Referring Patients for TBM Treatment

Tracheobronchomalacia (TBM) is a rare disorder characterized by weakness of the cartilage wall in the trachea and bronchi. Diagnosis is challenging, as the symptoms of TBM are similar to those seen in more common lung disorders such as asthma, COPD, and emphysema. Many patients go for months, even years, before TBM is diagnosed.

Symptoms of TBM include:

• Dyspnea or orthopnea (shortness of breath; labored breathing)
• Intractable bark-like cough
• Inability to clear secretions from lungs
• Recurrent respiratory infections

The sound of a cough before TBM treatment:

The sound of a cough after TBM treatment:

Please call 617-632-8252 or email us if you have questions or to schedule an appointment.

Along with the referral, please fax the following to our office as it will expedite the referral and eliminate duplicate testing:

• Most recent office note including current medication list
• Most recent pulmonary function test (PFTs), both pre- and post-bronchodilator and 6-minute walk
• Dynamic airway CT (send the CD and written report) (if available)
• Dynamic bronchoscopy report/images/video (if performed)
• Sleep study results (if available)
• ECHO (if available)
• GI testing/visits (if available)
• ENT testing/visits (if available)

Our fax number is 617-632-8253.

We offer a level of diagnostic expertise available at few other centers in the world.

Dynamic Flexible Bronchoscopy

Dynamic flexible bronchoscopy has become the gold standard for diagnosing TBM because it permits real-time examination of the airways and accurately captures dynamic airway properties. It also provides information on degree, extent, and location of pathology. This procedure is a specialized maneuver refined at BIDMC.

Patients receive local anesthetics to suppress the gag reflex and undergo the procedure with minimal sedation, to enable them to follow instructions. During the procedure, patients are asked to inhale deeply, hold their breath, and then forcefully exhale. Images are obtained at six different locations in the tracheobronchial tree and evaluated for degree of luminal narrowing.

Dynamic flexible bronchoscopy not only diagnoses TBM, but also facilitates the detection of other problems, such as vocal cord abnormalities or bronchitis, that may require treatment. During the procedure, we may also biopsy the mucosa or collect a sample of sputum for analysis.

Dynamic Expiratory CT Scan

This is a highly sensitive, noninvasive method for detecting TBM, with results that are concordant with dynamic flexible bronchoscopy. Patients are asked to breathe in, hold the breath, and then exhale forcefully so that the images reveal whether (and how much) the airway collapses when they breathe.

Assessing Severity of TBM

The airways are dynamic structures, and even healthy people experience some airway collapse while exhaling. Currently the accepted threshold for diagnosing TBM is a greater than 50% degree of airway collapse upon expiration.

Our research suggests that this threshold is too low, and may result in overtreatment. For that reason, we use the following parameters of central airway collapse to diagnose TBM and assess its severity:

• Less than 70 percent: Normal; consider alternative diagnosis
• 70 – 80 percent: mild TBM
• 81 – 90 percent: moderate TBM
• Over 90 percent: severe TBM

If a patient has mild or moderate TBM, we may recommend one or more options:

Airway Oscillatory Devices (Flutter Valve)

These hand-held devices help clear the airways by loosening phlegm and mucus.

External Percussion Vests

These inflatable vests are connected to a compressor and help clear the lungs of mucus and phlegm. These are usually used 2 or 3 times a day.

Expectorant

This medication makes it easier for patients to cough up mucus and phlegm, clearing the airways.

Pulmonary Rehabilitation

This involves education and training to improve a patient’s quality of life. Patients learn more about TBM, learn about different ways to breathe, how to conserve energy, and how to keep active.

Pursed Lip Breathing

This is a breathing technique in which patients inhale through the nose, while keeping their lips closed, and then exhale through tightly pursed lips. It is a simple yet effective way to slow breathing and alleviate shortness of breath.

CPAP or BiPap Devices

A continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPap) device is used while a patient is asleep. Both devices increase the air pressure in the throat, so that the airway doesn’t collapse and impede breathing.

Once severe, diffuse TBM is diagnosed, we evaluate patients to determine the extent and severity of TBM, and assess comorbidities that may require treatment before surgery. In our experience, 37% of patients have COPD, 23% have asthma, and 48% have GERD.

The surgery we have pioneered, tracheobronchoplasty, offers permanent relief for many patients with severe TBM. But to ensure that a patient will benefit, we conduct a thorough pre-operative evaluation and two-week stent trial.

Preoperative Evaluation

Prior to surgical evaluation, we evaluate whether the patient has any of the following conditions. If so, we offer treatment or coordinate with you to provide treatment:

• Vocal cord dysfunction
• GERD (gastroesophageal reflux disease)
• Other lung disorders (asthma, COPD)

Surgical Evaluation

• Physiologic assessment with pulmonary function testing
• A 6-minute walk test
• Patients are asked to fill out standardized questionnaires to determine functional status, symptoms, and quality of life, and cough-specific quality of life
• Pulmonary function testing may also be used after diagnosis of TBM to assess the degree of pulmonary comorbidity before intervention. This can help determine how well a patient may recover from tracheobronchoplasty.

Stent Trial

To ensure that a patient will benefit from surgery, we conduct a 2-week stent trial to assess how much symptoms will improve after the operation.

We place a Y-shaped silicone tracheobronchial stent into the airway sections that would be stabilized during surgery. While the stent remains in place, we monitor the patient closely to assess symptom relief and to manage any complications. In our experience, about 60 to 75 percent of patients with TBM receive enough symptom relief during the stent trial to suggest that surgery will help them permanently.

Tracheobronchoplasty

Tracheobronchoplasty is an open surgical procedure that typically lasts 6 hours. The surgeon opens the chest wall to reach the airways. A knitted polypropylene mesh is shaped into a Y-shaped posterior splint based on measurements we take of the patient’s anatomy. The mesh is sutured within the airway at the location where the cartilage wall has weakened. The mesh itself is flexible, but we use carefully placed sutures to create tension that holds it taut. (In a typical operation, the surgeon makes 60 to 80 sutures.) Over time, the mesh is incorporated by fibrosis, and the posterior membranous wall stiffens.

Patients spend a median of 3 days in the ICU. Median total hospital length of stay is 8 days. This may vary, depending on the patient's overall health and comorbidities.

After Surgery: Monitoring

We follow patients after surgery to ensure they are recovering well. This involves:

• Three months after surgery: Routine dynamic bronchoscopy and CT scans to establish a new baseline of function
• After the 3-month follow up, we assess patients annually for:
  • Respiratory quality of life
  • Functional status
  • Exercise tolerance

Permanent Stent Insertion

Patients with severe TBM who are not able to undergo tracheobronchoplasty may benefit from insertion of a tracheobronchial stent. This will improve shortness of breath and may alleviate the barking cough some patients experience. One challenge with stents is that some patients find them irritating, or the stents become infected. If that happens, we can remove the stent.

We are currently investigating ways to improve stents, by testing new materials that are less irritating. Our researchers are dedicated to advancing the field, in order to find new treatments for TBM.

We have patient education brochures and additional information available about TBM. If you would like to receive a packet of these materials to share with your patients, please contact us at 617-632-8252.