Frequently Asked Questions
What is a Newborn Blood-Spot Screen?
Why should my baby have the screening?
What conditions are currently screened in Missouri?
Are Newborn Screens required in Missouri?
How is the Screening done?
How are the screening results reported and to whom?
How do I find out about my baby's results?
If I get notified that my baby needs to have the screening repeated does it mean my baby has one of these 67 conditions?
Can these disorders be cured?
What is a Newborn Blood-Spot Screen?
A newborn blood-spot screen refers to tests performed on newborns before they are discharged from the hospital. These tests are done to protect them from the serious effects of conditions that otherwise may not be detected for several days, months, or even years. A screening test only finds those babies who may be at risk for a disorder. Only additional testing (diagnostic testing) can tell if the baby really has the disorder.
Why should my baby have the screening?
The conditions for which your baby will be tested for are individually rare. However, they are also very serious and can result in mental retardation and/or death if not treated. Babies with these conditions appear normal at birth. It is only with time the condition affects the baby's mental or physical development or causes other medical problems. By then the damage may be permanent. The newborn screen helps to identify the babies needing treatment, such as a special diet or medication. Only additional testing (diagnostic testing) can tell if the baby really has the disorder. About 1 in 600 of Missouri’s newborns are found to have one of the screening disorders.
The screening tests are very good but not fail-safe and may not always detect a disorder. In any case, if your baby does not seem well, talk to your baby's doctor as soon as possible.
What conditions are currently screened in the Missouri?
In Missouri all newborns are screened for the following metabolic or genetic conditions:
- Biotinidase deficiency - An enzyme deficiency that occurs when a baby lacks the enzyme biotindiase needed to recover and recycle the vitamin biotin. The symptoms of Biotinidase Deficiency include skin problems, frequent infections, hearing problems, seizures, and brain damage. Treatment consists of a daily dose of the vitamin biotin.
- Congenital adrenal hyperplasia (CAH) (con-JEN-I-tal ah-DREE-nal HIGH-per PLAY-ze-ah) - A condition that affects the adrenal glands, which are located on top of the kidneys. These glands produce three types of hormones called cortisol, aldosterone and androgens. Females with classical CAH are born with masculine appearing external genitals, but with female internal sex organs. Males with classical CAH appear normal at birth. Males and females with classical CAH are likely to have trouble retaining salt, a condition which can be life threatening. Treatment for CAH is life-long.
- Congenital hypothyroidism (CH) (con-JEN-I-tal HIGH-poe-THIGH-royd-ism) - Occurs when a baby does not have enough thyroid hormone. Without an adequate amount of thyroid hormone the baby may not develop normally, which can result in mental retardation and stunted growth. Treatment for these babies consists of medication needed for the rest of their lives.
- Cystic fibrosis (CF) - A disorder that causes thick, sticky mucus to build up in the lungs, digestive system, and other organs of the body. The symptoms of CF include lung infections, difficulty digesting food and nutrients, and poor growth. Treatment consists of enzyme supplements taken with meals and respiratory treatments to help with breathing. Regular medical care by a medical specialist is necessary.
- Galactosemia (Guh-LAK-toe-SEE-me-ah) - Occurs when a baby is not able to digest one of the sugars (galactose) in breast and cow's milk and baby formula. Without treatment of a special diet, the sugar builds up in the baby's blood causing liver damage, cataracts, mental retardation and possible death. Treatment consists of a special diet that eliminates galactose, a sugar found in milk and milk products from the baby's diet.
- Phenylketonuria (PKU) (FEE-nil-Kee-tone-u-ree-ah) - The baby is unable to use one part of the protein found in most foods. This protein builds up in the blood and may prevent the brain from developing normally. If left untreated, PKU causes mental retardation. Treatment consists of a special diet (low in protein) to be followed for life.
