Transcript Kind of lesions in dermatology 1

Skin Lesions
Kind of lesions in dermatology
1- Primary Skin Lesions
2-Secondary Skin Lesions
3- Special Skin lesions
PRIMARY LESIONS
PRIMARY
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MACULE
PAPULE
NODULE
VESICLE
BULLA
PUSTULE
WHEAL
Macule and Patch
• Description
– Circumscribed
– Flat
– Discoloration
– Smaller than 0.5 cm-macule
– Larger that 0.5 cm- patch
– May be brown, blue, red or hypogigmented
Macule and Patch
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Examples of Brown
– Café au lait spot
– Freckle
Examples of Blue
– Ink (tattoo)
– Mongolian spot
Red
– Rheumatic fever
– Drug eruptions
– Viral exanthems
Examples of Hypopigmented
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Vitiligo
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Tuberous sclerosis
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Tinea versicolor
Macule and Patch
TINEA VERSICOLOR
CAFÉ AU LAIT PATCH
Papule and Plaque
• Papule - Elevated, solid
lesion <0.5 cm in diameter
Ex: scabies/insect bites
• Plaque - Elevated,
solid”confluence of
papule”>0.5 cm in
diameter that lacks a deep
component
Ex: Psoriasis
Papule
• Description
– Lesion up to 0.5 cm in diameter
– Color varies: flesh, yellow , white, brown, red, blue or
violet
– May become confluent
– May form plaques
• Examples of flesh, yellow, white papules
– Flat warts
– Skin tages
– Closed comedo (acne)
– Molluscum contagiosum
Papules
• Brown
– Nevi
– Warts
– Melanoma
• Red
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Acne
Urticaria
Folliculitis
Insect bites
– Blue or Violaceous
examples
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Blue nevus
Kaposi’s sarcoma
Melanoma
Venous lake
Papule
Plaque
PSORIASIS
PLAQUE
PSORIASIS
NAIL
PITTING
Nodule
• Elevated, solid
lesion>0.5 cm in
diameter, a largerdeeper papule
• Lipoma
• Rheumatoid nudule
• erythema nodosum
Nodule
• Description
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Circumscribed
Often round
Solid lesion
More that 0.5 cm in
diameter
– Larger nodule is a tumor
• Examples of a nodule
– Warts
– Lipoma
– Neurofibromatosis
– Erythema nodosum
– Furnuncle
– Hemangioma
– Squamous cell and
metastitic CA,
Melanoma
LIPOMA
BENIGN TUMOR
Vesicle
• Vesicle - elevated,
circumscribed,
superficial fluid filled
blister less than 5 mm
in diameter
• Herpes simplex
• Herpes zoster
• Contact dermatitis
Vesicle
• Description
– Circumscribed collection of free fluid
– Up to 0.5 cm in diameter
• Examples of vesicle
– All the herpes
– Eczema
– Impetigo
Bulla
• Description of bulla
– Circumscribed collection of free fluid
– More that 0.5 cm in diameter
• Examples of bulla
– Fixed drug eruption
– Lupus erythematous
– Blister
Vesicles / Bulla
• Bulla - Localized fluid
collection>0.5 cm in
diameter, a large vesicle
• Pemphigus vulgaris
• Bullous pemphigoid
• Bullous impetigo
Pustule
• Papule that contains
purulent /pus material
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Folliculitis
Impetigo
Acne
Pustular psoriasis
CYSTIC
ACNE VULGARIS
ACNE
Pustule
• Description
– Circumscribed collection of leukocytes
– Free fluid
– Varies in size
• Examples of a pustule
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Impetigo
Herpes simplex, zoster, varicella
Acne
Keratosis pilaris
Wheal (Hive)
• elevated, irregular-shaped
area of cutaneous oedema;
solid, transient, changing,
variable diameter; red, pale
pink or white in colour
• Urticaria
• Urticaria pigmentosa
Wheal (hive)
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Firm
Edematous
Papule or plaque
Are transient
Result from infiltration
of fluid into the dermis
Examples;
Hives
Uticaria
Dermographism
SECONDARY
LESIONS
SECONDARY
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SCALES
FISSURE
SCAR
EROSIONS
ULCERS
ATROPHY
LICHENIFICATION
CRUSTS
Crust
• Is a collection of dried serum and cellular
debris- a scab
• Examples
– Acute eczematious inflammation
– Atopic on the face
– Impetigo- golden or honey colored
– Tinea capitis
Crust
• A collection of cellular
debris ,dried serum,and
blood
• A scab antecedent primary
lesion is usually a
vesicle,bulla or pustule
• Impetigo
• Capitis Tinea
Scale
• Thick stratum corneum that
results from hyperproliferation or
increased cohesion of
keratinocytes
• Scarlet fever
Scar
• A collection of new
connective tissue, may be
hypertrophic or atroohic
scar implies
dermoepidermal damage
• Burn
• Acne
Scar
• An abnormal formation of connective tissue,
implying dermal damage, after injury
• Are initially thick and pink, but become white and
atropic
• Examples
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Post surg.
