Different heart failure phenotypes of valvular heart disease: the role of mitochondrial dysfunction

Hereditary Diseases: Aerosol For Breathing

TIME

November 22, 1963 12:00 AM GMT-5

Though cystic fibrosis has had intense attention from medical researchers in the past few years, bedside doctors still find it bafflingly difficult to deal with the disease, which involves both the lungs and the digestive tract. It is not for lack of trying: they are using a dozen or more different drugs and other treatments. Now, Pediatrician Herman W. Reas of St. Louis Children's Hospital has found that boosting the patient's breathing efficiency twice a day with a new aerosol drug eases his distress and promotes his general health.

The victim of cystic fibrosis, almost invariably a child because the disease is usually fatal before adulthood, has an inherited enzyme defect that damages the oxygen-exchange cells in his lungs and reduces the elasticity of the lung walls. He does not breathe enough air in, nor let enough out. His windpipe and lungs become clogged with thick viscid mucus. The trick is to loosen and thin this mucus, and get rid of it.

Dr. Reas reports in the Southern Medical Journal that he used a new mist-making drug, N-acetylcysteine (trade named Mucomyst by Mead Johnson & Co.) on 28 patients aged 7 to 22. He clapped a face mask on his patients twice a day, before meals, and got them to inhale Mucomyst aerosol supplied under gentle pressure by a small pump. After 20 minutes, each bedridden child was turned into assorted head-down positions to help him spit out the mucus. Stronger children got rid of the mucus by taking a short but brisk run, which started them coughing.

Children with lung damage already far advanced received little benefit, but those with only moderate damage showed remarkable improvement after continuous treatment for two years or more. They could be far more active physically, they slept better (a boon to worn-out parents), they gained weight, and one grew three inches in 18 months as compared with only three-quarters of an inch in the preceding 18 months.

The Mucomyst spray is no cure, but it appears to be less irritating than previous aerosols. While its effects may be most dramatic in cystic fibrosis, the University of Mississippi's Dr. Watts R. Webb reports that the drug seems to be equally useful in other diseases in which mucus blocks breathing.

Understanding Idiopathic Pulmonary Fibrosis, Disease That Claimed Zakir Hussain's Life

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Simplifying... Inshort

Idiopathic Pulmonary Fibrosis (IPF), a disease more common in men and linked to age, smoking, and genetics, is a condition with symptoms like breathlessness, dry cough, and fatigue.

While there's no cure, treatments like medication, oxygen therapy, and even lung transplants can slow its progress and improve life quality.

Lifestyle changes, such as quitting smoking, healthy eating, regular exercise, and stress management, are also crucial in managing this disease.

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What's the story

Zakir Hussain, the legendary tabla maestro, passed away in San Francisco at the age of 73 on Sunday. He died due to idiopathic pulmonary fibrosis (IPF), a serious chronic lung disease. IPF is a disease that affects the tissues surrounding the air sacs or alveoli in the lungs, making them thick and stiff for unknown reasons. This causes permanent scarring, called fibrosis, which progressively makes breathing difficult.

Risk factors and symptoms of idiopathic pulmonary fibrosis

The risk of developing IPF increases with age, smoking habits, and family history. Men are more commonly affected by this disease than women. Genetic factors also play a significant role in the development of IPF, with mutations in certain genes like MUC5B increasing susceptibility to the disease. Symptoms of this condition include shortness of breath, prolonged dry cough, joint and muscle pain, fatigue, and unexplained weight loss.

Treatment options for idiopathic pulmonary fibrosis

Currently, there is no known cure for IPF. However, treatments are available that can slow lung damage and improve quality of life. Medications such as Nintedanib or pirfenidone may enhance lung function and prevent acute exacerbations. Other treatment options include oxygen therapy and ventilator support. In some cases, a lung transplant may be considered despite potential complications like infection or organ rejection.

Lifestyle changes recommended for managing idiopathic pulmonary fibrosis

Lifestyle modifications such as quitting smoking, maintaining a healthy diet, and regular exercise are recommended for managing IPF. Counseling and therapy can also help alleviate stress and anxiety associated with the disease. Current and past smokers, particularly those with emphysema, are at an increased risk. Furthermore, jobs that require prolonged exposure to pollutants, such as mining, farming, or construction, can increase vulnerability.

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