NEWBORNS & INFANTS
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Your baby has a positive Newborn Screen
for CF — now what?
If you live in New York State, and your baby's NBS came back positive for CF, it means there was a high level of IRT (a chemical made by the pancreas, which is often high in people with CF), and that a second test may have shown changes in the CF gene. A positive NBS does not definitively diagnose CF.
Most babies who have a positive (abnormal) screen for CF do not have CF, but it is important to be sure that they do not. Please bring the baby to the CF Care Center for sweat testing.
The sweat test can be done in the first few weeks of your baby's life, does not help, and you will be able to stay with the baby throughout the test. The technician will put a gel on the baby's arm or leg, cover the gel with small patches with wires which will make the baby sweat. The sweat is collected on a gauze pad or disk and taken to the lab to be analyzed.
If your baby's sweat test results diagnosed CF, please call our Pediatric CF Care Center at (516) 622 5280 to set up an appointment. Remember — we are here to help you and your baby, to enjoy a fulfilling life. We will teach you the best ways to keep your child healthy, and to work with you on treatments, therapies, education, financial and insurance arrangements, and moral support.
Click through to learn about nutrition, treatments, tests and other important information about living with your newborn's CF. You can also get more information at http://www.cff.org/AboutCF/Testing/NewbornScreening/
Electronic Medical Records:
We're pleased to announce that Electronic Medical Records (EMR) went live in the Pediatric Center on November 24, 2013. We can now order your medications online, and all the doctors who see a patient within the health system can see important data on the patient, improving the quality of multidisciplinary care.
Good nutrition is very important for infants with cystic fibrosis. A balanced diet, which includes adequate calories and the right vitamin and nutritional supplements, is key to good nutrition and health. Reaching and maintaining a normal body weight will help your baby be as healthy as possible. The CF dietician or care provider will teach you how to meet your baby's nutritional needs, and you can find more information here
The basic problem in CF is an error in the salt and water exchange in some cells. This causes the body to make thick, sticky mucus, which clogs the lungs and leads to infections. The body needs more calories to fight infection. This mucus can also keep pancreatic enzymes from reaching the intestines to digest and absorb food. Without enzymes, maldigestion and malabsorption of food can occur. This causes greasy, large bowel movements, slow growth and poor weight gain.
Some infants with CF gain weight and grow well, while many others do not. Therefore, there are two nutrition goals in CF. The first goal is to help those who need to gain weight "catch up" and reach a normal weight. The second goal is to help those who have already "caught up" or are already gaining weight well, to continue to do so.
A high-calorie diet is often needed, even if your baby does not have the digestive problems associated with CF. For most infants with CF, the extra calories help meet the high energy needs that go along with the disease.
Breast milk or infant formula is recommended for the first year of life. Do not routinely offer juice, water or other liquids, which are lower in calories or nutrition. If your baby's weight gain is slow, the CF dietician or care provider may suggest adding extra calories to breast milk or formula. After your baby's first birthday, ask your CF dietician if whole milk or a pediatric nutritional supplement is best for your child.
When introducing solid food into the diet of a baby who needs to gain weight, you may need to offer high-calorie formula or breast milk before feeding your baby solid food, or to choose baby foods with the most calories listed on the labels. Consult with your CF dietician as to the best way to give your baby more calories.
Along with a healthy diet, vitamin supplements are important for your baby. People with CF do not absorb vitamins well. Vitamin supplements will help prevent low levels of the fat-soluble vitamins A, D, E and K. Ask your CF care provider about the proper type and dose of vitamin supplements for your baby.
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A small amount of spitting up is normal for all babies. A large amount of vomiting is not normal and may mean the baby has reflux. This may lead to slow weight gain and poor growth. If you have concerns about spitting up, please consult your CF care provider or dietician.
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Pancreatic enzyme replacements
Your baby may need pancreatic enzyme replacements, or "enzymes," to help digest and absorb food. These enzymes must be prescribed by your doctor. Enzymes may be started if your baby has poor weight gain (despite a good appetite), frequent loose and/or large bowel movements, foul-smelling bowel movements, mucus or oil in bowel movements, excessive gas and/or stomach pain, and distention or bloating.
