Palliative Care in the Palliative Care in the Pediatric CVICUPediatric CVICU

Case StudiesCase Studies

Priya Bhat, MD, MSPediatric Palliative Care Conference

May 2, 2012

OutlineOutlineIntroduction

Pediatric death“A Good Death”Palliative Care defined

Case 1: IsaiahPrenatal and pre-operative counselingMedical education

Case 2: SaraHealthcare costsCompassion fatigue

Case 3: EthanParental perceptions

IntroductionIntroduction~54,000 children die annually in the US.

~7 million children worldwide could benefit from palliative care; 5000-8600 per day in US!

Infant mortality rate in US = ~7 per 1000 live births.

Death rate among children <1yo is higher than that in any other pediatric age group.

Most common etiology is congenital heart disease (CHD).

Most children die in the hospital, particularly the ICU.

>90% of these spend their entire life in the hospital.

A “good” death?A “good” death?“A decent or good death is one that is: free from avoidable distress and suffering for patients, families, and caregivers; in general accord with patients’ and families’ wishes; and reasonably consistent with clinical, cultural, and ethical standards.”Institute of Medicine. Approaching Death: Improving Care at the End of Life. 1997.

“Death is rarely good, but a constellation of factors add up to a better death.”

Gazelle G. A good death: Not just an abstract concept. J of Clin Onc. 2003.

“A good death is hard to define, however, for a good death is as complex as the good life of which it is an extension.”Kahn D, Steeves R. Narrative of dying aimed at understanding a ‘good death.’ Textbook of Palliative Nursing. 2001.

WHO Definition of PPCWHO Definition of PPC“…aims to improve the quality of life of patients facing life-threatening illness, and their families, through the prevention and relief of suffering by early identification and treatment of pain and other problems, whether physical, psychosocial, or spiritual.”

Total care of child’s mind, body, spirit + family support.

Begins when illness is diagnosed, and continues irrespective of whether or not child receives disease-directed treatment.

Emphasis on alleviating child’s physical, psychological and social distress.

Broad multidisciplinary approach including family and available community resources.

Can be provided in tertiary-care facilities, community health centers, home.

PPC in the CVICUPPC in the CVICUPrenatal diagnosis

~75% of all hypoplastic left heart syndrome (HLHS) cases diagnosed prenatally.

Atz et al. JTCVS. 2010.

Curative vs. palliative surgery; multiple operations.Promising short-term survival vs. uncertain long-term prognosis.

Considerable variation in mortality and morbidity among institutions.

Cardiac transplantation.High medical costs.Surgical vs. medical management philosophies.

Case 1Case 1Isaiah’s African-American parents presented to an outside ER intoxicated. Mom was in labor and had had limited prenatal care during her pregnancy.

Mom is 25 years old; this is her 4th living child and 6th pregnancy. She admitted to marijuana use during pregnancy. Dad is 19 years old, lives with GM.

Delivery was reportedly uncomplicated but the infant’s BAL was 0.17. He had facies characteristic of fetal alcohol syndrome. Birth weight was 2.0 kg.

Within the first 24 hrs of life, the infant developed respiratory distress and cyanosis. He was intubated.

At AHMC NICU, a diagnosis of HLHS was made. Prostaglandin infusion was started.

Hypoplastic Left Heart SyndromeHypoplastic Left Heart Syndrome

Surgical Options for HLHS- Stg 1Surgical Options for HLHS- Stg 1Norwood Operation with modified BT shunt (or Sano Modification)

Hybrid Stage 1- PDA stent, bilateral PA bands, atrial septostomy

Diagnosis ListDiagnosis ListHypoplastic Left Heart Syndrome

Severe tricuspid regurgitation

Fetal Alcohol SyndromeSmall for gestational ageEventration of right diaphragmHypoplastic right lung (20-25% decreased volume)Bilateral cryptorchidism

Parental alcohol addictionPossible FAS in siblings

Poor social situationOther family members/caretakers with alcoholism

Case 1Case 1NICU/Cardiology teams face massive denial from mother regarding diagnoses.

Isaiah is stable but requiring increasing ventilator support.

Staffing is arranged on DOL 11.

Mother is intoxicated in court the day prior and is subsequently hospitalized for substance abuse.

Custody of other 3 children given to MGM; parents retain medical decision-making rights.

Staffing is held with NICU and Cardiology teams, nursing and Dad.

