What is cystic fibrosis?

Cystic fibrosis (SIS-tik fi-BRO-sis), or CF, is an inherited disease of your secretory glands, including the glands that make mucus and sweat. "Inherited" means that the disease is passed through the genes from parents to children. People who have cystic fibrosis inherit two faulty cystic fibrosis genesone from each parent. The parents likely don't have the disease themselves. Cystic fibrosis mostly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs.
Mucus is a substance made by the lining of some body tissues. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. However, if you have cystic fibrosis, your mucus becomes thick and sticky.

The mucus builds up in your lungs and blocks your airwaysthe tubes that carry air in and out of your lungs. The buildup of mucus makes it easy for bacteria to grow. This leads to repeated, serious lung infections. Over time, these infections can severely damage your lungs. The thick, sticky mucus also can block tubes, or ducts, in your pancreas. As a result, the digestive enzymes that your pancreas makes can't reach your small intestine. These enzymes help break down the food that you eat. Without them, your intestines can't fully absorb fats and proteins. This can cause vitamin deficiency and malnutrition because nutrients leave your body unused. It also can cause bulky stools, intestinal gas, a swollen belly from severeconstipation, and pain or discomfort. Cystic fibrosis also causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. This can upset the balance of minerals in your blood and cause a number of health problems. Examples include dehydration (a condition in which your body doesn't have enough fluids), increased heart rate, tiredness, weakness, decreased blood pressure, heat stroke, and, rarely, death. If you or your child has cystic fibrosis, you're also at increased risk fordiabetes or a bone-thinning condition called osteoporosis. Cystic fibrosis also causes infertility in men, and it can make it harder for women to get pregnant.

What causes cystic fibrosis?

A defect in the CFTR gene causes cystic fibrosis (CF). This gene makes a protein that controls the movement of salt and water in and out of your body's cells. In people who have cystic fibrosis, the gene makes a protein that doesn't work right. This causes thick, sticky mucus and very salty sweat. Research suggests that the CFTR protein also affects the body in other ways. This may help explain other symptoms and complications of cystic fibrosis.

More than a thousand known defects can affect the CFTR gene. What type of defect you or your child has may influence how severe cystic fibrosis is. Other genes also may play a role in how severe the disease is.

Is cystic fibrosis inherited?

Every person inherits two CFTR genesone from each parent. Children who inherit a faulty CFTR gene from each parent will have cystic fibrosis. Children who inherit a faulty CFTR gene from one parent and a normal CFTR gene from the other parent will be "CF carriers." Cystic fibrosis carriers usually have no symptoms of cystic fibrosis and live normal lives. However, carriers can pass the faulty CFTR gene on to their children. The following image shows how two parents who are both cystic fibrosis carriers can pass the faulty CFTR gene to their children.

The image shows how CFTR genes are inherited. A person inherits two copies of the CFTR geneone from each parent. If each parent has a normal CFTR gene and a faulty CFTR gene, each child has a 25 percent chance of inheriting two normal genes; a 50 percent chance of inheriting one normal gene and one faulty gene; and a 25 percent chance of inheriting two faulty genes.

Who is at risk for cystic fibrosis?

About 30,000 people in the United States have cystic fibrosis (CF). Cystic fibrosis is one of the most common inherited diseases among Caucasians. About 1,000 new cases of cystic fibrosis are diagnosed each year. Cystic fibrosis affects both males and females and people from all racial and ethnic groups. However, the disease is most common among Caucasians of Northern European descent. Cystic fibrosis also is common among Latinos and Native Americans, especially the Pueblo and Zuni. The disease is much less common among African Americans and Asian Americans. About 12 million Americans are carriers of a faulty cystic fibrosis gene. Many of them don't know that they're cystic fibrosis carriers.

What are the signs and symptoms of cystic fibrosis?

