Managing the Patient with Amyloidosis

Jan D. Cannon; Richard L. Pullen; Jill D. Rushing

Disclosures

Dermatology Nursing. 2004;16(3) 

In This Article

Nursing Assessment and Patient Education

A head-to-toe assessment is conducted to evaluate health status, responses to medications and other therapies, and pain or discomfort, which may be affecting the patient's activities of daily living. Assessment of emotional support is also essential. The assessment includes an evaluation for protein in the urine, cardiopulmonary involvement, nerve damage, gastrointestinal function, including malabsorption, and cutaneous lesions including bruising.

The kidneys become hard and small with buildup of amyloid. It is important for the nurse to assess the extent of kidney involvement in amyloidosis and the development of the nephrotic syndrome. The nephrotic syndrome is suspected when there are high levels of protein in the urine, low levels of protein in the blood, and when the nurse assesses edema in the patient's feet, ankles, calves, scrotum, abdomen, and eyelids. Low protein levels may lead to an increase in total cholesterol and triglyceride levels rendering the patient more susceptible to cardiovascular disease and weakness, fatigue, and weight loss from malnutrition. It is not unusual for the patient with kidney involvement to lose up to 25 pounds and develop a distaste for certain protein-enriched foods. The patient may also experience an abnormally high or abnormally low blood pressure in nephrotic syndrome (DiSalvo et al., 2002; Khan & Falk, 2001).

Amyloid protein can limit the ability of the heart to fill properly, thus the cardiovascular system should be assessed to determine the presence of progressive heart failure, which constitutes 50% of the deaths in patients with primary amyloidosis. This includes assessing shortness of breath (even with the slightest exertion), peripheral edema, enlarged liver, and hepatojugular reflex and jugular vein distension. The nurse should also auscultate the cardiac valves for murmurs, gallops, or rubs, and the lungs for crackles, which may indicate heart failure. Electrophysiological changes in myocardial function commonly occur in patients with heart failure secondary to amyloidosis. The peripheral pulses should be evaluated for rate, amplitude, and regularity. Low-voltage electrocardiograms are common features in amyloidosis and should be evaluated for dysrhythmias. Patients should also be monitored for pulmonary hypertension accompanying heart failure, which is a marker of advanced amyloidosis (Bernardi et al., 2002; Dingli et al., 2001; Nakata & Shimamoto, 2003; Smith, 2003).

An assessment of the nervous system is necessary to determine the extent of autonomic dysfunction. Symptoms of postural hypotension occurring with renal and cardiac involvement are often indicators of progressive illness. Pupillary changes are common. The nurse should assess the pupils for size, symmetry, reaction to a light stimulus, and the six conjugate eye movements. Polyneuropathies may also occur. It is important to determine if the patient is experiencing neuropathic pain such as numbness, tingling, or burning sensations in the fingers, toes, or soles of the feet. The chronic pain may lead to progressive fatigue and weakness. In addition, one out of every four patients with amyloidosis presents with carpal tunnel syndrome (painful disorder resulting in numbness and tingling in response to pressure on the nerves around the wrist). Monitoring urinary patterns is also essential, since autonomic dysfunction can lead to neurogenic bladder. Also, if nerves controlling bowel function are affected, diarrhea alternating with periods of constipation may ensue (Khan & Falk, 2001; Nakata & Shimamoto, 2003; Smith, 2003).

The gastrointestinal tract may be affected by autonomic dysfunction or direct involvement of the bowels as a result of amyloid deposits. The patient should be assessed for abdominal symmetry, bloating, bowel sounds, and bowel patterns. Malabsorption of nutrients can occur as a result of chronic diarrhea. Changes in the characteristics of bowel sounds or absent bowel sounds may indicate bowel obstruction and is a life-threatening complication. Palpation of the abdomen often reveals hepatomegaly and splenomegaly. Decreased liver function is a late sign of the disease course. Protein accumulation in the spleen can contribute to an increased risk of splenic rupture due to trauma. Macroglossia (enlarged tongue) with swelling under the jaw may be present and can lead to swallowing difficulties, breathing difficulties, and sleep apnea. It is essential for the nurse to assess the patient's ability to chew and swallow food and characteristics of the respiratory rate, depth, and rhythm (Khan & Falk, 2001; Nakata & Shimamoto, 2003; Smith, 2003).

