Pathophysiology

Once you complete this section, you should be able to: 1. List common pediatric diseases 2. Discuss pathophysiology as it relates to chronic illnesses of childhood 3. List clinical signs and symptoms of common childhood diagnosis 4. List appropriate interventions and nursing considerations

Contents

Introduction ...... 133 Prematurity ...... 133 Bronchopulmonary dysplasia ...... 137 Asthma ...... 141 Status asthmaticus ...... 144 Apnea ...... 147 Seizures ...... 149 Gastroesophageal reflux disease (GERD) ...... 155 Cerebral palsy (CP) ...... 159 Neuromuscular diseases ...... 164

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Introduction There are numerous clinical diagnoses that can affect a pediatric home health care client. A small number of these are quite common and most of our clients are diagnosed with at least one of these frequently occurring conditions at one time or another. This chapter explains these disease processes, signs and symptoms or complications of each, and nursing considerations.

Prematurity A premature infant is defined as any infant born before 37 weeks of gestation. Each day in the United States, more than 1,300 infants are born prematurely. Preeclampsia, multiple gestation (twins, etc.), and other maternal diseases may contribute to preterm labor. Often the cause of prematurity is unknown.

A human takes approximately 40 weeks from the time of conception until the baby is fully developed. An infant born at less than 37 weeks gestation is called a premature or preterm infant.

Preterm infants are at increased risk for complications at birth and developmental problems as they get older. This is because their organs are not fully developed for survival outside the womb. The earlier the number of weeks from conception that the infant is born, the greater the risk for long-term physical and developmental problems.

Actual gestational age of a preterm infant is important to calculate (see chapter 3, Growth and development, Adjusted or corrected age). This helps physicians anticipate what complications an infant might exhibit in order to prepare for the infant’s needs. Knowing the gestational age of an infant is also helpful for anticipating what can be expected of a child developmentally. Some of a young infant’s development is strongly influenced by his or her experiences. On the other hand, much of their development is going to unfold as their body grows and evolves in a set biological sequence. A 2-month-old does not have the muscle strength or the neurological development to control their body in a sitting position. However, at around 6 months, the infant’s body has matured enough to be able to accomplish this task. A baby who was born early may be 6 months from birth, but in terms of corrected age, may be only 3 months old. A child’s nerves, brain, and muscles do not leap forward to “normal” in their development if the child was born early. It is best to adjust the infant’s age and expect them to progress at the rate they would if the child had been born at full term.

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Signs and symptoms • Low birth weight – usually < 2500 grams • Thin, fragile, translucent skin • Lanugo – fine body hair • Soft ear cartilage • Weak, ineffective, or uncoordinated suck and swallow reflex • Weak cry

Frequent complications • Respiratory distress syndrome • Intraventricular hemorrhage • Retinopathy of prematurity • Apnea of prematurity • Anemia of prematurity • Patent ductus arteriosis • Bronchopulmonary dysplasia • Infection/sepsis

Nursing considerations The nursing considerations related to respiratory distress syndrome and bronchopulmonary dysplasia will be discussed under the bronchopulmonary dysplasia section. The nursing considerations for apnea of prematurity will be discussed in the section on apnea.

Related to intraventricular hemorrhage Intraventricular hemorrhage (IVH) is the result of the rupture of fragile blood vessels in the brain of a preterm infant. They are graded on a scale from I to IV based on their severity, with I being the least severe and IV being the most severe. Consequences include possible developmental difficulties; cerebral palsy; impairment in speech, hearing, or vision; seizures; mental retardation; and hydrocephalus.

Family education should include reinforcement of the need for routine follow-up and developmental screenings for signs and symptoms of seizure activity.

For a client who has hydrocephalus, a ventriculoperitoneal (VP) shunt may be surgically inserted to relieve intracranial pressure. A client with a VP shunt should be observed for shunt malfunction, infection, or blockage 134

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of the shunt. Symptoms of possible malfunction of the shunt include headache, fever, drowsiness, poor feeding, vomiting, and seizures.

Related to retinopathy of prematurity Retinopathy of prematurity (ROP) is the abnormal growth of blood vessels within the retina and vitreous. It occurs in 16% of all preterm infants. Risk factors for ROP include a birth weight < 1500 grams, gestational age < 32 weeks, and probable treatment with high concentrations of oxygen. Long- term complications can include visual impairment from low vision to vision loss, glaucoma, retinal detachment, strabismus, and amblyopia. Family education should include reinforcement of the need for routine follow-up to observe for the development of complications in the eyes. The family should be aware of protecting the infant’s better eye, including the use of protective eyewear full-time when deemed appropriate, and strict adherence to physician orders regarding eye patching and eyeglasses.

Related to anemia of prematurity Anemia of prematurity (AOP) is a very common condition due to a decrease in the number of circulating red blood cells. This type of anemia is caused by one of the following: blood loss before and/or during delivery, tight umbilical cord around the baby’s neck, shorter lifespan of red blood cells compared with those of an adult, mismatch of the baby’s and mother’s blood types, and inability of the body to make enough red blood cells to keep up with rapid growth rate. Symptoms of AOP include pale skin color; tachypnea or increased number of apneic episodes; decreased activity or lethargy; tachycardia at rest; and tiredness with feeding or poor feeding habits plus slow weight gain.

Treatment works slowly over a few weeks and includes Erythropoietin in combination with iron supplement formulas, vitamins with iron, or iron drops.

Related to patent ductus arteriosis Patent ductus arteriosis (PDA) occurs when a blood vessel that is normal in utero fails to close after birth. While in utero, the mother provides oxygen and the baby’s lungs are filled with fluid. Blood flow during this time bypasses the lungs through a blood vessel that connects the pulmonary artery with the aorta.This blood vessel is called the ductus arteriosus. When it remains open after birth, the condition is called patent ductus arteriosus.

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In most babies, the ductus arteriosus remains open for a short time after birth, but in 90 percent of infants, it will be closed by 8 weeks. In most of the 10 precent whose ductus arteriosus does not close within 8 weeks, it will close during the first year of life. Small PDAs are usually asymptomatic and require no treatment. A heart murmur is often the only clue that a child has a PDA. If the infant is in heart failure, it will be detected by poor weight gain, frequent emesis, tachycardia, tachypnea, and hepatomegaly. Medications such as Digoxin or a diuretic may be prescribed to control cardiac symptoms until the child grows and the PDA diminishes or closes.

Children with PDAs are at an increased risk for subacute bacterial endocarditis (SBE). SBE can occur after a dental or other medical procedure but can usually be prevented by a dose of antibiotic prior to the procedure.

Related to sepsis Sepsis is more prevalent in premature infants because their immune systems have not developed enough to fight off overwhelming infections. Sepsis is usually caused by bacteria that make toxins that cause the body’s immune system to attack the body’s own organs and tissues. These bacteria can originate in the infants lungs, intestines, urinary tract, or gallbladder.

Symptoms include a rectal temperature of 100.4o F or below 96.8o F, lethargy, poor feedings, mottled skin, and signs and symptoms of respiratory distress.

Special attention to infection control is essential because an infection may be devastating for premature infants. Nurses must be vigilant in their assessment to ensure prompt referral for medical treatment.

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General considerations Keep these general considerations in mind:

Thermoregulation Premature infants lack the body fat to maintain their body temperature and it is necessary to keep the infant swaddled in blankets. Since a great deal of heat is lost through the head, a hat is also helpful in maintaining temperature.

Nutrition and growth Because of their accelerated rate of growth and their immature digestive systems, premature infants have special nutritional needs. Breast milk is an excellent nutritional source because it contains proteins that are easily digested and help prevent infection. If breastfeeding, the mother may need to augment her milk with either breast-milk fortifier or a high calorie premature formula.

Also available are specially designed formulas for growing preemies that have a high caloric content and contain supplemental vitamins, minerals, and calcium required for rapid growth. If an infant is younger than 32 to 34 weeks gestational age, he or she may not be able to be fed directly from the breast or bottle because of a weak, immature sucking reflex. These infants are generally fed through a feeding tube. Weekly weights are an important aspect of care for the premature infant.

Bronchopulmonary dysplasia Bronchopulmonary dysplasia (BPD) is a common lung disease related to neonates, usually preterm, treated with oxygen and positive pressure ventilation. Clinically, BPD is determined when the infant has (1) a persistent oxygen requirement at 28 days to maintain the arterial oxygen concentration at greater than 50 mm Hg and (2) abnormal changes on the chest x-ray. BPD is uncommon in infants born at more than 1,200 grams and after 30 weeks of gestation. Improved antenatal care, early surfactant therapy, and gentler modes of ventilation have greatly reduced the incidence of BPD.

Disease process The pulmonary tree consists of the trachea (also called the windpipe), which divides into two smaller main bronchi. Off of these form smaller tubes called bronchioles. These terminate into tiny air sacs called alveoli.

In the premature infant these alveoli are not fully developed or lined with surfactant, a fat-based fluid that decreases the surface tension to help the 137

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alveoli remain inflated. The combination of the inadequate numbers of alveoli and inadequate function makes the preterm lung “stiff.” The resulting respiratory distress in the newborn often requires treatment with oxygen and mechanical ventilation. Even though the lungs seem stiff, they are very fragile and damage easily.

In response to mechanical ventilation, some of these tiny air sacs may become overdistended and air may have difficulty entering and exiting. The lining of the air sac where gas exchange takes place at the cellular level becomes damaged and thickened. This makes the exchange of oxygen and carbon dioxide more difficult. Often this results in the need for treatment with supplemental oxygen for prolonged periods, which makes it even more difficult to get air into and out of the alveoli. In response to the resulting continued damage, the body’s normal immune response eventually causes inflammation, which in turn causes swelling of the airway, bronchospasm, increased mucous production, and eventually scarring. This sequence of events sets the stage for an infant with significant impairment in respiratory gas exchange.

Over time, the pressure required to perfuse blood through damaged lungs may result in changes in the heart. Hypertrophy of the right ventricle with resulting symptoms of heart failure may develop.

The increased work of breathing results in significantly increased metabolic needs. Infants with BPD may need considerably more calories to gain weight. Inability to handle large volumes of liquid, poor appetite, and gastrointestinal reflux disease may make gaining weight even more difficult. Medications to treat BPD may also affect absorption of certain vitamins and minerals, making these infants prone to low bone density.

Infants diagnosed with BPD often have prolonged neonatal hospital stays resulting in increased exposure to negative stimuli and less exposure to normal healthy stimuli. As a result, they may not handle external stimuli well as normal infants and may easily become agitated. They may also have less energy for developmental activities due to the excess energy expended to breathe. Their parents may be timid in handling them and as a result may not offer these infants the cuddling time usually afforded a newborn. All these factors, together with frequent episodes of hypoxemia, make developmental delays common in infants with BPD.

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BPD emergencies BPD emergencies are characterized by respiratory distress with tachypnea, grunting, nasal flaring, shallow ineffective respirations, tachycardia, wheezing, cough, fatigue, duskiness, and agitation. This can progress to a “BPD spell,” which is characterized by poor air entry, central cyanosis, diaphoresis, and bradycardia, even with positive pressure ventilation.

Signs and symptoms • Tachypnea • Coughing • Cyanosis • Feeding difficulties • Respiratory distress • Pulmonary hypertension • Wheezing • Oxygen dependency

Goals of treatment 1. Maintain adequate oxygenation by using the lowest possible amount of supplemental oxygen. 2. Wean from oxygen support as quickly as possible. 3. Develop and maintain a feeding plan that the client can tolerate and still gain height and weight consistently. 4. Progress from enteral to oral feeding if appropriate. 5. Prevent infection. 6. Progress appropriately for developmental age. 7. Develop competent caregivers in all aspects of infant care.

Response to acute respiratory distress 1. Maintain airway. 2. Do not feed during the episode. 3. Reposition to facilitate removal of secretions. 4. Suction PRN or as ordered. 5. Administer nebulizer treatment, oxygen, and chest physical therapy as ordered.

