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Caring for Scleroderma A Nurse’s Perspective Juliann Nederostek, RN, MAED, BC Clinical Nurse Specialist Thomas Jefferson University Hospital.

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Presentation on theme: "Caring for Scleroderma A Nurse’s Perspective Juliann Nederostek, RN, MAED, BC Clinical Nurse Specialist Thomas Jefferson University Hospital."— Presentation transcript:

1 Caring for Scleroderma A Nurse’s Perspective Juliann Nederostek, RN, MAED, BC Clinical Nurse Specialist Thomas Jefferson University Hospital

2 Epidemiology Scleroderma is a rare disease Less than 500,000 people in the United States Effects more women than men Possible hormone connection, but unclear Age most commonly is between 35-50 years Young children, young adults and older adults can also develop scleroderma

3 Epidemiology Family members may have other autoimmune diseases African Americans and Native Americans generally have more severe Scleroderma Everyone’s experience with Scleroderma is different Choctaw Native Americans in Oklahoma are 20 times more likely than the general population, to develop systemic scleroderma.

4 Pathology Immune System – is a system of biological structures and processes within an organism that protects against disease. Autoimmune Disorder – is when the immune system fails to properly distinguish between self and non-self, and mistakenly attacks healthy body tissue.

5 Pathology Over production of connective tissue (like scarring) on the skin under the skin Small arteries, arterioles and capillaries narrow causing a lack of oxygen to tissue Cells make collagen as if they are injured and need to be fixed, and end up making too much collagen Extra collagen interferes with normal organ function

6 Limited Scleroderma Thickened skin-usually just the fingers and/or face Milder form of scleroderma More common among Caucasians Less involvement of body organs

7 CREST syndrome C-calcinosis – calcium deposits under the skin and in tissues ( hard white dots) R-Raynaud’s phenomenon E-Esophageal dysmotility - Heartburn S-Sclerodactyly – Thick skin on fingers T-Telangiectasias – Enlarged blood vessels (Red spots on the face and other areas)

8 Diffuse Scleroderma Thickened skin involves arms, legs and trunk Inflammation of joints, tendons and muscles Joint motion becomes difficult Facial skin tightness Mouth opening is reduced

9 Diffuse Scleroderma (cont’d) Skin with pigment changes (light or dark) Hair loss on the limbs Decreased sweating Dry skin Involvement of Internal Organs: Heart, Lungs, Gastrointestinal Tract, Kidneys

10 Lung (ventilation defect) Alveoli-primary gas exchange unit of the lung Pulmonary Capillaries-blood vessels in alveoli Alveolar Space/Pulmonary Capillaries allow for oxygen exchange Alveoli experience inflammation that MAY lead to fibrosis Result: Interference in oxygen exchange

11 Lung (pulmonary hypertension) Right heart pumps blood into the lung Lung/blood vessel system is a low pressure system with very low resistance If blood vessels or lung tissue become diseased, increased pressure in both pulmonary circulation and the right heart result

12 Caring for Scleroderma - Lung Symptoms: Shortness of breath gradually develops overtime Not able to breathe easily when you are active Cough can be a symptom of lung disease or dry upper airway membranes, G I Reflux Usually no chest pain with lung disease

13 Caring for Scleroderma - Lung Diagnostics: Chest x-ray may be normal in early stages Lung CT identifies changes in lung tissue Pulmonary Function Tests identifies changes in lung function, but can’t differentiate between old and new fibrosis Cardiac catheterization for measurement of pulmonary pressure

14 Caring for Scleroderma - Lung Treatment for pulmonary hypertension: Oxygen at night helps to relax the smooth muscle of the pulmonary artery Water pills Blood thinners Prostacyclin analogues are helpful to treat pulmonary hypertension, but must be given intravenously

15 Nursing Considerations-Lung Nursing Care: Quit smoking Improve reflux Compliance with medications Compliance with diagnostic studies Home oxygen therapy if needed Research in process for new pills to treat Scleroderma pulmonary hypertension

