It's a birth defect as common as cystic fibrosis and muscular dystrophy, yet few people have ever heard about it. Nearly half of the babies with this defect don't see their first birthdays, but now, a drug millions of men are already very familiar with is saving little lives.
Not long ago, Daniel’s mother, Amber Dotson, thought she was going to lose him.
"He couldn't breathe, and everything was shutting down,” said Dotson.
Daniel was born with a life-threatening problem called CDH, or congenital diaphragmatic hernia.
Dr. Brad Warner, a pediatric surgeon at the St. Louis Children’s Hospital, explained, "The intestines go up into the chest, and that compresses on the developing lungs and pushes the heart over to the side and causes the lungs to be abnormally developed."
Before, these babies were rushed into surgery. However, now, doctors realize that does more harm than good, so they're finding less invasive and more unique ways to help.
"One of the ones that's really come along is actually Viagra. Viagra causes relaxation of muscle in different parts of the body, and one of those is the smooth muscle of the lungs,” said Dr. Warner.
In some babies, the little blue pill, in liquid form, helps improve blood flow to the lungs.
A new type of ventilation strategy, called gentle ventilation, is also helpful.
"Basically, the ventilator is like jiggling the baby, and every jiggle is a breath," said Dr. Warner.
Benjamin was also born with CDH. A combination of Viagra and other meds made him strong enough to leave the intensive care unit.
Benjamin’s mom, Kelly Hubble, said "He's off the oxygen. He's down to one medication a day. He's pretty much our miracle."
Babies who now have the chance to grow up thanks to treatment with a softer approach.
CDH is usually diagnosed on a prenatal ultrasound. The overall survival for CDH is 55-65%. The hope is that new treatment protocols and increased awareness can improve the survival rate in the future.
TOPIC: VIAGRA TREATS BIRTH DEFECT
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a hole in the diaphragm, according to Children's Hospital Boston. The hole is more likely to occur on the left side, yet, it does occasionally occur on the right side. Stomach, intestines, liver and spleen contents may travel through the hole making their way into a child's chest. When these contents travel into the chest, normal lung development is prohibited.
There are two main types of CDH. The first is called Bochdalek hernia. This type of hernia is caused when there is an opening in the back of the diaphragm, and stomach, intestines, spleen, or liver contents enter the chest. The second type of CDH is Morgagni's hernia. Morgagni's hernias are openings in the front of the diaphragm, typically behind the breastbone. This type of CDH is very rare and does not occur often. With Morgagni's hernias, liver and/or intestine contents move into the chest cavity.
CAUSES AND SYMPTOMS: CDH occurs as the child is developing in the mother's womb, around the seventh week of pregnancy. It is also important to note that during the seventh week of pregnancy, the stomach, esophagus, and intestines are forming. CDH develops if the intestines become trapped in the chest cavity, thus causing the diaphragm to improperly develop. Children's Hospital Boston also explains CDH occurs in about one in every 2,500 childbirths. Approximately 90 percent of all CDH cases are due to Bochdalek hernias.
Important symptoms to look for in babies to detect if they may have CDH are: fast breathing or difficulty breathing, a fast heart rate, bluish skin tones, a concaved abdomen, and abnormal chest development (usually one side is bigger or looks different than the other side).
TREATMENT: The erectile dysfunction drug Viagra helps treat some children with CDH. Viagra allows the child's muscles to relax, allowing him to breathe easier, according to Brad W. Warner, M.D., from St. Louis Children's Hospital and Washington University School of Medicine. Also, a new ventilation strategy called "gentle ventilation" puts less pressure on the child's fragile lungs and is now being used on babies with CDH. Dr. Warner says all patients will require repair of their CDH once they are medically stabilized, but these newer treatment options are offering a much needed alternative to rushing the baby into surgery immediately upon birth.
FOR MORE INFORMATION, PLEASE CONTACT:
Judy Martin, Associate Director of Media Relations
Washington University School of Medicine
St. Louis, MO