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CREST syndrome

Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. Progressive systemic scleroderma or systemic sclerosis, the generalised type of the disease, can be fatal. The localised type of the disease tends not to be fatal. The term 'localised, generalised sclerderma' can be used to describe cases where the disease covers a large area of the body - typically more than 40%. more...

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Signs and symptoms

Scleroderma affects the skin, and in more serious cases, it can affect the blood vessels and internal organs. The most evident symptom is the hardening of the skin and associated scarring. Typically the skin appears reddish or scaly in appearance. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage will weaken limbs and affect appearance.

The seriousness of the disease varies hugely between cases. The two most important factors to consider are, the level of internal involvement (beneath the skin), and the total area covered by the disease. For example there are cases where the patient has no more than one or two lesions (affected areas), perhaps covering a few inches. These are less serious cases and tend not to involve the internal bodily functions.

Cases with larger coverage are far more likely to affect the internal tissues and organs. Where an entire limb is affected, symptoms will almost certainly have serious consequences on the use of that limb. The heart and lungs will be affected when the disease covers this area of the torso. Some patients also experience gastrointestinal problems, including heartburn and acid reflux. Internal scarring may sometimes spread beyond what can be seen by the naked eye.

There is discoloration of the hands and feet in response to cold. Most patients (>80%) have Raynaud's phenomenon, a vascular symptom that can affect the fingers, and toes.

Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes, which are known as digital ulcers.

Types

There are three major forms of scleroderma: diffuse, limited (CREST syndrome) and morphea/linear. Diffuse and limited scleroderma are both a systemic disease, whereas the linear/morphea form is localized to the skin. (Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of scleroderma.)

Diffuse scleroderma

Diffuse scleroderma is the most severe form - it has a rapid onset, involves more widespread skin hardening, will generally cause much internal organ damage (specifically the lungs and gastrointestinal tract), and is generally more life threatening.

Limited scleroderma/CREST syndrome

The limited form is much milder: it has a slow onset and progression, skin hardening is usually confined to the hands and face, internal organ involvement is less severe, and a much better prognosis is expected.

The limited form is often referred to as "CREST" syndrome. CREST is an acronym for:

  • Calcinosis
  • Raynaud's syndrome
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia

These five are the major symptoms of the CREST syndrome.

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Sjogren's syndrome—implications for perioperative practice - Home Study Program
From AORN Journal, 3/1/03 by Lynn M. Petruzzi

SJOGREN'S SYNDROME--IMPLICATIONS FOR PERIOPERATIVE PRACTICE

The article "Sjogren's syndrome--Implications for perioperative practice," is the basis for this AORN Journal independent study. The behavioral objectives and examination for this program were prepared by Rebecca Holm, RN, MSN, CNOR, clinical editor, with consultation from Susan Bakewell, RN, MS, education program professional, Center for Perioperative Education.

A minimum score of 70% on the multiple-choice examination is necessary to earn 2.3 contact hours for this independent study. Participants receive feedback on incorrect answers. Each applicant who successfully completes this study will receive a certificate of completion. The deadline for submitting this study is March 31, 2006.

Send the completed application form, multiple-choice examination, learner evaluation, and appropriate fee to

AORN Customer Service c/o Home Study Program 2170 S Parker Rd, Suite 300 Denver, CO 80231-5711

or fax the information with a credit card number to (303) 750-3212.

BEHAVIORAL OBJECTIVES After reading and studying the article on Sj6gren's syndrome (SS), the nurse will be able to

(1) discuss the pathogenesis of the development of SS,

(2) describe the signs and symptoms of SS,

(3) explain the diagnostic tests used to differentially diagnose SS,

(4) identify treatment options available to patients with SS, and

(5) describe perioperative nurses' role in protecting patients with SS who are undergoing surgery.

This program meets criteria for CNOR and CRNFA recertification, as well as other continuing education requirements.

Sjogren's syndrome (SS), also known as sicca syndrome, is a chronic autoimmune disorder in which lymphocytes invade and destroy the exocrine glands, particularly the salivary and lacrimal glands, resulting in decreased saliva and tear production. Dryness also may affect the skin, sinuses, upper airway, gastrointestinal tract, and vaginal tissues. Systemic manifestations of SS include rash, Raynaud's phenomenon, fatigue, and nerve and muscle pain. (1)

Sjogren's syndrome is the second most common autoimmune rheumatic disease, surpassed only by rheumatoid arthritis, and even occurs more frequently than systemic lupus erythematosus. (2) Estimates of the prevalence of SS range from 500,000 to four million people, 90% of whom are women. (3) The typical patient is a Caucasian perimenopausal woman in her forties. Sjogren's syndrome frequently is unrecognized and untreated. The average time from onset of symptoms to diagnosis is at least three and one-half years. (4) The person often seeks the help of multiple physicians and specialists; only to repeatedly receive an incorrect diagnosis or no diagnosis at all. The symptoms continue or worsen, leading to anger and frustration. Delays in diagnosis can be explained by the insidious development of symptoms during a number of years, the lack of universally accepted diagnostic criteria, and the tendency of patients and medical personnel to trivialize the initial symptoms of the disorder.

