Janice Crawford and Summer Smith

SANDPOINT - Summer Smith resembles a young Jacqueline Onassis - classic features, creamy skin, dark eyes and dark hair.

The 8-year-old is the picture of a normal little girl, said her grandmother, Janice Crawford.

Like many American children, she is overweight. But unlike most, Summer has a disorder than cannot be seen and the steroids initially used to treat it that contribute to her weight.

Crawford pulls the collar of her granddaughter's T-shirt down to reveal a pink scar hiding a port beneath her skin that every month at $20,000 transports medication to boost her immune system.

Summer snuggles trustingly against the woman she calls “mom” - the woman who has raised her since she was less than a day old.

Smith is one of 360 Americans diagnosed with a rare disorder - opsoclonus myoclonus syndrome (OMS), an autoimmune disorder that attacks the brain and can cause memory loss, learning difficulties and other physical problems.

“I would have just as soon as have had her have cancer as of had this disease,” Crawford said.

With cancer, there is the possibility of a cure. That is not the case with OMS.

Summer was a bright baby who crawled at 4 and a half months, walked at 10 months and had a vocabulary at 18 months that was far ahead of most 3 and 4 year olds, Crawford said.

Now she may forget something that she knew the day before - like the location of her crayons in a stacked bin.

Crawford compares her granddaughter's memory to Swiss cheese.

Summer, who will enter the third grade this fall at Farmin-Stidwell, has an IEP for children with learning difficulties and cannot remember all the letters of the alphabet.

She learned how to ride a bicycle last summer - she has difficulties with balance - and would benefit from sports like gymnastics and swimming, but her grandmother's $850-a-month income does not stretch far.

Summer's first symptoms began when she was 20 months old and would not go to sleep. Her grandmother found her repeatedly bouncing up and down on the couch in the early house of the morning.

Her physician, Dr. George DeLand, could not figure out what was causing the problem, but he agreed with Crawford that it was something serious.

Crawford spent 40 days with her granddaughter at Sacred Heart Hospital in Spokane, then 52 days at Seattle Children's Hospital trying to discover a diagnosis

By then Summer could no longer walk, sit up or feed herself.

Crawford videotaped her granddaughter at Seattle Children's and gave a copy to Dr. DeLand. It would lead to the breakthrough her granddaughter needed.

By that time, Summer was on steroids and antibiotics. She could not stand to be restrained in a car seat because the syndrome causes extreme sensitivity and physical pain. The steroids made her behavior difficult and physically out of control.

Fortunately, Dr. DeLand passed the videotape of Summer along to a doctor at Barrow Neurological Institute in Arizona. The doctor called Crawford and referred her to a Web site: omsusa.org, created by the National Pediatric Myoclonus Center.

“I was 15 hours reading on that Web site,” Crawford said.

The disorder is caused by viral infections like Epstein-Barr, Coxsackie B, St. Louis encephalitis and other viruses or in half of the cases, like Summer's, it can be caused by neroblastoma or gandlioneroblastoma tumors, according to the center.

The result is a battle between the immune system and the virus or tumor, with the brain being caught in the middle. That battle can be especially difficult on the cerebellum, brainstem and limbic system.

Crawford then took Summer to a California hospital for evaluation. However, she became concerned that she was not getting enough answers and contacted the National Pediatric Myoclonus Center.

“I'm kind of forceful when I'm taking care of things, especially when it comes to kids,” she said.

By that time, Summer was taking a high dose steroid that caused extreme weight gain. When she was three, she weighed 75 pounds. Doctors at Shriners Hospital performed surgery on Summer, placing staples in the growth plates on outside of her legs because the weight was making them buckle sideways from the knees. The staples are designed to put her legs back in place as she grows.

When Summer first began having problems, Crawford also had broken her neck and wore a device to keep it immobilized. She also was raising Summer's younger sister, Janey, now 6.

As she searched for treatment options for Summer, Crawford also maxxed out her credit cards, eventually filing for bankruptcy. She and her grandchildren live on Summer's Social Security and a small income Crawford makes by working at the apartment building in which they live.

Treatment at the Illinois institute, which is headed up by Dr. Michael Pranzitelli, has helped Summer. The center conducts research, offers treatment, and is working to understand the scope of the problem and also increase awareness, according to its Web site.

Summer is the only person in Idaho with the syndrome - representing the sole red dot on the center's Web site map.

Every six months Summer goes for treatment at the center. Because of the cost and because Summer's immune system is compromised, flying on a public airplane is out of the question.

Instead, Summer and her grandmother are flown by Miracle Flights for Kids to St. Louis. Crawford then rents a car and drives to Springfield, where they stay at a Ronald McDonald House. Even the rental fee and the $8-per-day fee to stay at the Ronald McDonald House strains the family's limited budget.

Twice a day, every day for the past six years, Crawford has given Summer shots in the leg. Summer sometime smells because the steroids have caused early puberty and it possible that she will get cataracts, also because of the drug.

A playful tap on Summer's arm can cause extreme pain and combing her hair is difficult because her scalp is tender. She takes Valium for anxiety and another medication to help her sleep at night because she gets night terrors. She also is given medication that is used for ADHD, OCD, sleep apnea, although she has none of those problems.

She also have behavorial problems and can be aggressive toward other children - not because she is mean, but because she is ill, Crawford said.

Summer does not stay overnight with other children because she has to wear pull-ups when she sleeps. She had an accident during a sleepover that was not handled well by the parent, Crawford said. Her granddaughter also has repeatedly been made fun of for being fat.

“It's tough and people are mean to her,” Crawford said.

Janey has started to exhibit behaviorial problems - the result of having an older sister who receives a lot of attention and sometimes is taken out of school because of medical problems.

Crawford said Dr. DeLand's support and Christmas-time assistance from the Angels Over Sandpoint, along with local car dealer Scott Reynolds' willingness to help her purchase a reliable car at a decent price given her credit problems are very appreciated.

The ones (friends) I have are really good and really helpful,” she said.

The families of Summer and Gavin Scheel are the recipients of Jacey's Race which takes place Sunday at Sandpoint High School. The 5K walk begins at 8:15 a.m., while the 1K starts at 9 a.m. Registration is $30, except for children ages 12 and under who can walk or run for free.

Information: www.omsusa.org and jaceys-race.com