Adolescents with Sickle Cell Disease in a Rural Community: Are They Ready to Transition to Adulthood?

Key Points The transition to adult health care and adulthood in general poses many barriers to adolescents and young adults with sickle cell disease in a rural community. Identification of individual needs in the transition process and the engagement of community-based stakeholders can allow for the creation of locally based transition services. A transition program based in the medical home can assist adolescents and young adults in various domains to ease the transition to adulthood. In 1973, the median lifespan for patients with sickle cell disease (SCD) was estimated to be 14 years, with fewer than 20% of patients surviving beyond age 30.1 Forty years later, the median survival nears 50 years of age, with nearly all patients living to adulthood.2 SCD is no longer a “disease of childhood” but instead is a progressive, multisystem disease of chronic organ failure. In addition to the medical complications, patients often have compromised socioeconomic resources and a high incidence of neurodevelopmental disabilities that can affect school performance, employment, and other demands of independent adult life.3 Although life expectancy is improving, patterns of morbidity and mortality have been changing, with the young adult population appearing to be particularly vulnerable to increasing health complications and unexpected death. Compared with patients of other age groups, patients with SCD aged 18 to 30 years have higher acute care utilization and 30-day readmission rates.4 Mortality rates, although demonstrating decreasing trends in all childhood ages, now sharply increase after 19 years of age.2,5 Furthermore, the majority of deaths occur close to the time when patients transition to an adult healthcare provider, with the mean time between transition and death being less than 2 years.2 The increase in morbidity and mortality rates in the early young adult period highlights the need to conduct a careful and coordinated transition to adult health care and adulthood in general for adolescents and young adults with SCD. Accomplishing such a transition can be challenging, with patients, families, and providers facing numerous barriers, including the cultural differences between pediatric and adult medicine, lack of communication among providers during the transition period, adult providers’ knowledge of disease processes, and the maintenance of health insurance coverage.6 Patients in a rural location may face an even greater number of barriers, including a lack of reliable transportation and lack of access to an adult healthcare provider.7 The objective of this study was to define transition barriers for rural adolescents and young adults with SCD to develop a comprehensive program for their transition to adulthood and adult medical care. Methods The study was based in a South Carolina rural pediatric practice in collaboration with a tertiary care center. This rural practice is located in a county with a population of approximately 60,000, with 33% identified as black or African American. The primary city in the rural county in which the pediatric practice is located has a population of 9000, 57% of which is black or African American.8 The rural practice is located approximately 70 mi from the partnering tertiary care center and 125 mi from the other closest tertiary care center in the state. A community-based advisory committee was formed and included physicians from both the local area and academic center, nurses from a local community hospital, a community social worker, psychologists from the community and the county school district, a banking/finance representative, and a representative from the South Carolina Department of Health and Environmental Control. The committee assisted in the development of the needs assessment survey and provided insight into and access to resources to overcome identified barriers. After reviewing the literature, including previous surveys developed by Telfair et al9 and receiving input from the members of the committee, we created two surveys to identify barriers to transition. One survey was conducted in adolescents with SCD aged 13 to 19 years living in the catchment area of the local pediatric practice, and the other targeted young adult patients with SCD aged 20 to 28 years from the same area. The adolescent survey was piloted for clarity of questions before full administration. Adolescent participants were patients with SCD between the ages of 13 and 19 years who live in the rural practice catchment area and receive care at either the rural pediatric practice or the SCD clinic of the tertiary care center. Participants were initially identified by provider recall. International Classification of Diseases-9 codes for sickle cell anemia and variants (282.63 sickle-cell/HB-C disease without crisis, 282.41 sickle-cell thalassemia without crisis, 282.60 sickle-cell disease unspecified, and 282.68 other sickle-cell disease without crisis) also were used to identify any additional patients receiving care at the rural practice who met criteria for involvement in the study. The adolescent survey asked participants to rate their perceived ease in managing aspects of transition on a five-point scale (A, with great difficulty; B, with some difficulty; C, neither easy nor difficult; D, fairly easily; E, very easily). Topic areas included medical knowledge, communication with doctors and navigation of the healthcare system, and living as an adult. Participants were asked to identify their source of information in the transition topic areas, with choices including parents/family, doctors, other patients with SCD, the Internet, and their spiritual community. Participants also were