inherited bleeding disorders Flashcards
(78 cards)
1
Q
what is the coagulation cascade
A
- a continuous process
- as coagulation takes place, fibrinolysis also takes apace
2
Q
what is the general treatment principles of those with bleeding disorders
A
- treat as a normal person if on treatment
- treatment planning is important
- need to be aware of potential problems
3
Q
what may be some potential problems for dental treatment with those with bleeding disorders
A
- hygiene phase therapy
- local anaesthetic
- extractions and surgery
4
Q
why is prevention important
A
- if can prevent dental treatment then it makes life easier
5
Q
what can be done as part of prevention
A
- oral hygiene
- regular dental care
- fluoride supplements
- fissure sealants
- dietary advice
6
Q
what dental treatments are safe to do on those with bleeding disorders
A
- these are all safe as they don’t cause blood to be released into the mouth
- hygiene therapy
- removable prosthodontics
- restorative dentistry, including crowns and bridges
- endodontics
- ortho treatment
7
Q
what dental treatment need special care for those with bleeding disorders
A
- extractions
- minor oral surgery
- periodontal surgery
- biopsies
8
Q
what is important to ensure you do for extractions and surgery for patients with bleeding disorders
A
- may need to do preparations before = appropriate monitoring
- atraumatic Treatment
- consider antibiotics
- observe to ensure haemostats
- comprehensive post operative instructions
- need to liaise with haematologist
9
Q
what is an inherited bleeding disorder
A
- an acquired defect which affects the coagulation of the blood
- is a dynamic process
10
Q
what may inherited bleeding disorders affect
A
- coagulation cascade
- platelets
- in some people may affect both = a combined deficiency
11
Q
how can inherited bleeding disorders affect the coagulation cascade
A
- a reduction in one or more of the coagulation factors
12
Q
how can inherited bleeding disorders affect the platelets
A
- number and function
13
Q
what are some common inherited bleeding disorders
A
- factor VIII deficiency
- factor IX deficiency
- von Willebrand’s disease
- factor XI deficiency
14
Q
what is factor VIII deficiency also called
A
- haemophilia
- haemophilia A
15
Q
what is factor IX deficiency also called
A
- Christmas disease
- haemophilia B
16
Q
what is von Willebrand’s disease
A
- both factor VIII and platelet process don’t behave properly
- reduced factor VIII level
- reduced platelet aggregation
17
Q
where is factor XI deficiency common
A
- Ashkenazy Jew population
18
Q
what are rare bleeding disorders
A
- inherited defects of other factors in the coagulation pathway
- inherited defect of either the number or function of the platelets
- numbers in each group are small
- there are large numbers of different conditions registered in the UK = over 50
19
Q
what is the management of rare bleeding disorders
A
- complex due to the bleeding not always relate to the factors levels
- need to liaise with haematologists
20
Q
how common is haemophilia A in the UK
A
- 6480
- this will be the largest group that will have some impact on their dental care
21
Q
how common is haemophilia B in the UK
A
- 1372
22
Q
how common is VWD in the UK
A
- 9265
- most common
23
Q
how common is factor XI deficiency in the UK
A
- 1036
24
Q
how common are platelet disorders in the UK
A
- 815
25
what is the incidence of bleeding disorders in the general population
- haemophilia A = 1:10,000
- haemophilia B = 1:50,000
- VWD = 1:100-500
- factor XI = 1:50,000
26
how many patient are registered in Scotland with severe and moderate disease
- 500
| - these patients need to be monitored by haemophilia centre
27
how many patients are registered in Scotland with mild disease and carrier
- 2500
| - don't need much care
28
how can some people be carriers of haemophilia
- haemophilia is a sex linked recessive gene so only happens in girls with both X chromosomes with gene and in boys with the gene on their X chromosomes
- use defective gene in 50% of cells and non-defective in 50%
29
how much factor VIII can haemophilia A carriers make
- 50%
- still plenty for normal life though
- will only be a problem if their coagulation is tested
- each patient needs tailored treatment
30
what is autosomal recessive inheritance
- bad luck
- means both boys and girls can have it
- although some people may have the recessive gene though, doesn't mean they will have the bleeding disorders
31
what makes rare bleeding deficiency difficult
- generally, lack of clear correlation between bleeding and level of factor so it is more difficult to manage
32
what are inhibitors
- these are antibodies which develop to factor VIII and XI
- antibodies against clotting factors
- the amount of antibody developed varies between patients
33
what percentage of patients develop an inhibitor when they first start treatment
35-40%
34
what do inhibitors do
- if patient is given clotting factors that are not produced in their body then these factors can either fit the receptor and the pathway will continue of be detected by the immune system as a foreign material and the body will develop antibodies against the artificial Factor VIII
35
how can giving artificial clotting factors be a problem
- if they are used up quickly then it is fine
- if patient is given artificial factors everyday then you will have to anticipate that some of it will be destroyed and will need to give patient more to compensate this
- this needs to be considered when giving treatment = as need to allow inhibitor levels to drop to low levels for treatment
36
who gets haemophilia A and B
- sex linked recessive
| - males are affected and females are carriers
37
how did you use to know if someone had haemophilia A or B
- their joints would be very arthritic as there were bleeding at joints
38
how mush factor VIII is needed for body to wrk
- not a lot
| - around 1iu/ml
39
how much factor VIII is in haemophilia A and B
- severe cases = <0.02iu/ml
- moderate = 0.02-0.09iu/ml
- mild = 0.1-0.4iu/ml
- carries = <0.5iu/ml
40
how is severe and moderate haemophilia A treated
