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Archive for the ‘Cystic Fibrosis’ Category

What is this condition?

A chronic heart condition, cor pulmonale is an enlargement of the right ventricle that results from various lung diseases, except those that primarily affect the left side of the heart, or congenital heart disease. Invariably, cor pulmonale follows some disorder of the lungs, pulmonary vessels, chest wall, or respiratory control center. For instance, chronic obstructive pulmonary disease produces pulmonary hypertension, which leads to right ventricular enlargement and failure. Because cor pulmonale generally occurs late during the course of chronic obstructive pulmonary disease and other irreversible diseases, the prognosis is generally poor.

What causes it?

Approximately 85% of people with cor pulmonale have chronic obstructive pulmonary disease. And 25% of people with chronic obstructive pulmonary disease eventually develop cor pulmonale.

Other respiratory disorders that produce cor pulmonale include:

- obstructive lung diseases, such as bronchiectasis and cystic fibrosis

- restrictive lung diseases, such as black lung and scleroderma

- loss of lung tissue after extensive lung surgery

- diseases of the lungs’ blood vessels

- respiratory insufficiency without lung disease, as seen in muscular dystrophy and amyotrophic lateral sclerosis

- obesity hypoventilation syndrome (pickwickian syndrome) and upper airway obstruction

- living at high altitudes (chronic mountain sickness).

To compensate for the extra work needed to force blood through the lungs, the right ventricle dilates and enlarges. Eventually, this leads to right ventricular failure. Cor pulmonale accounts for about 25% of all types of heart failure.

Cor pulmonale is most common in areas of the world where the incidence of cigarette smoking and chronic obstructive pulmonary disease is high; it affects middle-aged to elderly men more often than women, but incidence in women is increasing. In children, cor pulmonale may be a complication of cystic fibrosis, upper airway obstruction, scleroderma, extensive bronchiectasis, or other disorders.

What are its symptoms?

At first, symptoms reflect the underlying disorder and occur mostly in the respiratory system. They include chronic cough, shortness of breath on exertion, wheezing respirations, fatigue, and weakness. As the disease progresses, symptoms include shortness of breath (even at rest) that worsens on exertion, rapid breathing, swelling, weakness, and discomfort in the right upper abdomen. A chest exam reveals findings characteristic of the underlying lung disease.

Drowsiness and alterations in consciousness may occur.

How is it diagnosed?

Pulmonary artery pressure measurements show increased right ventricular and pulmonary artery pressures as a result of increased pulmonary vascular resistance. Other useful diagnostic tests include echo cardiography (ultrasound of the heart) or angiography, a chest X-ray, arterial blood gas analysis, electrocardiography, pulmonary function tests, and hematocrit.

How is it treated?

Treatment of cor pulmonale is designed to reduce oxygen deficiency, increase the persons exercise tolerance, and, when possible, correct the underlying condition.In addition to bed rest, treatment may include administration of:

- digitalis glycosides (such as Lanoxin)

- antibiotics when respiratory infection is present

- potent pulmonary artery vasodilators (such as Hyperstat, Nipride, Aresoline, angiotensin-converting enzyme inhibitors, calcium channel blockers, or prostaglandins) in primary pulmonary hypertension

- oxygen by mask or, in acute cases, by a mechanical ventilator

- low-salt diet, restricted fluid intake, and diuretics, such as Lasix, to reduce swelling

- phlebotomy to reduce the red blood cell count

- anticoagulation with small doses of Calciparine to reduce the risk of thromboembolism.

Incoming search terms:

Omega 3 fatty acids particularly DHA and EPA are good for the treatment of inflammatory diseases including cystic fibrosis. Because of this reason omega 3 and cystic fibrosis are very much related to each other.

Omega 3 fats obtained from fish oil are required for the prevention of many diseases. The most prominent of these diseases is cardiovascular disease. These fats reduce inflammation throughout the body and also control high cholesterol levels in the body. They are excellent for the cure of high blood pressure, arthritis, prostatitis, and gallstones.

The connection between omega 3 and cystic fibrosis can be established by the fact that omega 3 fatty acids provide relief in nearly all kinds of inflammatory diseases. Omega 3 long-chain fatty acid known by the name of ALA is of particular interest in this context since it is primarily obtained from flax seed oil which is good for people of all ages.

The other rich source of omega 3 fats is fish oil. Since fish oil in its raw form is not easy to intake because of its bad taste, fish oil capsules are normally used by people who want to receive optimal levels of these fats on a daily basis.

