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Pulmonary Hypertension: A Dangerous Condition
High blood pressure in the lungs, known as pulmonary hypertension (PH), is a dangerous condition that disproportionately affects women. While experts have long recognized this disparity, very little is known about why it exists. Physician scientists at National Jewish Health are uncovering the mysteries behind PH in women and finding solutions from every angle, from prompt and accurate diagnoses to targeted treatments and disease management to the development of new therapies that may ultimately halt disease progression and save lives.
While PH can be caused by a wide range of conditions it can also manifest without any known cause, and its symptoms are vague and broad, such as fatigue and shortness of breath, making diagnosis a challenge. Many women go years with a misdiagnosis of things like asthma or anxiety, while pulmonary hypertension is only causing more damage to blood vessels and becoming more difficult to treat.
"It's a disease that requires input from multiple specialties, so we have built a comprehensive program where pulmonologists work together with cardiologists, rheumatologists and radiologists to thoroughly screen each patient, provide an accurate diagnosis and offer every available treatment," said Patricia George, MD, director of the Pulmonary Hypertension Program at National Jewish Health.
Through this comprehensive program, clinicians are able to give patients concrete answers and effective treatment, but there are still many lingering questions about exactly why PH develops so often in women and how to target its biological causes.
"We can ask specific questions based on what we see in the clinic, and then take these questions to the lab and try to design experiments to answer them," said Tim Lahm, MD, director of pulmonary vascular research at National Jewish Health. "At the same time, we can take the discoveries we make in the lab to the clinic to offer patients cutting-edge knowledge and therapies."
It's this bench-to-bedside approach that helps patients like Kira Cronk, who suffer from the most dangerous form of PH, pulmonary arterial hypertension (PAH).
"I was experiencing symptoms that prevented me from living a normal life for a couple of years before I had a diagnosis. Even just trying to climb a flight of stairs, I'd have to stop halfway up to catch my breath and my lips would turn purple," Cronk said. "Finally, I had a blood clot that caused my arm to swell up to twice its size and I knew it was time to figure out what was really going on."
At this point, it was determined that Cronk had congestive heart failure, but the root cause was still unknown. Finally, she was referred to pulmonary specialists at National Jewish Health, where comprehensive testing confirmed she was suffering from PAH, and while there is currently no cure, there are treatments to ease symptoms and help her manage the disease.
"Finally receiving effective medication and implementing some lifestyle changes to manage PAH has made an incredible difference in my symptoms," Cronk said. "Before my diagnosis, my body had essentially started shutting down — my heart was failing, my kidneys were affected and I was unable to even get up to shower on my own — but now, I'm able to drive myself to appointments, I can go shopping with my daughter, even take my dogs for a walk around the neighborhood. It's like night and day, and I feel like I got my life back."
"We truly value educating patients about their disease. Oftentimes, patients are their greatest advocates and we try to set them up for success," said Jordin Rice, RN, a pulmonary nurse practitioner at National Jewish Health. "Being able to teach patients like Kira about their medications and why their symptoms vary is a huge part of the comprehensive care we provide."
While current treatments work to relax blood vessels to treat PH, researchers are also developing and testing new treatments that address the biological cause of unexplained PH involving the thickening of blood vessel walls, which make it harder for the lungs to perform the critical tasks of taking in oxygen and releasing carbon dioxide.
"There are several drugs currently in clinical development that we are very excited about and feel will have a profound impact for patients suffering with pulmonary hypertension," said Dr. Lahm. "These novel therapies actively slow down the uncontrolled growth of the blood cells of the vessel wall, which is a whole new way to treat this disease."
There is additional research in the works that is providing more insight into cases of PAH without a clear cause, including a study recently published in CHEST exploring genetics, patients' knowledge of how their family history contributes to their disease and the role of genetic testing in assessing risk and customizing treatment.
"Genetic testing is a necessary component of comprehensive care and risk stratification for patients and family and can influence the treatment of pulmonary hypertension," said Rice, the lead author of the study. "Patients who undergo PAH evaluation should be given the opportunity to speak with a genetic counselor, but our study found that less than half of PAH patients were offered this resource."
Researchers continue to find clues about how the disease behaves uniquely in women and the specific biological pathways that hold the key to customizing treatment for women and men to one day even prevent the disease from developing. As that research evolves, it's important for anyone with unexplained symptoms to talk to their doctor about screening for PH. Early diagnosis is key to limiting damage to the heart and lungs and improving quality of life.
What To Know About The Pathophysiology Of Asthma
The pathophysiology of asthma refers to the changes that occur in the airways and lungs that lead to asthma symptoms. Understanding the pathophysiology can help with targeting asthma treatment.
