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Drug Treatments

Drug Treatments

The major target of medical intervention has been the maintenance of blood pressure in the normal or low normal range and prevention of surges in blood pressure with the intent to minimise the likelihood of arterial dissection or rupture.

Over time, a variety of medications have been suggested that include diuretics, b-adrenergic blockers, angiotensin processing blockers or receptor blockers, and other antihypertensive agents that may depend on knowledge and experience of the clinician.

Celiprolol and Losartan are two drugs available in the UK for patients diagnosed with vascular EDS, both drugs are approved by NICE and available on prescription through the NHS.

Currently Celiprolol is not available in the United States, as it is not an FDA approved drug. They can be purchased online in Canada. 

There is some debate with clinicians that study and treat vascular EDS. Some clinicians feel that since Celiprolol is the only medicine that has been studied in vascular EDS that if you can be on Celiprolol you should be. Other clinicians feel that the Celiprolol study had flaws due to patients in the cohort not being genetically confirmed with the COL3A1 gene.

If you are considering drug therapy please talk to your cardiologist or genetic counsellor.
Celiprolol

Celiprolol belongs to a group of medicines called beta-blockers. It works by slowing your heart rate or lowering your blood pressure.

By reducing heart rate and pulsatile pressure, celiprolol may reduce the mechanical stress on collagen fibers within the arterial wall and be of benefit in patients with vascular type Ehlers–Danlos Syndrome.

The largest investigation of celiprolol in vascular Ehlers–Danlos Syndrome was prematurely terminated due to significant benefit with celiprolol in reducing arterial events in patients with this condition. Celiprolol, therefore, represents a β-blocker that is unique from others in its class in both its pharmacology and clinical applications. 

Celiprolol is approved in many countries for the treatment of cardiovascular disorders; however, it is not yet approved in the United States for any indication. Acer Therapeutics plans to conduct a clinical trial in the U.S. to evaluate the efficacy of a medicine known as celiprolol for the treatment of vascular Ehlers-Danlos Syndrome (vEDS).
Celiprolol
A Unique Selective Adrenoceptor Modulator
View Publication
Effect of celiprolol on prevention of cardiovascular events in vascular EDS: 
a prospective randomised, open, blinded-endpoints trial.
View Publication
Losartan / Irbesartan

Losartan belongs to a group of medicines known as angiotensin-II receptor antagonists, it is widely used to treat high blood pressure.

Angiotensin-II is a substance produced in the body which binds to receptors in blood vessels, causing them to tighten. This results in an increase in blood pressure. Losartan prevents the binding of angiotensin-II to these receptors, causing the blood vessels to relax which in turn lowers the blood pressure. 

Losartan helps to prevent future strokes, heart attacks and kidney problems. It also improves your survival if you're taking it for heart failure or after a heart attack. This medicine is only available on prescription. It comes as tablets.
Losartan reduces aortic dilatation rate in adults
with Marfan syndrome: a randomized
controlled trial
View Publication
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