New York-based singer, songwriter and musician Chloe Temtchine has won rave reviews and awards for her solo work and collaborations with other top music industry artists. On March 29, 2014, Temtchine’s new song, “Be Brave,” will be released on iTunes, with fifty percent of the proceeds benefiting PHA. Diagnosed with pulmonary hypertension in 2013, Temtchine performs with her oxygen tank, which she has dubbed “Steve Martin,” alongside her. Community spoke with Temtchine about life, music and pulmonary hypertension.
When did you start singing and songwriting? Who and which styles are some of your biggest influences?
I started singing at about the age of six. My father used to take me to a Baptist church in Harlem, on Sundays, where I listened to gospel music for hours. That’s where it all began.
I’ve always had very eclectic taste in music: from artists like Edy Phenomene (French dancehall), to Smokie Norful and Kim Burrell (gospel), to Stevie Wonder and Sam Cooke (R&B/soul), to James Vincent McMorrow and Ray Lamontagne (singers/songwriters), to Eric Reed (jazz). The list could go on forever.
When and how were you first diagnosed with pulmonary hypertension? What were your initial symptoms?
In March 2013, after my cardiologist reviewed an echo and listened to my heart, I was diagnosed with severe pulmonary hypertension and was rushed to the ER. The shortness of breath, lung pain, and fatigue that had started five years previously had become progressively worse, and I had reached a point where I could barely move. Getting to the bathroom was a major accomplishment! Then my heart started beating out of my chest. And then, together with the continued chest pain that accompanied every breath, I suddenly put on 10 pounds of water weight overnight.
What is “Be Brave” about?
After being diagnosed, I found myself spending so much time trying not to die that I had forgotten to live. “Be Brave” is about making the choice to live.
Every time I went to the hospital, I came home feeling that there was very little hope. I realized that if I didn’t shift my consciousness quickly, my condition would continue to get worse.
I’m a big believer that our minds play a huge role in the state of our health. Keeping my mind in the right place is so important to me that I decided to write a song about it. I wanted to remind myself, and anyone else going through a similar experience, that any challenge had the potential to be an opportunity.
What inspires you in songwriting?
I’m much more comfortable expressing myself through song. Music captures the way I feel in a way that words without melody can’t seem to do.
Although I would do just about anything not to have pulmonary hypertension, I’ve understood through this process that there are many lessons I’m meant to be learning. I am very grateful for the perspective it has given me with regard to my life.
My biggest challenge was seeing a future when I was told that it was highly unlikely that I would have one. This continues to be my biggest challenge.
What do you think is most important for the newly diagnosed to know about pulmonary hypertension?
For the newly diagnosed, I would say: Surround yourself with positive people who instill hope in you, eat a very healthy diet (plant-based, if possible), go to pulmonary therapy, focus on other people’s success stories, and most importantly, believe that it is possible to get better no matter what you’ve been told.
How does being a singer, musician and songwriter help you deal with pulmonary hypertension?
I think that being inspired and passionate in general is helpful to anyone. I also think that it’s very important to express yourself in order to keep yourself in balance. Through music, I’m able to get out all of the things that I would otherwise potentially keep in.
What is next for you in terms of treatment for PH?
I’m not totally sure at the moment. Because there is a belief that I may have pulmonary veno-occlusive disease in addition to PH, my doctor is going very slowly with the medications, which I’m very grateful for. Lung transplantation has been suggested, and although I’m thankful that it exists as an option, my goal is to stay away from it, if at all possible.
What’s next for you in music?
My next goal in music is to finish writing the album I began when I got out of the critical care unit and to perform as much as possible.
What do you enjoy most about music?
I love that music has always had the ability to completely alter the way I feel. It has helped me be hopeful during very difficult times. I love the idea of creating something that could not only alter my own state, but that also has the potential to alter the state of someone else who may be in need of some state-altering!
—ELPosted in About Us, Diseases, Featured, Uncategorized | Tagged be brave, chloe temtchine, Lung Transplant, music, PH, PHA, Pulmonary Hypertension, Pulmonary Hypertension Association, Pulmonary Veno-Occlusive Disease, singing, veno occlusive | 3 Comments February 28, 2014
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Thomas L. Spray, M.D., is chief of the Division of Cardiothoracic Surgery at The Children’s Hospital of Philadelphia, director of the hospital’s Thoracic Organ Transplantation Program, and Professor of Surgery at the University of Pennsylvania School of Medicine. Community recently interviewed Dr. Spray about pediatric lung transplantation.