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6. Consider PRN sedation for agitation, if ordered 7. Notify physician if symptoms are unresolved 8. Activate 911 and initiate CPR, if indicated

Nursing considerations 1. Complete a respiratory assessment with vital signs and pulse oximetry at the beginning of each shift and note any changes in status as they occur. 2. Notify physician for significant changes from baseline. 3. Treat episodic bronchospasm per physician orders. 4. Monitor hydration status closely by observing for signs of overhydration or underhydration. 5. Cluster the care to provide for adequate rest and to decrease agitation. 6. Position infant to promote optimal ease of respirations. 7. Follow excellent hand hygiene practices and enforce with family and visitors. 8. Educate primary caregivers regarding the importance of immunizations and routine well-child checkups. 9. Provide proper disinfection of all reusable respiratory equipment such as nebulizer cups and suction equipment. 10. Provide small, frequent feedings. 11. Provide oral feedings in a calm and relaxed environment. Provide supplemental oxygen as needed to maintain acceptable saturation during feeding. Provide frequent rest periods during feedings, as needed. 12. If client is eating solid foods, offer foods high in caloric density. 13. Follow recommendations of therapy team (SLP/PT/OT) for developmental care. 14. Know your client. Preventing respiratory distress and BPD spells is better than treating them. Does your client need additional oxygen before suction or with feedings? Does your client need to be sedated prior to uncomfortable procedures?

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Asthma Asthma is a disease of the lungs affecting more than 15 million Americans and more than one-third of these are children. Asthma is the most common chronic condition of childhood. It is characterized by inflammation of the airway, increased mucous production, contraction of the bronchial smooth muscle, and episodic hyperactivity of the airways.

Disease process Asthma is defined by the presence of the “classical triad” of (1) eosinophilia of the mucosa or thickening of the airway, (2) increased mucous production, and (3) contraction of the smooth muscles of the airway. Hyper- responsiveness is also a characteristic feature (responsiveness is the ease with which the airway narrows in response to various stimuli such as inhaled irritants, exercise, hyperventilation, and cold air). With the events of the classical triad combined with hyper-responsiveness, the results are poor air exchange, air trapping, and shortness of breath.

With proper treatment and a team approach, most children can lead a normal life. However, asthma can be a life-threatening disease. Families must work together with health care professionals to develop a plan for the child.

Signs and symptoms • Cough • Wheeze • Tachypnea • Dyspnea • Prolonged expiration • Anxiety

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• Monosyllabic speech • Diaphoresis • Fatigue • Abdominal pain and vomiting • Tachycardia • Cyanosis

Goals of treatment 1. Achieve near-normal pulmonary function test results. 2. Maintain normal physical activity and sleep patterns. 3. Reduce exacerbations by elimination of exposures to allergens and triggers. 4. Reduce need for emergency visits for acute exacerbations and hospitalizations. 5. Reduce doses and side effects of medications. 6. Use of inhaled beta agonists < twice daily.

Treatment modalities Medications and nonpharmacological interventions are treatment modalities for asthma.

Long-acting medications • Inhaled or oral steroids (Flovent, Pulmicort, Orapred) • Cromolyn sodium (Intal) • Leukotriene antagonist (Singulair) • Long-acting beta-2 agonist (Serevent) • Methylxanthines

Rescue medications • Inhaled short acting beta-2 agonists (albuterol, Xopenex) • Inhaled anticholinergic (Atrovent) • Oral steroid

Nonpharmacological interventions • Control of precipitating factors (triggers)

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• Prevention of infection (ie, hand hygiene, adequate rest, avoidance of individuals with known illnesses) • Immunizations appropriate for age as well as annual influenza immunization • Education of client and primary caregivers related to signs and symptoms of asthma, triggers, symptom management, medications, use of peak flow meter, spacers or nebulizers, management of acute exacerbations

Assessment tools • Observation – ease of respirations, activity level, respiratory rate, color • Stethoscope – listen for a wheeze or other adventitious breath sound • Pulse oximeter – monitor oxygen saturations • Peak flow meter – hand-held monitor that measures the fastest rate at which air can move through the airways during forced expiration

Instructions for a peak flow meter 1. Assume an upright position. 2. Make sure indicator is reading zero. 3. Instruct client to take a deep breath, filling lungs completely. 4. Place mouthpiece in client’s mouth and have them lightly bite it and close their lips around it tightly. 5. Blow the air out as hard and as fast as possible in a single breath. 6. Remove the meter from their mouth. 7. Record the number that appears on the meter. 8. Repeat steps 1 to 7 two more times. 9. Record the highest of the three readings, which is their peak expiratory flow (PEF).

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Status asthmaticus Status asthmaticus is the term for a severe asthma attack that does not respond to the usual therapies. Although often reversible, it is a life- threatening emergency because it can lead to respiratory failure and cardiac arrest.

The symptoms include extreme difficulty with breathing, which causes restlessness and anxiety. Although coughing and wheezing are common symptoms of asthma, a child with status asthmaticus may not cough or wheeze because there is not enough airflow. Advanced symptoms include little or no breath sounds, inability to speak, cyanosis, and diaphoresis.

This is a medical emergency that is beyond the scope and practice of home health care. A child presenting with these symptoms should be referred for immediate medical care.

While awaiting transport, treatment may include supplemental oxygen to increase blood oxygen levels, inhaled bronchodilators to reduce inflammation, and anticholinergics to block smooth-muscle contraction and the production of excess mucus, as ordered by the physician.

Response to acute asthma episodes 1. Maintain airway. 2. Reposition to facilitate removal of secretions. 3. Suction PRN, as ordered. 4. Administer nebulizer treatments, oxygen, and CPT as ordered. 5. Follow emergency action plan 6. Do not feed during the episode 7. Notify physician if symptoms are unresolved 8. Call 911 and initiate CPR, if indicated

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Nursing considerations 1. Perform a complete respiratory assessment with pulse oximeter, if available, at the beginning of each shift and with any changes in status. 2. Maintain excellent infection control. Do not report to work with an untreated upper respiratory infection. 3. Know your client’s action plan for acute symptom management. 4. Know the location of all rescue medications and how they are administered. 5. Educate all caregivers for optimal management of symptoms and review frequently. 6. Ensure proper administration of inhaled medications. (children younger than 5 years should use a spacer, either with a mask or a nebulizer). 7. Educate yourself on your client’s asthma medications. 8. Know your client’s asthma triggers, which may include any of the following: • Trees, shrubs, weeds, or grasses • Mold • Pollen • Air pollution • Spores • Dust mites • Cockroaches • Odors • Sprays • Exercise • Cold air • Tobacco smoke • Sulfites • Animals • GE reflux • Medications • Cold/infection • Wood smoke • Food additives • Strong emotions

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Apnea Apnea is a lapse of spontaneous breathing for greater than 20 seconds, which may or may not be followed by bradycardia or color change.

Classifications of apnea 1. Central apnea – a pause in respiration caused by the failure of the brain to stimulate respiration. The cause may be immaturity of the breathing centers in the brain (as in apnea of prematurity), compression of the brain stem, brain injury, seizure, or other neurologic reason. 2. Obstructive apnea – a pause in respiration due to obstruction of the airway; such as enlarged tonsils and adenoids. This type of apnea occurs mostly during sleep when the oropharnyx is relaxed. It may occur in as many as 1 to 3 percent of healthy preschool children. Symptoms may include snoring, labored or noisy breathing while sleeping, gasping for air, restless sleep, frequent position changes, color change, or desaturations during sleep. 3. Mixed apnea – a combination of central and obstruction apnea.

Conditions associated with apnea Apparent life-threatening events (ALTEs) – ALTEs have many causes, which could include central apnea, obstructive apnea, GERD, cardiac arrhythmia, or seizure disorder. Most studies show a male predilection, with some studies showing the male-to-female ratio as high as 2 to 1. The typical age of presentation is 8 to 14 weeks and approximately 7% of those affected were born prematurely.

Childhood obstructive sleep apnea (OSA) – Obstructive sleep apnea (OSA) is a syndrome characterized by episodic upper airway obstruction that occurs during sleep. The obstruction may be complete or partial. In contrast to primary snoring, OSA is accompanied by varying degrees of oxygen desaturation, hypercapnea, autonomic stimulation, and sleep fragmentation. Common symptoms of OSA include restless sleep, loud night snoring (sometimes with audible pause followed by gasping or snorting), nocturnal enuresis, nightmares, and headaches upon rising. Long-term untreated OSA may result in failure to thrive, neurobehavioral issues, pulmonary hypertension, and cor pulmonale. OSA is graded on an apnea-hypopnea index (AHI), calculated by the number of apneic/hypopneic episodes that occur divided by the total number of hours of sleep. An AHI of 1 to 5 is considered very mild, 5 to 10 is mildly increased, 10 to 20 is moderately increased, and higher than 20 is severely abnormal. Most physicians will treat children whose AHI is greater than 5 or whose respiratory events include desaturations below 85%.

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Causes of childhood OSA – OSA in children is caused by craniofacial deformity, tonsillar hypertrophy, adenoid hypertrophy, and (rarely) fatty infiltration of the pharyngeal soft tissue due to obesity. Children with neuromuscular diseases like Duchenne muscular dystrophy, small muscle atrophy (SMA), and cerebral palsy may have a higher risk of developing OSA.

Treatment of OSA – OSA treatment varies greatly and is triggered by the cause. The most frequent cause of OSA in children is adenotonsillar hypertrophy, which would most likely be treated with a tonsillectomy and adenoidectomy. In cases not responsive to surgical correction of airway issues, continuous positive airway pressure (CPAP) is frequently used with an electronic device that delivers constant air pressure via a nasal mask. This leads to mechanical stenting of the airway and improved functional residual capacity in the lungs. The goal of CPAP is to find the optimal pressure that eliminates apnea but is still comfortable and does not lead to excessive air swallowing, gastric distention and air leak. Note: If the airway is compromised due to neurologic conditions, position the child to protect the patent natural airway.

Apnea of prematurity – Apnea of prematurity occurs in infants born before 34 weeks of gestation. It may be central, obstructive, or mixed and is usually caused by immature respiratory centers in the brain and a small pliable upper airway. Individual episodes of apnea generally respond to mild tactile stimulation. Treatments include positioning the child supine or side-lying with a small roll under the shoulders for optimal airway maintenance, treating with medications to stimulate the respiratory centers, use of CPAP or oxygen, and close cardio-respiratory monitoring.

Apnea of infancy – This condition occurs in infants following a full-term gestation and before age 1 year. It is usually self-limiting and does not cause desaturation, bradycardia, or other untoward effects. Following a complete medical work-up, apnea of infancy may be considered part of that infant’s normal breathing pattern.

Nursing considerations 1. Client may have an apnea/bradycardia monitor, an effective tool to alert parents and nursing staff to episodes of apnea or bradycardia. 2. Document the frequency, duration, and client’s response to apneic episodes. If you observe significant changes in your client’s pattern of apnea or your client requires increased intervention to resolve the apneic spell, notify the family, physician, and clinical manager. 3. Closely observe the infant at all times, despite monitor readings. Assess the client’s status before initiating interventions based on the monitor results. All monitors have the potential for false alarms,

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usually caused by poor skin contact with the leads or client movement. 4. Treatment for CPAP may cause side effects such as dry or stuffy nose, irritation of the skin on the face, stomach bloating, sore eyes, and headaches. Positioning during sleep, use of nasal sprays or humidified air, and adjusting the size and fit of the face mask are ways to alleviate the unpleasant side effects.

5. Provide education to primary caregivers regarding the proper use and Response to an maintenance of equipment in the home, how and when to use the apneic episode: apnea monitor, appropriate interventions for apneic episodes, • Gentle tactile medication administration, CPR, and how to activate EMS. stimulation– repositioning, 6. Document the family’s response to education and compliance with the rubbing back or plan of care. If poor compliance with the medical plan or continued chest lack of understanding despite reinforcement is observed, notify the • If normal breathing physician and clinical manager. does not resume, use more vigorous 7. Good sleep hygiene is important. A child with OSA may not have ever stimulation– experienced restful sleep. Habits become hard to break or modify. slapping the soles Healthy sleep hygiene involves setting a consistent bedtime so the of the feet. Never child averages 8 hours of sleep a night. After preschool years, naps shake an infant. are generally not needed, so bedtime may need to be reset. • If no response, Excessive daytime sleepiness is a sign that a child is not getting administer free- enough quality sleep. As a pediatric nurse, you can support improved flow oxygen and quality and quantity of sleep as well as lifestyle changes to benefit the then hand ventilate child. with a bag or mask if available Seizures Seizures are the result of abnormal electrical discharges in the brain. Symptoms may vary depending on what part of the brain is involved, but seizures often cause unusual sensations, uncontrollable muscle spasms, and loss of consciousness. Seizures may be caused by low blood sugar, infection, head injury, accidental poisoning, drug overdose, brain tumor, or cerebral anoxia. In some cases, the cause of the seizure is never discovered. More than 2 million people in the United States—about 1 in 100—have experienced an unprovoked seizure or been diagnosed with epilepsy.

Types of seizures Physicians have described more than 30 different types of seizures. Seizures are divided into two major categories – partial seizures and generalized seizures.

