Know A Good Doctor? We Do.

From our Sponsors

Caring for Kentucky’s Children

This slideshow requires JavaScript.

It’s a tough job – informing parents that their child has an incurable or potentially devastating disease and navigating that child’s long-term treatment. Yet pediatric specialists spend an entire career doing just that. In Kentucky’s major university healthcare settings, pediatric specialists are diagnosing enigmatic disorders, managing chronic conditions, and researching more effective treatments to allow children the simplest opportunity … to just be kids.

University of Louisville

University of Louisville Physicians-Pediatrics is the largest of the UofL Physicians service lines, with 175 medical specialists. Covering everything from adolescent medicine to infectious disease, these specialists, who are all faculty at the UofL School of Medicine, provide clinical care, conduct research, and train pediatric residents. They are the major providers of medical care at Kosair Children’s Hospital.

UofL Physicians-Pediatric Nephrology

A New Way of Thinking

Getting patients to change lifestyle habits is notoriously difficult. Changing the behavioral patterns of adolescents, who view themselves as invincible and are heavily influenced by the actions of their peers, is even harder. Adolescent hypertension is on the rise in the US but awareness is not nearly at the levels of diabetes or obesity.

David Kenagy, MD, joined the Pediatric Nephrology program in May 2012 and has his sights set on “taking a different approach to hypertension.” Kenagy says, “The nephrologist has a big part in figuring out why hypertension is happening and recommending treatment.” For some children, hypertension results from specific, treatable diseases of the kidney. But the greatest increase in hypertension is happening to adolescents who have a strong family history of hypertension, a sedentary lifestyle, a high sodium intake, and normal laboratory test results. He attributes the rising levels of hypertension to improved recognition by pediatricians and an increase in obesity, sodium intake, and sedentary lifestyles.

Kenagy envisions a new approach to adolescent hypertension, designed to change outcomes. He notes that traditional healthcare delivery systems have failed to stem this rising tide. His approach centers on the idea of a shared medical appointment. It is “a method of delivery of healthcare that’s been pioneered in a variety of different settings,” says Kenagy, including obstetrics and well child care. Bringing patients with the same diagnosis together to provide management tactics in a group setting allows patient to learn from and with their peers. Patients still get the benefit of seeing the physician one-on-one. “They’re not just learning from the nurse or dietician practical ways to put this into practice. They’re also in the same room with people who are similar to them from their perspective – similar age, similar gender, similar sociocultural group, similar disease process,” he advocates.

A Support Mission

Kenagy views the Nephrology division’s role as “a support mission for other specialties.” Kenagy and fellow pediatric nephrologist Sushil Gupta, MD, are often asked to consult on lupus management, blood pressure medications, or determine the cause of sudden changes in kidney function. “In the outpatient environment, a good portion of our work is helping children and their families to navigate the course after kidney disease begins but long before dialysis or transplantation is necessary,” Kenagy says. Kenagy and Gupta both have a decade of experience in general pediatrics, an advantage in evaluating the whole patient and augmenting the services of the general pediatrician.

Pediatric Dialysis Unit

Kenagy left Rainbow Babies and Children’s Hospital in Cleveland, Ohio, part of Case Western Reserve University, to join UofL last spring. “It was UofL’s enthusiasm about building a pediatric dialysis unit that attracted me to come here,” says Kenagy. In the US, children constitute only about 1% of patients with end stage renal disease (ESRD), meaning most receive dialysis in centers designed for adults. “Only a few locations in the country have made the decision to tailor their dialysis operations to children,” he says.

The pediatric dialysis unit is in development. The core staff is led by Liz Reed, RN, an administrator from Kosair Children’s, who has extensive experience in transplantation and directs the heart and kidney transplant programs. Drea Baker, RN, (peritoneal dialysis) and Kathy Baker, RN, (hemodialysis and transplantation) are experienced managers of kidney diseases and are already creating dialysis guidelines. Plans for the physical plant are in the works. The unit will be located in a new medical building, half a block from UofL’s current pediatric offices, which will house almost all the outpatient offices for every pediatric specialty and will be connected by pedestrian bridges to the hospital. Kenagy describes it as “a medical home … so the child who not only has a need for dialysis but also has a seizure disorder or lung disease – same building, same parking lot, and same location.” Construction is projected to be complete in 2014.

