Treating the special needs patient with a developmental disability: cerebral palsy, autism and down syndrome: recognizing signs of developmental delays and providing appropriate dental care.
Subject: Deinstitutionalization (Demographic aspects)
Dentists (Practice)
Child development deviations (Diagnosis)
Child development deviations (Care and treatment)
Child development deviations (Complications and side effects)
Developmental disabilities (Diagnosis)
Developmental disabilities (Care and treatment)
Developmental disabilities (Complications and side effects)
Mouth diseases (Risk factors)
Mouth diseases (Care and treatment)
Practice guidelines (Medicine)
Author: Jaccarino, Janet
Pub Date: 11/01/2009
Publication: Name: The Dental Assistant Publisher: American Dental Assistants Association Audience: Academic; Trade Format: Magazine/Journal Subject: Health; Science and technology Copyright: COPYRIGHT 2009 American Dental Assistants Association ISSN: 1088-3886
Issue: Date: Nov-Dec, 2009 Source Volume: 78 Source Issue: 6
Topic: Event Code: 200 Management dynamics
Product: Product Code: 8021000 Dentists NAICS Code: 62121 Offices of Dentists
Geographic: Geographic Scope: United States Geographic Code: 1USA United States
Accession Number: 222025790
Full Text: Developmental disabilities such as cerebral palsy, autism and Down syndrome affect the mind as well as the body and last throughout a person's life. It is important for the dental professional to be able to recognize signs of developmental delays and refer the patient to the appropriate expert. The earlier the recognition and treatment of delays is started the better the outcome for the child and his or her family.

As discussed in previous articles, deinstitutionalization has brought 80 percent of those with developmental disabilities into community residences or at home with their families. This population depends upon private local providers for needed dental services. Mental capacity, mobility problems, uncontrolled body movements, common oral findings of increased caries and periodontal disease will present a challenge in providing care. Many of these patients have medical issues such as cardiac and seizure disorders and vision and hearing impairments. A thorough medical history is essential and consultation with physicians, family and caregivers may be necessary. Adaptation of the skills you already have, flexibility, creativity and patience will allow your office to provide safe and effective care for this very special group of patients. (1) After reading this article, the reader should be able to:

* Explain the possible causes and characteristics associated with cerebral palsy, autism and Down syndrome.

* Describe the oral clinical findings commonly found in individuals with each disability.

* List the patient management approaches to providing dental care for patients with each disability.

* Discuss strategies for oral hygiene selfcare for patients with developmental disabilities.

* List the medical complications associated with Down syndrome and explain their relationship to dental care.

Cerebral Palsy (CP) (1-7)

Cerebral palsy is a nonprogressive chronic neurologic condition caused by damage to the immature brain. The damage can occur before or during the birth process or in infancy. The etiology of CP is outlined in Table 1 (page 8). CP is the second most common neurologic impairment in childhood, affecting one to four persons for every 1,000 live births. Seventy-five thousand Americans have the disorder.

While not curable, CP is not a disease process and does not get worse over time; however, affected individuals usually develop degenerative disorders such as osteoarthritis and respiratory problems. Use of adaptive devices, such as a powered wheelchair and communication tools, speech and occupational therapy, medication to reduce or control movement and possible surgery, can maintain and even improve function.

Characteristics

Symptoms of CP may vary from mild--needing no assistance, to severe--requiring use of a wheelchair and fulltime personal care. Uncontrolled body movements, seizure disorders, sensory impairment, and speech and communication defects may be present. Thirty to 50 percent of children with CP have some degree of mental retardation. Different types of CP are classified according to the specific area of the brain involved with each type having a different presentation.

Classification

* Spastic--most common type, stiff, rigid muscles one side of the body or all four limbs, can include the tongue, mouth and pharynx.

* Athetoid--low muscle tone, involuntary movements of the face, neck and trunk, may involve all limbs or just the limbs of the upper body.

* Ataxic--problems with balance, gait and depth perception.

* Mixed--is a combination of different types.

In general one or all of the following may be present: The muscles may be rigid and stiff with resistance to movement, involuntary muscle tremor may affect part or all of the body; and the muscles may be flabby and weak, making the patient unable to stand or raise his or her head.