Burns
Keloid
Post any herpes
Atrophy
• Thinning of the
epidermis, dermis or fat
that cause depression in
the skin surface
• Morphea
Atrophy
• A depression in the skin resulting from
thinning of the epidermis or dermis
• Examples
– Aging
– Lupus
– Striae
Fissure
• Vertical loss of
epidermis and dermis
with sharply defined
walls, crack in skin
• Perleche
• Eczema
Fissure
• A linear loss of epidermis and dermis with
sharply defined nearly vertical walls
• Examples
– Chapping – hands and feet
– Eczema on the finger tip
Erosion
• A partial focal loss of
epidermis, heals
without scarring
• Perleche
Ulcer
• A full-thickness, focal
loss of dermis, heals
with scarring
• Bed sore
• Syphilis
Ulcer
• A focal loss of epidermis and dermis, and heal
with scarring
• Examples
– Decubitus
– Ischemic
– Stasis ulcers
– Neoplasms
Pressure Ulcers
• are categorized into four stages depending on
the level of tissue involvement, or depth of
the sore
SPECIAL
SKIN
LESIONS
Excoriation
• Linear erosion included
by scratching
• Scabies
• Atopic Dermatitis
• Prurigo
Excoriation
• An erosion caused by scratching
• Are often linear
• Examples
– Scabies
– Atopic dermatitis
– Dry skin
Cyst
• Nodule that contains
fluid semisolidmaterial
• Acne
• Pilar cyst
Cyst
• A circumscribed lesion with a wall and a
lumen, it may contain fluid or solid matter.
• Examples
– Epidermal, pilar
Lichenification
• Focal area of thickened
skin produced by
chronic scratching or
rubbing
• Atophic Dermatitis
Lichenification
• An area of thickened epidermis induced by
scratching
• Skin lines are accentuated so it looks like a
washboard
• Examples
– Atopic dermatitis, chronic eczematous dermatitis
LICHENIFICATION
Petechiae
• A small purplish spot on
a body surface caused
by hemorrhage <0.5 cm
• Vasculitis
• DIC
Petechiae
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A circumscribed deposit of blood
Less than 0.5 cm in diameter
Does not blanch!!
Examples
– ITP, meningococcemia, vasculitis, leukemia, other
BAD stuff or could just be a minor injury site
Purpura
• A small purplish spot
• on a body surface
• caused by hemorrhage
>0.5 cm
• Platelet Disorder
Purpura
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A circumscribed deposit of blood
Greater that 0.5 cm in diameter
Does not blanch!!
