HIE HELP CENTER: MEDICAL INFORMATION Contents HIE Overview ...... 4 What Is Hypoxic-Ischemic Encephalopathy (HIE)? ...... 4 Overview: Treatment for HIE ...... 4 Types and Forms of HIE ...... 4 Prevalence and Incidence of HIE ...... 5 Life Expectancy for People with HIE ...... 5 More Detailed Information on HIE ...... 5 Identifying HIE ...... 6 Signs and Symptoms ...... 6 Tests, Evaluations, and Developmental Screening ...... 6 Tests for HIE ...... 6 Long-Term Outcomes ...... 11 Causes and Risk Factors for HIE ...... 12 High-Risk Pregnancy ...... 12 Umbilical Cord Issues ...... 14 Placental or Uterine Complications ...... 14 Cervical Issues ...... 15 Oligohydramnios/Polyhydramnios ...... 15 Infections in the Mother or Baby ...... 15 Intrauterine Growth Restriction (IUGR) ...... 16 Labor and Delivery Errors ...... 16 Neonatal Health Mismanagement ...... 18 Associated Conditions ...... 20 Behavioral and Emotional Disorders ...... 21 Cerebral Palsy...... 23 Epilepsy, Seizures and Seizure Disorders ...... 25 Fetal Stroke ...... 26 Hearing and Visual Impairments ...... 27 Intellectual and Developmental Disabilities ...... 30 Intracranial Hemorrhages (Brain Bleeds) ...... 32 Learning Disabilities ...... 32 Neurological and Mental Health ...... 34

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HIE HELP CENTER: MEDICAL INFORMATION Nutritional Health Concerns (Chewing, Eating, Nutrition and the Gastrointestinal System) ...... 36 Oral Health ...... 38 Orthopedic Health...... 41 Pain...... 42 Respiratory Health ...... 44 Skin Health ...... 46 Speech Delays and Language Disorders ...... 48 Orthotics Requirements ...... 52 Preventing HIE ...... 54 Betamethasone ...... 54 Delivery Methods: C-Section Delivery ...... 54 Fetal Heart Monitoring During Labor and Delivery ...... 55 Magnesium Sulfate ...... 56 Prenatal Testing ...... 56 Prenatal, Birth and Postnatal Care ...... 58 Preventing Premature Birth ...... 60 Treating HIE ...... 63 Hypothermia Therapy: Reducing Injury Severity ...... 63 Other Therapies for HIE ...... 64 For Additional Informational Packets ...... 65

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HIE HELP CENTER: MEDICAL INFORMATION HIE Overview

What Is Hypoxic-Ischemic Encephalopathy (HIE)? Hypoxic-ischemic encephalopathy (HIE, also known as birth asphyxia, perinatal asphyxia, or neonatal encephalopathy) is a dangerous and permanent brain injury. This form of brain damage occurs when blood or oxygen stop properly flowing to either a part or all of the brain in or around the time of labor and delivery. When oxygen or blood is cut off from the brain, brain cells begin to die off in a chain reaction that makes brain damage worse. When blood flow is cut off to parts of the brain, cells begin to break down and release lactic acid and other compounds which can disrupt normal cell function. All cells in the brain are impacted by HIE, but neurons (the cells that transmit and process information in the nervous system) are particularly vulnerable to damage due to hypoxia and ischemia in a kind of brain damage called selective neuronal necrosis. Other terms for HIE (and phrases commonly associated with HIE) include the following:  Oxygen deprivation at birth  Birth asphyxia  Fetal oxygen deprivation  Baby not breathing at birth

Overview: Treatment for HIE HIE is managed using a treatment called hypothermia therapy, where the baby’s brain or body is cooled down below normal temperatures to slow the cascade effect that causes widespread damage. This allows the baby’s brain to recover and reduce the level of disability they may have as they grow. The treatment must be given within 6 hours of birth. The therapy lasts for around 72 hours, allowing the baby’s metabolic rate to slow. This prevents an injury known as reperfusion injury, which occurs when normal oxygenation and blood flow are restored too quickly to the brain’s cells. While it may seem counter-intuitive that restoring flow quickly could cause further injury, the brain’s cells react differently to rapid oxygenation after being oxygen deprived. After oxygen deprivation injury, rapid oxygenation can cause more inflammation and the release of certain compounds that can harm cells further. Hypothermia treatment works to stabilize the brain’s cells and prevent or limit damaging inflammation. In addition to hypothermia therapy, medical staff should provide supportive care, which can mean helping the baby breathe, controlling and preventing seizures and low blood sugar, minimizing brain swelling and getting care from specialists.

Types and Forms of HIE HIE is classified into different categories depending on how severe the oxygen deprivation is. If they suspect HIE, medical staff can conduct brain imaging, such as ultrasound and MRI, to see how badly brain tissue in the brain is damaged. Caregivers also do a blood gas test to determine the pH of the baby’s blood, which can also provide some information about the baby’s oxygen levels. The severity of HIE is determined using Sarnat staging, which takes into account clinical presentation, exam results, seizure presence and illness duration. The results of the Sarnat staging are used together with EEG findings to determine the infant’s prognosis. Mild HIE may have a normal outcome (at least in the short run) but severe HIE has a significant mortality rate, with 80% of survivors showing signs of neurological sequelae. Mild HIE is classified as Sarnat Grade I, and severity increases up to a maximal Stage III.

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HIE HELP CENTER: MEDICAL INFORMATION Prevalence and Incidence of HIE HIE is estimated to occur in between 2 to 9 per 1,000 live births. Between 10-60% of infants with HIE die in the neonatal period (when they are newborns) and about 25% of those that live with significant brain damage and impairments. Most HIE occurs at the time of labor and delivery.

Life Expectancy for People with HIE The life expectancy of children with HIE can vary depending on the condition’s severity. In some cases, the children have life spans of normal length, but in other cases, they can be shortened, to some extent.

More Detailed Information on HIE For more information on HIE, please visit our Medical Information section, which can provide further information into the causes of HIE, treatment and prevention methods such as hypothermia therapy, associated conditions, and long-term outcomes. Please also explore the information library for additional caregiving and life care planning information.

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HIE HELP CENTER: MEDICAL INFORMATION Identifying HIE Medical staff will often diagnose HIE using head imaging – they take pictures of the baby’s brain using ultrasound or MRI to see if there are any abnormalities. They can conduct numerous tests, including:  CT scans  PET scans  MRIs  Blood glucose tests  Arterial blood gas tests  EEGs  Ultrasounds Doctors order diagnostic imaging when they suspect that the baby suffered oxygen deprivation. These suspicions can occur if the labor and delivery were traumatic or if there were complications during birth. In some cases, HIE- related brain damage will not show up until a child shows poor motor control, delayed developmental milestones or other concerning signs. Signs and Symptoms Hypoxic-ischemic encephalopathy can manifest in numerous ways. In many cases, parents first notice that something is wrong when their child first started to miss developmental milestones. In other cases, parents begin to suspect HIE when they think back to the pregnancy, birth and delivery process and believe a doctor may have made a mistake, delayed a procedure, or delayed delivery. Sometimes mothers will feel a decrease in the baby’s movement. Very often, one of the first signs of HIE is seizures once the baby is born. Seizures in a baby can often be very subtle and require EEG to track, but it is becoming more commonplace to have continuous EEG monitoring for high-risk infants, which means that medical staff can monitor seizure activity even if there are no outward physical signs of seizures. Other signs and symptoms of HIE include:  Medical staff resuscitating the baby after birth  Low APGAR scores for more than 5 minutes  Seizures within 24-48 hours of birth  Trouble feeding (when a baby can’t latch on, suck or swallow)  When the baby’s blood has a very low pH as shown in umbilical cord blood gas tests  Low muscle tone (the baby is floppy or limp)  The baby has multiple organ problems  The baby doesn’t have normal brain stem reflexes (like problems breathing or abnormal responses to light) or only has reflexes relating to blood pressure and heart function  Abnormal consciousness or coma Tests, Evaluations, and Developmental Screening Tests for HIE Hypoxic-ischemic encephalopathy (HIE) can be identified at different points. In many cases, parents first become suspicious that their child may have a developmental delay when their child starts missing developmental milestones. These parents can think back to their labor, wondering if something may have occurred during the process that may have caused the delay.

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HIE HELP CENTER: MEDICAL INFORMATION In other cases, medical professionals conduct tests immediately to determine if the baby has HIE. These tests occur if the medical staff suspect that HIE is possible, especially in cases of a difficult or prolonged labor, umbilical cord issues, and other events that they may suspect harbor a risk of causing oxygen deprivation-related brain damage, such as the need to resuscitate the baby after birth. This rapid diagnosis is absolutely critical for the baby, because the only current treatment for hypoxic-ischemic encephalopathy, hypothermia therapy, must be provided to the baby within 6 hours – the sooner, the better. Hypothermia therapy allows the baby’s brain to heal (to some degree) from hypoxic-ischemic injury, and it minimizes the severity of the baby’s disabilities. This treatment is critical to maximizing the baby’s functional abilities later in life. The tests that doctors prefer can vary, but they can include:

APGAR Scores The APGAR test is a test administered to all babies when they are born. It evaluates the baby’s general health by looking at five key parameters:  Appearance: What color is the baby? Blue or pale all over? Blue at the extremities? Pink all over? The letter “A” in “APGAR” stands for appearance.  Pulse: Does the baby have no heart rate? Is its heart rate slow (under 100bpm)? Is the heart rate fast (over 100bpm)? The letter “P” in “APGAR” stands for pulse.  Reflex irritability (grimace): Does the baby not respond to stimulation? Does it cry feebly and grimace? Does stimulation cause the baby to cry and pull away? The letter “G” in “APGAR” stands for reflex irritability, or grimace.  Activity: Does the baby have no activity? Does it have some flexion (joint movement)? Does the baby have flexes arms and legs that resist extension? The second letter “A” in “APGAR” stands for activity.  Respiratory effort: Is the baby not breathing? Is the baby’s breathing weak and irregular? Is the cry very strong? The letter “R” in “APGAR” stands for respiratory effort. APGAR tests are performed at one minute after birth and five minutes after birth, and they are repeated if the score remains low afterward. Each of the five areas are evaluated on a 0-2 point scale, and the points are added up to provide a quick overall indicator of the baby’s general health. The scores are broken down as follows:  1-3 points: critically low  4-6 points: below normal  7+ points: normal APGAR score If the baby’s APGAR scores are low and remain low, this may indicate that the baby has a brain injury like hypoxic- ischemic encephalopathy (HIE). The lower the baby’s APGAR scores, the more likely it is that the baby will need doctors to provide medical intervention.

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HIE HELP CENTER: MEDICAL INFORMATION

Brain Imaging MRIs and CT Scans MRIs and CT scans are two technologies that medical professionals can use to take pictures of your baby’s brain. These scans allow trained professionals to identify if your baby has been oxygen deprived and the extent of the injury. This is very important because brain injuries evolve over time. Medical professionals can request that the baby have multiple CT scans or MRIs, to make sure they can properly track what is going on in the baby’s brain. MRI is the best imaging method of diagnosing babies with moderate to severe HIE. MRIs can be performed as early as 12-24 hours after birth. They accurately show injury patterns as early as 1 day after birth (sometimes sooner), and are especially useful after day 4. This method uses magnetic fields and a scanner to make a detailed image of the human brain. MRIs can identify brain lesions (regions where the brain has been damaged) and can also sometimes help doctors determine the time when a baby had a brain injury. MRIs do take longer than other imaging types, and there are some cases when babies can’t have an MRI (if they are unstable or are on machines that have metal in them). Some incubators and ventilators do not use metal parts, allowing some babies to have MRIs even if using a machine. CT scans use X-rays to generate multiple ‘slices’ of images, which are ‘stacked’ together by a computer to form a 3D image of the brain’s structures. While it is not the preferred method of imaging a baby’s brain because it does use X-rays, CT scans are an alternative imaging method. For more information about brain imaging and its role in detecting brain injury, please click through to the following page on brain imaging techniques [External Link]. Ultrasounds Ultrasounds are another way of detecting hypoxic-ischemic encephalopathy, but this method has a lower sensitivity compared to other types of imaging. Ultrasound does not always image the outer parts of the cerebral cortex very well, and cannot always pick up on less-severe white matter abnormalities. It is, however, available at the baby’s bedside, and can show signs of hemorrhages (heavy bleeding) and abnormal ventricle sizes, as well as cerebral edema (swelling due to excess cerebrospinal fluid), increased echogenicity, and brain tissue necrosis (after 24 hours).

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HIE HELP CENTER: MEDICAL INFORMATION Developmental Screening One of the most common ways that parents obtain a diagnosis of HIE for their child is when the child begins to miss key developmental milestones, At each month and year of a baby’s life, there are certain developmental items they are generally expected to be able to do. If (barring genetic anomalies) a child misses a particular developmental milestone, or if a pattern of developmental delays persists, it may mean that the baby had HIE. Sometimes, a baby or child may be diagnosed with cerebral palsy; the underlying cause of cerebral palsy is sometimes HIE. The CDC has a collection of milestone checklists for new parents:  Developmental milestones at 2 months  Developmental milestones at 4 months  Developmental milestones at 6 months  Developmental milestones at 9 months  Developmental milestones at 1 year  Developmental milestones at 18 months  Developmental milestones at 2 years  Developmental milestones at 3 years  Developmental milestones at 4 years  Developmental milestones at 5 years These checklists can also be found in a single convenient packet here. Sometimes developmental difficulties are caught early. This can happen if a baby had difficulty with feeding (latching, sucking or swallowing). It can also occur if certain developmental reflexes don’t go away when generally expected, or if they are exaggerated. Screening generally occurs in conjunction with multiple medical and therapeutic specialists. Parents of children with HIE commonly consult pediatric neurologists, pediatricians, orthopedic surgeons, ophthalmologists, psychologists, and numerous other specialists in order to secure a diagnosis. Early diagnosis is critical for improving a child’s outcomes – the earlier a child starts therapy, the likelier it is that outcomes will be better for the child. Umbilical Cord Blood Gas Tests Immediately after a baby is born (especially if the mother had a high-risk pregnancy), doctors collect an umbilical cord blood sample. Research shows that a blood sample that is taken properly can help determine whether the baby was in fetal distress (whether there was a period of time where the baby didn’t get enough oxygen). The umbilical cord has one vein and two arteries. Blood drawn from a vein versus an artery will tell a different story; blood in the umbilical vein (which flows to the baby) reflects on oxygen flow in the uterus and placenta, while blood in the umbilical artery (which flows from the baby) reflects the fetus’ state along with the uterus and placenta. The best way to test umbilical cord blood samples is from an artery. Medical staff clamp the cord and draw blood for testing. Once the sample is collected, there are numerous tests that can be performed. In order to determine whether the baby had a hypoxic-ischemic event, the blood’s pH is analyzed along with other factors such as PCO2, HCO3 levels, PO2 and base excess. These numbers describe the relative proportion of dissolved gases in the baby’s blood; if the arterial blood is acidotic (a condition called fetal acidosis), it means that the baby suffered an oxygen- depriving event. Normal values differ significantly depending on whether the baby was born at term or preterm. Umbilical cord blood gas tests can also help determine what kind of acidosis occurred. This can help pinpoint what the cause of the oxygen deprivation was. For example, if a baby has a high PCO2 (a high level of dissolved carbon

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HIE HELP CENTER: MEDICAL INFORMATION dioxide in their blood), it means that more CO2 is being produced than the baby’s body can eliminate. This can, for example, occur in cases where the umbilical cord in compressed. Sometimes, however, if PCO2 is very different from normal values, blood gas analyzers may not accurately calculate HCO3- levels. This can cause medical staff to calculate a different value (base excess or base deficit). Umbilical cord blood gas testing isn’t always the best way to determine whether a baby likely had oxygen deprivation, however. Research has shown that some babies with HIE often have test results come back with a blood pH that is normal or very close to normal. This research also found that there can be catastrophic oxygen- depriving events that don’t show an acidic pH in the umbilical cord blood sample. If a baby has poor blood flow, it may develop acidic products in its blood, but those products won’t reach the sample site the blood was drawn from. This can also occur if the umbilical cord is occluded (blocked off completely) – if the umbilical cord is being sampled from a point downstream from an occlusion, the sample may not show any acidemia at all. Almost all newborn babies with severe birth asphyxia have poor or no blood flow, which means that blood isn’t circulating well through the umbilical cord. This means that the acidic products that umbilical cord blood gas tests look for won’t be present in the sample. Functionally, this means that an umbilical cord blood gas test significantly underestimates how acidic the baby’s blood actually is. Once the baby is resuscitated and their blood begins circulating better, the acidic products begin to clear their system via central circulation. Researchers found that analyzing a postnatal base deficit from a newborn within two hours of delivery is a more accurate measure of how acidic the baby’s blood was than the umbilical cord blood sample collected immediately after birth. This postnatal base deficit is a far more accurate predictor of neurological outcome than just umbilical cord blood gas testing alone. Tests for Concurrent Diagnoses HIE does not usually come as a single diagnosis; there can often be multiple diagnoses that a child may have. Some of the most common are intellectual and developmental delays or disabilities, as well as cerebral palsy. Sometimes children with HIE can have speech delays, motor disorders, or seizure disorders that are diagnosed first. In other cases, the baby may be diagnosed with intracranial hemorrhages (brain bleeds) or fetal stroke. In other cases, when the HIE is mild and the baby is treated with hypothermia therapy in a timely fashion, the baby may have very few to no impairments. In many cases, medical professionals run evaluations of a child once a parent has brought up developmental concerns. Children with HIE are sometimes diagnosed with cerebral palsy. This is usually a clinical diagnosis made when a baby or child has muscle spasticity. Sometimes this is seen when a baby begins missing developmental milestones. There are two additional tests that may be helpful. About 45% of children with cerebral palsy have seizures. EEG testing can be used to determine if a child has seizures or epilepsy, which is critical to preventing further brain damage. Another test that screens for coagulation problems is performed in children with hemiplegic CP or in children that show evidence of cerebral infarction (stroke) caused by HIE. This test determines if the child has a blood clotting disorder called prothrombotic coagulation disorder. Children with hemiparesis (weakness on one side of the body) should be tested for HIE.

