Common Disorders & FAQs

Liver

About the Liver

The liver is a marvellously resilient and vital organ that plays an indispensable role in nurturing and protecting your body everyday with clockwork precision. It has several key functions to perform – it helps filter and dispose off toxic materials from the blood, feeds your body the energy it needs to function, wards off viruses and infections, produces blood-clotting factors, regulates sex hormones, cholesterol levels and vitamin and mineral supplies in your body. Merely the tip of the iceberg, for the liver performs over 500 and odd functions, far more than any other organ in your body!

The need of the hour is to fully understand the critical role the liver plays in sustaining complete health. The liver supports almost every organ in the body and is vital for survival. It is tremendously important to understand the indispensable and central role that the liver plays in maintaining overall good health and vitality – only by doing so can you identify activities that help or harm this vital organ and do all you can to help protect it.

There are further advantages to be had from understanding your liver better – one, it helps you know exactly what must be done to keep it healthy and two, you will be able to actually cut down your risk of developing not only liver disease but other related health conditions such as diabetes and heart disease.

The process of caring is dual. Your liver depends on you to take care of it so it can, in its turn, do a better job taking care of you. It is an efficient multi-tasker and performs manifold functions – serves as your body’s engine, pantry, refinery, food processor, garbage disposal, trouble shooter and “guardian angel.” As you can see, a healthy liver is the key to achieving a healthy life. The trouble lies in the fact that this indefatigable worker carries out its work silently, often there’s no hint of trouble and any damage is usually far advanced by the time it makes its presence felt. Currently, there is no artificial organ or device capable of emulating all the functions of the liver. This heightens the importance of maintaining the continual good health of your liver.

Conditions of the Liver

Liver disease can prevent the liver from performing its numerous, vital functions. There are many kinds of liver diseases. Some common diseases of the liver like Hepatitis A, Hepatitis B and Hepatitis C are caused by viruses that attack the liver. Still other liver diseases can be the result of drug abuse, exposure to poisons or excessive consumption of alcohol.

Hepatitis A causes an inflammation of the liver and is primarily transmitted through contamination of food or drinking water with fecal matter. It can be effectively prevented by vaccine shots and sanitary precautions. Hepatitis B is another infection of the liver, primarily spread through blood or body fluid contact with an infected person. It is easily prevented with vaccination and by avoiding unprotected sex, contaminated needles, and similar sources of infection. Hepatitis C is spread by direct contact with infected blood and blood products. Currently there is no effective vaccine that affords protection against Hepatitis C.

Cirrhosis damages healthy liver cells and replaces them with scar tissue, preventing the liver from functioning efficiently. Liver cancer, caused by abnormal multiplication of cells can result from diseases such as Hepatitis B and C, alcohol abuse, exposure to chemicals, or congenital defects. Liver failure is a potentially life-threatening condition characterized by severe deterioration of liver function as a result of extensive damage to the liver.

Treatment of all liver diseases involves immediate medical care aimed at slowing the progression of the disease, minimizing the symptoms and reducing further complications.

More information on each kind of disease and its causes, symptoms, risk factors and tests and diagnosis are available from the links on the left.

Liver disease is a common and serious problem in our country. It is important for liver transplant candidates and their families to understand the basic process involved with liver transplants, to appreciate some of the challenges and complications that face liver transplant recipients (people who receive livers), and to recognize symptoms that should alert recipients to seek medical help.

What is liver transplantation?

Liver transplantation is surgery that is performed to remove a diseased liver in order to replace it with a healthy one. Such surgeries have been done for over 38 years. Several people who have had liver transplants go on to lead perfectly normal lives.

When does one require a liver transplant?

Liver disease severe enough to require a liver transplant can come from many causes. In adults, the most common reason for liver transplantation is cirrhosis. Cirrhosis is a condition in which the liver slowly deteriorates and malfunctions due to chronic injury. Scar tissue replaces healthy liver tissue, partially blocking the flow of blood through the liver. Cirrhosis can be caused by viruses such as hepatitis B and C, alcohol, autoimmune liver diseases, buildup of fat in the liver, and hereditary liver diseases. Many people who develop cirrhosis of the liver due to excessive use of alcohol also need a liver transplant. Abstinence from alcohol and treatment of complications for 6 months will usually allow some of them to improve significantly and these patients may survive for prolonged periods without a transplant. For patients with advanced liver disease, where prolonged abstinence and medical treatment fails to restore health, liver transplantation is the treatment.

In children, the most common reason for liver transplantation is biliary atresia. Biliary atresia is a rare condition in newborn infants in which the common bile duct between the liver and the small intestine is blocked or absent. Bile ducts, which are tubes that carry bile out of the liver, are missing or damaged in this disease, and obstructed bile causes cirrhosis. Bile helps digest food. If unrecognised, the condition leads to liver failure. The cause of the condition is unknown. The only effective treatments are certain surgeries, or liver transplantation.

Other reasons for transplantation are liver cancer, benign liver tumors, and hereditary diseases. Primary liver cancers develop at a significantly higher rate in cirrhotic livers as compared to normal livers, particularly in patients having liver disease secondary to Hepatitis B. Liver Transplantation at an early stage of liver cancer may result in long-term survival for select patients. However, cancers of the liver that begin somewhere else in the body and spread to the liver are not curable with a liver transplant. Sometimes the cause of liver disease is not known. Liver transplants can thus help both adults and children.