Treatment options for HLHSTreatment options for HLHS1. Palliative surgery (Norwood vs. hybrid stage I,

Stage II- Glenn vs. hemi-Fontan, Stage III- Fontan).5-yr survival = 38-72%.

2. Cardiac transplantation5-yr survival = 55-76%; however, if waitlist mortality is included, survival = 54%.

3. Palliative care*

Bidirectional Glenn

Fontan

Palliative Care for HLHSPalliative Care for HLHS2008 data from England, Australia:

Similar surgical outcomes as US.Parents counseled about availability of PPC; surgery is recommended.

Termination of pregnancy after prenatal dx of HLHS: ~20% of cases in US.

- Allen et al. J Ultrasound Med, 2005.

Doctrine of informed consent: 1. Recommended treatment option, 2. All reasonable alternatives, 3. Consequences of not choosing life-prolonging therapy.

The PPC DebateThe PPC DebateCon:

Outcomes for HLHS surgery are good, and similar to those for other CHD and critical pediatric illnesses.

Therefore, palliative care is not an acceptable alternative and should not be offered.

Pro:

As many parents and physicians would choose palliative care, it is a reasonable option regardless of surgical outcomes.

Many factors other than survival sway parental decisions.

No outcome statistic can override the reasonable person standard; therefore, palliative care is a reasonable treatment option.

Parental Counseling for HLHSParental Counseling for HLHSPrsa et al, Pediatrics, 2010: Survey of 749 pediatric cardiologists

and surgeons.

When counseling parents of newborns with HLHS,99.7% discussed staged palliative surgery,67% discussed cardiac transplantation,62.2% discussed compassionate care without surgery.Only 14.9% discussed all options; 76.2% recommended surgery over other options.

When counseling parents after prenatal dx of HLHS,98.8% discussed continuation of pregnancy + palliative surgery,53.5% discussed continuation of pregnancy + cardiac transplantation,56.9% discussed continuation of pregnancy + palliative care,74.3% discussed termination of pregnancy.36.5% discussed all options; 56% recommended surgery over other options.

What would YOU do?What would YOU do?Prsa et al. Pediatrics, 2010.

Post-natal dx of HLHS:Staged palliative surgery= 42%Cardiac transplantation= 2%Palliative care= 19%

Prenatal dx of HLHS:Continuation of pregnancy + surgery= 24%Continuation of pregnancy + transplantation= <1%Termination of pregnancy or continuation or pregnancy + palliative care= 47%.

Kon et al. Am J of Cardiol, 2003: 215 neonatologists, intensivists, cardiologists, surgeons.

Post-natal dx: 32%- surgery, 6%- transplant/bridge to transplant, 28% palliative care.Prenatal dx: 22%- continue pregnancy, 48%- termination.

~55% of parents of HLHS children opt for palliative care when presented with all treatment options.* (*Data from 2000, 2001).

Case 1Case 1Given choice of palliative care vs. Norwood operation, dad chose surgery.

On DOL 16, Isaiah had long and complicated operation but was able to separate from cardiopulmonary bypass for ~2hrs.

During diaphragm repair, he developed pulmonary hemorrhage, severe ventilatory difficulty, hypoxia.

After 2-3 hours of consideration, decision made to transition to ECMO.

Extracorporeal Membrane OxygenationExtracorporeal Membrane Oxygenation

Case 1Case 1Returned to PSHU coagulopathic, anuric.ECMO flow limited by small cannula size.Dad present at bedside daily. Mom present since POD #1.On POD #3, PRISMA initiated.

Disagreement among healthcare staff.Mom initially did not want to continue.Ultimately chose PRISMA x 48hrs.

Case 1Case 1Later that day, worsening lactic acidosis, progressive coagulopathy, capillary leak, shedding of bowel mucosa, GI bleeding.

Head US shows early evidence of ischemic injury; patient has not woken up since OR.

Lactate>14 all night.

Case 1Case 1Parents independently feel like Isaiah is suffering, dying, that “it was time.”

Deny offers for family support, chaplain.Request immediate withdrawal of support.

Father holds baby while supported on ECMO and extubated.

Isaiah is placed on bed, circuit clamped. Expires peacefully 15 minutes later with parents holding his hands.

Palliative Care EducationPalliative Care EducationHilden et al. J Clin Oncol, 2001: 228 pediatric oncologists in USA, Canada, UK

92% learned PPC through ‘trial and error.’38% had associated traumatic experience with pt.