16 Heart Heart disease may be the result of microvascular disease, tissue fibrosis, pericardial disease and inflammation of the heart muscle. Arrhythmias (irregular heart beat), Pericarditis (outside membrane of the heart), Heart Failure Routine risk factors for heart disease should also be evaluated: family history, cholesterol, smoking

17 Caring for Scleroderma - Arrhythmia Irregular Heart Beat Symptoms: light-headed or dizzy Palpitations irregular pulse fainting Diagnostic Tools: ECG Holter Heart Monitor Treatment: Referral to Cardiologist (Electrophysiology) Medication or Pacemaker

18 Nursing Considerations- Arrhythmia Nursing Care: If you are dizzy or light-headed, find a safe place to sit down to prevent a fall. Do not attempt to drive. If this is a new symptom, notify the doctor.

19 Caring for Scleroderma-Pericarditis Pericarditis is inflammation and swelling of the outside membrane of the heart Pericarditis Symptoms: Chest pain when you breath deeply Shortness of breath High fever Diagnostic Tools: ECG, Echocardiogram Physical Exam

20 Caring for Scleroderma-Pericarditis Severe Pericarditis is rare in Scleroderma Severe Pericarditis treatment is diagnosed by the doctor Mild Pericarditis usually does not require treatment, but is monitored by the physician Pericarditis is temporary and gets better

21 Nursing Considerations Pericarditis Nursing Care: Notify the doctor to describe any new symptoms. Cardiologist referral as needed. Medical examination and diagnostic studies as directed by the doctor. Compliance with medical care Pericarditis improves with treatment.

22 Caring for Scleroderma-Heart Failure Heart failure is an uncommon heart problem that could occur in the later stages of Scleroderma Right sided heart failure usually occurs with pulmonary hypertension. Symptoms of right sided heart failure: Difficulty breathing Legs are swollen and weight gain from retaining fluids may occur

23 Caring for Scleroderma-Heart Failure Left sided heart failure occurs when scar tissue develops in the heart muscle and it becomes a less effective pump. Symptoms of Left sided heart failure: Difficulty breathing when you are active Inability to lie flat in bed Wake up in the middle of the night with shortness of breath

24 Caring for Scleroderma - Heart Failure Diagnostics: ECG, Echocardiogram, Stress Test, and if necessary, a cardiac catheterization Treatment: Referral to Cardiologist Medications or procedures will be decided by the doctor.

25 Nursing Considerations Heart Failure Nursing Care Right Sided Failure: Report symptoms to doctor Elevate legs when sitting Keep head of bed up for ease of breathing Evaluate need for supplemental oxygen Monitor fluid intake Monitor body weight

26 Nursing Considerations Heart Failure Nursing Care Left Sided Failure: Report symptoms to the doctor Elevate head of bed Pace activity to allow for rest periods Monitor shortness of breath episodes Evaluate the need for oxygen Monitor fluid intake Monitor body weight (weight gain may indicate fluid retention )

27 Caring for Scleroderma - Kidney Kidney disease or renal crises occurs early in patients with diffuse scleroderma. Renal blood vessels constrict dropping the blood flow to the kidney. Untreated, the low blood flow leads to kidney damage and failure. Reversible condition with treatment now available. (ace inhibitors)

28 Caring for Scleroderma - Kidney Scleroderma Renal Crisis occurs when the blood pressure can go from normal levels to dangerously high levels in a matter of days. Kidney damage can occur in a matter of hours or days. In the early stages, there are no symptoms. After the first few days, malignant phase hypertension can cause: headaches, nausea, and vomiting

29 Nursing Considerations - Kidney Nursing Care: (How to Prevent Kidney Damage) Detect and treat high blood pressure quickly. Use a home blood pressure monitor. Call your doctor IMMEDIATELY if your blood pressure is high. (If the top number goes over 150 or the bottom number goes over 90)

30 Nursing Considerations - Kidney Get your blood pressure down right away Medication dose will be changed Laboratory studies to check kidney function If necessary, hospitalization to achieve rapid control of blood pressure *Take your blood pressure at the same time every day

31 Gastrointestinal Tract - Mouth Dry mouth is result of inflammation/fibrosis of the salivary glands Saliva is 98% water, enzymes, electrolytes, mucous, and anti-bacterial compounds Saliva lubricates and protects the tongue, teeth, and tissues of the mouth Saliva begins the digestive process and breaks down food caught in the teeth Dry mouth results in increased plaque, tooth decay and gum disease Decreased mouth opening may be the result of facial skin tightness.

