Adolescence. Adolescence may be a difficult time for both the of your child’s nutrition needs. If refusal of the medical food THE ROLE OF THE FAMILY AND OTHER Feeding an ill child can be very difficult. Often a child with child and the parents, regardless of MSUD! The influence of continues to be a problem, the use of a feeding tube may need CARE PROVIDERS IN MANAGING MSUD fever is restless and loses appetite. The illness might also friends and the struggle for independence may make dietary to be considered. Parents carry the bulk of the responsibility for the diet. If include stomach upset, nausea, or even vomiting. A child may compliance a challenge. They may feel that MSUD makes them Refusing Solids. A child may experience normal variations in possible, try to share the responsibility, so both parents can become very dehydrated because of the high body temperature different from their friends. Eating out with friends is part appetite or taste for certain foods. Or, the prescribed amount of prepare and monitor the diet. Other children in the family, as and a lack of adequate fluid intake. of growing up. Help your child develop the skills for eating Ketonex may be too high and the energy in it is causing her to well as the child with MSUD, should learn about the diet as If you suspect a cold or virus, or if your child has a fever, it out, traveling, and “sticking” to the diet when not at home. lose her appetite. is important to call your pediatrician or metabolic doctor Sometimes teenagers with MSUD would rather tell people they soon as they are old enough to understand it. Older brothers are a vegetarian or vegan than explain about MSUD. Help your Toddlers and preschoolers periodically have one of two and sisters should be encouraged to be involved in feeding the immediately. Illness in a child with MSUD can be very serious. teenager deal with her peers and not be self-conscious that she characteristic feeding behaviors that cause parents concern. child with MSUD so they become familiar with foods allowed Your metabolic doctor or nutritionist will help you decide has MSUD. Finding a peer with MSUD through support groups They may decide to stop eating—go on a “food strike,” or they and excluded. Make sure they understand the importance of on how to manage it. Ask your nutritionist for a “sick-day can be a great comfort for a teen. Ask the clinic for resources may go on a “food jag.” the diet for their brother’s or sister’s health. Brothers and plan” to use during illness. A sick-day plan provides adequate and suggestions. During a food strike when your child refuses to eat, offer food sisters should not feel sorry for the child with MSUD because calories to meet energy needs and helps keep your child at usual mealtimes and if she refuses the food, take it away. she is on a special diet. Treat your child with MSUD as out of the hospital. Ask about beginning medication such as FEEDING PROBLEMS Allow only water between meals. She will become hungry and normally as possible. acetaminophen (Tylenol®) to reduce fever and fluids to prevent Parents may be tempted to treat their child as a “sick” child then eat. Remember that Ketonex supplies most of your Grandparents love to spoil their grandchildren! It may be dehydration. and not follow their usual patterns of child rearing. The child child’s nutrient needs, so her medical food should never be difficult for them as they sometimes feel the child with Here are some suggestions of things to do when your child is ill with MSUD is a normal child who needs to manage food intake restricted. MSUD is “missing out.” It is important that they understand with fever. carefully. Ask your child’s doctor, nutritionist, public health Do not give in to the food strike and offer free foods or foods the diet and become actively involved as much as possible. A • Do not force-feed food or Ketonex, especially if your child is nurse, or social worker for support and help if any of the that are not on the BCAA-restricted diet. The nutritionist can grandmother may be the ideal person to experiment with low- nauseated or vomiting. Soda crackers may be the only food following problems should occur. help you during this trying time, so do not hesitate to call. It is protein cooking and provide special low-protein treats. he will feel like eating. Encourage intake of any allowed Loss of Appetite. Loss of appetite can result from a variety also very important for both parents to support each other in Explain MSUD to relatives, friends, day-care providers, baby- foods your child is willing to eat. of causes, including illness; eating too many sweet foods or managing a food strike. If a child is allowed to eat foods not on sitters, and all teachers. They should