Dental Problems of Children with Disabilities C. Chavarria DDS - October 18, 2006 Children with Disabilities The AAPD defines persons with special health care needs as individuals who: “have a physical, developmental, mental, sensory, behavioral, cognitive, or emotional impairment or limiting condition that requires medical management, health care intervention, and/or use of specialized services or programs. Health care for the special needs patients is beyond that considered routine and requires specialized knowledge, increased awareness, attention and accommodation” Currently, 56 million Americans have some type of disabling condition & 25 million Americans have a severe disability Patients with Special Health Care Needs can face many obstacles (such as financial resources, lack of preventive and therapeutic care, language and/or cultural barriers) in order to obtain oral health care Children with Disabilities Treatment Modification 1. Access to dental office 2. Radiographic examination 3. Preventive Dentistry Dental Office August 1984: Uniform Federal Accessibility Standards (barrier-free facility access) The Americans with Disabilities Act defines the dental office as a place of public accommodation. Follow accessibility guidelines (table 23.1) P. 567 (www.access-board.gov) Special Health Care Needs patients will continue to grow in number due to improvements in medical care. Many of the acute or fatal conditions have become chronic and manageable problems Mobility Problem Maintain clear path for movement throughout the treatment setting If you need to transfer your patient from a wheelchair to a dental chair, ask the patient or care giver Certain pts can’t be moved into dental chair, treat them in a wheelchair Dental Examination If a patient has a gagging problem, schedule an early morning appointment before eating or drinking Minimize the gag reflex by placing the patient’s chin in a neutral position or downward position If the patient has a swallowing problem, tilt the head slightly to one side and place the body in a more upright position Radiographic Examination X-ray are necessary for diagnosis and treatment plan Assistance from the parent, caretaker, dental assistant. Stabilize the films (Snap a ray; Bitewing loop with dental floss) Wear lead apron and/or gloves Preventive Dentistry Goal: Prevention of oral disease Factors: a) patient’s needs b) adequate communication with the parent/child in order to implement an appropriate preventive program for the patient c) direct involvement of the care takers and/or patient Home Dental Care • Parents/ caretakers are responsible for establishing good oral hygiene • Should begin during infancy • Unsupervised oral hygiene procedures in children with disabilities can have serious dental consequences • Plaque control program is essential • Brushing technique should be simple and effective • Various toothbrush–handle modification • Common positions of plaque removal: standing, upright wheelchair, reclining on couch or in bed, “leg lock” position, reclining on floor Diet and Nutrition • Nutrition influence dental caries by affecting the type and the virulence of the microorganisms in the dental plaque • Diet analysis is based on medical condition • Discontinuation of nursing bottle by 12 months of age • Cessation of at-will breast-feeding after teeth erupt (6 months of age) Fluoride Exposure • Emphasis should be placed on adequate systemic fluoride for patients with disabilities • Water analysis recommended prior to the use of fluoride supplementation • Professional topical fluoride treatments should be based on caries-risk assessment (fluoride varnishes) • Children at high risk for caries or children with active caries should be considered for additional fluoride therapy • Home fluoride programs using fluoride mouth rinses or brush-on fluoride gels should be recommended for use by children at high risk for caries • If a patient at high risk for caries cannot or will not comply with home fluoride therapy then frequent professional fluoride treatments may be substituted Preventive restorations • Pit and fissure sealants have been effective on occlusal caries reduction. • Stainless steel crowns are treatment of choice when a patient has Interproximal caries or severe bruxism • Recall visits may be required every 2, 3 or 4 months as indicated. Treatment Immobilization Mental Deficiency (AAPD Standards of Care for Behavior Management) Definition (American Association on Mental Deficiency): “(1) significant sub-avg. general intellectual functioning existing concurrently with (2) deficits in adaptive behavior and manifested during the (3) developmental period” Indications: 1. Lack of maturity 2. Mental or physical disability 3. Other behavior management techniques have failed 4. Safety Contraindications: 1. Cooperative patient 2. Underlying medical condition (e.g. Osteogenesis imperfecta) 3. Should not be used as a punishment Documentation includes 1. An informed consent 2. Indication of use 3. Type of immobilization used 4. Duration Mechanical aids for: Mouth opening: 1. Tongue blade 2. OPEN-WIDE® 3. Molt mouth prop • Reverse scissor action • Lip and/or palatal laceration • Never rest on the anterior teeth 4. Rubber bite block • Secured with dental floss • 3 sizes: small-child, child and adult size Body: Immobilization actually encourages relaxation and prevents undesired reflexes by keeping the patient’s arms in the midline of the body 1. Papoose Board • Has the option of a head attachment • A supporting pillow is needed • May develop hyperthermia 2. Triangular wrap 3. Pedi-Wrap • Does not have head support • Mesh fabric permits better ventilation 4. Bean bag dental chair insert 5. Safety belt 6. Extra assistant Extremities 1. Posey straps 2. Velcro straps 3. Towel and tape 4. Extra assistant Head 1. Forearm-body support 2. Head position 3. Extra assistant Attention Deficit Hyperactive Disorder (ADHD/ADD) • Neurobehavioral syndrome described by significant levels of inattention, impulsivity and overactivity • Learning problems • Poor peer relations and low self esteem • Affect 3-5% of school age children • Male >female = 4:1 • Onset 7 yrs old • Etiology is unknown/ familial disorder • Relation of maternal use of alcohol and tobacco during pregnancy Treatment: • Ritalin® (methylphenidate): stimulant, increase BP and HR • Adderall®, Concerta®, Strattera® • Counseling • 40% have symptom as adult Dental Management • Morning appointment (child rested and medication maximal level) • Avoid treatment during drug holiday • Short appointment • Simple instruction, repeat frequently Epidemiology • 1-3 % of US population has been identified as mentally disabled • 80% mild mental retardation Mental ”Disability” • General term used when an