Madisons Foundation - Moms And Dads In Search Of Needed Support

What

In the body, there is normally a balance between bleeding and clotting. When an individual is affected by a severe illness or infection, this balance can be disrupted. There may be excessive bleeding because clots are not maintained or due to excessive activation of the clotting pathway. This condition is known as disseminated intravascular coagulopathy (bleeding problem) or DIC. DIC is a final common pathway and is commonly described as a consumptive coagulopathy. In order to combat the numerous small clots that form throughout the body, anti-clotting factors are consumed leading to a bleeding tendency. This usually occurs with a fairly quick onset and for a short period of time (acute DIC) but can also go on for a longer duration (chronic DIC) which is usually associated with prolonged illness such as cancer.

Who

Any person can develop DIC regardless of race, age or gender. DIC most commonly occurs in critically ill patients such as those with life-threatening infections (sepsis), but can also occur in the setting of injury or trauma, as a complication related to pregnancy, or with cancer.

Signs and Symptoms

1. Bleeding is seen in up to 2/3 of patients with DIC and usually is at puncture sites on the skin, such as surgical or IV sites. Bleeding may also occur under the skin in the form of bruising or petechiae. Petechiae are small red hemorrhagic spots in the skin or mucus membranes. There may be bleeding within the intestinal tract with blood noted in the stool or with vomiting, and bleeding in the lungs and kidneys may result in blood in the sputum or urine respectively. 2. Clotting in the small blood vessels (thrombosis) can occur in any part of the body. When this occurs in the kidneys, the lack of blood and oxygen can lead to tissue death (necrosis), kidney failure and shock. Similarly, clots in the brain can lead to tissue necrosis, loss of brain function, and confusion. Clots in extremities can result in decreased blood flow to the fingers and toes. Low oxygen levels in the tissues are associated with severe muscle, back, abdominal and chest pains. 3. Complications related to both the bleeding and the clotting can occur and include shock, kidney failure, stroke and multiple organ failure.

Possible Causes

It is unclear why certain disorders lead to DIC, but in many patients, the trigger may be the entrance of a foreign protein (ie bacteria) into the blood circulation or injury to the blood vessel. Acute DIC may occur after severe infection. In addition to infection, other conditions where there is tissue death such as in burns, trauma, or after organ transplantation may activate the body’s clotting system. These patients should be closely monitored for bleeding complications. Other situations such as heat stroke and respiratory distress syndrome may also predispose patients to DIC. Chronic DIC results with prolonged illnesses such as cancer (especially acute promyelocytic leukemia), liver cirrhosis, and hemangiomas (collection of malformed blood vessels). It can also occur as a complication of pregnancy in the setting of pregnancy-induced hypertension (elevated blood pressure), pre-eclampsia, following a miscarriage or when fetal tissue remains inside the uterus. Chronic DIC tends to be subtler clinically but may still need to be treated.

Diagnosis

DIC is usually suspected when a patient begins to have uncontrolled bleeding or clotting. There is no one test to diagnose DIC, however there is a characteristic lab profile. This includes: 1. decreased platelet count or thrombocytopenia (platelets are cells which help make clots); 2. prolonged prothrombin time (PT) & partial thromboplastin time (PTT) (measures the effectiveness of clotting factors); 3. low fibrinogen, factor 5 and 8 levels (specific clotting proteins in the blood); 4. elevated fibrin split products and d-dimers (byproducts of clot breakdown); 5. Blood smear shows fragmented, abnormally-shaped red blood cells, which signify damage in small blood vessels. In a patient with chronic DIC, there may be fewer abnormalities on the above laboratory studies because it is a slower process and the body may be able to begin compensating for the loss of some of the clotting proteins.

Treatment

The main treatment for DIC is to treat the underlying illness or medical condition. If bleeding is significant, blood products such as fresh frozen plasma (FFP), platelets, or packed red blood cells (PRBCs) can be given to both replace the depleted clotting factors and also to replace lost blood. If the amount of fibrinogen is extremely low, it is sometimes necessary to give cryoprecipitate, which is a special preparation of blood plasma that has high concentrations of fibrinogen and factor 8. Heparin, which is medication that keeps blood from clotting, may be useful in the early stages of DIC to prevent clots, but in general is not effective in most patients.

Prognosis

The ability to survive DIC depends on early detection of the condition, the severity of the bleeding and clotting imbalance, the tissue damage caused by the clots, and how the patient responds to treatment. Focus on treating and controlling the underlying condition is essential.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Patient Plus from EMIS
A webpage from the UK organized similarly to the one above with some additional information about the causes of DIC.

Merck Manual
This article contains additional scientific information/explanations for DIC.

Henry Ford Health System
A nice summary with pictures of DIC and diagrams of the clotting system.

Healthopedia.com
Nice overview of the mechanism of DIC.

University of Alabama Health Sciences
Good links to support for parents.

Google Search for Disseminated Intravascular Coagulopathy (DIC)

References and Sources

Handbook of Pathophysiology, Springhouse Publishers, c.2001p.382-4 Leung, L. “Clinical features, diagnosis, and treatment of disseminated intravascular coagulation.” UpToDate. www.uptodate.com. Accessed September 10, 2005. Marino, BS. USMLE: Blueprints in Pediatrics. c.1998, p.116-7 McCance, K. Pathophysiology: the biologic basis for disease in adults and children 4th Edition, Mosby publishers, c.2002. p.891-4 Emedicine.com: http://www.emedicine.com/emerg/topic150.htm