You are on page 1of 14

TCA #5

Scoliosis
- Spinal Deformity
- May Be In One Or More of Three Spinal Planes (forwards, backwards, twist, all of the
above)
- May Be Congenital or Develop in Infancy, Childhood, or Early Adolescence. Most people
start showing signs of scoliosis in pre-adolescence. During adolescence the child is
going through puberty and the child gets concerned about how they look. So scoliosis is
not just a physical thing, it has some profound psychological implications.
- Not Understood Why it occurs. Keep in mind that when a diagnosis is made of scoliosis,
you have to rule out intraspinal disorders.
- If Less than 10 Degrees Not Considered Scoliosis but a possible deformity, will not be
treated
- If between 10 & 20 Degrees, and Not Progressing or Causing Problems, Not Going to be
treated – just going to keep an eye on it.

Types of Curvatures
- Kyphosis – Increased Convexity Of Thoracic Curve (hunchback – old women are
frequently hunched over due to osteoporosis)
- Loridosis – Exaggeration Of Lumbar Spinal Curve (pregnant women – center of gravity
changes and they get lord sis)
- Scoliosis – Lateral Curvature (goes off to the side)

Curve Can Be On Thoracic, Lumbar, Thoracolumbar, Or A Double (Combination of Two) or can


have a twist to it

Diagnosis
- Screenings With Observation of Asymmetry of Shoulder Height, Scapular, or Flank
Shape or Hip Height
o Need To See If They Are Inline, Uneven Hips, Curve In Spine, Uneven Shoulders
o Bend Forward To See Better The Unevenness Of The Back And Shoulders
- Standing X-Rays

Therapeutic Management
- Regular Clinical and X-Ray Evaluation
- Begin with Orthotic Interventions (Bracing, Exercises)
- Possible Surgical Intervention (Spinal Fusion) (For Major Curvatures That Cause
Problems)
- Best Managed by a Multidisciplinary Team
- If Curve of 40% or Greater Must Have Surgery

Treating Scoliosis
• Bracing – hard to get compliance from adolescents
- Most Common Types
o Boston Brace – big molded plastic thing – the nurse will have to talk to this client
about how to manage the skin up underneath this brace. Most of the time they are
designed that they can be worn outside of the clothes, but most 13 year olds are
not going to wear this brace on the outside of their clothes.
o TLSO (Thoracolumbosacral Orthotic) – also a molded plastic – this brace can be
pulled and tightened. These braces will be fitted and they will put pad in different
TCA #5

places to put pressure to move the spine or they will come in and mold it exactly
the way they want it based on the patient’s current condition. So if they gain or
lose weight some adjustments may be made with this brace.
o Milwaukee Brace – Only Occasionally Used In Scoliosis, It Is Mostly Used For
Kyphosis. It is going to push them back and stand them up straight. It does not
give as much to the lateral curve. Someone that has Kyphosis as well as Scoliosis
may go into the Milwaukee Brace.

• Surgical Management – this is for serious scoliosis (if the curve is


more than 40° they are almost always going to have to have some surgical intervention.
Most of the time bracing and exercise is to try and strengthen those muscles to get them
into alignment are not going to work if you have a curve of that much) - If the curve is big
this client is probably going to have some psychological issues. They will probably have
some respiratory issues. So a multidisciplinary team approach will need to be used. The
surgery is going to be realignment. They will do this with external fixation devices (metal).
They are going to go in and try to get it aligned as possible. Then they will take bone from
the ileac crest or donor bone and they are going to fuse those joints so that the movement
is not there. The type of surgery will do is going to depend on the physicians preference.
• Types of Surgeries:
- Herrington Rods
o Straighten Curve With Rods / Hooks (1 Yr Fusion Takes Place)
o Uses Bone Graft to Attach Rods or Hooks to Vertebra
o Need to be very careful post op:
 Logroll – this needs to be taught and told this pre-op
 Flat Bed – 7-10 Days
 Body Brace / Cast Usually 6 Months
o Usually Done In The Summer So Not To Miss School
- L – Rod Segmental Instrumentation
o Use Wires And Fusion
o Posterior approach – they are going into the back side of the spine
o Provides a little bit more stability initially, so one of the advantages is that the
patient does not have to be immobilized as long initially. They can be mobile in a
few days.
o There is a greater risk of nerve damage
- Multi Hook Instrumentation
o Combination of the previous two listed surgeries
o The advantage is that there does not have to be any post op stabilization
- Anterior Instrumentation
o Use screws that they screw into the vertebra body and then they connect a rod or
a cable to them. They will tighten them up basically to put some tension on them
so that they are straight.
o Provides really good correction because they can use the screws to tighten it up
o Requires immobilization post-op
- Spinal Fusion
o Will Wait As Long As Possible Because This Will Stop Growth.
- Cotrel Dubousset
o (France) Metal Rods Applied Differently With Hooking Vertebral With Increased
Movement. No Brace Post Op, Up To 2-5 Days Logrolls.
TCA #5

