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Course: Pediatric Nursing

Topic: Nursing Care of Child with Neuromuscular Disorders Part I

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Module Goals

Learners will be able to:

Describe the anatomy and physiology neuromuscular system in children.
Identify neuromuscular disorders common in children.
Identify risk factors associated with Guillain-Barre syndrome (GBS).
Identify common signs and symptoms of GBS.
List common diagnostic procedures for GBS.
Discuss the medical management of child with GBS.
Explain the role of the nurse in caring for children with GBS and nursing interventions commonly performed in the care of children.

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Neuromuscular System

Includes all the muscles in the body and the nerves serving them.
The nervous system provides the link between thoughts and actions by relaying messages from the brain to other parts of the body.
Nerves and muscles, work together as the neuromuscular system and make the body move.

Health Direct , 2021

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How does Neuromuscular System work?

Health Direct, 2021

Neurons that carry messages from the brain via the spinal cord to the muscles are called motor neurons.
Each motor neuron end sits very close to a muscle fiber called a neuromuscular junction.
Motor neurons release a chemical for muscle fibres which make muscles move.

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Common Neuromuscular Diseases in Children

Cerebral Palsy
Muscular Dystrophy
Multiple Sclerosis
Spinal Muscular Atrophy
Charcot Marie Tooth
Marfan’s Syndrome and Ehlers-Danlos Syndrome
Myelodysplasia, including Spina Bifida and Myelomeningocele
Arthrogryposis
Prader-Willi
Trisomy 21 (Down Syndrome)
Congenital Myopathy

Lee Health, 2022

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Critical Thinking Question

Guillain-Barré Syndrome occurs when the body's immune system attacks the myelin sheath on the nerves in the central nervous system.

True
False

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Guillain-Barre Syndrome (GBS)

A rare condition in which a individual’s immune system attacks the peripheral nerves
Called an autoimmune disease
Affects all age group but more common in adults and in males
Severe cases are rare, but can result in near-total paralysis

National Institute of Neurological Disorders and Stroke, 2021

WHO, 2016

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Causes

Exact cause of GBS is not known
Often preceded by a bacterial or viral infection
Infection with Campylobacter jejuni, which causes diarrhea, is one of the most common causes
Most cases usually start a few days or weeks following a respiratory or gastrointestinal viral infection
May also be triggered by vaccine administration or surgery
Increased incidence of GBS following infection with Zika virus

WHO, 2016

National Institute of Neurological Disorders and Stroke, 2021

CDC,2022

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Critical Thinking Question

Infection with which of the following is recognized as one of the most common risk factors for GBS? (Select all that apply)

Campylobacter jejuni
Escherichia coli
Toxoplasma gondii
Infection with zika virus

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Signs & Symptoms

Unexplained sensations often occur first

Tingling in the feet or hands, or pain (especially in children), often starting in the legs or back

Include weakness usually starting in the legs, and can spread to the arms and face
Difficulty or failure to walk
In 20%–30 % of people, chest muscles are affected, making it hard to breathe

National Institute of Neurological Disorders and Stroke, 2021

WHO, 2016

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Signs & Symptoms

In addition to muscle weakness, symptoms may include:

Difficulty with eye muscles and vision
Difficulty swallowing, speaking, or chewing
Pricking and needle sensations in hands and feet
Pain that can be severe, particularly at night
Coordination problems and unsteadiness
Abnormal heart beat/rate or blood pressure
Problems with digestion and/or bladder control

National Institute of Neurological Disorders and Stroke, 2021

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Critical Thinking Question

A nurse is assessing a child with Guillain Barre Syndrome (GBS). What are the sign and symptoms a nurse might see in the child?

Sudden weakness in legs
Diminished deep tendon reflexes
Tingling sensation in the legs
Absent deep tendon reflexes

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Diagnosis

Key diagnostic findings include:

Recent onset, within days or four weeks of symmetric weakness, usually starting in the legs
Abnormal sensations such as pain, numbness, and tingling in the feet that accompany or even occur before weakness
Absent or diminished deep tendon reflexes in weak limbs
Elevated cerebrospinal fluid protein without elevated cell count
Abnormal nerve conduction velocity findings, such as slow signal conduction
Sometimes, a recent viral infection or diarrhea

National Institute of Neurological Disorders and Stroke, 2021

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Critical Thinking Question

The child's lumbar puncture results are back. Which finding correlates with Guillain-Barré Syndrome?

high glucose with normal white blood cells
high protein with normal white blood cells
high protein with low white blood cells
low protein with high white blood cells

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Management and Care

No known cure for GBS
Hospitalization to monitor closely
Supportive care:

Monitoring of breathing, heartbeat and blood pressure
Monitor for complications (infection, blood clots)
Mechanical Ventilator to support breathing
GBS-related nerve damage can lead to difficulty handling secretions in the mouth and throat

