Symptom Control for Symptom Control for Pediatric PatientsPediatric Patients

A guide to the management of pain, nausea, and other symptoms in seriously ill children, with a focus on the social and medical aspects of end-of life care.

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The Jason Program

creating a community of care

Why Are You Why Are You Here?Here?

Be the caregiver you would want if you were in pain.

OutlineOutline Social AspectsCure vs. PalliationAccepting end-of-life careMaintenance of active medical careManaging death - Home or Hospital?

Medical CarePain ControlOther Common SymptomsNebulized Everything

Last Hours of Life

Cure vs. PalliationCure vs. Palliation

Cure -- fundamental hope is eradication of disease to achieve longevity -- assumes cure is worth a sacrifice

Palliation -- fundamental hope is comfort -- consequences of any intervention

that relieves suffering are acceptable

Curative / Life-Prolonging Therapy

Relieve Suffering - “Palliative” Care

Presentation

Death

A Better Viewpoint

Accepting End-of-Life Accepting End-of-Life CareCare

Hope is never lost

MD must accurately understand the medical situation and estimate the chance for cure

With the family, level of support is determined Previously established trust is helpful Clear communication and truth are necessary Shift towards increased family control Identify goals Situation is dynamic

Maintain Active Medical Maintain Active Medical CareCare

Socially Important Families need to know what is happening Families need to plan and adapt Feelings of security fostered Fears of abandonment eliminated

Medically Important Symptom relief necessary Maintain dignity Accomplish desired goals PROactive rather than REactive

Death at Home vs. Death at Home vs. HospitalHospital

Positive Home Death -- (Ida Martinson) More control over daily activities Medical care often better than in hospital Home is a safe, comfortable place Usually requires well functioning family Staff support of the home death concept helpful

Positive Hospital Death -- Family does not need to take a medical role Death at home may leave greater scars For some, sibling issues are easier Make hospital room feel like home

Medical Care IssuesMedical Care Issues

Pain Other Common Symptoms Venous Access Neonatal Pain Terminal Care Case Studies

Oncologic EmergenciesImmediate Intervention

RequiredCommon

Less Common

Pain

Fever with Neutropenia or Splenectomy

Airway Compression

Spinal Cord Compression

Brain Herniation

Hyperleukocytosis

Pain ManagementPain Management

Freedom From Pain: A Matter of Rights? T. Patrick Hill, M.A.

Ca. Invest., 12 (4), 1994

Pain Isolates: “We are probably never more alone than when severe pain invades us.”

Pain is Elusive: “Despite the fact that it is the result of biochemical processes, it is also ... a subjective experience, felt only within the confines of our individual minds.”

A Matter of AttitudeA Matter of Attitude

“Pain is unlike disease, and that to treat its symptoms clinically, physicians need above all to understand how the ravages of pain can reach beyond the body to the soul of the person, assaulting its very integrity.”

There exists “ a principle on which rests the human right to be free of pain and the corresponding obligation of health-care professionals to honor it. All patients are vulnerable, but none is more vulnerable than the patient in severe pain. The measure of medicine in general and of a physician in particular is ultimately their respect for the patient’s right to be free of pain.”

Barriers to Pain Barriers to Pain ControlControl

... “ the most pervasive and difficult to overcome relate to the fears among patients, families, and health professionals of opioid analgesics, which are the cornerstone of drug therapy for moderate to severe pain.

These fears include an exaggerated estimation of opioid addiction and tolerance, fear of opioid side effects -- most notably respiratory depression -- and ethical and regulatory concerns about using opioids.”

