Cub Chat | Winter 2006

Spring 2006

Taming Tantrums........2

Help for Eating Problems.........4

Baby Walker Warning........6

Volume 12, Number 1

Meet Keith and Steven of Newville, Pa.

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eith and Steven Martin, ages 5 and 3, are two contented, joyful little boys. It’s a sunny Tuesday morning when their father, Loran, brings them to Penn State Children’s Hospital for their regular checkups at the Stem Cell Outpatient Clinic on the seventh floor.

To watch them play in the children’s playroom, just across a toy-filled lobby, you’d never know that both of them suffered from the same life-threatening genetic disease. Not so long ago, they had a delicate procedure performed to save their lives. The brothers from Newville, Pa., were born with WiskottAldrich syndrome (WAS), an immunodeficiency disorder.

Normally, the immune system uses white blood cells and antibodies to attack potentially harmful substances.

Immunodeficiency means that the immune system fails to protect the body.

The disorder causes increased infection, more severe reactions, and delayed recovery from sickness. Those suffering from immunodeficiency are also more prone to developing cancer.

Diagnosis The road from diagnosis to recovery has been a rough one for Loran and his wife, Selesti. When the Martins’ second child and oldest son Keith was 4 weeks old, Selesti noticed a large bruise on his body as she was changing him.

Steven and Keith Martin play in the Children’s Hospital playroom.

Concerned, the Martins took him to their family physician. They were told to take Keith to the local emergency department if he didn’t improve. The bruise faded, but other sicknesses such as continual ear infections, asthma, skin rashes, pneumonia, and bloody diarrhea plagued Keith. At 8 months, while Keith was in the hospital to have tubes placed in his ears, blood work revealed the source of this young baby’s ailments: WAS. Keith’s doctors immediately referred the Martins to Penn State Children’s Hospital. His doctors prescribed a stem cell transplant.

What is stem cell transplantation?

In addition to immunodeficiency disorders, stem cell transplants are used to treat patients with cancer, leukemia, aplastic anemia, and disorders of hemoglobin production. “The sources of stem cells for transplant include bone marrow, umbilical cord blood, or stem cells collected from the blood,” explains Kenneth G. Lucas, M.D., director of the transplant program, Penn State Children’s Hospital. Here, stem cells are produced and mature to become red blood cells, white blood cells, and platelets. continued on page 2

Keith and Steven, continued from page 1

As they grow, they are released into the blood to perform their specific functions.

In the process of stem cell transplantation, the patient receives high doses of chemotherapy and/or radiation to destroy unhealthy bone marrow cells. Stem cells that have been collected from a donor’s bone marrow are then given back to restore the body’s production. The cells are transplanted through a broviac line, inserted surgically in a central vein before transplant. The central line allows all fluids and medications to be administered

Trishia Layden, R.N., takes Steven’s blood pressure. “They’re our most well-behaved patients,” she says.

through the same line, eliminating the need for multiple intravenous sticks.

For patients with immune deficiency like Keith, transplant provides healthy cells capable of fighting infection. Those with leukemia receive cells free of disease.

In cancer patients, stem cell transplants allow patients to receive higher levels of chemotherapy or radiation therapy than would otherwise be possible. Transplant is used most often in cancer patients when conventional treatments have been unsuccessful in destroying all of the cancer cells, or when patients experience a return of their cancer, known as a relapse. The higher doses of chemotherapy kill all of their bone marrow cells, but a transplant replaces the cells with healthy ones.

Two different sources for stem cell transplants exist. Allogeneic transplant stem cells are collected from either a matched related donor, a matched unrelated donor, or from cord blood. Autologous transplant stem cells are collected from the patient prior to transplant. This type of transplant is used most commonly with patients who have relapsed solid tumors including neuroblastoma, brain tumors, and lymphomas.

A different child In November 2000, 1-year-old Keith received an allogeneic stem cell transplant, with his 21/2-year-old sister, Grace, serving as donor. After the transplant, Keith remained in the hospital for a few weeks to allow the cells to engraft. At first, the Martins didn’t notice much difference.

