boston childrens hospital


Cautionary Tale: Vomiting Bouts And False Positive On Urine Test For Pot

Hollis Tufts, the teen who tested positive -- incorrectly -- for cannabis use in a cautionary tale published in the journal Pediatrics (Courtesy of the family)

Hollis Tufts, the teen who tested positive — incorrectly — for cannabis use in a cautionary tale published in the journal Pediatrics (Courtesy of the family)

The title got me: “13-Year-Old Girl With Recurrent, Episodic, Persistent Vomiting: Out of the Pot and Into the Fire.”

What pot? What fire? Oh, dear, recurrent vomiting. What troubling case report was this, in this week’s Pediatrics journal?

The case, written up by Dr. Diana Felton of Boston Children’s Hospital and colleagues, was indeed troubling, but also instructive, on two counts:

First, its main lesson: a medication for gastrointestinal problems — a proton pump inhibitor called pantoprazole — can cause a false positive result on a urine test for marijuana use.

And second, though marijuana is generally known to have anti-nausea effects, it has been increasingly recognized over the last decade that heavy, long-term use can bring on cycles of vomiting, a phenomenon called Cannabinoid Hyperemesis Syndrome. (It can also bring on compulsive bathing in hot water. I know. Sounds crazy. But I’m not making it up. More on that later.)

She remembers the thought, “What on earth are they saying? This is crazy! All we’ve ever done is care for our child…”

Dr. Felton and colleagues write that as the use of organic and synthetic cannabinoids — pot-like compounds — increases, “the number of patients with Cannabinoid Hyperemesis Syndrome will surely grow.”

Now to their tale. The patient was a 13-year-old Massachusetts girl who suffered from recurrent bouts of vomiting, a condition known as Cyclic Vomiting Syndrome. She was on her fourth bout of vomiting in six weeks when she was brought in to the Emergency Department, retching.

Hit by a stroke while still in the womb, the girl could not speak, and had been hospitalized repeatedly for such vomiting attacks. This time, among other tests, “the treating physician opted to send a urine toxicology screen to evaluate for possible Cannabinoid Hyperemesis Syndrome.” It came back positive for cannabinoids. Social services got involved. A protective order was filed.

“Given the patient’s severe physical and developmental limitations, it was clear that she was unable to access or administer the cannabinoids herself,” the paper says.

Let’s just pause for a moment to put ourselves in the place of the patient’s parents. I imagine myself exploding: “So you’re accusing us of giving our disabled daughter so much pot that it made her throw up? Are you out of your mind???”

Not too far off, says Jessica Tufts of Topsfield, whose daughter, Hollis, now almost 15, was the patient in the paper. She remembers the thought, “What on earth are they saying? This is crazy! All we’ve ever done is care for our child…”

“They bring me into a room and they say, ‘We just wanted to let you know that Hollis has tested positive for cannabinoids,” she recalls. “I said, ‘How on earth could she be getting it? We don’t smoke it. We don’t cook it. We never even touched it. So how is she getting it?’ I flew into a panic because she can’t say, ‘Somebody at school is feeding me pot brownies or whatever.’ We were trying to figure out all the points of contact that could possibly explain it.”

And, “We got progressively more terrified, because, as the mom of a child who’s going to be limited all her life and can’t tell you what’s going on, your worst nightmare is that some caregiver who is out of your control has done something to her.” Continue reading

How 3D-Printing Helped A Little Girl With A Rare Facial Defect

Check out this excellent story about a little girl named Violet born with a rare defect, a Tessier facial cleft, that left a fissure in her skull, and how 3D-printing is helping doctors take on these kinds of complicated surgeries. The piece is in today’s The New York Times and written by health reporter and CommonHealth contributor Karen Weintraub, who offers a little background:

Violet Pietrok was born nearly two years ago without a nose. Her eyes were set so far apart that her mom compared her vision to a bird of prey’s. There was a gap in the skull behind her forehead.

There was no question she would need drastic surgery to lead a normal life. But few surgeons have seen patients with problems as complex as Violet’s. Her parents, Alicia Taylor and Matt Pietrok, who live near Salem, Oregon, brought her to Boston Children’s Hospital, to Dr. John Meara, who had operated before on kids with Tessier facial clefts.

