7-year-old’s mystery illness baffles specialists

Gracie Lee Straus loves taking photographs of people and turning them into jigsaw puzzles on her iPad — from her friends and teachers to her doctors, nurses and therapists. She loves puzzles, even the old-fashioned, hands-on ones.

Gracie Straus, 7, enjoys two strawberry lollipops, one of the only solid foods she is allowed to have, in her family’s home in Dallas, Oregon, on Thursday, Sept. 25, 2014. Gracie lives with an undiagnosed disease that the family is working to get managed by doctors and hospitals all over the country. Anna Reed, Statesman Journal

Capi Lynn, Statesman Journal October 11, 2014

On the dining room at her home in Dallas is a stack of puzzle boxes, many of them of the Disney variety. Imagine dumping them all on the table, mixing them up and trying to put the various pieces together.

That is the jumbled picture of Gracie’s health. No matter how hard everyone tries, the pieces just don’t match, stumping her doctors and specialists and frustrating her parents, John and Mandy Straus.

“They have given diagnoses for her to get treatment, but some of the stuff doesn’t fit,” Mandy said. “She’s got a laundry list of diagnoses, but it’s not the big picture.”

Gracie has such a rare medical condition that it was in review by the National Institute of Health three years ago and will soon be in review again.

She has been diagnosed with cerebral palsy, epilepsy, spina bifida, autism spectrum disorder and global developmental delays. With each diagnosis, the puzzle has become more baffling, the treatment more elusive. For official medical purposes, she has an undiagnosed neuromuscular degenerative disease that resembles ALS, which attacks the nervous system.

Her life has been a maze of wires and tubes. A vagus nerve stimulation wire runs under her skin from the nerve near her collarbone to her brain stem, helping prevent electrical bursts in the brain that cause seizures. It’s a pacemaker for epilepsy. A gastrostomy tube in her abdomen delivers nutrition directly to her stomach. She has a catheter and is on oxygen.

Amid the medical chaos is a sweet girl who loves all things Disney and ice cream with sprinkles and who is blessed with a family that won’t stop looking for answers.

Gracie turned 7 on Saturday, Oct. 11, and celebrated by going horseback riding on her favorite steed, Goliath the TV-star-turned-therapy horse. Goliath was on the show “Dr. Quinn, Medicine Woman.”

Gracie Straus, 7, has leg braces with butterflies and Disney character straps as she plays in her family’s home in Dallas, Ore., on Thursday, Sept. 25, 2014. Gracie lives with an undiagnosed disease that the family is working to get managed by doctors and hospitals all over the country. Anna Reed, Statesman Journal

Miracle at birth

Gracie was born by emergency C-section because she had an ovarian cyst, which was growing fast and attaching to her organs. She weighed 8 pounds, 11 ounces and had a visible bulge on the left side of her tummy. The 1½-pound cyst was removed when she was 3 weeks old. The surgeon also had to take her left ovary and fallopian tube.

Her parents were told there had been only 22 previous cases like hers in the U.S.

“They told us at the very beginning Grace was a miracle,” mom Mandy said.

Gracie is the perfect model for the teal-colored T-shirt she wears, emblazoned in bright pink with the word “happy” and a smiley face. There is a bounce in her step — even though she’s wearing ankle-foot orthosis — as she zips down the hall to grab a game from the closet.

A 50-foot line to the oxygen machine gives her free run of the house. An IV pole accompanies her.

“She’s always had a happy disposition,” her mom said. “She lives life as it is and is everybody’s best friend. She gives hugs and kisses to everybody. She’s a therapy girl herself.”

One curse of Gracie’s condition has also been a blessing. She has been spared some pain because of the progressive deterioration of her nerves.

Her parents didn’t know just how fast it was progressing until a couple of years ago, when Gracie stepped on a rusty nail in the back yard and didn’t feel a thing. She was bleeding and had to go to the urgent care clinic, but all she was upset about was the hole in her favorite Toy Story Crocs.

