Case Presentation Webinar

Care of a long stay

critically ill child

Heide Kunzmann 4 February 2013

Presented in collaboration with

the Child Nurse Practice

Development Initiative

Introduction

Who is this child? • Name: Jacob (Name changed for confidentiality)

• DOB: 21\01\2011

• Age: 2 years

• Weight: 11.8kg

• Birth weight: 2.8kg

• Admission date:15\01\2013

• Admission reason: Acute flacid paralysis

Genogram

23yr 20yr

Jacob

2011

56yr 56yr

17yr 16yr

PURPLE blocks denote those

people that live together

Some background

• This child lives in a family where his maternal great

grandfather and great grandmother are the

breadwinners.

• His own father does not work, neither does his mom.

They both have grade 10.

• His two uncles dropped out of school and have no

income.

• They stay in a brick home, open plan with an enclosed

bathroom (running water, electricity and a toilet in the

house).

• This house is on the outskirts of George.

The story of Jacob…

• Born at term, NVD, no complications

• A well child till March 2012

March 2012

• He presented with a 8 day history of weakness in his legs

• He had a squint and had ophtalmoplegia

• Initially thought to be TBM because he had a positive TB contact (father)

• CT brain normal

• Deteriorated and required intubation and ventilation on 13\3\2012 in George hospital

• Transferred to Red Cross War Memorial Children’s Hospital (RCWMCH)

• Guillain-Barre Syndrome (GBS) was diagnosed and sucsessfully treated

• Stayed till 11\5\2012 at RCWMCH, then transferred back to George

• Well rehabilitated, walked in August 2012

Growth Chart

• Born on the 10th.

• Grew along the 25th

centile.

• Noted to track above

the 25th centile

during first admission

to hospital.

GBS pathophysiology

Background

• Identified in 2 french soldiers by physicians Guillain and

Barre in 1916.

• Initially thought to be a single disorder, but today there

are several variants.

• Guillian-Barre syndrome is a collection of clinical

symptoms of weakness and diminished reflexes.

• In countries where poliomelitis is under control, GBS is

the most important form of acute flassid paralysis [1]

GBS pathophysiology continued..

• Post infectious immune-mediated disease, usually

respiratory and gastrointestinal.

• During infection the body launches an immune response.

• Some of the antigens of the infectious agents capsules

are the same as those in the nerves.

• This leads to the bodies own immune response to cross

react with the gangliosides and glycolipids that are in the

myelin in the peripheral nervous system.

• This leads to immunologic damage to the peripheral

nervous system

• This leads to delay in conduction causing flaccid

paralysis

Disease pathophysiology

• Miller-Fisher syndrome is a variant of GBS

• 5% of all GBS

• Signs and symptoms include:

• Ataxia

• Areflexia

• Ophthalmoplegia -very important feature

• Limb weakness

• Ptosis

• Facial palsy

• Bulbar palsy

Prognosis

• Good recovery 1-3 months

• Peak weakness 10-14 days

• Average time on ventilator 50 days [1]

• Death in 2-12% [1]

commonly secondary to complications

related to GBS

• Respiratory distress

• Sepsis

• Thromboembolic disease, biggest cause is paralysis and

ventilation

• Persistent motor defect (foot drop, muscle wasting,

sensory ataxia) occurs in a significant percentage of

survivors

• Recurrence of GBS occurs in 2-5% of patients [2]

January 2013 Mom noticed something was not so

well with Jacob…

Jacob’s presentation at

George Hospital

8\01 10\01 12\01 13\01 11\01

• Head lag

• Drooling

• Poor

feeding

(mom

complained

about this,

only able to

eat some

jelly) • Dehydrated,

Hypovolemic

• Diplopia and

squinting\eyelids

normal

• Bulbar weakness,

pooling secretion

• Slight cough

• Flaccid arms,

reflexes

• Legs no

reflexes\power

3+, moving

against gravity

• Diaphragm

normal, but

bilateral LL

collapse

• Urine retention

• Investigations

done

Admitted to

George

hospital

Intubated

Referred to

RXH

How was the condition diagnosed

and treated? (George hospital)

• Investigations

• LP-proteine .21 (on admission)

• MRI\CT brain

• CXR done-not suggestive of PTB LL collapse

• Herpes\Enterovirus\Cocksackie

• LP-proteine now .43 (day 5)

• Assessments

• Clinical-differencial diagnosis (TBM,Herpes\bacterial

Meningitis)

• Medications

• Aciclovir

• TB treatment restarted

• Ceftriaxone

Current admission to RCWMCH…

• Came on a fixed wing plane

• At this point – strong suspicion of GBS

• Presentation at admission:

• Ophthalmoplegia - Paralysis of extraocular muscles reponsible for eye movements.