asked to self-assess the severity of their illness (mild/moderate/severe) and to identify whether transition to adulthood had been discussed by a physician (yes/no/don’t know). Finally, basic demographic information was collected, including age, sex, grade completed, insurance information, and frequency of periodic visits per year. The adolescent survey was administered in the clinical space in which the adolescent was being seen, and a research assistant was available to assist or read questions if needed. Parents or other family members also were permitted to assist the adolescent in completing the survey. Young adult participants were patients aged 20 to 28 years with SCD who were previous patients of the rural pediatric practice still living in the local catchment area whose current contact information was known or able to be obtained by the rural pediatric practice. Participants were identified via provider recall and International Classification of Diseases-9 codes as previously described for adolescent participants. Initial contact with participants came as an informative letter about the study followed by a telephone call to assess willingness to participate. For those agreeing to participate, the survey was mailed with a self-addressed stamped envelope for return. The young adult survey included topics similar to the adolescent survey but with questions framed to assess actual barriers encountered in the transition to adult health care and adulthood in general. The survey also asked participants to identify sources used to help overcome these barriers. Answer choices were identical to those in the adolescent survey with the additional option of “I didn’t know who to ask.” Participants were asked to rate their satisfaction with the transition process (very satisfied/somewhat satisfied/not satisfied), whether a transition program would be of benefit (yes/no), and suggestion as to services that a transition program should provide. Young adults also provided a self-assessment of severity of illness (mild/moderate/severe) and basic demographic information. Descriptive statistics were performed using SAS version 9.3 (SAS Institute, Cary, NC). This project was approved by the institutional review board of the Medical University of South Carolina. Results The adolescent survey was completed by 13 subjects. Demographics and self-assessment of illness severity are presented in Table 1. Upon receipt of survey data and evaluation of the distribution of answers, it was decided to dichotomize responses related to the assessment of transition barriers to identify all topics that adolescents believed they could not manage at least “fairly easily.” In the domain of transition to adult medical care, many adolescent respondents reported they could not at least “fairly easily” leave their pediatrician and see a doctor who caters to adult patients (62%), find a doctor who caters to adult patients (54%), use an adult emergency department (46%), or prepare for admission to a nonpediatric hospital (46%). Barriers in medical knowledge were identified as difficulty with discussing anemia and knowledge of how SCD differs from other types of anemia (54%). In consideration of the practical domains involved in the transition to adulthood, adolescents did not believe that they could at least “fairly easily” arrange transportation (54%), know how to pay for medications (46%), make a personal budget (46%), or understand or apply for medical insurance (46%). The majority had not or did not know whether their doctor had discussed their needs as they transition to adulthood (67%). A majority (85%) of adolescents believed that they could at least fairly easily graduate from high school, obtain an education after high school, and find a job. Furthermore, 70% of adolescents believed that they could at least fairly easily keep a job. Adolescents overwhelmingly selected parents/family as their source of information, with their sickle cell physician and pediatrician also selected as sources. Table 1 Patients’ demographics The young adult survey was completed by five subjects. The majority of respondents encountered difficulty leaving the doctors they knew the best (100%), finding a physician who caters to adults (80%), and keeping a job (60%). It is notable that 40% of the young adults surveyed were unemployed and another 20% were disabled and not working. Young adults also identified knowing what to expect in transition (40%) and knowing how to pay for medical care (40%) as being encountered barriers. Comparable results from the adolescent and young adult surveys are summarized in the Figure. Fig Areas of transition difficulty for adolescents and young adults with sickle cell disease (SCD) in a rural community. Two-thirds of young adult respondents were not satisfied with how their transition difficulties were managed and all believed that a transition program for youth with SCD would be helpful. Services suggested for a transition program to provide are listed in Table 2. The majority of young adults identified parents/family as sources of information, although 40% reported that they did not know whom to ask. Table 2 Young adults’ suggestions for services an SCD transition program should provide Discussion The transition to adulthood can be a challenge for any adolescent but may be especially so for adolescents with a chronic disease in rural areas, where resources may be scarce. To the authors’ knowledge, this is the first study to target a rural population of patients with SCD and identify the challenges that may be faced in their transition to adult health care and adulthood in general. Identifying barriers to transition can not only allow for tailoring of individual needs in transition but also can be a means to engage adolescents