Welcome to the Clinical Center Grand Rounds,
a weekly series of educational lectures for physicians and
health care professionals broadcast from the Clinical
Center at the National Institutes of Health in
Bethesda, MD.
The NIH Clinical Center is the world's largest hospital totally
dedicated to investigational research and leads the global
effort in training today's investigators and discovering
tomorrow's cures.
Learn more by visiting us online at http://clinicalcenter.nih.gov
OUR 5 PRESENTERS TODAY ARE:
DR. PRECILLA D'SOUZA, SHE
RECEIVED HER BACHELOR'S AND
MASTERS DEGREES IN NURSING AND
PEDIATRICS FROM THE COLLEGE OF
NURSING IN INDIA KNOW.
SHE COMPLEAD HER PEDIATRIC NURSE
PRACTITIONER TRAINING AND
DOCTORATE IN NURSING PRACTICE
FROM THE UNIVERSITY OF MARYLAND
SCHOOL OF NURSING SHE HONED HER
PEDIATRIC SKILLS IN PRIMARY
CARE, PEDIATRIC SURGERY,NYONATAL
INTENSIVE CARE, HOSPITALS
THROUGHOUT MARYLAND.
IN 2010 DR. D'SOUZA JOINED THE
NIH AND IN 2016 HE WAS RECRUITED
TO THE NATIONAL GENOME HUMAN
RESEARCH INSTITUTE NIH
UNDIAGNOSED DISEASES PROGRAM.
IN 2019 DR. CYNTHIA TIFT AND HER
COLLABORATORS AND HER
COLLABORATORS IN GENE THERAPY
FOR CHILDREN WITH GM1 INCASLIO
SIDOSEIS, A UNIFORMLY FATAL
LYSOSOMAL STORAGE DISORDER WITH
NO APPROVED TREATMENT.
MISS KRISTA CATO IS EXPERIENCE
IN CRITICAL AND CRITICAL
NURSING.
OVER THE LAST 6 YEARS SHE SERVED
AS SUPERVISORY MANAGER WITHIN
THE NURSING DEPARTMENT WHERE SHE
DIRECTED OPERATIONS TO PRODUCE
HIGH RELIABILITY SYSTEMS.
SHE RECENTLY TRANSITIONED TO THE
NATIONAL INSTITUTE OF ALLERGY
AND INFECTIOUS DISEASE AND NOW
SERVE AS AS A NURSE CONSULTANT
WITHIN THE DIVISION OF
MICROBIOLOGY AND INFECTIOUS
DISEASES.
DR. ROSA ROUSSEAU IS A
BEHAVIORAL NURSE AT THE CLINICAL
CENTER FOR OVER 25 YEARS OF
PEDIATRIC NEONATAL HIGH RISK
MATERNITY AND FLIGHT NURSING
EXPERIENCE.
DR. ROUSSEAU HOLDS A DOCTORATE
IN NURSING PRACTICE AND MASTERS
IN NURSING EDUCATION, SHE'S
EXPERIENCED IN HEALTHCARE
SIMULATION BEST PRACTICES AND
HOLDS CERTIFICATIONS AS A HELGTD
CARE SIMULATION EDUCATOR, HIGH
RISKNYONATAL HUSBANDERRING AND
IN PEDIATRICNYONATAL TRANSPORT.
SHARING HER PASSION FOR
ADVANCING THE SCIENCE OF
HEALTHCARE SIMULATION, C
ROUSSEAU HAS PRESENTED AT
NATIONAL AND INTERNATIONAL
CONFERENCES IN A VARIETY OF
SIMULATION TOPICS TO ENHANCE
PATIENT SAFETY AND QUALITY OF
CARE.
DR. PATRICIA TODD IS THE
CLINICAL PEDIATRIC SPECIALIST
FOR CRITICAL CARE AND SEDATION
SERVICES AT THE CLINICAL
TRUSTEES SINCE 2017.
DR. TODD'S PEDIATRIC EXPERIENCE
CROSSES IN-PATIENT AND
OUTPATIENT CARE AND HAS
PRACTICED IN NEONATAL INTENSIVE
CARE, PEDIATRIC CRITICAL CARE,
PEDIATRIC EMERGENCY, NURSING
MANAGEMENT, NURSING EDUCATION,
AND ADVANCED PROJECTIS NURSING
AS A CLINICAL NURSE SPECIALIST.
DR. MYRA WOOLERY IS A CLINICAL
SPECIALIST FOR PEDIATRICS AT THE
NIH CLINICAL CENTER.
SHE HAS BEEN COVERING THE
PEDIATRIC PROGRAM OF CARE WHICH
SEES A VARIETY OF MEDICAL
CONDITIONS SUCH AS ENDOCRINE,
GENETICS, HEMATOLOGY, ONCOLOGY,
IMMUNE DEFICIENCIES AND RARE
DISEASES FOR OVER 35 YEARS.
DR. WOOLERY RECEIVED HER BS FROM
THE YRT OF MARYLAND, MASTER IN
NURSING AND PARENT AND CHILD
NURSING FROM THE UNIVERSITY OF
WASHINGTON, IN SEATTLE AND HER
Ph.D. FROM THE UNIVERSITY OF
MARYLAND BALTIMORE WITH A
SPECIALIZATION IN SYMPTOM
MANAGEMENT AND MEASUREMENT.
SHE IS A CERTIFIED PEDIATRIC
ONCOLOGY NURSE AND AN APON CHEMO
BIOCHER ACTIVITIES AND PROJECTSY
INSTRUCTOR, LET US BEGIN WITH
DR. D'SOUZA.
>> THANK YOU DR. JORDAN.
CAN YOU SEE OUR SLIDES?
THEY'RE JUST POPPING UP.
>> YES.
>> SO THIS IS THE YEAR OF
DIVERSITY AND INCLUSION, HERE IS
OUR ALL WOMEN TEAM FROM 5
CONTINENTS FROM IN THE CENTER
YOU SEE DR. CYNTHIA CHEN HO IS
THE PRINCIPLE INVESTIGATOR AND
EXPERT IN GM1 AND 2 GANG
LIAISONOSEIS DISORDYS.
OR TEAM HAS BEEN STUDYING THE
NATURAL HISTORY OF THESE
DECEASES FOR OVER A DECADE.
NEXT SLIDE, PLEASE, WHAT YOU SEE
ON GINGLIO SIDOSEIS.
--WITH INCIDENCE OF 1 IN HUNDRED
THOUSAND TO 200,000.
MUTATION IN THE GENE CAUSES
ENZYME DEFICIENCY LEADING TO A
TOXIC ACCUMULATION OF THE
GANGLIO CITE PREDOMINANTLY IN
THE CENTRAL NERVOUS SYSTEM, BUT
ALSO IN PERIPHERAL TISSUES
INCLUDING HEART, LIVER, EYES AND
BONES.
[INDISCERNIBLE] ARE FORMALLY
FATAL AND THERE ARE NO MODIFYING
DISEASE TREATMENTS CURRENTLY
AVAILABLE.
MOST PATIENTS ARE GLB1 GENE,
NEXT SLIDE, PLEASE.
HUMAN GANGLIO SIDOSEIS, IS
DISEASE, DISEASE PROGRESSION
DEPENDING ON THE ACTIVITY, TYPE
1 FORM OR INFANTILE IS THE MOST
SERIES WITH SYMPTOM ONSET BEFORE
12 MONTHS OF AGE AND THAT OFFER
BEFORE AGE 2, THE CLINICAL
FINDINGS OF HYPER TONIA AND
DEVELOPMENT OF DELAY AND
RECOGNITION OF THE COMMON
FINDINGS, THE SYMPTOMS RESULTING
FROM SERIOUS CENTRAL NERVOUS
SYSTEM DEGENERATIONS ARE
EVIDENCE BY PRESENCE OF
[INDISCERNIBLE], OTHER SYMPTOMS
INCLUDE [INDISCERNIBLE],
SKELETAL DISPLASSIA AND CARDIO
MYOPATHY, TYPE 2 BROKEN INTO
WOSUBTYPES DEPENDING ON AGE OF
ONSET AND SEVERITY.
LATE INFANT -ILE AND JUVENILE.
LATE INFANTILE THE SYMPTOM
BETWEEN 12-18 MONTHS OF AGE
CHARACTERIZED BY DEVELOPMENT OF
[INDISCERNIBLE] OR REGRESSION.