How are candidates for liver transplant determined?

Evaluations by specialists from a variety of fields are needed to determine if a liver transplant is appropriate. The evaluation includes a review of your medical history and a variety of tests. The transplant team will arrange blood tests, X – rays, and other tests to help make the decision about whether you need a transplant and whether a transplant can be carried out safely. Other aspects of your health—like your heart, lungs, kidneys, immune system, and mental health—will also be checked to be sure you’re strong enough for surgery.

Many healthcare facilities offer an interdisciplinary approach to evaluate and to select candidates for liver transplantation. This interdisciplinary healthcare team may include the following professionals:

  • Liver specialist (hepatologist).
  • Liver Transplant surgeons
  • Transplant coordinator, usually a registered nurse who specializes in the care of liver-transplant patients (this person will be your primary contact with the transplant team).
  • Psychiatrist to help you deal with issues, such as anxiety and depression, which may accompany the liver transplantation.
  • Anesthesiologist to discuss potential anesthesia risks.
Can anyone with liver problems get a transplant?
  • You cannot have a transplant if you havecancer in another part of your body
  • serious heart, lung, or nerve disease
  • active alcohol or illegal drug abuse
  • an active, severe infection
  • inability to follow your doctor’s instructions

How is the transplant decision made?

The decision is taken in consultation with all individuals involved in the patient’s care, doctors as well as the patient’s family. The patient and family’s input is vital and it is important that they clearly understand the risks & benefits involved with transplantation.

Will liver transplantation be a treatment of last resort, when everything else has failed?

The answer would be both yes and no. If medical treatment is thought to prolong survival with good quality of life, transplantation would be considered at a later stage in the future. However, ideally, the surgery is undertaken before the terminal stage of the disease when the person is too ill to withstand major surgery. For patients with poor quality of life due to complications arising from liver cirrhosis, liver transplantation should be undertaken at an optimal state of health for a good prognosis.

Is Liver transplant Legal in India?

Yes, liver transplant is legal in India but is bound by certain clauses which have been framed to prevent commerical use of organs. On July 8, 1994, the President of India assented to the Transplantation of Human Organs Act (Act No.42, 1994) providing for “the regulation of removal, storage and transplantation of human organs for therapeutic purposes and for the prevention of commercial dealings in human organs and for matters connected therewith or incidental thereto”. As a result, various state legislatures prohibited all organ sales.

The Act’s preamble envisages the object of the legislation in a two-fold manner:

(1) Providing for regulation of removal, storage and transplantation of human organs for therapeutic purposes; and

(2) prevention of commercial dealings in human organs.

The Act necessitates that the donor must not be below 18 years of age, must agree voluntarily to his organ removal, and that his consent is informed. It further prohibits removal of organs by anyone other than a registered medical practitioner, and the transplantation must take place in a registered hospital.

Further, to prevent commercialisation of sales of human organs, Sections 18 & 19 criminalises such transactions, including supply of organs for payment, and making/receiving any such payment. Payment, however, does not include reimbursement for the cost of removing, transporting or preserving the organ to be supplied or any expenses/loss of earnings incurred by the donor which can be attributed to his supplying any organ from his body.

Section 9(1) provides that no human organ shall be removed and transplanted unless the donor is a close relative as defined in section 2(i) of the Act. And though an altruistic donor is permitted to donate organs, it is only with prior authorisation of the committee constituted under the Act. Section 9(5)&(6) lay down the procedure to be followed while obtaining the committee’s approval.

How long does it take to get a new liver?

If you become an active liver transplant candidate, your name will be placed on a waiting list . Patients are listed according to blood type, body size, and medical condition (how ill they are). Each patient is given a priority score based on three simple blood tests (creatinine, bilirubin, and INR). The score is known as the MELD (model of end stage liver disease) score in adults and PELD (pediatric end stage liver disease) in children.

Patients with the highest scores are transplanted first. As they become more ill, their scores will increase and therefore their priority for transplant increases, allowing for the sickest patients to be transplanted first.It is impossible to predict how long it will take for a liver to become available. Your transplant coordinator will always be available to discuss where you are placed on the waiting list. While you wait for a new liver, it would be best if you and your doctor discuss what you can do to stay strong for the impending surgery. You can also begin learning about taking care of a new liver.

Where does a liver for transplant come from?

There are two types of liver transplant options: living donor transplant and deceased donor transplant.

Living donor liver transplants are an option for some patients with end-stage liver disease. This involves removing a segment of liver from a healthy living donor and implanting it into a recipient. Both the donor and recipient liver segments will grow to normal size in a few weeks.

The donor, who may be a blood relative, spouse, or friend, will have extensive medical and psychological evaluations to ensure the lowest possible risk. Blood type and body size are critical factors in determining who is an appropriate donor. All living donors and donated livers are tested before transplant surgery. The testing makes sure the liver is healthy, matches your blood type, and is the right size so it has the best chance of working in your body.

Recipients for the living donor transplant must be active on the transplant waiting list. Their health must also be stable enough to undergo transplantation with excellent chances of success.

In deceased donor liver transplant, the donor may be a victim of an accident, brain hemorrhage or head injury. The donor’s heart is still beating, but the brain has stopped functioning. Such a person is considered legally dead, because his or her brain has permanently and irreversibly stopped working. At this point, the donor is usually in an intensive-care unit. The liver is donated, with the consent of the next of kin, from such individuals. Whole livers come from people who have just died. This type of donor is called a cadaveric donor. The identity of a deceased donor and circumstances surrounding the person’s death are kept confidential.

