A LOOK TO THE FUTURE While there is no test that can determine if another child will RECIPES Continuing the Diet. MSUD is a serious health concern. be affected with MSUD before a pregnancy, prenatal testing Treating a child as early in life as possible helps to prevent may be possible during the early part of the pregnancy. A ® ® developmental delay and neurological damage. Not following couple may want to discuss and consider all their options with Kool-Aid -Flavored Ketonex -1 the MSUD diet may cause mental and nervous system damage their metabolic doctor and genetic counselor before having Yield: 8 fl oz at any age. Lifelong nutrition support must be adapted to each another pregnancy. 40 g Ketonex-1 person’s needs. Metabolic doctors and nutritionists provide If you decide to have another child, give yourselves time to 3 Tbsp, level, sugar1 ® support that will help your child have a normal, productive life. adjust to the special needs of the first child. Make sure you 1/4 tsp Kool-Aid or Wyler’s Unsweetened Soft Drink Mix Current research is looking at alternative treatment options for have learned diet management for your child with MSUD Add water (room temperature) to ingredients to make 8 fl oz. Mix in a MSUD, such as enzyme replacement, gene therapy, and liver before having another child. blender at lowest speed no more than 4 seconds. Or, shake briskly in a transplantation. However, until these therapies can be proven Offspring of a Person with MSUD. All children born to a closed container for 10 to 12 seconds. Serve chilled. effective and safe, dietary treatment for life is essential. person with MSUD will be carriers of the gene. As shown in Nutrient 1 fl oz 8 fl oz Figure 6, all offspring of a parent with MSUD ( ) and a parent Family Planning. MSUD occurs approximately one time who is normal ( ) inherit one normal ( ) and one MSUD ( ) Isoleucine, mg 0 0 in every 185,000 live births. In the Old Order Mennonite gene. Each one of their offspring will be a carrier. Leucine, mg 0 0 population, MSUD occurs in approximately 1 in 175 newborns. If a person with MSUD has children with a carrier for MSUD, Valine, mg 0 0 The chance that two carriers of MSUD will have a child with approximately one-half (50%) of their offspring will have Protein, g 0.75 6 MSUD is 1 in 4, or 25%, for each pregnancy (Figure 4). The MSUD and one-half (50%) will be carriers (Figure 7). Energy, kcal 42 336 chance that two carriers will have a child who is a carrier is 1 in 2, or 50%, for each pregnancy. Childbearing by Women with MSUD. For the woman with 1 Osmolality (concentration of particles in solution) may be too high if more sugar is added, which may cause bloating and diarrhea. MSUD, having children may cause problems. A major concern 2 The amount of drink mix may be varied according to taste preference. Before a couple who has a child with MSUD has any more for women with MSUD is the stress of the pregnancy on her children, they should take time to seriously think about the metabolic control. Nutritional requirements can rapidly change special parenting tasks that parents of a child with MSUD during the course of a pregnancy. Pregnant women must be must manage. Genetic counseling is recommended to review monitored carefully for their own safety, as well as the health the risks and to discuss several reproductive options that are of their baby. Successful pregnancies require careful diet ® ® monitoring during the prenatal, labor, delivery, and postpartum Kool-Aid -Flavored Ketonex -2 available before and during pregnancy. Yield: 16 fl oz periods. 40 g Ketonex-2 3 Tbsp, level, sugar1 1/2 tsp Kool-Aid or Wyler’s Unsweetened Soft Drink Mix2 Mother’s Genes Father’s Genes Mother’s Genes Father’s Genes Add water (room temperature) to ingredients to make 12 fl oz. Mix in a blender at lowest speed no more than 4 seconds. Or, shake briskly in a closed container for 10 to 12 seconds. Serve chilled. Nutrient 16 fl oz A B Normal Gene A B Normal Gene Isoleucine, mg 0 MSUD Gene MSUD Gene Leucine, mg 0 C D C D Valine, mg 0 Protein, g 12 Energy, kcal 308 Figure 6. All children of a person with MSUD and a person who doesn’t Figure 7. Approximately half of the children of a parent with MSUD 1 Osmolality (concentration of particles in solution) may be too high if more sugar is added, which may cause bloating and diarrhea. have MSUD will be carriers of MSUD. who mates with a carrier of MSUD will have MSUD. 2 The amount of drink mix may be varied according to taste preference. 14 15