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
"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
||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
"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
"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
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
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
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."