JUVENILE, THE SIM TOM ONSET
BETWEEN 3-5 YEARS OF AGE, WITH
THE PLATEAUING AND DEVELOPMENT
OF MILESTONES, THE PRIMARY
SYMPTOMS ARE FREQUENT QUALS FOR
ACCORD NATION, [INDISCERNIBLE]
AND COGNITIVE DECLINE.
THE LAST TYPE, TYPE 3 LATE OR
ADULT ONSET BEGINS IN THE TEENS
OR 20S WITH THE EXTRA
[INDISCERNIBLE] SIGNS, GATE,
DISTURBANCE AND CARDIOMYOPATHY.
NEXT SLIDE, PLEASE.
HERE WE SHORTENED THE BRAIN MRI
AND MR SPECTROSCOPY DATA OF 15
LATE INFANTILE AND JUVENILE
PATIENTS WHO PARTICIPATE INDEED
THE NATURAL HISTORY.
UNFORTUNATELY WE DON'T HAVE
INFANTS DATA BECAUSE THEY WERE
TOO SICK TO TRAVEL.
THE DATA SHOWS THE SEVERITY OF
DISEASE IN LATE INFANTILE
COMPARED TO JUVENILE PATIENTS,
IN THE LEFT SLIDE, ATROPY
PLOTTED AS TD PERCENTAGE DEFICIT
RELATED TO THE PUBLISHED TOTAL
BRAIN WALLING FOR THE SUBJEKS
AGE.
THERE IS A CLEAR SEPARATION
BETWEEN THE LATE INFANTILE AS
SHOWN IN THE FIELD DIAMONDS AND
JUVENILE AS SHOWN IN OPEN
SQUARES AS TO THE GREAT VOLUME
LOSS IN THE RIGHT SLIDE YOU SEE
A STRONG CORRELATION IN THE
[INDISCERNIBLE] OR NAA DEFICIT,
TO THE PERCENTAGE OF ATROPY.
BUT THE SLOPES ARE DISTINCTLY
DIFFERENT FOR THE LATE INFANTILE
AGAIN IS SHOWED IN CLOSED
DIAMONDS AND JUVENILE SUBTYPES.
NEXT SLIDE, PLEASE.
HERE WE SHOW THE WALKING VIDEO
OF TYPE 2 JUVENILE PATIENT WHO
IS PART OF A [INDISCERNIBLE] AND
GANGLIO SIGHT KNOCK OUT MOUSE.
HAQUE IS THE RATIONAL FOR
TREATMENT OF GANGLIO SIDOSEIS
USING AAV9 GLB1 GENE REPLACEMENT
THERAPY, WE HYPOTHESIZED THAT
THIS COULD BE CORRECTED TO
LEVELS SUFFICIENT TO HALT OR
REVERSE THE PROGRESSION OF
DISEASE BY INTRODUCING A
TRANSGENE CONTAINING GLB1.
AAV9 HAS BEEN SHOWN TO CROSS THE
BLOOD BRAIN BARRIER THAT ARE
THEN EXPRESSED WIDELY IN THE
CENTRAL NERVOUS SYSTEM.
THE EFFICACY OF AAV9 GLB1
DELIVERY HAS BEEN DEMONSTRATE
INDEED PRECLINICAL MOUSE MODEL
OF TYPE 2 GM1 GANGLIO SIDOSEIS,
MICE RECEIVING A SINGLE
INTRAVENOUS DOSE SURVIVED 90%
LONGER THAN AVENGHTED CONTROL
MICE.
SURVIVAL WERE ACCOMPANIED BY
NEURAL MUSCULAR FUNCTION AND
COGNITIVE BEHAVIOR.
THE EFFICACY OF THE GENE
INTRAVENOUS DELIVERY HAS ALSO
BEEN DEMONSTRATED BY PRECLINICAL
STUDIES CONDUCTED AT AUBURN
UNIVERSITY IN FELINE MODEL OF
GANGLIO SIDOSEIS.
THE 2--THE 2 GM1 TREATED WITH
AAV9-GLB1, 42 MONTHS THE TREATED
ONLY DEVELOPED A SLIGHT HIND
LIMB WEAKNESS AND WHILE THE
HUMAN END POINT FOR
[INDISCERNIBLE] IS REACHED AT 8
MONTHS.
THE STRONG POSITIVE CLINICAL
RESPONSE AFTER INTRAVENOUS GENE
THERAPY IS SUPPORTED BY BRAIN
MRI AND THE MR SPECTROSCOPY
FINDINGS.
THE STUDIES CONDUCTED IN THE
MOUSE AND CAT MODELS PROVIDE A
RATIONAL FOR DESIGN OF A HUMAN
CLINICAL STUDY AND STRONGLY
SUPPORT POTENTIAL EFFICACY IN
HUMANS.
NEXT SLIDE, PLEASE.
HERE WE SEE VIDEO OF TREATED AND
UNCRETED CATS.
HERE ARE THE PROTOCOL DEAILS
AAV9-GLB1 IS A FIRST VECTOR
CONTAINING GLB1 GENE, IT IS
STAGE 1 SUBJECTS TREATED WITH
THE LOW DOSE AND THE HIGH DOSE
VECTOR, THE STARTING LOW DOSE
WAS CHOSEN BECAUSE IT SHOWS
STRONG EFFICACY IN THE MODEL OF
GM1, 2 TYPE FOLLOW THE SAME
IMMUNE MODULATION, AND SUBJECTS
WILL BE ENROLLED AT A HIGHER
DOSE, THE TYPE 2 PATIENTS WILL
BE ASSISTED AT 3 YEARS WITH THE
23 YEAR LONG-TERM FOLLOW UP AND
TYPE 1 PATIENT WILL HAVE OUTCOME
ASSIST AT 1 YEAR WITH THE 4 YEAR
LONG-TERM FOLLOW UP.
NEXT SLIDE, PLEASE.
THE CRITERIA ARE THOAN ON THE
LEFT.
ENZYME DEFICIENT SUBJECTS WITH
THE GLB1 WITH THE TYPE ONCE
DISEASE WHRO ARE BETWEEN 6 OF
AGE ARE ELIGIBLE FOR THE STUDY,
SIMILARLY, TYPE 2 PATIENTS AND
GENETICALLY DISEASE BETWEEN 1 OF
2 YEARS OF AGE AND WITH THE
STANDARD SCORE OF GREATER THAN
40 ON 1-3 BEHAVIORAL SKILL ARE
ELIGIBLE FOR ENROLLMENT.
THE KEY STUDY ENPOINTS ARE SHOWN
ON THE RIGHT ARE ENCLOSED SAFETY
AND TOLERABILITY, DISEASE
SEVERITY HAS THIS BY THE
CLINICAL GLOBAL SCALE.
UPRIGHT AND DEVELOPMENT ON THE
ASSESSMENT ON THE 1 AND 3.
THEN [INDISCERNIBLE] AND MR
SPECROSCOPY OF ANY NAA OR
[INDISCERNIBLE] WILL BE USED AS
OUTCOME MEASURE.
HERE WE SHOW THE PROTOCOL
TIMELINE.
WE JUST REVIEWED PRESCREENING
PORTION, THE BASE LINE INCLUDE
IN-PATIENT ADMISSION FOR
DIAGNOSTIC TEST AND PROCEDURES
SUCH AS EEG--NEXT ON DAY 0, THE
PATIENT IS PREMEDICATED WITH
ORAL BEN DRILL AND TYLENOL 30-60
MINUTES BEFORE THE SING WITH THE
GLB1 INFUSION, FOR THE NEXT 3
DAYS, THE PATIENT WILL REMAIN ON
AUTOSTEROIDS, NEXT SLIDE,
PLEASE.
NEXT SLIDE, PLEASE.
BASED ON OUR 8 TYPE 2 PATIENTS
SAFETY DATA, FDA APPROVAL IS
GRANTED TO TREAT INFANTS.
THEN WE OBTAINED CONDITIONAL
APPROVAL FROM CEO DR. GILMAN TO
TREAT INFANTS CONTINGENT UPON
PEDIATRIC AND RESPIRATORY
ELEMENTS IN THE CENTER DURING
THE PATIENT ADMISSION, INFUSION,
PROCEDURES.
HOSPITAL READINESS WAS INITIATED
FOR PATIENT SAFETY.
