What's the next step when a pediatrician decides to intubate a child with spinal muscular atrophy (SMA), but the parents refuse due to a previous child with the same illness who died from its sequelae?

Management of Parental Refusal of Intubation in a Child with Spinal Muscular Atrophy

The most appropriate next step when parents refuse intubation for a child with spinal muscular atrophy is to discuss the case with the ethics committee (option C). 1, 2

Understanding the Ethical Framework

When parents refuse a potentially life-saving intervention like intubation for their child with SMA, several key principles must be considered:

1. Parental Decision-Making Authority:

   • Parents are typically the most appropriate people to determine what is in their child's best interests 1
   • Their previous experience with a child who died from SMA significantly influences their perspective 2

2. Physician's Responsibility:

   • Physicians must determine if following parental wishes would put the child at significant risk of serious harm 1
   • When disagreements occur, physicians should explain the basis for disagreement and educate parents 1

3. Role of Ethics Committee:

   • The American Academy of Pediatrics explicitly recommends consulting institutional ethics committees for guidance in complicated ethical problems involving care and treatment of children 1
   • Ethics consultation is particularly valuable when concerns about forgoing life-sustaining medical treatment are raised 1

Why Ethics Committee Consultation is the Best Option

Why Not Simply Respect Their Wish (Option A)?

While respecting parental autonomy is important, physicians have a duty to protect the child's best interests. The AAP states that "in cases in which serious harm to a child is likely if the parent's wishes are followed, the pediatrician must get a second opinion and act to protect the best interests of the child." 1

Why Not Search for Solutions on the Internet (Option B)?

This option lacks professional rigor and fails to utilize established institutional resources designed specifically for these complex ethical dilemmas.

Why Not Ignore & Intubate (Option D)?

Ignoring parental wishes and proceeding with intubation would violate principles of:

• Informed consent
• Parental authority
• Professional ethics

The AAP guidance states that physicians should "explain the basis for the disagreement, educate the parent, and attempt to meet the child's needs within the constraints that exist" 1. Proceeding without parental consent would be justified only in true emergencies with no time for consultation.

The Ethics Committee Process

The ethics committee can help balance multiple considerations:

1. Medical Factors:

   • Current clinical status of the child
   • Prognosis with SMA (which varies by type)
   • Recent advances in SMA treatment that may affect outcomes 3
   • Potential benefits vs. burdens of intubation 1

2. Family Factors:

   • Parents' previous traumatic experience with another child
   • Their understanding of current SMA treatments and outcomes 4
   • Their values and goals of care for their child

3. Ethical Principles:

   • Best interests of the child
   • Parental authority
   • Physician's duty to prevent harm
   • Proportionality of interventions

Important Considerations in SMA Cases

• SMA outcomes have improved significantly with newer therapies, which may not have been available when the parents' first child was affected 3, 5
• Successful management of acute respiratory failure in SMA is possible with appropriate interventions 6
• The ethics committee can help determine if the parents' refusal is based on accurate, current information about SMA treatment options and outcomes 2

Conclusion

The ethics committee provides a structured, multidisciplinary approach to this complex situation, respecting both parental authority and the child's best interests while ensuring that decisions are based on current medical knowledge and ethical principles.