Many of these surgeries they are going in anteriorly with an abdominal incision. So if you are
taking care of a back surgery patient that had an anterior approach – not only do you have an
orthopedic surgery in the back, but you have an abdominal incision and you need to provide the
care that goes with abdominal surgery also.

Scoliosis Nursing Considerations


- Pre-Op
o Treatment With Multidisciplinary Team
o Teaching – very important do both pre- and post-op teaching.
o Pre Operative Care
 X-Rays
 Pulmonary Function Study
 ABG’s
 Routine Labs
 Autologous Blood Donations – may start this a month or two ahead of time
- Post-Op
o Wound, Vital Signs, Circulation
o Neuro & Neurovascular Checks
o Logroll
o Pain Control – most of the time will be on a PCA pump
o Skin Care – teach how to manage skin, but also assess it – if they are immobilized
need to use all of the skin precautions
o NGT Usually, For Paralytic Ileus – Assess Bowel Sounds (we do not want them to
start vomiting) If the bowel sounds are not there and they do not have an NG
tube and you start noticing some distension, will need to call for an order to put
down an NG tube.
o Not unusual for them to lose a significant amount of blood so you want to monitor
urinary output to make sure that they are not going into any type of acute renal
failure
o Physical Therapy will be visiting them
o Foley Frequently Used – once they get mobile, we want the Foley out – risk for
UTI
o Teaching
o They may get cranky so you may want to do some diversional activities

Club Foot
- The Short Tendons Are Long, The Long Tendons Are Short
- Common Deformity in Which the Foot is Twisted Out Of It’s Normal Shape or Position
- See It At Ankle
- Deformities Are Described According To The Position of The Ankle And Foot: (if pes is on
the end this means foot and talus means ankle – so there are foot and ankle issues)
o Talipes Vargus – means the heel is turned in and the foot goes out
o Talipes Valgus – the heel and the foot is turned out
o Talipes Equines – the foot is actually plantar flexed (toes are down) – if not treated
will be walking on their toes
o Talipes Calcaneus – when the foot is dorsiflexed. They are going to walk on the
inner sole of their shoe.
TCA #5

o Talipes Equinovarus (TEV) – most commonly see - the foot is pointed down and
then it extends inward to varying degrees
o The book says that metatarsus adductus is probably the most congenital foot
deformity. This is not considered clubfoot. The TEV is the clubfoot that is most
common.
- Usually Can Be With Other Disease’s Like Spina Bifida, Muscular Dystrophy
- Should be diagnosed or noticed in infancy (should be noticed in newborn nursery). The
orthopedic doctors are actually coming up there and casting these babies before they go
home with their mother.
- Most of the time unilateral, but it can be a bilateral deformity.
- It is more common in boys than girls

Classifications of Clubfoot
- Mild or Postural – may fix on its own, may teach mom some passive exercises which
involve stretching the tendons and moving them how they should be normally. They may
do some casting
- Tetralogic – frequently reoccurs – they are usually going to have to go in and do some
surgical intervention. If you work in the newborn nursery and they say that it is a
tetralogical defect, you need to be thinking that you wonder what else is going on
because this is frequently associated with Spina Bifida and these types of things
- Congenital Idiopathic (True Clubfoot) – bone is fixed – pretty severe, it is not just the
tendons and ligaments that are shortened. The bone is also involved.
o Must Have Surgical Intervention for congenital idiopathic.

