The mission of Special Olympics (SO) is to provide year-round sports training and athletic competition in a variety of Olympic-type sports for children and adults with intellectual disabilities (ID), giving them continuing opportunities to develop physical fitness, demonstrate courage, experience joy, and participate in a sharing of gifts, skills, and friendship with their families, other SO athletes, and the community (23). ID is defined as an intellectual quotient up to 75. However, standardized testing that demonstrates limitations in one or more of the following adaptive behaviors also qualify as ID:
- - Conceptual skills including language, literacy, money, time, number concepts, and self-direction
- - Social skills including interpersonal skills, social responsibility, self-esteem, gullibility, naïveté (i.e., wariness), social problem solving, and the ability to follow rules/obey laws and to avoid being victimized
- - Practical skills including activities of daily living (personal care), occupational skills, health care, travel/transportation, schedules/routines, safety, use of money, and use of the telephone
The SO as a sports movement began in the backyard of Eunice Kennedy Shriver in 1962. Through her innovation, there are currently more than 4.44 million athletes and 1.3 million coaches and volunteers from over 170 countries actively involved in the movement. Individuals with ID aged 8 and older compete in approximately 81,000 yearly competitions involving 32 different Olympic summer and winter sports. As such, there are more than 222 SO events held daily (23)! Games are held at local, regional, state, national, and international levels. The upcoming World Games will be held July 2015 in Los Angeles, CA.
SO recommends that athletes undergo a preparticipation physical examination (PPE) at a minimum of once every 3 years. The optimal place to undergo this evaluation is in the athlete’s primary care physician’s office. However, many people with ID have difficulty accessing health care. As such, the Healthy Athletes discipline MedFest was created to meet this need. There are a total of seven SO Healthy Athletes programs — Special Smiles, Opening Eyes, Healthy Hearing, Fit Feet, Fun Fitness, Health Promotion, and MedFest. Healthy Athletes has performed free screenings of over 1.4 million athletes from over 120 different countries (20). For more details on Healthy Athletes screening programs, the authors refer you to Dr. Holder’s review located in this issue. However, this article will highlight MedFest — a station-based mass screening PPE, which can occur prior to or during SO games.
MedFest provides a free sports physical to potential or current SO athletes. Two of MedFest’s primary goals are the same as traditional PPEs: 1) screen for conditions that are potentially life threatening or disabling and 2) screen for conditions that may predispose the athlete to injury or illness (2). Additionally, MedFest also strives to educate athletes about health issues and teach medical professionals about people with ID. As such, MedFest serves both as a recruitment and retention tool for both SO athletes and volunteers. Secondary goals include determination of general health status and provision of referrals to community health providers to care for abnormalities discovered during screening.
Each year, approximately 30,000 athletes are evaluated at a MedFest event. Analysis of these screenings has discovered that 22% of athletes had at least one previously undiagnosed medical condition and 25% were taking medications that could negatively affect their athletic experience. Identification of these issues highlights the potential impact a sports medicine provider can have on the overall health and well-being of SO athletes (20).
Common medical problems encountered in people with ID include motor impairment (20% to 30%), seizure disorders (15% to 30%), vision impairment (up to 25%), hearing impairment (up 32%), and behavioral disorders (15% to 35%). Additionally, if there is an underlying syndrome that has resulted in the ID, there are syndrome-specific primary and secondary associated disorders that need to be considered (21).
Basic Equipment Requirements
Table 1 shows equipment requirements. Pipe and drape or other partitioning devices should be available for patient privacy if a MedFest event is conducted in a large, open venue. SO International (SOI) has created standardized forms for the health history, physical examination, and referrals, which are available on the Web site (21).
Special Considerations in the Preparticipation Examination
Generally, the PPE for the SO athlete should follow the guidelines for other athletes. However, there are some specific areas of concern in this population, which are addressed below. MedFest events also specifically prohibit any type of examination that could be considered personally invasive, including hernia checks, genitourinary examinations, and direct palpation of femoral pulses.
