UNIVERSITY OF CALIFORNIA, SAN FRANCISCO

spring/summer 2014 V O L U M E 1 1 N U M B E R 2 newsC O N T E N T S

Perspective 2

Exploring Promising New Strategies for Combating Allergies 2

How Experts Use Sleep Medicine Advances to Improve Children’s Health 4

Successful Treatment for Amplified Pain Syndromes 5

More Effective Prenatal Screening, Treatment for Congenital Heart Disease 6

CME Courses 8

New Chest Scan Points the Way to Reducing Radiation

When UCSF radiologist Rebecca Smith-Bindman, MD, recently co-authored an op-ed piece in the New York Times titled “We Are Giving Ourselves Cancer,” it stirred long-standing concerns about radiation exposure from medical imaging. Smith-Bindman believes methods already exist to significantly reduce exposure – especially for children – but those methods are not yet widely used.

“Nearly everyone acknowledges the need to lower radiation doses per CT [computed tomography] scan and, where possible, reduce unnecessary CT scans,” she says. Many specialty children’s hospitals have already implemented procedures to ensure that happens. At UCSF Benioff Children’s Hospital San Francisco, for example, the average dose for an abdominal and pelvic CT scan in a child is around 3.0 millisieverts (mSv) – less than half the national average.

But access to children’s hospitals is limited, so most children are imaged at adult hospitals, where a preference for using higher-dose studies to achieve clearer images often outweighs radiation concerns. Modern equipment and education about radiological advances could change that mind-set for studies where reduced doses don’t compromise diagnostic accuracy.

CT Scan with X-ray DosageRecently, a team from UCSF Benioff Children’s Hospital developed a new CT protocol to image the chest wall for pectus excavatum (a depression of the anterior wall of the chest produced by a sinking in of the sternum). The scan’s radiation dosage is much closer to a chest X-ray than a typical CT scan, and the images – intended to view bones – turned out to be good enough to evaluate the lungs, the most common target for CT scans.

“In the past, a dose this low would get noisy, hard-to-interpret pictures that radiologists would not accept,” says radiologist Andrew Phelps, MD, of UCSF Benioff Children’s Hospital. “But advanced scanners and software

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Perspective

Donna Ferriero, MD Hanmin Lee, MD Allergies affect the lives of 1 in every 13 children under the age of 18 – and that number is rising. For food allergies alone, the prevalence among children increased approximately 50 percent between 1997 and 2011, according to a study released in 2013 by the Centers for Disease Control and Prevention.

Consequently, says allergist and immunologist Morna Dorsey, MD, there is a growing need for programs that quickly translate emerging science into clinical use and facilitate close, clinical collaborations among allergists, pulmonologists, dermatologists, gastroenterologists, psychologists, dietitians, nurses and others who work with children.

Dorsey, who is spearheading a new Allergy Diagnostic and Treatment Center at UCSF Benioff Children’s Hospital, says, “There are exciting approaches emerg-ing, some of which we can take advantage of right away.”

Food AllergiesFor example, an oral food chal-lenge – in which an allergist supervises a patient’s reaction to escalating levels of allergy-inducing food – can safely and more precisely identify a clinically significant food allergy.

One of the greatest advantages of an oral food challenge is that it helps families better under-stand what an allergic child can actually eat. This can be as important as figuring out what a child can’t eat, because it can help ensure proper nutrition and mitigate the emotional impact of telling kids their favorite foods are off-limits.

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One is never more aware of children’s vulnerability than in the presence of

illness. Our charge in pediatric medicine is to constantly seek ways to make

children less vulnerable to disease and to cure them of illnesses and injuries

when they do become vulnerable. This issue of UCSF Benioff Children’s

Hospital News offers five strong examples of how we do this work.

To avoid unnecessarily dangerous exposure to radiation, radiologists here are

successfully adapting computed tomography (CT) protocols without compro-

mising diagnostic accuracy. Allergies, sleep disorders and amplified pain

syndromes are three clinical concerns that are often misunderstood, overlooked

and/or difficult to treat. We are addressing all of these through comprehensive,

evidence-based approaches in dedicated clinics that successfully improve

quality of life for patients and families. And our fetal cardiology program,

a worldwide leader in diagnosing and treating children with congenital heart

disease, has now created a series of programs to help community sonographers

and physicians better screen for these life-threatening conditions.