Management
- Correction of Deformity
- Maintenance of the Correction Until Normal Muscle Balance Is Regained
- Follow Up Observation To Avert Possible Recurrence Of The Deformity

Treatment
• Serial Casting
o Begun Shortly (ASAP) After Birth When The Infants Foot Is Very Pliable (Flexibility
of Bones). Re-casted frequently, they are growing fast
o Done in Stages:
 Adduction Deformity
 Inversion Deformity
 Plantar Flexion Deformity
o Casting Is Done To The Other Extreme Hoping To End Up Somewhere In The
Middle
o 1-2 Week Will Change Position With New Cast
o Unless Mild Will Not Be Just One Casting
o Most of the time if casting is done appropriately within 8 to 12 weeks, they have
the maximum amount of flexion that that cast is going to provide. They will then
do some X-rays. They do not X-ray early on because a baby’s bones are not
developed and they do not show up on the film real well. The bones have to ossify.
o If don’t get aligned like they should, they will probably do surgery between 4
months to a year.
TCA #5

Teaching about Cast Care


• Keep it dry
• Make sure that they come back in to have it checked and redone
• Same care for the infant cast as you have for the cast on an adult
• Teach mother how to perform neurovascular checks
• Teach about skin care
• Importance of office visits
• Physical therapy and how to do the stretching and exercises

- Alternative Correction
o Dennis-Browne Splint
 After Casting Or When It Is Not Very Severe
 Shoes With Steel Bats Adjusted
 May Wear At Night
 Nurse Does Not Adjust
 They Hurt, When They Hit You In The Head

Major Nursing Considerations To Be Used When Caring For A Pediatric/Adolescent Client With
This Diagnosis
• Skin / Circulation Assessment
• Parent Education / Support Because They Will Be Doing Most Of The Care
• Regular Cast Change
• Reinforcing Orthopedics Instructions
• Care Of Cast Or Application (Neurovascular Checks)
• Encourage To Facilitate Normal Development

Developmental Dysplasia of the Hip (DDH)


- Describes a group of disorders related to abnormal development of the hip.
- Reflects a variety of hip abnormalities including shallow Acetabulum, subluxation, or
dislocation
- 10 out of every 1000 births have hip problems
- 60% of the time the left hip, 20% of the time the right hip, 20% of the time both hips, and
mostly white females.

Degrees of DDH
- Acetabular Dysplasia (Preluxation)
o Mildest form, no subluxation or dislocation
o Delay in the development of the acetabulum. The acetabulum roof is just not as
well developed. It is thin and shallow, but it is intact and for the most part the
femur head is intact. It is just a little bit loose.
- Subluxation
o Most common
o Implies an incomplete dislocation of the hip
o Femoral head remains in contact with acetabulum, but even though it is in contact
with the acetabulum it is pulling on the ligaments there. There is a big pull there
and it puts pressure and makes it pretty loose up in the socket.
- Complete dislocation
o Femoral head has lost all contact with acetabulum
TCA #5

o Usually have shortening of the tendons, so it is going to be pulled away, so the hip
is totally dislocated. It is usually pulled posteriorly and superiorly over that
fibercartilaginous rim.

