Congenital Diaphragmatic Hernia - Family Handbook

CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

Congenital Diaphragmatic Hernia Family Handbook

Researched and Prepared by: Jason Gien, MD & Mallory Mueller, MSN, RNC-NIC

With Support and Funding provided by:

Atlanta, GA | forehadley.com The Fore Hadley Foundation provides hope, opportunity, and support to the Congenital Diaphragmatic Hernia (CDH) community, and serves to inspire others to join the fight to eliminate this birth defect.

To learn more about opportunities for possible financial assistance, emotional support, or more information on our other CDH-centric programming, please visit forehadley.com or email info@forehadley.com.

Peer Reviewed and Approved by: The Children's Hospital Neonatal Consortium CDH Focus Group thechnc.org

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

Table of Contents What Is Congenital Diaphragmatic Hernia?.......................... 5 How is CDH Diagnosed?........................................................ 10 Treating CDH Before Birth and At Birth...............................13 The Delivery Room.................................................................19 Caring for Your Baby After Birth.......................................... 23 Ongoing NICU Care............................................................... 29 Your Role............................................................................... 33 Frequently Asked Questions in the NICU............................ 37 Glossary of Common Terms..................................................43

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What is congenital diaphragmatic hernia?

What Is Congenital Diaphragmatic Hernia?

Congenital diaphragmatic hernia (CDH) is a hole in the diaphragm. The diaphragm separates the chest from the abdomen and helps keep all the organs in the right place. CDH happens when the diaphragm doesn’t form correctly and it leaves a hole between the chest and the abdomen.

This opening lets the organs that are in the abdomen move up into the chest. This can cause your baby’s lungs to not develop and grow like they should.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

• The stomach and bowel are the organs that most often move into the chest • If the liver moves into the chest it causes more problems than only having the stomach or bowel in the chest • 85-90% of CDH happens on the left side • 10-15% of CDH happens on the right side and a few are on both sides • Most CDH are from a hole in the back of the diaphragm called a Bochadalek hernia • A hole in the front of the diaphragm is called a Morgagni hernia

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What is pulmonary hypoplasia?

Pulmonary hypoplasia means that the lungs are smaller than they should be based on how many weeks along your pregnancy is. It is caused when the organs from your baby’s abdomen move into the chest and push on the lungs which keep them from growing.

Pulmonary hypoplasia makes it hard for your baby to breath when they are born, which is why they will have a breathing tube put through their mouth into their windpipe (trachea), and they are put on a breathing machine called a ventilator.

What is pulmonary hypertension?

As a result of pulmonary hypoplasia, babies with CDH develop pulmonary hypertension. Pulmonary hypertension is high blood pressure in the lungs. With pulmonary hypoplasia there are fewer blood vessels in the lungs. In addition, the blood vessels are also thickened increasing the pressure in the blood vessels.

Blood vessels are small tubes that carry blood and oxygen throughout the body.

Pulmonary hypertension causes blood to bypass the lungs making it hard for your baby to breathe and get the oxygen they need. We figure out how bad the pulmonary hypertension is by comparing it to the blood pressure in the rest of the body.

Why does CDH happen?

We don’t know what causes CDH. About 60% of the time CDH is the only health problem. In 40% of babies, CDH happens along with other health problems, such as problems with the brain or heart. CDH is more likely to have a genetic cause when there are many problems with the baby. Genetic problems happen in 10-20% of babies with CDH. All babies with CDH will be checked for genetic problems. Other times CDH may be caused by environmental or unknown factors.

How often does CDH happen?

CDH happens in about 1 in 2500 babies or 0.04% of the time.

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How Is CDH Diagnosed?

How Is CDH Diagnosed?

CDH is usually found during an ultrasound. Your doctor may also do several other tests to find out how bad your baby’s CDH is. Below are the most common tests.

Ultrasound: A machine that uses sound waves to get pictures of the baby and their organs before they’re born. Ultrasound pictures are used to show us: • Where the organs are located: CDH is diagnosed by seeing the stomach and/ or bowel in the chest area and the heart in the right rather than left chest. The ultrasound can also show how severe the CDH is. When the liver is seen in the chest area it usually causes more severe problems. The position of the gallbladder, a small organ attached to the liver, can be helpful in figuring out which side the CDH is on and if the liver is up or down.

• The size of the lungs: comparing the size of the lung on the unaffected side to the size of the head, tells us how severe the CDH is. This is known as the lung head ratio (LHR).

MRI: A machine that uses magnets, radio waves, and a computer to get detailed pictures of the inside of the body. MRI is used to see: • The size of the lungs and the blood vessels in the lungs • Whether the liver is in the right place • Whether there is a sac (thin layer of tissue that surrounds the abdominal contents) • If there are other abnormalities with your baby other than CDH An MRI can be used to see whether there is a sac around the stomach and bowel.