The symptoms of cystic fibrosis vary from person to person and over time. Sometimes you will have few symptoms. Other times, your symptoms may become more severe. One of the first signs of cystic fibrosis (CF) that parents may notice is that their baby's skin tastes salty when kissed or the baby doesn't pass stool when first born. Most of the other signs and symptoms of cystic fibrosis develop later. They are related to how cystic fibrosis affects the respiratory, digestive, or reproductive systems of the body.

Figure A shows the organs that cystic fibrosis can affect. Figure B shows a cross-section of a normal airway. Figure C shows an airway affected by cystic fibrosis. The widened airway is blocked by thick, sticky mucus containing blood and bacteria.

Respiratory System Signs and Symptoms

People who have cystic fibrosis have thick, sticky mucus that builds up in their airways. This buildup of mucus makes it easier for bacteria to grow and cause infections. Infections can block the airways and cause frequent coughing that brings up thick sputum (spit) or mucus that's sometimes bloody. People who have cystic fibrosis tend to have lung infections caused by unusual germs that don't respond to standard antibiotics. For example, lung infections due to bacteria called mucoid Pseudomonas are much more common in people who have cystic fibrosis. An infection caused by this bacteria may be a sign of cystic fibrosis. People who have cystic fibrosis have frequent bouts of sinusitis, an infection of the air-filled spaces behind your eyes, nose, and forehead. Frequent bouts of bronchitis (bron-KI-tis) and pneumonia (nu-MO-ne-ah) also occur. These infections can cause long-term lung damage. As cystic fibrosis gets worse, you may develop more serious complications, such as pneumothorax (noo-mo-THOR-aks), or collapsed lung; orbronchiectasis (brong-ke-EK-ta-sis). Some people who have cystic fibrosis also develop nasal polyps (growths in the nose) that may require surgery.

Digestive System Signs and Symptoms

Mucus that blocks tubes, or ducts, in your pancreas and prevents enzymes from reaching your intestines causes most digestive system signs and symptoms. Without these enzymes, your intestines can't fully absorb fats and proteins. This can cause ongoing diarrhea or bulky, foul-smelling, greasy stools. Intestinal blockage also may occur, especially in newborns. Too much gas or severe constipation in the intestines may cause stomach pain and discomfort. A hallmark of cystic fibrosis in children is poor weight gain and growth. These children are unable to get enough nutrients from their food due to the lack of enzymes to help absorb fats and proteins. As cystic fibrosis gets worse, other complications may occur, such as:

Pancreatitis (PAN-kre-a-TI-tis). This is a condition in which the pancreas become inflamed, which causes pain. Rectal prolapse. Frequent coughing or problems passing stools may cause rectal tissue from inside you to move out of your rectum. Liver disease due to inflamed or blocked bile ducts.

Diabetes. Gallstones.

Reproductive System Signs and Symptoms

Men who have cystic fibrosis are infertile because they're born without a vas deferens. This is the tube that delivers sperm from the testicle to the penis. A woman who has cystic fibrosis may have a hard time getting pregnant because of mucus blocking her cervix or other cystic fibrosis complications.

Other Signs, Symptoms, and Complications

Other signs and symptoms of cystic fibrosis are related to an upset of the balance of minerals in your blood. Cystic fibrosis causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. This can cause dehydration (a condition in which your body doesn't have enough fluids), increased heart rate, tiredness, weakness, decreased blood pressure, heat stroke, and, rarely, death. Cystic fibrosis also can cause clubbing and low bone density. Clubbing is the widening and rounding of the tips of your fingers and toes. It develops late in cystic fibrosis because your lungs aren't moving enough oxygen into your bloodstream. Low bone density also tends to occur late in cystic fibrosis. It can lead to a bone-thinning disorder called osteoporosis

How is cystic fibrosis diagnosed?

Doctors diagnose cystic fibrosis (CF) based on the results from various tests.

Newborn Screening
Most States screen newborns for cystic fibrosis using a genetic test or a blood test. The genetic test shows whether a newborn has faulty CFTR genes. The blood test shows whether a newborn's pancreas is working.

