SPECIAL NEEDS POPULATIONS Perioperative Care of the Pediatric Patient With Down 1.

9 Syndrome
ANDREA TONGE, BSN, RN, CPN, CSRN www.aorn.org/CE

he care of children with special needs in the perioperative environment poses many challenges for health care workers. Childrens unique physical, physiological, and psychological needs require that the patient care team members possess extensive knowledge of growth and developmental milestones and the physiology of various body systems. This article addresses perioperative implications for pediatric patients with Down syndrome. Down syndrome is the most commonly occurring genetic disorder, and, because of its association with a high incidence of congenital abnormalities, many children with this disorder will require surgical procedures for correction and repair that will be performed with the patient under general anesthesia.1 Technological advances in the medical eld also will likely result in an increase in the number of surgical interventions for these patients.2 It is essential that perioperative staff members acquire a comprehensive knowledge base regarding coexisting conditions to properly manage care of this patient population. HISTORY Down syndrome, also known as trisomy 21, is an autosomal genetic disorder named for J. Langdon Down, a British physician, in 1866.3 Dr Down

sought to describe common characteristics of individuals affected by the disorder to distinguish these individuals from other individuals with cognitive delays. In 1959, a geneticist, Jerome LeJeune, established one of the causes of Down syndrome as an abnormality of structure (ie, trisomy) in the 21st chromosome.4 The two other causes of the disorder, translocation and mosaicism, were determined within the following three years.4 PATHOPHYSIOLOGY Human cells normally have 23 pairs of chromosomes, for a total of 46. These cells divide by the process of meiosis to produce gametes (ie, sperm and egg cells), which contain one copy of each pair of chromosomes. In Down syndrome, one of three different types of alterations in chromosomal structure occur during the process of gamete formationnondysjunction, translocation, or mosaicism.5 Nondysjunction results when, before conception, a gamete is produced with an extra copy of chromosome 21. When this gamete unites with a normal gamete from the other parent, the embryo develops with all cells having 47 chromosomes (instead of the normal of 46), each with three copies of chromosome 21 hence the term

indicates that continuing education contact hours are available for this activity. Earn the contact hours by reading this article, reviewing the purpose/goal and objectives, and completing the online Examination and Learner Evaluation at http://www.aorn.org/CE. The contact hours for this article expire December 31, 2014.
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Fer liza on Normal gamete Nondysjunc on Meiosis I TRISOMY

Meiosis II

Chromosome 21

Fer liza on Normal gamete MONOSOMY

Figure 1. In Down syndrome, nondysjunction of chromosome 21 occurs when it fails to divide properly, resulting in monosomy (which is lethal) or trisomy.

trisomy (Figure 1). The maternal gamete is implicated in 88% of these cases.5 In translocation, which accounts for 3% to 5% of Down syndrome cases, all or part of chromosome 21 is fused to another chromosome. This form of Down syndrome is usually hereditary and is not linked with an increase in parental age, whereas trisomy and mosaicism are linked with increased maternal age. Mosaicism refers to trisomy that occurs in only a minority of cells (Figure 2). With the majority of cells remaining normal in chromosomal structure, affected individuals present with less severe symptoms, with the degree of disability relative to the number of abnormal chromosomes.6 ETIOLOGY Down syndrome occurs in one in 800 live births, spanning all ethnicities and economic levels.7 One factor that increases the risk of having a child with Down syndrome is increased maternal chronologic age (ie, 35 years or older).8 Most people believe that maternal age is the primary predictor of Down syndrome and do not realize that 80% of children with Down syndrome are born to women who are younger than age 35. In addition to maternal age, other theories regarding the etiology of Down syndrome include environmental factors such as exposure to electromagnetic radiation and x-rays, toxic effects of

pesticides, smoking, and drinking.7 However, according to the National Down Syndrome Society,8 there has been no conclusive research that links Down syndrome with environmental factors or parental behavior before conception.

