# Presentation: 634193

## The Power of Stories over Statistics:  Illustrations from Neonatal Jaundice and Infant Airplane Safety

- Thomas B. Newman MD, MPH
- Professor of Epidemiology and Biostatistics and Pediatrics, University of California, San Francisco

- \sabbatical\stories&StatsForExeter.ppt

## Overview

- Introduction
- Jaundice in newborns and kernicterus
- Infant safety seats on airplanes
- Stories vs Statistics; Politics and Risk research
- Possible approaches and recommendations
- Questions and discussion

## Idealized public health decision making

- Recognize resources are limited
- Estimate the number of people who would benefit, and how much, from various courses of action
- Select course of action with highest expected benefit
- Alternative basis for decisions: stories

## Jaundice --Background 1

**Bilirubin**: Yellow breakdown product of heme (from red blood cells)
**Jaundice**: Yellow color of whites of eyes and skin due to high bilirubin*.  *Usually indicates liver or blood disease, but generally normal in newborns
**Hemolysis**: Destruction of red blood cells
**Kernicterus**: Rare, severe, permanent brain damage from very high bilirubin levels

## Jaundice --Background 2

- Phototherapy: Shining light on the babies skin -- helps lower bilirubin levels
- Exchange transfusion: replace baby’s blood with donor blood

## Jaundice – Background 3

- Exchange transfusion for Rh disease 1950s  much less kernicterus
- Phototherapy 1970s
- TN a resident in pediatrics 1980-83
- Phototherapy for Total Serum Bilirubin (TSB) > 14-15 mg/dl (240 uMol/L)
- Exchange transfusion for TSB > 20 mg/dl (340 uMol/L)
- Beginning of AIDS epidemic
- Watchko and Oski’s “Vigintiphobia” article (1983)*

- *Watchko JF, Oski FA.  Pediatrics 1983;71:660-63

## Jaundice--Current issues

- We know jaundice used to be over-treated
- “Kinder, gentler” approach (1992) and AAP jaundice guideline (1994) -- far less treatment
- Phototherapy for TSB >= 20 mg/dL
- Exchange for TSB > 25-30 mg/dL
- Concern about the return of kernicterus; evidence is poor
- New diagnostic technologies
- Formation of PICK, a parents group; push for universal bilirubin screening

## Phototherapy (PT) and Exchange Transfusion (ET) in 2 Hospitals in Israel Pre- and Post AAP guideline*

- *Seidman DS et al. Acta Paediatr 2001;90:292-5

-  63% reduction in phototherapy
-  85% reduction in exchange transfusions

## PILOT KERNICTERUS REGISTRY

***BIND CENTER - Pennsylvania Hospital***

**PILOT KERNICTERUS REGISTRY**
***Confirmed Cases of Acute Kernictrerus  (Voluntary Reporting)***
***“******Healthy” Term and Near Term Infants Born in 34 States***

- http://www.pickonline.org/presentation.ppt, accessed 7/10/03

## Concern about Return of Kernicterus

- Brown and Johnson registry: 90 cases in 15 years in the US
- Northern California Kaiser Permanente
- No cases in 111,000 births, 1995-8
- No cases found in 1991-4 (N~119,000)
- BUT: Evidence of increase in babies with very high bilirubin levels 1999-2003.

## Deaths from Kernicterus (773.4, 774.7), < 1 year-olds, USA*

- *CDC Wonder, Accessed 7/10/03

## Deaths from Kernicterus (773.4, 774.7), < 1 year-olds, USA*

- *CDC Wonder, Accessed 7/10/03, 9/4/05

## New Diagnostic Technologies

- Cost: $4000 + $7/use

## New Diagnostic Technologies -2

- Cost: $19,000 + $15 per use

## Stories: Cal Sheridan

- 36-37 wk 2900 g boy b.t.a. 35 y.o. G1 O+ Mom

## Nursing Notes, Age 17 hours 

- No bilirubin level sent.

