Cambodian Boy With Rare Affliction Gets Care, Home in US

lenox, Massachusetts – This town of old estates along tree-lined roads has been home to industrialist And­rew Carnegie, novelist Edith Whar­ton, musician James Taylor and more recently, severely disabled Cambodian orphan Roth Arun.

Ellen McDaniel Loftus knew from experience what to do when the 2-year-old arrived in the US in 2004. She stripped him in the airport bathroom, slathered him in scabies cream and dressed him in winter clothes, knowing that the November chill would be only the first of many shocks for the boy who had come from Phnom Penh’s Nutrition Center orphanage.

She did not know, however, how to say Khmer names, so “Arun” took the pronunciation “Aaron.” She also did not know whether or not doctors would be able to help him.

Arun was born with myriad de­formities and was abandoned as a baby. A hole in the front of his skull had allowed his brain to spill into his forehead as he grew, forcing his eyes apart and his nose down. His left arm and leg had only partially formed at birth, and his single foot had only four toes.

Doctors also found other less ob­vious abnormalities, like bubbles formed of excess fluid in his brain and no olfactory glands to give Arun a sense of smell.

Dr John Mulliken, a pediatric plastic surgeon at Children’s Hos­pital Boston who operated on Arun, said the boy’s variety of problems could be diagnosed as “oromandi­bular-limb hypogenesis syndrome,” which adds up to a list of symptoms with no known cause.

Dr Mark Proctor, a pediatric neurosurgeon at the hospital, was initially apprehensive about operating on Arun “because he was remarkably well adjusted to all these changes in the brain.”

If an adult sustained brain damage on the scale of Arun’s abnormalities, they would be reduced to a vegetative state. But the infant mind is amazingly plastic, so Arun’s brain found a way to function with no temporal lobe and only a partial parietal lobe on the right as well as large bubbles of fluid that had stretched and deformed everything on the left side.

Proctor said that Arun’s brain had somehow settled into a delicate but stable balance, and there was a danger surgery could “tip him over the edge.”

But it didn’t.

Mulliken and Proctor took about four hours to peel away the skin from the top of Arun’s head and face, remove a piece of skull between the eyes, cut away herniated brain tissue and use bone from the back of the skull to patch the front before sewing him back together.

As exacting as that may sound, it is comparatively simple as far as brain surgery goes, according to the July 2007 issue of the Journal of Neurosurgery: Pediatrics, which included an article about attempts by NGOs Rose Charities Cambodia and Medecins du Monde to make the procedure affordable in Cambodia.

This particular skull abnormality, called a frontoethmoidal meningoencephalocele, is rare in the West but common in Southeast Asia.

In Cambodia alone, every year approximately 75 babies are born with the problem, which has no clear cause. But while the July journal suggested surgeries could be performed for around $380 per person in Cambodia, it noted that only five neurosurgeons live and practice in Cambodia. And those costs assume there are no cerebral CT scans required, as most patients with the skull abnormality have otherwise normal brains.

But Arun’s case is more complicated. The excess fluid inside the skull went untreated for so long that it settled into stasis, so doctors decided to leave it undisturbed.

The surgery to remove the herniated brain tissue from Arun’s face means that the protruding mass no longer mars his face.

“Obviously in retrospect we’re glad we did it,” Proctor said of the operation.

Ellen Loftus is the youngest of 10 children, and she said growing up around older brothers prepared her for life as a police officer in Daytona Beach, Florida, before returning to Massachusetts to become the first female cop in her hometown of Cheshire. She divorced her husband and became a full-time single mother of two, but that wasn’t challenge enough. She also took on foster children, adopted, and most dramatically began bringing kids from around the world on a temporary basis for medical treatment in the US.

“Just two housewives who raised money by hand,” she said of the Children’s Medical Missions project she undertook with a friend. It took a “lot of begging, lot of schmoozing, lot of praying” to bring approximately 250 children to the US, where they underwent treatment and stayed in licensed foster homes-many in Ellen’s.

The permanently adopted kids living in the house all have nicknames: Munchkin, a five-year-old girl from India; Pie, also 5, an autistic Filipino boy; and Hopper, a 9-year-old Korean girl who was born with a face so dramatically cleft that an entire eye was missing in addition to deformities of the palate and lip. Ellen’s biological sons, Ryan, 21, and Kevin, 17, both still live at home. And then there’s five-year-old Arun.

Everyone who knows Arun describes him as being two things: very charming and even more stubborn. While that single-minded determination may make him a handful, many also credit it with his astonishing progress.

“It’s got to be on Arun’s terms,” physical therapist Jamie Bannon said of her work with him. Speech language pathologist Lisa Budzek-Burr was more blunt.

“He is stubborn,” she said. “That will serve him well in life because he won’t get run over…but it doesn’t bode well for teaching him to read and write.”

She added that Arun is on the cusp of normal development, approximately a year and a half behind, despite learning English as a second language without having properly absorbed Khmer as a first language. He is, perhaps unsurprisingly, good at making his wants and needs known, and can count to ten and identify some written letters.

Chronology and complex details pose more of a challenge, and he often gets lost in the day’s transitions: Failing somewhere in the three-step process of breaking from current activity, recognizing that some new task is at hand and identifying what it is.

But with a child whose speech is mostly limited to simple sentences like “I race around the world” and “You’re a potato-head,” evaluating whether his lack of comprehension is cognitive or behavioral is all but impossible for now.

“As he gets older and is expected to know more, you can see what the deficits are affecting,” Budzek-Burr said. “I think he’s in an ideal situation in preschool. Kindergarten will be a different scenario.”

Soon, Arun will have to sit still and pay attention for extended periods. And the coming years will pose other challenges. By second grade he will need to be much better at getting up and down stairs, for example. But as much hard work as Ellen expects ahead, she knows the groundwork has already been laid.

“When he first got his prosthetic [leg], he did not want his prosthetic. He wanted to be carried,” she said. “Whatever you would do for him, he would not do for himself.”

So Ellen pushed. If Arun wanted something, he had to walk to get it himself. She moved Arun’s toys around so he would have to get them, and even laid trails of candy for him to follow around the house.

“Now life’s a challenge, and he wants to grab it,” she said, adding that Arun displayed some of the same behaviors she had seen in other orphans, borne of a disjointed social life without a family and the routine of life in an institution.

“He was very mistrusting, and a lot of his charm was manipulative,” she said. “But now it’s a more genuine charm.”

Still, despite Arun’s many successes, Ellen knows she has to be prepared for problems to come.

“Is he Einstein bright? No,” she said, adding that years into the future “he may need guidance. He may need supervision. We know that. It’s ok.”

Proctor said Arun remains particularly vulnerable to head injuries, which could cause bleeding into the cerebral spinal fluid that fills approximately a third of his skull. He is at risk for seizures and should see a neurologist at least once a year. And after his face takes a more permanent shape in puberty, he may want more surgery.

And Ellen acknowledges that even aside from Arun’s unique challenges, there are other problems with bringing children from the developing world for medical treatment.

Especially with short-term stays, Ellen sometimes felt she “took children from West Africa and brought them here to show them everything they’ll never have.”

She also wondered whether improving medical care in the developing world might be a more economical and sustainable way to help in the long term.

“We bring a kid here” for thousands of dollars, she said. “How many could we have helped in-country?”

But nonetheless, Ellen believes in helping children who need it, one by one.

“If your country can’t do it, does that mean you don’t deserve it?” she asked. “Arun has a spirit. He needed every chance.”

 

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