DISSEMINATED INTRAVASCULAR COAGULATION

INTRODUCTION Disseminated intravascular coagulation (DIC), also known as disseminated intravascular coagulopathy or consumptive coagulopathy, is a pathological activation of coagulation (blood clotting) mechanisms that happens in response to a variety of diseases. DIC leads to the formation of small blood clots inside the blood vessels throughout the body. As the small clots consume coagulation proteins and platelets, normal coagulation is disrupted and abnormal bleeding occurs from the skin (e.g. from sites where blood samples were taken), the gastrointestinal tract, the respiratory tract and surgical wounds. The small clots also disrupt normal blood flow to organs (such as the kidneys), which may malfunction as a result. DIC can occur acutely but also on a slower, chronic basis, depending on the underlying problem.It is common in the critically ill, and may participate in the development of multiple organ failure, which may lead to death.

DEFINITION Disseminated intravascular coagulation (DIC) is a serious disorder in which the proteins that control blood clotting become abnormally active and is characterized by diffuse fibrin deposition in the microvasculature,consumption of coagulation factors and endogenous generation of thrombin and plasmin RISK FACTORS Risk factors for DIC include:
     

Blood transfusion reaction Cancer, especially certain types of leukemia Infection in the blood by bacteria or fungus Pregnancy complications (such as retained placenta after delivery) Recent surgery or anesthesia Sepsis (an overwhelming infection)

 

Severe liver disease Severe tissue injury (as in burns and head injury)

INCIDENCE RACE: Disseminated intravascular coagulation (DIC) occurs in all races. SEX: No particular sex predisposition exists for disseminated intravascular coagulation (DIC). AGE: Disseminated intravascular coagulation (DIC) affects individuals of all ages. CLINICAL MANIFESTATIONS GENERAL MANIFESTATIONS: y y y Petechiae Purpura Bleeding from opening in the skin -Venipuncture site -Surgical incision y y y y Bleeding from umbilicus,trachea(newborn) Evidence of GI bleeding Hypotension Organ dysfunction from infarction and ischaemia

SYSTEM-WISE MANIFESTATIONS CIRCULATORY SYSTEM

y y y y

Signs of spontaneous and life-threatening hemorrhage Signs of subacute bleeding Signs of diffuse or localized thrombosis Bleeding into serous cavities CENTRAL NERVOUS SYSTEM

Nonspecific altered consciousness/stupor

Transient focal/neurologic deficits

CARDIOVASCULAR SYSTEM
y y y

Hypotension Tachycardia Circulatory collapse RESPIRATORY SYSTEM

y y

Pleural friction rub Signs of adult respiratory distress syndrome (ARDS) GI SYSTEM

y y

Hematemesis Hematochezia GENITO-URINARY SYSTEM

y y y y y y

Signs of azotemia and renal failure Acidosis Hematuria Oliguria Metrorrhagia Uterine hemorrhage DERMATOLOGICAL SYSTEM

y y y y y y y y y

Petechiae Jaundice (liver dysfunction or hemolysis) Purpura Hemorrhagic bullae Acral cyanosis Skin necrosis of lower limbs (purpura fulminans) Localized infarction and gangrene Wound bleeding and deep subcutaneous hematomas Thrombosis

CAUSES Causes of DIC can be classified as acute or chronic, systemic or localized. DIC may be the result of a single or multiple conditions. ACUTE DIC Infectious
y y y y

Bacterial (eg, gram-negative sepsis, gram-positive infections, rickettsial) Viral (eg, HIV, cytomegalovirus [CMV], varicella, hepatitis) Fungal (eg, Histoplasma) Parasitic (eg, malaria) Malignancy

y y y

Hematologic (eg, acute myelocytic leukemias) Metastatic (eg, mucin-secreting adenocarcinomas) Neuroblastoma Obstetric

y y y y

Placental abruption Amniotic fluid embolism Acute fatty liver of pregnancy Eclampsia Trauma

y y y

Burns Motor vehicle accidents (MVAs) Snake envenomation Transfusion

y y

Hemolytic reactions Massive transfusion Other

Liver disease - Acute hepatic failure

y y y y y y

Prosthetic devices Shunts (Denver, LeVeen) Ventricular assist devices Acute pancreatitis Graft reactions Congenital malformations

