Dr Michelle Meiring

MBChB, FCPaeds, MMED (Paeds)

HPCA, PCSSA, UCT and

The Bigshoes Foundation

AWACC Conference

Durban 02/10/2009

8 year old boy from rural KZNSent to Jhb by his granny to live with his uncle ( a

miner) to access “better health care”WHO Class IV 3 disease - CD4 < 1 (0,1%)His mother had died from HIV related illness but

had not disclosed to her family- ?one of the reasons for the child’s late first presentation

He had a 5 year old brother who he was very close to who was HIV negative

Worked up and started on HAART as an outpatient

TB excludedHad to be hospitalized for complications

Complications included malignant bowel obstruction secondary to Non-Hodgkins Lymphoma

Surgeons unable to offer surgery due to his poor general condition

Developed severe abdominal pain that paradoxically seemed to worsen with morphine

Fortunately the obstruction resolved with conservative therapy and he was prepared for chemotherapy

During chemo developed excruciating peripheral neuropathy that was difficult to control

Complications included malignant bowel obstruction secondary to Non-Hodgkins Lymphoma

Surgeons unable to offer surgery due to his poor general condition

Developed severe abdominal pain that paradoxically seemed to worsen with morphine

Fortunately the obstruction resolved with conservative therapy and he was prepared for chemotherapy

During chemo developed excruciating peripheral neuropathy that was difficult to control

Fortunately peripheral neuropathy resolved with treatment

Despite a stormy course of chemotherapy did well and went into remission

Discharged back into care of uncle in JhBSent back to live with granny in rural KZN by

uncleAlthough granny tried to keep up with his

treatment, she struggled to collect his meds and keep appointments esp as she was also looking after his 5 year old brother and other grandchildren

Unfortunately ended up defaulting on treatmentSent up by granny to uncle in JhB againArrived in a critically ill stateRelapsed NHLDecision made to “palliate”Referred to Hospice Soweto for terminal careDied peacefully after a visit with his gran and

brotherFamily received assistance to bury him back in

KZN from hospice

What did the Bigshoes JhB Paediatric Palliative care team and Hospice Soweto do for the child?

8 year old boy from rural KZNSent to Jhb by his granny to live with his uncle ( a

miner) to access “better health care”WHO Class IV 3 disease - CD4 < 1 (0,1%)His mother had died from HIV related illness but

had not disclosed to her family- ?one of the reasons for the child’s late first presentation

He had a 5 year old brother who he was very close to who was HIV negative

Worked up and started on HAART as an outpatient

TB excludedHad to be hospitalized for complications

Sibling

support

Bereavement

counseling

Complications included malignant bowel obstruction secondary to Non-Hodgkins Lymphoma

Surgeons unable to offer surgery due to his poor general condition

Developed severe abdominal pain that paradoxically seemed to worsen with morphine

Fortunately the obstruction resolved with conservative therapy and he was prepared for chemotherapy

During chemo developed excruciating peripheral neuropathy that was difficult to control

Explanatio

n

Expert pain

control

Expert advice on Mx

ofMalignant bowel

obstruction

Fortunately peripheral neuropathy resolved with treatment

Despite a stormy course of chemotherapy did well and went into remission

Discharged back into care of uncle in JhBSent back to live with granny in rural KZN by

uncleAlthough granny tried to keep up with his

treatment, she struggled to collect his meds and keep appointments esp as she was also looking after his 5 year old brother and other grandchildren

Advice on the Mx of severe

mucositis –

magic mouth

wash

HIV

Disclosure Counseling

Second line tre

atment

Including motivating for

gabapentin (neurontin)

Unfortunately ended up defaulting on treatmentSent up by granny to uncle in JhB againArrived in a critically ill stateRelapsed NHLDecision made to “palliate”Referred to Hospice Soweto for terminal careDied peacefully after a visit with his gran and

brotherFamily received assistance to bury him back in

KZN from hospice

Assistance with

decision making re

withdrawal of

therapy

Provision of

holistic term

inal

care in a more

conducive

environment

Funeral and

bereavement

support to family

Spiritual care

8 year old boy from rural KZNSent to Jhb by his granny to live with his uncle ( a

miner) to access “better health care”WHO Class IV 3 disease - CD4 < 1 (0,1%)His mother had died from HIV related illness but

had not disclosed to her family- ?one of the reasons for the child’s late first presentation