What are the most important considerations for parents when making decisions about pediatric lung transplants?
The fundamental issue here is that, lung transplantation is, unlike heart transplantation, not associated with as good long-term survival. In some ways it’s somewhat palliative. Five-year survival for lung transplant is about 50 percent. And despite lung transplants being around now for about 25 years, there hasn’t been a lot of improvement in survival.
The lung is an immunocompetent organ, unlike most other organ transplants. It has the disadvantages of being connected to the outside, so it’s always at risk of infection. Also, there are lymph nodes in the lungs, which probably make them more susceptible to rejection and latent chronic rejection, which is where most lung transplants eventually fail; something called bronchiolitis obliterans—scarring of the small airways of the lungs—which is thought to largely be a chronic rejection problem.
Having said that, there are isolated patients who do extremely well and do well over the long term, but if you just take the averages, the average survival is about five years. So that’s the disadvantage.
On the other hand, for many patients, especially pediatric patients, their survival is going to be measured in a matter of a year or less if they don’t get a lung transplant. So it’s successful, but it’s not perfect by any means. It’s generally very much palliative for children as well.
To what degree should the child be involved in the decision?
To what degree the child should be involved in the decision is somewhat age-dependent. When you have a lung transplant, you’re basically exchanging one disease for another one, because you then have to be on medicines to prevent rejection, and you have a certain amount of medical surveillance necessary.
Unfortunately lung transplantation is still a procedure that has a significant risk associated with it. The lungs cannot work sometimes. The outcome cannot be positive if you get a viral infection of the transplant early on—it can destroy the lungs. And that’s very hard for anyone to survive.
I can remember some patients though, with cystic fibrosis, after lung transplant, even when the lungs went bad, and they ultimately died, having said during the time they were alive, that it was worth everything just to breathe normally for a change. So you have to put it in perspective.
When you get into the teenage years, I think it’s appropriate for the child to be involved in the decision. I think it’s especially important for teenagers, who unfortunately have a fairly high risk of not being compliant with medical management, and then they reject their organs and die.
Just being an adolescent is a risk factor for transplant, it appears. It’s a difficult time. That’s why when you’re talking teenagers, it’s very important that they be involved in the decision process, so that they recognize that they have to be involved in the medical management. They have to take their medicines faithfully. They have to be seen frequently. And if they aren’t willing to do that, then it’s kind of foolish to go down that pathway.
What have been some of the advances in pediatric lung transplantation?
Patient room at The Children’s Hospital of Philadelphia
Most of the advances in transplantation, in terms of survival, over the last 15 years, have been at the early period after transplant. In other words, we’ve been able to transplant more complex patients, who’ve had multiple previous operations, who were sicker waiting for transplant.
We’ve evolved to the point where it’s now possible to support patients on an assist device called ECMO while waiting for transplant and still have them able to be up and around and maintain their ability to breathe, so that it makes it easier after the transplant. So there are some advances, but there haven’t been any major advances in immunosuppression.
We have experience now transplanting newborns all the way up to adults. We’ve had experience with many kinds of complex congenital heart disease and lung transplants. We have experience in pulmonary hypertension. Those are the more common causes in children.
I think it’s important to note that in the pediatric world, the most common indications for transplant are considered the higher risk indications in the adult world. For example, in adult lung transplant, the most common indication is emphysema. Emphysema is a more straightforward condition to treat with lung transplant than any of the diseases that we see in children, and emphysema is virtually unheard of in children, so pediatric transplant by its very nature is a higher risk population.
What percentage of pediatric transplant patients have pulmonary hypertension?
At The Children’s Hospital of Philadelphia, at least 50 percent or more have congenital heart disease or pulmonary hypertension, and relatively fewer have cystic fibrosis. Part of that is because the management of cystic fibrosis has improved over the years such that there are relativity fewer children who require transplantation for cystic fibrosis than in the past. So, most people with cystic fibrosis can get to adulthood before they have enough lung deterioration to require transplant, and therefore get transplanted in adult centers now.
When we first started doing lung transplant in about 1990, when I was in St. Louis, cystic fibrosis was a common indication, because children under 18 with severe cystic fibrosis would come to St. Louis for transplant. In Philadelphia, we see less of that and more pulmonary hypertension and congenital heart disease.
What drew you to the lung transplantation field?
