Nearly 20% of the population of the United States has some form of disability–that’s about 56.7 million people. Of those, more than 12 million need help with daily living.1 It can be a challenge, sometimes, to sort out if the reason for the 9-1-1 call is related to a patient’s disability or not. Although it’s not practical to provide guidance on every possible disability, what follows are some general suggestions for working with patients who have special needs or disabilities, and who are experiencing either an unrelated medical or traumatic emergency or an exacerbation/complication of their current condition.
The Basics
Use person-first language. Remember, they are “a person with autism,” or “a person who is blind,” not “the autistic man” or “the blind kid.” They’re a person first and primarily. You can also follow their lead and listen to how they describe themselves and use similar language, if it’s respectful to themselves and others.
Some disabilities, such as cerebral palsy or autism, may make it difficult to know quickly if the person also has an accompanying intellectual disability. When in doubt, assume cognitive competence unless told otherwise. But also expect that some behaviors and language usage might be below age level. Unless your patient is a child, don’t treat them like a child. Remember that they’re adults and treat them as such, which includes speaking directly to the individual and not talking about and around them with caregivers. But, also be cognizant of the value of family and caregivers and use them as assets. When they tell you something, believe them!
When speaking to individuals with an intellectual disability,
don’t raise your voice (unless they’re hard of hearing),
take your time and don’t overload them with words.
Also be prepared for a service animal. More than just guide dogs for people who are blind, service animals can be used for detecting oncoming seizures and hypoglycemia, as well as used for opening doors, carrying items, and comfort. The Americans with Disabilities Act, however, only recognizes dogs, and in some cases miniature horses (under 34 inches), as service animals. Therefore, although you may be confronted with a service monkey, parrot or ferret, the only service animal you’re required (and expected) to transport in an ambulance is a service dog or miniature horse.2
Perhaps the most important suggestion, however, is to remember that the chief complaint might be completely unrelated to the existing condition. Don’t get tunnel vision by assuming that the chief complaint is another manifestation of an obvious disability. Make sure you conduct a thorough assessment as you would with any patient.
There are common characteristics of disabilities that can aid in your assessment and transport considerations. Keep in mind, however, that individuals with disabilities are unique. Not all people will exhibit
all characteristics.
Autism
Autism is a disability that impacts how individuals communicate and interact socially with others. Characteristics include difficulty with both verbal communication and interaction, but also with the nonverbal components of language. These language difficulties can range from individuals being completely nonverbal to having very sophisticated and complex, but pedantic, language.
People with autism may have difficulty understanding and answering questions as well as recognizing figurative language, like sarcasm, irony or wordplay. They may also not recognize or understand nonverbal communication such as body language, facial expressions and tone of voice. This can lead to confusion and frustration for both the patient and the EMS provider.
People with autism can also experience repetitive behaviors and language where they might flick their fingers, twirl their hair, rock back and forth, or repeat words over and over. Hyper and/or hyposensitivity to environmental stimuli are also very common with people with autism. This means that they may react strongly to being touched or to sights and sounds, all things that happen frequently at an emergency scene.
Take care when starting your assessment. Move slowly and explain what you’re going to do before you do it. Limit physical contact as much as possible and consider conducting your exam distal to proximal. Because of possible sensory issues, keep in mind that adhesive tape, ECG electrodes, finger sticks and the blood pressure cuff can cause excessive anxiety or fear.
Although as many as 70% of individuals with autism also have a mild to severe intellectual disability, many have average or above-average cognitive abilities.3 Even if your patient doesn’t speak, talk to them as you would to other patients. There are, however, some things to keep in mind.
It’s important to use concrete or literal language and avoid figurative language. For example, it may be common in your area to ask a potentially diabetic patient if they “have trouble with their sugar.” This may confuse a person with autism as they’re thinking about the sugar in their kitchen that they use in their coffee.
As is good practice with all patients, be sure to ask permission before you touch your patient and tell them what you plan to do before you do it. Be ready to give an explicit explanation as to why the procedure is necessary. Because anxiety and confusion–as well as reactions to sensory stimuli–can be severe, sometimes resulting in your patient trying to leave the scene or refusing care or treatment.
Transportation in an unfamiliar vehicle to an unfamiliar location with unfamiliar sights and sounds can be scary for many patients, but completely terrifying for patients with autism. Consider allowing a familiar face in the ambulance during transport. Dim interior ambulance lights if it doesn’t interfere with your care, and consider not using sirens if your patient is noise sensitive. Make sure to explain what will happen during transport and once you arrive at the hospital. Don’t forget to notify hospital personnel about your patient’s concerns and be prepared for longer turnover. In other words, be your patent’s advocate.
Cerebral Palsy
Cerebral palsy is a neurological disorder caused by a chronic, nonprogressive brain injury that occurs during the pre- or post-natal period. It affects body movement, muscle coordination, balance and posture. In addition, people with cerebral palsy may have associated conditions such as intellectual disability, seizures, and vision or hearing loss.
