M/S Module 5 study guide

Diagnostic testing for myasthenia gravis

-A standard series of laboratory studies is usually performed for patients with known or suspected MG. Thyroid function should be tested because thyrotoxicosis (excessive thyroid hormone) is present in a small number of myasthenic patients. -Serum protein electrophoresis evaluates the patient for immunologic disorders. Immunologic-based diseases, such as rheumatoid arthritis, systemic lupus erythematous, and polymyositis, may be associated with the disease -Several types of antibodies are found in the majority of patients with MG and include forms directed against the acetylcholine receptor (AChR) and the enzyme muscle-specific receptor tyrosine kinase (MuSK). -However, whereas a positive antibody test confirms diagnosis, a negative finding does not rule out the disease. -Some patients with MG have a thymoma, and therefore patients are assessed for this condition. The thymus, an H-shaped gland located in the upper mediastinum beneath the sternum, is where B- and T-cells interact, refining self-recognition of these white blood cells. It is hypothesized that thymic abnormalities cause the breakdown in tolerance that causes the immune-mediated attack on AChR in myasthenia gravis. A thymoma is seen on a chest x-ray or a CT scan. - Most common electrodiagnostic test performed to detect MG is repetitive nerve stimulation (RNS) of proximal nerves. diagnoses most cases of generalized MG but far fewer cases of ocular MG. Each nerve studied is electrically stimulated 6 to 10 times at 2 or 3 Hertz. The compound muscle action potential (CMAP) is recorded with surface electrodes over muscle. In MG, there is a progressive decline in CMAP amplitude (force, or strength) with the first 4 or 5 stimuli. During electromyography (EMG) to diagnose MG, a recording electrode is placed into skeletal muscle and the electrical activity of skeletal muscle can be monitored in a way similar to electrocardiography (ECG). A progressive decrease in the amplitude of the electrical waveform is a classic sign of MG. -Single-fiber EMG (SFEMG) is a newer and most sensitive form of electromyography in detecting defects of neuromuscular transmission. This test compares the stability of the firing of one muscle fiber with that of another fiber innervated by the same motor neuron. The time interval between the two firings normally shows a minor degree of variability, called jitter. Defective transmission increases jitter or actually blocks successive discharges. This test can diagnose almost all cases of generalized and ocular MG. -Pharmacologic tests with the cholinesterase inhibitors edrophonium chloride (Tensilon) and neostigmine bromide (Prostigmin) may be performed. This older test is often referred to as a Tensilon challenge test. Tensilon is used most often for testing because of its rapid onset and brief duration of action. This drug inhibits the breakdown of ACh at the postsynaptic membrane, which increases the availability of ACh for excitation of postsynaptic receptors. Tensilon testing may be used also to help determine whether increasing weakness in the previously diagnosed myasthenic patient is due to a cholinergic crisis (too much cholinesterase inhibitor drugs) or a myasthenic crisis (too little cholinesterase inhibitor drugs). In a cholinergic crisis, muscle tone does not improve after giving Tensilon. Instead, weakness may actually increase, and fasciculations (muscle twitching) may be seen around the eye and face. **The Tensilon test can cause cardiac dysrhythmias and cardiac arrest, but these reactions rarely occur. Be sure that atropine sulfate, the antidote for Tensilon, is available in case these complications occur.

Signs and symptoms + Assessment of myasthenia gravis

**Motor Manifestations • Progressive (proximal) muscle weakness that worsens with repetitive use and usually improves with rest • Poor posture • Ocular palsies • Ptosis; incomplete eyelid closure • Diplopia • Respiratory compromise • Loss of bowel and bladder control • Fatigue **Sensory Manifestations • Muscle achiness • Paresthesias • Decreased sense of smell and taste -Ask about specific muscle weakness. Although the onset of MG is usually insidious (slow), some instances of fairly rapid development have been caused by infection, pregnancy, or anesthesia. A temporary increase in weakness may be noted after vaccination, menstruation, and exposure to extremes in environmental temperature. Disease may have periods of exacerbation or flares when symptoms worsen. Ask the patient when symptoms worsen, specifically noting the affected muscle groups and any limitation or inability in performing ADLs. Anticipate worsening symptoms with repetitive muscle use. - Patients with MG are typically hospitalized for diagnostic evaluation, myasthenic/cholinergic crisis resulting in respiratory failure, or periods of exacerbation when gas exchange is threatened. - Additional areas of inquiry include any history of ptosis (drooping eyelids), diplopia (double vision), or dysphagia (difficulty chewing or swallowing) and the type of diet best tolerated. Assess history of respiratory difficulty, choking, or voice weakness. Other areas of assessment include asking about any difficulty holding up the head, brushing teeth, combing hair, or shaving. Assess for the presence of paresthesias or aching in weakened muscles. Finally, ask about a history of thymus gland tumor. The most common symptoms of MG are related to involvement of the levator palpebrae or extraocular muscles. These symptoms may last only a few days at the onset and then resolve, only to return weeks or months later. Pupillary responses to light and accommodation are usually normal. - For most patients, the muscles of facial expression, chewing, and speech are affected (bulbar involvement). Note the patient's smile, which may be transformed into a snarl. The jaw may hang so that the patient must prop it up with the hand. Chewing and swallowing difficulties, choking, and regurgitation of fluids through the nose may lead to considerable weight loss. - Ask about the patient's nutritional intake and any recent weight loss. He or she may have more difficulty eating after talking. After extended conversations, the voice may be weaker or exhibit a nasal twang. In some patients, the tongue has fissures (ulcers). - Because limb weakness is more often proximal (closer to the body), the patient may have difficulty climbing stairs, lifting heavy objects, or raising the arms overhead. Neck weakness may be mild or severe enough to cause difficulty in holding the head erect. -In the most advanced cases of MG, all muscles are weakened, including those associated with respiratory function and the control of bladder and bowel. In these severe cases, ask about bowel and bladder function. Assess respiratory rate, depth, pattern, and Spo2 frequently to ensure adequate gas exchange. Muscle atrophy, although rarely severe, occurs in a small percentage of patients with MG. The tendon reflexes should be assessed, but they are not often affected. Assess for pain, although this is seldom a major concern. Some patients report that their weakened muscles ache. If present, paresthesias (painful tingling sensations) affecting the muscles of the face, hands, and thighs are not associated with any loss of sensation. Lost or decreased sensations of smell and taste have been reported. Consciousness is not altered. - In Eaton-Lambert syndrome, a form of myasthenia often seen with small cell carcinoma of the lung, the muscles of the trunk and the pelvic and shoulder girdles are most commonly affected. Although weakness increases after exertion, muscle strength may temporarily increase during the first few contractions, followed by rapid decline. Diagnosis is confirmed by electromyography (EMG). Management differs somewhat from that of other types of MG. Treatment includes removing the tumor, managing the cancer, and administering drug therapy to release acetylcholine (ACh)

