Hypotension (Low Blood Pressure)

What Is Hypotension?

Hypotension (HI-po-TEN-shun) is low blood pressure. Blood pressure is the force of blood pushing against the walls of the arteries as the heart pumps out blood.

Blood pressure is measured as systolic (sis-TOL-ik) and diastolic (di-a-STOL-ik) pressures. Systolic blood pressure is the pressure when the heart beats while pumping blood. Diastolic blood pressure is the pressure when the heart is at rest between beats.

You will most often see blood pressure numbers written with the systolic number above or before the diastolic, such as 120/80 mmHg. (The mmHg is millimeters of mercury-the units used to measure blood pressure.)

Normal blood pressure in adults is lower than 120/80 mmHg. Hypotension is blood pressure that's lower than 90/60 mmHg.
General idea

Blood pressure changes during the day. It lowers as you sleep and rises when you wake up. It also can rise when you're excited, nervous, or active.

Your body is very sensitive to changes in blood pressure. Special cells in the arteries can sense if your blood pressure begins to rise or fall. When this happens, the cells trigger your body to try to bring blood pressure back to normal.

For example, if you stand up quickly, your blood pressure may drop. The cells will sense the drop and will quickly take action to make sure that blood continues to flow to your brain, kidneys, and other important organs.

Most forms of hypotension happen because your body can't bring blood pressure back to normal or can't do it fast enough.

Some people have low blood pressure all of the time. They have no signs or symptoms, and their low blood pressure is normal for them. In other people, certain conditions or factors cause blood pressure to drop below normal.

Hypotension is a medical concern only if it causes signs or symptoms, such as dizziness, fainting, or, in extreme cases, shock.
Point of View

In a healthy person, low blood pressure without signs or symptoms usually isn't a problem and needs no treatment. If low blood pressure causes signs or symptoms, your doctor will try to find and treat the underlying condition that's causing it.
Hypotension can be dangerous. It can make a person fall because of dizziness or fainting. Shock, a severe form of hypotension, is a condition that's often fatal if not treated right away. With prompt and proper treatment, shock can be successfully treated.

Types of Hypotension

There are several types of hypotension. People who always have low blood pressure have chronic asymptomatic hypotension. They have no signs or symptoms and need no treatment. Their low blood pressure is normal for them.

Other types of hypotension occur only sometimes, when blood pressure suddenly drops too low. The symptoms and effects on the body range from mild to severe.

The three main types of this kind of hypotension are orthostatic (OR-tho-STAT-ik) hypotension, neurally mediated hypotension (NMH), and severe hypotension linked to shock.

Orthostatic Hypotension

This type of low blood pressure occurs when standing up from a sitting or lying down position. It can make you feel dizzy or lightheaded, or even make you faint.

Orthostatic hypotension occurs if your body isn't able to adjust blood pressure and blood flow fast enough for the change in position. This type of low blood pressure usually lasts for only a few seconds or minutes after you stand up. You may need to sit or lie down for a short time while your blood pressure returns to normal.

Orthostatic hypotension can occur in all age groups. However, it's more common in older adults, especially those who are frail or in poor health. It can be a symptom of other medical conditions, and treatment often focuses on treating the underlying condition(s).

Some people have orthostatic hypotension, but also have high blood pressure when lying down.

A form of orthostatic hypotension called postprandial hypotension is a sudden drop in blood pressure after a meal. This type of low blood pressure mostly affects older adults. It's also more likely to affect people who have high blood pressure or a central nervous system disorder, such as Parkinson's disease.
Neurally Mediated Hypotension

With NMH, blood pressure drops after you've been standing for a long time. You may feel dizzy, faint, or sick to the stomach as a result. This type of low blood pressure also can occur if you have an unpleasant, upsetting, or scary experience.

NMH affects children and young adults more often than people in other age groups. Children often outgrow NMH.
Severe Hypotension Linked to Shock

People may say a person has "gone into shock" as a result of an upsetting event. But to doctors, the word "shock" has a different meaning.

Shock is a life-threatening condition in which blood pressure drops so low that the brain, kidneys, and other vital organs can't get enough blood to work properly. Blood pressure drops much lower in shock than in other types of hypotension.

Many factors can cause shock, such as major blood loss, certain severe infections, severe burns and allergic reactions, and poisoning. Shock can be fatal if it's not treated right away.

Other Names for Hypotension

• Low blood pressure
• Neurally mediated hypotension
• Neurogenic orthostatic hypotension
• Orthostatic hypotension
• Postprandial hypotension
• Postural hypotension
• Shock

What Causes Hypotension?

Factors or conditions that disrupt the body's ability to control blood pressure cause hypotension. The different types of hypotension have different causes.

Orthostatic Hypotension

Orthostatic hypotension has many causes. Sometimes two or more factors combine to cause this type of low blood pressure.

Dehydration (de-hi-DRA-shun) is the most common cause of orthostatic hypotension. Dehydration occurs when the body loses more water than it takes in. You may become dehydrated if you don't drink enough fluids or if you sweat a lot during physical activity. Fever, vomiting, and severe diarrhea also can lead to dehydration.

Orthostatic hypotension may occur during pregnancy, but it generally goes away after the birth.

Because an older body doesn't manage changes in blood pressure as well as a younger body, getting older also can lead to this type of hypotension.

Postprandial hypotension (a type of orthostatic hypotension) mostly affects older adults. Postprandial hypotension is a sudden drop in blood pressure after a meal.

Certain medical conditions can raise your risk for orthostatic hypotension, including:

• Heart conditions, such as heart attack, heart valve disease, bradycardia (a very low heart rate), and heart failure. These conditions prevent the heart from pumping enough blood to the body.
• Anemia (uh-NEE-me-eh).
• Severe infections.
• Endocrine conditions, such as thyroid disorders, Addison's disease, low blood sugar, and diabetes.
• Disorders of the central nervous system, such as Parkinson's disease.
• Pulmonary embolism.

Some medicines used to treat high blood pressure and heart disease can raise your risk for orthostatic hypotension. These medicines include:

• Diuretics, or "water pills"
• Calcium channel blockers
• Angiotensin-converting enzyme (ACE) inhibitors
• Angiotensin II receptor blockers
• Nitrates
• Beta blockers

Medicines used to treat conditions such as anxiety, depression, erectile dysfunction, and central nervous system disorders (like Parkinson's disease) also can increase your risk for orthostatic hypotension.

Other substances, when taken with high blood pressure medicines, also can lead to orthostatic hypotension. These substances include alcohol, barbiturates, and some prescription and over-the-counter medicines.

Finally, other factors or conditions that can trigger orthostatic hypotension include being out in the heat or being immobile (not being able to move around very much) for a long time.

Neurally Mediated Hypotension

Neurally mediated hypotension (NMH) occurs when the brain and heart don't communicate with each other properly.

For example, when you stand for a long time, blood begins to pool in your legs. This causes your blood pressure to drop. In NHM, the body mistakenly tells the brain that blood pressure is high. In response, the brain slows the heart rate. This makes blood pressure drop even more, causing dizziness and other symptoms.

Severe Hypotension Linked to Shock.

Many factors and conditions can cause severe hypotension linked to shock. Some of these factors also can cause orthostatic hypotension. In shock, though, blood pressure drops very low and doesn't return to normal on its own.

Shock is an emergency and must be treated right away. If a person has signs or symptoms of shock.

