NYMAC New York

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NYMACNew York Mid-Atlantic Consortium for
Genetic and Newborn Screening Services

• Katharine B. Harris, MT(ASCP), MBA NYS
  Department of Health

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Background on Newborn Screening

• NBS began in 1963 after Dr. Robert Guthrie
  developed an inexpensive way to test dried blood
  spots for phenylketonuria using special bacteria.
• NYS started screening for PKU in 1965
• Over 11 million infants have been screened for
  PKU.
• 511 NYS children have been diagnosed with PKU
  before the build-up of phenylalanine had the
  chance to cause severe mental retardation.

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Background on Newborn Screening

• Nationally all states include PKU, congenital
  hypothyroidism, galactosemia and sickle cell
  disease in their panels.
• HRSA, MOD and ACMG recommend a list of conditions
  that should be included in all state NBS panels
• Based on NYS data and the number of US births in
  2003, more than 9,000 infants would have been
  diagnosed with one of the diseases in the
  recommended panel
• Delay in diagnosing most of these conditions
  until clinical symptoms appear causes
  irreversible physical and/or mental retardation
  or death.

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So what happens when the lights go out or the
water rises?
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Emergency Preparedness

• To prepare procedures, agreements and collect
  information that will be needed in case of a NBS
  Program shut-down
• To test plans for flexibility, completeness and
  responsiveness

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Scope of Activity

• The state newborn screening level through
  Wadsworth Center
• The regional level through the (NYMAC) work group
• The national level through the National
  Coordinating Centers Emergency Preparedness work
  group

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Members of NYMAC Work Group 3
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What does it take to shut down a newborn
screening program?

• Natural disasters such as hurricanes, floods, ice
  storms, blizzards, earthquakes
• Terrorism
• Politics
• National Days of Mourning
• Accidents
• Equipment malfunctions
• Pandemics
• Other things that no one has yet thought of

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New Orleans, August 2005 Hurricanes
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Sidney, NY, June 2006 Floods
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Trenton, NJ, June 2006 - Floods
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Atlantic City, NJ, July 2006 Politics
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Adirondacks, January 1998 Ice Storms
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North Ridge, CA, October 1994 - Earthquake
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New York City, September 2001 Terrorism
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Washington, January 2007 Funerals
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Albany, NY, June 1993 Local Power Outages
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Northeast 2003 Regional Power Outages
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Ireland, February 2007 - Pandemics
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Any Place, Any Time - Equipment Failure
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Collection of Specimen Birth Hospitals
Newborn Screening Programs
X
Transport of Specimens by Couriers
Receipt of Specimen NBS Lab
Data Entry of Demographic Info
Testing for multiple analytes in several
laboratories using different methodologies
X
X
Posting results in data system
Reporting results to HOB, PCP, SCC
Contacting family for follow-up SCC, HOB, PCP
Ongoing access to Medical Care,
Drugs, Supplements, Special Foods, from
Pharmacies, Suppliers, Manufacturers
Clinical evaluation, confirmation
testing, diagnosis, treatment plan SCC, PCP
X
X
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Specimen Collection

• NBS Program must have contact information for all
  birthing centers
• Centers must have adequate supplies of blood
  collection forms
• Birthing centers must understand the relevance of
  NBS and the potential impact that delay in
  screening could cause
• Sufficient nursery staff must be trained in blood
  collection, handling and transporting

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Specimen Delivery

• Usual and alternate couriers must be identified
  and contact information maintained
• USPS
• FedEx
• UPS
• Private couriers

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Specimen Testing

• Lists with emergency contact information
• Lab staff
• Equipment maintenance contractors
• Reagent/consumable suppliers
• Memoranda of Understanding/ Agreements with other
  NBS Labs

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Reporting Specimen Results

• Lists of emergency contact information
• NBS Follow-up staff
• Hospitals of birth
• Primary care providers
• Specialty care centers

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Database Back-up

• Most programs have automatic back-up or real-time
  mirroring of the NBS database
• Storage should be off-site, ideally in more than
  one location with one of the locations
  geographically distant from the laboratory
• CDC is proposing to serve as a repository of
  state NBS back-up databases

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Follow-up of Children Diagnosed through NBS

• Lists of emergency contact information
• Specialty care centers
• Food/Supplement/Drug Suppliers
• Pharmacy chains
• Specialty care centers must maintain current
  lists of patient contact and medical status
  information off-site!

