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Course: Pediatric Nursing

Topic: Nursing care of child with Neuromuscular Disorders Part IV

The Nurses International Community

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Module Goals

Learners will be able to:

Identify risk factors associated with and/or causes of myasthenia gravis and botulism in children.
Identify common signs and symptoms of myasthenia gravis and botulism.
List diagnostic procedures for myasthenia gravis and botulism.
Discuss the common medical treatments and management of myasthenia gravis and botulism.
Explain the role of the nurse in caring for children with myasthenia gravis and botulism.

Contact info: info@nursesinternational.org

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Myasthenia Gravis (MG)

The term ‘myasthenia gravis’ comes from the Greek word:

‘myasthenia’ meaning muscle weakness and
the Latin word ‘gravis’ meaning severe

An autoimmune condition where antibodies destroy neuromuscular connections leading to muscle weakness
Affects voluntary muscles of eyes, mouth, throat, arms, and legs

Better Health, 2019

Contact info: info@nursesinternational.org

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Types of MG

Three types in children:

Transient neonatal MG

Temporary form in infants born to mothers with MG
Occurs when antibodies from MG cross over to the fetus
Usually lasts for a short time
Symptoms go away in weeks or months after birth, as the antibodies are naturally replaced
Not at greater risk of developing MG later in life

Stanford Children’s Hospital, 2022

Contact info: info@nursesinternational.org

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Types

Juvenile MG

Occurs in caucasian teen girls
Lifelong condition with periods of remission
Treatment often requires removal of entire thymus gland

Congenital MG:

Very rare form of MG which is not an autoimmune disorder
Inherited as an autosomal recessive condition
Symptoms usually start at birth and are lifelong

Stanford Children’s Hospital, 2022

Contact info: info@nursesinternational.org

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Causes

Acquired through antibodies passed to infants born to mothers with MG
Rare forms of MG are caused by gene changes
About 85% of clients produce antibodies against a protein called the ‘acetylcholine receptor’ (AChR)
Antibodies bind to acetylcholine receptors on the muscle surface and greatly reduce ability of receptors to receive chemical signal
As a result, an individual experiences muscle weakness

Stanford Children’s Health, 2022

Better Health, 2019

Contact info: info@nursesinternational.org

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Critical Thinking Question

Myasthenia gravis is due to blocked_________________ receptors that are destroyed by antibodies.

Acetylcholine
Norepinephrine
GABA
Dopamine

Contact info: info@nursesinternational.org

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Signs and Symptoms

Symptoms include:

Weak muscles
Weakness that improves after resting and gets worse after physical activity
Visual disturbances such as double vision, inability to hold a steady gaze and droopy eyelids
Fatigue
Swallowing difficulties
Breathing difficulties
Shortness of breath

Better Health, 2019

Contact info: info@nursesinternational.org

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Signs and Symptoms

Symptoms may occur differently in each child, and vary by type:
Transient neonatal MG

Infants may be weak and have poor sucking
Breathing problems
Sometimes respiratory muscles are so weak leading to difficulty in breathing and baby may need mechanical ventilation
Symptoms resolve as the mother’s antibodies disappear over time

Stanford Children’s Hospital, 2022

Contact info: info@nursesinternational.org

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Signs and Symptoms

Juvenile MG

Signs and symptoms may start slowly over weeks or months
May become very tired after very little activity
Have trouble with chewing and swallowing
Drooping eyelids may be severe, may have double vision
Symptoms are milder in the morning and worse later in the day or after exercise

Stanford Children’s Hospital, 2022

Contact info: info@nursesinternational.org

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Signs and Symptoms

Congenital MG

Usually noted at birth, but may begin during first year
Overall weakness in arms and legs
Delays in motor skills (crawling, sitting, and walking)
Trouble feeding or breathing
May have weak eyelids and poor head control

Stanford Children’s Hospital, 2022

Contact info: info@nursesinternational.org

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Critical Thinking Question

The nurse is caring for a client admitted with suspected myasthenia gravis.

Which finding is usually associated with a diagnosis of myasthenia gravis? (Select all that apply)

Weak muscles
Visual disturbances such as double vision
Swallowing difficulties
Breathing difficulties

Contact info: info@nursesinternational.org

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Diagnosis

Family health history
Physical exam
Tests:

Blood test: to detect presence of antibodies
Electromyography (EMG)
The ‘Tensilon test’ involves injection of a drug called Tensilon, which temporarily improves muscle strength
Chest x-ray, CT scan or MRI: examine the thymus gland, because abnormalities of the thymus are often linked
Genetic tests: look for conditions that tend to run in families

Better Health, 2019

Stanford Children’s Hospital, 2022

Contact info: info@nursesinternational.org

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Treatment

No known cure
Symptomatic Management:

Mechanical ventilator to breathe
Nutritional supports
Medicines:

Anti-acetylcholinesterase agents: prevent ACh destruction and increase accumulation of ACh
Corticosteroids – (e.g., prednisone): suppress antibodies that interfere with function of neuromuscular junction

Better Health, 2019

Stanford Children’s Hospital, 2022

Contact info: info@nursesinternational.org

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Treatment

Plasmapheresis:

Removes abnormal antibodies from blood and replaces them with normal antibodies from donated blood.

