What is CDH?
Congenital diaphragmatic hernia (CDH) occurs in an unborn baby when there is a hole in, or incomplete formation of, the diaphragm (the muscle under the lungs that is responsible for breathing). This hole allows the abdominal organs (eg. spleen, liver, intestines, stomach) to move into the chest, and prevents the lungs from developing normally because of the lack of space to grow. The defect can occur on the left side, right side, or both sides. The defect is usually discovered on ultrasound before the baby is born.
CDH affects about one in every 2,500 babies (1600 cases in the U.S. each year), and occurs as often as cystic fibrosis and spina bifida, yet most have never heard of it. On average, only 50% of these babies survive.
How often do I need to meet with my physician during pregnancy?
It is important that you consult with your doctors – they will closely monitor your pregnancy, with frequent exams especially in the last trimester. This close monitoring will help them determine if it is necessary to deliver early.
Do I need to deliver via C-Section or is a vaginal delivery safe?
This question is best answered by a maternal-fetal medicine specialist, but there isn’t any reason you can’t have a vaginal delivery - as long as your baby isn’t showing signs of distress or other complications that would make a vaginal delivery unsafe.
What should I expect the day my baby is born?
At birth, your baby will most likely need immediate resuscitation and ventilator help. A breathing tube is inserted into your baby’s airway so a ventilator can be attached to help with breathing. A tube going from the mouth or nose to the stomach will be placed to keep air from getting into the stomach and intestines causing less space for the lungs to expand. Intravenous and arterial lines will be placed in blood vessels of your baby’s arms, legs or belly button for administration of fluids and medications. Blood gases will be drawn from the arterial line to assess how well your baby can oxygenate, and determine the level of support your baby needs at that moment.
What is ECMO?
Extracorporeal membrane oxygenation, also known as ECMO, is a temporary heart-lung bypass technique used to oxygenate the blood and allow the lungs to rest. Under sterile conditions at the bedside and once your baby has received pain medication, the pediatric surgeon will place two tubes called cannulas into the artery and vein in your baby’s neck. The tube in the neck takes blood out of the body from the large vein, oxygenates the blood through the ECMO circuit and returns the now oxygenated blood to the baby by the carotid artery. ECMO is used when other treatments are unsuccessful. The lungs rest as the ECMO circuit does the work. In some cases, the baby may have the CDH repair while on ECMO. ECMO can have serious complications including bleeding and infection. Careful monitoring by an experienced ECMO specialist is critical.
Can I breastfeed my baby?
Pumping is always encouraged and there are numerous studies showing the benefits of breast milk. Once your baby shows signs of a normal healthy digestive system, and is receiving oxygen through a regular nasal cannula, you will typically get the go ahead to breastfeed. This can sometimes be a challenging part of the hospitalization. There are lactation specialists who can help guide you through this process and show you how to use pumps at the hospital. Many mothers have been able to get their baby to breastfeed, even after waiting as long as 9 weeks to attempt. Many others do just fine with a bottle.
I am worried about my baby getting sick. How do I let people know to stay away when they are sick?
This can be a sensitive subject since so many people will want to see your baby. It is a good idea to let people know before you come home what your expectations are. Let others know of the challenges of keeping your baby healthy, and how something like a common cold could result in hospitalization, especially during these first few years of life. Most people will respect and understand your wishes.
What side effects are common with CDH survivors?
There are many CDH children carrying out very normal lives, unbeknownst to the rest of us. The side effects can vary from child to child, and may diminish throughout the child’s life. Some common issues that can be present are: difficulty with eating, reflux (GERD), asthma, scoliosis, and developmental delays related to the life-saving procedures your child required to survive. Some require medications and oxygen after leaving the hospital.
What long-term outcomes can I expect for my baby?
The prognosis for CDH babies largely depends on the severity, more specifically whether the liver is up or down. When the liver is up, the lungs are even more crowded and smaller at birth. The outcome from your baby is also greatly influenced by the doctor and hospital where you choose to have your baby cared for. There are important factors to consider when choosing a team to take care of this very serious birth defect, and many places do not have necessary equipment, like ECMO machines, or very experienced CDH doctors to make critical decisions with regard to your baby's plan of care.
How is CDH evaluated and diagnosed?
Typically CDH is discovered on ultrasound, and less often after birth. Once discovered, parents are referred to Maternal Fetal Medicine for more evaluation and more diagnostic tests. A fetal echocardiogram will be ordered to evaluate the baby’s heart, and a fetal MRI to determine which organs, specifically the liver, are in the baby’s chest. Sometimes doctors can also get an idea of your baby’s lung size, though often it is difficult to see with all the organs in the way. High resolution ultrasounds will be performed numerous times to determine the lung to head ratio and look at other important factors. Genetic testing may also be offered to determine if there are any other defects that would affect your baby.
What is fetal intervention?
Some hospitals are offering fetal intervention as an option for very severe cases of CDH. Commonly knows as “tracheal occlusion,” this procedure is FDA-approved, but still in clinical trials, and considered experimental. Consult with your doctor for questions about the risks and potential benefits of this procedure.
About how many weeks gestation will I be when they schedule my induction or delivery?
Typically, an induction will be scheduled around 38-39 weeks gestation, but this will be decided between you and your physician.
How long after birth will my baby need surgery?
While there is no definitive answer for every baby, surgical timing is one of the most important decisions in your child’s outcome. Surgical repair of CDH after delivery depends on your baby’s progress.
What will happen to my baby during surgery?
Your baby’s repair will most likely be done in the ICU, because CDH babies are not stable enough to transport to the operating room (OR) for the procedure. Essentially, the OR will “come to” your baby. Your baby will receive general anesthesia and will be continually monitored by a pediatric anesthesiologist. An incision is made just below your baby’s rib cage, the organs in the chest are guided back down into the abdomen and the hole in the diaphragm is sewn closed. Length of stay in the NICU can vary widely and will be determined by needs for ECMO, ventilation support, and how well your baby eats.
I have so many records for my baby. How do I keep track of them all?
Many parents find it helpful to have a notebook or binder that contains all of their child’s most important records from the hospital stay. As you establish who your child’s regular doctors will be, you can let them make copies of your records, and add to them as your child gets older. Speak to the discharge planner at the hospital and he/she can help.
I am overwhelmed with the thought of leaving and taking care of my baby by myself. Any tips?
Going from a highly-structured environment where you have the support and resources of the hospital staff and doctors to your own home can definitely feel overwhelming. Try to take it easy as much as you can when you first get home and let family and friends help with anything they can like meals, laundry, grocery shopping, taking other children to school, etc. You will need to be patient with yourself as you establish your own routines and get comfortable caring for your baby and the increased needs he or she may have.
What if I live far away and need to fly home with my baby on oxygen?
Flying with oxygen can feel like a hassle, but there are companies that help coordinate this with the airlines to make sure your oxygen device is approved on your particular aircraft. You cannot use the tanks on the airplane, and will need to rent a portable oxygen concentrator instead. When you arrive, a home oxygen company will bring your supplies for your setup and make sure you know how to use your equipment.
I am worried about RSV. Can my baby get a vaccine to help protect him/her?
This is really up to your insurance company. Some companies are easier to work with than others. There are pharmacy assistance programs that can sometimes help supplement the costs of this expensive shot. Speak with your office manager at your pediatrician’s office and they can guide you through the process if your request is appealed and denied.