Certain severe infections can cause shock. This is known as septic shock. It can occur when bacteria enter the bloodstream. The bacteria release a toxin (a poison) that leads to a dangerous drop in blood pressure.

A severe loss of blood or fluids from the body also can cause shock. This is known as hypovolemic (HI-po-vo-LE-mik) shock. Hypovolemic shock can happen as a result of:

• Major external bleeding (for example, from a severe cut or injury)
• Major internal bleeding (for example, from a ruptured blood vessel or injury that causes bleeding inside the body)
• Major loss of body fluids from severe burns
• Severe swelling of the pancreas (an organ that produces enzymes and hormones, such as insulin)
• Severe diarrhea
• Severe kidney disease
• Overuse of diuretics

A major decrease in the heart's ability to pump blood also can cause shock. This is known as cardiogenic (KAR-de-o-JEN-ik) shock. Heart attack, pulmonary embolism, or arrhythmia (an irregular heartbeat) can cause this type of shock.

A sudden and extreme widening of the arteries and drop in blood pressure also can cause shock. This is known as vasodilatory (VA-so-DI-la-tory) shock. It can occur due to:

• A severe head injury
• A reaction to certain medicines
• Liver failure
• Poisoning
• A severe allergic reaction (called anaphylactic (AN-a-fi-LAK-tik) shock)

Who Is At Risk for Hypotension?

Hypotension can affect people of all ages. However, people in certain age groups are more likely to have certain types of low blood pressure.

Older adults are more likely to have orthostatic and postprandial hypotension. Children and young adults are more likely to have neurally mediated hypotension.

People who take certain medicines, such as high blood pressure medicines, are at higher risk for low blood pressure. People who have central nervous system disorders (such as Parkinson's disease) or some heart conditions also are at higher risk for low blood pressure.

Other risk factors for hypotension include being immobile (not being able to move around very much) for long periods and pregnancy. Hypotension during pregnancy is normal and goes away after birth.

What Are the Signs and Symptoms of Hypotension?

Orthostatic Hypotension

The signs and symptoms of orthostatic hypotension may happen within a few seconds or minutes of standing up after you've been sitting or lying down. You may feel that you're going to faint, or you may actually faint. Signs and symptoms include:

• Dizziness or feeling lightheaded
• Blurry vision
• Confusion
• Weakness
• Nausea (feeling sick to your stomach)

These signs and symptoms go away if you sit or lie down for a few minutes until your blood pressure adjusts to normal.

Neurally Mediated Hypotension

The signs and symptoms of neurally mediated hypotension (NMH) are similar to those of orthostatic hypotension. They occur after standing for a long time or in response to an unpleasant, upsetting, or scary experience.

The drop in blood pressure with NMH doesn't last long and often goes away after sitting down.

Severe Hypotension Linked to Shock

In shock, not enough blood flows to the major organs, including the brain.

The early signs and symptoms of reduced blood flow to the brain include lightheadedness, sleepiness, and confusion. In the earliest stages of shock, it may be hard to detect any signs or symptoms. In older people, the first symptom may only be confusion.

Over time, as shock worsens, a person won't be able to sit up without passing out. If the shock continues, the person will lose consciousness. Shock is often fatal if not treated right away.

Other signs and symptoms of shock vary, depending on what's causing the shock. When low blood volume (from major blood loss, for example) or poor pumping action in the heart (from heart failure, for example) causes shock:

• The skin becomes cold and sweaty. It often looks blue or pale. If pressed, the color returns to normal more slowly than usual. A bluish network of lines appears under the skin.
• The pulse becomes weak and rapid.
• The person begins to breathe very quickly.

When extreme widening or stretching of blood vessels (such as in septic shock) causes shock, a person feels warm and flushed at first. Later, the skin becomes cold and clammy, and the person feels very sleepy.

How Is Hypotension Diagnosed?

Hypotension is diagnosed based on your medical history, a physical exam, and results from tests. Your doctor will want to know:

• The type of low blood pressure you have and how severe it is
• Whether an underlying condition is causing the low blood pressure

Specialists Involved

A primary care doctor or specialist may diagnose and treat hypotension. The type of specialist most commonly involved is a cardiologist (heart specialist).

Other specialists also may be involved, such as surgeons, nephrologists (kidney specialists), neurologists (brain and nerve specialists), or others.

Diagnostic Tests

When a person is in shock, someone should call 9-1-1 right away because emergency treatment is needed.

For other types of hypotension, your doctor may order tests to find out how your blood pressure responds in certain situations. The results will help your doctor understand why you're fainting or having other symptoms.

Blood Tests

During a blood test, a small amount of blood is taken from your body. It's usually drawn from a vein in your arm using a thin needle. The procedure is quick and easy, although it may cause some short-term discomfort.

Blood tests can show whether anemia or low blood sugar is causing your hypotension.

EKG (Electrocardiogram)

An EKG is a simple test that detects and records the heart's electrical activity. It shows how fast the heart is beating and the heart's rhythm (steady or irregular). An EKG also shows the strength and timing of electrical signals as they pass through each part of the heart.

Holter and Event Monitors

Holter and event monitors are medical devices that record the heart's electrical activity. These monitors are similar to an EKG. However, a standard EKG only records the heartbeat for a few seconds. It won't detect heart rhythm problems that don't occur during the test.

Holter and event monitors are small, portable devices. You can wear one while you do your normal daily activities. This allows the monitor to record your heart longer than an EKG can.

Echocardiography

Echocardiography is a test that uses sound waves to create a moving picture of your heart. The picture shows how well your heart is working and its size and shape.

There are several different types of echocardiography, including a stress echocardiogram, or "stress echo." This test is done as part of a stress test (see below). A stress echo usually is done to find out whether you have decreased blood flow to your heart, a sign of coronary artery disease.

Stress Test

Some heart problems are easier to diagnose when your heart is working hard and beating fast. During stress testing, you exercise (or are given medicine if you're unable to exercise) to make your heart work hard and beat fast while heart tests are done.

These tests may include nuclear heart scanning, echocardiography, and magnetic resonance imaging (MRI) and positron emission tomography (PET) scanning of the heart.

Valsalva Maneuver

This is a simple test of the part of your nervous system that controls functions such as your heartbeat and the narrowing and widening of your blood vessels. If something goes wrong with this part of the nervous system, blood pressure problems may occur.

During this test you take a deep breath and then force the air out through your lips. You will do this several times. Your heart rate and blood pressure will be checked during the test.

Tilt Table Test

This test is used if you have fainting spells for no known reason. For the test, you lie on a table that moves from a lying down to an upright position. Your doctor checks your reaction to the change in position.

Doctors use a tilt table test to diagnose orthostatic hypotension and neurally mediated hypotension (NMH). People who have NMH usually faint during this test. The test can help your doctor find any underlying brain or nerve condition.

How Is Hypotension Treated?

Treatment depends on the type of hypotension you have and how severe your signs and symptoms are. The goals of treatment are to relieve signs and symptoms and manage any underlying condition(s) causing the hypotension.

Your response to treatment depends on your age, overall health, and strength. It also depends on how easily you can stop, start, or change medicines.

In a healthy person, low blood pressure without signs or symptoms usually needs no treatment.

If you have signs or symptoms of low blood pressure, you should sit or lie down right away. Put your feet above the level of your heart. If your symptoms don't go away quickly, you should seek medical care right away.

