1. WHAT IS CONGENITAL DIAPHRAGMATIC HERNIA? (CDH)
CDH is a birth defect that affects about 1 out of every 2500 babies. It occurs when the diaphragm either doesn't form correctly or doesn't close, leaving a hole through which digestive organs travel into the chest cavity. The national survival rate for CDH babies is somewhere between 50 and 60%.
2. WHY DID THIS HAPPEN TO JUDE?
There are ongoing research projects trying to find out what causes CDH. All that is known is that something happens between 7 and 12 weeks gestation that stops the diaphragm from closing. So far, no definitive answers as to genetics, heredity, or environmental factors have been found.
3. WHY IS IT SO SERIOUS?
In utero, the baby's heart and lung development are compromised by the organs developing in the chest cavity. CDH can frequently occur with other birth defects-- chromosomal anomalies, heart defects, etc. Or, as in our case, it can be a completely isolated defect not linked to anything.
4. WHAT DO YOU DO TO FIX IT?
The short answer to this is for a surgeon to place everything back into it's correct location and stitch up the hole.
5. THAT SOUNDS EASY.
Unfortunately, it's not. CDH babies are born with severely compromised respiratory and digestive systems. First, before repair surgery can even be discussed, the baby must be stable enough to withstand surgery. A large determining factor is the heart's ability to pump blood into the lungs for oxygenating the body. In utero, this process is unnecessary. Upon a baby's birth, many factors have to line up just right for the heart and lungs to work together. CDH babies have extra hurdles to get to that stage. Post op, the situation can get challenging as well-- just because the hole is repaired doesn't mean the baby can breathe, or eat.
6. WHAT CAN YOU DO BEFORE JUDE IS BORN?
Go to the doctor 1000 times to make sure there are no other complications. Stay pregnant as long as possible. Despite all of the doctor visits prenatally, there are no guarantees or concrete prognosis that can be made until the medical team sees Jude in person.
7. WHAT HAPPENS WHEN HE IS BORN?
He will be immediately resuscitated, sedated, and put on a ventilator. Since it's unknown how well his lungs will function, they will err on the side of caution and breathe for him until they get a better idea of what is going on. At no point do they want Jude deprived of oxygen. After he proves stable in the heart and lung department (i.e. tolerating the ventilator and maintaining good heart function), repair surgery will be done somewhere after 1 week postpartum.
8. WHAT IF HE'S NOT PROVING STABLE IN THE FIRST FEW DAYS?
There are lots of medications and ventilation techniques that will be tried. If those do not help, he will be placed on an ECMO machine. ECMO is the reason we have to relocate to another area instead of staying near Norfolk-- very few hospitals in the country have ECMO and it's vitally important that Jude be as close as possible to an ECMO machine if he needs it. There is also a possibility he might need it after his repair surgery if complications with his heart and/or lungs arise.
9. WHAT IS ECMO?
ECMO (extracorporeal membrane oxygenation) is a last resort intervention for very sick babies. If Jude is unable to oxygenate himself, he will deteriorate quickly. An ECMO machine is basically a heart/lung bypass machine that pulls the blood from his body, oxygenates it, and puts it back in. It's used to give a baby's heart and lungs time to rest and hopefully strengthen while the machine does the hard work. It has GREATLY increased the survival of CDH babies but it has its drawbacks. Being on ECMO for more than 3 weeks can severely damage a baby's brain and body. Blood clots can form inside the machine which spell disaster just as blood clots do for normal people. The bottom line with ECMO is that you really don't want to end up on it. If you do, chances are good that it's going to help the baby recover, it's just going to add some bumps along the way.
10. SO AFTER SURGERY...
A series of baby steps out the NICU door. Step one-- demonstrate ability of heart to function appropriately. Step two-- demonstrate ability of lung(s) to expand, grow, and start functioning on their own. Step three-- breathe without ventilation. Step four-- suffer through Baby Rehab as medications are knocked down and withdrawn. Step five-- demonstrate ability of newly rearranged digestive tract to tolerate feeding. It's a long haul (average 8-12 weeks NICU stay). It's a rollercoaster (these babies can go from thriving to a poor prognosis in seconds).