But suddenly in January 2001, the Martins noticed a dramatic, positive change. “He was just a different child,” says Selesti.

Déjà vu One year later, the Martin family welcomed their third child, Steven.

Physicians performed tests immediately to see whether he also had the gene for WAS. The tests were positive. “This time, we sort of knew what was coming,” says Selesti.

“It was easier and it wasn’t—because we knew what was coming.” Even though the same ailments that plagued Keith haunted Steven as well, as a whole, he was a healthier child than Keith.

Steven received his stem cell transplant when he was 2 years old. The stem cells were harvested from the Martins’ fourth child—a newborn sister whose umbilical cord provided the cells. Selesti says the

Techniques for Taming Tantrums Take steps to avoid them, but if that fails, know what calms your child

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our little one is having a kicking, screaming mega-meltdown in the frozen-food aisle. And you’re sure everyone in the market is thinking, “Why is that child carrying on, and how come the parent isn’t doing something to stop it?”

The period from 14 to 30 months is a peak time for tantrums, says Lynn Wegner, M.D., F.A.A.P., a developmental and behavioral pediatrician and adjunct professor at the University of North Carolina in Chapel Hill. Toddlers that age are learning to verbalize their feelings.

Young children whose “wants” are being blocked are apt to lose control, especially when hungry, tired or overstimulated. Parents in a chaotic public setting, focusing on the task at hand, are

less likely to notice shifts in their child’s mood. This means they may miss the chance to defuse matters before a fullblown tantrum breaks out.

Preventing a tantrum is much easier than stopping one. So before you step out with your child: Try to plan your outing for a time when crowds are few. That helps you avoid long lines and reduces embarrassment if it all falls apart anyway.

■ Ensure you and your child are well-fed, comfortably dressed and rested before you leave.

■ Offer some run-around time before you confine your child to a shopping cart or stroller.

■ Bring toys, snacks or books to entertain and distract if need be.

■

2 Penn State Children’s Hospital, www.pennstatechildrens.com

Even with preparation and planning, tantrums are bound to happen at times. When they do: ■

Make sure the environment is safe if your child is kicking, flailing, or throwing things. Remove the child if sharp corners, breakables or other objects pose injury risks.

[ health bits ] Kids’ Shots Are Often Late

Steven with his brother Keith improvement was more gradual in Steven.

Today, both he and his big brother are doing fine. Steven returns to Penn State Children’s Hospital every three months for checkups, while Keith visits every six months. By the smiles on their faces, they don’t seem to mind. ❖

Nearly two out of five U.S. children are “undervaccinated” for more than six months during their first two years of life, a study found. “It’s really important that kids get vaccinated on time, especially during the first two years, because that’s when they are at highest risk for many of the vaccine-preventable diseases,” says study author Elizabeth Luman, Ph.D., a National Immunization Program disease expert. Children need about 15 to 20 shots to head off measles, mumps, chickenpox, and the like before their second birthday.

While 73 percent of children get all the shots they need, just 9 percent get them all on time, researchers wrote in the Journal of the American Medical Association.

To check the vaccination schedule, ask your doctor or visit www.cdc.gov/nip.

To Learn More For more information about Stem Cell Transplantation at Penn State Children’s Hospital, visit www.pennstatepediatricstemcell.com.

Spacers on Inhalers Curb Asthma Attacks Fighting a child’s asthma attack could be as simple as sliding a plastic tube onto the end of an inhaler, University of Florida researchers say. Yet a lot of doctors don’t give parents this option, they add. The plastic tube is a holding chamber, or spacer. Using a metereddose albuterol inhaler with a spacer and increasing the number of puffs to treat breathing trouble works as well as a nebulizer, studies show. It also causes fewer side effects.

