Controversial Utah Cleft Palate Care -III

A summary of Part II re Cleft Palate Care at Primary Children’s

Part II deals with the the timing of jaw surgery in cleft lip and palate care at Primary Children’s Hospital in Salt Lake, Utah. At this children’s hospital, ProPublica asserts pediatric surgeons are taking controversial risks with how they care for kids with cleft lip and palate.

Experimenting With Bone Grafts

‘About seven years ago, spurred by what [Dr. Dana] Johns said was a “hypothesis we had,” Primary Children’s cleft team began experimenting with how surgeons bone grafted the upper jaw.

Without putting in place the protections of a formal research study, Johns and her colleagues decided the invasive surgeries could be done successfully when children were years younger — age 2 — than was standard practice in their field.

The “gold standard” for a bone graft is to use a piece of the child’s own hip. At 2, a patient doesn’t have enough bone to harvest. So over a period of about two years, Primary Children’s tested in its toddler-age patients different combinations of materials that would create a lasting bone graft.

Among the materials was a controversial product called bone morphogenic protein, or BMP, which stimulates bone growth. The BMP is put into the jaw where the child is missing bone and, in theory, spurs the body to naturally make bone, gradually filling the gap.

Some cleft doctors have hailed BMP as a less invasive way to treat patients since it first came to market in 2002 — but Primary Children’s believed it could be used in children years younger than their peers at other centers.

The parents of several patients told ProPublica they were never informed that their child was undergoing an untested procedure. In some cases the bone graft failed, necessitating a repeat of the painful operation.

In 2017, Gavin Rogers, 21 months old with the wispy blond hair of a baby, had bone graft and hard palate repair. Afterwards, “he was screaming all the time. He wasn’t sleeping, wasn’t eating. I had to squirt milk into his mouth,” his mom, Emily Rogers, recalled.

Primary’s decision to use BMP in toddlers was a risky bet. In 2015, the U.S. Food and Drug Administration issued a safety warning about using BMP in children. The warning said that BMP hadn’t been approved for children “because their bones may still be growing and using this product may cause serious injuries.” The FDA action followed revelations of injuries, including alleged deaths, related to the use of BMP in spine patients. BMP’s maker was subjected to a congressional investigation and paid hundreds of millions to settle civil suits.

The FDA recommended against routine use in children and told doctors to be sure to inform parents about the risks.

Doctors are allowed to use products like BMP for so-called off-label purposes and commonly do, often to the benefit of their patients. In 2017, Dr. Jeff Hammoudeh, a plastic surgeon at Children’s Hospital Los Angeles, published one of the largest studies of BMP in cleft patients and found that over nine years of use its safety and success was comparable to using bone from the child’s hip. But the average age of his patients was 11 and his youngest patient was 6. He said he wouldn’t use BMP in patients any younger than that because of concerns, shared by other experts, about the long-term growth of a child’s face and questions about the safe amount to use in a child of that size.

Johns, of Primary Children’s, said that Hammoudeh’s study was one of the reasons her team felt confident in using BMP.

Hammoudeh said doctors need to be up-front about what they are doing and build in clear guardrails. When he experimented with performing lip repairs in newborns, he did so through a supervised research study. Parents, he said, were given the choice to have the traditional protocol, just as they were for bone grafting with BMP.

Some doctors may eschew the word “experiment” when trying out a new protocol, he said, but that’s what it is — even if they deem it “innovation.”

“You can soften the terminology any which way, but if you don’t know what the results will be, you don’t know what the results will be,” he said.

Johns told ProPublica she doesn’t offer parents the choice to have a bone graft at the traditional age because she’s confident that her way is better. Giving them the option, she said, “means that we’re telling them, ‘OK, we’re going to allow you to have subpar results.’”

Johns said that since as early as 2018, the parents of patients have been told that the new protocol is superior to traditional care, a claim she said is supported by observational results from the team’s first patients.

The Rogerses and other parents who spoke with ProPublica said Siddiqi, the lead plastic surgeon at the time, led them to believe that grafting at toddler age was the standard of care.

“One hundred percent,” said one mom, whose daughter was not yet 18 months at the time of the procedure and had to repeat the surgery later. She said the conversation with a reporter “was the first I’m hearing this isn’t typical.”

After his surgery, Gavin happened to see a new doctor outside the hospital. Rogers said she stopped breathing when she learned that around the country, many doctors time bone grafts to when certain adult teeth come in. Gavin still didn’t have all his baby teeth.

And the bone graft didn’t work. Gavin faces a second bone graft surgery this summer at 8 years old — in the age range when most other doctors would have done it the first time.

