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Amyloidosis and its impact on patients with ESRD.

Proteins are the essential foundational tools for all body parts and processes and normally circulate throughout the body in the blood. Sometimes, cells in the body produce fragment or unusually shaped proteins that can settle as deposits in body tissues causing disease. These deposits of abnormal proteins are called amyloids and the disease process amyloidosis. There are different types of proteins that can form amyloid deposits, and two of them are closely related to kidney disease. In primary amyloidosis, abnormal protein production associated with plasma cell dyscrasia occurs as a first step and can lead to kidney disease. Dialysis-related amyloidosis (DRA), however, results from kidney disease (NIDDK, 2003).

Primary Amyloidosis

Primary amyloidosis occurs when the body produces abnormal protein fibers, which join together to form amyloid deposits in different organs, including the kidneys, where they cause serious damage (NIDDK, 2003). The kidneys become unable to function to remove urea and other wastes from the blood, resulting in the need for a dialysis prescription and therapy.

The initial sign that amyloidosis is present is the abnormally high amounts of protein in the urine or proteinuria (NIDDK, 2003). A kidney biopsy may be used to confirm the diagnosis of amyloidosis. No treatment has been found to be effective in reversing the effects of amyloidosis, although the use of melphalan and prednisone may help improve organ function and survival rates by interrupting the growth of cells that produce amyloid protein (NIDDK, 2003).

Dialysis-Related Amyloidosis

Patients, like those on dialysis, who have lost the ability to filter excess small proteins from their bodies, are at risk to develop DRA. When the kidneys do not work efficiently, a protein called beta-2-microglobulin can build up in the blood. Eventually, these molecules can form large deposits by forming a chain of small molecules into a large molecule and potentially damage surrounding tissues (NIDDK, 2003). Unfortunately, dialyzer membranes do not effectively remove these large molecules and as the blood levels become elevated, deposits begin forming in bone, joints, and tendons resulting in pain and/or stiffness.

Incidence

DRA has been found to be relatively common in patients on hemodialysis who have been receiving treatment for longer than 5 years, and especially among the elderly (NIDDK, 2003). DRA has also been found in patients who have been treated exclusively with CAPD (Cornelius et.al., 1989). The overall incidence of amyloidosis is 8 cases per million per year, representing 2,500 new cases annually in the United States. Peak occurrences of this disease are in ages 60-67 and are more common in males than females (Mayo, 2002).

Signs and Symptoms

Most symptoms of amyloidosis are nonspecific and often not recognized initially. Weight loss, fatigue, and weakness appear to be the most common symptoms reported. Others include: dyspnea, edema, and paresthesias. The primary signs and symptoms of DRA revolve around joint pain, fluid, and stiffness; neuropathy; nephropathy; bone fractures; ligament and tendon tears; and GI abnormalities (Sprague & Moe, 1996; Zingraff, 1990). Carpal tunnel syndrome is the most common complaint, occurring in about half of the patients who have DRA (NIDDK, 2003). Shoulders, back, hips, and neck joints are also affected in 20%30% of patients with DRA (Calal, Pavlovici, Mrzljak, & Jankovis, 1993).

Tests and Exams

Amyloidosis is uncommon and primary care physicians may not recognize when and how to test for the disease. Biopsy tissue that shows amyloid deposits on "Congo-red staining" is the only definitive confirmation of amyloidosis (NKF, 2003). Tissue that can be used for the biopsy includes: stomach fat aspirate, oral or rectal mucosa, bone marrow, or the specific organ affected (Mayo, 2002). Other tests commonly ordered to diagnose or prognose include: abdominal ultrasound, ECG, echocardiogram, nerve conduction tests, and renal function tests. BUN, serum creatinine, and urinary casts all act as predictors of disease and survival rates. Cystatin C. Serum -#82994 is a recently introduced test that provides a more sensitive indicator of glomerular filtration rate (GFR) and is proving very useful in diagnosing and monitoring nephritic syndrome caused by amyloidosis (Mayo, 2002).

Treatment

Unfortunately, no cure has been found, although a successful kidney transplant may stop DRA from progressing, according to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK, 2003). The National Kidney Foundation's K/DOQI Guidelines for Bone Metabolism and Disease in Chronic Kidney Disease suggests the use of high-flux dialyzers to slow the progression of the disease. Attempts are being made to develop membranes that can more efficiently remove beta-2-microglobulin from the blood during hemodialysis (NKF, 2003).