As part of Children’s Pediatric Simulator Program, Meara was able to get several 3D printed models made of Violet’s skull. By handling and slicing up the models, he got a better sense of what had gone wrong and how best to fix it.

Such 3D-printing is becoming more commonplace in complex surgeries, allowing doctors views and knowledge they can’t get on their screens.

Continue reading

If You Build A Crew Program For Overweight Kids, They Will Row — And Get Fitter

OWL On The Water participants bring a shell out of the Community Rowing boathouse in Brighton, and to a dock on the Charles River. (Jesse Costa/WBUR)Alexus Burkett, left, helps carry the shell toward the water. (Jesse Costa/WBUR)(Jesse Costa/WBUR)(Jesse Costa/WBUR)(Jesse Costa/WBUR)Coach Sandra Cardillo instructs the kids before they go out onto the water. (Jesse Costa/WBUR)(Jesse Costa/WBUR)Rowing coach Kate Simeon instructs the kids out on the Charles. (Jesse Costa/WBUR)An OWL On The Water boat moves past the Community Rowing facility as daylight fades. (Jesse Costa/WBUR)

There was no comfortable place for 17-year-old Alexus Burkett in her school’s typical sports program of soccer and lacrosse and basketball.

“They don’t let heavyset girls in,” she says.

Alexus was “bullied so bad about her weight,” says her mother, Angelica Dyer, “and there was no gym that would take her when she was 14, 15 years old. There was no outlet.”

But Alexus has found a sports home that is helping her bloom as an athlete: an innovative program called “OWL On The Water” that offers rowing on the Charles River specifically for kids with weight issues.

She has lost more than 50 pounds over half a year, but more importantly, says her mother, “They’ve given me my daughter’s smile back.”

Alexus Dwyer during warm-ups before instruction time. (Jesse Costa/WBUR)

Alexus Burkett stretches during warm-ups before “OWL On The Water” instruction time. (Jesse Costa/WBUR)

“It’s given me a lot of good strength and it’s making me more outgoing,” Alexus says. “We’re all best friends and we’re all suffering with the same problem — weight loss — so we’re more inspiring each other than we are competing against each other.”

OWL On The Water offers a small solution to a major national problem: According to the latest numbers, 23 million American kids are overweight or obese, and only about one quarter of 12-to-15-year-olds get the recommended one hour a day of moderate to vigorous physical activity. Heavier kids are even less likely to be active, and only about one-fifth of obese teens get the exercise they need, the CDC finds.

“I know I need to be active, but please don’t make me play school sports!” That’s what exercise physiologist Sarah Picard often hears from her young clients at the OWL — Optimal Weight for Life — program at Boston Children’s Hospital that sponsors OWL On The Water.

Many gym classes still involve picking teams, “and my patients are the ones that are always picked last,” she says. “You’re the biggest one, you’re the last one, you’re picked last, and you’re uncomfortable.”

They are strong, powerful people.

– Sarah Picard

School fitness testing is important, Picard says, but it, too, can be an ordeal: “I have kids who sit in my office and tell me that they didn’t go to school for a week because they wanted to miss the fitness testing,” she says.

While many a coach might see bigger bodies as poorly suited to typical team sports, Picard sees them as having different strengths. Particularly muscular strength.

“What I’ve observed is that these kids are much better at strength and power-based activities,” she says. And rowing is particularly good for them, she says, because though it is strenuous, it is not weight-bearing, and thus more comfortable for heavier bodies — yet a heavier, strong body can pull an oar much harder than a smaller person’s body. The program begins by building on that muscular strength, she says, and then works on aerobic fitness. Continue reading

Lessons From Boston Children’s: When Hackers Attack Your Hospital

Boston Children's Hospital (Wikimedia Commons)

Boston Children’s Hospital (Wikimedia Commons)

Last April, during the parents-versus-hospital custody dispute over teenager Justina Pelletier, Boston Children’s Hospital found itself under cyberattack, apparently by the hacker group Anonymous. The hospital’s website was flooded by traffic that hindered its operation, and other online operations were affected as well.

The assault brought widespread condemnation — BetaBoston called the attackers not activists but “criminals” — and subsided after about a week.

Now, Dr. Daniel Nigrin of Children’s offers some details and lessons from the assault in the latest New England Journal of Medicine: When Hacktivists Target Your Hospital.