As with autism, people with cerebral palsy may range from mildly affected to significantly impacted, including severe difficulty with speaking and controlling movement. People with cerebral palsy can have severe, seizure-like tremors of their torso and limbs, paralysis, paresis, contractures and/or rigidity (spasticity) of their limbs and joints, and difficulty with controlling their fine motor muscles to be able to speak clearly or at all, or swallow effectively and safely.4 These characteristics can make it difficult to conduct a complete assessment and determine the chief complaint.
You may have difficulty obtaining vital signs due to limb contractures or excessive movement. Consider obtaining vital signs manually instead of with the monitor to limit interfering movement. Remember that the monitor will pick up excess movement when doing cardiac monitoring or 12-leads
Because the patient may be unable to bend or straighten their limbs, be especially cautious when moving them to and from the stretcher. Ask them or their caregivers for the best way to lift and move them. Be sure to consider the patient’s position on the stretcher for comfort; don’t force limbs to move, and don’t forget to pad the voids.
Some patients with cerebral palsy will have chronic pain, paresis or paralysis, that result in injuries of which they aren’t aware. Consider this when conducting a trauma assessment.
Because more than 50% of people with cerebral palsy are also prone to seizures, be prepared for this and to treat if necessary.5
Make sure you also transport any equipment normally used by the patient (e.g., walker, wheelchair, communication devices) for use at the hospital and to facilitate discharge and travel home.
Spina Bifida
Spina bifida is a congenital birth defect where the spinal column doesn’t close completely during fetal development, resulting in the spinal cord exposed outside the body.6 Although the visible defect is typically corrected shortly after birth, the damage to the spinal cord prior to delivery can result in paresis or paralysis of the legs and torso. In addition, people with spina bifida can have Chiari malformations (i.e., brain tissue extending into the spinal canal), often requiring a cranial shunt, as well as bowel and bladder control problems, seizures, malformed feet, uneven hips and scoliosis.6,7 People with spina bifida also have an increased risk of latex allergy.8—10
As with cerebral palsy, people with spina bifida may not be aware they have a limb injury. Be aware of this as you conduct your assessment. They’re also at an increased risk for limb and spinal injury after a trauma, so give extra consideration to splinting and spinal precautions after a traumatic event.11
One study reports as many as 56% of individuals with spina bifida have a cranial shunt to treat hydrocephalus, a common comorbidity.7 Shunt malfunction happens in fewer than 50% of cases within the first year of placement but when it happens it can be life-threatening.7 Symptoms of a shunt malfunction include headache, visual disturbances, nausea, vomiting, drowsiness, bulging fontanel, clumsiness and cognitive decline.6 Shunt malfunction requires urgent transport to an appropriate facility. Pediatric patients should go to a pediatric hospital.
When planning for transport, the suggestions are similar as those for patients with cerebral palsy. Ask the patient or caregivers for the most appropriate way to move the patient safely and comfortably. Position carefully for comfort, remembering the patient may have limited-to-no control over their legs and feet, and bring mobility devices such as crutches, walkers and wheelchairs with you.
Intellectual Disabilities
In 2010, the terminology for this disability was changed in the U.S. from “mental retardation” to “intellectual disability,” although you may hear family members and caregivers still using the older term.12
An intellectual disability is defined as an IQ < 70 and concurrent deficits in adaptive behaviors, such as age-appropriate self-care and independence. Eighty-four percent of individuals with an intellectual disability have a comorbid mental health disorder or physical disability, or both, with a large majority also showing verbal, property and physical aggression.13 For example, people with Down syndrome are at higher risk for congenital cardiac defects, vision/hearing impairments, thyroid disorders, dementia and leukemia.14—16
People with an intellectual disability may have difficulty with communication, including limited speech and language; however, this isn’t true of all individuals. They may be susceptible to overstimulation, agitation, confusion or misunderstanding, especially when experiencing a medical or trauma emergency.
Some adults with an intellectual disability live at home with their families, but 75% live in sheltered housing, such as a residential group home, and are cared for by others.17 Due to the extensive mental and physical health needs of individuals with an intellectual disability, many family members who are also caregivers are exhausted and need respite. They may not understand the limits of the healthcare system. You may find yourself transporting a patient for what appears to be a break for the family. If the patient lives in a group home, there should be social, health and medical information on site.
You may have a difficult time sorting out the chief complaint or getting a complete history. Be prepared to ask about and address both medical and behavioral issues and encourage family or caregivers to stay with the patient to help with communication.
When speaking to individuals with an intellectual disability, consider your language usage. Don’t raise your voice (unless they are hard of hearing), take your time and don’t overload them with words. Listen to how the family or caregivers speak to the patient and adjust the complexity of your language. In addition, be sensitive to nonverbal cues indicating anxiety or concern and explain everything before doing it, even if they appear not to understand.