CLINICAL PRESENTATION Children with Down syndrome commonly present with abnormalities in physical features, congenital malformations of various organ systems, and impairment in physical and cognitive abilities.9 They appear smaller in size than their average counterparts and develop at a slower rate both mentally and physically.10 Fifty percent of affected children have congenital heart disease, and they are at increased risk for thyroid dysfunction; gastrointestinal, hematologic, and endocrine disorders; respiratory problems; sensory impairment; and mental disabilities.9 Physical features characteristic of the disorder include a at occiput; at facial features; slanted eyes with skin folds between the inner corner of the eyes and upper eyelids; speckling of the iris; small nose and ears; small mouth with a large, protruding tongue; short neck; and sparse hair. Other manifestations include increased exibility of certain joints, hypotonia, abbiness of abdominal muscles, a single palmar
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Figure 2. In Mosaicism, trisomy occurs in only a minority of cells. Reprinted with permission from the National Genetics Education and Development Centre (www.geneticseducation.nhs.uk).

crease in the hands, and short ngers, as well as small penis and undescended testicles in males.6 All children with Down syndrome have some measure of developmental delay, including language and motor skills.1 These become apparent during the rst year of life. Cognitive impairment is manifested more in verbal short-term memory than in visual processing.6 One study demonstrated that short-term memory and comprehension could be improved through visual imagery and cues.11 Social skills are usually not impaired in children with Down syndrome. Mental capabilities vary from mild to moderate, with an IQ ranging from 50 to 70 and 35 to 50, respectively.9 Some individuals will require extensive supervision to lead a life of optimal potential, whereas others can maintain a level of independence with fewer support systems.
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TREATMENT Clinical specialists gear the treatment of children with Down syndrome toward managing any compromise in physical, physiological, or cognitive function. The goal is to use early interventions and therapies to help affected individuals lead productive lives.12 Management includes assessing all physiological systems at birth; educating the childs parents and family members about diagnostic procedures, the potential for impaired development, and treatments and medications that are indicated for medical conditions commonly associated with Down syndrome; and making appropriate referrals. It is also important for health care providers to assist parents with their coping ability; to provide the childs caregivers with the necessary resources for short-term and long-term care involving physical, occupational, and speech

SPECIAL NEEDS POPULATIONS therapies; to discuss diagnostic procedures and therapeutic regimens; and to emphasize the importance of follow-up care for managing medical conditions.12 These interventions will help children with Down syndrome maintain a healthy lifestyle and promote their physical and psychological well-being during the entire lifespan. PERIOPERATIVE IMPLICATIONS According to Zuckerberg and Maxwell,13 8% to 10% of patients diagnosed with Down syndrome develop perioperative complications during noncardiac surgical procedures. These include obstruction of the airway, bronchospasm, and difculty with intubation. Cardiac anomalies with pulmonary hypertension also place these patients at risk from the use of anesthetic agents.1 Because of the signicance of these and other associated problems, a thorough preoperative evaluation with careful planning is essential to ensure the best outcomes for these patients. The preoperative evaluation should include assessment of the patients cardiac, pulmonary and respiratory, musculoskeletal, gastrointestinal, endocrine, hematologic, developmental/psychosocial, and neurological systems. The preoperative nurse must develop an individualized plan of care to guide the management of the patient during the entire perioperative period, based on comprehensive assessment data that addresses all commonly coexisting conditions (Table 1). Cardiac Approximately 40% to 50% of children with Down syndrome have congenital cardiac anomalies that will require corrective surgical repair.1 An echocardiogram with cardiology consult is indicated preoperatively for patients with a cardiac history because of the effects of anesthetics and the stress of surgery on the heart.14 Nurses in the presurgical clinic can play an important role in ensuring that the patient with Down syndrome is screened preoperatively by educating the patients parents on the importance of keeping pre-

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operative medical appointments. Nurses can also assist the anesthesia professionals in obtaining laboratory, radiology, and other diagnostic reports. On the day of surgery, the perioperative nurse should obtain baseline vital signs, including oxygen saturation level. To prevent endocarditis, patients may need antibiotics prophylactically 30 to 60 minutes before some surgical procedures. The nurse should screen for a history of allergies and perform a reconciliation of all of the patients medications to prevent possible adverse medication interactions. Pulmonary and Respiratory The presence of a small buccal cavity, large tongue, and small nasal passages predispose children with Down syndrome to an increased risk for airway obstruction and complications related to anesthetic agents and sedatives.1,15 Airway obstruction can occur while these patients are awake or asleep, which is of special consideration in patients who receive procedural sedation without an articial airway in place. Children with Down syndrome also have a higher incidence of subglottic stenosis and may require intubation with smaller-than-expected endotracheal tubes.7 Physicians and nurses must assess for a history of obstructive sleep apnea, stridor, barky cough, snoring, or unusual sleeping positions because of the possibility of subglottic stenosis. Nurses assisting with procedural sedation must anticipate the need for smaller airway adjuncts and have them available if necessary. Obesity, hypotonia, and cardiac disease also have been associated with both lower and upper airway problems in this population.7 When performing a preoperative assessment, the perioperative nurse also must consider these other factors. Musculoskeletal Approximately 20% of children with Down syndrome have atlantoaxial instability (AAI), a condition in which there is increased mobility between the two cervical bones at the top of the neck. This causes instability of the cervical spine
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TABLE 1. Perioperative Nursing Implications: Children With Down Syndrome