Dysarthria (problems with speech) is caused by the inability to control the muscles of speech and mastication. Dysphagia (difficulty swallowing and inadequate cough reflex) can result in choking, coughing and aspiration problems. The adjustment to a soft diet is often used to cope with altered function and chewing ability.

The addition of abnormal or primitive reflexes presents a challenge to providing care. Primitive reflexes are involuntary responses that affect posture and movement. The reflex can occur when the patient's head is moved, the patient is startled or during wheelchair transfer. The limbs straighten and become stiff. Often efforts to control the reflex may make the reaction more intense.

Oral Clinical Findings

Most of the oral findings in persons with CP are associated with disturbances of the oral muscles. Periodontal disease and caries are more common due to poor oral hygiene, physical ability and malocclusion. It is estimated that periodontal disease is three times higher in persons with CP than in the general population. Poor oral hygiene and gingival overgrowth caused by Dilantin (phenytoin) to control seizures are major contributors to the problem.

The prevalence of caries may be due to a soft diet with food retained in the mouth longer due to swallowing difficulty. Also placing these patients at risk for caries are increased enamel hypoplasia, mouth breathing and use of antidrooling drugs that create dry mouth. Class II malocclusion with anterior open bite may be due to abnormal muscle and tongue movements (tongue thrusting). Severe drooling may be a consequence of the anterior open bite, lack of lip closure and swallowing dysfunction. Falls and accidents are common and the anterior open bite with protruding teeth may result in trauma causing fracture and avulsion. Bruxism and TMJ problems are also common.

Dental Management

Allow extra time for the appointment and discuss treatment options and preferences with the patient. Uncontrolled body movements may present the biggest challenge to care. Place and maintain the patient in the center of the dental chair and allow the patient to settle into a natural position.

Try to anticipate the patient's movements and work around them. Moving the head can produce primitive reflexes, so the patient's head should be kept in the midline and can be gently cradled with the operator's forearms and hands during treatment. As discussed in previous articles, stabilizing and protective devices such as mouth props, body wraps and pillows or cushion bags may be necessary.

Standard intraoral radiographs may be difficult to obtain so make sure to review extra oral techniques before the appointment. Oral hygiene is especially important for patients with neuromuscular disorders who may be unable to wear a denture, tithe patient has a caregiver, make sure he or she is present for any instruction. Automatic and modified toothbrush and flossing devices are useful and the addition of antimicrobials like chlorhexidine, as well as a fluoride rinse and toothpaste may be indicated.

Autism (1, 3, 6-11)

Autism, also known as autistic spectrum disorder (ASD), is a complex, lifelong disability affecting one in 150 people. There are three million U.S. citizens with autism. Autistic disorders, which are more common in males than females (4:1), include different syndromes identified by their characteristics. Social interaction, language, behavior and cognitive functions are all limited.

While the cause of autism is unknown, it is believed that genetics may play a role with neurological damage, chemical imbalance and biochemical abnormalities being the most accepted causes. The relationship between vaccines and autism has not been proven. There is no medical test for autism; diagnosis is made by a team of specialists who observe behavior, perform educational and psychological testing and document reports from parents.

While the prevalence of autism seems to have increased in recent years, it is believed this increase may be due to better diagnostic criteria and a broader definition of the spectrum of disorders. Autism is found throughout the world in all racial, ethnic and social backgrounds.

While there is no cure, experts believe that early diagnosis followed by intensive behavioral training may allow for better language and social skills. Some patients have had success with holistic or naturopathic approaches that include vitamins, supplements and special diets, such as gluten-free and casein-free (wheat and milk are common allergens). Symptoms such as hyperactivity or self-injury may require medication. Other characteristics of ASD are outlined in Box 1 (below).

Oral Clinical Findings

There are no specific oral findings associated with autism except when other developmental disabilities are present. Damaging oral habits may include bruxism, tongue thrusting and self-injurious behavior such as picking at the gingival or biting the lips. Dental disease may be present due to lack of oral hygiene ability or because dental care may have been a low priority. Caries may be a problem due to dietary fixation by the patient, preference for soft sweet foods that require little chewing, sweets used as reward and food pouching instead of swallowing. Watch for the effects of medications that cause dry mouth and gingival overgrowth.

