What is Newborn (Blood Spot) Screening? Newborn Bloodspot - - PDF document

what is newborn blood spot screening
SMART_READER_LITE
LIVE PREVIEW

What is Newborn (Blood Spot) Screening? Newborn Bloodspot - - PDF document

10/23/2019 What is Newborn (Blood Spot) Screening? Newborn Bloodspot Screening, EVERY baby born in Nebraska gets the screen. What Home Visitors Collected at hospital after 24 hours but before 48 Need to Know(and then some) hours


slide-1
SLIDE 1

10/23/2019 Helping People Live better Lives. 1

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 1

Newborn Bloodspot Screening, What Home Visitors Need to Know…(and then some)

Recharge for Resilience Conference Kearney, Nebraska October 30, 2019

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 2

What is Newborn (Blood‐Spot) Screening?

  • EVERY baby born in Nebraska gets the screen.
  • Collected at hospital after 24 hours but before 48

hours after birth.

  • Five drops of blood from a heel‐stick.
  • Shipped to lab in Pennsylvania.
  • Tested for 32 rare diseases.
  • (will share the list in a bit)

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 3

Why is it done?

  • You can’t tell by how the baby looks or acts that they have

these diseases.

  • The diseases can cause severe damage to the brain,

deafness, blindness, organ damage, nerve and muscle damage, chronic illness and some even result in seizures, strokes or infant death.

  • The test is the best way to detect these diseases before

damage has occurred and symptoms begin.

  • For each of these rare diseases, there is an

effective treatment to prevent these problems!

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 4

Is it accurate?

  • The tests are highly sensitive and specific. However, they are a “screen” so:
  • Some will be False Positives
  • False Negatives are possible (extremely infrequent)
  • The screening tests are NOT diagnostic. A positive screen means that baby is at

higher risk of having the disease, and must have further testing.

  • Sometimes specimens are not acceptable for testing (e.g. not enough blood on

the filter paper cards) so repeat screens are needed.

  • Sometimes specimens are collected too early, or too close to a

transfusion, so they can’t be tested for some of the diseases.

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 5

What’s the hurry?

  • For some of the diseases the damage to the brain or other organs

begins in the first days to weeks of life.

  • The earlier we can get to a diagnosis and treatment the better it is for

the baby. If there are serious delays, there could be irreversible

  • damage. Treatment would still prevent further harm. But the goal is to

prevent harm.

  • For some of the diseases just not knowing, can result in infant death.

New moms might not recognize baby is not feeding well. If that baby has one of the metabolic diseases where “poor feeding” is a life threatening risk for triggering a metabolic crisis, they can die in the first days to weeks of life.

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 6

Just how rare are these diseases?

  • Some are found in only 1:100,000 babies, others as are as

common as 1:6,000.

  • Of the group of diseases screened, the most common diseases

are Cystic Fibrosis and Congenital Primary Hypothyroidism.

  • In Nebraska 1:500 to 1:600 babies will have one of these

screened diseases each year.

  • Collectively in Nebraska we usually find between 50‐60 babies

every year with one of these diseases.

slide-2
SLIDE 2

10/23/2019 Helping People Live better Lives. 2

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 7

What diseases are screened?

  • Metabolic diseases (Amino Acid, Fatty Acid and Organic

Acid disorders – 20)

  • Endocrine diseases (2)
  • Hemoglobinopathies (3)
  • Lysosomal Storage Diseases (2)
  • Other Metabolic (2)
  • Immunodeficiency (1)
  • Peroxisomal Storage Disease (1)
  • Cystic Fibrosis (Not characterized as a single type of

disease due to multiple systems affected).

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 8

  • Argininosuccinic Acidemia (AA)
  • Beta Ketothiolase Deficiency (BKT)
  • Biotinidase Deficiency (BIO)
  • Carnitine Uptake Defect (CUD)
  • Citrullinemia (CIT)
  • Congenital Adrenal Hyperplasia (CAH)
  • Congenital Primary Hypothyroidism (CPH)
  • Cystic Fibrosis (CF)
  • Galactosemia (GAL)
  • Glutaric Acidemia Type I (GAI)
  • Homocystinuria (HCY)
  • Isovaleric Acidemia (IVA)
  • Long Chain Hydroxy‐Acyl Co‐A Dehydrogenase

Deficiency (LCHAD)

  • Maple Syrup Urine Disease (MSUD)
  • Medium Chain Acylcarnitine Deficiency (MCAD)
  • Methylmalonic Acidemia (Mutase) (MMA)
  • Methylmalonic Acidemia (Cbl B & C) (MMA)
  • Muccopolysaccharidosis Type I (MPS‐I)
  • Multiple Carboxylase Deficiency (MCD)
  • Phenylketonuria (PKU)
  • Pompe Disease (PD)
  • Propionic Acidemia (PA)
  • Severe Combined Immune Deficiency (SCID)
  • Sickle Beta Thalassemia (S Beta‐Thal)
  • Sickle Cell Disease (S Disease)
  • Sickle Hemoglobin C Disease (SC Disease)
  • Trifunctional Protein Deficiency (TFP)
  • Tyrosinemia (TYR)
  • Very Long Chain Acylcarnitine deficiency (VLCAD)
  • 3‐Hydroxy‐3‐Methylglutaric Aciduria (3‐HMG)
  • 3‐Methylcrotonyl‐CoA Carboxylase