“Most doctors and patients misbelieve that a nebulizer is more effective than an inhaler,” says Florida professor Leslie Hendeles, Pharm.D., lead author of a report in the American Journal of Health-System Pharmacy. A nebulizer turns albuterol into a fine mist that patients breathe in through tubes. An inhaler uses albuterol at a lower dose. The spacer makes the inhaler more effective.

Air Bags Can Harm Kids 14 and Under Remove your child if you want to avoid disturbing others or ease your own stress. It may be easier to calm the child in a quiet place.

■ Don’t worry about other people’s reactions. Focus on your child, Wegner says. Pick actions based on your child’s unique needs and temperament. Some youngsters respond to being held or to a parent’s calm, repetitive words.

Others do best with watchful waiting.

■ Stay centered, take deep breaths and remember: This too shall pass. Hopefully, soon! ❖ ■

Children 14 and younger shouldn’t sit in the front seat of cars with air bags, says a study in Pediatrics. Federal rules say carmakers must warn of a risk for air bag injuries for children 12 and younger. But researchers looked at an eight-year sample of 3,790 children ages 1 month to 18 years who were seated in the right front seat during crashes. The study found kids 14 and younger were at high risk for serious injury from air bags. Air bags tended to protect children ages 15 and up. “When my 13-year-old nephew wants to sit in the front seat now, I won’t let him,” says study lead author Craig Newgard, M.D., an emergency medicine researcher at Oregon Health & Science University.

Inactive Girls Gain Weight Inactive teen girls gained an average of 10 to 15 pounds more than active girls during a 10-year study, The Lancet reports. At ages 9 and 10, there were slight differences in body mass index—about 4 to 5 pounds—between active and inactive girls. But the gap grew by the time the 2,379 girls in the study turned 19. The girls’ calorie intake rose just a bit and did not seem to be tied to the weight gains. “Just preventing the decline in physical activity that currently occurs among adolescent girls may be enough to prevent obesity,” says research nutritionist Eva Obarzanek, Ph.D., of the National Heart, Lung, and Blood Institute.

Penn State Children’s Hospital, www.pennstatechildrens.com 3

Feeding Program Helping children with feeding and nutrition

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oes your child throw tantrums at the dinner table?

Eat chocolate pudding and only chocolate pudding?

Maybe your child is physically incapable of chewing and swallowing. If any of these scenarios sound familiar, the Feeding Program at Penn State Children’s Hospital may be the answer.

Keith Williams, Ph.D., director of the Feeding Program at Penn State Children’s Hospital, and his staff are committed to helping children with a range of feeding and Keith Williams, nutritional problems.

The program at Ph.D. Children’s Hospital is one of only about a dozen comprehensive programs in the country, having cared for children from 21 states and four countries outside the United States.

One of those countries is Ireland, which doesn’t have a feeding program like the one at Penn State Children’s Hospital. Ann and John Cahill of Ashler Tulla, Ireland, had given up hope after taking their tube-dependent daughter Tinalee to doctors and feeding programs in Ireland. At the age of 6, Tinalee had never eaten solid foods or spoken a single word. She was also still in diapers. Yet even after surgeries to correct her physical problems, Tinalee still couldn’t eat or speak. She had become

traumatized by food, associating it with pain, and her mouth muscles weren’t strong enough to form words.

Children are enrolled in the Feeding Program for a variety of reasons. Some regularly refuse food or exhibit significant behavioral problems, such as throwing food or hitting, during meals.

Others eat extremely limited varieties of foods or textures that are not developmentally appropriate, such as baby food when they should be eating solids.

Some children, like Tinalee, have more obvious feeding problems, such as vomiting after or during meals, or relying on a feeding tube for nourishment.

But how do parents know if their child has a serious feeding problem that needs professional intervention or if it’s something they can handle themselves?

Williams says that when children are failing to thrive, it can affect height, weight, and brain development, and it’s definitely time to get help. Parents shouldn’t blame Tinalee with mother themselves, he Ann Cahill adds. Most feeding problems are a result of illness or other problems.