Sweat Test
If a genetic test or blood test suggests cystic fibrosis, a doctor will confirm a diagnosis using a sweat test. This test is the most useful test for diagnosing cystic fibrosis. It measures the amount of salt in sweat. For this test, doctors trigger sweating on a small patch of skin on an arm or leg. They rub the skin with a sweat-producing chemical and then use an electrode to provide a mild electrical current. This may cause a tingling or warm feeling.

Sweat is collected on a pad or paper and then analyzed. The sweat test usually is done twice. High salt levels confirm a diagnosis of cystic fibrosis.

Other Tests
If you or your child has cystic fibrosis, your doctor may recommend other tests, such as:

Genetic tests to find out what type of CFTR defect is causing your cystic fibrosis. A chest x ray. This painless test creates pictures of the structures in your chest, such as your heart and lungs. A chest x ray can show whether your lungs are inflamed or scarred or whether they trap air. A sinus x ray. This test may show signs of sinusitis, a complication of cystic fibrosis. Lung function tests. These tests measure the size of your lungs, how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood. A sputum culture. For this test, your doctor will take a sample of your sputum (spit) to see what bacteria are growing in it. If you have bacteria called mucoid Pseudomonas, you may have more advanced cystic fibrosis that needs aggressive treatment.

Prenatal Screening
If you're pregnant, prenatal genetic tests can find out whether your fetus has cystic fibrosis. These tests include amniocentesis (AM-ne-o-sen-TE-sis) and chorionic villus (ko-re-ON-ik VILus) sampling (CVS). In amniocentesis, your doctor inserts a hollow needle through your abdominal wall into your uterus. He or she removes a small amount of fluid from the sac around the baby. The fluid is tested to see whether both of the baby's CFTR genes are normal. In CVS, your doctor threads a thin tube through the vagina and cervix to the placenta. The doctor removes a tissue sample from the placenta using gentle suction. The sample is tested to see whether the baby has cystic fibrosis.

Cystic Fibrosis Carrier Testing

People who have one normal CFTR gene and one faulty CFTR gene are cystic fibrosis carriers. Cystic fibrosis carriers usually have no symptoms of cystic fibrosis and live normal lives. However, carriers can pass faulty CFTR genes on to their children. If you have a family history of cystic fibrosis or a partner who has cystic fibrosis (or a family history of it) and you're planning a pregnancy, you may want to find out whether you're a cystic fibrosis carrier.

A genetics counselor can test a blood or saliva sample to see whether you have a faulty cystic fibrosis gene. This type of testing can detect faulty cystic fibrosis genes in 9 out of 10 cases.

How is cystic fibrosis treated?

Cystic fibrosis (CF) has no cure. However, treatments have greatly improved in recent years. The goals of cystic fibrosis treatment are to:

Prevent and control lung infections Loosen and remove thick, sticky mucus from the lungs Prevent or treat blockages in the intestines Provide enough nutrition Prevent dehydration (a condition in which the body doesn't have enough fluids)

Depending on how severe the disease is, you or your child may be treated in a hospital.

Specialists Involved
If you or your child has cystic fibrosis, you may be treated by a cystic fibrosis specialist. This is a doctor who is familiar with the complex nature of cystic fibrosis. Often, a cystic fibrosis specialist works with a medical team of nurses, physical therapists, dietitians, and social workers. Cystic fibrosis specialists often are located at major medical centers.

Treatment for Lung Problems

The main treatments for lung problems in people who have cystic fibrosis are chest physical therapy (CPT), exercise, and medicines. Chest Physical Therapy CPT also is called chest clapping or percussion. It involves pounding your chest and back over and over with your hands or a device to loosen the mucus from your lungs so that you can cough it up. You might sit down or lie on your stomach with your head down while you do CPT. Gravity and force help drain the mucus from your lungs. Some people find CPT hard or uncomfortable to do. Several devices have been developed that may help with CPT, such as:

An electric chest clapper, known as a mechanical percussor.

