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Answers in support of



EB competency



Patients name………Fred……............…..



Carers name…………………………………. date / /



Carer to demonstrate knowledge of anatomy of skin









Epidermis

Skin pore



Dermis



Base layer







Label areas marked and list function

a) Epidermis – is the thin top layer of skin. It has no blood supply

and is supported by the dermis

b) Skin pore – helps fluid balance and temperature control in the

body

c) Dermis – is the thicker vascular layer of skin provides

elasticity and strength

d) Base layer – protects organs, bones and muscles



List 3 main functions of the skin

a) To keep the body together …

b) Temperature regulation ….…

c) Barrier to infection……..….…



Section 2

Carer to understand psychological implications of having EB



What is EB?

EB is a genetic condition where the lubricant between skin layers

is missing causing blistering with the least bit of friction.................

Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 1

Review date 2007

There are 3 types of EB, underline the type........Fred.... has

Dystrophic EB

Junctional EB

Simplex EB



In a short paragraph explain what happens to....Fred’s....... skin

As the skin moves it rubs together and a blister rises. The blisters

just get bigger unless the blister is popped to allow the skin to heal.



How does this affect.....Fred.....in the following areas?

a) Eating / drinking

EB affects the gastro-intestinal tract so his mouth can be very sore

and his oesophagus will contract with scaring

b) Mobility

His feet are permanently blistered and painful so his mobility is

poor

c) Eye site

EB also affects eye site so Fred’s eye site is poor and he needs

glasses

d) Healing

Fred heals well if he has good nutrition but as he blisters all the

time and gets infected it is a continuing process

e) Pain

Fred feels pain the same as anyone else at the same level

f) Continence

It is difficult to sit on a toilet and not worth the extra handling at

night, so he wears a nappy at night



How does this affect...........Fred’s..... body image?

He has said he wishes he didn’t have EB but he has a big

personality and the pain and practical problems worry him more

than body image



How does......Fred...talk about his/her condition?

He is very knowledgeable about his condition and will tell you

about it. Parents and child are also involved In Debra so he tells

you about others with the same problem



What could happen if.....Fred.....decides to move himself?

He could rub the skin from his bottom/ or damage his feet



Does...Fred...... experience pain at more the same level as

unaffected people? Yes No

Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 2

Review date 2007

If yes can you describe how you feel when

doing...Fred’s...dressings?

It can be very distressing if Fred is in pain. The smell from the

wound is occasionally very strong and upsetting



How does this affect......Fred......?

Fred has analgesia and midazolam to help with the dressings but

he hates his bath



How do the other people in...Fred’s...life cope with....Fred’s ...pain?

Mum

Mum accepts it as part of Fred but she is very involved in Fred’s

care and can get very upset if he has a bad bath and dressing

session

Dad

Does care for Fred but it tends to be mum who takes the lead role.

Dad is occasionally reluctant to give pain relief in case he gets to

used to it

Siblings

Fred has no siblings as parents are concerned the same condition

could occur

School staff

Fred has a 1-2-1 carer who can give pain relief when appropriate

Health care professionals

Most HCP’s have not seen EB prior to meeting Fred so it can be a

big shock. Most are willing to follow advice from specialist unit



Describe how.........Fred.....looks (including dressing)

Fred has a big wide smile! His mum buys larger sizes of trendy

clothes to cover his dressings. He now doesn’t wear shoes as

they caused friction



How does this affect...Fred.....?

Fred is quite secure as a person but obviously likes it better when

he needs fewer dressings



Children with EB have a lot of agencies supporting them. How

does the intrusion affect the family home?

It is difficult to fit in extra appointments on top of dressings and

medicines. Most EB children have to go to GOS which involves

travelling and overnight stays. Parents have to accept strangers in



Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 3

Review date 2007

their homes and are dependant on others which if they have

problems in their own lives may mean family care can fall down.



Underline any of the following words you recognise in relation

to........Fred........ and family situation



Dependant on others Lack of control of others

Coping Caring

Stressful Busy

Angry Tired

Mutually dependant Loving

Private Independent

Painful Sad

Happy Fun



Section 3

Carer to demonstrate awareness of Health and Safety in EB



Do you have a current moving and handling certificate?

Yes No



List 3 risks to you moving and handling

a) Trying to protect Fred I might over stretch

b) It is not safe to sling/hoist Fred

c) Difficult angles to life Fred from bed/floor



List 4 principles of good moving and handling with a child/young

person with EB

a) Use flat hands

b) Ask child for co-operation and tell them what you’re going to do

in advance

c) Use silks in bed

d) Use cushion/pillow to sit up from lying down



What are the risks of poor moving and handling technique for a

child with EB?

More skin damage and pain leading to friction and infection



Why may......Fred....choke?

Because his oesophagus is now so narrow that he has trouble with

his own secretions





Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 4

Review date 2007

List 3 things that may trigger a choking attack?

a) Lying down – secretions pooling in throat

b) Chest infection

c) Eating and drinking



Describe how to support.........Fred....when choking?

Follow protocol, keep calm. Sit Fred up and dispose of secretions



Who is you first line of support during a choking attack?