Preoperative care Schedule procedures for children with diabetes early in the day to decrease fasting time. Encourage parents to comply with preoperative screening of patients. Obtain a relevant history, including information about cardiac disease, obstructive sleep apnea, stridor, diabetes, hypothyroidism, gastroesophageal reux disease, vomiting, obesity, hypotonia, neck pain, changes in gait, muscle weakness, and changes in bowel and bladder function. Obtain baseline data regarding the childs developmental age and impairment in motor skills. Assist in obtaining relevant laboratory and diagnostic reports (eg, echocardiogram, cervical spine radiograph, blood glucose level, thyroid function tests). Obtain baseline vital signs on the day of surgery. Perform a medication reconciliation and obtain the patients history of allergies. Administer antibiotics prophylactically to prevent endocarditis. For procedural sedation cases, have a large selection of airway adjuncts available. Enforce practice guidelines governing infection control and prevention. Encourage family members to participate in all procedures by giving the child his or her medications, encouraging the child to communicate with staff members, providing comforting measures during invasive procedures, and incorporating the childs routine in the plan of care. Use visual aids and gestures when communicating with the child. Prepare the patients family members for a longer-than-usual observation period postoperatively. Intraoperative care Limit neck manipulation. Prepare to assist with cricoid pressure before anesthesia induction. Observe for adverse effects from anesthetics, sedatives, or analgesics. Assess for adequate cardiopulmonary function by monitoring vital signs and ventilation status as well as level of consciousness in sedation cases. Protect the patient from heat loss and hypothermia. Maintain proper body alignment and support of extremities during patient positioning and transfer. Monitor intake and output. Postoperative care Continue to monitor cardiopulmonary status. Observe for signs and symptoms of airway obstruction. Monitor for postoperative bleeding and drainage. Assess for adverse effects of muscle relaxants. Make clinical judgments regarding pain control based on cues and information from the patients family members. Communicate with patients who have sensory impairment to prevent feelings of isolation. Provide a favorite toy or other objects of comfort. Ensure patient safety to prevent physical injury. Provide discharge instructions to the patients family members based on the interdisciplinary plan of care.

and predisposes these children to spinal cord compression.1,16 The American Academy of Pediatrics recommends cervical spine radiographs of children with Down syndrome who are three to ve years old to detect possible AAI. Because the majority of children with Down syndrome are asymptomatic for AAI, it is important that the perioperative nurse obtain a pertinent history
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regarding neck pain, changes in gait, muscle weakness, or changes in bowel and bladder function before surgery.13 A history of increased exibility of joints in the ngers and elbows is associated with AAI.7 Because of the prevalence of hypotonia and delay in motor development in these patients,1 the perioperative nurse should obtain baseline data about any impairment in motor skills

SPECIAL NEEDS POPULATIONS preoperatively to properly assess for adverse events related to the administration of muscle relaxants. Gastrointestinal Abnormalities in the gastrointestinal system, including tracheoesophageal stula, pyloric stenosis, and Hirschsprung disease, occur in about 12% of children with Down syndrome, and many will need surgical repair in the neonatal period.1 Because gastroesophageal reux disease frequently occurs in individuals with Down syndrome, the preoperative nurse should assess for a history of vomiting because of the implication for possible aspiration during anesthesia induction.