individual’s intellectual development is significantly lower than the average and his or her ability to the environment is limited • A diagnosis of Mental Disability is not made based on IQ alone • Inadequate adaptive functioning and intellectual deficiency are required to comply with a diagnosis of Mental Disability 1983 Classification (15 point intervals) LEVEL IQ ABILITY Borderline 68-83 Slow learner Mild 52-67 Educable Moderate 36-51 Trainable Severe 20-35 Nontrainable Profound <20 Nontrainable Functional Goals LEVEL GOAL Mild Independence and self support: employment, parenting Moderate Some support & Supervision: academics of daily living, self-help, survival academics Severe Higher level or total support & supervision Etiology • Prenatal stage: single gene defects, chromosomal disorders, complex syndromes, toxin exposures, congenital infections • Perinatal stage: complications of prematurity, hypoxic-ischemic insults, infection • Postnatal stage: infection, toxins, metabolic disorders, trauma, severe deprivation Dental Management • Comprehensive medical & dental history • Assess patient’s behavior & cooperation • Approach mental age of patient • A brief tour of dental office • Allow parent/caretaker to escort patient • Be repetitive, speak slowly and in simple terms • Positive reinforcement • Modeling by parents, siblings • Give one instruction at the time • Actively listen to patient • Keep appointments short • Schedule patients early in the day • Rigorous preventive oral hygiene regimen, diet counseling • Fluoride, chlorhexidine • Frequent recalls • Premedication • Physical restraints for aggressive patients • Sedation, N2O, IV or general anesthesia Dental Treatment according to Mental Disability Classification: Mild • Simple preventive & op. procedures usu. tolerated Moderate • May require sedation, restraints or IV/GA Severe/Profound • Usu. totally dependent & cannot cope w/even simple tasks. • General anesthesia Down Syndrome • Most common cause of moderate to severe MR • Trisomy 21 is the most common chromosomal aberration • Incidence 1.5 per 1000; increases with maternal age • Mortality rate increases when cardiac defects, leukemia (10 to 20 fold greater incidence during infancy) & upper respiratory infections present Facial Characteristics • Mongoloid facies • Slanted palpebral fissures • Small, brachycephalic head • Midface deficiency • Broad & shortened hands, feet, digits • Transverse palmar crease (Simian) • Iris anomalies common • Flat facies with hypertelorism • Depressed nasal bridge • Protrusion of the tongue • Epicanthal folds • Small and misshapen auricles and anomalies of the folds • Wide gap between the first and second toes Oral Manifestations • Macroglossia, fissured tongue • Abnormal tooth morphology • Congenitally missing incisors • Delayed eruption • Caries~level of MR • Midface deficiency gives Class III appearance • Crowding of dentition • Mouth breathers • Dry, cracked lips • Periodontal disease • Gingival hyperplasia Systemic Manifestations • Cardiac (40%): Most of the patients experience mitral valve prolapse • Gastrointestinal, sensory and infectious complications • Hearing and visual loss • Spine instability • Onset of Alzheimer disease in adults during the third decade of life • Onset of dementia during the fifth decade of life • Increased rate of infections • T-cell and B-cell deficiency • Increased rate of Acute Lymphocytic Leukemia (ALL) during infancy • Increased hepatitis rate • Thyroid dysfunction Dental Management • Medical History (heart disease, ALL) • Emphasize oral hygiene due to high incidence of rapid periodontal dz • Poor vascularized gingival tissue • T-cell maturation defect or PMN chemotactic defect • May benefit from daily antimicrobial agents e.g. Chlorhexidine • Many children with Down syndrome are affectionate and cooperative • Some may require light sedation, immobilization or general anesthesia Pervasive Developmental Disorders Childhood Schizophrenia Asperger Disorder (or syndrome): Display some of the features of autism but may also possess a level of skill in some areas well above the average for their peers. Autism • Difficult to diagnose and has no cure • For many years, thought to be purely psychological w/o organic basis • Search for a biological cause- defective structure in brain • Closely associated with other clinical and medical conditions Prevalence: Earlier studies: 4 per 10,000 Recent studies: 10-15 per 10,000 • improved diagnosis • changing definitions of autism (milder forms now included) • boy:girl ratio is 3- 4:1 • Autism distinctive feature: restricted & stereotyped behavior patterns • Most children score below normal on IQ testing & experience significant developmental delay • The more severely affected express themselves minimally, show a low level of interest in exploring objects, avoid sounds, and engage in ritualistic behavior Characteristics: • Extreme aloneness • Language disturbances • Mutism • Parrot like, repetitive speech • Difficulty with the concept of “yes” • Nystagmus • Obsession with maintenance of sameness; routines • Inability to evaluate own thoughts • Unable to judge what others might be thinking • Spinning of objects • Sensitive to touch Associated Conditions • Maternal rubella • Early injury to brain • Infantile seizures • Chromosomal abnormalities • Mental Retardation • Metabolic disorders (phenylketonuria) • Fragile X syndrome • Rett syndrome • Williams syndrome • Neurocutaneous disorders(neurofibromatosis,tuberous sclerosis) Oral Manifestations • Drooling • Poor muscle tone • Poor tongue coordination • Pouch food instead of swallowing • Increases caries rate due to sweet and soft diet Dental Management • Repeated visits may desensitize • Use slow approach and quiet voice • T-S-D • Modeling by parents and siblings • Home rehearsals • Positive reinforcement with immediate reward • Minimize movements as easily distracted • Short visits, time out • Physical restriction • Sedation, N2O, GA Cerebral Palsy Definition: 1. There must be a difficulty in neuromotor control 2. A nonprogressive brain lesion 3. An injury to the brain that occurred before it was fully mature 4. Used only if static encephalopathy exists 5. Often associated with other symptoms • An estimated 764,000 children and adults in the United States manifest one or more of the symptoms of cerebral palsy. • Currently, about 8,000 babies & infants diagnosed each year. Incidence and Prevalence: • Prevalence: 1.5 to 3 per 1000 live births • There is a correlation between premature birth and incidence of CP • All children with cerebral palsy are w/ brain damage, but not all children with brain damages are with cerebral palsy. Etiology Brain insult may occur at the prenatal, perinatal or postnatal stage. 