NOW OUR COLLEAGUE WILL TALK WITH
[INDISCERNIBLE] ANALYSIS.
>> AS YOU SAW FROM DR. D'SOUZA'S
PRESENTATION, REGARDLESS IF
YOU'RE A POODIATRICIAN OR NURSE,
FINDING SOLUTIONS REPRESENT A
PIECE OF THE IMPORTANT WORK THAT
WE ALL DO HERE AT THE NIH, WITH
THE SUPPORT OF EXECUTIVE
LEADERSHIP TO INSURE CLINICAL
READINESS TO CARE FOR INSTABTS
IN THE CLINICAL CENTER, KEY
STAKEHOLDERS FROM 14
DEPARTMENTS, 4 SERVICES,
REPRESENTATIVES FROM CHILDREN'S
NATIONAL AND 1 AMAZING RESEARCH
TEAM CAME TOGETHER, TO CONDUCT
THE DEEP DIVE INTO THE STUDY.
THIS STARTED, NEXT SLIDE IS
PERFORMING AN FMEA, A PROCESS
MANY OF YOU ARE FAMILIAR WITH
AND OFTENUNESSED IN THE CLIPICAL
TRUSTEES TO EVALUATE RISKS.
FOR THIS FIRST IN HUMAN INFANT
GENE THERAPY PROTOCOL, THE
PROSPECTIVE ASSESSMENT
IDENTIFIED 194 PROTENTIAL RISKS,
OR FAILURE MODES AND 8 MAJOR
AREAS.
THIS ANALYSIS HELPED IDENTIFY
VULNERABILITIES AND INSURE
SAFETY MEASURES WERE IN PLACE
ACROSS THE CLINICAL CENTER PRIOR
TO ENROLLING ANY PATIENT.
NEXT SLIDE.
AS PRESILLA STATED THESE INFANTS
AND CHILDREN WOULD RECEIVE A
SERIES OF PROCEDURES AND
TREATMENTS SUCH AS MULTIPLE
BLOOD DRAWS, ANESTHESIA WITH
POSSIBLE INTERNATIONAL
CLASSIFICATION BATION, LUMBAR
PUNCTURES, RETUX MAB INFUSIONS,
AAV9-GLB1 INFUSION AND MORE, ALL
OF WHICH HAVE ASSOCIATED RISKS
ESPECIALLY FOR INFAPTS WITH LOW
TONE AND LOW WEIGHT.
TO MITIGATE THESE, PLANNING WAS
TAKEN TO ADDRESS KEY AREAS
WITHIN THE FMEA, THE EMERGENCY
RESPONSE PLAN NEEDED EXPANDING
TO INCLUDE ADDITIONAL AFTER HOUR
INTENSIVE COVERAGE AND MORE
PEDIATRIC CODE TEAM MEMBERS, THE
SYSTEMS REVEALED, THAT SOME OF
THE DEPARTMENTS DIDN'T HAVE
SUPPLIES NEEDED FOR CARE FOR
INFANTS.
THIS STEMMED FROM RESEARCH LAB
TUBES, BREAST FEEDING SUPPLIES
AND EMERGENCY EQUIPMENT.
POLICIES AND PROCEDURES REQUIRE
DEVELOPING USING BEST PRACTICE
MODELS WHICH NYRA WILL GO OVER
LATER AND IT ALSO BECAME
APPARENT THAT ADDITIONAL
TRAINING FOR CLINICIANS WOULD BE
REQUIRED AT NIH, CHILDREN LESS
THAN 3, WERE NONHISTORICLY
ADMITTED TO THE PEDIATRIC WARD
AND SINCE THESE INFANTS REQUIRE
ADMISSION, AND PROCEDURES AND
TESTS PERFORMED ACROSS THE
CLINICAL CENTER, EXPANDING
CLINICAL COMPETENCE WAS
PARAMOUNT.
NEXT NEXT.
TO BRIDGE THESE GAPS AS PRECIL
LA SHARED, THERAPISTS FROM
CHILDREN'S NATIONAL JOINED THE
CARE OF TEAM OF PROVIDERS INFANT
SUPPLY CARTS WERE ESTABLISHED
AND THE EMERGENCY EQUIPMENT SUCH
AS INFABT GO BAGS WERE CREATED.
NURSING PREPAREDNESS MEASURES
WERE EXTENSIVELY ADDRESSED AND
FURTHER EVALUATIONS WERE
PERFORMED WHICH TRISH WILL NOW
GO OVER.
NEXT.
>> THANK YOU.
>> A GAP ANALYSIS WAS FORMED
FOCUSING SPECIFICALLY ON
CLINICAL COMPETENCE AND THE CARE
OF IPT GREATER FANT, INFANT
SURVEYS FLIES AND NEW PRACTICE
GUIDELINES.
NEXT.
IN FOLK USING THIS ANALYSIS THE
GAPS CAME INTO AREAS OF FOCUSED
ON EDGEUS, THESE WERE INFANT
EQUIPMENT, 2 INFANT EMERGENCY
RESPONSE AND 3, EDUCATION
TRAINING AND COMPETENCIES IN THE
CARE OF INFANTS.
NEXT AS KRIOF THEA MENTIONED
BLOOD VOLUMES AND LAB TUBES
REQUIRED A DEEP DIVE, USING THE
BLOOD TRACKER CALCULATOR AND
WORKING CLOSELY WITH PRESILLA ON
PROCOLLABS WE WERE ABLE TO
INSURE THE BLOOD VOLUME LIMITS.
THIS ILLUSTRATED THE NEED TO
CONTINUED DILIGENCE AND WORK
CONTINUE TO WORK WITH YOUR
STAKEHOLDERS ON INSTITUTING
SMALL VOLUME COLLECTION TUBES.
AS FOR THE SUPPLY CART, 2 CARTS
WERE CREATED 1 FOR THE PEDIATRIC
IN-PATIENT AREA AND 1 FOR ICU.
IN THE EVENT THE INFANT NEEDED
ICU SUPPORT PRIOR TO THE
TRANSFER TO CHILDREN'S NATIONAL.
OBTAINING AND CREATING THE CART
WAS ONLY A PIECE OF IT.
STAFF NEEDS TO BE FAMILIAR WITH
THE CONTENTS, THE CART CONTAINED
CARDIOVASCULAR SUPPLIES, AIR WAY
SUPPLIES, SUCH AS BAG MASK,
NAISAL [INDISCERNIBLE], AND IV
EQUIPMENT AND [INDISCERNIBLE]
EQUIPMENT, FEEDING SUPPLIES,
AMONG OTHER THINGS.
ADDITIONALLY, HAVING AN INFANT
STAY IN PATIENT WHO WAS STILL
BREAST FEEDING GAME WITH ITS OWN
SET OF GAPS.
NEXT, PLEASE.
TO EXPAND EVEN FURTHER ON THE
EMERGENCY RESPONSE EFFORTS, CODE
ROLES AND RESPONSIBILITIES
SHIFTED SLIGHTLY AND IT WAS
ESTABLISHED IN THE EVENT THAT IN
A CODE RESPONSE WAS NEEDED THE
CHILDREN'S NATIONAL PICU
ATTENDING WOULD RUN THE CODE,
NURSES WERE TRAINED IN THIS
SIMULATION WITH CHILDREN'S
NATIONAL PICU ATTEND EXPTION
EMERGENCY RESPONSE CODE AND FOR
EMERGENCY RESPONSES.
AND INSCREMENTS FROM 5-KILOS TO
[INDISCERNIBLE].
IF A CODE WERE TO CONSIDER
DURING THE PROCEDURE AWAY FROM
THE IN-PATIENT UNIT, THE ICU
CODE RESPONSE IS USUALLY WITHIN
3-5 MINUTES.
BUT NOT ALL CODE CARTS
THROUGHOUT THE CLINICAL CENTER
WERE IN POSITION OF AN ICU CART
WHICH WAS REPLETE WITH INFANT
SUPPLIES, SO A RESPIRATORY GO
BAG WAS CREATED TO TRAVEL WITH
THE PATIENT THROUGHOUT THE
CLINICAL CENTER.
THIS CONTENT INCLUDED SUPPLIES
NEEDED FOR THE FIRST FAIF
MINUTES UNTIL THE IRIVAL OF THE
ICU CODE TEAM.
THIS INCLUDED INFABT AND CHILD
MASKS, NONREBREATHER, SUCTION
SUPPLIES AND IV STARTS AMONG THE
OTHER FEW THINGS.