Surgical Management for Myasthenia Gravis

For patients with MG, thymectomy (removal of the thymus gland) is usually performed early in the disease. The procedure is not always immediately effective. Those who have surgery within 2 years of the onset of myasthenic symptoms show the most improvement, but many patients do not experience a change in status despite thymectomy. • Because there is no way to predict whether remission or improvement will occur, it is important to avoid making promises but be optimistic. •Immediately before surgery, pyridostigmine (Mestinon) may be given with a small amount of water to keep the patient stable during and after surgery. If steroids have been used, they are also given before surgery and are tapered during the postoperative period. Antibiotics are administered immediately before or during the surgery. Plasmapheresis may be used before and after surgery to decrease circulating antibodies. •One of two surgical approaches may be used: the transcervical incision (minimal access technique) or the sternal split. -The transcervical approach is becoming more popular because it allows more rapid recovery with less discomfort after surgery, especially if done using the video-assisted thoracoscopic surgery (VATS) technique. However, this procedure is used only for patients who do not have a thymoma. Only a small dressing and an IV line are needed after surgery. -The older sternal split procedure is preferred when patients have a thymoma. It allows the surgeon to directly see the mediastinum and areas around the thymus. When thymoma is present, all surrounding involved structures (i.e., the pericardium, the innominate vein, a portion of the superior vena cava, and a portion of the lung) are removed. A single chest tube is placed in the anterior mediastinum. The patient is usually admitted to the critical care unit after surgery. Thymoma should be considered as a potentially malignant tumor requiring prolonged follow-up. •The presence of myasthenic weakness can still complicate its management. Although patients with adequate respiratory effort and gas exchange may be extubated immediately after surgery, most require a gradual weaning from the ventilator. Prolonged ventilatory assistance is rare. After the patient is extubated, pay special attention to respiratory status and maintaining a patent airway. Encourage the patient to turn, breathe deeply 3 to 6 times every 15 to 30 minutes in the hours after extubation, and use incentive spirometry.

thymectomy nursing care

For the patient having a thymectomy, monitor respiratory effort and promote effective gas exchange. Observe for signs of pneumothorax or hemothorax, including: • Chest pain • Sudden shortness of breath • Diminished or delayed chest wall expansion • Diminished or absent breath sounds • Restlessness or a change in vital signs (decreasing blood pressure or a weak, rapid pulse) If respiratory distress or symptoms of ineffective gas exchange occur, provide oxygen to the patient and raise the head of the bed to at least 45 degrees. Then report any of these signs and symptoms to the surgeon or Rapid Response Team immediately!

Other drug therapy for Rheumatoid Arthritis

Glucocorticoids (steroids)—usually prednisone (Deltasone)—are given for their fast-acting anti-inflammatory and immunosuppressive effects. Prednisone may be given in high dose for short duration (pulse therapy) or as a low chronic dose. Moderate-dose short-term tapering bridge therapy may be used when inflammation is symptomatic and other RA medications are insufficient or have not yet had an effect. Chronic steroid therapy can result in numerous complications, such as: • Diabetes mellitus • infection • Fluid and electrolyte imbalances • Hypertension • Osteoporosis • Glaucoma ◾Instruct patients taking chronic steroids to take calcium 1200 to 1500 mg daily plus vitamin D 400 mg daily to help prevent osteoporosis. Bisphosphonate drugs may also be prescribed. Bone density measurements (DEXA [dual-energy x-ray absorptiometry] scans) are done every 2 to 3 years to monitor for bone loss. ◾Patients with RA may experience one or a few joints that have more pain and inflammation than the others. Cortisone injections in single joints may be used to relieve local pain and inflammation. Have the patient ice and rest the joint for 24 hours after the procedure. Oral analgesics also are sometimes needed during that time. ◾Other immunosuppressive agents that may be used as a last resort are azathioprine (Imuran) and cyclophosphamide (Cytoxan). ◾Cyclophosphamide is sometimes given specifically to control RA vasculitis. Such immunosuppressive drugs may cause bone marrow suppression and occasionally leukemia or lymphoma. White blood cell counts are expected to decrease 7 to 14 days after the administration of IV cyclophosphamide; therefore monitor laboratory results closely to ensure safe limits. Hemorrhagic cystitis is a concern more with oral cyclophosphamide. Instruct the patient to drink water and void frequently (about every 2 hours while awake), which dilutes the urine and empties the bladder, thus decreasing opportunity for bladder irritation from residual drug. Hair thinning or loss can be seen with immunosuppressive medications. Cyclophosphamide may also cause sterility; strict birth control is recommended

