Robby's Story: Saving Babies With CDH - NewsChannel5.com | Nashville News, Weather & Sports

Robby's Story: Saving Babies With CDH

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CINCINNATI, OH (Ivanhoe Newswire) - Most babies have less than a 50 percent chance of surviving a condition that's diagnosed before they're even born. But delaying necessary surgery to treat the problem is greatly improving those odds.

Robby Dumford is on the move now but his mom, Barbie, said the tenacious toddler almost didn't make it out of her womb alive.

"It was a really hard time," Barbie Dumford told Ivanhoe.

Robby had congenital diaphragmatic hernia. It's a hole in the diaphragm that causes the organs to crowd up into the chest, so the lungs can't develop. It was one of the worst cases doctors had seen. Robby was given just a 20 percent chance of survival, and his mom had to decide whether to terminate the pregnancy.

"I remember just praying the whole time down at the hospital you know, please give us a sign," Barbie said.

The sign she believes, were the ultrasounds, where it looks like Robby is smiling and giving the thumbs up.

"Right then and there I knew that we were going to fight," Barbie said.

The fight brought the family to Cincinnati Children's Hospital.

"As our reputation has grown we are getting more of the severe infants," Paul Kingma, M.D., Ph.D., an assistant professor and neonatal director for the fetal care center at the Perinatal Institute Cincinnati Children's Hospital said.

Traditionally, the diaphragm is repaired within a few days of birth. The team waits up to six weeks to perform surgery. They also aggressively treat the baby's high pulmonary blood pressure with medication to give the lungs more time to grow.

"The benefit of that, by waiting is that when you take them to surgery and you are putting them through this stressful thing, their lungs are bigger, their pulmonary hypertension is less," Dr. Kingma said.

With the traditional approach CDH survival rates are about 42 percent in the U.S, for babies undergoing the newer treatment that number climbs to 75 to 90 percent.

As for Robby, his surgery was a success. Like most children with CDH, Robby has had trouble with acid reflux and has a higher susceptibility to heart and lung problems as well as developmental delays.

RESEARCH SUMMARY

BACKGROUND: A congenital diaphragmatic hernia (CDH) is when there is an absence of a child's diaphragm, or a hole in their diaphragm. This most commonly occurs on the left side, but can occur on either the left or right side. The contents of the child's abdomen, including their stomach, intestines, liver and spleen may go through the hole and into his or her chest. This prevents the normal development of the lung on that side, and may affect the growth of the other lung. (SOURCE: www.childrenshospital.org)

TYPES OF HERNIAS: There are two types of diaphragmatic hernias; the most common kind is a Bochdalek hernia. This type of hernia occurs when the stomach, intestines, and liver or spleen usually move up into the chest cavity because of an opening on the back side of the diaphragm. The second type of hernia- a Morgagni hernia- is considered to be rare and involves an opening in the front of the diaphragm, just behind the breastbone. With either of these hernias the liver or intestines may move up into the chest cavity. (SOURCE: www.childrenshospital.org)

CAUSES: A diaphragmatic hernia is caused by the incorrect joining of structures during fetal development. As a result, the abdominal organs such as the stomach, small intestine, spleen, part of the liver, and the kidney appear in the chest cavity. Therefore, the lung tissue on the affected side is not allowed to completely develop.

Most diaphragmatic hernias affect the left side. Having a parent or sibling with the condition slightly increases your risk. (SOURCE: health.nytimes.com)

SYMPTOMS: Some symptoms a child with diaphragmatic hernia may experience are ineffective movements of the diaphragm. Crowding of the lung tissue may also occur causing the infant to have severe difficulty breathing shortly after birth. The baby may turn bluish in color, have a rapid heart rate, and experience rapid breathing. (SOURCE: health.nytimes.com)

LATEST BREAKTHROUGHS: The method of treatment for infants with CDH is for a doctor to perform a surgery which places organs into the correct place and then closing the opening made in the diaphragm. Breathing support is needed after surgery. Sometimes, a heart/lung bypass machine is also used to help the lungs heal. The outcome of surgery depends on how well the baby's lungs have developed. Doctors at Cincinnati Children's hospital have shown that it is better to wait up to 6 weeks before performing the surgery. They have also found success by treating the baby's high blood pressure with medication. By waiting to perform surgery they allow the baby's lungs to grow, which has been shown to greatly increase their chance of survival. Mortality has decreased thanks to the Cincinnati Children's team, but CDH deaths continue to remain high with a mortality rate of about 20% to 60%. (SOURCE: www.cincinnatichildrens.org, www.ncbi.nlm.nih.gov)

INTERVIEW

Dr. Paul Kingma, Neonatologist, Co-Director of the Fetal Care Center talks about congenital diaphragmatic hernias.

Can you talk about the scope of the problem from Congenital Hernia?

Dr. Kingma: Congenital Diaphragmatic Hernia is an anatomical problem where infants very early on in development have a hole in their diaphragm. As a result of this defect, all of their abdominal organs, such as the intestines, stomach, and liver, herniate up through that hole which compress the lungs and inhibits lung development during the very critical early phases of lung development. So they end up with two big problems, the first is that they will have smaller lungs which is obviously not good and the second and more critical problem is that they end up with pulmonary hypertension. Which are basically smaller and more constricted blood vessels in the lungs restricts blood flow through the lungs then after they're born.

So obviously this can be deadly?

Dr. Kingma: Yes very much so. Unfortunately it is still in some cases, the severe cases it's still a lethal condition. We have significantly improved how we've taken care of these infants in the past decade, but yes they're still unfortunately are a lot of infants that die from Congenital Diaphragmatic Hernia.