Seeing a sac is a good sign because it usually keeps the abdominal organs from pressing as much on the lungs. An MRI can also be used to find out how severe your baby’s breathing problems will be after they are born. The larger the lungs the less severe the breathing problems and the better their chance of survival.

Measurements for pulmonary hypoplasia: • Percent Predicted Lung Volume (PPLV) - measured before 24 weeks into your pregnancy • Total Lung Volume (TLV) - measured at 34 weeks into your pregnancy

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

Fetal echocardiogram: An ultrasound that shows us if the heart has developed normally. In up to 30% of babies with CDH, the heart doesn’t develop normally in the womb.

Amniocentesis: a test of the amniotic fluid to look for genetic problems.

Amniocentesis is done by putting a small needle into the amniotic sac and taking out a small amount of the fluid for testing. We use ultrasound to make sure we get the needle in the right place. This test can help us learn if the CDH has a genetic cause.

Predicting the Severity of CDH There is no one thing that can tell us how bad the CDH is. Your team will look at many factors to help predict what kind of medical help your baby will need after birth.

These things include: 1. Whether there is a genetic cause of your baby’s CDH 2. Whether the heart developed normally in the womb 3. Whether there is liver or stomach in the chest and if there is a sac 4. The size of your baby’s lungs These 4 factors help determine the chance of survival and the need for treatment with a heart lung bypass machine called ECMO.

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Treating CDH before birth and at birth

Treating CDH before birth and at birth After the diagnosis of CDH is made there are several choices for treatment depending on the severity of the condition. The medical team will make recommendations for the level of care they think your baby will need based on the prenatal tests. Many babies with CDH will be able to do well with standard medical care to treat their CDH. If you want your baby to have aggressive medical treatment, your baby might need the following interventions to have the best chance of surviving. Not all treatment options are available at all facilities that treat CDH or are necessary for all babies. If your baby requires more advanced treatment, then you and your medical team will talk and determine which center is best for your baby to be delivered at or transferred to shortly after delivery. As we continue to understand CDH better, there are ongoing trials across the country for new treatment options. In some cases, your baby might be eligible to be enrolled in different research studies, such as FETO.

Fetal Endoluminal Balloon Tracheal Occlusion (FETO) This is a surgery that is done while your baby is still growing inside the mom for the most severe cases of CDH to help your baby’s lungs grow faster. This procedure, called tracheal occlusion, uses a balloon which is placed in your baby’s trachea (windpipe) while he/she is still inside the mom. The procedure keeps the fluid in your baby’s lungs, which speeds up the development of the lungs. The procedure is done while the mom is awake with local or regional anesthesia. Doctors are still learning about how helpful this procedure is. Research from Europe has shown that the baby’s chance of surviving is increased from 25-50% with this procedure. In about 1 in 3 cases, this procedure causes babies to be born several weeks early. This procedure is still in the research phase and long term follow-up studies are in progress. If your baby’s CDH is severe enough to meet the requirements for FETO your team will discuss this procedure with you and options for transfer to a center able to perform this procedure.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

ECMO Extracorporeal membrane oxygenation, known as ECMO or heart lung bypass, is a machine which is used to get enough oxygen in your baby’s blood. Surgery is needed to connect your baby to an ECMO machine. This is done by placing either one or two small tubes into your baby’s blood vessels, usually in their neck. The ECMO machine pulls blood out of the body, puts oxygen in the blood and then returns the oxygenated blood back into your baby. The machine works for your baby’s lungs so that they can rest and grow.

ECMO Complications: • In order to keep blood clots from developing a medicine called heparin is continuously running through the ECMO machine. This medicine can also cause your baby’s blood to not clot as quickly so they have a higher risk of bleeding. We will closely watch any incisions for bleeding and lab tests to try and minimize this risk.

• Babies also have very fragile blood vessels in their brain and being on ECMO increases their risk of bleeding from these vessels. This can cause an intraventricular hemorrhage (IVH), which is bleeding in the brain. We will use a head ultrasound to check for any IVH. • The longer a baby needs ECMO the harder it gets to make their heart and lungs do the work on their own.

Babies on ECMO are also at risk for infection, decreased heart function, and decreased kidney function.

ECMO and medical equipment

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In some cases, a baby with CDH may have additional complications that worsen their prognosis and a family might decide that extensive medical intervention is not the best choice for their family and their baby. In these cases, you can choose to either terminate your pregnancy or carry your pregnancy to term with the goal of care being comfort.

The medical team will shift their efforts to supporting you and your family. We will provide your baby medicines and other care to keep your baby comfortable as they pass away. The care plan shifts to supporting your family in spending as much time with your baby as possible. We will talk with you to understand what your wishes are for this time with your baby so we have a plan we all agree upon for when they are born.

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The Delivery Room

The Delivery Room Who will take care of my baby right after birth?