• Strabismus

• Bulbar weakness

• Dysphagia

• Drooling

• Diplopia

• Flaccid arms, reflexes

• Legs no reflexes\power 3+, moving against gravity

• Strong clinical evidence of Guillain-Barre syndrome (Miller

Fisher variant) due to the cranial nerve involvement.

15\01 17\01 25\01 27\01 18\01

• Polygam

• T\A, B\C

and urine

MC&S was

done

• Tracheostomy

• Aciclovir and

Cefotaxime

stopped

• All cultures

came back

negative

• Investigations

done for

mycoplasma,

cocksakie,

enterovirus

• Porphyria test

done

• MRI brain-

consistent

with GBS

• TB treatment

stopped

• Reinsertion of

urinary catheter

for urine

retention

• Picorna virus

came back

positive (Entero

virus)

• Augmentin

started due to

1000000 mixed

growth on urine

Jacob’s investigations and

treatment at RCWMCH

Looking for

cause of GBS

Nursing care 1

Facilitate mother-child interaction • Make sure that the mom understands what is

GBS. Have frequent contact sessions to ensure she understands.

• This will decrease her anxiety so that she can have a calming and reassuring influence on her child.

• Important to get the mom involved in caring for her child, this is going to be a long stay.

• Empowerment of the mom.

• As well as comfort to the child-making the child feel safe during an extreemly distressing time.

Nursing care 2

Non pharmacological Tx • Regular turnings and ROM

exercises, with proper positioning

to prevent shortening of muscles

• Distraction

• Multi-disciplinary team approach

• Eye patches to help with diplopia

• Tears to prevent dying out of eyes

• Full ventilation to comfort, do not

try to wean

Pharmacological Tx

• Morphine (stop after

tracheostomy done)

• Valeron (for stretches)

• Clonidine (taper down after a

week)

• Paracetamol

• Gabapentin (increase with

increments)

Manage pain and provide comfort • Patients with GBS describe the pain as severe and

distressing[1] - aching, throbbing and shock like pain,

particularly over the shoulders, buttocks and thighs,

Reason - immobility.

Nursing care 3

Promote good nutrition

• Ensure the dietician is consulted to work out the

calories needed.

• MVT, Folic and Zinc is added.

• Involve the speech therapist to promote

communication and establish safe swallowing.

Ensure hydration

• Monitor intake and output.

• Pay attention to ensenceable losses, due to

autonomic nerves system involvement they sweat a

lot.

Nursing care 4

Reduce microbial load • Implement the VAP bundle

• Adhere to basic infection control policies

Maintain skin and mucosal integrity • Due to diaphoresis (autonomic changes), ensure

you wash and dry them well.

• Let him lie on cotton.

• Dry his neck often due to the drooling.

• Regular turning due to paralysis-prevent pressure sores

• Ensure splints are applied properly

Nursing care 5

Support regulatory system

• Due to the autonomic nervous system

involvement, it is possible that the patient may

become bradycardic - ensure BP is adequate

and patient is saturating well.

• Urinary retention due to urinary sphincter

involvement - ensure bladder is not distended and

that patient is passing a good amount of urine –

Jacob failed 2 attempts to remove his catheter

• Bowel paresis and gastric dysmotility - ensure

patient does not become constipated (Sorbitol)

Nursing care 6

Promote development

• The groundwork for reading and writing is done

during the next year- reading to Jacob is important,

showing him the pictures.

• Math skills are learnt through solving problems like 8

piece puzzle sets or stacking rings - assist him in

playing these games.

• Creativeness is explored by shaking things to make

a sound, playing with clay - OT and the music man.

• Socialising by playing alongside another child - the

child in the bed space next to him also has GBS[3]

Reflecting back on the care of Jacob

• Essential to have a multi-disciplinary approach to a child diagnosed

with GBS.

• It takes a big team to care for this child (OT, speech therapist, physio,

dietician, music therapist, aromatherapist, nurses and doctors) and

the mom should be actively involved.

• Age appropriate care is important when doing daily activities.

• The biggest risk factors that can lead to complications are mechanical

ventilation and immobilisation.

• Pain control is important.

• Establishing ways of communication is very important.

• There is no way of caring for a child with GBS if you are not willing to

work in a team and actively involving the mother.

Questions?

References

1. Guillain-Barre Syndrome

http://emedicine.medscape.com/article/315632-

overview [Accessed 1st February 2013]

2. Das. A., Kalita, J. and Misra, U.K. 2004 Recurrent

Guillain Barre’ syndrome. Electromyography Clinical

Neurophysiology.; 44(2): 95-102

3. Child Development Tracker – Your two year old.

http://www.pbs.org/parents/childdevelopmenttracker/tw

o/ [Accessed 3rd February 2013]