32 Gastrointestinal - Mouth Nursing Care: Decreased mouth opening causes difficulty in performing dental care. Inform dentist/dental hygienist of this issue prior to examination Utilize pediatric toothbrush for ease of care Periodontist to follow patient every three to six months Biotene mouth wash and toothpaste may be used

33 Gastrointestinal – Esophagus – Stomach – Small or Large Bowel Gastrointestinal tract is involuntary smooth muscle that can be affected. Peristalsis is the normal esophageal, stomach and bowel wall motion that moves food through the upper and lower digestive tract Inflammation and fibrosis results in decreased motility or dysmotility of this smooth muscle (May cause pseudo obstruction)

34 Gastrointestinal Symptoms Dysphagia is difficulty swallowing. Dyspepsia includes symptoms of upper abdominal pain, belching, nausea, vomiting, and abdominal bloating. Satiety is the satisfied feeling of being full. Early satiety is feeling full sooner than normal or after eating less than usual. ( Result of early stomach filling/delayed emptying.)

35 Gastrointestinal Symptoms Typical gastrointestinal reflux is heartburn. Atypical gastrointestinal reflux is chest pain, gagging, or dry cough. If untreated, may result in esophageal inflammation, esophageal stricture, or gastrointestinal bleeding. Alteration in bowel elimination presents as constipation or diarrhea.

36 Caring for Scleroderma – Gastrointestinal Diagnostics: Endoscopy – direct visualization of the upper gastrointestinal tract/esophagus Colonoscopy – direct visualization of the lower gastrointestinal tract/small and large intestine Gastroenterologist has the ability to also complete biopsies along the tract to further evaluate any changes

37 Caring for Scleroderma - Gastrointestinal Medications: Antacids (Mylanta, Maalox, Tums, Di-Gel) H2 Blockers (Tagamet, Zantac, Pepcid, Axid) Proton Pump Inhibitors (Prilosec, Prevacid) Others (Carafate, Propulsid)

38 Nursing Considerations - Gastrointestinal Nursing Care: Dietary Advice to avoid certain foods and drink that might make heartburn worse: Alcohol and Chocolate Acidic foods (oranges, tomato sauce) Fried foods (high fat, fast food, nuts, dairy) Raw vegetables/Onions Spicy food

39 Nursing Considerations - Gastrointestinal Nursing Care: Don’t eat within 2 hours of bedtime Eat slowly and sitting up Chew food carefully and swallow before the next bite Drink sips of water between bites Eat small frequent meals

40 Nursing Considerations - Gastrointestinal Nursing Care: Elevate the head of your bed at least 4 inches on wooden blocks (This lifts your esophagus above your stomach) Decrease pressure on your stomach (limit bending, vigorous exercise or tight clothing) Try to keep your body weight in a healthy range.

41 Skin Assessment is completed utilizing a skin score. Palpation of skin determines skin thickening Determines classification of patient’s disease as limited or diffuse scleroderma Active disease is manifested by inflammatory signs such as edematous skin Inactive disease is manifested by thickened sclerotic skin similar to mature scar tissue

42 Caring for Scleroderma - Skin Symptoms: Dry skin-collagen in the inner layer of the skin destroys sweat and oil glands. The outer layer of the skin has fatty substances that moisten the skin. In Scleroderma, this outer layer gets thinner so there is less oil. Itching Ulcers Calcium deposits Discoloration Enlarged blood vessels (telangiectsias)

43 Nursing Considerations – Skin Stop Dry Skin: Turn the thermostat down. Use a mild soap. Limit soap to certain areas.(Under the arms and groin) Rinse well. Use warm, not hot water. Use baby oil for bathing.(Caution: Tub will be slippery)

44 Nursing Considerations - Skin Use special soaps and shampoos. (Hypoallergenic, fragrance free cosmetics) Use moisturizers and moisturize often. Use gloves (When handling household cleaners) Avoid certain ointments/medications. (Dermoplast or antihistamines as they dry) Avoid electric blankets except to warm your bed.

