Hereditary factor IX deficiency
Chapter 3:Diseases of the blood and blood-forming organs
ICD-10 D67 is a billable code used to indicate a diagnosis of hereditary factor ix deficiency.
Hereditary factor IX deficiency, also known as Christmas disease, is a rare genetic bleeding disorder caused by a deficiency of factor IX, a protein essential for blood coagulation. This condition is inherited in an X-linked recessive pattern, primarily affecting males, while females can be carriers and may exhibit mild symptoms. Patients with factor IX deficiency experience prolonged bleeding episodes, particularly after injuries or surgeries, and may also have spontaneous bleeding, especially into joints and muscles. The severity of the condition varies, with some individuals experiencing mild symptoms and others suffering from severe bleeding complications. Diagnosis is typically confirmed through laboratory tests that measure factor IX levels and assess the overall coagulation profile. Management of the disorder includes factor IX replacement therapy, which can be administered prophylactically or on-demand during bleeding episodes. Patients may also require supportive care and education regarding bleeding precautions. Regular monitoring and follow-up are essential to manage the risks associated with this condition effectively.
Detailed history of bleeding episodes, family history, and laboratory results.
Management of acute bleeding episodes, prophylactic treatment planning.
Documentation must clearly indicate the severity of deficiency and treatment response.
Genetic testing results, family pedigree, and counseling notes.
Genetic counseling for affected families, prenatal testing discussions.
Accurate coding of carrier status in females and implications for family members.
Used when obtaining blood samples for factor IX level testing.
Document the reason for blood draw and any relevant patient history.
Ensure that the specialty performing the procedure is noted.
The primary treatment is factor IX replacement therapy, which can be administered prophylactically or during bleeding episodes.
