Leslie A. Couch, MD

Cystic fibrosis is a progressive, genetic disease that predominantly affects the lungs and digestive system. This disease usually shows signs in early infancy and can by treated by a physician to manage complications and improve quality of life. Dr. Rodolfo Amaro-Galvez, director of UT Health’s Cystic Fibrosis Clinic, and Dr. Leslie Couch, pulmonologist, see both children and adults who have been diagnosed with this disease.

Causes and Testing

Cystic fibrosis is caused by a genetic mutation. People with this disease have inherited two copies of the defective cystic fibrosis gene -- one copy from each parent. Both parents must have at least one copy of the defective gene. It is most common in Caucasian parents, occurring in 1 in every 2,500 to 3,500 Caucasian newborns. There are tests available for children suspected of having this disease.

“A newborn screen for cystic fibrosis mutations is done in all the states in the U.S.,” says Dr. Amaro-Galvez. “If the newborn baby has two CF mutations and symptoms of CF such as chronic cough, wheezing, recurrent chest infections and/or failure to thrive, it may indicate that the child has CF. A quantitative sweat chloride test is the gold standard test when a child has suspected CF.” The sweat test is used because individuals with cystic fibrosis have more chloride in their sweat than people who do not have this disease.

The sweat test measures the amount of chloride (a component of salt) in the sweat. In the first part of the test, a colorless, odorless chemical (pilocarpine) and a little electrical stimulation is applied to a small area of the arm or leg to encourage the sweat glands to produce sweat. A person may feel tingling in the area, or a feeling of warmth. The sweat is then collected on a piece of filter paper or gauze or in a plastic coil. The collected sweat is then sent to a hospital laboratory to measure how much chloride is in the sweat.

Symptoms

In the United States, there are 1,000 new cases of cystic fibrosis diagnosed every year, with 75 percent of patients being diagnosed by age 2. “Symptoms vary among patients depending on the genetic defect,” says Dr. Couch. However, there are a number of common symptoms that parents can notice, mostly to do with lung function. “If the child has symptoms of cystic fibrosis such as chronic cough, wheezing, recurrent chest infections and/or failure to thrive, an evaluation in a cystic fibrosis clinic can be done,” says Dr. Amaro-Galvez.

Other symptoms:

• Very salty-tasting skin, which can be noticed when parents are giving their child a kiss
• Frequent lung infections, including pneumonia or bronchitis
• Shortness of breath
• Inflamed nasal passages or a stuffy nose
• Poor growth or weight gain in spite of a good appetite
• Frequent greasy, bulky stools
• Difficulty with bowel movements

Treatments and Research

Treatments vary from patient to patient due to the wide range of symptom severity and type. It’s up to the physician to create a custom plan that will work best for the patient’s unique needs. The Cystic Fibrosis Clinic at UT Health North Campus Tyler caters to those needs with a diverse team that not only has pulmonologists, but can also include a nutritionist, social worker, respiratory therapist, pharmacist, and/or physical therapist. The team can also care for patients throughout their lifetime, from childhood through adulthood, adapting the treatment as it changes. 

Dr. Couch highlights how diverse treatment can be. “Some patients take enzymes to digest food, take pulmozyme (mucolytic) to break up the thick mucus in lung, inhaled antibiotic and various forms of airway clearance. Some take insulin to control blood sugar, take special vitamins as they do not absorb well.”

Although there is no cure for cystic fibrosis, there are many trials and new treatments available that have bettered the quality of life and increased the lifespan of those with the disease. Both Dr. Couch and Dr. Amaro-Galvez are excited about some of the most recent developments. “There are many promising clinical trials testing different therapies in cystic fibrosis,” shares Dr. Amaro-Galvez. It was recently announced by Vertex, the company that has developed 3 CFTR modulators that one of them Kalydeco (Ivacaftor), has been approved to be use in children ages 6 months and older. Dr. Couch explains that this helps correct the genetic defect, allowing the chloride pump to work better.

Patients in the area will have access to new deployments as UT Health North Campus Tyler is a part of the Therapeutic Development Network for the Cystic Fibrosis Foundation. “This allows our patients access to new drugs being developed that are approved by the Cystic Fibrosis Foundation,” says Dr. Couch. “New drugs are new anti-inflammatory medications, newer genetic medications and newer mucolytic.”

Physicians at the Cystic Fibrosis Clinic are proud to put into practice all of the amazing advancements in treatments for those who have cystic fibrosis. “There is hope that exciting therapies are in the pipeline,” says Dr. Amaro-Galvez. “The outcome and perspective of this disease has changed dramatically over the past two decades.”

Learn more about cystic fibrosis care at UT Health North Campus Tyler visit https://uthealthnorth.com/services/lung-care or call 903-877-7916.