He had a 5 year old brother who he was very close to who was HIV negative

Worked up and started on HAART as an outpatient

TB excludedHad to be hospitalized for complications

Counselled mom on

her death bed about

disclosure and future

planning for her

children

Fortunately peripheral neuropathy resolved with treatment

Despite a stormy course of chemotherapy did well and went into remission

Discharged back into care of uncle in JhBSent back to live with granny in rural KZN by

uncleAlthough granny tried to keep up with his

treatment, she struggled to collect his meds and keep appointments esp as she was also looking after his 5 year old brother and other grandchildren

Home based care

and

HAART supervisio

n

Orphan care and

support

AND IT’S ROLE IN PAEDIATRIC HIV MANAGEMENT

WHO Definition:Palliative care for children represents a special,

albeit closely related field to adult palliative care. PPC is the active total care of the child's

body, mind and spirit, and also involves giving support to the family.

It begins when illness is diagnosed, and continues regardless of whether or not a child receives treatment directed at the disease.

WHO Definition continued:Health providers must evaluate and alleviate a child’s

physical, psychological and social distress.Effective palliative care requires a broad

multidisciplinary approach that includes the family and makes use of available community resources; it can be successfully implemented even if resources are limited.

It can be provided in tertiary care facilities, in community health centres and even in children's own homes.

WHO Definition:Palliative care for children represents a special,

albeit closely related field to adult palliative care. PPC is the active total care of the child's

body, mind and spirit, and also involves giving support to the family.

It begins when illness is diagnosed, and continues regardless of whether or not a child receives treatment directed at the disease.

PASSIVE

LIMITED TO

TERMINAL

CARE

THE ANTITHESIS OF

ACTIVE TREATMENT

(EG HAART)

WHO Definition continued:Health providers must evaluate and alleviate a child’s

physical, psychological and social distress.Effective palliative care requires a broad

multidisciplinary approach that includes the family and makes use of available community resources; it can be successfully implemented even if resources are limited.

It can be provided in tertiary care facilities, in community health centres and even in children's own homes.

TOO EXPENSIVE

LIMITED TO

HOSPICES

ONLY

Figure 1 Traditional palliative care services model

DIAGNOSIS

ACTIVE AGGRESSIVE

INTENT

PALLIATIVE INTENT BEREAVEME

NT

DIAGNOSIS

PALLIATIVE INTENT

BEREAVEMENT

Figure 2:Modified integrated palliative care services model (Frager, 1997)

You don’t have to die to get out of a paediatric palliative care programme

ACT CLASSIFICATION OF PPC CONDITIONSACT 1: Life threatening conditions that can be

cured but where treatment may fail (eg ALL,Malnutrition)

ACT 2: Life limiting conditions associated with premature death but which are manageable due to available treatments (HIV on HAART)

ACT 3: Non-curable conditions where treatment is exclusively supportive (eg IEM)

ACT 4: Non-progressive life limiting conditions associated with disability

It will not become obsolete!

PA

LL

IAT

IVE

TR

EA

TM

EN

T

ACTIVE TREATMENT

AZT : 1986

HAART: 1996

IRIS, RESISTANCE, DRUG TOXCICITIESMDR TB, XDR TB8O’s

90’s2000….

Still 60% of patients requiring HAART in SA not accessing this

Still 1000 people dying from HIV in SA every day

Increasing numbers of patients with IRISARV side effectsTreatment failures secondary to resistanceIncreasing number of orphans nearly 2

millionThe need for palliative care is great!

HAART is the most powerful palliative care drug around!

It does not cure the patientBy addressing the root cause of distressing

symptoms- immunocompromise – opportunistic infections

Improves quantity of lifeImproves quality of lifeDecreases hospitalisationsRestores hope!!

Pain in children with HIV/AIDS is a multifactorial, biologically complex problem associated with diminished quality of life and increased mortality (PACTG 219)

Pain in advanced HIV can be more complex than pain in children with cancer

Pain in cancer is more often related to treatment and procedures than the cancer itself

Pain in HIV is more commonly related to the disease and its complications

INCIDENCE OF PAIN IN HIV:Adult studies: 40 – 60% depending on stage

of HIVPaediatric studies: fewer than adults

Varies from 21-59%National Cancer Institute NIH:1996 (pre-

HAART): 59%Paediatric late outcomes study (PACTG-

219):2002 patients on HAART: 59%

WHAT CAUSES PAIN IN CHILDREN WITH HIV?HIV itself: peripheral neuropathy, cardiomyopathy,

myositis, arthritis, osteonecrosis of the hipGp 120: activates NMDA receptors on excitatory

neuronsHIV infected children are oversensitized to pain as a

result of neuronal damage during developmentAnimal data suggests that exposure to noxious stimuli

during development of the nociceptive neuronal circuitry (the pain pathway) results in a permanent rewiring of the central nervous system.