An inflatable therapy vest that uses high-frequency airwaves to force the mucus that's deep in your lungs toward your upper airways so you can cough it up. A small handheld device that you breathe out through. It causes vibrations that dislodge the mucus. A mask that creates vibrations that help break the mucus loose from your airway walls.

Breathing techniques also may help dislodge mucus so you can cough it up. These techniques include forcing out a couple of short breaths or deeper breaths and then doing relaxed breathing. This may help loosen the mucus in your lungs and open your airways. Exercise Aerobic exercise that makes you breathe harder helps loosen the mucus in your airways so you can cough it up. Exercise also helps improve your overall physical condition. However, cystic fibrosis causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. Thus, your doctor may recommend a high-salt diet or salt supplements to maintain the balance of minerals in your blood. If you exercise regularly, you may be able to cut back on your CPT. However, you should check with your doctor before doing this. Medicines If you have cystic fibrosis, you doctor may prescribe antibiotics, anti-inflammatory medicines, bronchodilators, or mucus-thinning medicines. These medicines help treat or prevent lung infections, reduce swelling, open up the airways, and thin mucus. Antibiotics are the main treatment to prevent or treat lung infections. Your doctor may prescribe oral, inhaled, or intravenous (IV) antibiotics. Oral antibiotics often are used to treat mild lung infections. Inhaled antibiotics may be used to prevent or control infections caused by the bacteria mucoid Pseudomonas. For severe or hardto-treat infections, you may be given antibiotics through a tube inserted into a vein. This type of treatment may require you to stay in the hospital. Anti-inflammatory medicines can help reduce swelling in your airways that's caused by ongoing infections. These medicines may be inhaled or oral. Bronchodilator medicines help open the airways by relaxing the muscles around them. These medicines are inhaled and often are taken just before CPT to help clear out mucus. You also may take bronchodilators before inhaling other medicines into your lungs.

Your doctor may prescribe mucus thinners to reduce the stickiness of your mucus and to loosen it up. These medicines can help clear out mucus, improve lung function, and prevent worsening lung symptoms.

Treatments for Advanced Lung Disease

If you have advanced lung disease and the level of oxygen in your blood is low, you may need oxygen therapy. Oxygen usually is given through nasal prongs or a mask. If other treatments haven't worked, lung transplant may be an option if you have severe lung disease. A lung transplant is surgery to remove a person's diseased lung and replace it with a healthy lung from a deceased donor.

Treatment for Digestive Problems

Cystic fibrosis can cause a number of digestive problems, including poor growth and development, bulky stools, intestinal gas, a swollen belly, severe constipation, and pain or discomfort. Nutritional therapy can improve your strength and ability to stay active. It also can improve growth and development in children. Nutritional therapy also may make you strong enough to resist some lung infections. A nutritionist can help you create a nutritional plan that meets your needs. In addition to having a well-balanced diet that's rich in calories, fat, and protein, your nutritional therapy may include:

Oral pancreatic enzymes to help you digest fats and proteins and absorb more vitamins. Supplements of vitamins A, D, E, and K to replace the fat-soluble vitamins that your intestines can't absorb. High-calorie shakes to provide you with additional nutrients. A high-salt diet or salt supplements that you take before doing vigorous exercise. A feeding tube to give you more calories at night while you're sleeping. The tube may be threaded through your nose and throat and into your stomach. Or, it may be placed directly into your stomach through a surgically made hole. Before you go to bed each night, you will attach a bag with a nutritional solution to the entrance of the tube. It will feed you while you sleep.

Other treatments for digestive problems may include enemas and mucus-thinning medicines to treat intestinal blockages. Sometimes surgery is needed to remove an intestinal blockage. Your doctor also may prescribe medicines to reduce your stomach acid and help oral pancreatic enzymes work better.

Treatments for Cystic Fibrosis Complications

A common complication of cystic fibrosis is diabetes. The type of diabetes that people who have cystic fibrosis develop often requires different treatment than other types of diabetes. Another common complication is the bone-thinning disorder osteoporosis. Your doctor may prescribe medicines that prevent your bones from losing their density.