Orthostatic Hypotension

There are a number of treatments for orthostatic hypotension. If you have this type of low blood pressure, your doctor may advise making lifestyle changes such as:

• Drinking plenty of fluids, like water
• Drinking little or no alcohol
• Standing up slowly
• Not crossing your legs while sitting
• Gradually sitting up for longer periods if you've been immobile (not able to move around much) for a long time due to a medical condition
• Eating small, low-carbohydrate meals if you have postprandial hypotension (a form of orthostatic hypotension)

Talk to your doctor about using compression stockings. These stockings apply pressure to your lower legs. They help move blood throughout your body.

If medicine is causing your low blood pressure, your doctor may change the medicine or adjust the dose you take.

Several medicines also are used to treat orthostatic hypotension. These medicines, which raise blood pressure, include fludrocortisone and midodrine.

Neurally Mediated Hypotension

If you have neurally mediated hypotension (NMH), you may need to make lifestyle changes. These may include:

• Avoiding situations that trigger symptoms. For example, don't stand for long periods. Try to avoid unpleasant, upsetting, or scary situations.
• Drinking plenty of fluids, like water.
• Increasing your salt intake (as your doctor advises).
• Learning to recognize symptoms that occur before fainting and taking action to raise blood pressure. For example, sitting down and putting your head between your knees can help raise blood pressure.

If medicine is causing your low blood pressure, your doctor may change the medicine or adjust the dose you take. He or she also may prescribe a medicine to treat NMH.

Children who have NHM often outgrow it.

Treating Severe Hypotension Linked to Shock

Shock is a life-threatening emergency. People who have shock usually need to be treated in a hospital or by emergency medical personnel.

The goals of treating shock are to:

• Restore blood flow to the organs as quickly as possible to prevent organ damage
• Find and reverse the cause of shock

Special fluid or blood injected into the bloodstream is often used to restore blood flow to the organs. Medicines can be used to raise blood pressure or make the heartbeat stronger. Depending on the cause of the shock, other treatments, such as antibiotics or surgery, may be needed.

Key Points

• Hypotension is low blood pressure. Blood pressure is the force of blood pushing against the walls of the arteries as the heart pumps out blood.
• Normal blood pressure in adults is lower than 120/80 mmHg. Hypotension is blood pressure that's lower than 90/60 mmHg.
• Some people have low blood pressure all the time. They have no signs or symptoms and their low readings are normal for them. In other people, certain conditions or factors cause blood pressure to drop below normal.
• In a healthy person, low blood pressure without signs or symptoms usually isn't a problem and needs no treatment.
• The three main types of hypotension that causes signs and symptoms are orthostatic hypotension, neurally mediated hypotension (NMH), and severe hypotension linked to shock.
• Orthostatic hypotension is low blood pressure that occurs after standing up from a sitting or lying down position. With NMH, blood pressure drops after you've been standing for a long time or due to severe emotional stress. Shock is a life-threatening condition in which blood pressure drops so low that the brain, kidneys, and other vital organs can't get enough blood to work properly. Many factors can cause shock, such as major blood loss, certain severe infections, severe burns and allergic reactions, and poisoning. Shock can be fatal if not treated right away.
• Factors or conditions that disrupt the body's ability to control blood pressure cause hypotension. The different types of hypotension have different causes.
• Hypotension can affect people of all ages. Older adults are more likely to have orthostatic and postprandial hypotension. Children and young adults are more likely to have NMH.
• The signs and symptoms of orthostatic hypotension and NMH include dizziness or feeling lightheaded, blurry vision, confusion, weakness, and nausea (feeling sick to your stomach). You may feel as though you're going to faint, or you may actually faint. Both of these types of hypotension can be dangerous if a person falls because of the dizziness or fainting.
• Signs and symptoms of shock include lightheadedness, sleepiness, and confusion. Over time, if shock worsens, a person won't be able to sit up without passing out. If the shock continues, the person can lose consciousness. Other signs and symptoms of shock include cold and sweaty skin, a weak and rapid pulse, and rapid breathing. If a person has signs or symptoms of shock, someone should call 9-1-1 right away.
• Hypotension is diagnosed based on your medical history, a physical exam, and results from tests.
• Treatment depends on the type of hypotension you have and how severe your signs and symptoms are. The goals of treatment are to relieve signs and symptoms and manage any underlying condition causing the hypotension. Treatments may include lifestyle changes, compression stockings, procedures, and medicines.
• Doctors often can successfully treat hypotension. Many people who have the disorder live normal, healthy lives.

(RLS) Restless Legs Syndrome

What Is Restless Legs Syndrome?

Restless legs syndrome (RLS) is a disorder that causes a strong urge to move your legs. This urge to move often occurs with strange and unpleasant feelings in your legs. Moving your legs relieves the urge and the unpleasant feelings.People who have RLS describe the unpleasant feelings as creeping, crawling, pulling, itching, tingling, burning, aching, or electric shocks. Sometimes, the feelings also occur in the arms.

The urge to move and unpleasant feelings occur when you're resting and inactive. They tend to be worse in the evening and at night and are temporarily relieved in the morning.Overview
RLS can make it hard to fall asleep and stay asleep. It may make you feel tired and sleepy during the day. This can make it hard to learn, work, and do your normal routine. Not getting enough sleep also can cause depression, mood swings, or other health problems.RLS can range from mild to severe based on:

The strength of your symptoms and how often they occurHow easily moving around relieves your symptomsHow much your symptoms disturb your sleepOne type of RLS usually starts early in life (before age 45) and tends to run in families. It may even start in childhood. Once this type of RLS starts, it usually lasts for the rest of your life. Over time, symptoms slowly get worse and occur more often. If you have a mild case, you may have long periods with no symptoms.Another type of RLS usually starts later in life (after age 45). It generally doesn't run in families. This type tends to have a more abrupt onset. The symptoms usually don't get worse with age.

Some diseases, conditions, and medicines also may trigger RLS. For example, it has been associated with kidney failure, Parkinson disease, diabetes, rheumatoid arthritis, pregnancy, and iron deficiency. When a disease, condition, or medicine causes RLS, the symptoms usually start suddenly.Medical conditions or medicines often cause or worsen the type of RLS that starts later in life.

Outlook

RLS symptoms often get worse over time. However, some people's symptoms go away for weeks to months.

If a condition or medicine triggers RLS, it may go away if the trigger is relieved or stopped. For example, RLS that occurs due to pregnancy tends to go away after giving birth. Kidney transplants (but not dialysis) relieve RLS linked to kidney failure.Treatments for RLS include lifestyle changes and medicines. Some simple lifestyle changes often help relieve mild cases of RLS. Medicines usually can relieve or prevent the symptoms of more severe RLS. Research is ongoing to better understand the causes of RLS and to find better treatments.

What Causes Restless Legs Syndrome?

Faulty Use or Lack of Iron

Research suggests that restless legs syndrome (RLS) is mainly due to the faulty use or lack of iron in the brain. The brain uses iron to make the chemical dopamine and to control other brain activities. Dopamine works in the parts of the brain that control movement.A number of conditions can affect how much iron is in the brain or how it's used. These conditions include kidney failure, Parkinson disease, diabetes, rheumatoid arthritis, pregnancy, and iron deficiency. All of these conditions increase the risk of having RLS.

People whose family members have RLS also are more likely to develop the disorder. This suggests that genetics may contribute to the faulty use or lack of iron in the brain that triggers RLS.