Partial seizures – These seizures occur in just one part of the brain. About 60 percent of people with epilepsy have partial seizure. 149

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In a simple partial seizure, the child will remain conscious but experience unusual feelings or sensations such as sudden and unexplainable feelings of joy, anger, sadness, or nausea. They may also hear, smell, taste, see, or feel things that are not real.

In a complex partial seizure, the child has a change in or loss of consciousness or consciousness may be altered, producing a dreamlike experience. They may display strange, repetitious behaviors such as blinks, twitches, mouth movements, or even walking in a circle. These repetitious movements are called automatisms. More complicated actions, which may seem purposeful, can also occur involuntarily. Clients may also continue activities they started before the seizure began, such as washing dishes in a repetitive, unproductive fashion. These seizures last just a few seconds.

Generalized seizures – These are a result of abnormal neuronal activity on both sides of the brain. These seizures may cause loss of consciousness, falls, or massive muscle spasms.

Absence seizure, also called petit mal seizures. The child may appear to be staring into space or have jerking or twitching muscles.

Tonic seizures cause stiffening of muscles of the body, generally those in the back, legs, and arms.

Clonic seizures are characterized by repeated jerking movements of muscles on both sides of the body.

Myoclonic seizures are characterized by jerks or twitches of the upper body, arms, or legs.

Atonic seizures are characterized by a loss of normal muscle tone. The affected client will fall down or may drop his or her head involuntarily.

Tonic-clonic seizures or grand mal seizures cause a mixture of symptoms including stiffening of the body and repeated jerks of the arms and legs as well as a loss of consciousness.

Nonepileptic events – Also called pseudoseizures, these events are called “nonepileptic” when people appear to have seizures even though their brains show no seizure activity. These events may be caused by narcolepsy, Tourette syndrome, or cardiac arrhythmia. Distinguishing between true epileptic seizures and nonepileptic events can be very difficult. It requires a thorough medical assessment to determine the origin.

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Causes of seizures Anything that disturbs the normal pattern of neuron activity—from illness to brain damage to abnormal brain development—can lead to seizures. Causes of seizures may include: • Metabolic disturbances – Disease of the kidney or liver can cause chemical disturbances in the body resulting in altered levels of sodium, calcium, or magnesium • Previous brain trauma – Traumatic head injuries such as those sustained in automobile accidents or serious sports injuries • Toxic substances – Many drugs and poisons can cause seizure in sufficient quantities or in susceptible individuals (Narcotic or alcohol withdrawal can result in seizures) • Infection – Inflammation of the brain such as in meningitis or encephalitis • Birth defects – Congenital malformations in infants and young children • Brain tumor – Although uncommon, brain tumor may cause seizure

Phases of seizures Aura phase – Only occurring with partial seizures, this phase is experienced by some clients as a warning or feeling that a seizure is coming. Common auras include changes in bodily sensations, changes in ability to interact with things happening outside the body, and changes in how familiar the outside world seems to the affected individual. Other warning signs that may occur hours before a seizure are depression, irritability, sleep disruption, nausea, and vomiting.

Ictal phase – The initiation of seizure activity. During this time the client is exhibiting symptoms based on the area of the brain involved in the seizure.

Post-ictal phase – The end to a seizure. This time period is a transition from the seizure back to the individual’s normal state and signifies the recovery period for the brain. It may last from seconds to minutes to hours, depending on several factors including which part(s) of the brain were affected by the seizure and whether the client was on antiseizure medication.

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Nursing care

Before a seizure 1. Familiarize yourself with the client’s specific pattern of seizure and related treatment plan. 2. Locate all emergency medications and equipment. 3. Ensure that all medications are within their expiration date. 4. Evaluate client’s and primary caregiver’s level of understanding regarding seizures, the individual specific seizure plan, and when to call EMS or the physician. 5. Provide education as needed. 6. Evaluate adequacy of safety precautions taken in the home. 7. Take only axillary temperatures. 8. Keep side rails padded and up while client is in bed. 9. Secure safety harness when in a wheelchair or other seating devices.

During a seizure 1. If necessary, remove client to a safe location. 2. Provide PRN medications as ordered. 3. Protect client’s head from injury. 4. Turn client on side to allow drainage of oral secretions and prevent aspiration. 5. Remove objects that may obstruct breathing or cause injury to the client. 6. Administer oxygen as needed, if ordered. 7. Do not forcibly restrain the client during the seizure.

Bus safety 1. Notify the bus driver that the client is having a seizure and request that he or she pull over and stop. 2. Remove the client from the wheelchair or car seat onto the floor of the bus. 3. Initiate treatment as ordered by the physician, maintaining the client’s privacy as much as possible 4. If necessary, activate EMS via the bus driver. 5. Monitor the client closely until the seizure resolves and the client is able to safely be returned to the wheelchair or car seat. 152

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After a seizure 1. Maintain client in a side-lying position until returned to baseline. 2. Limit stimulation. 3. Loosen restrictive clothing. 4. Obtain vital signs immediately following a seizure and then as ordered until they return to baseline. 5. Assess lips, tongue, and inside of mouth for injuries.

Documentation All the following information should be included in the Nurse’s Note related to any seizure activity: • All precautions taken • An aura observed or reported prior to the seizure • All interventions required during the seizure such as oxygen administration or • suctioning • All activity observed during the seizure including time, duration, location, • circumstances, color, respiratory status, and vital signs • Level of consciousness before, during, and after the seizure • Response to any medications administered • Any injury sustained during the seizure • Client’s post-ictal status

Vagus nerve stimulators Vagus nerve stimulation (VNS) is a treatment for intractable seizures. A small generator is surgically implanted under the skin below the left collar bone. This is connected to a lead with three coils at one end. These coils are wrapped around the vagus nerve in the left side of the neck.

The generator sends impulses from the vagus nerve in the neck to the brain and delivers therapy in two ways: 1. Initially, the physician will program the generator to deliver regular stimulation, using a computer and the “programming wand.” This

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regular stimulation is usually set to deliver cycles 30 seconds on and five minutes off continuously. 2. The generator can also be programmed to deliver additional stimulation to the vagus nerve on demand. Holding a special magnet near the implanted device causes the device to become active outside of the programmed interval. For clients with aura before their seizures, activating the stimulator with the magnet when the aura occurs may help prevent the seizure. For other clients, the generator is stimulated at the onset of a seizure or as directed by the physician.

Certain side effects are associated with VNS. The most commonly reported are temporary hoarseness or change in voice tone; sore or tickling throat; shortness of breath, and coughing. These problems hoarseness tolerate it well and notice it less over time. Other discomforts such as breathing difficulties can occur if the generator setting is too high initially or is increased too rapidly. If this happens, the physician can reprogram the stimulation settings.

Nursing considerations Clients with implanted VNS should avoid exposure to strong magnets, which may affect the stimulator settings. Areas with warning signs posted for people with pacemakers should also be a warning for clients with VNS.

The VNS is powered by a battery that lasts several years. It is replaced during an outpatient procedure under local anesthesia. If the client exhibits sudden decreased efficacy of vagus nerve stimulation, find out the last time the battery was changed.

With growth of the client, the stimulating electrode of the VNS may need to be modified. Replacement of the electrode is almost impossible due to scar tissue around the vagus nerve.

Ketogenic diet The ketogenic diet was designed in the 1920s to mimic the body’s response to starvation. This diet induces a ketotic state, shifting the body’s metabolism from carbohydrate to fat utilization for fuel. It appears that children under age 10 respond best to the diet from a physiologic perspective because they tend to be more prone to ketosis than older children or adults. The brain’s ability to extract ketone bodies and utilize them as an energy source decreases with age.

Successful control of seizures with the ketogenic diet tends to equal or surpass that of antiepileptic drugs. The ketogenic diet lacks the sedative

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and other adverse effects of antiepileptic drugs, carries none of the risks of surgery, and has shown dramatic positive results. Children who have become seizure-free on the diet may remain seizure-free even when the diet is discontinued.

Side effects • Possible long-term effects of high fats (cholesterol, triglycerides) • Growth retardation due to protein deficiency • Vitamin and mineral deficiencies • Constipation • Kidney stones • Elevated uric acid production • Impaired immune defenses • Metabolic acidosis • Liver failure

Nursing considerations 1. Strict adherence to the diet is required to produce effective results. 2. Monitoring of urinary ketones will be ordered by the physician along with response to nonketotic readings. 3. Liquid and chewable forms of medications may be prohibited due to glucose content. 4. Check with the pharmacist before administering new medications to ascertain that they will not interfere with induced ketosis.

Gastroesophageal reflux disease (GERD) Gastroesophageal reflux disease (GERD) is a digestive disorder that results when gastric acid flows from the stomach back into the esophagus. Gastroesophageal reflux is very common in infants, though it can occur at any age. It is the most common cause of vomiting during infancy.

Disease process Normal gastroesophageal reflux (GER) is not a disease or even a disorder. It is a retrograde movement of gastric contents into the esophagus and a physiologic process that occurs in everyone, particularly after meals. The most useful classification of GER divides the spectrum of reflux into 3 categories.

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1. Functional GER is a benign condition that does not require evaluation or treatment. It does not cause inflammation, lead to long- term complications, or affect growth and development. This category ranges from passage of refluxate into the distal esophagus to frequent regurgitation of gastric contents out of the mouth (typically seen in infants).

2. GERD, in contrast, necessitates intervention. Complications result from the reflux of gastric contents into the esophagus, such which leads to problems including failure to thrive, anemia, esophagitis, Barrett’s esophagus, apnea, pneumonia, wheezing, or chronic sinusitis.

3. Secondary GER is the term for the situation of some underlying condition causing the retrograde movement of gastric contents. The appropriate treatment is to address the underlying cause, as in pyloric stenosis. Other examples of disorders that may lead to secondary GER or GERD are metabolic defects, food allergy, infection, nasogastric tubes and neurological impairment.

Some studies have estimated that as many as 40 percent of healthy, thriving babies who are younger than age 6 months have abnormal amounts of acid reflux. The major symptom at this age is recurrent spitting and vomiting. By age 12 to 16 months, the number of infants with symptoms of GERD dramatically decreases. A pediatric physician office survey, however, indicates that up to 15 percent of healthy children and adolescents may still have symptoms of reflux, including heartburn and regurgitation. Infants and children with serious developmental and physical handicaps who spend long periods in the supine position are at high risk for GERD because they are unable to clear the esophagus efficiently. Children with brain injury secondary to infection, inherited metabolic disease, and tumors are also at a higher risk for GERD.

The Anatomy of GERD

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Causes GERD is the result of conditions that affect the lower esophageal sphincter; a muscle located at the bottom of the esophagus that opens to let food in and closes to keep food in the stomach. When this muscle relaxes too often or for too long, acid refluxes back into the esophagus, causing vomiting or heartburn.

Some foods seem to affect the muscle tone of the lower esophageal sphincter, allowing it to stay open longer than normal. These include but are not limited to chocolate, peppermint, and high fat.

Signs and symptoms • Irritability • Insomnia • Anorexia • Weight loss • Failure to thrive • Arching the back while feeding • Apnea • Increased salivation • Recurrent abdominal pain • Chest pain • Wheeze • Hoarse voice • Chronic cough at night • Frequent sore throat in the morning • Chronic cough, recurrent pneumonia, or upper respiratory infections • Sandifer syndrome – posturing episodes that are often mistaken for seizures • Rumination • Frequent, painful, or difficulty swallowing

Treatment Lifestyle change – For an infant, hold the baby upright for about a half hour after breast or bottle feeding to help keep reflux to a minimum. Feeding a baby smaller portions and feeding more often can help manage the spitting up. Some physicians recommend thickening the baby’s formula with rice cereal or using prethickened formulas. This may

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also decrease the frequency of emesis. Placing a baby in a semi-Fowler’s position as in an infant car seat makes GERD worse by increasing intra- abdominal pressure. Babies with GERD should sleep on their backs in a bed with the head of the bed elevated to a 30-degree angle.

For older children, the lifestyle change may be as simple as keeping them from lying down for 30 minutes after a meal. This allows the stomach to empty and the acidity of the gastric contents to decrease before assuming a position that will facilitate the back-up of gastric contents into the esophagus. Elevating the head of the bed 6 inches will also decrease the retrograde flow of gastric contents. Small frequent feedings will assist in controlling the amount of acid in the stomach. Clients that eat orally should avoid fatty foods, caffeine, mints and mint flavoring, spicy foods, citrus fruits, carbonated beverages, and tomatoes.