Living with cystic fibrosis

If you or your child has cystic fibrosis (CF), you should learn as much as you can about the disease. Work closely with your doctors to learn how to manage cystic fibrosis.

Ongoing Care
Having ongoing medical care by a team of doctors, nurses, and respiratory therapists who specialize in cystic fibrosis is important. These specialists often are located at major medical centers. It's standard to have cystic fibrosis checkups every 3 months. Talk to your doctor about whether you should get an annual flu shot and other vaccines. Take all of your medicines as your doctor prescribes. In between checkups, be sure to contact your doctor if you have:

Blood in your mucus, increased amounts of mucus, or a change in the color or consistency of your mucus. Decreased energy or appetite. Severe constipation or diarrhea, severe abdominal pain, or vomit that's dark green. A fever, which is a sign of infection. (However, you may still have a serious infection that needs treatment even if you don't have a fever.)

Lifestyle Measures
In between medical checkups, you can practice good self-care and follow a healthy lifestyle. An important part of a healthy lifestyle is following a healthy diet. A healthy diet includes a variety of fruits, vegetables, and whole grains. Talk to your doctor about what types and amounts of foods you should include in your diet. Other lifestyle measures include:

Not smoking and avoiding tobacco smoke Washing your hands often to lower your risk for infection Exercising regularly and drinking lots of fluids

Doing chest physical therapy (as your doctor recommends)

Other Concerns
Although cystic fibrosis requires daily care, most people who have the disease are able to attend school and work. Adults who have cystic fibrosis can expect to have a normal sex life. Most men who have the disease are infertile. However, modern reproductive techniques may help them. Men who have cystic fibrosis should still have protected sex to avoid sexually transmitted diseases. Women who have cystic fibrosis may find it hard to get pregnant, but they usually can have children. If you have cystic fibrosis, you should talk to your doctor if you're planning a pregnancy. If your child has cystic fibrosis, encourage him or her to learn about the disease and take an active part in his or her treatment.

Emotional Issues
Living with a chronic disease may cause fear, anxiety, depression, andstress. It's important to talk about how you feel with your health care team. Talking to a professional counselor also can help. If you're feeling very depressed, your health care team or counselor may prescribe medicines to make you feel better. Joining a patient support group may help you adjust to living with cystic fibrosis. You can see how other people who have the same symptoms have coped with them. Talk to your doctor about local support groups or check with an area medical center. Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.

What is the outlook for cystic fibrosis?

The symptoms and severity of cystic fibrosis vary from person to person. Some people who have cystic fibrosis have serious lung and digestive problems. Other people have more mild disease that doesn't show up until they're adolescents or adults. The symptoms and severity of cystic fibrosis also vary over time. Sometimes, you will have few symptoms. Other times, your symptoms may become more severe. As the disease gets worse, you will have more severe symptoms more often. Lung function often starts to decline in early childhood in people who have cystic fibrosis. Over time, permanent damage to the lungs can cause severe breathing problems. Respiratory failure is the most common cause of death in people who have cystic fibrosis.

As treatments for cystic fibrosis continue to improve, so does life expectancy for those who have the disease. Today, some people who have cystic fibrosis are living into their forties, fifties, or older. Early treatment for cystic fibrosis can improve both your quality of life and lifespan. Such early treatment includes nutritional and respiratory therapies, medicines, exercise, and other treatments.

