Neuromuscular/MSK Flashcards
Fractures
Rare in children and warrant an investigation to make sure it isn’t the result of abuse
Most common broken bone
Distal forearm
Most often from sports injuries
Common causes of fractures
Increase mobility and desire for freedom but immature motor skills
Trauma or abuse
Bone density
Birth injury (clavicle injury and sometimes that is intentionally done by MD to deliver an abnormally large baby or deliver rapidly one that is in distress)
Types of fractures: Simple or closed
Does not produce a break in the skin
Types of fractures: Open or compound
Fractured bone protrudes through the skin
Types of fractures: Complicated
Bone fragments have damaged other organs or tissues
Types of fractures: Comminuted
Small fragments of bone are broken from fractured shaft and lie in surrounding tissue
Types of fractures: Greenstick
When a bone isn’t broken all the way through, like a twig that you snap and breaks on one end but only bends on the other
Types of fractures: Spiral
Most often occurs in toddlers
Types of fractures: Transverse
break straight through
Types of fractures: Compound
Bones stick through skin
Growth plate (Epiphyseal plate) injury
Weakest point of long bone (cartilage)
Each long bone has 2 growth plates: at each end
Frequent site for damage during trauma. The injury may affect the future bone growth
Growth plate fracture treatment
May include open reduction and internal fixation to prevent growth disturbances
Not always seen on an xray so must get pedi ortho to evaluate
Growth plate Salter Harris classification system
Used to describe the degree of fracture I: transverse II: through and above the plate III: fracture below the plate IV: fracture through metaphysis and epiphysis V: crushing of physis
Fractures: Diagnostic
Radiography (only true way to diagnose) History taking (how it occured)
Fractures: Suspicion of fracture
In young child who refuses to walk or bear weight
Should be evaluated especially if child has specific point of injury, is specific about where and how it happen, and if they hear a crackling sound and if they have the inability to bear weight
Fractures: Manifestation
Pain Tenderness at site (could also be sprain/strain) Decreased ROM Immobility Deformity of extremity Edema Crepitus Ecchymosis Inability to bear weight Muscle spasm
Fractures: Goals
Reduction and immobilization
Restoring function
Preventing deformity
Fractures: Reduction
Repositioning of bones into normal alignment
Reduction can be done open or closed
Fractures: Reduction: Opened
Means needs an invasive procedure or surgery to realign the bone
Fractures: Reduction: Closed
Realigned bone without using an invasive procedure, although the setting of bone may occur w/anesthesia due to pain
Fractures: Retention or immobilization
The application of a device or mechanism to maintain the alignment of the bone until healing occurs, it can be with a cast, traction, plate, pin, or a combination of those
Fractures: Assessment
6Ps Pain Pallor Pulselessness Paresthesia Paralysis Pressure
Fractures: Casts application
Retention can be done through casting or traction
Give choices of color so the child can feel some sense of control
Explain procedure
When applying: provide distraction by talking about hobbies, pets…
The cast being applied reassures them its ok because they feel warmth from the application
Ensure proper padding of all boney prominences
Apply a stocking net before casting to protect the skin from any sharp edges after its dry
Fractures: Casts: Care management
Let the cast dry completely
Dry from the inside to out before putting any pressure on it
Use a fan or a cool air hair dryer may be helpful to get cast dry if it is very humid
Always use the palms of hands while its wet, using fingers leaves indentations which cause pressure points
Fractures: Cast at home care
Provide instructions on how to take care of cast. Keep the extremity elevated for the first 24hr to prevent swelling, observe for extremities for swelling and discoloration, checking for movement or sensation
Activity restriction, not allowing the child to put anything down the cast, it might get itchy and kids want to scratch it
Teach about crutch walking
Fractures: Cast: skin care
Teach hygiene because its important as well as how to pay special attention to skin care
Maintain child’s skin integrity while they are in casts
Fractures: Cast: assessment
Unusual odor beneath the cast
Tingling, buring, numbness of toes or fingers
Drainage through cast to toes or fingers
Swelling or inability to move toes or fingers
Toes/fingers that are cold, blue, or white
Sudden unexplained fever
Pain that is not relieved by comfort measures
Unusual pain or nre pain that the child begins tp experience
Neurovascular assessment (CSM)
Fractures: Cast Care teaching
Petaling the edges of the cast (Especially when its a spica cast. Apply transparent dressing to edge of the cast and another one to childs perineum that acts as a continuous water proof bridge between the cast and the perineum in order to prevent leakage and allows for observation of the skin and area beneath the dressing)
Drying of the cast
Prevention of swelling (elevate, or if elbow: teach how to properly use a sling to provide support to the arm)
Protecting the cast from damage (cover before bathing or swimming)
When to call the dr
Spica cast
Used to immobilize the hip and thighs so that bones and tendons can heal properly
Starts at the chest and can either extend down one or both legs
Bar is a hip abductor to keep the legs in the proper position
Be sure not to use bar as a handle
Opening at the middle of the cast allows for elimination
Pose specific challenges like eating, feeding, positioning, bathing issues
Use disposable diapers
Potty trained children can use bedpan
Breastfeeding: use a football home
Traction for fracture
Pull or force that exerted on one part of the body
Balanced skeletal traction is applied to: Allow physiological stability, Align bone fragments, Enable closer evaluation of the injured site
Newer technology: orthopedic fixation devices that allow for mobility
Purpose of traction
Relieve fatigue in involved muscle Position distal and proximal bone ends Immobilize fracture site Prevent deformity Immobilize healing bone and prevent further injury Reduce muscle spasms
Types of traction: Skeletal traction
Hardware is fixated to bone
Types of traction: Skin traction
Applying dressing to skin and traction is applied through dressing
Types of traction
Upper extremity Lower extremity Balance suspension Gardner-Wells tong Cervical (halo brace)
Types of traction: Lower extremity
Buck extension (Used for comfort measures for hip fractures) Bryant traction (Developmental dysplasia of hip, when pelvic harness isnt working to keep the hips in place, used for 2-3 wks prior to surgery to loosen muscle around the hip joint) Russell traction
Types of traction: Cervical
Used for about 6-8 wks before spinal instrumentation with scoliosis. Used for issues other than scoliosis
Corrects curvature greater than 80%, can be lessened to 50-60%
Continuous traditions
Children can ambulate despite having screws in their head
Traction care
Purpose: maintain bone alignment, prevent muscle spasms
Assessment of neurovascular status: don’t have any coldness, cyanosis, pulselessness
Maintain correct balance between traction pull and counter-action force
Care for weights: NEVER adjust without orders
Skin inspection
Pin care
Developmental concerns
Immobilization affect on development: Toddlers
Need to explore and the ability to imitate behaviors in order to develop a sense of autonomy
Help this by giving them different items of different textures to explore and have them classify them
Anything to promote sense of exploration so that they can work to develop sense of autonomy
Immobilization affect on development: Preschoolers
Expression of initiative is evidenced by their need for vigorous physical activity. This is very hard for them to do when they are in traction. So whatever they can do with their unaffected extremities is good
Immobilization affect on development: School age
Sense of industry is influenced by physical achievement and competition. Maybe have competition with other children on their unit or with parents or board games. Or any game they can participate in that has simple rules
Immobilization affect on development: Adolescent
Rely on mobility to achieve independence, one of the steps in creating their identity. Give them as much independence as you can.