- require the use of recombinant factor VIII produced by bacteria
- need to be treated with missing clotting factor
- some need it everyday as have so little that everyday activities cause bleeding
41
how are mild and carriers of haemophilia A treated
- majority of patients respond to DDAVP
- veyr mild cases only require tranexamic acid
- generally just need one or the other = if getting tooth out though, patient may need factor VIII given to them
42
how does DDAVP work
- releases factor VIII that is stuck on endothelial cell walls by competitive binding
- means more factor VIII is released into circulation without having to give it artificially
43
how does tranexamic acid work
- it is an inhibitor of fibrinolysis so slows down the rate that clots are destroyed
- turn-over of clotting may be low but using this returns the balance back to normal
44
how is haemophilia B treat
- do not respond to DDAVP as factor IX is not on endothelial walls
- prophylactic cover is required for recombinant factor IX = this is the only way to treat
45
how do people get VWD
- autosomal dominant
- both sexes are equally affected
- can be a spontaneous mutation or passed down by parents
46
what is VWD
- deficiency of von Willebrand's factor with a reduction in factor VIII levels
- factor VIII and platelet problems
47
what are the 3 types of VWD
- type 1 = dominant and mild
- type 2 = dominant and mild
- type 3 = recessive and severe
48
what type of VWD is less likely
- type 3
| - need copy from both parents so less likely to get it
49
how is VWD treated
- most patients respond to DDAVP
50
how do patients respond to DDAVP for VWD
- as need factor VII
- factor VIII sits on platelets and interact with platelets on blood so the action of platelets is inhibited if factor VII is stuck on walls
51
what do very mild cases of VWD require to be treated
- tranexamic acid
52
what are the treatment pathways for patients with bleeding disorders in theUK
- common treatment regimes for all patients
- treatment should be available locally
- hospital visits reduced
- if patient is mild and needs tooth extracted then get this at hospital then return to normal dentist for other treatment
- if patient is severe then get all treatment done at hospital
- most of the standard treatment depends on the risk of the patient
53
where are severe and moderate patients treated
- majority of the treatment in the hospital except for prosthodontics
- treatment by GDP/PDS by arrangement
54
where are mild and carriers treated
- treatment shared with GDP/PDS
| - patient reviewed at the hospital every 2 years
55
what are the dental treatment that required special care treatment
- administration of local anaesthetic
- extractions
- minor oral surgery
- periodontal surgery
- biopsies
56
where at the safe sites to administer LA
- buccal infiltration
- intralignmentary injections
- intra-papillary injections
57
where are the dangerous sites to administer LA
- inferior alveolar nerve block
- lingual infiltration
- posterior superior nerve block
58
why is the inferior alveolar nerve block dangerous
- issue wit tissue that you puncture as you give this
- if infiltrate upper then a pressure builds and prevents bleeding but with alveolar there is not a pressure build up and so bleeding occurs
59
what is the problem with extractions and surgery
- stopping the bleeding once the tooth is removed
60
what needs to be done if patient with bleeding disorder getting extractions or surgery
- appropriate cover from haemophilia unit = liaise with haematologist
- consider antibiotics
- observe for 2-3 hours after surgery
- comprehensive post operative instructions
61
why must you liaise with the haematologist
- they will work out how much clotting factors are needed before the extraction is done
62
why must you observe patient for at least 2-3 hours
- need this long as initial bleeding stops due to platelets ad plug formed doesn't form until a while later
63
what needs to be done for severe and moderate patients after extractions and surgery
- observed overnight
| - bed rest for patients as if they went home they would most likely not rest properly
64
what must be done for mild and carrier patients after extractions and surgery
- observed for 2-3 hours
65
where are edentulous patients with disease treated
- treatment carried out in GDP or CDS
66
what is the comprehensive care team needed to care for these patients
- haematologist
- physiotherapists
- nurses
- dental surgeon
- psycho-social workers
- lab technicians
- orthopaedic surgeon
67
what is thrombophilia
- opposite of haemophilia
- increased risk of clots developing
- over producing clots of not producing enough fibrinolytic products
- often an acquired condition superimposed on a genetic condition
68
what is common for patients with thrombophilia
- deep vein thrombosis
| - can get it in legs and it can break off and travel up to lungs and cause pulmonary embolism which is dangerous
69
what are the inherited syndromes of thrombophilia
- protein c deficiency
- protein s deficiency
- factor V leiden
- antithrombin III deficiency
70
what are acquired syndromes of thrombophilia
- antiphospholipid syndrome = lupus anticoagulants
- oral contraceptives
- surgery
- trauma
- cancer
- pregnancy
- immobilisation
71
what are platelet abnormalities of thrombophilia
- thrombocytopenia
- qualitative disorders
- thrombocythenia
72
what is thrombocytopenia
- reduced platelet numbers
73
what are causes of thrombocytopenia
- idiopathic
- drug related = alcohol, penicillin, heparin
- secondary to lymphoproliferative disorder
74
can you do dental treatment to patients with thrombocytopenia
- can proceed safely providing the platelet count is >50x10^9
75
what are qualitative disorders
- normal platelet count but abnormal function
76
what are inherited qualitative disorders
- rare
- Bernard Soulier syndrome
- Hermansky Pudlak
- Glanzmann's thrombasthenia
77
what are acquired qualitative disorders
- cirrhosis
- drugs
- alcohol
- cardiopulmonary bypass
78
what is thrombocythemia
- increased number of platelets
- uncommon disease
- usually on aspirin to prevent clot formation