Immunosuppressant use on myasthenia gravis

Immunosuppression may be accomplished with the use of corticosteroids, methotrexate, a chemotherapeutic agent, or rituximab, a biologic agent effective against B-cells. B-cells are lymphocytes active in antibody formation. -For ocular MG, corticosteroid treatment that does not cause significant systemic complications may significantly reduce the prevalence of generalized myasthenia gravis after 2 years on the drug. IV immunoglobulins (IVIGs) may also be used for acute disease management or as a long-term option for disease refractory to other treatment.

Emergency care: cholinergic Crisis

In cholinergic crisis, do not give anticholinesterase drugs while the patient is maintained with mechanical ventilation. Atropine 1 mg IV may be given and repeated, if necessary. When atropine is prescribed, observe the patient carefully. Secretions can be thickened by the drug, which causes more difficulty with airway clearance and possibly the development of mucus plugs. Unless complications such as pneumonia or aspiration develop, the patient in crisis improves rapidly after theappropriate drugs have been given. Continue to provide assistance as necessary because he or she tires easily after minimal exertion

Joint deformity in rheumatoid arthritis

Joint deformity occurs as a late, articular manifestation, and secondary osteoporosis can cause bone fractures. Observe common deformities, especially in the hands and feet. Extensive wrist involvement can result in carpal tunnel syndrome Gently palpate the tissues around the joints to elicit pain or tenderness associated with other rheumatoid complications, unless the patient is having severe joint pain. For example, Baker's cysts (enlarged popliteal bursae behind the knee) may occur and cause tissue compression and pain. Tendon rupture is also possible, articularly rupture of the Achillestendon.

Mangement of myasthenia gravis

MG is one of the most treatable neurologic disorders. The classic presentation of MG is muscle weakness that increases when the patient is fatigued and limits his or her mobility and ability to participate in activities. Management for this disease falls into two categories: • Treatment that affects the symptoms of MG without influencing the actual course of the disease (anticholinesterases or cholinergic drugs) • Therapeutic efforts for inducing remission, such as the administration of immunosuppressive drugs or corticosteroids, plasmapheresis, and thymectomy (removal of the thymus gland) -Both myasthenic crisis and cholinergic crisis increase muscle weakness and the patient's risk for respiratory compromise. The diaphragm and intercostal muscles may be affected, which inhibits the patient's ability to maintain adequate gas exchange, breathe deeply, and cough effectively. In addition, dysphagia may result in the aspiration of foods, liquids. Because of their respiratory muscle involvement, many pt have an increased risk for lung infections. The patient who cannot cough effectively may require oropharyngeal or nasopharyngeal suctioning. -Collaborate with the respiratory therapist (RT) to provide chest physiotherapy consisting of postural drainage, percussion, and vibration to mobilize secretions and improve gas exchange. -Because breathing difficulty or the inability to breathe easily is frightening, be aware of the patient's mental and emotional status during periods of respiratory compromise. Monitor his or her response to drug therapy for muscle weakness. Monitor for pulmonary congestion that can lead to respiratory complications like pneumonia and atelectasis. -Noninvasive mechanical ventilation (NIMV) can be used to support patients with acute respiratory failure from MG crisis while awaiting improvement from IV immunoglobulin (IVIG) therapy or plasma exchange. -Assess the patient's muscle strength before and after periods of activity. Provide assistance as necessary to prevent the patient from becoming fatigued. Schedule him or her for tests, treatments, and other activities early in the day or during the energy peaks after giving -Keep a bag-valve-mask setup (e.g., Ambu), equipment for oxygen administration, and suction equipment at the bedside of the patient with myasthenia gravis in case of respiratory distress. the prescribed drugs.

What is myasthenia gravis?

Myasthenia gravis (MG) is an acquired autoimmune disease characterized by muscle weakness. There are two types of MG: ocular andgeneralized. About two thirds of patients initially present with reports about vision that arise from disturbances of the ocular muscles. MG may take many forms—from mild disturbances of the cranial and peripheral motor neurons to a rapidly developing, generalized weakness that may lead to death from respiratory failure. MG can present at any age, and the incidence is slightly higher among men. It is a progressive disease. MG is caused by distorted acetylcholine receptors (AChRs) in the muscle motor end plate membranes. Antibodies are attached to the AChRs. As a result, nerve impulses are reduced at the neuromuscular junction; nerve impulses do not result in muscle contraction.

Emergency care: Myasthenic Crisis

Myasthenic crisis is often caused by some type of infection. For other patients, increasing muscle weakness leads to an overdose of anticholinesterase drugs. As a result, the patient may experience a mixed crisis. The Tensilon test, although not always conclusive, is important procedure for differentiation. Tensilon produces a temporary improvement in myasthenic crisis but worsening or no improvement of symptoms in cholinergic crisis. -The priority for nursing management of the patient in myasthenic crisis is maintaining adequate respiratory function to promote gas exchange. The acutely ill patient may need intensive nursing care for monitoring. He or she may require mechanical ventilation or other technologic support. -Cholinesterase-inhibiting drugs are withheld because they increase respiratory secretions and are usually ineffective for the first few days after the crisis begins. Drug therapy is restarted gradually and at lower dosages.

