Could your child have narcolepsy?

Victoria was 13 when she started experiencing unrelenting sleepiness. This was out of character for her and was confusing because she was getting the amount of sleep most kids her age need. She and her parents attributed the increased need for sleep to adolescence. They became much more concerned when she started falling to the ground unexpectedly.

She was assessed twice in the emergency room for this problem and admitted to the hospital after the second ER visit. The assessment for medical and neurologic causes of passing out (medical term is syncope) was negative, and the problem was attributed to stress.

Victoria was referred to a therapist but no stress was found outside of the stress of dealing with an undiagnosed medical problem. By the time she was referred to a psychiatrist, her “passing out” spells were increasingly associated with emotions such as laughter and joy.

The psychiatrist was suspicious of narcolepsy and referred her to the OHSU Doernbecher Pediatric Sleep Disorders Clinic where she was diagnosed with narcolepsy with cataplexy, which is loss of muscle tone associated with experiencing strong emotions, a phenomenon that occurs almost exclusively in patients with narcolepsy. The diagnosis involved taking a careful history, and overnight and daytime sleep studies.

Narcolepsy is a sleep disorder affecting 1 in 2,000 individuals. The onset typically occurs in adolescence, but there often is a delay in diagnosis. In the past, diagnosis sometimes took as long as 15 years. Fortunately, patients are now being diagnosed much sooner after symptoms appear.

Classic symptoms and signs of narcolepsy include:

  • Excessive daytime sleepiness (falling asleep suddenly often causing embarrassment)
  • Cataplexy
  • Sleep paralysis, or waking in the morning without ability to move major muscle group
  • Experiencing hallucinations as one falls asleep, much like starting to dream before one is asleep

These symptoms are often scary for patients and cause problems at school and with friends and family

We now know that narcolepsy is a neurologic condition involving a relatively small cluster of neurons in the hypothalamus of the brain. These neurons make a neuropeptide, hypocretin, also knows as orexin, which is involved in positively affecting alertness. In narcolepsy, especially the type of narcolepsy involving cataplexy, these neurons stop functioning and hypocretin levels plummet. Scientists hypothesize that these neurons become nonfunctional due to an autoimmune process much like the process responsible for multiple sclerosis.

Fortunately, once recognized, narcolepsy can be effectively treated although not cured. Treatment includes education about the condition, scheduled naps and medications. Ongoing management by a sleep physician is indicated.

Since being accurately diagnosed, Victoria has taken charge of her illness, finishing high school, competing in cross-country running, and now succeeding in college.

Victoria shared her story with KGW-TV a couple of years ago.

Kyle Johnson, M.D.
Director, OHSU Doernbecher Pediatric Sleep Disorders Clinic
Associate Professor of Pediatrics, Division of Child and Adolescent Psychiatry
OHSU Doernbecher Children’s Hospital

A note of thanks from 8-year-old former NICU patient Elle

Hi my name is Elle. I’m 8 years old and in the third grade. I was born in Silverton, Oregon, but was quickly taken to Doernbecher where I spent the first two weeks of my life in the Neonatal Intensive Care Unit.

Since then I have spent many days at the hospital with 10 different surgeries and lots of doctor’s visits. I am happy that Doernbecher is my hospital because they do everything they can do make a difficult situation better.

My favorite things are weekly Bingo and the prize cart, the ice cream, and lots of visits from fun people … and sometimes pets! My team of doctors is great. They help me grow stronger and make my life better.

Dr. Myall is my favorite doctor. He has been with me since I was a baby and I was sad that he retired last year.

Because so many people have donated toys, movies and their time to make my hospital time better, I wanted to give back. I chose to have a lemonade stand and donate the money to Doernbecher.

Lots of people stopped by and shared their own special stories about their experiences with the hospital.

I am happy we raised $150 for Doernbecher.

When I grow up I want to be a veterinarian. I would like to take care of animals just like Doernbecher has taken care of me.

My time at the hospital is unfortunately not over with at least 12 more surgeries to go. But I am happy that I have the very best people taking care of me.

Thank you to all the wonderful doctors and nurses that have helped me!

Love,
Elle

 

Vancouver’s Trey Foote Foundation raises $12K for OHSU Doernbecher, Knight Cancer Challenge

Dr. Charles Keller; Lea Foote; Jim Foote; Olivia; Olivia’s dad, Todd

The International Air and Hospitality Academy recently sponsored the second annual Trey Foote Foundation fundraising dinner to support OHSU Doernbecher Children’s Hospital — specifically Dr. Charles Keller’s Lab.

