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Ask Stanford Med, Pediatrics, Surgery

Surgery to find your voice: A Q&A with a pediatric otolaryngologist

Surgery to find your voice: A Q&A with a pediatric otolaryngologist

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When we’re in a noisy restaurant, it’s really difficult to hear my young niece speak. She can only talk very quietly, because she has a paralyzed vocal cord.

Like many children born very premature, the nerve going to her vocal cord was likely damaged when she had heart surgery soon after she was born. Her inability to be heard frustrates her, especially now that she is in school. However, a rare surgery may bring her the hope of a near-normal voice.

Stanford surgeons recently began performing laryngeal reinnervation surgery, which essentially rewires the paralyzed vocal cord with a new nerve supply. I recently spoke with Anna Messner, MD, a professor of otolaryngology and pediatrics who sees patients at Lucile Packard Children’s Hospital Stanford, about laryngeal reinnervation surgery.

What standard surgical procedures are used to treat unilateral vocal cord paralysis?

In general, the surgical procedures bulk up the paralyzed vocal cord to move it towards the midline of the body, making it easier for the other vocal cord to compensate and close. There are two standard surgeries. We can do injection laryngoplasty, where we inject a substance into the paralyzed vocal cord to thicken it. Unfortunately, this procedure often needs to be repeated multiple times, if it works at all. We can also insert a medialization implant in teenagers and adults, but this doesn’t work for growing kids. If we put an implant into a 2-year-old, it wouldn’t be an appropriate size when he is 10.

How does laryngeal reinnervation surgery work?

No matter what we do, we can’t make the vocal cord move. We can never make it perfect again. What we can do is hook up one of the other nerves in the neck to the recurrent laryngeal nerve that goes to the vocal cord. And that helps some new nerve fibers go to the vocal cord, making the vocal cord stronger and thicker. As a result, the voices on these kids improve significantly.

The surgery itself is fairly straightforward and only takes about an hour. The children typical go home the same day or just stay overnight, and they feel back to normal in a couple of days. But then we have to wait five to six months for the nerve fibers to grow before we can see real improvement in the voice. The only downside is that it takes a long time to see the effects of the surgery.

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Cardiovascular Medicine, Pediatrics, Transplants

Unusual bridge-to-transplant method helps teen get new heart and lungs

Unusual bridge-to-transplant method helps teen get new heart and lungs

bridge-to-transplant device
Earlier this year, Oswaldo Jimenez’s heart and lungs were failing. He needed a combined heart-lung transplant, but his doctors at Lucile Packard Children’s Hospital Stanford were worried that the 14-year-old from Salem, Oregon might not survive the wait for donor organs.

Stanford physicians have lots of experience with using external and implanted pumps that can support a patient’s failing heart. A few years ago, for instance, an 8-year-old patient spent 229 days with a Berlin Heart pump that moved blood through her body while she awaited a heart transplant.

Oswaldo’s case was different. Although his heart failure was significant, his failing lungs posed the biggest risk to his health. His doctors were concerned that his poor lung function would immobilize him – yet to benefit from transplanted lungs, he needed to stay fairly fit and mobile while he waited.

So the doctors decided to try an unusual bridge-to-transplant procedure called a “pulmonary to left atrial shunt,” which connected Oswaldo’s heart to a portable box outside his body that oxygenated his blood. Essentially, the team gave Oswaldo a temporary, artificial lung.

A press release from the hospital explains how it worked:

The procedure involved the insertion of a tube that redirected blood away from Oswaldo’s lungs into the oxygenator. This, in turn, provided oxygen to the blood and then returned it to his body, with his own heart providing the pump. Reports on this shunt device being able to sustain patients’ lives range from several weeks to six months, depending mostly on being able to prevent the blood from clotting while avoiding complications such as bleeding or stroke.

On July 12, Oswaldo made history by becoming the first child in the western United States to undergo this treatment — it saved his life and bought him time. Then, just one week after receiving the shunt, donor organs became available. Oswaldo received his heart and lung transplant on July 19.