NSAID Therapy for rheumatoid arthritis

NSAIDs are sometimes used for RA to relieve pain and inflammation. The choice of which one to prescribe depends on the patient's needs and tolerance, as well as the scientific evidence supporting the drug therapy. To decrease GI problems, the NSAID may be given with an H2-blocking agent, such as ranitidine (Zantac) or misoprostol (Cytotec). If there is no clinical change after 6 to 8 weeks, the health care provider may discontinue the current NSAID and try another one or change to a different drug class. It was once thought that celecoxib (Celebrex), a COX-2 inhibiting NSAID, should be given rather than the older NSAIDs like ibuprofen. However, all COX-2 inhibiting drugs have recently been associated with cardiovascular disease, such as myocardial infarction, and some have been taken off the market. The risk for GI bleeding is also high in patients taking Celebrex, and the drug cannot be given to those who have had recent open heart surgery

Complications of RA

Numerous extra-articular clinical manifestations are associated with advanced disease. Assess the patient to ascertain systemic involvement. In addition to increased joint swelling and tenderness, moderate to severe weight loss, fever, and extreme fatigue are common in late disease exacerbations, often called "flare-ups." ◾Some patients have the characteristic round, movable, nontender subcutaneous nodules, which usually appear on the ulnar surface of the arm, on the fingers, or along the Achilles tendon. These nodules can disappear and reappear at any time and are associated with severe, destructive disease. Rheumatoid nodules usually are not a problem themselves; however, they occasionally open and become infected and may interfere with ADLs. Accidentally bumping the nodules may cause discomfort. Occasionally, nodules occur in the lungs. ◾Inflammation of the blood vessels results in vasculitis, particularly of small to medium-size vessels. When arterial involvement occurs, major organs can become ischemic and malfunction. Assess for ischemic skin lesions that appear in groups as small, brownish spots, most commonly around the nail bed (periungual lesions). Monitor the number of lesions, note their location each day, and report vascular changes to the health care provider. Increased lesions indicate increased vasculitis, and a decreased number indicates decreased vasculitis. Also carefully assess any larger lesions that appear on the lower extremities. These lesions can lead to ulcerations, which heal slowly as a result of decreased circulation. ◾Peripheral neuropathy associated with decreased circulation can cause footdrop and paresthesias (burning and tingling sensations), usually in older adults. ◾Respiratory complications may manifest as pleurisy, pneumonitis, diffuse interstitial fibrosis, and pulmonary hypertension. ◾Cardiac complications include pericarditis and myocarditis. ◾Assess for eye involvement, which typically manifests as iritis and scleritis. If either of these complications is present, the sclera of one or both eyes is reddened and the pupils have an irregular shape. Visual disturbances may occur. Several syndromes are seen in patients with advanced RA. ◾The most common is Sjögren's syndrome, which includes a triad of: • Dry eyes (keratoconjunctivitis sicca [KCS], or the sicca syndrome) • Dry mouth (xerostomia) • Dry vagina (in some cases) Note the patient's report of dry mouth or dry eyes. Some patients state that their eyes feel "gritty," as if sand is in their eyes. Inspect the mouth for dry, sticky membranes and the eyes for redness and lack of tearing. ◾Less commonly observed is Felty's syndrome, which is characterized by RA, hepatosplenomegaly (enlarged liver and spleen), and leukopenia. ◾Caplan's syndrome is characterized by the presence of rheumatoid nodules in the lungs.C

What is Plasmapheresis

Plasmapheresis is a method by which antibodies are removed from the plasma to decrease symptoms. This is used as short-term management of an exacerbation of MG. •Six exchanges occur over a 2-week period with follow-up exchanges weekly or monthly as needed, usually as an ambulatory care patient.

What is Rheumatoid arthritis ?

Rheumatoid arthritis (RA) is a chronic, progressive, systemic inflammatory autoimmune disease process that affects primarily the synovial joints. Systemic means this disease affects the body system, affecting many joints and other tissues. The cause for this trend is not known. In RA, transformed autoantibodies (rheumatoid factors [RFs]) are formed that attack healthy tissue, especially synovium, causing inflammation. ◾The disease then begins to involve the articular cartilage, joint capsule, and surrounding ligaments and tendons. immunity and inflammation factors cause cartilage damage in patients with RA • CD4 T-helper cells and other immune cells in synovial fluid promote cytokine release, especially interleukin-1 (IL-1) and tumor necrosis factor-alpha (TNFA), which attack cartilage. • Neutrophils and other inflammatory cells in the joint are activated and break down the cartilage. • Immune complexes deposit in synovium, and osteoclasts are activated. • B- and T-lymphocytes of the immune system are stimulated and increase the inflammatory response. The synovium then thickens and becomes hyperemic, fluid accumulates in the joint space, and a pannus forms. The pannus is vascular granulation tissue composed of inflammatory cells; it erodes articular cartilage and eventually destroys bone. As a result, in late disease, fibrous adhesions, bony ankylosis, and calcifications occur; bone loses density, and secondary osteoporosis occurs. ◾Permanent joint changes may be avoided if RA is diagnosed early. Early and aggressive treatment to suppress synovitis may lead to a remission. ◾RA is a disease characterized by natural remissions and exacerbations.Preventing flares helps prevent joint erosion and permanent joint damage. Because rheumatoid arthritis is a systemic disease, areas of the body besides the synovial joints can be affected. Inflammatory responses similar to those occurring in synovial tissue may occur in any organ or body system in which connective tissue is prevalent. If blood vessel involvement (vasculitis) occurs, the organ supplied by that vessel can be affected, leading to eventual failure of the organ or system in late disease. its development.

