Thoracentesis (also known as a pleural tap) is a procedure to remove fluid or air from the pleural space. The pleural space is an area between the lung and the chest wall that usually contains just a thin layer of fluid. At times, air or more commonly fluid can collect in this space. If there is enough fluid, a patient can become symptomatic, for example develop shortness of breath. If that occurs, removal can provide relief of these symptoms. Additionally, fluid may need to be removed to help aide in the diagnosis of many conditions. The test is performed as follows: You will sit on the edge of a bed or chair. Your head and arms will rest on a table. The skin around the procedure site is cleaned and the area is draped. A local numbing medicine (anesthetic) is injected into the skin. You will feel a stinging sensation when the local anesthetic is injected. The thoracentesis needle is inserted above the rib into the pleural space and a catheter (small plastic tube) if introduced. You may feel pressure when the needle is inserted into the pleural space. Fluid is collected and will be sent to a laboratory for testing. This procedure has been performed by physicians since 1852!

Cystic fibrosis (CF) is an inherited condition caused by a defective gene (CFTR – Cystic Fibrosis Transmembrane Conductance Regulator) which causes the body to produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb food. This collection of sticky mucus results in life-threatening lung infections and serious digestion problems. The disease may also affect the sweat glands and a man’s reproductive system. Millions of Americans carry the defective CF gene, but do not have any symptoms. That’s because a person with CF must inherit two defective CF genes – one from each parent. An estimated 1 in 29 Caucasian Americans has the CF gene. The disease is the most common, deadly, inherited disorder affecting Caucasians in the United States. It’s more common among those of Northern or Central European descent. Most children with CF are diagnosed by age 2. A small number, however, are not diagnosed until age 18 or older. Occasionally, relatively mild symptoms may lead to frequent misdiagnosis or no diagnosis at all unless the symptoms become worse. The condition may be misdiagnosed as emphysema, asthma or chronic bronchitis. These patients usually have a milder form of the disease. About 30,000 Americans and 70,000 people worldwide are living with cystic fibrosis. Only a few decades ago, children with CF seldom survived elementary school. Today, thanks to earlier diagnosis and improved treatments, the median survival for those with CF is 47 years of age. With new, novel therapies known as CFTR modifiers this number is likely to climb.

Dr. Schaefer helped to establish the Adult Cystic Fibrosis Program at the UMass-Memorial Medical Center. He sees patients with CF at the CF Center at UMass-Memorial University campus, as well as in MLA’s Worcester office.

UMass-Memorial / UMass Chan Medical School Adult Cystic Fibrosis Program

The UMass-Memorial Adult Cystic Fibrosis Center, accredited by the national Cystic Fibrosis Foundation, specializes in the management of adults with cystic fibrosis. We provide a team approach to care that includes medical, nurse, registered dietician, physical therapy, social worker, and respiratory therapy cystic fibrosis specialists. We collaborate closely with our colleagues in the UMass-Memorial Pediatric Cystic Fibrosis Center to assist adolescents and young adults in the transition from pediatric to adult care.

Our services

• Comprehensive cystic fibrosis diagnosis including sweat testing and blood testing for all known mutations related to cystic fibrosis.
• Full range of treatments for all complications related to cystic fibrosis, including those involving the respiratory, sinus, digestive, endocrine, and reproductive systems.
• Up to date therapeutics including use of the newest CFTR Modulators
• Management of cystic fibrosis lung disease, including mucus clearance techniques, mucolytics, and preventive therapy for patients infected with pseudomonas.
• Comprehensive nutrition counseling and care, including pancreatic enzyme replacement therapy, screening for and repletion of vitamin deficiencies.
• Bronchoscopy is a procedure used to visualize the inside of the airways (bronchial tubes). It is used for both diagnostic and therapeutic purposes. The bronchoscope is a flexible instrument that is inserted into the airways, usually through the nose or mouth. It has both a light source and digital camera at its end. This allows the physician to examine the patient’s airways for abnormalities such as tumors, bleeding, foreign bodies, or inflammation. At the time of the bronchoscopy specimens may be taken from inside the lungs such as biopsies, fluid, or brushings. The procedure can be accomplished as an outpatient with the use of sedation, similar to that used for a colonoscopy.You may be referred to MLA by your physician for abnormalities such as a nodule or mass found on an x-ray or CT scan, or for the evaluation of persistent or recurrent pneumonia. Your MLA physician will review all the specifics of this test if he or she decides this would be the best study for you.
• Close collaboration with Nutrition, Psychology, Respiratory Therapy, Gastroenterology, Endocrinology (diabetes), Transplant Surgery and Palliative Care.

Pulmonary function tests are a group of tests that measure how well the lungs take in and release air and how well they move gases such as oxygen from the atmosphere into the body’s circulation.