50% of time etiology is unknown Multiple Classifications 1. Extremities involved 2. Type of motor disorder 3. Type of tone abnormalities 4. Characteristics of involuntary movements 5. Degree: mild, moderate, severe Anatomic Involvement • Monoplegia (one limb) • Hemiplegia (arm and leg on same side) Arm held in flexion Internal rotation Leg circumducted on involved side • Paraplegia (both legs) • Diplegia (both legs with minimal involvement of both arms) • Quadriplegia (all four limbs) Medical Management • Enrollment in an early intervention program -federal law mandates such a program in all states • Must address motor dysfunction and associated nonmotor deficits. • The final objective of CP management must be the socialization of the child with his/her motor, speech, or cognitive difficulties, which have to be checked and supported all thru his/her lifetime. Oral manifestations of CP • Periodontal disease due to poor oral hygiene and soft diet • Gingival hyperplasia due to seizure meds • Dental caries-conflicting data • Malocclusion-twice as common • Protrusion of max ant teeth, increase overbite/overjet, openbite • Bruxism • Trauma Classification by neuromuscular dysfunction: Spastic Cerebral Palsy: tense, contracted muscles (most common type) • Affects 70 to 80 percent of patients, muscles are stiff and permanently contracted. • Often described according to the affected limbs i.e. spastic diplegia (both legs) or left hemiparesis (left side of body). • In some cases, spastic cerebral palsy follows a period of poor muscle tone (hypotonia) in the young infant. • Spastic Diplegia: Scissoring of legs: excessive pull of hip adductors and internal rotators Toe walking Note that child is crouched due to hamstring tightness Athetoid or Dyskinetic: constant, uncontrolled motion of limbs, head, eyes • Affects about 10 to 20% of Cerebral Palsy patients • Characterized by uncontrolled, slow, writhing movements usually affecting the hands, feet, arms, or legs and, in some cases, the muscles of the face and tongue, causing grimacing or drooling. • The movements often increase during periods of emotional stress • Patients may also have problems coordinating the muscle movements needed for speech, a condition known as dysarthria. Ataxic Cerebral Palsy: poor sense of balance, falls & stumbles • Affects an estimated 5 to 10% of Cerebral Palsy patients • Affects the sense of balance and depth perception • Affected persons often have poor coordination; walk unsteadily with a widebased gait, placing their feet unusually far apart and experience difficulty when attempting quick or precise movements. Mixed • Affects 10% of the Cerebral Palsy patients • It is not unusual for patients to have symptoms of more than one form. • The most common mixed form includes spasticity and athetoid movements but other combinations are also possible. Rigidity: tight muscles that resist effort to make them move Tremor: uncontrollable shaking, interfering with coordination Dental Management • Thorough medical and dental history • Consider treating in wheelchair • Use 2 person lift if moving to dental chair • Do not force limbs into unnatural positions • Use rubber dam - protection from aspiration • Consider physical restraints for protection • Consider recommending mouthguard • Patience • Goal of treatment-comprehensive care • Prevention- frequent visits, parental involvement in home care, modifications to toothbrush, diet, fluoride • Restorative care with rubber dam, bite block tied with floss • Short appointments. Spina Bifida • Unknown etiology • Spina bifida is the most common neural tube defect • Affecting 1,500 to 2,000 babies each year (one in every 2,000 live births) • Occurs at the end of the first month of pregnancy • Evidence suggests that genes and/or environment may be involved • Folic acid during the first 6 weeks of pregnancy can prevent over 50% of neural tube defects Occurs in different forms, each varying in severity. • Spina Bifida Occulta (mildest form) Also known as Closed Neural Tube Defect Opening in one or more vertebrae w/o apparent damage to spinal cord Asymptomatic with no neurologic problems • Spina Bifida Manifesta: • Meningocele The meninges, or protective covering of the spinal cord, is pushed out through the opening in the vertebrae in a sac called the “meningocele” Spinal cord remains intact Can be repaired with little or no damage to the nerve pathways • Myelomeningocele The most severe form of spina bifida A portion of the spinal cord protrudes through the back Sacs can be covered by skin or tissue & nerves can be exposed • A high percentage of children with Spina Bifida are allergic to latex, possibly due to multiple genitourinary operations along with multiple urinary catheterizations for neurologically impaired bladders • Over 40,000 products contain latex rubber • Latex is a natural rubber; a complex mixture of phospholipoproteins, sugars, nucleic acids, lipids, minerals and proteins • The proteins are responsible for the severe immediate (Type I) anaphylactic reaction • Reaction to latex: Irritant contact dermatitis Nonimmunologic inflammation of the skin Erythema,dryness,fissuring, chapping, vesicle formation Allergic contact dermatitis Lesions appear 48-96 hrs post exposure Pruritus, erythema, scales, crusts, scabs, vesicles Immediate allergic reaction Caused by exposure to proteins in the natural latex rubber Immediate pruritus, erythema, edema Dental Management • Avoid all latex products: All equipment that comes in contact with the patient should be made of non-latex substitutes • Schedule dental appointments at the beginning of a working session • Treat the patient in a controlled setting • Emergency carts available at all times if an anaphylaxic rxn is present • Children with neural tube defects are at a high risk of caries due to poor oral hygiene, poor nutritional intake and long-term drug therapy • Be alert to medical history (history of allergies to banana, avocado, kiwi, pineapple, peach, nectarine, plum, cherry, potato, tomato, celery, and chestnuts) which may sensitize allergic patients to latex exposure • Pt. should wear a medical alert bracelet & carry an epi-pen at all times • Must have a latex free cart to simplify management • Patients with latex allergy need to be flagged, labeled and a latex allergy sign be posted outside the treatment room Treatment of latex anaphylactic reaction Terminate treatment. Use a cardiopulmonary monitor and oximeter Initiate basic life support: • Remove latex exposure • Discontinue IV or inhalation agents • Administer 100% Oxygen • Secure the airway with endotracheal tube if indicated (edema) • Administer epinephrine 1:1000 IV, SQ or intralingual 0.125-0.25 ml (child) 5 ml (adult) every 5-15 minutes as necessary • Administer