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GENDER AND LIFE SPAN CONSIDERATIONS

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A traumatic amputation is usually the result of an industrial accident, in which blades of heavy machinery sever part of a limb. A healthy young person who suffers a traumatic amputation with- out other injuries is often a good candidate for limb salvage. Reattachment of a limb will take place as soon as possible following the injury. The chief problems are hemorrhage and nerve damage. A closed amputation is the most common surgical procedure today. The bone is severed somewhat higher than the surrounding tissue, with a skin flap pulled over the bone end, usually from the pos- terior surface. This procedure provides more even pressure for a weight-bearing surface, promot- ing healing and more successful use of a prosthesis. (See Table 7 for levels of amputations.)

Amputation 63

•TABLE 7 Levels of Amputation

TYPE DESCRIPTION

Below the knee

Syme procedure

Transmetatarsal/toe amputation Hip disarticulation/

extensive hemipelvectomy Upper extremity

Most common surgical site, performed rather than above-the-knee amputation whenever possible because the higher the amputation performed, the greater the energy required for mobility

Partial amputation with salvage of the ankle; has advantages over loss of the limb higher up because it does not require a prosthesis and produces less weight-bearing pain

Involves removal of limited amount of limb; requires little rehabilitation; often followed by additional surgery at a higher level because of inadequate heal- ing when used for patients with peripheral vascular disease

Usually performed for malignancies; because of the surgery involved and the extremely bulky prosthesis required for ambulation, these procedures are used either as life-saving measures in young patients or as a surgery of last resort for pain control

Performed with salvage of as much normal tissue as possible. Function of the upper extremity is vital to normal activities of daily living. Much research is being conducted at the current time to manufacture and refine prostheses that can substitute for the patient’s arm and hand, providing both gross and fine movement. Cosmetic considerations are extremely important in the loss of the upper extremity for many patients.

Note:The terms “below the knee amputation,” or BKA, and “above the knee amputation,” or AKA, are also commonly used.

CAUSES

Peripheral vascular disease is much more frequently the cause of amputation than are traumatic injuries. Poor foot care, impaired circulation, and peripheral neuropathy lead to serious ulcerations, which may be undetected until it is too late to salvage the foot or even the entire limb. Congenital deformity, severe burns, and crushing injuries that render a limb permanently unsightly, painful, or nonfunctional may be treated with surgical amputation. Tumors (usually malignant) and chronic osteomyelitis that does not respond to other treatment may also necessitate amputation.

GENETIC CONSIDERATIONS

No clear genetic contributions to susceptibility have been defined.

women suffer traumatic amputations brought about by hazardous conditions in both the work- place and recreation. The greatest number of amputations is performed in older adults, espe- cially men over 60. Ethnicity and race have no known effects on the risk for amputation.

ASSESSMENT

HISTORY. Seek information in such areas as control of diabetes and hypertension, diet, smok- ing, and any other activities that may affect the condition and rehabilitation of the patient.

PHYSICAL EXAMINATION. Inspection of the limb prior to surgery should focus on the area close to the expected site of amputation. Because of limited circulation, the limb often feels cool to the touch. Any lacerations, abrasions, or contusions may indicate additional problems with healing and should be made known to the surgeon.

PSYCHOSOCIAL. The young patient with a traumatic amputation may be in the denial phase of grief. The older patient with a long history of peripheral vascular problems, culminating in loss of a limb, may fear the loss of independence. Patients may show hostility or make demands of the nurse that seem unreasonable. Incomplete grieving, along with depression and false cheerfulness, can indicate psychological problems that predispose to phantom limb pain and prolong rehabilitation.

Those patients who are reluctant to have visitors while they are hospitalized following an amputation may have problems with depression later.

64 Amputation

PRIMARY NURSING DIAGNOSIS

Impaired mobility related to loss of lower extremity

OUTCOMES. Balance; Body positioning; Ambulation; Bone healing; Comfort level; Joint move- ment; Mobility level; Muscle function; Pain level; Wound healing; Safety behavior: Fall prevention INTERVENTIONS. Positioning; Body image enhancement; Fall prevention; Exercise promo- tion; Exercise therapy: Ambulation and balance; Pain management; Positioning; Prosthesis care;

Skin surveillance; Wound care

PLANNING AND IMPLEMENTATION Collaborative

MANAGEMENT OF TRAUMATIC AMPUTATION. If complete amputation of a body part occurs, flush the wound with sterile normal saline, apply a sterile pressure dressing, and elevate the limb. Do not apply a tourniquet to the extremity. Wrap the amputated body part in a wet ster- ile dressing that has been soaked with sterile normal saline solution. Place the body part in a clean, dry plastic bag, label the bag, and seal it. Place the bag in ice, and transport it with the trauma patient. Do not store the amputated part on dry ice or in normal saline. Following reim- plantation surgery, assess the color, temperature, peripheral pulses, and capillary refill of the reimplanted body part every 15 minutes. If the skin temperature declines below the recom- mended temperature or if perfusion decreases, notify the surgeon immediately.