Medications – A physician may first recommend nonprescription medications, such as antacids and histamine-2 (H2) receptor blockers. Antacids help neutralize acid already in the stomach or esophagus. Some of these antacids have a foaming agent, which also helps prevent acid from backing up into the esophagus. Antacids can be used every day for 3 weeks. If taken longer, they can produce diarrhea, interfere with calcium absorption in the body, and build up magnesium, which can damage the kidneys. Infants are given antacids only in limited doses because of the risk of aluminum toxicity. H2 blockers should be taken 1 hour before meals because they block acid formation but have no effect on the acid already present in the stomach.

If the nonprescription medications are ineffective or the GERD is very serious, the physician will usually prescribe a proton-pump inhibitor to block the production of the enzyme that aids in the production of acid, a treatment that is more effective than antacids or histamine-2 receptor blockers in reducing gastric acidity. The physician may prescribe coating agents to protect the irritated mucous membranes that line the esophagus and stomach.

Fundoplication – This surgery is used to control GERD when lifestyle changes or medications are not effective or the reflux is severe. A fundoplication creates a valve mechanism by wrapping the upper part of the stomach (fundus) around the lower end of the esophagus. There are two types of surgeries. Thal fundoplication is a partial wrap and Nissen fundoplication is a total wrap.

With the Nissen operation, the fundus is pulled completely around the lower part of the esophagus and sewn together, helping to increase the pressure in the lower esophageal sphincter and preventing reflux. 158

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A gastrostomy tube (GT) is often placed into the stomach when the fundoplication is performed for feeding and relief of gastric distention. A child with reflux is often underweight and may require extra nutrition given through the GT. The gastric distention is due to the wrapping of the fundus, which inhibits the child’s ability to reflux and to burp. With the GT in place, you can relieve the client’s need to burp by opening the tube and allowing the gas to vent. Some infants require continuous venting during GT feedings through the use of a Ferrell bag. The frequency and method of gastric venting will be ordered by the physician prior to discharge.

Nursing considerations 1. Weekly or monthly weights as ordered by a physician. Track the weights on a growth chart. 2. Hydration status monitored frequently if the client is experiencing emesis. 3. Reflux medication dosages will need to be increased as the child grows in order to remain effective. If symptoms worsen, consider the last time the child’s dose was increased with weight gain in mind. 4. Ongoing assessments of caregiver’s understanding of GERD and the client’s treatment plan for successful management of the disease. Re- educate as needed throughout the caregiving process.

Cerebral palsy (CP) Cerebral palsy (CP) is a persistent disorder of movement and posture caused by nonprogressive defects or lesions of the immature brain.

Background information Cerebral palsy (CP), as defined above, refers to a condition of motor or postural abnormalities that are noted during early development. These abnormalities are thought to be associated with prenatal, perinatal, or postnatal events of varying etiologies. Many children with CP have normal or above-average intelligence but may not be able to express their intelligence due to oromotor, fine motor, or gross motor difficulties. Without appropriate intervention, these difficulties may impair the client’s development potential.

In the United States, the estimated prevalence of moderately severe or severe CP is 1.5 to 2.5 per 1,000 live births. The cause of damage remains unknown in many cases of CP. The following is a list of risk factors of CP: • Complications of prematurity • Very low birth weight (less than 1,000 grams or 2.2 pounds) 159

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• Fetal infections (CMV, herpes, toxoplasmosis) • Chemical or substance abuse during pregnancy • Infection (meningitis or encephalitis) • Intraventricular hemorrhage • Trauma • Complications of labor and delivery • Hypoxia – ischemia from meconium aspiration and persistent pulmonary hypertension • Birth defects • Perinatal CVA

Common presenting signs include but are not limited to the following: • Poor head control after age 3 months • Rigidity of the arms or legs • Arching of the back • Poor tone early on, usually replaced by spacticity • Use of only one side of the body to roll or crawl • Clenched fist after age 3 months • Excessive irritability or crying • Failure to smile by age 3 months • Inability to sit unsupported by age 8 months

Classifications and descriptions A child’s disease process will be described first in degree of severity as mild, moderate, or severe. Then it will be classified according to the type and degree of movement problems. • Spastic – Child has above-normal tone or stiffness of muscles • Athetoid – Damage to the part of the brain that influences and modifies electrical impulses sent from the brain to the muscles. Damage to this system can cause movements such as tremors, jerks, or writhing movements • Atonic – Child is weak, lacking normal muscle tone • Mixed/dystonic – A combination of any of the above • Ataxic – Involves the cerebellum, causing either an impaired sense of balance or a lack of coordinated movements 160

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Certain terms are used to denote the location of the movement problems. • Monoplegia – One arm or one leg is affected • Hemiplegia – The arm and leg on the same side are affected • Paraplegia – Only the legs are affected • Diplegia – Major movement problems with the legs and minor problems with the arms • Quadriplegia – Both arms, both legs, and spine are affected

All three descriptors will be used in discussing a child’s disease process. For instance, a child with severe full body spasticity would be classified with severe spastic quadriplegia.

Treatment There is no cure for CP. The goal is to enable affected children to attain their maximal developmental potential within the limits of their dysfunction. Treatment is primarily symptomatic and preventive.

Treatment aims to: 1. Establish mobility, communication, and self-help skills. 2. Achieve optimal appearance and integration of motor functions. 3. Correct associated defects. 4. Provide educational access adapted to the child’s individual limitations. 5. Encourage appropriate social interaction.

Detailed information regarding the team members involved in treating a child with CP as well as treatment devices (including those for positioning and splinting) will be discussed in the rehabilitation chapter of this book.

Baclofen pumps Spasticity is a motor disorder characterized by tight or stiff muscles that may interfere with voluntary muscle movements. Baclofen is a muscle relaxant medication that is often used to treat children with CP. It can be administered either orally or intrathecally. However, oral Baclofen causes systemic side effects and its short duration of effect requires frequent administration, which severely limits its usefulness in treating spasticity in children.

The intrathecal system for baclofen (ITB) delivery in small doses directly into the spinal fluid results in fewer, less severe side effects than those seen with oral dosing. The system consists of a catheter and a pump. The 161

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pump, a round metal disc about 1 inch thick and 3 inches in diameter, is surgically placed under the skin at the level of the abdomen. The pump stores and releases prescribed amounts of medication through the catheter. The pump has a 10 ml fluid capacity and can be refilled by

inserting a needle through the skin into a diaphragm in the center of the pump.

With a programmable pump, a tiny motor moves the medication from the pump reservoir through the catheter. Baclofen flows freely in the spinal fluid, decreasing the muscle hyperactivity. Adjustments in the dose, rate, and timing of the medication can be made by the physician using an external programmer. The pump system can be set to dispense medication continuously or at certain times of the day. The pump can also be easily reprogrammed, if needed.

The physician is responsible for the pump refills and adjustments to pump programming. Typically the pump requires a refill every 2 to 3 months. The pump is typically removed and replaced at the end of the battery’s life span, which is usually 3 to 5 years. Rapid reduction of baclofen administration may result in ITB withdrawal syndrome, a rare and life- threatening condition with symptoms of pruritus, rash, anxiety, disorientation, fever, and cardiovascular instability.

Since the problems caused by spasticity are numerous and multifaceted, a team approach for continuing management is required. Further treatment may involve stretching, strengthening, conditioning, and motor retraining. Serial casting or tendon release procedures can also be undertaken to achieve maximum elongation after spasticity has been successfully treated.

Nursing considerations with the baclofen pump 1. Call the physician if your client: • Is more sleepy than usual • Is weak 162

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• Is lightheaded • Is dizzy • Has headaches • Has nausea • Has seizures 2. If your client is very drowsy, consider the possibility of a baclofen overdose or a problem with the pump. This needs immediate attention. Contact the physician and prepare for transport to a medical facility. Take the ID card for the baclofen pump with you. 3. If your client has notable increases in spasticity, report to the physician because it may indicate a pump malfunction. One of the more common complications of a baclofen pump is catheter leakage caused by kinking or fracturing. This usually manifests as an apparent loss of treatment control such as increased spasticity in the patient or unpredictable variation in effectiveness of the drug. 4. Occasionally, the spinal catheter causes irritation of a lumbar nerve root, with resulting unilateral sciatica.

Botox therapy Botox therapy is the direct injection of the botulinum toxin into a specific muscle, with the aim of blocking the transfer of acetylcholine and alleviating muscle spasm caused by too much neural activity. There are seven different types of the neurotoxin but only the most potent, botulinum toxin type A, is in clinical use at this time.

Administered in small amounts, its therapeutic advantages are very promising in the treatment of uncontrollable muscle spasm due to CP. It can provide relief for as long as 6 weeks to several months. Over time, it appears that neurons resprout from the paralyzed nerve ending terminals, providing new avenues of communication with the muscle. After the neurons resprout, the muscle returns to its previously abnormal state of constant spasm.

Following the injections, clients usually receive some form of physical or occupational therapy to assist in stretching the muscle to lengthen the tissue and improve its function. Sometimes splinting devices or serial castings are used in conjunction with botox therapy to enhance and control the clinical results. The long-term effects (more than 5 years) of receiving regular injections of botox are not known at this time.

The main side effect of botox therapy is a short-lived weakness in the group of muscles that is being treated. In rare cases, clients have developed flu-like symptoms.

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Surgical intervention Surgery is recommended in clients suffering from extreme muscle contractions that do not respond well to other forms of treatment. These muscle contractions can be corrected by surgically lengthening the tendons and muscles involved.

Common comorbidity Epilepsy – Twenty to 40 percent of children with mental retardation and cerebral palsy also have seizures.

Osteopenia – Children with CP who are not able to walk risk developing poor bone density, which increases their risk for fractures.

Pain – Can be a problem for children due to spastic muscles and the stress and strain on parts of the body that are compensating for muscle abnormalities.

Other conditions – Clients with CP have higher-than-normal rates of other medical conditions such as bladder dysfunction and swallowing difficulties. Scoliosis is likely to progress after puberty, when bones have matured into their final shape and size. Children with CP also have a higher incidence of bone fractures that occur most frequently during physical therapy sessions. A combination of mouth breathing, poor oral hygiene, and abnormalities in tooth enamel increase the risk of cavities and periodontal disease.

Neuromuscular diseases Neuromuscular disorders are diagnosed in 1.5 million Americans annually. Weakness, paralysis, respiratory distress, and intractable pain caused by these diseases dramatically alter quality of life for both clients and families. Many groups of diseases such as muscular dystrophy, spinal muscular atrophy, inflammatory myopathies, diseases of the peripheral nerves, and metabolic diseases of the muscles fall into this category.

Muscular dystrophy (MD) Muscular dystrophy (MD) is not a single disease but rather a group of inherited diseases that cause the muscles to weaken and degenerate. This disorder affects structural proteins of the dystrophin glycoprotein complex (DGC) and related proteins, causing membrane instability leading to muscle fiber breakdown.

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Duchenne and Becker dystrophies cause a lack of dystrophin, while some of the limb girdle muscular dystrophies cause deficiencies in sacroglycans and other proteins. These proteins are necessary to maintain muscle function. When these proteins are absent, the membrane tears due to weakening of the sarcolemma, which in turn causes excess calcium leakage into the muscle fiber. This process initiates a cascade of events that leads to muscle-fiber necrosis. The body loses muscle tissue and replaces it with fat and connective tissue. This can make the muscles look larger than normal.

Despite the size of their muscles, children with MD may actually have significant muscle weakness, which makes walking difficult or even impossible. Currently, there is no cure for muscular dystrophy. The incidence of MD varies, depending on the specific type.

Duchenne muscular dystrophy is the most common condition. It is inherited on the X chromosome, primarily affects boys, and is the most severe type of the disease. Although women with the defective gene are carriers, they usually show no symptoms. There is an inheritance pattern of 1 case per 3,500 live births, and one-third of cases are caused by spontaneous new mutations.

Becker muscular dystrophy is the second most common form, with an incidence of 1 case per 30,000 live male births. It is also linked to the X chromosome.

Limb-girdle muscular dystrophy includes several different illnesses that can be inherited by both males and females.

Symptoms of muscular dystrophy • Progressive muscle wasting • Poor balance • Frequent falls • Walking difficulty • Waddling gait • Calf pain • Limited range of motion • Muscle contractures • Ptosis • Gonadal atrophy • Scoliosis

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• Inability to walk

Prognosis Varies according to the type of MD and its progression. Some clients have only mild symptoms with a normal lifespan, whereas others have severe symptoms and may die young. For example, children with Duchenne often die before age 18 because of respiratory failure, pneumonia, or other problems. In clients with Becker MD, death tends to occur later. Some complications associated with MD that lead to permanent, progressive disability are scoliosis, joint contractures, decreased mobility with inability to perform ADLs, mental impairment, cardiomyopathy, and respiratory failure.