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HIE HELP CENTER: MEDICAL INFORMATION Long-Term Outcomes The long-term outcomes of babies who have HIE can vary widely – those with the mildest HIE or those who received hypothermia therapy quickly enough may not have any impairments at all, while those with severe HIE may require around-the-clock care. Babies with HIE can also have the following, depending on the severity of the diagnosis:  Seizures/convulsions after birth  Long-term neurodevelopmental impairments  Intellectual disabilities  Learning disabilities  Cerebral palsy The severity of the HIE can depend on several factors, including:  How severe the oxygen deprivation was  How long the baby was oxygen-deprived  The baby’s condition prior to the oxygen-depriving incident  How members of medical staff manage the baby after the oxygen-depriving incident Generally, the longer the baby is oxygen deprived, the more severe and permanent the HIE will be.

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HIE HELP CENTER: MEDICAL INFORMATION Causes and Risk Factors for HIE HIE and other related health complications like cerebral palsy, intellectual and developmental disabilities (I/DD) and seizures stem from the root cause of brain injury due to oxygen deprivation. There are many ways that a brain injury of this type can occur. In some cases, brain injury can occur due to what a layman might think is an unrelated condition. There are some cases of HIE where the cause is unknown or difficult to identify, though medical mistakes often can factor into HIE diagnoses. HIE is sometimes a complicated diagnosis because oxygen deprivation in a baby can occur due to many different factors. In some instances, HIE may not be preventable. However, in many cases, oxygen deprivation occurs due to a health issue that medical staff missed or mismanaged during pregnancy birth or delivery. These health issues can occur in either the baby or the mother, and they need to be dealt with appropriately and quickly to prevent adverse outcomes. While the following is by no means a comprehensive list, it illustrates the wide variety of conditions that can cause or be risk factors for HIE. Because many of these conditions can feed into each other or (directly or indirectly) cause oxygen deprivation, it is important to get a second opinion on your child’s health status if you are concerned. A quick note on the difference between a cause and a risk factor: a cause is a factor that directly causes a particular disease, while a risk factor is an event, substance or condition that makes it more likely that the condition will be present, but doesn’t necessarily mean that a child is guaranteed to have a particular diagnosis. Multiple risk factors can build up, however, and form a ‘causal pathway.’ These pathways occur when several risk factors align in a specific order at a specific time to make it significantly more likely that a condition will occur. Some of the causes and risk factors for hypoxic-ischemic encephalopathy include: High-Risk Pregnancy Women with obesity, diabetes, high blood pressure, preeclampsia, a pregnancy involving twins, triplets or more, autoimmune disorders, and women who use alcohol or tobacco during pregnancy are considered high-risk. Women with a high-risk pregnancy are monitored differently than low-risk patients to make sure that any issues in pregnancy can be quickly addressed. One of the common risk factors for hypoxic-ischemic encephalopathy is mothers who are high-risk not being treated like they are high-risk. Physician guidelines for high-risk pregnancies differ between non-high-risk and high-risk pregnancies – high-risk pregnancies are usually monitored differently, with a greater number of appointments and ultrasounds to ensure the pregnancy is going smoothly. Other common testing includes non- stress tests (NSTs), a kind of test that ensures that the baby’s heart rate is reacting normally to their movement, and a biophysical profile (BPP), which includes a non-stress test and a fetal ultrasound. What Makes a Pregnancy High-Risk? A high-risk pregnancy is any pregnancy that has risk factors for the mother or baby developing a serious health issue. This includes a very broad range of health concerns, including hypoxic-ischemic encephalopathy (HIE). There are many factors that can make a pregnancy high-risk, and these can include (but are not limited to):  Maternal Obesity and High BMI  Diabetes in the mother (especially if undetected or improperly treated)  High Blood pressure  Preeclampsia  Multiple gestations, including twins, triplets or more children at once  Maternal immune system disorders (HIV/AIDS)  Maternal use of alcohol or tobacco during pregnancy  Intrauterine growth restriction (IUGR)  History of prior abruption, IUGR, or preterm birth

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HIE HELP CENTER: MEDICAL INFORMATION Just because a pregnancy can be classified as high-risk does not necessarily mean that the child will have a birth injury like hypoxic-ischemic encephalopathy. Often, the risk stems from medical staff not recognizing a high-risk pregnancy when they should have, and not taking the proper steps to properly inform mothers or conduct tests and monitoring. High-risk pregnancies must be properly monitored to allow the parents and doctors to work together to mitigate the risks of this kind of pregnancy. Maternal Obesity and High BMI High body weight can be a delicate subject because weight loss can be difficult for patients. Sometimes physicians can gloss over the health risks related to obesity because the topic is a difficult one to broach, but it is a necessary one. Some mothers are not aware of the health risks related to high BMI, which can include birth complications and restricted growth in the baby. Women who have a very high BMI have a bigger risk of having gestational diabetes, hypertension (high blood pressure), preeclampsia, and premature birth. There is also a risk that babies can be macrosomic (far larger than expected), which can make labor difficult. Alternatively, there are also cases where the babies of women with a very high BMI have had a condition called intrauterine growth restriction (IUGR), which means that the baby is not the size expected for their gestational age. Babies with IUGR tend to tolerate labor poorly and are at a higher risk of brain bleeds. Diabetes and Gestational Diabetes Diabetes is a condition that should be properly controlled and monitored for both the health of mother and child. In some cases, diabetes can be first diagnosed during a pregnancy. This kind of diabetes is called gestational diabetes. Uncontrolled or poorly managed diabetes can cause health problems in the mother, but also poses a health risk for the child, because these babies can become macrosomic (very large), prolonging labor. It is also more likely that macrosomic babies’ shoulders will get stuck on the mother’s pelvis due to their size, increasing their risk of a condition called Erb’s palsy, which is damage to the brachial plexus nerves. When a baby gets stuck in the birth canal, this is a direct factor that influences the baby’s risk of hypoxic-ischemic encephalopathy. High Blood Pressure Mothers and babies are connected to each other via the placenta and umbilical cord, which provide a constant flow of nutrients and oxygen to the baby. These nutrients and oxygen are carried to the baby through the blood. Blood flow is regulated by numerous factors, but one of the most important factors is blood pressure. Very low or very high blood pressure can compromise the free flow of blood between the two, which can be a risk for the oxygen deprivation injuries of hypoxic-ischemic encephalopathy. High blood pressure can also damage the mother’s kidneys, which can cause low birth weight in the baby/ intrauterine growth restriction. Typically, babies with IUGR need to be delivered via C-section before term, because they cannot tolerate labor as well as a normal- sized baby. Preeclampsia Preeclampsia is a subtype of high blood pressure, but it is specifically high blood pressure that is first diagnosed during a pregnancy. This is a particularly severe form of high blood pressure because there’s no way to ‘cure’ the preeclampsia other than delivering the baby. Preeclampsia can cause end-stage organ failure in the mother, and – if it is severe – can cause the mother to have seizures during birth and delivery, which can result in the death of both the mother and baby if improperly managed. Because preeclampsia is an exceptionally risky condition (and because it can progress from mild to severe very quickly), physicians deliver babies prior to term to protect both the mother and the baby. Preeclampsia is also a risk factor for IUGR. Multiparous Births (Multiple Births) Having multiples is a joyous occasion, but also requires some additional monitoring on the part of doctors. These births often occur when women have had fertility treatments or have a baby after age 30. These babies are at a greater risk for premature birth for several reasons, but the precise mechanisms of why are currently under investigation. Multiple births are a significant contributor to preterm births (more than half of women with twin pregnancies deliver at 37 weeks or earlier), which are associated with poorer outcomes including HIE.

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HIE HELP CENTER: MEDICAL INFORMATION Immune Disorders (HIV/AIDS) Mothers with immune system disorders are considered to be high-risk, as this population must be monitored and treated properly to reduce the risk of transmitting HIV to their child. Medical staff must inform mothers with HIV of advised steps, such as taking ART (antiretroviral therapy) and having a C-section rather than a vaginal birth. After birth, there are certain steps that doctors will carry out, such as advising mothers not to breastfeed and to follow treatment plans closely. Alcohol and Tobacco Use During Pregnancy It is the responsibility of medical staff to inform mothers who use alcohol and tobacco about the risks of substance use during pregnancy, as this can negatively affect the baby’s growth and development. With alcohol especially, infants are at risk for fetal alcohol syndrome (FASD), which is known to cause intellectual disabilities and developmental delays. Smoking is associated with IUGR and other long-term health difficulties. Physicians must inform pregnant patients who smoke of smoking cessation programs. Smoking cessation programs have been proven to help patients quit smoking. Umbilical Cord Issues There are many things that can be concerning in regards to the umbilical cord. Because the umbilical cord is the baby’s sole source of oxygen and nutrients, it is critical that the umbilical cord function properly. If it is occluded, compressed, or knotted, this can compromise the flow of oxygen and nutrients to the baby and cause injuries. Examples of umbilical cord issues can include:  Short cord  Long cord  Nuchal cord (when the umbilical cord is wrapped around the baby’s neck)  Prolapsed cord (when the umbilical cord exits the uterus before the baby does)  Compressed umbilical cord  Knotted umbilical cord  Infected or inflamed cord  Infarcted cord Placental or Uterine Complications The umbilical cord is attached to the placenta, which is a dense network of capillary beds and oxygenated tissue that feed the baby nutrients and oxygen. Issues with the placenta have the same impact as issues with the umbilical cord – compromising the flow of oxygen and nutrients will harm the baby. Issues can include: Uterine Rupture In some rare cases, a mother’s uterus may tear during delivery. This can cause the baby to move into the abdominal cavity (stretching or compressing the umbilical cord) and is accompanied by massive bleeding. This can happen when a mother attempts a vaginal birth in the presence of uterine scarring (from a prior C-section, hysterotomy, myomectomy or petroplasty). This can also be accompanied by placental abruption. When massive bleeding occurs, the mother’s blood pressure may drop and decrease blood flow to the baby. This emergency situation requires a C-section delivery. Placental Abruption Usually, the placenta stays attached to the uterus until delivery. In a placental abruption, the placenta separates partially or completely from the uterus early. This can compromise oxygen flow to the baby since the baby’s only source of air is through the placenta and the umbilical cord. If the placenta is partially or completely detached from the uterus, air and blood flow to the baby will decrease. The severity of an abruption can vary, but a mild abruption can turn severe very quickly.

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HIE HELP CENTER: MEDICAL INFORMATION Placenta Previa In most cases, the placenta connects to the uterus far away from the mother’s cervix. With placenta previa, however, the placenta attaches to the uterus close to the cervix, which can cause life-threatening bleeding during delivery. This condition can be detected if a mother reports on-and-off bleeding during the second half of her pregnancy; doctors should be able to diagnose this using ultrasound. In some cases, a low-lying placenta migrates out of the way as the pregnancy progresses, but if it does not, the risk of HIE can be reduced by delivering via C- section. Placental Insufficiency In some cases, the placenta can’t deliver enough blood to the baby. When this happens, the baby is often diagnosed with intrauterine growth restriction (IUGR) and the mother often has oligohydramnios (low amniotic fluid). Doctors should be monitoring these pregnancies closely with non-stress tests and biophysical profiles, as well as ultrasounds to measure the baby’s size, amniotic fluid levels and placental structure. They should also conduct Doppler ultrasounds to see how blood is flowing to the baby. There are usually no symptoms of insufficiency, but adequate monitoring should catch this condition. Vasa Previa Vasa previa occurs when the fetal blood vessels are exposed and cover the opening to the birth canal. Fetal blood vessels travel within the umbilical cord and attach to the central region of the placenta in normal uteroplacental circulation. However, when vasa previa is present, some of the fetal blood vessels travel within the fetal membranes and across the opening of the birth canal. Vasa previa can cause hypoxic-ischemic encephalopathy. Cervical Issues The cervix is a structure between the uterus and vagina that normally stays closed during pregnancy. If the tissue is weakened and it opens too early (cervical insufficiency), the protective membranes surrounding the baby can bulge through this opening and rupture before the baby can survive in an outside environment. In many cases, there are no symptoms, but doctors are responsible for screening for risk factors (such as prior cervical insufficiency, a history of D&C procedures, previous traumatic birth, prior premature rupture of membranes, or uterine anomalies). If a mother has risk factors, doctors should then perform a physical exam and multiple transvaginal ultrasound studies (TVS) over time to track cervix length. Doctors can help the cervix stay closed using cervical cerclage or progesterone treatment. Oligohydramnios/Polyhydramnios Oligohydramnios is a complication characterized by insufficient levels of amniotic fluid. Polyhydramnios is when there is too much amniotic fluid. As the baby develops, the amount of amniotic fluid tends to increase until the later parts of pregnancy, helping with nutrition and lung development. There are certain levels of amniotic fluid considered normal. If there is too much amniotic fluid, the fluid can push on the umbilical cord, compressing it and compromising blood flow. Cord compression may increase the risk of HIE if there is too little amniotic fluid as well. Risk factors for these include high blood pressure, diabetes, and placental issues. Doctors should be screening for amniotic fluid issues with physical examinations and ultrasound during the pregnancy. Infections in the Mother or Baby Some infections show no symptoms in the mother but can still cause HIE in the baby’s rapidly developing brain. Doctors should be gathering medical histories, conducting screens and tests for certain infections, and treating the mother so the infection doesn’t get passed to the baby during birth. In some cases, like when a mother has an active infection during delivery, it is imperative to give antibiotics and many times to deliver via C- section before the membranes rupture, so the baby is not exposed to infection via vaginal delivery. Some of these infections include:  Chorioamnionitis and villitis  Group B strep  Bacterial vaginosis

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HIE HELP CENTER: MEDICAL INFORMATION  Herpes simplex virus (HSV, also known as genital herpes) Depending on what the infection is, the baby may be diagnosed differently. The effects of neonatal infections are similar, however: infections can cause seizures, HIE, and a need for NICU admission. In many cases, adequate screening and proper prophylactic treatment (preventative measures) will prevent infection. Diagnoses related to infection include sepsis, meningitis, encephalitis, and in some cases pneumonia. Sepsis refers to an infection that circulates through the blood and tissues, meningitis is inflammation of the membranes around the brain and spinal cord, while encephalitis is brain inflammation caused by a virus. Pneumonia is a bacterial lung infection that can cause the baby to develop breathing issues and oxygen deprivation. Intrauterine Growth Restriction (IUGR) There are certain ranges of weights that babies should be at specific times in the developmental process. If a baby isn’t reaching appropriate size during pregnancy, it may have IUGR. While there are numerous factors for what causes IUGR (placental issues and underlying maternal health issues among them), medical staff should be screening for IUGR with regularly-scheduled ultrasounds and other tests such as Doppler flow, weight checks, amniocentesis, non-stress tests and biophysical profiles. Proper assessment of risk factors is critical. Once diagnosed, IUGR babies must be closely monitored and delivered early, as many of them do not fare well in labor. Lack of close monitoring and timely delivery during labor can result in HIE for babies with IUGR. Labor and Delivery Errors There are cases where emergency interventions are needed, both in high-risk and low-risk pregnancies. During labor, unforeseen complications require prompt responses from medical teams; failure to follow proper protocols can result in HIE. Common labor and delivery errors include:  Failing to prevent a preterm birth: Premature birth is a known risk factor for hypoxic-ischemic encephalopathy, as premature babies are not done developing yet, making their brains more fragile and susceptible to injury. There are interventions that doctors should be performing to help prevent premature birth. These interventions include:  Using a cervical cerclage if there is cervical insufficiency.  Treating a pregnancy with multiple gestations (twins, triplets, etc.) as a high-risk pregnancy and monitoring the pregnancy closely. Often, doctors recommend early delivery, usually via C-section, though induction can be an option as well.  If a mother has a history of preterm birth or has risk factors for preterm birth, she can receive progesterone treatment to prolong the pregnancy. Progesterone treatment is only effective in women with a singleton pregnancy, not with twins or triplets, etc.  Failing to prevent premature rupture of membranes (PROM): PROM occurs if a mother’s membranes rupture (‘water breaks’) more than 18 hours before labor starts. This can pose a risk for HIE and other birth injuries because the amniotic fluid that protects the baby from infection is gone. Doctors should administer antibiotics to decrease the risk of infection; in many cases, these babies need to be delivered via C-section because of the high risk of infection-related complications and umbilical cord compression issues. Doctors can also administer corticosteroids to mature the baby’s lung if they are preterm. Preventing PROM (and PPROM, which occurs together with preterm birth) means that doctors should be screening for infections. Cord compression is a serious risk with PROM.  Prolonging a pregnancy for too long: If a baby continues to gestate for longer than 40 weeks in a post-term pregnancy, he or she can develop post-maturity syndrome. After around 37 weeks, the placenta starts to break down in preparation for delivery. Usually, this is not a problem, but if a baby gestates for too long, it can be exposed to hypoxic (low-oxygen conditions) as the placenta continues to deteriorate. Indeed, as of June 2016, the American Congress of Obstetricians and Gynecologists recommend that women be induced at 39 weeks, as waiting longer greatly increases the risk of birth injuries and hypoxic-ischemic encephalopathy.