ETIOLOGY AND PATHOGENESIS

The trigger that initiates the autoimmune events of SS remains unknown. A variety of factors potentially may influence the development of the disease. Most research indicates that SS and other autoimmune diseases result from the interaction of specific, susceptible genes and environmental agents that fool the immune system into attacking a target organ. In SS, the exocrine or moisture producing glands are attacked. No single gene causes SS; however, the human leukocyte antigen 5 HLADQZ,DR3 occurs most frequently in Caucasian patients with SS. Apoptosis (ie, programmed cell death) of T lymphocytes and salivary acinar epithelial cells is a gene-regulated process that functions abnormally in patients with SS and appears to contribute to glandular destruction. Indirect evidence suggests that viruses may play a role as the environmental agent. Hormonal changes also may serve as an agent that influences the disease. (5)

An injury to the exocrine gland may initiate SS. As T lymphocytes invade the tissue, cytokines (ie, inflammatory messengers) are released locally, which perpetuates the immune inflammatory response. The cytokines may escape into the bloodstream, stimulating other parts of the body to make proteins that result in an increased erythrocyte sedimentation rate (ESR) and increased production of Creactive protein (CRP). T lymphocytes also stimulate B cells, causing antibody formation (eg, immunoglobulin G [IgG], immunoglobulin A [IgA], immunoglobulin M [IgM], antinuclear antibody [ANA], Sjogren's syndrome antigen A [SSA], Sjogren's syndrome antigen B [SSB]). Eventually the normal glandular tissue is replaced by fibrosed and fatty tissue. The autonomic nerves that send signals to the moisture producing glands also may be damaged. (6)

SIGNS AND SYMPTOMS

The signs and symptoms of SS are divided into the hallmark signs and symptoms and the extraglandular signs and symptoms. The hallmark signs and symptoms also are called the sicca symptoms.

Hallmark signs and symptoms. Xerostomia (ie, dry mouth) and xerophthalmia (ie, dry eyes) are the hallmark symptoms of SS. Xerostomia is the most prevalent and impairing symptom, although it often is intermittent early in the disease course. (7) Xerophthalmia may develop before, after, or simultaneously with xerostomia. (8) Although xerostomia and xerophthalmia seem insignificant, both can have a profound affect on a patient's health and quality of life.

Xerostomia. Three pairs of major salivary glands (ie, parotid, submandibular, sublingual) produce 95% of saliva. The remainder is produced by the minor salivary glands located inside the mouth. These glands are particularly numerous under the surface of the lips and palate. In patients with SS, both the major and minor salivary glands are affected, resulting in a reduction of salivary flow. (9)

Some people with SS complain of a feeling of dryness. Others describe

* a cotton mouth sensation,

* difficulty in swallowing food,

* an inability to eat dry foods,

* changes in taste,

* discomfort or burning sensation in the mouth, or

* problems wearing dentures. (10)

One of the earliest clinical signs of SS is a decreased sublingual salivary pool. The tongue and mucous membranes lose their glistening appearance and the buccal mucosa becomes sticky. Major salivary gland swelling, particularly in the parotid glands, occurs in episodes or as a chronic problem and can be quite painful. (11)

As the disease progresses, xerostomia leads to multiple oral problems. The tongue becomes erythematous, fissured, or ulcerated. Dental caries develop and may progress rapidly. Patients may exhibit an increased incidence of oral candidiasis and difficulty with phonation. Dysphagia (ie, difficulty swallowing), an altered sense of taste, mouth burning, and an intolerance of acidic or spicy foods may develop and lead to weight loss or malnutrition. (12) Lack of nocturnal saliva may cause sleep deprivation and trigger the onset of fibromyalgia. (13)

Xerophthalmia. Xerophthalmia, also known as keratoconjunctivitis sicca, results from a water deficiency of the tear film. The tear film is a three-layer structure consisting of the mucin, aqueous, and lipid layers. The aqueous layer produces 90% of tear volume. Ocular symptoms occur as tear volume diminishes. (14)

Patients typically complain of a scratchy or gritty sensation in the eye. Eye redness, itching, and burning frequently are reported. Xerophthalmia symptoms vary considerably, but most patients notice an increase in dryness as the day progresses. The eye also is affected by environmental conditions, such as low humidity, dust, and smoke. Diminished lacrimal production eventually can damage the comeal surface and lead to blurred vision, photosensitivity, and glare. Serious complications, such as infection, corneal ulceration, perforation, and vision loss, can result from undiagnosed or untreated xerophthalmia. (15)