Nerve Damage

Nerve damage in the legs or feet and sometimes in the arms or hands may cause or worsen RLS. Several conditions can cause such nerve damage, including diabetes.Medicines and Substances
Certain medicines may trigger RLS. These include some:

• Anti-nausea medicines (used to treat upset stomach)
• Antidepressants (used to treat depression)
• Anti-psychotics (used to treat certain mental health disorders)
• Cold and allergy medicines that contain antihistamines
• Calcium channel blockers (used to treat heart problems and high blood pressure)

RLS symptoms usually get better or may even go away if the medicine is stopped.

Certain substances, such as alcohol and tobacco, also can trigger or worsen RLS symptoms. Symptoms may get better or go away if the substances are stopped.

Who Is At Risk for Restless Legs Syndrome?

Restless legs syndrome (RLS) may affect as many as 12 million people in the United States. More than half of the people who have RLS have family members with the condition.
RLS can affect people of any race or ethnic group, but the disorder is more common in people of Northern European descent. RLS affects both genders, but women are more likely to have it than men.The number of cases of RLS rises with age. Many people who have RLS are diagnosed in middle age. However, in about 40 percent of RLS cases, symptoms start before age 20. People who develop RLS early in life usually have a family history of it.

People who have certain diseases or conditions or who take certain medicines are more likely to develop RLS. (For more information, see "What Causes Restless Legs Syndrome?")
For example, RLS is common in pregnant women. It usually occurs during the last 3 months of pregnancy. The disorder usually improves or goes away after giving birth. Some women may continue to have symptoms after giving birth. Other women may develop RLS again later in life.

What Are the Signs and Symptoms of Restless Legs Syndrome?

The four key signs of restless legs syndrome (RLS) are:

• A strong urge to move your legs. This urge often, but not always, occurs with unpleasant feelings in your legs. When the disorder is severe, you also may have the urge to move your arms.
• Symptoms that start or get worse when you're inactive. The urge to move increases when you're sitting still or lying down and resting.
• Relief from moving. Movement, especially walking, helps relieve the unpleasant feelings.
• Symptoms that start or get worse in the evening or at night.

You must have all four of these signs to be diagnosed with RLS.

The Urge To Move

RLS gets its name from the urge to move the legs when sitting or lying down. This movement relieves the unpleasant feelings that RLS sometimes causes.

Typical movements are:

• Pacing and walking
• Jiggling the legs
• Stretching and flexing
• Tossing and turning
• Rubbing the legs

Unpleasant Feelings

People who have RLS describe the unpleasant feelings in their limbs as creeping, crawling, pulling, itching, tingling, burning, aching, or electric shocks. More severe RLS symptoms may cause painful feelings. However, the pain usually is more of an ache than a sharp, stabbing pain.

Children may describe RLS symptoms differently than adults. Sometimes children with RLS are misdiagnosed as having ADHD.

The unpleasant feelings from RLS often occur in the lower legs (calves). But the feelings can occur at any place in the legs or feet. They also can occur in the arms.

The feelings seem to come from deep within the limbs, rather than from the surface. You usually will have the feelings in both legs. However, the feelings can occur in one leg, move from one leg to the other, or affect one leg more than the other.

People who have mild symptoms may only notice them when they're still or awake for a long time, such as on a long airplane trip or when watching TV. If they fall asleep quickly, they may not have symptoms when lying down at night.

The unpleasant feelings from RLS aren't the same as the leg cramps many people get at night. Leg cramps often are limited to certain muscle groups in the leg, which you can feel tightening. Leg cramps cause more severe pain and require stretching the affected muscle for relief.

Sometimes arthritis or peripheral arterial disease (PAD) can cause pain or discomfort in the legs. Moving the limbs usually worsens the discomfort instead of relieving it.

Periodic Limb Movement in Sleep

Most people who have RLS also have a condition called periodic limb movement in sleep (PLMS). PLMS causes your legs or arms to twitch or jerk about every 10 to 60 seconds during sleep. These movements cause you to wake up often and get less sleep.
PLMS usually affects the legs, but it also can affect the arms.
Not everyone who has PLMS also has RLS.

Related Sleep Problems

The symptoms of RLS can make it hard to fall or stay asleep. If RLS disturbs your sleep, you may feel very tired during the day.Lack of sleep may make it hard for you to concentrate at school or work. Not enough sleep also can cause depression, mood swings, or other health problems such as diabetes or high blood pressure.

How Is Restless Legs Syndrome Diagnosed?

Your doctor will diagnose restless legs syndrome (RLS) based on your symptoms, your medical and family histories, and the results from a physical exam and tests.
Your doctor will use this information to rule out other conditions that have symptoms similar to those of RLS.

Specialists Involved

Your primary care doctor usually can diagnose and treat RLS. However, he or she also may suggest that you see a sleep specialist or neurologist.

Symptoms

You must have the four key signs of RLS to be diagnosed with the condition.

Your doctor also will want to know how your symptoms are affecting your sleep and how alert you are during the day.

To help your doctor, you may want to keep a sleep diary. Use the diary to keep a daily record of how easy it is to fall and stay asleep, how much sleep you get at night, and how alert you feel during the day.
For a sample sleep diary, see the National Heart, Lung, and Blood Institute's "Your Guide to Healthy Sleep."

Medical and Family Histories

Your doctor may ask whether you have any of the diseases or conditions that can trigger RLS. These include kidney failure, Parkinson disease, diabetes, rheumatoid arthritis, pregnancy, and iron deficiency.

Your doctor also may want to know what medicines you take. Some medicines can trigger or worsen RLS.

Because the most common type of RLS tends to run in families, your doctor may ask whether any of your relatives have the disorder.

Physical Exam

Your doctor will do a physical exam to check for underlying conditions that may trigger RLS. He or she also will check for other conditions that have symptoms similar to those of RLS.

Tests

Currently, no test can diagnose RLS. Still, your doctor will likely order blood tests to measure your iron levels. He or she also may order muscle or nerve tests. These tests can show whether you have a condition that may worsen RLS or that has symptoms similar to those of RLS.

Rarely, sleep studies are used to diagnose RLS. A sleep study measures how much and how well you sleep. Although RLS can cause a lack of sleep, this sign isn't specific enough to diagnose RLS.

Researchers continue to study new tests to diagnose RLS.

Drug Therapy Trial

If your doctor thinks you have RLS, he or she may prescribe certain medicines to relieve your symptoms. These medicines, which are used to treat people who have Parkinson disease, also can relieve RLS symptoms. If the medicines relieve your symptoms, your doctor can confirm that you have RLS.

How Is Restless Legs Syndrome Treated?

Restless legs syndrome (RLS) has no cure. If a condition or medicine triggers RLS, it may go away or get better if the trigger is relieved or stopped.
RLS can be treated.

The goals of treatment are to:

• Prevent or relieve symptoms
• Increase the amount and improve the quality of your sleep
• Treat or correct any underlying condition that may trigger or worsen RLS

Mild cases of RLS often are treated with lifestyle changes and sometimes with periodic use of medicines. More severe RLS usually is treated with daily medicines.

Lifestyle Changes

Lifestyle changes can prevent or relieve the symptoms of RLS. For mild RLS, lifestyle changes may be the only treatment needed.

Preventing Symptoms

Many common substances, such as alcohol and tobacco, can trigger RLS symptoms. Avoiding these substances can limit or prevent symptoms.

Some prescription and over-the-counter medicines can cause or worsen RLS symptoms. Tell your doctor about all of the medicines you're taking. He or she can tell you whether you should stop or change certain medicines.