All babies with CDH have the best chance for survival when they are born in a specialized birthing center that can perform ECMO and has surgeons and nurses that can treat CDH. Several people are needed in the delivery room to stabilize babies with CDH after birth.

These include newborn intensive care specialists, neonatal nurse practitioners, NICU nurses and respiratory therapists (RTs).

What can I expect when my baby is born?

• Your baby may or may not cry after birth. This has no impact on your baby’s chance of survival.

• A baby with CDH needs help immediately after birth so you won’t be able to hold your baby when they are born.

• A breathing tube will be put in your baby’s mouth that goes to their lungs right away. Your baby will then be attached to a ventilator, which is a special breathing machine.

• We’ll put a second tube through your baby’s nose or mouth into the stomach to remove any air or fluid that was swallowed during delivery.

• We’ll put special IV lines in the part of the umbilical cord that goes through your baby’s belly button. Two IV lines will be put in,one into a vein to provide fluid and medicine to your baby, and one in an artery to measure blood pressure continuously and sample your baby’s blood.We may also put a small tube in your baby’s right hand to measure blood pressure and provide a way to get samples of blood. Your baby may also need other lines to provide fluid or medicine. These lines go into the big blood vessels around the heart and are called central lines.

• We will do a chest x-ray to check where the breathing tube is, where central lines are placed, and the size of the lungs.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

What will the team be checking right after my baby is born?

• Heart function. We use an echocardiogram, which is an ultrasound of the heart, to tell us how well your baby’s heart is working.

• Pulmonary hypertension. The echocardiogram will also help us measure the blood pressure in the lungs to check for pulmonary hypertension.

• Lung function. We will check how well the lungs are working. Blood samples are drawn from the IV lines in your baby’s arteries to tell us how well the lungs are working.

• Blood pressure in the body. We use an IV in the belly button or in the arm or leg to check blood pressure continuously. We often need to give babies with CDH medicines to keep the blood pressure in the normal range. If these medicines don’t help, your baby may need ECMO. Based on all of this the medical team will decide if your baby needs ECMO. Sometimes, even when all these assessments are normal your baby may still need ECMO to protect their lungs from needing very high support from the ventilator.

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Caring for Your Baby After Birth

NEONATAL INTENSIVE CARE UNIT

Caring for Your Baby After Birth Care for babies with CDH has evolved over time with several best practices identified that can help improve your baby’s chance for survival.

Gentle Ventilation All babies with CDH need a breathing machine to help them breathe after birth. Doctors used to think that using higher pressure settings on the ventilator was helpful. We now know that higher ventilator settings don’t help the baby survive and can cause damage to the lungs. Instead, we use “Gentle Ventilation” or low ventilator pressures to prevent damage to the lungs. “Gentle Ventilation” has been linked to better chances of surviving by protecting the small lungs from damage.

Treatment of Pulmonary Hypertension Pulmonary hypertension is a major reason why babies with CDH may not survive. We now have many more tools to treat pulmonary hypertension in CDH than ever before.

These tools include giving your baby oxygen and using several other medicines, inhaled nitric oxide being the most common. These tools are used to help lower the blood pressure in the lungs, which helps prevent a pulmonary hypertensive crisis (PPHN crisis). PPHN crisis happens when the blood pressure in the lungs is higher than the blood pressure in the body. This causes blood without oxygen to go to the body instead of going to the lungs, which causes your baby’s oxygen saturations to drop and your baby may often turn blue in color. We treat a PPHN crisis with sedation medicines, oxygen, and medicines to relax the blood vessels in the lungs.

• Oxygen itself can help relax the blood vessels in the lungs and is important for treating CDH. The amount of oxygen your baby gets is determined by the setting on the ventilator and is referred to as FiO2. Normal FiO2 that we breathe in without any extra oxygen is 21%. FiO2 ranges from 21-100%. Changes in FiO2 are made based on your baby’s oxygen levels, which is measured by a small device on the baby’s hand or foot called a pulse oximeter that determines the percentage of oxygen in the blood.

• Inhaled nitric oxide (iNO) is a special gas delivered through the breathing machine that works better than oxygen to relax and open the blood vessels in the lungs.

• Additional pulmonary hypertension medicines may include: Intravenous or oral sildenafil, inhaled or intravenous epoprostenol (Flolan) or oral bosentan in the most severe cases.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

Echocardiography We use many ultrasounds of the heart, called echocardiograms, to care for your baby. The echocardiogram can help us tell how well your baby’s heart is working, how severe the pulmonary hypertension is, and whether the medicines we are giving your baby are working. An echocardiogram can also tell us if your baby is ready to start coming off ECMO or the ventilator.

Low Blood Pressure Support Many newborns with CDH have low blood pressure in the body. Low blood pressure is often managed by giving your baby extra fluids. When fluids are not working or when we want to avoid giving too much fluid, we use medicines (Dopamine, epinephrine, vasopressin, and hydrocortisone) to stabilize the blood pressure. If none of these medicines work, ECMO is often needed.