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HIE HELP CENTER: MEDICAL INFORMATION  Prolonging labor and delivery for too long: While every pregnancy is different, and, therefore, the length of labor is different, there are certain indicators that medical personnel look for to see if labor is progressing normally. If labor is stalled, some medical interventions might be necessary to help the mother deliver the baby safely.  Traumatic birth: The process of birth requires the baby to have mechanical force exerted on its body, as with the contractions of labor and (when a mother might need some help delivering) assistive maneuvers from medical staff. Traumatic birth occurs when a baby is injured in the birthing process. These injuries can occur to the body, tissues, organs or brain. There are many reasons why a birth might be traumatic, but some of the most common include attempts at vaginal delivery when the baby is in a face or breech position, is too big to fit through the mother’s pelvis, or has a shoulder stuck on the mother’s pelvic bones. Birth trauma can also occur due to over-strong contractions stimulated by delivery drugs, or improper technique when using a forceps or vacuum extractor, which can cause brain bleeds, skull fractures and hypoxic-ischemic encephalopathy.  Attempting to continue a vaginal delivery when a C-section is safer: If a baby is large for its gestational age or has macrosomia, it has a higher risk of getting stuck on the mother’s pelvic bone in a condition called shoulder dystocia. If the baby is stuck or is too large to pass through the birth canal (cephalopelvic disproportion), there is a higher risk of oxygen deprivation. Some practitioners attempt to assist with a vaginal delivery using forceps or vacuum extractors, but this can increase the risk of a traumatic birth – a birth where physical force can cause bleeding, physical trauma, or fractures. There are also emergency situations where a baby has to be delivered in between 3 and 30 minutes (between decision to start C- section and the actual incision time), depending on the circumstances. Waiting longer in an emergency increases HIE risk significantly.  Attempting a vaginal delivery when the baby is positioned in a face-first or breech position: Usually, a baby is delivered head-first. If a baby is delivered with their face pointing outward first, or feet-first, it is more likely that they will have a traumatic birth or have an umbilical cord prolapse or compression. While there are methods of attempting to shift the baby, it is often recommended that these babies be delivered via C-section.  Attempting a vaginal birth after a history of C-section (VBAC): While in many of cases, a VBAC can be a safe experience for a mother and child, in others, a VBAC poses a heightened risk of HIE. After a C-section, uterine tissue can develop scars. These scars are points that can potentially rupture during labor, especially if mother had a classical incision or a low vertical incision. Uterine ruptures can lead to hemorrhages, and this bleeding can pose a health risk to both mother and child.  Not properly monitoring a baby’s heart rate: It is standard practice for fetal monitoring to begin once a mother is admitted to the labor and delivery ward. This monitoring helps doctors watch the baby’s heart rate – if the baby’s heart rate drops, it may mean the baby is having fetal distress, which can lead to HIE. Monitoring requires staff to be able to recognize and properly read heart tracings; if medical personnel fail to recognize signs of distress or respond quickly enough, it is likelier that a baby will suffer HIE.  Making mistakes in administering anesthesia: There are several medications that are commonly used in childbirth. For pain relief, women can undergo an epidural, or, in the case of C-section, anesthesia. This has risks like low blood pressure (which can compromise fetal blood flow, causing fetal distress), weaker contractions (which medical personnel compensate for using Pitocin or Cytotec), and a prolongation of labor as mothers can lose or decrease their ability to ‘bear down’ or push. All of these factors impact oxygen supply to the baby.  Making mistakes in administering medication: Cytotec and Pitocin are often used to increase the strength of contractions and speed up labor. Both of these carry risks of hyperstimulation, where the uterus contracts too hard or too fast. In labor, blood flow to the baby decreases during a contraction and resumes when the contraction ends; most healthy babies can recover from this very well. If hyperstimulation happens, however, there is little to no time between contractions,

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HIE HELP CENTER: MEDICAL INFORMATION keeping blood flow from resuming to the baby. This increases the risk of HIE. Additionally, the use of these drugs is associated with uterine rupture – if a woman has had prior C-section or other uterine surgery, the contractions can become intense enough to cause uterine rupture, which can cause hemorrhaging that compromises blood flow to the baby. Neonatal Health Mismanagement Babies can develop health issues after birth that require proper intervention. If intervention is done incorrectly, or is delayed, the impact of these health issues can become much worse. In many cases, however, these complications can be avoided with proper monitoring and adherence to standards of care. These health problems include:  Neonatal breathing problems: Babies who have gone through a difficult birth can sometimes need help breathing. If they are born not breathing at all, they are resuscitated, first with positive pressure ventilation (PPV), and then, if the procedure is not successful, with several alternatives in an attempt to get the baby breathing again. If resuscitation does not work, they are placed on a breathing machine called a ventilator. Medical staff must be able to place the tube properly, or they risk the baby not having air delivered to the lungs at all, along with stomach tears and lung collapse. Medical staff also have to regulate the pressure of the gases being passed into the baby’s lungs or the baby can be at risk of the lungs getting too distended and injured to work (pneumothorax). This type of lung injury can severely impact the baby’s ability to deliver oxygen to its tissues. Blood acidity and oxygen saturation are important metrics, as low pH and low oxygen saturation can indicate that the baby is still not getting enough air. These are indicators that the ventilator is over-ventilating (removing too much carbon dioxide from the baby’s blood). This can cause lung collapse and hypocarbia (low CO2 levels), which in turn cause HIE and an injury called periventricular leukomalacia (where the baby’s brain tissue starts to die and ‘soften’ around the periventricular area). This can in turn cause brain bleeds and fluid leakage into the ventricles (hydrocephalus), where cerebrospinal fluid leaks into the ventricles, enlarging them and interfering with the development of the cerebral cortex. This impairs the development of the growth of the cerebral cortex, a key part of the brain needed for memory, attention, cognition, thought, and consciousness.  Improperly treating meconium aspiration syndrome (MAS): If a baby has MAS, it is an emergency. In MAS, the baby inhales particles of his or her stool during labor, which has the potential to block airways, decrease oxygen to the baby’s brain, and cause infection and pneumonia. Babies with respiratory distress and MAS are admitted to the NICU, and are treated with some combination of airway clearance, ventilation, supplemental oxygen, surfactant therapy, steroid therapy, nitric oxide, ECMO or radiant warmer to maintain their body temperature, depending on the severity of the baby’s respiratory distress. If the baby’s MAS is not treated properly, it may suffer from HIE due to oxygen deprivation.  Improperly treating jaundice and kernicterus: Babies can develop yellow skin, poor feeding and lethargy due to the increased concentrations of bilirubin (a product of the breakdown of red blood cells) after birth. Babies can need some help in safely removing this bilirubin, however, especially if it develops in the first 24 hours after birth or between days 3-7 of life. Medical staff place the babies under special blue lights or under a fiber optic blanket in a process called phototherapy, which helps the body safely remove the extra bilirubin. This is a noninvasive and easy treatment which should prevent jaundice from getting worse. If the bilirubin level becomes too high, exchange transfusions will be needed. If the jaundice isn’t recognized in time or the proper treatment is not given, bilirubin can cross the blood-brain barrier and cause kernicterus, a form of brain damage, which often overlaps with HIE.  Improperly treating neonatal hypoglycemia: It is critical that babies’ blood sugar never drop too low. A baby’s developing brain depends exclusively on glucose for energy, so low blood sugar levels can cause brain cells to die, causing brain bleeds and HIE. This is one of the most common neonatal health issues, and one of the most easily solved. Hypoglycemia can occur if the baby has:  Too much insulin (a disorder called PHHI)  Intrauterine growth restriction (when the baby is small for their gestational age)  Premature birth

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HIE HELP CENTER: MEDICAL INFORMATION  Abnormally high body temperature (hyperthermia)  Abnormally high red blood cell mass (polycythemia)  Sepsis (bloodstream infection)  Growth hormone deficiency  Dysfunctions in the glucose generation or breakdown pathways  Depleted glycogen levels (due to oxygen deprivation or starvation) If a baby is at risk for hypoglycemia or shows signs of hypoglycemia, blood glucose concentration must be determined within minutes (ideally via lab testing, but with a testing strip later confirmed with lab results if lab results cannot be processed quickly). Hypoglycemia can be solved with extra feedings of breastmilk or formula. If blood glucose levels are extremely low, the baby may also be given glucose solution via IV. Treatment can last up to a week (or until the baby can maintain normal glucose levels); it can take longer in premature babies, those with infections or those with low birth weight. Continued low glucose can require further specialized treatment.

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HIE HELP CENTER: MEDICAL INFORMATION Associated Conditions Hypoxic-ischemic encephalopathy is a brain injury that can impact many different parts of the brain. In many cases, it can be difficult to know exactly what impairments a child will have as they age, because, although the brain injury itself does not evolve or worsen, it may have secondary effects that evolve as the child grows and matures. Sometimes issues like learning disabilities or cognitive delays may not always show up until the child reaches early adolescence. We’ve compiled a short list of conditions that have been associated with HIE, in order to help parents learn about, anticipate and prepare for potential health concerns. It is important to remember, however, that each child’s case of HIE is different. Some cases of HIE (if properly treated with hypothermia therapy and other treatments) may cause cognitive rather than visible physical functional effects on a child, while in other cases, a child may have more severe outcomes, including global developmental delays and cerebral palsy. There are ways to support the development of a child with HIE, particularly:  Hypothermia therapy within the first six hours of birth  The use of Early Intervention services and intensive therapy to help maximize a child’s abilities Some preliminary research finds that stem cell therapy can help some children with HIE, though research is still in the early stages and is not always broadly available thus far. Some parents choose to have their children undergo stem cell therapy outside the borders of the United States, but this requires careful evaluation, and such unregulated practices may have risks. The effects of some of the following conditions can be mitigated with optimal treatment and therapy, and supportive equipment and devices. To learn more about existing and potential future HIE treatments and therapies, please request our informational packet on treatments and therapies for HIE by calling (888) 329-0122.

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HIE HELP CENTER: MEDICAL INFORMATION Behavioral and Emotional Disorders Children with hypoxic-ischemic encephalopathy (HIE) may sometimes have behavioral and/or emotional disorders. These can be directly the result of brain injury to the portions of the brain that control emotional regulation, or they may stem from other issues, such as frustration at particular limitations, social situations, or other disability- related concerns. The severity of the emotional or behavioral disorder can vary with the severity of the condition and the degree of limitation that the child has, requiring active management with the help of a psychologist, psychiatrist or therapist. If a parent suspects that a child may have a behavioral or emotional disorder, they can seek out the assistance of a specialist trained in a program called ‘cognitive behavioral therapy’ (CBT), which aims to help children identify, explore, challenge and change certain behaviors and emotional responses. The method is rooted in the idea that certain patterns of thought and behavior are learned – because they are learned, they can be unlearned and modified to make them more productive. Signs and Symptoms of Behavioral or Emotional Disorders Because each child is different, the kinds of behaviors they can display can differ vastly. Generally, however, behavioral and/or emotional disorders tend to manifest themselves in maladaptive, negative or disruptive behaviors and emotional signs, including (but not limited to):  Anger or aggression  Anxiety  Antisocial behavior  Anxiety  Disinhibition/risk-taking behaviors  Depression  Difficulty in performing or executing tasks  Emotional distress  Feelings of helplessness  Frustration and irritability  Isolation  Loss of interest in things the child once loved  Poor academic performance  Moodiness  Peer rejection  Social difficulties  Repetitive hostile, defiant or disobedient behaviors Addressing Behavioral and Emotional Disorders If these behaviors become disruptive or occur in response to frustration, a child may benefit from CBT. It is also useful in situations where the child is deemed to be a possible harm to themselves or other individuals. In less severe circumstances, children and families may benefit from CBT if quality of life within the family unit is compromised or when the child seems to have difficulty bonding with other family members, friends or members of the community. It is also useful in situations where the child is being bullied and, as a result, feels irritable, isolated, rejected or depressed.

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HIE HELP CENTER: MEDICAL INFORMATION Parents can reach out to several different types of practitioners to provide cognitive-behavioral therapy, including licensed professional counselors, marriage/family therapists, psychotherapists, or psychiatrists. Each type of practitioner provides a slightly different perspective:  Licensed professional counselors: Specialize in behavioral therapy and developmental progress; focus on assisting clients with specific challenges relating to the tasks of everyday life.  Marriage/family therapists: Specialize in helping maintain or repair the family unit; focus on relationship dynamics.  Psychotherapists: Specialize in helping develop positive coping mechanisms, developing interpersonal relationships, and achieving full potential; focus on improving mental health, increasing self-awareness and improving perspective.  Psychiatrists: Specialize in assisting individuals that benefit from pharmacological interventions; focus on applied psychotherapy and the biopsychosocial model of mental illness. Each of these practitioners can provide a slightly different kind of service; however, the benefits extend not only to the child, but also to the family unit. In many cases, the therapist will work not only with the child, but also with parents and siblings to identify problematic behaviors and develop more positive responses to specific concerns the family may have. This can result in an overall improvement in the child’s ability to manage stress and frustration, the child’s ability to address academic and social challenges, and the family’s ability to positively adjust routines and daily living practices to accommodate for the child’s disability. As the child ages and is expected to transition into adulthood, the skills learned in cognitive-behavioral therapy will help them in coping with the stresses of increased responsibility and potential independence. Behavioral and Emotional Disorders: Additional Resources For further information on finding a therapist for a child with behavioral or emotional disorders, check out the following resources:  Kid’s Mental Health Information Portal  GoodTherapy.com: Children’s Mental Health and Therapy Overview  KidsHealth: Taking Your Child to a Therapist  Speaking to a Social Worker  How to Choose a Child Therapist  Identifying Emotional or Behavioral Disorders  Pacer Center: Emotional and Behavioral Disorders and Children’s Mental Health Issues

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HIE HELP CENTER: MEDICAL INFORMATION Cerebral Palsy One of the common manifestations of HIE is cerebral palsy, a musculoskeletal condition that can cause physical disabilities. The disorder is non-progressive, meaning that it does not get worse over time. Cerebral palsy (abbreviated as CP) manifests itself as either the loss or impairment of motor control or function, but cerebral palsy is caused by underlying brain damage. This brain damage can be caused by brain injury (during birth or soon after the baby is born). In most cases, this brain damage is from hypoxic-ischemic encephalopathy (HIE). Research suggests that a high percentage of cerebral palsy cases result from HIE occurring during pregnancy or during labor and delivery. There are numerous intersecting factors that can lead to cerebral palsy, including HIE, accidents, abuse, medical mistakes/negligence, and infections. What Does Cerebral Palsy Look Like? Cerebral palsy shows up first and foremost as a physical impairment, though the severity of the condition can vary dramatically. There are different levels of ‘involvement’ – some cerebral palsy cases are very mild while others are very severe. Mild cases might show impaired function in just one limb, while the most severe cases of cerebral palsy may cause patients to have impaired function in all of their limbs and their face. This impaired function can show up as muscle weakness, contractures (tightness), stiffness and painful positioning. In some cases, contractions can cause tremors, shaking or writhing motions. Cerebral palsy can also impact balance, hand-eye coordination, or posture, as it may be harder for children with cerebral palsy to grasp objects or perform actions like brushing teeth or buttoning clothes, as these require fine motor coordination. CP can also manifest itself in a child having trouble swallowing, eating, or speaking, or having poor facial muscle tone as well. In an infant, this can look like a baby having trouble latching or sucking. While cerebral palsy is a purely musculoskeletal disorder, it can also be accompanied by intellectual or developmental delays, seizures and other difficulties such as hearing or vision loss. This does not necessarily mean that every child with cerebral palsy will also have intellectual delays – the diagnosis’ severity is highly dependent on factors such as where the injury occurred, how long the child was oxygen-deprived, whether medical staff properly responded to fetal distress, and whether the child was properly treated with hypothermia therapy after the incident. Signs and Symptoms of Cerebral Palsy A sign is a clinically identifiable effect of a particular condition that a medical staffer uses to diagnose a patient. A symptom is an effect that a patient might identify or express to a physician but may be difficult to objectively verify. When a patient complains of symptoms, medical staff members can diagnose the patient using signs, clinical tests and exams. Sometimes, a baby may show signs or symptoms of cerebral palsy in infancy, but in other cases, cerebral palsy is diagnosed once the child begins to miss developmental milestones around ages 3-5. These developmental delays are often caught by parents, who can observe that their child might not be rolling over, sitting up, crawling or walking properly at the right developmental stage. They may bring these concerns up with a medical practitioner, who can conduct tests to look for signs of CP, including abnormal muscle tone, poor posture, or abnormal reflexes. In some cases, doctors may diagnose cerebral palsy very early if the baby had a traumatic or difficult birth and they suspect that multiple risk factors were at play. In other cases, the diagnosis might come late because CP tends to show up as the child’s brain continues growing. In some cases, cerebral palsy shows up when the child is at the developmental stage where they are developing higher-order brain processes but they fail to meet their milestones.