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creases their likelihood of developing acute leukemia.1 It has been documented in the literature that, although there is a high success rate in surgical procedures performed to repair cardiac anomalies in patients with Down syndrome, there is also an increase in postoperative complications and deaths that are possibly associated with recurrent infections.7 The nurse has an essential role in managing the perioperative environment to ensure that all members of the perioperative team comply with practice guidelines for infection control and prevention. Developmental and Psychosocial Compared with adults, young children in general have unique psychological responses to hospitalization in terms of feelings of separation anxiety, fear, and punishment.3 The perioperative nurse caring for the child with Down syndrome is faced with the added challenges of cognitive and sensory delays in these patients. Because these children vary in their degree of cognitive delay and may have vision and hearing decits, nurses need to know the level of impairment to communicate effectively with them. One way that nurses can do this is to collaborate with parents and family members to properly assess and determine the childs developmental age. These children also may have some difculty expressing their feelings verbally, which can result in added anxiety and

Endocrine There is an increased risk for diabetes and a prevalence of hypothyroidism in individuals with Down syndrome.1 Therefore, it is important for the preoperative nurse to collaborate with the anesthesia team in obtaining and reviewing relevant history and current laboratory values before the surgical procedure. Nurses must be knowledgeable about the signs and symptoms of these disorders to identify and report signicant ndings to the physician. An infant with hypothyroidism may present with a large tongue and an umbilical hernia. Older children with hypothyroidism might be noted with slow heart rate and intolerance to cold.15 Some perioperative implications for hypothyroidism include decreased cardiac output and delayed gastric emptying. Children with diabetes should be scheduled for Online Resources surgery early in the day to decrease their hours of National Human Genome Research Institute fasting. http://www.genome.gov/19517824 National Down Syndrome Congress Hematology http://www.ndsccenter.org/ Patients with Down synNational Down Syndrome Society drome have some measure http://www.ndss.org of immune system deciency National Dissemination Center for Children with Disabilities that renders them prone to a http://nichcy.org/disability/specic/downsyndrome higher incidence of infection relative to the general population and dramatically inWeb site access veried August 16, 2011.

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TONGE Neurological Individuals with Down syndrome may have a higher pain threshold than the general population and may react to pain more slowly.10 Research results have shown that these children also differ in their ability to precisely identify the location of painful stimuli and are less likely to communicate their pain verbally.18 Parents can provide the health care team with information about pain indicator behaviors that their child exhibits (eg, use of certain words, pointing, expressions of anger, acting out) to help effectively assess and control the childs pain. INTRAOPERATIVE CARE The goal in the intraoperative period focuses on patient safety while the patient is anesthetized or sedated. Precautions should be taken to limit neck manipulation in all children with Down syndrome because some patients are asymptomatic for AAI. The childs neck should be maintained in a neutral position with a soft collar to avoid extreme neck exion, extension, or rotation.7 Because of the incidence of airway abnormalities, it is important to have different sizes of airway modalities available to effectively manage respiratory complications. The intraoperative nurse should be prepared to assist anesthesia professionals with the application of cricoid pressure in the event of a rapid sequence induction in a child with a history of gastroesophageal reux and vomiting. Nurses administering or assisting with procedural sedation should have substantial knowledge of sedatives, analgesics, and reversal medications, and implications for patients with Down syndrome who have coexisting conditions. The sedation nurse should constantly assess for adequate cardiopulmonary function by monitoring vital signs, ventilation status, and level of consciousness. Infection control measures should continue to be instituted by the intraoperative nurse. In the event of hypothyroidism, coupled with the challenge of temperature regulation in the pediatric age group, care should be taken to protect against

frustration.8 Adolescent patients with Down syndrome might present with a history of emotional and behavioral problems such as aggression and exaggerated anger.17 The administration of anxiolytics or sedatives might be benecial. The nurse should communicate with the patient on the patients cognitive level by using simple instructions, preferably with visual aids and gestures. The nurse also can observe the childs social interaction with family members to identify cues that might decrease anxiety and fear. Nurses should explore their own feelings and attitudes about caring for these children to reveal possible biases and barriers that could affect their nursing care.6 According to Hayes,16 many children with Down syndrome resist changes in their routine. Nurses should try to incorporate the childs routine into the plan of care to decrease anxiety and promote cooperation. Parents can identify activities that the child can do independently (eg, changing clothes to don hospital attire, selfadministering oral medications with supervision). These activities should be encouraged to develop the childs sense of trust in the environment.6 The patients family members should be prepared with the knowledge that their child might need to be observed for a longer period postoperatively than is typically expected for the procedure, depending on the childs status after the procedure. Patients undergoing ambulatory surgery might require an overnight stay for further observation. It might be benecial to have the childs favorite toy, blanket, or other comforting object on hand in case the postoperative stay is prolonged. There is an increased rate of mental illness in older individuals with Down syndrome. In some health care institutions, care for the pediatric population extends past 18 years of age. Nurses and health care providers in these institutions need to be knowledgeable of state laws and regulations and of institutional policies that address the issue of consent for treatment for persons with mental illness.10
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SPECIAL NEEDS POPULATIONS heat loss and hypothermia. Proper body positioning and support of extremities are also important in this population because of possible hypotonia. Nurses must ensure that extra care is taken to appropriately support joints and limbs throughout patient transfer.