Questions should be directed to the athlete whenever possible. Some SO athletes are willing and capable communicators. In cases where the athlete is unwilling or unable to communicate effectively or the reliability of answers is in question, a caregiver should be available to provide assistance. If available, review of previously completed PPEs may be helpful in gathering any important historical data.
While seemingly mundane, the medication review is one of the most important, challenging, and potentially time-consuming portions of the PPE. SO athletes utilize medications at higher rates than most other athletes (21). Possible adverse effects of the medications include QT prolongation increasing the risk of potentially fatal arrhythmia, impairments to bone health increasing the risk of fracture, photosensitivity increasing the risk of sunburn, and weight gain increasing the risk of obesity. SOI has created a helpful list of medications with these potential adverse effects, which is available on their Web site. (21) While this is a valuable resource, the authors discourage the exclusive use of this reference during the screening process. The rapid introduction of new pharmaceuticals and recognition of previously unknown adverse effects for existing medications make it difficult for any list to be maintained with the most up-to-date information. Additionally, athletes taking multiple medications may experience drug interactions not identified by this list. A commercially available smart phone or tablet application such as Epocrates, Lexicomp, or Micromedex can be used to check for these adverse effects, identify individual components of combination preparations, and perform a drug interaction check for athletes using more than one medication.
We do, however, encourage familiarity with classes of medications with these adverse effects. Tricyclic antidepressants (4) and fluoroquinolone antibiotics (9) may cause QT prolongation. Proton pump inhibitors, thiazolidinediones, prolonged use of corticosteroid preparations, and many anticonvulsants may adversely affect bone health, increasing the risk of fracture (9). Photosensitivity may occur with angiotensin-converting-enzyme (ACE) inhibitors, sulfonylureas, thiazide diuretics, and tricyclic antidepressants (9). Weight gain is associated with tricyclic antidepressants (9), antipsychotics (5), hormonal preparations, and prolonged use of corticosteroids.
The most common prescriptions for patients with ID are psychotropic and anticonvulsant medications, with anticonvulsants frequently used for mood stabilization and behavioral difficulties in addition to seizure disorders. A recent study of adults with ID in New York state found psychotropic medications used at rates of 43.1% to 65.4%, with the lowest rate found in those living at home and the highest in those living in intermediate care facilities. The same population used mood stabilizers, primarily anticonvulsants, at rates ranging from 6.9% to 30.6% (25). Similar data from Australia showed 41% use of psychotropic medications and 31% use of anticonvulsants in adults with ID (6), demonstrating this is not a prescribing tendency unique to the United States. It appears that adolescent use may be slightly less, with 20% use of psychotropics and 15% use of anticonvulsants reported in Australia (6). Of note, it appears that older typical antipsychotic medications have been frequently replaced with newer atypical antipsychotics (25). At least two medications from this class, quetiapine and risperidone, have a risk of QT prolongation (9).
The history form developed by SOI asks for the medical cause of the athlete’s ID and any diagnosed syndrome. If known, this is very useful information, as syndromes may be associated with specific medical conditions requiring further investigation. Unfortunately, a SO survey discovered that only 40% of athletes knew their underlying neurodevelopmental diagnosis (22).
The cardiac screening questions in the history section of the PPE are of utmost importance due to the frequency of cardiac defects in this population and the possible use of medications causing QT prolongation. Cardiac defects are especially common in Down syndrome (40% to 50%), fetal alcohol syndrome (29% to 41%), Fragile X syndrome (up to 52%), Turner syndrome (up to 50%), and Williams syndrome (up to 75%). While these five diagnoses are some of the most frequently seen syndromes in SO athletes, other syndromes, which have a risk of associated heart defects, are also encountered (Table 2) (21). Cardiac warning signs and symptoms include the following:
- - Chest pain during or after exercise
- - Dizziness during or after exercise
- - Fainting during or after exercise
- - Headache during or after exercise
- - Irregular heartbeat
- - Loss of consciousness
- - Racing heartbeat
- - Shortness of breath during or after exercise
- - Skipped heartbeats
- - Syncope or presyncope
- - History of previous heart disease (congenital or acquired)
- - Heart murmur grade 3/6 or higher or a murmur suggestive of hypertrophic cardiomyopathy (HCM)
In a small study of SO athletes, any one of the mentioned symptoms doubles the possibility of finding a cardiac abnormality, while three or more triples the likelihood (22).