Sensory Impairments
Children and adults with blindness can have varying degrees of vision loss and may have limited vision even if legally blind. Provide a patient with limited vision as much verbal support as possible, being very sure to advise them every time you touch them, move them or perform a procedure. Children will be highly tactile so let them touch things before using them, if appropriate and practical, but keep in mind that young children will be more likely to put things in their mouths.
Many people who are considered deaf are able to hear something, but likely won’t be able to hear speech, especially in a moving ambulance. Some are skilled at lip reading, but it shouldn’t be relied on for clear communication. If they can read lips, don’t forget to face them when speaking to them. It seems obvious, but it’s very easy to turn away from someone while speaking without realizing it. If they use sign language as their primary means of communication, and they have someone who can interpret for them, consider allowing that individual to stay with them during transport. If they have a hearing aid or cochlear implant, watch for and don’t lose these devices.
Summary
The most important thing to remember when assessing and treating patients with disabilities is that they are people first. Use good assessment practices as you would with any other patient and use their knowledge, and that of their families, about their condition to help make good treatment decisions on their behalf.
References
1. Brault M. (July 1, 2012.) Americans with disabilities: 2010. Census. Retrieved Dec. 31, 2015, from www.census.gov/prod/2012pubs/p70-131.pdf.
2. U.S. Department of Justice. (July 12, 2011.) ADA revised requirements: Service animals. Americans with Disabilities Act. Retrieved Dec. 30, 2015, from www.ada.gov/service_animals_2010.htm.
3. Fombonne E. Epidemiological surveys of pervasive developmental disorders. J Autism Dev Disord. 2003;33(4):33—68.
4. Sewell M, Eastwood D, Wimalasundera N. Managing common symptoms of cerebral palsy in children. BMJ. 2014;349:g5474.
5. Anamaria T, Anca S, Cristian M, et al. Predictive factors in the onset of epilepsy in children with cerebral palsy. Acta Medica Marisiensis. 2015;61(3):200—205.
6. Matson M, Mahone E, Zabel TA. Serial neuropsychological assessment and evidence of shunt malfunction in spina bifida: A longitudinal case study. Child Neuropsychol. 2005;11(4):315—332.
7. Khalil A, Caric V, Papageorghiou A, et al. Prenatal prediction of need for ventriculoperitoneal shunt in open spina bifida. Ultrasound Obstet Gynecol. 2014;43(2):159—164.
8. Bernardini R, Novembre E, Lombardi E, et al. Risk factors for latex allergy in patients with spina bifida. Clin Exp Allergy. 1999;29(5):681—686.
9. Blumchen K, Bayer P, Buck D, et al. Effects of latex avoidance on latex sensitization, atopy and allergic diseases in patients with spina bifida. Allergy. 2010;65(12):1585—1593.
10. Bozkurt G, Sackesen C, Civelek E, et al. Latex sensitization and allergy in children with spina bifida in Turkey. Child’s Nerv Sys. 2010;26(12):1735—1742.
11. Szalay E, Cheema A. Children with spina bifida are at risk for low bone density. Clin Orthop Relat Res. 2011;469(5):1253—1257.
12. Diament M. (Oct. 5, 2010.) Obama signs bill replacing ‘mental retardation’ with ‘intellectual disability.’ Disability Scoop. Retrieved Dec. 30, 2015, from www.disabilityscoop.com/2010/10/05/obama-signs-rosas-law/10547/.
13. Crocker A, Prokić A, Morin D, et al. Intellectual disability and co-occurring mental health and physical disorders in aggressive behaviour. J Intellect Disabil Res. 2014;58(11):1032—1044.
14. Jensen K, Davis M. Health care in adults with Down syndrome: A longitudinal cohort study. J Intellect Disabil Res. 2013;57(10):947—958.
15. Glasson E, Dye D, Bittle AH. The triple challenges associated with age-related comorbidities in Down syndrome. J Intellect Disabil Res. 2014;58(4):393—398.
16. Roberts I, Izraeli S. Haematopoietic development and leukaemia in Down syndrome. Br J Haematol. 2014;167(5):587—599.
17. Woodman A, Mailick M, Anderson KA, et al. Residential transitions among adults with intellectual disability across 20 years. Am J Intellect Dev Disabil. 2014;119(6):496—515.
Resources for more information about special needs
Autism
Autism Speaks: www.autismspeaks.org/
Autism Society of America: www.autism-society.org/
Cerebral Palsy
My Child: www.cerebralpalsy.org
Centers for Disease Control: www.cdc.gov/ncbddd/cp/
Spina Bifida
Spina Bifida Association of America: www.spinabifidaassociation.org
Centers for Disease Control: www.cdc.gov/ncbddd/spinabifida
Intellectual Disability
American Association on Intellectual and Developmental Disabilities: www.aaidd.org
Centers for Disease Control: www.cdc.gov/ncbddd/developmentaldisabilities
Blindness
National Federation of the Blind: www.nfb.org
National Eye Institute: https://nei.nih.gov
Deafness
National Association of the Deaf: www.nad.org
American Society for Deaf Children: www.deafchildren.org
