Hernias in newborns: Lincoln’s story

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ST. LOUIS, Mo. (Ivanhoe Newswire) – It’s a condition that’s as common as spina bifida or cystic fibrosis – but you’ve probably never heard of it. Congenital diaphragmatic hernias occur in about one in every 2,000 births. They can be deadly, but now doctors are using a more aggressive treatment approach.

Two-year-old Lincoln Olds is happy and active today.

“He’s just a really fun kid. He likes to make people laugh,” Dorothy Olds, Lincoln’s mom, told Ivanhoe.

But when his mom was pregnant with Lincoln, an ultrasound revealed a devastating diagnosis.

“We just wanted to know if we were having a boy or a girl, that’s what we went to find out, and they told us that our child might not survive,” Dorothy said.

Lincoln had a congenital diaphragmatic hernia – essentially – a hole formed in his diaphragm.

“The intestines as a result of that go up into the chest, and they grow within the chest and actually prevent the lung from developing normally. If they make it to delivery, and they’re alive, the survival rate is still about 60 percent,” Brad W. Warner, MD, Surgeon-in-Chief at St. Louis Children’s Hospital, and Professor of Surgery at Washington University School of Medicine, told Ivanhoe.

Lincoln was placed on a life support machine known as ECMO – to give his body a chance to rest and oxygenate.

At first, he didn’t respond, but after 12 days, Lincoln was breathing better. He then had surgery to reposition his organs and repair his hernia. After 83 days in the hospital, Lincoln went home. Doctors expect him to live a normal life and be a normal kid.

“He’s a wonderful child. It’s really amazing,” Dorothy said.

Lincoln is small for his age. Because he breathes faster, his body requires more calories, so getting him to eat is important. He also takes speech and occupational therapy to address some of the complications of the condition.

Children with these types of hernias sometimes develop heart failure, scoliosis, hearing difficulties, and developmental delays.

BACKGROUND: A congenital diaphragmatic hernia (CDH) is a birth defect that occurs in about one in every 2,500 live births. It's a condition in which a hole in the diaphragm allows abdominal organs to move into the chest, blocking the development of the lungs. The hole develops very early on in fetal development, and most commonly occurs on the left side of the body. The condition can be life-threatening if not treated, because the lungs cannot provide enough oxygen support to the body. However, the survival rate is about 70 percent. (Source: The Children’s Hospital of Philadelphia)

CAUSES: There are many factors that contribute to the cause of CDH. Genetic and environmental factors both play a role in causing CDH. Some doctors think multiple genes from both parents create an unfortunate genetic combination which contributes to the development of the hernia. About 40 percent of CDH cases are associated with other birth defects, the most common being a congenital heart defect. The CDH is repaired after the birth. but in severe cases, if the liver has moved into the chest fetal therapy may be offered (Source: Columbia University)

NEW TECHNOLOGY: An ECMO machine, which stands for extracorporeal membrane oxygenation, is now being used to treat CDH. A catheter is placed in a vein and an artery to give cardiac and respiratory support. The amount of time the infant can stay on ECMO varies, depending on the condition and the recovery of the heart and lung functions. The maximum amount of time is usually two weeks, but some cases have gone longer. Complications from being on the machine include bleeding, infection, stroke, transfusion, or mechanical failure of the equipment. There are a variety of ECMO support groups online which help parents with the complications of having a child on the device. There is also a handy map of ECMO centers all over the world: http://batchgeo.com/map/d4b2377b0b3e2947ab91d80e0ae11423 (Source: www.uwhealth.org)

Brad Warner, M.D., Chief of Pediatric Surgery at Saint Louis Children’s Hospital, talks about a congenital diaphragmatic hernias in children.

What is congenital diaphragmatic hernia?

Dr. Warner: A congenital diaphragmatic hernia is basically when there is a hole that’s in the diaphragm when the baby is developing in the womb; the fetus is developing with a hole in the diaphragm. And the intestines, as a result of that, go up into the chest and they grow within the chest and actually prevent the lung from developing normally on the side of the hole. But it also compresses on the opposite lung and so both lungs are very abnormal. So when babies are born with a diaphragmatic hernia, they have really severe respiratory problems.

And it can it also impact their heart?

Dr. Warner: It can. And there’s an association of congenital diaphragmatic hernia with congenital heart disease as well. But the way it affects the heart really is it causes a very, very high blood pressure within the lungs which then can cause the heart to fail.

Is this is just like a hernia in adults, but because the baby is developing, that’s where it causes all the problems?