Is liver donation safe?

Liver donation is very safe. This is because the liver has great reserve and regenerates to its original size quickly (within 2-3 months) after a part of it is removed. The donor suffers from no long-term effects, does not have to take any medication beyond 2-3 weeks, and is back to normalcy in a month. He/she can resume strenuous physical activity (weight lifting etc) in 3 months.

What about Health Insurance?

You will need to check your health insurance policy to be sure it covers liver transplantation and prescription medicines. This is because you will require many prescription medicines after the surgery and for the rest of your life.

What happens in the hospital?

When a liver has been identified for you, you will be prepared for surgery. When you arrive at the hospital, additional blood tests, an electrocardiogram, and a chest X-ray will generally be taken before the operation. If your new liver is from a living donor, both you and the donor will be in surgery at the same time. If your new liver is from a person who has recently died, your surgery starts when the new liver arrives at the hospital.

How long would surgery take?

Liver transplants usually take from 4 to 14 hours. During the operation, surgeons will remove your liver and will replace it with the donor liver. The surgeon will disconnect your diseased liver from your bile ducts and blood vessels before removing it. The blood that flows into your liver will be blocked or sent through a machine to return to the rest of your body. The surgeon will put the healthy liver in place and reconnect it to your bile ducts and blood vessels. Your blood will then flow into your new liver. Because a transplant operation is a major procedure, surgeons will need to place several tubes in your body. These tubes are necessary to help your body carry out certain functions during the operation and for a few days afterward.

What happens during this recovery period?

Initially in the intensive care unit there is very careful monitoring of all body functions, including the liver. Once the patient is transferred to the ward, the frequency of blood testing, etc. is decreased, eating is allowed and physiotherapy is prescribed to regain muscle strength. The drug or drugs to prevent rejection are initially given by vein, but later by mouth. During the transplantation, frequent tests are done to monitor liver function and detect any evidence of rejection.

When will I be able to go home?

The average hospital stay after liver transplant is two weeks to three weeks. Some patients may be discharged in less time, while others may be in the hospital much longer, depending on how the new liver is working and on complications that may arise. You need to be prepared for both possibilities. Once you are transferred from the intensive-care unit to the regular nursing floor you will be given a discharge manual, which reviews much of what you will need to know before you go home. In the hospital, you will slowly start eating again. You will first start with clear liquids, then switch to solid food as your new liver starts to function.

You will learn how to take care of yourself and to use your new medications to protect your new liver. As you perform these functions regularly, you will become an important participant in your own healthcare. Before your discharge, you will also learn the signs of rejection and infection and will know when it is important to call your doctor. The patient’s willingness to stick to the recommended post-transplantation plan is essential to a good outcome.

What is rejection?

When the liver is transplanted from one person (the donor) into another (the recipient), the immune system of the recipient triggers the same response against the new organ that it would have against any foreign material, setting off a chain of events that can damage the transplanted organ. This process is called rejection. It can occur rapidly (acute rejection), or over a long period of time (chronic rejection). Rejection can occur despite close matching of the donated organ and the transplant patient.

Your body’s natural defences, the immune system works to destroy foreign substances that invade your body. The immune system, however, cannot distinguish between your transplanted liver and unwanted invaders, such as viruses and bacteria. Therefore, your immune system may attempt to attack and destroy your new liver. This is called a rejection episode. About 70% of all liver-transplant patients have some degree of organ rejection prior to discharge. Anti-rejection medications are given to ward off the immune attack.

Do immunosuppressants have any side effects?

Immunosuppressant drugs lower a person’s resistance to infection and can make infections harder to treat. Although these medications are meant to prevent rejection of the liver, they also decrease the ability of the body to fight off certain viruses, bacteria, and fungi. The organisms that most commonly affect patients are covered with preventive medications. However, avoiding contact with people who have infections is very important.

What are the signs and symptoms of rejection?

Here is a list of signs and symptoms that may indicate liver graft rejection:

  • Fever greater than 100°
  • Fatigue or excess sleepiness
  • “Crankiness”
  • Headache
  • Abdominal swelling, tenderness, or pain
  • Decreased appetite
  • Jaundice (yellow skin or eyes)
  • Dark (brown) urine
  • Itching
  • Nausea

None of these symptoms are specific for rejection; but they are important enough that when they occur, they should prompt a call to your doctor who will decide whether the situation warrants further investigation or should be observed for the time being.

Since rejection may have no symptoms at all, the standard strategy for post-transplant care is to regularly run blood tests that may be early indicators of liver graft rejection. Doctors will check your blood for liver enzymes, the first sign of rejection. In the beginning, these tests are run daily. For the first month or so after a liver transplant the tests are run at least weekly. Gradually the interval between measurement is increased as the months and years pass. When rejection is suspected it can be confirmed by a liver biopsy. In some instances a biopsy is not needed because rejection is strongly suspected. In other situations, a biopsy is critical. For a biopsy, the doctor takes a small piece of the liver to view under a microscope.

What are the other problems that can damage the liver transplant?

Onset of the problem that made the transplant necessary in the first place is the most common trouble for patients with liver transplants. Also, hepatitis C virus may damage a transplant if the patient was infected before the operation took place.