Pneumonic for traction care
T: Temp R: Ropes hang freely on the traction A: Alignment C: Circulation (6Ps) T: Type and location of fraction I: Increase fluid intake so they don't get dehydrated or constipation O: Overhead trapeze is there to help position them for comfort N: No weights ever on the floor or bed
Compartment syndrom
Very serious!!!! Medical emergency!!!!!! 6Ps Needs immediate intervention Fasciotomy is performed when it occurs Elevate extremity to the level of the heart
Developmental dysplasia of the Hip (DDH)
Refers to spectrum of disorders related to abnormal development of hip during fetal life, infancy, or childhood
Defined as condition where head of femur is improperly seated in the acetabulum of the pelvis
Girls are more commonly affected
Most are breech position
Strong family history
Cause is not completely known or understood
Developmental dysplasia of the Hip (DDH): Degrees: 1. Acetabular dysplasia (preluxation)
Mildest form
Delay in development of acetabulum defied by inadequate development of babies acetabulum.
Acetabulum is shallow and disk shaped rather than cup shape, osseous hypoplasia (obliquely inclined outward) of acetabular roof rather than normal horizontal orientation. Femoral head remains in the acetabulum
Developmental dysplasia of the Hip (DDH): Degrees: 2. Subluxation
Most common
Incomplete dislocation of hip, not complete dislocation, femoral head is in contact with acetabulum but it’s partially displaced
Developmental dysplasia of the Hip (DDH): Degrees: 3. Dislocation
Femoral head loses contact with acetabulum and is displaced posteriorly and superiorly over the rim so femoral head actually slides completely out of socket
Ligaments are elongated and taut
Developmental dysplasia of the Hip (DDH): Clinical manifestations: Infant
Newborn: appears in a lax hip joint rather than an outright dislocation
Hip joint laxity
Shortened limb on affected side
Restricted abduction of hip on affect side
Unequal gluteal folds when infant prone
Positive Ortolani test
Positive Barlow test
Ortolani test
Used to confirm Barlow findings
Thighs are abducted to test of subluxation and dislocation
Barlow test
Performed by adducting the hip bringing thigh to midline while applying light pressure on the knee and directing force posteriorly. Hip dislocates easily with this maneuver, then the test is considered positive
DDH: Early intervention
Longer the delay in treatment the more severe, more difficult treatment, and less favorable the prognosis is
Goal is to obtain and maintain a stable hip joint and promote normal hip joint development
DDH: Birth - 6 mo
Pavlik harness: maintaining abduction and flexion of the hip for about 3-5 months
Straps are checked and adjusted Q1-2 weeks due to rapid growth
Pavlik harness
there is one on each leg placed so that legs are held in important position to keep it stabilized during course of treatment
DDH: 6-24 mo
Dislocation unrecognized until child begins to stand and walk
Treatment: Closed reduction under general anesthesia, then placed in a hip spica cast for 2-4mo
If hip remains unstable: open reduction is performed
DDH: Older children
Operative reduction, include preoperative traction, or the performance of a tenotomy (surgical division of tendon), and possibly a hip osteotomy (surgical procedure where bones of hip joint are cit and reoriented and fixed back into a new position)
In older children, may have to perform more dramatic procedures to construct the acetabular roof. Correction after 4 yr is very difficult, not advised after 6yr
DDH: Nursing care
Teach parents to remove and apply harness appropriately
Only remove to check skin and bathing
Teach skin assessments, always put a shirt or onesie underneath the harness chest straps and put some type of long socks preferably up to the knees to prevent straps from rubbing against skin
Have parents check 2-3 x/day for any red areas under the straps
Provide gentle massage underneath the straps to stimulate circulation
Avoid any lotions, creams, ointments, or powder under the straps
Place diaper under the straps
Encourage cuddling to promote cognitive development and infant/caregiver bonding
Talipes equinovarus (clubbed foot)
Congenital abnormality in which the foot and ankle is twisted out of its normal position
Boys affected more than girls
Unknown cause
Talipes equinovarus (clubbed foot): Diagnostic evaluation
Deformity is apparent and detect prenatally through US or at birth
Talipes equinovarus: Described according to position of the ankle and foot
Talipes varus: inversion or bending inward (most common)
Talipes valgus: eversion or bending outward
Talipes equinus: plantar flexion with toes lower than the heel
Talipes calcaneus: dorsiflexion with toes higher than the heel
Most cases are a combination
Most commonly occuring being the composite deformity talipes equinovarus: foot pointed downward and inward in varying degrees of severity
Talipes equinovarus: Categories: Positional
Mild or postural and may correct spontaneously or necessitate some passive exercising or possibly serial casting. No bony abnormality but there could be tightening or shortening of soft tissue
Talipes equinovarus: Categories: Syndrome or teratologic
Often associated with other congenital abnormalities, usually necessitates surgical intervention, and with this there is a higher incidence of recurrence for these kids
Talipes equinovarus: Categories: Congenital
Its usually idiopathic, there is bony abnormality involvement and requires surgical procedure
Talipes equinovarus: Goal of care
Stretch the tightened tendons and ligaments gently and return foot to maximal anatomical position
Talipes equinovarus: Treatment stages
Correction of deformity
Maintenance of the correction until normal muscle balance is regained, and normal position is then achieved
Follow-up observation to avert possible recurrence
Talipes equinovarus: Serial casting/Ponseti method