Oswaldo is still recovering at the Ronald McDonald House, and his doctors think he’ll be able to go home close to the New Year. He’s looking forward to being a kid again, and his grateful family is thinking about how his case might benefit other kids in similar situations. “Now the doctors can use this therapy to treat other patients,” said Oswaldo’s mom, Carmen Hernandez.

Previously: Stem cell medicine for hearts? Yes please, says one amazing family, “Liberated from LVAD support”: One patient’s story and Image of the Week: First heart-lung transplant
Image of pulmonary to left atrial shunt courtesy of Lucile Packard Children’s Hospital Stanford

Behavioral Science, Mental Health, Pediatrics, Research, Stanford News

Stanford ingenuity + big data = new insight into the ADHD brain

Stanford ingenuity + big data = new insight into the ADHD brain

ask-the-brainAttention-focusing brain networks interact more weakly than usual in kids with attention deficit hyperactivity disorder, new Stanford research shows.

The research, published online today in Biological Psychiatry, is part of an ongoing effort to figure out how the brain differs from normal in people with ADHD. The disorder is both serious and common: It’s characterized by impulsiveness, hyperactivity and difficulty paying attention, and it has been diagnosed in more than 6 million U.S. children.

The new study focused on a particular set of linked brain regions called the salience network. From our press release:

“A lot of things may be happening in one’s environment, but only some grab our attention,” said Vinod Menon, PhD, a professor of psychiatry and behavioral sciences and the study’s senior author. “The salience network helps us stop daydreaming or thinking about something that happened yesterday so we can focus on the task at hand. We found that this network’s ability to regulate interactions with other brain systems is weaker in kids with ADHD.”

The research could lead to better diagnostics for ADHD, Menon said. That’s a big deal because, right now, diagnosis is based on subjective assessment of a child’s behaviors, and the threshold of behavior considered sufficient for diagnosis varies quite widely. Doctors worry about the risks of diagnosing ADHD in kids who don’t have it, or who actually suffer from a different psychiatric problem, and also about missing children who really should get a diagnosis.

But prior efforts to find an ADHD biomarker have been hampered by weak science. Many papers reporting brain-scan features of ADHD have not withstood attempts to replicate their findings.

The new study is different: Not only did Menon’s team find that their analysis could distinguish ADHD patients from controls with brain scans, it did so in three independent data sets. The data, from an open-source database of fMRI scans called the ADHD-200 Consortium, was collected using a different MRI scanner and slightly different clinical assessments at each site.

“We could use biomarkers developed from one site — New York — to classify ADHD children in another site, Beijing,” Menon said. The biomarkers also worked for the data from the third study site, which was Portland, Oregon. The fact that the findings held for all three sites gives an important level of real-world assurance that they’re meaningful.

More research is still needed to investigate whether brain scans can distinguish children with ADHD from those with other psychiatric conditions. But Menon thinks the work is on track to making a practical difference for better ADHD diagnostics.

Previously: A visual deluge may provide clues to ADHD treatment, Scientists reveal link between dopamine receptor subtype and ADHD diagnosis and Study finds many teachers, doctors mistaking immaturity for ADHD
Photo by Thomas Hawk

Genetics, Pain, Pediatrics, Precision health, Stanford News

Newly identified gene mutation explains why one family experiences unusual pain response to cold

Newly identified gene mutation explains why one family experiences unusual pain response to cold

snowy-handprintIf you’ve ever plunged your hand into a tub of ice water, you know about the overlap between cold and pain: that deep, biting ache makes you want to get your hand out of the water – fast. But while the protective value of that sensation is obvious, scientists have always been a bit mystified by how pain-sensing nerves register cold temperatures.

But now, research on a family with an extremely unusual gene mutation may help clarify what’s going on. The mutation, whose discovery was reported online this week in Nature, confers a heightened pain response to cold. The research was initiated by Stanford geneticists and expanded by scientists at three universities in Germany who specialize in hereditary pain syndromes.

