Hemolytic Uremic Syndrome

What is hemolytic uremic syndrome?

Hemolytic (he-mo-LIT-ik) uremic (u-RE-mik) syndrome (SIN-drome), or HUS, is a condition that affects the blood, small blood vessels, and kidneys. An injury to a blood vessel wall normally triggers platelets (blood clotting cells) to start clotting, which stops bleeding. With HUS, there is an uncontrolled clumping of platelets in the injured blood vessel. This decreases the amount of freely moving platelets in the blood and causes narrowing of the blood vessel. Red blood cells going through these narrowed blood vessels are often hemolysed (destroyed). Anemia (decreased red blood cells) and ischemia (decreased blood supply) may then occur. The small blood vessels of the kidneys are most often affected and cause kidney failure.

The kidneys are most often affected. The kidneys are two bean-shaped organs that remove unwanted chemicals and waste from the blood. These wastes are turned into urine by the kidneys. The kidneys are found in the back of the abdomen (stomach) on both sides of the spine. Other body parts that may be affected by HUS are the intestines (bowels), pancreas, and muscles.

What causes hemolytic uremic syndrome?

Eating food containing bacteria (germs) is the most common trigger in HUS. These bacteria, called E. coli, often release harmful substances that injure the blood vessels. Other things that may cause injury to the blood vessel wall include any of the following:

• Bone marrow transplant.
  
  
• Cancer and anti-cancer medicines.
  
  
• Conditions affecting the immune system, such as systemic lupus erythematosus (SLE) and human immunodeficiency virus (HIV) infection.
  
  
• Medicines, such as antiarrhythmia, antimotility, and antiplatelet agents.
  
  
• Pregnancy.

What are the signs and symptoms of hemolytic uremic syndrome?

You may have any of the following:

• Abdominal (stomach) pain, vomiting, and diarrhea (loose bowel movement).
  
  
• Bleeding from the lips, mouth, or nose.
  
  
• Coughing and trouble breathing.
  
  
• Decreased or no urine output, or blood in the urine.
  
  
• Fever
  
  
• High blood pressure.
  
  
• Pale or bluish color of skin, lips, or nails.
  
  
• Pinpoint reddish spots or purple-colored bruising of the skin.

How is hemolytic uremic syndrome diagnosed?

You may need any of the following tests:

• Blood tests: You may need blood taken to give caregivers information about how your body is working. The blood may be taken from your hand, arm, or IV.
  
  
• Percutaneous kidney biopsy: A percutaneous kidney biopsy is when a very small piece of your kidney is taken out and tested. For this procedure, you will need to lie face-down and hold very still. You may get medicine to help you relax before the biopsy. You may also get medicine to make the area numb (lose feeling) before the needle is put in. Caregivers put a needle into your back and through to your kidney. The needle has a sharp edge that will cut out a tiny piece of your kidney. The needle may have to be put in two or more times. After the needle is taken out, a bandage will be put over the area.
  
  
• Stool sample: A sample of your stool or bowel movement (BM) is sent to a lab for tests. The stool may show what germ is causing your illness. This helps caregivers learn what medicine is best to treat you.
  
  
• 24 hour urine test: During this test you will need to collect all of your urine for 24 hours. You will urinate into a container and the urine will be put into a jug. The jug will need to be kept cold. If you urinate during the night, you will need to save that urine. Caregivers will measure and record how much you urinate. At the end of 24 hours, the urine will be sent to a lab for tests.

How is hemolytic uremic syndrome treated?

You may need any of the following:

• Intravenous fluid therapy: You may need to stay in the hospital to receive intravenous (IV) fluid. This is to maintain the fluid and salt balance in your body. An IV is a tube placed in the vein for giving medicine or liquids. This tube is capped or connected to tubing and liquid.
  
  
• Medicines: These may be given to control symptoms that may occur with hemolytic uremic syndrome.
  
  
  • Anticonvulsant medicine: This medicine is given to control seizures. Take this medicine exactly as directed.
    
    
  • Blood pressure medicine: This is given to lower your blood pressure. A controlled blood pressure helps protect your organs, such as your heart, lungs, brain, and kidneys. Take your blood pressure medicine exactly as directed.
    
    
  • Steroids: This medicine may be given to decrease inflammation.

Your treatment may change if your HUS causes further problems, such as anemia and kidney failure. This is often decided after you have tests. You may have any of the following:

• Blood transfusion: You will get whole or parts of blood through an IV during a transfusion. Blood is tested for diseases, such as hepatitis and HIV, to be sure it is safe.
  
  
• Dialysis: Dialysis cleans your blood when your kidneys cannot. Extra water, chemicals, and waste products are removed from your blood by a dialyzer or dialysis machine. The dialysis machine does this by passing your blood through a special filter, then returning it back to you. You may need dialysis for a short time, or for the rest of your life. Caregivers will check your vital signs often during dialysis. You may also be given medicines or have blood taken for lab tests during dialysis.
  
  
• Plasma exchange: This treatment removes plasma (fluid portion) from your blood, and replaces it with plasma from a donor (from another person). A substitute fluid, such as albumin, may also be used. An IV tube is put in your vein and some of your blood is removed. A special machine spins and separates the plasma from the blood cells. Your plasma is then taken out and replaced by donor plasma or albumin. The blood cells, together with the replacement plasma or albumin, are then put back in your body through the IV. Ask your caregiver for more information about plasma exchange as a treatment option.
  
  
• Kidney transplant: In severe hemolytic uremic syndrome, where the kidneys are heavily damaged, a kidney transplant may be done. This is surgery to put a healthy kidney from another person into your body. The donated kidney does the work that your two failed kidneys used to do. Your diseased or injured kidneys will be removed during the transplant. Ask your caregiver for more information about kidney transplant as a treatment option.

With treatment, such as medicine and plasma exchange, your symptoms may be relieved and your quality of life may be improved.

Where can I find support and more information?

Having hemolytic uremic syndrome may be a life-changing disease for you and your family. Accepting that you have hemolytic uremic syndrome may be hard. You and those close to you may feel angry, sad, or frightened. These feelings are normal. Talk to your caregivers, family, or friends about your feelings. You may also want to join a support group. This is a group of people who also may have HUS. Contact any of the following for more information:

• National Heart, Lung and Blood Institute
  Health Information Center
  P.O. Box 30105
  Bethesda , MD 20824-0105
  Phone: 1- 301 - 592-8573
  Web Address: http://www.nhlbi.nih.gov/health/infoctr/index.htm
  

• National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
  Building 31, room 9A04 Center Drive, MSC 2560
  Bethesda , MD 208922560
  Web Address: http://www.niddk.nih.gov
  

• American Kidney Fund
  6110 Executive Boulevard, Suite 1010
  Rockville , MD 20852
  Phone: 1- 800 - 638-8299
  Web Address: http://www.akfinc.org
  

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.

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The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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