I started out as a congenital heart surgeon. Congenital heart surgeons all have to be adult heart surgeons first. I did adult and congenital heart surgery at St. Louis Children’s Hospital. I was recruited to come here to take over from William Norwood, a very well known surgeon, who went to Europe to start a new program.
When I came to Philadelphia, there wasn’t a lung transplant program in this area. So I started a pediatric lung transplant program when I came in 1994, having started the program in St. Louis in 1990.
I think my interest in lung transplant came from patients I saw in St. Louis who had no real, good option for repair of their heart, because they didn’t have good lungs to push blood into. There are certain types of congenital heart disease where that’s the case, where you could repair the heart defect if you had pulmonary arteries to pump blood into.
But children who don’t have that became progressively more cyanotic. I saw some of these children and I thought, ‘If we could just put new lungs in, we could fix the heart.’ So that’s what got me interested initially, and then that expanded to all the other potential reasons for lung transplant, of which there are many.
What are some of the differences in quality of life after a successful pediatric lung transplant?
Art therapy at The Childrens Hospital of Philadelphia
Quality of life depends on the patient’s condition before the transplant, but the majority in the pediatric population are extremely debilitated. They often have cystic fibrosis. They’re chronically infected. They have poor lung function. They have very little exercise tolerance. Patients with PH may have heart failure also. So the quality of life prior to transplant is very poor.
The waiting times are so long for lung transplant that most children deteriorate significantly while waiting in the hospital. They’re sometimes waiting in the hospital for a year or more.
So the quality of life after transplant, while vastly improved, takes a while for them to recuperate. I think what people often don’t realize is that if you’re sick for months and months prior to lung transplantation, there’s a lot of rehabilitation necessary. Even after a successful lung transplant, it’s not like patients are going home in a week. Many of them have to stay in the hospital for months while they’re literally recuperating and rehabilitating themselves from being chronically ill for the previous several years.
How much interaction do your patients have with the Child Life, Education and Creative Arts Therapy department at The Children’s Hospital of Philadelphia?
They have a great deal of interaction, especially while waiting. Children who are sick and in the hospital and literally waiting for sometimes months and months for organs to become available need that kind of interaction. They need to have play.
They need to have creative interactions, and that’s why the Child Life Department is so important for all the patients in the hospital, but even more important for those who are chronically in the hospital for long periods of time. They need to have that kind of stimulation. They need to be able to be involved in arts and crafts to keep them occupied, literally, to help them develop while they’re waiting.
What are some ways that families can cope with being on a waiting list?
Everyone, I think, does that differently. I think it’s always difficult for families to recognize that a waiting list is exactly that. You have no idea when organs might become available.
There are times when organs are available, and we tentatively accept them, and then they deteriorate to the point that we can’t use them. So the families have situations where their hopes are raised that they’ll have a transplant, and then it falls through, and then they’re back waiting again. So it’s a difficult thing for the families.
I think the families need to recognize that donor organs are hard to come by; they’re very scarce. The organ system is as fair and open as people can make it. It’s constantly trying to be as completely open and straightforward as possible and to not disadvantage anybody and make everybody on an equal playing field. But these are difficult things sometimes.
Transplantation in some ways is kind of a fundamentally flawed strategy, because you have to have a tragedy to have a miracle. Someone has to die for organs to be available, and as a physician and surgeon, I don’t want anyone to die. In a way, I don’t want there to be more donors.
On the other hand, I think the donors who are available should be used as maximally as possible, because there are so many children who need the organs. It’s a constant battle to try to use everyorgan you possibly can. But then some of them have problems and don’t work. That’s unfortunately the chance you take.
What programs are in place at The Children’s Hospital of Philadelphia to help families afford the cost of pediatric lung transplants?
Art Therapy at The Children’s Hospital of Philadelphia
There’s a whole process before someone is listed for transplantation that involves evaluating their financial situation. The hospital has many programs to try to get them into programs that will provide coverage. Because it makes little sense to do a lung transplant if you have no coverage for medications after the transplant, which happens in some crazy insurance arrangements.
The hospital sometimes will help families get Medicaid or some other government program that will at least provide them with follow-up medications and follow-up care. There’s a whole financial counseling group that works with families in relation to transplant, because you have to recognize it’s not a one-time deal. It’s like a new disease, if you will, transplantation.
What else is important to stress about how pediatric lung transplants are different from adult transplants?