The story began with a family who brought their young daughter to Lucile Packard Children’s Hospital Stanford to get help for her unusual episodes of pain. When cold, she experiences pain in her joints that radiates out to her arms and legs. The pain lasts 20 to 30 minutes at a time. The little girl’s father, paternal grandmother, paternal aunt and first cousin (the aunt’s daughter) also experience similar pain episodes, as the new paper explains in detail.

“When we saw her, we were really struck by the fact that the pain was going on in multiple generations of the family,” said one of the study’s authors, medical geneticist Jon Bernstein, MD, PhD. The pattern of inheritance made Bernstein suspect an autosomal dominant disease, in which only one bad copy of a gene causes symptoms. Although several hereditary pain syndromes are described in the medical literature, none matched the exact pattern of symptoms this family experienced, so the Stanford clinicians asked the German scientists to figure out what was going on.

The German team looked for rare mutations shared by the little girl and her cousin, finding one in a gene that codes for an electrical channel in nerve cell membranes. (Nerves transmit electrical signals via flow of charged ions through tiny protein tubes embedded in the cell membrane. There are several types of these channels.) The scientists’ experiments demonstrated that they had discovered a gain-of-function mutation – in which the encoded protein, instead of being rendered nonfunctional, instead alters what it does. In this case, there is a substitution of one amino acid for another in the structure of the affected electrical channel. That change causes pain-sensing nerves to fire at cool temperatures most people don’t find painful.

The same electrical channel, Nav1.9, was also identified as “a key determinant of cold pain sensation” in a paper published earlier this year that examined its activity in rats and mice. That study found that the channel was important to setting animals’ threshold for when cold begins to feel painful, and the new findings fit into that picture nicely.

The German team plans to continue studying the channel’s dynamics to help learn more about the normal threshold between cold and pain, Bernstein said. As for him? “I’m very much looking forward to working with the next family whose case is unsolved,” he told me.

Previously: One mutation, two people and two (or more) outcomes: What gives?, Crying without tears unlocks the mystery of a new genetic disease and Exploring the mystery of pain
Photo by Chris Geatch

Infectious Disease, Patient Care, Pediatrics, Public Health

Should doctors give up their white coats?

Should doctors give up their white coats?

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When you google ‘doctor,’ virtually every image shows a person in a long-sleeved white coat. The crisp white coat with a stethoscope around the neck has long symbolized the profession. However, there is controversy about whether doctors should give up their classic uniform, as described in a recent story in the Boston Globe.

Britain’s National Health Service banned white coats several years ago, requiring doctors to be bare below the elbows to avoid spreading infections. Many clinical departments in the United States have done the same. The argument goes something like this:

  • The sleeves of white coats are germ magnets.
  • Doctors don’t launder their white coats very often, so deadly infections can be spread from one patient to another.
  • Therefore, doctors shouldn’t wear long-sleeved white coats.

As a pediatric infectious disease specialist at Stanford, Charles Prober, MD, supports this theory. He told me during a recent interview: “In terms of the infectious disease risk, there is little question that one can carry bacteria or viruses on your clothing — whether it’s a white coat or the sleeve of your shirt, and one way to lessen that is to wash up to the elbows, especially when you’re going into high-risk environments like the ICU or nursery. Obviously you can only wash up to your elbows if they aren’t covered with something.

William Benitz, MD, division chief of neonatal and developmental medicine, agrees:

I find the summary reports highly credible and accept the contention that the long sleeves of white coats harbor infectious agents and carry them from patient to patient. We banned white coats in our NICU about 5 years ago, along with a mandate for baring arms to the elbow and hand cleansing upon entering any patient room. Part of the reason for the former is to reinforce and provide active visual evidence of the latter. We used to hear ‘but I won’t touch anything’ a lot, but that was often not so. Not an issue now.