Prenatal Factors
- Maternal hormone secretion
o Estrogen produces laxity of maternal pelvis at end of pregnancy and also affects
fetal joints
- Mechanical factors of intrauterine posture (the way the baby is positioned in utero)
o Higher incidence with Breech presentations and C-sections
o Twins
o Large infants

Diagnostic Evaluation of DDH


- The earlier diagnosis occurs – the better
- Best to diagnose and begin treatment prior to 2 months of age
- As a newborn is appears as a lax or loose hip joint
- Hip should be examined regularly until child begins to walk and normal gait is observed
- X-rays usually not done due to bone not being completely hard (especially if they are < 4
months of age)

To diagnose DDH
- Asymmetry of Gluteal and thigh folds (butt cheeks – does one hang lower than the other)
- Limited hip abduction, as seen in flexion (Allis sign)
- Apparent shortening of the femur, as indicated by the knees in flexion
- Ortolani click – if infant is less than 4 weeks of age, only special trained nurses or
physicians do this
- Positive trendelenburg sign or gait if child is weight bearing – they are constantly shifting
back and forth, but where they are going to end up leaving their weight on the affected
hip. This is because the pressure puts everything back where it is suppose to be.
- Waddling gait
- Lordosis
- See box on page 1224 of Wong

Therapeutic management of DDH


- Newborn to 6 months
o Pavlik harness – worn 24 hours a day
o Maintains hip in such a way that the head of the femur is where it should be in the
acetabulum
o Allows for some movement
o Pavlik harness requires a lot of parent teaching. It is set by the physician and then
it is not to be removed by the parent. It needs to be left on at all times, not even
for baths. There will be skin involvement. Need to be taught to clean under it and
not to use a bunch of powder, etc.
o Hip remains in abduction until the ligaments are stretched and the hip is back in
the socket where it should be.
o Usually wear this for about 3 to 6 months.
o If it is doing absolutely no good or when they take it off and there is another
problem there – then they are probably going to next go to a spica cast.
TCA #5

o Any time there is skin traction; there is a risk for skin breakdown.
- 6-18 months (before the problem has been noticed)
o By then the limb has shortened and they probably have some contractures in that
hip adductor and flexor.
o Gradual reduction by traction followed by cast immobilization
o Have to come up with alternate ways to help them explore their world because this
immobilizes the child.
o Make sure that you tell the parent that if there is a smell, or if they complain of a
hot area or if you feel a hot area when you touch that cast, you need to get the
child back in. They will cut out a window in the cast and make sure that there is no
skin breakdown under the cast.
- Older child
o Difficult to manage since secondary adaptive changes complicate the condition
o Open reduction (ORIF) required with casting and rehab
o IF NOT TREATED BY AGE 6 MAY BE UNREVERSIBLE WITHOUT MAJOR
SURGERY

Nursing care with DDH


- Assessment observation and referrals
- Teaching
o Skin care
o Cast care
o How to apply and maintain the device
o Toy selection
o Age/Developmentally appropriate activities
o Transport of the child

Spina Bifida
- Neural tube defect
o Largest group of congenital anomalies
- Occurs when the neural tube (bone and meninges) fails to close thereby producing
defects of varying degrees (it may be severe enough effect that the entire spinal column
is out)
- The bone has left open and through those openings that bone back there – it has allowed
parts of the spinal cord or the covering of the spinal cord to protrude through it.
- May involve the entire length of the tube or only a small portion
- These are defects that are derived from the embryonic neural tube.
- The higher up it is and the larger area it covers the more problems can develop
- Folic acid is something the mother can do or take to help prevent this
- These babies are going to the NICU
- If Spina Bifida is discovered while in utero – the baby will probably be delivered by c-
section

Types of Spina Bifida


- Spina Bifida Occulta
o Defect is not visible externally
o Bone is not closed but no protrusion of contents
o Usually occurs between L5-S1 (many times can see a little dimple)
TCA #5

- Spina Bifida Cystica


o Meningocele
 Midline defect through the osseous spine that encompasses the meninges
and spinal fluid but no neural elements
 No Neural defects due to no nerves involved
 Herniation protrusion of a saclike cyst filled with spinal fluid
 Clinically, not usually termed spina bifida
o Myelomeningocele
 Protrusion of meninges, spinal fluid, and nerves through a defect in the
osseous spine – is a visual defect
 Hernial protrusion of a saclike cyst containing meninges, spina fluid and a
portion of the spinal cord with its nerves
 Neurological deficit is present in varying degrees
 Degree of neurological deficit depends on anatomic level of defect – the
lower the defect; it involves the nerves below that area. The lower the
better.
 Hydrocephalus (fluid on the brain) is frequently present. A baby with
hydrocephalus is at risk for intercranial bleed, seizures.
 If there is a neural tube defect – this child is a greater risk for clubfoot,
developmental issues, etc. – you get in and you start seeing some other
defects.