He writes that the attack began with a warning message on Twitter relaying a set of demands, and then the hackers posted “the home and work addresses, phone numbers, and e-mail addresses of some of the people involved in the case (a tactic called ‘doxing’). The hackers also posted technical information about the hospital’s public-facing website, suggesting that it might become a target.” A few weeks later, the “distributed denial of service” — the flood of traffic — attack began.

Nigrin writes:

Over the course of the next week, the hospital was subjected to several other attacks that were intended to do more than affect its Internet connectivity. These included multiple attempts to penetrate its network through direct attacks on exposed ports and services, as well as through the use of “spear phishing” e-mails, which are intended to get recipients to click embedded links or open attachments that would provide a means for the attackers to gain access to the portion of the hospital’s network behind its firewall.

No patient data were damaged or exposed, Nigrin writes, but the experience underscores the important of planning for the possibility of losing Internet connectivity. Continue reading

Complex Cases Of ‘Parent-Ectomy,’ From New Yorker To Boston Globe

(An EPA photo, 1973, via Wikimedia Commons.)

(An EPA photo, 1973, via Wikimedia Commons.)

Is this a trend? A pendulum swinging? First came the heartbreaking story in the Dec. 2 New Yorker magazine of a single mother who left her three-year-old son alone in his crib one day to go to work, and lost custody of him — never to regain it again, no matter how she tried. It’s headlined ‘Where Is Your Mother?‘  I read every word with mounting horror as it became ever clearer that in the wake of her one mistake — admittedly a very bad one — and her inability to persuade the legal system to give him back to her, Niveen Ismail would lose her beloved son forever.

Now, the Boston Globe is running an investigation into cases of “medical child abuse” — in which parents are accused of hurting their children through medical interventions and can lose custody if they are ruled a threat. The Globe’s story focuses on a dispute between Boston Children’s Hospital staffers and a West Hartford couple, Linda and Lou Pelletier, whose 15-year-old daughter, Justina, has been hospitalized at Children’s for months. I read every word of this one, too, admiring the reporters for wading into a very contentious tale, and imagining the pain of parents deprived of contact with a very sick child. Then I got a shock at the end of today’s story: The case is still live, its ending unclear, a decision expected soon: From today’s story, Frustration On All Fronts In Struggle Over Child’s Future:

In the bitter cold of last Thursday morning, on the final day of the trial to determine Linda and Lou Pelletier’s fitness as parents, Juvenile Judge Johnston prepared to hear testimony from the witness at the center of it all. Justina was wheeled into the fourth-floor courtroom of the Edward Brooke Courthouse in Boston. Continue reading

Knees, Food, Periods: Top 10 Medical Tips If Your Daughter Plays Sports

(AP Photo/Gerald Herbert)

(AP Photo/Gerald Herbert)

You could call this “Title IX Medicine.”

Title IX, of course, refers to the landmark 1972 anti-discrimination law that gave huge added impetus to school sports programs for girls, helping create cohorts of more athletic grrrrrrrls.

In Title IX’s 40-plus years, American girls’ participation in high-school and college sports has jumped more than 10-fold to well over 3 million. That means many stronger, healthier girls — but it also means more girls at risk for sports-related injuries and what’s known as the Female Athletic Triad, a worrisome mix of poor nutrition, menstrual dysfunction and danger to bone health.

This week, Boston Children’s Hospital announced the creation of its new “Female Athlete Program,” aimed at treating “the entire female athlete – not just a single injury.”

“We know that the build of girls — both their musculature and bone structure — is different than boys’, as is their hormonal milieu,” said the program’s co-director, Dr. Kathryn Ackerman. “We really need to start tailoring our care of these athletes in a slightly different way.”

Certain specific issues need extra attention among girl athletes, she said. They’re at a five to eight times higher risk of anterior cruciate ligament knee injuries. “Aesthetic” activities like ballet tend to be linked with higher risks of eating disorders. If menstrual cycles become abnormal, bone development could suffer.

Some articles suggest that girls’ soccer is second only to men’s football in terms of concussions.

The new program aims to contrast with the traditional piecemeal approach to girls’ injuries and other health issues, Dr. Ackerman said. For example, “A girl comes in having sustained multiple stress fractures, and no one has asked her about her menstrual status or her calcium or Vitamin D intake or her overall caloric intake.” Some studies, she said, suggest that up to 60 percent of girl athletes have at least one component of the Female Athlete Triad: eating dysfunction, loss of menstrual cycle or low bone density.