Adopting good sleep habits can help you fall asleep and stay asleep-a problem for many people who have RLS. Good sleep habits include:

Keeping the area where you sleep cool, quiet, comfortable, and as dark as possible.

• Making your bedroom sleep-friendly. Remove things that can interfere with sleep, such as a TV, computer, or phone.
• Going to bed and waking up at the same time every day. Some people who have RLS find it helpful to go to bed later in the evening and get up later in the morning.
• Avoiding staying in bed awake for any long period in the evening or during the night.

Regular, moderate physical activity also may help limit or prevent RLS symptoms. Often, people who have RLS find that if they increase their activity during the day, they have fewer symptoms.

Relieving Symptoms

Certain activities can relieve RLS symptoms.
These include:

• Walking or stretching
• Taking a hot or cold bath
• Massaging the affected limb(s)
• Using heat or ice packs on the affected limb(s)
• Doing mentally challenging tasks

Choose an aisle seat at the movies or on airplanes and trains so you can move around, if necessary.

Medicines

You may need medicines to treat RLS if lifestyle changes can't control symptoms. Many medicines can relieve or prevent RLS symptoms, including many new medicines.

No single medicine works for all people who have RLS. It may take several changes in medicines and dosages to find the best approach. Sometimes, a medicine will work for a while and then stop working.

Some of the effective medicines used to treat RLS also are used to treat Parkinson disease. These medicines make dopamine or act like it in the parts of the brain that control movement. (Dopamine is a chemical that helps you move properly.)

If medicines for Parkinson disease don't prevent or relieve your symptoms, your doctor may prescribe other medicines. You may have to take more than one medicine to treat your RLS.

Always talk with your doctor before taking any medicines. He or she can tell you the side effects of each RLS medicine. Side effects may include nausea, headache, and daytime sleepiness.

In some cases, RLS medicines may worsen problems controlling excessive gambling, shopping, or sexual activity. Sometimes, continued use of RLS medicines may make your RLS symptoms worse.

Contact your doctor if you have any of these problems. He or she can adjust your medicines to prevent these side effects.

VON Willebrand Disease

What Is von Willebrand Disease?

Von Willebrand disease (VWD) is a bleeding disorder. It affects your blood's ability to clot. If your blood doesn't clot, you can have heavy, hard-to-stop bleeding after an injury. The bleeding can damage your internal organs or even cause death, although this is rare.

In VWD, you either have low levels of a certain protein in your blood, or the protein doesn't work the way it should. The protein is called von Willebrand factor, and it helps the blood clot.

Normally, when one of your blood vessels is injured, you start to bleed. Small blood cell fragments called platelets (PLATE-lets) clump together to plug the hole in the blood vessel and stop the bleeding. Von Willebrand factor acts like glue to help the platelets stick together and form a blood clot.

Von Willebrand factor also carries clotting factor VIII (8), another important protein that helps your blood clot. Factor VIII is the protein that's inactive or missing in people who have hemophilia, another clotting disorder.

VWD is more common and usually milder than hemophilia. In fact, VWD is the most common of all the inherited bleeding disorders. It occurs in about 1 out of every 100 to 1,000 people. VWD affects both males and females, while hemophilia mainly affects males.

Types of von Willebrand Disease

There are three major types of VWD.

Type 1

In type 1 VWD, you have a low level of von Willebrand factor, and you may have lower than normal levels of factor VIII. This is the mildest and most common form of VWD. About 3 out of 4 people who have VWD have type 1.

Type 2
In type 2 VWD, the von Willebrand factor doesn't work the way it should. Type 2 is divided into subtypes: 2A, 2B, 2M, and 2N. Different gene mutations (changes) cause each type, and each is treated differently. So it's important to know the exact type of VWD that you have.

Type 3
In type 3 VWD, you usually have no von Willebrand factor and low levels of factor VIII. Type 3 is the most serious form of VWD, but it's very rare.

Overview

Most people who have VWD have type 1, a mild form. This type usually doesn't cause life-threatening bleeding. You may need treatment only if you have surgery, tooth extraction, or trauma. If you need treatment, medicines and medical therapies are used.

Some people who have severe forms of VWD need emergency treatment to stop bleeding before it becomes life threatening.

Early diagnosis is important. With the right treatment plan, even people who have type 3 VWD can live normal, active lives.

What Causes von Willebrand Disease?

Von Willebrand disease (VWD) is almost always inherited. "Inherited" means that parents pass the gene for the disorder on to their children.

You can inherit type 1 or type 2 VWD if only one of your parents passes the gene on to you. You usually inherit type 3 VWD only if both of your parents pass the gene on to you. Your symptoms may be different from your parents' symptoms.

Some people have the genes for the disorder but don't have symptoms. However, they still can pass the genes on to their children.

Some people develop a form of VWD later in life as a result of other medical conditions. This form of VWD is called acquired von Willebrand syndrome.

What Are the Signs and Symptoms of von Willebrand Disease?

The signs and symptoms of von Willebrand disease (VWD) depend on the type and severity of the disorder. Many people have such mild symptoms that they don't know they have the disorder.

If you have type 1 or type 2 VWD, you may have the following mild-to-moderate bleeding symptoms:

• Frequent, large bruises from minor bumps or injuries
• Frequent or hard-to-stop nosebleeds
• Extended bleeding from the gums after a dental procedure
• Heavy or extended menstrual bleeding in women
• Blood in your stools from bleeding in your intestines or stomach
• Blood in your urine from bleeding in your kidneys or bladder
• Heavy bleeding after a cut or other accident
• Heavy bleeding after surgery

People who have type 3 VWD may have all of the symptoms listed above, as well as severe bleeding episodes for no reason. These bleeding episodes can be fatal if not treated right away. People who have type 3 VWD also may have bleeding into soft tissues or joints, causing severe pain and swelling.

Heavy menstrual bleeding is often the main symptom of VWD in women. Doctors call this menorrhagia (men-o-RA-je-a).

They define it as:

• Bleeding with clots larger than about 1-inch in diameter
• Anemia or low blood iron
• The need to change pads or tampons more than every hour

However, just because a woman has heavy menstrual bleeding doesn't mean she has VWD.

How Is von Willebrand Disease Diagnosed?

Early diagnosis of von Willebrand disease (VWD) is important to make sure that you're treated and can live a normal, active life.

Sometimes VWD is hard to diagnose. People who have type 1 or type 2 VWD may not have major bleeding problems. As a result, they may not be diagnosed until they have heavy bleeding after surgery or some other trauma.

On the other hand, type 3 VWD can cause major bleeding problems during infancy and childhood. As a result, children who have type 3 VWD usually are diagnosed during their first year of life.

To find out whether you have VWD, your doctor will review your medical history and the results from a physical exam and tests.

Medical History

Your doctor will likely ask questions about your medical history and your family's medical history. He or she may ask about:

• Any bleeding from a small wound that lasted more than 15 minutes or started up again within the first 7 days following the injury.
• Any extended, heavy, or repeated bleeding that required medical attention after surgery or dental extractions.
• Any bruising with little or no apparent trauma, especially if you could feel a lump under the bruise.
• Any nosebleeds that occurred for no known reason and lasted more than 10 minutes despite pressure on the nose, or any nosebleeds that needed medical attention.
• Any blood in your stools for no known reason.
• Any heavy menstrual bleeding (for women). This bleeding usually involves clots or lasts longer than 7 to 10 days.
• Any history of muscle or joint bleeding.
• Any medicines you've taken that might cause bleeding or increase the risk of bleeding. Examples include aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs), clopidogrel, warfarin, or heparin.
• Any history of liver or kidney disease, blood or bone marrow disease, or high or low blood platelet counts.