## Clinic Visit, 4.5 days

- (-9%)

## Return visit and outcome

- Next day, bilirubin 34.6 mg/dl Outcome: Kernicterus (?)
- Verdict for defense in malpractice suit, settled on appeal
- Same day: father diagnosed with spine tumor, misdiagnosed as benign

## Slide 19

## Parents of Infants and Children with Kernicterus (PICK)

- www.kernicterus.org

## “Never doubt that a small group of thoughtful, committed citizens can change the world.  Indeed, it's the only thing that ever has.”

- 	Margaret Mead (1901 - 1978) US anthropologist

## JCAHO Sentinel Alert

_**JOINT COMMISSION ISSUES ALERT: TREATABLE BLOOD DISORDER**__**PUTS BABIES AT RISK FOR BRAIN DAMAGE, DEATH**_

## JCAHO Sentinel Alert -2

- Sentinel Event Alert • Issue 18 - April 2001
***Kernicterus threatens healthy newborns***Kernicterus is a condition of newborns that leads to severely disabling brain damage or death. It results from hyperbilirubinemia that can be caused by a number of factors. Kernicterus is preventable with techniques currently available.
-  *"This is probably happening more than clinicians know about. With these recent cases, risk assessments were inadequate and unreliable, and bilirubin levels were not measured--or measured in time."****- Sue Sheridan***

## “In early 2001, a national support group for parents of children with kernicterus conducted a survey on kernicterus. A convenience sample of 15 families was identified by word-of-mouth or through the Internet... Among the sample families, seven did not complete the questionnaire, four had children who did not meet the case definition, and the remaining four had children who did meet the case definition..”

**1 /****Kernicterus in Full-Term Infants --- United States, 1994--1998****	**

- “In early 2001, a national support group for parents of children with kernicterus conducted a survey on kernicterus. A convenience sample of 15 families was identified by word-of-mouth or through the Internet... Among the sample families, seven did not complete the questionnaire, four had children who did not meet the case definition, and the remaining four had children who did meet the case definition..”

## Slide 25

**Notes:**
This slide is here because I remember trying to  watch this video, "Six Days, Seven Nights" at home one Friday evening when I was on call for the nursery.  Much to my wife's annoyance, I couldn't focus on the movie because I was so worried about a 5 day old baby with a bilirubin of 20.5 mg/dl (up from 19 the day before), whom I had heard about just before from the home health nurse.  She elected to continue home phototherapy rather than admit the baby to the hospital, because the breast milk had really come in.  Of course the baby did fine, but I vividly recall the uncomfortable "vigintiphobia."

## Neonatal jaundice/kernicterus summary

- No dispute about former over-treatment or current treatment thresholds
- No good data on current incidence of kernicterus, but it is rare
- Definition of the problem and proposed solution strongly influenced by 1 story (assisted by industry funding)
- Weak data, powerful stories
- Universal bilirubin screening is coming

## Press Release 

**AAP CALLS FOR AN END TO LAP TRAVEL FOR CHILDREN ON PLANES**

**AMERICAN ACADEMY OF PEDIATRICS**
- Committee on Injury and Poison Prevention
**Pediatrics 2001;108:1218-1221**

## Ending lap travel: Background

- Children under 2 can ride on parent’s lap with no ticket
- July 19, 1989: UAL #232 crash at Sioux City, Iowa.  Unrestrained infant dies.
- 1990:  US  NTSB recommends universal restraint
- July 12, 1994: Another “lap child” dies in crash, NTSB urges FAA to require infant restraint

**Notes:**
NTSB= National Transportation Safety Board
FAA= Federal aviation administration

## FAA Study, report to Congress, 1995

- Methods
-  Detailed analyses of survivability of previous crashes
- Models of price-sensitivity of travel decisions
- Results:
- Infant restraint would prevent maximum of 5 infant deaths over 10 years
- Net increase of 82 deaths over 10 years due to diversion to less safe modes of travel
- Rejected as “flawed” by NTSB and Congress

