diet and become actively involved as much as possible. A grandmother may be the ideal person to experiment with low- Mother’s Genes Father’s Genes Mother’s Genes Father’s Genes protein cooking and provide special low-protein treats. Explain the DLC to relatives, friends, day-care providers, baby- sitters, and all teachers. They should become familiar with foods allowed and excluded, and understand the importance A B Normal Gene A B Normal Gene of the diet. Give a list of the foods allowed and not allowed to DLC Gene DLC Gene anyone who feeds your child and explain the list as well as the exact menu. C D C D Tell everyone who cares for your child that even “just a little bite” of a high-protein food is not allowed. Emphasize what can happen if your child does not stay on the diet. Figure 6. All children of a person with a DLC and a person who does Figure 7. Approximately half of the children of a parent with a DLC Here are some suggestions of things to do when your child is ill not have a DLC will be carriers of a DLC. and a parent who is a carrier of a DLC will have a DLC. YOUR CHILD’S DIET DURING ILLNESS with fever. A body temperature greater than 98.6° F (37° C) or a rectal • Do not force-feed food or I-Valex, especially if your child is A LOOK TO THE FUTURE their metabolic doctor and genetic counselor before another temperature over 100° F (37.8° C) is a fever. During fever, the nauseated or vomiting. Soda crackers may be the only food pregnancy. body’s rate of using food for energy speeds up. If extra energy he will feel like eating. Encourage intake of any allowed Continuing the Diet. A DLC is a serious health concern. is not supplied during illness, the body will break down its foods that your child is willing to eat. Treating a child as early in life as possible may prevent If you decide to have another child, give yourselves time to own muscle protein and fat stores for energy. Muscle protein • Encourage your child to continue drinking his medical food developmental delay and severe neurological damage. Not adjust to providing for the special needs of the first child. breakdown (and fat breakdown in some DLCs) needs to be if he tolerates it. following the LEU restricted diet may cause mental and Parents will want to be skilled in diet management for the DLC prevented in children with a DLC because it will release too • Offer Pedialyte® Oral Electrolyte Solution with added neurological damage at any age. Lifelong nutrition support before having another child. much LEU and organic acids into the blood. LEU and organic carbohydrate, such as dextrose or table sugar (3 Tbsp sugar must be adapted to each person’s needs. Metabolic doctors and acids are carried to the brain and other body organs where they to 8 fl oz of Pedialyte); Pro-Phree®; non-cola carbonated nutritionists provide support that is essential for helping your Offspring of a Person with a DLC. All children born to a may have a harmful effect. Give your child extra low-protein beverages; sugar sweetened carbonated beverages; Kool- child have a normal, productive life. person with a DLC will be carriers of the gene or have a DLC. food, formula, and fluids during illness. The extra food will Aid®; Tang®; tea with sugar; vegetable broth; or fruit juices As shown in Figure 6, all offspring of a parent with a DLC ( decrease the amount of muscle protein broken down for body with some sugar added. Family Planning. The chance that two carriers of a DLC will ) and a parent who is normal( ) inherit one normal ( energy. • Dilute the I-Valex mixture, or use liquid Jell-O® if it is have a child with a DLC is 1 in 4, or 25%, for each pregnancy ) and one DLC ( ) gene. Each one of their children will be a tolerated. (Figure 4). Because you have a child with a DLC, you know carrier. If a person with a DLC has children with a carrier for Feeding an ill child can be very difficult. Often a child with • Freeze any of the beverages listed and make into chipped that both parents are carriers. That means you have a 25% risk DLC, approximately one half (50%) of their offspring will have fever is restless and has a loss of appetite. The illness might ice. Frequently feed small amounts of this chipped ice to with each pregnancy of having another child with a DLC. The a DLC and one-half (50%) will be carriers (Figure 7). also include stomach upset, nausea, or vomiting. A child may provide energy and prevent dehydration. chance that two carriers will have a child who is a carrier is 1 become very dehydrated because of the high body temperature • As your child’s appetite improves, gradually return to the of 2, or 50% for each pregnancy. Childbearing by Women with a DLC. For the woman with and a lack of adequate fluid intake. usual diet plan. a DLC, having children may cause problems. A major concern Before a couple with a child who has a DLC plan to have any for the DLC women is the stress of the pregnancy on her If you suspect a cold or virus, or if your child has a fever, it Emergency Letter. Individuals with DLC should have an more children, they should take time to seriously think about metabolic control. The nutritional requirements can rapidly is important to call your pediatrician or metabolic doctor emergency letter with them at all times. This letter provides the special parenting tasks that parents of a child with a DLC change during the course of a pregnancy. Pregnant women immediately. Illness in a child with a DLC can be very serious. important information such as the name of the condition, must manage. Genetic counseling is recommended to review must be monitored carefully for their own safety, as well as the Your metabolic doctor or nutritionist will help you decide on explanation of symptoms, the importance of timely treatment the risks, and to discuss several reproductive options that are health of their baby. how to manage it. and treatment strategies, and your metabolic doctor’s contact available before and during pregnancy. information. In times of illness or stress that may require DLCs are a serious health concern. However, by following Ask your nutritionist for a “sick-day plan” to use during illness. hospitalization, this letter can be presented. This letter can be While there is no test that can determine if another child will an LEU-restricted diet closely and keeping blood LEU levels A sick-day plan provides adequate calories to meet energy needs provided by your metabolic team. be affected with a DLC before a pregnancy, prenatal testing within the treatment range, the person with a DLC can lead and helps keep your child out of the hospital. Ask about using may be possible during the early part of the pregnancy. A a normal, productive life. Successful pregnancies require medication such as acetaminophen (Tylenol®) to reduce fever. couple may want to discuss and consider all their options with attentive diet monitoring during the prenatal, labor, delivery, and postpartum periods. 14 15