- Sickle cell anemia and unusual hemoglobin types (SICK-cul cell A-NEE-me-AH) - Is found mainly in African Americans and those of Mediterranean background. In sickle cell anemia, the red blood cells change from the normal round shape to an abnormal sickle shape. These sickle cells can clog blood vessels so not enough oxygen can be carried to the body. Treatment consists of the child being under the care of a health care provider early in life for medication and other treatment.
- Amino Acid Disorders
Babies born with one of these disorders cannot breakdown certain waste products from their blood, such as amino acids or ammonia. This can lead to problems with the eyes, skin or general development, liver failure, coma or death if untreated. Treatment can range from special diets to liver transplantation and special medications. A baby with an amino acid disorder must have regular medical care by an experienced physician. The Newborn Screening Program will screen for: - Argininemia (ARG, arginase deficiency)
- Argininosuccinic Acidemia (ASA, argininosuccinase)
- Citrullinemia type I (CIT-I, argininosuccinate synthetase)
- Citrullinemia type II (CIT-II, citrin deficiency)
- Defects of biopterin cofactor biosynthesis (BIOPT-BS)
- Defects of biopterin cofactor regeneration (BIOPT-RG)
- Homocystinuria (HCY, cystathionine beta synthase)
- Hyperphenylalaninemia (H-PHE)
- Hypermethioninemia (MET)
- Maple Syrup Urine Disease (MSUD, branched-chain ketoacid dehydrogenase)
- Phenyleketonuria (PKU, phenylalanine hydroxylase)
- Tyrosinemia type I (TYR-1, fumarylacetoacetate hydrolase)*
- Tyrosinemia type II (TYR-II, tyrosine aminotransferase)
- Tyrosinemia type III (TYR-III, hydroxyphenylpyruvate dioxygenase)
Fatty Acid Oxidation Disorders
Babies born with one of these disorders have trouble burning fat for energy. This can lead to vomiting, low blood sugar or more serious problems such as coma or death if untreated. Treatment depends on the disorder that a baby has, but may include a special diet and medication. A baby with a fatty acid oxidation disorder must have regular medical care by an experienced physician. The Newborn Screening Program will screen for:
- Carnitine/acylcarnitine translocase defect (CACT)
- Carnitine Uptake Deficiency (CUD, carnitine transport defect)*
- Carnitine palmitoyl transferase deficiency I (CPT-1a)
- Carnitine palmitoyl transferase deficiency II (CPT-II)
- Dienoyl-CoA reductase deficiency (DE-RED)
- Glutaric acidemia type II (GA-II, multiple acyl-CoA dehydrogenase deficiency)
- Long-chain hydroxyacyl-CoA dehydrogenase deficiency (LCHAD)
- Medium-chain acyl-CoA dehydrogenase deficiency (MCAD)
- Medium-chain ketoacyl-CoA thiolase deficiency (MCKAT)
- Medium/Short chain L-3-hydroxy acyl-CoA dehydrogenase deficiency (M/SCHAD)
- Short-chain acyl-CoA dehydrogenase deficiency (SCAD)
- Trifunctional Protein Deficiency (TFP)
- Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD)
Organic Acid Disorders
A baby with one of these disorders cannot remove certain waste products from their blood. Without treatment, the buildup of these waste products causes serious medical problems affecting the baby's health and development, including seizures, coma and brain damage. Treatment depends on the disorder, but may include special diets, supplements and medications. A baby with an organic acid disorder must have regular medical care by an experienced physician. The Newborn Screening Program will screen for:
- 2-Methyl-3-hydroxybutyric aciduria (2M3HBA)
- 2-Methylbutyryl-CoA dehydrogenase deficiency (2MBG, SBCAD)
- 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency (HMG)
- 3-Methylcrotonyl-CoA Carboxylase Deficiency (3MCC)
- 3-Methylglutaconic aciduria (3MGA, Type I hydratase deficiency)
- Beta Ketothiolase (BKT, mitrochondrial acetoacetyl-CoA thiolase, short-chain ketoacylthiolase)
- Glutaric acidemia, type I (GA-1, glutaryl-CoA dehydrogenase)
- Isobutyryl-CoA dehydrogenase deficiency (IBG)
- Isovaleric acidemia (IVA, Isovaleryl-CoA dehydrogenase)
- Malonic acidemia (MAL, malonyl-CoA decarboxylase)
- Methylmalonic Acidemia (Cbl A,B; vitamin B12 disorders)
- Methylmalonic acidemia (CBL C,D)
- Methylmalonic Acidemia (MUT, methylmalonyl CoA mutase)
- Multiple Carboxylyse Deficiency (MCD, holocarboxylase synthetase)
- Propionic acidemia (PROP, propionyl-CoA carboxylase)
Are Newborn Screens required in Missouri?