Weissman, David E. Home Health Care Consultant Vol. 2, No. 5, Sept. 1995

Treatment Treatment PrinciplesPrinciples

Correctly Assess Degree and Cause of Pain

Consider Psychosocial Factors Consider 24 hour Coverage

Children Severe or Chronic Pain Patient- Controlled Analgesia

Opioids Are Safe Respiratory Depression Overestimated Pharmacologic Dependence With Chronic Use

Never use a placebo

Pediatric Pain Pediatric Pain AssessmentAssessment Infant

HR, Resp, BP fever, sweating

Child Irritability, esp. paradoxical Refusal to walk or use a painful limb Functional changes (school, sports,

etc.) May be able to use pain scale

Adolescent Generally accurate reporter May be reluctant to participate

WHO 3-Step WHO 3-Step LadderLadder

Step 1 - Mild

Step 2 - Moderate

Step 3 - Severe

Aspirin

Acetaminophen

NSAIDs

Codeine

Hydrocodone

Oxycodone

Tramadol

Morphine

Hydromorphone

Methadone

Levorphanol

Fentanyl

Always consider adding an adjuvant Rx

Level I MedicationsLevel I Medications

Acetaminophen 12 - 15 mg/kg, Q 4hr, PO or PR

NSAIDs Ibuprofen

10 mg/kg, max 40mg/kg/day, Q 6hr, PO Ketorolac (variable efficacy)

0.5 mg/kg IV/IM, 5-10 mg PO, Q 6hr Cox 2 Inhibitors

Vioxx, oral solution, 0.5 mg/kg QD (effective) Occasional sedation Celebrex has better GI safety profile

Level II and III Level II and III MedicationsMedications

Pain Control Using Narcotics

Principles of Narcotic Principles of Narcotic DosingDosing

The Right Dose is the Dose that Works

Pain and the Reticular Activating System

“The respiratory depressant effect of opioid agonists can be demonstrated easily in volunteer studies. When the dose of morphine is titrated against a patient’s pain, however, clinically important respiratory depression does not occur. This appears to be because pain acts as a physiological antagonist to the central depression effects of morphine.”

Wall, R.D., ed. Textbook of Pain. Churchill Livingstone

Naive Pts. vs. Tolerance

Enteral NarcoticsEnteral Narcotics Codeine

1 mg/kg, Q 2-4 hrs, PO Ineffective for age >~10-12 years

Hydrocodone (Lortab) 0.1 mg/kg PO q 2-4 hours (very good for moderate pain)

Oxycodone 5 - 10 mg/ dose PO q 2-4 hours (Tylox)

Tramadol (Ultram) 0.7 - 2.0 mg/kg/dose PO Q 4-6 hours (variable efficacy)

Morphine (the gold standard) 0.3 mg/kg PO Q 2-4hr

Morphine SR (MS Contin)0.5 mg/kg, BID, PO (Do not crush)

Parenteral Narcotics Parenteral Narcotics Morphine

0.1 mg/kg IV bolus, Q 1-2hr .05 mg/ kg/hr, CI - IV or SQ

Hydromorphone (Dilaudid) Approximately 6 times stronger than morphine

Fentanyl Approximately 10 times stronger than morphine Wide dosing range 1-2 mcg/kg IV slow push 0.5-1.0 mcg/kg/hr, CI - IV or SQ Total hourly dose as a transderm patch

Patient-Controlled Patient-Controlled AnalgesiaAnalgesia

Age > 4 years (if able to play computer games) Home or Hospital Adequate observation

Medication Base Rate Bolus Dose Lockout “Max”/Hr

Morphine .03 mg/kg Same 6-10 min .15 mg/kg

Dilaudid 5 mcg/kg Same 6-10 min 25 mcg/kg

Fentanyl 1 mcg/kg Same 6-10 min 4 mcg/kg

Equianalgesic Narcotic Equianalgesic Narcotic

DosingDosingSource : McCaffery M, Pasero C. PAIN : Clinical Manual, 2nd Edition, Source : McCaffery M, Pasero C. PAIN : Clinical Manual, 2nd Edition,

Harcort Health Sciences Website, 2000. Harcort Health Sciences Website, 2000.

www.harcourthealth.com/PAIN/index.htmlwww.harcourthealth.com/PAIN/index.htmlOral/Rectal Dose (mg)