Other problems include

  • blockage of the blood vessels going into or out of the liver
  • damage to the tubes that carry bile into the intestine
What if the transplant doesn’t work?

Optimism is the need of the hour. Most liver transplant operations go well. About 80 to 90 percent of transplanted livers are still working after 1 year. Sometimes the liver takes a long time to work. There are varying degrees of failure of the liver, however, and even with imperfect function, the patient will remain quite well. If there are complications – say, the new liver fails to function or your body rejects it, your doctor and the transplant team will decide whether to replace the failing transplanted liver by a second (or even third) transplant operation. Unfortunately, there is no dialysis treatment for livers as is possible with kidneys. Researchers are experimenting with devices to keep patients with failing livers alive while waiting for a new liver.

How do I take care of my liver after I leave the hospital?

After you leave the transplant centre at the hospital, you will need to visit your doctor often to be sure your new liver is working well. You will also need to have regular blood tests to check that your new liver is not being damaged by rejection, infections, or problems with blood vessels or bile ducts. You will need to be careful about avoiding sick people and must immediately report any signs of illnesses to your doctor. Home care involves building up endurance to carry out daily life activities and recovering to the level of health that the patient had before surgery. This can be a long, slow process that includes simple activities. Walking may require assistance at first. Coughing and deep breathing are very important to help the lungs stay healthy and to prevent pneumonia. Diet may at first consist of ice chips, then clear liquids, and, finally, solids. It is important to eat well-balanced meals with all food groups. After about 3-6 months, a person may return to work if he or she feels ready and it is approved by the primary doctor. Besides a healthy diet and exercise you must abstain from alcohol, especially if alcohol was the primary cause of damage to your own liver. Before you take any medication, including ones you can buy without a prescription, you will need to check with your doctor whether it is safe for you. It is most important to diligently follow all that your doctor says to take good care of your new liver.

Can I go back to my daily activities?

Certainly. After a successful liver transplant, most people are able to go back to their normal daily activities. Getting your strength back will take some time, depending on how sick you were before the transplant. Your doctor will be able to tell you how long your recovery period is likely to be.

  • Work- After recovery, most people are able to resume work.
  • Diet – Most people can go back to eating as they did before. Some medication may cause weight gain, others may cause diabetes or a rise in your cholesterol. Meal planning and a balanced low-fat diet can help you remain healthy. Transplant patients have a tendency to gain weight because of their retention of water. They are advised to lower their intake of salt to reduce or eliminate this water retention.
  • Exercise – Most people can engage in physical activity after a successful liver transplant.
  • Sex – Most people return to a normal sex life after liver transplantation. It is important for women to avoid becoming pregnant in the first year after transplantation. You should talk to your transplant team about sex and reproduction after transplantation.

For further clarifications do consult your doctor before beginning any new activity.

How safe is it for women to become pregnant after transplantation?

Studies have shown that women who undergo liver transplantation can conceive and give birth normally, although they have to be monitored carefully because of a higher incidence of premature births. Mothers are advised against nursing babies because of the possibility of immunosuppressive drugs being transmitted to the infants through breast milk.

How can I donate my organs?

If you wish to be an organ donor, ensure that you carry an organ donor card and paste an organ donor sticker on your medical identification card. It is also important to discuss your views on organ donation with immediate family members since the process cannot be carried out without their consent. An organ donor card is easily available at the MOHAN Foundation.

How can I donate my organs?

If you wish to be an organ donor, ensure that you carry an organ donor card and paste an organ donor sticker on your medical identification card. It is also important to discuss your views on organ donation with immediate family members since the process cannot be carried out without their consent. An organ donor card is easily available at the MOHAN Foundation.

FAQ’s – Pediatric Liver transplant

What is liver transplantation?

Liver transplantation is the surgery to remove a diseased liver and replace it with a healthy one.

Who is a candidate for a liver transplant?

Children who suffer from end-stage liver disease due to various causes may be considered for liver transplantation. The most common indication in children is biliary atresia.

How is it decided that my child needs a liver transplant?

Eligibility is determined by a comprehensive medical evaluation by the transplant team.

Who can be the donor for transplantation?

There are two sources: cadaveric and living donors.

In Apollo Multispeciality Hospital, in accordance to the law of the land we do not do cadaveric transplant.

Living liver donors should be healthy adults, with a near normal body mass index (not obese) who have the ability to understand the procedure. The donor should have no medical, emotional, or psychological condition that could potentially increase the risk of this surgery.

What does a pre transplant evaluation process consists of?

This consist of checking all the body systems with regards to optimal function and presence of unexpected disease. Your child’s immunization records will be reviewed. Following transplantation, some vaccines cannot be given and others may not be as effective.

  • A detailed nutritional assessment will also be performed. Several tests will be performed:
  • Laboratory blood and urine tests.
  • Electrocardiogram (ECG) and a chest X-ray.
  • CT ofthe liver and blood vessels
What are the pros and cons of living donor transplantation?

The advantage of living related donor transplant is that the procedure can be scheduled effectively so that it works best for the donor and recipient. The disadvantage is that there is a very small risk of complications to the donor. Out of the 40 pediatric living related liver transplants performed at our center, there has been no significant complication in the donor population.

What is the average hospital stay for a donor?

Most donors are hospitalized for 7-10 days after surgery. The incision staples are usually removed about 7-10 days postoperatively.