Series of corrective casting and or use of splints, could even require surgical correction and series of cast to gently guide that clubfoot into position
Applies to hold foot in desired position
Begun right after birth, weekly manipulation and serial long leg cast to allow gradual repositioning
Extremities are casted and changed every 1-2 weeks until maximum correction is achieved usually 6-10wks
Talipes equinovarus: Casting: Nursing care
Cast care
Skin care
Education
Parental support
Talipes equinovarus: Casting: eduction
Parents need to understand the overall treatment plan, importance of regular cast changes and that they understand their role in overall effectiveness of therapy
Reinforce and provide instructions of cast care and skin care. Make sure parents know how to assess for any problems. They need to encourage normal G&D
Talipes equinovarus: Parental support
Parents understand their role
Active participation in the physical therapy treatments and child’s strength program
Help the parents understand the time commitment involved in caring for these kids
Very large commitment that parents have to follow through with physical therapy and doing it on consistent basis as well as strengthening program
Assess parents ability to monitor the child adequately for complications and confirm they understand the signs and symptoms of the complications that they might need to call the provider
Osteogenesis imperfecta
“brittle bone disease”
A group of heterogeneous inherited disorders of connective tissue. Parents often have a family history but most cases are generally due to new mutation
Defective periosteal bone formation and reduced cortical thickness of bones
Hyperextensibility of ligaments
Osteogenesis imperfecta: Characterized by
Excessive fragility/fracture and defects of bones/bone deformity
Osteogenesis imperfecta: Type I
Procollagen: Mutation on the genes that affect type 1 collagen, results in bone mineralization, abnormal construction of bone, and susceptibility of fractures. Most common mild bone fragility and may see kids have blue tinged to sclera, teeth usually look normal and may have some mild hearing loss
Osteogenesis imperfecta: Type II
Most lethal, results often in stillbirth or death in early infancy, it is a severe bone fragility with multiple fractures that may occur at birth, autosomal recessive inheritance
Osteogenesis imperfecta: Type III
Severe bone fragility (most cases), leading to severe progressive bone deformity, might have normal sclera, but will see very marked growth failure. Most autosomal recessive inheritance, some may be autosomal dominant
Osteogenesis imperfecta: Type IV: A
Mild to moderate bone fragility, normal sclera, short stature, variable deformities, autosomal dominant inheritance
Osteogenesis imperfecta: Type IV: B
Same as A but with abnormal dentition/teeth called dentinogenesis imperfecta
Represent about 6% of cases
Osteogenesis imperfecta: Type V
Similar to type IV may have hyperplastic callus and negative collagen mutation
Osteogenesis imperfecta: Type VI
Moderate to severe bone fragility
Osteogenesis imperfecta: Type VII & VIII
Osteochondroplasia and short stature
Osteogenesis imperfecta: Therapeutic management
Primary supportive care
IV bisphosphonate therapy, may need Calcium, GH.
No matter what fractures will occur
Children who get fractures from non-traumatic accidents need to be screen
Rehabilitative approach: (positional contractures and deformities, muscle weakness and osteoporosis, misalignment of lower extremity joints)
Osteogenesis imperfecta: Nursing implications
Careful handling
Changing diaper or tying a tourniquet can cause a fracture
Support when turning, positioning, and moving them
Educate parents on limitations and planning suitable activities to promote optimum development and to protect these kids from harm
Osteogenesis imperfecta: Clinical features
Weak bones, susceptible to fractures Scoliosis Kyphosis Blue tint to sclera (type 1) Early hearing loss (type 1) Hypermobility in joints (all) Hypoplastic discolored teeth (type IV B)
Scoliosis
Most common spinal deformity
Abnormal curvature of the spine
May be congenital or develop during childhood, many potential causes, most are idiopathic
Becomes noticeable after per-adolescent growth spurt, clothes may be ill fitting
Hips are slightly higher than the other
School screening, need to catch as soon as possible
Scoliosis: Causes: Idiopathic
Infantile, childhood/juvenile, adolescents (most common time of diagnosis)
Progresses faster in girls, and will more likely need surgical treatment
Becomes noticeable after pre adolescent growth spurt
Scoliosis: Secondary Cause: Neuro
Tethered cord
Cerebral palsy
Muscular dystrophy
Spinal tumor
Scoliosis: Secondary cause: MSK
DDH
Leg length discrepancy
OI
Scoliosis: Diagnostic
Based on Xray or observation
Assessment performed: standing behind the patient (should be in undergarments)
Asymmetry of shoulder height, scapular or flank shaped shoulders, or hip height and alignment should all be noted
Child bends forward and arms hanging, you’ll see asymmetry
Scoliometer used to determine the angle of trunk rotation
Radiography/Xray used to determine the degree of curvature
Scoliosis: Assessment findings
Trunk asymmetry
Unevenness of shoulders
Prominent scapula or uneven height of scapula
Rib hump
Uneven hips
In addition to primary curvature, compensatory curvature often present that aligns head with gluteal cleft
Scoliosis: Therapeutic management
Team approach (ortho, neuro, PT, nurses, social work)
Children should be observed regularly by provider and receive routine xrays for evaluation
Options are based on the magnitude of curve, location, and type of curve
Bracing or surgical intervention
Sometimes exercise
Scoliosis: Other factors to determine treatment
Age, skeletal maturity, and other underlying conditions
Growth plates closed?
Whats the degree?
Is the child going to grow more?
