Long term effects of chronic neurological disability

Question 1

what is spina bifida

Answer 1

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

Question 2

what is spina bifida linked with

Answer 2

hydrocephalus
70-80% suffer with both

Question 3

when is the neural tube closed

Answer 3

26

Question 4

what shows that there is a minor spina bifida defect and what is its name

Answer 4

SPINA OCCULTA
there is usually a tuft of hair (external)
internally there may be:
1. completely missing spinous process
2. poorly formed spinous process

Question 5

what is meningocele

Answer 5

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

Question 6

what is myelomeningocele

Answer 6

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

Question 7

what is myeloschisis

Answer 7

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

Question 8

what is the order of spina bifida forms from least to most serious

Answer 8

spina occulta
meningoceles
myelomeningocele
myeloschisis

Question 9

what are the neural consequences of spina bifida

Answer 9

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

Question 10

how do you treat affected area

Answer 10

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

Question 11

what is an issue with the valve involved in hydrocephalus

Answer 11

they can block
causing headaches, confusion, nausea
as there is compression in ventricles again

Question 12

what are some associated symptoms with spina bifida

Answer 12

renal anomalies
sphincter function
intellectual impairments (due to hydrocepahlus)
msk issues

Question 13

what feet are patients with spina bifida born with

Answer 13

often either
-rigid club foot
-vertical or rotated talus (rocker bottom feet- can be rerotated to horizontal plane and axial rotation)

Question 14

what is the issue with initial malformation of feet coupled with muscle weakness

Answer 14

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

Question 15

what is scoliosis due to
and how many people with spina bifida have it

Answer 15

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%

Question 16

how does the level of SB malformation affect the function of the patient

Answer 16

the lower the malformation, the milder the effect
(eg. cervical lesions lose much more)

Question 17

what is the prognosis of a patient for independent walking as an adult
(assuming there is no significant intellectual/psych impairment to walking)

Answer 17

thoracic and upper lumbar lesions- walking not possible
lower lumbar and sacral- can walk with ORTHOTICS to compensate for weak muscles

Question 18

is mobility the same as walking

Answer 18

NO
they may be mobile, able to drive, high function in wheelchair
but unable to walk

Question 19

what mobility challenges are there with an L4 lesion

Answer 19

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

Question 20

what mobility challenges are there with an L4 lesion

Answer 20

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

Question 21

what happens when there is a flexed knee gait in patients

Answer 21

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

Question 22

what are key factors of urinary tract for patient survival

Answer 22

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

Question 23

what does incomplete bladder emptying leads to

Answer 23

back pressure on ureters
renal parenchymal damage and failure
HYDRONEPHROSIS-> RENAL FAILURE

due to lack of control of sphincters

Question 24

how often to you scan kidneys in spinal injury patients

Answer 24

at least every 2 years

Question 25

what is therapeutic goal and treatment of urinary incontinence and why

Answer 25

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)

Question 26

What lesion comes with urinary and faecal incontinence

Answer 26

S2-4

Question 27

what is the treatment for faecal incontinence

Answer 27

daily rectal enema
laxatives/manual evacuation
stoma

Question 28

why do these patients get faecal incontinence

Answer 28

paralysis of external anal sphincter and mechanism to indicate a full rectum
may also have weak abdo and perineal muscles

Question 29

how is sexual potential affected

Answer 29

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

Question 30

what is education like for these patients

Answer 30

-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

Question 31

what is employment like with these patients

Answer 31

ABILITY rather than DISABILITY

discrimination so disability legislation to prevent this

day clinics (for social) if disability prevents employment

Question 32

what are the challenges for parents and caregivers

Answer 32

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?

Question 33

what is the optimal outcome for these patients to love independently

Answer 33

want:
-normal intellect
-social continence stability
-independently mobile- drive, wheel chair, walking aids
-independent living- household adaptions, telecare
-employment

Question 34

what are some challenges for these patients to live independently

Answer 34

-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