The Missing Factor living without Factor VII

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The Missing Factor- living without Factor VII

• Becky Sands

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Living without Factor VII

• Introduction

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Living without Factor VII

• About me!
• 22 years old
• Getting married in a months time
• Launching career as an Illustrator
• Graduated from Lincoln University in 2005 and
• the odd thousand in debt to the Student Loan
  Company
• nothing too different there!

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Living without Factor VII

• What is different the missing factor is a
  deficiency in Factor VII
• Effected my way of life and those around me
  parents and husband

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Living without Factor VII

• Today
• Bleeds on average once a week less frequent
  recently
• Inject myself at home but assistance required
  during a bleed
• Arthritis in ankle and elbow
• Some days difficulty walking and lifting

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Living without Factor VII

• Finding out about my Factor VII deficiency

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Living without Factor VII

• Diagnosis
• When young - bruising/pain in toes, ankles
  fingers - even tested for gout!
• In 1995 (aged 12) set myself 1.5km swim
  challenge on holiday, resulting in
• painful knee and anti-inflammatory treatment
  aspirin and at doctors later a black blue arm
• blood tests and diagnosed Factor VII deficient
  (level of 1.7 - severe with parents levels
  of 51 50)

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Living without Factor VII

• Dealing with the first incident
• In 1999 had first hospitalising bleed
• Weekend break 170 miles from home - VERY
  painful/swollen ankle did not realise it would
  get so bad
• Contacted Lincoln (home hospital) - negotiated
  admission to nearest H. Unit
• Supplies by emergency car from London to Bristol
  - no Factor VII for 24hrs from start of bleed!

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Living without Factor VII

• Treatment
• Initially, fresh frozen plasma but bad reaction -
  very low bp more needed
• Factor VII concentrate - this resulted in
  rash/stomach pains
• Continued to feel ill - in hospital 4 days

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Living without Factor VII

• Lessons from the first incident
• Afraid, especially in hospital away from home
• Difficulty of knowing you have a bleed,
  especially first time/in early days - so
  treatment delayed
• Realisation that few AE Depts/doctors have
  experience of Factor VII
• No supplies were on hand

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Living without Factor VII

• Response to the first incident
• Case reviewed/new 24hr arrangements established
  at Lincoln Hospital - battle plan including
  dosage, where to go for injection, reviews etc
• New arrangements were comforting and reassuring
  above all it all meant ...
• a realisation by me of a longer term problem

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Living without Factor VII

• Learning how to cope with the problem

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Living without Factor VII

• Problems remaining with new arrangement
• Lack of knowledge beyond the experts, especially
  in hospitals without H. Units - e.g. lack of
  urgency with treatment
• Finding files and supplies in the middle of the
  night!
• Worries regarding HIV/other infections - no
  NovoSeven available at that time

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Living without Factor VII

• After first incident
• Had regular bleeds in right ankle and then left
  elbow - treated with Factor VII concentrate,
  preceded by Piriton - administered in hospital
• Became better, but not good, at predicting bleeds
• Still had two particular problems at this time -
  the injection supplies

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Living without Factor VII

• Injecting myself
• First time I saw a needle ... I fainted
• Sister Whyatt began training me to inject myself,
  first ...
• using a model arm
• partly on my own
• on my own in hospital
• on my own at home
• in the BACK OF A CAR!!

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Living without Factor VII

• Inject into the back of right hand cannot put
  tourniquet on left arm
• Help needed with injection and for safety
• Multiple/frequent injections reduces options
• Husband now training to inject

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Living without Factor VII

• Improving supplies
• In 2000 recombinant Factor VIIA (NovoSeven)
  became available, via the hospital
• Safe product, easier to administer and store
• Four 120KIU boxes at home - supplied via hospital
• Jan 2004 12 boxes supplied direct to home

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Living without Factor VII

• Bleeds
• Less acute bleeds in ankle and elbow - inject
  120KIU as soon as happening/improvement after
  1hr/near full elbow extension in 1 to 2 days
• Acute bleed - no warning - several doses (e.g.
  120 x 2-6)
• Abdominal bleeds - possible endometriosis -
  hospital in Sheffield, Hallamshire (Paper) mini
  pill

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Living without Factor VIIFrequency of bleeds
2003
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Living without Factor VIIFrequency of bleeds
2004
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Living without Factor VIIFrequency of bleeds
2005
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Living without Factor VIIFrequency of bleeds
2006
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Living without Factor VII

• Issues surrounding bleeds and treatment
• Are pains always caused by a bleed?
• Finding places that I can inject
• hiccups!
• Nervousness dependency
• As a teenager, being taken seriously
• Side effects, increasing arthritis
• Pressure on parents and husband

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Living without Factor VII

• Arthritis
• Fairly severe in my right ankle because of bleeds
  before diagnosis
• Also now in right elbow
• Had operation to clean elbow steroids in
  ankle, at Sheffield Northern General -
  unsuccessful

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Living without Factor VII

• The impact on my life

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Living without Factor VII

• Practical issues - Factor VII
• Take NovoSeven Factor VII pack when away from
  home in UK abroad spare needles etc,
  tourniquet . even flask of hot water!!
• Storage in 12v fridge, especially for the car
  (good for beer!) check ahead for fridge in room
  or hotel bar!

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Living without Factor VII

• Practical issues - Factor VII
• Have to have someone who can help - difficulty if
  elbow has become painful
• Travel - medical insurance/security/ letter/ SOS
• Avoid getting knocked e.g. hard ball
  sports/rapids at Center Parcs!

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Living without Factor VII

• Practical issues - arthritis
• University - art degree (illustration)
• Uni job in bakery time-off, lifting and
  standing
• Social drinking (little alcohol!)/no dancing!
• Driving - automatic as changing gear a problem
• Career placements, travel, employed/self
  employed

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Living without Factor VII

• Some of the benefits
• Learning to cope with pain and hospitals!
• Learning more about oneself earlier in
  life/stronger
• Getting to come to Canada!

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Living without Factor VII

• Wish List - existing treatment
• Early screening
• More awareness knowledge of treatment in
  hospital/doctors
• Easier injections or not to have to inject at
  all!
• Longer lasting

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Living without Factor VII

• Wish List - Future treatment
• Protection of right elbow for drawing
• Prophylactic treatment?
• Gene therapy?
• Effective artificial joints (e.g. ankle)

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Living without Factor VII

• Living with NovoSeven
• Much less pain
• Less severe arthritis
• No fears regarding HIV

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Living without Factor VII

• Information for patients BPL/NovoSeven
• Being Me

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Living without Factor VII

• Thank you for inviting me

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Living without Factor VII

• Questions