in the transition process, making them an integral part of how transitions are managed. The present study provides information on the barriers that are anticipated by adolescents with SCD and assesses the barriers that were actually encountered by recently transitioned young adults. Although the constructs of the surveys do not allow a direct comparison of all of the results, it is interesting to note where the responses aligned and where they diverged. Both adolescents and young adults identified that leaving their pediatric healthcare team and finding a physician catering to adults were difficulties associated with transition. Both groups also identified concerns about paying for medical care. These concerns are similar to those noted in a national survey of adolescents and young adults with SCD by Telfair et al, which, using the surveys that served as a foundation for those used in the present study, also revealed concerns about paying for medical care and leaving their pediatric provider.9,10 Similarly, in a study of adolescents with SCD by Smith et al, primary concerns regarding transitions centered on leaving providers who know and understand their illness and getting to know new providers who may not have as much experience with SCD.11 A transition process that is initiated early and provides a multidisciplinary team approach, including social work and case management, can improve patient preparation and comfort with the process, allow for appropriate communication with adult providers, and ensure that all of the resources available to the patient are accessed. Although adolescents in the present study did not anticipate concerns with finding and keeping a job, maintaining employment was a barrier identified by many of the young adult respondents. This finding highlights that although it is important to determine the needs that adolescents can identify, there are challenging areas they may not anticipate that are equally important to address. High unemployment rates for patients with SCD are well known and difficulties with employment are probably multifactorial, including lost time for illness, chronic pain limitations, and psychosocial components.12,13 As such, although employment was not identified by the adolescents participating in this study as an anticipated barrier, vocational preparation should be an important component of any transition program for youth with SCD. Finally, the majority of adolescents either had not discussed or did not know whether they had discussed their needs in transition with their physician and 40% of the young adult respondents believed they did not know what to expect in the transition process. In addition, adolescents in this survey identified anticipated barriers in nonmedical domains of the transition to adulthood, including concerns about transportation and discomfort with financial considerations. This emphasizes the need for formalization of a transition process that is initiated by the provider and addresses concerns identified by the adolescent to more fully prepare these young patients for their transition to adulthood. This study is limited by a small sample size. The study involved only one practice in one rural community and does not encompass all adolescent and young adult patients in that community with a diagnosis of SCD. The goal of this study, however, although allowing a general assessment of how adolescents and young adults view transitions, was primarily to determine individual needs in the transition process that can be addressed through the medical home. The formulation of plans that address personalized concerns in the areas of medical knowledge and social skills such as banking and transportation allows for a more fully encompassing transition plan to be developed for each individual. Although a limited geographic area was used in this study, the barriers described here are likely similar to those found in other rural locations that are a significant distance from a tertiary care or subspecialty center, have limited or no public transportation access, and have few adult medical providers who are experienced in managing less common pediatric-onset conditions. With the knowledge gained from this survey, the adolescent study participants are the pilot patients entering a locally based SCD transition program. This program targets adolescents who are near the age of transition to adult health care, is centered in the patient’s medical home, and draws resources from the program’s community-based committee to address the specific needs of each individual. Emphasizing the provision of care in a medical home for patients with special healthcare needs has long been seen as a core concept and recent evidence supports the benefits of a medical home in the transition process.14–16 This transition program will collaborate with specialty care at a tertiary care center but will be uniquely providing transition services based in the primary care office in the patient’s home community. Focusing on transition at the local level will likely encourage greater engagement by patients and families, improved access to resources known locally, and deeper investment from local stakeholders. Conclusions Adolescents and young adults with SCD in a rural population identify numerous barriers to successful transition to adult health care and adulthood in general. Adolescents with SCD are not prepared to care for themselves or manage their own health issues and would benefit from a comprehensive transition program. Addressing transition barriers is a crucial component to ensure the ongoing health of this vulnerable population. Coordination of care at a local level may help ensure that patients have the preparation necessary to proceed to the next stages of their lives.