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Cancer, Pediatrics, Research, Stanford News

A cure is not enough for young cancer survivors

flower-887443_1920I survived Hodgkin’s lymphoma as a young adult about twenty years ago, thanks to the chemotherapy and radiotherapy that I received at Stanford Hospital as part of a clinical trial.

Even back then, the focus of the research was on fine-tuning my cancer treatment to maintain an excellent likelihood of survival, while minimizing the long-term health problems due to therapy. I knew Hodgkin’s was unlikely to kill me, so I had to worry instead about future health issues caused by my radiation and chemotherapy.

People that survive cancer at a young age are expected to live many decades after diagnosis and treatment, so they are the most vulnerable population to long-term damaging effects from cancer therapy. Stanford’s Karen Effinger, MD, MS, and Michael Link, MD, explore this issue in an editorial published today in JAMA Oncology.

The editorial explains that it is critical to directly study the late effects in young adult cancer survivors, rather than the common practice of extrapolating from studies of children and middle-aged adults.

In particular, they discuss a new study by Katherine Rugbjerg, PhD, and Jorgen Olsen, MD, DMSc, from the Danish Cancer Society Research Center, which used the national Danish registries to compare the long-term risk of hospitalization in almost 34,000 5-year survivors of adolescent and young adult cancers with that of more than 228,000 age- and sex-matched population controls. Reported in the latest issue of JAMA Oncology, Rugbjerg and Olsen found that adolescent and young adult cancer survivors had significant health issues due to their treatment; however, these treatment effects were different than survivors of childhood cancers.

The editorial also discusses the late effects of pediatric cancer treatment on survivors’ neurocognitive development, which impacts education, employment and quality of life. Effinger and Link specifically describe a new study reported in JAMA Oncology by Kevin Krull, PhD, and colleagues from the St. Jude Children’s Research Hospital, which compared the neurocognitive outcomes in 80 adult 25-year survivors of a pediatric cancer with 39 controls. Krull concluded that the risk of neurocognitive impairment from cancer treatment was related to the development of chronic health conditions — rather than directly from exposure to high-doses of chemotherapy, as expected — but longitudinal studies are needed to sort out possible modifying factors.

The editorial authors conclude:

Going forward, we must apply our knowledge of late effects to improve monitoring and interventions for patients. While the progress made in the management of cancer in children and young adults has been gratifying, we must remember the words of Giulio D’Angio, who reminds us that “cure is not enough.”

Jennifer Huber, PhD, is a science writer with extensive technical communications experience as an academic research scientist, freelance science journalist, and writing instructor.

Previously: Study highlights childhood cancer survivors’ increased risk of future health problems, Questioning whether physicians are equipped to care for childhood cancer survivors and A cancer survivor discusses the importance of considering fertility preservation prior to treatment
Photo by klimkin

Education, Pediatrics, Stanford Medicine Unplugged

Empathy and the darker side of pediatrics

Empathy and the darker side of pediatrics

Stanford Medicine Unplugged (formerly SMS Unplugged) is a forum for students to chronicle their experiences in medical school. The student-penned entries appear on Scope once a week during the academic year; the entire blog series can be found in the Stanford Medicine Unplugged category.

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I’m currently just over halfway through my pediatrics rotation, having recently finished up four weeks on inpatient peds and just started my month of outpatient peds.

Before this rotation, when I thought about pediatric medicine, I thought about diagnoses like asthma, croup, foreign body ingestions, and rashes. I never really thought about child abuse, or – as the medical terminology goes – “non-accidental trauma.” And yet, I saw all too much of it this past month on the wards.

Our 6 a.m. sign out one morning went something like, “Patient X, here for NAT, steadily improving, currently in CPS (Child Protective Services) custody, awaiting foster family placement.” When we met this young patient later that same morning, I found a strong mix of emotions stirring inside me: first disbelief, then overwhelming sadness, then – rapidly – anger.

How could ANY parent do this to their baby? This question repeated itself in my mind, over and over and over. I literally could not wrap my head around it. Here is this beautiful, helpless little human being – what could possibly make someone do harm to this child?