## Effects and Costs of Requiring Child Restraint Systems for Infants Traveling on Commercial Airplanes*

- Benefits: similar to FAA (6 deaths in 10 years)
- Risks: less than FAA when likely characteristics of drivers and driving considered (Net increase in deaths over 10 years 0-30 vs 82 )
- Costs: still high

- *Newman TB, Johnston B, Grossman D. Arch Pediatr Adol Med 2003;157:969-74

## Deaths caused or prevented

## Estimated Costs

- Assume no diversion to less safe modes of travel

- *Assumes 3% discounting

## Congressional Testimony: Cost effectiveness

- Congressman Geren: “... five million enplanements. Say it only costs $20. That's $100 million...If we gave you $100 million and you were entrusted with saving lives, where would you apply it, top priority?”
- Barry Sweedler, Director, Office of Safety Recommendations, NTSB: “There is a long list of safety issues that need addressing, and I think it's probably best for us to try to lay out what the issues are and try not to prioritize which safety issue is more important than other safety issues...this seems to be one of those areas where it just seems to make common sense to go ahead and take that extra step.”

## Congressional Testimony:  Evidence

- Coming into Washington we ran into some turbulence around some thunderstorms and there were some other lap children that the mothers were having trouble restraining. Many of them were crying and screaming. The little kid in the safety seat slept through the whole thing.
- So I think there is more than enough evidence that substantiates what we're trying to do.
- The question, I think, Mr. Chairman, comes down to how many more children must die, how many more have to be hurt before we reach the threshold of FAA's ghoulish cost/benefit ratio?			--Congressman Jim Lightfoot, Iowa

## “Real” vs “Theoretical” Children

- “The argument in support of the FAA’s resistance to the NTSB...is unreasonable on its face and ridiculous in its justification.  It protects theoretical children driving in cars at the expense of real flesh-and blood infants whose safety is unquestionably compromised when flown as a lap-baby”

- Nader R, Smith WJ.  Collision course: the truth about airline safety.  Blue Ridge Summit, PA: TAB Books, 1994.  Cited  by Beshai D. Arch Ped Adol med 2003;157:953-4

## Stories: UAL Flight 232: Jan Brown-Lohr

## Child Restraint on Airplanes: Summary 

- Good data
- Very little benefit
- Very high cost per benefit
- May cause net harm
- Notice of Proposed Rule-making issued by FAA in 2001
- Decision NOT to change rule 8/25/05
- Victory for data?

## AP Story: NTSB on FAA Ruling

- "During takeoff, landing and turbulence, adults are required to be buckled up, baggage and coffee pots are stowed, computers are turned off and put away, yet infants and toddlers need not be restrained," [NTSB Acting Chair] Rosenker said in a statement.

- Miller, L: Plane safety seat ruling unchanged for children http://www.buffalonews.com/editorial/20050827/1073311.asp, accessed 9/4/05

## AP Story –FAA Response

- FAA spokesman Greg Martin said requiring the seats would harm more children than it would protect. "A lap child held firmly by a parent doesn't result in second- or third-degree burns like an unsecured pot of coffee," Martin said.
- Three children who sat in their parents' laps were among the 309 who survived the fiery crash of an Air France Airbus A340 after it overshot the Toronto runway, Martin noted.

- Miller, L: Plane safety seat ruling unchanged for children http://www.buffalonews.com/editorial/20050827/1073311.asp, accessed 9/4/05

## Babies caught up in 'no-fly' confusion 

- WASHINGTON --Infants have been stopped from boarding planes at airports throughout the U.S. because their names are the same as or similar to those of possible terrorists on the government's "no-fly list."
- It sounds like a joke, but it's not funny to parents who miss flights while scrambling to have babies' passports and other documents faxed...