AnalgesicParenteral Dose (mg)

3 Morphine 120 Codeine 12

3 Hydrocodone --

0.75 Hydromorphone 0.15-(0.3 w/ PCA)

2 Oxycodone --

2 Methadone 1

25 mcg/hr Fentanyl Patch = 1 mg/hr IV MSO4

-- Fentanyl 10-20 mcg

30 Meperidine 7.5

Common Uncommon

Constipation Bad dreams / hallucinationsDry mouth Dysphoria / deliriumNausea / vomiting Myoclonus / seizuresSedation Pruritus / urticariaSweats Respiratory depression

Urinary retention

Opioid Side Effects

Demerol is not recommended due to its side effects

Addiction is NOT a side effect

CNS ExcitationCNS Excitation Eliminate primary cause Medications

Haldol (drug of choice) Age 3-12: Agitation: 0.01-0.03 mg/kg/day div QD - TID Age 3-12: Psychosis: 0.05-0.15 mg/kg/day div BID-TID Age >12: Acute agitation: 2-5 mg IM or 1-15 mg PO, Q1h PRN Age >12: Psychosis: same doses, IM Q 4-8 hr; PO div BID-TID

Benzodiazepenes (may exacerbate delirium) Dantrium - muscle spasms

4-8 mg/kg/day, PO, div QID 2.5 mg/kg by slow IV per dose, to effect

Narcotics are generally not indicated as these symptoms are usually uncomfortable, but not painful.

MyoclonusMyoclonus Melatonin in treatment of non-epileptic myoclonus in children

Developmental Medicine & Child Neurology 1999, 41: 255-259

Melatonin - pineal hormone regulates sleep Absence seizures; MLT is anticonvulsant 1.25µ/kg IV MLT causes EEG slowing and sleep Half-life < 1 hour

Case Reports: Three children with severe sleep disorders due to

myoclonus 1 had epilepsy, 2 without epilepsy

Case ICase I

15 month-old boy with holoprosencephaly & spastic quadriplegia; no epilepsy

Prolonged clusters of myoclonus only before sleep Lasted several hours crying and exhaustion No change in sensorium Benzodiazepenes failed 5 years of age:2.5 mg oral FR MLT QHS Myoclonus stopped after 2 days; returned if MLT

stopped 8 years of age: developed AM myoclonus; 4mg CR MLT

(replacing 5mg FR MLT) successful

AddictionAddiction

“…neurobehavioral syndrome with genetic & environmental influences that results in psychological dependence on the use of substances for their psychic effects.” ME Board of Licensure in Medicine

Compulsive use Loss of control over drugs Loss of interest in pleasurable activities Continued use of drugs in spite of harm A rare outcome of pain management

PseudoaddictionPseudoaddiction

“Pseudoaddiction” is a pattern of drug-seeking behavior of pain patients who are receiving inadequate pain management that can be mistaken for addiction.

Department of Professional & Financial Regulation, Board of Licensure in Medicine, a joint chapter with the Board of Osteopathic Medicine, Chapter 11: Use of Controlled Substances for

Treatment of Pain 

ToleranceTolerance

Reduced effectiveness of a given dose over time

Not clinically significant with chronic dosing

If dose is increasing, suspect disease progression

Physical dependencePhysical dependence

A process of neuroadaptation Abrupt withdrawal may abstinence

syndrome If dose reduction required, reduce by

50%every 2–3 days

Avoid antagonists

Substance AbusersSubstance Abusers

Can have real pain Treat with compassion Create protocols and contracts Consider a consultation with pain or

addiction specialists

More Options

Adjunctive Pain Adjunctive Pain TreatmentsTreatments

Radiotherapy External beam or brachytherapy Bone Metastases :

NSAIDs Hemibody XRT Radioisotopes

Anesthetic Procedures Epidural anesthetics Nerve Block

Neurosurgical Procedures Neurolysis

Orthopedic Procedures Stabilization of pathologic fractures

Complimentary Complimentary InterventionsInterventions

Acupuncture Relaxation Therapy Spiritual Assistance Hypnosis / Biofeedback / Massage Art Therapy

Summary

NIH Consensus NIH Consensus StatementStatement2121

“The introduction of acupuncture into the choice of treatment modalities that are readily available to the public is in its early stages. Issues of training, licensure, and reimbursement remain to be clarified. There is sufficient evidence, however, of acupuncture's value to expand its use into conventional medicine and to encourage further studies of its physiology and clinical value.”

