White Paper: Managing Chronic Conditions in the Neonatal

White Paper: Managing Chronic Conditions in the Neonatal Intensive Care Unit (NICU) For Health Plans, Medical Management Organizations and TPAs Trends in the NICU Care for premature babies and critically ill newborns in NICUs accounts for a significant percentage of all dollars spent for newborn care. A prolonged NICU stay for a chronic condition can easily exceed $1 million. While preterm births have been slowly declining in recent years, NICU length of stay has increased. Babies who would not have survived decades ago routinely survive today due to continually evolving medical and technological advances. Common Chronic Conditions Treated in the NICU Anemia of Prematurity All infants experience a decrease in hemoglobin concentration after birth. However, hemoglobin concentrations decrease more rapidly and more severely in premature infants than in term neonates. A number of factors may exaggerate the degree of anemia, including nutritional deficiencies of iron, vitamin E, vitamin B12, and folate, as well as blood loss and/or a reduced red cell lifespan. Many clinical findings have been attributed to anemia of prematurity, but they are neither specific nor diagnostic. Symptoms may include: poor weight gain despite adequate caloric intake; cardiorespiratory symptoms (e.g., tachycardia, tachypnea, flow murmurs); decreased activity, lethargy, and difficulty with oral feeding; pallor; increase in apneic and bradycardic episodes, and worsened periodic breathing; and metabolic acidemia (i.e., increased lactic acid secondary to increased cellular anaerobic metabolism in relatively hypoxic tissues). There are a number of laboratory studies that may be helpful in the diagnosis of anemia of prematurity. CBC count: White blood cell (WBC) and platelet values are normal in anemia of prematurity. Hemoglobin values are low (<10 g/dl) and may decrease to as low as 6 to 7 g/dl; the lowest levels are generally observed in the smallest infants. Red blood cell indices are normal (e.g., normochromic, normocytic) for age. Reticulocyte count: The reticulocyte count is low when the degree of anemia is considered, as a result of the low levels of erythropoietin (EPO). Conversely, an elevated reticulocyte count is not consistent with the diagnosis of anemia of prematurity. Peripheral blood smear: Red blood cell morphology should be normal. Red blood cell precursors may appear to be more prominent. Maternal and infant blood typing; direct antibody test (Coombs): The direct Coombs test result may be coinci dentally positive. Despite this, it is important to ensure an immune-mediated hemolytic process related to mater nal-fetal blood group incompatibility (hemolytic disease of the newborn) is not present. Serum bilirubin: An elevated serum bilirubin level should suggest other possible explanations for the anemia. These would include hemolytic entities, such as G-6-PD deficiency or other kinase/isomerase/enzyme deficiencies, or more common causes such as infection or hemolytic disease of the newborn. Lactic acid: Elevated lactic acid levels may be useful in determining the need for transfusion. Medical treatment options for anemia of prematurity include blood transfusion(s), recombinant EPO treatment, and observation. Drugs used to treat the condition are those that stimulate erythropoiesis and provide nutritional substrate needs. Ferrous sulfate/iron dextran, vitamin E, and folic acid, along with epoetin alfa, which stimulates red blood cell production, are among those administered. www.allmedmd.com AllMed Healthcare Management Inc. 1

Respiratory Distress Syndrome (RDS) Babies born before 34 weeks often develop this serious breathing problem, which is also known as hyaline membrane disease. In RDS, the infant s lungs are not fully formed or are not able to make enough surfactant, which is a liquid that coats the inside of the lungs and helps keep the small air sacs from collapsing. Most of the symptoms of RDS appear within minutes of birth. However, they may not be seen for several hours. Symptoms may include: bluish color of the skin and mucus membranes (cyanosis), brief stop in breathing (apnea), decreased urine output, grunting, nasal flaring, rapid breathing, shallow breathing, shortness of breath and grunting sounds while breathing, and unusual breathing movement. Tests that can help confirm a diagnosis of RDS include chest x-ray, blood tests to determine blood oxygen levels and to rule out infection, and echocardiography. Treatment for RDS usually begins as soon as an infant is born, sometimes in the delivery room. Most infants who show signs of RDS are quickly moved to the NICU. Babies who have RDS are given surfactant replacement therapy until their lungs are able to start making the substance on their own. Surfactant usually is given through a breathing tube, which allows the surfactant to go directly into the baby s lungs. Once the surfactant is given, the breathing tube is connected to a ventilator, or the baby may get breathing support from a ventilator or nasal continuous positive airway pressure (NC- PAP) machine. Infants who have breathing problems may get oxygen therapy through a ventilator or NCPAP machine, or through a tube in the nose. In addition, infants often receive antibiotics to control infections. Supportive therapy in the NICU helps limit stress on babies and meet their basic needs of warmth, nutrition, and protection. Treatment