Dr. Ackerman, herself a former national team rower, and the program’s co-director, Dr. Martha Murray, an orthopedic surgeon with a swimming background, kindly generated this list of their top 10 tips for parents of girl athletes. Dr. Ackerman expands in the comments below.

Dr. Kathryn Ackerman (Courtesy BCH)

Dr. Kathryn Ackerman (Courtesy BCH)

1. Your daughter can minimize her risk of ACL (anterior cruciate ligament) injury with a simple training program.

It would include hamstring strengthening, landing bio-mechanics, core stability and overall muscular balance. More details in the program’s ACL handout.

2. She needs to be getting good nutrition to play well, especially enough calories and the right amount of calcium and vitamin D.

Calorie counts depend on a girl’s level of activity and growth, but she should be getting 1,300 milligrams of calcium a day until she’s 19, then 1,000 milligrams a day until menopause, when calcium again needs an increase. Vitamin D recommendations vary, but many bone experts recommend at least 800 international units a day for a blood level of at least 30. More details on nutrition here. Continue reading

Mass. Health Care Shoppers Still Choosing ‘Nieman Marcus Hospitals’

Nieman Marcus in San Francisco (sjsharktank/Flickr)

Nieman Marcus in San Francisco (sjsharktank/Flickr)

If you buy all your clothes at Nieman Marcus, rather than at Banana Republic, TJ Maxx or Target, you’re spending a lot of money. Are the shirts, jeans or navy blue blazers that much better for four times the cost?

We almost never ask ourselves these questions in health care. We go to the most expensive hospitals in Boston for everything from an X-ray to a complex cancer treatment.

That habit means “the biggest hospitals have the highest price and get all of the payments,” said Aron Boros, director of the Center for Health Information and Analysis (CHIA). The result: We spend more money than we need to on routine care with no apparent benefit. The white shirt (say, a gall bladder removal) is of the same or better quality at Land’s End (your community hospital) as at Bloomingdale’s (a big Boston teaching hospital).

Boros just released the latest figures on the gap between hospitals that get paid very well in Massachusetts and those that are (barely) scraping by.

“This is more evidence that the market isn’t changing as rapidly as one would hope,” he said.

More evidence because this is the second report to show that four out of five health care dollars in Massachusetts go to half the hospitals, the most expensive ones. Continue reading

An Immune Element In Autism? Gene Screen Finds More Evidence

DNA double helix (National Human Genome Research Institute)

DNA double helix (National Human Genome Research Institute)

A better early blood test for autism.” That’s how the headlines tended to read in last week’s coverage of new findings by Harvard-led researchers published in the journal PLOS ONE.

But there was another aspect of the new research worthy of at least a grabby sub-headline: the analysis of gene activity in children with autism found a strikingly strong role played by genes connected to immune function.

‘The main point is: This is not a monolithic disease.’

That robust immune element surprised the work’s senior investigator, Dr. Isaac Kohane of Harvard Medical School and Boston Children’s Hospital. And it may have practical implications, says Dr. Martha Herbert, a Harvard neurologist and author of “The Autism Revolution.” The study looked at genes getting switched off and on, and “diet and environment could contribute to at least some of these genetic changes,” she said.

So “anti-inflammatory approaches to diet modification — such as a high nutrient plant- based diet, solidly adequate Vitamin D levels and plenty of antioxidants and essential fatty acids — could help to downregulate the more troublesome of the gene expression changes. Since these are low-risk interventions, they could be pursued even while we wait for this kind of blood test for autism to become more accurate.”

Dr. Kohane, a bio-informatics specialist, was a relative newcomer to autism research when he began the gene expression work, and any preconceptions stemmed from the dominant thinking in the field: that autism was all about synapses, the connections between neurons. He went into the gene analysis unbiased, he says, open to whatever signals the genes themselves would produce. As he tells it:

Remember, gene expression is not DNA, it’s genes getting switched on and off — it’s RNA. You’re looking at real-time physiology: genetic potential plus environmental influences. What I was expecting to see was either nothing, or a lot of synaptic genes. So I was really pleased when I looked at the gene expression signature — the sets of genes switched on and off in these children we studied — and sure enough, there was a good group of 55 genes that predicted with some accuracy who had autism and who did not.

