COLLABORATIVE

Therapy is targeted to amelioration of the underlying organ dysfunction through pharmacological therapy, but there is no known cure. Surgical procedures may be used to treat severe symptoms. The patient may develop a complication of the tongue called macroglossia. If this occurs, a tracheotomy may be necessary to maintain oxygenation. Patients with severe renal amyloidosis and azotemia may undergo bilateral nephrectomy and renal transplantation followed by immune therapy, although the donor kidney may be susceptible to amyloidosis as well. Strict control of blood glucose levels and treatment of hypertension are important for people with renal amyloidosis.

OTHER MANAGEMENT. A dietary consultation can provide the patient with a plan to supplement needed nutrients and bulk-forming foods based on the patient's symptoms. Unless the patient requires fluid restriction, he or she needs to drink at least 2 L of fluid per day. A referral to a speech therapist may be necessary if the patient's tongue prevents clear communication.

Pharmacologic Highlights

Medication or Drug Class	Dosage	Description	Rationale
Melphalan and prednisone combined therapy	Varies with drug protocol	Antineoplastic alkylating agent and corticosteroid	Interrupts the growth of the abnormal cells that produce amyloid protein; decreases amyloid deposits; no known effective therapy to reverse amyloidosis

Other Therapies: Dimethylsulfoxide (DMSO) and colchicine have been used at times to decrease amyloid deposits. To prevent serious cardiac complications in patients with cardiac amyloidosis, antidysrhythmic agents are prescribed. Digitalis is avoided because patients are susceptible to toxicity. Vitamin K is used to treat coagulation problems, and analgesics are prescribed for pain. As the disease progresses and malabsorption develops secondary to GI involvement, parenteral nutrition is used to meet nutritional needs.

INDEPENDENT

Maintain a patent airway when the patient's tongue is involved. Prevent respiratory tract complications by gentle and adequate suctioning when necessary. Keep a tracheotomy tray at the patient's bedside in case of airway obstruction. When the patient is placed on bedrest, institute measures to prevent atrophy of the muscles, development of contractures, and formation of pressure ulcers.

Provide a pleasant environment to stimulate the patient's appetite. Give oral hygiene before and after meals and assist the patient as needed with feeding. Note that the disease puts tremendous stressors on the family and patient as they cope with a chronic disease without hope of recovery. Encourage the patient to verbalize her or his feelings. Involve loved ones in the care of the patient, and involve the patient in all discussions surrounding his or her care. Present a realistic picture of the prognosis of the illness, but do not remove the patient's hope. This illness tends to be progressive and debilitating with significant dysfunction of the involved organs. Long-term health planning is essential. Refer the patient and family to the chaplain or a clinical nurse specialist for counseling if appropriate.