Athletes with Down syndrome also have a risk of atlantoaxial instability (AAI). This disorder is present in approximately 15% of individuals with Down syndrome and results in instability of the atlantoaxial (first and second cervical vertebra) joint. Historically, athletes with Down syndrome were required to have cervical spine films with flexion and extension views prior to SO participation due to risk of spinal cord injury and death. These films only needed to be accomplished prior to initial participation and were not required to be repeated. However, asymptomatic AAI is not associated with adverse outcome, and as such, routine screening films are no longer recommended (3) or required. SO is currently in the process of implementing a new PPE form and new medical policies surrounding AAI.
However, symptomatic AAI (present in 7% to 13% of individuals with radiographic findings of AAI) is associated with catastrophic injury. The signs and symptoms of symptomatic AAI are the following:
- - Burner, stinger, or pinched nerve in the neck, arms, shoulders/hands
- - New difficulty controlling bladder or bowels
- - New numbness or tingling in the arms, hands, legs, or feet
- - New onset of torticollis (head tilt)
- - New onset or worsening spasticity
- - New weakness or paralysis of the arms, hands, legs, or feet
- - Recent change in coordination
- - Recent change in the ability to walk
If any of these are present, emergent neurosurgical referral is required (21).
Menstrual history is important to determine, as some developmental syndromes causing ID also include hypogonadism with resultant amenorrhea (14). This increases the risk for osteopenia and osteoporosis (17). In fact, decreased bone mineral density (BMD) occurs in both men and women with ID. Data compiled by SOI found that 20% of participants had low BMD (20). A more recent study using total body dual-energy x-ray absorptiometry (DXA) scans performed on Caucasian adults aged 28 to 60 with ID showed decreased BMD compared with that in a non-ID control group. Patients with Down syndrome were more severely affected, with all measured sites showing significantly lower BMD than non-ID controls and a mean total z-score almost a full standard deviation below the norm. The cause is likely multifactorial, related to decreased ambulation and physical activity, nutritional deficiencies, medication use, and genetic factors related to the cause of ID (13,14). In spite of higher rates of osteoporosis and fractures in ID, BMD screening levels appear to be low in this population (8). A meta-analysis of screening guidelines for osteoporosis in the ID population concluded that BMD should begin at age 45 (26); however, at least one guideline recommends beginning as early as age 19 in high-risk individuals (1). Of note, these guidelines currently are based on expert opinion and not on evidence.
Age, height, weight, temperature, blood pressure in each arm, pulse rate, and pulse oximetry should all be collected. SOI has created a referral form for adults for vital measurements falling outside normal parameters, and the authors encourage use of these guidelines for adult referrals. However, normative data for blood pressure and pulse rates in the pediatric population are significantly different. For blood pressures that are greater than or equal to the 90th percentile, recheck is recommended in 6 months. If greater than or equal to the 95th percentile, referral for further evaluation is recommended (10,24).
There is some debate whether blood pressure norms are the same in the ID and non-ID populations. In a study of people with Down syndrome, it was shown that blood pressures tend to be lower than that in the general population (7). Until more normative data become available in the ID population, we have little choice but to rely on general population norms for adults, children, and adolescents.
Body mass index (BMI) is calculated by kilograms/meters squared and therefore can easily be determined when the patient’s weight is reported in kilograms and height is in meters. Height should be recorded to the nearest 0.1 cm, and weight, to 0.1 kg. A normal BMI is between 18.5 and 25 kg·m−2, and measurements greater than 30 kg·m−2 indicate obesity. A referral for nutritional counseling should be considered for athletes with a BMI falling outside this range. A large data set examining obesity in the SO population was collected from 2005 to 2010 and involved over 6,000 adults participating in SO in the United States. The prevalence of obesity in males ranged from 33.1% to 45.5% depending on age and the year measured. These rates were high but are similar to those of the general population over the same time period. Women demonstrated higher prevalence, with rates of 46.4% to 56.7%, which tended to be higher than that of the general female population as well (12). Over the same time period, data collected from the SO population aged 8 to 11 showed high rates of obesity but again are similar to the general population data. However, in the age group of 12 to 18, the rates of obesity were noted to be higher than those in the general population from 2007 to 2010, suggesting that rates may be increasing (12).