Prenatal Diagnosis of Spina Bifida


- Can determine some major neural tube defects prenatally
- Ultrasound scanning of uterus
- Increased levels of alpha-fetoprotein (AFP) in the amniotic fluid can indicate the presence
of Spina Bifida. Can be diagnosed in-utero
o Elevated levels HIGH risk for Spina Bifida
o Test usually done at 16-18 weeks
o Decreased may be Downs Syndrome
o MRI, Ultrasound, CT, Myelography
- Amniocentesis with 2+ AFP test
- Chorionic villus sampling (CVS) – going to be done between 9 – 11 weeks into the
pregnancy – this can tell if something is wrong. Go in and take fetal cells out and analyze
them. There is a little risk for miscarriage.
- Made on basis of clinical manifestations and examination of the meningeal sac
- MRI and CT scan
- Myelography
- Labs will be checked for organisms known to cause common, major problems – risk for
infection – need to monitor for signs and symptoms of meningitis – UTI’s are common
because they have a lot of urinary retention.

Clinical manifestations
- Frequently no observable manifestation
- May be associated with cutaneous manifestation
o Skin depression
o Port-wine angiomatous nevi
o Dark tufts of hair
TCA #5

o Soft cutaneous lipmas


- May be neuromuscular disturbances
o Progressive disturbances
o Bowel and bladder control problems
o Gait, foot weaknesses
o May be a lot of anal-sphincter problems ----FYI – never take and anal temp on a
newborn
o May see some actual sensory things down below the normal when doing a
neurological check – they may not respond like would be typically expected.

Cystica Spina Bifida


- Sensory disturbances usually parallel motor dysfunction
- Below L2
o Flaccid, partial paralysis of lower extremities
o Will have major bladder and bowel issues
- Below S3
o May not see any motor impairment at all
o May have “saddle anesthesia” – this is where they are numb where they would be
if they were sitting in a saddle on a horse.
o May see joint deformities

Management of Myelomeningocele
- Teaching
- Multidisciplinary approach for the many abnormalities in cardiac, respiratory, neuro, etc.
systems
- Assessment of intactness of sac – a huge portion of a nurse’s job is to keep that sac
protected. You want it to stay intact, clean. Usually there will be an order to keep sterile
saline gauze over it to keep it moist.
- Keep pressure off of defect – these babies are going to be laying prone until the defect is
repaired
- Prevent infection, assess of s/s infection
- Neurological assessment and observation for anomalies
- Measure head circumference at birth and daily - hydrocephalus
- Assess fontanels for bulging/tension - hydrocephalus
- Assess output, may have retention
- Avoid rectal temps
- Family support
- Myelomeningocele should be closed early – usually within first 12-18 hours but definitely
before 72 hours
- Watch for skin breakdown because these babies have to lay prone
- They need to eat, but they have to lay prone – may do OG tube – they will still have suck
reflex and will need a pacifier.

Post-op (Myelomeningocele)
- Same as any other surgery Plus
o Close observation for cerebrospinal fluid
o Some of the neurosurgeons – after the repair will let the baby lay on its side, but
this will depend on if the baby has developmental hip problems.
TCA #5

o These babies often have major urinary retention and they will probably on in & out
caths. Therefore, at risk for UTI’s.
o Want to limit latex exposure. These babies are at huge risk for latex allergies.

