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. 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.

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: You have the right to understand your health condition in words that you know. You should be told what tests, treatments, or procedures may be done to treat your condition. Your doctor should also tell you about the risks and benefits of each treatment. You may be asked to sign a consent form that gives caregivers permission to do certain tests, treatments, or procedures. If you are unable to give your consent, someone who has permission can sign this form for you. A consent form is a legal piece of paper that tells exactly what will be done to you. Before giving your consent, make sure all your questions have been answered so that you understand what may happen.

IV: An IV is a tube placed in your vein for giving medicine or liquids. This tube is capped or connected to tubing and liquid.

Vital signs: This includes taking your temperature, blood pressure, pulse (counting your heartbeat), and respirations (counting your breaths). To take your blood pressure, a cuff is put on your arm and tightened. The cuff is attached to a machine which gives your blood pressure reading. Caregivers may listen to your heart and lungs by using a stethoscope. Your vital signs are taken so caregivers can see how you are doing.

Medicines: Your caregiver may give you the following kinds of 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.
  
  

Tests:

• Blood gases: This test is also called an arterial blood gas or ABG test. Blood is taken from an artery (blood vessel) in your wrist, arm, or groin. The groin is the area where your abdomen meets your upper leg. Your blood is tested for the amount of "gases" in it, such as oxygen, acids, and carbon dioxide.
  
  
• 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.
  
  
• 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 also called an echo. It is a type of ultrasound, using sound waves to show pictures of the size and shape of your heart. An echo also looks at how your heart moves when it is beating. These pictures are seen on a TV-like screen.
      
      
    • This test is done while lying down on your back. Clear jelly will be squirted on your chest to help the ultrasound sensor slide easily. The sensor will be rubbed across your chest to see your heart from different angles. You may hear a whooshing noise, which is the sound of your blood flow. Caregivers may ask you to pedal a bike during the test (exercise echo) or you may get medicine before the test to increase blood flow to your heart muscle (stress echo). This test can tell how well your heart is pumping. An echo can also find problems, such as fluid around the heart or problems with your heart valves.
    
    
  • 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 © 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.

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