Cystic fibrosis is an inherited genetic disease that causes mucus to build up and clog body organs, especially the lungs and pancreas. It mostly affects Caucasian families -- one out of every 28 Americans carry the defective gene that causes cystic fibrosis, and one of every 3,300 Caucasian babies inherit these genes from both parents and develop the disease. In comparison, only about one in 17,000 African-American babies and one in 11,500 Hispanic babies get cystic fibrosis (it's even rarer among Asians).
"Most babies who have cystic fibrosis appear healthy at birth," says Susanna McColley, M.D., director of the Cystic Fibrosis Center at the Ann & Robert H. Lurie Children's Hospital of Chicago. That's why all states test for the disease as part of mandatory newborn screenings. If you are told that your baby has cystic fibrosis, you'll need to take extra steps to make sure she gets the nutrients she needs to thrive and that her airways stay clear and healthy. Here's a look at what you might expect during her first year.
Feeding. Cystic fibrosis causes mucus to block tubes or ducts in your baby's pancreas; this keeps digestive enzymes from reaching the intestines. As a result, nutrients from fats and protein aren't properly absorbed. This can lead to intestinal blockages, diarrhea, malnutrition, poor weight gain, and growth. To aid proper digestion, you will need to give your baby pancreatic enzyme supplements prescribed by his doctor at the start of each feeding. "These look like small beads and are usually mixed with a dab of applesauce and fed by spoon prior to nursing or bottles," Dr. McColley says. (The enzymes tend to work better when mixed with soft, acidic foods and not with milk products.) Although the enzymes are tasteless, it can take a little time for infants to get used to the texture and learn how to swallow the beads. If your baby spits them out, gently scoop up the bead mixture with a spoon and feed it to him again. Talk to your doctor if your infant seems to be spitting out more medicine than he's taking in. Because babies get hungry often, you should always carry enzymes and baby food with you (keep a stash in the diaper bag).
When your infant starts to eat solid foods, you will have to give the enzymes before every meal and snack (as well as before bottles or breast). But because the enzymes work for about a half hour, you do not need to give new enzymes for each food that is consumed. You also don't have to give enzymes when feeding your child certain foods composed of simple carbohydrates that his body can easily digest. These include juice, fruit, fruit snacks, infant rehydrating drinks, and Popsicles. If your independent tot starts becoming stubborn about taking the enzymes, try giving him a choice of two options: "Would you like to take your beads in applesauce or pears today?"
Cystic fibrosis also disrupts the normal function of cells that make up the sweat glands in skin. As a result, infants can lose large amounts of salt when they sweat, placing them at high risk for dehydration. Any extra salt intake should be dosed in accordance with her doctor or dietitian's recommendation. Your baby also may need supplemental fat-soluble vitamins such as A, D, E, and K.
Reaching Milestones. You can expect your baby to reach developmental milestones -- sitting up, crawling, walking, and talking -- on target with her peers. When your child starts preschool or kindergarten, she may be eligible for an individualized education plan (IEP) under the Individuals with Disabilities Education Act. An IEP ensures that your child can continue her education through tutoring or other arrangements if she is ill or hospitalized and can also include necessary measures to keep her in school, such as administering enzymes and having extra snacks.
Respiratory Therapy. Infants with cystic fibrosis are at high risk for lung infections because the thick mucus in their airways is difficult for them to expel. To keep your baby healthy, you'll need to do therapy to clear her airways. "During chest physical therapy, you use a cupped hand or a small soft device called a percussor to clap over different areas of the lungs," Dr. McColley explains. Breathing treatments, in the forms of a nebulizer or inhaler, may be given prior to this therapy. Therapy sessions may last anywhere from 10 to 30 minutes and are best done either before your child eats or two hours after a feeding.
You probably have a lot of questions about raising a child with cystic fibrosis. It can help to connect with other families who are in similar situations. Contact the Cystic Fibrosis Foundation to find other families in your community and to get answers to frequently asked questions.
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