Common meds used in client’s spina bifida


Ditropan – anti-spasmotics – given for bladder spasms
- Detrol
- Pro-banthine
- Various antibiotics for UTI – prophylactic - Bactrim
- Stool softeners

Prevention of Spina Bifida


- Folic Acid 0.4 mg/day should be given to all women of childbearing age.
- For women with history of pregnancy with NTD’s increase intake of 4 mg folic acid/ day
under supervision of physician beginning 1 month before a planned pregnancy

Cerebral Palsy
- Group of disorders characterized by early onset of impaired movement and posture
- Non-progressive (does not get worse)
- Abnormal muscle tone, and coordination are primary disturbances
- Most common permanent disability of childhood
- More premature babies are at a greater risk for cerebral palsy than term babies

Causes
- Prenatal Brain abnormalities??
- Perinatal problems, especially birth hypoxia???
- Anorexia secondary to other causative factors

Classifications
- Spastic
o Balance, posture, coordination
o More you try to control it gets worse
o Jerky spastic movements – can be one side or both sides
o Most have a problem with fine and gross motor skills – the harder they try to do
something the more they shake
- Dyskinetic/Athetoid
o Abnormal movement is more like a worm movement when they walk
o May have trouble with pharynx and swallowing
o May have speech problems
o Also have trouble with drooling
o As they get older and get into adolescence they seem to get into more problems
(involuntary movements, if they get under stress- they have more of the
involuntary movements)
- Ataxic CP
o Wide base gait
o Very rapid and repetitive movements
- Mixed type/Dystonic
o Combination of all
TCA #5

o Involuntary movements and more jerking movements

Diagnosis
- Neurological exam and history are primary modalities for diagnosis (when start seeing
some of these things, the first thing to do is rule out brain tumor)
- Very hard to recognize CP in the early months of life (if notice that baby is not feeding
well and coughing and choking as they try to eat – this is a good sign to look at)
- Nurse needs to know developmentally what is going on
- Observation of signs of any of the classifications
- EEG
- Tomography
- Screening for metabolic defects
- Electrolytes

Management
- Goal is for early recognition and promotion for optimum development within constraints of
their brain dysfunction
- Requires a multidisciplinary approach (PT, OT, orthopedic doctor, neurologist, etc.)
- Treatment is very individualized
- Establish locomotion, communication and self help
- Gain optimum appearance and integration of motor functions
- To correct associated defects as effectively as possible
- To provide individualized educational opportunities
- To promote socialization experiences with affected and non affected persons
- PT is one of the most frequently used conservative treatments
- Exercises are individualized
- Exercises include
o Stretching
o Passive, active and resisted movements applied to specific muscle groups
o Water exercise
- Speech therapy
- Support groups

Therapeutic devices for CP


- AFO’s (Ankle Foot Orthotics)
o Brace to help keep straight
o Prevent deformities
o Makes their gait more efficient
- Other mobilization devices
o Scooters
o Go-carts
- Technical aids
- Therapeutic aids

Ortho Surgical
- Surgery is only for children who do not respond to PT. Usually the surgery is going to be
to correct orthopedic problems or to go in and put a suprapubic catheter in because they
have so much urinary distension. Surgery is to promote function and to help their
development as much as possible. There are a lot of tendon lengthening procedures
TCA #5

because they will get some fractures because of the spasticity that will not allow them to
stretch like they should.
- Selective dorsal rhizotomy (neuro) – they go in and put electrical stimulation along those
dorsal roots of the nerve. What was now spastic is now flaccid. If now flaccid, these
patients have to relearn to walk and such. This procedure requires a lot of physical
therapy after it.
- Drugs
o Skeletal muscle relaxants (Robaxin)
o Anti-anxiety – to possibly help with spasticity
o Valium – sometimes they will give this (not over a long period of time), they will
give it to them and relax them and actually get good range of motion and do this
for a while until they can get the ROM and then they will go off of the Valium and
continue their stretching. The ROM exercises must be done many times a day.
o Botox
o Local nerve blocks
o Little effect on overall function

Combination neurosurgical and Pharmacological Management


- Involves implantation of a pump to infuse baclofen directly into the intrathecal space
surrounding the spinal cord, this will prevent the side effects of oral administration and
they are getting the medication right where they need it. Before this is done there will be
a little trial done. This procedure is done in the OR by a neurosurgeon. The patient will
have to return on a regular basis to have the pump refilled. This procedure is showing a
lot of promise. Also used for patients with Tourette’s syndrome.