Deficiency (3‐ MCC)

  • X‐linked Adrenoleukodystrophy (X‐ALD)

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 9

  • Argininosuccinic Acidemia (AA)
  • Beta Ketothiolase Deficiency (BKT)
  • Biotinidase Deficiency (BIO)
  • Carnitine Uptake Defect (CUD)
  • Citrullinemia (CIT)
  • Congenital Adrenal Hyperplasia (CAH)
  • Congenital Primary Hypothyroidism (CPH)
  • Cystic Fibrosis (CF)
  • Galactosemia (GAL)
  • Glutaric Acidemia Type I (GAI)
  • Homocystinuria (HCY)
  • Isovaleric Acidemia (IVA)
  • Long Chain Hydroxy‐Acyl Co‐A Dehydrogenase

Deficiency (LCHAD)

  • Maple Syrup Urine Disease (MSUD)
  • Medium Chain Acylcarnitine Deficiency (MCAD)
  • Methylmalonic Acidemia (Mutase) (MMA)
  • Methylmalonic Acidemia (Cbl B & C) (MMA)
  • Muccopolysaccharidosis Type I (MPS‐I)
  • Multiple Carboxylase Deficiency (MCD)
  • Phenylketonuria (PKU)
  • Pompe Disease (PD)
  • Propionic Acidemia (PA)
  • Severe Combined Immune Deficiency (SCID)
  • Sickle Beta Thalassemia (S Beta‐Thal)
  • Sickle Cell Disease (S Disease)
  • Sickle Hemoglobin C Disease (SC Disease)
  • Trifunctional Protein Deficiency (TFP)
  • Tyrosinemia (TYR)
  • Very Long Chain Acylcarnitine deficiency (VLCAD)
  • 3‐Hydroxy‐3‐Methylglutaric Aciduria (3‐HMG)
  • 3‐Methylcrotonyl‐CoA Carboxylase Deficiency (3‐

MCC)

  • X‐linked Adrenoleukodystrophy (X‐ALD)

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 10

Why is this relevant to Home Visitors bef before re babies are born?

  • New mom’s have so much to absorb.

Preparing them ahead of time makes it easier for them after baby is born.

  • Home visitors can help prepare moms

before baby arrives with simple messages:

  • It’s the law for every baby to get it.
  • It’s one small heel prick.
  • It can give your family and the baby’s doctor

valuable information.

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 11

Wh Why is is this this re relevant to to Hom Home Vi Visit sitors af after bab babie ies ar are born born?

  • Results are available on average at about 5 days of age.
  • Ask mom if her baby’s doctor provided the results to her.
  • Some baby’s need repeat screens.
  • Ask mom if her baby needed to have a repeat screen or any
  • ther follow‐up testing. If she hasn’t had it done, find out if

there are barriers.

  • If mom has any paperwork for the follow‐up, it may

explain why it’s needed. (NNSP provides Parent Fact Sheet to baby’s doctor to give to parent when results need confirmatory testing.)

  • You may be able to help her understand the

information if she is unclear.

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 12

Other ways Home Visitors can help

  • If there is a delay in getting the follow‐up, or any reluctance:
  • You can remind mom, it is important to do the follow‐

up in case baby does have the disease, so treatment can begin as soon as possible.

  • If mom is experiencing anxiety about the results:
  • You can remind mom, it’s just a screen result, not a
  • diagnosis. It could be a false positive. But again, if her

baby does have the disease, it’s treatable! The sooner we begin treatment, the better!

  • If mom has more questions:
  • You can refer her to contact her baby’s doctor,
  • r to call the NNSP.
slide-3
SLIDE 3

10/23/2019 Helping People Live better Lives. 3

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 13

Nebraska Specific Resources

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 14

National Resources

Baby’s First Test

Very good site for parents and the general public.

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 15

National Resource

NewSTEPS

National newborn screening resource center Good site for comparing differences between State Newborn Screening Programs (what they screen for, what it costs, etc.)

H el pi ng Peopl e Li ve B et t er Li ves. H el pi ng Peopl e Li ve B et t er Li ves. 16

dh dhhs.ne.gov

@NEDHHS @NEDHHS NebraskaDHHS

DHHS Program Manager II Nebraska Newborn Screening Program

Juli Julie Lued Luedtk tke

AFTER HOURS LINE 402 471‐0374

Julie.Luedtke@Nebraska.gov

402 471‐6733

http://dhhs.ne.gov/Pages/Newborn‐Screening.aspx