How does donation affect the donor’s ability to work?

The recovery time for this type of surgery varies, but most donors are advised that they will require up to 3 months for complete recovery of normal health and activity.

How long will the operation take?

A typical liver transplant can last from 8-12 hours. The surgery for the donor lasts approximately 5-6 hours.

What are the risks of transplant surgery?

There are risks with transplant surgery just as with any major surgery. Some immediate complications can include bleeding and blood clotting problems, respiratory problems and malfunction of the donor liver. Long term complications include rejection (when the child’s immune system does not accept the new liver) and infection. Fortunately, most of these complications are treatable.

What is the postoperative period like for the child who undergoes liver transplantation?

After your child’s surgery, he/she will be taken to transplant ICU where he/she will stay for a week. After your child is transferred out of ICU to the pediatric floor, the length of stay will depend on how quickly he/she recovers. Average length of hospital stay is about 3 weeks.

What medications will my child take at home after transplant?

Your child will take 2 major types of medications in addition to multivitamins and health supplements to prevent rejection. If your child misses a dose, you need to contact our team immediately.

Once my child leaves the hospital, what happens?

Initially your child has to come to the transplant clinic twice a week for laboratory work up and physical examination or as frequently advised by our team. As recovery progresses, these visits become less frequent.

Who will look after my child long term?

Your child will be looked after by the primary pediatrician who will be supported by our team. Reports will be communicated to us via e-mail or fax.

What restrictions will my child have during her recovery?

For the first six weeks after surgery, your child should avoid strenuous exercises.

What lifestyle changes are associated with liver transplants?

Most patients can return to a normal or near-normal lifestyle six months after a successful liver transplant. Recipients should avoid exposure to people with infections. Maintaining a balanced diet, and staying on prescribed medications are vital to stay healthy. Children can attend school and participate in sports and other age-appropriate activities and can have a normal married life with no fertility issues.

What is the survival rate for children with liver transplants?

Survival rates vary from centre to centre around the world. Our results are comparable to the most well established centers from across the world.

What is the cost of a liver transplant?

A pediatric liver transplant at our centre is cost effective. Post Transplant, a patient subsequently requires Rs.8-10,000 month for lifetime immunosuppression.

 

Kidney

Diabetic Kidney Disease

Diabetes is a disease that keeps the body from using glucose, a form of sugar, as it should. If glucose stays in the blood instead of breaking down, it can act like a poison. Damage to the nephrons from unused glucose in the blood is called diabetic kidney disease. Keeping blood glucose levels down can delay or prevent diabetic kidney disease. Use of medications called angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) to treat high blood pressure can also slow or delay the progression of diabetic kidney disease.

High Blood Pressure

High blood pressure can damage the small blood vessels in the kidneys. The damaged vessels cannot filter wastes from the blood as they are supposed to. A doctor may prescribe blood pressure medication. ACE inhibitors and ARBs have been found to protect the kidneys even more than other medicines that lower blood pressure to similar levels. The National Heart, Lung, and Blood Institute (NHLBI), one of the National Institutes of Health, recommends that people with diabetes or reduced kidney function keep their blood pressure below 130/80.

Glomerular Diseases

Several types of kidney disease are grouped together under this category, including autoimmune diseases, infection-related diseases, and sclerotic diseases. As the name indicates, glomerular diseases attack the tiny blood vessels, or glomeruli, within the kidney. The most common primary glomerular diseases include membranous nephropathy, IgA nephropathy, and focal segmental glomerulosclerosis. The first sign of a glomerular disease is often proteinuria, which is too much protein in the urine. Another common sign is hematuria, which is blood in the urine. Some people may have both proteinuria and hematuria. Glomerular diseases can slowly destroy kidney function. Blood pressure control is important with any kidney disease. Glomerular diseases are usually diagnosed with a biopsy-a procedure that involves taking a piece of kidney tissue for examination with a microscope. Treatments for glomerular diseases may include immunosuppressive drugs or steroids to reduce inflammation and proteinuria, depending on the specific disease.

Inherited and Congenital Kidney Diseases

Some kidney diseases result from hereditary factors. Polycystic kidney disease (PKD), for example, is a genetic disorder in which many cysts grow in the kidneys. PKD cysts can slowly replace much of the mass of the kidneys, reducing kidney function and leading to kidney failure.

Some kidney problems may show up when a child is still developing in the womb. Examples include autosomal recessive PKD, a rare form of PKD, and other developmental problems that interfere with the normal formation of the nephrons. The signs of kidney disease in children vary. A child may grow unusually slowly, vomit often, or have back or side pain. Some kidney diseases may be silent-causing no signs or symptoms-for months or even years.

If a child has a kidney disease, the child’s doctor should find it during a regular check-up. The first sign of a kidney problem may be high blood pressure; a low number of red blood cells, called anemia; proteinuria; or hematuria. If the doctor finds any of these problems, further tests may be necessary, including additional blood and urine tests or radiology studies. In some cases, the doctor may need to perform a biopsy.

Some hereditary kidney diseases may not be detected until adulthood. The most common form of PKD was once called “adult PKD” because the symptoms of high blood pressure and renal failure usually do not occur until patients are in their twenties or thirties. But with advances in diagnostic imaging technology, doctors have found cysts in children and adolescents before any symptoms appear.

Other Causes of Kidney Disease

Poisons and trauma, such as a direct and forceful blow to the kidneys, can lead to kidney disease.