When it comes to treatment for renal insufficiency, Kenagy much prefers peritoneal dialysis to hemodialysis where appropriate. Offered in the child’s home and administered while the child sleeps, peritoneal dialysis requires more effort on the part of parents but offers a more physiologic, less disruptive, and more convenient care model for many patients. Hemodialysis is conducted in a dialysis center and requires patients to come to the center three to four times a week for two to four hours at a time. The new center will provide comprehensive kidney care: hemodialysis for some, peritoneal dialysis training and support for others. But for all, the center will prepare for transplantation and provide state-of-the-art care afterwards.

UofL Physicians-Pediatric Rheumatology

A Detective’s Work

“Basically, rheumatologists all over end up being the depository for patients who don’t fit anywhere else,” says UofL pediatric rheumatologist Kenneth N. Schikler, MD, who likens his job to that of a detective. He and colleague Kara Schmidt, MD, see patients that are referred to them by primary care physicians, infectious disease specialists, oncologists, and orthopedic surgeons for unexplained joint or musculoskeletal pain, general malaise, abnormal lab tests, as well as unexplained fevers, rashes, or chronic pain.

A veteran on the UofL campus, Schikler joined the faculty in 1976 as the director of Adolescent Medicine for the Department of Pediatrics. Long before pediatric rheumatology was a board-certified subspecialty, Schikler began seeing children with chronic, undiagnosed rheumatologic conditions, which led to him to “retool” himself in the subspecialty of pediatric rheumatology. In the mid-80s, Schikler was appointed as director of Pediatric Rheumatology by Dr. Billy F. Andrews, the chairman of the Department of Pediatrics at the time.

Fortunately, according to Schikler, most of the complaints children and adolescents are referred for have explanations. Central pain processing disorders, such as fibromyalgia, are the most common diagnoses Schikler and Schmidt see, numbering well over 200 patients. A chronic musculoskeletal pain condition, not an inflammatory disease, fibromyalgia is “associated with fatigue, recurring headaches, recurrent abdominal pain, lightheadedness or passing out frequently in addition to chronic widespread musculoskeletal pain for at least three months,” he says. It often goes unrecognized, as lab tests and x-rays should be normal and not indicative of an inflammatory process.

Juvenile idiopathic arthritis (JIA) is the most common of the rheumatic inflammatory diseases and is characterized by unexplained arthritis that is present for at least six weeks. It affects between 10 to17 out of every 100,000 children. Other conditions include: systemic lupus erythematosus (SLE), juvenile dermatomyositis, scleroderma, less common vasculitides, and mechanical pain issues.

Remission, Not Cure

“We never talk about cure. “We talk about inducing remission,” says Schikler, whose treatment goal is “to have kids function the way kids are supposed to function and have normal childhood and adolescent lives as they progress to adulthood.”

Treatments for inflammatory conditions include NSAIDS and immunomodulatory drugs, which can include corticosteroids, chemotherapeutic agents, intravenous immunoglobulin (IVIg), and biologic agents. The biologics are being studied in a number of different clinical trials but are already approved and being used in the clinic for JIA, vasulitides, and lupus. Intra-articular injections of corticosteroids for arthritis and rehab services (physical and occupational therapy) are also employed.

For central pain processing, treatments include pharmacological agents, aerobic exercise, and cognitive behavioral therapy, an area they found to be of benefit in a study in which they participated and which was published this year. Schikler chairs the Pain Committee for the Childhood Arthritis and Rheumatology Research Alliance (CARRA), which has put together a toolbox for treating patients with juvenile fibromyalgia.