I was told that basically you've been able to double the survival rate here versus nationally.

Dr. Kingma: Yes we have significantly improved our survival rates and really the trend started to shift in the positive direction around 2000. And that was with the institution of the CDH team here and the changes to our approach to the CDH babies at that time. We've really seen a significant improvement in the outcome of these infants. During that time we've also taken care of more and more complex CDH infants as our reputation has grown and referring hospitals are sending us the more severe infants. So we've actually improved our survival rates while facing even more challenging cases.

What exactly have you done to improve survival to make it a better institution?

Dr. Kingma: The first is the CDH team and I would say that's the most important thing that we've done. This is a fairly rare condition, so as a normal neonatologist you wouldn't see it very often during your career. So we decided that if we had a single group of select physicians that would agree to basically see all of the CDH babies and take care of these infants from the very beginning of their evaluation during the pregnancy, during their entire hospital stay, and even during their follow up care, basically take care of these infants from day one to the very end, two things would happen if we did that. One we would get to be very good at taking care of these infants. All across medicine we know that the more you do something the better you get at it. And so if we had a select group of physicians that were willing to dedicate their time and see these infants every day and follow them from the very beginning to the very end that this group of physicians would significantly improve their skills at taking care of this type of infant. And the second thing with the CDH team is that we would improve the continuity of care. So I know exactly what happens to my CDH infants all the time and I know what happened to them ten days ago and I know what happened to them a hundred days ago and everybody on the team knows that as well. The CDH team is not only a dedicated group of physicians but we also have a dedicated group of nurse practitioners that follow these infants from the beginning to the end. Then we also have a specific group of nurses and a specific group of respiratory therapists. So all aspects of the team is a group of people who basically said we want to do a better job of taking care of these infants and we're willing to follow them from beginning to end and see them every day that they're here. And that's really made a big difference. The second method that we've done is we have taken a very aggressive approach to treating the pulmonary hypertension. Because that's really one of the biggest problems that they face after they're born. So we now start our infants on anti-pulmonary hypertension medications like inhaled nitric oxide and inhaled prostacyclin and we really are aggressive about treating their pulmonary hypertension. And that pays dividends in the long run. The third method that we've done with the CDH team is we've taken a delayed approach to repairing the defect in their diaphragm. Historically the feeling was that the faster you fixed the diaphragm the better off the babies were. But what we've determined is that once they're born the damage to the lung is already done and fixing the diaphragm is just housekeeping. It doesn't really fix the lungs it just moves things around and it doesn't fix the lungs. We've found that if you treat them with anti-pulmonary hypertensive medications and just give them supportive care for a long time the lung actually grows. And it remodels and those blood vessels improve and the pulmonary hypertension decreases even though the intestines and everything else are still in the chest. And the benefit of that by waiting is when you take them to surgery and expose them to this stressful procedure, their lungs are bigger and their pulmonary hypertension is less and so they have less underlying disease during the stressful surgery and so they do better during the surgery and recover better after the surgery. So whereas other institutions would repair in the first twenty four hours or in the first three to four, maybe five days we'll take as long as a month and a half before we send them to surgery. And what we've found is they just get better and better during that waiting period even though their diaphragm isn't repaired.

Because their lungs have had a chance to grow?

Dr. Kingma: Right, because their lungs have had a chance to develop during that time. And the fourth strategy that we've taken is the EXIT to ECMO approach which we are doing in our very severe cases. So if we evaluate an infant during the pregnancy, during the fetal period and we know that this infant has a really bad Diaphragmatic Hernias and their lungs are very small, we know that they are going to have a very difficult time when they are delivered. Therefore we try to give them maximum support when they are in the delivery process before they come off of the support of the placenta. During the delivery, when they are still being provided oxygen and everything else from the placenta like they would be when they were still in utero, we transfer them to a ECMO circuit which is basically for simpler terms is a heart-lung bypass machine. What that does is it eliminates that stressful period of delivery and then we support them on this ECMO circuit for usually two to three weeks. This gives them again more time for their lungs to grow and develop in the absence of any significant stress. The last strategy has been our approach to ventilation of the infant's lungs. Even though these infants often require significant support on the ventilator in order to survive, we know that the ventilator can damage the delicate lung tissue. Therefore we always strive to use a gentle ventilation strategy so that we expose the infant's lungs to the absolute lowest level of ventilator support needed. We have also started using a new mode of ventilation over the past 3 years that achieves better oxygenation and ventilation while using pressures that are 10-20% lower than pressures required with conventional ventilation. So those are the main things that we've done to try to improve the biggest factors we've had in improving the outcome of our CDH infants.

Can you talk about your partnership with the fetal care unit?

Dr. Kingma: The CDH team was actually created in 1999, and then in 2003 the fetal care center was created. What happened then with the creation of the fetal care center and the partnering between the CDH team and the fetal care center was that instead of becoming involved with these infants just at the time of their delivery and essentially coming in as a neonatologist and saying here's the infant see what you can do. Now because of the fetal care unit we evaluate these infants and we come up with a treatment strategy during the pregnancy. We can see how the lungs are changing during the pregnancy and we can measure the size of their lungs, we can get some predictive markers to determine how well the infant will do. Most importantly, we can get to know the family and the infant and formulate the best treatment strategy for that infant long before the infant is actually born. We can be ready with the EXIT to ECMO team or whatever else might be needed for this infant at the time of delivery rather than scrambling to bring things together at the last minute.

FOR MORE INFORMATION, PLEASE CONTACT:

Paul Kingma MD, PhD
Cincinnati Children's Hospital Medical Center
(513) 803-0957
paul.kingma@cchmc.org

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