Children can be referred by their doctors, therapists, parents, nurses, and teachers. Ann Cahill found the Feeding Program at Children’s Hospital online and decided to call Williams in a

4 Penn State Children’s Hospital, www.pennstatechildrens.com

last-ditch effort. Williams asked Cahill to send Tinalee’s medical records and a video of her trying to feed her daughter.

He called the Cahills within two weeks and said that Children’s Hospital would welcome Tinalee into the program.

In June 2004, Ann and Tinalee flew stateside while John stayed home with their two other children. After a week of intensive therapy, Tinalee was taking her first bites of solid foods, requiring only water through her feeding tube.

In one month, Tinalee was completely off the tube. “It was a huge, unreal change,” says Ann. Tinalee was enrolled in the intensive treatment program, an all-day alternative to inpatient therapy used for children who are dependent on supplemental feedings or at severe nutritional risk.

Intensive treatment entails between five and 10 feeding sessions per day.

Partnership Helps With Patient Care Bailey Ann Schwartz of Beach Lake, Pa. (in Wayne County), needed specialized care for her systemic scleroderma. She and her family found assistance close to home thanks to the partnership between Penn State Children’s Hospital and Wyoming Valley Health Care System in Kingston. Specialists from Children’s Hospital regularly travel to Wyoming Valley to offer the care that area children need. Services include the following: ■ Cardiology ■ Gastroenterology ■ Hematology/oncology ■ Nephrology/hypertension ■ Pulmonology ■ Rheumatology

Bailey Ann Schwartz

The feeding program also offers three other treatment plans: outpatient feeding therapy, the oral motor clinic, and an evaluation clinic. Outpatient feeding therapy is the most common treatment and includes a single appointment where therapists work with children to tackle specific problems. The oral motor clinic cares for children who are physically unable to eat.

The multidisciplinary clinic features specialists from pediatric gastroenterology, behavioral psychology, and many other specialties. No matter the treatment plan, the goal is the same: teaching the parents and caregivers how to maintain their child’s healthy eating habits at home. Parents are able to watch feeding sessions in observation rooms attached to each treatment room. In addition, sessions are videotaped with the therapist narrating each technique. The session is

burned to DVD and sent home with the parent or caregiver.

For the Cahills, the experience has been life-changing. Tinalee has become independent and vibrant, feeding herself, using the bathroom, and speaking “loads of words,” says Ann. “She came to life.” In addition, Ann has gone back to work part-time, and she and John can go out together again. “Before, everywhere we went, Tinalee came.” Now other caregivers are comfortable taking care of Tinalee. “We owe them so, so much.” ❖

To Learn More For more information about how the Feeding Program at Penn State Children’s Hospital can help your family, visit www.hmc.psu.edu/childrens/feeding.

E-mail: feedingprogram@hmc.psu.edu Telephone (717) 531-7117

To Learn More For more information, ask you child’s pediatrician or call Penn State Children’s Hospital at (800) 243-1455.

Penn State Children’s Hospital, www.pennstatechildrens.com 5

For Obese Teens, Surgery Is the Last Resort While such operations are up, they’re not for all youths

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xtreme obesity plagues more than a million teens and young adults, experts estimate. The youths tend to be at least 100 pounds or 100 percent above their ideal body weight.

So far, the toll for that extra weight includes a sharp rise in type 2 diabetes among the young. And the future may not be any healthier for these teens, who aren’t likely to grow out of their obesity.

What’s a parent to do? More and more are looking at the same last resort as adults—bariatric surgery. Such surgery, in effect, reduces the stomach’s size. You feel full sooner, so you eat less.

Have tried but failed at organized weightloss attempts of six months or longer.

■ Have a body mass index (body weight adjusted for height) of at least 40.

■ Have finished most of their skeletal growth. That generally means girls must be at least 13 years old and boys 15.

■ Have obesity side effects that weight loss would help fix.

■

“If the child does not change behaviors, then the expected weight loss may not occur or, with time, could be regained.”