Cystic Fibrosis At A Glance

Cystic fibrosis (CF) is an inherited disease of your secretory glands, including your mucus and sweat glands. Cystic fibrosis mostly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs. It doesn't affect the brain. If you have cystic fibrosis, your mucus becomes thick and sticky. It builds up in your lungs and blocks your airways. This leads to repeated, serious lung infections that can damage your lungs. Lung function often starts to decline in early childhood in people who have cystic fibrosis. Over time, permanent damage to the lungs can cause severe breathing problems. The thick, sticky mucus also can block tubes, or ducts, in your pancreas. As a result, the digestive enzymes that your pancreas makes can't reach your small intestine. Without these enzymes, your body can't absorb fats and proteins. This can cause vitamin deficiency and malnutrition. Cystic fibrosis also causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. This can upset the balance of minerals in your body and cause a number of health problems. Examples include dehydration, increased heart rate, tiredness, weakness, decreased blood pressure, heat stroke, and, rarely, death. A defect in the CFTR gene causes cystic fibrosis. This gene makes a protein that controls the movement of salt and water in and out of your body's cells. Every person inherits two CFTR genesone from each parent. Children who inherit a faulty gene from each parent will have cystic fibrosis. Children who inherit one faulty gene and one normal gene will be "CF carriers." Cystic fibrosis carriers usually have no symptoms of cystic fibrosis, but they can pass the faulty gene on to their children. About 30,000 people in the United States have cystic fibrosis. It is one of the most common inherited diseases among Caucasians. About 1,000 new cases of cystic fibrosis are diagnosed each year. The symptoms of cystic fibrosis vary from person to person and over time. Sometimes, you will have few symptoms. Other times, your symptoms may become more severe. Doctors diagnose cystic fibrosis based on the results from various tests. Most States screen newborns for cystic fibrosis.

Cystic fibrosis has no cure. However, treatments have greatly improved in recent years. Treatment may include nutritional and respiratory therapies, medicines, exercise, and more. Early treatment for cystic fibrosis can improve both your quality of life and your lifespan. If you or your child has cystic fibrosis, you should learn as much as you can about the disease. Ongoing care and lifestyle measures can help you manage the disease. As treatments for cystic fibrosis continue to improve, so does life expectancy for those who have the disease. Today, some people who have cystic fibrosis are living into their forties, fifties, or older.

In-Depth

We understand that you may have a lot of questions when your child is diagnosed with cirrhosis. What exactly is it? What are the causes of cirrhosis? What are potential complications in my childs case? What are the treatments? What are possible side effects from treatment? How will it affect my child long term? Weve tried to provide some answers to those questions here, and when you meet with our experts, they can explain your childs condition and treatment options fully. What is the liver, and what does it do? The liver is the bodys second largest organ, located in the right side of the abdominal cavity below the diaphragm and above the right kidney and intestines. The liver helps the body in hundreds of ways: All of the blood coming from the stomach and intestines passes through the liver through a large vein called the portal vein. The liver turns nutrients from the food we eat and chemicals from the medicines we take into forms that the rest of our bodies can use. The liver helps clean the bloodstream of harmful substances and poisons. The liver makes bile, which contains chemicals to help us digest the food we eat. The liver helps control blood sugar and cholesterol levels The liver makes the proteins that allow blood to clot normally. What is cirrhosis? While the liver has an amazing capacity to heal itself if damaged by illness or injury, in the case of long term damage the liver may become scarred. The most severe form of scarring is called cirrhosis. What happens to the liver in cirrhosis? The normal healthy liver has a smooth, shiny surface. Over time, as the liver tries to recover from long-term illness or injury, hard scar tissue can replace the livers healthy tissue. When this happens, blood cannot flow through the liver as easily and the liver cannot work as well. Once it has started, if the damage continues, the scar tissue in the liver will gradually replace the livers healthy tissue, and the organ can actually start to shrink and take on a shriveled appearance. When the scarring (also called fibrosis) gets to the point of causing nodules instead of a soft, smooth liver, it is called cirrhosis.