Screening People in the Waiting Room for Vestibular Impairments

Key Points A description of true vertigo (the illusory sensation of self-motion rather than a vague sense of dizziness) should be considered when taking the history. Dix-Hallpike maneuvers should be performed during office screening. Romberg standing balance tests on foam with eyes closed are somewhat useful; head impulse tests are less useful for screening. Few studies validate screening tests of the vestibular system in the absence of the lengthy, expensive, and equipment-intensive battery of objective diagnostic tests (ENG battery). The ENG battery often includes low- and high-frequency sinusoidal rotatory chair tests of the vestibulo-ocular reflex (VOR); bithermal water caloric tests; Dix-Hallpike maneuvers (D-H), and other positional tests of the VOR; vestibular evoked myogenic potential tests; and tests of saccadic, smooth pursuit, and optokinetic eye movements. When an ENG battery is unavailable or too expensive or if the physician wants to determine whether the ENG battery is necessary, a screening battery with head movements, D-H, and balance tests may be used in conjunction with the health history, as was done in a study to determine whether people with human immunodeficiency virus/acquired immunodeficiency syndrome are at increased risk for vestibular disorders.1 The D-H is a widely known test,2 performed by turning the patient’s head toward the side and having the patient rapidly lie supine with his or her neck hyperextended. Eye movements (nystagmus) are easily observed when recorded with infrared video oculography (VOG). A classic, positive response on this test (nystagmus beating toward the test side) is pathognomonic for benign paroxysmal positional vertigo.3,4 The head impulse test (HIT) is widely used for screening the horizontal VOR. The examiner sits in front of the subject, instructs the subject to stare at the examiner’s nose, and briskly rotates the head approximately 20° left or right in yaw rotations, stopping suddenly and approximating a step of velocity. The response is positive if the examiner observes a saccade contralateral to the head movement. A positive response may be consistent with impaired horizontal semicircular canal function.5–8 One version of the Romberg test for standing balance, the Clinical Test of Sensory Interaction and Balance (CTSIB), is used widely among clinicians.9,10 The patient stands with feet together on the floor or on compliant foam with eyes closed. The condition on foam with eyes closed is considered a screening test for vestibular impairments, although testing with the head still has limited sensitivity and specificity.11 Younger (21–59 years old) and older (60 years old and older) adults differ in the time taken to perform the test, and sensitivity and specificity improve when the head oscillates in pitch at 0.33 Hz. The ability to perform five or more pitch rotations of the head during the test is consistent with normal vestibular function.12 We used both head still and head pitching conditions. The goal of this preliminary study was to determine whether the results of a screening battery accurately predicted the outcome of the ENG battery. Volunteers took the screening battery and some people then agreed to take the ENG battery. Methods Subjects Initially we recruited 300 volunteers, 21 to 79 years old, from the waiting room of a primary care clinic and from staff and other visitors to our institution. All were ambulatory without the use of gait aides. Exclusion criteria included a history of known otologic disease or surgery; use of vestibular-suppressant medication (eg, meclizine, diazepam, benzodiazepines); musculoskeletal conditions that prevented independent gait; recent surgery if the surgeon had not yet approved return to full activities; and history of significant neurologic disease (eg, stroke, Parkinson disease, multiple sclerosis, lower extremity peripheral neuropathy, cerebellar disease, dementia that precluded giving informed consent). Because CTSIB performance does not differentiate vestibular disorders from presbystasis or various neurologic conditions, and many people have more than one disorder, we relied on subjects’ histories to rule out nonvestibular disorders. Subjects reported their age, sex, and history of vertigo. Except for one subject with a strongly positive D-H response, participants were not told whether their screening results were normal or abnormal. Subsequently, 69 subjects volunteered to visit the diagnostic laboratory for the full ENG battery. Other subjects declined the ENG battery because of the time needed for the battery (2.5 hours), the need to take the tests at another time, the inconvenience traveling between campuses, or lack of interest. All of the subjects gave informed consent before participating in the screening. This study was approved by the institutional review board for our institution. Data were collected between June 2011 and November 2011. Screening Battery The 15-minute screening battery included HIT, D-H, and tests of standing balance using the modified CTSIB, given in a private room in random order. Screening tests were performed by one of three laboratory technicians who had 8 to 25 years of experience in research and diagnostic testing with patients suspected of having vestibular disorders. In advance of performing HIT, the examiner determined that the subject had at least 30° of yaw cervical range of motion. This brief examination also allowed the subject to become used to having the examiner move his or her head passively. Then the examiner gave the subject two trials of HIT to each side. A response was considered positive if the examiner observed a saccade on both trials to one side. D-H was performed on a stretcher with the wheels locked and on a firm foam mattress. Eye movements were recorded with infrared VOG. A positive D-H response was defined as three or more beats of nystagmus with a latency to onset of at least 2 seconds. A classic response was defined as having quick phases up-beating, with horizontal and torsional components beating ipsilaterally.3 A positive but nonclassic response was defined as any other response with three or more beats of nystagmus. Subjects also were asked