Pharmacy Program

Delivering Integrated Care and Cost Management

The IHTC works collaboratively with payors to optimize care. We ensure that the patients and families we serve have access to care and therapies, thereby helping to contain costs and reduce both bleeding events and utilization of resources.

The IHTC Pharmacy’s ability to purchase clotting factor through the Public Health Service 340B discount program and our overall pricing structure benefit payors and patients by dispensing clotting factor at significantly reduced prices.

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Signs and Symptoms of Thrombosis

The clinical spectrum of venous thromboembolism (VTE) ranges from deep vein thrombosis (DVT) to pulmonary embolism (PE). The symptoms of VTE depend on the location of the affected vessel and whether the vessel is totally or partially occluded by the clot.

Clinical Spectrum of VTE

Deep vein thrombosis (DVT)

  • Blood clots may form in the deep blood vessels, most commonly in the legs and groin, and can block normal blood flow returning from the legs to the heart.
  • Venous clots that form in regions of slow to moderate flow are composed of a mixture of red cells, platelets, and fibrin and are known as mixed platelet fibrin thrombi.
  • Partially occlusive venous thrombosis of the deep veins in the legs or abdomen may present with subtle symptoms and sometimes may not present until significant collateral circulation* has developed.

Pulmonary Embolism (PE)

  • PE results from a piece or all of a blood clot that breaks off and is carried by the blood stream to the lung where it obstructs the blood vessel.
  • The size of the clot and the site of the obstruction of blood flow in the vessel determine the extent and severity of the pulmonary embolus.
  • Proximal vein thrombosis is more likely to lead to fatal PE as compared to calf vein thrombosis.
  • The incidence of fatal PE can be markedly reduced if DVT is treated with anticoagulant therapy.

Superficial Thrombophlebitis

  • Superficial thrombophlebitis is due to blood clots that form in veins that are closer to the surface of the skin and are associated with inflammation.
  • Superficial thrombophlebitis is often observed in individuals who are heterozygous or homozygous for the factor V Leiden mutation.

Signs and Symptoms

Deep Vein Thrombosis

  • Pain
  • Swelling of the affected extremity/area with erythema and warmth over the vicinity of the clot
  • Discoloration including a bluish or suffused color

Signs and symptoms on physical examination

  • Positive Homan’s sign: pain with dorsiflexion of the foot
  • Swelling
  • Pain on palpation
  • Presence of a palpable cord
  • Evidence of collateral circulation, usually manifested by increased prominence of superficial veins
  • Some people with a DVT may be asymptomatic

Pulmonary Embolism
Pulmonary emboli may present subtly with the following complaints listed in order of frequency:

  • Dyspnea
  • Rapid breathing, fast heartbeat and chest pain especially with inhalation
  • Pleural pain: Some patients notice only a dull ache in their chest
  • Apprehension, anxiety
  • Cough
  • Hemoptysis
  • Sweats
  • Syncope
  • Fatigue

Signs and symptoms on physical examination:

  • Tachypnea
  • Tachycardia
  • Rales
  • Fever
  • Sweating
  • Thrombophlebitis
  • Accentuation of the pulmonary closure sound
  • Gallop heart sound
  • Cyanosis
  • Some people with a PE may be asymptomatic

Signs and Symptoms in Thrombophilic Conditions

Pregnancy loss

Inherited Deficiencies

  • Mesenteric vein thrombosis is a rare but characteristic thrombotic episode of inherited thrombophilia
  • Family history of thrombosis
  • First thrombosis at a young age, often younger than 40 years
  • Frequent recurrence
  • Neonatal purpura fulminans is a rare condition associated with homozygous protein C and S deficiencies
  • Most patients with inherited or acquired thrombophilia may be asymptomatic for a significant period of time; diagnosis may result from either a personal or family history suggestive of thrombophilia

Superficial Thrombophlebitis

  • These clots often partially block blood flow in affected veins and may cause pain and irritation.
  • Redness and inflammation along the vein may occur; if hard and erythematous, the affected vein is often visible and most commonly occurs in the legs or arms.
  • Other associated symptoms include warmth and tenderness, surrounding purities and swelling.
  • Pain along the vein: patients may report a throbbing or burning sensation beneath the skin’s surface; these symptoms may interfere with sleep as they progress.
  • Fever: Patients with venous inflammation may develop an elevated temperature associated with an episode of thrombophlebitis.

Hyperhomocysteinemia

  • Clinical manifestations of hyperhomocysteinemia are similar to those associated with deficiencies of other thrombophilic disorders.
  • Reported rates of thrombotic events in cases of significant hyperhomocysteinemia include
    • DVT either with or without PE: ~64%
    • Superficial thrombophlebitis: ~24%
    • Thrombosis of cerebral or mesenteric veins: ~12%
  • Thrombotic manifestations are often associated with the presence of precipitating factors such as use of oral contraception, trauma/surgery, pregnancy, the puerperium, and immobilization.
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