NEXT PLEASE.
EXTENSE OF EDUCATION AND
TRAINING METHODOLOGIES WERE PUT
IN PLACE FOR OUR PEDIATRIC
NURSES, OUR ICU NURSES, OUR PICU
NURSES AND IR NURSES, AFTER
DISCUSSIONS WITH THE RESEARCH
TEAM TO BETTER UNDERSTAND THE
COMPLEXITIES OF DISEASE PROCESS
AND TREATMENT, WE WERE WAIBL TO
DEVELOP A TRAINING PROGRAM WITH
THE FOCUS ON POTENTIAL EMERGENCY
SITUATIONS.
THESE INCLUDED ANAPHYLAXIS DUE
TO GENE TRANSFER TREATMENT,
SHOCK MANAGEMENT AND SEIZURE
MANAGEMENT TO DUE TO THE DISEASE
PROGRESSION AND CARDIAC
ARRHYTHMIA SPECIFICALLY AVT DUE
TO FEMORAL C-LINE, THE TRAINING
FELL INTO 4 BUCKETS, DIDACTIC
WHICH INCLUDED INFANT CARE,
EMERGENCY RESPONSE AND
MANAGEMENT OF POTENTIAL RISKS TO
THE SKILLS LAB INCLUDED HANDS ON
SKILLS SUCH AS INFANT MASK,
RESPIRATORY ADJUNCTS, IO
PLACEMENT AND RAPID SEQUENCE
INTERNATIONAL CLASSIFICATION
BATION AND CHEMO DYNAMIC TRIP
MANAGEMENT AND ARRHYTHMIA
MANAGEMENT.
AND THE STIMULATION LAB,
MULTIDISCIPLINARY NIH TEAMS
WORKED TOGETHER TO PRACTICE THE
MANAGEMENT OF ANAPHYLAXIS SVT,
USER MANAGEMENT AND SHOCK
MANAGEMENT.
THE INSTITUTE SIMULATION
INVOLVED OUR NIH
MULTIDISCIPLINARY TEAMS SUCH AS
P A C TEAM AND ICU NURSES AND
CHILDREN'S NATIONAL INTSEMMIST
AND THEIR RESPIRATORY THERAPIST
ON 1 UNIT.
ROSA WILL NOW WILL TAKE A DEEPER
DIVE AND PRESENT OUR EDUCATION
AND TRAINING PROGRAM.
OUR PROVIDERS AND NATURAL
PROVIDERS UNFAMILIAR WITH OUR
CLINICAL SETTING.
THIS THAT IS WHERE IT WAS
CRUCIAL TO REVERSE EVERY
POTENTIAL COMPLICATION WITH THE
TEAMS, THE PRIMARY PURPOSE OF
THE EDUCATION AND TRAINING
PROGRAM WAS TO PREAND SUFFERING
FROM GM 1 IS ADMITTED TOA A NEW
AND LEARNING NEEDS ASSESSMENT
AND IDENTIFIED THE CARING FOR
UNFAMILIAR CARING POPULATION
WITH A HIGH RISK OF
COMPLICATIONS CREATED A GAP IN
NURSES CALL FOR LEVEL,
INCREASING ANXIOUS, STRESS AND
AND FUNCTION EFFECTIVELY IN THE
EVENT OF AN EMERGENCY.
THOSE WERE ICU NURSES, WHO AS
PART OF THE CODE BLUE TEAM WOULD
RESPOND TO AN INFANT EMERGENCY,
OUR PEDIATRIC CLINICAL RESEARCH
NURSES AS A PRIMARY CAREGIVERS,
OUR PERIOPERATIVE ANESTHESIA
UNIT AND RADIOLOGY NURSES,
CARING FOR INFANTS WITH A TEAM
WE DECIDED MULTIMODAL
EVIDENCE-BASED APPROACH
COMBINING LECTURES AND HANDS ON
SKILLS, AND SIMULATIONS WOULD
PROVIDE THE BEST OPPORTUNITY FOR
NURSES TO IMPROVE THE
CONFIDENCE, KNOWLEDGE AND SKILLS
NEEDED TO ENHANCE PATIENT SAFETY
AND PATIENT OUTCOMES.
NEXT.
EIGHT SESSIONS, OF INTENSE
EDUCATIONAL TRAINING OCCURRED
OVER A PERIOD OF 3 MONTHS.
IN PREPARATION FOR THE FIRST
INFANTS ADMISSION, WE PURPOSELY
MIXED THE NURSING DISCIPLINES
ATTENDING EACH CLASS SUCH AS
PEDIATRIC AND ICU NURSES TO
ENHANCE TEAM COLLABORATION AND
COMMUNICATION.
OUR INTERVENTION WAS DIVIDED IN
3 PHASES, IN THE FIRST FACE WE
WERE PRESENTED WITH THE DISEASE
PROCESS AND PROTOCOL, POETIC
TEBTIAL COMPLICATIONS AND INFANT
FOCUSED EMERGENCY CARE.
NURSES PARTICIPATED ON CANCELED
SKILLS WHICH INCLUDED
FAMILIARIZATION WITH EMPHASIZED
EQUIPMENT, RESPIRATORY SUPPORT
AND WEIGHT BASED MEDICATION
ADMINISTRATION.
NEXT SLIDE, PLEASE.
IN FACE 2 WE CONDUCTED A TOTAL
OF 24 INTEGRATED HIGH FIDELITY
SIMULATION WTION OUR NIH
CLINICAL PROVIDERS, THE
SIMULATIONS WERE CONDUCT INDEED
A SAFE NONTHREATENING LEARNING
ENVIRONMENT, THAT ALLOWED TO
EXPERIENCE HIGH RISK SITUATIONS,
THAT COULD LATER TRANSFER
FOCUSED ON REAL LIFE.
SIMULATION SPECIALIST ALONG WITH
CRITICAL CARE AND NURSING
EXPERTS DEVELOP 3 CASE SCENARIOS
THAT WILL DRIVE THE FOCUS
SIMULATIONS, MANAGEMENT OF
SEIZURES AND SUPER VENTRICULAR
TACHYCARDIA, THE SIMULATIONS
WERE BRACE OFFICE OF DIVERSITY
POTENTIAL COMPLIKAIGHTZS OF
DISEASE AND CLINICAL TRIAL.
NEXT SLIDE, PLEASE.
LASTLY IN PHASE 3, WE CONDUCTED
A TOTAL OF SIMULATIONS BRING
TOGETHER ICU, NIH NURSES AND
PROEDUCATIONALLERS CHILDREN'S
NATIONAL PROVIDERS AND HIGH
INS10ITY SIMULATIONS.
NEXT.
NEXT SLIDE.
FOCUSED ON EVALUATE OUR PROGRAM
OUTCOMES, 2 INSTRUMENTS WERE
USED, THE FIRST FRIEWMENT IS A
PEDIATRIC ADVANCED LIFE SUPPORT
SKILL SELF-EFFICACY INVENTORY ON
THE P A LS SSEI, THIS AN
EVALUATOR TOOL WITH CRONBACH'S A
.88 AND CVI = 1.
AND THIS IS AN EVALUATION BY
COMPARING PREE AND POST
INTERVENTION RESULTS.
THE SECOND INSTRUMENT WE USED
WAS A 5 QUESTION ANONYMOUS
SURVEY TO,A SESES SIMULATION
BASED EXPERIENCE.
NEXT SLIDE, PLEASE.
THE IPT GREATER VENTERY TOOL IS
A SCALE WITH 19 QUESTIONS.
THAT ALLOWS NURSES TO
[INDISCERNIBLE] SELF-CONFIDENCE
WITH PERFORMS PEDEIAT VERSE US
[INDISCERNIBLE] SKILLS.
THE SURVEY ON THE CATEGORIES OF
AMERICAN HEART ASSOCIATION
PEDIATRIC AND SLICE SUPPORT
PSYCHOMODAL COURSE OBJECTIVES.
THAT ADDRESS EVALUATION,
IDENTIFICATION, AND ISHT
VENTION, ADVANCED INTERVENTIONS,
AND TREATMENT IN PEDIATRIC
EMERGENCY, NEXT SLIDE, PLEASE.
THE MEASUREMENT OF INTEREST TO
US WAS WHETHER PARTICIPANTS
CONFIDENCE SCORES CHANGED WITH
THE TRAINING OF SIMULATIONS.