Primary amyloidosis is treated with combination drug therapy. Currently, melphalan (a common chemotherapy drug) and prednisone are used to slow progression of the disease (NIDDK, 2003). Clinical trials are presently underway using such drugs as dexamethasone, interferons, and thalidomide. Stem cell transplantation is also under investigation. Symptomatic treatment is the only option in many cases. Dialysis, renal transplantation, carpal tunnel release, and heart transplantation (in very selected cases) are some of the treatments available to relieve symptoms of amyloidosis.

Prognosis and Survival Rates

Drug therapy and good management may potentially slow the progression of the disease, but the prognosis remains bleak. Congestive heart failure is the single greatest prognostic indicator in patient survival (Mayo, 2002). Echocardiogram results are abnormal in one-third of patients at diagnosis, and almost one-half of patients have septal thickness of 15 mm or more. The thickness apparently reflects the amount of amyloid that has deposited in the myocardium and is also a predictor of patient survival. Patients who do not exhibit congestive heart failure have up to a 5-fold increase in estimated survival time (Mayo, 2002). Recent studies have demonstrated that high counts and percentages of peripheral blood plasma cells (PBPC) are associated with shorter survival in amyloidosis.

While mortality from DRA is rare, the disease can cause significant morbidity and is a major cause of immobility in patients on long-term dialysis. The K/DOQI Clinical Practice Guidelines for Bone Metabolism and Disease in Chronic Kidney Disease recognize the lack of quality studies in this area and suggest that it is a reflection of the slow progressive nature of the disease as well as the difficulty in diagnosing when symptomology is vague (NKF, 2003). The guidelines do not recommend routine screening of the patients for the presence of beta-2-microglobulin.

Implications for Practice

Implications for practice include patient teaching, optimal pain management, fall risk management, and early diagnosis. Patient teaching for amyloidosis needs to be specific to the disease process, appropriate pharmacological and nonpharmacological pain management alternatives, musculoskeletal concerns, and appropriate lab tests. Patients also need to be aware of current options in treatment management: use of high flux dialyzers, transplantation, surgery, etc. Safety concerns for this population would include a fall risk plan and interventions due to the musculoskeletal pain and weaknesses. Safety equipment for the home and/or work area are recommended. The impact of carpal tunnel syndrome on an arm that already contains a graft or fistula for hemodialysis should be explored with the patient and lifestyle alterations considered.

Pain management techniques include teaching on a pain assessment scale, evaluation of interventions, and the use of safety devices. Teaching on appropriate pain relief medications is imperative, especially if it is dialysis-related amyloidosis. The medications must also be kidney friendly.

Finally, early diagnosis is essential but difficult. Since many symptoms are vague, the diagnosis can be easily missed. Community education and education in the dialysis arena could trigger earlier lab tests and the use of preventive techniques (such as type of dialyzer). Patients and family members need to be aware of resources (see Table 1) and support groups available to them for more information on current clinical trials, treatment modalities, and emotional reinforcement.

This offering for 1.0 contact hour is being provided by the American Nephrology Nurses' Association (ANNA). ANNA is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's Commission on Accreditation. ANNA is a provider approved by the California Board of Registered Nursing, provider number CEP 00910.

The Nephrology Nursing Certification Commission (NNCC) requires 60 contact hours for each recertification period for all nephrology nurses. Forty-five of these 60 hours must be specific to nephrology nursing practice. This CE article may be applied to the 45 required contact hours in nephrology nursing.

References

Calal, S., Pavlovicl, D., Mrzljak, V., & Jankovis, N. (1993). Symptoms of dialysis-related amyloidosis in diabetic patients. Professional paper: Croatia Nephrology and Dialysis Department.

Cornelis, F., Bardin, T., Faller, B., Verger, C., Allouache, M., & Raymond, P., et al. (1989). Rheumatic syndromes and beta-2-microglobulin amyloidosis in patients receiving long-term peritoneal dialysis. Arthritis & Rheumatology, 32, 785-788.

Mayo Reference Services. (2002). Mayo Clinic communique: The laboratory diagnosis and monitoring of amyloidosis. Rochester, MN: Mayo Reference Services.

