At the hospital - CDH Australia

Babies with Congenital Diaphragmatic Hernia (CDH) will respond to life outside the womb in different ways. It is not always predictable how a baby will respond after birth, so the below is simply a guide for the most common steps that many CDH babies undertake. All babies born with CDH need to be managed in a Neonatal Intensive Care Unit (NICU).

Because a baby with CDH has underdeveloped lungs, they need to be put onto a breathing machine straight after birth (this process is known as intubation). The baby is then stabilised and put on drugs such as morphine to keep them calm and out of pain.

NICU

Depending on where you are located during the birth, the baby will be transferred to a Neonatal Intensive Care Unit (NICU) so that they can be stabilised. CDH needs to be managed in a NICU with nurses, physicians and surgeons who specialise in the condition. In Australia this may mean your baby will need to be moved in the first 24 hours from a NICU in the birthing hospital to a NICU in a Children’s Hospital.

When in NICU, babies have one-to-one care where they are very closely monitored 24 hours a day by specialised medical and nursing staff. Babies will be placed in special humidicribs where they are kept warm. Babies will receive help from various machines, with a number of tubes and attachments to their body – these will likely include: breathing tube through their mouth that is connected to a breathing machine (ventilator), a nasogastric from their nose to their stomach to decompress gas in the stomach and later supply vital nutrients (CDH babies are not able to initially breast or bottle feed), a heart and breathing monitor, a number of different types of cannulas (small plastic tubes) placed into their veins and arterials for blood tests, giving fluids and medications.

Download: NICU tips

Surgery

After a baby is stabilised, the next step to recovery is usually surgery. The average time for a baby to have surgery is 3–10 days. The surgery itself usually takes around one hour, however the entire process of preparing the baby and having the baby come out of surgery is around three to four hours. Parents are usually advised to remain close to the hospital during this time.

Surgery involves opening up the baby’s abdomen around the ribcage and moving the abdominal organs back to the abdominal region. Then the hole in the diaphragm will be repaired. Depending on the size and location of the hole, surgeons may either close the hole by suturing the ends of the diaphragm together, or use a patch made of a special material. Although uncommon, sometimes the abdomen may not have enough room to house the organs straight away. In these cases the baby will need to have abdominal organs outside of the body until the abdomen grows. A second surgery will then be conducted to replace the abdominal organs in the correct location.

Sometimes surgery is performed by making an incision (cut) along the skin under the ribs (called an open repair), at other times the surgery may be done via key-hole surgery with a smaller incision. There are pros and cons of each type of CDH repair, and your surgeon will discuss their choice and reasons with you before the surgery.

After surgery it is important that the baby has time to re-stabilise. Parents are usually able to visit their baby a few hours after surgery is complete.

Breathing

Once the baby’s organs are in the correct place after surgery, the next big step towards recovery is to have the baby breathe on their own. Again, each baby is unique and not all babies follow a standard path. Some babies may skip a step, some may go back and forward between steps, some babies will require long-term breathing assistance, some babies may require on and off breathing assistance. When your baby is in hospital, your medical team will be able to talk you through your baby’s requirements and situation in detail.

The most common steps to help a baby breathe on their own involve:

Intubation / mechanical ventilation
This is where a tube is inserted into the baby’s mouth and windpipe so that a special machine can move air in and out of the baby’s lungs. A baby will very likely be intubated straight after birth and this will remain post surgery. There are a number of different types of mechanical ventilators (breathing machines) that your baby may receive. The most common type is called conventional mechanical ventilation where the doctors try to support regular breathing patterns. Some babies will receive high-frequency oscillatory or high-frequency jet ventilation. These are specialised types of mechanical ventilators which give very small but very fast inflations (240–900 breaths a minute) to the lung (like a dog panting). They are usually used if the doctors are worried about lung injury or a baby needs a high amount of oxygen. The choices around breathing machines can be complex so make sure your doctors and nurses explain them and how your baby is responding.

Continuous Positive Airway Pressure (CPAP)
CPAP is breathing assistance that uses air pressure to keep airways open without a breathing tube. A soft face mask or small rubber prongs are placed in the nose and additional pressure and oxygen placed into the back of the mouth to help babies whilst they are breathing. This is the type of breathing assistance used during sleep by people with sleep apnoea. CPAP is usually used once a baby can breathe without a tube.

High flow / Low flow oxygen
Respiratory support usually through a small nose prong. This air support reduces the work of breathing and allows additional oxygen to be given.