I was distressed and distracted throughout rounds that morning, until finally – unable to stay quiet – I confided my thoughts to one of the other members on the medical team, who said: “I know it feels hard to understand. But, take just a moment, and think about it from the parents’ perspective: They are no longer allowed to visit their own child. And they have to explain to literally everyone they know – friends, family, colleagues, other children in the home – why they no longer have their baby.”

Her words stunned me into momentary silence. Never had I thought to empathize with the parents of our NAT patient.

Somehow, I had taken that 2-minute sound bite uttered during morning sign-out and transformed it into a mental battlefield, with the health-care team on one side, fighting valiantly to protect this child, and the parents on the other, a medical story of “good” vs. “evil.” But my fellow team member was right: This situation was awful all around. These parents no longer had a child, the child no longer had parents, and sometimes – if the patient had siblings – the siblings too were taken into CPS custody.

This experience taught me how absolutely vital it is to find ways to empathize with every patient and every family, no matter what the circumstances. 

I feel like this is particularly relevant in the inpatient setting, where we see patients for days – which sometimes become weeks and months – at a time. We connect with our patients: They’re often the first people we see when we get into the hospital and the last ones we see when we leave. We feel like we know all about them, about their families, about their values. But we don’t.

We’re witnessing this small window of their lives that has brought them to the hospital. And as easy as it is sometimes to ask question and judge – particularly in the setting of something as sensitive as child abuse – it’s not our place to do so.

Hamsika Chandrasekar is a third-year student at Stanford’s medical school. She has an interest in medical education and pediatrics. 

Photo by 3rdparty

Ethics, In the News, Parenting, Patient Care, Pediatrics, Stanford News

Parents now help doctors decide what care is right for the sickest babies

Parents now help doctors decide what care is right for the sickest babies

Today, NPR’s Morning Edition featured an in-depth story on the evolution of decision-making in neonatal intensive care units – hospital nurseries for the sickest infants. Parents now have much more say in their babies’ care than in the past, and Stanford experts who were on the front lines of the change, including William Benitz, MD, chief of neonatology at Lucile Packard Children’s Hospital Stanford, explained how it happened.

As medical care for premature and other at-risk babies advanced in the 1970s and early 1980s, doctors gained the ability to save many infants who would once have died soon after birth. But some children in the new category of survivors had lifelong disabilities, with lasting implications for them and their families.

At first, doctors did not realize that this change would affect parents’ desire to participate in planning medical decisions for fragile infants:

“It never occurred to anyone that that might be a reasonable conversation to have,” Benitz says. “We were in unexplored territory.”

As technology improved and doctors tried to save sicker babies, and some born even earlier in gestation, there were new decisions to make: Should the health team put the tiny child on a ventilator? Attempt heart surgery? Those interventions helped many infants survive. Others did not fare as well.

“A lot of them ended up with significant impairments,” Benitz recalls. And doctors started to get pushback. “In the mid-80s we began to hear from families that maybe that wasn’t consistent with their goals for their children.”

As a result, neonatologists began having in-depth conversations with parents about the possible outcomes of different treatments for their infants. The practice is now widespread, and it means a lot to parents like Karin and Chris Belluomini, whose daughter, Joy, was born in May 2015 with Down syndrome, several heart defects and fluid around her lungs.

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Cancer, In the News, Pediatrics, Stanford News

“Earth angel” brings smiles to sick kids in hospital

"Earth angel" brings smiles to sick kids in hospital

6552156879_1cd906fbab_zHere’s a tale that will tug on your heart strings: It begins in a small town in Pennsylvania, in 1926, at the birth of Joe Manfrey, the fifth child in a family that would eventually grow to nine.

Like many his age, Manfrey served in World War II; his ship was part of the battle of Iwo Jima.