—Ronald Williams, M.D., director of Penn State Children’s Hospital Multidisciplinary Weight Loss Program

U.S. doctors did about 150 such procedures on teens from 1991 to 2000, says Thomas H. Inge, M.D., Ph.D., surgical director of the Comprehensive Weight Management Center at Cincinnati Children’s Hospital Medical Center. No one’s sure how many have been done since. Still, they’re clearly on the rise.

About 60 teens have had the surgery just at Cincinnati Children’s since 2001.

But such surgery isn’t right for all obese teens. In a recent Pediatrics article, Inge wrote that bariatric surgery is warranted in most cases only when adolescents:

“If we sense a child is not ready to make a major change in his or her diet, or if they haven’t made a very valiant effort with some sort of supervised weight-loss program for at least half a year, that’s a real red flag,” says Inge.

“If the child does not change behaviors, then the expected weight loss may not occur or, with time, could be regained,” says Ronald Williams, M.D., director of Penn State Children’s Hospital Multidisciplinary Weight Loss Program. Parents and children must also understand the risk for complications, including the very slight risk for death.

Are you looking at such surgery for a child? Experts suggest you seek a center experienced in the procedure. The center should have a weight-management team with experts in different fields—dietitians, psychologists or psychiatrists, and surgeons—who can evaluate your child.

Long-term follow-up is a must. So is a strong commitment from you and your child. “The operation is a tool, not a cure or quick fix,” says Inge. “It takes lifelong compliance with a dietary regimen, vitamin and mineral supplements, and exercise to maintain a healthy weight and avoid regaining the excess pounds.” ❖

6 Penn State Children’s Hospital, www.pennstatechildrens.com

High chairs are great for older children, who can safely play with toys on a tray.

Babies Need “Tummy Time” Put infants to sleep on their backs, but act to avoid flat heads while they’re awake A simple piece of advice in 1992 cut the death rate from sudden infant death syndrome (SIDS) by more than half. That was the year the American Academy of Pediatrics (AAP) told parents to put babies to sleep on their backs.

Now, the experts have some new advice to reduce the odds of flattened heads, a possible result of babies spending so much time on their backs.

No one’s sure how common flat heads are. Statistics vary a great deal, “from one in five cases for a mild form to one in 500 to 600 cases,” says AAP spokesman John Persing, M.D., a professor and chief of plastic surgery at

Toss Your Baby Walker, Pediatricians Say Children can roll themselves into danger Safety is your top concern for your child.

Just as you put your infant in a car seat, you may think that putting your child in a baby walker is safe, too.

“Safety is a misperception,” says pediatric emergency physician Joseph Wright, M.D., a member of the American Academy of Pediatrics (AAP) Committee on Injury, Poison and Violence Prevention. The AAP terms baby walkers dangerous and says you should throw them out. “Even when parents are supervising a child, he or she can move a walker at 3 feet per second,” he warns.

An estimated 4,360 children were injured due to baby walkers in 2004, according to the U.S. Consumer Product Safety Commission. Walkers can cause children to: ■ Roll down stairs, causing head injuries and even death. This is the most common way kids get hurt in walkers.

■ Get burned. Children in a walker may

Yale University School of Medicine. But doctors have seen a “significant increase” in flat heads in the past decade, says a 2003 article Persing wrote in Pediatrics.

“It’s very important for infants to get some tummy time when they are awake and supervised,” says John Kattwinkel, M.D., chairman of the AAP Task Force

be able to reach a hot cup of coffee on a table or a pot on the stove.

■ Drown. A child can roll into a pool or fall into a bathtub or toilet.

■ Be poisoned. A child may be able to reach poisonous items you thought were out of reach.

■ Pinch fingers or toes. A child’s tiny digits can get caught between the walker and furniture.

“Parents should avoid all mobile walkers,” Wright says. For safety’s sake, also make sure there are no baby walkers anywhere your child spends time.

Walkers also slow development, Wright says. “Motor development is delayed compared with infants who are not placed in walkers.”