Treatment There is currently no cure for any form of muscular dystrophy. Research into gene therapy may eventually develop treatment to stop the progression of some types of muscular dystrophy. Current treatment is designed to help prevent or reduce deformities in the joints and the spine and to allow people with MD to remain mobile as long as possible. Treatments may include various types of physical therapy, medications, assistive devices, and surgery.

Spinal muscular atrophy (SMA) SMA is characterized by progressive muscle weakness resulting from degeneration and loss of the anterior horn cells (ie, lower motor neurons) in the spinal cord and the brain stem nuclei. Onset ranges from before birth to adolescence or young adulthood. Poor weight gain, sleep difficulties, pneumonia, scoliosis, and joint contractures are common complications. Classification by age of onset and maximum function achieved is useful for prognosis and management.

Classification The SMA classification is based on age of onset and maximum function attained:

Prenatal • Arthrogryposis multiplex congenita (ie, congenital joint contractures involving at least two regions of the body) • Weakness at birth • Facial weakness minimal

SMA I • Onset 0 to 6 months 166

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• Poor muscle tone • Muscle weakness • Lack of motor development; never achieves ability to sit without support • Facial weakness; minimal or absent • Fasciculation of the tongue seen in most but not all affected individuals • Postural tremor of the fingers seen occasionally • Mild contractures; often at the knees, rarely at the elbows • Absence of tendon reflexes • No sensory loss • Alert appearance • Normal cerebral function including intellect

SMA II • Onset of muscle weakness usually after age 6 months • Achieves ability to sit independently when placed in a sitting position • Finger trembling almost invariably present • Flaccidity • Absence of tendon reflexes in approximately 70 percent of individuals • Average intellectual skills during the formative years and above average by adolescence

SMA III • Onset usually after age 10 months but in some cases earlier • Achieves ability to walk at least 25 meters • Proximal limb weakness; the legs more severely affected than the arms • Weakness manifest as frequent falls or trouble walking up and down stairs at age 2 to 3 years

SMA IV “ Adult” onset of muscle weakness.

Incidence – The SMAs are the second most common autosomal- recessive inherited disorders after cystic fibrosis. Spinal muscular atrophy affects 1 in 6,000 to 1 in 10,000 people.

Prognosis – The mortality and/or morbidity rates of SMA are inversely correlated with the age at onset. High death rates are associated with 167

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early-onset disease. In clients with SMA type I, the median survival is 7 months, with a mortality rate of 95 percent by age 18 months. Respiratory infections account for most deaths. In SMA II, the age of death varies, but the death is most often due to respiratory complications.

Diseases of the peripheral nerves Guillain Barré syndrome (GBS) is a disorder that affects the peripheral nervous system and is the most common cause of acute generalized paralysis. Incidence is about 0.4 to 1.7 cases per 100,000 people each year. While the cause is unknown, the syndrome is thought to be mediated by the immune system. The main symptoms are muscle weakness and loss of reflexes, which usually begin about 10 days after a viral infection, such as an upper respiratory tract infection or gastroenteritis. The weakness is symmetric, beginning in the muscles of the legs and then slowly advancing upward toward the trunk and arms over a period of days to weeks. Other symptoms can include difficulty walking, breathing, swallowing, and presence of pain and paresthesia. Most children with GBS begin recovering 2 to 3 weeks after the symptoms begin. About 80 percent have complete recovery, but some have residual weakness and there is an approximate 3 percent mortality rate. Children have a 7 percent chance of relapse of symptoms.

Inflammatory myopathies A myopathy is a disorder of any muscle. Inflammatory myopathies are autoimmune diseases that can cause muscle weakness.

Dermatomyositis is one member of this group of inflammatory myopathies. Hallmarks of dermatomyositis are a widespread rash and muscle weakness. Some early signs of myositis include trouble rising from a chair, climbing stairs, or lifting the arms; fatigue with standing or walking; and difficulty swallowing or breathing.

Prognosis The disease is seldom fatal, although muscle weakness can persist for life.

Metabolic diseases of the muscles Acid maltase deficiency disease is a rare genetic disorder estimated at 1 in every 40,000 births. Caused by mutations in a gene that create an enzyme called alpha-glucosidase (GAA), the disease disables the heart and muscles.

Normally, the body uses GAA to break down glycogen, a form of sugar stored and used for energy. In this disease, excessive amounts of glycogen accumulate everywhere in the body, but the cells of the heart and skeletal muscles are the most seriously affected. The disease is 100 percent fatal. 168

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Individuals affected with infantile acid maltase deficiency generally die before reaching age 1 from heart or respiratory failure. Young people affected with childhood acid maltase deficiency generally die from respiratory failure between the ages of 3 and 24. Persons affected with adult acid maltase deficiency generally die of respiratory failure within 10 to 20 years of the onset of symptoms.

Carnitine deficiency is one of a group of muscle diseases that interfere with the processing of fats to draw energy from food. It is usually diagnosed in childhood, presenting with cardiac disease; muscle weakness in the hips, shoulders, and upper arms and legs; and weakness in the neck and jaw muscles. It is genetically based and generally slowly progressive.

Treatment of neuromuscular diseases Specific treatment for a client with neuromuscular disease will be determined by the physician based on the: • Child's age, overall health, and medical history • Extent of the condition • Type of neuromuscular disease • Child's tolerance for specific medications, procedures, or therapies • Expectations for the course of the disease process • Family’s opinion or preference

Many aspects of care and treatment are available for children with neuromuscular diseases and opinions are widely varied as to the “right” course of action. The decisions parents need to make are difficult and each family's situations and beliefs are different. As a skilled nurse in the home it is important to respect the family’s decisions and avoid judgmental behaviors that will only serve to increase the family’s already elevated stress level.

The goals of treatment are to manage respiratory compromise, ensure adequate nutrition, prevent orthopedic deformity, encourage optimal developmental progress and social interaction, and to prevent infection.

Respiratory care Early and effective pulmonary rehabilitation is essential if hospitalizations for lung infection or acute respiratory failure are to be avoided. Coughing and deep breathing should be encouraged to assist with airway clearance and prevent atelectasis. Cough suppressants and sedatives should be avoided because they can interfere with the client’s ability to clear mucus

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from the lungs. Excessive muscular fatigue should be avoided, but as much activity as tolerated without pain or fatigue is encouraged.

For many children with neuromuscular weakness, the beginning of respiratory failure comes on slowly and may be mistaken for other problems. Shortness of breath may not occur in these children, especially when weakness prevents them from exercising.

Hypoventilation at night is often the first symptom observed in these children because of the natural decrease in respiratory drive and compression of the diaphragm by the abdominal contents when a person is sleeping or lying down. Lack of strength in the voice and a feeble, nonproductive cough indicates that the respiratory muscles are weakening.

The result of weakening respiratory muscles is that the “vital capacity” of the lungs, which is the amount of air that a person can exhale after taking in a deep breath, decreases over time.

The primary focus of respiratory therapy applied to children with neuromuscular disorders is to reduce the speed at which the vital capacity decreases. Slowing the progression of the disorder is accomplished in stages throughout disease progression with different methods of ventilatory assistance. The goals of treatment are to manage respiratory compromise, ensure adequate nutrition, prevent respiratory infection, and maintain independence.

An incentive spirometer is used to help open alveoli. This device provides a goal volume for a deep breath and the child is encouraged to hold that volume for 10 to 15 seconds. From 15 to 20 deep breaths are suggested four to six times per day. Children are often started on this form of respiratory therapy when their vital capacity measurement falls below 75 percent of the normal, or predicted, value.

Nebulized medications may be ordered to help fight or prevent infections, to aid in thinning secretions, and to decrease inflammation of the airway. Typical inhaled medications include: • Mucolytics, which break down thick mucus • Decongestants, which decrease swollen tissues • Antibiotics, which combat infections • Bronchodilators, which relax smooth muscles in the airway and may assist with airway clearance

An intrapulmonary percussive ventilation (IPV) machine can be used to assist in mucus clearance during an acute respiratory tract infection. It is most frequently used when the vital capacity has declined, when the 170

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child uses a wheelchair full time, and when chest physiotherapy must be frequently performed with the child in one position. An IPV machine delivers aerosolized medication with small pulses of pressure gently loosening mucus from airway walls. This ventilation is delivered through a mouthpiece much like a nebulizer and requires 15 to 20 minutes to complete. It is frequently ordered 3 to 4 times a day during respiratory illness.

An in-exsufflator, also called a coughalator, is a device that can assist a weak cough effort and make it effective in expelling mucus. The device can push a volume of air into the lungs and quickly pull that same volume of air out of the lungs, imitating a strong cough effect. It is often utilized in clients when their own muscle strength is inadequate to clear the airways.

Noninvasive ventilatory supports in the form of continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BIPAP) are often valuable in the treatment of children with neuromuscular-related respiratory problems. If the vital capacity has dropped below 40 percent of normal, CPAP or BiPAP may be initiated during sleeping hours, when the child is most likely to hypoventilate. Hypoventilation during sleep is determined by a sleep study.

As the vital capacity declines to less than 30 percent of normal, noninvasive ventilatory support may also be required while awake. The client gradually increases the amount of time using the ventilator during the day, as needed. A mouthpiece can be used in the daytime and a nasal or face mask can be used during sleep. This is sometimes referred to as a “sips” ventilator, as the client inhales through a mouthpiece much like straw to augment his or her own respirations on demand. It is an easily portable system.

As the client’s dependence on ventilatory supports increases, some families opt for placement of a tracheostomy tube to facilitate improved clearance of secretions and delivery of ventilatory support. Sometimes the client’s inability to protect his or her own airway due to weakness of the laryngeal musculature or neurologic compromise results in the need for placement of a tracheostomy tube. Some clients and their families decline placement of tracheostomy tubes. Maintaining a patent natural airway can become a great nursing challenge.

Positioning of the client to prevent aspiration of oral contents, frequent suctioning, frequent respiratory assessments (with pulmonary hygiene as indicated), frequent position changes, and optimal hydration will all be required to promote optimal air exchange and prevent mucous plugs. Respiratory care for these clients may occupy large portions of time over the day.

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Nutritional care Obesity should be avoided because extra body weight restricts breathing and impedes mobility. Avoiding weight gain may be difficult because children with neuromuscular disease often have low caloric needs due to limited movement compared to their same-aged peers. Small frequent feedings are recommended to avoid gastric distention, which can result in upward pressure on the diaphragm with resulting respiratory compromise. It can also exacerbate GERD, a frequent comorbidity. Some physicians may use nutritional supplements as part of the child’s treatment plan.

The child’s ability to protect his or her airway during oral feedings needs to be frequently assessed because of the inability to chew foods effectively and ultimately the progressive weakness may require switching to a soft, chopped, or pureed diet. Thickened liquids may prevent aspiration while drinking as the child’s oromotor muscles weaken. Thorough documentation of changes in the client’s abilities is essential for proper treatment. Many children with neuromuscular disease eventually require enteral feeding through a gastrostomy or jejunostomy tube.

Orthopedic care Prevention of contracture with frequent prosthetic range of motion and splinting devices (as ordered by the physician) is paramount to maintain existing mobility. Physical and occupational therapy facilitate clients in maintaining optimal levels of independence for as long as possible. Assistive devices may be recommended by therapists based on the client’s status.

Maintaining the client’s safety while using assistive devices such as walkers, wheelchairs, and lifting devices is an essential aspect of care. Safety restraints should always be secured. Routine inspection and maintenance of equipment is essential to client safety.

Scoliosis is a common complication in neuromuscular disorders. It is caused by weakening of the muscles that normally support the spine and prevent full expansion of the chest, which leads to loss of breathing capacity. Tracking the degree of scoliosis is important for a growing child, whose spinal curvature may progress surprisingly quickly.

Significant scoliosis develops in 90 percent of children with Duchenne MD and more than 90 percent of children with severe, early-onset SMAs. Maintaining a straight spine not only permits children to sit comfortably and helps them avoid being confined to bed but it also slows the progression of respiratory compromise.