CCN Parent Key worker

(please underline)



What must you do to help keep ...Fred.. calm during an attack?

Phone 999 Keep calm Shout for help

(please underline)



Is it safe for.......Fred...to eat and drink? Yes No



Can....Fred....have anything by mouth? Yes No



List 3 potential hazards when lancing a blister

a) Needlestick injury to child

b) Needlestick injury to self

c) Contact with bodily fluids

d) Introduction of infection



List 3 people who may be put at risk when lancing a blister

a) Child/ young person

b) Parent

c) Self



What is clinical waste?

Waste that has bodily fluid on it



Describe in a short paragraph safe disposal of clinical waste

Clinical waste must be disposed of safely for incineration. It should

go in a yellow bag – the council will collect these separately



Who does safe disposal of clinical waste protect?

Everyone – self, child/young person, family and refuse collector



What risks are associated with use of needles?

Needle stick injury

Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 5

Review date 2007

List 3 ways of preventing those risks

a) Not re-sheathing needles

b) Lancing blisters at the correct angle

c) Closing sharps box when it is three quarters full



Should you re-sheath needles? Yes No



What must you do if you get or cause a needle stick injury?

a) Squeeze site to make blood flow freely

b) Put under fast flowing water

c) Contact on call and fill in incident form



Where do you record it?

In care plan



Who do you report it to?

On call - CCN



Where is the needle stick injury guidelines in......Fred’s...house?

In his care plan



Section 4

Carer to be aware of strategies to prevent damage to skin/

organs of child/young person with EB



How does blistering occur in a child with EB?

There is no collaboration between layers of skin, so any movement

can cause friction



List 3 organs that may be affected in a child with EB

a) Skin

b) Oesophagus/ bowel

c) Mouth



List 3 ways of preventing damage

a) Use of correct moving and handling

b) Taking care with soft foods

c) Ensure dressings are always well applied



What does friction mean?

Friction means rubbing where there is no lubrication so there is

force surface against surface



Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 6

Review date 2007

If.......Fred....slides himself what might occur?

Blistering of back of legs or buttocks



How would you ask him to move himself safely?

Support him to lift himself up and down, and not slide.



Is it possible to move himself safely? Yes No



As.....Fred’s....skin heals under its dressing does it get itchy?

Yes No

If yes how can we help to manage this?

Pat the skin with flat hands



Can he scratch? Yes No



When......Fred......is in bed are there any ways we can help move

in bed? Yes No



If yes, what?

Use of silks. Use the pillow to lift him holding the pillow



If.....Fred....was choking in bed and you wanted to sit him/her up

quickly, what might you use?

Use the pillow to hold



What problems could wrinkled dressings cause?

More friction and blisters



Is it safe to straighten them? Yes No



How must the dressing be fixed?

Undone and re-applied



What damage could you cause if you pulled then straight?

More blistering



What does the term ‘flat hands’ mean?

That I don’t grip the child/ young person but hold him with straight

hands

How does ‘flat hands’ make it safer to move......Fred...?

It makes it safer because I place the hands were they will cause

least friction



Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 7

Review date 2007

EB affects the gastro-intestinal tract . So if.....Fred......has anything

by mouth please describe the texture and consistency?

Sips water only



Why is this important?

It can affect his nutrition which he needs to help support his

healing and growth



When should blisters be lanced?

a) At next bath

b) When gets home from school

c) As soon as they are noticed



Please give reasons for answers

The blisters will just get bigger and bigger if not burst



Section 5

Carer to demonstrate safe lancing of blisters



Hand washing is an important part of any care you give.

How many steps are in the Trust hand washing policy?

2 5 7 8 9



How often do you need to repeat each step?

2 4 5 7 times



How often do you need to wash your hands when caring

for……Fred…?

a) At beginning and end of shift

b) As required throughout the shift

c) Before and after any care you give

(please underline)



List what equipment you require prior to child/young person’s

bath/dressings

.....Towels.................... .....Silks.......................

.....Pre-cut dressing...... .....Green towels.........

.....Cream..................... .....Sharps...................

.....Pyjamas ................. .....Sharps boxes.........

.....Gloves..................... .....Gauze.................

.....Disposal Bag.........





Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 8

Review date 2007

Describe a suitable environment for....Fred..... bath and dressings

Warm bathroom with room to move



List 3 essential things for this environment?

a) Privacy

b) Warmth

c) Ways of supporting child length of procedure – distraction



Are.........Fred’s...... dressings a sterile technique?

Yes No



How would you describe the dressing technique?

It is a clean technique, it is as clean as possible



What are the universal procedures?

Using gloves and apron and hand washing technique as form of

protection for child/ young person and myself



Who do they protect?

Child/Young person Staff Both

(please underline)



At what angle do we lance the blisters?

Approx 180 degrees



How do we ensure blister is completely drained?

a) Make as many puncture holes as it requires to ensure all fluid

drained

b) Put needle in and tear blister

c) One puncture hole is always sufficient

(please underline)



List 3 things we need to ensure when lancing a blister?

a) Child knows what we are doing and has consented

b) Sharps box is available

c) Swab is ready to collect fluid



How do we collect the fluid from the blister?

