A new hip for a patient with rare neuromuscular condition

It has only been one month since 16-year-old Malena had hip replacement surgery, but the operation has been a game-changer.

Prior to the surgery, Malena was bed-bound, suffering pain and problems with her joints like many teen and adult patients with spinal muscular atrophy (SMA). The rare genetic disease affects nerve cells and causes muscles to become weak.

When Malena developed arthritis between the ball and socket joint of her hip, she sought pain relief, as well as an increase in function and movement, through a complete hip replacement.

“Her left hip was so painful that it was difficult for Malena to transfer from her wheelchair, participate in regular activities or remain in an upright position for several hours a day,” said Nina Daya, Malena’s mother. “We were looking for the best solution, and Dr. Pereira made that happen for us.”

Internationally renowned pediatric orthopaedic surgeon Jon Davids had referred Malena from Shriners Children’s Northern California to consult with UC Davis orthopaedic surgeon Gavin Pereira. Pereira specializes in complex hip and knee replacements and revisions of failed joint replacements within the UC Davis Department of Orthopaedic Surgery.

Pereira had extensive discussions with Davids about the surgical options available to Malena. Davids was well versed in operations on the hip. He felt that a hip replacement might be a good option if it was technically possible.

Pereira met with Malena for an evaluation and heard her desire for a hip replacement.

“After spending time talking to Dr. Pereira, Malena felt like he understood her vision for herself and thought he was a perfect provider to make this a reality,” Daya said. “The relationship between Malena and Dr. Pereira is special and built on trust.”

No reported cases in medical literature

Pereira had never seen a patient like Malena before. After evaluating her, he looked in the medical literature.

“I found no case reports on hip replacements performed on a patient with SMA. If it has been done, it’s not been reported,” Pereira said. “Most surgeons would say that a hip replacement is not indicated in a patient who is not able to walk, and they would be right.”

While many patients with SMA are not viable candidates for hip replacement surgery for many reasons, including softness of their bones, Malena had been treated quarterly with Spinraza (Nusinersen), the first FDA-approved drug to treat SMA, which can strengthen patients’ bone mineral density.

Pereira was up for the challenge. He saw two options that could help Malena:

• He could remove the ball of the thigh bone or femur and leave Malena without a hip joint, known as a Girdlestone operation. But without the ball of the thigh bone, he worried that she would still experience the sensation of bones rubbing against each other or the range of motion of the hip would reduce significantly. It also did not guarantee that she would not have chronic pain.

• He could give her a hip replacement, but he was concerned this was a more extensive operation than required and would expose her to more complications. She also had a very small frame, and he didn’t know if the smallest implant on the market would fit her.

He wouldn’t know what path he would need to choose until he was in the operating room. But Pereira began surgical planning with military precision. He worked through all of the details of the surgery with his team, using three-dimensional printed models of CT scans of her pelvis.

He also ensured that her team of specialists would be able to care for Malena after her surgery to make certain that she was stable. The team of specialists included:

• pediatric anesthesiologists Nina Schloemerkemper, Kenneth Furukawa, Cathleen Lammers and Amrik Singh
• pediatrician Robert Byrd
• pain specialist Cherie Ginwalla
• pediatric gastroenterologist Sunpreet Kaur
• pediatric pulmonologist Rory Kamerman-Kretzmer
• pediatric hospitalist Heather McKnight
• Physical Medicine and Rehabilitation physician Nanette Joyce
• division chief of the pediatric intensive care unit (PICU) Jennifer Plant
• division chief of general pediatrics Serena Yang
• chief of pediatrics Satyan Lakshminrusimha

They were all part of a single email chain, so everyone was aware of the dedicated role they would play in support of her care. In addition, nurses Julia Pasterski, Cindy Anderson and Melissa Wulff, along with operating room nurse Inna Murzin and principal surgical technician Jeff Rogers, were heavily involved in the planning.

“Everything was finely tuned. Everyone knew their role. We had planned for every possible outcome in surgery,” Pereira said.

This surgery is life-changing as it will open many doors for Malena’s overall well-being and confidence through increased mobility.” —Nina Daya

A new lease on life

Pereira performed a hip replacement surgery for Malena last month without complications. Her bones were solid, and the replacement left hip fit her frame.

He saw Malena after surgery that evening on his rounds in the Pediatric Intensive Care Unit.

“She was sitting up in bed like nothing had happened to her. She was comfortable. It was remarkable,” Pereira said.

Daya said she is proud that Malena is the first SMA patient to have had hip replacement surgery. It has taken away Malena’s pain and given her a brighter future.

“This surgery is life-changing as it will open many doors for Malena’s overall well-being and confidence through increased mobility,” Daya said. “I can see opportunities with adaptive sports and possible robotic options to assist her in the future. Life is exciting! We are blessed that we have UC Davis.”