The NCI estimated 15,590 children and adolescents ages 0-19 were diagnosed with cancer in 2018. In comparison to the past 20 years, the overall childhood cancer survival rate continues to rise. In fact, over 429,000 U.S. childhood cancer survivors were reported to be alive in 2015.
Children diagnosed with high-risk cancers are considered medically complex and seen routinely for ototoxicity monitoring; however, barriers can continue to arise throughout and beyond their course of cancer treatment. With an increased incidence of childhood cancer and aggressive medical management, children diagnosed with cancer are at risk for decreased quality of life. Several barriers impact this population, including hearing loss due to ototoxicity, impact of hearing loss on speech and language, school reintegration, and parent coping mechanisms. Health care providers play an integral part in the multidisciplinary approach to advocate for treatment options that will help patients overcome these barriers and have a better quality of life.
Ototoxic Hearing Loss
Ototoxic hearing loss occurs when chemotherapeutic agents cause degeneration of hair cells. Sensorineural hearing loss due to ototoxicity is more often bilateral and symmetrical, originating in the higher frequencies. This can be progressive with increasing cumulative dose.
Platinum-based chemotherapy agents such as cisplatin and carboplatin are common treatment regimens for pediatric cancers including neuroblastoma, hepatoblastoma, and medulloblastoma. Cisplatin tends to be the more ototoxic although carboplatin can cause significant hearing loss at higher doses. Platinum-based therapy coupled with cranial irradiation has a higher risk of hearing loss, but this requires the hearing mechanism to be within the radiation field (Int J Audiol 2017;57(sup4):S62-S68). Research has shown that children younger than 5 years old are 21 times more likely to develop moderate to severe high-frequency hearing loss than older children given the same cumulative dose (Eur J Cancer 2004;40:2445-2451).
Impact On Speech & Language
Speech production and language development require children to be able to perceive and differentiate sounds at all frequencies. Ototoxic hearing loss negatively impacts the high frequency speech sounds s, f, th, sh, h, k, and t. These sounds account for approximately 50 percent of the consonant sounds in the English language (J Clin Oncol 2005;23(34):8588-8596). Reduction in the audibility of these consonants impact pre-, peri-, and post-lingual children. Pre- and peri-lingual children often experience articulation errors, unintelligible speech, a decrease in low-frequency speech cues, plus difficulty with tenses, plurals, and possessives. Children with pre-existing speech and language skills may appear to be less impacted by a high-frequency hearing loss; however, minimal hearing loss can significantly alter language development, verbal abilities, and reasoning skills in young children (Int J Audiol 2017;57(sup4):S62-S68; Pediatrics 2007;120(5):e1229-e1236). According to Bass, et al, 37 percent of children with minimal hearing loss failed at least one grade in school compared to the normative rate of 3 percent (2013, ASHA https://bit.ly/2kvejB8).
School Reintegration
The concerns of the student, parents, or teacher can affect a child's re-entry to school after a cancer diagnosis. Table 1 identifies common concerns of the child, parent, and teacher when preparing for the child's reintegration into school. About 11 percent of children diagnosed with cancer in childhood or adolescence have school phobia compared with 1.7 percent of the population at large (J Pediatr Oncol Nurs 2003;20(4):159-174). These challenges may occur during or after treatment. When a child can make a prompt return to school, having social support from teachers and classmates can help improve his or her self-esteem and reduce depression and anxiety (J Pediatr Oncol Nurs 2003;20(4):159-174, Pediatr Blood Cancer 2015;62(S5):S805-S817).
Parent Coping Mechanisms
Parents of a child diagnosed with cancer need to cope with the diagnosis and be the child's primary support system. Children are affected by the parents' coping behavior. Parents who have a strong family support system, positive marital relationship, optimistic behavior, reduced concurrent stressors, and open communication are associated with good coping outcomes among childhood cancer survivors (J Pediatr Oncol Nurs 2003;20(4):159-174).
Similarly, poor parental coping mechanisms are associated with poor outcomes of young cancer patients. Parent behaviors-such as agitation, stress, anxiety, denial, criticism, apology, and high levels of reassurance during or after painful medical procedures-lead to higher levels of patient distress (J Pediatr Oncol Nurs 2003;20(4):159-174). Physicians advocate for an honest and direct approach in communicating with patients, even at a young age. Evidence shows a more positive adjustment in patients who are informed about their diagnosis. These patients, even those who are young, show lower levels of anxiety about treatments, less defensive posture, closer relationships with parents and caregivers, lower psychological distress, and basic satisfaction with the self (J Pediatr Oncol Nurs 2003;20(4):159-174).
Addressing Barriers
Outside the standard audiologic interventions (i.e., hearing aids and cochlear implants), numerous solutions are available to help health care providers manage these barriers with patients and their families. Adopting a multidisciplinary team approach can help during diagnosis, treatment, and follow-up. The treatment team should include physicians, social workers, therapists (including speech-language pathologist, occupational therapist, physical therapist), audiologists, and educational liaisons. With this multidimensional approach, the providers can focus on treating the patient while ensuring that the patient's quality of life is supported (JAMA Oncol 2015;1(4):423).
ASCO and the American College of Surgeons have recommended integrating early palliative care into standard therapy to improve patient's quality of life, for both adults and children (JAMA Oncol 2015;1(4):423). Palliative care is frequently confused with end-of-life and hospice care, but this specialized medical care plays a larger role in managing all chronic disease states, not just terminal illness.
Palliative services include, but are not limited to, symptom control, psycho-social support, and impairment-driven rehabilitation measures. Palliative care promotes emotional and physical functions, and supports quality of life across the chronic disease continuum affecting both adults and pediatric patients. It also includes psychosocial and rehabilitation services. Figure 1 shows a multidisciplinary team approach with integration of palliative care services to help balance the need for survival prolonging therapies and improved quality of life (JAMA Oncol 2015;1(4):423).
As pediatric cancer treatment continues to improve-with patients living longer with their chronic illnesses or being cured altogether-young cancer patients and their families need additional support when re-integrating into the community post-treatment. From an audiological perspective, the main barriers to a successful recovery include ototoxic hearing loss, the impact of hearing loss on speech and language, school reintegration, and parent coping mechanisms. Aside from referral to audiology and ENT services for standard hearing loss interventions, establishing a multidisciplinary team to also include other physician specialties, social workers, therapists, and educational liaisons help to improve the quality of life of patients and their caregivers.
BRITTNEY SPROUSE, AUD, is Director of Audiology and a pediatric audiologist at the University of Chicago Medical Center. She also serves as President of the Illinois Academy of Audiology. MICHELLE KETCHAM GOZDECKI, AUD, is a pediatric audiologist at UChicago Medicine.