If growth plates are closed then its sure that the pt wont grow anymore and the curvature wont increase
Scoliosis: Bracing
Not curative, but may shol curvature down in order to allow for skeletal maturity. Compliance is a problem because teens are concerned with appearance so that can make them feel very awkward
Most common braces: Boston, Wilmington, which are customized, pre-fabricated plastic shells.
Used for moderate curvature
Scoliosis: Surgical intervention
Severe curvature (instrumental and fusion)
Greater the 30-40 degree require fusion
Involves realignment with in an internal fixation and bony fusion of the realign spine
Variety of rods that may be used during surgical fusion (most common: Harrington, Luque)
Posterior spinal fusion is best choice for thoracic curves.
Anterior spinal fusion is the best treatment for lumbar curves.
Scoliosis: Exercise
May be prescribed but really rarely of any value in managing scoliosis but can maintain and strengthen abdominal muscles to provide support
Scoliosis: Care management
Concerns of body image is the priority psychoscolcial nursing diagnosis for the adolescent. Explain the importance of the benefits
Scoliosis: Pre-op teaching for spinal fusion
What to expect before and after surgery
Demonstrate how to use incentive spirometer, coughing , and deep breathing
Show them all the equipment that might be used: foley, IVs chest tubes, O2 mask
Teach them about the PCA pump and explain it is for their pain
Demonstrate how to log roll and how to assist them out of bed
Scoliosis: Post-op Care for spinal fusion
Make sure child maintains airway, do neurovascular assessment of lower extremities, log roll every 2 hours depending on orders
Typically they will be out of bed and ambulating at least 4 times a day
Most of the time adolescent so we want to encourage them doing ADL they usually do, start ambulating post op 2-3 days
Scoliosis: Family issues
Provide resources and information on support groups
Family is encouraged to become involved so that they will be comfortable caring for them at home
Osteomyelitis
Infection of the bone that can occur at any age, when in children more frequently at the age of 10 and younger
Boys more than girls
Caused by exogenous or hematogenous sources
Osteomyelitis: Most common causative agent
Staphylococcus aureus
Group B strep in neonates
Osteomyelitis: S/S
Begin abruptly and resemble symptoms of arthritis and leukemia
Starts off with severe pain, fever, irritability, tenderness to the area, limited ROM, swelling over the area, and pain with movement
Osteomyelitis: Diagnostic
Blood cultures to identify organism and abx susceptibility
MUST TREAT THE BACTERIA
Bone cultures obtained from biopsy is rare but will happen if the blood culture is inconclusive
Supportive evidence: marked leukocytosis, elevated ESRT, C-reactive protein
Xray
Bone scan
MRI - most definitive test and most sensitive diagnostic tool
Osteomyelitis: Xray
May appear normal at first, may take up to 2 -3 weeks for signs to become evident
Osteomyelitis: Bone scan
Shows areas where there is increase blood flow which can occur in early stages of infected bone and that can be useful in locating multiple sites
Not diagnostic
Osteomyelitis: Types
Acute hematogenous
Exogenous
Subacute
Chronic
Osteomyelitis: Acute hematogenous
Blood borne infection that goes on to cause infection in bone
Osteomyelitis: Exogenous
Acquired directly from puncture wound, open fracture, surgical contamination, or from tissue infection from adjacent wound.
Osteomyelitis: Subacute
Longer course
Caused by less virulent microbe that created a walled off type abscess that typically in the tibia
Osteomyelitis: Chronic
Progression of acute Osteomyelitis, characterized by dead bone, bone loss, drainage as well as the development of sinus tracts in the bone
Osteomyelitis: Management
IV abx Education Surgery Pain management Monitoring
Osteomyelitis: Presentation
May be subacute, with walled-off abscess rather than spreading infection
Osteomyelitis: IV abx
#1 priority Prompt, vigorous IV abx for extended period of time (3-4wk up to several months)
Osteomyelitis: Treatment for S. aureus
Nafcillin, clindamycin
Nurse should advocate for a PICC line as soon as Osteomyelitis is diagnosed because of long term abx therapy
Osteomyelitis: Educate parents
IV administration, typically because kids are sent home for home care with PICC lines. Make sure parents know how to set those up and set up flushed. When one dose, have to draw up flushes.
Osteomyelitis: Surgery
Necessary if there is no response tot specific abx, if there is a penetrating injury, or if the infection spreads to the joint
Osteomyelitis: Pain management
Movement can cause severe discomfort. Position in a comfortable position with affected bone or limb supported
No weight bearing
Long term pain meds may be needed
Osteomyelitis: Monitor hematologic
Renal and hepatic responses to treatment
When using abx, need to monitor renal and liver function and see how they are responding
Juvenile idiopathic rheumatoid arthritis (JIA)
Small amount of children have R-factor Chronic autoimmune disease that causes inflammation in the joints and tissues Occurs before the age of 16 Peak onset of 1-3yr Often go undiagnosed Heterogeneous group of diseases
Juvenile idiopathic rheumatoid arthritis: Cause
Systemic arthritis
Oligoarthritic
Polyarthritic
Psoriatic
Juvenile idiopathic rheumatoid arthritis: Systemic arthritis
Involves one or more joints
Associated with at least 2 weeks of high fever, rash, hepatosplenomegaly, pericarditis, pleuritis, serositis (inflammation of serous tissues like the lining of the heart, lungs and abdomen), lymphadenopathy
Juvenile idiopathic rheumatoid arthritis: Oligorarthritic
Involves ≤ 4 joints for the first 6 months
Juvenile idiopathic rheumatoid arthritis: Polyarthritic
Involves ≥5 in the first 6 months with a negative R-factor or a positive R-factor
Juvenile idiopathic rheumatoid arthritis: Psoriatic
Enthesitis and undifferentiated where patients has psoriasis skin condition, nail pitting in their nail bed
Enthesitis (inflammation on the site where tendons and ligaments enter into the bone)
Involves sacroiliac or lumbosacral pain or could also have acute anterior uveitis (inflammation in the interior chamber of the eye)
May also affect patient with inflammatory bowel disease
Juvenile idiopathic rheumatoid arthritis: Clinical manifestations
Rarely life threatening, may cause disability
S/S come and go, and include chronic inflammation of synovium with joint adhesion (increased intra articular joint fluid)
Includes destruction of cartilage as well as stiffness of joints as the disease progresses, especially in the morning or after long periods of inactivity
Swelling
Loss of motion
Areas that are warm to touch, but seldom red
Joints can be tender to touch or may be painless
Juvenile idiopathic rheumatoid arthritis: Diagnostic
No definitive test. Diagnosis of exclusion of other disease processes.