diphenhydramine 1-2 mg/kg PO or IM, max 50 mg • Activate EMS • Transport the child to an appropriate medical facility Management of the Medically Compromised Patient C. Chavarria DDS - October 19, 2006 Acquired Immunodeficiency Syndrome • The Joint United Nations Programme on HIV/AIDS (UNAIDS) estimates 40 million people living with HIV or AIDS worldwide. • In the U.S. approximately one million people have HIV or AIDS, and 40,000 Americans become newly infected with HIV each year. • AIDS is threatening children as never before: Children under 15 account for 1 in 6 global AIDS-related deaths and 1 in 7 new global HIV infections. A child under 15 dies of an AIDS-related illness every minute of every day A young person aged 15–24 contracts HIV every 15 seconds. (UNICEF) Infants & children w/ AIDS have clinical findings similar to those in adults: • Weight loss and failure to thrive, • hepatomegaly or splenomegaly, • generalized lymphadenopathy, and • chronic diarrhea • HIV infects cells of the immune system (lymphocytes and macrophages) These white blood cells contain the greatest number of CD4 cell surface receptors (glycoproteins) which permit attachment with viral surface proteins and enhance host-cell invasion and infection The viral genome is integrated into the host-cell genome and leads to progressive and eventually irreversible immunosuppresion by producing more virus and further killing the CD4 (T4) helper-inducer lymphocytes that are important modulators of the immune system • Immunodef’cy allows opportunistic infections, malignancies & autoimmune dz Diagnosis is made by screening the serum for antibodies to HIV & is confirmed by Western blot analysis Ongoing management is guided by the patient’s CD4 cell count • A person can receive a clinical diagnosis of AIDS, as defined by the U.S. Centers for Disease Control and Prevention (CDC), if he/she has tested positive for HIV & meets one or both of these conditions: 1) The presence of one or more AIDS-related infections or illnesses 2) A CD4 (T-cell) count that has reached or fallen below 200 cells per cubic millimeter of blood (CD4 count ranges from 450 to 1200 in healthy individuals) • Treatment: Antiretroviral drugs target the virus at several steps: 1. The fusion of the virus at the host cell 2. The transcription of DNA from viral RNA by reverse transcriptase 3. The cleavage of viral proteins by the viral protease enzyme 4. The most effective treatment strategies use a combination of several drugs to inhibit the virus at several steps • Oral Manifestations of HIV Infection Fungal infections • Oral candidiasis: 1. Pseudo-membranous lesion: creamy white/yellow plaques easily removed from mucosa leaving a red, bleeding surface 2. Hyperplastic lesion: white plaques that cannot be easily removed 3. Erythematous (atrophic) lesion: red or spotty areas on the mucosa 4. Angular cheilitis: fissures radiating from lip/mouth commissures • Systemic treatment 1. Ketoconazole (Nizoral) 200-400 mg daily with food 2. Fluconazole (Diflucan) 100 mg daily 3. Amphotericin B or fluconazole IV • Topical treatment (one to two weeks is usually effective) 1. Nystatin (Mycostatin) rinses (100,000 U, three to five times daily) 2. Clotrimazole (Mycelex) troches Viral infection • Herpes group viruses (HSV) can produce recurrent episodes of vesicles (oral acyclovir –Zovirax) • Herpes zoster (VZV) can produce oral ulcerations accompanied by skin lesions restricted to one side of the face (oral acyclovir) • Oral hairy leukoplakia (HL) caused by Epstein-Barr virus, white lesion that does not rub off located on lateral margins of tongue, seen on HIV patients (high doses of acyclovir) Bacterial Infection • Mycobacterium avian-intercellulare • Klebsiella pneumoniae Neoplasms • Kaposi sarcoma most common malignancy seen in AIDS • Occurs in 15 to 20% of AIDS patients • May be red, blue, or purple, flat or raised and solitary or multiple Kaposi sarcoma is a vascular, malignant tumor that can involve the skin, mucous membranes, or internal organs. The palate is a common site of oral involvement Lesions often are purple or brown in color, and can be flat, raised, or nodular. Idiopathic Processes/Lesions • Ulcers of unknown etiology resemble apthous ulcers • Salivary gland swelling • HIV associated gingivitis and HIV associated periodontitis (aggressive curettage, Peridex, and/or antibiotic treatment) • Dental Management of AIDS Consult with Physician Emphasis on Preventive Care Palliative treatment as needed Asthma Sickle Cell Anemia • a diffuse obstructive lung disease • Affecting 1:10 children (3/4 : mild) • Prepubertal: boy>girl • Adolescence/adulthood: male= female • Characterized by inflammation and bronchial constriction • Caused by: 1. edema of mucous membranes, 2. increased mucous secretion, and 3. spasm of smooth muscle • Autosomal recessive hemolytic disorder • Predominantly: African, Italian, Greek, Arabian and Indian descent • Hemoglobin S-decreased oxygen-carrying capacity Decreased oxygen tension causes sickling of the cells • Radiographically: stepladder trabeculation, hair on end • Painful crisis Possible splenectomy Progressive deterioration of cardiac, pulmonary and renal failure • May need SBE • Etiology: Biochemical Immunologic Infectious Endocrine Psychological factor • Precipitating Factors: Acidosis Dehydration Hypoxia Fever Hypothermia Infection Hypotension Hypovolemia Stress • Onset: Acute (exposure to irritants) Insidious (precipitated by viral infection) • Dental Management Short dental appointments Aggressive preventive program No dental treatment during a sickle cell crisis Occasionally infarcts in the jaw If GA for dental procedures, consult with hematologist and anesthesiologist Patients with less than 7 g/dl and 20% hematocrit may require transfusion • Signs and symptoms Coughing Wheezing on expiration Chest tightness Dyspnea (difficulty or labored breathing) Severe asthma: Dyspnea Wheezing Tachypnea Profuse perspiration Cyanosis Hyperventilation Tachycardia Chest pain • Medications used in asthma Bronchodilators ßeta-agonists: Albuterol (Ventolin™, Proventil™), Salmeterol (Serevent™) Anticholinergic: Ipratropium (Atrovent™) Theophylline Anti-inflammatory Steroid: Beclomethasone (beclovent) Other: Cromolyn (Intal™) Cystic Fibrosis • Autosomal recessive disorder predominantly affecting the exocrine glands • Approximately 5% of the population is carriers & 1 in 2000 live births affected • The most common lethal genetic disorder affecting Caucasians • The abnormal gene has been located on the long arm of chromosome 7 • basic abnormality: elevation of viscosity of the mucous secretions • pancreas: obstruction of secretory ducts, exocrine insufficiency & malabsorption • extensive bronchi plugging: obstruction, infection, destruction