Is usually raised
Examples
– Rocky mt spotted fever, HSP, platelet
abnormalities, advanced BAD stuff
Differences in skin
Pediatric differences: thinner skin
• Newborn's skin thinner, more easily hurt by endotoxins and infection, shearing and tearing forces
• Child's body absorbs more of topical products
• Harder to regulate body temperature
Factors in skin condition
• Age
• Hydration and nutrition
• Soap, laundry detergents, and topical products
• Medications
• Infectious processes (viral, bacterial, fungal)
• Mechanical forces (tearing, friction, shearing) & vascular damage
• Tape and adhesive products
• Allergy
• IV infiltration
• Temperature
• Bodily secretions: urine, stool, ostomy drainage, fistula
DIAGNOSTIC TESTS
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SKIN BIOPSY
PATCH TESTS
SKIN SCRAPING
TZANCK SMEAR
WOOD’S LIGHT EXAM
PHOTOGRAPHS
KOH TEST - FUNGUS
Specific Test
Nursing Considerations
Complete blood count (CBC)
Standard CBC includes:
1) white blood cells (WBC)
2) red blood cells (RBC)
3) hemoglobin content (Hgb)
4) hematocrit (Hct)
5) mean corpuscular volume (MCV)
6) mean corpuscular hemoglobin (MCH)
7) mean corpuscular hemoglobin concentration (MCHC)
8) platelet count and volume
Typically there are no specific pre-testing restrictions, although the
client should avoid eating a fatty meal prior to the test
Cultures - No test prep is needed
blood is placed in culture bottles to grow both aerobic and anaerobic
specimens to determine causative agent and potential treatment
Usually 2 to 3 blood samples are collected at timed intervals and/or from
different veins
Enzyme-linked immunosorbent assay (ELISA); also known as EIA
The enzyme catalyzes a color reaction when exposed to substrate
(enzyme immunoassay)
1) specific color changes indicate specific diseases
One of the tests used to diagnose human immunodeficiency virus (HIV) 2) the greater the density of color, the more advanced the disease
An assay that uses an enzyme-bound antibody to detect antigen
Most types of biological samples can be used, e.g., plasma, serum, urine,
cell extracts
Eosinophil count - absolute
Measures the number of eosinophils (white blood cells)
Usually helps to confirm a diagnosis
Immunoglobulin A (IgA) - Antibody testing focusing on IgA
Often used in allergy testing (and in the diagnosis of celiac disease)
IgA is usually measured as part of radioallergosorbent testing (RAST) or allergy skin
testing
Immunoglobulin E (IgE) - The allergen-specific IgE antibody test
Monitors immunotherapy or to see if a child has outgrown an allergy
The level of IgE does not correlate to the severity of an allergic reaction
IgE is usually measured by radioallergosorbent testing (RAST) or allergy skin testing
Radioallergosorbent testing (RAST) - Used to identify measurable allergen-specific
antibodies, e.g., IgE
Tests for reaction to certain respiratory and food allergy stimulants
Blood test - no special prep is needed
Specific Test
Nursing Considerations
Cultures - Used to detect a bacterial wound infection and to
determine and isolate the specific type of bacteria
A sterile swab is used to collect cells or pus; an aspiration of
fluid from deeper wounds may be indicated
Patch testing - Indicated when allergic contact dermatitis
(ADC) is suspected and does not respond to treatment
Procedure
1) standard contact allergens are applied to the upper back
using adhesive-mounted patches or plastic (Finn) chambers
containing minute amounts of allergen
2) skin under the patches is evaluated 48 to 96 hours after
application
•Skin/wound Studies
Skin scrapings - Used to diagnose fungal infections and
scabies
Scales are taken from the border of the lesion and placed onto
a microscope slide
Skin biopsy - Bleeding is controlled by medication,
electrodesiccation; large incisions are closed by sutures
Types
1) punch biopsy: a tubular punch (4 mm diameter) is inserted
into deep dermal or subcutaneous tissue to obtain a specimen
2) skin shaving: superficial lesions are biopsied using a skin
scalpel or razor blade
Tzanck testing - Used to diagnose viral infection, e.g., herpes Performed when active intact vesicles are present
simplex and herpes zoster (but does not differentiate
Uses a scraping from the vesicle, which is transferred to a
between the two)
slide, stained, and examined microscopically
Wood's light (black light) - Used to distinguish
hypopigmentation from depigmentation
Fluorescence color changes seen under the black light:
1) erythrasma fluoresces bright orange-red
2) tinea capitis (caused by microsporum canis and
microsporum audouinii) fluoresces a light, bright green
3) pseudomonas infection may be green fluorescence
Wounds
Assessment
