In 2008, Bianca Morgan was pregnant with her second child when she developed breathing problems. The Trenton woman felt out of breath from seemingly simple activities. She attributed it to the pregnancy and the likely need to lose weight.
But after the birth of her daughter, her shortness of breath gradually worsened. She made an appointment with her primary care physician who initially diagnosed her with seasonal allergies. Months later, his respiratory problems remained.
Her doctor suggested she may have asthma, a chronic condition that causes inflammation of the airways. He prescribed two maintenance inhalers to help prevent inflammation and control his symptoms. (These medications are different from the rescue inhalers used to relieve an attack.) They offered some relief, but she still didn’t feel back to normal.
Over the years, the 41-year-old non-smoker normalized her breathing issues even as they got worse. She remembers a trip to Disney with her kids where she had to take several breaks just walking to the park entrance.
But one morning in 2017, Bianca’s breathing was different. She couldn’t get air in or out. She noticed that her lips had turned a bluish gray color and her feet and ankles were swollen. She dropped her children off at school and drove to the nearest emergency room.
Doctors discovered that his oxygen saturation was dangerously low. They put her on oxygen and admitted her to the hospital.
When her breathing and swelling were brought under control, Bianca was sent home with oxygen.
A few days later, at home, Bianca suddenly lost her breath, feeling like her throat was tight. She returned to the emergency room, where doctors told her she had a collapsed lung. It happens when air enters the chest cavity and creates pressure against the lung, causing it to collapse.
After a few days in the hospital with no progress in reinflating her lung, she requested a transfer to intensive care at Temple University Hospital.
At Temple, Bianca underwent video-assisted thoracoscopic surgery (VATS), a minimally invasive procedure in which a thoracic surgeon used a tiny camera to guide the removal of lung tissue that was allowing air to escape into the chest. . The surgeon also biopsied a piece of his lung.
Analysis of the biopsy showed signs of sarcoidosis, a rare inflammatory disease that causes the immune system to overreact.
People with sarcoidosis have clusters of inflamed tissue called “granulomas” in different parts of the body, although they most commonly affect the lungs. As granulomas form and grow, scar tissue may form in the affected organs, causing various symptoms.
Although the cause of sarcoidosis is unknown, research has shown that it occurs more often in black people. Women are also at higher risk of contracting the disease.
Because sarcoidosis symptoms can be vague and mimic other illnesses, patients are often misdiagnosed for months or even years. Often, by the time patients are correctly diagnosed, they have more advanced disease.
The severity of the disease varies. Some patients have mild disease and the granulomas may stop growing or shrink without treatment. Bianca was diagnosed with advanced pulmonary sarcoidosis, an advanced form that slowly gets worse over the years and can cause permanent scarring of the lungs known as fibrosis. This can reduce the amount of air the lungs can hold and cause abnormal stiffness, leading to breathing problems.
Advanced fibrosis can also lead to other complications such as pulmonary hypertension and infections. Patients with advanced disease have a higher risk of death and disability than patients with milder disease.
Despite advances in research, there is no cure. Limited treatment options focus on preventing further organ damage, reducing symptoms, and improving quality of life. Patients with advanced pulmonary sarcoidosis may need a lung transplant.
Bianca did not meet the eligibility criteria for a transplant and was referred to me to help manage the disease while waiting to be reassessed for a transplant.
Advanced sarcoidosis can affect multiple organs, each in a different way, so it’s best to seek treatment from a team of specialists who can work together to design a comprehensive treatment plan.
While I was working on a treatment plan, Bianca’s illness progressed rapidly to the point where she had to move her bedroom to the first floor of her house because she could no longer climb the stairs. His kidney function also declined.
Once I identified the cause of her symptoms – a combination of inflammation, fibrosis and pulmonary hypertension – we began a holistic treatment plan. To treat her fibrosis and help her breathe better, I recommended she continue taking supplemental oxygen daily and started her on a pulmonary rehabilitation program.
I also prescribed medication to treat pulmonary hypertension, a condition in which scar tissue narrows or blocks the arteries in the lungs, causing high blood pressure. Anti-inflammatory medications helped improve symptoms and organ function.
Patients with advanced disease need close follow-up (every two to three months) to monitor symptoms and adjust treatment as needed.
Bianca has seen her quality of life improve dramatically, now comfortably climbing stairs and resuming other daily activities that previously kept her out of breath. A transplant is still an option because her lung damage is irreversible, but she enjoys greater independence now that her disease is properly managed.
Rohit Gupta is Director of the Sarcoidosis Center of Excellence at Temple University Hospital and Associate Professor of Thoracic Medicine and Surgery at the Lewis Katz School of Medicine at Temple University.