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CONNECTIVE TISSUE DISORDERSJimmy Hall, RN, BSN
OBJECTIVES
Describe care for patients with systemic lupus erythematosus (SLE)
Describe systemic effects of SLE Devise a teaching plan for the SLE patient Identify modifications in lifestyle
CONNECTIVE TISSUE DISORDERS
Involves a family of similar disorders Rheumatoid Arthritis (RA) Systemic Lupus Erythematosus (SLE) Polymyositis Scleroderma Polymyalgia rheumatica
COMMON FEATURES
Women are affected more frequently “Multisystem” diseases Many overlapping features
For example: Lupus/ Scleroderma Symptoms: Scleroderma features thickened skin from
excess collagen, leading to scar tissue while SLE features a rashes due to skin inflammation and a “butterfly” rash to face related to photosensitivity
Lupus/ Rheumatoid ArthritisSymptoms: Lupus (joint pain), RA (joint swelling)Lab tests: Sed rate, C-reactive Protein, ANA
Polymyositis
Inflammation of many muscles Causes muscle weakness
Shoulders, hips, thighs 5-10 cases per million adults per year
Treatement: high-dose corticosteroid therapy or immunosuppressants
Scleroderma “Hard Skin” due to
collagen deposits in skin and other parts of the body
18-20 people per million per year
Can be localized (to hands or feet) or can be systemic (affecting heart, lungs, kidneys, and intestines)
No one test can diagnose
Treatment depends on symptoms
Scleroderma Features Raynaud’s Phenomenon Swelling in hands Hard and rigid skin Dry skin (because sweat
secretion is suppressed) Extremities stiffen Expressionless face Heart failure Difficulty swallowing
(esophageal stiffening) Lungs scarred (affecting
respiration) Hardening of intestinal
mucosa Renal failure
Polymyalgia Rheumatica Involves proximal muscle discomfort and
joint swelling Neck, shoulder, and pelvic muscles
Occurs predominantly in Caucasians Patients are usually in people 50 years or
older 52 cases per 100,000 people annually Usually have a first-degree relative with
disease Genetic marker HLA-DR4
Diagnosis made by elimination Treatment: corticosteroids, NSAIDs
Polymyalgia Rheumatica Symptoms
Fever Weight Loss/Anorexia Fatigue Depression Giant cell arteritis
Headache with changes in vision and jaw claudication
LUPUS ERYTHEMATOSUS
Four types:1. Discoid lupus erythematosus (DLE)2. Drug-induced lupus erythematosus3. Neonatal lupus4. System lupus erythematosus (SLE)
**Most common and most severe**
DID YOU KNOW?
What does “lupus” mean in Latin? a. Evil b. Wolf c. Suffering d. Butterfly
WOLF
Some doctors thought the rash resembled the pattern of fur on a wolf's face, and some thought that the rash resembled an attack of an animal.
One account claims that the term was given after a French mask that women reportedly wore to conceal the rash on their faces. The mask is called a loup, French for “wolf.”
DISCOID LUPUS ERYTHMATOSUS
Chronic skin condition of sores with scarring and inflammation Often affects the face, scalp, and ears Sensitive to sunlight Corisone ointment used to slow progression and
to improve the lesions. Plaquenil is often prescribed – yearly eye exam!
DRUG INDUCED LUPUS ERYTHEMATOSUS
Results from long-term use of certain medications
Occurs often in men over age 50 because of the higher rate of having chronic diseases that require these medications.
Symptoms Often complain of flu-like symptoms
Muscle pain Joint Pain Fever Arthritis
DRUG INDUCED LUPUS ERYTHEMATOSUS
Medicines often linked to this are used to treat: Heart Disease Thyroid Disease Hypertension Neuropsychiatric disorders
Over 38 drugs have been linked Procainamide (Pronestyl) – for arrhythmias Hydralazine (Apresoline) – for hypertension Isoniazid (Laniazid) – antibacterial
It is important to identify which medication is causing the problem and to stop the medication!
NEONATAL LUPUS
Rare disorder caused by the transplacental passage of maternal autoantibodies
Only 1% of infants with positive maternal autoantibodies develop neonatal lupus erythematosus.