• acute or chronic: etiology of pressure, shearing, trauma
• measurements: depth, size, location, "tunneling", open, closed
• staging: partial or full thickness, complex (involving muscle, bone)
Factors impacting wound healing
• perfusion, oxygenation, nutritional deficiencies, infection, underlying systemic condition, topical
products utilized, medications
Principles of successful wound management
• remove or eliminate causative factors, provide appropriate systemic support: fluids, nutrition,
apply appropriate topical products: encourage moist wound healing and process of occlusion
Evaluation of successful wound healing
• type: primary, secondary or tertiary wound closure
• measurement: decreasing size, viable tissue, decreased exudate, color
Modes of caring for skin disorders
• non-surgical: skin care products, nutrition, healthy lifestyle
• surgical: debridement, skin grafts and flaps
Noninfectious Skin Conditions
Atopic dermatitis (eczema): inflammation of the skin
etiology: genetic tendency, multifactorial with family history of allergies or asthma; exacerbated by stress and certain
foods
pathophysiology: trigger- increased histamine release with inflammatory response, e.g., itching and findings of
exacerbation
Findings: dry skin, itching, erythema, macule, papule, pustule and even vesicles possible, acute weeping areas with
excoriated red plaques
Diagnostics: family history, physical exam, laboratory tests: eosinophils; immunoglobulin E (IgE )
management: control itching, moisturize, remove irritants and allergens, and prevent secondary problems (infections)
products: wet compresses, occlusive dressings, creams/ointments, hydrocortisone cream, mild detergents
pharmacologic: antihistamines
nursing interventions: remove allergens, keep child's fingernails short, clothe lightly to decrease
sweating, apply emollient preparation immediately after bathing, provide rest periods,
provide hypoallergenic diet if appropriate, teaching points; provide family support
regarding nutrition and recurrent nature of this disorder
Seborrheic dermatitis (called "cradle cap" in infants)
– definition: recurrent inflammatory reaction of skin that
may also involve the eyelids and external ear canal
– etiology: probable dysfunction of sebaceous glands
– findings: usually non-pruritic oily scales on scalp,
forehead and eyebrows or behind ears
– management: scalp hygiene with mild baby shampoos,
hydration creams, baby oil
– teaching points: teach parents how to clean scalp and shampoo hair
Head Lice - Head lice infect the scalp and hair and can be
seen at the nape of the neck and over the ears. Head lice
spread easily and quickly but do not carry disease as other
lice do
Diaper dermatitis ("diaper rash")
– definition: inflammatory skin disorder caused directly or indirectly by wearing of diapers
– etiology
• ammonia in urine, fecal enzymes, detergents, moisture, heat
• can lead to secondary fungal infection (especially candida albicans)
– pathophysiology
• prolonged and repetitive contact with irritant (especially urine ammonia, which is
formed by urea breakdown from fecal bacteria); inflammation
• excoriation, with macules or papules and erosion
• concern: secondary infection.
– findings: red, excoriated macules and papules; maceration
– management
• cleaning and frequent diaper changes
• skin protectants and moisture barriers to keep skin dry
• topical antifungals and steroids
• if diarrhea, treat underlying cause
• Contact dermatitis
– definition: inflammatory reaction of the skin to chemical substances natural or
synthetic
– etiology: multiple factors and irritants foods, solutions, allergens, plants
– findings: irritant and allergic types
• irritant: causes dry, inflamed, pruritic lesions where irritant touched
• allergic: blisters after weeping, pruritic, lesions
– management
• topical applications: anti-inflammatory and antipruritic
• cold compresses
• supportive care, i.e., prevent further exposure to offending substance if possible
• Acne vulgaris
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definition: most common skin problem treated by doctors during adolescence
etiology: multifactorial (heredity, hormones, emotions)
pathophysiology
• puberty: increased androgens; involves hair follicle and sebaceous gland complex
• increased sebaceous glands secrete more sebum
• pores become plugged and dilated
• fatty acids are oxidized on skin and form blackheads or
• internal fatty acids form whiteheads
• rupture causes local inflammation, sometimes with pustules
findings: whiteheads (closed comedones), blackheads (open comedones), papules, pustules, nodules, red
and excoriated skin
management
• general: good cleansing of skin, nutrition, and no squeezing or picking at lesions
