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Paediatric Cancers

Wendy

Saegenschnitter

Objectives

Children’s Cancer

Incidence and survival

Organisation of service

Treatment

Oncology Emergencies & complications

Cancer develops from one cell that has damaged DNA

http://people.bath.ac.uk/pr1cemb/Tumour.htm

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Rare (only about 1% of all cancer) but Diagnoses are very different from those in adults

Cure rates have improved to a remarkable extent

Remains an important cause of death in childhood

Successful treatment contributes a very significant

number of ‘life years gained’

Treatment conveys important future health and personal risks to many survivors

Childhood cancer has provided important insights into cancer biology and aetiology

Cancer incidence in relation to some other important childhood conditions

Cystic Fibrosis 1:2500

Down’s Syndrome 1:1000

Cancer 1:600

Diabetes 1:500

Distribution of childhood malignancies

brain

tumours

25% leukaemia

36%

bone

5%

sarcomas

6% Embryonal

tumours

16%

lymphoma

12%

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Cases per year

STATISTICAL DATA cases per year

INCIDENCE RATES OF ACUTE LEUKAEMIA

1996-2005

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Survival rates 2006 - 2008

Organisation of care for South West

Bristol the designated Principle Treatment Centre for Region

– Diagnosis, treatment plan by consultant & MDT, specialist services i.e. radiotherapy, surgery, BMT, PICU, Neuro

– Teams (Oncology, NeuroOnc, Haematology, BMT, Non Malignant)

– Bones, Retinoblastoma, Liver cancers; link with Birmingham specialist services

Paediatric Oncology Shared Care Unites (POSCU’s) – Truro, Bath, Barnstaple, Gloucester, Exeter, Plymouth, Taunton, Yeovil, (Swindon)

Each POSCU has different level of services

– All about working together – Safe care as close to home as possible

BMT has larger referral catchment e.g. South Wales, Southampton, Cambridge, Oxford

Teenagers & Young Adults with Cancer (TYA)

– 16–18yrs must be given choice between Paediatric/Adult care

– Ward 35 and Area 61

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Patient Pathway

Presentation

Diagnosis

Treatment

Follow-up

– Long term follow-up

– Relapse…

– Palliative Care

– End of Life

Treatment of Cancer

Depends of type of cancer

– May require parallel planning while having active treatment i.e.

wishes discussions with family while pursuing curative

treatment

Most children are on evidence based clinical trials run by

international/national groups

Single or combination therapy with

– Surgery

– Chemotherapy

– Radiotherapy

– Other e.g. immune therapy

Treatment

Surgery – If early stage & can remove or after chemo has shrunk tumour

Chemotherapy – acts on cells when actively in cell cycle (process to replication)

– Cannot discriminate between normal cells and cancer cells

– Effect rapidly dividing cells (Bone marrow, Gastro-intestinal Tract, Hair)

– Key side effects – anaemia, thrombocytopenia, neutropenia, mucositis

– Other organs can also be effected

Radiotherapy – Never first choice in young children due to side effects

Growing bones

Neurological development of child less than 3yrs

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Stages of treatment

1) Induction 2)

Consolidation 3) Interim

maintenance 4) Delayed

Intensification 5)

Maintenance

R1 R2

Oncology Emergencies

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Cancer can develop in any area of the body

May be a mass or lump (single or multiple) – in the case of leukaemia systemic

Symptoms or complications at diagnosis can be related to location of tumour or metobolic changes e.g. Wilm’s (kidney tumour) often associated with malignant hypertension

Lets think of other complications…..

Oncology Emergencies

Complications are related to

– Underlying disease process

Rapid tumour growth

Site of tumour

Bone Marrow Failure

Clotting disorders

Electrolyte imbalance

– Treatment

Chemotherapy/drugs

Radiotherapy

Surgery

Stem Cell Transplant complications

Complications at presentation & Diagnosis of childhood cancers

Tumour Lysis Syndrome

High white cell count (leucocytosis)

Superior vena cava obstruction

Disseminated Intra-vascular Coagulation

Raised ICP

Infection/Sepsis

Electrolyte disturbance

Hypertension

Bleeding

Spinal cord compression

And many others

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Complications of treatment

Rapidly dividing cells most effected i.e.