whether they had vertigo, defined as the illusion of self-motion. CTSIB trials were given for a maximum of 30 seconds, in the same order, in increasing order of difficulty, and with feet together and arms crossed. Tests were given on the floor before being given on 10-cm, medium density, compliant Sunmate foam (Dynamic Systems, Leicester, NC). Tests were given with eyes open before being given with eyes closed. Tests were given without augmented head motions (head still) before being given with pitch head rotations (pitch). For tests given with head pitch, the 0.33-Hz frequency of head movement was cued with the use of an iPod (Apple, Cupertino, CA) portable music player, attached to desktop amplifiers or an earbud earphone, and played at a comfortable intensity level. Every subject wore a 28.3-g inertial motion sensor (Xsens North America, Los Angeles, CA) on a plastic band atop his or her head. A single data acquisition program (LabView, National Instruments, Austin, TX) collected time-synchronized data from the inertial motion sensors, which were sampled at 100 Hz. Data were used to verify the trial duration and the number of head movements during head still and head pitch CTSIB trials. CTSIB trials had two dependent measures: time (trial duration) and number of head motions the subject could make during the test. Time scores were considered abnormal if the trial duration was <1 standard deviation of the age-based scores for “normal” people 21 to 59 years old and 60 to 79 years old, and head nodding was considered abnormal if the subject made fewer than five head motions during the trial. These normative values were based on previously collected data from our laboratory.12 ENG Battery All of the subjects were invited to undergo the standard clinical ENG battery used at our institution. The laboratory at which the ENG battery was given is located in the Department of Otolaryngology, in a separate facility, 3.54 mi away; therefore, subjects would have to travel between campuses to have undergone the ENG battery on the same day as the screening battery. Because of the distance, the time needed for the ENG battery, and the possibility of nausea elicited by bithermal water caloric testing, patients and research subjects must avoid eating for 3 hours before testing. As such, ENGs usually are scheduled in advance. Subjects were tested within 1 month of the day of screening, as their schedules allowed. The ENG technician was blinded to the outcome of the screening tests. Before beginning the ENG battery, the technician ascertained that the subject had not undergone a change in health status. The ENG battery included cervical vestibular-evoked myogenic potentials (VEMP); low-frequency sinusoidal tests of the VOR in darkness in the horizontal plane using the computerized rotatory chair (Contraves-Goertz, Thermo Fisher Scientific, Waltham, MA) at 0.0125, 0.05, and 0.2 Hz ± 60°/second, while eye movements were recorded with electronystagmography; and tests of smooth pursuit and saccades using a light bar; D-H, positional tests, and bithermal water caloric tests while eye movements were recorded with infrared VOG as the subject lay on a firm, padded examination table. Because test results were interpreted by the neurotologist who reads all of the ENGs from the laboratory, he was blinded to the study. If the ENG results were abnormal, then the neurotologist summarized the response pattern as nonlocalizing, peripheral vestibular unilateral weakness; central impairment; superior canal dehiscence; or benign paroxysmal positional vertigo. We were concerned with finding any possible abnormality that may indicate a vestibular impairment, not with specific diagnoses; therefore, for the purposes of this study, the summary result of the overall ENG battery was scored as either normal or abnormal. Subjects were provided with copies of their test results and, if results were abnormal, subjects were advised to consult their physicians. Statistical Analysis We compared patients with normal and abnormal ENG scores by t tests for continuous variables and χ2/Fisher exact tests for grouped variables. Logistic regression was used for the estimation of odds of abnormal ENG by screening test result. Adjustments were made for potential confounders, specifically age and history of vertigo, by including those variables on the regression model. P < 0.05 was considered statistically significant. Results Although we recruited 300 volunteers for the screening battery, 231 of them declined to return for the ENG; 156 of the initial subjects who declined the ENG had abnormalities on at least one subtest of the screening battery. The rest of this section describes only the 69 subjects who had both the screening battery and the ENG. The age of subjects with normal ENG results approached being significantly less than subjects with abnormal ENG results (P = 0.07). Every 1-year increase in age increased the odds for ENG by 4% (odds ratio [OR] 1.04). ENG results were not related to subjects’ sex. History of vertigo was four times more prevalent among subjects with abnormal ENG scores than among subjects with normal results (OR 4.0), although this difference did not reach statistical significance (P = 0.12), possibly because of the small sample size (Table). Table Odds for abnormal ENG results by patient characteristics at screening D-H Maneuvers During the screening, 40% of the 69 subjects had positive responses to D-H. One response was classic; the other responses were nonclassic. During the ENG, 35% to 39% of subjects had positive responses on D-H in supine and sitting, respectively. Abnormal D-H (supine) responses were more than three times more common in subjects with abnormal ENG compared with subjects with normal ENG results (OR 3.23; Table). The results approached significance univariately (P = 0.09), but after adjustment for potential confounders (OR 3.2, P = 0.11), they were not statistically significant, probably because of the relatively small sample size. These results suggest that in a larger sample the results may have been significant. CTSIB The percentage of subjects who took the ENG and who had abnormal CTSIB pitch nodding scores did not differ between groups with normal and abnormal ENG scores (Table). On