The AAP suggests you use a stationary jumping device instead. “Nonmobile jumping devices are safer and can provide a level of freedom for parents,” Wright says. Such devices don’t have

on SIDS and a pediatrics professor at the University of Virginia School of Medicine.

To avoid a flat head, Persing and Kattwinkel offer these tips: Parents should still place babies on their backs for sleep.

■ When babies are awake, put them on ■

wheels; instead, they have seats that rotate and bounce.

Playpens are safe for children learning to sit, crawl, and walk. High chairs are great for older children, who can safely play with toys on a tray. ❖

Playpens are safe for children learning to sit, crawl, and walk.

their tummies for a while. This eases pressure on the back of the head and helps babies build shoulder and neck strength. “This time must be supervised, 100 percent of the time,” Persing says. “Don’t even run to the bathroom and leave an infant on the tummy.”

■ Relieve pressure on the back of the head when you lay an infant down for sleep “by very gently turning their head 45 degrees to the left one night, then 45 degrees to the right the next night,” says Persing.

■ Change the crib’s position a few times a week. As your child looks around the room, the head will be in a new position because of that change.

■ Don’t overuse car seats when the child is not in a car. When in a car, move the car seat often from one side to the other.

■ If your child develops a flat spot on the head, see your doctor. Such flat spots usually form on the back or side of the head. ❖

Penn State Children’s Hospital, www.pennstatechildrens.com 7

Teaching the Next Generation of Healers

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eaching future doctors how to provide child-friendly and family-centered care is as much an art as it is a science. While few in number, children’s hospitals train almost one-third of our nation’s pediatricians and half of all pediatric specialists, such as neurologists or cardiologists. If you have children, they’ve probably been cared for by a pediatrician or a family practice physician who trained at a children’s hospital at some point in his or her career.

Because children’s hospitals often take care of children with very serious and complex conditions, such as cancer,

To Learn More To learn more about the importance of graduate medical education to children’s health and children’s hospitals, visit www.childrenshospitals.net.

cystic fibrosis, or heart transplants, they must provide the most technologically advanced care available. Doctors in training at children’s hospitals get specialized education and unique experience that no other hospital can provide.

But teaching great physicians takes time and money. While Medicare pays for training physicians in adult hospitals, children’s hospitals don’t qualify for this funding because they don’t treat adult patients. That’s why, in 1999, the National Association of Children’s Hospitals (NACH) successfully lobbied Congress to create the Children’s Hospitals Graduate Medical Education payment program. This program provides federal funding to nearly 60 children’s hospitals that train physicians and ensures children’s hospitals can continue to provide quality care while they train the next generation of healers.

However, NACH and children’s hospitals must appeal to Congress for this

funding each year.

Ask your children’s hospital how you can help make sure children’s hospitals get the money they need to train the doctors who care for children.

Children’s hospitals also train nurses, occupational therapists, social workers, dentists, and other health care professionals. By receiving professional training in a children’s hospital, our nation’s future health care professionals gain an appreciation for the specialized needs of children and develop the skills and compassion needed to care for families. ❖

NACHRI National Association of Children’s Hospitals and Related Institutions www.childrenshospitals.net

Articles in this newsletter are written by professional journalists or physicians who strive to present reliable, up-to-date information. But no publication can replace the care and advice of medical professionals, and readers are cautioned to seek such help for personal problems. ©2006 Health Ink Communications, 780 Township Line Road, Yardley, PA 19067, (267) 685-2800. Some images in this publication may be provided by ©2006 PhotoDisc, Inc. All models used for illustrative purposes only. Some illustrations in this publication may be provided by ©2006 The Staywell Company; all rights reserved. (106)

Cub Chat is a complimentary quarterly newsletter produced by the Office of Strategic Services at Penn State Children’s Hospital.

For questions or additional copies, please call (717) 531-8606. www.pennstatechildrens.com

A. Craig Hillemeier, M.D. Medical Director and Chairman

CHI-3227-06