Which brings us back to the fact that over the long term, we share responsibility

with you for addressing these and many other health concerns. It is reassuring

to know that when these children return to their home communities, they are

in wonderful hands.

Donna Ferriero, MD Hanmin Lee, MDPhysician in Chief Surgeon in Chief UCSF Benioff Children’s Hospital UCSF Benioff Children’s Hospital

Exploring Promising New Strategies for Combating Allergies

Morna Dorsey, MD

“That’s why providing education to families and the community is paramount to reducing the stress of managing food allergy,” says Dorsey. “It’s important to have the practitioner, family and school work together to create a safe, supportive environment.” The UCSF clinic employs a nurse, social worker and dietitian to address this need.

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A Comprehensive Approach to Diagnosis and TreatmentThe center is also modeling comprehensive clinical manage-ment for other complex allergic manifestations in children.

A program treating eosinophilic gastrointestinal disorders brings together allergists with gastro-enterologists, dietitians, therapists and social workers. Parallel specialty combinations are on the horizon for treatment of atopic dermatitis and allergic airways disease and asthma.

“When you get these tough cases, you can’t work in a silo,” says Dorsey.

Clinicians and scientists at UCSF are also collaborating on – and well positioned for – clinical research projects to improve diagnosis and treatment. Among the possibilities:

• Component resolved diagno-sis (CRD): “Food is a complex mix of allergen epitopes, and CRD can improve the accuracy of allergy testing. Right now, patients can test positive for certain foods, but there is often no clinical significance to the finding,” says Dorsey.

• Immunoglobulin E (IgE) profiling can help characterize the range of clinical reactions to foods by looking at each patient’s IgE profile. This can help stratify risk for severe reaction.

• Adjunct oral immunotherapy could go beyond immediate desensitization to produce long-term nonresponsiveness, the ultimate goal of food allergy treatment.

• Nonspecific preventive approaches include the use of probiotics and prebiotics that can influence the microbiome – as well as dietary interventions such as vitamin D.

“Ultimately, allergy is a complex, immune-based disease that will require a variety of approaches to achieve successful treatment,” says Dorsey.

For more information, call the Pediatric Allergy and Asthma Clinic at (415) 353-7337 or go to www.ucsfbenioffchildrens.org/allergy.

Dr. Dorsey works with a young pediatric patient.

“There are exciting approaches emerging, some of which we can take advantage of right away.”

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How Experts Use Sleep Medicine Advances to Improve Children’s Health

Gwynne Church, MD

Jessica Litwin, MD

• Abnormal nighttime behaviors

• Attention-deficit/hyperactivity disorder (ADHD)

• Circadian rhythm abnormalities

• Excessive daytime sleepiness

Refer a child to the Pediatric Sleep Disorders Center with any of the following symptoms:

• Excessive leg kicking during sleep

• Insomnia

• Limb pain and restlessness at bedtime

• Mouth breathing

• Night terrors

A comprehensive look at a child’s sleeping and breathing patterns, snoring, heart rate and oxygen level, a sleep study facilitates an accurate diagnosis and individualized treatment.

At the Pediatric Sleep Disorders Center, two board-certified sleep specialists work with a multi-disciplinary team to diagnose and treat all known sleep disorders for newborns through young adults. The team includes neurologists, pulmon-ologists, otolaryngologists, dentists and registered sleep technologists. The sleep studies take place in a tranquil room at San Francisco’s Hotel Tomo, a unique arrangement that helps create a more sleep-friendly, nonclinical environment.

Tailored treatment plans can include:

• Behavioral modification

• Continuous positive airway pressure (CPAP)

The Difference a Sleep Lab MakesChurch tells of seeing a child with severe scoliosis, restrictive lung disease and a previously undiag-nosed myopathy who was admitted to UCSF Benioff Children’s Hospital with carbon dioxide narcosis. A sleep study revealed severe nocturnal hypoventilation and obstructive sleep apnea.

“Subsequent bi-level titration improved his nighttime ventilation sufficiently that his daytime carbon dioxide levels returned to normal, and he felt dra-matically better,” says Church. “Stories like these demonstrate how expert diagnoses and treatment can improve sleep and change the lives of children and their families.”

For more information, call the Pediatric Sleep Disorders Center at (415) 476-2072 or go to www.ucsfbenioffchildrens.org/sleeplab.

“Sleep is central to how children and families function,” says pediatric pulmonologist and sleep specialist Gwynne Church, MD, director of the Pediatric Sleep Disorders Center.