The clinical pattern of amyloid involvement with the skin occurs in about half of all cases of both primary and secondary amyloidosis and in all cases where there is inflammation of the peripheral nerves, thus making skin assessment essential. It is important for the nurse to assess the location and characteristics of lesions. Waxy-appearing papules may present on the face and neck, the groin, axillae, anal area, tongue, or in ear canals. Edema, purpura (hemorrhage beneath the skin), dry mouth, and hair loss may also occur. Lichenoid lesions form papules and plaques on exterior skin surfaces and are associated with pruritus (see Figure 1). Nodular cutaneous lesions result from amyloid deposits throughout the dermis and often present with facial involvement (Figures 2, 3, & 4). Periorbital and facial bruising such as petechia, purpura, and eccyhmoses result from vascular fragility induced by the deposition of amyloid (Figures 3, 4, & 5). Unexplained low clotting indices should alert the nurse and other health professionals to the possibility of this condition, where factor X deficiency may occur as a result of its binding to amyloid fibrils (Chotzen et al., 1995; Hung, Wang, Hong, & Kuo, 2003).

Figure 1.

Nodules in amyloidosis. Photo courtesy of EMH Swiss Medical Publishers.

Figure 2.

Facial involvement with amyloidosis. The patient has extensive nodules with purpura involving the eyelids and the naso-labial folds. Photo courtesy of Regina Gandour-Edwards, MD, Associate Professor and Pathologist, University of California Medical School at Davis.

Figure 3.

Amyloid nodule involving the upper lip, a typical site of extensive involvement with amyloidosis. Photograph courtesy of Regina Gandour-Edwards, MD, Associate Professor and Pathologist, University of California Medical School at Davis.

Figure 4.

Amyloidosis of the trunk. The upper abdomen and lower chest are involved with nodules and purpura. Photograph courtesy of Regina Gandour-Edwards, MD, Associate Professor and Pathologist, University of California Medical School at Davis.

Figure 5.

Periorbital hemorrhage in amyloidosis. Photo courtesy of EMH Swiss Medical Publishers.

It may take time to determine a definitive diagnosis of amyloidosis. Differential diagnoses should be considered (see Table 3 ). Numerous clinical trials are being conducted to improve the care and management of patients with amyloidosis. These clinical trials are available on the National Institutes for Health Web page (www.clinicaltrials.gov). The following supportive therapies will help to improve the quality of life for many individuals with amyloidosis. Encourage them to:

  • Eat a well-balanced and nutritious diet. Although amyloid is an abnormal protein, the amount of protein in the diet does not affect the onset of the disease. A diet low in protein may be necessary when the kidneys are involved.

  • Limit fat intake in the diet and seek treatment for hyperlipidemia.

  • Maintain ideal body weight, weigh daily, and monitor blood pressure and pulse daily.

  • Limit salt intake to reduce fluid retention.

  • Get up slowly from a sitting or lying position to prevent lightheadedness or dizziness.

  • Have frequent rest periods throughout the day. Talk with the health provider about activities that are appropriate.

  • Perform activities of daily living cautiously. Large amounts of amyloid in the bone can make the patient susceptible to fractures.

  • Report side effects and/or adverse effects of medications and other treatments to the health care provider.

  • Undergo regular eye examinations (especially in patients with familial amyloidosis) due to amyloid deposition behind the lens.

  • Seek genetic counseling to learn about the risks of passing the condition to their children when hereditary amyloidosis is suspected.

  • Participate in self-help groups and professional organizations that help individuals with amyloidosis (see Table 4).

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