Some over-the-counter medicines can be poisonous to the kidneys if taken regularly over a long period of time. Anyone who takes painkillers regularly should check with a doctor to make sure the kidneys are not at risk.

Acute Kidney Injury

Some kidney problems happen quickly, such as when an accident injures the kidneys. Losing a lot of blood can cause sudden kidney failure. Some drugs or poisons can make the kidneys stop working. These sudden drops in kidney function are called acute kidney injury (AKI). Some doctors may also refer to this condition as acute renal failure (ARF).

AKI may lead to permanent loss of kidney function. But if the kidneys are not seriously damaged, acute kidney disease may be reversed.

Chronic Kidney Disease

Most kidney problems, however, happen slowly. A person may have “silent” kidney disease for years. Gradual loss of kidney function is called chronic kidney disease (CKD) or chronic renal insufficiency. People with CKD may go on to develop permanent kidney failure. They also have a high risk of death from a stroke or heart attack.

End-stage Renal Disease

Total or nearly total and permanent kidney failure is called end-stage renal disease (ESRD). People with ESRD must undergo dialysis or transplantation to stay alive.

What are the signs of chronic kidney disease (CKD)?

People in the early stages of CKD usually do not feel sick at all.

People whose kidney disease has gotten worse may

  • need to urinate more often or less often
  • feel tired
  • lose their appetite or experience nausea and vomiting
  • have swelling in their hands or feet
  • feel itchy or numb
  • get drowsy or have trouble concentrating
  • have darkened skin
  • have muscle cramps

Blood Pressure Measurement

High blood pressure can lead to kidney disease. It can also be a sign that the kidneys are already impaired. The only way to know whether a person’s blood pressure is high is to have a health professional measure it with a blood pressure cuff. The result is expressed as two numbers. The top number, which is called the systolic pressure, represents the pressure in the blood vessels when the heart is beating. The bottom number, which is called the diastolic pressure, shows the pressure when the heart is resting between beats. A person’s blood pressure is considered normal if it stays below 120/80, stated as “120 over 80.” The NHLBI recommends that people with kidney disease use whatever therapy is necessary, including lifestyle changes and medicines, to keep their blood pressure below 130/80.

Microalbuminuria and Proteinuria

Healthy kidneys take wastes out of the blood but leave protein. Impaired kidneys may fail to separate a blood protein called albumin from the wastes. At first, only small amounts of albumin may leak into the urine, a condition known as microalbuminuria, a sign of deteriorating kidney function. As kidney function worsens, the amount of albumin and other proteins in the urine increases, and the condition is called proteinuria. A doctor may test for protein using a dipstick in a small sample of a person’s urine taken in the doctor’s office. The color of the dipstick indicates the presence or absence of proteinuria.

A more sensitive test for protein or albumin in the urine involves laboratory measurement and calculation of the protein-to-creatinine or albumin-to-creatinine ratio. Creatinine is a waste product in the blood created by the normal breakdown of muscle cells during activity. Healthy kidneys take creatinine out of the blood and put it into the urine to leave the body. When the kidneys are not working well, creatinine builds up in the blood.

The albumin-to-creatinine measurement should be used to detect kidney disease in people at high risk, especially those with diabetes or high blood pressure. If a person’s first laboratory test shows high levels of protein, another test should be done 1 to 2 weeks later. If the second test also shows high levels of protein, the person has persistent proteinuria and should have additional tests to evaluate kidney function.

Glomerular Filtration Rate (GFR) Based on Creatinine Measurement

GFR is a calculation of how efficiently the kidneys are filtering wastes from the blood. A traditional GFR calculation requires an injection into the bloodstream of a substance that is later measured in a 24-hour urine collection. Recently, scientists found they could calculate GFR without an injection or urine collection. The new calculation-the eGFR-requires only a measurement of the creatinine in a blood sample.

The eGFR calculation uses the patient’s creatinine measurement along with age and values assigned for sex and race. Some medical laboratories may make the eGFR calculation when a creatinine value is measured and include it on the lab report. The National Kidney Foundation has determined different stages of CKD based on the value of the eGFR. Dialysis or transplantation is needed when the eGFR is less than 15 milliliters per minute (mL/min).

Blood Urea Nitrogen (BUN)

Blood carries protein to cells throughout the body. After the cells use the protein, the remaining waste product is returned to the blood as urea, a compound that contains nitrogen. Healthy kidneys take urea out of the blood and put it in the urine. If a person’s kidneys are not working well, the urea will stay in the blood. A deciliter of normal blood contains 7 to 20 milligrams of urea. If a person’s BUN is more than 20 mg/dL, the kidneys may not be working at full strength. Other possible causes of an elevated BUN include dehydration and heart failure.

Additional Tests for Kidney Disease

If blood and urine tests indicate reduced kidney function, a doctor may recommend additional tests to help identify the cause of the problem.

Kidney Imaging

Methods of kidney imaging-taking pictures of the kidneys-include ultrasound, computerized tomography (CT) scan, and magnetic resonance imaging (MRI). These tools are most helpful in finding unusual growths or blockages to the flow of urine.

Kidney Biopsy

A doctor may want to examine a tiny piece of kidney tissue with a microscope. To obtain this tissue sample, the doctor will perform a kidney biopsy-a hospital procedure in which the doctor inserts a needle through the patient’s skin into the back of the kidney. The needle retrieves a strand of tissue less than an inch long. For the procedure, the patient lies face down on a table and receives a local anaesthetic to numb the skin. The sample tissue will help the doctor identify problems at the cellular level.