Breathing on their own
No breathing support required by the baby. Some babies will continue to require ongoing breathing assistance after leaving hospital. If your baby requires this, the hospital will coordinate training and equipment.

Heart

Many babies born with CDH have heart problems in the first few weeks after birth. Your baby will have continuous monitoring of oxygen levels and blood pressure, and at least one heart scan (called an ECHO). If your baby has any heart problems it is likely that a group of medications called inotropes will be started. There are different types of inotropes that maybe used depending on the type of heart problem. All are given via a continuous intravenous infusion. Many babies also need to have a gas called nitric oxide added to the oxygen. Nitric oxide helps relax the blood vessels in the lungs.

Feeding

Once a baby is able to breathe on their own, the next hurdle is to introduce feeding.

Post birth and until surgery, your baby cannot feed – instead your baby receives vital nutrients via a Total Parental Nutrition (TPN) tube. After surgery, your baby is monitored to see when it is appropriate to introduce feeding – usually once the intestines appear to be ready. Once ready, a tube via mouth or nose will be inserted that leads directly to the baby’s stomach. It is through this tube that breast milk or formula can be given to the baby. This feeding tube is necessary while the baby requires respiratory support or if they are having trouble feeding orally.

Again, each baby is different. Feeding can be a difficult process for some babies; it can be very tiring for them and patience is required. Breastfeeding, bottle feeding with expressed breast milk, formula or feeding tube are all ways for your baby to get the vital nutrients they require. Hospitals will have staff to assist parents with the feeding process.

In many cases, breastfeeding is possible if the mother is able to, and chooses to do so. After giving birth, midwives at the birth hospital should assist in teaching mothers how to express milk from the breasts by hand and by machine. Hospitals should be able to supply mothers with the equipment they will need for this, but it may be worth looking into hiring or purchasing a breast pump if you are wanting to breastfeed or provide expressed breast milk. If you would like to breastfeed or use breast milk, it is important to keep milk supply up by regularly expressing milk. Please talk to a lactation consultant or midwife at the hospital about this. It is important to remember all women are different and breastfeeding is not always viable or the right option for every mother.

Download: Feeding your child with CDH

Parental care and interaction while at hospital

Depending on how stable your baby is, you will likely only be able to look at, sit next to and touch your baby with your hands when they are first taken to NICU. Holding your baby will be dependent on how stable they are and what your medical team thinks is best for the baby at various stages of their journey.

It is a great idea to interact with your baby as best you can while they are in hospital. To begin with, touching may be limited but you can still talk to your baby, read your baby books and be present for them. Most hospitals will work closely with parents to ensure parents are actively involved in their baby’s care – this may mean cuddles, skin to skin contact, feeding, changing diapers, changing bedding, washing and cleaning your baby, or undertaking milk therapy.

Many babies will not open their eyes for the first while as they will be sedated and likely bloated or puffy from medication and surgery. Due to their breathing tube, babies will not be able to use their vocal chords, so you will not hear your baby crying to begin with. But your baby will know you are there with them and will be comforted by your presence and love.

Discharge

At various stages above, as your baby progresses they might be moved from one-to-one NICU care to less intensive care. Your hospital team will talk you through this process. In order for your baby to be discharged and taken home, the hospital team will be looking to ensure the baby is feeding and gaining weight, breathing comfortably and generally doing well.

Going home

This is usually as daunting as it is exciting. When the time comes to finally take your baby home, it could be any variation of scenarios: no breathing assistance and no feeding tube to some assisted breathing or feeding. If any assistance is required your hospital team will ensure you are fully trained to handle your baby’s needs. Follow up care will be coordinated between the hospital staff and parents. This could include at-home visits and scheduled hospital follow ups.

The organisations listed below provide specialised support for children who need special care at birth and beyond.

Life’s Little Treasures Foundation
Australian charity dedicated to providing support, friendship and information, specifically tailored for families of premature or sick babies.

Miracle Babies Foundation
Miracle Babies Foundation provides vital programs and resources to support and enhance a family’s experience from a threatened pregnancy, hospital journey with a premature or sick newborn, the transition to home and beyond.

What do I need at home?

To get your baby home from the hospital you will need a professionally fitted baby seat in your car, or a suitable pram/bassinet for other modes of transport. At home you will need the same set up as any other baby – a safe sleep environment, feeding equipment (if not exclusively breastfeeding), clothing, change table etc. If any medical equipment is required, this will be discussed and coordinated before your baby is discharged from the hospital.