– Bone Marrow; low red cells, white cells and platelets

– Mucosal lining of GI tract; mucositis

– Hair; Alopecia

Impact on other systems depend on individual

drug profile

– Nausea and Vomiting

– Constipation, loss of appetite, Haemorrhagic

cystitis, infertility, loss of hearing

– Renal, cardiac, neurological, liver

Tumour Lysis Syndrome

A metabolic complication

Due to a rapid release of intracellular metabolites

– uric acid,

– potassium,

– phosphate

From the distruction (lysis) of malignant cells

– Initiation of treatment (chemotherapy)

– Large tumour volume which is rapidly dividing

In quantities that exceed the excretory capacity of the kidneys

(Gibson & Soanes 2008)

Tumour Lysis Syndrome

The metabolic abnormalities are

– hyperuricaemia,

– hyperkalaemia,

– hyperphosphataemia

– with resultant hypocalcaemia

May be in isolation or combination

Usually seen within 12-72 hours from start of treatment

Signs & symptoms are directly related to metabolic changes eg hyperuricaemia – crystals block kidneys -renal failure – life threatening

Most common in ALL, NHL (high white count), but can occur in other tumours

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Tumour Lysis Syndrome

Prevention is key principle of care – Identify those at risk

– Hyperhydration before start of treatment 2 – 2.5L/m2/day 21kg = 0.82 x 2.5 = 2050ml/day = 85ml/hr

– Allopurinol or Rasburicase drug therapy

– Monitor closely Electrolytes (6 hourly bloods)

Renal function (urine output and Urea/Creatinine)

fluid balance and weight, may need frusemide to prevent pulmonary oedema

– Leukopheresis in very high WCC

– Haemodialysis if necessary (high K, Phosphate, anuria)

– Start treatment but with caution

Tumour Lysis Syndrome

Can lead to:

– Acute renal failure

– Multi organ failure

– Pulmonary oedema

– Cardiac arrhythmias

– Seizure

– Death

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Senario Day 1

4 year old boy presented to local ED

Short of breath, abdo pain, vomiting (coffee ground)

On examination: clear chest, tachycardia, sunken eyes

1/12 history of abdo pain, tonsillitis (treated by GP with

antibiotics)

2/52 history vomiting, fever, decreased oral intake,

weight loss (approx 2kg in 3 weeks)

Bloods; Hb 44, plts 17, WBC 120, Cr 470, Urea 30

– What are our concerns?

Day 1

Transferred to PICU for haemofiltration as in

renal failure

– Short term dialysis CVC in R groin

Day 2

? Leukaemia

Further tests to diagnose

AML m5

Potassium and phosphate are normal so not

felt to be tumour lysis,

? Disease infiltrates in kidney or obstruction

But due to high cell count as risk of tumour

lysis

– What is our care?

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Day 3

Ongoing haemofiltration

Start chemo

– Reduce one drug due to renal toxicity

– Other drugs at normal dose

Day 4

Post day 2 chemotherapy

– Moved to Oncology Ward

– BD haemodialysis on Renal Ward

Day 5

HB 82, Plts 35, WBC 13

– Shows response to treatment

PO4 3.95, Calcium 1.62, Potassium 5.2

Hold Day 5 chemotherapy

– Concern in tumour lysis

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Day 6

Review post dialysis

– PO4 3.48 pre dialysis, post 2.37

– Ca 1.75 pre, post 2.11

– K 5.2 pre, post 2.11

Restarted chemotherapy between dialysis

Monitor bloods 4 hourly

Hourly urine measure

Strict fluid balance, no hydration

Sepsis and Cancer

Why are our patients vulnerable?

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Why are our patients vulnerable?

Compromised immune system

– Due to disease

– And/or treatment

Factors leading to vulnerability

Immune suppression due to

– Disease impact on bone marrow

– Drugs (chemo, steroids, ciclosporin, monoclonal

antibodies)

– Radiotherapy

– NEUTROPENIA IS CRITICAL FACTOR

Other factors to consider

Degree of neutropenia

Length of neutopenic phase

Age (very young and very old)

Central line (number of lumens)

Mucositis

Nutritional status

Environmental risk factors

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Why are neutrophils so important?

Campbell, Biology 4th Ed. (1996)

Blood cell lineage and differentiation

Lines of Defence

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Phagocytes

Neutropenic Fever

Neutrophils < 0.5

Temperature > 38°c

No other obvious symptoms

No Paracetamol prior to review

No Brufen

Child needs to be seen and commenced

treatment within 30 mins.

Why?

Signs and Symptoms of Infection

What are signs and symptoms of infection?

– Sore throat, diarrhoea, burning when passing urine,

redness or swelling around CVC exit site,

– just not feeling well or

In a neutropenic child, THERE MAY BE NO

CLEAR FOCUS OF INFECTION

WHY IS THIS?

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Neutropenic Sepsis: Prevention & Management of Neutropenic Sepsis in Cancer Patients. NICE Guidelines 2012

Treat neutropenic sepsis as an acute medical

emergency

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Septic Shock

A systemic response to pathogenic micro-organisms in

the blood

Bacterial toxins trigger haemodynamic & metabolic

changes

Septic syndrome can lead to poor cardiac output, poor

tissue oxygenation, renal failure.

If allowed to progress multi organ failure and death

Patients with fever and neutropenia should be treated

as bacterial sepsis until proven otherwise.

Very small window of opportunity to treat

Signs and Symptoms in Neutropenic Sepsis

Temperature may be the only early indication

If the child is taking steroids, even this can be

absent.

If the child is unwell, sepsis must be

considered and treatment started.