Enzymes come in capsule form. Inside each capsule are many small "beads" that contain digestive enzymes. For babies, the capsules are opened up and the beads are sprinkled in a small amount of acidic food, such as baby applesauce, and given from a spoon. Infants who do not digest their food well need enzymes with every feeding (breast milk, formula and most foods).
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People with CF lose more salt in their sweat than those without CF. Your CF care provider or dietician may suggest adding a small amount of salt to your baby's food or formula, especially during hot weather.
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Some infants with CF have a very difficult time gaining weight. Feeding formula through a tube in the baby's stomach is an excellent way to help your baby gain weight. Tube feedings should not be seen as a last resort and do not mean failure. They can be a great way to help your baby grow and feel better.
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for important information on treating your child's cough or cold
Postural Drainage and Percussion
Postural Drainage and Percussion (PD & P), also known as chest physical therapy, is a widely accepted technique for helping people with CF breathe with less difficulty and stay healthy. PD & P uses gravity and percussion
to loosen the thick, sticky mucus in the lungs so it can be removed by coughing. Unclogging the airways is critical to reducing the severity of lung infections.
The goal of PD & P is to clear mucus from each of the five lobes of the lungs
by draining mucus into the larger airways so it can be coughed out. The right lung is composed of three lobes: upper, middle and lower, while the left lung has two lobes: upper and lower. The lobes are divided into smaller divisions called segments. The left and right upper lobes are made up of three segments each: apical, posterior and anterior. The left upper lobe also includes the lingual, which corresponds to the middle lobe on the right. The lower lobes each include four segments: superior, anterior, lateral basal and posterior basal.
Each segment of the lung contains a network of air tubes, air sacs and blood vessels. These sacs allow for the exchange and carbon dioxide between the blood and air. These are the segments that PD & P seeks to drain.
Performing PD & P
In infants, PD & P must be performed by a caregiver. PD & P involves a combination of techniques including multiple bronchial drainage positions, percussion, vibration, deep breathing and coughing
that help move mucus from the lungs to the throat. Once the infant is in one of the several prescribed drainage positions, the caregiver performs percussion on the chest wall.
Here are the positions for performing PD & P
on infants and the lung sections that each one targets.
Infant/ Upper Lobes/Apical Segments: Lean the infant back from a sitting position at a 30-degree angle on a pillow in your lap. Percuss and vibrate over the muscular area between the collarbone and the top of the shoulder blade on both the left and right sides.
Infant/Right Middle Lobe/Lateral and Medial Segments: Place the infant over the extended legs of the therapist, with a pillow on the legs if desired. Place the infant head-down on their left side and rotate the torso one-quarter turn backward and percuss over the right nipple.
Infant/Left Upper Lobe/Lingular Segment: Place the infant over the extended legs of the therapist, with a pillow on the legs if desired. Place the infant head-down on their right side and rotate the torso one-quarter turn backward and percuss over the left nipple.
Infant/Lower Lobes: Place the infant face-down on a pillow in your lap and clap over the middle of the back below the tip of the shoulder blade on either side of the spine.
Infant/Upper Lobes/Apical Segments: Place the infant in your lap, leaning back at a 30-degree angle and clap over an area between the collarbone and the shoulder blade on the infant's left side, then repeat on the right side.
Infant/Upper Lobes/Anterior Segments: Place the infant on his/her back on your lap. Clap between the collarbone and nipple on each side of the chest.
Infant/Upper Lobes/Posterior Segments: Place the infant on your lap, leaned forward at a 30-degree angle. Clap over the upper back on each side.
Infant/Lower Lobes/Anterior Basal Segments: Place the infant head-down over the extended legs of the therapist, with a pillow on the legs if desired. Turn the infant on their left side and clap the lower ribs just beneath the right armpit. Then turn the infant onto their right side and clap the lower ribs just beneath the left armpit.
Infant/Lower Lobes/Lateral Basal Segments: Place the infant head-down over the extended legs of the therapist, with a pillow on the legs if desired. Turn the infant's torso one-quarter turn to the left, and clap over the uppermost portion of the lower ribs on the right side. Then rotate the infant's torso so that it is turned one-quarter to the right, and clap over the uppermost portion of the lower ribs on the left side.
Infant/Lower Lobes/Posterior Basal Segments: Place the infant head-down on his/her belly over the extended legs of the therapist, with a pillow on the legs if desired. Clap over the lower rib cage on both sides of the chest.