Abnormality with

Test Normal Result Condition Explanation

Diagnostic Highlights

Ankle arm index

(AAI) AAI !1.0 AAI "0.6 Ratio of the blood pressure in the leg to that in the arm; identifies people with severe aortoiliac occlusive disease Other Tests:Limb blood pressure; Doppler ultrasonography; ultrasonic duplex scan- ning; plethysmography; computed tomography

POSTOPERATIVE MANAGEMENT. Many interventions can help improve the patient’s mobility—for example, care of the surgical wound, control of pain, and prevention of further injury. If any excessive bleeding occurs, notify the surgeon immediately and place direct pres- sure on the area of hemorrhage. Occasionally, a patient returns from surgery with either a Jobst air splint or an immediate postsurgical fitted prosthesis already in place. Explain that the device is intended to aid ambulation and prevent the complications of immobility.

A particular danger to mobility for the patient with a lower limb amputation is contracture of the nearest joint. Elevate the stump on a pillow for the first 24 hours following surgery to control edema. However, after 24 hours do not elevate the stump any longer, or the patient may develop contractures. To prevent contractures, turn the patient prone for 15 to 30 minutes twice a day with the limb extended if the patient can tolerate it. If not, keep the joints extended rather than flexed.

Amputation 65

Medication or

Drug Class Dosage Description Rationale

Pharmacologic Highlights

Analgesia Varies by drug The amputation procedure is painful and generally requires narcotic analgesia

Relieve pains and allows for increasing mobility to limit surgical complications Other Therapies: Intense burning, crushing, or knifelike pain responds to anticonvulsants such as phenytoin (Dilantin) and carbamazepine (Tegretol). The severe muscle cramps or spasmodic sensations that other patients experience respond better to a central-acting skeletal muscle relaxant such as baclofen (Lioresal). Besides the usual narcotics, a beta blocker such as propranolol may be used for the constant, dull, burning ache.

Independent

Postoperative pain located specifically in the stump can be severe and is not usually aided by positioning, distraction, or other nonpharmacologic measures. True stump pain should be short term and should decrease as healing begins. Stump pain that continues to be severe after healing progresses may indicate infection and should be investigated. Phantom limb pain, very real physical discomfort, usually begins about 2 weeks after surgery. It may be triggered by multiple factors, including neuroma formation, ischemia, scar tissue, urination, defecation, and even a cold temperature of the limb. The sensations of phantom limb pain may be described in two basic patterns and are treated with different types of medication. Some nonpharmacologic treat- ments have also shown success, especially transcutaneous electrical nerve stimulation, hypnosis, whirlpool, and massage therapy.

Grieving over loss of a body part is a normal experience and a necessary condition for suc- cessful rehabilitation. It becomes excessive when it dominates the person’s life and interferes seriously with other functions. Explore concerns, and help the patient and family determine which are real and which are feared.

Looking at the stump when the dressing is first changed is usually difficult for the patient. It triggers a body image disturbance that may take weeks or months to overcome. The nurse’s calm manner in observing and caring for the stump encourages the patient to move toward accepting the changes in her or his body.

Regardless of the type of dressing, stump sock, or temporary prosthesis, inspect the surgical area of an amputation every 8 hours for healing. The goal of bandaging is to reduce edema and shrink the stump into shape for a future prosthesis. Keep the wound clean and free of infection.

Many problems of safety or potential for injury exist for the patient who re-establishes her or his center of balance and relearns ambulation. Urge caution, especially with the young patient who denies having any disability. Safety concerns play a prominent role in planning for dis- charge. The nurse or physical therapist assesses the home situation.

DRG Category: 240 Mean LOS: 6.4 days

Description: MEDICAL: Connective Tissue Disorders with CC

DOCUMENTATION GUIDELINES

• Condition of the surgical site, including signs of irritation, infection, edema, and shrinkage of tissue

• Control of initial postoperative pain by pain medication

• Measures, both effective and ineffective, in control of phantom limb pain

• Ability to look at the stump and take part in its care

• Participation in physical therapy, including reluctance to try new skills

• Presence of any complication: Bleeding, thrombophlebitis, contractures, infection, return of pain after initial postoperative period

DISCHARGE AND HOME HEALTHCARE GUIDELINES

PREVENTION OF COMPLICATIONS. Teach the patient to wash the stump daily with plain soap and water, inspecting for signs of irritation, infection, edema, or pressure. Remind him or her not to elevate the stump on pillows, as a contracture may still occur in the nearest joint.