What is Stevens-Johnson syndrome

Stevens-Johnson syndrome is often a drug-induced skin reaction caused by an immunologic mechanism, similar to toxic epidermal necrolysis. The disorder may be mild with only skin involvement, or it may be severe and systemic. The skin lesions are widely distributed, including mucous membranes, and varied in appearance • The patient has a mix of vesicles, erosions, and crusts. With severe involvement, the patient may have respiratory problems, excessive fluid loss, kidney failure, and blindness. •Removal of the offending drug is critical. Mild forms of the disorder are usually self-limiting in 10 to 14 days unless the episode was triggered by a bacterial infection. Then, antibiotics are needed. •Severe problems require high doses of steroids to suppress the inflammation. Supportive care may include fluid replacement, mechanical ventilation, and even renal replacement therapy.

S/S Steven Johnson syndrome

Symptoms of this condition include: ◾Facial swelling ◾Tongue swelling ◾Hives, itching of skin ◾Skin pain ◾A red or purple skin rash that spreads within hours to days ◾Blisters on your skin and the mucous membranes of your mouth, nose, eyes and genitals ◾Shedding of your skin ◾Fever ◾General ill feeling ◾Joint aches Multiple skin lesions: Start quickly and may return, May spread, May appear as a nodule, papule, or macule and may look like hives, Central sore surrounded by pale red rings, also called a "target", "iris", or "bulls-eye", May have vesicles and blisters of various sizes (bullae), Located on the upper body, legs, arms, palms, hands, or feet, May involve the face or lips, Usually even on both sides (symmetrical) Other symptoms that may occur with this disease: ◾Bloodshot eyes ◾Dry eyes ◾Eye burning, itching, and discharge ◾Eye pain ◾Mouth sores ◾Vision problems

Manifestations of Rheumatoid arthritis

The onset of rheumatoid arthritis (RA) may be acute and severe or slow and progressive; patients may have vague symptoms that last for several months before diagnosis. The onset of the disease is more common in the winter months than in the warmer months. The manifestations of RA can be categorized as early or late disease and as articular (joint) or extra-articular ◾Early Disease Manifestations. The patient with RA typically reports joint signs and symptoms of joint inflammation,generalized weakness, and fatigue. Anorexia and a weight loss of about 2 to 3 pounds (1 kg) usually occur early in the disease process. Persistent low-grade fever may accompany these manifestations. In patients with early disease, the upper-extremity joints are involved initially—often the proximal interphalangeal (PIP) and metacarpophalangeal (MCP) joints of the hands. These joints may be slightly reddened, warm, stiff, swollen, and tender or painful, particularly on palpation (caused by synovitis). The typical pattern of joint involvement in RA is bilateral and symmetric (e.g., both wrists). The number of joints involved usually increases as the disease progresses. In early disease, the patient may report migrating symptoms known as migratory arthritis. The presence of only one hot, swollen, painful joint (out of proportion to the other joints) may mean the joint is infected. Refer the patient to the health care provider (generally the rheumatologist) immediately if this is the case. Single hot, swollen joints are considered infected until proven otherwise and require immediate long-term antibiotic treatment. ◾Late Disease Manifestations. As the disease worsens, the joints become progressively inflamed and very painful. The patient usually has frequent morning stiffness (also called the gel phenomenon), which lasts for 45 minutes to several hours after awakening. On palpation, the joints feel soft and look puffy because of synovitis and effusions (joint swelling with fluid, especially the knees). The fingers often appear spindle-like. Note any muscle atrophy (which can result from disuse secondary to joint pain) and a decreased range of motion in the affected joints. Most or all synovial joints are eventually affected. The temporomandibular joint (TMJ) may be involved in severe disease, but such involvement is uncommon. When the TMJ is affected, the patient may have pain when chewing or opening the mouth. When the spinal column is involved, the cervical joints are most likely to be affected. During clinical examination, gently palpate the posterior cervical spine and identify it as cervical pain, tenderness, or loss of motion.

Drug therapy for myasthenia gravis

Two groups of drugs are typically prescribed for the treatment of myasthenia gravis (MG): anticholinesterases and immunosuppressants. Be sure to give these drugs on time to maintain blood levels and thus improve muscle strength. Monitor and document the patient's response to drug therapy. Provide information for the patient and the family about the indications for, effectiveness of, and side effects of the drugs used in the treatment of MG -Cholinesterase (ChE) inhibitor drugs are the first-line management of MG. These drugs are also referred to as anticholinesterase drugs or antimyasthenics. They enhance neuromuscular impulse transmission by preventing the decrease of ACh by the enzyme ChE. This increases the response of the muscles to nerve impulses and improves muscle strength. The ChE inhibitor drug of choice is pyridostigmine (Mestinon, Regonol). -Expect a day-to-day variation in dosage depending on the patient's changing symptoms. -Administer ChE inhibitors with a small amount of food to alleviate GI **Instruct the patient to eat meals 45 minutes to 1 hour AFTER taking ChE inhibitors to avoid aspiration. This is especially important if the patient has bulbar involvement. -Drugs containing magnesium, morphine or its derivatives, curare, quinine, quinidine, procainamide, or hypnotics or sedatives should be avoided because they may increase the patient's weakness. Antibiotics such as neomycin and certain tetracyclines impair transmitter release and also increase myasthenic symptoms **A potential adverse effect of ChE inhibitors is cholinergic crisis.Sudden increases in weakness accompanied by hypersalivation, sweating, and increased bronchial secretions help identify this as a cholinergic crisis rather than a myasthenic crisis. -A cholinergic crisis is more likely to be associated with nausea, vomiting, and diarrhea. Teach the patient and family to monitor for these two types of crises: 1. Myasthenic crisis—an exacerbation (flare-up or worsening) of the myasthenic symptoms caused by not enough anticholinesterase drugs 2. Cholinergic crisis— acute exacerbation of muscle weakness caused by too many anticholinesterase drugs