But unlike many of his peers, Manfrey, now 89, is still volunteering twice a week in the cancer unit in the Lucile Packard Children’s Hospital Stanford where he spreads good cheer, hope and sock monkeys. Manfrey was featured in a recent story in the San Jose Mercury News:

“Joe embodies the great values of the Greatest Generation,” said Leslie Griffith, a nurse in the Bass Center for Childhood Cancer and Blood Diseases. “He is funny, engaging and soothing to parents, who can be crying or overwhelmed by a surreal sadness. But just when they feel lost, someone kind and upbeat like Joe arrives — an Earth angel, a beautiful spirit.”

But there’s a twist to Manfrey’s story.

His own son, now 47, survived acute lymphoblastic leukemia as a child, at a time when its survival rates were much lower than they are now. He shows patients a photo of his son as a bald, 5-year-old, and then one as a healthy adult.

“By showing them the pictures together, it lets them know that I personally know what they are going through. It also gives them hope because they can see that Rob grew up to be healthy and strong,” Manfrey said in the article.

“This is not about me,” he emphasized in the story. “I do this for the kids and the parents, in order to take their minds off the difficulties they are going through. I try to make them feel a little more comfortable.”

Previously: California collaboration focuses on analyzing pediatric cancers, A look at the dramatic improvement in pediatric cancer survival rates and Children’s hospital volunteers snuggle infants to soothe tiny patients and reassure their parents
Photo by Clyde Robinson

Chronic Disease, Patient Care, Pediatrics, Stanford News

Helping kids with chronic medical conditions make the jump to adult care

Helping kids with chronic medical conditions make the jump to adult care

With just one dramatic example from her practice, Stanford pediatric critical care specialist Yana Vaks, MD, recently illustrated for me the importance of better adult health care for children who survive a catastrophic childhood illness or endure an incurable medical diagnosis.

“There was an 18-year-old who came to the hospital in crisis,” she said. “He had a liver transplant when he was 8, but when he turned 18 he wanted to start a new life and decided he was done with all that extra health consciousness his transplant meant.” The patient had stopped taking the drugs necessary to keep his body from rejecting the transplant and neglected to see his doctor regularly. By the time Vaks saw him, his transplanted liver had begun to fail, starting a catastrophic process that affects all body systems. “It was a shocking case,” she said. The teenager died the next day.

His mother told Vaks that the biggest challenge had been the 18th birthday, that legal coming of age where parents can no longer control what medications their children take.

As I did the reporting for a Stanford Medicine story called “When I Grow Up,” I was shocked to learn just how many young adults fall into the categories of survivor or chronically ill: They may soon represent 10 percent of the U.S. population ages 15 to 25. Before advances in treatment began saving so many lives, that population was just 1 percent.

The specialists who treat these growing children have long recognized the challenges related to this patient population: Young adults may be grown in body, but they aren’t always ready psychologically or socially to take full responsibility for consistently following complicated medical routines and practicing lifestyle restrictions. Nor are most adult care doctors trained in the after-effects of childhood cancer, for instance, or the lifelong need to monitor adults with childhood heart repairs.

What’s needed is something called transition care — but no one had studied just what that should look like. The Clinical Excellence Research Center, established in 2010 to study, design and demonstrate ways to improve health care while reducing costs, identified transition care as a good candidate for the changes it hopes to effect with its work. For two years, CERC gathered information, reviewed research, interviewed patients and families and visited hospitals around the country, and it has launched pilot programs – including one at Stanford Children’s Health – to test its recommendations:

The CERC team’s recommendations emphasize that pediatricians and pediatric specialty teams must be guides in this process: equipping patients and parents with information so they can anticipate the transition, coaching patients to develop the confidence and skills needed to manage their health, and locating and being available to specialists and primary care physicians who will need certain medical knowledge to care for their patients as adults.

Previously: Stanford Medicine magazine tells why a healthy childhood mattersStudy highlights childhood cancer survivors’ increased risk of future health problemsQuestioning whether physicians are equipped to care for childhood cancer survivors and Chronic illness in childhood: One patient’s story
Illustration by Daniel Horowitz

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