The rheumatologist at CHKD knew right away that Nora was suffering from a chronic inflammatory disease.
Nora Doudna just wasn’t her bubbly self last winter. Throughout December, the 4-year-old had a low-grade fever and frequently complained that her legs hurt.
Several times, Meagan Doudna took her daughter to the doctors at a primary care center.
“They thought it was a bug that was going around,” Meagan recalls. “The doctor told me not to worry, it would go away.”
Nora complained of being tired and achy throughout the holiday season. “After a while, she seemed to accept feeling bad,” her mother said. “She just couldn’t keep up with the rest of the kids. And I noticed she was walking funny.”
Through January, Meagan kept Nora home from preschool. She lost count of how many times she took her daughter to the doctor. “I knew something was wrong. A mother just knows,” she said.
One day Meagan was telling a friend how frustrating it was that no one had found out what was wrong with Nora and how frightened she was for her daughter. The friend told her about Children’s Hospital of The King’s Daughters.
“I’m from Ohio,” Meagan said. “I hadn’t heard of CHKD before. I didn’t think I could just take my child there.” Meagan is a Navy wife. Her husband, Navy gunnersmate Benjamin Doudna, is stationed at Little Creek Amphibious Base.
Then, in early February, Nora’s legs and face became swollen and a rash appeared on her face, hands and neck. Meagan took her to the emergency room at the Naval Medical Center in Portsmouth. The Virginia Beach mom says she was relieved when the ER doctor told her that Nora needed to go to CHKD for care.
“I remembered what my friend said, and I knew I was finally going to get help for Nora,” Meagan said.
Pediatric rheumatologist Christos Gabriel knew within minutes of seeing Nora at CHKD that she was suffering from juvenile dermatomyositis – JDMS – a chronic inflammatory disease that affects the skin and muscles and sometimes other organs.
“It’s not something you see every day,” he said, “but there are clear signs that set it apart from other autoimmune diseases, such as lupus.”
Nora’s rash, muscle weakness, fever, swelling and breathing problems indicated a very advanced case. Her diagnosis was quickly confirmed through blood tests, which showed an elevated count of muscle enzymes.
“Basically, all her muscles, even the involuntary ones, were inflamed,” Dr. Gabriel said. “She needed very aggressive treatment right away to save her life.”
Dr. Gabriel told Meagan and Ben that Nora was very weak because of the extensive inflammation in her muscles and skin. Her kidneys were barely functioning, her breathing very shallow.
In CHKD’s pediatric intensive care unit, critical care specialists catheterized her to relieve the swelling in her kidneys and bladder, hydrated and fed her intravenously, and initiated chemotherapy to control the inflammation in her body. A ventilator was used to help her breathe.
Meagan recalls the agonizing days she watched over Nora in the PICU. “It was a very slow process because she was so sick,” Meagan said. “She had all these tubes in her and she was so weak. Her nurses were wonderful. They really helped me while they helped her.
“I knew Nora was finally in the right place to get better.”
Once her crisis was over and Nora began to recover, she was moved from intensive care and began inpatient physical therapy to strengthen her weakened muscles. After a few weeks, she was strong enough to go home.
“The good news is that the disease can be managed on an outpatient basis with steroids and anti-inflammatory medications to reduce the symptoms that make life difficult for kids with JDMS,” Dr. Gabriel said. “There is no cure as such, but with proper care and chemotherapy, a significant percentage of patients will go into remission.”
Meagan learned to give Nora her weekly chemo shots and daily medications at home. Once a month, she returns to CHKD for an intravenous infusion of a combination of chemo drugs, similar to what cancer patients receive. “It controls the inflammation and keeps the muscle enzymes in check,” Dr. Gabriel said.
“Today Nora is doing very well,” he added. “The chemotherapy is working for her, and she can be as normal as any 5-year-old.”
Besides the chemo and medications, children with JDMS need regular activity to keep their joints from stiffening and a balanced diet rich in protein and calcium to ensure healthy growth, strong bones, muscle repair and joint lubrication.
Dr. Gabriel and his staff remind Meagan of the benefits of exercise to strengthen Nora’s weak muscles. Today she keeps active without additional therapy. With a playground near their home, her mother says Nora doesn’t need much prompting to play outside.
“We know that winter will be a challenge for us because Nora has a weakened immune system and needs to be extra careful avoiding germs,” Meagan said. “We also have to make sure she has plenty of sunscreen on when she goes out because her skin is very sensitive.”
But Meagan predicts this winter will be a far cry from the past one. With her daughter well, the future looks much better.
“Nora loves going back to CHKD for her checkups with Dr. Gabriel,” she said. “She doesn’t even mind the monthly chemo.
“I’m just so glad CHKD was there when we needed it,” she added.
“My friend was right. CHKD did have the answers.”
Dr. Gabriel practices with Children’s Specialty Group PLLC at CHKD.
This story was featured in the fourth quarter 2010 issue of KidStuff, a publication of Children's Hospital of The King's Daughters. Click to read more patients' stories.