Extraglandular signs and symptoms. Extraglandular manifestations occur in up to one-third of patients with primary SS. Occasionally, the extraglandular symptoms may overshadow the sicca symptoms, and they also are the presenting manifestations of SS. Systemic involvement can lead to significant morbidity and mortality. In addition, other autoimmune or lymphoproliferative disorders may develop at any time in a patient with SS. A systems review of extraglandular manifestations of SS is listed in Table 1. (16)

Fatigue is a common complaint of patients with SS and may have many causes. Active systemic inflammation is one cause and may be detected in the patient's blood test results. Fatigue also may be related to poor sleep. Inadequate sleep may be caused by joint or muscle pain, nocturia due to large liquid consumption to relieve dryness, insomnia due to steroid use, or physiological sleep disorders. (17)

DIAGNOSIS

A number of conditions can cause sicca symptoms, such as those seen with SS (Table 2). Other disorders, like sarcoidosis, head and neck radiation, mouth breathing, allergies, chronic sialadenitis (ie, inflammation of the salivary gland) and more than 400 medications can produce dryness. (18) Identification of SS, however, as the cause of the patient's sicca symptoms not only will lead to more effective treatment but also facilitate more effective and timely medical evaluation when extraglandular organs are involved.

Primary SS is diagnosed when a person with no known connective tissue disease presents with xerophthalmia and xerostomia. Secondary SS occurs when a person with a defined connective tissue disorder develops sicca symptoms. Approximately 50% of patients with SS have the secondary form. Conditions associated with secondary SS include

* rheumatoid arthritis;

* lupus;

* scleroderma, including the CREST variant (ie, calcinosis, Raynaud's disease, esophageal dysmotility, sclerodactyl, tele-, angiectasia);

* mixed connective tissue disease; and

* relapsing polychondritis (ie, inflammation of cartilage) and polymyositis (ie, simultaneous inflammation of many muscles). (19)

Although there are no uniformly accepted criteria for the diagnosis of SS, diagnosis is based on symptoms as well as documented evidence of

* xerophthalmia,

* xerostomia,

* serologic autoimmunity, or

* positive salivary gland biopsy.

As a general rule in clinical practice, meeting three of four criteria indicates a high probability of SS. The objective evidence and significance of the four criteria are identified in Table 3. (20)

TREATMENT

Treatment for SS is a two-pronged approach. Health care providers focus on treating the sicca symptoms as well as treating the underlying systemic disease.

Treating the sicca symptoms. The sicca symptoms of SS can be treated in a variety of ways.

General measures to decrease dryness include

* reviewing the patient's medications to consider replacing one or more drying agents with medications that have fewer anticholinergic effects; (21)

* avoiding environmental factors, such as wind, dust, smoke, and low humidity; (22) and

* increasing fluid intake, especially water. (23) Measures to treat xerostomia include

* using artificial saliva and oral lubricants, such as topical vitamin E oil; (24)

* avoiding alcohol and mouth washes containing alcohol, which can irritate and dry the mouth; (25)

* using correct mouth breathing, which helps minimize oral symptoms; (26)

* stimulating salivary production with the use of medications, such as pilocarpine tablets, which, if used regularly, produce subjective and objective improvement in salivary flow in most patients and also may stimulate other exocrine glands and provide relief from dry eyes, skin, nose, and vagina (27) or cevimeline, which has US Food and Drug Administration approval for use with SS xerostomia;

* using sugarless gum and candy and eating smaller, more frequent meals to stimulate salivary flow; (28) and

* practicing good dental hygiene for caries prophylaxis through frequent brushing with a fluoride-containing toothpaste, regular flossing, frequent dental visits, and using topical fluoride; however, oral candidiasis should be identified and treated promptly. (29)

Measures to treat xerophthalmia Include

* using preservative-free artificial tears and ocular lubricants and ointments; (30)

* using lacrimal inserts (ie, a small pellet that slowly dissolves producing a film over normal tears); (31)

* undergoing punctal occlusion or closure of the tear duct to decrease drainage of tears and provide an increased tear volume; (32)

* making frequent visits to an ophthalmologist for examination and evaluation of treatment; (33)

* avoiding use of excessive eye makeup, particularly on the eyelid, because it can soften and enter the eye, creating more concentrated tears; (34) and

* using moisture-chamber glasses specifically designed to protect the eye from irritants and hold sponges that increase the humidity surrounding the eye. (35)

Systemic treatment. Extraglandular involvement is treated with two major groups of systemic medications--nonsteroidal anti-inflammatory drugs (NSAIDs) and immunomodulating medications. The choice of therapy is specific to the individual and has not been shown to affect the sicca symptoms of SS. (36)

Arthralgias, myalgias, and polyarthritis commonly are treated with NSAIDs. Hydroxychloroquine can improve fatigue, musculoskeletal symptoms, lymphadenopathy, and parotid swelling and is the most commonly used immunosuppressive medication. Corticosteroids, either oral or IV, generally are reserved for patients with vasculitis or when the patient's nervous system or an internal organ (eg, lung, kidney) is involved.