Waiting to Repair CDH Doctors used to think it was best to repair CDH within hours of birth. Today we wait until the blood pressure in the lungs has gone down before starting to repair the CDH. In mild to moderate CDH this may happen within 3 to 5 days. But in moderate or severe CDH this may take 10 to 14 days. We have seen better results for babies by waiting. When a baby goes on ECMO, it may be better to have the repair earlier. Your surgeon and care team will discuss the benefits and risks of repair on ECMO versus delayed repair after ECMO. When your surgeon decides it is time to repair the hole in the diaphragm, there are several methods available to close the hole.

1. If the hole is small, we may be able to stitch it closed. This is called primary closure.

2. If the hole is too big to be fixed with just stitches, we can cover it with a patch made from Gortex or mesh.

3. Sometimes we can use a part of your baby’s muscle from the abdominal wall to close and cover the hole. This method is called a muscle flap and is preferred because the muscle grows with your baby and there is less risk for infection.

Often during surgery, a small tube is put in your baby’s chest. This lets fluid and air be taken out of the chest so that the lungs can grow and work properly.

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Nutrition Studies have shown that many babies with CDH don’t grow as fast as they should, which can impact survival. We have a designated nutrition specialist that will monitor your baby’s nutrition on a daily basis. We also use a proactive plan for feeding babies with CDH. We start giving calories through IV nutrition called TPN. Later we feed babies with CDH high calorie breast milk or formula through a feeding tube.

A few days after your baby’s CDH is repaired, we will put a feeding tube through the nose into the stomach and we’ll slowly start feeding your baby by giving breast milk or formula through this tube. Over time we will lower the amount of TPN your baby gets and increase the amount of breast milk or formula that is provided. Eventually your baby should be getting all their nutrition from breast milk or formula, which we call full feeds. Babies with CDH may have reflux where milk comes back up their throat and causes frequent spitting up. For babies with reflux, a feeding tube is placed that goes from your baby’s nose down into their intestines. This is called a transpyloric tube (TP tube). Once your baby is growing and is on full feeds the feeding tube is pulled back from the small intestine into the stomach. We’ll keep watching your baby closely for reflux to make sure they are growing, as some babies with CDH may need surgery called a Nissen Fundoplication to treat their reflux.

Airway Assessment Some babies with CDH have problems with their airway being soft and floppy and not able to open all the way. This happens from having the stomach and other organs pressing on the airway. This is called tracheomalacia if it involves the big part of the airway or bronchomalacia if it includes the smaller parts of the airways.

If this happens it makes their pulmonary hypertension even worse and they may not respond well to the treatment we’re giving them. This can be treated by having the ventilators use more pressure to help keep the airway open and help your baby breathe. If the airways don’t get better on their own, your baby may need a tracheostomy (a breathing tube through the neck) so that they can remain on the ventilator for a longer period of time while the airways get better.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

Cardiac Catheterization The assessment of pulmonary hypertension done by echocardiogram is only an estimate, and sometimes more reliable tests are needed. Doctors may suggest a test called cardiac catheterization to measure the pressure in the lungs if your baby is not getting better. A catheter is put in a vein in the leg and guided up to the heart to measure the pressure in the pulmonary artery. This test helps us see how well the medicines we’re using to treat the high pressure are working or to help us learn if there are problems with the structure of the lungs.

Genetics Sometimes genetic testing by amniocentesis is not done before your baby is born. In these cases, it may be recommended, especially if your baby has multiple anomalies, to send blood samples to test for a genetic cause to your baby’s condition. Most centers have genetic specialists who can help identify if your baby has an isolated case of CDH or if your future babies would be at risk for CDH. Sometimes CDH happens as part of a genetic syndrome so the medical team will check for other features that would indicate your baby has a syndrome. Other tests like ultrasounds may also be used to help guide the medical team to fully understand if other organs are impacted by your baby’s condition.

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Ongoing NICU Care

Ongoing NICU Care After your baby has had their CDH repair, we can start working on weaning your baby’s respiratory support and starting them on feedings.

Babies with CDH may still face other problems after their repair. Their journey towards home can often have many ups and downs.

Respiratory Care Once your baby’s pulmonary hypertension is controlled we’ll begin to slowly wean them off the ventilator. We will decide that they are ready to come off the ventilator by looking at blood gases and chest x-rays. These will show us how big the lungs are and how well they are working. After they are off the ventilator, if they still need help breathing we may use: • Continuous Positive Airway Pressure (CPAP): CPAP is a mask that goes over the nose and a machine helps push air in and out of the lungs so your baby doesn’t have to work as hard to breathe.

• Non-Invasive Positive Pressure Ventilation (NIPPV): NIPPV is another way to help your baby breathe by pushing air in and out of the lungs. This kind of respiratory support keeps some pressure in the lungs so that they do not close all the way.

