
Several years ago, before we matched with Amigrace’s birthmother, we tried to adopt a new born with Cystic fibrosis. We did quite a bit of research on this life threatening disorder and even found out where our local support groups met. We understood that the disease causes severe lung damage and nutritional deficiencies but we were committed. Therefore, we were quite disappointed when another family was chosen to adopt the baby girl. However, God had another plan for us so that one wasn’t meant to be. Sometimes that is hard to accept in adoption especially when you have waited so long for a baby.
Cystic Fibrosis (CF) is hereditary condition. It affects the cells that produce mucus, sweat, saliva, and digestive juices. These secretions are normally thin and slippery, but with cystic fibrosis, a defective gene causes them to become thick and sticky. The secretions are supposed to act as a lubricant, but instead they plug up tubes, ducts, and passageways, especially in the pancreas and lungs. The most dangerous consequence of CF is respiratory failure. However, CF also prevents the body from absorbing key fat-soluble vitamins A, D, E, and K by having the thick sticky secretions block the pancreatic enzymes that help digest fats and proteins. The target of treatments for cystic fibrosis is relieving symptoms and complications.
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Specific signs and symptoms vary with the severity of the CF disease. One child may have respiratory problems but not digestive problems, while another child with CF may have both. The signs and symptoms of cystic fibrosis may vary with the child’s age. However, one of the first symptoms people may notice is salty tasting skin when they kiss their child. People with cystic fibrosis tend to have higher than normal amounts of salt (sodium chloride) in their sweat
Newborns may have a blockage in their intestines (meconium ileus). This occurs when the tarry, greenish-black stools that an infant normally passes a day or two after birth becomes so thick it can't move through the intestines. Newborns may also fail to grow, have bulky greasy stools, and frequent respiratory infections.
These same symptoms may also occur in older children along with coughing or wheezing, thick sputum, chest or sinus infections with recurring pneumonia or bronchitis. Other symptoms in older children are a protrusion of part of the rectum through the anus and enlargement or rounding (clubbing) of the fingertips and toes.
Children need to inherit two copies of the recessive gene that causes CF, one from each parent, in order to have the disease. If children inherit only one copy, they will be carriers of CF and possibly pass the gene to their own children but won't develop cystic fibrosis themselves. Carriers are usually healthy with no symptoms so it is possible to be a carrier.
A family history of the disease is the greatest risk factor for cystic fibrosis. Having Northern European ancestry also increases your risk to a one in 29 chance of carrying the gene. Hispanics have a one in 46 chance, black Americans have a one in 65 chance, and Asian Americans have a one in 90 chance of carrying the gene.
Men with cystic fibrosis are usually infertile because thick secretions often block the tube connecting the testes and prostate gland. Women with CF are often less fertile than other women.
Photo Credit: The official Web site of the Centers for Disease Control and Prevention (CDC). It is a public domain Web site, which means you may link to CDC.gov at no cost and without specific permission.