CHRONIC DIC Malignancies

y y

Solid tumors Leukemia Obstetric

y y

Retained dead fetus syndrome Retained products of conception Hematologic

y y

Myeloproliferative syndromes Paroxysmal nocturnal hemoglobinuria Vascular

y y

Rheumatoid arthritis Raynaud disease Cardiovascular - Myocardial infarction Inflammatory

y y y

Ulcerative colitis Crohn disease Sarcoidosis Localized DIC

y y

Aortic aneurysms Giant hemangiomas (Kasabach-Merritt syndrome)

y y

Acute renal allograft rejection Hemolytic uremic syndrome

BLOOD CLOT FORMATION

Blood clotting normally occurs when there is damage to a blood vessel. Platelets immediately begin to adhere to the cut edges of the vessel and release chemicals to attract even more platelets. A platelet plug is formed, and the external bleeding stops. Next, small molecules, called clotting factors, cause strands of blood-borne materials, called fibrin, to stick together and seal the inside of the wound. Eventually, the cut blood vessel heals and the blood clot dissolves after a few days. BLOOD CLOTS

Blood clots (fibrin clots) are the clumps that result when blood coagulates.

PATHOPHYSIOLOGY DIC occurs when the first stage of the coagulation process is abnormally stimulated. Two distinct phases can be identified. PHASE I: When clotting mechanism is triggered in the circulation

Thrombin is generated in greater amounts than can be neutralized by the body

There is rapid conversion of fibrinogen to fibrin with aggregation and destruction of platelets If local and widespread fibrin deposition in blood vessels takes place

Obstruction and eventual necrosis of tissues occurs PHASEII The fibrinolytic mechanism is activated

Cause extensive destruction of clotting factors

With the deficiency of clotting factors the child is vulnerable to uncontrollable hemorrhage into vital organs

Can also cause Damage and hemolysis of RBC DIAGNOSTIC TESTS Tests that may be used to diagnose DIC include:

1. D-dimer test. This blood test helps determine whether a person's blood is clotting normally by measuring a substance (fibrin) that is released as a blood clot breaks up. Ddimer levels are often higher than normal in people who have abnormal blood clotting. 2. Prothrombin time (PT/INR). This blood test measures how long it takes blood to clot. At least a dozen blood proteins, or clotting factors, are needed to clot blood and stop bleeding (coagulation). Prothrombin, or factor II, is one of several clotting factors produced by the liver. A long prothrombin time can be a sign of DIC. 3. Fibrinogen. This blood test measures how much fibrinogen is in the blood. Fibrinogen is a protein that plays a part in blood clotting. A low fibrinogen level can be a sign of DIC. It happens when the body is using fibrinogen faster than the body can make it. 4. Complete blood count (CBC). A complete blood count (CBC) involves taking a blood sample and counting the number of red blood cells and white blood cells. CBC results cannot diagnose DIC, but they provide information to help the doctor make a diagnosis. (DIC often causes the platelet count to drop.) 5. Blood smear. In this test, a drop of blood is smeared on a slide and stained with a special dye. The slide is then examined under a microscope. The number, size, and shape of red blood cells, white blood cells, and platelets are recorded. Blood cells often look damaged and abnormal in people who have DIC.