Physical Exam

Your doctor will do a physical exam to look for unusual bruising or other signs of recent bleeding. He or she also will look for evidence of liver disease or anemia (a low red blood cell count).

Diagnostic Tests

No single test can diagnose VWD. Your doctor may recommend a combination ofblood tests to diagnose the disorder. These tests may include:

• Von Willebrand factor antigen. This test measures the amount of von Willebrand factor in your blood.
• Von Willebrand factor ristocetin (ris-to-SEE-tin) cofactor activity. This test shows how well your von Willebrand factor works.
• Factor VIII clotting activity. Some people who have VWD have low levels of factor VIII activity, while others have normal levels.
• Von Willebrand factor multimers. This test is done if one or more of the first three tests are abnormal. It shows the structure of your von Willebrand factor. The test helps your doctor diagnose what type of VWD you have.
• Platelet function test. This test measures how well your platelets are working.

You may have these tests more than once to confirm a diagnosis. Your doctor also may refer you to a hematologist (a blood disease specialist) to confirm the diagnosis and for followup care.

How Is von Willebrand Disease Treated?

Treatment for von Willebrand disease (VWD) is based on the type of VWD you have and how severe it is. Most cases of VWD are mild, and you may need treatment only if you have surgery, tooth extraction, or an accident.

Medicines are used to:

• Increase the release of von Willebrand factor and factor VIII into the bloodstream
• Replace von Willebrand factor
• Prevent the breakdown of blood clots
• Control heavy menstrual bleeding in women

Specific Treatments

Desmopressin (DDAVP) is a synthetic hormone that you usually take by injection or nasal spray. It makes your body release more von Willebrand factor and factor VIII into your bloodstream. DDAVP works for most people who have type 1 VWD and for some who have type 2 VWD.

Von Willebrand factor replacement therapy is an infusion of a concentrate of von Willebrand factor and factor VIII into a vein in your arm. This treatment may be used if you:

• Can't take DDAVP or need extended treatment
• Have type 1 VWD that doesn't respond to DDAVP
• Have type 2 or type 3 VWD

Antifibrinolytic medicines help prevent the breakdown of blood clots. They're mostly used to stop bleeding after minor surgery, tooth extraction, or an injury. These medicines may be used alone or together with DDAVP and replacement therapy.

Fibrin glue is medicine that's placed directly on a wound to stop bleeding.

Treatments for Women

Treatments for women who have VWD with heavy menstrual bleeding include:

• Combined oral contraceptives (birth control pills). The hormones in these pills can increase the amount of von Willebrand factor and factor VIII in your bloodstream and reduce menstrual blood loss. Birth control pills are the most recommended birth control method for women who have VWD.
• A levonorgestrel intrauterine device. This is a contraceptive device that contains the hormone progestin. The device is placed in the uterus (womb).
• Aminocaproic acid or tranexamic acid. These antifibrinolytic medicines can reduce bleeding by slowing the breakdown of blood clots.
• DDAVP.

For some women who are done having children or don't want children, endometrial ablation is done. This procedure destroys the lining of the uterus. It has been shown to reduce menstrual blood loss in women who have VWD.

If you need a hysterectomy (surgical removal of the uterus) for another reason, this procedure will stop menstrual bleeding and possibly improve your quality of life. However, hysterectomy carries its own risk of bleeding complications.

Living With von Willebrand Disease

If you have von Willebrand disease (VWD), you can take steps to prevent bleeding and stay healthy. You should:

• Avoid over-the-counter medicines that can affect blood clotting, including aspirin, ibuprofen, and other nonsteroidal anti-inflammatory drugs (NSAIDs).
• Always check with your doctor before taking any medicines.
• Tell your doctor, dentist, and pharmacist that you have VWD. Your dentist can talk to your doctor about whether you need medicine before dental work to reduce bleeding. You also may want to tell people like your employee health nurse, gym trainer, and sports coach about your condition.
• Consider wearing a medical ID bracelet or necklace if you have a serious form of VWD (for example, type 3). In case of a serious accident or injury, the health care team treating you will know that you have VWD.
• Exercise regularly and maintain a healthy weight. Exercise helps keep muscles flexible. It also helps prevent damage to muscles and joints. Always stretch before exercising.

Some safe exercises and activities are swimming, biking, and walking. Football, hockey, wrestling, and lifting heavy weights are not safe activities if you have bleeding problems. Always check with your doctor before starting any exercise program.

Your parents, brothers and sisters, and children also may have VWD. Consider telling them about your diagnosis and suggesting that they get tested too.

Pregnancy and von Willebrand Disease

Pregnancy can be a challenge for women who have VWD. Although blood levels of von Willebrand factor and factor VIII tend to increase during pregnancy, women who have VWD can have bleeding complications during delivery. They also are likely to have heavy bleeding for an extended time after delivery.

However, you can take steps to lower the risk of complications during pregnancy. Consult a hematologist and an obstetrician who specialize in high-risk pregnancies before you become pregnant.

A hematologist is a doctor who specializes in treating blood diseases and disorders. An obstetrician is a doctor who treats and provides care for pregnant women.

Consider using a medical center that specializes in high-risk obstetrics and has a hematologist on staff for prenatal care and delivery.

Before you have any invasive procedure, such as amniocentesis (AM-ne-o-sen-TE-sis), discuss with your doctor whether anything needs to be done to prevent serious blood loss.

During your third trimester, you should have blood tests to measure von Willebrand factor and factor VIII to help plan for delivery.

You also should meet with an anesthesiologist to review your choices for anesthesia (AN-es-THE-ze-a) and to discuss taking medicine to reduce your bleeding risk. The term "anesthesia" refers to a loss of feeling and awareness. Some types of anesthesia temporarily put you to sleep, while others only numb certain areas of your body.

With these precautions, most women who have VWD can have successful pregnancies.

Children and von Willebrand Disease

If your child has VWD that's severe enough to pose a major risk of bleeding, anyone who is responsible for him or her should be told about the condition.

For example, the school nurse, teacher, daycare provider, coach, or any leader of afterschool activities should know, especially if your child has one of the more severe forms of VWD. This information will help them handle the situation if your child has an injury.

Links to Other Information About von Willebrand Disease

NHLBI Resources

Blood Diseases and Resources Information

"In Brief: Your Guide to von Willebrand Disease"

Non-NHLBI Resources

Bleeding Disorders (MedlinePlus)

Von Willebrand Disease (MedlinePlus)

Clinical Trials

Current Research (ClinicalTrials.gov)

NHLBI Pediatric Clinical Trials

What is Cirrhosis of the Liver?

Cirrhosis of the liver is a liver disease characterized by a gradual annihilation of the liver cells. These cells are progressively replaced with fibrous tissue, which then leads to hardening. Cirrhosis is irreversible chronic injury of the liver. It often has no symptoms. Your health care provider will diagnose cirrhosis based on your medical history, a physical examination, and laboratory tests.

Signs

The signs and symptoms of cirrhosis can range from an absence of symptoms (in 10 to 20 percent of patients) to liver failure. Cirrhosis can also have symptoms such as jaundice (yellowing of the skin), weight loss, abdominal pain, testicular atrophy (in men), menstrual irregularity (in women), swelling and fluid in the abdomen, and enlarged veins.