Patients with systemic symptoms usually are quite ill. Corticosteroids also can be injected locally to treat an area of specific inflammation, such as tendonitis or bursitis: In the most serious cases, other immunomodulating agents may be prescribed with the intent of halting the inflammatory process, inducing remission, or preventing devastating complications. These immunomodulating agents can be used alone or as concurrent therapy with NSAIDs and corticosteroids. They are very slow acting and may require weeks to months to produce results. Medications in this category include methotrexate, cyclophosphamide, and azathioprine. (37)

CARE OF THE PERIOPERATIVE PATIENT

Patients undergoing surgery for other reasons have special needs because of their SS diagnosis. The psychological and physical stresses related to surgery can exacerbate the patient's symptoms or lead to a flare up of the disease process. Anesthetic gases are drying, and general anesthesia causes a decrease in tear production beyond the effects of SS. (38) The use of local or regional anesthesia, therefore, is encouraged when appropriate. The most common postoperative problems that patients with SS experience include burning eyes (eg, corneal dryness, abrasion), sore throat, and exacerbations of other sicca symptoms. (39)

Before surgery. The preoperative nurse may be the first to discover that a patient has SS during the preoperative visit or telephone call. The nurse should assess the patient's general status, including medication usage, and determine whether recent laboratory studies have been obtained. It is important to determine whether there is extraglandular involvement. A rheumatologist most likely is the coordinator of the patient's care and can provide information the patient cannot. The anesthesia care provider and perioperative team members should be notified of the patient's diagnoses, and the plan of care should be discussed (Table 4).

Preoperatively, the patient should be instructed to continue his or her usual eye and oral care and medication regimen. The patient also should be instructed to bring an adequate supply of his or her favorite artificial tears, saliva, and other treatments to the hospital. Patients should be asked whether they regularly use vitamin E oil. If indicated, vitamin E oil should be discontinued two weeks before surgery because it may have an anticoagulant effect. A prolonged NPO status should be avoided, and the patient should be allowed clear liquids until two hours before surgery, if possible. (40)

Day of surgery. When the patient arrives in the preoperative area, the perioperative nurse performs a full assessment of the patient's ocular and oral status, skin condition, and mobility. All information obtained about the patient's SS status should be documented. The perioperative nurse should ensure that preoperative and intraoperative medications with anticholinergic effects, such as atropine, diphenhydramine, glycopyrrolate, and promethazine, are avoided. (41) Patients taking long-term steroid medications should receive stress doses for surgery. This should include IV steroids on call to the OR followed by perioperative stress doses until the patient is clinically stable. (42) The circulating nurse turns up the temperature in the OR before the patient is brought to the room because patients with SS are susceptible to Raynaud's phenomenon.

Intraoperatively, the circulating nurse should observe several precautions. Associated arthritis or fibromyalgia can affect the movement and positioning of the patient on and off the OR bed. The circulating nurse, therefore, coordinates the care provided by the entire perioperative team during this process, or if possible, the circulating nurse should have the patient transfer and position himself or herself.

If possible, regional anesthesia is preferred because anesthetic gases are drying and cause a decrease in tear production. If general anesthesia is required, the anesthesia care provider adds a humidifier to the rebreathing system. Additionally, he or she lubricates and places the endotracheal tube or laryngeal mask airway very carefully. The anesthesia care provider may place a dental guard if the patient has multiple caries and thin oral mucosa. Intubation may be difficult if the rheumatic process involves the cervical spine or temporal mandibular joints. The anesthesia care provider should lubricate the patient's eyes every 30 minutes and gently tape the patient's eyelids, avoiding pressure.

The circulating nurse notifies the postanesthesia care unit (PACU) nurse of the patient's SS diagnosis. The PACU nurse ensures that the oxygen administered to the patient is humidified. He or she avoids administering pain medications, such as meperidine, that have a drying effect. (43) The nurse establishes a baseline by assessing the patient's ocular and oral status upon arrival in the PACU and notes any changes from the patient's preoperative condition. The nurse provides the patient with ice chips, liquids, or artificial saliva as soon as possible. He or she moisturizes the patient's lips with a petroleum-based product and administers eye drops frequently. The nurse places soothing warm water compresses over the patient's eyes.