DIAGNOSTIC FINDINGS
Test Platelet count Fibrin degradation product (FDP) Factor assay Prothrombin time (PT) Activated PTT Thrombin time Fibrinogen D-dimer Antithrombin ABNORMALITY Decrease Increase Decrease Prolonged Prolonged Prolonged Decreased Increased Decreased

TREATMENT

The only effective treatment is the reversal of the underlying cause and elimination of aggravating factors such as acidosis, electrolyte disturbances,hypoxia,shock etc in order to prevent continuous activation of coagulation factors

All medication should preferably be administered intravenously keeping venepunctures to the minimum.

y y

Packed cells are administered to correct anemia. Hydrocortisone may be used in presence of purpura fulminans or meningococcemia infection

Vitamin K is given to correct vitamin K dependent factors

REPLACEMENT THERAPY: Fibrinogen and other coagulation factors can be replaced by the administration of cryoprecipitate or FFP.Fibrinogen level should be raised to over 150mg/dl.Infants may be given 10-15mL/kg of FFP along with to 1 unit of platelet concentrates every 12-24 hourly.Platelet counts should be maintained above 50,000/microlitre while fibrinogen level should be maintained above 75mg/Dl.

HEPARIN: Heparin is an activator of the physiologic thrombin system and inhibits number of proteolytic enzymes and factors such as thrombin, IXa and Xa. In most patients heparin therapy does not alter the course of DIC because of the underlying disease process. y Heparin therapy may reduce the severity of bledding and thromboembolic manifestation along with an improvement in the laboratory parameters. y The elevated levels of FSP fall rapidly, coagulation factors return to normal and accelerated fibrinolysis, if present, disappears. y It is recommended as 15-20 units/kg hour as continuous drip or 50-70 units/kg/6 hourly. y Administration of heparin is controlled by estimation of APTT which should be maintained at a maximum of 1.5-2 times the normal.

EXCHANGE TRANSFUSION Double volume exchange transfusion helps by providing the replacement factors and removing the a) circulating fibrin split products(FSP) b) activated procoagulant c) toxins Platelets concentrates should be administered after the exchange for the successful management of DIC.

PROGNOSIS Prognosis varies depending on the underlying disorder, and the extent of the intravascular thrombosis (clotting). Mortality in acute DIC is high and varies between 5080%.Prolongation of PT (>1.5 times) and APTT (>2,5 times)indicate poor prognosis.

NURSING MANAGEMENT:-

NURSING ASSESSMENT
Subjective and Objective data should be obtained from patient or parents SUBJECTIVE DATA:PAST HEALTH HISTORY :-recent hemorrhage, excessive bleeding, HIV infection, cancer (especially leukemia or lymphoma) systemic lupus erythematosus, cirrhosis, DIC, exposure to radiation. MEDICATIONS:-use of thiazide, diuretics, furosemide, aspirin, acetaminophen, estrogen, NSAIDs, penicilline, streptomycin,quinine, phenytoin and chemotherapy drugs. FAMILY HISTORY of bleeding problems and malaise,bleeding gingival,easy bruising.hematuria,dark or bloody stools fatigue, weakness, fainting, epistaxis, hemoptysis, pain and tenderness in bleeding areas, headache, menorrhagia, metorrhagia. OBJECTIVE DATA:General:-fatigue, lethargy Intugementary:- petechiae, ecchymoses, purpure Gastrointestinal:- splenomegaly, abdominal distension,

1.NURSING DIAGNOSIS:-Impaired oral mucous membrane related to low platelet counts as evidenced by oral bleeding and blood filled bullae. Goal:-Experiences lesion free oral mucosa without bleeding.

Interventions:-Monitor lips, tongue, mucos membranes, and gums for moisture, colour, texture, presence of debris and infection using good lighting and tongue blade to provide information for planning interventions. y Assist the patient to select soft, bland and nonacidic foods to decrease irritation of oral mucosa. y y Use a soft toothbrush for removal of dental debris. Use toothettes or disposable foam swabs to stimulate and clean cavity with minimal trauma to gingiva. y Instruct and assist patient to perform oral hygiene after eating and as often as needed to avoid breakdown of oral mucosa. y Avoid use of lemon-glycerin swabs to prevent excessive drying of mucosa.