The most common clinical manifestations are cardiac, dermatologic, and hepatic.
The onset of neonatal lupus erythematosus occurs between birth and a few months of life.
SYSTEMIC LUPUS ERYTHEMATOSUS
Chronic, autoimmune disease affecting skin, joints, and possibly other organs
An increase in autoantibody production, resulting from suppressor T-cell function, leads to immune complex deposition and tissue damage
Inflammation stimulates antigens, which in turn stimulate additional antibodies, and this repeats
The body attacks own tissues and organs anywhere in the body Skin Kidneys Heart
SLE cause
Genetic factors Hormonal factors Environmental
factors Medications
HOW IS SLE DIFFERENT FROM HIV/AIDS?
HIV/AIDS is an immune deficiency disorder, where the immune system fails in fighting off infection.
SLE involves a hyperactive immune system that involves the immune system fighting itself.
SLE FACTS
1 per 1,000 to 2,000 people It is a disease of flare-ups Women age 15-44 are most affected. Women develop SLE ten times more often
than men. It is three times more common in African
Americans than Caucasians
SYSTEMIC LUPUS ERYTHEMATOSUS Sypmtoms
Fatigue Fever Weight loss or gain Joint Pain, stiffness and swelling Butterfly Rash on face* Skin lesions Memory Loss
Mouth sores Hair loss Raynaud’s
phenomenon Chest pain Dry eyes Easy Bruising Anxiety Depression
SLE and the body Pericarditis is the most common
cardiac manifestation Serum creatinine levels and
urinalysis are used to screen for renal involvement
Renal involvement can lead to hypertension
A butterfly rash occurs in more than 50% of SLE
patients
DID YOU KNOW?
Renal failure and intercurrent infection are the most common causes of death associated with SLE – Lupus Foundation of America
http://www.lupus.org/webmodules/webarticlesnet/templates/new_learnunderstanding.aspx?articleid=2238&zoneid=523
SLE DIAGNOSIS
Diagnosis is based on a complete history, physical exam, and blood tests.
The American College of Rheumatology (ACR) has listed 11 criteria for diagnosis of SLE.
ACR states that if you have 4 or more of the 11 criteria you probably have lupus.
http://www.lupusresearchinstitute.org/lupus/lupus_diagnosis
SLE CRITERIA SET FORTH BY ACR Scaly, raised rash, called a discoid rash Facial rash that is butterfly shaped and covers the
bridge of the nose and spreads across the cheeks Mouth Sores, usually painless Sun-related rash, appearing after exposure to sunlight Joint pain and swelling that occurs in two or more
joints Kidney disease Swelling of the linings around the lungs or the heart Low blood counts, such as anemia, thrombocytopenia,
or leukopenia A neurological disorder, such as seizures or psychosis Positive ANA test Other positive blood tests that may indicate an
autoimmune disease
SLE DIAGNOSIS
No single lab tests confirms SLE Lab tests reveal
Anemia Thrombocytopenia Leukocytosis Positive ANA
SLE Diagnosis
Lab testsAntinuclear Antibody (ANA) TestErythrocyte Sedimentation Rate (Sed
Rate)Coomb’s testC-reactive Protein
Other tests: Complete Blood Count (CBC) Urinalysis Kidney and Liver assessment Chest X-ray Electrocardiogram Syphilis test
Antinuclear Antibody (ANA) Test Antinulcear antibodies
are a unique group of autoantibodies that have the ability to attack structures in the nucleus of cells.
Nonspecific A positive (+) ANA test
can indicate autoimmune disease
Erythrocyte Sedimentation Rate (Sed Rate) Checks for inflammation
within the body Nonspecific The normal
sedimentation rate is: In males is 0-15
mm/hour In females is 0-20
mm/hour
Coomb’s Test
Looks for antibodies that bind to blood cells and cause premature red blood cell destruction
Two types: Direct – used to detect if antibodies are bound to
the surface of the red blood cell; often used to detect cause of anemia or jaundice
Indirect – searches for unbound antibodies; often used to detect if a reaction to a blood transfusion
A positive result indicates that there are antibodies acting against your red blood cells
C-Reactive Protein Test
CRP is produced by the liver It is present when there is inflammation
present in the body Often used to detect if treatment is working Elevated CRP levels indicated that there is an
acute inflammation present.