• Heated high flow nasal cannula: oxygen flow is provided by a nasal cannula placed in the nose with flow provided at greater than 1 liter per minute. Oxygen is given and mixed with air (blended) so the right amount of oxygen is provided to your baby.

• Low flow nasal cannula: Oxygen flow is provided by a nasal cannula put in the nose with flow provided at less than 1 liter per minute. Babies can go home with low flow nasal cannula.

If your baby can’t be weaned off the ventilator or remains on CPAP, they may need a tracheostomy tube (called a trach) to help them breathe. A trach is a breathing tube that is surgically put in the airway through the neck instead of the mouth. A trach lets a baby get whatever pressure or ventilator support is needed and is a way for them to go home once stable on a home ventilator. A trach also makes it easier when your baby still needs a ventilator for you to hold your baby and for them to grow normally; learn to sit, crawl and walk.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

Nutrition Support Your baby will be able to start eating by mouth when the medical team and therapists feel they are on low enough oxygen support and not breathing too fast. A special feeding team (OT and speech therapists) will help your baby learn to eat. The medical team and therapists will check your baby every day to see if they are breathing comfortably and ready to take food by mouth. Breast feeding and bottle feeding are both options.

If your baby needs more respiratory support or is not able to eat enough milk or formula to grow, they may need a gastrostomy tube or G-tube. This is a small device called a button that is put in through the belly that goes into the stomach and is held in place by a small balloon. Babies can go home with a G-tube. Some babies are close to being able to eat all of their feedings by mouth when they are ready to go home. In these cases, they might go home with a nasogastric (NG) feeding tube for just a few weeks.

Many babies with CDH spend most of their energy on breathing and growing. It may be too much work for them to try and eat by mouth. A NG or G-tube lets them get all the calories they need to grow, while still working on taking food by mouth if they can do so.

Reflux Almost all babies with CDH will also have gastroesophageal reflux, where milk comes back up their throat and can cause frequent spitting up. Many babies with reflux will need anti-reflux medicines and some will need anti-reflux surgery. If your baby has severe reflux they may need a surgery called Nissen Fundoplication. This surgery is recommended for babies with CDH and severe reflux because it is vital that your baby grows, which also lets the lungs get bigger. This surgery also protects the lungs from milk coming up the throat and going into the lungs.

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Developmental Care Babies with CDH are at risk for delays in motor skills, mental development, and problems with hearing or vision. CDH babies need therapies during their stay in the NICU and after discharge with: • physical therapy / PT (large muscle motor development) • occupational therapy/ OT (small muscle motor development) • speech therapy (feeding, speech and audiology/hearing) CDH babies also need long term follow up for heart and lung issues. We suggest that all CDH babies get follow up with a peditrician or follow up clinical that has expereince with CDH to make sure all their needs are being met.

Palliative Care Based on how severe the CDH is, some babies have very long hospital stays. Sadly, some babies do not survive. We know this is a very difficult time for parents and families. To support you, your baby and your family some centers involve a palliative care team.

The goal of palliative care is to improve the quality of life for patients and their families when facing a life-limiting illness. Our medical team will talk to you often about your baby’s medical progress, suggested medicines and treatments, etc. We will also work closely with you to discuss your goals of care for your baby and family.

NICU palliative care room

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Your Role

Your Role Getting to Know Your Baby When your baby is first born, they are going to be quite sick and need a lot of medical equipment and specialized care. Even though you won’t be able to hold your baby there are many ways you can interact with your baby to bond with them.

Your nurses will help you know when it is a good time to interact with your baby.

Babies born with CDH can often be very sensitive to noise, light, and sound so we want to create a healing setting. For these reasons your nurse may ask you to only have a few visitors in the baby’s room at a time, and to use quiet speaking voices.

Here are some things you can do even when your baby is very sick: • Hold your baby’s hand or place your hand on their head. It is best to not stroke the baby.

• Talk to your baby in a soothing calm voice- you can read them stories or sing a lullaby • Provide mouth care with breast milk or sterile water during care times • Change your baby’s diaper with the nurse’s help • Place a scent cloth near your baby’s head to help them feel close to you • Rub lotion on your baby’s feet and legs and provide gentle massage • Each hospital involves families in care, but this may look different depending on where your baby is being treated. Your nurses will help you know what is best for your baby.

Caring for Your Baby Sleep is very important to helping babies heal so we often try and combine all our tasks every 3-4 hours. These are called care times. During care time we will check your baby, move them into a new position, change their diaper, give medicines, and perform tests such as x-rays or ultrasounds.

As your baby gets better your nurse will help you become more involved in their cares.

This includes helping with: • Bathing • Feeding • Holding • Learning to care for their medical equipment if needed, such as G-tubes or tracheostomy tubes, and oxygen.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

Many parents may be nervous to help during care times or even touching their baby when they are first born and very sick. We will help you become more comfortable with caring for your baby. We will make sure you feel confident to provide all your baby’s care before you go home.