Causes
What causes cirrhosis? Because cirrhosis is always linked to some kind of long-term disease or injury to the liver, it can be caused by a large number of disorders. In adults, chronic alcohol ingestion, viral hepatitis, and fatty liver are the most common causes. In children, biliary tract disorders and genetic conditions top the list of causes:

Biliary atresia, a condition that occurs in the first month or two of life, a blockage of the ducts that bring bile from the liver to the gallbladder and intestines. Autoimmune hepatitis, in which the bodys immune system turns against the liver Cystic fibrosis, an inherited chronic disease in which the body produces unusually thick, sticky secretions. Alpha-1 antitrypsin deficiency, a genetic disease that prevents the liver from building a protein it normally releases into the bloodstream. Chronic viral hepatitis caused by the viruses hepatitis B or C. These viruses cause the liver to swell, which can encourage scar tissue to form. Primary sclerosing cholangitis, an acquired condition which causes inflammation and narrowing of the bile ducts inside and/or outside of the liver Wilson disease, an uncommon inherited disorder in which the body cannot rid itself of excess copper found in many foods; the copper then builds up in the liver. Non-alcoholic fatty liver disease (NAFLD), a buildup of fat in the liver most commonly caused by obesity. Many children with NAFLD have a more serious condition called non-alcoholic steatohepatitis (NASH), which can lead to severe liver scarring. Some congenital (inherited) heart defects Other less common disorders While in adults overconsumption of alcohol is the most common cause of cirrhosis, it is important to stress that no amount of alcohol consumption by a parent, even during pregnancy, can cause a child to develop cirrhosis .

Signs and symptoms

What are the symptoms of cirrhosis? Cirrhosis itself generally produces no symptoms early on, though your child may experience symptoms related to the underlying medical condition that caused the liver damage. As it worsens, cirrhosis may cause: Loss of appetite Nausea or vomiting Weight loss or difficulty gaining weight Weakness Abdominal pain or swelling The appearance of spider-like blood vessels on the skin With time, cirrhosis may also lead to additional serious problems, including: A yellowing of the skin or the whites of the eyes (called jaundice) Bruising or bleeding easily, or nosebleeds Swelling of the legs or abdomen from build up of fluid. In the legs this fluid buildup is called edema; in the abdomen it is called ascites. Confusion or difficulty thinking (encephalopathy), caused by a buildup in the bloodstream of waste products from food. Failure of the kidneys to work properly Because in cirrhosis blood cannot flow as easily through the liver, the pressure in the vein entering your childs liver, called the portal vein, may increase, a condition called portal hypertension. This condition can cause its own symptoms and complications, and has its own treatments. The increased blood pressure in the portal vein can also affect how blood circulates in the lungs, causing conditions called hepatopulmonary syndrome and portopulmonary hypertension. How is cirrhosis diagnosed? Doctors usually make a diagnosis of cirrhosis based on a combination of symptoms, medical history, physical exam, and blood tests. In some cases, our doctors may order a liver biopsy to confirm the diagnosis, which will help them see the extent of the scarring in the liver. For details, see Tests. How do you treat cirrhosis? While in most cases there is no way to cure cirrhosis, our doctors will work with you and your child to control it and keep it from getting worse. They will also help understand and treat the underlying illness or damage that is causing the liver to scar. Cirrhosis can also raise your childs risk of developing liver cancer. And in advanced cases, cirrhosis can cause the liver to start to fail altogether. If that should happen, we may refer your child for a liver transplant. For details, see Treatment and Care. How can cirrhosis affect my child in the long term?

Because a damaged liver cannot break down medicines as quickly as if it were healthy, medicines including over-thecounter medicines and vitamin or herbal supplements may work more strongly than before, or may not be made into the active forms by the sick liver. For this reason, if your child has cirrhosis, you should always talk to his or her doctor before starting any new medications or supplements, even vitamins. Cirrhosis may make it harder for your child to gain or maintain his weight and healthy nutritional status. Cirrhosis may be an additional risk factor if your child has other medical problems or needs surgery. If your child has portal hypertension from cirrhosis, she may have more long-term effects. Cirrhosis is a chronic condition, and for this reason your child will likely have to seek care for it for the rest of his or her life. The Center for Childhood Liver Disease can help you and your child plan for the eventual transition from pediatric to adult care.