CTSIB with head still and with head pitch, the time scores were not related to the total ENG score (P = 0.18 and P = 0.62, respectively). The score on CTSIB with head still was significantly related to having a normal/abnormal score on VEMP when adjusted for age (P = 0.04), but CTSIB with head pitch was not significantly related to VEMP. The score for nodding during CTSIB, either <5 or ≥5, was examined. The age-adjusted relation between the score on VEMP and CTSIB approached significance for head still (P = 0.09), but it was not significant for head pitch (P = 0.68). For subjects younger than 50 years, no relation was found between VEMP or the total screening score (TSS) and nodding during head still or head pitch. For subjects older than 50 years, no relation was found between nodding and VEMP or the TSS during head still; however, the relation approached significance during head pitch (P = 0.08). When adjusted for age, no relation was found between the total ENG score and the nodding score with head still or head pitch. HITs Only one subject in the abnormal ENG group had a positive HIT (Table). The total HIT score was not related to the total ENG score (χ2 0.38, P = 0.99); the rotatory score (P = 0.99); or the caloric score (P = 0.21). Of the 20 subjects with abnormal scores on caloric testing and/or rotatory chair testing, only that one subject also had a positive HIT. History The association between a history of vertigo and a positive finding on the ENG battery approached significance (P = 0.12). Having a history of vertigo was not related to having a normal or abnormal subtest response including VEMP (P = 0.16), bithermal water caloric tests (P = 0.25), or rotatory chair tests (P = 0.85). The ENG was abnormal among 89.5% of subjects with a history of vertigo, but among only 68% of subjects without a history of vertigo (Table). TSS The total screening battery also was scored as either normal or abnormal. No significant relation was found between the TSS and the total ENG score (χ2 0.19, P = 0.65), but the TSS was significantly related to the VEMP score after controlling for age (P = 0.02). No relation was found between the TSS and the score on bithermal water caloric testing (Wald χ2 0.68) or the rotatory chair test score (Wald χ2 0.80), after controlling for age for both tests. Discussion Several variables from the screening battery hold promise for use in future studies. History of vertigo had a particularly high OR. Responses to D-H testing—to the maneuver itself and to sitting up after the maneuver—also had high ORs. Somewhat lower ORs were found for CTSIB, but those responses, especially to time and nodding for the head still condition, are likely to be useful for screening. The absence of positive HIT responses suggests that this screening measure may have limited utility despite its popularity among clinicians. Subjects were recruited regardless of their histories of vertigo, although no subjects were actively vertiginous. The age difference between participants with and without histories of vertigo confirms previous findings.13 Similarly the finding that subjects with positive responses to D-H testing were older than subjects with negative responses was consistent with previous work showing that benign paroxysmal positional vertigo and central vertigo occur in middle and older age.14 We expected to find that a preponderance of subjects with positive, classic D-H responses would be female; however, the sample of people in that category was so small that the analysis by sex probably is not meaningful. The discrepancy between the screening D-H and the ENG D-H has several explanations. The slight differences in compliance of the surfaces used for testing may have had an effect. At screening some patients may have felt slightly unwell without having positive responses on the day of testing; however, that sense of being unwell may have been their reason for volunteering for the ENG. During routine clinical testing patients sometimes have negative responses when tested initially but have positive responses on another day because of the variability of vestibular responses. The lack of a statistically significant relation between the ENG and screening results was probably the result of the relatively small sample size. Most components of the ENG battery evaluate the VOR elicited by stimulation to the lateral semicircular canal or to the superior branch of the vestibular nerve. The HIT is approximately two sharp ramps of increasing and then decreasing head velocity with peak velocities of 150°/second to 350°/second and accelerations of 4000°/sec2 to 5000°/sec2.6,7 Bi-thermal caloric testing approximates low frequency VOR testing at 0.003 Hz and 0.58°/sec2.15 Although some studies have shown that patients known to have unilateral weakness have impaired responses to HIT6,7 other investigators have suggested that the test sensitivity is low, 0.34 to 0.63 .16–18 All of those studies had small samples and as such, the sensitivity of HIT remains unclear. The velocity profiles of the stimuli in the ENG battery and the head impulse are different. We gave rotatory tests at three frequencies, 0.0125, 0.05, and 0.2 Hz, none of which matched HIT. These differences may account for the lack of relation between tests, but this point remains to be clarified. Age, history of vertigo, D-H supine responses, CTSIB still time, CTSIB still nodding (ie, number of head rotations), and CTSIB pitch nodding had ORs that approached significance. As such, those variables may be useful for screening although this suggestion should be tested and confirmed in larger, adequately powered studies. Except for D-H tests, we cannot state with assurance which screening variables are directly related to particular ENG variables. The VOR tests and CTSIB measure different constructs. Balance testing (eg, CTSIB) is more likely to detect abnormalities associated with the vestibulospinal tracts, which receive their input primarily from the otoliths, including both utricular and saccular inputs, although the vertical canals probably also contribute.19,20 During CTSIB with pitch, vertical canal signals also must be involved. The ENG battery did not include the relatively new test of ocular VEMP21–23 because standard clinical procedures are not yet well defined. Ocular VEMP may have shown a relation to CTSIB performance. The comparison between the screening and ENG results may have been affected by a sampling bias caused by the smaller sample of subjects who had the ENG. Unfortunately, because of the constraints of time and funding, we could not continue to recruit until we had the planned complement of 300 subjects who had undergone the ENG. The strategy of recruiting subjects by asking strangers in a waiting room to participate may not have been optimal. Had the local physicians been able to encourage participation we may have recruited a larger sample; however, we may have had a bias of a different type.