Classes of Myasthenia gravis

• Class I: Any ocular muscle weakness; may have weakness of eye closure; all other muscle strength is normal • Class II: Mild weakness affecting other than ocular muscles; may also have ocular muscle weakness of any severity: • Class IIa: Predominantly affecting limb, axial muscles, or both; may also have lesser involvement of oropharyngeal muscles • Class IIb: Predominantly affecting oropharyngeal, respiratory muscles, or both; may also have lesser or equal involvement of limb, axial muscles, or both • Class III: Moderate weakness affecting other than ocular muscles; may also have ocular weakness of any severity: • Class IIIb: Predominantly affecting oropharyngeal, respiratory muscles, or both; may also have lesser or equal involvement of limb, axial muscles, or both; • Class IV: Severe weakness affecting other than ocular muscles; may also have ocular muscle weakness of any severity: • Class IVa: Predominantly affecting limb, axial muscles, or both; may also have lesser involvement of oropharyngeal muscles • Class IVb: Predominantly affecting oropharyngeal, respiratory muscles, or both; may also have lesser or equal involvement of limb, axial muscles, or both; use of a feeding tube to avoid aspiration and maintain nutrition • Class V: Defined by the need for intubation, with or without mechanical ventilation, except when used during routine postoperative management

Collaborative Care for myasthenia gravis

•Occupational and physical therapists evaluate patients for assistive-adaptive devices. In collaboration with the nurse, they also teach the patient and family energy conservation techniques and ideas for making work and self-management easier after discharge from the hospital. •Weakness of the speech and facial muscles often results in dysarthric (slurred) and nasal speech. In collaboration with the speech-language pathologist (SLP), determine the patient's ability to communicate. Instruct the patient to speak slowly while attempting to lip-read. Repeat what the patient says to check that it is correct. Questions that can be answered with "yes" or "no" or by gestures may be used along with other communication systems such as eye blinking, notebook and pencil, computer, handheld mobile devices, and picture, letter, or word boards. •The patient with myasthenia gravis (MG) may have difficulty maintaining an adequate intake of food and fluid because the muscles needed for chewing and swallowing become weakened and tire easily. In collaboration with the dietitian, occupational therapist, and speech language pathologist, evaluate the patient's nutritional status and his or her ability to receive adequate oral nutrition. High-calorie snacks are often well tolerated. Monitor the effectiveness of the nutrition program by recording the patient's calorie counts, intake and output, serum prealbumin levels, and daily weights If cannot swallow, a feeding tube may be used. •The patient's inability to completely close the eyes may lead to corneal abrasions and further decrease vision and comfort. During the day, apply artificial tears to keep the corneas moist and free from abrasion. A lubricant gel and shield may be applied to the eyes at bedtime to provide more extensive coverage. To help relieve diplopia, cover the eyes with a patch for 2 to 3 hours at a time, one eye at a time. At times, patients tape their eyes shut at night.

Management for rheumatoid arthritis

◾ A synovectomy to remove inflamed synovium may be needed for joints like the knee or elbow. -Total joint arthroplasty (TJA) may be indicated when other measures fail to relieve pain. Initially, most patients are managed with disease-modifying antirheumatic drugs (DMARDs). As the name implies, these drugs are given to slow the progression of the disease. First-Line Disease-Modifying Antirheumatic Drugs. ◾Methotrexate (MTX) (Rheumatrex), an immunosuppressive medication, in a low, once-a-week dose (generally 25 mg or less per week orally) is the mainstay of therapy for RA because it is effective and relatively inexpensive. It is a slow-acting drug, taking 4 to 6 weeks to begin to control joint inflammation. Observe for desired therapeutic drug effects, such as a decrease in joint pain and swelling. Monitor patients for potential adverse effects, such as decreasing WBCs and platelets (as a result of bone marrow suppression) or elevations in liver enzymes or serum creatinine ***Patients taking MTX are at risk for infection. Teach them to avoid crowds and people who are ill. Remind patients to avoid alcoholic beverages while taking MTX to prevent liver toxicity. Teach them to observe and report other side and toxic effects, which include mouth sores and acute dyspnea from pneumonitis. Rarely, lymph node tumor (lymphoma) has been associated in those who have RA and are taking MTX. ◾Folic acid, one of the B vitamins, is often given to those who are taking MTX to help decrease some of the drug's side effects. Pregnancy is not recommended while taking methotrexate because birth defects are possible. Strict birth control is recommended for childbearing women who are in need of MTX to control their RA. If pregnancy is ever desired, instruct the patient to consult the rheumatologist as well as an obstetric/gynecologic (OB/GYN) health care provider. Generally, the health care provider will discontinue the drug at least 3 months before planned pregnancy. MTX may be restarted after birth if the patient does not breast-feed ◾Leflunomide (Arava) may be prescribed for some patients. It is a slowacting immune-modulating medication that helps diminish inflammatory symptoms of joint swelling and stiffness and improves mobility. The drug is generally prescribed as a loading dose of 100 mg orally daily for 3 days followed by 20 mg orally daily thereafter. Inform the patient that Arava takes 4 to 6 weeks and sometimes up to 3 months before maximum benefit is realized. Arava is a potent medication that is generally tolerated, but side effects of hair loss, diarrhea, decreased WBCs and platelets, or increased liver enzymes have been reported. Teach patients to report these changes, and monitor laboratory results carefully. Remind them to avoid alcohol. Inform them that Arava can cause birth defects, and therefore recommend strict birth control to women of childbearing age. Tell patients to contact the health care provider immediately if pregnancy occurs while taking the drug. Cholestyramine (Questran) is available to help block the drug's action. ◾Another DMARD sometimes used for RA is hydroxychloroquine Plaquenil). This drug slows the progression of mild rheumatoid disease before it worsens. It is an antimalarial drug that helps decrease joint and muscle pain. Patients generally tolerate Plaquenil quite well. In a few cases, mild stomach discomfort, light-headedness, or headache has been reported. **The most serious adverse effect of Plaquenil is retinal damage. Teach pts to report blurred vision or headache. Remind them to have an eye examination before taking the drug and every 6 months to detect changes in the cornea, lens, or retina. If this rare complication occurs,the health care provider discontinues the drug

