Integrating the Child with Chronic Illness into General Pediatric Nursing Practice

8/14/2019 Integrating the Child with Chronic Illness into General Pediatric Nursing Practice

1/28

Integrating the Child with Chronic Illness into General

Pediatric Nursing Practice:

The Model of Childhood Cancer Survivorship

Holly DeLuca, MSN, PNP-BC, CPON

Debbie Lafond, MS, PNP-BC, CPON

Center for Cancer and Blood Disorders

Childrens National Medical CenterJune 2009

Objectives

1. Discuss landmarks in pediatric oncologyand identify demographics of childhoodcancer survivors today

2. Define late effects of childhood cancertherapy

3. Discuss the etiology of long term effects

of childhood cancer treatment andassociated risk factors


2/28

Objectives Continued

4. Recognize challenges faced by childhood cancer

survivors and survivors of other chronic and/or life

threatening childhood illnesses today including:

medical, educational, social/emotional, legal and

financial

5. Identify components of a long term follow-up program

and the importance of the pediatric nurse as a key

member of the survivorship team.

6. Discuss the role of the pediatric nurse in helping this

unique population of patients integrate the chronic /late effects of illness and treatment to live productive

and meaningful lives

Landmarks in Pediatric Oncology

1970s- Recognition that curewas possible

- Proliferation ofrandomized clinical

trials

- Effective multi-modality

protocols


3/28

Landmarks: continued

1980s- Tailoring therapy to riskfactors

- Defining late effects

- Reducing radiationdose

- Substituting effectivedrugs for radiation

Landmarks Continued

1990s

- Understanding of dose related effects

- Initiating efforts to track and educate

survivors

2000s

- Surveillance for late effects based on risk

- Interventions to reduce late effects- Transition to adult health care


4/28

Childhood Cancer Survivors Now

Over 270,000 childhood cancer survivors livingin the United States

1 in 1000 is a childhood cancer survivor

1 in 570 is a childhood cancer survivor in thoseage 20-34 years

What does survivorship mean?

5 year survival rate isapproaching 80%

2/3 will experience atleast 1 late effect

1/3 will experience asevere or life threatening

late effect


5/28

Late Effects of

Childhood Cancer Treatment

Definition persistent and

adverse changes thatare directly related todisease process,treatment process orboth

Disease

Location of tumor

Treatment Surgery

Chemotherapy

Radiation

Late Effects: Causes


6/28

Late Effects: continued

Risk Factors

Age and developmental stage

Gender

Co-morbidities

Family history and/or geneticpredispositions

Location of tumor

Types and dose ofchemotherapy agents

Site, dose and type of radiation

Types of Late Effects

Physical

Endocrine

Neurocognitive

Second malignancies

Psychosocial

Emotional


7/28

Physical Late Effects

Head and Neck

Central Nervous System

Pulmonary

Cardiovascular

Gastrointestinal

Genitourinary

Musculoskeletal

Renal

Integumentary

Hematopoetic

Head and Neck

Why this area affected? Sensitive structures

Common site for soft

tissue tumors

Development during

childhood is essential to

maximum health in

adulthood

Common Late Effects:

1. Hypoplasia of bone

- > 30 GyRadiation

2. Skin necrosis/ulceration

- > 70 GyRadiation

3. Growth disturbances

- Radiation & Chemo

4. Xerostomia

- > 30 Gy Radiation

5. Sensorineural hearing loss

- Radiation & Chemo


8/28

Ocular Late Effects

Hyperkeratosis

Chronic injection ofconjunctiva and sclera

Cataracts

Glaucoma with prolongedsteroid use

Retinopathy

Deformities in orbital bone

growth

Effects on Hearing

Conductive hearing loss

Sensorineural hearingloss

Chronic otitis externa

Stenosis or necrosis ofear canal

Excess cerumen


9/28

Dental Late Effects

Delayed dentition

Missing orunderdeveloped

permanent teeth

Increased Caries

Blunting, thinning andshortening of roots

Hypocalcification

CNS Late Effects

Age at diagnosis andduring treatment is an

important factor

Paralysis

Neuropathies

Seizures

Neurocognitive problems

Shunt function


10/28

Pulmonary Late Effects

Obstructive, restrictive,interstitial or

combination lung disease

Pneumonitis

Pulmonary Fibrosis

Frequent pneumonias

Non-cardiogenicpulmonary edema

Cardiovascular Late Effects

Coronary artery disease

Pericarditis

Cardiomyopathy

CHF

Valve damage

Arrhythmias


11/28

Gastrointestinal Late Effects

Hepatitis/Hepaticdysfunction/Cirrhosis

Feeding disorders

Chronic GVHD

Esophageal strictures

Esophogitis/GERD

Adhesions

Enteritis

Genitourinary Late Effects

Renal function

Urethral strictures orfibrosis

Bladder and/or boweldysfunction

Vaginal fibrosis,malformation, fistulas

Testicular/Prostatedysfunction


12/28

Musculoskeletal Late Effects

Hypoplasia

Leg length discrepencies

Prosthetic devices

Scoliosis

Amputation

Rhabdyomyolisis

Slipped capitofemoralepiphysis

Musculoskeletal Effects:

continued

Exostosis

Osteonecrosis

Fibrosis/Contractures

Bone density changes

-osteopenia / osteoporosis


13/28

Integumentary Late Effects

Pigment changes

Alopecia

Melanoma/Skin cancers

Skin necrosis

Telangiectasia

spidery blood vessels beneaththe skin surface

Fibrosis/contractures/scarring/ striae

Acceleration of skin aging

Hematopoetic Late Effects

Immune reconstitution

Re-immunization

Chronic anemia

Chronicthrombocytopenia

Eosinophilia

Especially after stem cell

transplant


14/28

Endocrine Late Effects

Thyroid

Growth

Fertility

Gonadal

Pituitary

Adrenal insufficiency

Diabetes

Osteopenia

Hypothalamic obesity

Thyroid Late Effects

Hypothyroidism

Hyperthyroidism

Thyroid nodules

Silent thyroiditis


15/28

Effects on Growth

Growth hormone

deficiency

Microcephaly

Catch up growth aftercompletion of therapy

Effects on Gonads and Fertility

Precocious or delayedpuberty

Ovarian dysfunction

Primary

Secondary

Egg harvest or donation

Testicular dysfunction

Primary Secondary

Sperm banking or donors


16/28

Adrenal Insufficiency & Diabetes

ACTH deficiency

SIADH/Salt wasting

syndrome

Diabetes mellitus

Diabetes insipidis

Osteopenia

May be

asypmtomatic

Risk of fractures

Bone density

screening

Calcium and Vitamin

D supplements Exercise with impact


17/28

Hypothalamic Obesity

Unrelenting weight gainthat does not respond to

diet or exercise

modifications

Risk greater for braintumor survivors

Age < 6 years at dx

Radiation

Tumor location

Risk greater with presenceof other endocrinopathies

Neurocognitive Late Effects

Learning disabilities

Loss of IQ points

Developmental delay

Attention deficit

Behavioral abnormalities

Fine and gross motorcoordination difficulties

Decrease in processingspeed

Memory loss

Leukoencephalopathy


18/28

Second Malignancies

Thyroid carcinoma

Leukemia or MDS

Bone tumors

Meningiomas

Brain tumors

Skin cancers

Breast cancer

Psychosocial/Emotional Effects

Post traumatic stress

Quality of life

Social functioning

Functional impact

Depression

Anxiety

Fatigue Sleep disorders

Body image


19/28

Challenges for Survivors

Medical

Educational

Social/Emotional

Employment

Legal

Financial

Medical Challenges

Survivor of childhooddisease

Integration into adult healthcare

Health insurance

Americans with Disabilities

Act

Comprehensive Omnibus

Budget Reconciliation Act SSI

HIPPA


20/28

Educational Challenges

Right to education

Individuals with DisabilitiesAct

Americans with Disabilities

Act

IEP & 504 plans

Cognitive remediation

Neurocognitive testing

Need to readjust personalgoals

Social/Emotional Challenges

High risk behaviors

-smoking

-alcohol abuse

-drug use

-sexual promiscuity

Difficulty finding peergroup

Intimacy

Anxiety

Depression

Sleep disturbance


21/28

Challenges with Employment

Physical disabilities

Psychosocial disabilities

Neurocognitive issues

Insurability

Chronic fatigue

Maintaining employment

Job discrimination

Inability to serve in military

(some cases)

Financial Challenges

Income

Lower paying job due todisabilities

Job discrimination leading to

lower salary and promotions

Medical bills

Debt from treatment

Costs incurred from medicalcare for late effects and

follow-up


22/28

Care of Survivors

Challenges

Lack of knowledge ofprimary care providers

Long term follow-up

programs

Pediatric Cancer Centers

Adult cancer centers

Role of Pediatric Nurse

Program coordination

Screening

-History and phsycial exam

-COG long-term follow-upguidelines

Educating Survivors

-treatment summaries

-recommendations forlong-term follow-up


23/28

Other Childhood Illnesses

Cystic Fibrosis

Sickle Cell disease

Hemophilia

Congenital CardiacDefects

Others

Models of Integration

Empowering patients

and families

Integration of

chronic/late effects

Advocacy

Transition to adult

care


24/28

Case Study

Daniel is now a 13 year old survivor of a

Medulloblastoma, a type of childhood brain tumor.

He was diagnosed at the age of 4 years.

Prior to diagnosis, he was an active child and had

exceeded normal developmental milestones. He

was attending preschool and enjoyed spending

time with his siblings who were 6 years and 9 years

at the time he was diagnosed.

Daniel underwent a gross total resection of his

tumor and there was no evidence of metastatic

disease. He required a VP shunt for obstructive

hydrocephalus.

He recovered well post-operatively with mildcerebellar mutism syndrome, characterized by

mild ataxia and fine/gross motor abormalities and

halting speech.

These deficits were recovering but he required

several subsequent shunt revisions that left him

more permanently impaired.


25/28

He was enrolled on a COG clinical trial and

received craniospinal radiation therapy (2400 cGy)

with adjuvant chemotherapy including Cisplatin,

CCNU and Vincristine. Therapy was completed in

18 months.

Daniel received intensive rehabilitation servicesincluding PT, OT, and speech therapy and did

have some improvements.

Daniel is now a 9 year cancer survivor.

He was unable to attend school during therapy but

did have a home tutor and was able to complete

elementary with an IEP and 504 plan in place. He

is currently in a 6th grade special education class.

Late effect of therapy include:

Cerebellar mutism syndrome

Severe ataxia requiring wheelchair for mobility Sensorineural hearing loss requiring bilateral hearing aides

Hypothyroidism

Adrenal insufficiency

Growth hormone deficiency


26/28

Late effects: continued

Tanner Stage I delayed puberty

Esophageal strictures requiring gastrostomy tube

Pulmonary fibrosis

Central sleep apnea requiring tracheostomy and ventilatorysupport at night

Hypertension related to renal tubular acidosis

Obesity related to hypothyroidism

Chronic fatigue syndrome

Integrating Daniel into your pediatric

practice

What issues/challenges do you foresee forthis patient and family?

What screening needs to be done on anannual basis (at a minimum)?

What resources might you suggest for thisfamily?


27/28

Challenges

Where to receive LTFU

Multiple medical specialties needed

Financial impact on family

Impact on siblings

Psychosocial issues

Neurocognitive issues

School re-entry issues

High school diploma track appropriate?

Vocational track?

Thank you!

Questions?


28/28

Contact info

[email protected] Holly DeLuca

[email protected] Debbie Lafond

Documents

Integrating the Child with Chronic Illness into General Pediatric Nursing Practice