Head, Eyes, Ears, Nose, and Throat (HEENT)
Oral hygiene and tooth decay is a significant problem in the ID population. Prior data gathered by SOI found that 39% of athletes had evidence of obvious untreated tooth decay (20). A study of New York City Special Olympic participants from 2005 to 2008 revealed that 28% of examined athletes had untreated caries and 32% had signs of gingival disease (11). Evidence of impaired oral hygiene should prompt a referral to the SO Special Smiles Program or a local dental clinic.
Since hearing disorders are more common in people with ID than the general population, audiology examinations were initially recommended at MedFest. It was later realized that this was better performed at Healthy Hearing events due to the large amount of ambient noise than often accompanies a MedFest event. Otoscopic examination is still recommended as part of the PPE, as cerumen impaction may be present in up to 1/3 of patients and is an easily correctible cause of mild hearing loss in this population (16).
Lea Symbol vision charts should be used for the screening visual acuity examination, as this does not require knowledge of letters or numbers. An athlete is considered to be functionally one-eyed if the best corrected vision in one eye is less than 20/40 (18). In such cases, both the American Academy of Ophthalmology and the American Academy of Pediatrics recommend mandatory eye protection (2), which can be provided free of charge at an Opening Eyes event. There is limited data regarding the prevalence of visual impairments in the SO population, but a small study involving 79 SO athletes with Down syndrome in Europe found ocular pathology in 32% of examined eyes and uncorrected refractive errors in 18% (15).
The emphasis of the cardiovascular examination should be detecting underlying cardiac defects and arrhythmias. Blood pressure is obtained in both arms to evaluate for coarctation of the aorta, which has a higher prevalence in Fragile X and other syndromes (21). The heart should be auscultated in both the supine and standing positions, as murmurs associated with HCM tend to be louder in the standing position due to decreased venous return (2). Referrals should be made for evaluation of an irregular cardiac rhythm, murmurs suggestive of HCM, cardiac murmurs rated 3/6 or higher, and suspicion of coarctation of the aorta.
Clinicians should remember that there is no upper age limit to SO participation. The authors have even cared for SO athletes in their 80s. As such, in addition to congenital heart disease, coronary artery disease (CAD) signs, symptoms, and risk factors need to be considered. Yet, it is unclear if the risk factors for CAD in SO athletes are the same as those in the general population. Although those with Down syndrome tend to have high rates of traditional risk factors for CAD such as elevated BMI, total body fat percentages, elevated triglycerides, elevated CRP levels, and decreased physical activity, the rates of CAD appear to actually be lower in this population (7).
Findings of hepatomegaly or splenomegaly are considered contraindications to sports participation until resolved or further evaluation and clearance is obtained.
Knowledge of the baseline neuromuscular examination is critical, as some athletes will have spasticity, range of motion limitations, or strength deficits as their norm. Caregivers should be utilized to assist in detection of any baseline deviations. Increase in spasticity may represent any underlying infection or unrecognized painful stimulus or simply inadequate spasticity management. Evaluation should include looking for signs of myelopathy, which if new may represent underlying AAI.
If the athlete uses any type of assistive device, these should be inspected to insure good repair and fit. This is essential to avoid injury to the SO athlete and other participants.