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POSTOPERATIVE CARE Postoperative care of the pediatric patient with Down syndrome focuses on continued monitoring of cardiopulmonary status, with an emphasis on observing for signs and symptoms of airway obstruction, laryngospasm, and postextubation croup. The patient care team in the postanesthesia care unit should determine the critical aspects of the patients status that could affect his or her recovery.3 Nurses should monitor for postoperative bleeding or drainage, which could affect hemodynamic status. Strict intake and output should also be observed to prevent possible uid overload.3 The patient care team should use baseline data to assess for adverse effects of muscle relaxants because of the prevalence of motor delays in these children. Nurses must make clinical judgments regarding pain control and the administration of analgesics, because children with Down syndrome may not show obvious evidence of discomfort.10 The nurse might need to make a decision based on the type of procedure and the anesthetic agents and medications used during the procedure.3 In patients with a history of hearing loss or vision impairment, nurses must be especially sensitive to anticipate the childs needs and communicate with him or her to prevent feelings of isolation.6 Patient safety must be considered, and the childs immediate surroundings must be secured to prevent physical injury. Discharge plans from the postoperative area should incorporate an interdisciplinary approach, addressing the patients physical, physiological, and psychosocial needs, and should be based on the preoperative assessment and on intraoperative and postoperative events.

CONCLUSION Pediatric patients with Down syndrome pose considerable challenges for the entire perioperative team. A careful preoperative screening and evaluation is crucial to target issues of special concern relative to this population. Nurses can function as facilitators to ensure that a multidisciplinary approach is used in anticipation of perioperative complications and in the development of a comprehensive plan of care. The psychosocial needs of these children must be given special attention because of the incidence of cognitive and sensory impairment, and effective communication with parents and families must be used to meet these needs. Extra time must be secured to allow for the management of possible adverse events related to coexisting medical conditions. With the application of the most current knowledge, evidence-based practice, and careful planning, the health care team can prevent complications and ensure a positive perioperative experience for pediatric patients with Down syndrome. Acknowledgement: The author thanks Michelle Byrne, PhD, RN, CNE, CNOR, associate professor and coordinator for the Master of Science in Nursing Education Program at North Georgia College and State University, Dahlonega, GA, for her guidance and support in the preparation of this manuscript.
References
Hach M, Sun L. Perioperative management of patients with Down syndrome. Curr Ped Rev. 2009;5(3):180183. 2. Allsopp D. Improving hospital experiences for children with complex needs. Paediatr Nurs. 2006;18(9):24. 3. Phippen M, Ulmer B, Wells M. Competency for Safe Patient Care During Operative and Invasive Procedures. Denver, CO: Competency and Credentialing Institute; 2009. 4. Leshin L. Trisomy 21: the story of Down syndrome. Updated May 2009. http://www.ds-health.com/trisomy .htm. Accessed August 16, 2011. 5. McCance K, Huether S, Brashers V, Rote N. Pathophysiology: The Biologic Basis for Disease in Adults and Children. 6th ed. Maryland Heights, MI: Mosby Elsevier; 2010. 1.

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view/Point-of-Care-Quick-Reference/397156/3 [membership required]. Accessed July 4, 2011. Kliegman RM. Anesthesia, perioperative care and sedation. In: Kliegman RM, Stanton BMD, St Geme J, Schor N, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011. Motoyama EK, Davis PJ. Smiths Anesthesia for Infants and Children. 7th ed. Philadelphia, PA: Mosby Elsevier; 2006. Hayes J. Perioperative care planning for a Downs syndrome patient. J Perioper Pract. 2010;20(2):70-73. Bawle E. Chapter 258: Down syndrome: managing the child and family. In: American Academy of Pediatrics. Textbook of Pediatric Care. Pediatric Care Online. 2008. https://www.pediatriccareonline.org/pco/ub/view/ Point-of-Care-Quick-Reference/397156/3 [subscription required]. Accessed July 19, 2011. Davies RB. Pain in children with Down syndrome: assessment and intervention by parents. Pain Manag Nurs. 2010;11(4):259-267.