The event was a huge success with more than 100 people in attendance. Dr. Keller spoke about the advances his team and his fellow researchers from OHSU Doernbecher and the Knight Cancer Institute are making, but the true star of the night was Olivia.

Olivia is a 15-year-old young lady who is currently battling leukemia. She spoke to the audience about her disease, what her life was like before cancer, some of the challenges she faces as her fight continues, but more importantly, she shared the hopes and dreams she has once her cancer is cured.

Trey Foote Foundation Board

“It was a real reminder why we are all here tonight and how important it is to support Olivia, support Dr. Keller, and more importantly, do our part to make the Phil and Penny Knight challenge a reality,” said Lea Foote. “What a rare opportunity we have to play such active role in fighting cancer.”

The event raised $12,000, and 100 percent will be donated to Dr. Keller’s lab and will put us $12,000 closer to fulfilling Phil and Penny Knight’s $500 million Knight Cancer Challenge.

Together we will raise $1 billion for OHSU to fight cancer.

Jim Foote
Father of Trey Foote and President of the Trey Foote Foundation
Vice President and Chief Business Security Officer
ADP Dealer Services

 

New heart defibrillator is game-changer for tweens, teens with heart arrhythmia

This week the pediatric cardiovascular team at OHSU Doernbecher Children’s Hospital, in cooperation with the OHSU Knight Cardiovascular Institute, made history by implanting a first-of-its kind heart defibrillator that is less invasive and poses fewer risks to patient than current heart defibrillators.

Kenneth Clark, 17, is the first pediatric patient in Oregon to receive the implant.

Clark recently was diagnosed with hypertrophic cardiomyopathy. Additional testing suggested he was at risk for lethal fast heart rhythms and a defibrillator was recommended. Boston Scientific had recently released a new defibrillator called the S-ICD, and Clark was a perfect candidate. His procedure was uneventful and smooth.

The main advantage of the S-ICD (subcutaneous implantable cardiac defibrillator) is there are no wires inside the heart. All components of the defibrillator are located under the skin on the chest and in the armpit. This minimizes the risk of potential complications associated with conventional defibrillators, including heart perforation, wire dislodgment, heart infections, blocked veins, heart blood clots, and the later possibility of having the wires extracted, which can be risky.

Due to size of the defibrillator, this technology is not yet appropriate for very small children. But for teens and some pre-teens, it promises to be a very useful advancement. It is only a matter of time before this technology is applicable to even smaller children.

View an animation of the procedure:

YouTube Preview Image.

Seshadri Balaji, M.D.
Professor of Pediatric Cardiology
OHSU Doernbecher Children’s Hospital

 

Q: Are the steroids in my daughter’s asthma inhaler safe?

A: If your daughter has chronic asthma, it means that the airways that carry air to and from her lungs are inflamed, so that inflammation needs to be reduced to help her breathe.

Inhaled corticosteroids help ease inflammation in the lungs. The steroid in an inhaler is set at an extremely low dose and is not absorbed in any significant amount.

Even if your daughter is feeling better, being consistent with medication for childhood asthma is important: She still needs to continue using the inhaler to prevent the inflammation in her lungs from returning.

If you have more questions about dosage and long-term use of corticosteroids, talk to your child’s doctor.

Alexandra Cornell, M.D.
Assistant Professor of Pediatric Pulmonology
OHSU Doernbecher Children’s Hospital

Resilience

In my work as a pediatric brain and spine surgeon at OHSU Doernbecher Children’s Hospital and OHSU Hospital, I get to meet some pretty amazing children. Alex Shepherd most certainly counts among them.

Alex came to Doernbecher after a seizure led to diagnosis of a brain tumor. Despite the presence of an 11 cm long ‘C’-shaped mass in the depths of Alex’s brain, he remained a charismatic and enthusiastic 10-year-old. Except for the moments of his seizures, he also remained tremendously precocious and bright, with an amazing ability to tell stories and engage with the doctors and nurses around him.

In fact, Alex seemed to be helping his parents and all of us cope with the challenges of his illness ahead.

At Doernbecher, children come from as far away as the distant corners of Oregon and even Idaho and Alaska for care of difficult to treat brain and spinal cord tumors. Even so, Alex’s very complex tumor posed a serious challenge to all members of our multi-specialty team. To help him, we needed to remove the entire tumor despite its extent and depth within the brain, as well as deliver additional therapies.

In May 2013, Alex’s therapy began with a series of four brain operations to completely remove different components of his tumor from the front and back regions of the brain.

Recovering from the fourth operation, Alex, we immediately realized, was still Alex, with his sense of humor, intelligence, and engaging personality intact.

