Prehospital Assessment of Patients with Physical Disabilities

Emergency medical providers are regularly called upon to care for special populations with unique healthcare needs. While it is estimated approximately 20% of the population in the United States has some form of disability,1 those patients with developmental conditions, in particular, may pose distinct challenges in the prehospital setting. These conditions may either have unique medical issues associated with them, or may force the EMS provider to use different approaches for common issues.

It is important for EMS providers to recognize the basic elements of many disabilities. Detailed appreciation of certain aspects, such as pathophysiology or long-term management, can be helpful in some cases, but is often less pertinent in the emergent situation. On the other hand, these groups are at a greater risk than the general population for conditions such as respiratory failure or infections (e.g., urinary tract infections associated with long-term indwelling Foley catheters, or respiratory infections which may result from motor weakness or severe scoliosis). Still, the initial stabilization of these infections and many other conditions is largely the same.

Therefore, this article will instead focus on the unique factors affecting prehospital care or possible complications, reviewing some common disabilities healthcare providers should be prepared to encounter in the field. We will focus on those aspects of care which are managed differently from the general population. In all aspects of care for patients with disabilities, awareness, preparation and flexibility in a provider’s approach are crucial.

It is important to note, however, that each condition will only be covered as a brief overview. This article intends to be a primer, with EMS providers encouraged to explore each of these disabilities in more detail as pertinent to the specifics of their practice setting.

Cerebral Palsy

Cerebral palsy (CP) is a non-progressive disorder of movement and posture caused by a brain injury in early development. Cerebral palsy is not a disease; it is a collection of symptoms, and most common is the spastic variety (70%–80%).2, 3

Issues affecting patient care: Medical conditions associated with cerebral palsy include malnutrition due to swallowing and feeding problems; mental disabilities; seizures; urinary incontinence; difficulty with hearing, vision or speech; and spasticity with joint contractures.3 To decrease this spasticity, patients may have a baclofen pump implanted. EMS providers should note the possibility for pump failure.4

Orthoses are braces that support or correct musculoskeletal deformities, and may similarly address flaccidity, spasticity or contractures. They are generally named for the body parts they cover, such as the ankle-foot orthotic (or “AFO”).5 Still, spasticity may persist, and it is important to position these patients with supportive pads under areas of high pressure, or in the gaps between the body and the stretcher. Do not attempt to straighten a spastic extremity for any reason, such as taking blood pressures, starting IVs or splinting. Extremities should be splinted in their baseline position. Spastic extremities may also give unreliable blood pressure readings.

Because individuals with CP may have dysarthria, healthcare providers should be prepared to communicate with augmentative communication devices such as a communication board. Communicating in whatever way is easiest for the patient will assist patient care. The provider must be careful, however, not to make assumptions regarding intelligence based on communication difficulties. Although there are high rates of cognitive disabilities associated with CP, many individuals are highly intelligent.3,6 As with all patients with disabilities, the provider must gauge an individual’s ability to comprehend and interact on a case-by-case basis.

Muscular Dystrophy

Muscular dystrophy is a motor disorder associated with structural abnormalities of dystrophin proteins involved in muscle function. This results in progressive weakness and motor dysfunction.7

Issues affecting patient care: All muscle function is affected by muscular dystrophy. This affects mobility and patients often require assistance with transfers. Wheelchairs are often utilized as the patient gradually loses the ability to ambulate. As the disease progresses the patient will require an increased amount of support, and eventual involvement of the diaphragm and respiratory muscles will cause respiratory compromise. Patients can ultimately become ventilator dependent, or may have Do Not Resuscitate orders that should be honored. Respiratory muscle weakness combined with dysfunction of swallowing apparatus and upper airway muscles may lead to pneumonia, which is a common cause of death in this population.7 Given these concerns, a low threshold for supplemental oxygen should be considered for transport, especially when there are signs of respiratory distress or impending failure.