Biological Response Modifiers. for Rheumatoid Arthritis :2

◾Anakinra (Kineret) is another biological response modifier. Instead of affecting tumor necrosis factor-alpha (TNFA), however, it works to inhibit a different protein signal of the immune system called interleukin- 1 (IL-1). IL-1 is also a pro-inflammatory protein that signals the immune system to increase inflammation. It is thought that IL-1 is a weaker protein than TNF, but having an alternative drug that targets a different receptor site is helpful when a patient cannot take other biologics. Those who have multiple sclerosis or tuberculosis cannot take TNF inhibitors, but Kineret can be used with this population. Injection site reactions occur more often with Kineret compared with other BRMs. Ice and hydrocortisone 1% cream are recommended. Remind patients to rotate injection sites. Kineret is administered with a simple jet for self-administration. The patient has the option to use the simple jet or administer the subcutaneous injection traditionally. ◾Abatacept (Orencia) and rituximab (Rituxan, MabThera) require IV infusions every 2 weeks to start and then may be more spread out, depending on the drug. Like the results of the other BRMs, patients usually report feeling a benefit from these drugs in 2 weeks, but it may take months for the maximum benefit to be seen. ◾Golimumab (Simponi) is the first biologic that is administered only once each month for both RA and psoriatic arthritis. Teach patients that this drug has a black box warning for serious infections that may lead to hospitalization or death from opportunistic pathogens ◾Tocilizumab (Actemra) is given when the patient cannot tolerate other drugs. Tocilizumab is different from other biologics because it is the first humanized interleukin-6 (IL-6) receptor-inhibiting monoclonal antibody that is available for patients with RA. It can be used alone or in combination with other DMARDs. Teach patients about adverse drug effects, including hypertension, GI distress, infection, and an increase in low-density lipoproteins (LDLs) and liver enzymes. Like for other biologics, teach the patient about the high risk of infection (e.g., tuberculosis) when taking Actemra. ◾One of the newest biologics is tofacitinib (Xeljanz), which has been approved for moderate to severe RA as monotherapy or in combination with methotrexate. This drug is a tyrosine kinase inhibitor (TKI). Tyrosine kinases usually facilitate cytokine-mediated (e.g., interleukin) signals that promote the inflammatory process. Teach patients that tofacitinib carries a black box warning alerting patients about its potential for serious opportunistic infections, tuberculosis, lymphoma, and other cancers.

Biological Response Modifiers. for Rheumatoid Arthritis

◾As a group, biological response modifiers (BRMs), sometimes called biologics, are one of the newest classes of DMARDs. Most BRMs neutralize the biologic activity of tumor necrosis factor-alpha (TNFA) by inhibiting its binding with TNF receptors. Any one of the BRMs may be tried. If one drug is not effective, the health care provider prescribes another drug in the same class. All these drugs are extremely expensive at this time, and insurance companies may not completely pay for their use. ◾Teach patients receiving any one of the BRMs that they are at a high risk for developing infection. Instruct them to stay away from people with infections and to avoid large crowds if possible. Remind patients with multiple sclerosis (MS), tuberculosis (TB), or a positive TB test that they should not receive TNF inhibitors because they make patients susceptible to flare-ups of these diseases. ◾Determine whether the patient has had a recent negative purified protein derivative (PPD) test for TB. If not, a PPD skin test is typically administered and the selected BRM is not started until a negative test result is confirmed. ◾Etanercept (Enbrel) is given subcutaneously as 25 mg twice weekly for most patients. Immunosuppression with medications such as methotrexate is generally tried before using Enbrel or other biological response modifiers. Methotrexate is often given in combination with biologic therapies because the combination may be more effective than either drug alone . Most patients tolerate Enbrel or Enbrel and methotrexate together; however, laboratory monitoring is important. Combination therapy requires CBC, serum creatinine, and a liver panel to be drawn regularly, generally every 4 to 8 weeks. -Injection site reaction and systemic infection (especially respiratory) are possible adverse effects. Ice and hydrocortisone 1% cream can be used if a red, itchy rash at the injection site develops. ◾Infliximab (Remicade), first approved to treat Crohn's disease, is given in a single IV infusion over several hours. The initial dose generally used for RA is 3 mg/kg of body weight. The drug dosage is repeated at weeks 2 and 6. After these first three infusions, a maintenance dose of 3 mg/kg of body weight is given every 8 weeks, depending on the response of the patient. For patients who do not respond to the first three infusions, the drug dosage may be increased up to 10 mg/kg of body weight given at 4- week intervals. The risk of side effects and adverse effects increases at higher doses. Patients typically take methotrexate before starting Remicade and continue on combination therapy***Teach the patient to report and observe for symptoms of Remicade infusion reaction: chest discomfort, tachycardia, shortness of breath, or light-headedness. If any of these symptoms are reported, decrease the IV rate or discontinue it!Acetaminophen and Benadryl are medications often given before the start of Remicade and are often used at the time of reported infusion reaction. Those who experience serious adverse effects, such as hypertension or anaphylaxis, require permanent discontinuation of the drug. ◾Adalimumab (Humira) is the first fully human TNFA inhibitor and is given by subcutaneous injection. Symptoms of inflammatory arthritis tend to decrease with the use of Humira, including less joint swelling, less stiffness, and better mobility. Injection site reactions and adverse effects similar to the other TNFA inhibitors have been reported. Careful monitoring, especially with combination therapy of Humira and methotrexate or other drug that affects the body's immunity, is important and similar to combination therapy with other BRMs.