of bronchial walls • confirmation of CF: elevation of sweat concentration of Na & Cl > 60mEq/L • Clinical manifestations vary, some pts are asymptomatic for long periods of time • More than 85% of affected children show evidence of malabsorption • Median life expectancy is 31 years • Death results f/ pneumonia & anoxia after long period of respiratory insufficiency • Oral Findings: High caries rate Low salivary flow High palatal vault More posterior crossbite Greater overjet Increased facial height Chronic mouth breather • Questions to ask: Age of onset Frequency/severity Triggering agent Hospitalizations? ER visit Last episode Medications Limitation of activities • Dental Management Evaluate the patient MD consult Well controlled asthmatic patients may be treated safely in the office Poorly controlled asthmatic patients should postpone elective dental treatment Severe asthmatic pts on steroids may need add’l steroids to cover the stress of the procedure Upright or semi upright position may be beneficial Should have a bronchodilator available prior to treatment Reduce anxiety Nitrous Oxide,Vistaril or Valium help alleviate anxiety Narcotics are contraindicated: Histamine release leads to bronchospasm Aspirin and NSAIDs are contraindicated Acetaminophen is recommended • Emergency Treatment Discontinue dental treatment Reassure the patient, administer inhalant bronchodilator give 2-3 inhalations Administer supplemental 0xygen Keep airway open Consider SQ epinephrine 1:1000, 0.25 cc or 0.01 mg/kg Call medical assistance • Oral Findings of CF: Low incidence of caries High incidence of tooth discoloration (systemic tetracycline during formation) High incidence of mouth breathing / open bite (chronic nasal/sinus obstruction) • Multidisciplinary Approach… • Dental Management of CF: need a high caloric intake, therefore preventive advice is essential MD consultation Patients may prefer upright position (clear secretions more easily) Avoid sedatives that interfere with pulmonary function Nitrous oxide is contraindicated on pts with history of severe emphysema Endocarditis Prophylaxis (PO 1 hour before dental procedure) Heart Disease Congenital Heart Disease: Ventricular septal defect Patent ductus arteriosus Tetralogy of Fallot Transposition of the great vessels Atrial septal defect Pulmonary stenosis Coarctation of the aorta Aortic stenosis Tricuspid atresia All other 22% 17% 11% 8% 7% 7% 6% 5% 3% 14% • Etiology • Aberrant embryonic development of a normal structure • Failure to progress beyond early stage of embryonic developm’t • Maternal rubella • Chronic maternal alcohol abuse • Hereditary predisposition • Acyanotic Congenital Heart Disease Left to the right: 1. VSD 2. ASD 3. patent ductus arteriosus Obstructive defect: 1.aortic stenosis 2. coarctation of the aorta • Cyanotic Congenital Heart Disease Right to left shunt: 1. Transposition of great vessels 2. pulmonary stenosis 3. tricuspid atresia 4. tetralogy of Fallot Congestive heart failure pulmonary congestion heart murmur labored breathing cardiomegaly Congestive heart failure labored breathing Cyanosis hypoxia poor physical development heart murmur clubbing of the fingers Tetralogy of Fallot 1. VSD 2. aorta overriding 3. pulmonary stenosis 4. RV hypertrophy Acquired Heart Disease: • Rheumatic fever: Heart Murmurs Innocent (functional) murmurs = sounds from turbulence w/o cardiac abnormality & do NOT require antibiotic prophylaxis Organic murmurs = sounds from pathologic abnormality in the heart & do require antibiotic prophylaxis Rheumatic heart disease requires antibiotic prophylaxis • Bacterial endocarditis: Microbial infection of heart valves or endocardium in the area of a heart defect Acute Fulminating disease Result from high pathogenic microorganism: staphylococcus, group A streptococcus and pneumococcus Normal heart suffers of erosion and destruction of the heart valves Subacute (SBE) Caused by viridians streptococci Embolization is a characteristic feature of infective endocarditis Microorganism→ Blood stream→ Endocardium/valvular heart defect→ Form vegetations Symptoms: Low irregular fever Sweating Malaise Anorexia Weight loss Arthralgia Heart murmur Painful fingers and toes Amoxicillin (standard regimen) Clindamycin (‘cillin allergy) Dosage forms: Amoxicillin: Clindamycin: adults 2.0 g, children 50mg/kg adults 600 mg, children 20mg/kg 125 or 250 mg/5ml or 250, 500 tabs 150, 300, 450, 600, 750, 900 mg/tab Recommended for: (pts with high & moderate risk conditions) Ext. (& reimplanting avulsed teeth) Perio (surgery, prophy, S/RP, probing, recall, implants) Endo (RCT or surgery only beyond the apex) Subgingival fibers/strips Initial placement of ortho bands but not brackets Intraligamentary LA injections When bleeding is expected … HIGH-RISK Prosthetic valves Previous bacterial endocarditis Complex cyanotic congenital heart dz Sugically constructed shunts/conduits MODERATE-RISK Most other congenital malformations Acquired valve dysfunction (e.g., rheumatic heart dz) Hypertrophic cardiomyopathy MVP w/regurgitation &/or thickened leaflets NOT Recommended for: (unless bleeding is expected) Restorative (op & prosth) w/ or w/o cord LA injections (non-intraligamentary) Intracanal endo; post placement & buildup Rubber dam placement Post-op suture removal Removable or ortho appliance placement Taking impressions Ortho adjustment Shedding of primary teeth … NEGLIGIBLE-RISK (normal) Isolated secundum ASD Surgically repaired ASD, VSD, or PDA (after 6 months) Previous coronary artery bypass graft surgery MVP w/o regurgitation Physiologic (functional, innocent) heart murmurs Previous Kawasaki dz w/o valve dysfunction Previous rheumatic fever w/o valve dysfunction Cardiac pacemaker or implanted defibrillator • Dental Management of Heart Disease Update dental and medical history Cardiology consultation SBE prophylaxis Behavior management techniques (sedation, general anesthesia) CPR equipment Pulp therapy in primary teeth is not recommended Endo treatment in permanent teeth Refer as needed Definitive treatment is recommended Once amoxicillin oral suspension is mixed with water, it will expire in a week Wait 2-4 weeks between each visits to allow penicillin resistant organisms to disappear from the oral flora and allow repopulation of the mouth with antibiotic susceptible flora If needed, the alternation of medication may be indicated Consider hospital dentistry Make the parent(s) aware that poor dental hygiene and periodontal or periapical infections my produce bacteremia even in the absence of dental procedures. Individuals who are at risk for developing bacterial endocarditis should establish and maintain the best possible oral health to reduce potential sources of bacterial seeding Ventricular Septal Defects (VSD) • The most common of cardiac malformations • Small defects can be asymptomatic • May be found during routine physical examination • Large defects with extensive pulmonary flow are responsible for breathlessness, feeding difficulties and poor growth • 30-50% of the small defects close spontaneously within the first year of life • Larger defects are usually closed surgically in the second year of life, • Defects involving other cardiac structures may require complex surgery or even transplantation Tetralogy of Fallot • Consists of a combination of: 1. An obstruction to the right ventricular outflow ( pulmonary stenosis) 2. VSD 3. Dextroposition of the aorta 4. Right ventricular hypertrophy • Cyanosis is one of the most obvious sign as the child grows, the obstruction of blood is further exaggerated • Oral mucous membranes, nail-beds show signs of cyanosis • Growth and development may be markedly delayed Atrial Septal Defects (ASD) • Not as common as VSD in children • More significantly on adult • Even an extremely large ASD rarely produces heart failure in children • Symptoms usually appear in the third decade Acquired cardiovascular disease: Rheumatic fever • Group A streptococcal infection of the upper respiratory tract • Usually between 5 -15 years • Higher among lower socioeconomic groups • Clinical onset is usually acute and occurs 2-3 weeks after a sore throat • Joint pains • Carditis is the most serious manifestation • Fever (low grade) is usually present • Most of the carditis resolves except the lesions on the cusps of the heart valves which become fibrosed and stenotic; may affect mitral, aortic, tricuspid, and pulmonary valves Pulmonary stenosis • Mild to moderate stenosis of the pulmonary valve usually present no symptoms • Severe stenosis may cause exercise intolerance and cyanosis Disease of the myocardium and pericardium Bacterial infections such as : diphtheria and typhoid; fungal and parasitic infections: rheumatoid arthritis: systemic lupus erythematosus; uraemia, thalasemia, hyperthyroidism, neuromuscular diseases, such as muscular dystrophy and glycogen storage diseases Viral hepatitis Viral hepatitis is an infection that results in the inflammation of the liver cells, leading to necrosis or cirrhosis of the liver Hepatitis A virus (HAV) Hepatitis B virus (HBV) Hepatitis delta virus (HDV) Non-A, non-B hepatitis virus (NANB) or Hepatitis C (HCV) - parenterally transmitted Patent ductus arteriosus (PDA) • During fetal life most of the pulmonary arterial blood is shunted through the ductus arteriosus into the aorta, bypassing the lungs. • Functional closure of the ductus arteriosus usually occurs at birth NANB or hepatitis E virus - enterically transmitted Leukemia • Hematopoietic malignancy: proliferation of abnormal leukocytes in bone marrow and dissemination of these cells into peripheral blood, tissue and organs • Second to accidents as the leading cause of death in children • Affects 5 in 10,000 children in the US (Peak incidence 2 and 5 years of age) • Our primary objective should be prevention, control and eradication of oral inflammation, hemorrhage and infection • Physically debilitated patients with ulcerative lesions require close attention, in order to avoid the potential of a fatal viral, fungal or bacterial infection • • Infection = 1º cause of death in approx. 80% of children w/ leukemia • Bleeding is the second most common cause • Candida infection is common in leukemia patients (debilitated, immunosuppressed, poor OH, prolonged antibiotic therapy) • Nystatin is recommended • Etiology: Unknown Ionizing radiation Chemical agents Genetic factors • Classified according to: 1. Morphology of predominant abnormal white blood cells in the bone marrow 2. Clinical course (acute or chronic) 3. Degree of differentiation or maturation • Signs and Symptoms History at presentation: Irritability, lethargy, persistent fever, vague bone pain, easy bruising Common findings on physical examination: Parlor, fever, adenopathy, hepatosplenomegaly, petechiae, cutaneous bruises, gingival bleeding, and evidence of infection Clinical manifestation of acute leukemia: anemia, thrombocytopenia and granulocytopenia • Once diagnosed, the patient is stabilized and prepared for chemotherapy Treatment for ALL is prolonged (2.5 to 3.5 years) The treatment regimen depends on the prognosis and parameters evaluated • Phases of Treatment: 1. Induction (use of antileukemic drugs 4 week regimen) 2. Consolidation (intensify prophylactic CNS treatment) 3. Interim maintenance (combo of relatively non toxic agents, monthly visits) 4. Delayed intensification (intensify antileukemic therapy) tends to improve survival rate in patients with ALL 5. Maintenance phase (two years for girls and three years for boys) • Oral Manifestation: Regional lymphadenopathy Mucous membrane petechiae Gingival bleeding ( due to anemia, granulocytopenia and thrombocytopenia) Gingival hypertrophy (due to direct infiltrate of leukemic cells) Pallor Nonspecific ulcerations • Evidence of skeletal lesions is visible on dental radiographs in up to 63% of children, although not pathognomonic, we should be alert of the following changes: 1. Generalized loss of trabeculation 2. Destruction of the crypts of developing teeth 3. Loss of lamina dura 4. Widening of the periodontal ligament space 5. Displacement of teeth and tooth buds • Dental Management: Pulp therapy on primary teeth is contraindicated Avoid the use of drugs that may alter platelet function (salicylates) The pt’s hematologist & oncologist or 1° care physician should be consulted Primary medical diagnosis Anticipated clinical course and prognosis Present and future therapeutic modalities Present general state of health Present hematologic status Blood cell profile and platelet count Platelet Count 150,000-400,000 50,000-100,000 20,000-50,000 <20,000 normal bleeding time prolonged, most routine procedures OK moderate risk of bleeding; Defer elective surgical procedures significant risk for bleeding; Defer elective dental procedures 100,000/mm³ is adequate for most dental procedures < 20,000/mm³ can cause spontaneous bleeding (all intraoral mucosal tissues hemorrhaging) Absolute neutrophil count (ANC) >1500 normal 500-1,000 some risk of infection (bacteremia) Defer some elective procedures 200-500 pt. should be admitted to hospital; moderate risk of sepsis; defer all elective dental procedures <200 Significant risk of sepsis ANC=(% of neutrophils+ % of bands) x total white count ÷ 100 Indicates the host’s ability to suppress or eliminate infection < 1,000/mm³ defer elective dental treatment Management of the Medically Compromised Patient ( II ) Carmen Chavarria DDS - October 25, 2006 Hearing Loss • Affects 1.8 million people • 1 in 600 neonates