The nurse may need to cut or crush oral medications, depending on the degree of dryness and difficulty swallowing the patient is experiencing. The nurse also should help the patient move into an upright position before administering oral medications and then provide the patient with a full glass of water. Dry foods, such as crackers, which typically are given to patients postoperatively, should be avoided.

Postoperative nurses should be prepared to provide extra assistance and comfort measures because most patients with SS experience fatigue and achiness after surgery, particularly if a general anesthetic has been used. These symptoms may be more pronounced in patients with SS, particularly if fatigue, arthralgias, and myalgias were experienced preoperatively.

CASE STUDY

Mrs M, a 45-year-old Caucasian female with a past history of primary SS, gastroesophageal reflux disease, and chronic gastritis, is scheduled for a laparoscopically assisted vaginal hysterectomy under general anesthesia. Mrs M suffers from severely dry eyes for which she underwent punctal occlusion of both lower tear ducts. Other recent problems include

* dry mouth,

* recurrent vaginal yeast infections with antibiotic use,

* arthralgias,

* fatigue, and

* occasional Raynaud's phenomenon during cooler weather.

Mrs M's rheumatologist indicates that her systemic disease is well controlled and all recent laboratory results remain within normal limits.

During a previous laparoscopic procedure, the patient experienced a severe flare up of ocular and oral dryness and sore throat. She was unable to wear contact lenses postoperatively. Additionally, the patient experienced transient postoperative numbness and tingling of the extremities that spontaneously resolved.

Current treatment includes systemic medications and eye and oral care. Systemic medications include

* pilocarpine 5 mg six times per day;

* hydroxychloroquine 200 mg twice per day;

* pantoprazole sodium 40 mg once per day, increasing to twice per day, if needed;

* lorazepam two 1-mg tablets at bedtime; and

* ibuprofen two to three 200-mg tablets every four hours as needed.

Mrs M's eye care includes self-administration of preservative-free artificial tears six times per day and at bedtime. Her oral care includes using prescription fluoride toothpaste two times per day. Additionally, Mrs M uses topical fluoride oral rinse in the morning and applies petroleum jelly to her lips at bedtime.

During the preoperative telephone call, the perioperative nurse discussed the status of Mrs M's SS. Having consulted with the anesthesia care provider and the surgeon before calling the patient, the nurse instructed the patient to

* continue her usual eye and oral care and medication regimen on the day of surgery;

* bring her favorite artificial tears, lubricating gel, prescription fluoride toothpaste, fluoride oral rinse, and other special treatments to the hospital; and

* remain NPO after 4 AM rather than midnight. Preoperatively, the circulating ensured that

* the temperature of the OR was increased to minimize problems with Raynaud's phenomenon,

* a humidifier was added to the anesthesia rebreathing system,

* adequate lubrication was available for the anesthesia care provider to use during endotracheal tube insertion, and

* adequate ocular lubrication was available for the anesthesia care provider to apply to the patient's eyes every 30 minutes during the procedure. Intraoperatively, the circulating nurse

* worked with the anesthesia care provider to keep the patient's extremities covered and assess the patient every 30 minutes for signs and symptoms of Raynaud's phenomenon,

* allowed the patient to transfer herself onto the OR bed preoperatively, and

* ensured the availability of adequate personnel and equipment to gently transfer the patient postoperatively. Postoperatively, the perioperative nurse ensured that

* humidified oxygen was used,

* Mrs M's eye and oral care was resumed,

* ice chips were provided as soon as possible and the patient's diet was progressed when possible after surgery, and

* postoperative incisional pain was assessed and treated separate from Mrs M's arthralgias.

During the postoperative telephone call made three days after Mrs M was discharged, the perioperative nurse discussed Mrs M's hospital experience, especially focusing on care provided in regard to her diagnosis of SS. Mrs M was very pleased with the care she received. The only problem that Mrs M described was her excessive fatigue. In light of the fact that many patients with SS also suffer from fibromyalgia, nurses should have assessed Mrs M's level of fatigue during the postoperative period. Had this been occurring, they would have identified a problem. The perioperative nurse discovered that Mrs M's room was located by the nurse's station. As a result of the fibromyalgia that Mrs M suffers, she requires uninterrupted sleep as much as possible. The patient should have been placed in a room away from the noise of the unit nurse's station, kitchen, and staff member lounge. Additionally, sleeping medications may have been needed routinely rather than as requested. Although her excessive fatigue was not identified during her hospitalization, Mrs M is recovering well and has not suffered the numerous complications that she experienced during her previous hospitalization.