Clinical features

Temperature

Tachycardia

Tachypnea

Rigors

Or just not right.

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Full set of observations (Temp, Pulse, BP,

Sats, Capillary refill)

Blood culture, FBC, U&E’s, LFT’s

Give IV antibiotics (dependent on allergy)

Piptazobactim

Then additional septic screen as appropriate

(Chest Xray, urine, stool, CVC swab etc)

Neutropenic Sepsis

Sam is 18 months old with Neuroblastoma

10 days after chemotherapy

Presented with temperature of 38.8 deg C

How should Sam be cared for?

Antibiotics are commenced

Bloods results are:

Hb 7.5g/L, Plts 15, WBC 0.1, Neut 0.1

How should we care for Sam?

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24 hours later

Sam is distressed, crying

You review him……….ABCDE

Sam starts an NCA with morphine

48 hours later Mum is not happy, Sam is still

distress now complaining of painful abdomen,

has not had bowels open for several days.

You assess him……..ABCDE

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Surgeons review with oncologist

Tiphlitis

Now nil by mouth

Start TPN

NCA background increased

Now on night shift

You review Sam, he is much less distressed

ABCDE

Sam is given a fluid bolus……What do we give?

We start ½ hourly observations

Respiratory rate stays the same & he is still febrile

Pulse 130 bpm

BP 90/50

Cap refill - 2 sec

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3 hours later…

Respiratory rate 50 bpm

Pulse 170 bpm

BP 70/30

Cap refill 4 sec

Sam is pale and motley

Parents say he is very sleepy and not interacting like he normally does

How should Sam be cared for?

Sam is moved to the HD unit.

2 hours later Sam’s condition deteriorates:

A: he is groaning

B: increased work of breathing with recession, rate 54, sats 92% in air

C: increasing tachycardia, 180 and you are unable to obtain a cuff BP. Capillary refill is 4 seconds.

D: groaning to painful stimuli

What immediate treatment does Sam need?

Sam is intubated, ventilated and receiving

inotropic support.

What specific problems and issues does Sam

pose to the ITU team?

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Senario

Patient presented to ED

Facial swelling

Thought to be a drug reaction

Further investigation, Chest Xray

Large mediastinal mass

Superior Vena Cava Obstruction

Most often ALL, NHL, tumour causes mediasinal mass leading to possible blockage;

– Puffy face, distended veins, headache, pain, decreased LOC, death

Rapid assessment of tumour (imaging) & monitor

Strict fluid monitoring

At risk of Tumour Lysis Syndrome

May not be able to put in a CVC

Don’t lie flat

Avoid upper limb cannulation

Start treatment including steroids

Spinal Cord Compression

Can be caused by a range of tumours

Either at diagnosis or relapse

Risk is due to proximity of tumour to spinal cord. – Further deterioration can be caused by oedema and obstruction of vascular drainage

Critical signs are – back pain,

– tingling in extremities, – sensory changes at level of tumour,

– sensory loss, motor loss, reflex loss,

– bladder or bowel dysfunction

Urgent intervention and support critical to reduce further loss or deterioration – Surgery

– Steroids

– Radiotherapy

Nursing care related to monitoring, stabilizing spine, support Depending on level of tumour will depend on risk

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Disseminated Intravascular Coagulation (DIC)

A cluster of symptoms not a disease

Secondary to malignancy (APML) or infection or drug/transfusion reaction

Trigger of coagulation process

Leads to consumption of platelets and clotting factors

Micro clots (ischaemia) and haemorrhage

Life threatening complications

Management of DIC

Treat underlying cause

Supportive therapy

– Manage coagulopathy

– broad spectrum antibiotics

– Red cell transfusion as required

Neurological complications

Wide range of potential challenges – Fitting, reduced level of consciousness, confusion, agitation, loss of

function, death

Related to – Underlying disease

Brain tumours causing raised ICP

Metabolic

– Treatment and its complications Drugs i.e. steroids, opiodes, cyclosporin, methotrexate, ifosfamide

Infection; bacterial, viral, fungal, shunt related

Bleeding

Stroke related to clotting

etc

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Mucositis

A frequent complication of chemotherapy or

radiation

Death of mucosal cells throughout GIT

– Pain (PCA)

– Nutrition (PN)

– Bleeding (transfusion)

– Sepsis

High dose therapy give prophylactic antifungal, septrin

(PCP), antiviral but not antibiotic

Febrile neutropenia guideline (currently undergoing review)

– Will resolve when count recovered

Stem Cell Transplant (SCT) complications

Mucositis

Graft vs Host Disease

– Skin, gut, liver

Pulmonary Hypertension (Hurlers)

Veno Occlusive Disease (VOD)

Haemorrhagic cystitis

– Related to type of chemo and polyomer virus

Infection

– Short term and long term immune difficiency

– Viral, fungal, bacterial infections

– Pneumocystis carinii, adenovirus