He moved on to other parts of Doernbecher Children’s Hospital and clinics to carry on the rest of his therapies. It has been a joy for me to follow his progress through occasional visits back to my clinic in neurological surgery.

Upon emerging from months of intensive surgical and medical care, Alex also made a big impression on a number of other people, including television personality and host, Ellen Degeneres and her huge national audience.

On Feb. 3, 2014, Alex and his parents appeared on Ellen and told some of his story to a rapt audience. America could see what we have seen for months: the tremendous spirit and resilience of a terrific child facing a difficult disease with energy and courage, and maintaining everything of himself that his parents and his community celebrate.

If that were not enough, Ellen and her wonderful team provided a huge boost to the Shepherds. Recognizing the burden of constant long drives from southern Oregon for Alex’s treatments at Doernbecher, and the impact of missed work for his parents who are dedicated to his care, Ellen surprised the Shepherds with a new car and $10,000 cash to help cover expenses!

There was not a dry eye in the television studio or at Doernbecher, where doctors and nurses crowded around screens to watch the touching interview.

Alex and his family, and countless patients and families like the Shepherds, remind us of why we are blessed to do the work we do. Thanks Alex.

Alex’s interview with Ellen:A New Car for an Inspiring Family.”

Nathan Selden, M.D., Ph.D.
Mario and Edie Campagna Chair of Pediatric Neurosurgery
Director, OHSU Neurological Surgery Residency Program
OHSU Doernbecher Children’s Hospital

 

Defining the best practices

Doernbecher Children’s Hospital has the largest program in Oregon for the repair of skull birth defects called cranial synostosis. In synostosis, one or more of the skull growth plates is missing, which can cause a severely misshapen head, pressure on the brain, developmental delay and even blindness.

Surgery to repair synostosis requires an accredited specialty team comprising a pediatric neurosurgeon and a pediatric craniofacial plastic surgeon.

At Doernbecher, I am privileged to do this work with one of the best craniofacial plastic surgeons in the country, Dr. Anna Kuang.

We typically perform more than 40 major reconstructive operations for skull deformity in infants each year. Unfortunately, even after surgical repair of a skull deformity as a baby, there is a risk in the first few years of life that the problem will recur.

At Doernbecher, Dr. Kuang and I were anxious to predict which children might suffer this rare complication. To tackle this problem, we analyzed the results of surgery on more than 150 children undergoing treatment at Doernbecher.

From this study, Dr. Kuang and I learned two important things. First, thankfully, recurrence rates at Doernbecher are among the lowest reported. Second, and even more importantly, we identified a small subset of patients at more risk for recurrence and a strategy for timing their surgery to improve the chances of a permanent result from the first operation.

Along the way, we identified some findings on routine post-surgical follow up testing that could confuse surgical teams into over-diagnosing recurrence and undertaking unneeded operations.

Dr. Kuang and I reported these important findings during the past year in two major surgical journals: Plastic and Reconstructive Surgery and the Journal of Neurosurgery. We each also travelled to national surgical meetings to present our results immediately to other program leaders from around the country (and the world).

By taking these additional steps, we can help make sure that all children benefit from the research and quality improvement efforts taking place at Doernbecher and OHSU. Our mission is not only the best care for any child that comes to us from Oregon and the region, but also for every child no matter where their care is given.

Nathan Selden, M.D., Ph.D.
Mario and Edie Campagna Chair of Pediatric Neurosurgery
Director, OHSU Neurological Surgery Residency Program
OHSU Doernbecher Children’s Hospital

What parents should know about childhood palpitations, abnormal heartbeats

Unpleasant sensations of fast or forceful heartbeats are called palpitations. Young children with palpitations usually tell their parents that their heart is “beeping” or “going crazy” or words to that effect. Older children typically are able to describe the feeling more effectively. What is a parent to do?

The most important thing is not to panic unless the child passes out, which is extremely rare. Should this happen, call 911 right away and consider doing CPR.

Abnormal heartbeats in children are fairly common and almost never life-threatening. If possible, put your finger to their pulse in the elbow or the wrist and try to count the pulse rate or put your hand on their chest and try to feel their heart. If the heart rate is abnormally fast due to an abnormal heart rhythm, also known as an arrhythmia, it will be over 180 beats per minute and this is too fast to count. If this happens, take your child to the nearest emergency room.

If the heartbeat is not that high and/or the racing heartbeats are happening in short bursts, it is important to see your pediatrician or family physician, who will do an ECG/EKG, or electrical recording of the heart. If that recording is normal, your physician may recommend a heart monitor, usually a heart event recorder and ask the child to try and “capture” one of the fast heartbeats.