First formal study of Cleft Team

Four years after Primary Children’s started the protocol, the hospital did its first formal study — a review of 14 patients’ medical records. From around 2017 to mid-2019 — when Johns was telling patients the protocol was a success — the cleft team used different surgical techniques and a combination of bone products, including the BMP, that proved unreliable, and some patients needed additional grafts, according to the study and a university spokesperson.

A hospital spokesperson said its bone grafting procedures have advanced since patients such as Gavin had the operation. Khosla, the Stanford doctor who reviewed the protocol, reported in his summary that newer hospital data demonstrates good early results.

Johns and Agarwal said their new protocol was a slow evolution and didn’t need to be overseen by independent reviewers. Performing a bone graft sooner, they said, would solve a common problem in cleft patients in which the arch of their mouth is narrow and collapses inward as they grow. Moving to age 2 was a minor progression, Johns said, noting that the mouths of a 5-year-old, the youngest age most doctors would do a bone graft, and a 2-year-old are similar.

“There was nothing that felt outlandish about it,” Johns said.

Johns concedes they don’t know yet how their patients’ faces will grow over time. So far, she said the outcomes are trending in the right direction, “but we recognize that we’ve got eight more years to continue to see if it holds up.”

She’s clear about this uncertainty with her patients, she said, as well as how Primary’s protocols diverge from the standard. She said if parents are uncomfortable, she encourages them to seek a second opinion. (One mother said Johns’ colleague told her that he would no longer treat her son after she sought a second opinion.)

Primary Children’s Hospital & Jessica Bernstein’s son

Jessica Bernstein, whose 6-year-old son is a cleft patient at Primary Children’s and whom the hospital connected with ProPublica, said she had the utmost confidence in the care received and felt Johns and the other doctors carefully thought out decisions. She said they told her that the hospital’s timeline for care was based on the latest, best research. Although she said she wasn’t informed that aspects of the hospital’s protocol differ from the standard or that BMP was off-label for children, she felt her son was being treated in the right place.

Other cleft doctors said previous attempts to fix the upper jaw line between birth and 2 years old — known as “primary bone grafting” — were abandoned because of poor outcomes.

Dr. Ron Hathaway recalls when he was at the University of Indiana and participated in a study that compared outcomes at five cleft centers. One fared significantly worse. The upper jaws of many of its patients didn’t grow as expected, leaving them with underbites. Hathaway was stunned to learn it was his program.

Indiana, it turned out, was the only one that grafted before age 2. The others waited until patients were school age. Hathaway had been involved in such grafting at Indiana since 1992, and now he faced the humbling data that his patients were three times more likely than the study’s best center to need invasive jaw surgery because of it.

Indiana eliminated primary bone grafting, and when the study was repeated 10 years later, Hathaway said, outcomes had drastically improved. While this type of research can’t definitively prove causation, he said, “a thinking person would say primary bone grafting causes [poorer growth] of the upper jaw.”

The original study was published in 2011, joining a body of research over decades that showed such grafting was “significantly detrimental,” Kirschner, of Nationwide, said.

Nevertheless, in 2016 the cleft team at Primary Children’s began questioning whether operating earlier would be better, said Agarwal, the chief of plastics. He and Johns said what they do is different — in terms of a slightly older age and specifics of the procedure — from the primary bone grafting of the past. Khosla, of Stanford, wrote in his review summary that the protocol challenges conventional wisdom, but “their rationale and the theoretic benefits are sensible.”

For Cindy Anderson, whose daughter’s bone graft echoed Gavin’s in both timing and failure, it wasn’t just that her daughter had to repeat a surgery. Had they been informed of the innovative nature, Anderson said they might have agreed because of their faith in the doctors. But not to be given a chance to consent made them feel duped.

She said they’re left wondering about Primary Children’s: “What the heck are they doing?”’

So what do you think of Primary Children’s Cleft Team?

Anxious to hear now that you’ve finished the three part Pro Publica article. 

Unnecessary surgeries? Is BMP a good idea? Other thoughts?

___

Visual depiction of the Halo Procedure by a doll fitted with a model of a metal device that a young patient will have to wear after an intensive jaw surgery. Primary Children’s hospital needs the doll to explain the procedure because its doctors perform the operation regularly on patients much younger than peers in the medical community, who wait until patients are finished growing.

 

My latest book, PRIVILEGED KILLERS, is a true story about a half-dozen Dark Triad people in my everyday life - narcissists, manipulators, and psychopaths. Three of 'em murdered people, and one came after my wife and me. Print and e-book versions of this (and CLEFT HEART) available at Amazon and elsewhere online. Also at your local bookstore.