• pharmacologic
– topical applications: vitamin A, topical antibiotics, topical benzoyl peroxide
– oral antibiotics
– possible estrogen, e.g., birth control, for girls
teaching points
• possibility of scarring
• possible bacterial invasions
• body image
• education needed on myths and realities of causative agents and t
reatment modalities
• stress importance of compliance to treatment program
• Infectious Disorders
– Impetigo contagiosa
• definition: bacterial infection of skin
• etiology: staphylococcus aureus, group A beta-hemolytic streptococcus
• pathophysiology: starts in area of broken skin - highly contagious for seven to ten days
• findings
– primary: pustules or vesicles
– secondary: honey-colored crusts, superficial erosion, easily bleeds, pruritic
– tends to heal without scarring unless secondary infection
• management
– careful removal of crusts or debris with warm soapy solution
– short fingernails
– pharmacologic
» topical antibiotics for early small lesions
» systemic antibiotics - treatment of choice
• concerns: highly contagious, therefore good hand washing; no sharing towels or eating
utensils
• Cellulitis
– definition: infection of dermis and/or subcutaneous tissue
– etiology: streptococcal bacteria, e.g., Streptococcus group A or Streptococcus pyogenes;
Staphylococcus aureus; Haemophilus influenza
– pathophysiology
• starts in area of broken skin
• highly contagious for days
• can occur on any part of the body
– findings
• affected area: red, edematous, tender, occasional discoloration
• enlarged lymph nodes; fever, malaise, headache; "streaking" frequently seen
– diagnostics
• CBC and blood cultures
• possible skin culture
– management
• oral or parenteral antibiotics
• warm moist compresses
• possible incision and drainage
• monitor size of area by marking and dating red area
– concerns: secondary infection, alteration in skin integrity
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Temperature-Related Disorders
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Causedby cold
• Definitions
– chilblain: skin (usually extremities) becomes red and swollen when it is intermittently exposed to
cold temperatures
– frostbite: tissue damage when ice crystals form in tissue
– hypothermia: cooling of the body's core temperature to injurious levels (below 95 degrees
Fahrenheit or 35 degrees Celsius)
Findings
– chilblain: redness, edema of hands and feet, may burn or itch
– frostbite
» blanching, decreased sensation, mottled
» second degree (cold after rewarming): blisters and possible bulla
» third degree: cyanosis, mottling- then red with swelling, local necrosis, hemorrhagic
vesicles
» fourth degree: complete necrosis, gangrene, loss of body part
– hypothermia: cooling of the body's core temperature to injurious levels (below 35 degrees
Celsius)
Management for frostbite
• cover area immediately but no massaging
• rewarm in 100 to 108 degree Fahrenheit water - immerse affected part
• give analgesics and sedatives for severe pain during rewarming
• possible surgery - escharotomy
hypothermia - 3 categories of rewarming
• for mild hypothermia - passive external rewarming (PER): client is placed
in warm environment and covered with insulation
• active external rewarming: heat is applied to skin over trunk of body
only
• active core rewarming (most effective way to rapidly increase core
temperature)
• warmed, humidified air
• peritoneal dialysis
• heated irrigation
• extracorporeal - most rapid means of rewarming
• Sunburn
– Definition: dermatitis due to overexposure to the sun
– Etiology: overexposure to ultraviolet light waves UVA (minor burning) UVB (tanning, burning,
harmful effects)
– Prevention
• avoid sun
• wear good clothing with a tight weave and eye wear
• apply child appropriate topical sunscreens and blocks liberally
• no direct sun between ten am and three pm
– Management: like a first degree burn
Thermal injuries (burns)
– Definition: injuries to skin resulting from extreme heat sources
• partial thickness: first and second degree burns
• full thickness: third and fourth degree burns
– Etiologies: thermal (most common in children), chemical, electrical, secondary to irradiation
• hot water scalding more common in toddlers
• flame-related burns more common in older children
– Pathophysiology
• severity: determined by the amount of systemic disruption beyond what the body could
normally compensate
– minor: less than 10% total body surface area
– moderate: 10-20% total body surface area
– uncomplicated major: more than 20% total body surface area
• severity affected by: location, concurrent inhalation injury, trauma, underlying condition,
age of child