Other Common Other Common SymptomsSymptoms

Neurologic Pain Anxiety Depression Breathlessness When All Else Fails Nausea Constipation

Narcotic PruritusNarcotic Pruritus Due to mast cell destabilization Routine skin care ? Reduce dose or change narcotic Antihistamines

Claritin (or other non-sedating antihistamines) 1- 6 years 5 mg PO QD >6 years 10 mg PO QD

Benadryl 1 mg/kg, IV or PO, Q 4-6 hr

H2 Blockers may be effective Narcotic receptor blockade

Narcan, 0.005 mg/kg/hr, IV or SQ

SedationSedation

Distinguish from exhaustion due to pain Tolerance develops within days Treatment – Stimulants

Ritalin, start @ 5-10 mg PO BID Consider SR, 20 mg BID Maximum 20 mg QID Adderall is an alternative

Physiology of NauseaPhysiology of Nausea CTZ

•All transmitters

Cortical Anticipation

GI Tract

•Serotonin -- vagal

•ACH - peristalsis

•? Dopamine

Other CNS

•Vestibular ACH, histamine

•ICP

Vagal •acetylcholine

Pharmacologic Pharmacologic ManagementManagement

Serotonin Blockage -- “Wonder Drugs”

Zofran (Ondansetron) 0.15 mg/kg PO or IV Q 4-8 Hr Oral forms: Solution: 4mg/5ml, Disintegrating tab: 4,

8 mg, Tabs, 4, 8, 24 mg Approved for chemo, post-op, gastroenteritis No significant adverse effects Less effective with delayed nausea Kytril (Granisetron)

1 mg PO QD or BID Oral forms: 1 mg tab, Solution, 2mg/10 ml

Pharmacologic Pharmacologic ManagementManagement

Dopamine Blockade

Phenothiazines (Compazine, Trilafon) Butyrophenones (Droperidol, Haldol) Benzimidazoles (Metaclopramide,

Domperidone) Modestly effective; Sedation occasionally

useful Side effects common: sedation, EPS,

xerostomia, hypotension

Other MeasuresOther Measures Steroids

Most effective Rx for post-chemo nausea Anxiolytics

Amnesia / Sedation / Relaxation Propofol @ Sub-Hypnotic Doses Canabinoids (THC)

Oral: variable side effects, often unpleasant ? Inhaled GI Agents

Prokinetic Rx Proton Pump Inhibitor Octreotide (Useful in GI obstruction)

Non-Pharmacologic Interventions Avoid negative associations (taste, odors, emesis basin) Pt. may prefer nausea to medication

Not Not RecommendedRecommended

Meperidine Normeperidine is a toxic metabolite

longer half-life (6 hours), no analgesia if dosing q 3 h, normeperidine builds up accumulates with renal failure psychotomimetic effects, myoclonus, seizures nausea

Propoxyphene (no proven efficacy) Mixed Agonists/Antagonists (toxicity)

Federal Foolishness Federal Foolishness & Marijuana& Marijuana

Jerome P. Kassirer, M.D.

NEJM, January 30, 1997

“Thousands of patients with cancer, AIDS, and other diseases report they have obtained striking relief from these devastating symptoms by smoking marijuana....I believe that a federal policy that prohibits physicians from alleviating suffering by prescribing marijuana for seriously ill patients is misguided, heavy-handed, and inhumane.”