Developmental care Encouraging as much independence as possible is essential. Scheduling play time into a day filled with respiratory treatments, feedings, and 172

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medications can be a challenge. Exposing these children to “normal” childhood experiences and recreation is an important aspect of care. Early intervention, occupational therapy, and physical therapy can be extremely helpful in modifying routine daily activities so the disabled child can participate. Encouraging the family to develop routines that include the client in daily household activities can help make a very unnatural situation seem more acceptable. Families often benefit from support groups or supportive counseling as they try to assimilate some very difficult realities into their lifestyle. Remember to be respectful of each family as they make their very difficult journey through the disability spectrum. They sometimes may not adapt as well or a quickly as we would like. This will require patience, kindness, and often a great sense of humor from the home health care nurse.

Prevention of infection Immunizations should be up to date. Appropriate flu vaccinations are encouraged annually. Good hand washing and infection control procedures are vital. Avoidance of individuals with known upper respiratory infections or gastroenteritis is also important. This can be a great challenge once the child begins to attend school. Classroom education regarding hand hygiene for the child’s classmates can also be beneficial if permitted by the child’s teacher. Hand hygiene prompts placed near the sinks and making hand sanitizer available in the classroom may also improve outcomes and decrease infections.

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Before continuing on to the next section, answer the following questions by circling the appropriate letter. Check your responses with the answers below.

1. At how many weeks of gestation is an infant born considered to be premature? (A) 40. (B) 30. (C) 35. (D) 37.

2. Common complications of prematurity include all of the following EXCEPT: (A) Patent ductus arteriosus. (B) Apnea of prematurity. (C) Bronchopulmonary dysplasia. (D) Hyperthermia.

3. You arrive at the home and find your client, a 4-month-old with bronchopulmonary dysplasia (BPD), asleep in his crib. Mom tells you the pulse oximeter was going off all night and she turned it off so she could sleep. Your FIRST action should be to: (A) Call your clinical manager and let her know the family is noncompliant with therapy. (B) Place the infant on oxygen. (C) Assess your client to ensure he or she is well-oxygenated and in no respiratory distress, place him on the pulse oximeter, and check the alarm limits to see that they comply with the physician orders. (D) Reeducate the parent on the importance of following the physician’s monitoring orders.

4. You are caring for Baby Joey today. He is 4 months old and has been diagnosed with BPD. He requires oxygen with feedings and PRN via nasal cannula. He receives nebulizer treatments of albuterol sulfate 0.125 ml in 2 cc NSS QID and PRN. On your visit to Dr. Smith’s today, he requests that you get blood work drawn on the baby on your way home from the office. After the lab draw is complete, you note that Joey is dusky, diaphoretic, tachycardic, and very agitated. What should you do for Joey FIRST? 174

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(A) Swaddle him, place him prone in his stroller, and gently pat him to help him fall asleep. (B) Activate EMS. (C) Position him for optimal air exchange, administer oxygen and/or nebulizer treatments as ordered, suction if needed, place on pulse oximeter if ordered, and monitor closely. Hold feedings until Joey is at baseline. (D) Hold feedings until Joey’s color improves. Consider sedation if ordered. Administer oxygen and advise Mom to keep him on oxygen overnight.

5. You assume care for Lauren, a 3-month-old preemie with mild BPD. Your report from Mom includes that she has been fine, except feeding a little less than usual. On assessment, you note that Lauren is pale and has a slightly elevated heart rate at rest. She has no fever. She has not gained weight in 3 weeks. You have difficulty getting Lauren to complete her minimum feeding volume by mouth today. What is your appropriate response? (A) Try to administer oxygen PRN during the next feeding to see if that helps. (B) Reassure Mom that “some days babies just aren’t hungry, and the baby will make it up later.” (C) Notify the physician and clinical manager of the changes in Lauren’s behavior and her lack of weight gain. (D) Suggest to Mom that she might want to take Lauren to the emergency room because you think she might be septic.

6. Bobby is an 8-year-old with asthma and cerebral palsy. You accompany Bobby to school during the day. After a walk outside, you notice that Bobby is coughing more than usual and seems anxious. What would you do for Bobby? (A) Ask Bobby how he is feeling and assess his lung sounds. If wheezing is noted, initiate his asthma action plan. (B) Take Bobby to the school nurse. (C) Call Mom and have Bobby picked up from school. He may be catching a cold. (D) Give Bobby his PRN bronchodilator and then check a peak flow reading and assess his lung sounds.

7. Triggers for asthma include all of the following EXCEPT: (A) Pollen and molds. (B) Tobacco smoke. (C) Strong emotions. 175

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(D) Changes in altitude.

8. A peak flow meter measures: (A) The amount of air a person can exhale after taking a deep breath. (B) The fastest rate at which air can move through the airways during a forced expiration. (C) The amount of air in each breath. (D) How deeply a person can inhale.

9. Nonpharmacologic methods of controlling asthma include all of the following EXCEPT: (A) Prevention of infection. (B) Minimizing active play activities. (C) Educating the client and family regarding the client’s asthma action plan. (D) Minimizing exposures to triggers.

10. Treatment of gastroesophageal reflux disease (GERD) includes all of the following EXCEPT: (A) Keeping an infant up in a car seat or infant seat for 30 minutes after feedings. (B) Small frequent feedings. (C) Elevating the head of the bed. (D) Administering antacids per physician orders.

11. Childhood obstructive sleep apnea can be caused by all of the following EXCEPT: (A) Enlarged tonsils and adenoids. (B) GERD. (C) Craniofacial deformity. (D) Neuromuscular disease.

12. Parents’ knowledge and competency in the use of an apnea monitor should be reviewed in all of the following areas EXCEPT: (A) CPR. (B) How to apply the leads or belt to the infant. (C) How to change the alarm settings on the monitor. (D) How to charge and maintain the monitor.

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13. Your client is newly discharged from the NICU. She is a preemie with a history of apnea and bradycardia. She has been stable with no episodes for 4 days. She has been discharged on an apnea monitor. During the night her alarm goes off. You check her and she is not breathing. What should you do? (A) Provide gentle stimulation by rubbing her chest or back and watch for the return of respirations. If they return easily, document the episode and continue to monitor closely. (B) Get the oxygen and set up for blow by. (C) Bag and mask her until she starts crying. (D) Begin CPR and activate EMS.

14. Generalized seizures: (A) Are abnormal neuronal activity in both hemispheres of the brain. (B) Are abnormal neuronal activity in one part of the brain. (C) Appear to be seizures but the brain shows no seizure activity. (D) Are the most common type of seizures.

15. The ictal phase of a seizure is: (A) The recovery phase. (B) The initiation of seizure activity. (C) The warning or feeling a seizure is coming on. (D) The time period after a medication has been administered to stop the seizure.

16. A child with moderate spastic hemiplegia can be described as having: (A) Moderate amount of tremors of the legs or the arms. (B) Moderate tightness of all four extremities and the spine. (C) Moderate tightness of one side of the body. (D) Generalized lack of coordination and an impaired sense of balance, mostly affecting one side of the body.

17. All of the following are true about muscular dystrophy EXCEPT that it: (A) Is a group of inherited diseases that causes muscles to weaken and degenerate.

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(B) Is curable with physical therapy. (C) Has varying prognoses based on the type of MD. (D) Primarily affects males. 18. Most deaths in end-stage neuromuscular disease result from: (A) Bowel perforation. (B) Malnutrition. (C) Respiratory infection or failure. (D) Sepsis.

19. Symptoms of chronic hypoventilation include all of the following EXCEPT: (A) Sleep disturbances. (B) Morning headaches. (C) Shortness of breath. (D) Poor appetite and weight loss.

Answers: 1. D, 2. D, 3. C, 4. C, 5. C, 6. A, 7. D, 8. B, 9. B, 10. A

11. B, 12. C, 13. A, 14. A, 15. B, 16. C, 17. B, 18. C, 19. C

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Resources Neonatology. Emory University School of Medicine Department of Pediatrics. http://www.pediatrics.emory.edu/neonatology/dpc/faq.htm#6. February 7, 2004.

Vision Channel Webpage, HealthCommunities.com. Retinopathy of prematurity. ,. http://www.visionchannel.net/retinopathy/. March 7, 2007.

Leroy, S. “Patent ductus arteriosus. University of Michigan website. http://www.med.umich.edu/cvc/mchc/parpat.htm, Oct. 1999.

“Vagus nerve stimulation in epilepsy action. Epilepsy.org website. http://www.epilepsy.org.uk/info/vagal.html. July 18, 2005.

,. Seizures and epilepsy: hope through research. National Institute of Neurological Disease and Stroke website. http://www.ninds.nih.gov/disorders/epilepsy/epilepsy.htm. March 19, 2007.

Status asthmaticus. Penn State University Milton S. Hershey Medical Center, Health and Disease Information website; 2004. http://www.hmc.psu.edu/childrens/healthinfo/s/statusasthmaticus.ht m. October 31, 2006.

Gastroesophageal reflux disease (GERD)/heartburn. Digestive and Liver Diseases; Stanford University Lucile Packard Children’s Hospital website; 2007. http://www.lpch.org/diseasehealthinfo/healthlibrary/digest/gerd.html.

Liburd J. Gastroesophageal reflux. WebMD. http://www.emedicine.com/ped/topic1177.htm. February 18, 2005.

Peeke K, Hershberger M. Obstructive sleep apnea syndrome in children. Pediatric Nurse. 2006;32(5):489–494. ©2006 Jannetti Publications, Inc.

Thorogood C, Alexander M. Cerebral palsy. WebMD. http://www.emedicine.com/pmr/topic24.htm. July 15, 2005.

Cerebral palsy. MedicineNet.com. http://www.medicinenet.com/cerebral_palsy/article.htm. April 22, 2002.

Boosara R. Cerebral palsy. Web MD. http://www.emedicine.com/NEURO/topic533.htm. August 9, 2005.

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Cerebral palsy. McKesson Health Solutions; Hackensack Medical Center Institute for Child Development. http://www.med.umich.edu/1libr/pa/pa_cp_hhg.htm. September 29, 2004.

Harding AE: Inherited neuronal atrophy and degeneration predominantly of lower motor neurons. In: Dyck PJ, Thomas PK, eds. Peripheral Neuropathy. 3rd ed. Philadelphia: WB Saunders; 1993:1051-1064.

Tsao B, Stojic A. Spinal muscular atrophy. Web MD. http://www.emedicine.com/neuro/topic631.htm. November 2, 2006.

Do T. Muscular dystrophy. Web MD. http://www.emedicine.com/orthoped/topic418.htm. June 21, 2006.

Breathe easy: respiratory care for children with muscular dystrophy. MDA Publications. http://www.mda.org/publications/breathe/be_c.html. September 2004.

Gastroesophageal reflux. Texas Pediatric Surgical Associates Webpage. http://www.pedisurg.com/PtEduc/Gastroesophageal_Reflux.htm.

Grand mal seizure. MayoClinic.com. website. http://www.mayoclinic.com/health/grand-mal- seizure/DS00222/DSECTION=3. December 29, 2006.

Health Information – CHEO Bronchopulmonary Dysplasia Children’s Hospital of Eastern Ontario Webpage. http://www.cheo.on.ca/english/9250.shtml. December 2001.

Fisher R, Long L, et al. Guide to the Care of the Patient with Seizures. Glenview, IL: AAnn Reference Series for Clinical Practice; 2004.

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Once you complete this section, you should be able to: 1. Discuss fluid and caloric intake for each age group 2. List causes of gastric dysfunction 3. Discuss alternative feeding methods 4. Demonstrate placement, care, and maintenance of feeding tubes 5. Discuss development issues related to fluid imbalance 6. List components of physical assessment of hydration status 7. List signs, symptoms, and degrees of dehydration

Contents

Introduction ...... 184 Caloric intake ...... 184 Fluid intake ...... 185 Gastrointestinal dysfunction ...... 185 Alternate feeding methods ...... 189 Formulas and feeding ...... 195 Assessing hydration status ...... 196

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Introduction A nutritional assessment is an important part of a complete physical assessment. The initial nutritional assessment will be completed by the clinical manager upon admission. Information regarding dietary intake begins with a dietary history, which helps assess adequate, deficient, or excess nutrition. You will need to obtain a head circumference for children younger than age 1, along with their weight and height/length. A child within the 5th to 95th percentiles for height, weight, and head circumference is considered having adequate nutritional status. A child who falls below the 5th percentile has a deficit of nutrition, whereas a child that is above the 95th percentile has an excess of nutrition.

Special health care needs can have a direct or indirect effect on a child’s nutritional status. Side effects from medications can affect appetite, intake, and tolerance of feedings. It may be necessary to time medication administration to assure effectiveness of medications and tolerance of feedings.

Caloric intake The caloric intake and needs of the population we care for vary. A child may have decreased physical activity but may also expend a large amount of energy to survive. The recommended daily dietary caloric intake is listed below.