WE CONDUCTED A DESCRIPTIVE
STATISTICAL ANALYSIS AND
CALCULATED THE CHANGE FOR EACH
GROUP BEFORE AND AFTER EACH
SIMULATION.
THE GRAPH HERE DEPICTS EACH
NURSING GROUP, THE HIGHER THE
MEAN VALUE, THE HIGHER THE
SELF-REPORTED IMPROVEMENT, WE
SEE THAT THE AVERAGE HIGHEST
OVERALL RESULTS WERE 3
QUESTIONS, ASKING THE CONFIDENCE
LEVEL, ADMINNESTERS OXYGEN VIA
NONREBREATHER, ASSISTING WITH
THE PEDIATRIC AIR WAY AND
ASSISTING WITH THE PEDIATRIC
PATIENT.
THESE RESULTS REFLECT OUR
TRAINING EMPHASIS ON THE
IMPORTANCE OF MANAGING A
PEDIATRIC AIR WAY ESPECIALLY
THAT OF AN INFANT OVER AN ADULT
AIR WAY.
OVER ALL EVERY SINGLE
PSYCHOMOTOR SKILL.
BUT BEYOND THE STATISTICALLY
IMPROVEMENT, IT IS CRITTAL TO
UNDERSTAND THE CLINICAL
SIGNIFICANCE OF IMPROVING THE
NURSES CALL FOR LEVEL, ABILITY
TO ACT PROMPTLY WHEN TIME IS OF
THE ESSENCE.
NEXT SLIDE, PLEASE.
THE SECOND TRIEWMENT SIMULATION
BASED EXPERIENCE SURVEY
DEMONSTRATED ACTIVITIES WERE
WELL RECEIVED BY ALL WITH NURSES
SHARING OW SIMULATIONINGS HELP
WIDE TEAM COMMUNICATION AND
UNDERSTANDING THE PROTOCOL, AND
INCREASING THE CONFIDENCE TO
CARE FOR THIS POPULATION.
I WANT TO BRING TO YOUR
ATTENTION THE COMMENT ON HOW
THEY APPRECIATED A SIMULATION OF
AN ALLERGIC REACTION.
NEXT SLIDE, PLEASE.
WE WANTED TO SHARE A STORY, THAT
REFLECTS THE IMPACT OF THE
SIMULATIONS AND TRAINING, IF YOU
REMEMBER WE CONDUCT A
SIMULATIONS OF INFANT
EXPERIENCING ANAPHYLAXIS ON A
FOLLOW UP OUTPATIENT VISIT, 1 OF
OUR INFANTS SUFFERING FROM GM 1
DEVELOPED SEVERE ANAPHYLAXIS, A
CODE WAS CALLED OUR ICU TEAM
PEDIATRIC NURSES, PROVIDERS AND
CHILDREN'S HOSPITAL TEAM WORKED
IN SYNCHRONY QUICKLY REVERSING
THE SEVERE ALLERGIC REACTION, 1
OF THE PEDIATRIC NURSES LATER
CAME TO ME, SAID SIMULATION
HELPED ME REMEMBER WHAT TO DO.
AND NOW, I WILL TURN IT OVER TO
MY COLLEAGUE DR. MYRA WOOLERY.
>> THANK YOU ROSA, NOW I WILL
REVIEW THE POLICIES AND
PRACTICES OF RELATED TO
PEDIATRIC CARE FOR INFANT
POPULATION, FOR PURPOSE OF THIS
PRESENTATION I WILL FOCUS 3
AREAS, VENOUS ACCESS AND
MANAGEMENT AND MANAGEMENT, CHEMO
THERAPY, BIOTHERAPY
ADMINISTRATION AND BREAST
FEEDING AND EXPRESSED BREAST
MILK.
FIRST EXISTING VENOUS ACCESS TO
DEVICE PLACEMENT AND MANAGEMENT
OF DOCK YOU WANTS WERE REVIEWED.
FOLLOWED BY BENCHMARKING WITH
INSTITUTIONS THAT ADMIT INFANTS
AND PLACE CENTRAL ACCESS
DEVICES, THE DATA WAS SUMMARIZED
AFTER REVIEW AND ANALYSIS, THERE
WAS DISCUSSION AMONG THE
RESEARCH TEAM AND DISCIPLINARY
TEAM MEMBERS TO DECIDE THE BEST
APPROACH.
PERIPHERALLY INSERTED CENTRAL
VENOUS CATHETERS REFERRED TO AS
PICCPLACED IN THE FEMORAL VAIN
WAS THE BEST OPTION FOR THIS
STUDY.
DUE TO LIMITED EXPERIENCES
PLACING PICKS IN SMALL CHILDREN
AND INFANTS IT WAS DECIDED THE
PIC WOULD BE INSERTED AT THE
CHILDREN'S NATIONAL HOSPITAL
ASSISTING PICK FLUSH GUIDELINES
WERE MODIFIED FOR INFANT AND
FEMORAL PLACEMENT, A DOCUMENT
CREATED REGARDING THE CARE,
MAINTENANCE AND TROUBLE SHOOTING
OF PICCs IN INFANTS.
NEXT SLIDE, PLEASE.
ALTHOUGH RETUX MAB A MONOCLONAL
ANTIBODY IS ADMINNISTED IN MANY
POPULATIONS, ADMINISTRATION AT
THE INFANT POPULATION HAS NOT
OCCURRED AT THE CLINICAL CENTER.
THIS WAS IDENTIFIED FOR
MONITORING INFANTS WITH RETUX
MAB, [INDISCERNIBLE] ONCOLOGY
NURSES CHEMO THERAPY, BIOTHERAPY
INSTRUCTIONS.
BASED ON THE REVIEW OF LITIATURE
AND, MAAING IT WAS DETERMINED
THAT THE VITAL SIGN MONITORING
WAS CONSISTENT WITH CLINICAL
CENTER NURSING DOCUMENTS
INCLUDING OBTAINING VITAL SIGNS
AT BASE LINE AND WITH EACH DOSE
ESCALATION WHICH IS TYPICAL WITH
RETUXA MAB, HOWEVER, PLACING
INFANTS OF THE MONITORS WAS VERY
LOW.
IN THE IN-PATIENT SETTING MOST
OF THEM DID PLACE THEM ON
CARDIAC MONITORS DUE TO THE
AVAILABILITY OF THE MONITORS,
HOWEVER, IN THE OUTPATIENT THIS
WAS DEPENDENT ON WHETHER OR IN
THE THEY HAD CARDIAC MONITOR
AVAILABILITY, HOWEVER IF THEY
DID NOT HAVE CARDIAC MONITOR
AVAILABILITY, ALL OF THEM
REPORTED PLACING INFANTS ON THE
SYMMETRY DURING INFUSION, NEXT
SLIDE.
THE MOMS WHO PARTICIPATED IN
THIS ACTIVITY EXPRESSED DESIRE
TO BREAST FEEDING THEIR BABIES,
THERE ARE MANY REASONS TO
CONTINUE PREOF THE FEEDING FOR
THE BOND BETWEEN MOTHER AND BABY
AND HELP BOOST THE IMMUNE
SYSTEM, THE CLINICAL CENTER
HOWEVER HAS LIMITED DOCUMENTS
REGARDING BREAST FEEDING MOMS
AND EXPRESSED BREAST MILK
BECAUSE EXISTING NIH POLICY
NURSING MOTHERS PROGRAM FOCUSES
ON MOTHER'S EXPRESS INDEED
BREAST MILK, LACTATION ROOM AND
SERVICES AVAILABLE.
NEXT SLIDE.
AFTER DISCUSSION WITH THE
LACTATION COORDINATOR THE
VISITING THING MOTHER'S LIST WAS
OBTAINED ALONG WITH A LIST OF
CONTACTS, I WAS ABLE TO SCHEDULE
AN OPPORTUNITY FOR A FEW OF US
TO SEE THE LACTATION NURSE.
SUBSEQUENTLY THE DECISION WAS
MADE TO NOT USE THE LACTATION
ROOM DUE TO LOGISTICS AND
PROXIMITY TO THE NURSING ROOM TO
THE LACTATION AREA.
NEXT SLIDE.