Your NICU Room • After your baby is stabilized in the delivery room they will be moved to the Neonatal Intensive Care Unit, where they will get all the care they need.

• Sometimes we will need to do procedures that are sterile. During these times, we will ask you to step out of your baby’s room, but will let you know when it’s ok to go back in.

• There may often be different alarms going off in your baby’s room from the medicine pumps, monitor, or breathing equipment. Our nurses know which alarms need attention right away, and they will teach you what the different alarms mean. The alarms can seem scary at first, but please know your nurse will respond as soon as they can.

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Frequently Asked Questions in the NICU

Frequently Asked Questions in the NICU Who is on my baby’s Care Team?

CDH babies need help from many team members including: • Bedside Nurse • Respiratory Therapist (RT) • Neonatal Nurse Practitioner (NNP) • Neonatal Fellow • Attending Neonatologist • Surgical Fellow • Surgical Attending

What is a primary nurse?

Most hospitals have primary nursing teams where there are a few nurses on the day and night shifts that will consistently care for your baby. This will help the nurses get to better know you and your baby throughout their hospitalization.

What happens during Rounds?

During Rounds, the medical team will talk about what happened during the last 24 hours. We will then create a plan for the day, including plans for feeding your baby, labs or tests that are needed, changes to your baby’s medicines, changes to your baby’s breathing support, and taking care of your baby’s pain. Rounds allow parents to be involved in the medical decision making and receive updates from the providers.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

What should I bring to the NICU? The NICU will provide all the items that your baby needs including blankets, diapers, burp clothes, bath supplies, and all the supplies for pumping breastmilk.

You may want to bring: • Cute blankets for your baby’s bed • Socks, hats, or headband for your baby. At first, your baby will not be able to wear clothes due to all their medical equipment, but you can put some small items on your baby • Pictures from home • Anything else to make you feel more comfortable (pillows, blankets from home) • Entertainment for you (books, IPad, games, etc.) You will spend a lot of time in the NICU and often it may feel like the days just stand still. We encourage you to bring things to do and to also take breaks from your baby’s room.

What is an arterial blood gas (ABG)?

Blood gases are used to check your baby’s ability to take in oxygen and get rid of carbon dioxide. It also tells us about their acid base balance. Based on their ABG we may make changes to their ventilation or fluids.

Normal ABG values Value

Normal Range

What it tells us?

pH

7.30-7.45

Level of acid in the blood can be impacted by respiratory status or metabolic status

Carbon Dioxide (CO2)

35-45 mmHg

Ventilation

PaO2 (oxygen)

60-80 mmHG

Oxygenation

Bicarb (HCO3)

19-26 MEq/

Metabolic balance

Base Excess

-4 to +4

Acid-Base balance

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What are all the numbers reported?

Making sure your baby doesn’t get too much or too little fluid is very important to helping them get better. Each day the medical team checks how much fluid your baby had in and out, and reports the results of any lab tests changed by fluid status and labs that track kidney and liver function.

When will my baby start to get milk?

For babies to start getting breast milk through a feeding tube, they need to be able to keep up their blood pressure on their own and have good oxygen going to their stomach and intestines. After your baby has had their CDH repair, we’ll put a feeding tube into their intestines that can then be used to slowly start milk when they are ready.

When can I hold my baby?

Your baby can be held when the CDH has been repaired, your baby is on a conventional ventilator, off of blood pressure medicine, and their PPHN is stable enough to tolerate being held. Your bedside nurse and medical team will help decide when it is the right time for your baby to be held.

When can my baby eat by mouth instead of through a tube?

Your baby will need a feeding tube until he/she can have their breathing tube removed, and can breathe comfortably on an appropriate amount of oxygen. It is not safe to feed babies who breathe too fast (more than 60 times/minute) and who need more than several liters of oxygen. Your nurses, doctors, and a team of therapists will help you decide when it is the best time to breastfeed or give your baby a bottle.

How long will we be here?

The length of stay varies greatly for all babies with CDH. Based on the severity of the CDH, your medical team will help direct this time frame for you. Basic rules for your baby going home include: • taking all food by mouth or G-tube/NG • gaining weight • tolerating low flow nasal cannula • off inhaled nitric oxide Some babies with very mild CDH can go home as early as a month or two, but many need to be in the hospital for around 6 months. The most severe cases of CDH may need to be in the hospital for up to a year or more.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

What is the next goal for my baby at this point in his/her care?

Your baby will meet many milestones during their hospital stay! Some of these milestones are: • starting tube feeds • getting the CDH repaired

• getting extubated (taking their breathing tube out)

• seeing the lungs open up on x-ray for the first time

• being able to make noise while crying

• weaning off the ventilator

• moving around on their own

• opening their eyes

These are just a few. Your medical team will help you decide what the milestones are for your baby each day and will set goals to work on.

