Islets of Hope disorders associated with diabetes
Edited by Lahle Wolfe
Additional resources used:
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Alex, The Life of a Child
Alexandra Deford, a precious and precocious girl, was just eight years old when she died in 1980 following a battle against the debilitating effects of cystic fibrosis, the number-one genetic killer of children. Her poignant and uplifting story touched the hearts of millions when it was first published and then made into a memorable television movie. A new introduction contains information on the latest cystic fibrosis research, and a touching postcript reveals how the Deford family came to terms with the loss of Alex.
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Key Points From NIH
"Cystic fibrosis (CF) is an inherited disease of your mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs.
"In CF, an abnormal gene called the cystic fibrosis transmembrane conductance regulator (CFTR) gene causes mucus to become thick and sticky. The mucus builds up in the lungs and blocks the airways, creating an environment that makes it easy for bacteria to grow. This leads to repeated serious lung infections that can damage your lungs.
"The mucus can also block tubes, or ducts, in your pancreas so that the digestive enzymes it produces cannot reach the intestines where they are needed to break down food.
"You have extremely salty sweat. When you perspire, your body loses large amounts of salt. This can upset the balance of minerals in your blood, which can cause a heat emergency.
"The most common symptoms of CF are frequent coughing with phlegm, frequent bouts of bronchitis and pneumonia, salty-tasting skin, dehydration, poor growth, and infertility, mostly in men.
"The sweat test is the most common diagnostic test for CF. It measures the amount of salt in your sweat.
"Other tests that can be used to help diagnose CF include a chest x ray, sinus x ray, lung function tests, analysis of sputum cultures and/or stool samples, and genetic testing of a blood sample.
"Prenatal genetic testing can help you find out if your baby is likely to have CF.
"Antibiotics are the primary treatment for lung problems in CF. They treat airway infections. Other treatments include chest physical therapy, exercise, mucus-thinning drugs, and other medications to reduce inflammation in your airways and help open them up.
"Lung transplantation is an option for some people with CF.
"The digestive problems in people with CF can be managed with nutritional therapy, enemas, mucus-thinning drugs, and medications to reduce stomach acid.
"Ongoing medical care from a team of health care providers who specialize in CF is important.
"Good self-management includes eating a healthy diet, avoiding tobacco smoke, exercising frequently, doing chest physical therapy every day, drinking lots of fluids, and washing your hands often to reduce your chances of infection."
Cystic Fibrosis - Part 1 of 2
What is Cystic Fibrosis (CF)?
CF is a chronic, genetic (hereditary), debilitating disease where the body over produces mucous. Excess mucous can clogged the pancreas contributing to the secondary onset of type 2 diabetes. CF affects approximately 30,000 children and adults in the U.S. It can lead to serous life-threatening lung infections because the thick mucous clogs the lungs. This mucous can also block ducts in the pancreas causing permanent damage that can lead to the onset of type 1 diabetes.
Cystic fibrosis related diabetes (CFRD) shares features of types 1 and 2 diabetes, but it is a unique and distinct form of diabetes that requires a special management approach.
Most persons with CF are diagnosed prior to age three, with approximately 1,000 new cases diagnosed each year.
Other names for cystic fibrosis are:
What Causes Cystic Fibrosis?
Cystic fibrosis (CF) is caused by a defect in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This gene makes a protein that controls the movement of salt and water in and out of the cells in your body. In people with CF, the gene does not work effectively. This causes the thick, sticky mucus and very salty sweat that are the main features of CF.
Each of us inherits two CFTR genes, one from each parent.
CF carriers usually have no symptoms of CF, live normal lives, but can pass the abnormal CFTR gene on to their children
When two CF carriers have a baby, the baby has a:
About 30,000 people in the United States have cystic fibrosis (CF):
What Are Symptoms of Cystic Fibrosis?
Most of the symptoms of cystic fibrosis (CF) are caused by the thick, sticky mucus. The most common symptoms include:
CF can also lead to other medical problems, including:
Tests and Diagnosis of CF
The sweat test is the most useful test for diagnosing cystic fibrosis (CF). It measures the amount of salt in your sweat. For this test, doctors rub a small amount of a chemical called pilocarpine (pi-lo-KAR-pen) on your arm or leg. They then attach an electrode to this spot. The electrode provides a mild electric current that produces sweat. This may cause tingling or a feeling of warmth. They then cover the area with a gauze pad or filter paper and wrap in plastic. After 30 to 40 minutes, they remove the plastic so the sweat that collected on the pad or paper can be analyzed. The test is usually done twice. High salt levels mean CF.
Your doctor may also do the following tests to understand more about your condition and how to treat it:
CF Carrier Testing
You may want to check whether you are a CF carrier, if:
A genetics counselor at your local hospital can take a blood or saliva sample to see if it contains the abnormal CFTR gene that causes CF. It will detect 9 out of 10 cases of CF. Some insurance plans cover genetic testing.
On to next section (treatment, management of digestive disorders, and living with CF)
Page Updated 03/17/2006