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Collection of Specimen
Newborn Screening Programs
Communication with everyone
Transport of Specimen
Receipt of Specimen
Data Entry of Demographic Info
Testing in several laboratories using several
methodologies
Posting results in data system
Reporting results to HOB, PCP, SCC
Contacting family for follow-up
Clinical evaluation, confirmation
testing, diagnosis, treatment plan
Ongoing access to medical care,
drugs, supplements, foods
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The NBS Program must provide information to all
concerned about program and emergency status to
reduce confusion

• Government officials
• Staff
• Back-up laboratories
• Suppliers
• Courier services
• Parents

• Hospitals
• Treatment centers
• Physicians
• Public health programs
• The public

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All modes of communication must be considered and
availability determined during the emergency

• Telephone
• E-mail
• Public Service Announcements
• School Watch Broadcasts
• Websites
• Emergency Management Sites

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When to Declare an Emergency

• The nature of the emergency
• What part of the program is down?
• How long will the program be affected?
• The extent of the emergency
• Are neighboring states affected?
• How important is newborn screening in relation to
  the scope of the emergency?

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Back-up Lab Considerations

• What are the number of births, NBS panel, testing
  methodologies and interpretation of results in
  affected state?
• What is the capacity and technology of the
  back-up lab?
• National Newborn Screening and Genetic Resource
  Center (NNSGRC) has information about all state
  programs on their website

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• MEMORANDUM OF AGREEMENT
• between
• State of West Virginia
• Department of Health and Human Resources
• Bureau for Public Health
• Office of Laboratory Services
• and
• State of Maryland
• Department of Health and Mental Hygiene
• Laboratories Administration

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Identifying One or More Back-up Laboratories

• Many NYMAC states have formal agreements for
  back-up
• Other states have found that formal negotiations
  between and among state legal authorities can bog
  down regarding payment, ownership,
  responsibilities and liability
• Informal agreements seem to be easier

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Emergency Management Assistance Compact - EMAC

• All 50 states, DC, Puerto Rico, Virgin Islands
  are signators
• EMAC provides form and structure to interstate
  mutual aid.
• Through EMAC, a disaster-impacted state can
  request and receive assistance from other states
  quickly and efficiently
• Two key issues are resolved by the compact
  liability and reimbursement.

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Choices

• Can one, or more, other programs meet the needs
  of the affected program?
• For example, New York screens as many as 1300
  specimens each work day
• Neighboring states are much smaller
• NYS would have to divide specimens, between two
  or more other states

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Choices

• Can the back-up lab(s) test for all the analytes
  on the affected states panel?
• Can some analytes/conditions be delayed until the
  emergency is resolved? i.e. sickle cell, cystic
  fibrosis
• Are some conditions so time-dependent that
  delaying screening would cause irreparable harm?
  i.e. PKU, MSUD, Krabbe disease

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Choices

• Can blood spot cards already screened for
  selected analytes be used to screened for missed
  analytes
• Can the affected lab retroactively screen
  specimens delayed or lost in transport?
• Can infants not screened be tested once the
  emergency is resolved?

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Choices

• Who owns the specimens?
• How will the back-up lab be compensated for
  costs?
• How will differences in cut-off levels be
  handled?
• How will the database of the affected lab be
  brought up-to-date once the emergency is
  resolved?
• Etc.

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Central Laboratory Proposal

• NYMAC, APHL and CDC have been discussing the
  possibility of creating a separate, full-service
  newborn screening laboratory that would have all
  capabilities and all methodologies.
• Perhaps at CDC
• State lab staff could train there annually to
  maintain proficiency and drill emergency
  procedures

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Central Laboratory

• Iowa Hygienic Laboratory went to 24/7 operation
  after Katrina to do Louisianas NBS
• They are developing plans for permanent capacity
  to act as back-up during emergencies

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Emergency Preparedness Drills

• Regardless of the efforts made to develop back-up
  plans, it is vital that the plans be tested
• The effectiveness of drills must be evaluated
• CDC and APHL are involved with states in training
  for public health emergencies
• Region 5 Heartlands has proposed a project to
  develop drill scenarios.
• NBS must work with state emergency programs in
  their system drills