Signs and Symptoms of Cerebral Palsy

Sign/Symptom What does this look like?

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HIE HELP CENTER: MEDICAL INFORMATION

Abnormal muscle tone Limbs that are abnormally floppy, flaccid, stiff, or rigid Involuntary muscle spasms Joints that are fused together Muscle spasms accompanied by rhythmic contractions

Trouble with Spastic movements, ‘scissoring’ legs, writhing movements, trouble Movement, with balance or fine motor control, difficulty walking Coordination or Muscular Control

Abnormal Reflexes The presence of certain primitive reflexes that are normally expected to disappear (asymmetrical tonic reflex, spinal gallant reflexes, palmar grasp reflex, placing reflex, startle reflex)

Development of Early Children usually start preferring one hand over another when they Hand Preference are about two years old; earlier preferences can indicate that something is wrong

Undeveloped or Late  When an infant doesn’t develop the Landau reflex around Postural Responses 4-5 months  When an infant doesn’t develop a Parachute response around 8-10 months  When an infant doesn’t develop head righting around 4 months  When an infant doesn’t develop trunk righting around 8 months

Trouble Balancing Inability to sit without using hands for support, swaying while standing, unsteady walking, abnormal gait, trouble making fast motions, and needing to use hands to support themselves for activities that need balance

Trouble with Gross Inability or delayed development of the ability to make large Motor Function coordinating movements using multiple limbs and muscle groups (such as walking, running, jumping, balancing, or rolling, sitting up, crawling, standing, walking or balancing in a baby)

Trouble with Fine Motor Intention tremors as children get closer to completing a task, Function difficulty with grasping small objects, holding objects between thumb and forefinger, or doing tasks like setting objects down gently, coloring, or turning a page

Oral Motor Function Trouble with using the lips, jaw or tongue, including difficulty with Difficulty speaking, swallowing, feeding/chewing, and drooling

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Epilepsy, Seizures and Seizure Disorders One of the most common signs that a baby might have hypoxic-ischemic encephalopathy (HIE) is the presence of seizures after birth. Usually, a baby’s brain has normal electrical activity, but, in the case of seizures, these patterns of electrical activities are in some way abnormal. These can cause involuntary jerking motions and visible symptoms, though in some cases, the signs are very subtle. Sometimes, the best (and sometimes only) way to detect a baby having a seizure is using an EEG monitor to track the baby’s brain activity. Overall, about half of newborn seizures are subtle seizures, which can be hard to see. An addition ¼ of infant seizures are clonic seizures, marked by a slow rhythmic jerking or twitching of one body part. 5% of infants have tonic seizures, marked by sustained contractions, sometimes accompanied by rolling eyes and apnea. The most severe type of seizures is myoclonic, because myoclonic seizures indicate very severe brain damage. These are marked by very fast twitching or jerking and are most often found in premature babies. Seizures are not just concerning for the parent, but can actually make the damage from hypoxic-ischemic encephalopathy worse. What Do Seizures Look Like in a Baby? Seizures may manifest differently in different babies, but many babies show one or more of the following. In addition to jerking or twitching motions, a baby having a seizure can have:  Apnea (a time period where they stop breathing)  Repetitive movements of the face  “Bicycling,” which appear as pedaling motions of the feet  Staring into space  Stiff or tight muscles groups  Abnormal bending or stretching of the arms or legs Recognizing the Risk Factors for Seizure Activity Because seizures are associated with cognitive difficulties and cerebral palsy, recognizing when they occur is crucial to making sure your child gets proper treatment. While seizure diagnosis is carried out by medical professionals using EEGs, MRIs and CT scans, parents can learn about when seizures are more likely to occur and bring up any concerns they may have. If a baby is born not breathing, has a low APGAR score, or needs to go to the NICU after they are born, these can be signs that the child is likely to be at risk for seizures. The risk factors for seizures are the same as the risk factors for hypoxic-ischemic encephalopathy; please see our page on risk factors for HIE here.

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HIE HELP CENTER: MEDICAL INFORMATION Fetal Stroke Hypoxic-ischemic encephalopathy is, at its core, an injury caused by a lack of oxygen and/or blood flow to a part of the brain. Often, if a baby is diagnosed with a fetal stroke (also known as a perinatal stroke), they also have HIE, as it is very common for them to co-occur. Both diagnoses require prompt treatment to minimize damage. Both must also be promptly diagnosed to allow medical practitioners to begin rehabilitation as soon as possible. Stroke can happen in as little as three minutes. It can occur after a traumatic injury, where brain bleeds begin to form blood clots in important areas of the brain. These blood clots get stuck in the pathways that allow blood to circulate, preventing oxygenated blood from reaching tissues. This kind of stroke is called an ischemic stroke. After three minutes, oxygen deprivation begins to affect the tissues, which die off in a ripple pattern from the location of the blockage that caused the compromised blood flow. Hemorrhagic stroke can occur as a result of birth trauma, where the brain vessels rupture due to too much mechanical force exerted on them, often as a result of misused forceps or vacuum extractors. Fetal stroke often leads to seizures, which can either be apparent or ‘silent’ in a baby. Seizures can cause further brain damage, so it is exceptionally important that babies with a stroke diagnosis be continuously monitored using EEG – EEG monitoring traces the baby’s brain activity, and can detect abnormal electrical activity even in cases where the baby might not be showing any externally recognizable symptoms, like:  Trouble feeding  Trouble breathing  Lapses in breathing (apnea)  Favoring one hand over another The difficulty with such symptoms is that these symptoms can appear after the critical 6-hour time frame for preventive hypothermia therapy treatment is over, which is why close early observation and EEG monitoring is so critical. Hospitals that can’t do continuous monitoring should still be doing near-continuous EEG testing. If a parent learns that their child has had a stroke, there are certain tests that can be done to determine where and how the brain is damaged. These tests can help inform what kinds of treatments will be necessary:  MRIs  Blood tests  MRAs  MRVs  CT scans  CTA  Cranial ultrasounds  Lumbar punctures There are ways to treat fetal stroke, including providing IV fluids and blood thinners. Babies who have had one stroke are often at risk for another one because existing brain injury make the brain’s cells fragile and prone to bleeding. When the tissues are prone to bleeding, they are also susceptible to more clotting. Blood thinners decrease the risk of further clots and help stabilize the baby’s condition. These two treatments, however, do not help repair some of the damage that the stroke already caused. The only treatment that researchers have identified that can help repair some of the brain damage of fetal stroke and hypoxic-ischemic encephalopathy is hypothermia therapy, a process where the baby’s brain is cooled down enough to slow metabolism and help the brain repair itself. While the effectiveness of the treatment can vary (some children treated with hypothermia therapy show no signs of disability, while others have moderate to severe disability), hypothermia therapy has been clinically demonstrated to reduce the level of disability when administered in a timely fashion (within 6 hours of birth, and the sooner the better).

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HIE HELP CENTER: MEDICAL INFORMATION Hearing and Visual Impairments The brain injury caused by an oxygen-depriving event can often cause impairment in a child’s vision and/or hearing. Generally, newborn babies are screened at birth for sensory impairment issues and then are screened periodically throughout their development. If parents have concerns about hearing or vision, they can speak to their child’s pediatrician about evaluations and interventions (such as glasses or a hearing aid). If the child needs assistance, they can then be referred to an ophthalmologist (for vision evaluation and care) or an audiologist (for hearing evaluation and care). These medical professionals can provide recommendations regarding what kind of interventions the child will need – sometimes this may involve glasses or a hearing aid, though there are certain circumstances in which surgical intervention may be appropriate. Many children with HIE-related cerebral palsy are known to have vision impairments, which can include acuity loss, field loss, oculomotor problems, sensory processing difficulties, and hearing loss. Up to 13% of children with CP have hearing loss. Hearing loss associated with HIE can fall into one of two categories: sensorineural or conductive. Sensorineural hearing loss is more difficult to treat because it means that the nerves that transmit signals to the brain are damaged. This can be remedied with a combination of a cochlear implant and hearing aid. The cochlear implant bypasses the injured nerves entirely, acting as a conduit for sound. Conductive hearing loss is simpler to treat, as it tends to involve damage to the physical portions of the middle ear rather than the nerves themselves. This damage is often caused by inflammation and fluid buildup. When this inflammation is addressed, the problem often decreases in severity. For severe cases or recurrent conductive hearing loss, hearing aids and surgery are also options. What Signs Should Parents Look For If They Are Concerned About Their Child’s Hearing or Vision? Signs of Visual Impairment  Difficulties with focusing while looking at objects  Blurred vision  Field vision loss  Problems making fast eye movement  Trouble recognizing familiar faces  Covering eyes while trying to read  Slow speech and language development skills  Moving the head while reading (as opposed to moving eyes)  Underdeveloped hand/eye coordination  Eye squinting when reading or focusing on objects  Holding books or other materials close to the eyes when reading  Frequent headaches Signs of Hearing Impairment  (In babies) Not reacting to human speech  (In babies) Not being calmed by what should be a familiar voice  (In babies) Not startling or waking up at loud noises  Lack of response to simple requests  Delays in language acquisition and speech  Does not show signs of listening to stories or songs

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HIE HELP CENTER: MEDICAL INFORMATION  Not repeating sounds or words  Does not respond correctly to questions Strabismus Associated with Hypoxic-Ischemic Encephalopathy (HIE) One sign of a visual disturbance is called ‘strabismus,’ a term that means a child’s eyes are improperly aligned, making them turn in different directions. When this happens, the set of muscles around one eye may work harder than the other. Because the eye muscles do not work together, the brain may have difficulty combining the visual images from the two eyes. This can mean that the individual may be more prone to an awkward gait, accidents or collisions with their surroundings because their eyes are sending inaccurate images of their surroundings to the brain. An ophthalmologist may be able to correct strabismus and ensure the eyes can work together properly. When Should a Child be Referred Early to a Hearing or Vision Specialist? There are certain HIE-associated diagnoses that indicate that a child should be referred to an ophthalmologist or audiologist early. These include: Indications for Early Referral to an Ophthalmologist  If a child has Stage III HIE  If a child has Stage II HIE with either an abnormal neurological examination or reduced visual examination at discharge from the hospital  If a child has an HIE-associated stroke Indications for Early Referral to an Audiologist  If a child has intrapartum asphyxia, they should be tested for sensorineural hearing loss after birth and before they are discharged from the hospital.  If the child has persistent pulmonary hypertension (high blood pressure) along with HIE, the child may be at risk for late-onset hearing loss and should be tested repeatedly throughout childhood. Vision and hearing are critical for a child’s development, as they are two of the most important sense through which a child perceives the world. Hearing is especially critical for language acquisition, making early intervention an important component of ensuring the child will be able to interact with family, friends, peers and teachers. If a child has some delays in language, speech or social development, hearing aids and glasses – in conjunction with intensive speech/language therapy, specialized educational interventions, and behavioral/social therapies – can help mitigate delays. Additional Information on Visual Impairment  Research: Visual Problems as a Result of Brain Damage in Children  Visual Impairment and Cerebral Palsy  American Association for Pediatric Ophthalmology and Strabismus: Vision Screening  American Association for Pediatric Ophthalmology and Strabismus: Strabismus Facts  Vision Screening in Young Children Additional Information on Hearing Impairment  Hearing Loss and Cerebral Palsy  Checking for Hearing Loss in Young Children  The Importance of Early Hearing  CDC: Hearing Loss Additional Information on Visual Impairment, Hearing Impairment and HIE  Children with HIE: Intensive Followup in the Newborn Period Before Hospital Discharge

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HIE HELP CENTER: MEDICAL INFORMATION  Red Flags for Vision and Hearing Loss

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HIE HELP CENTER: MEDICAL INFORMATION Intellectual and Developmental Disabilities With HIE, there can be a wide range of outcomes when it comes to the level of cognitive or intellectual impairment that a child might experience, depending on numerous factors. The terminology surrounding cognitive and intellectual disabilities can be somewhat confusing, so here is a brief explanation of the differences between these categories.  Intellectual disability: intellectual disability is a broad category of persistent disorders that reduce an individual’s cognitive capacity. It is accompanied by trouble in carrying out the activities of daily living and adaptive behaviors like managing money, schedules, and social interactions. By definition, intellectual disabilities arise before a child is 18 years old. Typically, individuals with intellectual disabilities require supportive programs throughout their adulthood.  Developmental delay: A developmental delay occurs when a child misses developmental milestones at a particular age. Sometimes these go away when a child continues to grow. If they persist, the child is then said to have a developmental disability.  Developmental disabilities: Developmental disabilities are severe long-term disabilities that can impact either cognitive or physical abilities or both. These appear before a child is 22 years old; it is likely to be lifelong and is not expected to improve. Testing for Intellectual and Developmental Disabilities The most well-known test of cognitive ability is the IQ test. The level of impairment that a child may experience can vary quite widely. Outcomes can vary from mild to very severe. Cognitive disabilities can impact both intellectual function (IQ scores of 70-75 or lower) and adaptive behaviors (the ability to apply social and practical skills in everyday life):  Mild cognitive disability: IQ 55-70; usually included in mainstream classrooms.  Moderate cognitive disability: IQ 30-55.  Severe cognitive disability: IQ under 30; often have few communication skills and need direct supervision. While a child with HIE may not always necessarily have a cognitive impairment, a significant portion do. The more severe the oxygen-depriving episode, the more likely a child may have a cognitive disability. Conditions Associated with Intellectual and Developmental Disabilities Cognition covers a very broad range of the brain’s processes, so a cognitive disability might impact numerous different functional areas of the brain, including comprehension, planning, reasoning, decision-making, emotional processing, reading, learning, attention, computation, memory, problem-solving, recognition, speech/language and executive function. Because HIE is fundamentally a brain injury, and because brain injuries can impact numerous areas of the brain, cognitive impairments can be associated with other conditions (co-morbidities):  Anxiety disorders  ADHD  Behavioral difficulties  Mood disorders (such as depression)  Fatigue  Psychological disorders  Sleep difficulties  Autism spectrum disorders Individuals with cognitive or intellectual impairments can benefit from supportive services, as well as specialized education plans (IEPs) to maximize their functional abilities. These educational plans include input from a number of professionals, including occupational therapists, physical therapists, doctors, psychologists, special education

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HIE HELP CENTER: MEDICAL INFORMATION teachers and speech/language pathologist. The Americans with Disabilities (ADA) Act set forth ground rules for providing services to individuals with disabilities; more information about special education law can be found at Wright’s Law, a legal resource specifically devoted to helping navigate special education. Early Detection of Intellectual and Developmental Disabilities Cognitive disabilities can be difficult to detect while a child is very young; typically, parents bring up concerns with their pediatrician when their child does not meet developmental milestones. However, even from a young age, children respond to their environment. The best way to determine if a child has a cognitive disability is to observe them at home and to see whether the child responds to environmental stimuli. If the child doesn’t respond or doesn’t seem interested in what’s going on around them, this may potentially be a cause for concern. In an infant, a lack of response to a parent’s touch or the sound of their voice is cause to go to a doctor immediately. Other signs of cognitive impairments can potentially include:  A child disliking being touched  Delays in language  Trouble with concentration, attention or learning  Trouble with processing information  Outbursts and poor temper  Poor memory  Trouble with social interactions  Trouble speaking or responding to other people

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HIE HELP CENTER: MEDICAL INFORMATION Intracranial Hemorrhages (Brain Bleeds) Intracranial hemorrhages are another term for bleeding that occurs inside the brain or skull. While they can range between very mild to severe, they must be quickly diagnosed in order to properly initiate treatment. They are also related to extracranial hemorrhages, which refer to bleeding that occurs in the tissues outside the skull. Types of Intracranial Hemorrhages Brain bleeds are classified differently depending on the location where the bleeding is occurring. The location and extent of the bleeding determine what kind of damage the bleeding will cause. Cerebral Hemorrhage Bleeding within the brain itself; classified as a type of stroke caused by a ruptured blood vessel. Subarachnoid Hemorrhage Bleeding between the brain and the inner membrane that covers it. This is the second most common brain bleed in full-term babies and can cause seizures, breathing issues and lethargy. This serious bleeding is linked to hydrocephalus, PVL, cerebral palsy and brain damage. Intraventricular Hemorrhage The most serious type of brain bleed where blood is forced into the brain’s ventricles, where spinal fluid is produced. It is most common in premature and low-birthweight infants. Subdural Hemorrhage and Hematoma The most common brain bleed in babies where blood collects on the brain’s surface. This bleeding causes seizures, jaundice (high bilirubin levels), a head that grows too fast, poor Moro reflexes and retinal bleeding. Cephalohematoma Cephalohematomas (or cephalhematomas) are bleeding outside the baby’s skull. These are usually not severe and show up as raised bumps on the baby’s head. These typically go away on their own, but can lead to infections or calcifications if they require treatment and are left untreated.n lead to infections or calcifications if they require treatment and are left untreated. Signs, Diagnosis and Treatment of Intracranial Hemorrhages The symptoms of a brain bleed can vary, but they overlap with the symptoms of HIE. These symptoms can include lethargy, seizures, apnea and breathing issues, feeding issues, bulging soft spots on the baby’s head, the baby being too floppy or tense, and issues with staying conscious. Brain imaging is absolutely critical for diagnosing an intracranial hemorrhage. If medical staff suspect that the baby had a brain bleed, they should be doing head imaging studies using a CT scan or MRI. They can also do ultrasound tests and tests of cerebrospinal fluid as well. Unfortunately, treatment for intracranial hemorrhages tends to be supportive (though surgery might be necessary for relieving pressure in some cases). Some babies with brain bleeds recover very well, while those with severe bleeding (and resulting hypoxic-ischemic encephalopathy) may have intellectual or developmental disabilities and/or cerebral palsy. Learning Disabilities A learning disability does not necessarily mean that a child has an intellectual disability, though some children with learning disabilities do also have intellectual disabilities. A learning disability is a disorder that means a child may have trouble with certain specific academic skills, like reading (dyslexia), writing (dysgraphia) or math (dyscalculia), because their brain has difficulty with processing, storing or retrieving certain kinds of information. People diagnosed with learning disabilities in the absence of intellectual disabilities can do well academically, provided they receive quality instruction and are provided with proper adaptive strategies for creating workarounds, often through an Individualized Education Plan (IEP). Children with learning disabilities in the absence of intellectual disabilities can have average or above-average intelligence and potential, but a learning disability can lower