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Commentary on “Adolescents With Sickle Cell Disease in a Rural Community: Are They Ready to Transition to Adulthood?”

The life expectancy of patients with sickle cell disease (SCD) has improved significantly. It is no longer a disease of childhood. Most children with sickle cell anemia (93.9%) and nearly all children with milder forms of SCD (98.4%) live to become adults.1 Significant numbers are older than age 50 and some are now living longer, into the seventh and eighth decades. Consequently, the importance of transitioning from a pediatric care provider to adult healthcare facilities has become a critical step in the healthcare management plan. In this issue of the Southern Medical Journal, Mennito and colleagues discuss the challenges and barriers to a successful transition process of adolescents with SCD in a rural community.2 Although life expectancy is improving, patterns of morbidity and mortality have been changing, with the young adult population appearing to be particularly vulnerable. The pattern of mortality varies with age. Unfortunately, the risk of death among those older than 20 years is not easily assigned to a single preventable cause.3 Several factors contribute to making the transition to adulthood difficult. In adolescents there is often a tendency to deny illness and reluctance to go to a new adult healthcare facility. Adolescents desire independence as adults but may not be ready to face new responsibilities for appointments and mandatory medications. They also need support to cope with issues such as contraception, family planning, and sexually transmitted infections. Insurance companies often seek groups of young healthy people, and therefore patients with chronic illnesses such as SCD frequently lose medical coverage when they become legally independent. Integrated transition programs can provide age-appropriate treatment and continuity of care. Support from a multidisciplinary team is important from both pediatric and adult facilities. All involved parties—patients, providers, and facilities—should agree beforehand on a plan for transition. Before this process begins,4 the idea of transition is introduced approximately 1 year in advance. Mennito and colleagues raise important questions about how to move forward in facilitating a seamless transition in the rural population of adolescents with SCD, where the barriers are even greater than in the urban population and tertiary centers. The purpose of this study is to determine the readiness of a rural SCD population for transition to adulthood and adult medical care, and this is the first study to target a rural population of patients with SCD. The objective of this study was to define transition barriers for rural adolescents and young adults with SCD to help develop a comprehensive individualized program for their transition to adulthood and adult medical care. The study was based in a rural pediatric practice in collaboration with a tertiary care center. The authors created two surveys to identify barriers to transition. One was conducted in adolescents with SCD aged 13 to 19 years who were living in the catchment area of the local pediatric practice, and the other targeted young adult patients with SCD aged 20 to 28 years from the same area. Adolescents reported difficulty leaving their pediatrician (62%) and knowing how SCD differs from other types of anemia (54%). They had concerns about finding an adult physician (54%), arranging transportation (54%), preparing to use an adult hospital (46%), and handling financial issues (46%). It was interesting to see that up to two-thirds of young adults were not satisfied with their transition and all believed a formalized transition program would be helpful. Similarly, both adolescents and young adults identified that leaving their pediatric healthcare team and finding an adult physician were difficulties associated with transition. In addition, adolescents in this survey identified anticipated barriers in nonmedical domains of the transition to adulthood, including concerns regarding transportation and discomfort with financial considerations. Both groups also identified concerns about paying for medical care. Although this study is quite limited by a small sample size and does not encompass all adolescent and young adult patients with a diagnosis of SCD in that community, it has highlighted challenges in a rural population, which are even greater than in the urban areas and tertiary centers. Strategies by which this can be achieved could include outreach programs and peer-led instruction and peer education, because these hold great promise as approaches that are adolescent centered.5 Mennito et al question how to move forward in facilitating a seamless transition process, and the notable findings highlight the importance of standardizing the transition process in a rural area. This can be addressed through the medical home and by drawing resources from the program’s community-based committee to address the specific needs of each individual.5 In conclusion, the authors have initiated important new research directions and requirements in a rural population as opposed to major urban areas. Optimal care should not be a ZIP code lottery; therefore, the coordination of local care and the standardization and development of transition at the local level will likely result in greater engagement by patients and families, improved access to resources known locally, and deeper investment from local stakeholders in the future.

