Nephrogenic Systemic Fibrosis

WHAT YOU SHOULD KNOW:

• 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. It is not clear exactly what causes NSF. It may be caused by a gadolinium dye used during a magnetic resonance imaging (MRI) test. Surgery and certain medicines and conditions may increase your risk for having NSF.
  
  
• With NSF, you may have itchy or painful skin-colored or reddish bumps on your skin. Your hands and feet may swell and you may have pain and weakness in your arms and legs. You may have chest pain or see yellow spots on your eyes. Blood tests and a deep skin biopsy may be needed to learn more about your condition. A computed tomography (CT) scan, magnetic resonance imaging (MRI), and an echocardiogram may also be used. Treatment may include medicines, dialysis, kidney transplant, photopheresis, and physical therapy. Having NSF found and treated early may decrease your symptoms and prevent further damage to your body.

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.

RISKS:

You may be allergic to some medicines for pain, and they may cause rashes or trouble breathing. With surgery, you may bleed more than usual, get an infection, or blood clots. Nerves, blood vessels, muscles, intestines, and other organs may get damaged. Without treatment, your symptoms will get worse. Your skin and muscles may continue to tighten causing deformities. You may have little or no movement and have to use a wheelchair. NSF may cause damage to your heart and lungs, and may cause death as your condition gets worse. Ask your caregiver if you have questions about your condition, treatment, or care.

WHILE YOU ARE HERE:

Informed consent:

A consent form is a legal document that explains the tests, treatments, or procedures that you may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.

IV:

An IV (intravenous) is a small tube placed in your vein that is used to give you medicine or liquids.

Vital signs:

Caregivers will check your blood pressure, heart rate, breathing rate, and temperature. They will also ask about your pain. These vital signs give caregivers information about your current health.

Medicines:

You may be given the following medicines:

• Pain medicine: Caregivers may give you medicine to take away or decrease your pain.
  
  
  • Do not wait until the pain is severe to ask for your medicine. Tell caregivers if your pain does not decrease. The medicine may not work as well at controlling your pain if you wait too long to take it.
    
    
  • Pain medicine can make you dizzy or sleepy. Prevent falls by calling a caregiver when you want to get out of bed or if you need help.
  
  

Tests:

• Blood gases: This is also called an arterial blood gas, or ABG. Blood is taken from an artery (blood vessel) in your wrist, arm, or groin. Your blood is tested for the amount of oxygen and carbon dioxide in it. The results can tell caregivers how well your lungs are working.
  
  
• 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.
  
  
• 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.
  
  
• Imaging tests: These tests may be done to let caregivers check for any damage inside your body from NSF. Certain imaging tests use a special dye to help pictures show up better. People who are allergic to iodine or shellfish (lobster, crab, or shrimp) may be allergic to some dyes. Tell your caregiver if you are allergic to shellfish, or have other allergies or medical conditions.
  
  
  • Computed tomography scan: This is also called a CT scan. A special x-ray machine uses a computer to take pictures of your body. It may be used to look at bones, muscles, brain tissue, and blood vessels.
    
    
  • Echocardiogram: This test is a type of ultrasound. Sound waves are used to show the structure, movement, and blood vessels of your heart.
    
    
  • Magnetic resonance imaging: This test is also called an MRI. During the test, pictures of your body are taken. An MRI may also be used to look at your brain, muscles, joints, bones, or blood vessels. You will need to lie still during an MRI. Never enter the MRI room with an oxygen tank, watch, or any other metal object. This can cause serious injury.

Treatment options:

• 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.

Copyright © 2011. Thomson Reuters. 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.

Learn more about Nephrogenic Systemic Fibrosis (Inpatient Care)