It has been found that people who eat fish or seafood regularly are at reduced risk of developing coronary heart disease, breast and prostate cancer, diabetes and Alzheimer’s disease. Polyunsaturated fats obtained from fish also prevent and cure prolonged depression and anxiety.

It is advisable to consume at least 300 mg of DHA and EPA on a daily basis. A single fish oil capsule provides up to 180 mg of EPA and 120 mg of DHA. You can consume 2 to 3 capsules every day, one after each meal to receive your daily required amount of these fats.

For maximum absorption of these nutrients by the bloodstream, try to take capsules after a gap of 4 to 5 hours. This will ensure proper absorption and will also keep a check on the balance of omega 3 and omega 6 fats in the body. The recommended ratio of these fats is three parts of omega 6 to one part of omega 3 fats.

Plant oils particularly hemp oil and olive oil provide excellent concentration of both types of fats. However, if you can digest fish oil then it is the best and most healthful source of omega 3 fats known to date.

Cystic fibrosis (also known as CF, mucovoidosis, or mucoviscidosis) is a hereditary terminal disease of the lungs and pancreas marked by severe coughing and malnutrition. It affects the exocrine (mucus) glands of the lungs, liver, pancreas, and intestines, causing progressive disability due to multisystem failure.

Thick mucus production results in frequent lung infections. Diminished secretion of pancreatic enzymes is the main cause of poor growth, fatty diarrhea, and deficiency in fat-soluble vitamins.

Often, symptoms of CF appear in infancy and childhood. Meconium ileus is a typical finding in newborn babies with CF. Individuals with cystic fibrosis can be diagnosed prior to birth by genetic testing. Newborn screening tests are increasingly common and effective (although false positives may occur, and children need to be brought in for a sweat test to distinguish disease vs carrier status).

Cystic fibrosis is one of the most common life-shortening, childhood-onset inherited diseases

In the US alone, about 1 in 4,000 are born with CF. It appears to be most common among western European populations and Ashkenazi Jews. That is to say about one in twenty-two people of Mediterranean descent are carriers of one gene for CF, making it the most common genetic disease in these populations. Incidentally, Ireland has the highest rate of CF carriers in the world, about 1 in 20.

The pancreas contains the islets of Langerhans, which are responsible for making insulin, a hormone that helps regulate blood glucose. Damage of the pancreas can lead to loss of the islet cells, leading to diabetes that is unique to those with the disease. Cystic Fibrosis Related Diabetes (CFRD), as it is known as, shares characteristics that can be found in Type 1 and Type 2 diabetics and is one of the principal non-pulmonary complications of CF. Vitamin D is involved in calcium and phosphorus regulation.

Poor intake of Vitamin D in the diet causes the bone disease Osteoporosis in which weakened bones are more vulnerable to fractures. Besides, people with CF also often develop clubbing of their fingers and toes due to the effects of chronic illness and low oxygen on their tissues.

Younger children with cystic fibrosis in the United States call their disease 65 Roses because the words are easier to pronounce. This trademarked phrase has been popularized by the Cystic Fibrosis Foundation.

Sixty-Five Roses, the book is the heart-wrenching, yet beautiful account of two sister’s love for each another as one sister battles daily for her life with cystic fibrosis. It seems to be a lifetime story that leaves no emotion in a safe harbor. Her life seems to be a daily struggle, crammed with treatments, hospitalizations, false starts and faint hope, setbacks and unfulfilled dreams. She communicates well the complicated feelings that long-term illness can breed in families.

From the few pages that I have read, I can assure you is that it is told with complete honesty and with a soft conscience. Although I haven’t got down to finishing it, it appears that it surely would be one of the most moving memoirs that I may have ever read.

Readers tell me that each page is breathtaking, pulsating and leaves you wanting to never stop reading as the story is fascinating from beginning to end.

I can quite understand the support and love in a family towards the person inflicted with cystic fibrosis and highlights the complicated feelings that long-term illness can breed in families. Like in any battle they live and fight it together and learn to triumph over it in as many ways. Must truly be one helluva journey.

It was nominated as the Globe and Mail Best 100 Books of 2006

Read this book: your life will never be the same claims the Storycircle Book Review.

Now that I have it, I must get down to finishing it one of these days.

Since 1965, the term “65 Roses” has been used by children of all ages to describe their disease. But, making it easier to say does not make CF any easier to live with. The “65 Roses” story has captured the hearts and emotions of all who have heard it. The rose, appropriately the ancient symbol of love, has become a symbol of the Cystic Fibrosis Foundation.