Does an elevated SED rate diagnose someone with SLE?
No. It only indicates that there is an inflammation.
Assessment Skin
Check for rashes or pigment changes Ask if there has been any skin changes and about
sensitivity to sunlight/UV light Heart
Auscultation for pericardial friction rub, which is associated with pleural effusions and myocarditis
Mouth Check for ulcerations (GI involvement)
Neurological Psychosis, depression, or seizures
Joints Tenderness, swelling, warmth, pain during movement,
and edema Joint involvement is symmetric and similar to that of
RA
TREATMENT
Goals: Prevent progressive loss of organ function Reduce the likelihood of acute disease Minimize disease-related disabilities Prevent complications from therapy
TREATMENT
Corticosteroids Nonsteroidal anti-inflammatory drugs
(NSAIDs) Antimalarial Drugs
Other potential medications: Antidepressants Narcotics
CORTICOSTEROIDS***Single most important medication for
treatment*** Used topically, orally, or intravenous (IV) Action
Block the production of substances that trigger allergic and inflammatory actions, such as prostaglandins.
They also impede the function of white blood cells which destroy foreign bodies and help keep the immune system functioning properly.
Side effects: Osteoporosis Hyperglycemia Infection
Nonsteroidal anti-inflammatory drugs (NSAIDs) Acions:
Block cyclooxygenase (COX) enzymes and reduce prostaglandin
Reduce pain, inflammation, and fever Often taken with corticosteroids to minimize the
requirement of corticosteroids Commonly used NSAIDs:
Aspirin Naproxen (Aleve) Ibuprofen
Side effects: Stomach ulcers Bleeding
Antimalarial Drugs Hydroxychloroquine (Plaquenil) is most commonly
prescribed. Mechanism of action is unknown Plaquenil improves:
Inflammation Muscle and joint pain Fatigue Skin inflammation
HYDROXYCHLOROQUINE (PLAQUENIL)
Side Effects: Nausea Stomach cramps Photosensitivity
Avoid UV light exposure Wear Sunscreen
Eye problems Color blind Loss of vision
HYDROXYCHLORQUINE (PLAQUENUIL)
One of the worst side effects of taking Plaquenil is damage to the retina at the back of the eye.
Have a baseline eye exam Have eye exams every 6-12 months after
initiating therapy Problems mostly occur when:
kidney problems are present Taking medication for over 10 years
1. Sit in an area with good lighting, wear your reading glasses and look at the grid at a comfortable position (30-40cm).
2. Cover one eye 3. Look directly at the center dot. While looking directly at the dot note whether all lines of
the grid are straight or if any areas are distorted, blurred or dark. 4. Repeat the test with the other eye
If you experience: distortion or curvy lines blurring holes or spots in some areas of the grid
SEE YOUR OPHTHALMOLOGIST IMMEDIATELY!
http://sw-eng.com/plaquenil-eyes.html
TREATMENT CONTINUED
Immunosuppressives Medications that suppress the immune system Used mainly in severe cases of SLE who have not
responded to other therapies Serious Side Effects (ex: infection, liver damage,
infertility, increased risk of cancer) Commonly used:
Cyclophosphamide (Cytoxan) Azathioprine (Imuran, Azasan) Methotrexate Mycophenolate (CellCept)
ALTERNATIVE MEDICINE
Omega-3 Fish Oil Flaxseed
Cayenne (Capsicum) creams - analgesic
FISH OIL Contains two omega-3: eicosapentaenoic acid
(EPA) and docosahexaenoic acid (DHA) Actions:
Fight inflammation Reduce fatigue Stimulate circulation Also: reduce triglycerides, heart rate, blood
pressure, atherosclerosis, and increases brain activity
Side effects: Bloating Belching Gas Diarrhea
FLAXSEED OIL
Contains an omega-3 called alpha-linolenic acid (ALA)
Decreases inflammation in the body Improves kidney function
Common Side Effects: Bloating Abdominal pain
DID YOU KNOW?
