HAEMORRHAGIC AND COAGULATION DISORDERS (THROMBOCYTOPENIA (CLINICAL…
HAEMORRHAGIC AND COAGULATION DISORDERS
is a disorder that occurs when there is a reduction of platelets, resulting in bleeding disorders, it may be congenital or acquired.
Inadequate production of platelets by the bone marrow
Increased and premature destruction of circulating platelets by antibodies, malignancy, immune disorders, bacterial and viral infections and drugs such as sulphonamides and quinine
Loss or increased utilisation of platelets in patients with mechanical prosthetic heart valves.
Secondary to haemolytic anaemia and disseminated intravascular coagulopathy
Abnormal distribution of platelets as a result of hyperplenism and hypothermia
Regardless of the cause, thrombocytopenia affects coagulation and haemostasis, resulting in bleeding tendencies.
A history of prolonged and sometimes uncontrollable bleeding following minor trauma
Spontaneous bleeding from the nose, gums, urinary system, gastrointestinal tract and excessive menstruationin women
Petechia in the skin as well as ecchymosis
Signs of anaemia as a result of bleeding
Joint pains due to extravasations into tissues
Fever, lethargy and weakness
The goal of management is to prevent bleeding, therefore the patient should be put on bed rest with the head elevated to prevent intracranial bleeding.
Advise patients to use a soft-bristled toothbrush for cleaning their teeth, and use an electric for shaving to prevent bleeding
Instruct patients in measures to avoid constipation, as straining could cause bleeding
A splenectomy may also be perfomed and where this has been successful, it has produced a lasting remission in the majority of patients with thrombocytopenia.
Replacement therapy with blood or components is also an option.
Teach the patient to recognise signs of bleeding and how to prevent episodes
Encourage the patient to take medication as prescribed
Methods of arresting bleeding include gentle application of pressure on bleeding sites, as well as the use of cold compresses.
Is an inherited sex-linked chromosomal defect where there is a deficiency of one or more of the clotting factors carried on the X chromosome, hence females are carriers and males are sufferers.
TYPES OF HAEMOPHILIA
Also known as classic haemophilia caused by a deficiency of clotting factor VIII, it can be mild, moderate or severe
Also called christmas disease -due to a deficiency of clotting factor IX, it can be mild, moderate or severe
Normally, haemostasis is a regulated process that depends on the interaction between the blood vessels, blood platelets and coagulation factors.
Clotting factors are multiple plasma proteins (numbered I to XIII) that cause cascades of enzymatic reactions in blood plasma.
These cascades of reactions are often divided into an intrinsic, extrinsic and a common clotting pathway.
Patients with haemophilia A have an abnormality of the gene coding for coagulation factor VIII, leading to abnormally low plasma levels of factor VII.
This factor is involved in the intrinsic pathway of coagulation.
Haemophilia B is the result of an abnormality of the gene coding for coagulation factor IX.
Because factor IX is also involved in the intrisic clotting pathway, the low levels of factor IX give rise to a disease with clinical manifestations identical to those of haemophilia A.
The coagulating factor deficiency in haemophilia results in failure of blood to clot and therefore a tendency to bleed spontaneously, after minor trauma and after major trauma and surgery.
There are, considerable variations in the degree of this bleeding tendency among patients.
Haemarthroses causes pain, redness and swelling of the larger joints, commonly the knees, elbows and hips.
Repeated episodes can result in limitation of movement and ankylosis, which can be crippling without adequate treatment.
Bleeding into muscle is common after relatively minor trauma or intramuscular injections.
Haemophilia is not curable, but bleeding episodes can be adequately controlled.
Give a transfusion of blood and fresh frozen plasma as prescribed to replace blood loss.
REPLACEMENT OF DEFICIENT CLOTTING FACTORS IS ACHIEVED BY THE ADMINISTRATION OF:
Factor IX concentrates during bleeding
Cryoprecipitate infusion to replace deficient factor VIII, Factor XIII and fibrinogen.
ADMINISTER PRESCRIBED MEDICATION AS FOLLOWS:
Analgesics as prescribed for pain, but aspirin and NSAIDS must be avoided because these increase the incidence of bleeding.
Aminocaproic acid in cases of clot formation to slow down the dissolution of clots.
Synthetic vasopressin (DDAVP) can be administered intravenously, subcutaneously or intranasally to produce a transiet rise in factor VIII concentration in patients with haemophilia.
CARE OF THE JOINTS SHOULD BE DONE IN THE FOLLOWING WAY:
Apply splints and other supportive devices to immobilise painful and swollen joints.
Elevate the affected joint to improve venous return.
Apply cold compresses to joints gently.
Handle the swollen joints gently.
Joint mobilisation and physical terapy.
Joint replacements are sometimes required.
Assist with passive or active range of motion exercises daily
PREVENT BLEEDING AS FOLLOWS:
Avoid, whenever possible, intramuscular injections.
Observe the patient for signs of bleeding.
Teach the patient and their family about the nature of the condition in order to promote understanding and cooperation.
Stress the importance of preventing injury.
Encourage the patient to seek medical help for any suspected or obvious bleeding.
Instruct the patient on procedures to stop bleeding, such ass application of cold compresses, gentle direct pressure and elevation of the affected area.
Inform the patient about the importance of notifying dentists and other doctors about the condition for when dental care is required or illness occurs
It is for paramount importance to avoid aspirin or other drugs that may impair blood platelet function and cause severe bleeding.
Follow-up care and blood transfusion may be required
DISSEMINATED INTRAVASCULAR COAGULOPATHY
Is an acute disorder characterised by widespread blood clotting and haemorrhage.
In DIC, the flow of blood is disrupted by precipitating factors, resulting in the formation of tiny clots within the micro-circulation
These clots consume platelets and clotting factors and the blood loses its ability to clot, which causes bleeding.
Acute onset with rapid development of the signs and symptoms
Bleeding from the body, which may be mild or severe ( signs of bleeding that may be observed include petechiae, ecchymosis, purpura, bleeding from mucous membranes, lungs and from venipuncture sites)
Signs of hypolaemic shock will be evident.
Treat the underlying cause.
Improve oxygenation tissue hypoxia.
Give fluid replacement therapy: transfusion of blood, RBCs and cryoprecipitate as prescribed.
Low doses of heparin may be prescribed, although this is controversial.
Monitor vital signs carefully
Keep intake and output records.