If intubation is mandated, succinylcholine, a paralytic often used in rapid sequence intubations, is contraindicated for patients with muscular dystrophy. Succinylcholine is a toxin to unstable membranes in any patient who has myopathy. It is associated with rhabdomyolysis, pathologic hyperkalemia and death.8–10 Intubation without a paralytic may be an option, or a non-depolarizing agent like rocuronium may be used, although this agent has been shown to have a longer time to onset and prolonged recovery in patients with muscular dystrophy.11 Sugammadex has been used as a reversal agent.12

Spina Bifida

Spina bifida results from incomplete neural tube development, likely caused by folic acid deficiency in utero. The extent of associated symptoms varies with the degree to which the spinal cord remains open.13

Issues affecting patient care: Because spina bifida affects the spinal cord, it is significantly associated with lower-extremity motor deficits and mobility issues. Patients with spina bifida should not be expected to ambulate on their own, although many can. Others may use crutches or wheelchairs as mobility aids. When possible, EMS personnel should identify the patient’s preference for transfers and facilitate independence. At the same time, as with other disabilities affecting movement or sensation, providers should be alert to the increased risk of pressure ulcer formation. Due to sensory deficits, or in some cases cognitive deficits, patients may not be able to identify ulcers as they are forming. Motor limitations may preclude shifting of weight or repositioning. Careful cushioning for transport is therefore of great importance in such cases.

Many patients with spina bifida will have a ventriculoperitoneal shunt, used to drain cerebrospinal fluid. These shunts may become a locus of infection, or can fail and produce symptoms of increased intracranial pressure. An increase in pressure in children is suggested by irritability, vomiting or bulging fontanels. Adults may be nauseous or vomit, may complain of headache, or have decreased alertness.14 Shunt failure must be considered in the differential of any patient with vague symptoms or altered mental status, and may suggest transport to a center with neurosurgical capabilities once stable.

All healthcare providers must be aware that a large percentage of patients with spina bifida have a latex allergy.15,16 Latex allergies should be presumed and all medical equipment and gloves should be latex-free.

Down Syndrome

Down syndrome is a congenital disability due to an extra copy of the 21st chromosome (trisomy 21). It is associated with mild to severe cognitive delay, and a characteristic pattern of resultant pathology and physical manifestations.17

Issues for patient care: Down syndrome is associated with several medical problems including leukemia, early-onset Alzheimer’s disease, osteoporosis, sleep apnea, cataracts and congenital heart defects.17–19 Patients may be more sensitive to medications, although reports of greater sensitivity to agents such as atropine remain subject to debate.20

In addition, there are a myriad of distinct physical features, some affecting the airway. Among these are dental abnormalities, thickened and fissured lips or tongue, and progressive protrusion of the mandible due to a large tongue in a small oral cavity. These patients may have large tonsils and adenoids, choanal stenosis, or glossoptosis. Patients with Down syndrome tend to have a small tracheal diameter.21 Because of these anatomic concerns, the healthcare provider should use an endotracheal tube at least two sizes smaller than generally used, given the risk of airway trauma.22 EMS providers must recognize the difficult airway and should intubate only with a back-up plan in place.

Unfortunately, alternative airway options have associated concerns as well. Inflation of the large pharyngeal cuff of the Combitube or King airway device exerts a great deal of pressure against the vertebral bodies in the upper cervical spine, as does insertion of a laryngeal mask airway (LMA).23 Individuals with Down syndrome are prone to atlantoaxial dislocation due to cervical spine instability, and in cases of trauma, a very low threshold for spinal stabilization should be adopted.24,25 EMS providers should minimize cervical spine trauma and may decide to hold off on intubation with an alternative airway device until the patient is in the ER. In many cases, simple bag-valve mask ventilation may be ideal until the patient can be transported to a facility with adequate resources.

Additional Information

Patients with disabilities may present with a variety of challenges that might not be evident upon initial assessment.