The diseases are different, and they’re more complex. Many of the [pediatric] patients have had previous surgeries, which complicate the transplant significantly, in terms of bleeding and other issues.
I’ve said many times that the hardest part of transplantation is not putting the new organs in; it’s getting the old ones out. That can be extremely difficult due to the scarring and inflammation and previous infections. It can sometimes be extremely difficult just to get the old organs out without damaging other important structures. They can all be stuck together in the chest.
That’s one major challenge with lung transplant, especially in children. It’s not as common in adults. Most of the adults with emphysema have not had significant previous surgeries; they don’t have a lot of extra blood vessels.
Many of the children who need transplant are also ‘blue.’ They’re cyanotic. They don’t have normal oxygen levels. That stimulates development of blood vessels in the chest that can be very difficult to control at the time of transplant, and bleeding is much more of an issue.
Then of course we’re dealing with different sizes. In adults, they’re using mostly adult lungs and some teenage lungs. But in pediatric transplantation, we have to list patients in a very discrete size range, because we do transplants in patients all the way from newborns up to adult-size teenagers. So we have to have the ability to put very small lungs in small children.
We do occasionally use lobes or parts of lungs from adults in small children, which is something the adult world rarely, if ever does, because we have to deal with this wide size range.
FOR IMMEDIATE RELEASE
Lundbeck’s SABRIL® (vigabatrin) Now Approved by U.S. FDA as an Adjunctive Treatment Option for Children 10 and older with Refractory Complex Partial Seizures
Deerfield, Ill., October 28, 2013 – The U.S. Food and Drug Administration (FDA) approved SABRIL (vigabatrin) as add-on therapy for the treatment of refractory complex partial seizures (CPS) in children 10 years of age and older who have inadequately responded to several other treatments and if the possible benefit outweighs the risk of vision loss.1 This approval expands upon the age range of SABRIL’s previous indication as adjunctive therapy for adults with refractory CPS. SABRIL is not indicated as a first-line agent for refractory CPS.
Of the more than two million Americans affected by epilepsy,2 approximately 35 percent have CPS, which originates from a single region of the brain and can cause impaired consciousness.3 Approximately 30 to 36 percent of those with CPS continue to have seizures despite trying multiple therapies, and are considered to have refractory CPS.4,5,6
“It is crucially important for people with challenging seizures like refractory CPS to not give up and continue striving for improved seizure management, and this expanded Sabril indication provides another consideration for the treatment of those ten and older with refractory CPS,” said Philip Gattone, president and CEO of the Epilepsy Foundation. “We encourage people living with such challenging seizures and their loved ones to have ongoing conversations with their doctor about available options to help manage this intractable seizure disorder.”
When SABRIL was first approved in 2009, a patient registry was established to collect information on all patients who are prescribed SABRIL. To date, more than 5,600 patients have been treated with SABRIL, a substantial number of whom have been treated for refractory CPS.7 In evaluating whether to start SABRIL, doctors, patients and their caregivers work together to assess the risk of permanent vision loss versus the benefit of seizure reduction. There are other serious risks associated with SABRIL. Please see the important safety information below for more details.
“With so many children still having seizures due to refractory CPS, we are very pleased that the FDA has approved SABRIL for patients 10 and older who may benefit from a new add-on treatment option,” said Amy Magro, Director of Epilepsy Marketing at Lundbeck. “For those caring for a child as young as 10, we hope this new indication provides encouragement to speak with their child’s doctor about the risks and potential benefits of adding SABRIL for refractory CPS.”
In addition to its refractory CPS indication, SABRIL is approved for use in babies one month to two years of age with infantile spasms if the possible benefit outweighs the potential risk of vision loss.
For more information, please visit www.SABRIL.net.
About SABRIL® (vigabatrin) 1
SABRIL is a prescription oral antiepileptic drug developed in the United States by Lundbeck. SABRIL is available in 500-mg tablets or 500-mg packets of powder for oral suspension. Because of the risk of permanent vision loss, SABRIL is available only through a restricted program under a REMS called the SHARE Program. (1-888-45-SHARE).
SABRIL (vigabatrin) is a prescription medicine used with other treatments in adults and children 10 years of age and older with refractory complex partial seizures (CPS), who have not responded well enough to several other treatments, and if the possible benefits outweigh the risk of vision loss. SABRIL should not be the first medicine used to treat CPS.