may include: using a radiant warmer or incubator to keep infants warm and reduce the risk of infection; ongoing monitoring of blood pressure, heart rate, breathing, and temperature through sensors taped to the babies bodies; using sensors on fingers or toes to check the amount of oxygen in the infants blood; giving fluids and nutrients through needles or tubes inserted into the infants veins (later, babies may be given breast milk or infant formula through feeding tubes that are passed through their noses or mouths and into their throats); and checking fluid intake to make sure that fluid does not build up in the babies lungs Bronchopulmonary Dysplasia (BPD) Bronchopulmonary dysplasia is characterized by inflammation and scarring in the lungs. Most infants who develop BPD are born more than 10 weeks before their due dates, weight less than 2 pounds at birth, and have breathing problems. Infections that occur before or shortly after birth also can contribute to BPD. Many babies who develop BPD are born with serious RDS and need oxygen therapy. Infants who are born early (usually >10 weeks before their due dates) and still need oxygen therapy by the time they reach their original due dates are diagnosed with BPD. Bronchopulmonary dysplasia can be mild, moderate, or severe. The diagnosis depends on how much extra oxygen a baby needs at the time of his/her original due date. It also depends on how long the baby needs oxygen therapy. Tests that can help confirm a diagnosis of RDS include chest x-ray, blood tests to determine blood oxygen levels and to rule out infection, and echocardiography. Since RDS can lead to BPD, some or all of the treatments used for RDS will continue in the NICU once BPD is diagnosed. In addition, infants with BPD may also receive bronchodilators (e.g., albuterol) to help keep airways open and/or diuretics (e.g., furosemide) to reduce buildup of fluid in the lungs. Apnea of Prematurity Premature babies sometimes do not breathe regularly. A baby may take a long breath, then a short one, then pause for 5 to 10 seconds before starting to breathe normally. This is called periodic breathing and is usually not harmful, and the baby will outgrow it. However, some premature and sick babies may stop breathing for 15 to 20 seconds or more. This apnea may also be accompanied by a slow heart rate (i.e., bradycardia). Apnea of prematurity can result from: RSD and other pulmonary conditions; infections (e.g., pulmonary pneumonia, bacteremia, meningitis, fetal or neonatal inflammatory response syndrome); hypoglycemia and other metabolic diseases; and central nervous system (CNS) pathology (e.g., trauma, intracranial hemorrhage, anoxia and/or ischemia, stroke). Diagnostic procedures for apnea of prematurity may include: physical examination; blood tests (e.g., blood counts, electrolyte levels, infection, blood oxygen levels); x-rays of the lungs, heart, or gastrointestinal system; and an apnea study, which monitors breathing effort, heart rate, and oxygenation. Useful laboratory studies include: www.allmedmd.com 2

CBC count and cultures of blood, urine, and spinal fluid if a serious bacterial or fungal infection is suspected Tests for ammonia, amino acid profiles in blood or urine, and organic acid levels in blood and urine if a meta bolic disorder is suspected Serum electrolyte, calcium, magnesium, and glucose levels can be useful for diagnosing a recent stressful condi tion, a metabolic process, or chronic hypoventilation Analysis of the stool for different toxins related to botulism may reveal a cause in an infant with apnea, constipa tion, clinically significant hypotonia, difficulty swallowing or crying, or absent eye movement Tactile stimulation by touching or patting the skin is usually enough to terminate an isolated apneic event. If the upper airway is obstructed, repositioning the baby s head and neck or gently elevating the infant s jaw may alleviate the occlusion. Use of a high-flow nasal cannula may open the airway enough to reduce obstructive apnea. Supplemental oxygenation or bag-mask ventilation is indicated in infants with signs of bradycardia or desaturation. Medical treatment is indicated when apneic episodes number 6 to 10 or more per day; when the infant does not respond to tactile stimulation; or when an event requires oxygenation and/or bag-mask ventilation to terminate apnea, bradycardia, and/or desaturation. Some infants may be placed on continuous positive airway pressure (CPAP). Medications used to treat apnea of prematurity include caffeine and aminophylline, which are methylxanthines. Methylxanthines reduce the frequency of anemia of Congenital Heart Defects A congenital heart defect is a defect in the structure of the heart and great vessels that is present at birth. Many types of heart defects exist, most of which either obstruct blood flow in the heart or vessels near it, or cause blood to flow through the heart in an abnormal pattern. Severe congenital heart defects generally are diagnosed during pregnancy or soon after birth. Risk factors for congenital heart defects include the following: Family history: hereditary diseases, previous sibling with defects Prenatal: viral infections, maternal medicine, maternal illness Postnatal: poor feeding, cyanosis, tachypnea, tachycardia, edema Signs of a heart defect include cyanosis, shortness of breath, rapid breathing, and signs of heart failure. Echocardiography, electrocardiogram, chest