Bracing Against the Curve: Anakalia’s Story of Strength and Support from Shriners Children’s

Anakalia was born with osteogenesis imperfecta (OI).

Unlike many families, her parents were fully prepared to care for a child with this disorder. Her dad, Mike, was born with the same condition. “They asked me if I wanted to do testing to see if she had OI,” said Anakalia’s mom, Krystine. “I said no – I knew I wanted to keep her either way.” Krystine got the news that her baby would have OI during an ultrasound, showing that little Anakalia already had fractures.

Commonly referred to as brittle bone disease, osteogenesis imperfecta is a rare, permanent genetic disorder that prevents the building of strong bones. Children with the disorder have bones that break easily and they may have deformed bones. OI can also result in fragile teeth, spine curvatures, hearing loss and weak muscles.

The cause of osteogenesis imperfecta can be traced back to genetics; more specifically, a gene defect that influences collagen production. Collagen is a protein that is vital to healthy bone development. With OI, either the body does not produce enough collagen, or the collagen that is produced does not work properly. Most children with OI inherit the gene defect from one or both parents. In Anakalia’s case, it was inherited from her dad. “I’ve been through almost all of the same things she has,” said her father, Mike, who was also treated at Shriners Children’s Northern California.

Anakalia’s osteogenesis imperfecta is categorized as OI Type III. Infants with OI Type III are typically born having already experienced fractures in utero. A shorter stature, curved spine, rounded rib cage and other bone conditions are characteristic of Type III OI.

OI Expertise at Shriners Children’s Northern California

Anakalia, who recently turned 13, has been receiving treatment from Shriners Children’s Northern California since she was a baby. “Most providers in our area don’t know how to treat OI,” said Krystine. “They wrap her up and send us to Shriners Children’s.”

Given the nature of her condition, Anakalia is prone to frequent bone fractures. Four years ago, she underwent surgery with Debra Templeton, M.D., to get rods placed in her legs. These rods assist with both strength and stability. “I haven’t broken a bone in my legs since I got the surgery,” Anakalia said.

When Anakalia fractures something, she and her parents have a well-organized system for getting her treatment. They quickly wrap up the affected area and drive her to the local hospital in Redding. There, she is then placed in a splint before being transferred to Shriners Children’s Northern California for surgery. After about a month in a cast, she is done until the next fracture occurs. “It kinda hurts, but I’m kinda used to it by now,” she said. “It’s not as bad after a while.”

Along with having fragile bones, Anakalia’s spine has also been affected. Spinal deformities are known to occur in anywhere between 39% to 78% of all patients with OI.

For Anakalia and other patients with spine and spinal cord issues, Shriners Children’s Northern California has a robust spine and spinal cord program that has been able to treat them. Led by a team of highly skilled orthopedic surgeons, the program offers comprehensive care tailored to each child's unique needs. From scoliosis and kyphosis, to spinal cord injuries and congenital deformities, every aspect of treatment is approached with compassion, innovation and a commitment to maximizing function and quality of life.

Straightening Out With Halo

Prior to her halo traction treatment, Anakalia had an 87° curvature in her spine, with a slight twist. To put this in perspective, any spinal curve exceeding 60° is classified as severe. This curvature made it difficult for her to maintain proper posture while sitting and standing, and if left unaddressed, it could lead to further complications in the future. Chief of Staff Rolando Roberto, M.D., recommended that she undergo halo traction to assist in straightening her spine.

Halo traction is typically a first step in correcting severe scoliosis, kyphosis and other spine deformities. Patients remain in the hospital the entire time they are in traction, typically three to eight weeks. Usually after halo traction, patients have spinal fusion surgery to permanently stabilize the spine. Halo traction reduces the risk of damaging the nerves or soft tissues that surround and support the spine during surgery.

During surgery, an orthopedic surgeon attaches a lightweight metal ring (halo) to the patient’s skull with small pins. Once the halo is in place, it is attached to a pulley system, which is on the patient’s bed, wheelchair and walker. During the following several weeks, weight is added to the pulley to slowly straighten the spine.

Even though the surgery was a significant decision, Anakalia and her family were confident it was the best option and felt adequately prepared. Given the unique curvature and twist in Anakalia's spine, there was a realization that achieving a complete correction might not be feasible. However, this did not deter them, as they understood that any degree of improvement would be a step forward from her previous condition.

“I was actually excited to get the halo,” Anakalia said. “It seemed fun and I was excited to straighten out and be taller.”

After the initial surgery to get the halo on, she said the first day was the worst of it. It was a bit of an adjustment at first: learning how to do her hair, adjusting the pulley and finding a comfortable sleeping position. After the first week, the whole family got into a routine and it was seamless from there.

For four weeks, Anakalia walked around Shriners Children’s Northern California with her halo. She decorated her room, played games in the GUM Pavilion and socialized with other patients. Within the first week, she said there was already a noticeable difference in her posture. By the last week, the change was incredible, and she was ready to have the halo taken off. She went into surgery extremely excited.

On the Road to Recovery

“At the end of her halo therapy, her curvature was in the 50° range,” said Krystine. “So, going into her surgery, we were prepared for that, or a degree close to that. Instead, Dr. Roberto informed us that he was able to correct her spine to 34°, which was way more than we anticipated. We were doing the surgery in hopes of stopping any internal organ damage or pain that could happen the more it curved. Knowing he was able to correct her more than we thought made the whole experience better and even more worth it.”

After a month recovering from surgery to remove the halo, Anakalia returned to Shriners Children’s Northern California for a follow-up appointment with Dr. Roberto. Her scars from the halo were already fading quickly, and she was all smiles in her hot pink back brace. “She’s making a really excellent recovery,” said Dr. Roberto.

Dr. Roberto was also able to show the before-and-after X-rays to Anakalia, her parents and her grandmother. The improvement in her spine’s curve was immediately evident. “Though every child will have a different experience with halo traction, we would highly recommend it, especially to someone who is suffering from other issues because of the curving in their spine,” said Krystine.

Lasting Connections

Since her family makes the long drive to Sacramento from Redding, Anakalia wanted to make the most of her time back at the hospital. She visited her physical therapy team and her favorite nurses with handmade cards and hugs to show her appreciation. “I’m 4 feet tall now!” she said, chatting with her favorite physical therapist, Erin Lombardi, PTA.

Erin took the time to play air hockey with Anakalia, their favorite game during her hospital stay, and helped her practice walking without her walker. Anakalia was all smiles throughout the visit, which made her parents and grandmother ecstatic. “I’m so proud of her and the strength she has had throughout this whole journey,” said Krystine.