• anatomy and physiology of burns
– depth
» amount of local tissue destroyed or damaged
» related to length of exposure and temperature
– third degree: involves muscle, fascia and bone; wound appears
dull and dry and ligaments, tendons and bone may be exposed
Findings
– superficial (first degree) burn: localized pain, dry surface, blanches with pressure, redness,
possible blister
– partial thickness (second degree) burn: open wound, very painful, denuded skin; blistered, moist
– full thickness (third degree) burn
• tough, leathery, dull dry, with variable pain (often severe)
• color: brown/tan/black/red: dependent on severity
• may be life threatening; may affect many body systems
– full thickness (fourth degree) burn
• wound dull and dry
• ligaments, tendons, bone may be exposed - not painful
Burn injuries differ in children
– thinner skin, so tissue damage is more severe
– fluid volume changes faster than cardiovascular system can respond
– relatively large surface area increases risk for losses of fluid and heat
– increased risk for dehydration and acidosis due to: diarrhea, insensible fluid loss, and
because child's body requires higher proportion of water than adult's
– immature immune system increases risk of infection
– long term: scars mature more slowly and keloids (see image below) may develop
extent of burn determines responses: local or systemic
local response
• cellular damage and fluid movements - edema
• fluid leaks into interstitial spaces - fluid lost to air
• fluid is lost to circulating volume - the "oliguric" phase
• burn damages tissue
multi-systemic response and potential complications
• cardiovascular: dehydration; "burn shock"
• pulmonary: respiratory distress, possible post-inhalation injury, adult respiratory
distress syndrome (ARDS), aspiration pneumonia, pulmonary edema
• GI: ischemia - decreased bowel sounds, possible ileus, Curling's ulcer
• GU: decreased fluids, increasing BUN and creatinine
• metabolic: increased basal metabolic rate, vital signs
• neuroendocrine: increased ADH and aldosterone
• CNS: possible encephalopathy, seizures, coma, altered LOC
• integumentary: burned, infection, scar tissue formation and poor healing
• anemia: associated with major burns
NURSING DIAGNOSIS
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HIGH RISK FOR IMPAIRED SKIN INTEGRITY
PAIN/ITCHING
SLEEP PATTERN DISTURBANCE
BODY IMAGE DISTURBANCE
KNOWLEDGE DEFICIT
Pediatric Hematology
Blood Components
Erythrocytes: RBC- carry oxygen to cells and move carbon dioxide back to lungs
average life span: 120 days
hemoglobin: the iron component of RBCs
blood typing :
persons with type A can receive type A or type O blood
persons with type B can receive type B or type O blood
persons with type O can receive only type O blood
Leukocytes increase with infections or inflammation:
Plasma proteins: include albumin and globulins
- measuring plasma proteins helps assess
nutritional status
Chromosome Composition
• Autosomes
– 22 pairs of chromosomes alike in males and
females
• Sex chromosomes
– Make up the 23rd pair, determining sex of person
• All males have an X and Y
• All females have two X chromosomes
Disorders of Single-Gene Inheritance (Mendelian)
• Autosomal Dominant
– A single mutant allele from an affected parent is
transmitted to an offspring regardless of sex
• Autosomal Recessive
– Manifested only when both members of the gene
pair are affected (both parents unaffected, but
carriers)
• X-Linked Recessive
– Always associated with the X chromosome;
inheritance pattern is predominately recessive
Genetic Traits
• Recessive Inheritance
Both parents carry a normal gene (N), and a faulty recessive gene (n).
The parents, although carriers are unaffected by the faulty gene.
Their offspring are affected (25%), not affected (25%), or carriers
(50%). Ex: Sickle cell, cystic fibrosis, B-Thalasemia, PKU
• Dominant Inheritance
One parent has a single, faulty dominant gene (D), which overpowers
its normal counterpart (d), affecting that parent. When the affected
parent mates with an unaffected and non-carrier mate (dd), the
offspring are either affected(50%) or not affected(50%), but they are
not carriers. Ex: Von Will brand
• Carrier
– A person who is heterozygous for a recessive trait and does not
manifest the trait
X-Linked Dominant
• An affected male has an affected mother because males
inherit their X chromosome from mothers
• Some affected females may be less severely affected
than males because of X-inactivation
• There may be an excess of female offspring in the family,
as some X-linked dominant genes are lethal in the male
• Male to male transmission is not seen. Males transmit
their X chromosome only to their daughters.