Research to Target Treatments

Stem cell transplantation is currently an end of the line treatment for children with some forms of arthritis, lupus, and systemic sclerosis, but Schikler is closely watching current research in adults with stem cell infusions. The ultimate goal is to find more targeted treatments. “Things like steroids and methotrexate are fire hoses that target the entire immune system,” he says. “Every JIA isn’t necessarily caused by the same alteration in one’s immune system or connective tissues. We’re trying to be more precise in understanding the specific defects that would enable more narrow focused targets in treatment, even beyond the great strides provided by the narrower focus of our current array of biologic agents.”

Research currently being conducted at UofL includes approximately 10 rheumatologic studies looking at biologic agents directed at inflammatory cytokines and modulation of the immune system and behavioral outcomes in patients with JIA. Schikler is also the chair of the Section on Rheumatology of the American Academy of Pediatrics, where he is helping to determine national policy in the field.

University of Kentucky Department of Pediatric Hematology-Oncology

Research, Guidance, and Prevention

Let’s start with some good news.

Most children will survive childhood cancer. According to UK pediatric oncologist John A. D’Orazio, MD, PhD, up to 70% of all childhood cancers are curable.

“These are a very special group of kids who, through no fault of their own, get leukemias and lymphomas, tumors, and other cancers,” says D’Orazio. “The good thing about this field is that most of them can be cured. Fifty years ago, a child with leukemia would come in and the physician would give the family his deepest condolences and morphine, and it would be done in six weeks. But today, leukemia – ALL leukemia in particular – is 85%-plus curable. Some circles even say 90%, and that is directly the result of decades of clinical and science research. We are absolutely dedicated to that cause.”

D’Orazio’s commitment, as a physician-scientist, means he attends to the children on UK’s pediatric hematology-oncology service and studies melanoma risk and prevention at the Markey Cancer Center.

“My research impacts the children that we see here in a more preventative way,” he explains. “Right now, we advise patients to stay out of the sun, wear sunblock, and please don’t go to tanning salons. But, I am working on new ways to apply topical creams and sprays that would actually reduce their chances of getting melanoma.”

Many people with fair complexions have a deficiency in their ability to produce melanocyte-stimulating hormone (MSH), the hormone responsible for one’s ability to tan, and face a significant risk for melanoma. D’Orazio’s research shows that the application of the drug forskolin can correct this deficiency and produce a UV-protective tan. Furthermore, the drug is shown in mice studies to repair the mutations caused by UV exposure in as little as 24 hours.

Forskolin, says D’Orazio is “too indiscriminate to be practical,” but as research narrows in on other ways to manipulate the hormonal axes of the skin, D’Orazio is hopeful that his research will have real benefit to pediatric cancer survivors for whom melanoma is “one of the overrepresented secondary malignancies later on.”

To help pediatric cancer survivors monitor the lifelong effects of chemotherapy and radiation, UK opened the state’s first Long-term Follow-up Clinic in 2008. Led by Sherry L. Bayliff, MD, MPH, who is board certified in pediatrics and board eligible in pediatric hematology/oncology, the clinic utilizes decades of research contained in the Children’s Oncology Group’s survivorship guidelines to help patients and their physicians better manage their health.

“We look for late effects of our chemoradiation, surgery, and all the interventions we use to help cure or manage the disease,” says Bayliff, “and so move away from just surveillance or observation for relapse.” By taking a more predictive stance that, for example, a brain tumor survivor may face radiation-associated osteosarcoma 10 or 15 years later, Bayliff helps educate patients about the process of survivorship, being mindful of what to avoid and what to report quickly to their physicians. “Our goal is to show them that to be a survivor means to beat all odds.”

Hemangiomas and Vascular Malformations

Guidance is a prominent term in Bayliff’s vocabulary. In addition to her work with pediatric cancer patients and survivors, she helps patients, their families and pediatricians manage some very difficult, often disfiguring vascular disorders through the Pediatric Vascular Anomaly Clinic.

