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Vol. LVII, No. 24
December 2, 2005

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Tanzanian 'Children of the Moon' Bring Rare Brand Of Sunshine to NIH

On the front page...

Dr. Kenneth Kraemer still seems a little amazed at what some NIH staff and a few notable others were able to pull off this past summer. As the NCI scientist sits at his desk — his computer monitor displaying a thematic backdrop of sub-Saharan Africa's zebras and shorebirds — he recounts the compelling tale of how two young boys blinded with xeroderma pigmentosum, or XP, traveled from their rural village in Tanzania to NIH to receive medical testing, treatment, and, for one of them, life-saving surgery.


"It takes a village," he says with a smile — a reference to the African proverb that teaches that raising a child often requires the time, effort and resources of an entire community.

  Contributing to the successful treatment of Ally's cancer were NIDCD research nurse practitioner Susan Rudy, Dr. Carter VanWaes (c) and Dr. Brian Driscoll.

He isn't kidding. In one month, the re-nowned XP researcher, together with Deborah Schmidt-Tamura, a research nurse at NCI, had solicited the assistance of dermatologists, a social worker, a pediatrician, an ophthalmologist, a genetic counselor, a dentist, an oral surgeon, an ear, nose, and throat nurse practitioner, two head and neck surgeons, translators, ethics consultants, medical photographers, and umpteen students, nursing support staff and Children's Inn staffers. In all, six NIH institutes and centers took part in the monumental effort, including NCI, CC, NHGRI, NEI, NIDCR and NIDCD. And that's just the NIH side of things — that doesn't include the unrelenting energy and optimism brought to the table by a college student from Maine named Emerald Russell.

Russell, an affable woman with a bottomless drive to make a difference in the world, had met the boys, Ally Sufian, age 9, and Emmanuel Tenga, age 11, the previous fall when volunteering at a school for blind children in Tanzania. While making plans to bring the boys to the United States to attend a special camp for kids with XP, she contacted Kraemer to see if there was a way the boys could receive some much-needed medical attention during their stay. (Ally, in particular, had a worrisome non-healing lesion on his lip, despite previous attempts to remove it.) Kraemer, who is conducting clinical studies on XP patients, was interested in examining the boys since they would be the first Africans he's encountered with the disease.

XP is a rare inherited disease that affects people of all races and causes a person to be extremely sensitive to harmful UV radiation such as sunlight. Sun exposure damages DNA, which, under normal circumstances, is repaired by special cellular machinery. Individuals with XP, however, aren't able to repair their DNA. For this reason, they are 1,000 times more at risk for skin cancer than people who don't have XP, and they are also susceptible to cancer of the eyes, tip of the tongue and lips. In addition, XP patients can experience nerve damage, leading to such problems as neurological disorders and hearing loss. During daylight hours, people with XP should wear protective clothing and sunglasses if they go outside and cover windows when indoors. As a rule, however, they are strongly advised to stay indoors during the day and to save their outdoor- time for the night, so they are sometimes referred to as "children of the moon."

It Takes a Village — Plus Faxes, Emails, Cell Phones.

Bringing two young Tanzanian boys to the States to participate in a clinical study isn't a task for the shy or easily discouraged. While Russell was busily raising cash for Ally and Emmanuel's airfare, Kraemer was grappling with another issue: getting the green light from the boys' parents — who work as subsistence farmers and speak only Swahili — for the boys to participate in the study.

  Emmanuel Tenga (seated, l) and Ally Sufian (in sunglasses) pose during their visit to NIH. With them are (front, from l) medical students Salma Faghri and Jared Jagdeo, teacher and guardian Dastan Anthony. At rear are (from l) a local translator, Emerald Russell and Dr. Kenneth Kraemer.

"One of our biggest concerns was informed consent. We wanted everything to be done the same way we do things here — but in Swahili," he said with a smile. Schmidt-Tamura found a staff person at the NIH Library who would arrange for translation of the necessary forms and then asked the school principal, Damas Kimoso, to deliver the forms to the parents for signatures.

"We felt it was important to let the parents know that we weren't going to cure the disease," Kraemer notes. "We didn't want to raise their expectations too high." In addition, the parents had to formally designate a teacher at the boys' school — a young man named Dastan Anthony — to serve as guardian for Ally and Emmanuel while they were visiting NIH.