Prognosis
- Moderate disability
o Survival rate is about the same as for an unaffected child for the first 20 yo
- Severe disability
o 50% probability of surviving 20 years or more

NURSING DIAGNOSIS
• Risk for injury (falls and aspiration)
• Self care deficit
• Impaired physical mobility
• Fatigue
• Body image
• Impaired verbal communication
• Altered Family Processes (this effects the whole family)

Muscular Dystrophies
- Largest and single most important group of muscle disease of childhood
- All MD’s
o Are hereditary
o Are progressive
o Cause a characteristic, selective pattern of weakness and loss of strength
TCA #5

Patho
- Cause is unknown but appears to be related to a metabolic disturbance
- Serum creatinine phosphokinase is consistently elevated and assists in diagnosis and
early detection

Types of MD’s
- Duchenne (Pseudohypertrophic) Muscular Dystrophy is the most severe and the most
common muscular dystrophy of childhood. This is an X-Linked disorder there fore, males
are affected almost exclusively, it is transmitted by the mother to the male. Females are
usually going to be the carriers. This means that male children are at risk for symptoms
and the female children are at risk for carrying it. If someone is diagnosed with a
muscular dystrophy, the parents will be referred to genetic counseling. 50% of the males
born in that family are going to be infected and 50% of the females born in that family are
going to be carriers.

CHARACTERISTICS OF DUCHENNE MUSCULAR DYSTROPHY


• Early onset, usually between 3 and 5 years of age. This is because at 3 years the child
has more muscle. You can ask most parents of a child diagnosed with MD if they
crawled and walked when he/she should have and most of the time they will tell you that
the child was a little bit slow or late.
• Usual distribution for the Duchenne’s is hip, shoulders and quadriceps
• Progressive muscular weakness, wasting, and contractures. Increases with age
• Calf muscle hypertrophy (pseudohypertrophy) is a classic feature in most patients. The
muscles have fat come in so what looks like big muscles are really fat. So even though it
looks like their legs should be strong – they are not.
• Loss of independent ambulation by 9-11 years of age – Usually in wheelchair
• After they are able to run up stairs; they will stop and begin crawling or slowly up stairs.
• Slowly progressive, generalized weakness during teenage years
• Relentless progression until death from respiratory or cardiac failure
• Eventually all muscles in the body are involved.

Facioscapulohumeral MD
- Onset in early adolescence
- Distribution – shoulder girdle, neck, face, pelvic girdle
- Progression is slow
- Lifespan is usually unaffected
- With type they will have trouble raising their arms over their heads, they may have a
problem with facial mobility and the shoulders may float to the front.

Limp-Girdle MD
- Autosomal recessive disease
- Onset – Late childhood or adolescence
- Slow progression
- Proximal shoulder and pelvic girdle muscles

Late stage MD
- Ventilator is possible

Diagnosis
TCA #5

- Serum enzymes – creatine phosphokinase, adolase and AST levels are extremely high in
the first 2 years of life before the onset of clinical weakness. They diminish with muscle
deterioration but do not reach normal levels until severe muscle wasting and
incapacitation have occurred.
- Muscle biopsy – degeneration of muscle fibers with fibrosis and fatty tissue replacement
- Electromyography (EMG) – a slow decrease in amplitude and duration of motor unit
potentials

Management
- No effective treatment exists for childhood MD
- Teaching
- Primary goal is to maintain muscle function in unaffected muscles for as long as possible
- ROM exercises
- Surgery to release contractures deformities
- Bracing
- Assistance with ADL’s
- Genetic counseling
- Referrals – transportation, support groups, etc…

Nursing considerations
- Major emphasis is coping
- With Duchenne MD help family cope with fatal outcome
- ADL’s
- Encourage independence if possible

You might also like