Intravenous immunoglobulin:

Stops immune system’s production of its own antibodies.

Surgical thymectomy:

Removal of thymus gland is recommended for some individuals.

Better Health, 2019

Stanford Children’s Hospital, 2022

Contact info: info@nursesinternational.org

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Nurses Role

Assess ventilation and oxygenation
Special nutritional consideration while breastfeeding or feeding
Counsel parents in promoting a lifestyles that minimizes stress and maximizes relaxation
Strenuous activity is discouraged to minimize myasthenia crisis
Psychological support
Educate parents and family about importance of accurate administration of medications with emphasis on side effects, danger of choking, and respiratory distress

Hockenberry & Wilson, 2007

Contact info: info@nursesinternational.org

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What Would the Nurse Do?

The most significant initial nursing observations that need to be made for a client with myasthenia include:

Ability to chew and speak distinctly
Degree of anxiety about her diagnosis
Ability to smile an to close her eyelids
Respiratory exchange and ability to swallow

Contact info: info@nursesinternational.org

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Botulism

A rare but serious illness caused by a toxin that attacks the body’s nerves and causes difficulty breathing, muscle paralysis, and death
Toxin is made by bacteria:

Clostridium botulinum
Clostridium butyricum and Clostridium baratii
Produce the toxin in food, wounds, and intestines of infants

Is a medical emergency and may cause death if untreated

CDC, 2021

Better Health 2017

Contact info: info@nursesinternational.org

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Types of Botulism Common in Children

Infant botulism:

Most common form of botulism
Children under age 12 months are most susceptible
Caused by consuming food, particularly honey, dust or soil containing Clostridium botulinum spores

CDC, 2021

Better Health 2017

Contact info: info@nursesinternational.org

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Types of Botulism common in Children

Wound botulism:

Rare form of botulism
Caused by contaminated soil or gravel invading a wound
Intravenous drug users are susceptible
Symptoms occur after four days to two weeks

Foodborne botulism:

Common sources are homemade foods improperly canned, preserved, or fermented

CDC, 2021

Better Health 2017

Contact info: info@nursesinternational.org

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Critical Thinking Question

The majority of childhood botulism cases are:

Pre-schoolers
In infants
In teens
School aged children

Contact info: info@nursesinternational.org

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Signs and Symptoms

Signs and Symptoms of botulism in infants include:

Constipation
Poor sucking and feeding
Choking and gagging
Weak, feeble cry
Reduced movements of the limbs
Inability to control head movements
Increasing weakness and floppiness
Paralysis
Breathing difficulties

Better Health, 2017

Contact info: info@nursesinternational.org

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Who is at Risk?

Children under twelve months of age are commonly at risk of intestinal botulism
Older children and adults are not usually affected
People who eat home canned fruit, vegetables or meats are at risk of foodborne botulism
Open wounds that are not properly cleaned
Injecting drug users

NSW, 2018

Contact info: info@nursesinternational.org

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Critical Thinking Question

Which is the food most likely to be contaminated with botulism toxin?

Raw tuna
Home-canned foods
Raw eggs
A mayonnaise-based salad at a summer picnic

Contact info: info@nursesinternational.org

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Diagnosis

Based on history and assessment of signs and symptoms
To confirm diagnosis:

Stool culture and
Direct toxin assay

Obtained from stool, serum, or gastric contents

Most sensitive test: mouse inoculation test performed by CDC
Polymerase chain reaction (PCR) to detect spores
Important to rule out meningitis by performing a lumbar puncture

NSW, 2018

Contact info: info@nursesinternational.org

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Management

Antitoxin to prevent toxin from causing severe complications
Hospitalisation is usually required
Intensive care with mechanical ventilation may be needed if muscles involved in breathing have been affected
Wound botulism sometimes needs surgery to remove source of bacteria and child may need to take antibiotics
Infants may require immunoglobulin

NSW, 2018

CDC, 2021

Contact info: info@nursesinternational.org

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Prevention in Infants

Avoid giving honey to infants under 12 months of age
Keep newborns away from soil until umbilical stumps have dropped off and navel has completely healed
Take care when preparing, handling, and storing solid foods

Better Health, 2017

Contact info: info@nursesinternational.org

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Nurses Role

Assess for signs of poor feeding, constipation, and muscle impairment in child
Intensive care when child is admitted to hospital
Parental support and reassurance
Advise parents/caretakers that normal bowel activity may not return for several weeks

Hockenberry & Wilson, 2007

Contact info: info@nursesinternational.org

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Critical Thinking Question

One way parents can help prevent botulism in an infant is to:

Have the child vaccinated
Not to give honey to a child under a year old
Stay away from swimming pools
All of the above

Contact info: info@nursesinternational.org

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Red Flags

Reduced movements of the limbs
Inability to control head movements
Increasing weakness and floppiness

Contact info: info@nursesinternational.org

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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

Contact info: info@nursesinternational.org

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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

Contact info: info@nursesinternational.org