Date:
Patient:
Reason for Visit:
Follow-up for LAM
Evaluation of symptoms (e.g., dyspnea, cough, pneumothorax)
Assessment of lung function and response to treatment
History:
Present Illness:
Onset, duration, and severity of symptoms suggestive of LAM:
Dyspnea (shortness of breath) – progressive, worse with exertion
Chronic cough (may be productive)
Recurrent pneumothorax (collapsed lung)
Chylothorax (accumulation of lymphatic fluid in the pleural space) – less common
Chest pain (pleuritic)
Fatigue
Functional limitations due to symptoms
Past Medical History (PMH):
Diagnosed with LAM and date of diagnosis
Presence of underlying tuberous sclerosis complex (TSC) (associated with LAM in some cases)
History of prior pneumothorax or chest tube placement
Social History:
Smoking history (can worsen LAM symptoms)
Pregnancy history (pregnancy can exacerbate LAM)
Occupational exposures (塵埃 chén’ēi – dust, fumes)
Physical Exam:
Vital Signs: Assess for tachypnea (rapid breathing) and hypoxia (low oxygen levels).
General: Look for signs of respiratory distress (use of accessory muscles).
Pulmonary:
Auscultate for breath sounds (presence of crackles, wheezing).
Assess for chest wall asymmetry (suggestive of prior pneumothorax).
Diagnostic Tests (may be ordered depending on clinical presentation):
Chest X-ray: May show signs of cysts or hyperinflation.
High-Resolution CT scan (HRCT): Gold standard for diagnosing LAM, showing characteristic thin-walled cysts throughout the lungs.
Pulmonary function tests (PFTs): Measure lung function (reduced lung volumes and airflow obstruction).
Blood tests:
Serum lactate dehydrogenase (LDH) levels – may be elevated in LAM
TSC genetic testing (if TSC not previously diagnosed)
Assessment:
Confirmation of LAM: Based on clinical presentation, HRCT findings, and supportive evidence (e.g., TSC diagnosis).
Severity of lung disease: Based on PFT results and symptoms.
Presence of complications: Recurrent pneumothorax, chylothorax.
Plan:
Treatment plan based on disease severity and individual patient factors:
Hormonal therapy: Leuprolide (GnRH agonist) to suppress estrogen production (estrogen can worsen LAM)
Mammalian target of rapamycin (mTOR) inhibitors: Medications like everolimus to inhibit cell growth and proliferation (may be used alone or in combination with hormonal therapy).
Lung transplant: Considered in advanced cases with severe respiratory failure.
Pleurodesis: Surgical procedure to create scar tissue between the lung and chest wall to prevent recurrent pneumothorax.
Chest tube placement: For treatment of pneumothorax, with possible need for pleurodesis to prevent recurrence.
Supportive care: Oxygen therapy for hypoxemia, pulmonary rehabilitation to improve exercise tolerance.
Referral to a pulmonologist with expertise in LAM: For complex cases or consideration of advanced therapies.
Referral to a genetic counselor (if TSC is diagnosed)
Patient education: Provide information about LAM, the potential association with TSC, treatment options, potential side effects, importance of smoking cessation, and the importance of regular follow-up.
Discuss risks and benefits of pregnancy in LAM patients.
Follow-up:
Schedule for regular follow-up appointments to monitor symptoms, assess lung function with PFTs, monitor for complications (pneumothorax), and adjust medications as needed.
Discuss the importance of long-term management for LAM, as it is a chronic condition.
Disclaimer: This information is for educational purposes only and should not be interpreted as medical advice. Please consult with a qualified healthcare professional for diagnosis and treatment of pulmonary lymphangioleiomyomatosis.