Dental Management

Patients with autism will exhibit a wide variety of function, understanding and ability to cooperate with dental treatment so modifications should be patient-specific

As discussed in previous articles, a desensitization appointment may be useful to evaluate the patient for ability to cooperate. The patient can become familiar with the office, staff and equipment through a step-by-step process. Desensitization may take several visits to accomplish.

It is essential to find out what type of communication technique the patient will respond to. Some rely on verbal and non-verbal cues while others do not understand nonverbal language at all. The "tell-show-do" approach to communication may work best. Explain each procedure before it occurs, then show or demonstrate what you have explained. Use short simple sentences.

Keep instruments out of sight and light out of the patient's eyes. Autistic patients may have unusual sensitivity to sensory stimuli such as sound, bright colors and touch. It is best to minimize distractions. If possible use the same staff, dental operatory and appointment time.

Praise and reinforce good behavior; ignore inappropriate behavior. Some patients respond to soft music for relaxation or may gain comfort from a stuffed animal or blanket or a caregiver nearby:

Immobilization techniques should be used only when necessary to protect the patient and staff during treatment. Consent from a legal guardian is necessary. Make sure to check your State Dental Practice Act guidelines before using any form of restraint. Appointments should be short and positive. Provide care when you can, and reschedule if necessary.

Down Syndrome (DS) (1, 2, 7, 12-15)

Down syndrome, which affects one in 800 to 1,000 live births, is the most common and frequently observed chromosomal abnormality. More than 400,000 persons in the U.S. have this developmental disability.

Three manifestations of abnormality can occur with chromosome 21 during cell division. The abnormality causes a combination of physical characteristics that is constant throughout this population; individuals with DS tend to resemble one another. The disability can affect people of all races, cultures and economic levels in every geographic region. Some intellectual impairment in the mild to moderate range may be present.

Those with DS are at increased risk for many systemic conditions such as heart defects, respiratory and hearing problems, Alzheimer's disease, childhood leukemia, and thyroid conditions. Common medical problems for this population are outlined in Table 2 (page 34). Because these conditions are treatable many persons with DS have a life expectancy of 60 years of age.

Oral Clinical Findings

Individuals with DS have a higher incidence of periodontal disease due more to decreased immune function rather than oral hygiene alone. The disease can begin as early as six years of age and by adulthood virtually all persons with this disability are affected. Other contributing factors include chronic mouth breathing leading to dry mouth, fissured lips and tongue.

Patients with DS generally have obstructed airway issues due to macroglossia (enlarged tongue), increased secretions, obesity, enlarged tonsils and adenoids. These factors also put this population at risk for upper respiratory infections. Hypotonia or reduced muscle tone can affect the mouth contributing to an open bite and problems with chewing, swallowing, drooling, and speaking. Aphthous ulcers, oral candida infection and acute necrotizing ulcerative gingivitis are common.

Delayed tooth eruption can be as long as two to three years. Malocclusion is found in most people with DS. The small maxilla creates a mandibular overset and posterior crossbite. Orthodontic treatment may be considered but monitored carefully due to periodontal conditions.

It is interesting to note that children and young adults with DS have fewer caries than those without the disability. Delayed eruption of primary and permanent teeth, missing permanent teeth and small-sized teeth with wide spaces between them may be contributing factors. As the individual with DS ages there is an increased risk of caries due to xerostomia and poor food choices.

Dental Management

Due to the many medical considerations with these patients a thorough medical and pharmacologic history is essential. The dental team must be knowledgeable in all aspects of each individual condition the patient may present with; this may include communication techniques and treatment modalities specific for each condition.

Heart problems may require a physician consult and possible antibiotic premedication. A compromised immune system and a decreased number of T-cells may lead to more systemic and oral infection, tithe patient has a seizure disorder the dental team should be prepared to handle an emergency. Be sure to notice any oral side effects of medications.

