Caring for Scleroderma

A Nurses 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
Everyones 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 nonself, 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-Raynauds 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
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 (contd)

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

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 cant
differentiate between old and new
fibrosis
Cardiac catheterization for

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 ConsiderationsLung

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 SclerodermaPericarditis

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 SclerodermaPericarditis

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:
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

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

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

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, DiGel)
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:
Dont 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 patients
disease as limited or diffuse
scleroderma
Active disease is manifested by
inflammatory signs such as
edematous skin

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

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

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
doctors 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 cant 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

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
patients 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/Scl
eroderma/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/