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Glossary of Common Terms

Glossary of Common Terms Cardiac Monitor: Provides important vital signs for your baby including: • Heart rate (green)

• Arterial Blood Pressure (Red)

• Oxygen Saturations (blue)

• Respiratory rate (white)

Central Line: A long narrow tube that enters your baby’s vein and ends at the baby’s heart. This tube can provide medicines and fluids to your baby for longer periods of time.

Peripheral Arterial Line: A small catheter placed into an arterial vessel that allows for continuous blood pressure monitoring, and allows us to draw blood from your baby for lab tests without poking them each time.

Medication Pump: A machine that can be set to a specific rate to deliver all the different medications and fluids your baby may need. Some medications will be running continuously on a pump, while others will be given over a set amount of time such as 30 minutes.

Foley Catheter: A small plastic tube inserted through an opening that goes to the bladder. This allows for urine to be removed from your baby’s body so that we can closely monitor how much fluid they are getting rid of.

Endotracheal Tube (ETT): A narrow plastic tube that is inserted into your baby’s trachea (windpipe). and is connected to a ventilator.

Ventilator: A machine that connects to an ET tube that provides a set number of breathes each minute. It also can provide pressure to help keep your baby’s lungs open so that they can receive oxygen and get rid of carbon dioxide. Ventilators also provide set amounts of oxygen ranging from 21% (room air) to 100%.

Oscillator: A special type of ventilator that gently allows your baby’s lungs to stay open, which can help them grow. This ventilator does not provide regular breathes, but provides lots of breathes which causes their chest to wiggle. Many babies with CDH may initially need an oscillator due to their very small sized lungs.

Nitric Oxide: An inhaled gas that is delivered through different breathing devices and breathed into your baby’s lungs. This gas helps relax the blood vessels of your baby’s lungs when they have pulmonary hypertension.

Oro/Nasogastric feeding tube: A small plastic tube placed either through your baby’s mouth or nose that goes down their esophagus and into their stomach. This allows your baby to receive milk and medicines when they are not able to take it by mouth.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

Chest Tube: A plastic tube inserted into the chest by a physician that allows air or fluid that builds up inside the chest to be removed. This tube is hooked up to a collection chamber that measures how much comes out, and can also be connected to suction to help actively remove the air or fluid.

Tracheostomy (Trach): Some babies with CDH may need to go home with a ventilator. For these babies a tracheostomy tube is placed by a surgeon. A small incision is made in their neck that goes into their trachea (airway) and a breathing tube is placed in the opening. This kind of breathing tube allows your baby to move around a lot more and go home with the breathing equipment they may need. Not all babies with CDH end up needing a trach.

Gastrostomy Tube (G-tube): A surgically inserted feeding tube that provides milk, formula, or medicine directly into the stomach. Babies who cannot eat all they need to grow may need to go home with a G-tube.

Commonly Used Medical Terms and Tests Lung Head Ratio (LHR): Measured by ultrasound and is an assessment of the area of the unaffected lung divided by the head circumference. This provides a ratio that is helpful, but only when the ratio is extremely above or below what is expected. If the lung to head ratio is low this indicates smaller than expected lungs.

Observed to Expected Lung to Head Ratio: A modified LHR in which the measured (observed) LHR is compared to what the LHR should be at a given number of weeks into your pregnancy (expected LHR). The reason for this is that between 12 and 32 weeks of your pregnancy, normal lung area increases four times more than head circumference. This provides a more accurate interpretation of the ratio for the actual age in weeks of the developing baby.

Total Lung Volume (TLV): A measurement done by MRI at 34 weeks into your pregnancy that can determine the size of the lungs. Babies with a TLV of greater than 40 mls have a 90% chance of survival and a 10% need for ECMO, but when babies have less than 20 mls TLV their survival chances decrease to 35% and 86% will need ECMO. Percent Predicted Lung Volume (PPLV): A calculation done to estimate what the expected lung volume would be if there was no CDH. This measurement is helpful early in the pregnancy at less than 24 weeks. If the PPLV is more than 20% chances of survival are 100%, but if the PPLV is less than 15% chances of survival decrease to 40% and all require ECMO. Systemic: Refers to the blood pressure in the rest of the body (not in the lungs).

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Suprasystemic: The blood pressure in the lungs is higher than the blood pressure in the body. Measured by echocardiogram. Means pulmonary hypertension is bad.

Subsystemic: The blood pressure in the lungs is lower than the blood pressure in the body. Is reported as a percentage of the blood pressure in the rest of the body (Example: 80% of systemic blood pressure).

Commonly Used Medications TPN: A liquid form of nutrition that provides glucose and electrolytes that is given through their central line.

Lipids: A liquid form of fat to help your baby grow.