Yes, state law RSMo 191.331 requires all newborns born in Missouri to be screened.
A parent may decline due to religious grounds. These grounds must be stated in writing and the written objection filed with the attending physician, certified nurse, midwife, public health facility or hospital. A copy of the written objection will be sent to the Department of Health and Senior Services.
How is the Screening done?
The newborn's heel is pricked and a few drops of blood are put on a special filter paper. This is done before your baby is discharged from the hospital. The filter paper is sent by courier or mailed to the Missouri State Public Health Laboratory, Newborn Screening Laboratory.
If your baby is born at home, you need to make an appointment with your baby's doctor to have a newborn blood-spot screening test. Preferably, this test should be done at 24 – 48 hours after birth.
How are the screening results reported and to whom?
The test results are reported by the Newborn Screening Laboratory, by mail, three to five days after the filter paper sample was received, to both the doctor who saw your baby while in the hospital and to the hospital where your baby was born. If your baby has a high risk result, the Newborn Screening Laboratory calls and faxes a copy of the high risk result immediately to the doctor who saw your baby in the hospital. This is often done within 30 hours or less after the Newborn Screening Laboratory receives the newborn screening sample.
It is very important that the hospital and the doctor have the parent's correct last name, telephone number, and address, in case a repeat newborn screen is needed. Infants with unclear or borderline results need to be rescreened. If the repeat newborn screen indicates that further evaluation is needed, the Newborn Screening Laboratory will contact the baby’s doctor immediately.
If you don't have a telephone, please leave the phone number of a neighbor or relative with the doctor or hospital. You can also help by notifying your baby's doctor if you move after the baby is born, then if your baby should need to be retested, your baby's doctor will know where to reach you. Remember, time is very important. If your baby's doctor is someone other than the physician at the hospital, then your current physician will not have a copy of the baby's newborn screen result. It is important that parents tell hospital staff who their baby’s doctor is going to be.
How do I find out about my baby's results?
Your doctor should go over the results of your baby's newborn screening results with you during a well baby visit. If not, please ask.
If I get notified that my baby needs to have the screening repeated does it mean my baby has one of these 67 conditions?
Not necessarily. There are several reasons why your baby's doctor or the hospital may tell you that your baby needs to be retested. Some reasons include:
Unsatisfactory specimen: The filter paper sample cannot be tested because: there was not enough blood to complete all the required screening tests, there was too much blood on the sample, or due to other reasons.
"Too Early" specimen: If the blood specimen was collected before the baby was 24 hours old, a repeat newborn screen should be done as soon as possible to avoid missing a disorder.
Abnormal: The Newborn Screening Laboratory will call the doctor right away on all results that are moderate to high risk. Screening results that are low to borderline risk usually only require a repeat screen.
Note: Premature or low birth weight newborns are more likely to have out of the expected range test results on the first specimen, even if a disorder is not present.
Again, if your baby needs a repeat newborn screen or a diagnostic test, it is important that you follow through with the doctor or hospital’s recommendations for additional screening or testing. This will help to avoid missing a disorder.
Can these disorders be cured?
At this time there is no known cure for these disorders. The serious effects of many of the disorders can be prevented or lessened if a special diet, medical treatment, or other intervention is started early.
Missouri Department of Health & Senior Services