Severe reduction in eGFR (15 to 29)

The patient should continue following the treatment for complications of CKD and learn as much as possible about the treatments for kidney failure. Each treatment requires preparation. Those who choose hemodialysis will need to have a procedure to make veins in their arms larger and stronger for repeated needle insertions. For peritoneal dialysis, one will need to have a catheter placed in the abdomen. A catheter is a thin, flexible tube used to fill the abdominal cavity with fluid. A person may want to ask family or friends to consider donating a kidney for transplantation.

Kidney Failure (eGFR less than 15)

When the kidneys do not work well enough to maintain life, dialysis or a kidney transplant will be needed.

In addition to tracking eGFR, blood tests can show when substances in the blood are out of balance. If phosphorus or potassium levels start to climb, a blood test will prompt the health care provider to address these issues before they permanently affect the person’s health.

Treatment for Chronic Kidney Disease

What can be done about Chronis Kidney Disease (CKD)?

Unfortunately, CKD often cannot be cured. But people in the early stages of CKD may be able to make their kidneys last longer by taking certain steps. They will also want to minimize the risks for heart attack and stroke because CKD patients are susceptible to these problems.

  • People with reduced kidney function should see their doctor regularly. The primary doctor may refer the patient to a nephrologist, a doctor who specializes in kidney disease.
  • People who have diabetes should watch their blood glucose levels closely to keep them under control. They should ask their health care provider about the latest in treatment.
  • People with reduced renal function should avoid pain pills that may make their kidney disease worse. They should check with their health care provider before taking any medicine.

Controlling Blood Pressure

People with reduced kidney function and high blood pressure should control their blood pressure with an ACE inhibitor or an ARB. Many people will require two or more types of medication to keep their blood pressure below 130/80. A diuretic is an important addition when the ACE inhibitor or ARB does not meet the blood pressure goal.

Changing the Diet

People with reduced kidney function need to be aware that some parts of a normal diet may speed their kidney failure.

Protein

Protein is important to the body. It helps the body repair muscles and fight disease. Protein comes mostly from meat but can also be found in eggs, milk, nuts, beans, and other foods. Healthy kidneys take wastes out of the blood but leave in the protein. Impaired kidneys may fail to separate the protein from the wastes. Some doctors tell their kidney patients to limit the amount of protein they eat so the kidneys have less work to do. But a person cannot avoid protein entirely. People with CKD can work with a dietitian to create the right food plan.

Cholesterol

Another problem that may be associated with kidney failure is high cholesterol. High levels of cholesterol in the blood may result from a high-fat diet.

Cholesterol can build up on the inside walls of blood vessels. The buildup makes pumping blood through the vessels harder for the heart and can cause heart attacks and strokes.

Sodium

Sodium is a chemical found in salt and other foods. Sodium in the diet may raise a person’s blood pressure, so people with CKD should limit foods that contain high levels of sodium. High-sodium foods include canned or processed foods like frozen dinners and hot dogs.

Potassium

Potassium is a mineral found naturally in many fruits and vegetables, such as oranges, potatoes, bananas, dried fruits, dried beans and peas, and nuts. Healthy kidneys measure potassium in the blood and remove excess amounts. Diseased kidneys may fail to remove excess potassium. With very poor kidney function, high potassium levels can affect the heart rhythm.

Not Smoking

Smoking not only increases the risk of kidney disease, but it also contributes to deaths from strokes and heart attacks in people with CKD.

Treating Anemia

Anemia is a condition in which the blood does not contain enough red blood cells. These cells are important because they carry oxygen throughout the body. A person who is anemic will feel tired and look pale. Healthy kidneys make the hormone EPO, which stimulates the bones to make red blood cells. Diseased kidneys may not make enough EPO. A person with CKD may need to take injections of a form of EPO.

Preparing for End-stage Renal Disease (ESRD)

As kidney disease progresses, a person needs to make several decisions. People in the later stages of CKD need to learn about their options for treating the last stages of kidney failure so they can make an informed choice between hemodialysis, peritoneal dialysis, and transplantation.

Dialysis

The two major forms of dialysis are hemodialysis and peritoneal dialysis. Hemodialysis uses a special filter called a dialyzer that functions as an artificial kidney to clean a person’s blood. The dialyzer is a canister connected to the hemodialysis machine. During treatment, the blood travels through tubes into the dialyzer, which filters out wastes, extra salt, and extra water. Then the cleaned blood flows through another set of tubes back into the body. The hemodialysis machine monitors blood flow and removes wastes from the dialyzer. Hemodialysis is usually performed at a dialysis center three times per week for 3 to 4 hours. A small but growing number of clinics offer home hemodialysis in addition to standard in-clinic treatments. The patient first learns to do treatments at the clinic, working with a dialysis nurse. Daily home hemodialysis is done 5 to 7 days per week for 2 to 3 hours at a time. Nocturnal dialysis can be performed for 8 hours at night while a person sleeps. Research as to which is the best method for dialysis is under way, but preliminary data indicate that daily dialysis schedules such as short daily dialysis or nocturnal dialysis may be the best form of dialysis therapy.