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Dr. Amaro specializes in pediatric pulmonology, the study and treatment of children’s lung diseases. He has two decades of experience treating children with cystic fibrosis, pneumonia, whooping cough and other respiratory diseases. In addition, he is one of only a couple of board-certified specialists in pediatric sleep disorders in Northeast Texas.

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Dr. Couch is a pulmonary expert who has treated patients with chronic obstructive pulmonary disease (COPD) and other lung diseases for more than 20 years. Her specialty is treating patients who have recurring infections that damage the airways to the lungs. To help treat this condition, Dr. Couch has conducted research to determine how effective inhaled antibiotics are in preventing infection.

Chronic Obstructive Pulmonary Disease, commonly referred to as COPD, is a term used to describe several different lung diseases that affect the lung functionality of over 16 million Americans. Even more suffer from COPD without being diagnosed. Although there is no cure, this disease progresses slowly and patients who seek out help can be prescribed treatments that will slow the disease and make it easier to breathe.

UT Health East Texas North Campus Pulmonary Clinic has many physicians with expert knowledge and experience in treating COPD. These pulmonary experts share their knowledge in hopes of bettering the quality of life for people with COPD.

The Effects of COPD on Your Lungs

Within normal lungs, the bronchial tubes branch into thousands of smaller, thinner tubes called bronchioles that end in bunches of tiny, round air sacs called alveoli. Small blood vessels, called capillaries, run through the walls of the alveoli. When air reaches the alveoli, oxygen passes through the walls into the blood in the capillaries. At the same time, carbon dioxide moves from the capillaries into the alveoli. This processes is called air exchange. The bronchioles and alveoli are elastic. With inspiration (inhaling or breathing in), each alveoli fills up with air like a small balloon. With expiration (exhaling or breathing out), they deflate and the air goes out. In COPD, less air flows in and out of the airways because of one or more of the following:

1.            The bronchioles and alveoli lose their elastic quality.

2.            The walls between many of the alveoli are destroyed.

3.            The walls of the bronchioles become thick and inflamed.

4.            The bronchioles make more mucus than usual, which can clog them.

Symptoms of COPD

COPD can have seemingly benign symptoms, such as a nagging cough, so people suffering from this disease often avoid seeing a physician. However, since treatments can slow the disease and improve quality of life, it’s important to know what to look out for and get tested.

COPD is diagnosed based on signs and symptoms. Be aware if it’s an ongoing cough, how long it’s been present, how much or how often you cough, and how much mucus is produced. Recall details such as medical and family histories, including questions regarding smoking history, contact with lung irritants, such as secondhand smoke, air pollution, chemical fumes or dust. The main test for COPD is spirometry, which measures the amount of air and how fast the air can be breathed out. Other tests include a chest XRAY or a CT scan that can show signs of COPD. An arterial blood gas test (ABG) helps determine the severity of COPD and whether oxygen therapy is needed. Also, a six-minute test is used to determine exercise tolerance and to qualify a patient for oxygen therapy.

After getting tested, there are a lot of lifestyle changes a patient can make that will improve their functionality and overall health.

After Being Diagnosed

The first step in slowing disease’s progression is to prevent exposure to harmful lung irritants, such as cigarette smoke, heavy dust, fumes and air pollution. After that, slow changes should be adopted into your everyday routine, including how you eat, exercise and get around your home.

You should eat smaller, more frequent meals; rest before eating, and take vitamins or nutritional supplements as well. Physical activity can strengthen the muscles that help with breathing and improve overall wellness, but do activities slowly. Put frequently used items in one place that’s easy to reach, find simple ways to cook, clean and do other chores. Use a small table or cart with wheels to move things around and a pole or tongs with long handles to reach things. Ask for help moving things around your house so that you don’t have to climb stairs as often. Keep clothing loose, and wear clothes and shoes that are easy to put on and take off. Don’t forget to also ask friends and family for help with daily tasks.

Help from Your Community Providers

There are whole programs, departments and providers dedicated to helping those who are affected by COPD and other lung diseases. Our physicians, along with many other experts, are continually researching COPD, looking for ways to relieve symptoms, slow and eventually eliminate this disease.

UT Health East Texas has a lung (pulmonary) rehab program with the physical therapy department that can help patients learn to manage the disease. A team of health professionals can provide counseling, teaching and research for patients diagnosed with COPD. We are always looking for research participants to assist our organization in finding better treatments and even cures for disease processes. I would highly encourage anyone who might be interested in being involved with pulmonary research to contact our clinic or hospital so that we can assist in getting them enrolled in research studies.

If you are having trouble breathing, or are worried about your lung health, please make an appointment with a physician. To find a physician, please call 903-596-DOCS. If you are interested in joining a research study, talk to your physician. Most importantly, if you are a smoker struggling to quit, please contact a physician. There are many services and providers at UT Health East Texas that are there for you. For more information about UT Health East Texas Pulmonary Institute visit uthealtheasttexas.com/services/lung-care.