Dopamine: A continuous medication to help your baby maintain their blood pressure.

Epinephrine: A medication that may be given in the delivery room to help increase your baby’s heart rate or provided continuously to help maintain their blood pressure.

Milrinone: A medication given continuously that help’s your baby’s heart contract more efficiently, especially when it must work harder against higher pressures in the lungs.

Fentanyl: A medication given either continuously or intermittently to help decrease pain and keep your baby comfortable.

Versed or Ativan: Medications given to help keep your baby sedated which means they will be sleepy so that they do not move around a lot and fight the machines needed to help them breathe.

Precedex: A medicine given continuously to help keep your baby calm and comfortable so that the medical machines can work to help them breathe.

Lasix: A diuretic, which helps your baby make more urine (pee) so that they get rid of extra fluid that might make them look puffy.

Antibiotics: Medications given to treat an infection. May be given before and after surgery to prevent infections. Common antibiotics used are ampicillin, gentamycin and vancomycin.

Sildenafil: A medication used to treat pulmonary hypertension by relaxing the blood vessels in the lungs but not the rest of the body. Given through a vein continuously or given into the stomach through a nasogastric tube or G-tube.

Flolan: An inhaled medication provided through the ventilator that helps treat severe pulmonary hypertension.

Hydrocortisone: Due to the stress babies go through when they are born they often have low cortisol levels, which is an important hormone made by the body.

Hydrocortisone is the medication version of cortisol that will be given to help your baby regulate some of their systems such as maintaining a good blood pressure.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

Electrolyte Replacement: Your baby needs many different electrolytes for their body to work properly. Sometimes when they are very sick they use these electrolytes faster than their body can keep up with so we have to give them extra. The most common electrolytes given are calcium and potassium.

Prostaglandin (PGE’s): When babies are born, there is a small opening off their heart called a Patent Ductus Arteriosus (PDA) that closes after birth. If your baby has a possibility of heart defects or very bad pulmonary hypertension when they are born, we may want to prevent the PDA from closing by giving a medicine called Prostaglandin.

Appendix A: CDH Respiratory Management Pathway

Intubation in Delivery Room

Conventional Ventilation

CDH Repair

Wean ventilator settings: rate, PEEP, pressure support, oxygen

Remove breathing tube.

Go on CPAP or RAM cannula

Switch to High flow nasal cannula, and wean down to 1 L

High Frequency Oscillation

Once lungs are open on x-ray and on low oscillator settings can switch to conventional ventilator

ECMO: If your baby requires ECMO they will still be on a ventilator. They may have their CDH repair while on ECMO. In order to come off ECMO we will look at x-rays, wean ECMO flow, and do clamp trials to determine when your baby is ready to come off.

If unable to come off ventilator or CPAP, airway consultation for Tracheostomy

Transition to home ventilator before going home

Wean to low flow nasal cannula. Can go home.

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CONGENITAL DIAPHRAGMATIC HERNIA FAMILY HANDBOOK

Discrimination is Against the Law. Children’s Hospital Colorado complies with applicable Federal civil rights laws and does not discriminate on the basis of race, color, national origin, age, disability, or sex. Children’s Hospital Colorado does not exclude people or treat them differently because of race, color, national origin, age, disability, or sex.

Children’s Hospital Colorado provides free aids and services to people with disabilities to communicate effectively with us, such as: Qualified sign language interpreters, written information in other formats (large print, audio, accessible electronic formats, other formats).

Children’s Hospital Colorado provides free language services to people whose primary language is not English, such as: Qualified interpreters, information written in other languages.

If you need these services, contact the hospital main line at 720.777.1234.

If you believe that Children’s Hospital Colorado has failed to provide these services or discriminated in another way on the basis of race, color, national origin, age, disability, or sex, you can file a grievance with: Corporate Compliance Officer, 13123 E 16th Avenue, B450, Aurora, Colorado 80045, Phone: 720.777.1234, Fax: 720.777.7257, corporatecompliance@childrenscolorado.org. You can file a grievance in person or by mail, fax, or email. If you need help filing a grievance, the Corporate Compliance Officer is available to help you.

You can also file a civil rights complaint with the U.S. Department of Health and Human Services, Office for Civil Rights, electronically through the Office for Civil Rights Complaint Portal, available at https://ocrportal.hhs.gov/ocr/portal/lobby.jsf, or by mail or phone at: U.S. Department of Health and Human Services 200 Independence Avenue, SW Room 509F, HHH Building Washington, D.C. 20201 1-800-368-1019, 800-537-7697 (TDD) Complaint forms are available at http://www.hhs.gov/ocr/office/file/index.html.

Children’s Hospital Colorado complies with applicable Federal civil rights laws and does not discriminate on the basis of race, color, national origin, age, disability, or sex.

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NICU_160138133_2018-07_Congenital Diaphragmatic Hernia Parent Handbook-Fore Foundation