CREATION OF THE BREAST FEEDING
AND DOCUMS OF THE REVIEW OF
LITERALLURE AND BENCHMARKING THE
CDC STORAGE AND PREPARATION OF
BREAST MILK WAS IDENTIFIED IN
SEVERAL HOSPITALS ACROSS THE
COUNTRY SHARED BREAST FEEDING
AND EXPRESSED POLICIES AND
GUIDELINES.
THE IN THIS ROUTINE MEANT TO
REVIEW DOCUMENTS AND PROVIDE
INPUT, FEEDBACK WAS OBTAINED
ALSO FROM THE BEDSIDE NURSES,
NEXT SLIDE.
THE STANDARD OF PRACTICES AND
PROCEDURE WAS ORGANIZED BY THE
FOLLOWING CATEGORIES AND
DEFINITIONS OF TERMS, ESSENTIAL
INFORMATION, COLLECTING BREAST
MILK, STORING EXPRESSED BREAST
MILK, PREPARATION, WARMING
ISSUES ARE, CLEANING SANIFIESING
OF THE SUPPLIES, NEXT SLIDE.
IT TAKE AS I A VILLAGE TO CREATE
A COMP REHAPPENSIVE DOCUMENT,
DISCUSSIONS WITH THE LACTATION
COORDINATOR TO DETERMINE
FEASIBLE OF USING LACTATION ROOM
AS PREVIOUSLY DISCUSSED WAS PART
OF THE PROCESS HOWEVER THIS WAS
NEEDED.
DRAFT DOCUMENTS WAS CIRCUTED FOR
INPUT FROM NURSING AND OTHER
DEPARTMENTS SUCH AS HOSPITAL
EPIDEMIOLOGY FOR INPUT ON
COLLECTION AND THE STORAGE AND
TEMPERATURE MONITORING, ALL
FEEDBACK WAS INCORPORATED IN A
FINAL DRAFT TO THE DOCUMENT WAS
SENT OUT FOR REVIEW IPT GREATER
PUT.
NEXT SLIDE.
THE EXPRESS BREAST MILK
COLLECTION PROCESS INCLUDING
LATE [INDISCERNIBLE] OUTLINED IN
THE DOCUMENT, LABELS WERE
CREATED THATICALLILY IDENTIFIED
THE FRESH NEVER FROZEN
REFRIGERATED FROZEN OR THAT
YOUD.
PRIOR TO ADMISSION, NUTRITION
FOR PRINTED THE LABELS AND
BROUGHT THEM TO THE NURSING
UNIT.
ONCE THE BREAST MILK WAS
EXPRESSED, THE MOTHER FILLED IN
THE INFORMATION ON THE LABEL AND
PLACED THE LABELOT CONTAINER.
THE EXPRESSED BREAST MILK WAS
USED TO USED TO FEED THE INFAPT
OR REFRIGERATED OR FROZEN FOR
LATER USE.
NEXT SLIDE.
ONCE THE BREAST MILK IS
EXPRESSED, IF NOT IMMEDIATELY
USED TO FEED THE INFANT, IT
NEEDS TO BE STORE INDEED A
REFRIGERATOR OR A FREEZER.
THE BIOMED POLICY FOR
REFRIGERATORS AND FREEZERS IN
PATIENT CARE AREA WERE REVIEWED,
A SMALL REFRIGERATOR, WOULD
FREEZER TO PLACE IN THE PATIENT
ROOM AND A LARGER 1 TO PLACE IN
THE LOCKED NUTRITION ROOM
[INDISCERNIBLE] WERE OBTAINED
FROM BIOMED.
EACH REFRIGERATOR FREEZER WAS
LABELED WITH EXPRESSED BREAST
MILK ONLY AS NO OTHER ITEMS CAN
BE PLACED IN THOSE REFRIGERATORS
OR FREEZERS.
EACH WAS EQUIP WIDE A
TEMPERATURE GAUGE AND A LOG WAS
COMPLETED AND MAINTAINED WHEN IT
USE TO DOCUMENT THE TEMPERATURE,
BIOMED WAS NOTIFIED FOR ANY
TEMPERATURES OUTSIDE THE
ACCEPTABLE RANGE.
FOR THIS STUDY, THE EXPRESSED
BREAST MILK WAS STORED IN THE
PATIENT'S ROOM.
IF IT WERE TO HAVE BEEN FROADZEN
IT WOULD HAVE BEEN STORE INDEED
A LARGER REFREJERATOR, FREEZER
DOWN IN THE NUTRITION ROOM SINCE
IT IS EASIER TO MAINTAIN
CONSISTENT TEMPERATURES IN IN
FREEZER COMPARTMENT.
THE CHILDREN CHILDREN'S INNFERS
ALSO CONTACTED TO SET UP TO SET
UP PROCESSES FOR WHEN THE WOMEN
WERE DISCHARGED.
STUDIES AND RESOURCES WERE
PROVIDED TO THEM.
NEXT SLIDE, PLEASE.
A DESIGNATED AREA FOR THE
PATIENT ROOM WAS ESTABLISHED FOR
CLEAN BREAST MILK SAVING AND
SUPPLIES AND STORING THE CLEAN
SURVEYS LIES.
THE CLEANING PROCEDURE WAS
OUTLINED IN THE DOCUMENT
REQUESTMENT, A MICROSTEAM BAG
WAS USED TO SANITIZE THE
SUPPLIES, IN THE DESIGNATED
HITTER MICROWAVE LOCATED IN THE
NUTRITION ROOM ON THE PEDIATRIC
NURSING UNIT.
NEXT SLIDE.
THE DOCUMENTS CREATED WERE ADDED
TO THE RESOURCE MANUELSA 1
MOTHER WEST, DAY HOSPITAL AND
ALSO IN THE ICU.
NEXT SLIDE.
WE WANTED TO TAKE THIS
OPPORTUNITY TO THANK THE BEDSIDE
NURSES AND PC Ts WHO
CONTRIBUTED TO THE SUCCESS AND
IMPLEMENTING THIS STUDY IN SO
MANY WAYS.
THOSE WHO CARED FOR THE INFANTS
AND THEIR CAREGIVERS, THOSE WHO
ASSISTED IN INSURING THE
INTEGRITY OF THE RESEARCH STUDY
AND PROVISION OF SAFE CARE FOR
THE WILLINGNESS EMBRAISE THE NEW
SKILLS FOR SHARING THEIR
EXPERIENCE AND PROVIDING
FEEDBACK THAT ASSISTED US TO
REFINE THE PLANS AND MAKE
IMPROVEMENTS FOR THEIR
COLLABORATION IN REVIEWING THE
VARIOUS DOCUMENTS THAT WERE
DEVELOPED IN PROVIDING FEEDBACK
TO IMPROVE THE RESOURCES.
NEXT SLIDE.
AS YOU WOULD HAVE HEARD
THROUGHOUT THIS PRESENTATION,
THERE WERE PROCESSES THAT HAD TO
BE QUEPED INCLUDING THE FAMILY
RESEARCH TEAM, THE TEAM, MEMBERS
AND THE NURSES AND PC Ts WITH
1 NORTHWEST DAY HOSPITAL, ICU,
THE PEDIATRIC CLINIC, THESE BY
NO MEANS REPRESENT ALL OF THE
STAFF WHO ASSISTED US WITH THIS
STUDY, NOW I WILL TURN IT OVER
TO PRESILLA TO PROVIDE AN UPDATE
ON THE INFANTS.
>> THANK YOU.
AS YOU HEARD FROM OUR SPEAKERS,
WE HAVE TREATED 2 INFANTS AT THE
CLINICAL CENTER SO FAR, THIS IS
A FIRST INFANT WHO CAME TO US AT
8 MONTHS OF AGE AND WAS TREATED
AT 9 MONTHS.
UNFORTUNATELY SEE SUCCUMBED TO
HER DISEASE AT 17 MONTHS, 8
MONTHS AFTER GENE TRANSFER.
NEXT SLIDE, PLEASE.
HERE WE SHOW THE VARIOUS
PICTURES OF THE SECOND INFANT
DIAGNOSED AFFAIRS TEAM LEADER 4
MONTHS OF AGE AND JOINING OUR
PROTOCOL AT 6 MONTHS AND TREATED
AT 7 MONTHS NOW SHE'S 15 MONTHS
OLD, SO FAR SHE'S MAKING SLOW
PROGRESS AS SHOWN LEER, NOW I
WILL TALK ABOUT THE FUTURE
IMPLICATIONS.
NTHANK YOU PRESILLA.