National Kidney Foundation. (2003). Clinical practice guidelines for bone metabolism and disease in chronic kidney disease. Retrieved January 8, 2006, from www.kidney.org/professionals/ kdoqi/guidelines_bone/Guide 10.htm

National Institute of Diabetes and Digestive and Kidney Diseases. (NIDDK). (2003). Retrieved January 8, 2006, from www.kidney.niddk.nih.gov/kudiseases/pubs/ amyloidosis

Sprague, S.M., & Moe, S.M. (1996). Clinical manifestations and pathogenesis of dialysis-related amyloidosis. Seminars in Dialysis, 9, 360-369.

Zingraff, J.J., Noel, L.H., Bardin, T., Atienza, C., Zins, B., Drueke, T.B., & Kuntz, D. (1990). Beta-2-microglobulin amyloidosis in chronic renal failure. New England Journal of Medicine, 323, 1070-1071.

Sandra D. Copeland, MSN, RN, BC, is Clinical Nurse Specialist for Renal Care Services, Medical Center of Central Georgia, Macon, GA. She is a member of the Masters City Chapter of ANNA.</p> <pre> Table 1 Amyloidosis Resources The following support groups and information sites exist for patients with amyloidosis, their families, and healthcare providers: Amyloidosis Support Network 1490 Herndon Lane Marietta, GA 30062 www.amyloidosis.org Amyloidosis Network International, Inc 7118 Cole Creek Drive Houston, TX 77092-1421 1-888-AMYLOID Amyloid Treatment and Research Center http://amyloid.bu.edu/amyloid/amyloid1.htm Association for Neuro-Metabolic Disorders 5223 Brookfield Lane Sylvania, OH 43560-1809 1-419-885-1497 http://www.kumc.edu/gec/support/neuro-me.html National Institutes of Health National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse One AMS Circle Bethesda, MD 20892-3675 1-301-495-4484 www.nih.gov/niams/healthinfo National Kidney and Urologic Diseases Information Clearinghouse 3 Information Way Bethesda, MD 20892-3580 1-800-891-5390 www.niddk.nih.gov National Kidney Foundation 30 East 33rd Street Suite 1100 New York, NY 10016 1-800-622-9010 www.kidney.org </pre> <p>Amyloidosis and Its Impact on Patients With ESRD

Posttest--1.0 Contact Hour Posttest Questions

(See posttest instructions on the answer form, on page 35.)

1. Which statement is correct in comparing and contrasting primary and dialysis-related amyloidosis (DRA)?

A. Primary amyloidosis is a result of kidney disease where as DRA is caused by overproduction of proteins.

B. Effective treatments are available for both primary and dialysis-related amyloidosis.

C, X-rays are useful in diagnosis of primary and dialysis-related amyloidosis.

D. Primary amyloidosis may lead to kidney disease.

2. Which patient is most likely to have DRA?

A. 75 year-old male patient on hemodialysis for 1 year.

B. 50 year-old female patient on hemodialysis dialysis for 5 years.

C. 81 year-old male patient on peritoneal dialysis for 6.2 years.

D. 57 year-old female patient on peritoneal dialysis for 2.5 years.

3. The most common complaint of a patient with DRA is

A. stiffness.

B. joint pain.

C. neuropathy.

D. carpal tunnel syndrome.

4. The most common complaint of a patient with primary amyloidosis is

A. joint paint.

B. fatigue and weakness.

C. shortness of breath.

D. gastric reflux.

5. Progression of DRA can be stopped with

A. kidney transplant.

B. heart transplant.

C. hi-flux dialysis.

D. daily dialysis.

6. What statement is true in comparing and contrasting the prognosis of primary and dialysis-related amyloidosis?

A. Congestive heart failure is the greatest prognostic indication of DRA.

B. Septal thickness is a predictor of patient survival in primary amyloidosis.

C. Primary amyloidosis causes significant immobility contributing to increased morbidity.

D. DRA offers a bleak prognosis for most patients.

7. Nursing care of patients with primary or dialysis-related amyloidosis may include:

A. pain management only.

B. pain management and patient teaching concerning the disease process only.

C. pain management, patient teaching concerning the disease process, and a fall risk plan.

D. pain management, patient teaching concerning the disease process, a fall risk plan, and use of high-flux dialyzers.

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Title Annotation:Continuing Education; End-Stage Renal Disease
Author:Copeland, Sandra D.
Publication:Nephrology Nursing Journal
Geographic Code:1USA
Date:Jan 1, 2006
Words:2048
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