x-ray, and/or pulse oximetry may be used to diagnose congenital heart defects. Treatment options for congenital heart defects include cardiac catheterization, surgery to repair the structural defect, and medications (e.g., diuretics lower the amount of extra fluid in the body; digoxin increases strength of heartbeats; vasodilators widen blood vessels so blood can flow more easily; antiarrhythmics treat and prevent irregular heartbeats). The most common heart problem in premature babies is patent ductus arteriosis (PDA). In PDA, the ductus arteriosis, which connects the aorta and the pulmonary artery before birth and closes after birth, remains open. This causes abnormal blood flow between the aorta and the pulmonary artery. In some cases, nonsteroidal anti-inflammatory drugs, such as ibuprofen, or indomethacin may close the passageway. Surgery may be required if medications do not work. The aorta is the large artery that sends blood from the heart to the rest of the body. In coarctation of the aorta, the aorta may be too narrow for the blood to flow evenly. The narrow part can be cut away and the open ends sewn together, and the constricted section can be replaced with man-made material or patched with part of a blood vessel taken from elsewhere in the body. Sometimes, this narrowed area can be widened by inflating a balloon on the tip of a catheter inserted through an artery. Some babies are born with heart valves that are narrowed, closed, or blocked and prevent blood from flowing smoothly. Some infants may require placement of a shunt (artificial graft) to allow blood to bypass the blockage until the baby is big enough to have the valve repaired or replaced. A septal defect refers to a hole in the wall (septum) that divides the two upper or lower chambers of the heart. Because of this hole, the blood cannot circulate as it should, and the heart has to work extra hard. Surgery can be performed to close the hole by sewing or patching it. Small holes may heal by themselves and not need repair at all. In tetralogy of Fallot, a combination of four heart defects keeps some blood from getting to the lungs. As a result, the baby www.allmedmd.com 3

has episodes of cyanosis and may grow poorly. New surgical techniques allow early repair of this complex heart defect. When transposition of the great arteries occurs, the positions of the two major arteries leaving the heart are reversed. Each artery arises from the wrong pumping chamber. Surgical advances have enabled correction of this defect in the newborn period. Intraventricular Hemorrhage (IVH) Intraventricular hemorrhage refers to bleeding in the brain, generally occurring near the ventricles in the center of the brain. It is most common in the smallest premature babies (<3 1/3 lbs). Brain bleeds usually are given a grade from 1 to 4, with 4 being the most severe: Grade 1: Bleeding confined to one small area Grade 2: Blood is also within the ventricles Grade 3: More blood in the ventricles, usually with the ventricles increasing in size Grade 4: A collection of blood within the brain tissue Mild hemorrhages may not cause any symptoms. Symptoms of more severe IVH include increased episodes of apnea and bradycardia, decreased muscle tone, and decreased reflexes. Many hospitals use ultrasound of the head to routinely screen all premature babies for IVH within the first week of life and again before discharge. Currently, there is no way to stop bleeding associated with IVH. Most brain bleeds are mild (grade 1 or 2) and resolve themselves with no or few lasting issues. Blood transfusion may be given to improve blood pressure and blood count. A spinal tap may relieve pressure if swelling on the brain develops; if this is not possible, surgery may be needed to place a tube or shunt in the brain to drain fluid. Necrotizing Entercolitis (NEC) In premature infants with NEC, the lining of the intestinal wall dies and the tissue falls off. This condition may result from decreased blood flow to the bowel, preventing the bowel from producing mucus that protects the gastrointestinal tract. Bacteria in the intestine may also cause NEC. Babies with NEC develop feeding problems, abdominal swelling, and other complications such as vomiting, diarrhea, blood in stool, reduced or completely absent bowel sounds, and no bowel movements. Abdominal x-rays or ultrasound can be used to diagnose NEC. There are a number of medical treatments for NEC, including stopping all regular feedings and using an intravenous catheter to provide nutrients. In addition, a nasogastric tube extending from the nose into the stomach can suction air and fluids from the stomach and intestine. Antibiotic therapy may be used to treat possible infections. Stools should be checked for blood, and frequent blood tests should be performed to check for signs of infection and other problems. Oxygen therapy or mechanical ventilation should be used if abdominal swelling interferes with breathing, and platelet and red blood cell transfusion may be necessary in severe cases. Surgery is needed if the baby does not respond to medical treatment, or if there is a hole in the intestines. Part of the bowel may be removed; the goal is to remove only the part of the bowel that has no chance of recovery. www.allmedmd.com 4