Recommended dietary caloric intake for infants and children Age Kcal/kg/d Infant Birth–6 months 108 6 months–1 yr 98 Children 1–3 yrs 102 4–6 yrs 90 7–10 yrs 70 Males 11–14 yrs 55 15–18 yrs 45 18–21 yrs 40* Females 11–14 yrs 47 15–18 yrs 40 18–21 yrs 38*

*Based on recommended daily allowances and increased physical activity.

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Fluid intake Maintenance fluid is the amount of fluid the body requires to replace fluid loss through normal function of the skin, urinary, gastrointestinal, and respiratory tracts. A well child usually drinks more than the recommended daily amount of fluid. If less fluid were taken in, the child would gradually become dehydrated. Requirements vary with the age of the child. Infants have a higher requirement per kilogram than older children.

Calculating daily maintenance fluid requirements

Calculations: 100 ml/kg for the first 10 kg of body weight + 50 ml/kg for the next 10 kg of body weight + 20 ml/kg for each kg of body weight over 20 kg

Examples: Child weighs 10 kg: 10 kg x 100 ml/kg = 1,000 ml Child weighs 15 kg: (10 kg x 100 ml/kg) + (5 kg x 50 ml/kg) = 1,250 ml Child weighs 25 kg: (10 kg x 100 ml/kg) + (10 kg x 50 ml/kg) + (5 kg x 20 ml/kg) = 1,600 ml

Medical conditions also affect the requirements. If a child has a fever, diarrhea, diabetes, burns, or is sweating, fluid requirement increases. Meningitis, heart failure, and renal failure require decreased amounts of fluid. For every degree Celsius of body temperature above 99.5 F (rectally), daily maintenance fluids should be increased by 12 percent. A physician’s order is required to increase fluids.

Gastrointestinal dysfunction Failure to thrive (FTT) – FTT is a decrease in the rate of established growth or being consistently below the 5th percentile for height and weight on standard growth charts. The definition of FTT is a bit vague because it describes a condition, not a disease. Causes of FFT are: 1. Social factors – Physicians may not be able to identify a medical problem, but may discover that the parents are responsible for the lack in weight gain and growth. Some parents restrict caloric intake for their children because of the fear of their child getting “fat.” They may not be giving the child enough to eat for reasons such as “they are distracted by other household functions,” lack of interest, or the inability to provide a child with the necessary nutritional requirements. 2. Medical conditions, chronic illnesses, or medical disorders Disorders such as GERD cause the esophagus to become irritated. The child may refuse to eat because it causes pain. Chronic diarrhea can interfere with absorption of the nutrients that are taken in. Malabsorption disorders such as 185

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cystic fibrosis, chronic liver disease, and celiac disease limit the body’s ability to absorb nutrients. Some disorders such as prematurity and cleft lip or palate make it difficult for a child to consume the amount of calories needed. Other conditions such as endocrine, respiratory, and cardiac disorders require an increased caloric intake, making it difficult to keep up with the caloric need. 3. Intolerance to milk protein – This condition can initially lead to difficulty absorbing nutrients until it is diagnosed and treated. It can also restrict an entire class of food from the child’s diet. 4. Infections – Infections place energy demands on the body which cause it to use nutrients rapidly and appetite may also be impaired, which can lead to short- or long-term FTT. 5. Metabolic disorders – Can limit a child’s capacity to utilize most of the calories consumed. Metabolic problems may also make it difficult for the body to break down, process or derive energy from food or they can cause a buildup of toxins during the breakdown process.

Gastroesophageal reflux disease (GERD) – GERD is a malfunction of the lower esophageal sphincter (LES) muscle, which is responsible for closing and opening the lower end of the esophagus and is essential for maintaining a pressure barrier against contents from the stomach. If it weakens or loses tone, the LES cannot close up completely after food empties into the stomach. When this happens, acid from the stomach backs up into the esophagus. Dietary substances, drugs, and nervous system factors can also weaken the LES and impair its function. GERD can cause feeding and oral aversions associated with pain related to feedings. 1. Impaired stomach functions – Studies have shown that over half of GERD clients showed abnormal nerve or muscle function in the stomach. These abnormalities cause impaired motility because stomach muscles cannot contract normally, which causes delays in stomach emptying and increases the risk for acid back-up 2. Abnormalities in the esophagus – Some studies suggest that clients with atypical GERD symptoms such as hoarseness, chronic cough, and constant feeling of something in their throat have abnormalities of the esophagus 3. Drugs that increase the risk of GERD – These products include but are not limited to nonsteroidal anti-inflammatory drugs, inhaled bronchodilators, calcium channel blockers (treat high blood pressure), anticholinergics (treat urinary tract disorders and allergies), beta-adrenergic agonists (for asthma and obstructive lung diseases), sedatives, antibiotics, potassium, and iron

Vomiting – This is the forceful ejection of gastric contents. Projectile vomiting is vomiting accompanied by forceful peristaltic waves. A common myth is that vomiting is controlled by the stomach when in fact it is controlled by an area in the brain referred to as the vomiting center. The cause of vomiting depends on the source that the brain is getting the information from. Some sources that trigger the vomiting center in the brain include the following: 186

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• Stomach and intestines – Infection, injury or food irritation, or (in early infancy) a blocked intestine • Inner ear – Dizziness, motion sickness • Brain – Head injury, brain infections or tumors, migraines • Other causes – Overeating or overfeeding and cough

Nausea – An unpleasant feeling of sickness in the stomach, nausea is accompanied by an inclination to vomit. It can originate from problems in the brain or organs of the upper GI tract including the esophagus, stomach, small intestine, liver, pancreas, or gallbladder. Nausea can also be caused by pain, motion, medications, and diseases of nongastrointestinal organs. All stimuli that cause nausea work through the vomiting center in the brain which gives rise to the sensation of nausea and coordinates the physical act of vomiting.

Constipation – The passage of stool fewer than three times per week is considered constipation. The stool may be firm or hard and dry and difficult to eliminate. Constipation is a symptom, not a disease. Constipation occurs when the colon absorbs too much water or if the muscle contractions of the colon are slow or sluggish, causing the stool to move through the colon too slowly. Common causes of constipation are: • Not enough fiber in the diet • Lack of physical activity • Medications such as pain medications, antacids that contain aluminum and calcium, calcium channel blockers (for high blood pressure), antispasmodics, antidepressants, iron supplements, diuretics, and anticonvulsants • Milk • Irritable bowel syndrome • Abuse of laxatives • Ignoring the urge to have a bowel movement • Dehydration • Problems with the colon or rectum • Problems with intestinal function

Diarrhea – An increased number of stools with increased water content, diarrhea can be caused by: • Viral infection • Food poisoning • Medications (antibiotics, laxatives containing magnesium, chemotherapy)

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• Medical conditions such as malabsorption syndromes, irritable bowel syndrome, inflammatory bowel diseases, and celiac disease

Abdominal distention – Distention of the abdomen is a protuberant contour of the abdomen secondary to delayed gastric emptying, an accumulation of stool or gas, or presence of inflammation or obstruction. Common causes of abdominal distention include: • Overeating/overfeeding • Lactose intolerance • Swallowing air • Irritable bowel syndrome • Partial bowel obstruction • Constipation

Flatulence – Accumulation of excessive amount of air or gases in the stomach or intestines occurs when food does not break down completely in the stomach and small intestine. Some possible causes include: • Swallowed air • Eating or feeding too quickly • Bowel fermentation • Fundoplication surgery • Lactase deficiency • Lactose intolerance • Irritable bowel syndrome • Chronic digestive diseases • Celiac disease • Food intolerances

Gastric dysmotility – Delayed or rapid emptying of the stomach and/or intestines. 1. Rapid gastric emptying (dumping syndrome) is the rapid or early emptying of undigested food or fluid from the stomach into the lower small intestine. This usually occurs immediately after (but may occur during) a meal or feeding. The most common cause is gastric surgery of any type. Symptoms may include: • Diarrhea • Cramping • Palpitations • Nausea

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• Vomiting • Bloating • Dizziness 2. Delayed gastric emptying (gastroparesis) is late or disordered emptying of gastric contents caused by delayed digestion and movement of food from the stomach to the small intestine. This occurs when the vagus nerve becomes damaged and cannot control production of gastric acid and the movement of food through the digestive tract. This can be associated with low blood glucose levels. Causes may include stomach surgeries involving the vagus nerve, diabetes, hypoglycemia, drugs that decrease intestinal contractions, smooth muscle diseases, nervous system disorders, and metabolic disorders. Symptoms may range from mild to severe and include: • Slow digestion • Vomiting • Poor appetite • Stomach spasms • Bloating • Weakness • Weight loss • Sweating • Dizziness • Nausea • Sleepiness • Extreme fatigue

Alternate feeding methods If a child cannot consume food orally or take in a sufficient amount of nutrition, it may be necessary to utilize alternative methods for feeding. Delivery of nutrition Clinical alert: can be accomplished by the use of a feeding tube. The various types of feeding • Check with your tubes will be explained in detail. When an alternative method for feeding is used, state’s Scope of check the physician’s orders for: Practice to determine your role • Type and size of feeding tube in the use, placement, • Type, amount, and frequency of feeding replacement, and emergency • Method of delivery – pump or gravity procedures for • feeding tubes Time frame over which the feeding is to be given • Type and amount of fluid for flushing • Frequency of tube changes • Venting of the tube or use of a continuous venting system

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• Emergency procedures if the tube comes out • Method of de-clogging the tube • Stoma care

A feeding can be given with the use of a feeding bag that is appropriate for that particular pump or with extension tubing and a large syringe.

Types of feeding tubes Nasogastric (NGT) or orogastric (OGT) tube – This type of tube passes through the nasal passage (NGT) or mouth (OGT), through the throat, and into the stomach. Before the tube is inserted, it must be properly measured by placing the end of the tube at the ear, then to the tip of the client’s nose, and then to slightly below the xyphoid process. The measurement should be marked on the tube. The end of the tube is lubricated with water or a water-soluble lubricant and inserted into one of the external nares or through the mouth and advanced to the measured mark on the tube. If the client begins to gag during the insertion, you may use a pacifier to help. If the client is able to drink, sips of water may help. Care should be taken not to inadvertently insert the tube into the trachea and down into the lungs. To check placement of the NGT or OGT, inject air (0.5 to 1 ml for infants and up to 5 ml for older children) into the tube while listening with a stethoscope over the stomach. If air is heard, the tube is correctly placed.

Another method for checking placement is to aspirate a small amount of fluid from the tube with a syringe. The fluid is then placed on a small piece of pH paper to determine the acidity of the fluid. A pH of 5.5 or below is a good indication that the tube is in the correct position. Once positive placement is confirmed, stabilize the tube by securing it to the cheek. An effective method of securing the tube is to apply a sterile flexible dressing to the cheek then secure the tube in place with tape or a thin transparent dressing.

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Check with the manufacturer for the recommended frequency of replacing the tube. Most tubes used in home health care are replaced monthly or as needed if there is an obstruction or dislodgement. On some occasions, the tube will be reinserted before each feeding. To clean the area around the tube, use a cotton-tipped applicator and warm water. If the nostril becomes irritated, apply a water-soluble lubricant. If irritation continues, remove the tube and replace in the other nostril, if possible.

After administration of medications or a feeding, the tube should be flushed. This will help keep the tube from becoming obstructed. The usual fluid for flush is tap water. The amount of the flush is normally 1½ times the volume of the tube. Smaller tubes usually range from 1 to 2 ml where larger tubes may require 5 to 15 ml. To determine the volume of the tube, completely fill an unused tube with water, then measure the contents. If the tube holds 5 ml, then your flush would be 7.5 ml.

To unblock an obstructed tube, flush with warm water. If that does not resolve the issue, attach a syringe with plunger in place to the end of the tube. Using a gentle motion pull back on the plunger and then push. You may have to repeat this action several times. If you are unable to unblock the tube, removed it and insert a new one.

Contraindications and complications NGTs or OGTs may be contraindicated in clients with facial trauma, esophageal abnormalities, nasal abnormalities, or impaired airway. Complications of these tubes may include nosebleeds, sinusitis, sore throat, erosion of the nasal passage, esophageal perforation, and pulmonary aspiration

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Gastrostomy tube (GT, G-tube) – This feeding tube is placed into a surgical opening of the stomach. This tube may be necessary for children when the passage of the NGT or OGT is contraindicated or impossible; to avoid the constant irritation of the NGT or OGT; or for clients requiring long-term enteral feedings. The GT can be vented to relieve gas, per physician’s orders, by opening the plug and attaching a large syringe (preferably a 60 cc syringe). When connecting a skin-level feeding tube, attach the extension tubing to the feeding tube, then attach the large syringe. Venting can also be achieved with the use of a continuous venting bag.