Michael Jackson had Lupus
LIFESTYLE CHANGES
Avoid Sunlight Control Infection Birth Control Get plenty of rest Don’t smoke Eat a healthy diet
Therapy
Physical therapy can be beneficial in order to: Improve range of motion and functional mobility Identify exercises that are beneficial for the
patient Occupational therapy can benefit the patient
by Restoring functional independence (bathing,
feeding, toileting) Identifying appropriate equipment (raised toilet
seats, splints, elastic shoelaces)
Nursing Diagnoses… What to do? Fatigue
Sleep Rest Exercise Treat Depression
Impaired skin integrity Avoid sun/UV light
exposure Wear sunscreen/clothing
when exposed
Body image disturbance Support groups Education: rash can come
and go Medication
Lack of knowledge for self-management decisions Educate patient Disease involves multiple
systems Avoid sunlight Dietary changes due to
increased risk for cardiovascular disease
Potential side effects Follow up care
ANY QUESTIONS?
Post Test
Which of the following is not a feature of Systemic Lupus Erythematosus:
It affects women more than men It affects Caucasians more that African
Americans. It primarily affects those ages 15-44. It is commonly associated with a butterfly rash.
Which of the following is not a feature of Systemic Lupus Erythematosus:
It affects women more than men It affects Caucasians more that African
Americans. It primarily affects those ages 15-44. It is commonly associated with a butterfly rash.
When caring for a patient taking hydroxychlorquine (Plaquenil), the nurse overhears the patient’s conversation. What statement by the patient indicates teaching is needed?
“Me and my friend are going to eat seafood this afternoon.”
“It doesn’t matter if I am around my child when taking this medicine.”
“It’s been 5 months since I got my eyes last checked.”
“I am taking my first trip to the beach next week, and I am so excited!”
When caring for a patient taking hydroxychlorquine (Plaquenil), the nurse overhears the patient’s conversation. What statement by the patient indicates teaching is needed?
“Me and my friend are going to eat seafood this afternoon.”
“It doesn’t matter if I am around my child when taking this medicine.”
“It’s been 5 months since I got my eyes last checked.”
“I am taking my first trip to the beach next week, and I am so excited!”
Systemic Lupus Erythematosus is an autoimmune disorder characterized by a hyperactive immune system attacking itself.
True False
Systemic Lupus Erythematosus is an autoimmune disorder characterized by a hyperactive immune system attacking itself.
True False
A positive ANA is diagnostic that a patient has Systemic Lupus Erythematosus
True False
A positive ANA is diagnostic that a patient has Systemic Lupus Erythematosus
True False
A 27 year old female, diagnosed with Systemic Lupus Erythmatosus, has recently started taking Methotrexate for her disease. She informs her nurse that she and her husband have decided to have a baby. What should the nurse’s response be?
“So, that’s why the doctor started you on Methotrexate. I am so excited for you.”
“That is great! Your disease must be doing better.” “That’s not a great idea considering you are taking
Methotrexate ” “I would not recommend that you have a baby.
Women who have children always pass on the disease to their children.”
A 27 year old female, diagnosed with Systemic Lupus Erythmatosus, has recently started taking Methotrexate for her disease. She informs her nurse that she and her husband have decided to have a baby. What should the nurse’s response be?
“So, that’s why the doctor started you on Methotrexate. I am so excited for you.”
“That is great! Your disease must be doing better.” “That’s not a great idea considering you are
taking Methotrexate ” “I would not recommend that you have a baby.
Women who have children always pass on the disease to their children.”
THE END…
References: Lupus Foundation of America, Inc. (2009). Prognosis and a hopeful
future. Retrieved February 15, 2010, from http://www.lupus.org/webmodules/webarticlesnet/templates/new_learnunderstanding.aspx?articleid=2238&zoneid=523
Lupus Research Institute. (n.d.). Lupus diagnosis. Retrieved February 15, 2010, from http://www.lupusresearchinstitute.org/lupus/lupus_diagnosis
Smeltzer, S., Bare, B., Hinkle, J., & Cheever, K. (2008). Textbook of medical- surgical nursing (Eleventh ed.). New York: Lippincott, Williams & Wilkins
Software Engineering. (n.d.). Plaquenil & your eyes. Retrieved February 15, 2010, from http://sw-eng.com/plaquenil-eyes.html