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Hockenberry MJ, Wison D, Winkelstein M, Kline N. Wongs Nursing Care of Infants and Children. 7th ed. St Louis, MO: Mosby; 2003. Meitzner MC, Skurnowicz JA. Anesthetic considerations for patients with Down syndrome. AANA J. 2005;73(2):103-107. What causes Down syndrome? National Down Syndrome Society. http://www.ndss.org/index.php?option com_content&view article&id 60:what-causesds&catid 35:about-down-syndrome&Itemid 77. Accessed August 16, 2011. Ostermaier KK. Clinical features and diagnosis of Down syndrome. UpToDate. http://www.uptodate .com/contents/clinical-features-and-diagnosis-of-downsyndrome?source search_result&selectedTitle 1%7E150. Accessed August 16, 2011. Hayes C. Down syndrome: caring holistically in primary health care. Br J Community Nurs. 2007;12(9): 404-410. Lecas JF, Mazaud AM, Reibel E, Rey A. Using visual strategies to support verbal comprehension in an adolescent with Down syndrome. Child Lang Teach Ther. 2011;27(1):84-96. Pinto S, Schub T. Down syndrome. In: Pravikoff D, ed. Cinahl Information Systems. Glendale, CA; 2010. http://search.ebscohost.com/login.aspx?direct true&db rzh&AN 5000000373&site ehost-live. Accessed August 16, 2011. Zuckerberg AL, Maxwell LG. Chapter 62: preoperative assessment. Down syndrome. In: American Academy of Pediatrics. Textbook of Pediatric Care. Pediatric Care Online. https://www.pediatriccareonline.org/pco/ub/

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Andrea Tonge, BSN, RN, CPN, CSRN, is a resource staff nurse, Childrens Healthcare of Atlanta, GA. Ms Tonge has no declared afliation that could be perceived as posing a potential conict of interest in the publication of this article.

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EXAMINATION
CONTINUING EDUCATION PROGRAM

1.9
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Perioperative Care of the Pediatric Patient With Down Syndrome

PURPOSE/GOAL
To educate perioperative nurses about caring for the pediatric surgical patient with Down syndrome.

OBJECTIVES
1. 2. 3. 4. 5. Discuss the pathophysiology of Down syndrome. Identify the etiology of Down syndrome. Describe the clinical presentation of Down syndrome. Describe treatments used for children with Down syndrome. Discuss implications of perioperative care for a patient with Down syndrome.

The Examination and Learner Evaluation are printed here for your convenience. To receive continuing education credit, you must complete the Examination and Learner Evaluation online at http://www.aorn.org/CE.

QUESTIONS 1. In Down syndrome, the types of alteration in chromosomal structure that may occur during the process of gamete formation include 1. mosaicism 2. nondysjunction 3. translocation 4. trisomy a. 1 and 2 b. 3 and 4 c. 1, 2, and 3 d. 1, 2, 3, and 4 2. Down syndrome occurs in one in _________ live births. a. 800 b. 1,000 c. 1,200 d. 1,500 3. Children with Down syndrome commonly present with
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1. abnormalities in physical features. 2. congenital malformations of various organ systems. 3. heightened cognitive abilities. 4. impaired physical abilities. a. 1 and 2 b. 3 and 4 c. 1, 2, and 4 d. 1, 2, 3, and 4 4. Children with Down syndrome are at an increased risk of 1. congenital heart disease. 2. gastrointestinal, hematologic, and endocrine disorders. 3. respiratory problems. 4. sensory impairment. 5. thyroid dysfunction. a. 1 and 5 b. 2, 3, and 4 c. 1, 2, 4, and 5 d. 1, 2, 3, 4, and 5
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5. intolerance to heat. 6. increased heart rate. a. 1, 3, and 5 c. 1, 2, 3, and 4

5. Interventions that can help children with Down syndrome maintain a healthy lifestyle include 1. assessing all physiologic systems at birth. 2. educating the childs parents and family members about diagnostic procedures, the potential for impaired development, and treatments and medications that may be needed. 3. assisting the parents with their coping abilities. 4. referring patients for physical, occupational, and speech therapies as appropriate. a. 1 and 3 b. 2 and 4 c. 1, 2, and 3 d. 1, 2, 3, and 4 6. Research indicates that __________ of patients with Down syndrome develop perioperative complications during noncardiac surgical procedures. a. 8% to 10% b. 12% to 15% c. 20% to 25% d. 33% to 38% 7. Children with Down syndrome have a higher incidence of subglottic stenosis and may require intubation with smaller-than-expected endotracheal tubes. a. true b. false 8. Pediatric patients with Down syndrome who have endocrine disorders may present with 1. a large tongue. 2. an umbilical hernia. 3. decreased cardiac output. 4. delayed gastric emptying.