She says poor sleep patterns and sleep disorders at some point affect between 25 and 40 percent of children – those who are developing normally and those with chronic conditions that include:

• Allergic rhinitis

• Asthma

• Attention-deficit/ hyperactivity disorder (ADHD)

• Cerebral palsy

• Chronic headaches

Yet physicians often overlook or misdiagnose sleep disorders. This is especially disturbing, since prop-erly diagnosing and treating sleep disorders can, among other things, improve school performance, diminish a child’s crippling headaches, improve problems associated with hyperactivity and inat-tention and significantly reduce autistic symptoms.

“Primary care providers, neurologists and pulmon-ologists should screen for sleep disorders regularly,” says pediatric neurologist and sleep specialist Jessica Litwin, MD. “If after setting age-appropriate sleep expectations, a problem persists, refer as early as possible to a pediatric sleep specialist.”

Sleep Labs Provide ExpertiseSleep specialists take an extensive history, conduct a physical exam, discuss a child’s symptoms, review various treatment options and, when necessary, initiate or refer for a sleep study.

• Problems with attention

• Regular snoring

• Sleep deficit interfering with patient and/or family functioning

• Sleepwalking

• Hypertension

• Neurodevelopmental disorders

• Processing and learning disorders

• Obesity

• Seizure disorders

• Medication

• Surgery

Sleep Lab room at Hotel Tomo

Amplified pain syndromes – which include everything from complex regional pain to fibromyalgia – are often misunderstood, says pediatric rheumatologist William Bernal, MD, of UCSF Benioff Children’s Hospital.

Yet, he says, when clinicians diagnose these syndromes correctly, an evidence-based rehabilitation program can restore functionality and, within six months, relieve pain for 90 percent of children.

to send pain signals not just to the brain, but also to the vascular nerves. In turn, blood vessels constrict, leading to lactic acid buildup in a repetitive cycle that continually amps up the pain.

Over time, chronic amplified pain results in altered pain signal processing by the brain. “The brain is no longer able to assess severity accurately – and nothing is ever just a mild ache for these kids,” says Bernal.

• Allodynia (light touch pain)

• Disproportional loss of function (relative to inciting trigger or injury)

• Distractible pain

• Incongruent affect (a child calmly reporting severe pain)

The Rehabilitation ProgramRehabilitation revolves around simultaneously addressing the physical and emotional issues driving these painful patterns. The goals are to restore function, decrease or eliminate pain and give patients the skills to lead functional lives.

“The first step is to break the cycle of inappropriate signals by tailor-ing a program that repeatedly stimulates the nerves through aerobic conditioning and desen-sitization,” says Bernal. In this phase, the primary goal is restor-ing function, with the expectation that pain reduction will follow.

Unlike traditional injury rehabili-tation, the process is not “limited by pain.” Rather, pushing through the pain is how the nervous system resets itself.

But that won’t occur, says Bernal, if patients and therapists don’t also address the emotional aspect of amplified pain. “Chronic pain is stressful, and most of these kids have accompanying anxiety, and CBT is the psychological ap- proach with the most evidence for successfully addressing psycho-logical and emotional factors.”

The combination of exercise and CBT, he says, empowers patients who are tired of an endless regimen of drug taking with little to show for it. “This is a growing population that needs something different.”

For more information, call our 24/7 Access Center at (877) 822-4453 [877-UC-CHILD].

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William Bernal, MD

Successful Treatment for Amplified Pain Syndromes

Dr. Bernal examines a patient with indications of amplified pain.

Providers should refer patients with chronic pain to a specialty rehabilitation program when they cannot determine the cause of the pain or have exhausted their treatment options and are seeing important loss of day-to-day function.

Even at that point, however, Bernal says diagnosis is a chal-lenge, because patients worry they are being told their debili-tating pain is all in their head.

“But this is real pain, just for the wrong reasons,” he says.

He adds that while there are no specific tests for amplified pain, there are classic signals:

“Typically, clinicians address diverse symptoms as they appear, but over time, a pattern of diffuse pain complaints with normal workup emerges,” says Bernal, who recently initiated a rehabilitation program at UCSF Benioff Children’s Hospital.

“What unifies these symptoms is a nervous system gone haywire. We need to reset that system through a tailored exercise pro-gram combined with cognitive behavioral therapy (CBT).”