45 Nursing Considerations - Skin Moisturizers: Eucerin Cream or Lotion Neutroderm Lubriderm Alpha- Keri Lotion Eutra Lotion

46 Nursing Considerations - Skin Candermyl Cream Neutrogena – Norwegian Formula Aquaphor Ointment Lacticare Lotion Moisturel Complex – 15

47 Nursing Considerations – Skin Bland Soaps: Alpha Keri Aveeno Bar (Dry Skin) Neutrogena(Dry Skin) Basis Unscented Bar Eucerin Dry Skin Cleansing Bar Moisturel Sensitive Skin Cleanser Dove Cetaphil

48 Nursing Considerations – Skin Shampoos: Neutrogena Regular Shampoo DHS Clear Shampoo Duplex Progaine

49 Nursing Considerations – Skin Sunscreen: Only use sunscreen containing Sun Protection Factor (SPF) greater than or equal to 15 Avoid the sun if you are on medications that make you sun sensitive Avoid: All sunscreens that contain Paba or Paba Ester skin reactions may occur.

50 Nursing Considerations - Skin Laundry Detergents: All Powder Dash Powder Ivory Snow Safeskin Rinse out of clothes well so reduces itching and irritation

51 Musculoskeletal Muscle weakness is due to: Autoimmune mediated inflammatory myositis Non-inflammatory fibrotic myopathy Disuse or wasting from deconditioning Malnutrition Side effect from medications such as corticosteroids or lipid lowering agents Tendon friction rubs and contractures

52 Nursing Considerations – Musculoskeletal Occupational Therapist: Hand and finger exercises Hand splints Range of Motion Parafin baths Massage

53 Nursing Considerations – Musculoskeletal Physical Therapy: (Prior to the start of physical therapy, pain and fatigue have to be controlled.) General exercise Water aerobics *All exercise must be supervised by a doctor and physical therapist

54 Psychological Emotional response to the disease: Mood disorders – depression Self image – affects the face and the hands Anxiety/panic – fear of the disease, distress over the unknown Pain – impacts on mood, sleep cycle, and effective coping

55 Psychological Societal role – maintain relationships within the family and society Chronic disease takes control away from the patient and adds fear of the unknown Patient should be a recognized participant in their care as part of the health care team

56 Sexuality Sexual function is often impaired, but rarely discussed. Males may have erectile dysfunction secondary to microvascular changes and tissue fibrosis. Females may have dry membranes, causing discomfort or pain with sexual intercourse. Professional counseling can be helpful.

57 Communication Guide Prepare a list of questions for your doctor’s appointment. Communicate what you would like to discuss. Initiate your questions as the doctor may not cover this information in your visit. Let doctor know if you can’t follow plan of care that is outlined.

58 Communication Guide Review your treatment with the doctor. Do not change medications or dose without speaking to the doctor. Discuss financial issues as needed. Talk to the doctor when you are dressed. Important medication information: (name, purpose, dose and schedule, should I avoid certain foods or drink?)

59 Communication Guide Identify the possible side effects of the medication. Identify the contact number for the office if you are having issues. The patient needs to develop an active role in participating in their health care. Scleroderma cannot be cured, but the symptoms can be managed.

60 Quality of Life Health care needs should consider the patient’s ability to have control and enjoy other aspects of their lives. Quality of life depends on personality traits of the patient, the physician/patient relationship, and social/family support. Patient education, expert care, and comprehensive medical management positively influence the quality of life.

61 References and Resources International Scleroderma Network Johns Hopkins Scleroderma Center National Institute of Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse Info/Scleroderma/default.asp Info/Scleroderma/default.asp

62 References and Resources National Institute of Arthritis and Musculoskeletal & Skin Diseases (NIAMS) Sclerodema Clinical Trials Consortium Scleroderma Research Foundation

63 References and Resources The Arthritis Foundation www.arthritis The Scleroderma Foundation

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