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HIE HELP CENTER: MEDICAL INFORMATION academic achievement. It is important to screen for learning disabilities properly, as an improper screen may diagnose a child as having an intellectual disability when they instead have a learning disability such as Dyslexia or Dyscalculia. Generally, if a child is not having academic success, the child is provided with ‘Response to Intervention’ services before being formally evaluated for a learning disability. These services help distinguish learning difficulties due to poor schooling from learning difficulties related to learning disabilities specifically. If Response to Intervention services are ineffective, the child is then screened for learning disabilities as part of mandated IDEA protocols. Related Reading: Learning Disabilities For more information on learning disabilities and intervention strategies, please visit the following sites:  LDA America: Types of Learning Disabilities  How to Get Help for Children with Learning Disabilities  Understood.org: Learning Disabilities Overview  Successful Strategies for Teaching Students with Learning Disabilities  National Joint Committee on Learning Disabilities: Responsiveness to Intervention and Learning Disabilities  Individualized Education Plans (IEPs) for Disabilities

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HIE HELP CENTER: MEDICAL INFORMATION Neurological and Mental Health Hypoxic-ischemic encephalopathy (HIE) is a form of brain damage caused by a lack of oxygen and/or blood flow to the brain. This often puts people with HIE at increased risk for cerebral palsy and other disorders and/or pathologies of the central nervous system, including neurological and mental health concerns. The boundary lines between neurological health and mental health are often somewhat hazy, as health concerns relating to the brain can manifest in ways both clinical (as seen, for example, in diagnostic imaging) and psychological (such as with behavioral or mood disorders). Before delving deeper into the specific health concerns of children with HIE, it is useful to define the differences between the concerns addressed by neurologists and those addressed by psychiatrists or psychologists. What Is the Difference Between a Neurological and Psychological Disorder/Disease? Although there is significant debate about the exact boundary lines between these two disciplines, neurological disorders are generally defined as those with a specifically organic cause (physical diseases of the nervous system). Often, neurological diseases or disorders show up in clinical diagnostic testing. Psychological disorders (or mental health disorders) are functional disorders relating to emotional, social or mental stressors. Neurological issues are handled by neurologists or neurosurgeons, while mental health concerns are handled by psychiatrists or psychologists. Some disorders straddle the line between the two, giving rise to the field of neuropsychiatry. Practically, medical professionals evaluate the best way to treat a specific case, and can provide recommendations regarding the kind of care that would be most suitable to the specific needs of your child. It is worth noting that, in the US, the Board Certification for psychiatry and neurology are combined under the American Board of Psychiatry and Neurology. What Are Some of the Specific Neurological and Mental Health Concerns Associated with HIE? Hypoxic-ischemic encephalopathy (HIE) can impact different parts of the brain, so it is difficult to generalize the disorders that may be present in a given individual. However, we do know that a significant percentage of individuals with HIE have cerebral palsy, which is associated with intellectual and developmental disabilities (I/DD) and a higher rate of behavioral and emotional disorders such as depression, anxiety or ADHD, among others. Hypoxic-ischemic encephalopathy is, by definition, an injury to the brain, which means that there is the potential for comorbid neurological disorders as well. What Can Parents Do To Help a Child with Neurological or Mental Health Concerns? Parents of children with HIE may develop a treatment plan together with their primary care physician in order to address any mental or neurological health concerns. This may require referral to a specialist medical professional such as a neurologist, psychiatrist or psychologist. Depending on the results of the medical professional’s evaluation, parents can sometimes provide medication, schedule therapy sessions, or find local support resources for mental health concerns such as depression. State Departments of Behavioral Health and Developmental Disabilities are sometimes able to provide parents with direction regarding what kind of services to seek out for their child, as well as information to ask therapists about during sessions. Learn More About HIE and Neurological/Mental Health:  Cerebral Palsy and Mental Health  SAMHSA Behavioral Health Treatment Services Locator  Mental Health America: Guide to Finding Help and Find an Affiliate  Find a Social Worker  National Institute of Mental Health: Common Mental Health Concerns  [Research] Long-term outcome after neonatal hypoxic-ischaemic encephalopathy  [Compilation] Mental Health Resources  CHIP Insurance for Children’s Mental Health

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HIE HELP CENTER: MEDICAL INFORMATION  [PDF Guidebook] Guidelines for Understanding and Serving People with Intellectual Disabilities and Mental, Emotional, and Behavioral Disorders

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HIE HELP CENTER: MEDICAL INFORMATION Nutritional Health Concerns (Chewing, Eating, Nutrition and the Gastrointestinal System) A significant percentage of children with hypoxic-ischemic encephalopathy (HIE) may require specialized dietary plans due to a wide variety of reasons – some cannot chew or swallow properly (as with cerebral palsy), some are at risk of malnutrition due to physical difficulty eating, and others may sometimes have dysfunctional reflexes that can pose a choking hazard. Dysphagia occurs most commonly in individuals with central nervous system injury, which can include individuals with HIE, cerebral palsy, stroke, or traumatic head injury. For these children, it may be necessary to modify the calorie density of their meals, change the form in which they are provided, or change mealtime strategies to maximize eating efficiency. This should be done under the supervision of a physician, nutritional counselor and/or registered dietician, who can provide help and advice regarding numerous nutritional concerns. Here, we’ll discuss some of the issues that can impact nutritional health. Dysphagia Dysphagia is an ‘oral-motor dysfunction’ that makes swallowing difficult. When a child has dysphagia, they are at increased risk of choking and/or aspirating their food. Because of this, monitoring during mealtimes may be needed. Generally, this occurs with children who have a moderate-to-severe case of cerebral palsy, where the muscles of the face and neck cannot execute the complex movement needed to move food and liquid from the child’s mouth to the stomach. There are two different kinds of dysphagia:  Oropharyngeal dysphagia: The result of abnormalities in the muscles and nerves of the oral cavity, pharynx and esophageal sphincter. This is most often related to nerve and muscle malfunctions that weaken the throat muscles, making it difficult to move food from mouth to throat.  Esophageal dysphagia: A result of the muscle malformations or a malfunction of the lower esophageal sphincter. This type of dysphagia can issues with the movement of food down the esophagus to the stomach. Dysphagia can manifest in different ways – while the most common sign is difficulties with eating, dysphagia can also cause problems with speaking, which uses the same muscles as eating and chewing. Often, the physical difficulty of eating can reduce the amount of food a child will be able to eat, as dysphagia can also make a child tire easily during eating. The following chart outlines some signs of dysphagia: Dysphagia-related Other Potentially Dysphagia-related issues related to Issues directly related to Dysphagia-related Health speech feeding Concerns Falling asleep during Ataxic dysarthria Back or chest pain feeding Difficulty controlling vocal cords Aspirating food Choking (resulting in nasal speech) Apnea during feeding Difficulty voicing or articulating (falling asleep during Constant cough speech feeding) Delayed and/or absent Spastic dysarthria Drooling swallowing reflex Difficulty and/or Verbal apraxia Dry mouth unwillingness to feed Spasms in the esophagus Fatigue

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HIE HELP CENTER: MEDICAL INFORMATION Sensation of throat Heartburn obstruction Pain while swallowing Nasal regurgitation Difficulty or inability to Sluggishness, lack of

make mouth movements energy Taking a long time to eat Sore throat Resistance to feeding Tongue thrust Unexplained weight loss Secondary Malnutrition Because dysphagia often means that children with HIE eat smaller quantities of food, they are at risk of not receiving enough nutrients. Food intake should be closely monitored and parents should work with a nutritionist to ensure the child has adequate macronutrients, vitamins, minerals and other dietary components. Sometimes this might mean supplementation or consultation with a dietary specialist to help figure out the best way to ensure the child intakes enough. Malnutrition can increase the risk of a child failing to thrive, grow or develop according to expected values, and can exacerbate existing conditions. Secondary Dehydration If a person loses more fluid than they retain, brain swelling, low blood volume shock, kidney failure and other adverse events may occur. Low fluid intake (due to fear of choking, aspiration, or breathing issues) must be monitored and remedied. Respiratory Issues Respiratory issues may stem from dysphagia-related aspiration. Aspiration of food or liquid into the lungs can cause pneumonia, a serious infection of the lungs. Consulting with Specialists Parents who are concerned about their child’s nutritional intake can consult with one of several specialists trained in helping to recognize, understand and mitigate nutrition-related health concerns. Often, this requires assessment and evaluation by multiple specialists, including clinical dieticians, occupational therapists, speech-language pathologists, pediatricians, radiologists, neurologists and otolaryngologists (ear/nose/throat doctors), each of which plays a different role in developing a comprehensive nutritional plan. To learn more about the concrete steps that specialists and parents can take to mitigate the risks of malnutrition with HIE, visit our Care Considerations > Eating, Feeding and Meal Planning page.

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HIE HELP CENTER: MEDICAL INFORMATION Oral Health Children with hypoxic-ischemic encephalopathy (HIE) may require additional oral and dental care, especially in situations where there is bruxism (teeth grinding), seizures, cerebral palsy, or intellectual/developmental disabilities. Dental care is one of the most common unmet needs of individuals with disabilities, and access to care may be limited, especially for children with cerebral palsy, autism, or intellectual/developmental disabilities (I/DD). Why Might Children with Disabilities Experience Oral Health Issues? Children with disabilities are often at risk for developing cavities (caries) due to:  Diet (caused by prolonged or frequent feedings)  Insufficient saliva production (due to certain medications)  Limitations in performing oral hygiene (such as neuromuscular or cognitive disability that inhibits teeth brushing)  Oral aversion and behavioral difficulties  Difficulty swallowing and oral motor hypotonicity  GERD and chronic vomiting  Use of medicine containing sugar  Dysphagia (which can leave food in the mouth)  Weak tongues can inhibit the cleaning of oral surfaces  Gagging may inhibit complete brushing of the teeth  An inability to spit may cause toothpaste swallowing.  Malocclusion/teeth crowding (which is more common with I/DD or cerebral palsy)  Crowding makes teeth harder to clean, increasing risk of periodontal disease and cavities. Detecting Oral Health Issues in Kids with HIE Parents of children with HIE or other disabilities should watch out for:  Gingival hyperplasia (gum enlargement), especially if the child is prescribed antiepileptic medications for seizures such as phenytoin, calcium channel blockers or cyclosporin A  Gum redness and swelling (a sign of gingivitis, also known as gum disease)  Periodontal disease (gum infection)  Trauma to the head and face (which is more common in children with seizures, developmental delays, poor muscle coordination, or abnormal protective reflexes), which can damage oral structures  Bruxism (teeth grinding, especially during sleep or in times of stress)  Malocclusion (‘bite’ misalignment, which can cause abnormal facial shape, drooling and biting/chewing difficulty)  Tooth decay and/or cavities (caries) due to excessive drooling (sialorrhea) or due to acid from reflux or vomiting Creating a Daily Preventative Care Regimen to Prevent Tooth Decay Because children with special needs may have unique health concerns, parents should speak to a dental or other medical professional (such as an occupational or speech therapist) to see how they can tailor daily preventive care regimens to their child. Some suggestions parents may receive:

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HIE HELP CENTER: MEDICAL INFORMATION Type of Oral Care Concern Possible Solution Use only a small amount of toothpaste Brushing teeth Toothpaste swallowing (a ‘smear’) or use non-fluoridated toothpaste Brushing teeth Brushing triggers gagging Brush teeth with fluoride mouthwash Use electric/battery-powered brush or extend brush handle with Brushing teeth Limited dexterity tongue depressor or widden brush handle with wrapping or use a mouth prop Ask for a referral to a pediatric dentist or Child cannot tolerate or does not a specialist with training in sedation cooperate with routine cleanings, Going to the dentist (local anesthesia, nitrous oxide sedation, restorative procedures, or minor oral oral conscious sedation or general surgery anesthesia)

Primary care physician should complete examination checklist or refer to a dental professional comfortable with Oral examination may be more treating children with special healthcare Screening for Oral Problems difficult needs. Children with special healthcare needs are considered ‘high risk’ and should be referred to a dentist by age 1 year.

Many children with special healthcare needs qualify for Medicaid, which can Children’s dental care not covered Insurance Coverage help qualify them for the Early and by insurance Periodic Screening and Diagnostic and Treatment (EPSDT) program.

In some situations, it may not be possible to brush, floss and rinse regularly throughout the day. If rinsing with water is not an option, caregivers should use a disposable applicator swab to clean the mouth, and continue scheduling regular dental appointments for cleaning, fluoride treatment and sealants. Dental Care Specialists for Kids with HIE and Special Needs You should consult with your child’s physicians to determine what best meets their individual needs. However, there are several dental care professionals that may help a child with special needs:  Dentists (who provide cleaning, fillings, minor surgery, and fluoride or sealant treatment)  Orthodontists (who treat malocclusions and improve tooth positioning)  Periodontists (who treat infections, plaque, and gingivitis)  Cosmetic dentists (who improve the aesthetic appearance of teeth)

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HIE HELP CENTER: MEDICAL INFORMATION Other professionals the child may see include dieticians (who can provide advice regarding meal plans that minimize tooth decay) and speech-language pathologists (SLPs), who can help a child control their facial muscles to improve chewing and swallowing ability. How Do I Find a Dentist Who Can Help My Child with Special Needs or HIE? Not all dentists are well-equipped to address the unique health concerns of individuals with disabilities. Dental practitioners with experience in serving individuals with disabilities can often be difficult to find, and there are often barriers to oral health care, including barriers to building accessibility, financial availability (due to the costliness of restorative work), and lack of insurance coverage. However, there are several organizations devoted specifically to dental professionals with disability-related training, and parents can check to see if there is a dentist near them that can help keep their child’s teeth healthy:  The Special Care Dentistry Association and SCDA Care Locator  Dental Lifeline Network: Dedicated to helping individuals with disabilities find dental care  Find a State Oral Health Program  Grottoes of North American: Dental Care for Kids with Special Needs (USA)  Find a Dentist: USA and Canada These medical professionals have experience in addressing special healthcare needs, which can include handling breathing difficulties, reducing aspiration risk, doing wheelchair transfers, controlling seizures and acid reflux, sedation, pharmaceutical interactions, and the control of involuntary movements, shaking and seizures – topics that do not always apply with traditional dental practice. They can also provide specific advice to caregivers regarding daily health maintenance routines. To learn more about ways to help maintain your child’s oral health, please see the following resources:  [Resource Guide] South Carolina Department of Health and Environmental Control: Oral Health for Families with Special Health Care Needs  Oral Health Care For Children With Special Health Care Needs: A Guide for Family Members/Caregivers and Dental Providers  [Powerpoint download] Protecting All Children’s Teeth: American Academy of Pediatrics  A Caregiver’s Guide to Good Oral Health for Persons with Special Needs  Practical Oral Care for People With Cerebral Palsy  Colgate Oral Care Center: Dental Health Care For Children With Special Needs Paying for Special Needs Dental Care Some insurance plans provide dental health insurance coverage, while other do not. Most dental clinics have payment plans and dental care packages that can help families save money, but it is best to inquire about pricing prior to receiving services.

