Standard of Care
The following procedures may or may not apply to your case. I have tried to encompass everything that *may* happen but this is by no means meant to be comprehensive.
The big day is here! Your baby arrives. So what’s next from a medical standpoint?
You should be fully informed and educated by your medical staff before any procedure takes place. You will be asked to sign consent forms. The doctors and nurses will answer any questions that you have and there is no such thing as a stupid question. You NEED information in order to make the best choices for your particular situation.
If your baby’s diagnosis was made prior to birth, he or she will probably be intubated immediately after birth. This is necessary to help the child breathe and compensate for small lungs and poor lung function.
Intubation involves the insertion of an ET (endotracheal tube) in the throat. Tubes leading to a mechanical ventilation machine can then be attached to the ET tube or a hand bag can be attached. It is most likely that the baby will be hand bagged in the delivery room and switched to either a conventional ventilator or to a high frequency oscillatory ventilator (HFOV) in the shortly thereafter or in the NICU.
A diagnosis may not be made until birth. Often the first clue that anything is wrong is difficulty breathing after birth. In that instance, intubation will occur quickly as well.
Your baby's heart rate, respiration rate, oxygen saturation levels and blood pressure will probably be continuously monitored and visible on a monitor (or monitors) by the bed. If you are unsure of what you are seeing, ASK! You will become very familiar with this monitor as it gives you a concise picture of how your baby is doing overall. It is sometimes difficult to tear your eyes away from the monitor. Our surgeon told us that he was going to cover it up because we were constantly watching it! Just keep in mind that these numbers do not tell the entire story of how good or bad your baby's health is.
If fluid or air is accumulating around the lungs, a chest tube (or 2 or 3) may need to be inserted. This is a minor surgical procedure inserting a tube through the chest wall and ribs. The doctor will decide how deeply and where exactly in the chest it needs to be inserted and may use a few stitches to keep it in place. A dressing will be applied over the insertion site. The tube can be set to one of three setting: gravity, positive pressure or suction and it employs a water seal to drain off fluid or pull out air without returning air to the chest cavity. Additional information on how chest tubes work can be found here.
Do not be alarmed to see one or two lines extending out of the umbilical cord stump (belly button, navel). In newborns, this is a fairly standard procedure. These lines allow direct access to an artery and vein and by leaving these lines in place as long as possible, repeated needle sticks can be avoided. Blood is taken from these lines for blood gasses and they have additional uses. The umbilical line is removed when the umbilical cord stump falls off.
If an umbilical line cannot be inserted for some reason or after the umbilical cord stump falls off, a PICC line may be inserted. PICC stands for peripherally inserted central catheter. It is inserted in a vein, usually the arm but it can be inserted elsewhere, and a thin, flexible catheter is threaded through the vessel until it rests in the superior vena cava or right atrium of the heart. There are distinct advantages to PICC lines. "It is easy to insert, poses a relatively low risk of bleeding, is externally unobtrusive, and can be left in place for months to years for patients who require extended treatment. The chief disadvantage is that it must travel through a relatively small peripheral vein and is therefore limited in diameter, and also somewhat vulnerable to occlusion or damage from movement or squeezing of the arm (1)."
A central line can appear frightning but it is a great help to the medical staff and easier on the patient. It is much like a PICC line in may respects but it does not use a peripheral vein; the catheter is inserted via a subclavian, internal jugular or femoral artery and threaded into the heart as described above. Because of a more direct access, stronger medications can be administered with a central line than with a PICC line. Also, larger catheters can be used since the vessels used are larger. This means that more fluid and medications can be administered through just one line (1).
Frequent blood gasses will be taken. It is best to use arterial blood but in some instances, this is not possible. Be aware that a blood gas taken from a vein (non-oxygenated blood) is not as precise as one taken from an artery (oxygenated blood). A blood gas can measure many levels but in CDH children, it most commonly measure the carbon dioxide level, oxygen level and ph level in the blood. A normal CO2 reading should be between 35 and 45; oxygen as close to 100% as possible; normal ph levels should be between 7.35 and 7.45. The frequency of these gasses will depend on your doctors and how well or bad you baby is doing.
Inhaled nitric oxide (INO, not to be confused with nitrous oxide) is often used to dilate arteries and increase blood flow to the lungs and organs. The INO machine is hooked up to the mechanical ventilator and administered in that manner. INO is generally used in an attempt to avoid ECMO however, it does not always work and can be used in conjunction with ECMO. You baby will have to be weaned off of INO.
There may come a time when you are presented with placing your child on ECMO. ECMO stands for extracorporeal membrane oxygenation and is a life support machine that allows the hearts and lungs to rest while the machine does the work. It is basically a heart/lung bypass. Much more detailed information on ECMO can be found here.
For a period of time, your child will have a foley catheter inserted into their bladder to remove urine and measure urine output. It is extremely important to accurately measure urine output to see how the kidneys are working. When the medical staff feels that the catheter is no longer needed, it will be removed and urine output will be measured by weighing soiled diapers.
When the time comes for your baby to start eating breastmilk or formula along with their IV nutrition, a feeding tube will be used. There are various types of feeding tubes that can be used but the most common is known as an NG tube, or nasogastric tube. With this type of feeding, a small tube is is put into a nostril and threaded down the throat and esophagus to rest in the stomach. This type of feeding tube is only used short term (2). Another type of feeding tube can be inserted through the mouth rather than the nose, pass through the throat and into the stomach like a NG tube. Your child may very well need a feeding tube long term and that will be different from an NG tube. Additional information on feeding tubes can be found here.
Surgery to repair the diaphragmatic hernia can take place whenever the surgeon feels that the child is stable enough. There are various studies examining late vs. early repair but it all boils down to the child and how they are doing. During surgery, your child will receive a general anesthetic that makes them sleep. They may be hand bagged throughout the surgery. An incision is made in the abdomen and the abdominal contents located in the chest are gently place back into the correct position. Many times the surgeon will take the organs out, examine them for defects and then place them in the correct position. If the hole is small, the diaphragm will be stitched up. If the defect is larger, a patch may be needed. Gore-tex is the most common material used to patch up the hole. Because of the empty abdomen, the organs may be a tight fit. Additional surgeries may be needed down the line.
When your child is able to be removed from mechanical ventilation, they will most likely still receive oxygen via a nasal cannula. This is a minimally invasive method of delivering oxygen via the nostrils. Your child can remain on this indefinitely or may come off of it very quickly depending on how the lungs perform.
---------------------
References