Clinical criteria from American College of Rheumatology: onset before age 16, involves development of arthritis in one or more joints for at least 6wks or more, ESR is elevated or not.
Presence of antinuclear antibodies common, but not specific for JIA
Leukocytosis is frequently present on exacerbation
Juvenile idiopathic rheumatoid arthritis: Management
No specific cure
Primarily outpatient
Juvenile idiopathic rheumatoid arthritis: Goal
Control pain and relieve symptoms
Preserve Function and ROM of joints involved, minimize effects of inflammation such as joint deformities
Promote normal G&D
Juvenile idiopathic rheumatoid arthritis: Pharmacological Treatment
NSAIDS
Corticosteroids
Cytotoxic agent
Biologic agent
Juvenile idiopathic rheumatoid arthritis: NSAIDS
First line Ibuprofen Indomethacin Naproxen Effective and safe for use in children with few SE other than GI upset. Monitor for GI bleeding
Juvenile idiopathic rheumatoid arthritis: Corticosteroids
Given during time of flare up
Always give the smallest dose for the shortest period of time
Juvenile idiopathic rheumatoid arthritis: Cytotoxic agent (Methotrexate)
May be given weekly.
Sulfasalazine, Hydroxychloroquine, used in children when NSAIDS alone have failed
Pt edu about SE, and their responsibility of birth defects and importance of avoiding alcohol
Juvenile idiopathic rheumatoid arthritis: Biologic agent
Slow-acting antirheumatic drugs: Etanercept, Cyclosporin
Used in severe cases
Juvenile idiopathic rheumatoid arthritis: Care management
PT (can be several times a week, using a pool to provide less gravitational pull), OT
Opioids are avoided except for short term use
Promotion of general heath: well balanced diet, adequate calories, adequate rest
Facilitating compliance
Encouraging the use of heat and exercise
Warm bath before therapy can help with comfort and mobility
Caution with hot pack for thermal burns
Support child and family
Physical limitation may interfere with self-care, participation in school and activity and their ability to engage in recreational activities.
Family encouraged to maintain normal activities but also be able to work with and create adaptations for child
Cerebral Palsy
Group of permanent disorders of the development of movement and postures, causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing fetus
Most common permanent physical disability in childhood
Viable causes, most are unknown, babies weighing < 2000g at birth can develop CP
Cerebral Palsy: Manifests as..
A lack of movement or associative movement disorder
Associated with epilepsy, speech problems, issues with vision and cognitive dysfunction
Cerebral Palsy: Prognosis
Dependent on the type and severity
Moderate impairment: 85% will go on to achieve ambulation
30-50% with CP will develop cognitive impairments
Growth can be affected, their survival depends on any comorbid conditions that they may be experiencing
Cerebral Palsy: Characterized by
Abnormal muscle tone and coordination
Cerebral Palsy: Causes
Prenatal brain abnormalities (exact cause is unknown, can be genetic or other prenatal birth abnormalities)
Perinatal ischemic stroke
Perinatal neonatal brain lesions or abnormal birth development
Postnatal factors
Cerebral Palsy: Cause: Other prenatal birth abnormalities
Clotting disorder
Gross abnormalities or malformation of the brain
Vascular occlusion
Effects of low birth weight
Laminar degeneration
Anoxia or hypoxia infarction or hemorrhage
Cerebral Palsy: Causes: Postnatal factors
Bacterial meningitis, multiple births (twins, triplets…) usually from decreased oxygenation baby may experience during birth, child abuse (shaken baby syndrome)
Cerebral Palsy: Types
Dependent on where the brain is damages Spastic Dyskinetic Ataxic Mixed
Cerebral Palsy: Spastic
Most common
Muscles appear stiff and movement are very stiff and jerky
Spasticity is a form of hypertonia or increased muscle tone
Both groups of muscles will turn on at the same time and in some instances the wrong group turns on making movement difficult or impossible (only one specific group should turn on at a time)
Cerebral Palsy: Dyskinetic
Variable movements that are involuntary.
Uncontrolled movements become very noticeable when they try and move
Movements can be a twisting or repetitive movement to the trunk or from extremities called Dystonia
Slowed movements called Athetosis/Athetoid
Dance like jerky irregular unpredictable movement is Athetoid, involuntary jerky slow worm like movements
Muscles involved are extremities, trunk, facial muscles, and tongue
Cerebral Palsy: Ataxic
Wide based gait, rapid repetitive movement performed poorly
Cerebral Palsy: Mixed
Combination of spastic and dyskinetic
Labeled mixed when no specific motor pattern is dominant
Cerebral Palsy: Diagnostics: Infants -6mo
Careful assessment
Good history and physical
Talking to parents, assessing for developmental milestones
Head lag when you pick them up, ask the parents if the baby feels stiff when being picked up or feel floppy, should not feel floppy as they get older
Cradled in arms: does the child overextend the neck or back or feel like they are pushing away from you?
Child is picked up: do their legs get stiff or do they scissor/cross?
