What is Bronchiectasis?

Bronchiectasis is a condition of the lung characterized for abnormal dilatation and loss the architecture of the airway/bronchi. The bronchi is the tubing within the lungs in charge of transporting oxygen into the lungs and CO2 out of the lungs. These changes in the airway are cause by chronic inflammation and infections.

Most patients with Bronchiectasis complain of cough and mucous production. As the airway gets damage, the normal muco-ciliary mechanism in charge of moving the mucous is also affected causing pooling of the mucous in these areas and accumulation, allowing bacteria to grow an colonize the lungs.

DIAGNOSIS

About half of the patients with bronchiectasis have a cause. The other half are consider idiopathic meaning there is no clear cause causing this disease.

The most common causes of bronchiectasis includes:

  • Past severe lung infections that damage the lungs.
  • Non-tuberculous mycobacterium infection.
  • Genetic diseases like Cystic Fibrosis, Alpha-1 Antitrypsin Deficiency, and Primary Ciliary Dyskinesia.
  • Conditions affecting the Immune system making it difficult to fight infections.
  • Allergic bronchopulmonary aspergillosis. An allergic reaction to specific fungus colonizing the airway.
  • Aspiration. Breathing in stomach content, liquid or food.
  • Other conditions like connective tissue disease, inflammatory bowel disease, etc.

MANAGEMENT

The principal course of treatment for patient with bronchiectasis is airway clearance, inhaled antibiotics and anti-inflammatory medications. This consist in a series of nebulized medications to help coughing and bringing mucous up as well as controlling the bacteria proliferation. At the same time, several devices can be used to assist with moving mucous up mechanically by inducing airway vibration. 

Patients with bronchiectasis can have exacerbations. This are events when their cough and sputum production increase. These episodes can also be associated with shortness of breath and blood in the sputum. During a bronchiectasis exacerbation, patients are treated with antibiotics and depending on the severity, they might need admission to the hospital.

PROGNOSIS

In general, the prognosis for patients with bronchiectasis continues to improve. Most patients are able to have a normal lifespan. The goals of treatment are to reduce the number of exacerbations and to slow the decline of the lung function.  Patients that continue to progress despite aggressive medical management can be refer and consider for lung transplant.

What is Non-tuberculous Mycobacteria Lung disease?

NTM-LD is a chronic infection of the lungs cause by a group of bacteria’s call non tuberculous mycobacteria. Patients usually present with nonspecific complains including, fatigue, lack of energy, weight loss, cough, shortness of breath. Most patients have an underlying lung condition, including bronchiectasis, chronic obstructive pulmonary disease, asthma and interstitial lung disease, that predispose them to being colonize first and later infected by these bacteria’s.

Nontuberculous mycobacteria, or NTM, are opportunistic pathogens that cause lung disease. With over 150 different species of NTM widely distributed throughout the environment, the pathogens are most commonly found in water and soil particles.

NTM lung disease (NTM-LD) is not contagious, but poses a threat to those with compromised immune systems and those with pre-existing lung conditions. Traditionally it was thought to affect middle-aged and elderly women who are underweight. However, we know now it can affect multiple populations specially patients with underlying lung disease and immunosuppress. People can live with the condition for months, even years, without knowing because the symptoms are similar to those of other lung conditions.

An estimate of 1 out of 7 NTM-LD patients live in Florida,  symptoms including cough, fatigue and shortness of breath. However, it can also cause infections in the skin, lymph nodes and any organ, leading to symptoms like fever, night sweats, abdominal pain and diarrhea. Diagnosis requires clinical evaluations and tests, including high-resolution computed tomography scans and sputum cultures, to determine any radiologic abnormalities and microbiologic cultures.

Depending on the severity of the infection, some patients with NTM lung disease do not require treatment, as the bacteria can just colonize the airway without affecting the lungs. When patients develop symptoms and radiographic changes, it requires ongoing treatment; treatment includes multiple antibiotics for an average of 16-18 months. While NTM disease has lasting effects on the individual, it is rarely linked to death.

At UF Health Shands Hospital, the NTM Lung disease program’s mission is to seek a better understanding of NTM disease to provide patients with better diagnoses and management. This is accomplished through clinical trials, trained providers and integration of different focus areas within the program.

Because NTM disease is most commonly known to affect the lungs and those with pre-existing lung damage or diseases, current clinical trials focus mainly on this region. The team at UF Health has been researching how involving inhaled antibiotics and medications may battle the bacteria, with plans to broaden their scope of the implications and treatment of the disease.

We already have several clinical trials going on for this particular condition and expect to grow more on the area, we do clinical trials mostly on inhale antibiotics and inhale medications that can improve the clearance of the bacteria and improve outcomes.

Due to the varying levels of severity in NTM lung disease diagnosis and prognosis, treatment needs to be carefully determined on a case-by-case basis. This requires ongoing monitoring of patients’ situations, and the flexibility to alter treatment once it has begun. The program’s pharmacology team is educated on NTM disease, and is trained to understand when and why certain treatment is necessary.

“Our pharmacology program has been extremely helpful in getting and assessing the levels of the drugs so we can make sure that we’re treating the patients the right way and adjust properly,” Lascano said.

In contrast to other similar programs, UF Health’s team takes a multidisciplinary approach by allowing their researchers and physicians to diagnose and treat patients under one roof – making care more accessible and timely. This approach to care allows the team to have all hands on deck when making any decisions regarding assessment or treatment, and gives patients more places to turn for answers to any questions they may have.

“It’s important that we have this clinic in Florida, which is one of the hot spots for NTM disease. The greatest value of this program is all the coordination between microbiology, physicians and pharmacology,” Lascano said. “Traditionally, samples will be collected, then sent to the state lab to be identified, then to Denver to identify the bacteria, but we can do most of that in house. It cuts down the amount of time that we have to wait for results and make the whole process much quicker.”