Local SMA patients, parents determined to fight disease

Published 12:00 am Wednesday, October 10, 2001

In most ways, Miranda Cremeens and Lauren Ball are like your average girls.

Wednesday, October 10, 2001

In most ways, Miranda Cremeens and Lauren Ball are like your average girls.

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Cremeens, 11, enjoys going to the movies with her friends and shopping. The sixth-grader at Rock Hill middle school also likes sports. She passes her time dribbling a basketball, she’ll tell you with a shy grin on her face.

"She’s kind of bashful," her mother, Becky is quick to point out. "But she basically likes to do the same things every other kid her age likes to do."

Five-year-old Lauren Ball’s favorite thing to do is drawing. She also has fun playing with her friends who live in her neighborhood and her classmates at West Ironton Kindergarten and she loves playing with her Barbie dolls.

Really, the only difference between them and their peers is they are confined to wheelchairs. Miranda, the daughter of Rick and Becky Cremeens of Kitts Hill and Lauren, the daughter of Jeff and Beth Ball of Ironton, suffer from spinal muscular atrophy (SMA), a disease which affects the motor neurons of the spinal cord and brain stem.

As they motored around Spare Time Recreation Tuesday, it was obvious they have developed a strong friendship even though several years separate them. They shared laughs and took turns playing video and other games at the complex.

"Really, Lauren and Miranda are just like any other child," said Mrs. Ball. "They just get around differently."

SMA causes the muscle cells to become which, in turn, weakens the muscles of the limbs and, in some cases, the muscles used for swallowing and breathing. The disease is the top inherited killer of children under two.

Recent studies of the disease confirm that most children and adults with SMA have inherited this disorder by receiving one gene from both their mother and their father. Between one in 40 and one in 80 healthy men and women carry the gene for SMA. If both a man and woman carry the gene, the chances are 25 percent that their children will be born with SMA.

Intelligence is unaffected and studies have shown that physicians who work with children who have SMA are impressed that they tend to be alert, interactive and gifted.

"That’s very important to let people know," said Mrs. Cremeens. "Just because they are in wheelchairs doesn’t mean they are stupid or different. They are just ordinary kids."

Generally, there are four types of SMA: Type I, Type II, Type III and Type IV (Adult). Miranda is a Type III patient and Lauren a Type II.

Type I SMA is the most severe form of the disease. Virtually all children with Type I SMA show symptoms of weakness before they are eight months old. Children with Type I SMA are never able to sit without assistance.

The disease also affects the muscles used for chewing and swallowing and the muscles of the arms and legs. The weakness of the chest wall muscles makes it difficult for the child to breathe deeply or generate a strong cough. They also are at high risk of pneumonia.

In children with Type II SMA, the weaknesses are less severe. Symptoms are usually noticed between the ages of 6 and 18 months, the age Lauren was when she was diagnosed. The clinical features are similar to those found in children with Type I.

Children with Type II SMA usually have the ability to sit independently, but are very rarely able to walk or stand without help.

Children with Type III typically develop symptoms after 18 months of age. Miranda, for example, was not diagnosed until she was 2. These children achieve independent walking and may remain ambulatory for years after the onset of symptoms. Other patients develop slow progressing symptoms that usually result in a loss of independent walking.

Respiratory and swallowing complications are uncommon in Type III patients.

Type IV SMA (Adult) begins in adulthood with most patients noticing symptoms in their mid-30’s. Only a small number of patients eventually requiring wheelchair assistance.

Though there is not a cure or a real effective treatment for SMA, both Miranda and Lauren do receive physical therapy to keep their muscles from degenerating even more. Research of SMA, however, has brought forth some advanced care for the disease. It has also made it

easier to identify.

For example, in 1992 Miranda had to undergo a muscle biopsy for her diagnosis. Just five years later when Lauren was diagnosed, doctors were able to detect the disease through a DNA blood test.

With research coming this far in such a short period of time, though, both the Balls and the Cremeens are optimistic about what the future may hold for their children.

"I think as medical technology improves, things are going to get better," Mrs. Ball said. "In the last 10 years, there has been so much come out of the research. Hopefully, they’ll find a cure or at least treatment so Lauren and Miranda can have more endurance and strength."