Cerebral Palsy: Diagnostic: >6mo
Do they roll over in one direction or the other or both? 🚩if they dont roll over
If they cant bring their hands togther 🚩
Difficulty putting hands in mouth 🚩
Do they reach out only with one hand and the other fisted? 🚩
Cerebral Palsy: Diagnostic: >10 mo
Should be crawling. Crawl in lopsided manner, may push off with one hand and leg and drag with the other🚩
May scoot around on buttocks or hop on knees but cannot coordinate crawling on all 4s 🚩
Cerebral Palsy: Diagnostic: Neurologic exam and history
Primary means of diagnosis
Cerebral Palsy: Diagnostic: Neuroimaging
Used for diagnosis , MRI preferred over CAT scan
Cerebral Palsy: Metabolic and genetic testing
If not able to identify structural abnormalities with MRI
Cerebral Palsy: Clinical manifestations
delayed gross motor development abnormal motor performance alterations in muscle tone abnormal posture reflex abnormalities associated disability poor head control after 3mo stiff rigid arms or legs arching back floppy body posture 8mo cannot sit without support 3mo if hands remain clenched, hands remaining tightly fisted leg scissoring persistent or primitive reflexes seizures
Cerebral Palsy: Behavioral signs
extreme irritability crying feeding difficulties thrusting of tongue after 6mo little interest in surrounding and excessive sleep
Cerebral Palsy: Goals
Early recognition, promotion of optimal development, reach maximum potential
Establish locomotion, communication, self help skills
Achieve optimal appearance and integration of their motor functions
Correct associated defects as effectively as possible
Provide educational opportunities that are adapted to the child’s needs and capabilities
Promote socialization experiences is essential for normal growth and development
Cerebral Palsy: Management
Individual basis Ankle and foot braces Orthopedic surgery Pharmacological Botulinum toxin A injections Dental hygiene Physical/occupation therapy
Cerebral Palsy: Ankle and foot braces
May be worn, used to help reduce deformities or prevent them. Increase their ability to ambulate and control the alignment of these affected muscle groups
Cerebral Palsy: Orthopedic surgery
Correct spastic deformities, including tendon lengthening, releasing os spastic muscles, correction of hip muscle spasticity or muscle contraction
Cerebral Palsy: Pharmacological agents
Treat pain related to spasms and seizures, muscle spasms can be painful
Cerebral Palsy: Botulinum Toxin A injections
Used to reduce the spasticity in specific targeted muscles
Cerebral Palsy: Dental hygiene
Regular dental check ups are essential, proper brushing, flossing and fluoride is encouraged
Brushing teeth can be a major ordeal if their facial muscles are involved
Cerebral Palsy: PT/OT
Stretching, passive, active, and resistive movements to maintain and increase ROM, strength and endurance
Cerebral Palsy: Care management
Assist the family in devising and modifying equipment and activities
May need G tube and extra feeding to ensure adequate wt gain, but also need to give orally to maintain oral motor control
Medication admin: G tube if jaw is compromised, if PO then support jaw (Holding middle finger of non-dominant hand under the jaw with thumb placed on bottom of the lip, index finger placed along the jaw), also used during feedings
Safety precautions (aspiration)
Recreational activities
PT, OT, speech and language therapy
Support family
Neural tube defects
Largest group of congenital anomalies
Neural tube defects: Normally
The spinal cord and cauda equina are encased in a protective sheath of bone and meninges during development
Neural tube defects: Failure of neural tube closure during development
Produces defects of varying degrees may involve entire length of neural tube or be restricted to a small area
Neural tube defects: Incidence
Occurs more often in White and Hispanic children, studies have shown that it may be due to Hispanic diet.
They are less likely to take folic acid supplements and folic acid are known to help reduce neural tube defects
Supplementation may not be as protective in overweight woman according to some studies, this is important for Hispanic women because they 1/2 times more likely to give birth to a baby with spina bifida or neural tube defects
Neural tube defects: Types
Anencephaly
Myelodysplasia
Spina bifida occulta
Spina bifida cystica
Anencephaly
Congenital malformation where both of the cerebral hemispheres are absent
Condition is basically absence of brain. Incompatible with life, even if infant had some brain and is able to control temp, cardiac, and respiratory function, the may live for a few hours to weeks but eventually die from respiratory failure
Myelodysplasia
Any malformation of spinal canal and cord
Failure of the bony spine to close is termed spina bifida (2types)
Spina bifida occulta
Defect not visible externally, occurs most frequently in lumbosacral area, not apparent unless other associated manifestations or neuromuscular disturbances
Mild form of SB that many people aren’t aware they have. Outer part of vertebrae are not completely closed, the splits are so small that the spinal cord does not protrude. The skin at site and lesion may be normal or may have small hairs growing from it. May be a dimple in the skin or birthmark
Spina bifida cystica
Visible defect and usually occurs with external sac like protrusion 2 forms (Meningocele, Myelomeningocele)
Spina bifida cystica: Meningocele
Encases meningeal and spinal fluid and not neural elements involved
Spina bifida cystica: Myelomeningocele
Contains meninges, spinal fluid and nerves, usually associated with varying degrees of neurologic deficits
Neural tube Defects: Patho
Failure of the neural tube to close during the embryo’s early development (3-4weeks after conception). Some vertebrae and spinal cord do not fully formed and remain unfused and open. If opening is large enough then can cause some spinal cord to protrude through opening of those bones. There may not be a fluid filled sac that surrounds the spinal cord. May be genetic. Degree of neurologic dysfunction the child will experience depends on where the sac protrudes through the vertebrae, level of defect is, and amount of nerve tissues involved
Neural tube Defects: Patho: Multifactorial causes
Drugs, radiation, maternal malnutrition, chemical exposure, genetic mutation
Neural tube Defects: Patho: Additional causative agents
Maternal obesity
Maternal Dm
Low vitamin B12 level
Neural tube Defects: Prenatal detection
Elevated alpha-fetoprotein level in amniotic fluid at 16-18 wk gestation
Uterine ultrasonography
Chorionic villus sampling (CVS) can be done first. Not recommended before 10 wk of gestation because the CVS carries certain risks
Neural tube Defects: Clinical manifestations
Hydrocephalus associated with spina bifida, flaccid paralysis of lower extremities, absence of deep tendon reflexes, lack of response to pain, presence of constant urinary dribbling
Neural tube Defects: Therapeutic management: Postnatal care
Prevention of infection, performing neurological assessment, observing for abnormalities, dealing with the impact that it might have on the patient and family
Early closure of meningocele, typically with in the first 24-72 hr of life, favorable outcomes.