SABRIL (vigabatrin) is a prescription medicine used in babies, 1 month to 2 years old, with infantile spasms (IS), if the possible benefits outweigh the possible risk of vision loss.
Important Safety Information
WARNING: VISION LOSS
See Medication Guide and full Prescribing Information for complete information
In all people who take SABRIL:
• You are at risk for vision loss with any amount of SABRIL
• Your risk of vision loss may be higher the more SABRIL you take daily and the longer you take it
• It is not possible for your healthcare provider to know when vision loss will happen. It could happen soon after starting SABRIL or any time during treatment. It may even happen after treatment has stopped.
Please see SABRIL Medication Guide, full Prescribing Information including Boxed Warning, and Instructions for Use; go to www.sabril.net, or call toll-free 1-888-45-SHARE (1-888-457-4273).
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.
About Lundbeck in the U.S.
A wholly owned subsidiary of H. Lundbeck A/S of Denmark, Lundbeck in the United States is headquartered in Deerfield, Illinois, and is committed to providing innovative specialty therapies that fulfill unmet medical needs of people with central nervous system (CNS) disorders, including several therapies for people with challenging seizure disorders.
With a special commitment to the epilepsy community, Lundbeck actively supports and participates in hundreds of community-based initiatives. Learn more about our epilepsy community programs at http://www.lundbeck.com/us/our-commitment/community-involvement.
About H. Lundbeck A/S
Lundbeck is a global pharmaceutical company highly committed to improving the quality of life of people living with brain diseases. For this purpose, Lundbeck is engaged in the entire value chain throughout research, development, production, marketing and sales of pharmaceuticals across the world. The company’s products are targeted at disorders such as depression and anxiety, psychotic disorders, epilepsy, Huntington’s, Alzheimer’s and Parkinson’s diseases. Lundbeck’s pipeline consists of several mid- to late-stage development programs.
Lundbeck employs more than 5,800 people worldwide, 2,000 of whom are based in Denmark. We have employees in 57 countries and our products are registered in more than 100 countries. We have research centers in Denmark, China and the United States and production facilities in Italy, France, Mexico, China and Denmark. Lundbeck generated revenue of approximately DKK 15 billion in 2012. Lundbeck’s shares are listed on the stock exchange in Copenhagen under the symbol “LUN.” Lundbeck has a sponsored Level 1 ADR programme listed in the US (OTC) under the symbol “HLUYY.” For additional information, we encourage you to visit our corporate site www.lundbeck.com.
SABRIL is a registered trademark of Lundbeck.
Actelion receives U.S. FDA approval of Opsumit (macitentan) for the treatment of pulmonary arterial hypertension.
Allschwil, Switzerland, October 18, 2013 – Actelion Ltd (SIX: ATLN) announced today that the United States Food and Drug Administration (FDA) has approved the use of the orally available endothelin receptor antagonist Opsumit® (macitentan) 10 mg once daily for the treatment of pulmonary arterial hypertension (PAH) to delay disease progression.
Opsumit is indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to delay disease progression. Disease progression included: death, initiation of intravenous (IV) or subcutaneous prostanoids, or clinical worsening of PAH (decreased 6-minute walk distance, worsened PAH symptoms and need for additional PAH treatment). Opsumit also reduced hospitalization for PAH.
Effectiveness was established in a long-term study in PAH patients with predominantly WHO Functional Class II-III symptoms treated for an average of 2 years. Patients were treated with Opsumit® monotherapy or in combination with phosphodiesterase-5 inhibitors or inhaled prostanoids. Patients had idiopathic and heritable PAH (57%), PAH caused by connective tissue disorders (31%), and PAH caused by congenital heart disease with repaired shunts (8%).
Dr. Vallerie McLaughlin, Director of the Pulmonary Hypertension Program in the Division of Cardiovascular Medicine at the University of Michigan, commented: “Over the past twenty years, great strides have been made in treating PAH patients. However, there has been a medical need for innovative treatments that improve long-term outcomes. Opsumit® is the first clinically proven and only oral treatment option indicated to delay disease progression and reduce the need for PAH hospitalization.”
Dr. McLaughlin concluded: “These effects were demonstrated in SERAPHIN, the first and largest PAH outcome study to date, where Opsumit® was given on average for 2 years, as a monotherapy or in combination with phosphodiesterase-5 inhibitors or inhaled prostanoids. I am very pleased that PAH patients will have this new treatment option.”