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HIE HELP CENTER: MEDICAL INFORMATION Orthopedic Health Orthopedic health revolves around the function of the musculoskeletal system, including the bones, joints, tendons, ligaments, muscles and supportive tissues. Orthopedic health concerns for children with hypoxic-ischemic encephalopathy (HIE) generally revolve around cerebral palsy, as HIE can cause cerebral palsy. This disability can necessitate intensive physical therapy, orthotics, early intervention, and other preventative and health maintenance services in order to maintain range of motion, walking ability, and physical capabilities. While more than 40% of individuals with cerebral palsy have some form of cognitive disability, orthopedics focuses on an individual’s’ physical ability to move, walk, and carry out the tasks of daily living on their own. Orthopedic impairments can reduce a person’s independence, prompting an emphasis on early intervention to reduce complications such as contractures, pain, foot drop and tissue shortening. Orthopedic Health Concerns Related to HIE The orthopedic concerns of HIE overlap with the orthopedic health concerns of cerebral palsy. With cerebral palsy, a person’s balance, fine and gross motor skills, muscle tone, coordination and muscle control, oral-motor function, posture and reflexes are all impacted. This brain injury can cause nerves to erroneously fire signals to the muscles, causing tightening and other complications. In severe cases, this can result in bone and joint deformities, contractures, severe pain, hip dislocations, and spinal curvatures. There are numerous types of cerebral palsy, which can manifest as too much muscle tone, not enough muscle tone, uncontrolled motions, writhing or tremors. Treating Orthopedic Health Concerns A child’s orthopedic health can be safeguarded in multiple ways, each of which is unique to the child’s medical history and circumstances. Physical and occupational therapy are designed to help children gain or improve their physical function, which certain medications (such as baclofen or Botox) can help control spasticity. Assistive devices can help children walk and complete the Activities of Daily Living (ADLs), and orthotic devices can help keep the joints in alignment, support the trunk, feet, knees and joints, and help prevent maladaptive habits such as toe-walking. Children can be taken to their primary care physician or pediatrician for evaluation when very young, in order to maximize the benefits of therapy and formalized Early Intervention (EI) programs, along known as Zero Through Three Programs in some states. In some cases, primary care providers will provide referrals to orthopedic surgeons who specialize in helping children with musculoskeletal conditions. Learn More About Orthopedic Health and HIE  Orthopedic Concerns in Cerebral Palsy  Common Orthopedic Concerns in CP

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HIE HELP CENTER: MEDICAL INFORMATION Pain Hypoxic-ischemic encephalopathy (HIE) is, at its core, a brain injury. This injury can impact a child cognitively, emotionally, intellectually, and physically. HIE can impact a child physically by damaging neural pathways, including the nerves that send signals to the muscles, in a condition called cerebral palsy. Cerebral palsy can often cause pain, as certain types of cerebral palsy can cause muscle spasms, contractures and limited range of motion, which can make movement difficult and painful. Individuals with hypoxic-ischemic encephalopathy may also need certain surgical procedures or interventions in some cases, and pain can sometimes be a factor after the completion of such interventions. In other instances, pain can be the result of secondary conditions, such as immobility or the inability to reposition the body after a set period of time. Pain commonly results from spasticity, scoliosis, hip dislocation/malpositioning, nerve injury, UTIs, and respiratory, esophageal or intestinal issues. Other considerations include nerve impingements, skin breakdowns, and arthritis due to bone displacement. Specialized Cases: How to Determine When a Nonverbal Child Is In Pain One aspect of childcare that can be difficult is determining whether a nonverbal child is in pain, because of two factors: nonverbal children may not be able to identify or describe what they are feeling, and parents may have limited training in interpreting signs of pain. Individuals who can communicate should be encouraged to communicate their pain levels, but for those who cannot report at all, there are behavioral pain assessment tools available, such as the American Society for Pain Management Nursing (ASPMN)’s “Pain Assessment in the Nonverbal Patient: Position Statement with Clinical Practice Recommendations.” Other behavioral pain assessment tools include:  FLACC Assessment Tool  CHEOPS in Young Children  CHIPPS, NIPS and CRIES Scales for Neonates  RIPS for Infants  PIPPR-R for Neonates  COMFORT Behavior Scale Treating and Managing Pain from Conditions Related to HIE Parents who are worried about their child’s physical pain should consult with a medical professional regarding methods of minimizing pain symptoms and controlling underlying causes of pain. There are several ways that physicians can recommend parents assist in relieving their child’s pain, including therapy, medication, or surgical procedures. The kind of treatment a child will receive will depend on their condition’s severity and other factors. Pain management is necessary to improve the child’s quality of life, as pain can impact all aspects of a child’s daily living, including social, physical and cognitive functioning. The child’s primary care physician will likely be the first point of contact for the family. Depending on the root cause of the pain, the physician may refer the child to a specialist, or continue treating the child themselves. They may interface with occupation and/or physical therapists, orthopedists, neurosurgeons, rheumatologists, neurologists, physiatrists, chiropractors or massage therapists to develop a comprehensive pain management approach. Parents may also consider taking their child to a specialized pain management specialist or clinic. In many cases, physical and/or occupational therapy can help reduce or prevent painful HIE-related physical issues before they worsen, as these therapies can increase strength, range of motion, stretching, endurance, and physical stability. All of these factors can reduce the risk of painful contractures and musculoskeletal deformities. Medications are the next line of defense against pain. Over-the-counter medications (such as Tylenol, Motrin or Advil) may be common, though physicians can sometimes provide prescription pain medications that prevent pain receptors in the brain from firing. The type of medication prescribed largely depends on the pain’s cause. Children with spasticity-related pain may be prescribed anticholinergics or antispastic medication (such as Baclofen, administered as ITB therapy), which children with severe cerebral palsy may be given opioids such as oxycodone. Children with seizures may be given anticonvulsants to reduce seizure activity and prevent pain from seizure-

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HIE HELP CENTER: MEDICAL INFORMATION related injuries. Other medications for pain-related issues include anti-inflammatories, stool softeners (for constipation-related gastric pain), and antidepressants (to relieve emotional/psychological pain in the form of depression and/or anxiety). All medications must be monitored by a medical professional, including over-the- counter medications, to ensure the child does not have adverse reactions or drug interactions. Other solutions (which may be appropriate for some people but not others), include:  Pulsed radiofrequency  Neuromodulation  Botox injections  Spinal cord stimulators to block pain receptors (experimental)  Surgery (including orthopedic surgery to making walking less painful, selective dorsal rhizotomy to reduce spastic pain, and spinal surgery to provide trunk stability)

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HIE HELP CENTER: MEDICAL INFORMATION Respiratory Health Children with hypoxic-ischemic encephalopathy (HIE) may require breathing aids due to a number of factors, including muscle weakness and prematurity-related conditions. In cases of moderate to severe HIE, multiple organ systems may be impacted by the oxygen deprivation at birth, and this can sometimes impact the respiratory system as well. Throughout this page, we’ll discuss considerations related to respiratory health issues in children with HIE and other special needs. Causes of Respiratory Health Issues in Children with HIE and Special Needs Children with hypoxic-ischemic encephalopathy may have respiratory conditions due to a wide variety of factors:  Lower quantities of exercise due to physical limitations  Greater risk of swallowing or feeding limitations (dysphagia), which can promote choking and aspiration  Chest wall deformity due to spinal curvature causing labored breathing, restricted lung function, and respiratory difficulty  Prematurity, which increases the risk to babies for bronchopulmonary dysplasia and respiratory distress syndrome (RDS)  More difficulty in obtaining sufficient nutrition, which can cause weakness and/or atrophy, which in turn impacts lung function  Difficulty clearing airways due to muscular dysfunction Diagnosis, Treatment and Therapy for Respiratory Health Conditions Children with chronic chest infections, pneumonia, aspiration or coughing should be closely monitored and potentially referred to a certified respiratory therapist (CRT) or other professional (such as a gastroenterologist, dietician or speech-language pathologist (SLP). These professionals can screen for risk factors that contribute to serious health conditions (such as aspiration, pneumonia, and respiratory failure). They will measure a patient’s lung capacity, blood oxygen levels, blood pH, and CO2 levels, and make note of any physical issues that make breathing harder (such as scoliosis, kyphosis, pectus excavatum, pectus carinatum, or barrel chest). They can then recommend respiratory interventions that will help reduce health risks and improve respiratory function. These interventions can include respiratory exercises, vest therapy, inhalers/nebulizers, modified diets, speech therapy, oxygen treatment, prophylactic antibiotics or short-term intubation. They can also help the child develop ways to cough and clear airways more efficiently, exercise their lungs to improve breathing efficiency, and develop ways to position their bodies to improve ventilation. They may also initiate therapies such as hydrotherapy, high frequency chest wall oscillations, percussion and chest wall vibrations and suctioning to help remove mucus and build up, and remedy obstructions. Respiratory therapy is not something that a child would receive on a regular schedule the same way that they would receive physical or occupational therapy. Respiratory therapists provide general advice for daily living and can monitor children with feeding tubes, as these individuals are more likely to develop a respiratory condition that may need addressing. Respiratory therapy can address:  Breathlessness, chest rattling, postural drainage, secretions that obstruct airways, wheezing and strained breathing  Dysphagia and related issues (GERD, aspiration, gagging, trouble chewing/swallowing)  Drooling  Pneumonia, chest infections and recurring cough  Bronchospasms  Aspiration of food and/or liquids  Unexplained fatigue

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HIE HELP CENTER: MEDICAL INFORMATION  Asthma, bronchitis and bronchiolitis  Prevention of serious complications, including failure to thrive, aspiration, heart and lung trouble, malnourishment, and respiratory failure. Learn More About Respiratory Health and HIE  Aetna – Chest Physiotherapy  Airway Clearance and Lung Care  Respiratory Therapy Magazine  American Association for Respiratory Care  Standing for Easier Breathing  Chest Physiotherapy  Link Between CP and Respiratory Disease

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HIE HELP CENTER: MEDICAL INFORMATION Skin Health Children with hypoxic-ischemic encephalopathy (HIE) can often be at an increased risk of skin breakdowns, especially when they are non-ambulatory or have paraplegia. These children are more likely to use wheelchairs and other assistive devices. It is important to check that these devices do not cause pressure sores, irritation, or skin breakdowns, because these can become painful and uncomfortable for the child. When skin breaks down, it makes the area more susceptible to infections and inflammation. In many cases, if a child’s skin is broken or irritated, there are simple solutions to reducing redness and irritation, such as keeping the area clean and dry, repositioning the body away from the sore area, and ensuring that adaptive equipment is properly fitted. In other cases, over-the-counter solutions may be available. Prescription- strength treatments are available for particularly severe or persistent skin conditions; obtaining these treatments may require a visit to the child’s primary care provider to secure a referral to a dermatologist. Skin conditions to watch out for include:  Infections (yeast, bacterial, viral, parasitic, fungal, etc.)  Skin ulcers or pressure sores  Skin redness of irritation  Boils  Folliculitis  Acne  Psoriasis What Causes Skin Conditions in Children with HIE and Special Needs? There are numerous reasons why a child with HIE or another disability may develop skin irritation, including:  Skin breakdowns, pressure and rubbing from improperly-fitted adaptive devices or rigorous physical therapy  Allergic reactions to medications  Voluntary or involuntary self-injury, which can be co-occurrent with intellectual/developmental disability (I/DD)  Oral motor dysfunction causing excessive drooling: excessive salivation can irritate skin  Hearing/vision impairments, which increase the risk of injury due to environmental hazards There are other factors that can predispose a child to having skin conditions, including genetic predisposition, nutritional deficiencies, exposure to certain pathogens, poor hygiene, and prolonged irritation. Preventing or Minimizing Skin Breakdown General skin health can be supported by keeping the skin dry, clean and moisturized, just as with any individual. This is especially critical for individuals using adaptive equipment that can cause added friction. Other tips for promoting skin health include:  Washing hands often  Keeping hair clean and moisturized to prevent itching  Maintaining orthotics properly for fit and function  Washing and changing clothing often  Making sure clothing and shoes fit properly (not too tight or too loose)  Keeping nails trimmed to prevent scratching

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HIE HELP CENTER: MEDICAL INFORMATION  Keeping existing open wounds clean  Checking for allergic reactions to deodorant, moisturizer/lotion, laundry products, soaps and perfumes, medications, etc.  Changing bedding regularly  Changing incontinence products (diapers, incontinence pads, etc.) as needed  Checking bedding, clothing and skin for bed bugs, lice and dust mites, which can irritate skin  Positioning the child for increased circulation  Checking for environmental irritants:  Check bedding for bedbugs and dust mites  Check hair for lice  Check skin (especially underarms, buttocks, groin and limbs) for signs of redness/irritation Learn More About Skin Health  Managing Skin Breakdown  University of Washington Rehabilitation Medicine: Skin Care & Pressure Sores  Nutrition, Skin Integrity, and Pressure Ulcer Healing in Chronically Ill Children: An Overview  Bedsore Prevention  Preventing Pressure Sores

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HIE HELP CENTER: MEDICAL INFORMATION Speech Delays and Language Disorders Brain injury due to hypoxic-ischemic encephalopathy (HIE) is rarely confined to a simple area of the brain, though it is certainly possible in certain mild cases. Because oxygen deprivation affects the connections in the brain on a global level, it is often possible that children with HIE will have multiple interrelated delays in development. Children with HIE can sometimes have delays in developing speech and language. These delays can sometimes be mitigated, while in other, more severe circumstances, children may remain non-verbal and require the use of alternative or augmentative communication (AAC) technologies to assist them in communicating their thoughts, needs, and desires. Developing a method for communicating helps these children interact with others, develop relationships, learn, work and socialize. Speech and language are clearly highly inter-related, but they are not interchangeable. Speech refers to the physical act of expressing words and sounds, while language refers to communicating in a systematic and meaningful way. Because language is related to intelligence, disorders in language acquisition and expression are generally considered more serious than speech disorders. There are two broad categories of language disorders:  Expressive language disorder: Relates to the ability to communicate meaning to others  Receptive language disorder: Relates to the ability to understand someone else’s message Language disorders can be expressive, receptive, or mixed receptive-expressive. The signs and symptoms of these disorders can vary depending on the type of disorder a child has: Symptoms of Expressive Language Disorders  Difficulty constructing grammatically correct or complex sentences  Difficulty using standard grammar conventions  Difficulty finding words  Overuse of placeholder holders like ‘um’ or ‘uh’  Smaller vocabulary than is age-appropriate  Repetition of certain phrases  Echoing of questions in responses Symptoms of Receptive Language Disorders  Difficulty understanding speech  Difficulty following spoken direction  Difficulty organizing thoughts Speech Delays and Language Disorders Associated with HIE There are several conditions that are common in certain children with HIE. These conditions can be improved via intensive speech-language pathology treatment, and can include:  Aphasia (A disorder in understanding written or spoken language)  Dyspraxia (Difficulty in physically pronouncing words) and Articulation disorders  Dysprosody (Disorders in speech timing/cadence)  Dysarthria (Abnormal facial muscle tone)  Fluency Disorders (Stuttering)  Dysphagia (Trouble swallowing or breathing, resulting in increased choking risk)  Resonance Disorders (Abnormalities in the vocal tract) When Should a Child Get Speech-Language Therapy?

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HIE HELP CENTER: MEDICAL INFORMATION If a child is not acquiring speech or language at the usual expected rate, it is recommended that parents work with the child’s pediatrician to have the child evaluated as soon as possible (during infancy or very early childhood). Sometimes, children may require some extra assistance or therapy to help them acquire language, or may need extra intervention in the case of concurrent developmental issues. It is critical to make these diagnoses early so the child may be enrolled in an Early Intervention (Zero to Three, Head Start, Early On) program to mitigate some of the difficulties that may be associated with poor communication. These can include future academic struggles, emotional or behavioral problems associated with poor social interaction, and difficulties in occupational or psychological development. These programs are most effective when a child is very young, as beginning these programs at an older age will mean that the child must also take the extra step of unlearning old frameworks and developing new ones. It is also worth noting that sometimes speech and/or language delays can result from hearing-related deficits, as hearing problems may make it appear that a child has a language or speech issue when they only require a hearing aid or medical intervention to get their speech and/or language development on track. What Causes Speech Delays and Language Disorders? Delays in speech and/or language can be due to several root causes. In some children, HIE-related speech/language disorders are due to damage to the part of the brain responsible for processing language, which can require a different approach from speech/language disorders arising from damage to parts of the brain that control the muscles of the throat, mouth and jaw (a common feature of cerebral palsy stemming from HIE). A licensed speech-language therapist will be able to determine the best way to assist a child with a speech/language disorder, working with the child’s caregiver team of physicians, physical therapists, psychologists and social workers to help the child overcome cognitive or developmental difficulties that can make communication difficult. Therapy for Speech Delays and Language Disorders Usually, when a child is evaluated and found to have a speech or language disorder, they can be referred to a speech and language pathologist – an American Speech–Language–Hearing Association (ASHA)-certified professional (with a minimum of a Master’s degree in speech and language pathology) that helps individuals with difficulty communicating adapt and compensate to maximize their communication abilities. These professionals have training in pathology, audiology, anatomy and physiology, linguistics and phonetics, human development, and American Sign Language (ASL), among other courses, which allows them to effectively assist children with specialized developmental needs. Parents seeking a speech-language pathologist for their children can request assistance at a variety of locations, as speech-language pathologists are often available at clinics, community health centers, hospitals, rehabilitation centers, public schools, nursing homes and state health departments. The location a child will receive care will depend on several factors, including:  The child’s age  The child’s medical history  Availability of services in their community  Whether insurance will cover care Under ideal circumstances, children receive one-on-one services with additional exercises that they practice at home with parents outside of therapy sessions. In other circumstances, speech-language pathology services are provided in a small group setting as well. Sometimes, these services can be provided in a home setting without having to travel. What Happens During Speech Language Therapy? First and foremost, it is important to remember that each child is different, so the exercises that a speech-language pathologist may recommend may differ very substantially child-to-child. For children who are verbal, speech- language therapy can help children:

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HIE HELP CENTER: MEDICAL INFORMATION  Pronounce words more clearly  Listen to and understand conversation  Use words properly  Understand grammar  Have a bigger vocabulary  Comprehend conversation  Speak in complex sentences  Speak conversationally  Improve memory and recall  Understand the differences and similarities between written text and spoken words A speech-language pathologist will not only help the child physically form words needed for communication but will also develop a training regimen designed to help the child’s brain interpret and understand words, sounds, numbers and gestures. Some children with HIE may be non-verbal. When working with children who are nonverbal, speech-language pathologists work with the child to create alternate communication strategies. These can include strategies such as sign language or the use of cue cards or picture boards, or they can be more technological in nature. Technological aids such as Alternate and Augmentative Communication (AAC) systems are often used; speech- language pathologists can help evaluate which systems would be most suited to a particular child’s abilities, preferences and cognitive abilities. Additional Benefits of Speech-Language Therapy Speech-language therapy revolves around the use of the muscles involved in the functioning of the throat, mouth and jaw. While communication is the primary goal of speech-language therapy, it often has the added benefit of helping children drink, eat, breathe and chew more effectively as well. Often, children with HIE who have cerebral palsy will have a degree of impairment in eating and drinking (dysphagia); speech-language pathologists can assist these children in improving complex muscle coordination. This decreases the risk that a child will aspirate their food (which can lead to choking and/or pneumonia), have abnormal breathing, increased coughing, dehydration or malnutrition. Why is Speech-Language Therapy Important? Communication is the underpinning of social interaction, which makes early intervention critical. Developing a communication system helps to ensure that the child will have frameworks in place for inclusion and socialization, allowing them to more fully engage with their surroundings, participate in activities in school or at home, learn new skills, and develop new hobbies or interests. This therapy also increases the likelihood that other interventions will be effective. Because other types of therapies are dependent on the child’s ability to comprehend and follow instruction, speech-language therapy is a crucial part of a child’s multidisciplinary therapy regimen. Speech-language therapy supports learning, education, literacy, and community integration. There are no risks to speech-language therapy. Indeed, delaying speech-language pathology can increase the risk of isolation or depression, and can mean that other areas of development (social, educational, psychological, etc.) may also slow without continued communication-related support. Additional Speech and Language-Related Resources  University of Michigan: Speech and Language Delay and Disorder Quick Reference  What Does Normal Speech Acquisition Look Like?  Speech Therapy: How it Works, What to Expect

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HIE HELP CENTER: MEDICAL INFORMATION  Apraxia of Speech: Frequently Asked Questions  Encyclopedia of Children’s Health: Language Delays  A Parent’s Guide to Speech and Language Disorders  What to Expect from Speech-Language Therapy  Communication Development: A Parent’s Guide

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HIE HELP CENTER: MEDICAL INFORMATION Orthotics Requirements Individuals diagnosed with hypoxic-ischemic encephalopathy (HIE) are often at higher risk for motor disorders and limitations in coordinating movement. Sometimes they may need orthotic devices, which help brace, stabilize and support a person’s limbs, spine or trunk in order to help them maximize their mobility and independence and help with bodily stability and alignment. Orthotics can take many forms, ranging from limb braces to sets of progressive shoe inserts designed to help children walk. Some children will need orthotics for the duration of intensive therapy only, while in other circumstances they will need them throughout their lives. These braces are sometimes prefabricated, but in certain situations can be modified or customized to address a child’s specific health needs. What Are Orthotics Used For? Some of the conditions orthotics are designed to treat include:  Dislocation of the joints  Spasticity  Bone and spinal deformations  Scoliosis  Drop-foot  Inversion or eversion  Instability in gait  Low- or high-tone pronation Orthotics can also be preventative in nature. When properly applied, they can help prevent flexible deformities, stop progressive deformities from worsening, decrease the effects of spasticity on the body, and help children strengthen muscles and joints to help them ambulate more stably or successfully. Types of Orthotics Orthotics are divided up by the type of body part they support:  Lower limb orthoses: Support the lower limbs, including the legs, knees and ankles  Foot orthoses: Shoe inserts made to support the arches and and foot joints; also include orthopedic shoes, shoe modifications, arch supports and heel modifications  Ankle-foot orthoses (AFOs/foot drop braces): External braces made to support the ankle and foot, correct deformities, correct foot drop, or immobilize fractured limbs  Knee-ankle-foot orthoses (KAFOs): Orthoses designed to limit or assist the plane of motion of the sagittal, coronal or axial planes of motion of the lower limbs; often use to help individuals who have limited range of motion in the legs  Hip-knee-ankle-foot braces (HKAFOs): Help position a person upright while centering knees  Knee orthoses (KOs): Knee braces designed to support or align the knee, prevent extension instability, or relieve pressure on the joint due to inflammatory conditions  Trunk-Hip-Knee-Ankle-Foot Orthotics (THKAFOs): HKAFOs with spinal orthoses to help with trunk control and spinal alignment; often used by individuals with paraplegia  Spinal orthoses (back braces): Used to treat scoliosis and abnormal spinal curvatures, as well as spinal fractures. Sometimes used to provide support for children with limited trunk control.  Upper-limb orthoses: Orthotic devices applied to the arms to help restore or improve function  Clavicular and shoulder orthoses

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HIE HELP CENTER: MEDICAL INFORMATION  Arm orthoses  Elbow orthoses  Forearm-wrist orthoses  Forearm-wrist-thumb orthoses  Forearm-wrist-hand orthoses  Hand orthoses What Happens When a Child Receives an Orthotic Device? During an initial appointment, the child is evaluated by a pediatric orthopedic surgeon for possible issues that orthotic devices can correct. Once they identify such issues, they can refer the child to a licensed orthotist who specializes in orthotic devices. These professionals are certified by the National Commission on Orthotic and Prosthetic Education (NCOPE) or the American Board for Certification in Orthotics, Prosthetics & Pedorthics. The orthotist develops a treatment plan in conjunction with the child’s primary care provider and orthopedic surgeon, and they can make recommendations for certain types of devices. Some devices might be off-the-shelf, while some might need some customization. For custom devices, the orthotist may make a cast or take measurements, and the amount of time it takes to receive the device can vary. Once the device is made or provided, the orthotist will request the child come back for a fitting, where the device is adjusted and the child and family are shown how to properly use, adjust and remove the device. After fitting, the child will be given a schedule for wearing the device that includes break-in time, followed by a follow-up appointment where the orthotist will determine if the orthotic device is effective or needs further adjustment. It is important to follow all follow-up guidelines because a child’s needs and abilities can change over time, necessitating further evaluation, modification or replacement of devices. It is also important to check in with the orthotist about ways to help your child become comfortable with an orthotic device, especially since they can be uncomfortable at first (especially if the orthotic device must be worn continuously). Ways to improve your child’s experience include:  Making sure the device is not too tight or too loose  Making sure the skin under the device is not red, raw or broken  Making sure there is a barrier (such as socks or sleeves) between skin and device when the orthotist recommends it  Making sure the child adheres to the wear schedule  Making sure the device is well-maintained, clean and in good repair  Making sure barrier clothing is dry to prevent irritation and discomfort  Making sure the child understands why they are wearing a device and how it will help them Learn More About Orthotics and HIE  Orthotic Management of Children with Cerebral Palsy  When are orthoses appropriate?  The interplay of early intervention and orthotics  Orthotics for Hypotonia

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HIE HELP CENTER: MEDICAL INFORMATION Preventing HIE It is very important to remember that hypoxic-ischemic encephalopathy is a diagnosis stemming from oxygen deprivation to the fetus. Preventing the oxygen deprivation that causes the HIE is key. To prevent oxygen deprivation, pregnancies must be properly monitored and cared for throughout gestation, labor, delivery and the neonatal period. In many cases, the underlying risk factors and causes can be mitigated and controlled. Indeed, one of the common threads that tie together many preventable cases of HIE is failure on the part of medical professionals in carrying out the standards of care. These can include a failure to deliver a baby in time, a failure in recognizing that the baby’s heart rate is abnormal on the fetal monitoring strips, or a failure to resuscitate or intubate the baby in time. Sometimes this can also be due to a failure of basic communication between the various care providers – results of various tests or procedures can get lost, especially during shift changes or between departments. Because the prevention of oxygen deprivation is so heavily dependent on medical care providers, one of the best ways parents can try to ensure their baby’s safety is choosing a good medical care provider and getting accurate information regarding birth and delivery procedures, including what medical staff do in case of complications or emergencies. Betamethasone While cerclage and progesterone are given to women at risk of preterm birth who have not yet gone into labor, there are methods to help babies who are about to be born preterm (especially those born before 34 weeks). Preterm babies aren’t fully developed and are often very fragile, which means that they may need help in maturing. For example, it is very common for preterm babies to having trouble breathing because their lungs can’t produce enough surfactant (a molecule that helps keep the lungs flexible and pliant). This means they may need ventilation or breathing assistance when they are born. To help the baby’s lungs mature more quickly, medical staff can give the mother betamethasone while the baby is still in utero in two doses 24 hours apart. This decreases the risk that the baby will have trouble breathing or need breathing help. That said, it is very common for babies to still need some extra surfactant to help them breathe nonetheless. Reducing the need for breathing assistance and developing the baby’s lungs once a preterm birth is imminent helps reduce the risk of hypoxic-ischemic encephalopathy and other associated conditions like Periventricular Leukomalacia (PVL) and hydrocephalus by cutting down on the risk that the baby’s tissues will not get an adequate oxygen supply after birth. Delivery Methods: C-Section Delivery A C-section delivery is a surgical procedure for delivering a baby. In some cases, C-sections are planned beforehand (especially if a mother has had a prior C-section, preeclampsia, placental insufficiency, or pelvic issues that would make it dangerous to deliver vaginally). In other cases, C-sections are necessary in emergency situations where the health of the mother or baby are threatened and delivery needs to happen immediately. Essentially, emergency C- sections are procedures done where immediate delivery is the only way to access the baby to provide medical care safely. Potential Indications for a C-Section There are many situations where C-sections are safer for the baby than a vaginal birth. These include the following. Potential Indications for C-Section in the Mother  Placental abruption, insufficiency, placenta previa or placenta accreta  Brain hemorrhage or aneurysm  Vasa previa  Uterine rupture  Stalled labor  Prior C-sections  Preeclampsia

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HIE HELP CENTER: MEDICAL INFORMATION  Diabetes  Active genital herpes or other maternal infections  Twins or triplets Potential Indications for C-Section in the Baby  Signs of fetal distress  Umbilical cord complications:  Umbilical cord wrapped around the neck (nuchal cord)  True knot in the cord  Short umbilical cord  Umbilical cord compression  Umbilical cord prolapse  Breech or face-presenting position  Birth defect  Shoulder dystocia (the baby’s shoulder is stuck on the pelvic bone)  Prematurity or extreme prematurity In these situations, time is of the essence in delivering the baby, because prolonging labor can increase the risk of birth injury to the baby and health issues for the mother. There are specific time frames in which medical staff must perform a C-section, usually in less than 30 minutes. For some health conditions (cord prolapse, uterine rupture and fetal heart rate under 60 beat per minute), however, the time limit is far shorter (10-20 minutes or less). Medical staff should be monitoring fetal heart rate continuously when a mother is admitted to the labor and delivery unit. If the baby’s heart rate drops or begins to show signs of abnormal heart rate tracings, it means that the baby needs to be delivered immediately. If vaginal delivery can’t be done quickly enough, a C-section is needed to immediately deliver the baby to provide it with needed medical care (like resuscitation or oxygen). Delayed C-Sections and the Risk of HIE One of the things to look out for in preventing HIE is the risk of waiting too long to have a C-section. Sometimes medical staff can be reluctant to perform a C-section if a baby is showing signs of fetal distress, and they will attempt to speed up labor and vaginal delivery using delivery drugs (like Pitocin or Cytotec) or delivery instruments like forceps or vacuum extractors. The difficulty with delivery drugs is that mothers can react unpredictably to them – a dose which will have no effect in one mother may have a massive effect on another, causing the uterus to contract strongly and decrease blood flow even further to an already- distressed baby. Delivery instruments require specific training to use properly, as improper use can cause brain bleeding and trauma. Oftentimes, C- sections in emergency situations are safer than attempting to speed up a vaginal delivery. One of the ways parents can mitigate the risk of having a C-section unsafely delayed is asking hospitals and doctors ahead of time about their C-section policies. Fetal Heart Monitoring During Labor and Delivery When a mother is admitted into the labor and delivery unit, doctors must monitor the baby’s heart rate continuously. This is a very important step because fetal heart rate indicates how well a baby is handling labor. Medical staff are trained in reading the printouts (called EFM tracings) that come from the electronic fetal monitoring unit, and should be able to identify if the baby’s heart rate is abnormal. If these tracings are abnormal, it is likely that the baby is experiencing fetal distress (which is nearly always caused by oxygen deprivation). EFM readings look like the following:

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HIE HELP CENTER: MEDICAL INFORMATION In most cases, babies can tolerate labor well, but there are some conditions that can make labor dangerous for the baby. If a baby is extremely premature, extremely small, or too large to fit through the birth canal, it is likely they are at higher risk of birth injury. This can mean it is safer for them to be delivered via C-section. Other conditions where it is likely that a baby will show signs of abnormal fetal heart rate can be found here. Magnesium Sulfate Magnesium sulfate (along with betamethasone) should be administered to a baby about to be born preterm (24- 32 weeks) in order to protect against cerebral palsy. While the baby is still in utero, the mother receives magnesium sulfate, which helps increase blood flow to the baby’s brain, decrease the levels of inflammatory molecules in the brain that can cause damage, reduces excitotoxicity, stabilizes cell membranes and prevents large blood pressure changes that can cause brain damage. Medical personnel administer magnesium sulfate to women about to give birth to a premature baby within 24 hours whether or not their membranes have ruptured preterm. Generally, they begin with a 4g dose via IV and provide 1g of a maintenance dose every hour for 24 hours or until the baby is born (whichever is sooner). If it is likely labor will take more than 24 hours, doctors should hold off on beginning magnesium sulfate therapy until closer to the time of delivery. However, if the baby is not doing well and needs to be delivered immediately, doctors should not delay delivery to finish providing magnesium sulfate. Magnesium sulfate isn’t for every pregnancy, however. Women with certain neuromuscular diseases or heart or kidney issues should be very carefully evaluated to prevent magnesium toxicity and should be very carefully monitored. If doctors are using a tocolytic to prevent preterm labor in conjunction with magnesium sulfate, the kind of tocolytic they should use is indomethacin. Prenatal Testing Whether or not a mother is high-risk or not, there are certain tests that doctors make sure mothers get to ensure their babies are developing properly. The kind of tests they administer can vary depending on numerous factors (such as the mother’s medical history, pre-existing health conditions, among others), These tests (commonly called prenatal tests) are used to identify babies who are at risk of having a brain injury like hypoxic-ischemic encephalopathy and to take steps to reduce their risk of adverse health outcomes (a general term for health conditions that negatively impact a baby’s development). Mothers who are high-risk and/or have the following conditions are monitored and/or tested particularly frequently to decrease risk:  Mothers with high blood pressure and preeclampsia  Diabetes or gestational diabetes  Other medical conditions that impact pregnancy  The baby is small for their gestational age due to fetal growth restriction  The baby has a decrease in movement  The mother has had an external cephalic version to turn the baby into the correct position for birth  The baby has received a 3rd trimester amniocentesis to check lung maturity or infection status  The mother previously lost a baby during the second ½ of pregnancy  The baby has been diagnosed with abnormalities or birth defects One of the best ways to prevent HIE is to closely monitor pregnancies, especially those with one or more risk factors for HIE. Proper prenatal care and monitoring is crucial for managing and identifying conditions that may affect the baby’s health and development. What Do Prenatal Tests Screen For? Different prenatal tests look for different things. Because the list of tests that a mother goes through during her prenatal care is extensive, this page focuses on the tests that directly relate to hypoxic-ischemic encephalopathy

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HIE HELP CENTER: MEDICAL INFORMATION (HIE). Other tests (such as those that test for UTIs/BV or Group B Strep) are equally as important from a birth injury standpoint but will be discussed separately. There are several factors that prenatal testing surrounding the oxygenation of the baby can focus on. These include:  Fetal heart rate  Blood flow  Fetal movement  Amniotic fluid levels If a baby is being oxygen deprived (hypoxic), their heart rate will slow down, they will move less to conserve energy. These are warning signs of a condition called fetal distress, where the baby is beginning to suffer damage from oxygen deprivation. Additionally, if amniotic fluid levels decrease or are low, there is a risk that the umbilical cord can be compressed and cause fetal distress. During pregnancy, birth and delivery, medical professionals must monitor the baby’s health in order to address such signs as soon as they occur, because prolonged inaction can cause permanent damage. Prenatal Testing: Non-Stress Tests One of the tests administered during pregnancy is called a non-stress test (NST). It is commonly provided between weeks 38-42 (or much earlier depending on risk factors), and is provided to mothers are high-risk or have a post- term pregnancy (a pregnancy that lasts longer than 40 weeks). Just as in adults (whose heart rates increase during exercise), a baby’s heart rate should increase when they move around or kick. An NST tests how well their heart rate responds to movement, If the baby isn’t getting enough oxygen, their heart rates don’t increase when they move around, producing a non-reactive test. If a baby is getting enough oxygen, their heart rate increases, pointing to a reactive test. This reaction (acceleration) shows up as an increase of at least 15 beats per minute for at least 15 seconds. This is an extremely important measure because these accelerations can indicate how well a baby will go through labor. Prenatal Testing: Contraction Stress Tests (CSTs) After 34 weeks’ gestation, this test determines whether a baby will stay healthy during labor and delivery. During labor and delivery, the uterus contracts, reducing oxygen availability for the duration of the contraction. In between contractions, oxygen levels increase back to normal. Most babies are able to tolerate this well, but some are not. CSTs determine if a baby can safely go through the labor process. During this test, a mother lies on her left side while a fetal heart rate monitor and a device that records contractions are placed in specific positions on her abdomen. A machine prints out the results of these monitoring tests, allowing a physician to interpret the results and provide feedback to the mother about her baby’s ability to tolerate labor. If a mother doesn’t have contractions for the first 15 minutes of the tests, staff may give Pitocin to stimulate them. CSTs are rarer now, as they are riskier and more expensive than NSTs. Much of the risk steps from the use of Pitocin, as it is very hard to predict how a mother’s uterus will react to Pitocin. There are also some conditions where Pitocin shouldn’t be used (such as if a mother has a low-lying placenta) because it can cause hemorrhaging and other adverse health outcomes. Prenatal Testing: Amniotic Fluid Volume (AFV) Tests Amniotic fluid ( the fluid inside the uterus that cushions and protects the baby) must be at a precise level to effectively protect the baby and ensure that it develops properly. Too much amniotic fluid or too little amniotic fluid can cause issues with the flow of nutrients and oxygen to the baby through the umbilical cord, as well as other health conditions like preterm birth, premature rupture of the membranes, placental abruption and hypoxic- ischemic encephalopathy. To test how much amniotic fluid there is, medical professionals use ultrasound to get a measurement called an amniotic fluid index (AFI) by measuring amniotic fluid depth in 4 different sections of the amniotic sac. Results are generally expected as follows:

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HIE HELP CENTER: MEDICAL INFORMATION  AFI 9-18 cm: Normal  AFI 5-8: borderline  AFI 5 or less: abnormal  Sudden decrease in AFI at any range abnormal A healthy AFI at 20-35 weeks gestation is ~14cm. At weeks 34-36, amniotic fluid volume begins dropping in anticipation of birth. Generally, amniotic fluid volume increases to about 1L total by 34-36 weeks, and then decrease as much as 25% a week, up to 150mL/week between weeks 38-43. AFI tests can indicate the presence of oligohydramnios (too little amniotic fluid) or polyhydramnios (too much amniotic fluid). Polyhydramnios is defined as having more that 2L of amniotic fluid, more than 8cm maximum pool, or an AFI of more than 25cm. In either case, the baby may need to be delivered early, though that is dependent on a host of factors, including lung maturity, the presence of fetal distress, and other causes that medical professionals evaluate. A different amniotic fluid volume test is called the maximum pool, where medical professionals measure the single deepest vertical pocket of amniotic fluid using ultrasound. This is done as part of the biophysical profile (BPP). Prenatal Testing: Biophysical Profiles (BPP) This approximately 30-minute test uses ultrasound to evaluate the baby. The test takes into account the results of the non-stress test (NST), amniotic fluid volume (AFV) tests, as well as the presence of absence of fetal breathing movements, gross body movements, and the presence or absence of reflex and extension movements. The test uses a point system to evaluate if a baby has been having acute or chronic hypoxia. The BPP can also be modified to measure acute oxygenation and longer-term oxygenation. This test can predict whether a baby is at risk for fetal asphyxia (severe oxygen deprivation) and risk of fetal death during the short period of time directly after birth (the antenatal period). If a medical professional identifies a baby with oxygen deprivation, they have to take immediate steps to prevent the baby from having brain damage or death due to acidosis. One of these steps is an emergency C-section. Prenatal Testing: Doppler Velocimetry This test measures how well blood is flowing through the uteroplacental structure and how the baby responds to physiological changes. If the placenta’s blood vessels aren’t properly developing, this test will show progressive changes in areas like fetal blood flow, blood pressure and heart rate which show up as circulation changes. This test can show if there is major dysfunction in crucial arteries and veins like the umbilical cord. This is important because major dysfunction can indicate a likelihood for hypoxic-ischemic encephalopathy. This test is highly detailed and specific, and can pinpoint the flow of blood in different blood vessels. Abnormal Doppler findings must be closely monitored and a plan must be quickly made to determine when the baby should be delivered. Prenatal, Birth and Postnatal Care One of the ways that parents and doctors can prevent oxygen deprivation in a baby is proper prenatal care. Ultimately, parents and doctors work together to ensure a successful pregnancy, but it is key that doctors follow standards of care and keep parents informed of what they should be doing throughout pregnancy. Each pregnancy is different, and medical staff must be able to adjust their approach to each situation appropriately. There are certain tasks that doctors can do to decrease the risk of a child having HIE. The Role of Medical Professionals in Your Baby’s Care Proper Prenatal Care  Proper prenatal testing to identify problems early  Insisting on keeping prenatal care on schedule  Informing and educating families about healthy pregnancy, birth and childcare practices

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HIE HELP CENTER: MEDICAL INFORMATION  Advising mothers about proper prenatal diet and supplements (such as folic acid)  Advising mothers about health exercise habits  Advising others not to drink or smoke during pregnancy  Helping mothers manage their health  Treating a mother’s underlying health conditions (like infections or sexually transmitted diseases)  Ordering tests to identify genetic anomalies  Addressing emergency situations like bleeding during pregnancy  TORCH testing  Keeping up-to-date on standards of care  Avoiding medical malpractice and birth injuries Proper care during birth and delivery  Properly monitoring the baby for signs of fetal distress  Properly recording health indicators like APGAR scores  Properly undertaking birthing techniques  Adequately managing emergency situations  Following standards of care in delivering the baby  Recognizing and addressing delayed or stalled labor Proper postnatal care  Ensuring the child is up-to-date on immunizations to avoid infectious disease  Checking the newborn for jaundice  Scheduling and keeping well-baby check-up visits to ensure the baby is developing properly The Role of Parents in Neonatal and Pediatric Care Parents can seek out information from their physician about healthy pregnancy habits and monitoring, in order to take an active role in decreasing a pregnancy’s risk. They should seek the advice of medical professionals in regards to diet, exercise, supplements and other personalized advice given to them about their medical history and risk factors. This is especially true of mothers with risk factors that make their pregnancy high-risk, such as diabetes, high blood pressure, preeclampsia, sexually transmitted diseases, or obesity. High-risk patients sometimes need extra monitoring and support, and it is critical to follow the advice of medical professionals to minimize pregnancy risk. Parents can also check out their doctor’s credentials and medical history to make sure their doctor and the hospital they plan to deliver at don’t have a record of medical malpractice or mistakes during delivery. Parents may also explore their policies regarding scheduling doctors and procedures. Individual doctors can have different availabilities after-hours and are sometimes assigned on a ‘rotating’ schedule. In practice, this means that the doctor assisting in delivery might potentially not be the same one who did prenatal care. Other questions parents can ask when choosing doctors and hospitals include:  Does the hospital have a full-time OB/GYN on staff?  Is the OB/GYN A, B, or C-listed?  If you need emergency care, will you have access to a well-trained emergency staff?  What’s the hospital’s medical malpractice record? Do they have a record of birth injuries?

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HIE HELP CENTER: MEDICAL INFORMATION  What would the doctor do in an emergency situation?  Would they monitor fetal heartbeat?  How do they make the decision to do a C-section?  What do they do if a delivery takes a long time?  What do they do if the baby gets stuck? Would they use forceps?  Is the baby shows signs of distress, what would they do?  Do they induce labor? What medications do they use?  If a baby’s or mother’s health is in danger, what would they do? After a baby is born, parents should:  Monitor the baby for signs of jaundice (yellow skin) or other illness  Keep vaccines up-to-date  Bring developmental or health concerns up with a medical professional  Avoid injury to the baby by using proper car seats, high chairs, strollers and bikes If a baby needs medical care, parents can take an active role by keeping informed about any procedures their baby must undergo. Questions can include:  What is the purpose of the procedure?  What are the risks?  Are there alternatives? What are they?  How often has the doctor done the procedure?  What are the doctor’s qualifications and experience? Being informed is one of the best things that a parent can do to ensure their child will be healthy. They must also, however, be aware that unforeseen medical emergencies at birth sometimes do occur, and that medical professionals are required to properly provide medical interventions if their baby is in distress. Preventing Premature Birth One of the key ways that medical practitioners can avoid hypoxic-ischemic encephalopathy (HIE) in their patients is by taking all appropriate actions to avoid a baby being born prematurely. Premature babies are very fragile because they are still in the process of developing – this fragility extends to the baby’s brain as well. Preemies are far more prone to having brain bleeds (intracranial hemorrhages) and other blood-pressure-, nutrition-, and perfusion-related issues that can cause HIE. When a medical practitioner closely monitors a pregnancy and takes action when there is a suspicion that a child will be born early, it can help decrease the incidence of HIE. There are several procedures that medical practitioners can do in cases where there are indications that a woman may give birth prematurely, including:  Using a cervical cerclage to help support the cervix (when there is cervical insufficiency)  Prolonging pregnancies using progesterone when a mother has a prior history of preterm birth  Closely monitoring pregnancies to ensure high-risk conditions are not missed For other strategies for preventing hypoxic-ischemic encephalopathy, please visit our main prevention page. Cervical Cerclage There are certain factors that can predispose a mother towards having a premature birth. These factors are detectable via proper prenatal care and screening. One of these factors is called ‘cervical insufficiency’ (previously known as ‘incompetent cervix’).

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HIE HELP CENTER: MEDICAL INFORMATION The cervix is a structure that separates the uterus from the vagina. At the beginning of pregnancy is it closed and rigid but it tends to soften as the pregnancy progresses. With a cervical insufficiency, it begins to soften and open up too soon. When this happens, the membranes surrounding the baby bulge through the opening and eventually break, leading to an early water breaking (premature rupture of membranes). This early rupture leads to increased contractions, which, if not halted, can lead to premature birth or miscarriage. Certain women can benefit from a procedure called a cervical cerclage, a method of support that involves the placement of a stitch into the cervix. This helps keep the cervix closed throughout the pregnancy until it is removed between 36-38 weeks to avoid problems related to labor. For a cerclage to be effective, however, it must be placed fairly early in the pregnancy (typically before 14 weeks of pregnancy). In other situations, it can be placed between 14 weeks and 16 weeks, but the absolute latest it can be placed is at 24 weeks. When Might a Cerclage Be Placed? Certain risk factors can predispose a mother to a weak cervix. In these cases, doctors should consider placing a cerclage. Procedures and events that can cause a weak cervix include:  If the mother had an insufficient cervix before  If the mother has had a prior second-trimester stillbirth  If the mother’s water broke early before (Preterm premature rupture of membranes/PPROM)  If the other has had a cervical biopsy for diagnostic purposes (cone biopsy/cervical conization) or a LEEP (loop electrosurgical excision procedure)  If the mother’s cervix was torn in a previous birth  If the mother has had prior repeat or late-term abortions  If the mother has uterine abnormalities/anomalies  If the mother was environmental exposure to DES  If the mother has had a D&C procedure It is critical that a woman’s obstetrician take a full medical history to identify risk factors for cervical insufficiency. In many cases, cervical insufficiency produces only mild symptoms between weeks 15-20 (such as mild discomfort, pelvic pressure, backache, mild cramps, or light vaginal bleeding); between 16-28 weeks, cervical dilation (opening) may show no signs at all. Once they see a doctor regarding discomfort or concern, they may have significant dilation (2cm+). Usually a dilation of 4 cm or more triggers contractions or rupture of membranes. In addition to taking a mother’s medical history, prenatal screening can identify cervical insufficiency. During prenatal care exams, OB/GYNs typically do a pelvic exam during the second or third trimester. Cervical insufficiency is sometimes revealed during these exams, when (between 16-24 weeks) a mother shows signs of a dilated cervix. If a woman has a prior history of cervical insufficiency, a cerclage should be placed and the pregnancy should be monitored more closely using transvaginal ultrasounds (TVS) after 16 weeks. TVS helps monitor cervical length and can help identify if preterm birth is likely – if the cervical length is less than 25mm between 14-24 weeks. To reduce the risk of preterm birth, the mother is also typically given progesterone, which helps prolong pregnancy. Cervical cerclages aren’t always indicated, however. Even if a pregnant woman does have a cervical insufficiency, she should not have a cerclage when:  Active labor has begun  Placental abruption is present  The mother has an infection  Water broke early (PPROM)  The mother has active bleeding

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HIE HELP CENTER: MEDICAL INFORMATION Progesterone Up to ¼ of premature births in the second trimester are due to an insufficient or short cervix , which means that a significant number of preterm births could be prevented with proper care. During prenatal care, pregnant women should have their cervixes measured between about 19-24 weeks. If the OB/GYN finds that the cervix is short, the OB/GYN should consider placing their mother on progesterone. This hormone helps prolong pregnancy and prevent preterm labor with single pregnancies. Usually (when a mother has a history of prior preterm birth), OB/GYNs begin progesterone therapy around 16-20 weeks until about 36 weeks. This is done via weekly intramuscular injection. If a woman has a shortened cervix without a prior history of preterm birth, progesterone is administered vaginally beginning around 18 weeks. This is something typically done in conjunction with a cervical cerclage to maximize the chances of prolonging a pregnancy to a sufficient length.

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HIE HELP CENTER: MEDICAL INFORMATION Treating HIE Hypothermia Therapy: Reducing Injury Severity Right now, there is only one treatment available for treating hypoxic-ischemic encephalopathy. This treatment is known as hypothermia therapy, and can also be known as ‘cooling therapy,’ ‘cooling treatment,’ or ‘hypothermia treatment.’ This treatment involves cooling the baby down to a temperature below homeostatic temperature to allow the baby’s brain to recover from a hypoxic-ischemic injury, usually to about 33 degrees Celsius (91 degrees Fahrenheit). There are two ways that hypothermia treatment can be administered: using a cooling cap for selective brain cooling (‘head cooling,’) or using a cooling blanket for ‘whole-body cooling.’ Either of these options can be effective; the choice to use one over the other is dependent on what protocols are in place and what equipment a particular NICU has. Selective Brain Cooling and Infant Cooling Caps A cooling cap is a flexible cap that runs cold water or another coolant through channels in the cap. This cold liquid draws heat from the infant’s body and reduces the temperature of the brain. The baby’s temperature is reduced for about 72 hours (3 days), and the baby is warmed back up very slowly. Whole-Body Cooling and Cooling Blankets With whole-body cooling, the infant is placed on a cooling blanket while naked (except for a diaper). This cooling blanket drops the temperature of the baby’s entire body. The cooling process lasts for three days, until the baby is slowly re-warmed by degrees. During both kinds of cooling therapy, doctors, nurses and other medical staff watch the baby’s vital signs, including:  Respiration  Oxygenation  Heart rate  Brain wave activity By monitoring the baby closely, they can determine how well the baby is responding to treatment, and they can make adjustments as necessary. When Should My Baby Get Hypothermia Therapy? If your baby was diagnosed with hypoxic-ischemic encephalopathy (HIE), doctors have to begin hypothermia therapy within six hours of the initial brain injury, which is – in many cases – birth and delivery. The sooner they start hypothermia therapy, the greater the chance that the baby’s potential disabilities will be minimized. There are certain criteria that babies have to meet in order to qualify for the therapy, and these can vary slightly depending on the hospital your baby is in. Usually, this means that the baby has to be at least 36 weeks’ gestation, and show at least two of the following signs of moderate-to-severe HIE:  Lethargy  Stupor  Coma  Abnormal tone/posture  Abnormal reflexes  Decreased/absent spontaneous activity  Problems with breathing, heart rate or visual reflexes  Metabolic acidosis  Seizures

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HIE HELP CENTER: MEDICAL INFORMATION Re-Warming the Baby After Hypothermia Therapy Although hypothermia therapy lasts about 72 hours, treatment for brain injuries continues even after the cooling is done. The baby is warmed up by degrees to prevent a condition known as reperfusion injury. Reperfusion injury occurs when blood rushed back too quickly into an area that has already been damaged. When tissue is damaged, the cells are in a state where inflammatory compounds are being released from damaged cells. The rush of blood back into the area can damage cell membranes already unstable due to injury, releasing compounds that then cause further damage to the cells in a cascade reaction. Hypothermia therapy helps to stabilize the cell membranes and prevent this from occurring. Gradual re-warming (as opposed to fast rewarming) helps keep cells stable, minimizing brain damage together with hypothermia therapy. Where Do Babies Get Hypothermia Therapy? Not all hospitals will be equipped to provide hypothermia therapy because not all NICUs are the same. Community hospitals, for example, may not have brain cooling equipment, but they should have the ability to transfer the baby to a bigger or more specialized hospital that can provide cooling. Generally, neonatal care units are divided into Grade I, II, III, and IV, and only Grade III or IV units are able to provide hypothermia therapy to a baby. It is a good idea to inquire about what kind of care a neonatal care unit can provide when researching hospitals. Other Therapies for HIE Hypoxic-ischemic encephalopathy is a diagnosis whose severity can be in part mitigated with the appropriate physical, occupational, speech and other therapies, depending on the child’s unique health situation. For a complete primer on available therapies and treatments for HIE, please call us or send us a form submission requesting the ‘HIE Treatments and Therapies’ information packet. We’re available 24/7 at HIEHelpCenter.org or via phone at (888) 329.0122.

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HIE HELP CENTER: MEDICAL INFORMATION For Additional Informational Packets Never hesitate to reach out to us for more educational information on hypoxic-ischemic encephalopathy (HIE!) We’d be happy to send you a copy of our comprehensive primers on HIE-related care, including:  HIE Medical Information  HIE Treatments and Therapies  HIE Caregiving: Resources for Families Also, please check out https://hiehelpcenter.org/resources/ for tip sheets, quick reference items, our HIE medical glossary, and links to special needs parenting videos and podcasts online!

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