Diagnosis ChallengeBernal explains that amplified pain is an abnormal short-circuit-ing of the spinal cord causing it

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Anita Moon-Grady, MD

Shabnam Peyvandi, MD

“Congenital heart disease is the number one killer of infants due to birth defect in this country,” says pediatric cardiologist Anita Moon-Grady, MD, who directs the Fetal Cardiovascular Program at UCSF Benioff Children’s Hospital. “But routine prenatal screening misses over half of congenital heart disease cases.”

That’s part of the reason why 2013 guidelines from the Inter-national Society of Ultrasound in Obstetrics and Gynecology require more detailed assess-ment of the fetal heart – including outflow tracts – at every mid- trimester screening.

EducationTo help community sonographers and physicians meet the guide-lines, a UCSF team has created a menu of CME options that touch on fetal cardiac screening, functional assessment of children with other birth defects and other uses of imaging technology. Experts can deliver the classes in a variety of formats.

“Improved prenatal screening gives parents more options and facilitates more appropriate referrals to specialty cardiac centers, which may lead to better short- and long-term outcomes

for these kids,” says pediatric cardiologist Shabnam Peyvandi, MD, who recently arrived at UCSF after a fellowship in fetal cardiology at Children’s Hospital of Philadelphia.

Diagnosis and Treatment in a Specialty CenterBetter outcomes stem from a combination of experience, expertise and collaboration. For example, the UCSF Fetal Cardio- vascular Program, which works closely with the UCSF Fetal Treatment Center and Pediatric Heart Center, offers carefully coordinated care drawn from teams that include:

• Geneticists and genetic counselors

• Maternal-fetal specialists

• Obstetricians

• Pediatric cardiologists

• Pediatric cardiothoracic surgeons

• Social workers

These teams can reliably uncover heart disease using fetal echo-cardiography as early as 13 to 14 weeks into pregnancy. This gives families more time to consider treatment choices and helps health care teams better manage

the pregnancy and reduce the risks of potential procedures.

“In select cases, we can even treat congenital heart problems before birth. When we do, we perform these fetal interventions with a collaborative, multidisci-plinary team, using echocardio- graphy to monitor the heart,” says Peyvandi.

Even if fetal intervention is not indicated, early referral eases the transition to giving birth at UCSF Benioff Children’s Hospital, where mother and child can remain together. During the pregnancy, UCSF staff coordi-nates closely with local resources, which enables most prenatal care to take place closer to home. For postnatal surgery or interventional cardiac catheter-ization, experts from the UCSF Pediatric Heart Center are available 24/7 to perform any necessary procedures.

In addition, research informs the clinical care. Peyvandi, for example, is collaborating with neuroradiologists, neurologists and pediatric intensivist Patrick McQuillen, MD, to better under-stand prenatal brain development in children with heart disease. She hopes the work will lead to fetal interventions to reduce the risk for neurological abnormali-ties common in children with repaired or palliated congenital heart disease.

The Fetal Cardiovascular Pro-gram is also running the only active clinical trial for fetal treat-ment of aortic stenosis, testing a procedure that could prevent progression to hypoplastic left heart syndrome.

For more information, call the Fetal Cardiovascular Program at (415) 353-1887 or go to www.ucsfbenioffchildrens.org/fetalheart.

More Effective Prenatal Screening, Treatment for Congenital Heart Disease

Dr. Moon-Grady conducts a sonogram.

Dr. Peyvandi makes a presentation at a meeting of the Fetal Treatment Center.

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“All scanners can be adjusted to some extent, and we can certainly do things like limiting the area where we scan – for example, omitting the belly when doing a chest scan, or adjusting the strength or amount of dose per unit of tissue,” says Phelps.

To reduce the variability in doses currently used for CT, facilities need to assess their practice, says Smith-Bindman. “We aren’t providing standardized numbers that specify a targeted effective dose for each type of scan and each

patient group. That’s what I’m targeting in my research, as once we have agreed-upon standards – and benchmarks – I am sure all facilities will try to reach them.”

She also acknowledges that lowering doses too far could occa-sionally lead to repeating a scan to obtain necessary diagnostic information, but says, “We have not had that problem at UCSF, and the alternative is to overdose everyone; I’d rather keep most people as low as possible, with only the occasional patient needing to be scanned again.”

For more information, go to www.radiology.ucsf.edu/pediatric.

