“The biggest mistake I made is that I feel guilty because all I saw was her disability. I let it kind of take over. All I saw was what Ivy could not do, through (her twin sister) Isabel. You have to change the way you think. Once I changed that, once I realized she is in there, I knew she was alright. Your child is not their diagnosis. Your mentality, and how you take it, can make or break you.”
This is the mantra of Shelly Raley, 35.
She’s a mother of 3-year-old twin daughters, but there’s nothing identical about her girls.
Isabel walks, talks, laughs, explores and asks questions about the world as she watches it unfold.
Ivy, on the other hand, does none of this.
She cries with frustration, as she can’t express her emotions.
She wails in pain, trying to figure out why everything hurts so much.
She doesn’t walk.
She doesn’t talk.
And every day is a battle to simply keep this little girl alive.
Thousands of people around the world have been following “Mighty Ivy” on Facebook, as this Chatsworth, Georgia, family struggles to find normalcy after being dealt an extraordinarily challenging hand in life.
“She didn’t meet milestones,” Shelly says. “Sitting, rolling, crawling. None of that. We were in and out of doctors. Something wasn’t right. She could cry for eight hours straight, and nobody listened. I went through three pediatricians, and they all told me to stop comparing her to Isabel.”
Thankfully, Shelly found a godsend in October 2014.
“We went to Dr. Judy Gay, and right away, she knew something was wrong.”
Ivy was scheduled for a battery of metabolic and genetic tests, brain MRIs, blood panels and several appointments with specialists.
All tests came back normal.
Everything except the screening for Rett syndrome, a genetic mutation that currently has no cure. Infants seem healthy for the first six months of life, then rapidly lose coordination, speech and use of hands.
“The doctor noticed a key sign,” Shelly says. “Ivy was putting her hands in her mouth … she had stopped using them. What they were doing was verifying the diagnosis and I should have known. I think (they wanted to be absolutely sure before they told me) because the diagnosis is so catastrophic.”
On Jan. 9, 2015, it was confirmed that Ivy was deteriorating because of Rett syndrome.
It was a moment that Shelly will never forget.
“I’m sure every mother with a sick child remembers the day,” she says. “Ivy was really sick with the flu, I laid down with her and woke up to John laying at the bed crying. What’s odd is that I knew in my heart that it was exactly what she had. All the hope, though, that it may be something else was gone when the doctor said that. She said, ‘I don’t want to have to tell you this over the phone, but I know you are on pins and needles …’ ”
The doctor was extremely straight-forward and didn’t attempt to sugarcoat the situation’s grim reality.
“She told me that it’s only going to get worse,” Shelly recalls. “Ivy needed a permanent feeding tube, and basically, her overall prognosis was unknown because it all depended on the specific mutation.”
Over the next couple days, the family’s house was flooded with medical supplies, therapy machines and all the equipment that would now become part of their life.
For the next year, Ivy’s health rapidly declined. She never learned to crawl and was losing the skills she previously had acquired.
“Things happened that we didn’t even see happening,” Shelly says. “The skills she was losing, I didn’t even see happening until I went back. That’s the way Rett works. It’s like, when we went for her two-year birthday pictures, I realized she completely lost the skill of sitting alone.”
Shelly describes Rett syndrome as a combination of autism, cerebral palsy and Parkinson’s disease. Ivy also suffers from seizures, which were moderately under control until earlier this week, when she was hospitalized in the intensive care unit after a horrific episode.
Ivy is currently at home and stabilized, but her discomfort is continuous.
“The worst part is the pain and the crying,” Shelly says. “There is nothing I can do to alleviate it. It’s gotten better, but only because of very strong narcotics. What’s odd is things like ibuprofen help more than the narcotics, but she can’t take them because she gets a gastrointestinal bleed. They think it’s nerve pain, so it’s hard to treat and control.”
On average, both Ivy and Shelly sleep just three hours per night. Days are spent trying to keep the pain at bay while attempting to give Ivy some semblance of a normal routine.
“She can tap a button to play with toys, but that’s starting to get hard,” Shelly says. “She likes things that respond with music when she hits the button.”
As far as interacting with her sister, Ivy can’t communicate her needs – but their bond transcends the disability.
“Isabel knows Ivy is different, but it’s normal to her,” Shelly explains. “She doesn’t realize everyone doesn’t have an Ivy. Sometimes, she comes up to Ivy when she’s crying, and she stops. She responds well to her. Isabel knows that we have to help her sister, and she doesn’t get upset or frustrated.”
Thankfully, Shelly discovered the Tobii, an eye-tracking device that helps Ivy communicate. She describes it as a touch-screen computer with a retina reader.
“It makes you realize how smart she is,” Shelly says. “We found an ear infection because of the Tobii. And her stomach, which was twisted earlier this year, every day, Ivy would (say) ‘My stomach hurts … Will you help me?’ It’s hard to make people understand that, mentally, she is on par with Isabel, but she’s trapped inside her deteriorating body.”
Her Only Wish...
Eventually, Rett syndrome plateaus, something Shelly is eagerly awaiting. But Ivy’s gastrointestinal issues are so severe that further complications can arise.
“She has aspirated twice and has gastroparesis,” Shelly says. “She had complete intestinal failure, and the medication, Reglan, is like playing Russian roulette. It can onset tardive dyskinesia.”
For Shelly, the hardest part of watching her daughter suffer is the constant guessing game when it comes to Ivy’s needs.
“If she could just talk to me, I think things could be more manageable.”
Rett syndrome affects less than 1,000 children per year in the United States, so it tends to fly under the radar. Shelly hopes to raise awareness, eventually leading to a cure.
But in the meantime, she focuses her energy on being the best mother she can be to both her daughters.
Despite the horrific circumstances, Shelly reflects on what this journey has taught her about life.
“The biggest mistake I made is that I feel guilty because all I saw was her disability,” she says. “I let it kind of take over. All I saw was what Ivy could not do, through (her twin sister) Isabel. You have to change the way you think. Once I changed that, once I realized she is in there, I knew she was alright. Your child is not their diagnosis. Your mentality, and how you take it, can make or break you.”