Types of G-tubes include but are not limited to: Foley catheter, mushroom tip, wing tip, or skin-level device.

Gastric tube Mushroom tip (Bard) Low profile (skin-level) G-tube with balloon

Non-bolus extension tube Bolus extension tube

The advantages of the skin-level gastrostomy tube include that it is small and flexible; there is minimal extrusion from the abdomen so that it is cosmetically 192

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unobtrusive; it is easy to care for being totally immersible; and it contains a one- way valve to minimize reflux and eliminate clamping.

The plastic bolster of the gastrostomy tube should be positioned just above the surface of the skin by approximately 2 to 3 mm (a dime could slide under the bolster comfortably). A tight tube, which could lead to pressure necrosis, may be an indicator that the tube size needs to be changed because the child has grown. The gastrostomy button should be rotated at least daily to prevent the tube from adhering to the skin and to provide relief from tube pressure on the skin. Do not rotate transgastric tubes because that could cause the tube to become blocked or dislodged.

Percutaneous endoscopic gastrostomy (PEG) – This is a surgical procedure to place a feeding tube without having to perform an abdominal laparotomy. A gastrostomy is made percutaneously (through the skin) using an endoscope to determine where to place the feeding tube in the stomach and secure it in place. This procedure takes less time to perform, has a lower complication rate, and costs less than the surgical gastrostomy, making PEG preferred over laparotomy. Indications for a PEG include neurologic conditions associated with impaired swallowing; birth defects of the mouth, esophagus, or stomach such as esophageal atresia or tracheal esophageal fistula; and inability to consume sufficient quantities to meet the body’s needs. Complications can occur after the PEG placement, including client pain at the site, leakage of stomach contents around the tube site, aspiration, bleeding, or perforation. With the tube, complications may include dislodgement or malfunction.

Percutaneous endoscopic jejunostomy – PEG can be modified to a percutaneous endoscopic jejunostomy to provide both jejunal feeding and gastric decompression. Modifications of the standard PEG technique allow transpyloric placement of a jejunostomy tube. Feeding can be started after fluoroscopic confirmation that the tube is in the distal duodenum or jejunum. Clients with GERD are at increased risk for recurrent aspiration of gastric feedings and may benefit from simultaneous aspiration of gastric contents while

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continuous jejunal feeding is provided. Clients with gastric motor disorders may also benefit from this tube.

Jejunostomy tube (JT, J-tube) – Surgically inserted directly into the jejunum, this tube is indicated in children who cannot receive nutrition through the stomach due to congenital anomalies, surgery, inadequate gastric motility, severe gastric reflux, or who are at high risk for aspiration. Do not check for residuals of jejunal tubes. There is no opportunity for residual feeding to collect in the jejunum since emptying of the content is continuous and feeding is delivered at a much lower rate over a longer period of time than gastrostomy tube feedings.

Low profile J-tube J-tube

Combination gastrostomy and jejunal tube (GJT, GJ-tube) – A combination tube may be used to allow for gastric drainage or venting and medication administration through the G-tube port with the enteral feeding through the J-tube port. In most cases, if the J-tube becomes obstructed or dislodged, it is replaced by a physician. Bolus feedings should not be given via jejunal ports because “dumping syndrome” could result.

Gastro-jejunal feeding tube

Care and maintenance of feeding tubes and stomas Cleaning 1. Extension tubes should be washed with warm soapy water and rinsed well after each use. 194

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2. Stoma should be cleaned per physician’s orders. Best practice is to clean a new stoma using sterile water or normal saline solution with a cotton-tipped swab, washcloth, or gauze. An established stoma should be cleaned with soap and water and dried well. 3. It is not recommended to clean the stoma with hydrogen peroxide solution because the solution washes away the normal flora, leaving the area at risk for fungal infection. Chronic use of hydrogen peroxide damages healthy tissue. 4. Too frequent use or overuse of bactroban to the stoma site should be avoided due to a risk of resistance to the bactroban for the treatment of MRSA. The skin will lose its sensitivity to the effect of the ointment if overused. 5. Chronic use of antibiotic ointments to the stoma site should also be avoided so there will be sensitivity to the medication when it is needed for an infection.

Maintenance 1. Obstruction is the primary cause for failure of the tube. This is especially true with the longer gastric-jejunal tubes. The most important preventive measure is to flush the tubing. Follow the physician’s orders for flushing, which should include: • Type of fluid and amount • Before and after each feeding • Before and after giving medications • Every 4 to 6 hours if the client receives continuous feeds • After checking for stomach content residuals

It is recommended that medications be given in thin liquid form. Thick liquid medications can be thinned with water or formula. If a medication is only available in tablets or capsules, these must be crushed or dissolved completely and mixed with a small amount of water or formula

Formulas and feeding The formula used will be ordered by the physician. Various formulas are available for enteral feedings. Infant formula should be used for the first year of life. There are isotonic pediatric formulas to meet the nutritional needs of children between the ages of 1 and 10 years. They are complete and balanced with 100% of the daily recommended allowance of vitamins and minerals. When a physician selects a formula, he is taking into consideration a number of factors including the protein and caloric requirements; age and medical condition; history of food intolerance or allergy; intestinal function; route of delivery; and formula characteristics such as osmolality, viscosity, nutrient content, convenience, and cost.

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Complete formulas are considered nutritionally complete because they are made of complex proteins, fats, carbohydrates, vitamins, and minerals. These formulas are designed for clients who have normal digestion but cannot orally ingest sufficient calories and nutrients.

Elemental formulas are made of predigested nutrients including amino acids or Clinical alert: hydrolyzed protein, carbohydrate, and fat in the form of medium-chain Formulas that have triglycerides or essential fatty acids. They contain all the essential vitamins and been opened and minerals and require little or no digestion. The stool volume is low, with minimal refrigerated should stimulation of bile production and pancreatic secretions. If infused too quickly, be used within 24 they may cause diarrhea and cramping. These formulas are more expensive and hours. Due to the risk of bacterial growth, it would have no advantage to a child who can tolerate the isotonic formulas. Some is recommended that of the clinical indications for elemental formulas are short bowel syndrome, a formula not be used malabsorption disorders, irritable bowel syndrome, and gastrointestinal fistulas. if left unrefrigerated for more than 4 hours. Home prepared blenderized feedings are less expensive than commercially prepared formulas but are time-consuming to prepare. Often supplements are needed to complete the nutritional needs of the client. It is not recommended that these formulas be given via nasogastric tubes due to the tendency of clogging the tube.

Modular formulas are not complete; they contain only specific nutrients which can be added to commercially or home-prepared formulas to meet special nutrient needs. Some examples of these formulas are medium-chain triglycerides, which are fats in an easy-to-digest form used for additional calories; readily digested carbohydrates used for additional calories; and protein and specific amino acid preparations.

Assessing hydration status The hydration assessment is done initially by the clinical manager, and then it becomes a continual part of the process performed by the home health care nurse. Each time you perform an initial physical assessment, hydration status is assessed and then is ongoing throughout the duration of your shift. The nurse assesses for: • Any change in mental status • Presence of thirst or a dry mouth • Changes in heart rate • Quality of pulses • Changes in breathing • Sunken eyes (and fontanels in infants) • Production of tears • Moisture of the lips and mucous membranes of the mouth

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• Skin turgor recoil • Timing of capillary refill • Temperatures of extremities • Urinary output Dehydration is a common body-fluid imbalance that occurs when the total output exceeds the total intake. It can be caused by diarrhea, vomiting, fever, or sweating and also occurs when the intake of fluids is decreased or doesn’t meet the body’s requirements.

Signs and symptoms of dehydration

Degree of Dehydration

Symptom Minimal or none Mild to moderate Severe <3% loss of 3-9% loss of >9% loss of body weight body weight body weight

Mental status Well, alert Normal, fatigued, Apathetic, restless, or irritable lethargic, unconscious

Thirst Drinks normally, Thirsty, eager to Drinks poorly, might refuse liquid drink unable to drink

Heart rate Normal Normal to Tachycardic; increased bradycardic in severe cases

Quality of pulses Normal Normal to Weak, thread, or decreased impalpable

Breathing Normal Normal, fast Deep

Eyes or fontanels Normal Slightly sunken Deeply sunken

Tears Present Decreased Absent

Mouth and tongue Moist Dry Parched

Skin fold Instant recoil Recoil in <2 Recoil in >2 seconds seconds

Capillary refill Normal <2 Prolonged >2 Prolonged, minimal seconds seconds

Extremities Warm Cool Cold, mottled,

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cyanotic

Urine output Normal to Decreased Minimal to none decreased

Before continuing on to the next section, answer the following questions by circling the appropriate letter. Check your responses with the answers below.

1. The 5-month-old infant you are caring for weighs 5 kg. The recommended caloric intake for this age group is 108 kcal/kg/day. How many calories should this client receive daily? (A) 580 calories. (B) 1020 calories. (C) 540 calories. (D) 250 calories.

2. Using the calculation information in the bullets below, what are the fluid requirements for a client who weighs 66 pounds? • 100 ml/kg for the first 10 kg of body weight • + 50 ml/kg for the next 10 kg of body weight • + 20 ml for each kg of body weight over 20 kg (A) 2000 ml. (B) 1000 ml. (C) 2500 ml. (D) 1700 ml.

3. GERD (gastroesophageal reflux disease) is a malfunction: (A) Of the pylorus. (B) Of the lower esophageal sphincter. (C) In gastric emptying. (D) Of the stomach.

4. Failure to thrive may be caused by: (A) Parents’ inability to provide adequate nutrition. (B) GERD. (C) Milk protein intolerance. (D) All of the above.

5. How do you measure an infant or child for nasogastric tube placement? 198

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(A) Ear to nose to umbilicus. (B) Nose to ear to slightly below the xyphoid process. (C) Ear to slightly below the xyphoid process. (D) Nose to umbilicus.

6. Which statement is FALSE? Check placement of a nasogastric tube: (A) By instilling a small amount of air while auscultating over the stomach.

(B) By aspirating a small amount of fluid and testing the pH. (C) By instilling 5 to 10 ml of water while auscultating over the stomach. (D) Before administration of medications or feedings.

7. All statements regarding jejunal tubes are true EXCEPT: (A) Rotate the tube at least once daily to prevent adhering to the skin. (B) Do not check residual. (C) Bolus feedings should not be given. (D) If the jejunal tube becomes occluded or dislodged, it is replaced by a physician.

8. Which of the following is NOT an essential component of hydration status? (A) Changes in mental status. (B) Skin turgor recoil. (C) Timing of capillary refill. (D) Pulse oximetry.

9. Hydration assessment is: (A) Done at the time of assessment by the clinical manager. (B) Part of your assessment at the beginning of your shift. (C) Is ongoing. (D) All of the above.

10. Signs and symptoms of mild to moderate dehydration include all of the following EXCEPT: (A) Fatigue, restlessness, irritability. (B) Slightly sunken eyes (and fontanels in infants). (C) Bradycardia. 199

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(D) Decreased urine output.

11. Which of the following is FALSE? (A) Minimal or no dehydration = <3% loss of body weight. (B) Mild to moderate dehydration = 5% loss of body weight. (C) Mild to moderate dehydration = 3–9% loss of body weight.

(D) Severe dehydration = >9% loss of body weight.

Answers: 1. C, 2. D, 3. B, 4. D, 5. B, 6. C, 7. A, 8. D, 9. D, 10. C, 11. B

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References http://www.hc-sc.gc.ca/fnih-spni/pubs/nursing infirm/2001_ped_guide/chap_04_e.html http://www.kidney.org/professionals/kdoqi/guidelines_updates/nut_p05.html http://www.bmed.com/products/eftmain.htm http://travismedical.com/shoponline.php?&category_id=279 http://www.southwestmedical.com/Nutrition_Feeding/Feeding_BagsPump _Sets/400c0 http://www.bcchildrens.ca/Services/SurgeryAndSurgSuites/GeneralSurgery/ TubeFeeding/TypesOfTubes/default.htm http://www.clevelandclinic.org/health/health- info/docs/2000/2000.asp?index=4911&src=newsp http://www.hopeforhlhs.com/images/crosssection.jpg

Whaley & Wong’s Nursing Care of Infants and Children. 6th ed. St Louis: Mosby; 1999.

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