has a congenital hearing loss • 14 million hearing-impaired individuals in the US • Table 23-3 of your textbook demonstrates how speech and psychological problems relate to various degrees of hearing loss • Early identification and correction of hearing loss is essential for normal development of communication skills • Hearing impairment = problem with or damage to one or more parts of the ear. Conductive: problem with the outer or middle ear, including the ear canal, eardrum, or ossicles. • Usu. can be corrected with medications or surgery. Sensorineural: damage to the inner ear (cochlea) or the auditory nerve. trouble hearing clearly, understanding speech, & interpreting sounds. • Hearing loss is permanent. • Treated with hearing aids or, in severe cases, a cochlear implant. Mixed both conductive and sensorineural hearing problems. • Etiology: Prenatal Factors: 1. Viral infections 2. ototoxic drugs (aspirin, streptomycin, neomycin, kanamycin) 3. Congenital syphilis 4. Hereditary disorders Perinatal Factors: 1. Toxemia late in pregnancy 2. Prematurity 3. Birth injury 4. Anoxia 5. Erythroblastosis fetalis Postnatal Factors: 1. Viral infections (mumps, measles, chickenpox, flu, &/or meningitis) 2. Injuries 3. Ototoxic drugs (aspirin, streptomycin, neomycin, kanamycin) • Management Prepare the pt & parent before the first visit with a welcome letter Let the pt & parent determine how the patient desires to communicate Assess speech, language ability and degree of hearing impairment Identify the age of onset, type, degree and cause of hearing loss Make sure the patient understands what the dental equipment is What is going to happen, and how it will feel Reassure the patient with physical contact Allow extra time for all appointments Avoid blocking the patient’s visual field Adjust the hearing aid before the handpiece is in operation (hearing aid amplifies all sounds Use of an interpreter is extremely helpful Visual impairment • Total visual impairment affects more than 30 million people • A person is considered to be affected by blindness If visual acuity doesn’t exceed 20/200 in the better eye w/ corrective lenses OR If acuity greater than 20/200 but w/ a visual field of no greater than 20 degrees • Etiology Prenatal Factors: Optic atrophy, microphthalmos, cataracts, colobomas, dermoid and other tumors, toxoplasmosis, syphilis, rubella, tuberculous meningitis, developmental abnormalities of the orbit Postnatal causes: Trauma, retrolental fibroplasias, hypertension, premature birth, polycythemia vera, hemorrhagic disorders, leukemia, diabetes mellitus, glaucoma • The capabilities of a child with blindness are difficult to assess, therefore an affected child could be misdiagnosed as developmentally delayed • Children with blindness may exhibit self-stimulating activities, such as eye pressing, finger flicking, rocking or head banging • Listening, touching, tasting, and smelling are extremely important for the affected children in helping to learn coping behavior • Dental management: Determine the degree of visual impairment Establish rapport Do not grab, move or stop the patient without verbal warning, encourage the parent to accompany the child Paint a picture in the mind of the visually impaired child by describing the office setting and treatment Introduce the office personal When making physical contact, do so reassuring Allow the patient to ask questions Protect the patient’s eyes with eyeglasses Use the touch, taste, or smell approach A patient may reject strong tastes, use small quantities of dental materials Use audio & Braille dental pamphlets explaining specific dental procedures Ideally, limit providers of the patient’s dental care to one dentist Maintain a relaxed atmosphere Von Willebrand disease • Hereditary bleeding disorder • Abnormality of von Willebrand Factor (VWF) • found in plasma, platelets, megakaryocytes and endothelial cells • Circulates as carrier protein for factor VIII • Important in platelet adhesion to the sub endothelium via collagen and formation of the primary platelet plug; • impairment may result in bleeding from skin and mucosa, bruising, and prolonged bleeding after surgical procedures Hemophilia • sex-linked genetic disorder Inherited bleeding disorder affects 1 in 7500 males • def’cy or absence of one of the clotting proteins in plasma. results in delayed clotting, bleeding involves muscle and joints • There is no cure for hemophilia, the goals of treatment are early recognition of bleeding episodes and appropriate intervention to prevent complications. Factor VIII deficiency (Hemophilia A or Classic Hemophilia) • VIII = antihemophilic factor • X-linked recessive trait (Males affected, females carriers) • It accounts for about 85% of all hemophilias (most common) • joint and muscle hemorrhages, easy bruising prolonged & potentially fatal hemorrhage after minor cuts or abrasions • If joint hemorrhages go untreated, severe limitation of motion occurs Factor IX deficiency (Hemophilia B or Christmas Disease) • IX = plasma thromboplastin component • X- linked recessive trait disorder • One fourth as prevalent as factor VIII • Factor XI = plasma thromboplastin antecedent def’cy Inherited as an autosomal recessive trait Male and female offspring equally affected Most frequently observed in those of Ashkenazi Jewish descent • Degrees of severity of Hemophilia A and B: (normal levels: 55-100%) Mild Pro-coagulant levels greater than or equal to 5% Bleed infrequently and only in association with surgery or injury May never have a bleeding problem Rarely have joint problems Diagnosis of mild deficiency is found during presurgical evaluation or when bleeding occurs in association with surgery or trauma Moderate 1%-5% factor activity levels Can bleed with slight injury May bleed 4-6 times/yr depending on activity level & stage of May have joint problems if not treated Develop a target joint (repeated episodes at same joint) • Hemophilia A & vWD: Hemophilia A Normal ↑↑ ↓↓ Normal Normal Normal vWD Normal ↑↑/ Normal ↓ ↓ ↑ Abnormal • Physical Exam and Medical/Family History: Documentation of presenting symptoms Family history including bleeding history Pt's bleeding Hx, including previous injuries, trauma response, prior surgeries. (If female, menstrual history including detailed assessment of duration, frequency, & amount of bleeding during cycle) • Positive Indications for Potential Bleeding Disorder: Excessive bleeding Swelling to soft tissues, muscles or joints Frequent mucosal bleeds (nosebleeds, oral bleeds) Excessive bleeding after trauma or surgery History of laboratory coagulopathy Hematomas noted after IM injections and/or venipunctures PT represents the extrinsic pathwayif abnormal, may be due to deficiency in FI, II, V, VII, X. If PT alone is abnormal indicative of FVII deficiency. PTT represents the intrinsic pathway if abnormal, may be due to deficiency of FI, II, V, VIII, IX, XI, XII. If PTT only is abnormal may indicate FVIII, IX, XI or XII. Normal Factor Activity levels usually range from 50%-150% or 60%-200% depending on specific laboratory parameters. • Treatment: Use of either purified concentrate made from pooled plasma or factor produced through recombinant technology; one-time correction of procoagulant to approx. 