Long term effects of chronic neurological disability Flashcards
what is spina bifida
a group of congenital neural tube malformation conditions in which:
-incomplete development/covering of the brain/ spinal cord
-caused by a failure of the fetal spine to close normally in the first month of pregnancy
what is spina bifida linked with
hydrocephalus
70-80% suffer with both
when is the neural tube closed
26
what shows that there is a minor spina bifida defect and what is its name
SPINA OCCULTA
there is usually a tuft of hair (external)
internally there may be:
1. completely missing spinous process
2. poorly formed spinous process
what is meningocele
it is when the sac (found outside the body due to there being no spinal body) contains the meninges and CSF but not the spinal cord
spinal cord is in the normal position
what is myelomeningocele
spinal cord and therefore extended sensory and motor nerves are displaced and exposed to the external environment- only protection is meningeal layer (sac)
enlarged sub-arachnoid space with CSF
what is myeloschisis
v serious
exposed neurons- no meningeal covering
continual leakage of CSF as central canal is open
–no covering
incompletely developed and non-functional spinal cord
therefore risk of INFECTION
what is the order of spina bifida forms from least to most serious
spina occulta
meningoceles
myelomeningocele
myeloschisis
what are the neural consequences of spina bifida
paralysis
loss of sensation distal to abnormality
no formation of reflexes distal etc
CSF circulation disruption causing hydrocephalus
high risk of foetal meningitis- infection where spinal cord isnt covered
how do you treat affected area
close/stitch it to hold fluid
if hydrocephalus:
put a shunt in
out through one way valve
drain into peritoneal space
pressure that build in ventricle forces CSF to go down catheter and through valve
empty ventricle to normal pressure
what is an issue with the valve involved in hydrocephalus
they can block
causing headaches, confusion, nausea
as there is compression in ventricles again
what are some associated symptoms with spina bifida
renal anomalies
sphincter function
intellectual impairments (due to hydrocepahlus)
msk issues
what feet are patients with spina bifida born with
often either
-rigid club foot
-vertical or rotated talus (rocker bottom feet- can be rerotated to horizontal plane and axial rotation)
what is the issue with initial malformation of feet coupled with muscle weakness
signals that brain normally gets to develop (stimulate bone with weight and stimulate with vertical column of weight) not there
so two bones in leg develop at slightly different rates
-shortening of fibula exacerbates problems==> wedging in the ankle
-this causes walking on the insoles
-cant feel where feet are touching ground or shoes–> sores–>infections
LATERAL WEDGING OF TIBIA CAUSES VALGUS OF FEET
what is scoliosis due to
and how many people with spina bifida have it
due to a combination of congenital abnormalities of the spine and spinal muscle weakness
poor motor control- we need it to keep spinal column nicely aligned therefore unequal stimulation of bone growth, so grow unequally
LATERAL CURVES
50%
how does the level of SB malformation affect the function of the patient
the lower the malformation, the milder the effect
(eg. cervical lesions lose much more)
what is the prognosis of a patient for independent walking as an adult
(assuming there is no significant intellectual/psych impairment to walking)
thoracic and upper lumbar lesions- walking not possible
lower lumbar and sacral- can walk with ORTHOTICS to compensate for weak muscles
is mobility the same as walking
NO
they may be mobile, able to drive, high function in wheelchair
but unable to walk
what mobility challenges are there with an L4 lesion
child grows- demands on muscles increases
muscle strength doesn’t change as limited muscle innervation
therefore muscle fatigue
knee pain- due to stance
therefore become more reliant on wheelchair- self-propelling, or electric
what mobility challenges are there with an L4 lesion
child grows- demands on muscles increases
muscle strength doesn’t change as limited muscle innervation
therefore muscle fatigue
knee pain- due to stance
therefore become more reliant on wheelchair- self-propelling, or electric
what happens when there is a flexed knee gait in patients
weak or paralysed calf muscles results in excess ankle dorsiflexion and more strain on ankles (reliant on quads)
energy inefficient gait
muscle fatigue
this causes further bone problems
lose spinothalamic pathways- cant feel pain so get joint deterioration- bone on bone as cartilage goes and get growth spurts
what are key factors of urinary tract for patient survival
bladder should fill to good capacity
should empty fully- otherwise bacteria left to ferment=infection-> stones and blockages
emptying should be under voluntary control
filling and emptying should not be detrimental to renal function
what does incomplete bladder emptying leads to
back pressure on ureters
renal parenchymal damage and failure
HYDRONEPHROSIS-> RENAL FAILURE
due to lack of control of sphincters
how often to you scan kidneys in spinal injury patients
at least every 2 years
what is therapeutic goal and treatment of urinary incontinence and why
controlled continence by end of childhood
can cause social isolation- affect quality of life
-permanent bladder catheterisation is not good long term- UTI risk
-so use INTERMITTENT CATHERTERISATION- carer/ patient
-URINARY DIVERSION stoma bag (ureter to stoma bag)
What lesion comes with urinary and faecal incontinence
S2-4
what is the treatment for faecal incontinence
daily rectal enema
laxatives/manual evacuation
stoma
why do these patients get faecal incontinence
paralysis of external anal sphincter and mechanism to indicate a full rectum
may also have weak abdo and perineal muscles
how is sexual potential affected
libido may be normal
sensation absent (eg. S2-4 area)
men- erections might still be possible but poorly maintained
usually incontinence and ejaculatory dysfunction- not sperm count issue
women- fertility normal, menstruation causes social difficulty
what is education like for these patients
-mainstream school possible (with less hydrocephalus) as not intellectually challenged– social and less isolation, social skills for independence
-special needs school
-college to acquire life skills with learning difficulties
what is employment like with these patients
ABILITY rather than DISABILITY
discrimination so disability legislation to prevent this
day clinics (for social) if disability prevents employment
what are the challenges for parents and caregivers
emotional and physical support
provide assistance with daily physical tasks- bathing, dressing, mobility
sibling/partner neglect?
aging parents and what will happen to children later?
what is the optimal outcome for these patients to love independently
want:
-normal intellect
-social continence stability
-independently mobile- drive, wheel chair, walking aids
-independent living- household adaptions, telecare
-employment
what are some challenges for these patients to live independently
-learning difficulties
-behavioural difficulties (may have been isolated/social issues)
-cannot transfer independently- needs hoisting, care washing, toilet
-aging parents
-some might live sheltered/ residential facility and be looked after by a team of carers- but limited and expensive