CONCLUSION

Sjogren's syndrome is more prevalent than most health care providers realize. Nurses can have a positive impact on the patient's surgical experience and outcome by improving their knowledge of SS and the care that is required perioperatively. A knowledgeable and skilled perioperative team enhances the care of the patient with SS by decreasing the patient's stress level, providing maximum comfort, preventing complications, and avoiding exacerbation of symptoms.

Examination

SJOGREN'S SYNDROME--IMPLICATIONS FOR PERIOPERATIVE PRACTICE

1. -- (ie, programmed cell death) of T-lymphocytes and salivary acinar epithelial cells is a gene-regulated process that functions abnormally in patients with Sjogren's syndrome (SS) and appears to contribute to glandular destruction.

a. Acarinosis

b. Alkalosis

c. Apoptosis

d. Aspergillosis

2. Which of the following factors does not influence the development of SS?

a. bacterial agents

b. gerletic susceptibility

c. hormonal changes

d. viral agents

3. The hallmark symptoms of SS are

a. xerasia and xerostomia.

b. xerophagia and xerophthalmia.

c. xerasia and xerophagia.

d. xerostomia and xerophthalmia.

4. Secondary SS is diagnosed when a person with no known connective tissue disease presents with xerophthalmia and xerostomia.

a. true

b. false

5. Overall, 70% to 80% of patients with SS have elevated levels of

a. antinuclear antibody.

b. anti-SS antigen A.

c. gamma globulins

d. anti-SS antigen B.

6. Salivary flow may be stimulated by all of the following except

a. cevimeline.

b. chewing sugarless gum.

c. pilocarpine.

d. undergoing punctal occlusion.

7. -- is the immunomodulation medication most commonly used to treat the extraglandular involvement of SS.

a. Corticosteroids

b. Cyclophosphamide

c. Hydroxychloroquine

d. Methotrexate

8. Preoperatively, the patient should be instructed to do all of the following except

a. discontinue regular vitamin E use.

b. continue usual eye and oral care and medication regimen.

c. maintain an NPO status after midnight.

d. bring his or her favorite artificial tears and saliva to the hospital on the day of surgery.

9. Which of the following pertains to the intraoperative care of patients with SS?

a. encourage use of medications with anticholinergic effects to minimize SS symptoms

b. use general anesthesia because anesthetic gases stimulate the endocrine system

c. keep the OR cooler than usual to avoid complications of Raynaud's phenomenon

d. allow patients with associated arthritis or fibromyalgia to transfer themselves and assist with their own positioning

10. Measures to increase comfort postoperatively should not include

a. using ice chips, liquids, or artificial salivas, as permitted.

b. selecting meperidine for pain relief because it has the least drying effect.

c. administering eye drops frequently and applying warm compresses to the patient's eyes.

d. ensuring that the oxygen administered to the patient is humidified.

AORN is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's Commission on Accreditation. AORN recognizes these activities as continuing education for RNs. This recognition does not imply that AORN or the American Nurses Credentialing Center approves or endorses products mentioned in the activity. AORN is provider-approved by the California Board of Registered Nursing, Provider Number CEP 13019.

Answer Sheet

SJOGREN'S SYNDROME--IMPLICATIONS FOR PERIOPERATIVE PRACTICE

Please fill out the application and answer form on this page and the evaluation form on the back of this page. Tear the page out of the Journal or make photocopies and mail to:

A score of 70% correct on the examination is required for credit. Additionally, please verify by signature that you have reviewed the objectives and read the article, or you will not receive credit.

Signature --

Event # 03079 Session #7328

Contact hours: 2.3

Fee: Members $11.50; Nonmembers $23.00

Program offered March 2003.

The deadline for this program is March 31, 2006.

1. Record your AORN member identification number in the appropriate section below. (See your member card.)

2. Completely darken the spaces that indicate your answers to examination questions one through 10. Use blue or black ink only.

3. Our accrediting body requires that we verify the amount of time you required to complete this 2.3 contact hour (115 minutes) program. --

4. Enclose fee if information is mailed.

Learner Evaluation

SJOGREN'S SYNDROME--IMPLICATIONS FOR PERIOPERATIVE PRACTICE

The following evaluation is used to determine the extent to which this Home Study Program met your learning needs. Rate the following items on a scale of 1 to 5.

OBJECTIVES

To what extent were the following objectives of this Home Study Program achieved?

(1) Discuss the pathogenesis of the development of Sjogren's syndrome (SS).

(2) Describe the signs and symptoms of SS.

(3) Explain the diagnostic tests used to differentially diagnose SS.

(4) Identify treatment options available to patients with SS.

(5) Describe perioperative nurses' role in protecting patients with SS who are undergoing surgery.

PURPOSE/GOAL

To educate the perioperative nurse about Sjogren's syndrome and how it affects the patient's perioperative experience.

(6) Did this article increase your knowledge of the subject matter?

