What's the next step when parents of a child with spinal muscular atrophy (SMA) refuse intubation due to a previous child's fatal outcome from the same illness?

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

The next appropriate action is to discuss the case with the ethics committee (Option C). When parents refuse a potentially life-saving intervention like intubation for their child with spinal muscular atrophy (SMA), especially based on their previous experience with another child who died from the same condition, ethical consultation is the most appropriate next step 1.

Understanding the Ethical Framework

When faced with parental refusal of medical treatment for a child, several key principles must be considered:

1. Respect for parental decision-making: Parents are typically the most appropriate people to determine what actions are in their child's best interests 1. Their previous experience with another child who died from SMA provides important context for their decision.

2. Physician's obligation to the child: The pediatrician must determine if the child would be put at significant risk of serious harm by following the parents' wishes 1.

3. Ethical resolution of conflicts: When pediatricians and parents disagree about medical care, the American Academy of Pediatrics (AAP) recommends:

   • Explaining the basis for disagreement
   • Educating the parents
   • Attempting to meet the child's needs within existing constraints
   • Seeking to understand the reason for disagreement 1

Why Ethics Committee Consultation is Appropriate

The ethics committee should be consulted because:

• The AAP explicitly states that "institutional ethics committees should be consulted for guidance, education, and advice regarding unusual or complicated ethical problems that involve the care and treatment of children" 1.

• In cases involving life-sustaining medical treatment (LSMT), ethics consultation "may be useful to the health care team and to families when ethical concerns about forgoing LSMT are raised or require clarification" 1.

• The committee can help balance the parents' previous traumatic experience against the current child's best interests.

Why Other Options Are Not Appropriate

• Option A (Respect parents' wish): While respecting parental decisions is important, immediate acceptance without further evaluation may not be appropriate if intubation is urgently needed to prevent imminent harm.

• Option B (Search for solution on internet): This is not a professional approach to medical decision-making and does not follow established ethical guidelines.

• Option D (Ignore & intubate): Performing a procedure against parental wishes without proper ethical consultation violates the principle of informed consent and parental authority, except in true emergencies where there is no time for consultation 1.

Considerations Specific to SMA

For children with SMA, several factors should inform the ethics committee discussion:

• SMA is a progressive neurodegenerative disease with high morbidity and mortality, particularly in types I and II 2, 3.

• Recent research shows that children with SMA types I or II can be successfully treated with noninvasive ventilation (NIV) and aggressive airway clearance during acute respiratory failure 2.

• When intubation is required, successful extubation can often be achieved with NIV transitional support combined with aggressive airway clearance 2.

• The landscape of SMA treatment has changed significantly with the approval of disease-modifying therapies 4, 5, which may affect the long-term prognosis and quality of life.

Recommended Approach

1. Urgently consult the ethics committee while providing necessary supportive care.

2. Document thoroughly the parents' refusal, their reasoning, and your assessment of the child's condition.

3. Explore with the ethics committee:

   • The parents' understanding of their child's condition
   • Whether their decision is based on accurate information about current SMA treatments
   • The immediacy of the need for intubation
   • Possible alternatives to traditional intubation
   • The legal framework applicable in your jurisdiction

4. Consider a time-limited trial of intervention if appropriate, with clear endpoints and goals.

5. Maintain open communication with the family throughout this process.

Remember that while forgoing life-sustaining medical treatment is ethically supportable when the burdens outweigh the benefits to the child 1, this assessment must be made carefully with input from multiple perspectives, especially in light of evolving treatments for SMA.