PHANTOM LIMB PAIN. Teach the patient to apply gentle pressure to the stump with the hands to control occasional phantom limb pain and to report frequent phantom limb pain to the physician.

PHYSICAL THERAPY. Give the patient the physical therapy or exercise schedule she or he is to follow and make sure she or he understands it. If the patient needs to return to the hospital for physical therapy, check on the availability of transportation.

SUPPLIES AND EQUIPMENT. Make sure that the patient is provided with needed supplies and equipment, for example, stump socks and a well-fitting prosthesis.

ENVIRONMENT. Instruct the patient to avoid environmental hazards, for example, throw rugs and steps without banisters.

SUPPORT SERVICES.Many communities have support services for amputees and for patients who have suffered a loss (including a body part). Help the patient locate the appropriate service, or refer him or her to the social service department for future support.

66 Amyloidosis

Amyloidosis

A

myloidosis is a rare, chronic metabolic disorder that is characterized by the extracellular dep- osition of the fibrous protein, amyloid, in one or more sites of the body. Eight people out of one million develop amyloidosis and the average age of diagnosis is approximately 65 years of age.

Organ dysfunction results from accumulation and infiltration of amyloid into tissues, which ulti- mately puts pressure on surrounding tissues and causes atrophy of cells. Some forms of amyloi- dosis cause reticuloendothelial cell dysfunction and abnormal immunoglobulin synthesis.

The associated disease states may be inflammatory, hereditary, or neoplastic; deposition can be localized or systemic. Although primary amyloidosis is not associated with a chronic disease, secondary amyloidosis is associated with such chronic diseases as tuberculosis, syphilis, Hodgkin’s disease, and rheumatoid arthritis, and with extensive tissue destruction. Amyloidosis occurs in about 5% to 10% of patients who have multiple myeloma, the second most com- mon malignant tumor of bone. The spleen, liver, kidneys, and adrenal cortex are the organs

most frequently involved. For a patient with generalized amyloidosis, the average survival rate is 1 to 4 years. Some patients have lived longer, but amyloidosis can result in permanent or life-threatening organ damage. The major cause of death is renal failure.

CAUSES

The precise causes of amyloidosis are unknown, although some experts suspect an immunobio- logical basis for the disease. The disease has complex causes, with both immune and genetic fac- tors involved. It may be due to an enzyme defect or an altered immune response. Some forms of amyloidosis appear to have a genetic cause. Another form of amyloidosis appears to be related to Alzheimer’s disease.

GENETIC CONSIDERATIONS

While not specifically a genetic disease, several genes have been associated with amyloidosis. These include the apolipoprotein A1 gene (APOA1), the fibrinogen alpha-chain gene (FGA), and the lysozyme gene (LYZ). Amyloidosis can also occur as a feature of diseases that are heritable, such as Familial Mediterranean fever (FMF), which is an autosomal recessive disease more common among persons of Mediterranean origin. FMF causes fever and pain in the joints, chest, and abdomen.

GENDER, ETHNIC/RACIAL, AND LIFE SPAN CONSIDERATIONS

Amyloidosis is seen more in adult populations. Elderly individuals, especially those with Alzheimer’s disease, are also at risk. Little is known about gender considerations. Patients whose origins are Portuguese, Japanese, Swedish, Greek, and Italian seem to be more suscepti- ble. Some studies show that African Americans have a higher risk for cardiac-related amyloido- sis leading to cardiomyopathy than do other populations. The male to female ratio is 2:1.

ASSESSMENT

HISTORY. Establish a history of weakness, weight loss, lightheadedness, or fainting (syncope).

Ask the patient if she or he has experienced difficulty breathing. Determine if the patient has experienced difficulty in swallowing, diarrhea, or constipation, which are signs of gastrointesti- nal (GI) involvement. Determine if the patient has experienced joint pain, which is a sign of amyloid arthritis. Elicit a history of potential risk factors.

PHYSICAL EXAMINATION. Assess for kidney involvement by inspecting the patient’s feet for signs of pedal edema and the patient’s face for signs of periorbital edema. Take the patient’s pulse, noting changes in rhythm and regularity. Note any changes in the patient’s blood pressure.