Pulmonary function tests are done to:

• Diagnose certain types of lung disease (most commonly asthma, chronic bronchitis and emphysema)
• Help find the cause of shortness of breath
• Assess the effect of medication to treat your lung disease
• Assess the effect of medication prescribed to treat your lung disease
• Assess the effect of medication that may negatively affect your lung function (for example, amiodarone)
• Measure progress in disease progression
• To assess perioperative pulmonary risk prior to undergoing surgery

The most commonly performed test is called “spirometry.” With this test you will breathe into a mouthpiece that is connected to an instrument called a spirometer. The spirometer records the amount and the rate of air that you breathe in and out over a period of time. For some of the test measurements, you can breathe normally and quietly. Other tests require forced inhalation or exhalation after a deep breath. You will be asked to wear a nose clip so we can collect all the air that comes out of your mouth. “Lung volume” is measured in other ways. In one way you will be asked to sit in a sealed, clear box that looks like a telephone booth (body plethysmograph) while breathing in and out into a mouthpiece. Changes in pressure inside the box help determine the lung volume.

Lung volume can also be measured when you breathe nitrogen or helium gas through a tube for a certain period of time. For one test known as a “methacholine challenge test” you will have to breathe in medication before the test.

Do not eat a heavy meal before the test. Do not smoke for 4 – 6 hours before the test. You’ll get specific instructions if you need to stop using bronchodilators or inhaler medications prior to the test. The tests are usually very well tolerated. Since the test involves some forced and rapid breathing, you may have some temporary shortness of breath or light-headedness.

For some patients with asthma avoidance of triggers and inhaled (and oral) medications are not enough to control their asthma.  Recently, several new medications, known collectively as “biologics,” have been approved for the treatment of moderate-to-severe asthma. The term “biologic” is used for medications and other healthcare products that are made from living animals, plants, or cells, instead of through a chemical process. Biologics include a wide range of products, such as vaccines and blood products, as well as medications used to treat certain medical conditions (e.g., insulin for diabetes). Biologic therapies offer new ways of treatment because they target different molecules in the body that contribute to asthma. Biologic therapies can treat some types of severe asthma by helping to stop body processes that cause inflammation of the airways. This inflammation that may be caused by allergies or by high levels of a cell in the body called an eosinophil. Eosinophils are a type of white blood cell linked to inflammation. People with severe asthma sometimes have high levels of eosinophils in the blood.

If your provider is considering using a biologic to treat your asthma, the biologic would be added to the reliever and controller medications you are currently taking. Biologics do not replace these medications, although some people may find that they are able to reduce the dose of their controller medication after several doses of a biologic. Please note that you should only reduce the dose of your controller if your specialty provider tells you to do so.

Several options are available for biologic therapy in treating severe asthma with the so called allergic or eosinophilic phenotype. Severe allergic asthma where severe symptoms are triggered by allergies. This type of asthma mostly starts in childhood. Omalizumab is a biologic that targets severe allergic asthma, reducing the allergic response. Eosinophilic asthma where severe symptoms are triggered by higher levels of cells called eosinophils. This type of asthma is usually associated with adult-onset asthma. Mepolizumab, reslizumab and benralizumab are all biologics that target severe eosinophilic asthma, by reducing eosinophils.  Severe asthma with type 2 inflammation where severe asthma symptoms are driven by both allergies and high levels of eosinophils. Dupilumab is a biologic targeting severe type 2 inflammatory asthma. It works by damping down the inflammatory response. The most recently approved biologic is a drug known as Tezepelumab. This targets an entirely different aspect of the asthma inflammatory cascade.  Most of the therapies are given as an injection, either in the office, or using pre-filled syringes at home. Reslizumab is given through an intravenous infusion. Biologic treatment is given as an injection at your doctor’s office or special clinics, usually every 2 to 4 weeks, depending on the particular medication.

As with all medicines, biologic treatments can have risks and side effects. Some common side effects (affecting up to 1 in 10 people) include: headache, sinus pain, sore throat, soreness at the injection site (this should pass in a few days and may improve after you’ve had the injection a few times). Biologic treatments for asthma have an increased risk of a severe allergic reaction (anaphylaxis). The risk is rare (1 in 1000 or more) and more likely if you have a history of allergic reactions or anaphylaxis.  This is not a complete list of the side effects that may happen with biologics; if your doctor is considering a biologic for you, please make sure to ask about the possible side effects that could happen with the specific medication you will be using.

There are currently no set recommendations on how long a patient should be on a biologic.  Guidelines recommend trialing the medication for at least four months to see if it is helping improve your asthma.  Your specialist may use tests to assess your status such as spirometry, FeNO, and blood tests. Whatever biologic you are prescribed, you will need to review your treatment regularly with your provider. Your doctor will work with you to decide how long to keep you on a biologic if your asthma is under good control. Contact your healthcare team if your asthma symptoms are not improving or are getting worse. Do not stop taking either your usual asthma medicines or your biologic treatment unless your doctor tells you to. If your treatment’s not working for you, or you’re getting difficult side effects, your specialist may stop treatment and look at other options.

A New Treatment Option for Severe Emphysema

Your doctor offers a new procedure to help you with severe emphysema. It is called the Zephyr® Valve treatment. The Zephyr Valve treatment is a procedure that allows a doctor to place one or more small valves in your airways, which release trapped pockets of air to improve your ability to breathe. It is not a medicine and it is not surgery.

Other Treatment Options

Emphysema cannot be cured; however, treatment may help
reduce symptoms, improve quality of life, and slow progression of the disease.

Current Emphysema Treatment Options:

• Stop smoking
• Medication
• Long term oxygen therapy
• Pulmonary rehabilitation
• Surgical lung volume reduction
• Lung transplant