Disorders of Red Blood Cells
Sickle cell anemia (SCA): one of a group of diseases in which normal adult
hemoglobin A (HbA) is partly or completely replaced by abnormal sickle hemoglobin
(HbS)
etiology: autosomal recessive disease; 1 in 2 African-Americans carries the trait, while
1 in 375 is affected with sickle cell disease
pathophysiology
trigger - RBC's shape becomes sickled and clump
generalized microvascular occlusion (vaso-occlusive crisis)
Sickle cell anemia (SCA):
Findings: result from obstruction caused by sickled RBCs, RBC destruction, hypoxia
organ dysfunction (spleen, liver, kidney) due to ischemia and infarction
painful exacerbations called crises:
vaso-occlusive - painful distal ischemic usually hands and feet
sequestration crisis - pooling of blood in liver and spleen
aplastic crisis - diminished RBC production
hyperhemolytic crisis increased destruction of RBC
diagnostics
newborn screening
electrophoresis (hemoglobin)
Sickle cell anemia (SCA):
therapeutic management aims at therapy to prevent sickling phenomenon
hydration
oxygen
analgesics
antibiotics (prophylaxis with penicillin recommended)
folic acid
genetic counseling
nursing interventions
formulate teaching plan for patient and family
encourage fluids
apply to affected areas as ordered
accurate I & O
medicate for discomfort as ordered
teaching points
how to administer prophylactic antibiotics
findings of infection
how to increase fluid intake especially in hot weather
Specific Test
Activated partial
thromboplastin time (PTT or
aPTT)
Nursing Considerations
Used to:
1) investigate a bleeding or thrombotic episode
2) monitor unfractionated (standard) heparin anticoagulant therapy
Blood draw
Basic metabolic panel (BMP or Standard BMP includes (8 specific tests): glucose, calcium, electrolytes (sodium potassium,
chemistry panel)
carbon dioxide, chloride, bicarbonate), kidney tests (blood urea nitrogen [BUN]
& creatinine)
Blood draw - may be collected on a random basis or drawn after fasting 10 to 12 hours
prior to the blood draw
When more information is needed, a complete metabolic panel (CMP) may be ordered
Blood smear (Red blood cell
Microscopic examination of red blood cell irregularities, including size, shape and color
morphology)
Blood draw; can also use finger or ear prick; heel prick with infant - no prep required
Complete blood count (CBC) Typically there are no specific pre-testing restrictions, although the client should avoid
eating a fatty meal prior to the test
Coagulation factors
D-dimer
Ferritin
Standard CBC includes:
1) number of white blood cells (WBC)
2) number of red blood cells (RBC)
3) total amount of hemoglobin content (Hgb)
4) hematocrit (Hct) - fraction of the blood composed of RBCs
5) mean corpuscular volume (MCV) - the size of RBCs
6) mean corpuscular hemoglobin (MCH)
7) platelet count and volume (note: decreased platelet numbers increase bleeding
tendency)
Tests to measure the function of specific coagulation factors; evaluate bleeding episodes
Blood draw - no specific prep
To help diagnose or rule out thrombotic diseases and conditions
Blood draw - no specific prep
Used to determine the body's total iron storage capacity
Blood draw - client should be NPO 12 hours before the test; water is permitted
B-Thalassemia
definition: inherited blood disorder characterized by deficiencies in rate of production
of specific globin chains in hemoglobin
etiology: autosomal recessive disorder, also known as Cooley's anemia
pathophysiology: abnormal, chronic production and destruction of RBC's resulting in
insufficient amounts of normal circulating hemoglobin
findings
severe anemia, pallor
microcytic RBCs
impaired growth
splenomegaly
diagnostics
hemoglobin and hematocrit
hemoglobin electrophoresis
x-rays of involved bones
management
chronic transfusion therapy to maintain hemoglobin of 10 gm/dL
referral for chelation counseling
genetic counseling
Bleeding disorders
Idiopathic thrombocytopenic purpura (ITP)
definition: an acquired hemorrhagic disease
etiology: unknown but often occurs one to two weeks after a febrile viral illness
Pathophysiology, auto-immune disorder, platelets are killed and fewer are made
may be acute and self-limiting or chronic
