SOUTHWICK – When Kelly and Jason Clendenin found out they were pregnant with their daughter Violet in 2010, it was a welcome surprise.
The couple had a one-year-old daughter at the time and Jason’s daughter was 12. The new baby arrived Oct. 24, 2011, and was a healthy 8lbs, 9 oz, and 20 inches long and was the spitting image of big sister Ember.
When the Clendenin’s brought baby Violet home three days later, she began to cry.
“She cried all day and all night,” Kelly said.
Several trips to the pediatrician brought the same diagnosis: colic. Then, on Nov. 19, Violet’s doctor gave the same diagnosis at yet another visit, until the doctor noticed her arm was limp. An appointment was made for that same day with a neurologist and the Clendenin’s lives would soon change.
“When my husband took Violet to the neurologist appointment, he sent me a text message not even 15 minutes into the appointment telling me they were going to admit her to the hospital,” said Kelly. “At that very moment my heart sank and I knew – I just knew – it wasn’t good.”
Kelly’s instinct was right and before she could even think about the possibilities, they had their answer. Violet, not yet a month old, was diagnosed with Spinal Muscular Atrophy, Type 1. (SMA).
SMA is an incurable autosomal recessive disease caused by a genetic defect. Spinal muscular atrophy manifests in various degrees of severity which all have in common general muscle wasting and mobility impairment. Other body systems may be affected as well, particularly in early-onset forms. SMA is the most common genetic cause of infant death. Some children with SMA can live to age 10 and there have been some cases of people with very mild SMA that live into adulthood. However, most children with SMA die very young.
The Clendenins were given the worst prognosis they could imagine.
“Basically they told us she had six-12 months to live,” said Kelly. “When the doctor said she had SMA, we had no idea what that was. Baystate had seen maybe one or two cases.”
That night, in the hospital, Kelly began researching SMA online and learned all that she could. She and Jason opted for hospice care right away and through hospice, found the Families of SMA Massachusetts chapter. The connections made through that organization are what kept the family going.
Called a “rare” disease, Kelly said through the FSMA network she found many families who had gone through what she was going through and they offered a wealth of knowledge.
“It’s a horrible thing to go through, but I met the most amazing group of people,” she said.
What the Clendenins found out was that one in 40 people unknowingly carry the gene that causes SMA and that the child of two carriers has a one in four chance – or 25 percent – of developing SMA.
“And they’re now saying it’s more like one in 35 for caucasians,” Kelly added.
The gene that causes SMA was discovered just five years ago and Kelly said many cases of SIDS (Sudden Infant Death Syndrome) were likely due to SMA.
While the support of family, friends and the FSMA, the Clendenins enjoyed every moment they could with Violet, who began having to receive morphine almost daily to relieve her pain.
“Our mission was to keep her pain-free and comfortable,” said Kelly.
In addition to morphine, little Violet had a feeding tube and other medications to ease her pain. Her struggle ended fairly quickly and Violet passed away in Kelly’s arms on Jan. 24, 2011 at exactly three months old.
The Clendenins remain part of FSMA and are keeping Violet’s memory alive through fundraising and awareness efforts, including the Victory for Violet Dance Away SMA event set for this Saturday from 4-9 p.m. at The Cove on Point Grove Road, Southwick.
Tickets are $20 each and children under 10 are free.
The event includes a buffet dinner, DJ music, dancing, raffles and a cash bar.
“We have two Shriner’s clowns – Poppy and Caddy – doing face painting,” said Kelly. “It is a family event.”
Mama Cakes of Westfield will provide its own special Violet Cupcake. Kelly said the cupcakes can also be purchased at the bakery, located on Elm Street, and $1 of every Violet Cupcake sold goes to SMA research.
Kelly and Jason are happy to share their story so that people, especially those considering having children, are aware of SMA. There are no prenatal screenings for SMA yet, but Kelly said a simple cheek swab will determine if a person carries the gene.
The Clendenins have chosen not to have other children, but are in the process of adopting another child
“The risks are high,” said Kelly. “We have one healthy child and Jason’s daughter is healthy.”
Playing the odds now that they know they are both carriers is not a risk they’re willing to take after experiencing the heartbreak of Violet’s struggle.
Victory for Violet Dance SMA Away is open to the public. Donations can also be made through the website www.fsma.org.
In support of the Clendenin family and their quest to raise awareness, the Southwick Board of Selectmen declared August SMA Month and offerd the family a proclamation.
Selectmen Chairman Arthur Pinell said the proclamation was “the least we could do to help people in this situation.”