WHAT CAUSES PAIN IN CHILDREN WITH HIV?HIV itself: peripheral neuropathy, cardiomyopathy,

myositis, arthritis, osteonecrosis of the hipGp 120: activates NMDA receptors on excitatory

neuronsHIV infected children are oversensitized to pain as a

result of neuronal damage during developmentAnimal data suggests that exposure to noxious stimuli

during development of the nociceptive neuronal circuitry (the pain pathway) results in a permanent rewiring of the central nervous system.

WHAT CAUSES PAIN IN CHILDREN WITH HIV?

Secondary and opportunistic infections: Lymphadenitis Parotitis Oral and oesophageal thrush Apthous ulcers Pneumonia Osteomyelitis, Meningitis: bacterial, tuberculous MAC infection, Herpes Zoster Sinusitis UTI Impetigo

What causes pain in children with HIV?Repeated painful proceduresHIV monitoring, diagnostic (esp LP), thoracocentesisChildren do not get used to repeated painful

proceduresPain may worsen with each procedure as a result of

considerable anticipatory anxiety from previous experiences

Untreated pain esp in the newborn period causes lifelong decrease in pain threshold through neural rewiring

Pain is more than just remembered, it changes the brain!

What causes pain in children with HIV?Toxcicities and adverse drug reactions:

d4T and EFZTB RX: INH: peripheral neuropathyChemotherapy: vincristine, cispaltin, etoposide,

inteferon

WHAT CAUSES PAIN IN CHILDREN WITH HIV?

NON-AIDS conditions:Same things that cause pain in children

without HIVDental diseaseMigraine tension headachesTrauma

WHAT CAUSES PAIN IN CHILDREN WITH HIV?

Psychosocial stressors – emotional painLiving with a chronic illnessPovertyLong period of hospitalisation, maternal

separationPotential or actual loss of a parentClinical depressionBeing teasedNormal anxieties of childhood

Untreated acute pain is responsible for considerable morbidity and even mortality

HIV infected children with pain were 5X more likely to die than patients without

Untreated acute pain can lead to chronic painUntreated acute pain can reset the pain

threshold for the rest of the child’s life

Copyright ©2002 American Academy of Pediatrics

Gaughan, D. M. et al. Pediatrics 2002;109:1144-1152

Fig 1. Survival probability curves by (A) baseline pain and (B) baseline pain and CD4 percentage

The story of Jeffrey Lawson: Born 198526 week prem weighing 750 gmsHad a PDA ligation at 2 weeks of lifeProcedure (included a mini thoracotomy)

lasted 1 ½ hoursFollowing the surgery… things started going

wrongNumerous electrolyte disturbances and other

signs of catabolismDeveloped an intra-ventricular haemorrage

and died a few weeks post op

Mom decided to investigateDiscovered the PDA ligation had been done

under pancuronium onlyThe anaesthetist had not given any analgesia

as she thought he was too sick (BP too low)During surgery BP and pulse increased

considerably

Catabolic reactions noted post op were secondary to stress response from pain

Untreated pain had been the trigger leading to his death

Public outcry when her letters were published in the Washington Post

1 year later AAP issued a policy statement recommending analgesia in newborns including prems

Recent studies suggest HIV infected children may be more sensitive to morphine than non-HIV infected children and that increases should be made more gradually

Sometimes accept side effects if they are less severe than the pain and especially if tolerance will develop in a few days (esp: drowsiness, nausea and vomiting)

Treat side effects with anti-emetics, laxatives if necessary

Palliative care developed out of the modern hospice movement

Dame Cecily Saunders started the first adult hospice St Christopher’s Hospice in London in 1967

First Children’s Hospice was opened in Oxford (UK) in 1982

First hospital based PPC service started at St Mary’s Hospital in New York in 1985,GOSH followed in 1986

Palliative Medicine became recognised as a full medical speciality in the UK in 1989 under the Royal College of Physicians

Rapidly developing speciality in paediatrics worldwide

2 academic chairs in PPC in GermanyRecently created chair in GOSH (UK)Increasing evidence base in paediatric pain

and its managementStill need increasing evidence base for drugs

used to treat non pain syndromes (many still used off code esp in children < 2 years)

TOWARDS EXCELLENCE WITHOUT APOLOGY!!!

ANY QUESTIONS?