Neurologic PainNeurologic Pain

Caused by diseased neurons Characterized as burning, tingling,

electric Medications

Amitryptiline, start at 25 mg PO HS and increase as tolerated to relief

Neurontin, 1800 - 3600 mg/day div TID Narcotics are also useful – Methadone may an effective agent NMDA Blockers - High dose dextromethorphan

Under investigation now @ ~ 400 mg/day

AnxietyAnxiety

Non-Pharmacologic Compassionate Exploration of issues Alternative medical approaches

Pharmacologic Benzodiazepenes - Choose by half-life Valium: 0.1 mg/kg IV or PO; rectal gel - 0.2-0.5 mg/kg Ativan: 0.05 mg/kg, PO, IV, or SL Versed: 0.05 mg/kg IV; 0.5 mg/kg PO

Long

Short

DepressionDepression Risk Factors

Poorly controlled pain Physical impairment Poor social supports Spiritual pain

Symptoms Hopelessness Loss of self-esteem Helplessness Suicidal ideations Do you feel depressed most of the time?

Medication Ritalin, 5-10 mg BID SSRI

BreathlessneBreathlessnessss

Sense of drowning Medical Management

Correct the underlying problem Oxygen

Placebo vs. Cool Air? Opioids Anxiolytics

Non-Medical Management Cool room with open window Relaxation, hypnosis, minimize loneliness Eliminate irritants

ConstipationConstipation Guaranteed to Work -- Miralax

PEG - Brings water into the bowel lumen

Tasteless in orange juice Prevention

~ ½-1 cap (17 gm) per 8 ounces juice QD - BID

“Cleanout” 1-1.5 gm/kg QD X 3 days

When All Else FailsWhen All Else Fails

Butyrophenones Droperidol 0.025 - 0.05 mg/ kg IV Q 4-6 hr prn

Barbiturates Pentobarbital 2 - 8 mg/ kg IV,PO, PR, IM, Q 1-4 hr prn Special Considerations

Barbiturates in the Care Barbiturates in the Care of of The The Terminally IllTerminally Ill

Barbiturates: Reliably produce sedation and unconsciousness

(comfort) Are used in the execution of prisoners by lethal

injection

Ethical Considerations: The Principle of Double Effect --

Distinction between intended effects and unintended although foreseen effects.

•Truog, Robert D., et. al. NEJM, Vol. 327, No. 23, 1678-81

Barbiturates Are Barbiturates Are Justified Justified

To relieve physical suffering when all reasonable alternatives have failed

To produce unconsciousness before terminal extubation

Produce deep sedation and unconsciousness as a means of relieving nonphysical suffering

Venous Venous AccessAccess

Concept Placement of a venous access device to

allow for treatment without repeated veinipunctures.

Advantages Minimizes pain Nearly eliminates extravasation Permits delivery of central TPN Facilitates care in home and hospital settings

Disadvantages Infection Thrombosis

OptionsOptions

PICCPICC

PAS PortPAS Port

CookCook

BroviacBroviac

Port-a-CathPort-a-Cath

External VAD

CookCook

HickmanHickman

BroviacBroviac

PICCPICC

WalrusWalrus

VAS-CathVAS-Cath

SQ VAD

Port-a-CathPort-a-Cath

MediportMediport

PAS portPAS port

Pain in NeonatesPain in Neonates

Consensus Statement for the Preventionand Management of Pain in the Newborn

K. J. S. Anand, MBBS, DPhil; and the International Evidence-Based Group for Neonatal Pain

Arch Pediatr Adolesc Med. 2001;155:173-180

Management of pain must be Management of pain must be considered an important considered an important component of the health care component of the health care provided to all neonates, provided to all neonates, regardless of their gestational regardless of their gestational age or severity of illnessage or severity of illness..

ConclusionConclusion

Management of PainManagement of Pain 1. Pain in newborns is often unrecognized and

undertreated. Neonates do feel pain, and analgesia should be prescribed when indicated during their medical care.