Usually no symptoms appear until the disease has entered the late stages as the disease tends to come on gradually. When they occur, symptoms can include:

· nausea, vomiting, and loss of appetite.

· unusual gain or loss of weight.

· yellow coloration of the skin and eyes (see Jaundice).

· dark urine.

· bloody, black stools, or unusually light-colored stools.

· vomiting of blood.

· thinning hair

· abdominal swelling.

· itching.

· swollen feet or legs.

· red palms.

· sleep disturbances and confusion.

· fatigue or loss of stamina.

· enlarged breasts in men.

· loss of sex drive and menstrual problems in women.

· spider like blood vessels on chest and shoulders.

· in severe cases, mental disturbances

Causes

The most common cause of cirrhosis is alcoholism. Consuming a lot of alcohol daily (32 to 48 oz. of beer, 4 to 8 oz. of liquor, 16 to 32 oz. of wine) for 10 years or more increases your chances of developing cirrhosis. How much alcohol you drink and for how long are more important than the type of alcohol ingested. Between 5 and 10 percent of people in the United States are alcoholics. Of these, 10 to 15 percent will develop liver disease. Cirrhosis can also be caused by the ingestion of drugs and toxins, infections, inherited medical conditions, and cardiovascular diseases. About 10 percent of cases have no known cause.

Herbs

Herbs may be used as dried extracts (capsules, powders, teas), glycerites (glycerine extracts), or tinctures (alcohol extracts). Unless otherwise indicated, teas should be made with 1 tsp. herb per cup of hot water. Steep covered 5 to 10 minutes for leaf or flowers, and 10 to 20 minutes for roots. Drink 2 to 4 cups per day.

Due to the high doses required and the need to avoid alcohol, the preferred form of these herbs is powdered.

• Milk thistle (Silybum marianum): 100 mg three times per day prevents free radical damage in the liver.
• Barberry (Berberis vulgaris): 250 to 500 mg per day corrects metabolic abnormalities in liver cirrhosis.
• Catechin (Uncaria gambir): 400 mg three times per day, is antioxidant, antiviral, and helps regenerate liver tissue.

What is primary biliary cirrhosis?

Primary biliary cirrhosis is a chronic disease that causes the bile ducts in the liver to become inflamed and damaged and, ultimately, disappear. Bile is a liquid produced in the liver that travels through the bile ducts to the gallbladder and then the small intestine, where it helps digest fats and fat-soluble vitamins A, D, E, and K. When the bile ducts become damaged from chronic inflammation, bile builds up in the liver, injuring liver tissue.

Normal liver and biliary system.

Injured liver tissue from chronic inflammation and the buildup of bile leads to cirrhosis, a condition in which the liver slowly deteriorates and malfunctions. Scar tissue replaces healthy liver tissue, partially blocking the flow of blood through the liver. Scarring also impairs the liver’s ability to

• control infections
• remove bacteria and toxins from the blood
• process nutrients, hormones, and drugs
• make proteins that regulate blood clotting
• produce bile to help absorb fats—including cholesterol—and fat-soluble vitamins
• effectively replace its own cells when they become damaged

Primary biliary cirrhosis develops over time and may ultimately cause the liver to stop working completely. Most people are diagnosed early, before the disease progresses. Early treatment delays—but does not stop—the eventual onset of cirrhosis and liver failure. When a person has end-stage liver disease, a liver transplant is necessary for survival.

Primary biliary cirrhosis usually occurs between the ages of 40 and 60 and affects women more often than men.

What causes primary biliary cirrhosis?

The cause of primary biliary cirrhosis is unknown. Most research suggests the disease is an autoimmune condition. The immune system usually protects the body from harmful substances such as bacteria and viruses by attacking and destroying them. In autoimmune diseases, the immune system instead attacks the body’s own tissues. In primary biliary cirrhosis, the immune system attacks the bile ducts.

Genetic factors may make a person prone to develop primary biliary cirrhosis. Primary biliary cirrhosis is more common in people who have a parent or sibling—particularly an identical twin—with the disease.

Genetic factors may also make some people prone to develop other autoimmune diseases. People with primary biliary cirrhosis may have other autoimmune conditions such as rheumatoid arthritis or autoimmune thyroiditis.

A person who has genetic factors for primary biliary cirrhosis may be more likely to develop the disease after exposure to chemicals or infections, such as urinary tract infections.

What are the symptoms of primary biliary cirrhosis?

Most people with primary biliary cirrhosis are diagnosed before symptoms begin. The first and most common symptoms people with this condition experience are

• a general feeling of tiredness, or fatigue
• pruritus—itchy skin—and darkened skin in itchy areas due to scratching

Other symptoms may develop, including

• dry eyes and mouth
• jaundice—darkening of the urine and yellowing of the skin and whites of the eyes—which occurs when the diseased liver does not remove enough bilirubin from the blood. Bilirubin is the pigment that gives bile its reddish-yellow color.

How is primary biliary cirrhosis diagnosed?

The first indication of primary biliary cirrhosis may occur when results of routine blood tests to check liver function are abnormal. The doctor will then order one or more tests to confirm the disease:

• Anti-mitochondrial antibody (AMA) blood test. The presence of AMA is detected in 90 percent of people with primary biliary cirrhosis.
• Alkaline phosphatase blood test. Primary biliary cirrhosis is likely if two blood tests performed at least 6 months apart reveal alkaline phosphatase—a liver enzyme—to be abnormally high.
• Liver biopsy. A liver biopsy can confirm the diagnosis but is not always necessary. A biopsy may help determine the extent of liver damage. The biopsy is performed with a needle inserted between the ribs or into a vein in the neck. Precautions are taken to minimize discomfort. A tiny sample of liver tissue is examined with a microscope for scarring or other signs of cirrhosis. Sometimes a cause of liver damage other than cirrhosis is found during biopsy.

The doctor may also order the following tests:

• Cholesterol blood test. People with primary biliary cirrhosis may have abnormally high levels of cholesterol in the blood. However, these high cholesterol levels are usually less harmful to people with primary biliary cirrhosis than to those without the disease.
• Abdominal ultrasound. An ultrasound shows whether the liver and bile ducts are inflamed. A handheld device, which a technician glides over the abdomen, sends sound waves toward the abdomen. The sound waves bounce off the liver and other organs, and their echoes create a picture of the liver and biliary system on a video monitor.

What are the complications of primary biliary cirrhosis?

Some people develop one or more complications of primary biliary cirrhosis when the disease progresses to cirrhosis.

Osteoporosis. The disease may cause bones to become fragile and more likely to break. Osteoporosis can also result from steroid use as a treatment of primary biliary cirrhosis.

Maldigestion. When a person with primary biliary cirrhosis has jaundice, the small intestine cannot easily absorb fats and fat-soluble vitamins. Maldigestion may result in diarrhea and fatty stools and can lead to weight loss in the late stages of the disease.

Portal hypertension. Normally, blood from the intestines and spleen is carried to the liver through the portal vein. But primary biliary cirrhosis may cause inflammation in the portal tract, leading to increased pressure in the portal vein. This condition is called portal hypertension.