Sisters Izzy, 5, left, and Gracie Straus, 7, play in their bedroom in Dallas, Ore., on Thursday, Sept. 25, 2014. Gracie lives with an undiagnosed disease that the family is working to get managed by doctors and hospitals all over the country. Anna Reed, Statesman Journal

All in the family

Gracie has two sisters, Maddy, 11, and Izzy, 5. Izzy is the protective one. Their parents learned just how protective when Gracie recently needed to be on an NPO diet, an abbreviation meaning nothing by mouth.

Mandy and John noticed Gracie was often clearing her throat and gurgling in her speech. Doctors discovered the muscles in her digestive track were deteriorating. She had been unable to digest her food. It was collecting in a clump in her stomach, increasing the risk of acid backup. After the obstruction was removed, doctors recommended a formula-only diet through her G-tube.

“That was probably the hardest thing we had to do,” Mandy said. “She cried for almost a week, saying ‘I’m hungry.’

“It was brutal — physically for her and emotionally for us.”

Little sister Izzy tried to help Gracie at night, sneaking fish crackers into the bedroom. Older sister Maddy blew the whistle. Their parents explained to Izzy that they had to keep Gracie’s stomach safe.

Gracie has recently been able to add ice cream and Popsicles back into her diet, to everyone’s delight. She also can have lollipops, and she digs with gusto into a bin full of Dum Dums looking for her favorite flavor, strawberry. She emerges from the kitchen two-fisted, grinning for the camera.

Gracie Straus, 7, moves her tower for her feeding tube and oxygen around the living room so she can set up a board game in her family’s home in Dallas, Ore., on Thursday, Sept. 25, 2014. Gracie lives with an undiagnosed disease that the family is working to get managed by doctors and hospitals all over the country. Anna Reed, Statesman Journal

The fight for treatment

John and Mandy have insurance through his employer. He is a sheriff’s deputy at the Marion County jail. Even with insurance — they are covered by Kaiser Permanente — they say they have had to scratch and claw for treatment they believe their daughter deserves, especially recently.

To be fair, Kaiser has covered Gracie since she was born. It approved an expensive whole exome sequencing test a year ago and multiple visits at Doernbecher Children’s Hospital, where Gracie has been treated since she was 5 months old.

The most recent battle centered on a change in Gracie’s formula prescription. Gracie lost six pounds and was malnourished before the change was finally made, and the family says that happened only after Rep. Jim Thompson, R-Dallas, got involved. The family filed a complaint with the state Insurance Division.

Thompson confirmed that he met with Kaiser officials to plead the Strauses’ case.

“I’m flying blind trying to force people to sit down and negotiate a treatment settlement for a very sick little girl,” Thompson said. “I can bring them to the table, and I have the power of shame — I use that liberally — but I don’t have the authority to effect treatment by such and such a date.”

The family says providers at Kaiser have urged them to put Gracie on palliative and hospice care, which they refuse to do.

“She’s an active, joyful little girl,” Mandy said. “She goes to church and school. We’re not going to give up on her. We’re still enjoying life with Grace, and she’s still enjoying life with others.

“We know it’s not there yet. She’s still happy and functioning. All we’re asking for is continued care.”

Asked for comment, Kaiser on Friday released this statement: “Our hearts go out to families caring for a sick child. We take it very seriously when any patient is disappointed in their care experience. Kaiser Permanente is recognized nationally and in the Northwest for providing the highest quality health care. Due to patient privacy, we cannot discuss specific patient care.”

Open enrollment for John’s insurance coverage is in November and they plan to switch carriers, even though it will probably be more expensive.

Everything in their power

“We honestly feel like they’ve been waiting for her to die, and we’re still actively seeking and researching treatment,” said Mandy, a stay-at-home mom who had worked as a certified nursing assistant and a medical assistant.

