Neonates, Infant, Newborn, Baby, Preemie
What is Cystic Fibrosis?
Cystic Fibrosis is a genetic disease that causes the body to produce extra mucus, leading to potentially life-threatening complications. There is a gene that controls the movement of salt throughout the body, and with cystic fibrosis, there is a flaw with this particular gene that results in the thick secretions.
Potential complications from the thick mucus can lead to difficulty breathing, as well as building up harmful bacteria, leading to lung infection. These factors can eventually contribute to damaging the lungs themselves, and even death.
Cystic Fibrosis is considered one of the leading genetic diseases that ultimately leads to a shorter life.
The following is courtesy of Genetics Home Reference: [Source]
"Cystic fibrosis is an inherited disease of the mucus glands that affects many body systems. The disorder's most common signs and symptoms include progressive damage to the respiratory system and chronic digestive system problems.
Mucus is a slippery substance that lubricates and protects the linings of the airways, digestive system, reproductive system, and other organs and tissues. In people with cystic fibrosis, the body produces mucus that is abnormally thick and sticky. This abnormal mucus can obstruct the airways, leading to severe problems with breathing and bacterial infections in the lungs. These infections cause chronic coughing, wheezing, and inflammation. Over time, mucus buildup and infections result in permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs.
Most people with cystic fibrosis also have digestive problems because thick, sticky mucus interferes with the function of the pancreas. The pancreas is an organ that produces insulin (a hormone that helps control blood sugar levels). It also makes enzymes that help digest food. In people with cystic fibrosis, mucus blocks the ducts of the pancreas, preventing these enzymes from reaching the intestines to aid digestion. Problems with digestion can lead to diarrhea, malnutrition, poor growth, and weight loss. Some babies with cystic fibrosis have meconium ileus, a blockage of the intestine that occurs shortly after birth.
Cystic fibrosis used to be considered a fatal disease of childhood. With improved treatments and better ways to manage the disease, many people with cystic fibrosis now live well into adulthood. Adults with cystic fibrosis experience medical problems affecting the respiratory, digestive, and reproductive systems. For example, most men with cystic fibrosis are unable to father children (infertile) because the tubes that carry sperm (the vas deferens) are blocked by mucus and do not develop properly. This condition is known as congenital bilateral absence of the vas deferens (CBAVD). Infertility is also possible, though less common, in women with cystic fibrosis."
There is no known cure, but there are many treatments and medications available to help with CF complications. Babies with Cystic Fibrosis will need antibiotics to fight potential infections, as well as medications to help break up mucus.
Parents need to be consistent with helping keep their baby's chest clear by administering medications as well as manually loosening mucus if necessary. This can be done by gently tapping on your baby's chest with a cupped hand. Have a pediatrician demonstrate this method before using it.
Survivability has increased vastly over the years, with many childrne born with CF living well into their 30s, and sometimes longer.
See Also:
• BabyCenter - Cystic Fibrosis
• Cystic Fibrosis Foundation
• Livestrong - Infant Cystic Fibrosis