Commentary on “Successful Use of Intravenous Dexmedetomidine for Magnetic Resonance Imaging Sedation in Autistic Children”

In this issue of the Southern Medical Journal, Ahmed and colleagues present their case–control study examining the effectiveness of intravenous dexmedetomidine for achieving deep sedation for magnetic resonance imaging (MRI) among 56 children with an autism spectrum disorder (ASD) compared with 107 children in the control group.1 The authors found that children with ASD could be sedated for MRI successfully using dexmedetomidine and found relatively few significant differences in response between the children with ASD and the control group. Overall, the results provide supportive evidence for implementing this approach in clinical practice and may be a useful guide for clinicians. This study is a valuable contribution to practice for several reasons. There is consensus that the prevalence of ASD is increasing, with data from the Centers for Disease Control and Prevention citing a prevalence of 1 in 68 children at age 8 years.2 The etiology of ASD is known to be diverse and substantial additional work is needed to understand the cause. Practice guidelines recommend neuroimaging in selected cases as indicated by examination or history.3 In addition, seizure disorders affect 20% to 25% of individuals with ASD,4 and intellectual disability affects approximately 31%,2 which also may prompt MRI.5 As such, the demand for neuroimaging with sedation among children with ASD is likely to continue and possibly increase. Given the often substantial behavioral challenges in this population, evidence-based approaches to effective sedation are needed. There are a number of significant strengths to this study. The authors have made use of a valuable database collected in the course of clinical care to share important information that may have great utility to other clinical centers. Diagnoses of autism were established by a neurologist, lending confidence to the validity of the diagnosis, which often is challenging. Careful inclusion and exclusion criteria were applied. A detailed description of the sedation protocol is provided, which included a highly standardized approach. The patient population is well characterized such that the reader can determine how similar or dissimilar this cohort is to his or her own patient population. A number of limitations to the study should be attended to by the reader, however. The diagnostic criteria for ASD based on the Diagnostic and Statistical Manual of Mental Disorders changed in 2013 with the release of the fifth edition,6 such that a number of the participants in this study who had a diagnosis of ASD would likely no longer meet the criteria. This change simply implies that the types of patients presenting with a diagnosis of ASD in the coming years will be different from those described in this study. This does not invalidate the results of this study, but the reader should be aware that future patients may not be comparable with those included in this study. An additional limitation that the reader should take into consideration is that the control group was not matched to the ASD group with regard to a number of characteristics. Specifically, the ASD group was older and were boys. Indeed, children with ASD are much more likely to be boys than girls and this prevalence reflects the characteristics of the disorder. The reader, however, must be aware that when the case and control groups are dissimilar, differences noted between groups may not result from ASD but from these other confounding characteristics. The authors have therefore appropriately controlled for sex, age, and weight in their analyses and it is these controlled analyses that should be attended to by the reader. Finally, it is important to note that although some comment can be made regarding safety, the small sample size of this study limits the ability to detect uncommon adverse effects of the medication. One may conclude, then, that there was no evidence of increased prevalence of relatively common adverse effects among the group with ASD as compared with controls; however, one cannot draw any conclusions about the prevalence of uncommon adverse effects because the study was not powered to detect these. In conclusion, this study provides valuable information regarding one institution’s experience with dexmedetomidine for sedation for MRI within the pediatric ASD population. The study provides supportive evidence for the effectiveness of this approach. Of course, the gold standard would be a randomized controlled trial, but in the absence of such data, the results of this study can be applied by clinicians to their own clinical practice while taking into consideration the appropriate caveats to interpretation of the study results.