Dr. Warner: Exactly. When you think of hernias, you think of hernias that are in the groin or in the abdominal wall. But a hernia on the inside, in the developing fetus, is really a very, very, difficult thing for them to overcome; because the intestines and everything are developing in the wrong cavity and pushing on the developing lungs as well, so there are a lot of dynamic things going on.

How often do you see this?

Dr. Warner: It’s about 1 in 2,500 live births. It’s as frequent as cystic fibrosis or spina bifida; it’s as common as those, but this is something that we don’t talk as much about. And one of the other things that it’s important to realize is that many babies actually do not make it to delivery with this diagnosis. Many babies that are still born or that die in utero have diaphragmatic hernias, so there is a hidden mortality that’s even greater.

So really the problem is you don’t know how treatment is going to help these babies?

Dr. Warner: Right, it’s still about a 60 percent survival rate for babies that are born with diaphragmatic hernias. And again, those are babies that are born alive. A good percentage of babies die before they’re born, so we don’t really know what the exact percentage is, but if they make it to delivery and they’re alive, the survival rate is still about 60%.

How do you treat them?

Dr. Warner: There are many things that we do and many of them really depend on how the baby presents. So sometimes you don’t know that the baby has this condition and they’re born somewhere without any prenatal testing or anything, so it’s just discovered when they’re born as soon as they clamp the umbilical cord, they turn blue. Or another presentation is that the babies do well for a period of time and get sent off into a normal nursery and within about six or eight hours they suddenly start to have increasing respiratory problems. They start to turn blue and then it starts to spiral. Then, very rarely, probably less than 5% of babies, will present and have a diaphragmatic hernia and it never gets discovered until sometime as an older child or an adult. We actually had a case this past year, the 17-year-old girl that was involved in a motor vehicle accident and we got a chest x-ray and it showed her intestines in her chest. Based on the history of trauma, we surmised that she had a traumatic tear of her diaphragm. So we operated on her right away and in fact it was a congenital hernia that she’s had all of her life.

Can you fix this?

Dr. Warner: Yes, absolutely. Actually fixing the diaphragm is not the most difficult part of it. The most difficult in managing these children is really the amount of lung that they are born with. Some babies are born with such a small amount of lung that they’ll never grow that back in time, and those are the babies that no matter what you do, they are not going to do well. Then there are babies that are born with enough lung tissue, but the blood vessels in their lungs are very spastic and they respond by tightening down with response to any kind of stimulus which would include turning the lights on in the room or loud noises in the room.

Is it a short window of time, like 12 hours or 10 hours, that you can actually save those kids who make it through delivery?

Dr. Warner: It depends on how they present. If a baby, whether they’re known to have a diaphragmatic hernia or not, if you clamp the umbilical cord and they turn blue, those babies need immediate attention. One life-saving thing that we can do is put them on what’s called ECMO or extracorporeal membrane oxygenation, and we do an emergency operation. We make an incision in the neck and we put two tubes in the neck, one in the artery and one in the vein and we draw the baby’s blood off, add oxygen to it, take away the CO2, and deliver it back to the baby. So it’s like a heart lung machine. And we can put those babies on within hours of birth. The problem though is, we never really know whether they’ve got underdeveloped lungs that will not be consistent with survival. If that’s the case, they go on ECMO and we end up waiting several weeks and they don’t get better because they’re not growing enough lung. Those are the babies sometimes where we have to terminate therapy because there’s nothing else that can be done.

Will the underdeveloped lungs get stronger and grow?

Dr. Warner: There are two things: there are underdeveloped lungs and there is high blood pressure in the lungs. You can only really do something about the high blood pressure in the lungs. If a baby is born with underdeveloped lungs, it generally is going to take them months-to-years to grow enough lung tissue to be able to survive. That’s too long of a window to allow us to support them. So, babies that are born with underdeveloped lungs from the start are probably not going to do well no matter what.

Do the babies who survive have long term effects from this?

Dr. Warner: They do. From a lung standpoint, it can vary, but there’s no reason to think that if they survive in the newborn period, that they won’t be able to grow lung tissue and function and be normal kids. So, we do provide that as hope for the families, but there are other issues that are associated with diaphragmatic hernias. One of which is the congenital heart disease and that obviously impacts survival. But they also have such things as scoliosis or a curvature of the spine associated with repairing the diaphragm and having them being a little tight on one side relatively to the other. They often times have hearing difficulties; we think that it’s due to the antibiotics and things they’re exposed to during a long course in the hospital.