In peritoneal dialysis, a fluid called dialysis solution is put into the abdomen. This fluid captures the waste products from a person’s blood. After a few hours when the fluid is nearly saturated with wastes, the fluid is drained through a catheter. Then, a fresh bag of fluid is dripped into the abdomen to continue the cleansing process. Patients can perform peritoneal dialysis themselves. Patients using continuous ambulatory peritoneal dialysis (CAPD) change fluid four times a day. Another form of peritoneal dialysis, called continuous cycling peritoneal dialysis (CCPD), can be performed at night with a machine that drains and refills the abdomen automatically.

Transplantation

A donated kidney may come from an anonymous donor who has recently died or from a living person, usually a relative. The kidney must be a good match for the patient’s body. The more the new kidney is like the person receiving the kidney, the less likely the immune system is to reject it. The immune system protects a person from disease by attacking anything that is not recognized as a normal part of the body. So the immune system will attack a kidney that appears too “foreign.” The patient will take special drugs to help trick the immune system so it does not reject the transplanted kidney. Unless they are causing infection or high blood pressure, the diseased kidneys are left in place. Kidneys from living, related donors appear to be the best match for success, but kidneys from unrelated people also have a long survival rate. Patients approaching kidney failure should ask their doctor early about starting the process to receive a kidney transplant.

What do the Kidneys do?

The kidneys are bean-shaped organs, each about the size of a fist. They are located near the middle of the back, just below the rib cage, one on each side of the spine. The kidneys are sophisticated reprocessing machines. Every day, a person’s kidneys process about 200 quarts of blood to sift out about 2 quarts of waste products and extra water. The wastes and extra water become urine, which flows to the bladder through tubes called ureters. The bladder stores urine until releasing it through urination.

Wastes in the blood come from the normal breakdown of active tissues, such as muscles, and from food. The body uses food for energy and self-repairs. After the body has taken what it needs from food, wastes are sent to the blood. If the kidneys did not remove them, these wastes would build up in the blood and damage the body.

The actual removal of wastes occurs in tiny units inside the kidneys called nephrons. Each kidney has about a million nephrons. In the nephron, a glomerulus-which is a tiny blood vessel, or capillary-intertwines with a tiny urine-collecting tube called a tubule. The glomerulus acts as a filtering unit, or sieve, and keeps normal proteins and cells in the bloodstream, allowing extra fluid and wastes to pass through. A complicated chemical exchange takes place, as waste materials and water leave the blood and enter the urinary system.

At first, the tubules receive a combination of waste materials and chemicals the body can still use. The kidneys measure out chemicals like sodium, phosphorus, and potassium and release them back to the blood to return to the body. In this way, the kidneys regulate the body’s level of these substances. The right balance is necessary for life.

In addition to removing wastes, the kidneys release three important hormones:

  • erythropoietin, or EPO, which stimulates the bone marrow to make red blood cells
  • renin, which regulates blood pressure
  • calcitriol, the active form of vitamin D, which helps maintain calcium for bones and for normal chemical balance in the body.
What is renal function?

The word “renal” refers to the kidneys. The terms “renal function” and “kidney function” mean the same thing. Health professionals use the term “renal function” to talk about how efficiently the kidneys filter blood. People with two healthy kidneys have 100 percent of their kidney function. Small or mild declines in kidney function-as much as 30 to 40 percent-would rarely be noticeable. Kidney function is now calculated using a blood sample and a formula to find the estimated glomerular filtration rate (eGFR).

The eGFR corresponds to the percent of kidney function available. Some people are born with only one kidney but can still lead normal, healthy lives. Every year, thousands of people donate one of their kidneys for transplantation to a family member or friend.

For many people with reduced kidney function, a kidney disease is also present and will get worse. Serious health problems occur when people have less than 25 percent of their kidney function. When kidney function drops below 10 to 15 percent, a person needs some form of renal replacement therapy-either blood-cleansing treatments called dialysis or a kidney transplant-to sustain life.

Why do Kidneys Fail?

Most kidney diseases attack the nephrons, causing them to lose their filtering capacity. Damage to the nephrons can happen quickly, often as the result of injury or poisoning. But most kidney diseases destroy the nephrons slowly and silently. Only after years or even decades will the damage become apparent. Most kidney diseases attack both kidneys simultaneously.

The two most common causes of kidney disease are diabetes and high blood pressure. People with a family history of any kind of kidney problem are also at risk for kidney disease.

How do Kidneys Fail?

Many factors that influence the speed of kidney failure are not completely understood. Researchers are still studying how protein in the diet and cholesterol levels in the blood affect kidney function.

What medical tests detect kidney disease?

A person’s eGFR is the best indicator of how well the kidneys are working. An eGFR of 90 or above is considered normal. A person whose eGFR stays below 60 for 3 months or longer has CKD. As kidney function declines, the risk of complications rises.

What are the stages of CKD?

Many factors that influence the speed of kidney failure are not completely understood. Researchers are still studying how protein in the diet and cholesterol levels in the blood affect kidney function.

What happens if the kidneys fail completely?

Total or nearly total and permanent kidney failure is called ESRD. If a person’s kidneys stop working completely, the body fills with extra water and waste products. This condition is called uremia. Hands or feet may swell. A person will feel tired and weak because the body needs clean blood to function properly. Untreated uremia may lead to seizures or coma and will ultimately result in death. A person whose kidneys stop working completely will need to undergo dialysis or kidney transplantation.

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