If it is possible prior to transport, it is important for the EMS provider to obtain as much additional information as he or she can from family members or caretakers. These sources can provide supplemental information pertinent to the patient’s care, such as information on medications, allergies, specialist care, feeding tubes, Foley catheters or shunts. EMS providers should ask about these, and ask open-ended questions about whether there is anything else that healthcare providers should know. In group homes, patient medication lists or recent medical documents may be available. However, obtaining further information may be complicated if the available staff is not familiar with the patient, or if relevant events occurred when the current staff was not on duty.

Medical care for individuals with disabilities is often fractured—many do not have a “medical home” or adequate support in coordinating care.26 While this may make it difficult for a receiving hospital to access records pertinent to the patient’s care, it helps to encourage transport of an individual to a medical center where that individual’s care is centered and staff is familiar with the patient’s needs, if possible.

In addition to obtaining information, prior to transport the EMS provider should take time to make sure feeding tubes, Foley catheters and any other medical devices are secure. It is imperative the medical provider does not pull any of these tubes out. One can ensure this does not happen by making certain these devices are secured with tape or gauze prior to moving the patient, and being aware of tubes at all times throughout the transport.

It is important to be aware that the patients themselves often become experts in their disease and can be crucial resources. At the same time, there can be a component of distrust or frustration with medical providers. Available literature suggests those with disabilities can face additional barriers to care or have difficulty finding providers who understand their condition.27, 28 Engaging all patients can facilitate care, and this is particularly true for those with disabilities.

EMS providers should ask the patient about his or her condition and always explain what they are doing to the patient. This should be done in reassuring, calm language at a level appropriate for the patient’s cognitive abilities. In all discussions with patients with disabilities, word choice is important. Certain terms have negative connotations. For example, while “retardation” used to be a medical term, it is no longer accepted, and “retarded” is considered derogatory by many patients and advocates. It is important to use person-first language, such as “a person with a disability” rather than “a disabled person.”29

EMS providers must realize that not only does each disability have a distinct pattern of characteristics, but also that each person with a disability is different. An open-minded and thorough assessment is crucial. At the same time, communicating with the patient and family, anticipating and addressing associated medical issues, and providing supportive measures and appropriate modifications will help EMS providers provide safe, effective care for these patients.