Management of Steven Johnson syndrome

◾Since S-J syndrome is a medical emergency, those with this case are often admitted at the ICU or the burn unit. ◾Non-essential medications are to be stopped since the first and most important step in treating Stevens-Johnson syndrome is to discontinue any medications that may be causing it. Fluid replacement and nutrition may also be provided as supportive care because of the skin loss which can also result in significant loss of fluid from your body. Fluids and nutrients may be received via a nasogastric tube (NGT). ◾Wound care is also important. Here, the healthcare provider may gently remove any dead skin and place a medicated dressing over the affected areas. This may also be done through applying cool, wet compresses that will help soothe blisters while they heal. ◾Eye care may also be provided and the patient may be referred to an ophthalmologist. Aggressive lubrication of the ocular surface may be done. As inflammation and cicatricial changes ensue, most ophthalmologists use topical steroids, antibiotics, and symblepharon lysis. ◾As for the medications, these are the most commonly used for Stevens-Johnson syndrome: Pain medication to reduce discomfort, antihistamines (to relieve itching), Antibiotics to control infection, and topical steroids to reduce skin inflammation. ◾If left untreated, certain complications may arise, such as: Secondary skin infection (cellulitis), Blood infection (sepsis), Eye problems, damage to internal organs, and permanent skin damage.

Diagnostic testing for rheumatoid arthritis

◾The test for rheumatoid factor (RF) measures the presence of unusual antibodies of the immunoglobulins G (IgG) and M (IgM) types that develop in a number of connective tissue diseases. Many patients with RA have a positive titer (greater than 1 : 80), but not all positive results indicate the disease, especially in older adults. ◾The antinuclear antibody (ANA) test measures the titer of a group of antibodies that destroy the nuclei of cells and cause tissue death in patients with autoimmune disease. ◾The fluorescent method is sometimes referred to as FANA. If this test result is positive (a value higher than 1 : 40), various subtypes of this antibody are identified and measured. ◾When RA patients also have Sjögren's syndrome (SS) or if the syndrome occurs as a separate disease, several unusual anti-SS antibody types may be present. In particular, anti-SS-A (Ro) and anti-SS-B (La) antibodies are present in about 60% to 70% of those with Sjögren's syndrome or those with secondary Sjögren's and RA ◾Serum complement proteins, especially C3 and C4, are usually decreased in autoimmune diseases, including RA and lupus. ◾An elevated erythrocyte sedimentation rate (ESR), or "sed rate," can confirm inflammation or infection anywhere in the body. An elevated ESR helps support a diagnosis of an unspecified inflammatory disease. The test is most useful to monitor the course of a disease, especially for inflammatory autoimmune diseases. In general, the more severe the disease gets, the higher the ESR rises; as the disease improves or goes into remission, the ESR level decreases. ◾The high-sensitivity C-reactive protein, or hsCRP, is another useful test to measure inflammation and may be done with or instead of the ESR. As the name implies, it is more sensitive to inflammatory changes than the ESR. It is also very useful for detecting infection anywhere in the body. ◾The presence of most chronic diseases usually causes mild to moderate anemia, which contributes to the patient's fatigue. Therefore monitor the patient's complete blood count (CBC) for a low hemoglobin, hematocrit, and red blood cell (RBC) count. An increase in white blood cell (WBC) count is consistent with an inflammatory response. ◾A decrease in the WBC count may indicate Felty's syndrome, a complication associated with late RA. Thrombocytosis (increased platelets) can also occur in patients with late RA. ◾A standard x-ray is used to visualize the joint changes and deformities typical of RA. A CT scan may help determine the presence and degree of cervical spine involvement. ◾An arthrocentesis is an invasive procedure that may be used for patients with joint swelling caused by excess synovial fluid (effusion). A large-gauge needle inserted into the joint (usually the knee) to aspirate a sample of synovial fluid to relieve pressure. The fluid is analyzed for inflammatory cells and immune complexes, including RF. Fluid from patients with RA typically reveals increased WBCs, cloudiness, and volume. Teach the patient to use ice and rest the affected joint for 24 hours after arthrocentesis. Often the health care provider will recommend acetaminophen as needed for pain. If increased pain or swelling occurs, teach the patient or family to notify the health care provider immediately. **After an arthrocentesis, monitor the insertion site for bleeding or leakage of synovial fluid. Notify the health care provider if either of these problems occurs. ◾A bone scan or joint scan can also assess the extent of joint involvement. MRI may be performed to assess spinal column disease or other joint involvement.