If sac is leaking then closure if recommended even earlier (24hr)
Neural tube Defects: Therapeutic management: Shunt
Provide some relief from progressive hydrocephalus
Early surgical closer if myelomeningocele sac, pre-fetal birth surgery can reduce the need for placement of shunt
Can shoe improvement of mental and motor function
Neural tube Defects: Therapeutic management: Orthopedic considerations
Prevention of joint contracture, correction of existing deformities if possible, prevention or minimization of motor and sensory deficit, want best possible function of lower extremities.
Skin care is important with use of braces, walking devices, wheel chairs
Neural tube Defects: Therapeutic management: Of GU function
Myelomeningocele is a common cause of neurogenic bladder which is lack of bladder control due to nerve innervation that causes them to lack bladder control
Predisposes the child to UTI because of not emptying the bladder completely
Might need intermittent catheterization
Teach parents how to do it and eventually taught the child when they are older
Neural tube Defects: Therapeutic management: GU Goal
Preserve renal function and manage the urinary incontinence
Neural tube Defects: Therapeutic management: GU surgical procedure
Vesicostomy- bladder is surgically brought out to the abdominal wall to allow continuous drainage
Appedovasacotomy- where catheterized channels is created from appendiz, ureter or bowel so catheter can be inserted through the channel. This is done when kids get older around 10 because they are old enough to use this to perform a self-catheterization, and it fosters independence
Neural tube Defects: Therapeutic management: Latex allergy
Risk for allergy is highest with spina bifida
Always place on a latex allergy precaution
Food items: bananas, kiwi, avocado and nuts
Educate the child and parents
Advocate for an Epipen
Neural tube Defects: Therapeutic management: Bowel control
Some degree of fecal continence may be achieved with regular bowel habits
Neural tube Defects: Prognosis
Depends on extent of neurological deficit and early surgical repair, preventing infection and correcting hydrocephalus. Many are intelligent and will achieve independence. Remember coordinated care is important
Neural tube Defects: Emergent nursing care
Prevent trauma to the membranous cyst, usually baby us placed in incubator or warmer to maintain temp. Avoid rectal temps can cause irritation and prolapse, sphincter function is effected.
Before surgical closure, prevent from drying out by covering with a sterile moist non-adherent dressing, usually sterile NS. When changing dressing look for leaks, abrasions, irritations and signs of infection. Carefully clean if soiled/contaminated
Look for signs of ICP: monitor FOC Q2H
Keep baby prone, no clothing or covering because don’t want to irritated it.
Turn baby head Q2H
Neural tube Defects: Post-op care
Monitor VS, I&O, provide nutrition through OG, NG, TPN, or PO if able to
Prone right after surgery or side lying
Close observation for any CSF leak
Monitor for signs of infection, and pain
Ventriculoperitoneal Shunt: FOC measurements, signs of increased ICP, careful with positioning of head
Bladder surgery: Look for signs of infection, make sure its healthy, no unusual odor or drainage. (want pink,no bleeding)
Orthopedic surgery
Neural tube Defects: Prevention
Supplementation of folic acid: 0.4mg/day begin before conception
Food that have folate: Green leafy veggies, citrus, fortified foods (rice, grits, pasta, breads,)
If history of neural tube defects take 4mg/day
Duchenne muscular dystrophy
“pseudohypertrophic muscular dystrophy”
Most severe and most common of muscular dystrophies in childhood
X-linked recessive inheritance pattern, 1/3 of cases results from fresh mutations and mother is not a carrier
Duchenne muscular dystrophy: Characteristics
Most kids reach their appropriate early developmental milestones, but evidence begins to occur in early childhood (3-5yr)
Difficulty running, climbing stairs, riding the bike
A progressive of muscle weakness, wasting contractures then begin
Hypertrophic calf muscles occur in most patients due to enlargement from fatty infiltrations occur- feels firm or woody
Progressive generalized weakness
Death from respiratory or cardiac failure between 15-30
Duchenne muscular dystrophy: Diagnostics
Prenatal diagnosis is possible as early as 12 weeks of gestation
Made through PCR where they look for dystrophic chain gene mutation
Confirmation of diagnosis by electromyography, muscle biopsy (reveal fatty fibrous tissue that gradually replaces muscle, protein dystrophin is low in muscle of these patients) and serum enzyme measurement
Positive family history and display of the usual characteristics of the disease
Duchenne muscular dystrophy: Clinical manifesations
First sign: delayed walking, tired easily, difficulty running, climbing, riding bike
Waddling gait, frequent falls, gower sign (characteristic sign of rising up from squatting or sitting position from the floor)
Lordosis from weaken pelvic muscles
Progressive symmetric muscle wasting that occurs
Enlarged or hypertrophic muscles, especially in thighs and upper arms
Profound muscular atrophy in later stages
Mild to moderate mental impairment
Obesity is common (contribute to premature loss of ambulation, and restricted because of dystrophy, and physical activity, become bored and end up consuming more calories )
Diet needs to be closely monitored
Deficiency of dystrophin isoforms in brain tissue is what causes cognitive mental impairment
Duchenne muscular dystrophy: Therapeutic management
No effective treatment
Keep child as active as possible: ROM exercises, bracing, performance of activities of daily living, surgical release of contractures as needed
Maintain vital lung capacity, surgery to release and muscle contractures or deformities, help them in their performance of ADLs
Ensure the child gets all vaccines due to a risk of respiratory infection, everyone around the child needs vaccines also. Avoid anyone who has a respiratory infection
Develop abnormal breathing patterns= inadequate oxygenation
Long term ventilation may be required
Genetic counseling for family
Duchenne muscular dystrophy: Goal
Maintain function in unaffected muscles (ambulations and independence as long as possible)
Duchenne muscular dystrophy: Care management
Multidisciplinary team helps child and family cope with chronic, progressive, debilitating disease
Design program to foster independence and maintain activity as long as possible
Teach child self-help skills
Provide appropriate health care assistance as child’s needs intensify
Take care of their skin and family as well because they can be immobilized or with assistive devices that can cause problem for their skin
Maintain some sort of bowel and bladder functioning and protect them from respiratory infection
Diet monitoring to prevent obesity
Guillain-Barre syndrome
Infectious polyneuritis
Acute demyelinating polyneuropathy with progressive flaccid paralysis
Occurrence in children is rare but happens between 4-10y/o
Child’s own immune system begins to damage their nerve cells, causing muscle weakness, which then leads to paralysis
Symptoms that can last for a few weeks. Most people fully recover but some people may be left with serious nerve damage. Rare occasions people have died from this disease, death occurs due to difficulty breathing
Guillain-Barre syndrome: Hallmark
Acute peripheral motor weakness. Occurs after 10 days after a nonspecific viral infection. Very rare occasion been reported after administration of certain vaccines, usually the influenza vax.