(7) Was the content clear and organized?

(8) Did this article facilitate learning?

(9) Were your individual objectives met?

(10) How well did the objectives relate to the overall purpose/goal?

TEST QUESTIONS/ANSWERS

(11) Were they reflective of the content?

(12) Were they easy to understand?

(13) Did they address important points?

LEARNER INPUT

(14) Will you be able to use the information from this Home Study in your work setting?

a. yes b. no

(15) I learned of this Home Study via

a. the Journal I receive as an AORN member.

b. the Journal that I obtained elsewhere.

c. the AORN web site.

d. SSM Online.

(16) What factor most affects whether you take an AORN Journal Home Study?

a. need for contact hours

b. price

c. subject matter relevant to current position

d. number of contact hours offered

What other topics would you like to see addressed in a future Home Study Program? Would you be interested or do you know someone who would be interested in writing an article on this topic?

Topic(s): --

Author name(s) and address(es): --

Home Study Program

ENDOVASCULAR REPAIR OF ABDOMINAL AORTIC ANEURYSMS

The article "Endovascular repair of abdominal aortic aneurysms," is the basis for this AORN Journal independent study. The behavioral objectives and examination for this program were prepared by Rebecca Holm, RN, MSN, CNOR, clinical editor, with consultation from Susan Bakewell, RN, MS, education program professional, Center for Perioperative Education.

A minimum score of 70% on the multiple-choice examination is necessary to earn 3.2 contact hours for this independent study. Participants receive feedback on incorrect answers. Each applicant who successfully completes this study will receive a certificate of completion. The deadline for submitting this study is March 31, 2006.

Send the completed application form, multiple-choice examination, learner evaluation, and appropriate fee to

BEHAVIORAL OBJECTIVES

After reading and studying the article on endovascular repair of abdominal aortic aneurysms (AAA), the nurse will be able to

(1) discuss the anatomy of the normal abdominal vascular system in regard to the development of an AAA,

(2) describe the diagnostic tests used to diagnose an AAA,

(3) explain how the anatomic measurements of an AAA allow care providers to identify candidates for the endovascular approach to AAA repair,

(4) describe the preoperative preparation of a patient scheduled for endovascular AAA repair,

(5) identify the circulating nurse's role in endovascular AAA repair,

(6) explain the steps of an endovascular AAA repair procedure, and

(7) describe the postoperative phase of the patient undergoing endovascular AAA repair.

This program meets criteria for CNOR and CRNFA recertification, as well as other continuing education requirements.

NOTES

(1.) S Carsons, E K Harris, eds, The New Sjogren's Syndrome Handbook (New York: Oxford University Press, 1998) 3-10; F B Vivino, "Diagnosis and treatment of Sjogren's syndrome." The Female Patient 24 (August 1999) 65-72.

(2.) R I Fox, "Sjogrens syndrome: New approaches to treatment," Medscape, http://www.medscape .com/viewprogram/182 (accessed 19 Dec 2002).

(3.) N Talal, "Sjogren's syndrome: Historical overview and clinical spectrum of disease," Rheumatic Disease Clinics of North America 18 (August 1992) 507-515.

(4.) F B Vivino, C H Huang, "Clinical and laboratory manifestations in 58 patients with Sjogren's syndrome," Arthritis and Rheumatism 36 suppl 2 (September 1993) S253.

(5.) A D Askari, F B Vivino, Current Concepts in the Evaluation and Treatment of Sjogren's Syndrome (Arlington Heights, Ill: ACCESS Medical Group, Department of Continuing Medical Education, 1999) 1-14; Carsons, Harris, eds, The New Sjogren's Syndrome Handbook, 3-10; R I Fox, T Maruyana, "Pathogenesis and treatment of Sjogren's syndrome," Current Opinion in Rheumatology 9 (September 1997) 393-399.

(6.) Carsons, Harris, eds, The New Sjogren's Syndrome Handbook, 3-10; M H Friedlaender, "Ocular manifestations of Sjogren's syndrome: Keratoconjunctivitis sicca," Rheumatic Disease Clinics of North America 18 (August 1992) 591-608; Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72.

(7.) F B Vivino, W A Katz, "Sjogren's syndrome: Clinical picture and diagnostic tests," Journal of Musculoskeletal Medicine 12 (March 1995) 40-52.

(8.) Askari, Vivino, Current Concepts in the Evaluation and Treatment of Sjogren's Syndrome, 1-14.

(9.) Carsons, Harris, eds, The New Sjogren's Syndrome Handbook, 53.

(10.) Askari, Vivino, Current Concepts in the Evaluation and Treatment of Sjogren's Syndrome, 1-14; Vivino, Katz, "Sjogren's syndrome: Clinical picture and diagnostic tests," 40-52.