1970s: 6% of medical schools incorporated palliative care in curriculum; 1990s: 30% (mostly pre-clinical or elective).

Typical 1st exposure is during residency.Regardless of level, method, or type of educational intervention or evaluation, improvement is seen in outcome measures.

Barriers to PPC education = lack of time, lack of knowledgeable faculty and mentors.

Jan 2010: 74 active palliative care fellowships.Role for specially trained, board-certified palliative care NPs.

Case 2Case 2Sara is a 4mo female infant with prenatally suspected chromosomal trisomy and unbalanced AV septal defect.

Mom is 39yo, dad is 42yo; middle-class, Hispanic, Catholic.

Due to religious beliefs, they choose to continue pregnancy to term.

Upon delivery, she is diagnosed with trisomy 21 (Down Syndrome), UAVSD (R-dominant), coarctation of aorta. Parents request full medical and surgical treatment.

Unbalanced atrioventricular septal defectUnbalanced atrioventricular septal defect

Diagnosis ListDiagnosis List

Unbalanced AV septal defectRight-ventricle dominant

Bilateral superior venae cavae

Single ventricle

Coarctation of the aorta

Trisomy 21 (Down Syndrome)

Bilateral clubbed feet

Cystic right kidney

Tracheomalacia

Case 2Case 2Sara undergoes neonatal coarctation repair, followed by pulmonary artery band placement a month later.

She is discharged home where she grows, develops and returns at age 4 months for her bilateral bidirectional Glenn and Damus-Kaye-Stansel operations.

Her operative course is complicated by 3 CPB runs for hypoxemia, bradycardia, ventricular dysfunction.

She is returned to the PSHU with an open sternum with snares in place for possible ECMO cannulation.

Case 2Case 2Cardiac arrest, followed by E-CPR ensues. Sara develops severe capillary leak; Glenn pressures are noted to be elevated.

Serial head US demonstrate evidence of hypoxic ischemic injury.

10 days after initial surgery, she undergoes takedown of her Glenn and placement of central shunt. She is taken off ECMO successfully.

She remains an ECMO candidate.

Parents kept updated regularly and continue to request full support.

Case 2Case 2Sara’s condition continues to deteriorate over the next month with: capillary leak, arrhythmia, thrombocytopenia, copious chest tube output (despite thoracic duct ligation and chemical pleurodesis), diastolic dysfunction, kidney failure.

She has another brief cardiac arrest. Head US demonstrates evolving global ischemic injury.

Parents are informed of her grim condition but struggle with their choices. She remains full code.

The medical team decides that she is no longer an ECMO candidate.

Economic$ of $ingle ventricle $urgeryEconomic$ of $ingle ventricle $urgeryBetween 1998-2007, cumulative median length of stay, costs and charges for 3-stage palliative surgery:

LOS: 44 daysCosts: $171,672Charges: $376,403

Increasing costs thought to be due to increasing survival: 57%82%.

Cardiac transplantation associated with highest resource utilization and costs.

Highest cost for palliative surgery followed by transplantation.26-36% likelihood of death or transplantation following stage I surgery.

Costs comparable to other major congenital malformations, prematurity…?

Appropriateness of use of limited societal resources.

Dean et al. Pediatrics, 2011

Case 2Case 2Sara has 4 cardiac arrests in 24 hours, many more over following month.

Massive work-up initiated: EEG, head/abd US, specialized blood tests.

Dialysis is started and poorly tolerated.

Anasarca worsens. Tremendous skin breakdown with weeping and blisters occurs.

Medical staff struggles with caring for infant because of difficulty with pain control.

Parents want Sara to “make the decision.” Somewhat mixed messages from medical team. She remains full code.

Case 2Case 2“What are we doing?”

Sara becomes septic. She has cardiac arrests nearly every day, many of which are witnessed by her family.

Inotropic support is drastically escalated.

During her final cardiac arrest, MD suggests that mom hold her. She passes away.

Parents seem stunned, distraught.

Medical staff are supportive but drained.

Compassion FatigueCompassion FatigueDescribed in 1992 when studying burnout in ER nurses: “A unique form of burnout that affects people in caregiving professions.”

Sx: Fatigue, avoidance, depression, overeating, headaches, bodyaches, cynicism, boredom, loss of compassion, poor communication, more sick days, decreased productivity.

Aggravated by high workloads, staff shortages.