We saw a group of genes that were characteristic of the synapse, so that was very reassuring, that in the blood you could see something developing in the brain. But then I saw there were a number of other genes that were involved in inflammation.

I wondered whether I was seeing this because I was looking at white blood cells. But why would it be that I saw it in one group of kids, the ones with autism, and not in the control group? And so then I started looking through the literature and there was this whole other parallel set of literature about the immune gene signatures in autism. Continue reading

First Treatment Found For Rapid-Aging Disease In Children


Researchers have found the first treatment for progeria, a rare “rapid aging” disease in children. (Courtesy of the Progeria Research Foundation)

I first heard of progeria in “When Bad Things Happen To Good People,” by Harold Kushner. The author, a Natick rabbi, lost his 14-year-old son to the disease, a rare genetic defect that causes accelerated aging and effectively turns children into little old people, afflicted by strokes and heart attacks. They die young, of old age.

So my first reaction to today’s big news about the first promising treatment for progeria was: “Now at least the bad things that happen to some good people may not be quite so bad.”

A paper just published in the Proceedings of The National Academy of Sciences reports on a clinical trial for the first known treatment for progeria, and the findings are highly promising. The drug used, Lonafarnib, originally aimed at fighting cancer, appeared to help with weight, bone structure, and most importantly, artery health in 28 children with progeria.

That the drug appeared not only to slow but to reverse some aspects of damage to the children’s blood vessels “is a tremendous breakthrough, because cardiovascular disease is the ultimate cause of death in children with progeria,” said Dr. Leslie Gordon in the press release. She is the lead author of the study, medical director for the Progeria Research Foundation, and the mother of a child with progeria. (She’s also affiliated with Boston Children’s Hospital, Harvard, Brown and Hasbro Children’s Hospital.)

I challenged her on “breakthrough” — I tend to be so cautious with that word that I’m downright allergic to it. Her justification:

“This is a 100% fatal pediatric disease and we had no idea whether it could be influenced in any way by any drug treatment.’

“I do think it can be called a breakthrough, and the reason is that prior to this study finding, we had no idea whether we could offer anything for progeria at all. This is a 100% fatal pediatric disease and we had no idea whether it could be influenced in any way by any drug treatment.

So to me, the breakthrough here is that we have findings that show that progeria can be altered. Not only in the rate of weight gain but that the vasculature can be influenced and the bone can be influenced. That’s a real breakthrough that gives us tremendous hope that by finding more treatments and more ways to get at the disease process in progeria — with the protein called progerin — that we can actually have an impact on the disease.”

The clinical trial was only 2-1/2 years long, she noted, so it is not yet known whether the drug’s benefits will translate into longer lives. But “the breakthrough element is really, ‘Oh my gosh, for the first time, we know the disease can be influenced.'”

I also spoke today with Dr. Mark Kieran, senior author of the progeria study and director of pediatric medical neuro-oncology at Boston Children’s Hospital and Dana Farber Cancer Institute. Our conversation, lightly edited:

So it sounds like this is one of those times when science works just as it’s supposed to: Researchers found the gene, figured out what it did, and then corrected it, at least partially?? Continue reading

Boston Children’s Hospital Cuts 255 Jobs, Lays Off 45 Staffers

WBUR’s Martha Bebinger

WBUR’s Martha Bebinger reports that top management at Boston Children’s Hospital sent word to employees today that the hospital was cutting a total of 255 positions, most through attrition but 45 through elimination. Savings total $89.5 million, for a 3% margin.

From the letter:

“During FY12, 255 positions were eliminated from the hospital’s operating budget through consolidation of positions and operations, transfers and not filling vacancies. This process affected employees at all levels of the organization. Decisions were made thoughtfully and carefully, with an eye toward ensuring we did not affect our ability to deliver the best and safest patient care and with a minimum of impact on employees.

Do the budget pressures necessitate a reduction in force?

From our employee base of 9,600, approximately 45 employees’ jobs are being eliminated and those employees are not able to be transferred to other positions. These eliminations include management and non-management positions. Human Resources is supporting those employees by providing a competitive severance package, continuation of health and dental benefits for the severance period, and Employee Assistance Program counseling.”