The diaphragm forms at about 8 weeks gestation, and in babies with CDH, for some reason the diaphragm does not form properly. Sometimes it is almost entirely absent, and sometimes there is just a small hole. the hole can be found on either side of the diaphragm, but left-sided CDH is much more common than right-sided, and the survival rate for left-sided is higher. Internal organs that normally rest in the abdomen come up through the hernia and compress the lung(s) and heart. This prevents the proper growth of the lungs, especially on the side where the hernia has occurred. In right-sided hernias it is more likely that the liver is up in the chest, and in left-sided hernias it is more likely that the stomach, bowels, spleen and kidneys have traveled up.
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| Illustration courtesy of www.cdhresearch.org |
Ideally, this defect is found on ultrasound before the birth. However, when it is seen very early in the gestation that can sometimes mean that the hernia is large, since the organs are already shifting up. Typically when it is detected via ultrasound, it is because the stomach is seen up in the chest cavity. The position of the heart is also a good indicator, because when other organs float up into the chest cavity, the heart is often pushed to the opposite side. Heart defects and many other problems often accompany diaphragmatic hernias. Oftentimes an amniocentesis and other genetic testing will be performed because CDH is sometimes linked to a chromosomal abnormality.
It is very hard to have a good idea of how well a baby will do after they are born with this a diaphragmatic hernia. If a chromosomal abnormality is found, the chances of survival go down. Sometimes an MRI is done, as that is the best indicator of how many organs have shifted up through the hole. Some babies appear to have a lot of lung tissue, but they sadly do not survive. Others appear to have no lung at all, but they are somehow able to thrive. The size of the hole in the diaphragm can't be determined until the corrective surgery is performed. This usually occurs within the first week of life.
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| Rivers several hours after he was born |
CDH babies generally spend a lot of time in the NICU after they are born. Some even spend a year or more at the hospital. They are intubated (put on a ventilator that does most or all of their breathing) and they can only get nutrients through an IV until after their surgery is completed. After the surgery, if things have gone well, the baby must learn how to breathe and eat on their own,and their pulmonary hypertension needs to be under control. This is a very hard task after all that they have been through, and many babies need supplemental oxygen, and some need feeding tubes for quite some time after they are discharged from the hospital. After they go home, they should be kept away from any and all illness, as their lungs are still usually smaller than normal, and any respiratory disease can send them straight back to the hospital.
Rivers is not a typical CDH case. The fact that he currently has no further complications from his hernia is a complete miracle. The majority of babies born with this defect who survive have a very rough road ahead. We are EXTREMELY lucky and VERY blessed that, despite a rough start, Rivers has shown no other delays in growth and development so far. There is still a chance that he can re-herniate in his lifetime (some babies re-herniate many times, and the corrective surgery has to be re-done each time.)
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| Rivers at 3 months old |
For more information about diaphragmatic hernias, check out CHERUBS, the amazing non-profit organization working very hard to find the cause of this defect. It is still undetermined whether or not the cause is genetic or environmental, but if you have had a child with CDH in the past, your chances of having another jump to 1 in 50 (so from 0.0004% up to 2%.) It is our hope that someday the cause will be determined and that most cases may be prevented!
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