Your lungs are one of the strongest muscles in your body, but they can also fall prey to disease. Pulmonary Arterial Hypertension (PAH) is labeled by the World Health Organization (WHO) as group 1 of five groups for pulmonary hypertension. The five classifications were created as a way to understand the underlying causes of the disease. The classifications also help researchers understand the varying statistics (such as morbidity, national mortality, etc.), so proper studies and treatments can be delivered. PAH requires diagnosis through catherization and a multitude of other tests. PAH causes those diagnosed with it to have their arteries that connect their lungs and heart to stiffen leading to higher blood pressure. Although the disease can be fatal, it can also be helped with the use of OPSUMIT (macitentan) by reducing the risk of progression from the disease.
What Is Pulmonary Arterial Hypertension?
Straight up pulmonary hypertension is a severe lung disease that is distinguished as high blood pressure in the pulmonary arteries (the arteries that bring blood to the lungs). As discussed earlier, there are five different groups of pulmonary hypertension. Pulmonary Arterial Hypertension (WHO Group 1) is associated with blood vessels narrowing in the lungs. This can include both cases where the underlying cause is known and not known. Some of the known underlying causes include heredity, certain drugs or toxins, lupus, congenital heart problems, high blood pressure in the liver, HIV, and sickle cell anemia.
Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) can be hard to distinguish from PAH and thus are included in the PAH classification. However, they do not respond to the same treatments as PAH does, which means OPSUMIT is not aimed at these patients. Speak with your doctor immediately about treatment for any Pulmonary Hypertension disease.
It’s also worth noting that newborn infants can also be born with PAH. However, when this is the case, it’s typically called persistent pulmonary hypertension of the newborn (PPHN). When present from this age, the treatment can be very different, though very effective. Most often they are treated with a mixture of oxygen and inhaled nitric oxide.
What is OPSUMIT?
OPSUMIT is an endothelin-receptor antagonist that reduces contraction and narrowing of blood vessels. This allows the body to lower blood pressure and resistance to blood flow within your lungs. This makes it an ideal treatment for PAH. When taken daily, patients were shown to have 47% lower resistance than before treatment after just 16 weeks.
Once taken, patients can see improvement in their ability to exercise, compared to their peers. When measured using the 6-minute walk distance, the study found the patients who took OPSUMIT walked 22 meters further on average than patients not taking OPSUMIT. It can also improve symptoms of PAH and slow progression of the disease. With all of this, OPSUMIT also helps patients lower their chance of needing to be hospitalized for PAH. For PAH patients who also have pulmonary vascular resistance (PVR), the combination medication also reduced those symptoms.
Actelion Pharmaceuticals Ltd, a Janssen Pharmaceutical Company of Johnson & Johnson aided in the testing of OPSUMIT, in combination with the PDE5 inhibitor tadalafil. They just revealed their findings of blood flow improvement to the lungs at the CHEST Annual Meeting in October. This study was conducted on 46 patients who were recently diagnosed with PAH with the goal to evaluate efficacy, safety, and tolerability of the combination medication.
Side Effects of OPSUMIT
During testing, the researchers found the combination of OPSUMIT and tadalafil was well-tolerated as a means to treat PAH. However, many patients did report some side effects during the process. One of the most common side effects shown in patients was peripheral edema, which is basically fluid build-up. However, the full list included a multitude of other side effects. Let’s take a look:
• Peripheral edema
• Headache
• Diarrhea
• Shortness of Breath
• Anemia
• Lack of Energy
• Nasuea of vomiting
• Pain in the upper right stomach
• Loss of appetite
• Skin or whites of your eyes turn yellow
• Dark Urine
• Fever
• Itching
Severe reactions were also found in 13 patients, causing three to discontinue drug treatment and three others died during the study. These symptoms they experienced included:
• Cardiac arrest
• Heart failure
• Multi-organ failure with sepsis
Despite these side effects, studies have found the combination of the two drugs to be beneficial to the patient’s overall well-being. And helped lower the risk of mortality for those who have PAH. If you’ve been recently diagnosed with PAH and looking into OPSUMIT, speak with your doctor about the risks and benefits of the medication. Be sure to note any and all side effects and bring them to your doctor right away.
Frequently Asked Questions About OPSUMIT
• How long does it take for OPSUMIT to work?
From the most recent study, we can see that there was a 47% decrease in resistance if taken on a regular basis. At approximately 6 months, patients were walking 22 meters further on average than those not currently taking OPSUMIT. After 2 years, improvement was maintained.
• How much does OPSUMIT cost?
At Canadian Pharmacy King, the cost of the 10 mg oral tablet is approximately $150 per tablet.
• Who cannot take OPSUMIT?
Women who are pregnant, plan to become pregnant, or become pregnant while taking OPSUMIT should avoid or cease its use immediately. You should not breastfeed while on this medication, either, as it may pass into breastmilk. This medication can cause serious birth defects. You should also speak with a doctor if you are currently on any other medications, vitamins, and supplements, as these may affect each other’s side effects. At this time, it is not currently known if OPSUMIT is currently safe or effective in children who have PAH.
• How do you take OPSUMIT?
The dosage for OPSUMIT is 10 mg taken once daily orally. This can also be taken with or without food but must not be chewed, crushed, or split. If you forget a dose, you will need to take it as soon as you remember and take the next at the normal time. However, do not take two at the same time if you miss a dose.
For patients suffering with pulmonary arterial hypertension, the diagnosis can feel daunting and scary. However, with new advances in treatments for PAH, such as OPSUMIT or the combination therapy with tadalafil, there is hope that you can go on to live your life with a lower risk of early mortality. Talk to your doctor about what medications and treatments might be right for you and be sure to bring up OPSUMIT in the discussion.
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