WE DISCOVERED THAT THE METHODS
OF EICATION, TRAINING AND COMP
TEBSY DEVELOPED FOR THIS
PROTOCOL, AND WE HAVE SINCE HAD
THAT OPPORTUNITY TO USE THIS
PROCESS FOR OTHER STUDIES, THIS
ENDEAVOR DEMONSTRATED THE
IMPORTANCE OF CLOSE MONITORING
OF TRAINING PLANTS AND SAFETY
AND BEST PRACTICES IN BLOOD
COLLECTION AT PRESENT, OUR IT
COLLEAGUES ARE PILOTING AN
ENHANCEMENT OF THE BLOOD TRACKER
TOOL FOR THE L. O. P. WHERE THEY
CAN REVIEW THE AMOUNTS OF BLOOD
AND HAVE THE OUTPATIENT SETTING
MODIFY LAB ORDERS TO STAY WITHIN
PATIENT AND SAFETY GUIDELINES,
IN AUGUST OF 2021, THE PEDIATRIC
PLANNING GROUP WAS ESTABLISHED
WITH THE GOALS OF OF 1
EVALUATING PEDIATRIC CARE AT THE
NIH CLINICAL CENTER AND 2,
CONDUCTING AN ASSESSMENT OF THE
FEASIBILITY OF EXPANDING PATIENT
VOLUME INCREASING THE ABILITY TO
MANAGE HIGHER ACUITY PATIENTS
AND REDUCING THE LOWER LIMIT FOR
ADMISSIONS FROM 3 YEARS TO 6
MONTHS.
SUBGROUPS WERE CREATED AND
DIVIDED AND PROVIDED DETAILS
RECOMMENDATIONS IN 3 AREAS.
PEDIATRIC EMERGENCY MANAGEMENT,
PEDIATRIC CRITICAL CARE AND
PEDIATRIC SUBSPECIALTY COVERAGE.
A PROPOSAL WITH THE DETAILED
RECOMMENDATIONS HAVE BEEN
SUBMITTED TO THE NIESH H
GOVERNING BOARD.
ALL OF THIS WOULD NOT HAVE COME
TO FRUITION WITHOUT THE
INVOLVEMENT OF SO MANY.
WE WANT THIS PRESENTATION TO
SERVE AS AN ACKNOWLEDGMENT TO
EVERYONE'S EFFORTS.
AS CLINICIANS WE SHOULD BE
PROUD, THIS STUDY SERVED AS A
REMINDER THAT THERE CAN BE HOPE
FOR FAMILIES AND WHY WE DO THE
IMPORTANT WORK THAT WE DO AND
NOW I WILL TURN IT OVER TO
DR. JORDAN FOR CLOSING REMARKS.
>> THANK YOU TRISH AND THANK YOU
TO ALL OF OUR PRESENTERS.
I THINK THIS HAS BEEN A FABULOUS
EXHIBITION OF HOW TEAMS COME
TOGETHER TO PLAN FOR SOMETHING
COMPLEX AND SOMETHING THAT HAD
NOT BEEN DONE IN RECENT YEARS,
AT THE CLINICAL CENTER.
I HAVE NOT RECEIVED ANY
QUESTIONS.
I WOULD LIKE TO REMIND THE
AUDIENCE IF HAVE YOU QUESTIONS
ENTER IT VIA CLICKING THE BUTTON
THERE, BUT I WOULD LIKE TO ASK
THE PRESENTERS YOU KNOW WHAT WAS
MOST CHALLENGINGLY FOR YOU IN
DEVELOPING THE EDUCATION AND THE
PLAN FOR THE PATIENTS?
SO PERHAPS WE COULD START OR GO
IN ORDER AS FAR AS CHALLENGE
WITH DR. D'SOUZA.
WHAT DID YOU FIND MOST
CHALLENGING?
>> IT'S A VERY GOOD QUESTION.
IT HAS BEEN--BRINGING INFABTS TO
THEIN KLF-TWOICAL CENTER WAS A
PRETTY HARD THING.
YOU KNOW YOU KNOW FOR ME, IT WAS
DEALING WITH THE FAMILY, DEALING
WITH THE SYSTEM, DEALING WITH
THE WHOLE MULTIINSTITUTIONS
BEING THE PRIMARY PROVIDER AND
DR. CYNTHIA [INDISCERNIBLE] AS A
TEAM, YOU KNOW COORDINATING WITH
THE MULTIPLE PEOPLE,
DEPARTMENTS.
I THOUGHT THAT WAS A HARD THING
FOR THESE 2 INFANTS.
ULTIMATELY WE HAD A GOOD
COOPERATION FROM ALL--THROUGHOUT
THE HOSPITAL, WHEREVER WE WENT,
YOU KNOW, THE--ESPENLLY THE--THE
NURSES, THE TECHNOLOGIST, THE
ANESTHESIA TEAM, WHO ARE
AVAILABLE ON INFANT EVENT, THEY
TOOK WELL CARE OF OUR INFANT AND
PROVIDED NECESSARY INFORMATION
FOR OUR PROTOCOL.
>> THANK YOU.
>> KRISTA WOULD YOU LIKE TO ADD
COMMENTS TO THAT OR OTHER
THOUGHTS THAT YOU EXPERIENCED?
>> YEAH, 1 OF THE GREATEST
CHALLENGES IT BRIDGING GAPS WAS
RECOGNIZING THE INDIVIDUAL
EXPERTISE WITHIN THE NURSING
DEPARTMENT AS WELL AS THE
VARIOUS DEPARTMENTS.
I THINK INSURING THAT WE
LISTENED TO THIS THE
STAKEHOLDERS AND EVERYBODY THAT
WOULD BE PROVIDING CARE AND
WOULD BE INVOLVED IN THE CARE
TRULY WAS PARAMOUNT TO MAKING
SURE THAT WE BRIDGE THE GAPS
THAT EXISTED, AND WE MET WEEKLY
FOR THOSE FMEAs ARE
APPROXIMATE MONTHS AND MONTHS
AND 50 PLUS + PEOPLE THERE AND I
THINK THAT THAT WAS INCREDIBLY
IMPORTANT AS WELL AZIZZ WELL AS
SURVEYS THAT CONDUCTED FOR
EDUCATION TO MAKE SURE THOSE
VOICES WERE HEARD AND EVERYBODY
WAS REPRESENTED.
THAT WAS PROBABLY THE HARDEST
PART WAS JUST MAKING SURE THAT
DIDN'T LEAVE ANY STONE UNTURNED
AND EVERYBODY HAD A VOICE TO
MAKE SURE THAT THEIR CONCERNS
AND IDEAS WERE BROUGHT FORWARD
AND IT REALLY DID TAKE A
VILLAGE, IT WAS AN INCREDIBLE
HONOR TO WORK WITH SUCH AN
AMAZING TEAM.
>> SO I WOULD LIKE TO TURN FROM
CHALLENGES TO WHAT DO YOU SEE AS
SOME OF THE GREATEST SUCCESSES
FROM THIS WORK?
I WILL ASK ROSA TO COMMENT.
>> THANK YOU DR. JORDAN.
I THINK THE GREATEST SUCCESS AS
MENTIONED IN CLOSING IS WE WERE
ABLE TO REPLICATE IT, AND WE CAN
CONTINUE TO REPLICATE IT IN
DIFFERENT SETTINGS, WE'RE
ALREADY WORKING ON DIFFERENTS
SPECTS TO JUST USE THE SAME TIME
OF EDUCATION AND TRAINING WITH
SIMULATION, TO INTRODUCE AND
PREPARE NURSES FOR NEW CLINICAL
TRIALS.
>> THANK YOU.
TRISH, DO YOU WANT TO COMMENT
FURTHER?
>> SURE, THANK YOU.
I THINK THE--1 OF THE GREATEST
THING SYSTEM TO--YOU KNOW ALL
THE TEAMS WE WORK WITH, ALL THE
GROUPS WE WELCOMER WITH, REALLY
WANTED WHAT WAS BEST FOR THESE
CHILDREN AND WERE WILLING TO DO
WHAT WAS NEEDED TO MAKE SURE
THAT THEY WERE SAFE AND THAT WE
WERE ABLE TO CARRY OUT THIS
PROTOCOL.