2.DIAGNOSIS:-Risk for injury related to low platelet counts and treatments. Goal:-1.maintains tissue integrity 2.Has no evidence of bleeding or bruising. Interventions:y y Monitor for signs and symptoms of persistent bleeding to detect internal bleeding. Monitor coagulation studies,including prothrombin time(PT),partial thromboplastin time(PTT),fibrinogen and platelet counts to determine bleeding risk. y Avoid injections(IV,IM or surrounding puncture site. y Protect patient from trauma that cause bleeding to reduce tissue trauma and subsequent bleeding into tissue. y Administer blood products(e.g platelets,fresh frozen plasma)toreplace subcutaneous)to prevent bleeding into tissue

coagulation factors. y Teach patient to avoid aspirin or other anticoagulants to prevent additional bleeding risk.

3.DIAGNOSIS: Risk for infection related to exchange transfusion procedure Goal: To prevent the child from infection during and after transfusion Interventions: y y y y y y Assess the condition of the child Always use sterile techniques while starting transfusion. Clean the transfusion site with the aseptic technique. Send the sample of blood for laboratory diagnosis to detect the infection. Always do strict handwashing before touching the child. Check the vital signs regularly.

4.DIAGNOSIS:-Ineffective management of therapeutic regimen related to lack of knowledge of disease process, activity and medication as evidenced by frequent questioning about disease management,anxiety and restlessness. Goal:- verbalises required knowledge and skills to manage disease process at home. Interventions:y Appraise patients current knowledge related to specific disease process to plan appropriate interventions. y y y y Describe disease process. Describe common signs and symptoms of disease. Discuss treatment options to decrease anxiety and prevent complications. Discuss lifestyle changes that may be required to prevent future complications so patient will be knowledgeable and able to manage own care. y Refer patient to local community agencies /support groups.

GLOSSARY Fibrin- an insoluble protein that is essential to clotting of blood, formed from fibrinogen by action of thrombin Fibrinogen- a coagulation factor I, a glycoprotein; administered to increase the coagulability of the blood Fibrinolysin- plasmin, a preparation of proteolytic enzyme formed from profibrinolysin(plasminogen); to promote dissolution of thrombi Hemostasis- the condition in which the external and internal environment of a cell remains relatively constant Thrombin- an enzyme resulting from activation of prothrombin, which catalyzes the conversion of fibrinogen to fibrin. Thrombosis- formation,development or presences of a thrombus Thrombus- an aggregation of blood factors, primarily platlets and fibrin with entrapment of cellular elements, frequently causing vascular obstruction at the point of formation. Activated PTT- aPTT tests the intrinsic and common pathways D-dimer- an antigen formed as a result of plasmin lysis of cross-linked fibrin clots, documents the presence of thrombin Fibrin degradation product(FDP)- degradation products increase as plasmin biodegrades fibrinogen and fibrin,levels are elevated in 85-100% of patients with DIC Prothrombin Time(PT)- tests the extrinsic and common pathways Blood Components: used to correct abnormal homeostasis. Used to correct the clotting deficiencies caused by the consumption of blood components during the DIC process. -Platelets: contain platelet factor III, which functions as a mechanical plug

-Fresh frozen plasma (FFPs): used for volume expansion and contains clotting factors V,VIII,XIII and antithrombinIII. -Packed Red Blood Cells (PRBCs): used to increase RBCs and clotting factors. Used instead of whole blood to help with fluid overload and reduce development of antibodies. Cryoprecpitate: contains fibrinogen and factor VIII.

BIBLIOGRAPHY Wongs Essentials of pediatric nursing,Pp-956,Year 2007,Published byElsevier. Ghais Essential Pediatrics,Pp-329, Edition Sixth,Year 2005 Published byCBS. http://www.webmd.com/a-to-z-guides/disseminated-intravascular-coagulationdic-topic-overview11-10-11 http://emedicine.medscape.com/article/199627-c,11-10-11