Clearance of the SO Athlete
At the conclusion of the MedFest experience, a final clearance status is granted to the athlete, which can be full clearance, full clearance with referrals, partial clearance with referrals, or no clearance pending further evaluation. The goal of MedFest is not to exclude SO athletes from participation but, instead, to find activities that are safe for them to participate in despite their medical and musculoskeletal problems. Historically, the majority of individuals who are not able to be cleared for participation in MedFest events are temporarily restricted due to failure to complete the required history section of the PPE form. This highlights the importance of not permitting individuals who have not completed the prerequisite paperwork to be screened. Of those with completed PPE history forms, complete sports restriction is only seen in 1% to 2% of athletes (21). This is due to significant cardiac defect or arrhythmia, symptomatic AAI, acute infection, acute musculoskeletal injury, hepatomegaly, splenomegaly, hypertension stage II or higher, or hypoxia. Those with significant cardiac abnormalities or symptomatic AAI and/or signs of spinal cord compression require appropriate evaluation and treatment by cardiology or neurosurgery, respectively, prior to clearance. SO athletes with an active infection or musculoskeletal injury are typically cleared for participation once the acute illness or injury resolves. Chronic infection, such as human immunodeficiency virus or hepatitis B, however, does not disqualify the athlete.
As previously mentioned, seizure disorders are common in SO athletes. During competition, seizure threshold may be decreased by increased adrenal activity, decreased sleep, elevated core temperature, electrolyte imbalances, and skipped doses of anticonvulsants. Pill omission may be accidental (forgetting to take the pills) or intentional for ergogenic purposes. In individuals with uncontrolled seizures, restriction of participation should be considered in sailing, swimming, diving, equestrian, downhill skiing, powerlifting, and cycling (19).
Although the majority of SO athletes receive full clearance, up to 85% may require some form of follow-up for one or more issues discovered during MedFest. Prior to the event, organizers of MedFest should have an established system in place for routine and urgent athlete referrals so as to efficiently address any issues that may arise. In addition to medical issues uncovered during the PPE (e.g., uncontrolled hypertension), the athletes may require referral for auditory, visual, dental, and/or podiatric pathology. Having multiple Healthy Athlete programs with open lines of communication at the SO venue is very beneficial in such cases. In the case of unknown underlying neurodevelopmental diagnosis, referral for evaluation for the underlying etiology of ID is important for overall athlete health, as the information gained has the potential to open doors to services that may otherwise be unavailable to the athlete. However, an unknown cause of the neurodevelopmental diagnosis should not by itself be considered disqualifying.
One distinct challenge of conducting a MedFest during SO games is the athlete who is disqualified from participation but has traveled from afar to compete. This emotionally charged situation can be difficult for the athlete, family, coach, and physician alike. However, athlete safety and well-being are paramount, and as such, clinician objectivity must be maintained. To prevent this from occurring, it is ideal to hold a MedFest prior to the scheduled competition with ample time to address any issues that may arise. When an athlete is disqualified and/or further care is required, the athlete or coach is given a referral form with the recommended follow up care. The program coordinator for the MedFest event should follow up with the athlete to make sure the recommended evaluations occurred.
Despite the challenges, participation in MedFest is a rewarding experience for athletes and providers alike. The authors challenge readers to join the SO movement by volunteering for local MedFest events and/or sideline coverage for competitions. It is a life-changing experience that will remind you of the joy that sport can bring to all people from all backgrounds and abilities.
MedFest is a free mass PPE screening for SO athletes that may be conducted as a free-standing event or during SO games. The goals of MedFest are to screen for conditions that are potentially life threatening or disabling or may predispose the athlete to injury or illness (2). Additionally, MedFest also strives to educate athletes about health issues and teach medical professionals about people with ID. As such, MedFest serves both as a recruitment and retention tool for both SO athletes and volunteers. Secondary goals include determination of general health status and provision of referrals to community health providers to care for abnormalities discovered during screening. The majority of athletes screened during a MedFest event will be cleared for sports participation, but many will require some type of referral for further care. It is important for the organizers of the MedFest to have prearranged protocols to ensure that the athletes efficiently receive the required evaluations.
Dr. Seidenberg is a paid consultant for Special Olympics, Inc., and serves on the Board of Directors for Special Olympics of Pennsylvania. He has received honoraria for writing articles for this journal in the past.
Dr. Eggers has nothing to disclose.
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