MATTAWAN - After three earlier times to get her hair trimmed, a Mattawan youngster Monday afternoon re-ceived her first “big haircut,” this time donating 13 inches of her golden curls to “Locks of Love.”
Brielle Kennedy, who recently turned five, went to Renee’s Nails, 51797 CR 652, Mattawan, for that special cut. She made the trip with her mother, Sarah, and younger sister, Brooke, age three.
Under the skillful hands of stylist Jessica Jenkins, Ken-nedy sat up straight in the chair, without flinching, while Jenkins gathered up her locks and put them into two pony tails, prior to cutting them off.
“She always wanted to grow it out,” said Sarah.
When Brielle was 16-months old, Sarah and her husband, Eric, received devastating news that Brielle was diagnosed with Spinal Muscular Atrophy (SMA) Type II.
Doctors told the Kennedys there was no treatment or cure for the disease, but the Kennedys remain optimistic and are encouraged by research currently being done.
According to Sarah, Brielle was accepted as part of a compassionate study through the University of Utah SMA Research Center, aimed at arresting symptoms and assisting in rebuilding strength.
The study requires Brielle to take potent and highly toxic medications several times daily.
According to Sarah, while the risks of this treatment are great, Brielle has shown huge improvement in muscle strength and respiratory function since beginning the study in June of 2009.
Brielle also takes part in physical therapy that includes:
• Daily home exercises focusing on building and maintaining muscle strength.
• Hippotherapy at the Cheff Center, in Augusta, twice a week.
• Aquatic therapy twice a week at Agility Health.
• Early On at home therapy weekly.
• Occupational Therapy – Periodic check-in with Early On; focusing on fine motor strength and keeping an eye on her hand tremors.
Sarah said that blood is drawn from Brielle regularly to monitor the medication levels and organ function from the trial medications. They also travel to Children’s Hospital Boston SMA Clinic and The University of Utah for once-a-year evaluations.
Sarah said Brielle has already faced many challenges in her short life, but meets each new hurdle with her favorite saying, “I Can and I Will!”
The Kennedys have three friends who are facing childhood cancer. “It hits close to home,” said Sarah.
As the degenerative disease has taken away Brielle’s muscle strength, it didn’t take away her ability to grow her curly hair,” said Sarah. “She wanted to help other children who have been diagnosed with cancer.”
This fall, Brielle will begin kindergarten at Mattawan Early Elementary School.
With the help of State Senator Tonya Schuitmaker and State Representative Aric Nesbitt, Gov. Rick Snyder proclaimed August as “Spinal Muscular Atrophy Awareness Month.”
It has an extra special meaning for the Kennedys, whose youngest daughter, Brooke, was diagnosed with SMA one month after her birth.
Brooke was admitted into a clinical “single key-drug therapy” study being conducted by the University Of Utah School Of Medicine. The study is funded by FightSMA and led by Dr. Kathryn Swoboda. The study is called the “Prospective Phase I/II Study to Evaluate Effects of Sodium Phenylbutyrate in Pre-symptomatic Infants with Spinal Muscular Atrophy” or “STOP SMA.”
Sarah said that by beginning treatment pre-symptomatic, Dr. Swoboda is setting out to prove that the earlier treatment begins, the greater the potential benefit in delaying onset and ultimately lessening severity of SMA symptoms.
At age two, Brooke did not show the signs of deterioration her sister experienced at this same stage. Sarah said she was crawling extensively, pulling to stand and walking with a gait trainer.
Starting around 12 months of age, Sarah said Brooke’s greatest challenge was her inability to gain weight.
As is often the case with experimental treatments in small children and infants, it is believed her medication, so apparently successful at delaying onset of SMA symptoms, may indeed have been the culprit for these complications, according to Sarah.
At 15 months of age, she weighed only 15 pounds and her doctors determined it was critical to abandon her current study medication regimen and start her on the same path as her sister.
Brooke’s inability to gain weight resulted in emergency laparoscopic nissen fundoplication surgery and insertion of a gastrostomy tube (G-tube). They are both doing a lot of physical therapy. They are trying real hard to preserve the muscle they have now,” said Sarah.
The two girls travel twice a week to the Cheff Center, where they do stretching, reaching and balancing exercises on a horse while it is in motion.
Sarah said her daughters, despite their setbacks, keep an upbeat spirit as they continue their medical challenges.
Wednesday, September 12, 2012 at 11:35AM 