Since some patients with DS maybe intellectually impaired it's important to determine the level of ability and communication. Generally these patients are content and affectionate but can become aggressive and unmanageable if confused or disoriented. The behavior may be due to fear or past traumatic dental experiences so be sure to explain procedures and keep the atmosphere calm.

Patients and their caregivers need to be educated about prevention including use of fluoride and treatments aimed at relieving dry mouth. Since the tongue may be fissured and can harbor bacteria, instruct the patient about cleaning the tongue every day with the toothbrush.

It is important to provide nutritional counseling to the patient and caregiver because delayed tooth eruption can cause a decrease in the number of teeth which decreases the patient's ability to chew. Even though caries rates may be lower in this population, young avoidance of poor food choices, such as a soft carbohydrate rich diet, should be stressed to help prevent later decay and obesity.

Oral disease should be treated aggressively: Remember to provide a nonoil-based lip balm during treatment. Topical and systemic antimicrobial agents and early periodontal therapy may be necessary to treat gingival disease.

Ask if the patient needs help getting to the dental chair. Sensory impairments can complicate communication so use of the interaction skills discussed in previous articles becomes vital. Make sure to record in the chart what strategies were successful. Once the patient is seated position him or her in the safest way to allow movement of the head and neck.

Due to a tendency for upper respiratory infections, it is especially important to practice good fluid suction techniques. Make sure to be aware of the increased gag reflex when using suction. Pillows or a blanket will stabilize patients and make them more comfortable. Early morning appointments when patient and operator are more rested may be best.

Conclusion

Individuals with developmental disabilities have both oral and systemic complications that can affect dental health. The mouth may have been neglected because dental treatment may not have been sought out at an early age due to other more pressing medical issues, therefore more oral disease may be present.

This population does present challenges to care but most can be treated in your office. The articles in this series will help the dental team employ techniques already known and create slight modifications to treatment. Being prepared and using your imagination will make the appointment safe, effective and comfortable and enable your office to provide dental care for everyone.

References

(1.) Practical Oral Care for People with Developmental Disabilities. U.S. Department of Health and Human Services, National Institutes of Health, National Institute of Dental and Craniofacial Research (Continuing education series of 7 booklets), http://www.nidcr.nih.gov/OralHealth/Topics/ DevelopmentalDisabilities/ContinuingEducation.htm. Accessed: June 2009.

(2.) Wentworth, L.E., Rowe, D., "Persons with Neurologic and Sensory Disabilities" In: Darby and Walsh. Dental Hygiene Theory and Practice. 2nd ed. St. Louis, Mo.: Saunders; 2003: 794-815.

(3.) Surabian, S., R., "Developmental Disabilities: epilepsy, cerebral pals35 and autism." JCDA. 2001. http://www.cda.org/page/Library/cdamember/ pubs/journal/jour0601/epilepsy.html. Accessed: June 2009.

(4.) Pediatric Dental Health. "Oral and Dental Health for Children with Cerebral Palsy." Available at: http://dentalresource.org/topic32cp.htm. Accessed: June, 2009.

(5.) Southern Association of Institutional Dentists. Cerebral Palsy, a review for dental professionals. Self Study Course (a series of 15 Modules). http://saiddent.org/modules.asp. Accessed October 10, 2008.

(6.) DECOD Program (Dental Education in Care of the Disabled). Module II. Dental treatment oft he patient with a developmental disability. 2nd ed. (a series of 12 booklets). Seattle: DECOD, School of Dentist, University of Washington; 1998.

(7.) Wilkins, E.M. "The patient with a mental disability. In: Clinical Practice of the Dental Hygienist. 10th ed. Philadelphia, Pa.: Lippincott Williams & Wilkins; 2009: 967-80.

(8.) DePalma, A., Raposa, K., A., Building Bridges: Dental care for patients with autism. The Academy of Dental Therapeutics and Stomatology. http://www.ineedce.com/courses/1554/PDF/AutismSpeaks.pdf. Accessed: June 2009.

(9.) Williams, K. S., Autism and the clinical implications for dental hygiene. Access. 2009; Feb: 34-35.