become familiar with • Encourage your child to continue drinking his medical food desserts that satisfy the appetite and decrease the desire to eat her diet, blood ILE, LEU, and VAL levels will not be controlled. foods allowed and excluded and understand the importance of if he tolerates it. the foods prescribed; getting too much Ketonex, which may On a food jag, a child wants to eat the same food or foods for the diet. Give a list of the foods allowed and excluded to anyone • Offer Pedialyte® Oral Electrolyte Solution with added depress the appetite for other foods; or having a lower than long periods. If the foods are nutritious and are in the diet, who feeds your child and explain the list as well as the exact carbohydrate, such as dextrose or table sugar (3 Tbsp sugar normal blood BCAA level. Some children have a poor appetite there is no reason for concern. Remember that most of your menu. to 8 fl oz of Pedialyte); Pro-Phree®; non-cola carbonated despite adequate treatment and dietary management. In such child’s nutrient needs are supplied by Ketonex. beverages; sugar-sweetened carbonated beverages; Kool- cases, it may be necessary to use a feeding tube to ensure Tell everyone who cares for your child that even “just a little Aid®; Tang®; tea with sugar; vegetable broth; or fruit juices nutrition needs are met. Inappropriate Feeding Behavior. Inappropriate behaviors, bite” of a high-protein food is not allowed. Emphasize what can with some sugar added. Unusual Hunger. This may be an indication that the diet such as refusing to give up the bottle and/or difficulty in eating happen if your child does not stay on the diet. • Dilute the Ketonex mixture, or use liquid Jell-O® if it is needs to be adjusted. The amount of Ketonex may need to be solids, chewing, or self-feeding, may result from a variety tolerated. increased because the table foods prescribed are not satisfying of causes, including a delay in offering solid foods; delay in YOUR CHILD’S DIET DURING ILLNESS • Freeze any of the beverages listed and make into chipped needs. Providing low-protein foods is a great way to manage teaching the child to drink Ketonex by cup; or not allowing ice. Frequently feed small amounts of this chipped ice to hunger without increasing protein intake. the child to self-feed either with his fingers or a spoon. Always A body temperature greater than 98.6° F (37° C) or a rectal provide energy and prevent dehydration. keep a positive attitude and make feeding a pleasurable event. temperature over 100° F (37.8° C) is a fever. During a fever, the • As your child’s appetite improves, gradually return to the Refusing Medical Food. A child may refuse Ketonex because Try not to feed a child longer than necessary at mealtime to body’s rate of using food for energy speeds up. If extra energy usual diet plan. of normal variations in appetite, and this should not be of encourage self-feeding. Remember that small amounts of food is not supplied during illness, the body will break down its own concern, especially if average intake over a week is adequate. If are usually wasted when a child first learns to selffeed, but muscle and fat stores for energy. Muscle protein breakdown Emergency Letter. Individuals with MSUD should have an Ketonex is not offered regularly, a child may decide to refuse this is normal. Keeping food records will help your nutritionist needs to be prevented in children with MSUD, because it will emergency letter with them at all times. This letter provides it. Improperly mixed Ketonex can also be a cause—too much estimate your child’s intake. release too much BCAA into the blood. BCAA is then carried important information such as the name of the condition, water makes the volume too great; too little water makes the to the brain where it may have a harmful effect. Give your child explanation of symptoms, the importance of timely treatment medical food mixture too thick. A child may refuse Ketonex A child may be using his diet as a way of getting attention or extra low-protein foods, formula or fluids during illness. This and treatment strategies, and your metabolic doctor’s contact as an attention getting device, especially if he senses that his manipulating parents. If your child has any of these problems, extra food energy will decrease the amount of muscle protein information. In times of illness or stress that may require parents are anxious for him to drink the Ketonex mixture. call the nutritionist. The nutritionist will give you support and broken down for body energy. hospitalization, this letter can be presented. This letter can be Remember, Ketonex plays an important role in providing most offer suggestions to help solve the problem. provided by your metabolic team. 12 13