IN THE EDUCATION IN AND THE
TRAINING FROM THE IT FOLKS, FROM
JUST EVERY AS SPECT, OUR NURSES,
OUR ICU NURSES WHO HAD TO REALLY
GO OUT OF THEIR SCOPE OF
PRACTICE AND TAKE ON SOMETHING
SO NEW AND CHALLENGING.
IT JUST--IT WAS FUN TO JUST WORK
WITH THESE FOLKS AND IT WAS EASY
IN THAT SENSE BECAUSE EVERYBODY
WANTED TO THE SAME OUTCOME.
>> THANK YOU.
I RECALL THE DAY WHEN THE 1
CHILD DID HAVE THE ANAFLACTIC
REACTION AND GOING TO THE ICU
AND 1 MIGHT EXPECT AN AIR OF
FRENZ SKPE BAKUGAN IT WAS CALM,
CONTROLLED AND EVERYONE WAS
COMFORTABLE, THEY KNEW WHAT TO
DO AND THINGS WENT VERY WELL AND
WE HAD A GOOD OUTCOME AND I
THINK THE TRAINING AND EDUCATION
THAT WAS PROVIDED AND ESPECIALLY
THE SIMULATION, YOU KNOW REALLY
PREPARED PEOPLE.
I WOULD ALSO LIKE TO COMMENT AND
PERHAPS HERE FURTHER FROM MYRA
REGARDING ALL THE DETAIL
PROVIDED WHILE PREPARING FOR
BREAST FEEDING, 1 WOULD THINK WE
ARE A HOSPITAL, WE SHOULD HAVE
THESE KINDS OF THINGS IN PLACE
AND READY AND YET, WE DID NOT
SO, WHAT DID YOU FIND, AGAIN,
SOME OF YOU ARE EXPERIENCING IN
CREATING THIS PROTOCOL AND IS
THIS SOMETHING NOW THAT WE CAN
USE MOVING FORWARD?
>> WELL, DR. JORDAN, THAT IS A
GREAT QUESTION, I HAVE TO GIVE
KUDOS TO SO MANY DIFFERENT
DEPARTMENTS AND SO MANY PEOPLE
ACROSS THE COUNTRY THAT HELPED
US OUT.
THIS--YOU WOULD THINK LIKE YOU
SAID, WE ARE A HOSPITAL BUT WE
HAVEN'T HAD INFANTS FOR A LONG
PERIOD OF TIME SO JUST BEING
PREPARED IF ARE THAT PART OF IT,
BUT I CAN'T SAY ENOUGH ABOUT THE
NUTRITION DEPARTMENT WHO MANY
DIFFERENT PLAYERS ALONG THE WAY
HELPED IN IDENTIFYING VARIOUS
ASPECTS, YOU KNOW THROUGHOUT
THAT PARTICULAR PROCESS BIOMED
WHO WEIGHED INOT REFRIGERATORS
AND TEMPERATURES, HOSPITAL
EPIDEMIOLOGY WHO GAVE ENORMOUS
IMPACTS TO MAKE SURE THAT WE
WERE ADHERING TO THE BEST SAFE
GUIDELINES TO MAKE SURE WE
DIDN'T HAVE ANY TRANSMISSION OF
INFECTIOUS ACCIDENTALLY OR
LOOKING AT THE ENVIRONMENT FROM
THAT STANDPOINT, LIKE I SAID, MY
COLLEAGUES, YOU KNOW THROUGH THE
ASSOCIATION OF FUTURE
[INDISCERNIBLE] AT
[INDISCERNIBLE] ACROSS THE
NATION WHO SHARED WHEN I REACHED
OUT TO THEM AND EXPRESS WHAT WAS
GOING ON, THEY SHARED THEIR
BREAST MILK VARIOUS POLICIES AND
GUIDELINES THAT HELP ME TO
FURTHER FINE TUNE WHAT WOULD
WORK HERE IN THE CLINICAL CENTER
VURSS NOT A LACTATION
COORDINATOR WHO PROVIDED A LOT
OF INFORMATION ALTHOUGH WE
DIDN'T END UP USING THAT, THAT
WOULD BE BENEFICIAL BECAUSE I
ADDED THAT TO THE DOCUMENT THAT
IS NOW VERY PROBUST, THE NEXT
STEP WILL BE TO MOVE THIS INTO A
CLINICAL CENTER DOCUMENT, THIS
IS AVAILABLE NOT ONLY FOR THESE
MOTHERS, ANY OF OUR MOTHERS THAT
COME TO THE PEDIATRIC AREA, BUT
IF THERE'S OTHERS ALSO WITHIN
THE CLINICAL CENTER THAT NEED TO
EXPRESS EXPRESS BREAST MILK ON
OTHER STUDIES WHERE THAT'S SAFE
FOR THEM TO DO THAT AND STORE
THAT FOR FUTURE USE, IT WILL BE
INPUTTING THAT AS WELL.
>> THANK YOU.
SO WE DID RECEIVE A COMMENT AND
QUESTION.
CONGRATULATIONS FOR THIS
PIONEERING WORK, TO ALL THE
TEAMS INVOLVED, VERY APPROPRIATE
CHOICE OF TOPIC FOR NURSING WEEK
CELEBRATION.
THANK YOU.
AND THE QUESTIONER ASKS, CAN YOU
COMMENT ON OUR LAB'S ABILITY TO
RUN LABS ON PEDIATRIC VOLUME
TUBES OR WAS TESTING DONE AT
CHILDREN'S NATIONAL.
>> SO I KNOW PRESILLA CAN ADD TO
THIS BETTER THAN I, WHEN WE WORK
TOGETHER ON THE LABS WE LOOKED
AT WHAT THE PROTOCOL HAD PLANNED
OUT AND THEN WITH PRESILLAS KEEN
A AND HER TEAM LOOKED TO SEE
WHEN LABS COULD BE COMBINED
WHICH COULD BE MOVED TO ANOTHER
TIME WHICH COULD BE A LOWER
VOLUME IN THE COLLECTION WHICH
WAS A BIG PART OF IT BUT I DO
BELIEVE MOST OF THEM WERE RUN
HERE AM I RIGHT BY SAYING THAT
PRESILLA.
>> THAT'S CORRECT.
>> SO, YEAH, WHAT TRISH SAID WAS
CORRECT.
ALL THE LABS, CLINICAL LABS WERE
DONE HERE AND WHAT WAS AVAILABLE
TO THE LABORATORY OF MEDICINE,
WE HAVE A--WHAT YOU CALL, WE
HAVE WONDERFUL LAB TEAM UP HERE,
I WOULD MAKE A PHONE CALL, THEY
WOULD ANSWER SAYING THAT HEY, I
HAVE AN INFANT OF 6 KILOS, COULD
YOU RUN THIS, COULD DO XYZ TEST
WITH 1 ML OF BLOOD.
THEY WOULD CARRY THE TUBE TO THE
HEMATOLOGY TO CHEMISTRY AND THEY
WOULD RUN THE TEST SO THIS WAS 1
OF THE WAY.
WE WERE ABLE TO STAY WITHIN THE
CLINICAL CENTER GUIDELINES ABLE
TO DO ALL THE TESTS.
>> THANK YOU.
SO THE REMAINING COMMENTS THAT
WE'VE SEEN ARE ALL
CONGRATULATIONS WHICH I WILL
SHARE WITH OUR PRESENTERS BUT I
JUST AGAIN WANT TO THANK EACH OF
YOU AND DR. [INDISCERNIBLE]
BEHIND THE SCENES WITH THE
SLIDES HERE ON ORGANIZING AN
EXCELLENT PRESENTATION BUT TO
THANK EVERYBODY WHO WAS INVOLVED
WITH THE PROTOCOL AND WITH, YOU
KNOW FROM OUR EXECUTIVE
LEADERSHIP THROUGH OUR
INSTITUTE, PARTNERS AND
COLLEAGUES AND THE AT CLINICAL
CENTER, WE REALLY SET THE GROUND
WORK FOR FUTURE STUDIES IN THE
RESEARCH OF INFANTS AND AGAIN,
THANK YOU PRESENTERS.
REMIND PEOPLE TO SUBMIT IT FOR
THEIR CME CREDIT AND AGAIN, I
WISH ALL NURSES A HAPPY NURSES
WEEK AND I THANK YOU ALL AND
THANK YOU FOR THE OPPORTUNITY TO
HAVE OUR WONDERFUL NURSE
COLLEAGUES PRESENT TODAY, THANK YOU