Taking the long-term view in nursing a child with Down Syndrome Presented by Lauren Rees (RN)

2 April 2012

www.childnursepracticedevelopment.org.za

Learning objectives

• To know what discharge planning is.

• To understand why discharge planning is important.

• To understand the importance of long-term nursing care planning in a child with Down Syndrome.

•Lulama – second born child of Zukiswa

•Born: 1 March 2009 (now 3 yrs old)

•Current weight:11 kg

•Birth weight: 2,92 kg at term

•Immunisations up to date

•Admission : 2 February 2012

Context

All names changed to protect patient confidentiality

Diagnosis

• Down Syndrome

• Atrioventricular septal defect with

Eisenmenger Syndrome

• Microcytic anaemia

Genogram

42 30

9 months 3

25

× 25

3 15

62

= people who support Zukiswa

X X X

X

X

X

X

X X

.

Pathophysiology • Down Syndrome is a chromosomal condition

caused by an extra 21st chromosome (all or part)

• Complete atrioventricular septal defect is a defect in the inferior part of the atrial septum and a ventricular defect beneath the AV valve.

– Shunting of blood occurs leading to mixing of blood and potential for pulmonary hypertension

• Eisenmenger Syndrome occurs when a left-to-right shunt leads to pulmonary hypertension and a subsequent right-to-left shunt.

Assessment

• Down Syndrome phenotype

• Global developmental delay

• Central cyanosis with saturations below 90% on nasal prong oxygen

• Clubbing

• Pallor

• Cardiomegaly with epigastric pulsation

• No murmur but loud P2

• Mild tachycardia

Course of admission • Feb 2012 - Cardiac investigations-echocardiogram,

ECG and bloods

• Cardiac catheterisation delayed due to RSV pneumonia

and rhinovirus A cultured on nasopharyngeal aspirate

• 7 March- Cardiac catheterisation. Cardiac lesion found to

be inoperable. Prognosis poor.

• 23 March – Planned transfer to Cecilia Makiwane

Hospital, but did not occur due to transport difficulties

• 24 March - Acute gastroenteritis developed

• 30 March – Transfer to Cecilia Makiwane by air

Nursing care-1 • Facilitate mother-child interaction

• Devoted mother. Very good bonding with Lulama

• Zuki needs financial support

• Zuki identified need to see a social worker

• Link Zuki to a Down Syndrome support group in the Eastern Cape

• Physical, social and emotional support required for needs of caring for a child with Down Syndrome but also for end of life care

• Manage pain and provide comfort

• Child appeared comfortable generally.

• Use FLACC scale.

• Kept trying to pull off sats probe. Use intermittently.

• Mother at bedside and held child when distressed. Support nursing child in a comfortable position for her.

• Nasal prong oxygen did not bother child but check position

• End of life care will require expert pain management

Nursing care-2

• Promote good nutrition • Promote current full ward diet with supplementary full cream milk feeds • Monitor intake and output • Describe stools • Weigh Lulama daily • Provide micronutrient supplementation as prescribed • Support optimal nutrition at home

• Ensure hydration • Current ward diet and full cream milk supplementation 150ml x 4 calculated

to meet Lulama’s maintenance requirements • Check hydration daily • During acute gastroenteritis, administer oral rehydration solution after

each loose stool as prescribed • More frequent hydration checks required • Administer anti-failure medication as prescribed • Describe urine • Urinalysis on admission and during acute gastroenteritis as indicated • Ensure that mother knows how to prepare oral rehydrate at home

in a sterile manner

Nursing care-3

• Reduce microbial load

• Wash hands

• Nasal prong oxygen tubing kept clean

• Infection control measures when RSV pneumonia

• Provide additional immunisations

• Educate mother about limiting contact with other children when ill

• Encourage discharge as susceptible to nosocomial infections

• Maintain skin and mucosal integrity

• Daily bath

• Moisturise skin

• Care of nostrils to prevent pressures sores

• Nappy care

• Mouth care

• Promote exercise as tolerated

Nursing care-4

• Support regulatory system

⁻ Observe, report and record temperature, pulse and respirations 6 hourly

⁻ Observe, report and record blood pressure daily

⁻ Promote sleep and encourage daytime sleep as well

• Promote development

⁻ Provide stimulation for the child

⁻ Encourage involvement of the multi-disciplinary team

⁻ Educate Zuki concerning stimulation and exercises to provide at home

⁻ Assist Zuki to access support services

Thank you