b. 2, 4, and 6 d. 1, 2, 3, 4, 5, and 6

9. To address the patients unique psychological responses to hospitalization, the perioperative nurse should 1. collaborate with the patients parents to determine the childs developmental age. 2. communicate by using simple instructions with visual aids, keeping gestures to a minimum. 3. avoid administering anxiolytics or sedatives. 4. incorporate the childs routine into the plan of care. 5. identify cues that might decrease anxiety and fear by observing the patients interaction with his or her family members. a. 2 and 3 b. 1, 4, and 5 c. 1, 3, 4, and 5 d. 1, 2, 3, 4, and 5 10. During the intraoperative period, the perioperative nurse should 1. be prepared to assist with applying cricoid pressure in the event of rapid sequence induction. 2. ensure different sizes of airway modalities are available to manage respiratory complications. 3. take precautions to limit neck manipulation. 4. take extra care to support joints and limbs throughout patient transfer. a. 1 and 4 b. 2 and 3 c. 1, 2, and 3 d. 1, 2, 3, and 4

The behavioral objectives and examination for this program were created by Kimberly Retzlaff, editor, with consultation from Rebecca Holm, MSN, RN, CNOR, clinical editor, and Susan Bakewell, MS, RN-BC, director, Center for Perioperative Education. Ms Retzlaff, Ms Holm, and Ms Bakewell have no declared afliations that could be perceived as posing potential conicts of interest in the publication of this article.

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LEARNER EVALUATION
CONTINUING EDUCATION PROGRAM

1.9
www.aorn.org/CE 9. Will you change your practice as a result of reading this article? (If yes, answer question #9A. If no, answer question #9B.) 9A. How will you change your practice? (Select all that apply) 1. I will provide education to my team regarding why change is needed. 2. I will work with management to change/ implement a policy and procedure. 3. I will plan an informational meeting with physicians to seek their input and acceptance of the need for change. 4. I will implement change and evaluate the effect of the change at regular intervals until the change is incorporated as best practice. 5. Other: 9B. If you will not change your practice as a result of reading this article, why? (Select all that apply) 1. The content of the article is not relevant to my practice. 2. I do not have enough time to teach others about the purpose of the needed change. 3. I do not have management support to make a change. 4. Other: 10. Our accrediting body requires that we verify the time you needed to complete the 1.9 continuing education contact hour (114-minute) program:

Perioperative Care of the Pediatric Patient With Down Syndrome

his evaluation is used to determine the extent to which this continuing education program met your learning needs. Rate the items as described below. OBJECTIVES To what extent were the following objectives of this continuing education program achieved? 1. Discuss the pathophysiology of Down syndrome. Low 1. 2. 3. 4. 5. High 2. Identify the etiology of Down syndrome. Low 1. 2. 3. 4. 5. High 3. Describe the clinical presentation of Down syndrome. Low 1. 2. 3. 4. 5. High 4. Describe treatments used for children with Down syndrome. Low 1. 2. 3. 4. 5. High 5. Discuss implications of perioperative care for a patient with Down syndrome. Low 1. 2. 3. 4. 5. High CONTENT 6. To what extent did this article increase your knowledge of the subject matter? Low 1. 2. 3. 4. 5. High 7. To what extent were your individual objectives met? Low 1. 2. 3. 4. 5. High 8. Will you be able to use the information from this article in your work setting? 1. Yes 2. No

This program meets criteria for CNOR and CRNFA recertication, as well as other continuing education requirements. AORN is accredited as a provider of continuing nursing education by the American Nurses Credentialing Centers Commission on Accreditation. AORN recognizes these activities as continuing education for registered nurses. This recognition does not imply that AORN or the American Nurses Credentialing Center approves or endorses products mentioned in the activity. AORN is provider-approved by the California Board of Registered Nursing, Provider Number CEP 13019. Check with your state board of nursing for acceptance of this activity for relicensure.

Event: #11525; Session: #0001 Fee: Members $9.50, Nonmembers $19 The deadline for this program is December 31, 2014. A score of 70% correct on the examination is required for credit. Participants receive feedback on incorrect answers. Each applicant who successfully completes this program can immediately print a certicate of completion.

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