“As a mom, you want to do anything and everything in your power for your child. My job is to advocate for everything she needs. I do that for my oldest child and my youngest child, but I have to do a little more for Gracie.”

Gracie has an independent streak, as you would expect from a 7-year-old. When Mandy jumped up to assist as a beeping alarm signaled the end of Gracie’s feeding, Gracie demonstrated that independence.

“I want to do it,” she said, turning nob and detaching the tube.

When Doodle, their half golden retriever-half poodle therapy dog, inadvertently lies down on her oxygen line, she gets a bit annoyed and nudges the dog away.

Doodle has been a welcome addition. They rescued him this summer after he was abandoned at a dog groomer’s office. They tracked his medical records to a veterinarian clinic and learned he was a trained therapy dog. He is sweet and attentive and knows to camp right next to Gracie’s monitors at night.

Strength in numbers

Mandy and John are fortunate to have an incredible support system, between their family, their church, John’s employer and the girls’ school.

They attend Salem Evangelical Church, where members have provided moral and prayer support. John’s team at the jail donated $550 in gas cards for an upcoming road trip to see two genetic specialists in California.

All three girls attend Faith Christian School in Dallas. Gracie goes to school for three hours in the morning, and her mom volunteers as an aide so she can be there if Gracie has a seizure or her catheter needs changing.

As a fundraiser to help the family, the school sold 2,000 pounds of apples that were donated by Perryhill Farm in Perrydale and an anonymous donor. The kindergarten and third-grade classes — 32 students, along with parents — picked and sorted the apples. Izzy is in the kindergarten class.

Other classes did their own fundraisers, including a lemonade stand, a baked sale and a movie night. A parent organized a car wash.

“When word spread that one class decided they wanted to do something, then every class wanted to do something,” said Stephanie Earhart, who works part-time in the office and is president of the parent teacher fellowship. “These kids just have a heart for this type of work. They go nuts for raising money for somebody who needs it.

“The school just wrapped their arms around this family.”

On Friday, during chapel, Principal Michele Stein announced in front of her 125 students that they had raised more than $6,500 for the family.

The Strauses were overwhelmed and grateful. They will use the money to purchase a portable oxygen concentrator, which will allow Gracie to extend her school day. It would normally run around $4,800, but they’ve found a used one that a man is selling for $1,800. It belonged to his late wife.

They also will purchase for around $2,400 a pulse oximeter, which measures the oxygen in Gracie’s blood and sounds an alarm when it drops too low.

Searching for answers

Gracie and her sisters are counting down the days until their trip to California, which will include a visit to Disneyland. Their parents are more excited about getting second and third opinions from a pair of geneticists.

Gracie has appointments at Lucile Packard Children’s Hospital at Stanford University and at Children’s Hospital of Orange County in Anaheim, with doctors specializing in undiagnosed neurodegenerative diseases. Neither visit will be covered by insurance.

John and Mandy hope to expand on the journey that began a year ago with the exome sequencing test. Blood and DNA samples of Gracie and both of them were sent to the genetics testing lab at Baylor College of Medicine in Texas. Six months later, they received a 15-page genetic report that looks at abnormalities, transitions and deletions. They didn’t really get any answers, but they hope the information will someday help identify Gracie’s disease, even if it’s five or 10 years down the road.

In the meantime, they will continue to seek answers.

Mandy shared a voice mail from a doctor at Doernbecher Children’s Hospital who offered care even if insurance did not cover it. “There is no one like Grace. She is unique and special in our hearts,” the doctor said.

“They’re not giving up on Grace,” Mandy said. “They can’t fix it, but they can support us.”

Forward This appears Wednesdays and Sundays and highlights the people, places and organizations of the Mid-Willamette Valley. Contact Capi Lynn at clynn@StatesmanJournal.com or (503) 399-6710, or follow her the rest of the week at Twitter and Facebook.

Source Statesman Journal


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