Auscultate the patient’s heart sounds for the presence of dysrhythmias, murmurs, or adventitious sounds. Auscultate the breath sounds and observe for dyspnea.

Observe the patient’s tongue for swelling and stiffness, and assess the patient’s ability to speak and swallow. Auscultate bowel sounds, noting hypoactivity. Palpate the patient’s abdomen, noting any enlargement of the liver. Observe the patient for signs of abdominal pain, and check the patient’s stool for blood.

Assess the patient’s skin turgor and color, noting any evidence of jaundice. Malabsorption occurs with GI involvement, leading to malnourishment. Observe the patient’s skin for the pres- ence of lesions that may indicate nutrient or vitamin deficiencies. Palpate the axillary, inguinal, and anal regions for the presence of plaques or elevated papules. Inspect the patient’s neck and mucosal areas, such as the ear or tongue, for lesions. Observe the patient’s eyes, noting any peri- orbital ecchymoses (“black-eye syndrome”). Neurological testing may reveal decreased pain sensation and muscle strength in the extremities.

PSYCHOSOCIAL.Because the patient with amyloidosis may be asymptomatic, the sudden- ness of the revelation of the disease can be traumatic. Patients with facial lesions may be upset at the change in their appearance.

Amyloidosis 67

68 Amyloidosis

Abnormality with

Test Normal Result Condition Explanation

Diagnostic Highlights

Serum protein electrophoresis, urine protein electrophoresis, serum and urine protein immu- noelectrophoresis

Biopsy: usually taken from the rectal mucosa, abdominal fat pads, skin, and gums

Negative

Negative

Identification of abnormal immunoglobulins

Positive for amyloid usually with Congo red staining techniques

To determine the immune response

Identifies the presence of amyloid with appro- priate stains Other Tests:Electrocardiogram; serum alkaline phosphatase; urinalysis; blood urea nitrogen and creatinine.

PRIMARY NURSING DIAGNOSIS

Ineffective airway clearance related to tongue obstruction (macroglossia) OUTCOMES. Respiratory status: gas exchange and ventilation; Oral health

INTERVENTIONS. Airway insertion; Airway management; Airway suctioning; Oral health promotion; Respiratory monitoring; Ventilation assistance

PLANNING AND IMPLEMENTATION Collaborative

Therapy is targeted to amelioration of the underlying organ dysfunction through pharmacologic 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 tra- cheotomy 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.

OTHER MANAGEMENT. A dietary consultation can provide the patient with a plan to sup- plement 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 refer- ral to a speech therapist may be necessary if the patient’s tongue prevents clear communication.

Medication or

Drug Class Dosage Description Rationale

Pharmacologic Highlights

Melphalan and prednisone com- bined 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 mal- absorption develops secondary to GI involvement, parenteral nutrition is used to meet nutritional needs.

DRG Category: 012 Mean LOS: 6.3 days

Description: MEDICAL: Degenerative Nervous System Disorders

Independent

Maintain a patent airway when the patient’s tongue is involved. Prevent respiratory tract com- plications 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 pres- sure 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 tremen- dous 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 real- istic 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.

DOCUMENTATION GUIDELINES

• Physical findings: Adequacy of airway, degree of hydration or dehydration, presence of lesions, macroglossia, edema

• Ability to use extremities: Range of motion, weakness, gait, activity tolerance

• Nutritional status

• Changes in heart and lung sounds, presence of dysrhythmias

• Response to medications, speech therapy, counseling, surgery

• Emotional responses to disease: Degree of hope, resiliency, family support, ability to cope

• Understanding of and interest in patient teaching

DISCHARGE AND HOME HEALTHCARE GUIDELINES

MEDICATIONS. Teach the patient the purpose, dosage, schedule, precautions and potential side effects, interactions, and adverse reactions of all prescribed medications.

COMPLICATIONS. Teach the patient to examine her or his legs daily for signs of swelling.

Instruct the patient to monitor urinary output for a decrease in quantity. Teach the patient to test the stool for bleeding. Advise the patient to report breathing difficulties or irregular heart beats.

FOLLOW-UP. Explain to the patient and significant others that a variety of counseling and social supports are available to help as the disease progresses. Give the patient a phone number to call if some health assistance is needed.

Amyotrophic Lateral Sclerosis 69

Amyotrophic Lateral Sclerosis

A

myotrophic lateral sclerosis (ALS) is the most common progressive motor neuron disease of muscular atrophy. There are 20,000 Americans living with the disease, and each year in the United States, there are 5000 new cases. ALS is often referred to as Lou Gehrig’s disease after the baseball

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