Findings: excessive bruising, petechiae, internal bleeding
diagnostics
laboratory: platelet count, bleeding time, bone marrow aspiration
management
pharmacologic
corticosteroids
intravenous immunoglobulins (IVIG)
immunosuppression
immunoabsorption apheresis (to filter antibodies from bloodstream)
splenectomy for chronic disease
Nursing interventions
monitor for bleeding episodes
provide for age appropriate diversional activities
teaching points
should not participate in contact sports
should not use aspirin; use acetaminophen (Tylenol) to relieve pain
Von Willebrand's Disease
definition: hereditary bleeding disorder
etiology: congenital (autosomal dominant) bleeding disorder; affects both males
and females
Pathophysiology: body makes too little Von Willebrand's factor and factor VIII
prolonged bleeding time
findings: excessive bleeding of mucous membranes: gums, epistaxis (nosebleed),
menorrhagia
diagnostics: bone marrow aspiration
management: replacement of missing clotting factor and/or administration of
antidiuretic, e.g., desmopressin acetate (DDAVP)
Aplastic anemia
definition: bone marrow failure
Etiology: congenital, e.g., Fanconi syndrome
acquired due to exposure to overwhelming infection, e.g., hepatitis, HPV
causative agents such as antineoplastic agents, chemicals, chloramphenicol
Pathophysiology: bone marrow stops making erythrocytes, leukocytes, and
platelets (thrombocytes)
result: pancytopenia
findings: petechiae, bruising, pallor, fatigue, myelosuppression
diagnostics: bone marrow aspiration
therapeutic management
anti-thymocyte globulin (ATG), anti-lymphocyte globulin (ALG)
if severe, bone marrow transplant is treatment of choice
Hemophilia
definition: group of bleeding disorders in which there is a deficiency of
one of the factors necessary for coagulation of blood
etiology: x-linked recessive disorder
pathophysiology: missing or defective factor VIII or factor IX blood components necessary for
blood coagulation
Findings: mild-severe prolonged bleeding; most often in muscles and joints (hemathrosis),
longterm loss of range of motion of affected joints
Diagnostics: history of bleeding episodes, partial thromboplastin time (PTT)
assay procedures for specific factor deficiencies
Management: replacement of missing clotting factor, factor VIII concentrate, desmopressin
acetate (DDAVP) - an anitdiuretic that aids blood clotting, prophylactic treatment with clotting
factor before surgery and some other procedures, pressure to bleeding site, and ice, rest,
elevation, immobilization
nursing interventions: prevent bleeding with appropriate exercise, brushing teeth with soft
toothbrush, recognize and control bleeding, support family, assess school activities
teaching points: child should wear MedicAlert® identification, genetic counseling for parents
Disseminated intravascular coagulation (DIC)
definition: disorder of coagulation
Etiology: secondary disorder of coagulation that complicates other disorders
itriggered by endothelial damage such as trauma, shock, infections, hypoxia, liver disease
Pathophysiology: the first stage of the coagulation process is abnormally stimulated, clotting
mechanism is triggered in circulation, thrombin is generated in greater amounts than the body
can neutralize, rapid conversion of fibrinogen to fibrin with aggregation and destruction of
platelets, local and widespread fibrin deposition in blood vessels causes obstruction and
necrosis, fibrinolytic mechanism causes extensive destruction of clotting factors
Findings: bleeding, bruising, petechiae, altered serum levels of clotting-related factors
(increased PT, PTT, TT, decreased platelets, degraded fibrinogen), clotting, hypoxemia,
intracranial hemorrhage, progressive organ failure, diagnostics: prothrombin time (PT), partial
thromboplastin time (PTT) and thrombin time, platelets, fibrin degradation products, fibrinogen
Management: control of underlying etiology, factor replacement, platelets, fresh frozen
plasma (FFP) and RBC transfusions, vitamin K, oxygen
nursing Interventions: monitor IV infusions, blood transfusions, administer Heparin as
ordered, monitor any severely ill child to recognize presence of this disorder