The UAB Neurofibromatosis Clinic provides comprehensive, integrated care to adult and pediatric patients with all forms of NF, including NF1, NF2, and schwannomatosis. Because pediatric patients frequently have health concerns that are different from those of adult patients, we provide a dedicated pediatric NF clinic that delivers specialized care for pediatric patients from a multidisciplinary team of specialists who collaborate in the treatment and management of NF and its complications. This month’s blog features insight from two physicians who provide care to pediatric NF patients.
UAB and Children’s of Alabama pediatric neuro-oncologist Katie Metrock, MD, leads the pediatric neuro-oncology clinic within our pediatric NF program, which assists in the management and treatment of brain and spinal tumors, optic pathway gliomas, complex neurofibromas, and malignant peripheral nerve sheath tumors (MPNSTs). Children’s of Alabama Division Director of Pediatric Hematology and Oncology Girish Dhall, MD, provides specialized care to pediatric patients with brain tumors, including patients with NF. Dr. Metrock and Dr. Dhall discuss their roles in providing care to pediatric NF patients and the unique types of support the pediatric NF clinic provides in managing the lifelong medical, psychological, and social implications of an NF diagnosis.
How does providing a dedicated pediatric NF clinic and a multidisciplinary approach to care benefit patients?
Dr. Metrock: I see patients each Tuesday afternoon during our weekly pediatric NF clinic at Children’s of Alabama. Having a dedicated pediatric NF clinic means that our patients can receive more of their care at one time. Many of our patients have multiple health concerns and complex cases, so having access to several specialists in one location streamlines care for our patients and allows providers to integrate care more effectively.
Our team of specialists also meets weekly as part of the neuro-oncology tumor board, either in person or on a conference call, to discuss aspects our program and our patients’ cases. Genetics and neuropsychology are part of the call, as well as adult and pediatric neuro-oncology. It’s important to have adult neuro-oncology in these meetings because our pediatric patients will need adult care at some point. The adult neuro-oncologists are often familiar with the pediatric cases when they move on to the adult clinic, and this helps to ensure continuity of care.
The NF Clinical Trials Consortium team also participates in the meeting to share information about any clinical trials that could be relevant, as well as our physician scientists who may glean information that is helpful in planning future research. These meetings are not only beneficial for patient care, but they also help to advance continuing medical education because we learn from interacting with other specialists.
Dr. Dhall: While Dr. Metrock is our NF physician who sees patients in the UAB Pediatric NF Clinic, I provide care to pediatric patients with brain tumors, including those with NF, each Wednesday in a dedicated clinic at Children’s of Alabama. Our group also has close collaboration with pediatric endocrinologists and neurosurgeons, who are often an integral part of the care team for pediatric patients with brain tumors.
We also have a weekly tumor board meeting with all neuro-oncologists to discuss pediatric brain tumor patients and their treatment progress, and some of these are NF patients. Also, we have a bi-weekly tumor board meeting for all other tumor types, including pediatric patients with NF, which includes neuro-oncology, neurosurgery, neuropathology, and neuroradiology. Having a diversity of disciplines involved in these meetings ensures integrated, comprehensive care for these patients.
In what ways are pediatric NF patients’ needs different from those of adult patients?
Dr. Metrock: Pediatric patients have unique health concerns. First, they are growing, and they also have NF, so we monitor them carefully for any abnormalities in growth and development. For example, brain and spinal tumors can cause precocious puberty in some children, resulting in faster growth and development. Also, peripheral nerve sheath tumors and plexiform neurofibromas can affect development.
Dr. Korf is on the front line of providing care to NF patients and identifies many of these issues. By the time they come to me, we already know about a tumor. In other cases, we may perform imaging and identify a tumor.
A significant part of the care I provide is working with patients and families to decide when it is appropriate to treat a tumor or when it is best to watch and monitor it. We do have effective therapies for brain and spinal tumors, and MEK inhibitor medications are effective for treating plexiform neurofibromas. However, all medications have side effects, so we weigh treatment against other factors, such as the location of the tumor, whether it is impacting other structures, and how it is affecting daily life and its potential to impact the patient’s life going forward.
Dr. Dhall: For pediatric patients with brain tumors, the needs are unique because the psychosocial component is more significant in terms of how they perceive the disease and the level of disruption that might occur to their daily lives and routine. Brain tumors in younger patients can be more aggressive, and the psychological cost of the disease can be higher. For example, if a child loses vision due to a brain tumor, it can be devastating due to the many years of development they have ahead of them.
For this reason, we are much more aggressive with treatment in pediatric patients. However, because we want to avoid significant side effects, we focus on achieving a fine balance between effective treatment and side effects.
What other aspects of the UAB Pediatric NF Clinic address the unique needs of pediatric patients?
Dr. Metrock: We provide our patients with an exceptional neuropsychology team to help children leverage their strengths, maximize their abilities, and reach their goals. As a physician, I value that we have specialists on our team who can address the psychological development of our patients. The neuropsychology team provides neuropsychology testing to diagnose learning differences or other related developmental issues. They regularly meet with families to discuss test results and determine the next best steps.
We also have a school liaison on our team who is a trained, experienced teacher. She works closely with the neuropsychology team to communicate necessary information to the child’s school, which involves meeting with school counselors in person to ensure our patients are receiving all needed resources, including IEPs and 504 plans. If one of our patients is undergoing treatment, she also sometimes speaks to the child’s class to help them better understand what their classmate is experiencing and what to expect.
In addition to these resources on our clinical team, the Children’s of Alabama Hope and Cope team includes a patient navigator and social worker that regularly checks in on families to identify any stressors and provide help and support. We have many patients who travel long distances to come to the clinic, and the Hope and Cope team also assists these families with finding affordable lodging and other resources.
Dr. Dhall: The exceptional transition of care between pediatric and adult oncology is a unique strength of UAB and Children’s of Alabama that benefits patients by ensuring continuity of care. Dr. Korf serves as the glue to bridge the gap between pediatric and adult patients. We also have monthly meetings about adult and pediatric patients, and we routinely discuss transition of care. We typically care for patients up to age 21 and begin thinking about transition of care at age 18.