by Richard Meade
Spearman School Area, January 15, 2015 – Thursday was cold and overcast with temperatures in the low 40’s and a slight breeze, not a typical day for any child to go out and play. But Ariel, age 3 and not a typical child, was excited and ready to go outside to see her new pirate ship playhouse which was built specially for her by the Make-A-Wish® Foundation.
You could see the excitement in Ariel’s eyes and hear her small voice expressing eagerness to go outside. Excitement also spread to a crowd which had assembled inside the house and out in the back yard waiting to see Ariel’s reaction. (The crowd included friends, family, Make-A-Wish staff, a nurse, church friends, an early interventionist and others.)
There were pirate hats, masks, balloons and a pirate cake because “Ariel loves pirates” her Grandmother Kathy Barker said.
As Ariel was rolled down the long ramp toward the big pirate ship with a black pirate flag flying over the bow, it was obvious that the big playhouse met her approval. The playhouse includes a ramp so she can be rolled up to the main deck and access the helm, a slide, three swings with one designed for Ariel’s frail frame.
With a little help, she tried them all. When asked she said her favorite was the swing.
Why all the fuss? Ariel suffers from Spinal Muscular Atrophy (SMA), a genetic disease affecting the part of the nervous system that controls voluntary muscle movement.
SMA is characterized by the loss of nerve cells in the spinal cord called motor neurons, muscle controlling nerve cells. Long wire-like projections connect the motor neuron to the muscle in the limbs and trunk. Normally signals from the nerves to the muscles cause the muscles to contract. In SMA, motor neurons are lost and muscles can’t function.
The muscles most affected are those closest to the center of the body, such as those of the shoulders, hips, thighs and upper back. Special complications occur if the muscles used for breathing and swallowing are affected.
Sensory, mental and emotional functioning are entirely normal in chromosome-5 SMA.
This is evident in Ariel’s case as several people who work with her say she is very smart and wants to be independent. Although weak, Ariel at one time could sit, grasp objects, breathe and swallow. The disease has taken all these functions away to the point Ariel now has a breathing device that does her breathing for her and has to be constantly suctioned because she can’t swallow which has also forced her to have a feeding tube.
So why the playhouse? The Make-A-Wish® Foundation grants wishes to children with life-threatening medical conditions to enrich the human experience with hope, strength, and joy. Ariel’s wish was to be outdoors more because she LOVES to be outdoors with her family and friends. (see related story furnished by Katlyn Gould, Wish Manager for Make-A-Wish® SC.)
Ariel’s Story: Ariel is being cared for by her grandparents Kathy and Steve Barker who have joint custody with her dad, Joshua Barker. Joshua is in the Army and stationed in Savannah, Ga. He comes home once a month. Ariel’s mom Nicky, lives in Texas and checks on her regularly.
“We first noticed something at age six months when she wasn’t rolling over and her hips seemed weak. We got the diagnosis of SMA two months later,” said Kathy.
Ariel was able to sit with assistance, hold her head up, and move her arms. Then they noticed she was losing her ability to swallow.
“November 27, 2012 was the most horrifying day of my life Kathy said. Ariel stopped breathing because she couldn’t swallow and the mucus blocked her airway.” We spent 16 days in the hospital and came home with tons of equipment, heart monitor, feeding tube, breathing machine. That’s when it hit us just how sick she was. She was 16 months old.”
Since then she gradually lost muscle tone and suffered several fractures because her bones were so fragile. Once just from putting her into the stroller. She has been getting an infusion with a medicine that older people take to help strengthen bones. She has had no fractures for the last 8 months, Kathy said.
Taking care of Ariel is a full time job. She has a home care nurse come in 12 hours each day, occupational therapy two times each week, physical therapy once a week, speech therapy once a week, and a teacher four times a week.
Ariel has to be suctioned to clear the mucus 5 to 10 times an hour, care for the feeding tube, constant movement and massages for her arms and legs, move her head from side to side and breathing treatments.
“I couldn’t imagine doing anything else. That’s what family is for” said Kathy.
Ariel will be four in July. Kathy says she is very smart and her vocabulary is above most 3 year olds at this point. She is socially brilliant and will carry on a conversation with someone when they walk into the room. Her brain power that would be used to move her arms and legs has been channeled to help her advance in this area Kathy said.
“We do everything we can to make sure she is safe, but we do everything we can to help her be as normal as possible. We took her sledding when it snowed, we have taken her roller skating (pushing her stroller in the rink with other children).
“We would rather see her have a fuller life than a longer life,” Kathy said.
When asked about Ariel’s love for pirates, Kathy said her dad has always been a pirate fan, so she took after him. Ariel loves to be the captain, barking out orders to Grandfather Steve who wears a pirate hat and uses a pirate voice, and to others getting them to swab the deck and other commands. When we took her to the skating rink, we used cardboard to transform her stroller into a pirate ship. The flag we used is now hanging over the bed in her room.
When asked about Ariels’s prognosis, Kathy teared up a bit and said the biggest problem is she can’t swallow and doesn’t have the muscles to cough, so her airway gets blocked.
You’ve got to stay on top of it she said. When she gets blocked this is the most horrifying. You have to suction it out sometimes with a tube that goes up the nose and down the throat.
Once you get her clear, 5 minutes later she is fine and ready to play. It’s horrifying, but you move on.
“One thing this journey has taught us is to not take anything for granted. We are not promised tomorrow. We have to be in the right place at the right time to bring her back or a single moment could take her away,” Kathy said.
“As long as she is with us, we are going to live life to the fullest. We have been blessed to have her,” she said.