- Miller, L. http://homelandsecurity.osu.edu/focusareas/transportation.html accessed 9/4/05

## What makes stories powerful?

- More interesting than numbers
- More understandable than numbers
- Emotional connection to story teller (a real person,” not scientific or political establishment)
- Passion, sustained commitment of story teller

## Stories

- ““Humans are primates who tell stories.”		Stephen Jay Gould, PhD
- “Humans are primates whose cognitive capacity shuts down in the absence of a story.”		Robyn M. Dawes, PhD		Cognitive psychologist		Author: “Everyday Irrationality”

## Stories and Politics

- “Rather than understanding policy-makers as problem-solvers who apply objective, scientific, and value-free methods to cure society’s ills...think of policy-makers as performers who seek to persuade an audience...
- A policy argument, in consequence, tells a story: it provides a setting, points to the heroes and villains, follows a plot, suggests a solution, and, most importantly, is guided by a moral.*

- *John Adams and Michael Thompson.  Taking account of societal concerns about risk: Framing the problem. A report for the Health and Safety Executive.

## Risk perceptions and preferences

- Involuntary risks perceived as worse than voluntary risks
- Identifiable victims/lives saved valued much more than statistical lives saved
- Cure valued more than prevention
- Preventing rare disease valued more than decreasing common disease even if fewer lives saved
- Equity is valued

## Jaundice Story

- Heroic PICK mothers fighting ignorance and managed care efforts to save money
- Solution: Universal bilirubin screening
- Moral: need to protect the vulnerable
- Risk involuntary: PICK moms not warned  that jaundice can cause* brain damage.*
- Identifiable victims: KI considered separately from Cerebral Palsy

## Safety Seat Story

- Story -- heroic, dedicated flight attendants, congressmen against unfeeling bureaucrats
- Solution: Require universal child restraint
- Risk is both involuntary (to infant and other passengers) and voluntary (parents decide)
- Plane crash victims are identifiable; car crash victims an anonymous “drop in the bucket”
- Equity: Infants deserve same level of protection

## Possible approaches/ Recommendations -1

- Divide and conquer
- Recognize and publicize the problem
- Get stories (and story tellers) that support best public health choices
- Harder to get stories that support NOT doing something that is only too expensive
- Avoid polarization -- meet with the opposition.

**Notes:**
Divide and conquer -- let the specoial interests with their vivid stories fight with each other for resources -- not desirable

## Possible approaches/ Recommendations -2

- Focus on deliberative bodies and opinion leaders
- Try to discuss how decisions should be made separately from the specifics of a particular decision
- Get passionate about good decision making

## Slide 50

## Slide 51

## Slide 52

## Children Under 5 Years Old

- Road fatalities to all vehicle occupants under 5 years old decreased 12.4% between 2000 and 2002.
- Injuries decreased 11.9% between 2000 and 2002.
- Estimated resident population of children under 5 years old increased 1.9% between 2000 and 2002.

## Summary

- Even in extreme cases, the historic relationship between diversion from air travel and increased risk of fatality or injury in highway accidents is not clearly apparent.
- Accident risk is not evenly distributed among all drivers, vehicle types, and trip conditions.
- No evidence was found to suggest an increased risk for children under 5 years old.

## Additional Application: Pseudodisease

- Pseudodisease: disease that looks just like real disease pathologically but would cause no problems
- Identified with screening tests, causing net harm
- Can’t tell from disease in an individual patient
- Hard for people to grasp because there are no *stories*

## Alternative story line

- Villains -- emotional, irrational, nonscientific people and for-profit corporations
- Heroes -- rational, scientific, impartial researchers who do not accept industry money
- Moral: follow the money, keep the broadest possible perspective!

**Notes:**
It is of course tempting to buy into my own story line, in which people like me are heroes...

- www.kernicterus.org

**Information for Parents: The Jaundiced Baby**
***Jaundice in Newborns and its Treatment***
- About 60% of newborn infants in the United States are jaundiced, that is they look yellow. Excessive jaundice in newborn infants may cause brain damage.