References

1. CDC. Prevalence and most common causes of disability among adults-United States 2005. MMWR, 2009; 58(16): 421–426.
2. Zeldin A. Cerebral palsy. Medscape, www.Emedicine.com.
3. Krigger KW. Cerebral palsy: an overview. Amer Fam Physician, 2006; 73(1): 91–100.
4. Shirley KW, Kothare S, Piatt JH Jr., et al. Intrathecal baclofen overdose and withdrawal. Pediatr Emerg Care, 2006; 4: 258–261.
5. Morris C. A review of the efficacy of lower-limb orthoses used for cerebral palsy. Dev Med Child Neurol, 2002; 44(3): 205–211.
6. Odding E, Roebroeck ME, Stam HJ. The epidemiology of cerebral palsy incidence, impairments and risk factors. Disabil Rehabil, 2006; 4: 183–191.
7. Emery AEH. The muscular dystrophies. Lancet, 2002; 23: 687–695.
8. Caro DA & Laurin EG. “Neuromuscular blocking agents.” In Walls RM & Murphy MF (eds.), Manual of Emergency Airway Management, 3rd ed. Lipincott Williams & Wilkins, 2008.
9. Nathan A, Ganesh A, Godinex RI, et al. Hyperkalemic cardiac arrest after cardiopulmonary bypass in a child with unsuspected Duchenne muscular dystrophy. Anesth Analg, 2005; 100(3): 672–74.
10. Larach N, Rosenberg H, Gronert G, et al. Hyperkalemic cardiac arrest during anesthesia in infants and children with occult myopathies. Clin Pediatr, 1997; 36(1): 9–16.
11. Ihmsen H, Viethen V, Forst J, et al. Pharmacodynamic modeling of rocuronium in adolescents with Duchenne muscular dystrophy. Eur J Anaesthesiol, 2009; 26(2): 105–110.
12. De Boer HD, Van Esmond J, Booij LH, et al. Reversal of rocuronium-induced profound neuromuscular block by sugammadex in Duchenne muscular dystrophy. Pediatric Anaesth, 2009; 19(12): 1226–1228.
13. Honein M, Paulozzi L, Mathews J, et al. Impact of folic acid fortification of the U.S. food supply on the occurrence of neural tube defects. JAMA, 2001; 285: 2981–2986.
14. Lee P, DiPatri AJ. Evaluation of suspected cerebrospinal fluid shunt complications in children. Clin Ped Emer Med, 2008; 9(2): 76–82.
15. Leger RR, Meeropol E. Children at risk: latex allergy and spina bifida. J Pediatr Nurs, 1992; 7(6): 371–376.
16. Mazagri, R, Enrique C, Ventureyra G. Latex allergy in spina bifida patients. Crit Rev Neurosurg, 1999; 9(3): 189–192.
17. Roizen NJ, Patterson D. Down’s syndrome. Lancet, 2003; 361(9365): 1281–1289.
18. Dinani S, Carpenter S. Down’s syndrome and thyroid disorder. J Ment Defic Res, 1990; 34(2): 187–193.
19. Tubman TR, Shields MD, Craig BG, et al. Congenital heart disease in Down’s syndrome: two year prospective early screening study. BMJ, 1991; 302(6790): 1425–1427.
20. Coté CJ, Lerman J, Ward RM, Lugo RA, Goudsouzian N. A Practice of Anesthesia for Infants and Children, 4th ed. Saunders, 2009, pp. 145.
21. Kozak FK & Ospina JC. “Characteristics of Normal and Abnormal Postnatal Craniofacial Growth and Development.” In Flint P, Harker L, Haughey B, et al (eds.), Cummings Otolaryngology: Head & Neck Surgery, 5th ed. Elsevier Mosby, 2010.
22. Scott SR. Down syndrome: analysis of airway size and a guide for appropriate intubation. Laryngoscope, 2000; 110(4): 585–592.
23. Todd M. Cervical spine anatomy and function for the anesthesiologist. Can J Anaesth, 2001; 48(6): R1–R5.
24. Pueschel SM, Scola FH, Perry CD, et al. Atlanto-axial instability in children with Down syndrome. Pediatr Radiol, 1981; 10(3): 129–132.
25. Morton RE, Khan MA, Murray-Leslie C, et al. Atlantoaxial instability in Down’s syndrome: a five year follow up study. Arch Dis Child, 1995; 72(2): 115–119.
26. Child and Adolescent Health Measurement Initiative. 2005/06 National Survey of Children with Special Health Care Needs, medicalhomedata.org/content/medicalhomedata.aspx.
27. U.S. Department of Health and Human Services. The Surgeon General’s Call to Action to Improve the Health and Wellness of Persons with Disabilities, 2005.
28. Eddey GE & Robey KL. Considering the Culture of Disability in Cultural Competence Education. Acad Med, 2005; 80(7): 706–712.
29. Foreman P. Language and disability. J Intellect Dev Disabil, 2005; 30(1): 57–59.

Kathryn E. Gersch, RN, FNP-BC, CCRN, CFRN, EMT-B, works as an FNP at Crouse Hospital and is also a flight nurse at Mercy Flight Central. She credits her sister, Marie, a special-education teacher, with stimulating her interest in disabilities.

Taylor R. Spencer, MD, MPH, is an emergency medicine physician in Albany (NY) Medial Center. As a college student, he worked at a summer camp for individuals with physical and developmental disabilities. As a medical student at the University of Connecticut, he organized the disabilities interest group and earned a Master’s in Public Health with a focus on disabilities. He has previously lectured to EMS personnel on disabilities issues.

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