Esophageal varices and gastropathy. When portal hypertension occurs, it may cause enlarged blood vessels in the esophagus, called varices, or in the stomach, called gastropathy, or both. Enlarged blood vessels are more likely to burst due to thin walls and increased pressure. If they burst, serious bleeding can occur in the esophagus or upper stomach, requiring immediate medical attention.

Splenomegaly. When portal hypertension occurs, the spleen frequently enlarges and sequesters or holds white blood cells and platelets, reducing the numbers of these cells in the blood. A low platelet count in the blood may be the first evidence that a patient has developed cirrhosis.

Edema and ascites. When the liver dam-age progresses to an advanced stage, fluid collects in the legs, called edema, and in the abdomen, called ascites. Ascites can lead to bacterial peritonitis, a serious infection.

Bruising and bleeding. When the liver slows or stops producing the proteins needed for blood clotting, a person will bruise or bleed easily.

Sensitivity to medications. Cirrhosis slows the liver’s ability to filter medications from the blood. When this occurs, medications act longer than expected and build up in the body. This causes a person to be more sensi-tive to medications and their side effects.

Hepatic encephalopathy. A failing liver cannot remove toxins from the blood, and they eventually accumulate in the brain. The buildup of toxins in the brain—called hepatic encephalopathy—can decrease mental function and cause coma. Signs of decreased mental function include confusion, personality changes, memory loss, trouble concentrating, and a change in sleep habits.

Insulin resistance and type 2 diabetes. Cirrhosis causes resistance to insulin—a hormone produced by the pancreas that enables the body to use glucose as energy. With insulin resistance, the body’s muscle, fat, and liver cells do not use insulin prop-erly. The pancreas tries to keep up with the demand for insulin by producing more, but excess glucose builds up in the bloodstream causing type 2 diabetes.

Liver cancer. Hepatocellular carcinoma is a type of liver cancer that can occur in patients with cirrhosis. Hepatocellular carcinoma has a high mortality rate, but several treatment options are available.

Other problems. Cirrhosis can cause immune system dysfunction, leading to the risk of infection. Cirrhosis can also cause kidney and lung failure, known as hepato-renal and hepatopulmonary syndromes.

How is primary biliary cirrhosis treated?

Early Treatment

Initial treatment for primary biliary cirrhosis is aimed at relieving symptoms. Vitamin replacement therapy, calcium supplements, and drugs to treat itching are usually prescribed.

A specific treatment that stops or reverses the progression of primary biliary cirrhosis has not been found. However, medication prescribed during the early stage of the disease may slow liver damage.

Ursodiol (Actigall). Ursodiol is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis. Ursodiol assists the liver in moving bile through the ducts to the gallbladder and small intestine. Studies have shown that ursodiol prescribed early in the disease improves liver function, slowing the time it takes to progress to liver failure and the need for a liver transplant.

Researchers are studying the effects of sev-eral other medications on the progression of primary biliary cirrhosis. To date, none have shown the positive effects of ursodiol.

Treatment for Cirrhosis and Its Complications

When the disease progresses to cirrhosis, the goals of treatment are to slow the progression of scar tissue in the liver and prevent or treat the complications of the disease. Hospitalization may be necessary for people who have cirrhosis with complications.

Eating a nutritious diet. Because malnutrition is common in people with cirrhosis, a healthy diet is important in all stages of the disease. Health care providers recommend a meal plan that is well balanced. If fluid overload develops, a sodium-restricted diet is recommended. A person with cirrhosis should not eat raw shellfish, which can contain a bacterium that causes serious infection. To improve nutrition, the doctor may add a liquid supplement either for drinking or administration with a nasogastric tube—a tiny tube inserted through the nose and throat that reaches into the stomach.

Avoiding alcohol and other substances. People with primary biliary cirrhosis are encouraged not to consume alcohol or illicit substances, as both will cause more liver damage. Because many vitamins and medications—prescription and over-the-counter—can affect liver function, a doctor should be consulted before taking them.

Treatment for cirrhosis also addresses specific complications. For edema and ascites, the doctor may recommend diuretics—medications that remove fluid from the body. Large amounts of ascitic fluid may be removed from the abdomen and checked for bacterial peritonitis. Oral antibiotics may be prescribed to prevent infection. Severe infection with ascites requires intravenous (IV) antibiotics.

The doctor may prescribe a beta-blocker or nitrate for portal hypertension, which can lower the pressure in the varices and reduce the risk of bleeding. Gastrointestinal bleed-ing requires an immediate upper endoscopy to look for esophageal varices. The doctor may perform a band-ligation using a special device to compress the varices and stop the bleeding. People who have had varices in the past may need to take medicine to prevent future episodes.

Hepatic encephalopathy is treated by cleans-ing the bowel with lactulose—a laxative given orally or in enemas. Antibiotics are added to the treatment if necessary. Patients may be asked to reduce dietary protein intake. The condition may improve as other complications of cirrhosis are controlled.

People with cirrhosis who develop hepatorenal syndrome must undergo regular hemodialysis treatment, which uses a machine to clean wastes from the blood. Medications are also given to improve blood flow through the kidneys.

Liver Transplantation

A liver transplant is the only treatment that will cure primary biliary cirrhosis. A liver transplant is considered when complications cannot be controlled by treatment. A liver transplant is a major operation in which the diseased liver is removed and replaced with a healthy one from an organ donor. A team of health professionals determines the risks and benefits of the procedure for each patient. Survival rates have improved over the past several years because of drugs that suppress the immune system and keep it from attack-ing and damaging the new liver.

The number of people who need a liver transplant far exceeds the number of available organs. A person needing a transplant must go through a complicated evaluation process before being added to a long transplant waiting list. Generally, organs are given to people with the best chance of living the longest after a transplant. Survival after a transplant requires intensive follow-up and cooperation on the part of the patient and caregiver.

Points to Remember

• Primary biliary cirrhosis is a chronic disease that causes the bile ducts in the liver to become inflamed and damaged and, ultimately, disappear.
• Injured liver tissue from chronic inflammation and the buildup of bile leads to cirrhosis, a condition in which the liver slowly deteriorates and malfunctions.
• The cause of primary biliary cirrhosis is unknown. Most research suggests the disease is an autoimmune condition.
• Primary biliary cirrhosis is more common in people who have a parent or sibling—articularly an identical twin—with the disease.
• Most people are diagnosed early, before the disease progresses. The disease is often discovered when routine blood tests to check liver function are abnormal.
• Many people with primary biliary cirrhosis do not have symptoms until after the disease is diagnosed.
• The first and most common symptoms of the disease are itching, called pruritus, and fatigue. Other symptoms include dry eyes and mouth and jaundice.
• The anti-mitochondrial antibody (AMA) blood test, the alkaline phosphatase blood test, and a liver biopsy may be necessary to confirm a diagnosis of primary biliary cirrhosis.
• Some of the complications of primary biliary cirrhosis are osteoporosis and maldigestion.
• Early treatment delays—but does not stop—the eventual onset of cirrhosis and liver failure.
• Ursodiol (Actigall) is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis.
• Liver transplantation is the only treatment that will cure primary biliary cirrhosis. A liver transplant is considered when complications cannot be controlled by treatment.

Hope

The National Institute of Diabetes and Digestive and Kidney Diseases’ Division of Digestive Diseases and Nutrition supports basic and clinical research into liver diseases—including primary biliary cirrhosis—and liver transplantation. Researchers are studying

· the mechanisms of liver injury and regeneration

· ways to improve outcomes of liver transplantation

· how autoimmune liver diseases develop in the body