Caring for Scleroderma
A Nurse’s Perspective
Juliann Nederostek, RN, MAED, BC
Clinical Nurse Specialist
Thomas Jefferson University Hospital
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
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.
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.
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
Limited Scleroderma
• Thickened skin-usually just the fingers and/or
face
• Milder form of scleroderma
• More common among Caucasians
• Less involvement of body organs
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)
Diffuse Scleroderma
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Thickened skin involves arms, legs and trunk
Inflammation of joints, tendons and muscles
Joint motion becomes difficult
Facial skin tightness
Mouth opening is reduced
Diffuse Scleroderma (cont’d)
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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
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
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
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
Caring for Scleroderma - Lung
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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
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
Nursing Considerations-Lung
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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
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
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
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.
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
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
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.
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
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
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.
Nursing Considerations Heart Failure
Nursing Care Right Sided Failure:
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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
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 )
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)
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
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)
Nursing Considerations - Kidney
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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
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.
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
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)
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.)
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.
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
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)
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
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
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.
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
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)
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)
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.
Nursing Considerations - Skin
Moisturizers:
• Eucerin Cream or Lotion
• Neutroderm
• Lubriderm
• Alpha- Keri Lotion
• Eutra Lotion
Nursing Considerations - Skin
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Candermyl Cream
Neutrogena – Norwegian Formula
Aquaphor Ointment
Lacticare Lotion
Moisturel
Complex – 15
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
Nursing Considerations – Skin
Shampoos:
• Neutrogena Regular Shampoo
• DHS Clear Shampoo
• Duplex
• Progaine
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.
Nursing Considerations - Skin
Laundry Detergents:
• All Powder
• Dash Powder
• Ivory Snow
• Safeskin
• Rinse out of clothes well so reduces itching
and irritation
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
Nursing Considerations –
Musculoskeletal
Occupational Therapist:
• Hand and finger exercises
• Hand splints
• Range of Motion
• Parafin baths
• Massage
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
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
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
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.
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.
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?)
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.
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.
References and Resources
• International Scleroderma Network
• www.sclero.org
• Johns Hopkins Scleroderma Center
• National Institute of Arthritis and Musculoskeletal
and Skin Diseases Information Clearinghouse
• http://www.niams.nih.gov/Health
Info/Scleroderma/default.asp
References and Resources
• National Institute of Arthritis and Musculoskeletal
& Skin Diseases (NIAMS)
• www.niams.nih.gov
• Sclerodema Clinical Trials Consortium
• www.sctc-online.org
• Scleroderma Research Foundation
• www.sclerodermaresearch.org
References and Resources
• The Arthritis Foundation
• www.arthritis
• The Scleroderma Foundation
• www.scleroderma.org
• www.sclero.org/chapter/delaware_valley/