While hemangiomas affect about 10% of newborns, Bayliff cautions that there are still a lot of misunderstandings around the condition. “Some people call any congenital vascular anomaly a hemangioma, but that is a misnomer,” she says. Rather, hemangiomas are benign tumors of the capillaries in one select area, often in the head and neck, with a specific life cycle beginning at or within a few months of birth and lasting until age 10. “The capillaries continue to grow for a period of time and will reorganize, collapse, and then start to melt away until they are completely absorbed into the body.” She says much of her work is in helping parents form realistic expectations and guiding them toward trustworthy resources for information and support.

About one-fifth of hemangiomas require intensive medical care, especially when they interfere with vital structures. Some obscure vision or compromise breathing and feeding. “Babies with greater than five hemangiomas may also have them internally on their organs,” Bayliff advises, “and if they grow at a faster pace, they can cause a steal of blood flow and interfere with the function of the heart.” Further complications come from the breakdown and potential ulceration of the hemangioma, and some, if allowed to grow to their full potential can be very disfiguring. In these cases, coordinated interventions are necessary.

Vascular malformations are managed in a similar manner, and while congenital and causing lesions that last a lifetime, they may also manifest later in childhood or be discovered at the onset of puberty. Vascular malformations may involve venous, lymphatic and arterial vessels, or a combination of any the above.

“Klippel-Trenaunay syndrome is the most well-known of these abnormalities,” says Bayliff, “but treating it is very complicated.” Focusing on improved quality of life through symptom management, Bayliff prescribes compression garments, physical therapy, and occupational therapy to alleviate the discomfort and improve appearances. Orthopedics can help with a myriad of complications arising from the syndrome’s bony overgrowths, and plastics can improve surface bleeding through laser resurfacing of lymphatic malformations. Bayliff points out that new medications “will soon be part of the frontline studies,” including the immunosuppressant agent sirolimus, which works to reduce the potential for swelling in lymphatic malformations. “What to expect from these conditions, even among pediatricians” says Bayliff, “is not very well known, so providing guidance from the consultation through the management of medications is one of my important roles.”

Hemophilia and Clotting Disorders

Here are three truths about the patients that come into this busy department: They have very special needs. They require preventative care to control symptoms and improve quality of life issues. They require coordinated treatments across specialties and shared management with their primary physicians. In brief, this is not just a pediatric hematology/oncology clinic but, for many, a medical home.

“Hemophilia was one of the first disorders to use the medical home concept about twenty years ago,” says Vlad C. Radulescu, MD, pediatric hematologist/oncologist and director of UK’s Hemophilia Treatment Center. “We try to help patients with their lifestyle, work, and medical needs in order to prevent bleeds from occurring. Then, we try to achieve this at a minimum expense. It has been proven that in a coordinated setting, the treatment of hemophilia is superior in terms of effectiveness and cost when compared to treatment by a primary care physician.”

Utilizing federal and state funding, the Hemophilia Treatment Center helps patients of all ages, particularly from central and eastern Kentucky, prevent bleeds and the significant impairments they can cause. According to Radulescu, clinical research at the center aims to optimize the timing of the administration of coagulation factor concentrate so as to reduce the risk of developing inhibitors. “Inhibitors not only destroy the clotting factor, which is very expensive,” he says. “They can lead to a situation in which it is very difficult to stop the bleed.”

Radulescu takes a similarly coordinated approach to the treatment of sickle cell disease and other clotting disorders. Complications from sickle cell disease are well-known to impact the lives of middle aged adults, but the disease can be life-threatening to even the youngest patients. The center monitors these patients periodically to assess organ function, prevent infections, intervene with pain crises, and screen for risk of stroke.

Success in delivering quality care for clotting disorders, he says, is making sure that the team is very familiar with individual patients and their needs, and then being as forthcoming with the primary care physicians as possible.

“Thus, their care is more efficient and effective for the patient,” says Radulescu. “If they can come to a place where the nurse knows everything about them, where all their medical needs can be met in one place, it is much better than going from one doctor to another.”

Physicians will also be interested to learn that the preliminary data from the center’s research into pediatric thrombosis, a clotting disorder that has seen steeply increased incidence in recent years, “suggest that obesity may tip the balance over to clotting. More work needs to be done to determine whether that’s true and to understand the pathways to which obesity leads to clotting,” he says.