There were other hurdles too. While Russell was working to obtain passports and visas for Anthony and the boys — an undertaking that required Kimoso to stand in long lines for a week in a town 10 hours away — Kraemer was trying to locate a medical facility that could help in completing the necessary paperwork as well as provide health care for the boys when they returned home. Conducting an online search for medical centers near the boys' school in Moshi, he came upon the Regional Dermatology Training Center, an internationally funded medical center located at the foot of Mount Kilimanjaro. His concerns further dissipated when he noticed a photo taken at the center's doorstep in 1992. There, smiling with members of the board of the Internation-al Foundation for Dermatology, was Dr. Stephen Katz, director of NIAMS, who played a role in the facility's creation.

"I'd seen Steve earlier that day at lunch and now, there he was — front and center!" Kraemer laughed.

Ally and Emmanuel's American Adventure

When Ally and Emmanuel finally arrived at NIH on July 11 — their sun-ravaged eyes glued closed — they were warmly welcomed by the staff of the Children's Inn, their home away from home for the next 7 days, along with other medical staff and translators. The plan was for the boys to visit NIH for one week of evaluation and diagnostic tests and then to attend camp in New York for the second week. While the evenings were filled with unbridled joy — they ate all the food they wanted and played with as many toys as they could lay their hands on — the days were consumed with a flurry of medical tests. Nevertheless, except for the needles, even the doctors' pokes and prods possessed some element of intrigue for the boys.

"These are bigwigs," said Russell, describing the NIH doctors and nurses who attended to them during their stay. "I mean, you type in 'XP' on the computer, and up pop their names. These kids were sitting on Dr. Kraemer's lap! They come from a country where there's one doctor for every 50,000 people, but here were all these people in white coats joking with them — spending time on our boys."

Among the battery of tests performed on Ally and Emmanuel were examinations for cancer of the eyes and tongue, hearing loss and vitamin deficiencies. Although the three former tests turned out negative, both boys were found to be chronically malnourished. Vitamin supplements and a more healthful diet helped bring their nutritional levels back to normal and sunglasses and eyedrops helped clear up their acute eye problems.

A Life-Saving Procedure

Ally's non-healing lip lesion was of concern to everyone due to a recurrent cancer growing there. (In Tanzania, doctors had tried removing the cancerous tissue twice before. To keep the infection down, they put salt in the wound, which left Ally in constant pain.)

"The cancer could have been fatal if it had metastasized," said Kraemer.

After a CAT scan revealed that the cancer had not spread, Dr. Jaime Brahim, an oral surgeon with NIDCR, performed a biopsy on the boy's lip and found that it indeed tested positive for cancer, a finding that was confirmed by Dr. Carter VanWaes, a head and neck surgeon with NIDCD. Because cancers involving the head and neck can have a major impact on voice, speech and swallowing, NIDCD's Head and Neck Surgery Branch is often consulted for such cancers at the Clinical Center.

A confluence of factors — most notably, having the right team of specialists available at NIH and having identified a facility that could provide a continuity of care in Tanzania — led the research team to decide to operate on the lesion. The following week, while Ally and Emmanuel were away at camp, the team drew up a second informed-consent form — this one filled with diagrams and descriptions of the surgery — for Ally's mother to sign. Schmidt-Tamura then made the call to Russell, asking her to bring the boys back to NIH when camp was over.

"The operation involved the removal of a wedge of lip down to the chin, which required some plastic surgery," explained VanWaes. Dr. Brian Driscoll, an NIDCD head and neck surgeon with training in facial plastic surgery, did the procedure.

"The surgery went very well," remarked Kraemer. "One concern was that Ally wouldn't be able to open his mouth following the surgery since the skin surrounding the mouth gets tight from the disease. When the surgery was over, Ally's mouth opened just fine and he experienced no post-op pain at all, which was remarkable."

A Joyous Homecoming

"I must say I cannot believe that Ally is now free from cancer!" wrote Kimoso in an exuberant emai l to Russell relaying the school-wide celebration that took place on the boys' return.

"You cannot believe that we celebrated the whole night to see our kids back home healthy and safe. In fact, all the kids, their parents and the teachers were jumping here and there, hugging, singing all the songs they ever knew on seeing Dastan, Ally and Emm. The small kids.were beating drums, cans, sticks and whatever came in their hands as if they had some sort of madness. But it was all happiness. Even the evening wind that was blowing softly made the branches of trees swang [sic] here and there and you would probably think that even the trees were dancing a beautiful African Ngoma."

Kraemer, though not quite as effusive as Kimoso, was no less delighted with the outcome: "I'm just very pleased to know that this old government institution has a warm heart," he reflected. "Practically everyone we approached bent over backwards to help these boys."

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