NEW CHEST SCAN POINTS THE WAY TO REDUCING RADIATION

(CONTINUED FROM COVER)

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Rebecca Smith-Bindman, MD

Andrew Phelps, MD

eliminate nearly all the unwanted noise.” UCSF is now testing whether the protocol’s image quality consistently maintains diagnostic accuracy; if it does, common radiation doses to the chest could be dramatically reduced.

Existing Options UnderusedBut even absent advanced scanners and new protocols, Phelps and Smith-Bindman say there already exist underused ways to reduce radiation.

A CT scan using model-based iterative reconstruction uses roughly the same effective dose (0.08 mSv) as a two-view chest X-ray.

Pediatric CT Doses at UCSF vs. National Averages*

Effective Dose† CTDIvol DLP SSDE

Abdomen 3.0 mSv 8.0 mSv 3.9 mGy 7.6 mGy 151 mGy-cm 294 mGy-cm 4.1 mGy 12.7 mGy and pelvis

Chest 2.5 mSv 3.6 mGy 102 mGy-cm N/A

Head 1.4 mSv 25 mGy 378 mGy-cm N/A 

Sinus 1.6 mSv 33 mGy 290 mGy-cm N/A

CT, computed tomography; CTDIvol, volumetric computed tomography dose index; DLP, dose-length product; mGy, milligray; mGy-cm, milligray-centimeter; mSv, millisievert; SSDE, size-specific dose estimates.

*Average CT dose estimates from 2009 data on patients aged 1 day to 18 years Source: Goske MJ, Strauss KJ, Coombs LP, et al. Diagnostic reference ranges for pediatric abdominal CT. Radiology. 2013;268(1):208-218.

†For purposes of comparison, the average effective dose of radiation for a chest X-ray is 0.02 mSv.

CME Courses For more information, visit www.cme.ucsf.edu

Course Title Dates Location

47th Annual Advances and June 5-7, 2014 The Westin Controversies in Clinical Pediatrics San Francisco

UCSF Eating Disorders Conference June 6, 2014 UCSF Laurel Heights Auditorium San Francisco

Pediatric Hospital Medicine June 19-21, 2014 UCSF Medical Center, Boot Camp 2014 Parnassus Campus, San Francisco

CME Courses

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Children’s Hospital Access Center

For consultation on a difficult diagnosis, to refer a patient to one of our specialty practices, or to admit or transfer a patient, please call (877) 822-4453 [877-UC-CHILD]. Available 24/7, our Access Center staff ensures that you receive fast and efficient access to all of our services, including safe, expedient interfacility transport of your patients to UCSF Benioff Children’s Hospital.

To contact the Access Center, please call (877) 822-4453 [877-UC-CHILD].

Physician Liaison Service

Physician liaisons visit referring physicians and practice repre-sentatives throughout Northern California and Nevada to learn more about their referral needs and to provide information about the services and faculty expertise available at UCSF Benioff Children’s Hospital.

To contact the Physician Liaison Service, please call (800) 444-2559.

UCSF Benioff Children’s Hospital News is published semiannually for referring physicians by the Marketing Department of UCSF Medical Center. It is written by Andrew Schwartz and designed by Robin Awes Everett.

Mark R. Laret, Chief Executive Officer, UCSF Medical Center and UCSF Benioff Children’s Hospital

Kim Scurr, RN, Executive Director

Donna Ferriero, MD, Physician in Chief

Hanmin Lee, MD, Surgeon in Chief

Images: © Don Mason/Blend Images/ Corbis, p. 1; Elisabeth Fall, pp. 3, 5, 6; Miguel Farias, p. 4

To read back issues of this and other physician newsletters, visit www.ucsfhealth.org/newsletters

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30% post-consumer waste fibers.

© 2014 The Regents of the University of California

UCSF Benioff Children’s Hospital at Mission Bay to Open SoonIn 2015, UCSF Benioff Children’s Hospital will move into a brand new, freestanding, 183-bed facility that will advance the health of children in multiple ways for years to come. At Mission Bay, many of the world’s finest clinicians will continue to deploy the most advanced medical technologies for services that include urgent, emergency and specialty outpatient care. Now they will be delivering those services in a higher- capacity, state-of-the-art physical space tailored to the special needs of children. A transformative design enhances healing through connections to nature, a focus on sustainability and a renewed emphasis on patient and family support.

For more information, visit www.missionbayhospitals.ucsf.edu.