Once the rapid heart rate has been documented by ECG, a heart specialist can determine whether it is an abnormal heart rhythm. Palpitations are almost always due to a heart arrhythmia or an anxiety or panic attack. The heart event recorder can help establish which one it is. Rare glandular problems like thyroid abnormalities form the rest, and can be excluded by a simple blood test.

In these days of busy families and over-worked kids, stress-related anxiety attacks are not uncommon in kids. If the recorder suggests that the palpitations are not due to an arrhythmia, but likely due to stress, your doctor will recommend methods to address them that may involve seeing a psychologist or a counselor to get at the cause of the stress.

It is important to realize that the anxiety attacks are not “brought on” by the child; they happen unconsciously and are disabling and scary to the child. It is important to be sympathetic and help the child through his/her issues. Sometimes families see a diagnosis of anxiety or panic attacks as a stigma and insist on more heart tests because, in their opinion, there is no way their child could be stressed. Unfortunately doing more tests is usually unnecessary, expensive and, more importantly, can make the child feel that there is something seriously wrong, which only adds to his/her stress.

Arrhythmias, on the other hand, are managed with daily medications or a day procedure known as a heart electrophysiology, which tests electrical-function, and ablation, which cauterizes or destroys the spot in the heart causing the abnormal heart beats.

Whether your child has an arrhythmia or stress-related palipitations, consulting your primary care physician right away and relying on the partnership between him/her and the heart specialist is the right way to go.

Seshadri Balaji, M.D.
Professor of Pediatric Cardiology
OHSU Doernbecher Children’s Hospital

Q: I think my child has the flu. Should we go to the Emergency Room?

A: You should first contact your pediatrician, but if your child has an extremely high fever — more than 104 degrees — or is acting unusual, for example very sleepy or irritable, you should consider seeking immediate medical attention.

Winter is a common time for infectious illnesses such as pneumonia, bronchiolitis, or lung infections, or the flu; ensuring your child gets a flu shot can prevent or reduce the severity of influenza.

Flu shots are especially important for children with chronic illnesses, such as asthma.

If your child does need emergency care, OHSU Doernbecher Children’s Hospital has an emergency room just for kids 24/7 and staffed by specially trained emergency medicine doctors and nurses who treat only children.

David M. Spiro, M.D., M.P.H.
Medical Director, Pediatric Emergency Medicine
OHSU Doernbecher Pediatric Emergency Department

Doernbecher Fanconi Anemia specialist invites you to walk/run for a cure

As pediatricians we care for children with a wide spectrum of illness and injury. Some of them are life-threatening, some more mundane, most heal. Children grow, parents are grateful and pediatricians thrive on the validation both provide. Only sometimes the news is that things will not get better, will last a long time and children will not grow to be their unencumbered, exuberant and irreverent selves.

Fanconi Anemia, or FA, is a rare, 1 in 180,000, illness that spells a long chronic course of office visits for the children and sleepless nights for the parents. The list of complications is long and the medical needs are daunting. It is easy to get lost in the complexity and heartbreaking to watch when lives are cut short after long suffering.

When Peg Padden’s son was diagnosed with Fanconi Anemia and subsequently passed away, she was not content to sit by. Determined to make a difference and help find cures so others would not have to go through the same agony, she organized the Valentine Fanconi Anemia Run/Walk 5K/8K/12K.

Through the years, she has found many friends and supporters that brave the inevitable drizzle and occasional cold that are the hallmarks of a February morning in Portland — more than 1000, last year. Many participants learn more about the disease as they meet other runners. Some turn out to be patients, some parents, others are doctors, nurses, medics, FA researchers, students or running enthusiasts. Altogether:  COMMUNITY.

What motivates Peg to organize the run and us to join? A commitment to the patients and a strong conviction that research will eventually offer a cure and help translate biological insight to tangible benefit — bench to bedside.

OHSU has been a center of excellence for FA research for many years and researchers here have made landmark discoveries that provide a deeper understanding of the disease. OHSU Doernbecher also serves as a home for the comprehensive care that FA patients with their medically challenging situation require.

Over the past nine years, the Valentine Fanconi Anemia Run has motivated 6,500 of us to get up early on a Sunday in February, lace up and join Peg. Along the way she has raised $350,000 for Fanconi Anemia research. Pretty amazing, really!

Come join us, Sunday, Feb. 9, 2014, at 8 a.m., and support the patients and the research to find cures.

Peter Kurre, M.D.
Associate Professor of Pediatric Hematology/Oncology
OHSU Doernbecher Children’s Hospital

Doernbecher Children's Hospital

Doernbecher Children’s Hospital

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