Nephrogenic Systemic Fibrosis

GENERAL INFORMATION:

What is nephrogenic systemic fibrosis? Nephrogenic systemic fibrosis is also called NSF. It is a condition normally found in people with kidney disease. With NSF your skin becomes thick and hard, mostly on your arms and legs. Your muscles, joints, organs, and the tissue that covers your brain may also be involved. NSF may progress quickly causing very bad pain, and may be life threatening. Treatment of NSF is done to improve your kidney function and decrease your pain and other symptoms.

What causes nephrogenic systemic fibrosis? It is not clear what causes NSF. Caregiver's believe the condition may be caused by a contrast dye called gadolinium. This dye may be given to you during a magnetic resonance imaging (MRI) test. An MRI take pictures of your body and the dye helps your caregiver see the pictures better. Normally, this dye is removed from your body when you pass urine. With kidney disease you either do not pass urine, or you pass it in very small amounts. People with kidney disease may have trouble removing the dye from their body. When this happens, the dye can be harmful to your body and cause damage.

What increases my risk of having nephrogenic systemic fibrosis? Having kidney disease with any of the following may increase your risk of having NSF:

• Medicines: These may include medicines that cause lactic acidosis or increase the red blood cells in your body. Lactic acidosis occurs when medicines decrease the amount of oxygen and blood flow to your tissues. Ask your caregiver for more information about these medicines.
  
  
• Surgery: Having surgery may increase your risk of having NSF. This most often happens when you have vascular surgery. Vascular surgery is done to repair your blood vessels.
  
  
• Other medical conditions: These may include hepatitis, pulmonary fibrosis, deep vein thrombosis, or other blood disorders. Metabolic acidosis or having too much iron or phosphate in your body may increase you risk. High blood and tissue levels of metals such as copper and zinc may also increase your risk. Ask your caregiver for more information about these disorders.

What are the signs and symptoms of nephrogenic systemic fibrosis? You may have any of the following:

• Chest pain.
  
  
• Hair loss.
  
  
• Painful or itchy skin.
  
  
• Skin colored or reddish bumps on your skin.
  
  
• Swelling of your hands and feet, and pain and muscle weakness in your arms or legs.
  
  
• Thick, hard, or dimpled skin like that of an orange peel.
  
  
• Yellow spots or redness on the whites of your eyes.

How is nephrogenic systemic fibrosis diagnosed? Your caregiver may ask questions about your health and any tests or surgeries you have had. Your caregiver may also ask about medicines you have taken or are presently taking. You may also need the following tests:

• Blood tests: You may need blood taken for tests. The blood can be taken from a blood vessel in your hand, arm, or the bend in your elbow. It is tested to see how your body is doing. It can give your caregivers more information about your health condition. You may need to have blood drawn more than once. Deep skin biopsy: This is when a sample of your skin is taken and sent to a lab for tests. Your skin is cleaned before a numbing medicine is put into it to control pain. A bandage is put on the biopsy area after the tissue sample is taken.
  
  

How is nephrogenic systemic fibrosis treated? The goal of treatment is to improve your kidney function, decrease your pain, and improve you joint movement. You may need any of the following:

• Medicines:
  
  
  • Pain medicine: Caregivers may give you medicine to take away or decrease your pain. Medicine may be given regularly, or may only be given if you ask caregivers for it. Tell caregivers if your pain does not decrease enough for you to feel better. Do not wait to ask for your pain medicine until the pain is very bad. The medicine may not work as well at controlling your pain if you wait too long to take it. Ask your caregiver for help getting out of bed if you feel tired or dizzy.
    
    
  
  
• Physical therapy: This may include activities such as swimming and deep massage. These forms of therapy may help prevent, or slow down joint contractures. A contracture is when your skin or muscles tighten, causing problems with how you move your joints.
  
  
• Procedure or surgery:
  
  
  • 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.
    
    
  • Kidney transplant: This is surgery to replace your damaged kidney with a new one from a donor (another person). The donor may be a family member, close friend, or someone you do not know.
    
    
  • Photopheresis: This is when your blood is removed from your body and treated with ultraviolet (UV) light and medicine. After treatment, your blood is then returned to your body. This may help to decrease you symptoms and improve your joint movement.

When should I call my caregiver? Call your caregiver if:

• Your arms or legs are swollen, or you have trouble moving them.
  
  
• Your skin becomes red or painful.
  
  
• You have questions or concerns about your condition, treatment, or care.

When should I seek immediate help? Seek care immediately or call 911 if:

• You suddenly have trouble breathing.
  
  
• You have worsening pain that does not get better after taking medicine.

Where can I find more information? Contact the following:

• National Kidney Foundation
  30 East 33rd Street
  New York, NY 10016
  Phone: 1-212-889-2210
  Phone: 1-800-622-9010
  Web Address: http://www.kidney.org
  

CARE AGREEMENT:

You have the right to help plan your care. To help with this plan, you must learn about your health condition and how it may be treated. You can then discuss treatment options with your caregivers. Work with them to decide what care may be used to treat you. You always have the right to refuse treatment.

Copyright © 2008 Thomson Healthcare Inc. All rights reserved. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes.

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.

Link to Page

Print Page

Email Page