(11.) Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72; Vivino, Katz, "Sjogren's syndrome: Clinical picture and diagnostic tests," 40-52.

(12.) T E Daniels, P C Fox, "Salivary and oral components of Sjogren's syndrome," Rheumatic Disease Clinics of North America 18 (August 1992) 571-589; Vivino, Katz, "Sjogren's syndrome: Clinical picture and diagnostic tests," 40-52; F B Vivino, S E Odin, "Sjogren syndrome: Giving dry mouth and dry eye the full treatment," Journal of Musculoskeletal Medicine 17 (June 2000) 350-367.

(13.) Askari, Vivino, Current Concepts in the Evaluation and Treatment of Sjogren's Syndrome, 1-14.

(14.) Carsons, Harris, eds, The New Sjogren's Syndrome Handbook, 40-41; Vivino, Katz, "Sjogren's syndrome: Clinical picture and diagnostic tests," 40-52.

(15.) Carsons, Harris, eds, The New Sjogren's Syndrome Handbook, 39-52; Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72; Vivino, Orlin, "Sjogren syndrome: Giving dry mouth and dry eye the full treatment," 350-367.

(16.) Askari, Vivino, Current Concepts in the Evaluation and Treatment of Sjogren's Syndrome, 1-14; Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72; Vivino, Katz, "Sjogren's syndrome: Clinical picture and diagnostic tests," 40-52.

(17.) Fox, "Sjogrens syndrome: New approaches to treatment."

(18.) L M Sreebny, S S Schwartz, "A reference guide to drugs and dry mouth," Gerontology 5 (Autumn 1986) 75-79; Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72; Vivino, Orlin, "Sjogren syndrome: Giving dry mouth and dry eye the full treatment," 350-367.

(19.) Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72.

(20.) Askari, Vivino, Current Concepts in the Evaluation and Treatment of Sjogren's Syndrome, 1-14; Carsons, Harris, eds, The New Sjogren's Syndrome Handbook, 29-36; Fox, "Sjogrens syndrome: New approaches to treatment;" Vivino, Katz, "Sjogren's syndrome: Clinical picture and diagnostic tests," 40-52.

(21.) Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72; Vivino, Orlin, "Sjogren syndrome: Giving dry mouth and dry eye the full treatment," 350-367.

(22.) Askari, Vivino, Current Concepts in the Evaluation and Treatment of Sjogren's Syndrome, 1-14.

(23.) Ibid.

(24.) Vivino, Orlin, "Sjogren syndrome: Giving dry mouth and dry eye the full treatment," 350-367.

(25.) Ibid.

(26.) Ibid.

(27.) Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72; F B Vivino et al, "Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjogren's syndrome: A randomized, placebo-controlled, fixed-dose, multicenter trial," Archives of Internal Medicine 159 (January 1999) 174-181.

(28.) Fox, "Sjogrens syndrome: New approaches to treatment."

(29.) Carsons, Harris, eds, The New Sjogren's Syndrome Handbook, 156-160.

(30.) Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72; Vivino, Orlin, "Sjogren syndrome: Giving dry mouth and dry eye the full treatment," 350-367.

(31.) Ibid.

(32.) Carsons, Harris, eds, The New Sjogren's Syndrome Handbook, 47; Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72.

(33.) Vivino, Orlin, "Sjogren syndrome: Giving dry mouth and dry eye the full treatment," 350-367.

(34.) Carsons, Harris, eds, The New Sjogren's Syndrome Handbook, 50.

(35.0 Ibid, 189; Vivino, Orlin, "Sjogren syndrome: Giving dry mouth and dry eye the full treatment," 350-367.

(36.) Askari, Vivino, Current Concepts in the Evaluation and Treatment of Sjogren's Syndrome, 1-14.

(37.) Ibid' Carsons, Harris, eds, The New Sjogren's Syndrome Handbook, 147-155; Vivino, "Diagnosis and treatment of Sjogren's syndrome," 65-72.

(38.) Carsons, Hams, eds, The New Sjogren's Syndrome Handbook, 174-175.

(39.) Ibid.

(40.) Ibid.

(41.) Ibid.

(42.) Ibid.

(43.) Ibid.

Lynn M. Petruzzi, RN, MSN, is an RN at West Shore Surgery Center, Mechanicsburg, Pa.

Frederick B. Vivino, MD, is a clinical associate professor of medicine at the University of Pennsylvania, Philadelphia; director, University of Pennsylvania Sjogren's Syndrome Center, Philadelphia; and chief of rheumatology, Presbyterian Medical Center, University of Pennsylvania Health System, Philadelphia.

COPYRIGHT 2003 Association of Operating Room Nurses, Inc.
COPYRIGHT 2003 Gale Group

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