, indicating a spontaneous mutation. Ethan was born without teeth, tear ducts, and the ability to sweat.
Medical Challenges
Despite the serious medical challenges, EEC syndrome does not affect life expectancy or personality traits. Ethan's mother, Natalie, recalls, "When Ethan was born, he had a cleft lip and palate, clefts in his hands and feet, urological challenges, and gastrointestinal issues." Due to a cleft scrotum and GI complications, Ethan requires multiple catheter changes daily. "It's something I need to do every three to four hours during the day, but at night, he can sleep through the night," explains Natalie.
To address the blockage in his urinary system, Cranig underwent a colostomy procedure, which collected waste that would normally pass through the rectum and anus during toilet use. Ethan used the bag until he was old enough to undergo reconstructive surgery to resolve the blockage issues. "The bag is gone now," says Natalie, "but when he was a little baby, he would have been unable to function without it."
However, the complications with his elimination system may return. "The doctors aren't sure how long this will last or if it will come back," she adds.
Natalie emphasizes that Ethan's lack of tear ducts and sweat glands poses the greatest challenge, especially during summer. "I have to keep his eyes moist," she explains. "We apply lubricating eyedrops every hour. He can't overheat or he'll start seizing, so I have to keep his temperature down, which is really hard during the summer." Due to his missing teeth, Ethan must wear dentures daily. "How many nine-year-olds do you know who have dentures?" jokes his mother.
Surgeries and Treatments
Ethan has endured 29 different surgeries throughout his young life. One of his most recent surgeries took place on April 21, 2017, when he traveled to Johns Hopkins Hospital in Baltimore, Maryland, where pediatric surgeons performed a corneal procedure on his eyes.
Ethan required the procedure because his eyes were starting to fail as a result of his condition. Prior to the surgery, he was legally blind in his right eye and had 55% vision loss in his left eye. "He was getting all these new cells growing on his eyes because they can't keep themselves moist," explains Natalie. "When the cells grow over the pupil, that's when he starts losing his vision."
According to his mother, the corneal procedure involved cauterizing the blood vessels in Ethan's eyes to stop the extra cells from growing. The procedure has shown promising results in improving Ethan's vision.
However, there have been side effects. Ethan has developed ulcers on his cornea, which can be painful and, in turn, become blinding as they heal and scar. "As the ulcers heal up, the scarring can cause him to lose his vision," says Natalie. "Obviously, we want the ulcers to go away, so we're going back to Baltimore on August 16th for Ethan to have another treatment. He has been receiving Avastin, which slows down the growth of the cells on his eyes, but we had to hold off on that for 30 days because it can block the eyes from healing properly."
The numerous procedures have taken a financial toll on the Cranig family, but Natalie emphasizes their importance in helping Ethan live a fulfilling life. "It's expensive," she says, "but every procedure is necessary to help him function as normally as possible."
Development and Support
Despite being born with EEC syndrome, Ethan's demeanor mirrors that of any other child his age. Natalie credits her older son, Wyatt, with playing a significant role in helping his younger brother enjoy his childhood to the fullest. "Wyatt is 11, and Ethan mimics everything he does," she says. "Ethan has been able to accomplish every milestone, like walking and talking, because he's trying to keep up with his brother."
"Ethan goes to a regular classroom with a speech therapist, a physical therapist, and an occupational therapist who come throughout the day. He does everything that every other kid his age can do, he just does it differently."
Concerns and Hope
Today, Ethan's eyesight remains the primary concern for Natalie. "We have met other kids who have the same condition as my son, and some of them have lost their vision completely," she says. "I'm determined that's not going to happen to Ethan."
"It's a struggle. It can be very expensive, especially because it's hard to find doctors who are familiar with what Ethan has and will treat him with compassion."
To help raise money for Ethan's medical expenses and ongoing surgeries, the Cranig family has created a YouCaring page.
