The popular diet drug fen-phen was one of the primary causes of PPH before is was taken off the market. Even though the drug was recalled, not much research went into understanding why fen-phen was causing cardiovascular problems. Now, small pharmaceutical firms think they have figured it out, and they are developing new obesity drugs designed to be safe.

Dr. Bryan Roth, a psychiatrist at the University of North Carolina, thinks that the fen-phen drug combination activated a cellular receptor known as 5-HT2b. That receptor is very close to another, which is known to suppress appetite. Roth thinks that 5-HT2b might be triggering the cardiovascular complications.

San Diego-based Arena Pharmaceuticals has developed a prototype which targets the related receptor without activating 5-HT2b. After 12 months of testing, no heart problems have arisen in the trial group. A company in Cleveland, Athersys Inc., claims they too have developed a diet drug that is even more selective than Arena’s. Though Arena is ahead in the development stage, it will be interesting to see where this race for pharmaceuticals will go.

In 2000, 16-year-old Cassie Tessier’s life completely changed. She was diagnosed with primary pulmonary hypertension (PPH) and is now waiting on a life-saving double lung transplant. In the meantime, she has not let her illness stop her from attending school and church, as well as spending time with her friends. Her health regimen includes a pump that sends medicine directly to her heart, blood transfusion, occasional use of oxygen, and pills four times a day. Cassie now suffers from two bleeding disorders as a result of the medications she takes for her PPH.

Doctors told Cassie last summer that she needs the double lung transplant. Since that time, Cassie’s bag is always packed as she and her mother wait patiently for the phone to ring. To help alleviate the costs of this expensive procedure, classmates and friends are hosting a pasta supper at the Easthampton High School on April 11th. While health insurance covers Cassie’s expensive medication and the procedure, Cassie and her mother may need to stay in New York for months after the transplant is over. For now, Cassie and her mom are anticipating the day the phone will ring, which will bring Cassie not just a new set of lungs but a whole new quality of life.

For more information about Cassie, click here.

Dakota Falls is just like any other 11-year-old boy who loves to play video games and spend time with his family. Unlike most children, however, Dakota is fighting for his life. In the summer of 2006, Dakota was diagnosed with pulmonary arterial hypertension, and he is currently at stage 4 - the most critical stage.

A pump sits on his waist all day pushing medicine through a tube into his chest. He can’t swim, run, or even walk down the hallway without becoming breathless. As his mother Terri says, Dakota will be up at night “sick as a dog” and never once complains. His strength and optimism is something that should inspire all of us.

Dakota is presently up for a lung transplant, which would improve his quality of life tremendously. His family, however, needs help with medical bills and other finances before Dakota can receive his new lungs. The Falls are hosting a benefit concert for Dakota on Sunday, March 30 in Waynesboro, VA - admission is free, but donations are appreciated.

For more information regarding Dakota and the benefit concert, click here.

For $195 million, Pfizer Inc. has decided to buy Encysive Pharmaceuticals Inc. with the intention of pushing the drug Thelin into the United States. Thelin, which has been approved in Europe but not yet in the US, is used to treat primary arterial hypertension – an often fatal condition, distinguished by extremely high blood pressure in the arteries between the lungs and heart.

Pfizer plans to conduct a “Phase III trial” of the drug so it will soon be authorized by the FDA. Thelin is a once-daily pill which blocks receptors to the protein endothelin, which causes the tightening of the blood vessels. Since Thelin inhibits these receptors, it prevents the vessels from contracting, allowing blood flow.

It could prove to be exceptionally beneficial for primary arterial hypertension sufferers if Pfizer is able to launch Thelin in the United States.

Full article here.

Oliver Sherwood, a two-year-old from the UK, suffers from pulmonary hypertension. To control the disease, he takes four doses of Viagra, also called Sildenafil, a day, which helps improves Oliver’s blood flow. Even though Viagra is more famous for treating erectile dysfunction, it can help open the veins and capillaries to improve circulation. While the condition is very rare in children, it is also fatal: the survival rate is only about five years, even with medication.

Even though Viagra can be expensive, it is still one of the cheaper drugs out there to treat PH. Impending cuts in health expenditures, however, raise fears that he may need to switch to more expensive treatments. The Government’s drug rationing agency, the National Institute for Health and Clinical Excellence, is considering whether to continue prescribing the drugs and Oliver’s mother has started a petition calling for the Health Service to keep funding the treatments. A spokesman has said there is evidence that Viagra is “clinically effective and cost-effective in treating PAH.”

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Alicia House was an avid hiker and swimmer until 2004, when she was told she might be an asthmatic. But as her condition worsened to the point were House couldn’t even work, she started looking for answers to her “asthma.” From her research, she discovered that she displayed the symptoms of pulmonary hypertension – an often misdiagnosed disease.

Since symptoms are vague, they can be associated with other illnesses. Breathlessness, chronic fatigue, swollen ankles, legs, and abdomen, chest pain, and fainting are all indications of pulmonary hypertension. As House points out, many asthmatics that have had “poor control of their disease” are now finding out they have pulmonary hypertension. Pulmonary Hypertension is a progressive and fatal disease with continuous high blood pressure that can result in an enlarged heart.

There are two types: primary and secondary, and it’s a rare disease with only one-to-two people per million diagnosed. Treatment options do vary depending on the progression of the disease, but there is no known cure for PH. Almost 50 percent of patients die within three years of their diagnosis. House is advocating for the passage of the PH Research and Education Act, which will open up more research options, prepare physicians for treatment, and increase public awareness.

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Looked at one way, a spike in hospital stays for a rare and deadly lung disease is a bad thing. Or it may reflect progress in diagnosing and treating a disease that has puzzled medical science for at least a century. Which is it?

Whatever the cause, the findings are troubling, because pulmonary hypertension is so often fatal. If untreated, about half of patients die within three years. The disease occurs when there’s too much resistance in arteries that carry blood to the lungs from the right side of the heart, typically in patients with heart failure, chronic obstructive pulmonary disease and pneumonia. In about 1.5% patients, doctors can’t identify a cause.

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