2. If a procedure is painful in adults, it should be considered painful in newborns, even if they are preterm.

3. Newborns may experience a greater sensitivity to pain compared with older age groups and are more susceptible to the long-term effects of painful stimulation.

4. Adequate treatment of pain may be associated with decreased clinical complications and decreased mortality of neonatal pain.

ContinuedContinued

5. Environmental, behavioral, and pharmacological interventions can prevent, reduce, or eliminate neonatal pain.

6. Sedation does not provide pain relief and may mask the neonate’s response to pain.

7. Health care professionals have the responsibility for assessment, prevention, and management of pain in neonates.

8. Clinical units providing health care to newborns should develop written guidelines and protocols for the management

Pain Scales

Analgesic MedicationsAnalgesic Medications

Nebulized EverythingNebulized Everything

Guaifenesin (glycerol guaiacolate) The idea: “If the cough reflex is strong,

loosen secretions with nebulized saline and guaifenesin.”26

Opioids for Dyspnea Lidocaine for cough & hiccoughs

Managing secretionsManaging secretions2525

Saliva produced in the oral cavity under neurologic control 3 pints/day

Sputum mucous secretion produced by pulmonary

epithelium <100 ml/day bronchorrhea is > 100 ml/day production

Improve Mucociliary Improve Mucociliary ClearanceClearance Guaifenesin - creosote derivative

amount of upper airway fluid25

fluid surface tension & adhesiveness25

?except in chronic bronchitis34

efficacy enhanced by strong cough25

Safety 100 mg/kg = horse anesthesia 150 mg/kg = pig EEG changes of sedation No side effects in chronic bronchitis @ 1600

mg/D34

Our experience

Opioids for DyspneaOpioids for Dyspnea Pharmacology

“The individual relative bioavailabilities of inhaled morphine varied from 9% to 35%, with a mean of 17%.”28 (50mg neb, 10mg po, 5 mg IV)

“The systemic bioavailabilities of morphine were5 +/- 3% and 24 +/- 13% for the nebulized and oral routes respectively.” 29(50mg neb, 10mg po, 5 mg IV)

“Peak plasma morphine concentrations were achieved more rapidly after nebulized than oral morphine, occurring within 10 min in all subjects.” 29

EfficacyEfficacy Pediatrics. 2002 Sep;110(3):e38.

20-kg boy with end stage cystic fibrosis Dose: 2.5 12.5mg (0.125-0.625 mg/kg) Venous pCO2 < 4mm; 9mm at 12.5 mg dose Conclusions:

“…a mild, beneficial effect on dyspnea, with minimal differences found between the lowest and highest doses.”

“More studies are needed to determine what, if any, the optimum dose of nebulized morphine is for children.”

Nebulized LidocaineNebulized Lidocaine Pediatric Safety36

6 severely asthmatic patients followed in the Pediatric Allergy and Immunology Section, Mayo Clinic, 1996

Dose: 0.8 mg/kg/dose to 2.5 mg/kg/dose TID-QID

Mean duration of therapy: 11.2 mos (7-16 mos) Toxicity: None “lidocaine may prove to be the first non-toxic,

steroid alternative to patients with severe steroid-dependent asthma.”

Pediatric SafetyPediatric Safety

New York Medical College37, 1997 In flexible bronchoscopy - 20 pts., not intubated, no cardiac or hepatic

disease Dose: 8 mg/kg or 4 mg/kg of nebulized 2%

lidocaine by face mask prior to bronchoscopy (randomized)

Safety: serum lidocaine levels much < toxic Conclusion: “Nebulized lidocaine in doses up to

8 mg/kg appears to be safe and moderately effective as a topical anesthetic for flexible bronchoscopy in infants and children.”