National Institute of Neurological Disorders and Stroke, 2021

WHO, 2016

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Management and Care

Acute phase:

Typically treated with immunotherapy, such as plasma exchange to remove antibodies from the blood
Intravenous immunoglobulin therapy (IVIg)
Often beneficial when initiated 7 to 14 days after symptoms appear

In cases where muscle weakness persists after the acute phase of the illness:

May require rehabilitation services to strengthen their muscles and restore movement

National Institute of Neurological Disorders and Stroke, 2021

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Management

Rehabilitative Care:

Regain strength and resume activities of daily living.
Blood thinner injections to prevent dangerous blood clots in leg veins.
Inflatable cuffs may be placed around the legs to provide intermittent compression to prevent blood stagnation and sludging.

National Institute of Neurological Disorders and Stroke, 2021

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Nursing Management

Assessment:

Vital signs (Respiratory rate, quality of respirations, and decreasing vital capacity)
Respiratory assessment (shallow and irregular breathing, the use of accessory muscles, and difficulty in clearing secretions)
Assess for problems associated with immobility
Assess pain related related muscle changes
Skin, bowel function

Nguyen, Taylor, & Renwanz Boyle, 2021

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Nursing Management

Monitoring:

Respiratory impairment
Cardiac dysrhythmias
Weakness and paralysis in extremities
Changes in skin due to immobility
Fluid and electrolyte imbalance
Changes in bladder and bowel function
Pain
Psychological problems

Nguyen, Taylor, & Renwanz Boyle, 2021

Contact info: info@nursesinternational.org

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Nursing Management

Intervention:

Monitor vital signs
Perform passive ROM exercises at least twice daily
Position changes every two hours
Ensure the child is positioned on a foam, air, water, or gel support surface bed
Use of anti-embolism stockings and compression boots to prevent DVT
Maintenance of adequate hydration to decrease risk of pressure ulcers

Nguyen, Taylor, & Renwanz Boyle, 2021

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Nursing Management

Intervention:

Management of gastrostomy feeding
IV fluid administration, or the administration of parenteral nutrition to ensure necessary nutrients
Communication strategies such as eye blinks, use of pictures, or computer graphics
Pain management
Educate family members about GBS including the symptoms, disease progression, medical current treatments

Nguyen, Taylor, & Renwanz Boyle, 2021

Contact info: info@nursesinternational.org

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What Would the Nurse Do?

A 14 yr old girl with unexplained paralysis in both legs came to Emergency unit. According to the child a few days ago her feet were feeling “weird” and had trouble walking and now unable to move lower extremities. The physician suspects Guillain-Barré Syndrome. Which finding below during a nursing assessment requires immediate nursing action?

The child reports a headache.
The child has a weak cough.
The child has diminished reflexes in the lower extremities.
The child has paresthesia in the upper extremities.

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Red Flags

Weak cough
Difficulty breathing

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Cultural Considerations

Religion, culture, beliefs, and ethnic customs can influence how families understand and use health concepts:

Health beliefs: In some cultures talking about a possible poor health outcome will cause that outcome to occur.
Health customs: In some cultures family members play a large role in health care decision-making.
Ethnic customs: Differing gender roles may determine who makes decisions about accepting & following treatment recommendations.

AHRQ, 2020

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Cultural Considerations (Continued)

Religion, culture, beliefs, and ethnic customs can influence how families understand and use health concepts:

Religious beliefs: Faith and spiritual beliefs may effect health seeking behavior and willingness to accept treatment.
Dietary customs: Dietary advice may be difficult to follow if it does not fit the foods or cooking methods of the family.
Interpersonal customs: Eye contact or physical touch may be ok in some cultures but inappropriate or offensive in others.

AHRQ, 2020

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References:

CDC. (2022). Guillain Barre Syndrome. https://www.cdc.gov/campylobacter/guillain-barre.html

Health Direct. (2021). Neuromuscular System. https://www.healthdirect.gov.au/neuromuscular-system

Lee Health. (2022). Neuromuscular Diseases in Children. https://www.leehealth.org/our-services/pediatric-orthopedics/neuromuscular-disease-in-children

WHO. ( 2016 October 31). Guillain Barre Syndrome. https://www.who.int/news-room/fact-sheets/detail/guillain-barr%C3%A9-syndrome

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References:

National Institute of Neurological Disorders and Stroke. (2021). Guillain Barre Syndrome. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Guillain-Barr%C3%A9-Syndrome-Fact-Sheet

Nguyen, T.P., Taylor, R.S, & Renwanz Boyle, A.G. (2021). Guillain Barre Syndrome (Nursing). Treasure Island (FL): StatPearls Publishing. Available from: https://www.ncbi.nlm.nih.gov/books/NBK568815/

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