(10.) Green, D., Flanagan, D., "Understanding the autistic dental patient." General Dentistry. 2008; March/April: 167-71. https://www.agd.org/publications/articles/?ArtID=2968. Accessed: June 2009.

(11.) Friedlander, A., H., Yagiela, J., A., Patemo, V., I., Mahler, M., E., "The neuropathology, medical management and dental implications of autism." JADA. 2006; 137(11): 1517-27.

(12.) Southern Association of Institutional Dentists. Down Syndrome, a review for dental professionals. Self Study Course (a series of 15 Modules). http://saiddent.org/modules.asp. Accessed: October 2008.

(13.) Morgan, J., "Why is periodontal disease more prevalent and more severe in people with Down syndrome?" Spec Care Dentist. 2007; 27(5): 196-201.

(14.) Surabian, S., R., Developmental disabilities and understanding the needs of patients with mental retardation and Down syndrome. 2001 June; 29(6):415-23.

(15.) Pilcher, E.S., "Dental Care for the patient with Down syndrome." Original article published in J Down Syndrome. 1998; 5 (3), 111-16. http://www.ds-health.com/dental.htm. Accessed: June 2008.

Janet Jaccarino, CDA, RDH, MA

Janet Jaccarino, CDA, RDH, MA, is an Assistant Professor in the Department of Allied Dental Education, in the School of Health Related Professions at the University of Medicine and Dentistry of New Jersey. She has been teaching dental hygiene and dental assisting students since 2000 and can be reached at jaccarja@umdnj.edu.
Box 1
CHARACTERISTICS OF AUTISM (3, 7)

1. Social Interaction

* Relate to objects rather than people.
* Impairment in use of nonverbal behavior such as eye contact, facial
  expression and gestures.
* Failure to develop peer relationship appropriate to developmental
  level.
* Lack of social or emotional reciprocity.

2. Communication

* Delay or lack of development of spoken language impairment to
  initiate or sustain a conversation with others.
* Repetitive use of language.
* Lack of spontaneous make-believe play.

3. Repetitive and Stereotyped Patterns of Behavior

* Inflexible routines or rituals.
* Repetitive body movements.
* Persistent preoccupation with parts of objects.


Table 1
Etiology of CP (4,5)

Before Birth            During Birth        After Birth

Prematurity             Apnea               Trauma
Low birth weight        Hypoxia             Brain tumors
Maternal infections     Birth injury        Infections
  (rubella, syphilis,   Prolonged or          (encephalitis,
  herpes)                 difficult labor     meningitis)
Maternal dysfunctions                       Toxins
  (diabetes,                                (lead)
  hypertension,
  thyroid disorder)
Blood type
  incompatibility
Radiation
Drugs of abuse
  (alcohol, cocaine)

Table 2
COMMON MEDICAL PROBLEMS FOR PATIENTS WITH DOWN SYNDROME

Cardiac         * Mitral valve prolapse (MVP) is seen
                  in more than half of all adults.

Disorders       * Valve dysfunction leading to
                  congestive heart failure.

                * A shunt may be present.

Compromised     * Leads to more frequent oral and
                  systemic infection.

Immune System   * Chronic respiratory infections
                  contribute to mouth breathing, dry
                  mouth and fissured lips and tongue.

                * 100% of persons with DS over 35
Alzheimer         years old develop neurological
                  signs of the disorder.

Dementia        * 40-50 years old is the average age
                  of onset.

                * Impaired ability to understand and
                  remember.

                * Reduced degree of muscle tone.

Hypotonia       * When the mouth is involved it leads
                  to open bite, problems with
                  chewing, swallowing, drooling and
                  speaking.

                * Ability to communicate may be
                  affected.

Sensory         * 77% of persons with DS have hearing
                  impairments.

Impairment      * Crossed or misaligned eyes,
                  glaucoma and cataracts may be
                  present.

Seizures        * The mouth is at risk of chipped
                  teeth or a bite to the tongue or
                  cheeks during a seizure.

Additional      * Obesity (high blood pressure,
                  breathing problems).

Factors         * Below average height with a
to Consider       stooping posture (need body
                  stabilization in the dental chair).
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