Core Clinical Management Areas
Currently, KD is managed through coordinated, multidisciplinary care, with promising therapies actively being investigated. While no disease-modifying therapy is currently approved in the US, optimized symptom management, anticipatory guidance, and proactive monitoring can significantly improve patient outcomes and quality of life.
Below is a practical summary of what frontline clinicians should assess and manage.
1. Neuromuscular Function
What to Assess
- Progressive weakness (typically proximal and symmetric)
- Fatigue, cramps, fasciculations
- Functional status: transfers, gait, endurance
- Frequency of falls or near-falls
Provider Actions
- Encourage safe, sustainable exercise
- Avoid overexertion; emphasize low-impact aerobic exercise and gentle strengthening
- Recommend early mobility aids when indicated
- Refer to PT/OT for strengthening, fall prevention, and assistive devices
- Track progression through manual muscle testing (MMT), patient-reported function, and gait analysis
2. Bulbar and Swallowing Function
Dysphagia is highly prevalent and a major contributor to morbidity.
Recommended Care
- Early referral to SLP for swallowing/speech assessment
- Consider tongue, lip and pharyngeal strengthening exercises
- Yearly video swallowing studies to monitor for progression, or sooner if change in symptoms
- Early diet modification with progression of swallowing difficulty
- Monitor for signs of silent aspiration, weight loss, and dehydration
What to Watch For
- Difficulty swallowing pills, solids, or liquids
- Drooling, coughing during meals, “wet” voice
- Unexplained weight loss
- Recurrent pneumonia or aspiration events
Aspiration is a major preventable complication — early intervention is essential.
3. Respiratory Health
KD typically causes mild respiratory involvement, but complications arise from aspiration, reduced cough strength, or sleep-related breathing issues. Respiratory impairment is generally mild but should not be overlooked.
Suggested Monitoring
- Baseline and periodic pulmonary function tests
- Nocturnal oximetry if sleep-disordered breathing suspected
- Address aspiration risk as a respiratory complication
Provider Actions
- Obtain baseline pulmonary function tests
- Screen for nocturnal symptoms (snoring, daytime sleepiness)
- Consider nocturnal oximetry or sleep study if indicated
- Address aspiration aggressively
- Aggressive mucous management for those with allergies, chronic sinus issues
- Cough assist devices in later stages
4. Endocrine and Metabolic Health
Common KD-specific metabolic abnormalities:
- Reduced insulin sensitivity
- Dyslipidemia
- Gynecomastia and androgen insensitivity features
- Mild hepatic enzyme elevations (commonly due to fatty liver as well as elevated CK)
Recommended Screening
- Fasting glucose or HbA1c
- Lipid panel
- Liver function tests
- Consider baseline hormone panel
- Consider baseline fibroscan of liver and monitoring every 3 years
Provider Actions
- Conduct routine metabolic screening
- Avoid exogenous testosterone therapy unless correcting deficiency
- Collaborate with endocrinology for complex cases
- Refer to endocrinology for complex metabolic or hormonal issues
5. Nutrition and Weight Stability
Both overweight and underweight states worsen KD outcomes.
Provider Actions
- Monitor BMI and weight trend
- Refer to a dietitian for individualized meal planning
- Address dysphagia-related modifications early
- Encourage adequate hydration
- Monitor weight regularly; both weight loss and obesity exacerbate weakness
- Implement nutritional interventions early in the context of dysphagia
- Hydration monitoring is essential
6. Mobility and Rehabilitation
- Encourage safe, sustainable physical activity
- Falls prevention with balance and proprioception training, core stabilization
- Assess fall risk routinely
- Train in fall recovery techniques
- Recommend appropriate assistive devices, orthotics, bracing, or mobility aids
- Consider home safety evaluation
7. Mental Health and Psychosocial Support
Chronic progressive conditions can impact mood, coping, and family dynamics.
Provider Actions
- Providing patient education and long-term planning support
- Screen for depression and anxiety
- Provide referral to counseling, peer support, or community programs
- Encourage participation in patient organizations such as KDA
8. Cardiac Monitoring
Provider Actions
- Provider Actions
- Screening for Brugada Syndrome or Brugada pattern with ECG
- Early screening for cardiovascular disease given associated metabolic disease and increased CVD risk
- Appropriate management of CVD risk factors