EfficacyEfficacy Hiccups38

58 yr.-old man, 5 mos. Hiccups Dose: 3ml, 4% topical lidocaine, QD X 3 D Resolved for 3 weeks, retreated

successfully Cough39,40

Type: Intractable, Habit Dx.: Asthma, COPD Efficacy: Very effective

Breathlessness41 (terminal care in adults) Ineffective

Protocol VariationsProtocol Variations

Bronchodilator pre-treatment lidocaine can cause bronchospasm

Cardiac monitoring lidocaine arrthymias

+/- 1.0 ml 0.5% bupivicaine NPO for 1-several hours after Rx

Loss of gag reflex

Last Days of Living - Last Days of Living - Social Aspects Social Aspects

Preparation DNR Letting Go Physical Presence at Time of Death Mechanism of Death Autopsy Follow-up

Last Days of Last Days of Living - Medical Living - Medical

AspectsAspects Weakness & Fatigue Dehydration Respiratory Distress Temperature Changes Increased Secretions Pain May Increase Anxiety Two Roads to Death

Two Paths to Two Paths to DeathDeath

Usual

Difficult

Sleepy

LethargicObtunded

Comatose

Death

Restless

Confused Tremulous

Hallucinations

Delirium

Seizures

Myoclonic Jerks

Thanks for listeningThanks for listening

In ClosingIn Closing --- Moldow, D.G. and Martinson, I.M., 1984

“On December 17, 1978, Shawn, a 10 year old boy, died of ... cancer. Shawn’s disease had reached a stage where there was no hope for a lasting cure.... Shawn chose to discontinue treatment and to return home for the final days of his life. Shortly before his death he stated in his own words...

And I decided not to take the treatment, because I had been through all that and it was hard. And it wouldn’t guarantee that I would live....days don’t count unless they’re good days....You just have as much fun as you can, and make use of it, it’s like each day is a gift.

Shawn died at home with his family.”

Thanks for ListeningThanks for Listening

Gary Allegretta, M.D.

Kennebunk Pediatric Center

Phone: 207-985-6770

E-Mail:medicaldirector@jasonprogram.org

Fax: (206) 338-2426

Web: www.jasonprogram.orgBreak Time!

Case ICase I

Two day-old infant due for a circumcision

Case IICase II

Five year old boy, 25 kg, with relapsed neuroblastoma and bony metastases. He is receiving palliative chemotherapy. He has had slowly increasing pain, despite the use of Tylenol with codeine, scheduled Q 4H. He presents for a routine visit, where he is comfortable at rest. The parents carry him because he refuses to walk.

Case IIICase III

17 year old girl with advanced cystic fibrosis. She has severe thrombocytopenia, fatigue, and poor urinary output, but strongly wishes to attend her sister’s wedding next month. She complains of no dyspnea, but her PCO2 is 70 and her PO2 is 60. How “aggressive” would you be?

Case IVCase IV

10 year old girl, 40 kg, with far advanced abdominal malignancy and intestinal obstruction. Receiving morphine at 100 mg/hr without relief. Her parents would like her to be awake for the arrival of a relative tomorrow, but don’t want her to suffer.

Case VCase V

15 year old girl with an advanced CNS tumor. She is becoming restless and has periods of confusion. The family wants to stay at home at all costs. Is this possible? How would you plan for the future?

Case VICase VI 12 year old girl with Werdig-Hoffman’s disease, which is a severe,

progressive, congenital neuropathy. She lives in a nursing home, as her parents are incapable of caring for her at home. She carries a DNR order as well as an order not to transfer her to another institution for mechanical ventilation if needed. She often requires an external ventilator for survival when pulmonary infections or asthma occur, and has recently been dependant for the past 5 weeks due to recurrent infections and malnutrition. She is lucid and intelligent. Her mother, who is mentally unstable, has recently given sole responsibility of her care to her father, who has not visited in three years. The ventilator now partially fails. The father upholds the DNR and no transport orders, but wishes Grace to have IV fluids, pain control, and antibiotics, despite the patient’s desire to avoid the IV.

How would you manage this situation?Next Topic

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