40% will achieve hemostasis Factor concentrates are generally accessible, easy to handle and store, virally inactivated, and lead to a more consistent hemostatic result The level necessary for hemostasis is the same for Factor IX as for Factor VIII for Hemophilia A: Factor VIII concentrate DDAVP (1- deamino-8-Darginine vasopressin) used for mild factor VIII-deficient hemophilia (minor hemorrhagic episodes) peak level one hour after post administer The half- life of Factor VIII is approximately 12 hours for Hemophilia B : Purified coagulation factor IX concentrate The half-life of Factor IX is approximately 24 hours for von Willebrand disease: DDAVP • Complications: Inhibitors are antibodies that may develop: …in approximately 28% of patients with severe factor VIII deficiency …in 3% to 5% of patients with severe factor IX deficiency Hemophilic patients with inhibitors should be managed only in conjunction with a hemophilia center Patients with inhibitors: High responders Low responders Arthritis: Degenerative joint disease secondary to recurrent bleeding Blood borne viral infections (transmitted via blood used for therapy) dev’pment Severe < 1% factor activity level Can bleed with no injury (spontaneous) May bleed two to four times/month Common sites of bleeding include joints, muscles and skin Hemarthoses (joint bleeding): pain, stiffness, limited movement) Chronic musculoskeletal problems Pseudotumor (hemorrhagic pseudocyst) of the jaw PT PTT Factor VIII vWF BT PLT aggregation • Abnormal Test Results: • Dental Management prior to treatment 1. Be aware of the patient’s specific type of bleeding disorder 2. Severity 3. Frequency of bleeding 4. Inhibitor status 5. Consult with hematologist in regards to the extent & invasiveness of the dental procedures, anticipated bleeding & anesthetic technique to be used • Dental Management 1. Prevention of dental disease (home dental care information, use of systemic or topical fluorides, routine dental exams) 2. Periodontal therapy 3. Restorative procedures (rubber dam, high-speed vacuum & saliva ejectors must be used with caution) 4. Pulpal therapy 5. Oral Surgery 6. Surgical complications 7. Antibiotic Prophylaxis 8. Orthodontic treatment 9. Dental Emergencies • Antifibrinolytics: control oral bleeding (prevent lysis of clots w/in oral cavity) Amicar (epsilon aminocaproic acid) Cyklokapron (tranexamic acid) can be used in mucous membrane bleeding Bone Marrow Transplantation • A bone marrow transplant (BMT), also called a stem cell transplant, is a procedure in which diseased or damaged bone marrow cells are replaced. This procedure is performed after a patient had high-dose chemotherapy or radiation treatment for conditions that did not respond to standard doses. • There are three types of bone marrow transplants: 1. Allogeneic — from a donor who may or may not be a relative. 2. Autologous — The patient receives his or her own stem cells that were collected and frozen before the high-dose chemotherapy or radiation treatment. 3. Syngeneic – stem cells from a healthy identical twin • Transplant Conditioning • goal is to destroy abnormal cells or cancer cells throughout the patient's body. • based on the type of disease, previous treatment, & clinical trial participation. • may consist of chemotherapy, radiation therapy or both. Radiation may be given before the transplant as part of the conditioning or it may be given following recovery from transplant. • During and after hemopoietic stem cell transplantation, the most common cause of serious morbidity and mortality is infection (endogenous opportunistic organisms are usually the cause of life-threatening infections) • Oral Complications: Ulcerations Mucositis Transient salivary gland dysfunction Thrombocytopenic gingival bleeding • Graft-Versus-Host Disease: • interaction of donor cells & recipient cells that display disparate antigens Acute form: involves the lymphoid system, skin, liver and GI tract Chronic form: involves cutaneous and oral mucosal involvement (mucosal erythema, lichenoid eruptions, ulcerations) • Hematopoietic Cell Transplantation (HCT) Phases: I. Pre-transplantation In HCT, the patient receives all the chemotherapy and /or total body irradiation in just a few days before the transplant There will be prolonged immunosuppresion following the transplant Elective dentistry will need to be postponed until immunological recovery occurs (9-12 months after HCT) II. Conditioning/neutropenia Oral complications are related to conditioning regimen / medical therapies Mucositis, xerostomia, oral pain, oral bleeding, opportunistic infections and taste dysfunction III. Initial engraftment to hematopoietic reconstitution Intensity and severity of complications begin to decrease normally after 3 to 4 wks after transplantation. Emphasis on Oral hygiene and topical fluoride applications. Invasive dental procedures should be done only if authorized by the HCT team because of the patient’s immunosuppression IV. Immune reconstitution/ late post-transplantation After day 100 post-HCT, oral complications are related to the chronic toxicity associated with the conditioning regimen, including salivary dysfunction, late viral infections, oral chronic GVHD Invasive dental treatment should be avoid in patients with profound impairment of immune function • Dental treatment guidelines Consult with oncologist Definitive dental care before chemotherapy/ radiation or at intervals between chemo cycles Restore all carious teeth or source of irritation and/or active infection Institute periodontal therapy and oral hygiene instructions Establish a diagnosis for all oral lesions Institute appropriate fluoride treatment Remove orthodontic and other appliances that may cause irritation Only minimum care during active phases of myelosuppression • Medical Guidelines Each patient should be considered individually Elective dental procedures when: ANC > 1000 / mm³ Platelet count >40,000 / mm³ Prophylactic antibiotic coverage according to current AHA if ANC < 1000 / mm³ OR patient has a central venous catheter Educate patients / parents about possible long-term sequelae of chemo or radiation therapy Place the patient on a 3 to 6 month recall schedule to monitor dental condition and detect long- term complications