What questions should be asked during a routine follow-up for a patient with Amyotrophic Lateral Sclerosis (ALS) who is already on Bilevel Positive Airway Pressure (BiPAP) therapy?

Key Assessment Questions for ALS Patients on BiPAP During Routine Follow-up

During routine follow-up of ALS patients on BiPAP therapy, assessment should focus on respiratory function, nutritional status, and bulbar symptoms to optimize survival and quality of life.

Respiratory Function Assessment

• Ask about adherence to BiPAP therapy, including average hours of daily usage (aim for >8 hours/day for optimal survival benefit) 1, 2
• Inquire about comfort with the BiPAP mask, presence of air leaks, skin irritation, or other interface issues 3
• Assess for symptoms of hypoventilation despite BiPAP use, such as morning headaches, daytime sleepiness, or disturbed sleep 3
• Ask about changes in breathing pattern, increased shortness of breath, or orthopnea 3
• Evaluate the effectiveness of cough and ability to clear secretions (consider adding cough assist device if not already using one) 2
• Question about any recent respiratory infections or hospitalizations 3

Nutritional Status Assessment

• Inquire about weight changes since last visit (weight loss >5-10% is associated with decreased survival) 3
• Ask about changes in appetite, meal duration, and fatigue during or after eating 3, 4
• Assess for progression of dysphagia symptoms using structured questions about:
  • Difficulty with specific food textures or consistencies 4
  • Coughing or choking during meals 4
  • Need for dietary modifications (thickened liquids, pureed foods) 3
  • Time required to complete meals 4
• If not already using enteral nutrition, assess readiness for discussion about PEG placement before respiratory function significantly deteriorates 3, 5

Bulbar Function Assessment

• Evaluate for progression of bulbar symptoms by asking about:
  • Changes in speech clarity or volume 4
  • Increased drooling or difficulty managing saliva 4
  • Nasal regurgitation of food or liquids 4
  • Poor lip seal leading to food or liquid leakage 4
  • Difficulty forming or manipulating food bolus in mouth 4
• Assess for signs of aspiration such as coughing during meals, wet-sounding voice after swallowing, or recurrent pneumonia 3, 4

Quality of Life and Functional Assessment

• Ask about changes in overall functional status using structured questions based on ALS Functional Rating Scale-Revised (ALSFRS-R) domains 6
• Inquire about sleep quality and whether BiPAP has improved sleep 3
• Assess for symptoms of anxiety or depression related to disease progression 7
• Evaluate caregiver burden and need for additional support services 7

BiPAP Settings and Equipment Review

• Review current BiPAP settings (IPAP, EPAP, backup rate) and assess if adjustments are needed based on symptoms 3
• Check if supplemental oxygen is being used with BiPAP and review settings 3
• Confirm that the patient has backup equipment and knows how to troubleshoot common problems 3

Monitoring Recommendations

• Schedule regular assessment of oxygenation and ventilation (arterial blood gas, end-tidal CO2, or transcutaneous PCO2) 3
• Plan for videofluoroscopy if dysphagia symptoms have worsened to assess for silent aspiration 3, 6
• Recommend screening for malnutrition (BMI, weight loss) every 3 months 3
• Consider Volume-Viscosity Swallow Test (V-VST) for clinical screening of dysphagia progression (92% sensitivity, 80% specificity) 3, 6

Red Flags Requiring Immediate Attention

• Significant weight loss (>10% of body weight) since last visit 3
• New or worsening signs of respiratory failure despite BiPAP use 3
• Recent aspiration events or pneumonia 3
• Inability to tolerate BiPAP for recommended duration 1, 2

Follow-up Planning

• Schedule more frequent follow-ups if there is rapid progression of symptoms 4
• Consider earlier referral for PEG placement if significant dysphagia is present but respiratory function is still adequate 8
• Ensure coordination between neurology, pulmonology, nutrition, and speech therapy services 4, 8