## The Nuclear Non Proliferation Treaty, Article VI

- “Each of the Parties to the Treaty undertakes to **pursue negotiations in good faith** on effective measures **relating to cessation of the nuclear arms race at an early date and to nuclear disarmament**, and on a treaty on general and complete disarmament under strict and effective international control.”
- Signed by the U.S. 1968, ratified 1969

- http://disarmament.un.org:8080/TreatyStatus.nsf

**Notes:**
Here’s the second underappreciated fact.  It is that article 6 of the Nuclear nonproliferation treaty states:

## What does if mean if a treaty is ratified?

- Article VI, U.S. Constitution:
- “This Constitution... **and all treaties made, or which shall be made, under the authority of the United States**, shall be the **supreme law of the land**; and the judges in every state shall be bound thereby...
- “The Senators and Representatives...and the members of the several state legislatures, and all executive and judicial officers, both of the United States and of the several states, shall be bound by oath or affirmation, to support this Constitution...”

**Notes:**
Now, what does it mean if a treaty is ratified?  Here’s the third under-appreciated fact.  It means that we have to follow it.  Article VI of the US constitution states...<slide>

So when all of our government officials swear to be bound by the constitution, that means they swear to be bound by article VI of the NPT, as  the supreme law of the land.

## AAP Guidelines (1994)

## Reemergence of Kernicterus In Babies Discharged As Healthy In 1990s: Review of 5 Decades of Reports in the Literature.                                                                                                       Vinod K. Bhutani and Lois H. Johnson 

**Plus new Cases**

**PAS Meeting 2003: Abstract**

- N=154

- http://www.pickonline.org/presentation.ppt, accessed 7/10/03

- 6

- 6

## Kernicterus Registry: Definition and Eligibility*

- Acute phase kernicterus
- Irritability and hypertonia with early retrocolis and opisthotonus and drowsiness, poor feeding, high-pitched cry, alternating tone or failed ABR
- Chronic kernicteric sequelae: Abnormality in at least 2 of the following, at least one severe:
- Extrapyramidal movement disorder
- Gaze abnormalities
- Auditory disturbances
- Intellectual deficits
- Enamel dysplasia of deciduous teeth

- Johnson LH, Bhutani VK, Brown AK. J Pediatr 2002; 140: 396-403

## How common is kernicterus?

- Pilot kernicterus registry: 90 cases in 15 years in the US
- Northern California Kaiser Permanente
- No cases in 111,000 births, 1995-8.  (11 had TSB > 30 mg/dl1 )
- No cases found yet in 119,000 births, 1991-4
- ? 2 potential cases 2003?
- California Cerebral Palsy Project2
- 1 case in 155,636.
- (Total of 192 cases of CP in that study.)
- Denmark, 1994-8 ~1/65,0003

- 1 Newman TB et al.  Pediatrics 2003;111:1303-11
- 2 Cummins SC. Personal communication.  3Ebbesen F Acta Paediatr 2000;89:1-5

## How Common Is Kernicterus?

- Problems:
- No uniform surveillance
- Trade-off between sensitivity and specificity in case definition – no standard
- Diagnosis of kernicterus often delayed, uncertain and contentious
- Probably between 1/100,000 and 1/250,000 = 16-40 cases per year in the US
- MUCH more common in some other countries, especially in Africa
- Southern Nigeria: 9/20 infants readmitted with TSB > 15 mg/dl (45%)1

- 1 Slusher TM et al. Pediatr Res 2003 Abst #2865

## Pathophysiology

- CO

## MMWR Case Reports: missing information

- #2 (Cal Sheridan) The follow-up visit when OM diagnosed
- #3 The child has congenital nephrotic syndrome -- a low albumin probably contributed to risk
- #4  At 4 days Mother declined a home health visit and reported that jaundice appeared to be fading