Retinopathy of Prematurity (ROP) Retinopathy of prematurity is an abnormal growth of blood vessels in the eye. It can lead to bleeding and scarring that can damage the retina, and may result in vision loss. In addition to prematurity, other risk factors for ROP include apnea, heart disease, high levels of carbon dioxide in the blood, infection, low blood ph, low blood oxygen, respiratory distress, bradycardia, and transfusions. Stage 1: There is mildly abnormal blood vessel growth Stage 2: Blood vessel growth is moderately abnormal Stage 3: Blood vessel growth is severely abnormal Stage 4: Blood vessel growth is severely abnormal and there is a partially detached retina Stage 5: There is total retinal detachment Symptoms of severe ROP include abnormal eye movements, crossed eyes, severe nearsightedness, and leukocoria (white pupil). Retinal exams are typically routine for all babies less than 30 weeks gestation or weighing less than 3 pounds at birth. Treatments for ROP include laser therapy (photocoagulation), cryotherapy, and scleral buckling surgery and/or vitrectomy (stage 4 or 5 ROP). Sepsis Sepsis is a bacterial infection in the blood. Infants may come in contact with bacteria during pregnancy, birth, or from the environment after birth. Risk factors for sepsis include maternal group B Streptococcus (GBS) status, premature rupture of membranes, prematurity, and chorioamnionitis. The clinical signs of sepsis are nonspecific and can include body temperature changes, breathing problems, diarrhea, hypoglycemia, reduced movements, reduced sucking, seizures, slow heart rate, swollen abdomen, vomiting, and jaundice. Systemic physical assessment should include observation, auscultation, and palpation. Helpful diagnostic laboratory tests may include: CBC and differential, blood, and cerebrospinal fluid cultures; measurement of C-reactive protein and other infection markers. Lumbar puncture or imaging studies, such as chest x-ray, CT scan, MRI, and ultrasound of the head, may also be necessary. Antibiotic therapy is used to treat sepsis. Combined IV aminoglycoside and expanded-spectrum penicillin antibiotic therapy provides coverage for Gram-positive organisms (e.g., GBS) and Gram-negative bacteria (e.g., Escherichia coli). Health Plan Coverage for Treating Chronic Conditions in the NICU Insurance companies often base coverage decisions on criteria adapted from clinical guidelines developed by the American Academy of Pediatrics (AAP) and the American College of Obstetricians and Gynecologists (ACOG). The AAP Committee on the Fetus and Newborn (COFN) studies issues and current advances in fetal and neonatal care, makes recommendations regarding neonatal practice, collaborates with ACOG to consider perinatal issues on which the practices of obstetrics and pediatrics merge, and works cooperatively with ACOG on guidelines for perinatal care. The Role of Independent Medical Review in Determining Medical Necessity for Treatment in the NICU An independent medical review, which is normally used by healthcare payers, looks at whether or not a specific therapy or procedure was medically necessary. An independent review organization (IRO) provides ready access to specialists, thereby allowing for unbiased and timely determinations of whether the requested diagnostic test or treatment falls under medical necessity guidelines. The board-certified physician specialists who work with IROs keep up-to-date with the latest medical research literature and the latest standard of care, staying on top of continually evolving diagnostic advances and therapies as they are studied more extensively and potentially accepted into clinical guidelines. Independent medical review also avoids conflicts of interest, which can relate to economics, a lack of specialists to review cases, or having the same doctor who denied a case review an appeal. www.allmedmd.com 6

Conclusions The NICU is an intensive care unit specializing in the care of ill or premature newborn infants. Although not all babies in the NICU have the same illness or condition, some diagnoses are common to newborns who need intensive care. How long babies remain in the unit depends on the severity of their illness. Bibliography American Academy of Pediatrics, American College of Obstetricians and Gynecologists. Guidelines for Perinatal Care. 7th ed. 2012. Centers for Disease Control and Prevention. Preterm Birth. March 2013. Available at: http://www.cdc.gov/reproductivehealth/maternalinfanthealth/pretermbirth.htm. Accessed June 20, 2013. Martin JA, Osterman MJK, Sutton PD. Are preterm births on the decline in the United States? Recent data from the National Vital Statistics System. NCHS Data Brief. No. 39. May 2010. Schmidt B, Roberts RS, Davis P, et al. Caffeine therapy for apnea of prematurity. N Engl J Med. 2006;354:2112-2121. About AllMed AllMed Healthcare Management provides physician review outsourcing solutions to leading health plans, medical management organizations, TPAs and integrated health systems, nationwide. AllMed offers MedReview (SM), MedCert (SM), and Medical Director staffing services that cover initial pre-authorizations and both internal and external appeals, drawing on a panel of over 400 board-certified specialists in all areas of medicine. Services are deployed through PeerPoint, AllMed s state-of-the-art medical review portal. For more information on how AllMed can help your organization improve the quality and integrity of healthcare, contact us today at info@allmedmd.com or visit us at www.allmedmd.com 621 SW Alder St., Suite 740 Portland, OR 97205 800-400-9916 www.allmedmd.com www.allmedmd.com 6