Use a gauze swab to collect fluids



How do we dispose of this fluid?

Put swab in disposal bag



Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 9

Review date 2007

How do we dispose of sharps?

In sharps box



How full should a sharps box be when it is sealed?

Three quarters



Why is the box sealed at that level?

In case anyone is damaged by sharps in box when placing a sharp

in the box



Describe what to do if a needle stick injury occurs to

a).....Fred......

Tell Fred and parents – follow injury client document – accident

form

b) Others, i.e. Parents

Follow client – report – contact GP for instructions – accident form

c) Self

Follow client – contact on call



What form must you fill in?

Incident form



Section 6

Carer to demonstrate how to support child if choking



Why is the incident of choking more likely in......Fred.....?

Because his oesophagus is narrow



Describe in a short paragraph.....Fred’s....care plan for managing

these episodes.

These episodes happen mostly at night so Fred must be sat up

using pillows and not be left throughout the episode

Where is the protocol kept?

In care plan



Is it accessible at all times? Yes No



Is there anything specific that....Fred.....likes you to do during

choking episodes? Yes No



If yes, please explain

Hold bowl and towel and take them away a.s.a.p



Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 10

Review date 2007

Section 7

Carer to be aware of the importance of good nutrition in EB



How does a ‘normal’ child obtain good nutrition?

Through a healthy diet



List 3 reasons why.....Fred... may not have good nutrition?

a) His problems with narrow oesophagus

b) Sore blisters in his mouth– may make it painful for him to eat

c) Not able to take enough calories in



Why may......Fred.....require better nutrition than other children of

this age?

He needs a lot of calories for the extra healing he requires



How is this managed for.......Fred..... to support his nutrition?

Fred has as gastrostomy for all his nutritional needs – he also has

small feeds overnight. Fred only has sips of water so he also has

extra water especially in summer



Section 8

Carer to recognise importance of record keeping



Why is it important to speak to ……Fred….. in a manor that is age

appropriate?

So he understand what is said as well as not feeling patronised



List 3 words that will reflect how you speak to parents/carers

a) Politely

b) Pleasantly

c) Reflecting their expertise



What do you understand by accurate, appropriate documentation?

Record keeping that reflects exactly what happened in an ordered

manner. It is about Fred and the instructions he required and only

involves others where they are required to Fred’s care……………



Who do you need to write about?

Child-1 family-3 interventions-2



Does it matter when you write the document? Yes No



If so when should it be written?(please list in order)

Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 11

Review date 2007

As soon as possible …1...

Within shift …2...

Within 24 hours …3...



Who should read this document?

Only people who have permission and need to know....................



What colour should you write this in?

Red blue black purple



List three things you must do to identify the child

a) Name...................

b) Address................

c) Date of birth - community number..........



Can you leave spaces for others to fill in later? Yes No



Who can you share information with about this child?

Only those you have permission to do so with….....…



Do you always need parent’s permission to share information?

Except in cases of patient safety and child protection..................



Underline the words, which apply to good recordkeeping

Factual patient condition

Consistent legible

Accurate understandable

Fiction fit for purpose

Consecutive current

Abbreviations concise

Jargon subjective comments

Contemporaneous



List 3 things you must do to identify who wrote the record and

when the record was written

a) Name print and sign.......

b) Date and time…….........

c) Job title…………..…..…



List who you would report record keeping problems to?

CCN Team…………..............................……………………





Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 12

Review date 2007

What information would it be suitable to discuss with…Fred’s

teacher in school?

Only information I’d been asked to discuss by parent or Fred…



Section 9

Carer to demonstrate awareness of issues of privacy and

dignity



In a short sentence, explain what privacy means to you.

Privacy means own space, own secrecy, solace…...…………



In a short sentence, explain what dignity means to you.

Dignity means being treated with respect, opinion and beliefs

being respected and feeling valued



List how you would know if …Fred……was

Happy He smiles but does not laugh a lot........

Sad He’ll tell you...............................................

In pain See below.............................................

If possible, give examples

If Fred is in pain he will complain of a poorly tummy even if the

pain is not in his tummy. We then play a game pointing at bits of

the body till he says ‘yes’



How can we preserve ……Fred’s…dignity especially during bating

and changing dressings?

Ensure Fred’s consent is gained prior to bath. Ensure he has

adequate pain relief. Ensure he is listened to throughout the

procedure and his wishes are taken into account where ever

possible





What does child specific mean to you?

This only allows me to look after Fred – no one else









Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 13

Review date 2007

Questionnaire assessed by

_________________________________print



I certify I am currently registered with the

NMC_____________________sign Assessor



NB. Your responses to the questions in this document are

used to assist in the assessment of your competency to

undertake the particular procedures detailed in the

accompanying competency document and satisfactory

completion of this document is only part of the overall

competency assessment. You may NOT carry out child or

young persons care independently until the competency

document has been signed by your assessor evidencing your

competence, yourself, and your employer if you are a non

NHS Trainee.









Liz Herd July 2005 – Reviewed by Carolyn Bishop August 2005 14

Review date 2007



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