Patho of acquired neuromuscular disorders
Immune-mediated disease
Associated with viral or bacterial infections or administration of vaccines
Inflammation and edema in spiral and cranial nerves: progresses to impair nerve conduction and the produces partial or complete paralysis of muscles innervated by involved nerves
Patho of acquired neuromuscular disorders: Associated with
Infectious mononucleosis, measles, mumps, gastroenteritis, cytomegalovirus, Epstein barr virus, H. pylori, lyme disease
Guillain-Barre syndrome: Diagnostic
Based on paralytic manifestations, electromyographic findings or both
CSF analysis: may have increased protein concentrations; other lab tests results may be within normal limits
Symmetric nature of paralysis helps differentiate from other diagnosis
Guillain-Barre syndrome: Clinical manifestations
Very rapid onset
Begins with flu like symptoms
Initially: muscle tenderness, sore throat, paresthesia, muscle weakness
Weakness progresses to paralysis: may be ascending or descending, rapidly ascending from lower extremities; may involve trunk, arms, and face
Flaccid paralysis with areflexia which is a loss of reflexes
Intercostal and phrenic nerve involvement (breathlessness, vocalization and shallow and irregular respirations may be noted)
Urinary incontinence or retention and constipation (recovery of function usually begins 2-4wks after the progression stops)
Guillain-Barre syndrome: Therapeutic management
Treatment: symptomatic
Hospitalized in the acute phase due to respiratory involvement that may require ventilation assistance or even the use of a temporary trach tube
Treatment is acute phase is aggressive (IV steroids, IV immunoglobulin, plasmapheresis)
Respiratory support
Medications to prevent complications
Most patients may make full recovery in several weeks but it can take up to several months
Guillain-Barre syndrome: Acute phase
When the symptoms begin until new symptoms stop appearing; may last for as long as 4weeks
Guillain-Barre syndrome: Plateau phase
Symptoms remain constant without deterioration and may last from several days up to a few weeks
Guillain-Barre syndrome: Recovery phase
Patients begins to improve and progress on to their optimal recovery period
Guillain-Barre syndrome: Course and progrosis
Better outcomes are associated with younger ages
Muscle function begins to return 2 days- 2 weeks after onset of symptoms, but period to complete recovery is prolonged
Guillain-Barre syndrome: Care management
Supportive care
Observation for early signs of respiratory distress/difficulty swallowing (NPO, NG/G tubes)
Maintain an open airway with suctioning; postural changes to prevent pneumonia; monitoring O2 status
Temporary urinary catheter
Prevention of complications (prevent muscle or joint contractures so nurse or PT may provide passive ROM)
Pain management (Neurontin)
Physical therapy (passive ROM in the acute phase, later in the recovery phase: active therapy may be indicated)
Support for child and family
Down’s syndrome
Most common chromosomal abnormality
Decreased muscle tone, which can begin to compromise respiratory expansion. The underdeveloped nasal bone also causes inadequate drainage of mucus
Caused by an extra chromosome 21 = trisomy 21
The degree of cognitive and physical development is related to the percentage of cells with the abnormal chromosomal makeup
Down’s syndrome: Caused by
Translocation of chromosomes 15,21,22
Associated with advanced maternal age >35
Down’s syndrome: Physical features
Separated sagittal sutures Brachycephaly (short head) Flat occiput Inner epicanthal fold of the eye Specking of the iris (Brushfield spots) Small nose with the depressed nasal bridge Small ears (conductive hearing loss) High arched palate Protruding tongue Delayed eruption of teeth Excessive neck skin folds (detected on prenatal ultrasound) Neck laxity Very short broad neck Pectus excavatum (sunken chest) Pectus carinatum (pigeon chest) Congenital heart defects Round protruding abdomen Umbilical hernia Small penis Cryptorchidism Bulbous vulva Broad short hands with stubby fingers and transverse palmar crease Plantar crease between the big and the second toe Broad short feet with stubby toes Short stature Hyperflexibility Muscle weakness Hypertonia Atlantoaxial instability Dry cracked fissuring of skin Mottling of skin
Down’s syndrome: Brushfield spots
White dots ringing the iris
Down’s syndrome: Considerations
Speech, PT, OT: to promote the child’s optimal development
Give the family reassurance and resources and tell them about support groups
Down’s syndrome: Higher risk for
Strabismus Leukemia Cardiac defects Obesity Respiratory infections Hearing and vision loss Hypothyroidism
Down’s syndrome: Atlantoaxial instability
Increased mobility at the articulation of the 1st and 2nd cervical vertebrae
Most of the time it is something that is asymptomatic. Some who have it have normal x-ray and then have an abnormal x-ray following, some who have it have abnormal x-rays then have normal x-rays following
All children with Down’s who want to play sports should have a cervical spine x-ray to check for this (if negative then no need for more x-rays)
Children who do not have instability should be encouraged to participate in any sports they want
Down’s syndrome: Atlantoaxial instability: Cause
Not well understood but it may be an abnormality of the ligament which maintains the articulation, could also be from bony abnormalities of the cervical vertebrae… could be both
Down’s syndrome: Hypotonicity of muscle and Hyperflexibility of joints
Complicate position of children
Limp, flaccid extremity resembles posture of ragdoll so holding the infant can be difficult
Extended body position can also promote heat loss so parents should be encouraged to swaddle or cover the infant before picking them up
Decreased muscle tone compromises the respiratory expansion, and affects gastric motility- predisposing the child to constipation
Increase fiber and water to prevent constipation