Asthma is a long-term lung disease that affects the bronchial tubes, which are the airways that carry oxygen to the lungs. People with asthma may develop inflammation and narrowing of the airways, which makes breathing difficult.
Pathophysiology refers to the ways a disease alters or affects normal bodily function.
The pathophysiology of asthma is the process or ways asthma alters the lungs. The exact process and impact on the lungs may differ slightly depending on the type of asthma someone has. However, the pathophysiology remains similar. Understanding the pathophysiology of asthma helps in determining effective treatment.
The prevalence of asthma in the United States is about 8%. According to the National Heart, Lung, and Blood Institute (NHLBI), the exact cause of asthma remains unknown. However, a combination of environmental and genetic factors likely contribute to the development of the disease.
For instance, environmental factors, such as exposure to cigarette smoke as a young child, may affect lung development and increase the risk of asthma. Frequent viral infections as a child may also affect a person's asthma risk.
Some people may experience environmental risks and never develop asthma. However, others may have a genetic predisposition that makes them more likely to develop the condition.
Bronchial hypersensitivity, also known as bronchial hyperresponsiveness or bronchial hyperreactivity, is a crucial feature of asthma. People with asthma have airways with an increased sensitivity to certain triggers.
Triggers are normally harmless. However, in someone with asthma, their airways are hypersensitive and become irritated. This hyperresponsiveness leads to increased mucus production and constriction or narrowing of the airways.
Additionally, some people react to environmental triggers that cause an allergic reaction, leading to asthma symptoms. About 80% of children with asthma are sensitive to environmental allergens.
Common allergens that cause hyperresponsive or hypersensitive airways include:
Airflow obstruction, which occurs due to a reduction in the diameter of the airways, also develops as part of the asthma process.
The airways become narrow due to the activation and release of immune cells, such as eosinophils, neutrophils, and mast cells. Inflammation develops as a response to the release of the cells, leading to the airways swelling.
Once this inflammatory response develops, it causes a cascade of adverse effects on the airways. For instance, it causes the airway's smooth muscle to contract, leading to bronchoconstriction. The inflammation also causes excess mucus production in the airways, possibly forming a mucus plug.
When persistent airflow limitation is not fully reversible, experts may refer to it as asthma with fixed airflow obstruction.
Acute asthma is reversible, which means treatment may reverse airway changes, such as inflammation and narrowing. However, in some situations, the airway changes are no longer reversible.
People with chronic asthma with recurrent asthma attacks may develop airway remodeling. With repeated asthma attacks, a prolonged immune response, and chronic inflammation, irreversible changes to the airways may occur.
Structural changes to the walls of the airways may develop. The makeup of the extracellular matrix, which is a mixture of fibrous proteins, also changes.
Airway remodeling may include:
Experts associate an increase in airway smooth muscle with decreased lung function in people with severe asthma.
Doctors break down asthma symptoms into two main phases: the early and late phases. The early phase of asthma occurs when the immune system detects an allergen. Because the airways are hypersensitive in someone with asthma, this response triggers the release of immunoglobulin E (IgE) antibodies.
In turn, IgE attaches itself to certain types of white blood cells, such as basophils and mast cells. The mast cells then release prostaglandins, histamine, and leukotrienes. These cells cause the smooth muscle in the airways to contract or narrow, causing bronchospasm, which triggers an asthma attack.
The late phase is the second phase of asthma. If left untreated, the late phase of an asthma attack occurs over the next few hours after the initial response to the allergen.
The cells released by the immune system, including eosinophils, helper T cells, and mast cells, travel to the airways and increase inflammation and constriction. This leads to airflow obstruction, making it harder to breathe. The immune system response also stimulates the goblet cells, producing excess mucus.
The first step in asthma treatment involves determining the triggers and assessing the severity of symptoms and frequency of flare-ups.
Treatment for asthma should recognize the three mechanisms that develop during an asthma attack. These include:
Doctors may prescribe a combination of medications to treat the different physiological factors that develop in an asthma attack. For instance, medications called bronchodilators help relax the smooth muscles of the airways, allowing them to dilate or widen.
Steroids help treat inflammation, which also reduces airflow obstruction. Medications are also available that inhibit eosinophils, such as monoclonal antibody therapy.
The best treatment plan may depend on a person's type of asthma, severity of symptoms, and response to initial treatment.
The pathophysiology of asthma is how the disease affects the normal function of the airways. It includes hypersensitivity of the airways, airflow obstruction, and reversibility.
However, in some people, if asthma remains untreated and frequent attacks develop, the inflammation can cause structural changes in the airways. Developing airway remodeling, such as thickening and stiffening of the airway smooth muscle, can further cause airflow obstruction.
Asthma treatment typically addresses both constriction and inflammation to decrease airflow obstruction.
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