Thirteen-year-old Nick LeGrand, like many other teenage boys, is a huge sports fan. He would love to actually participate in sports like baseball, basketball, and football, but is unable to because he suffers from primary pulmonary hypertension. Nick is attached to an oxygen tank twenty-four hours a day and only has nineteen percent of his lung function. Nick is constantly out of breath, making it very difficult for him to walk short distances let alone play a competitive sport.

Nick was diagnosed with PPH when he was just three-years-old, at which time he was lucky enough to receive a double lung transplant. Unfortunately, just ten years later, the lungs are beginning to fail. Nick is currently experiencing severe chronic rejection of the lungs. As Nick puts it, “[his] lungs are saying, ‘Hey, these aren’t my lungs,’ and fighting against them.”

Nick was on a transplant list back home, but the hospital lost their certification. He and his mom now wait for the lifesaving call in Houston, Texas. They have not seen the rest of their family in six months. This is a tragic situation that Nick and his family find themselves in, however they are sticking together, keeping their spirits high and waiting for that life altering call.

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Italian researchers have reported that the drug bosentan has reduced the clinical decline in patients suffering from early stages of pulmonary arterial hypertension. Previous studies had shown that bosentan improved exercise capacity and pulmonary vascular resistance (PVR) and slowed clinical decline in patients with later stages of PAH.

The researchers conducted this most recent study using one-hundred and eighty-five people ranging from age twelve and up. They all had early-stages of PAH and were separated into two groups. One group received the drug bosentan and the other received a placebo. The patients’ PVR and distance they were able to walk in six minutes was measured at the beginning of the study then again six months later. At the end of the six months, the PVR mean improved significantly in those taking bosentan versus those on placebo. Three percent of patients on bosentan and fourteen percent of those on placebo experienced clinical worsening, some as severe as hospitalization and even death.

Although this study needs more research and data to support its initial findings, the initial results indicate that bosentan may be an effective and useful drug in battling this difficult disease.

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The use of stem cell therapy for people suffering from Pulmonary Hypertension is becoming a reality. At the Jewish General Hospital for Pulmonary Vascular Disease in Montreal they have begun treating patients with Pulmonary Hypertension with their own modified stem cells. This clinical study is unique because it is the only one in the world using genetically modified stem cells.

The stem cells used have been genetically engineered with synthetic DNA in an extremely specialized lab, in order to produce nitric oxide. This chemical compound is a crucial molecule involved in the repair and protection of blood vessels. These enhanced stem cells are administered in a heart catheterization suite, and they lodge in the lung where hopefully they will stimulate repair and regeneration of blood vessels in the lung.

The procedure has cured many laboratory rats and doctors hope it will be as successful with humans. They are moving in the right direction and hopefully they will find an end all cure for this tragic disease.

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The amount of pregnant woman using antidepressants has increased substantially through out the past 10 years. There has been much debate about whether it is safe for a pregnant mother to take medications like Paxil, Prozac and Zoloft. The U.S. Center for Disease Control (CDC) recently told Americans that they didn’t need to worry about the effects of these drugs on newborns. However, the actual research suggested something very different.

In 2006 it was reported that 13% of infants who were exposed to antidepressants while in utero had withdrawal symptoms. More seriously then withdrawal symptoms however, is the report released by the FDA also in 2006. They found that infants born to mothers who took selective serotonin reuptake inhibitors (SSRIs) after their 20^th week of pregnancy were 6 times more likely to have Persistent Pulmonary Hypertension (PPHN) than infants born to mothers who took no antidepressants during pregnancy.

When a newborn has PPHN their arteries to the lungs constrict, limiting the blood flow to the lungs. This also limits the amount of oxygen in the bloodstream. The FDA also stated that neonatal PPHN is strongly linked to morbidity and mortality among the infants. These findings hopefully are enough to keep mothers from endangering their infants by taking harsh medications like antidepressants during their pregnancy.

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Dr. Jean-Paul Clozel is responsible for developing Tracleer, a drug that allows patients with Pulmonary Arterial Hypertension to live a longer healthier life. Although it is not a cure to this disease, it nonetheless improves the quality and length of life for those battling PAH. Jean-Paul (he prefers to leave out the Dr. prefix) spent 12 years researching at the pharmaceutical company F. Hoffmann-La Roche located in Switzerland. This is there where he developed the first T-channel blocker (Tracleer) to help treat Hypertension.

In 2007 Tracleer had more than $1 billion in sales. Around 20,000 people all around the world with PAH have been treated with this medication. He currently has 12 drugs in clinical development for many diseases, including a new drug for Pulmonary Hypertension. The Clozel’s were determined to improve the lives of those suffering from disease, and I think they are on the right path. I hope that soon we will find an end all cure for this disease, and Jean-Paul undoubtedly instills hope that it is possible.

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According to a new study by the University of Pennsylvania, patients suffering from Primary Arterial Hypertension (PAH), have a very poor chance of survival and a high rate of right-heart failure when their serum sodium levels (called hyponatremia, or HN) are low. Paul R. Forfia, M.D. at UPenn said, “This is the first study to show the powerful prognostic significance of low blood sodium in these patients.” Since serum sodium is easily measured, understanding the importance of HN in patient outcomes could make it a key clinical marker in patients with PAH.

The study was conducted by examining the blood sodium of 40 patients with PAH. The results found that 13 patients had sodium levels less than 136 mEq/L. The patients whose HN was below normal had significantly poorer kidney function, and were three times as likely to have lower extremity edema, with evidence on echocardiography of significantly worse right heart function. The most remarkable thing came when the doctors performed the follow up. According to Dr. Forfia, “All ten patients with a serum sodium less than 136 mEq/L died, whereas all 16 subjects with sodium concentration of 140 mEq/L or greater survived.”

The generalized findings of the report reads, “Serum sodium has important implications regarding right heart dysfunction, clinical right heart failure, and patient outcome and should not be overlooked in the clinical assessment of patients with PAH.”

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Gilead Sciences of Foster City, California announced last Thursday, the purchase of Cicletanine from Navitas Assets for an undisclosed amount. Cicletanine which has been approved by several European countries is an oral, once-daily agent used in the treatment of Pulmonary Arterial Hypertension (PAH).

Gilead is awaiting the approval of Cicletanine from the FDA for use in the United States. However Gilead is planning to begin Phase II testing by the end of this year. Gilead believes that Cicletanine will provide the needed assistance for people suffering from PAH in the U.S. based on its success overseas. Gilead President and Chief Operating Officer John F. Milligan said “Our agreement with Navitas is reflective of Gilead’s commitment to building a pipeline of therapies for people living with PAH including treatments with potentially novel mechanisms of action.”

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