Disenfranchised grief; ongoing fluctuation between focusing on loss (experiencing grief) or repressing it (avoidance).

Need for greater support for healthcare professionals.

Case 3Case 3Ethan is a Caucasian male neonate with prenatally diagnosed HLHS.

Post-natally, he is noted to have multiple congenital anomalies.

32yo mother, 38yo father, 5yo sister, 17yo step-brother. Middle-class, employed.

Palliative care never offered.

Stage I surgery performed on DOL 11.

Diagnosis ListDiagnosis List

Hypoplastic left heart syndrome

Coffin-Siris syndrome

Absent corpus callosum, coprocephaly.

Abnormal gut anatomy, delayed gastric emptying, pyloric hypertrophy

Unilateral choanal atresia

Case 3Case 3First extubation attempt occurs 3 weeks post-op followed by multiple failed attempts and 3 cardiac arrests over next 3 mos.

He is treated for necrotizing enterocolitis, has significant feeding difficulties, undergoes gastrostomy, jejunostomy.

Successfully extubated 3 mos after initial surgery.

Over next 2 mos, has more feeding difficulties, failure to thrive.

Case 3Case 3At 6 mos age, 3.5-kg, undergoes bidirectional Glenn, TV plasty.

Fails extubation repeatedly. Has multiple cardiac arrests.

Develops junctional rhythm requiring atrial pacing via temporary pacing wires, which he pulls out.

Access is difficult because of vessel occlusion; however, indwelling catheters have to be removed because of infections.

Undergoes Nissen, pyloroplasty at 7 mos.

Ethan’s R diaphragm is plicated; new invasive lines are placed.

Case 3Case 3Ethan tolerates feeds but fails to gain weight.

He fails extubation repeatedly; bronchoscopy demonstrates airway pathology.

At 8 mos of age, tracheostomy is discussed with parents.

Cardiac cath demonstrates elevated Glenn pressures, collateral vessels.

Case 3Case 3Ethan’s Glenn is taken down at 9 mos age, 6-mm central shunt and new temporary pacing wires placed.

Develops slow junctional rhythm and evidence of pacemaker dependence.

Fails extubation repeatedly. Tracheostomy at 11 mos. Transitions to trach collar.

Starts having intractable seizures, requires 3 anticonvulsants for control.

Head CT scan shows diffuse hypoxic ischemic injury.

“When our son…”

Case 3Case 3Ethan celebrates 1st birthday in PSHU.

Mom researches and suggests switching him to ketogenic formula.

After discussion with Neurology, Ketocal is started.He finally starts gaining weight.

He develops complete pacemaker dependence; when temporary wires malfunction, he rapidly decompensates.

When presented with need for PPM, parents hesitate. Option of palliative care is presented.

“The staff was…”

Parental PerceptionsParental PerceptionsImportance of effective communication:

Honest, complete information.Delivered in timely, compassionate manner.Understandable language.Availability of medical staff for discussions.Conflicting information, “too many cooks in the kitchen” = mistrust.Displaying empathy, emotion = support.

Sensitivity, interpersonal skills felt to be more important than professional status.

Loss of controlInvolvement in decision-making.

Spirituality and religion.Prayer, access to clergy, faith.Connection to child.

Case 3Case 32 weeks later, a staffing is held. Parents request LET (limited end-of-life therapy) orders.

A week later, parents disclose that they would like to discontinue pacing soon.

They request comfort care in the interim, minimal blood draws, positive energy, respect of their privacy.

Ethan develops respiratory illness; parents consent to work-up, antibiotics.

Though initially hesitant, his parents consent to transfer to Step-down Unit.

“After exhausting every…”

Parental PerceptionsParental PerceptionsTranscendent quality of parent-child relationship.

Value of parental contribution to child’s care.Importance of time alone with child.Evaluation of meaning of loss.

AltruismOrgan donation.

Relief of external signs of pain, suffering.

Continuation of bereavement support after death.

Sense of community within hospital.MDs, nurses often considered more involved during dying process than other family, friends.

Case 3Case 3On the selected day, at nearly 14 mos age, Ethan is transferred to a private room.

Feeds and medications are discontinued. Fentanyl and versed infusions are started for comfort.

Family and medical staff are invited to say their goodbyes.

That evening, per his parents’ wishes, his pacemaker was stopped and wires were cut.

“On Ethan’s final day…”

“Anyone can slay a dragon. It takes a real hero to wake up every day and learn to love the world all over again.”