Jean-Paul Clozel, M.D. and Chief Executive Officer of Actelion commented: “Today’s approval of Opsumit® by the FDA is providing the PAH community with a unique treatment option, the only oral PAH medicine that has proven to delay disease progression. Over the last 14 years, Actelion has worked tirelessly to first discover and then develop Opsumit® in the largest, longest and first-ever outcome study in PAH. I would like to express my gratitude to all the members of the PAH community. Without their contribution, Opsumit® would not have become a reality. We will now leverage our existing PAH expertise and infrastructure to bring Opsumit® to patients within the coming weeks.”
The US label for Opsumit® carries a Boxed Warning alerting patients and health care professionals that the drug should not be used in pregnant women because it can harm the developing fetus. Female patients can receive the drug only through the Opsumit REMS Program. All female patients must be enrolled in the program, comply with pregnancy testing requirements and be counselled regarding the need for contraception.
The most common adverse reactions (more frequent than placebo by 3% or more) observed in patients treated with Opsumit® were anemia, nasopharyngitis/pharyngitis, bronchitis, headache, influenza, and urinary tract infection.
Physicians are advised to measure hemoglobin and liver enzymes prior to initiation of Opsumit® and repeat during treatment as clinically indicated.
In the United States, Actelion expects Opsumit® to become available to patients in November. Outside of the United States, Actelion continues to work with health authorities to obtain regulatory approval for Opsumit® .
The FDA approval was based in part on data from the landmark phase III SERAPHIN study. Published in the New England Journal of Medicine in August 2013, the SERAPHIN study showed the risk of the first occurrence of a morbidity or mortality event, the primary endpoint of the study, was reduced by 45% (p<0.0001) with macitentan 10 mg compared to placebo. This effect was observed irrespective of whether or not patients were already treated with other therapies for PAH. SERAPHIN also showed a risk reduction in PAH related hospitalization and death of 50% (p<0.0001) compared to placebo. .
Pulmonary arterial hypertension (PAH) is a chronic, life-threatening disorder characterized by abnormally high blood pressure in the arteries between the heart and lungs of an affected individual. The symptoms of PAH are non-specific and can range from mild breathlessness and fatigue during normal daily activity to symptoms of right heart failure and severe restrictions on exercise capacity and ultimately reduced life expectancy.
NOTES TO THE EDITOR
ABOUT OPSUMIT® (MACITENTAN)
Opsumit® (macitentan) is a novel dual endothelin receptor antagonist (ERA) that resulted from a tailored drug discovery process with the target of developing an ERA to address efficacy and safety .
ABOUT THE SERAPHIN STUDY
SERAPHIN (Study with an Endothelin Receptor Antagonist in Pulmonary arterial Hypertension to Improve cliNical outcome) was the largest and longest randomized, controlled study in PAH patients to include a clearly defined morbidity/mortality primary endpoint . The pivotal Phase III study was designed to evaluate the efficacy and safety of Opsumit®(macitentan) – a novel dual endothelin receptor antagonist that resulted from a tailored drug discovery process – through the primary endpoint of time to first morbidity and all-cause mortality event in patients with symptomatic PAH.
Global enrollment was completed in December 2009 with a total of 742 patients. Patients were randomized 1:1:1 to receive two different doses of macitentan (3 mg and 10 mg once daily) or placebo. Patients were allowed to receive PAH background therapy throughout the study, either PDE-5 inhibitors or oral/inhaled prostanoids. This event-driven study was conducted in 151 centers from almost 40 countries in North America, Latin America, Europe, Asia-Pacific and Africa and was completed in the first half of 2012, with 287 patients having an adjudicated event.
Dr. McLaughlin is a consultant to Actelion and was an investigator in the SERAPHIN trial.
ABOUT SERAPHIN STUDY DATA
Patients were randomized to placebo (n=250), macitentan 3 mg (n=250), or macitentan 10 mg (n=242). The primary end point occurred in 46.4%, 38.0%, and 31.4% of the patients in these groups, respectively. The hazard ratio for macitentan 3 mg versus placebo was 0.70 (97.5% CI, 0.52 to 0.96; p=0.0108) and the hazard ratio for macitentan 10 mg versus placebo was 0.55 (97.5% CI, 0.39 to 0.76; p<0.0001). Worsening of pulmonary arterial hypertension was the most frequent primary end point event. The effect of macitentan on this end point was observed irrespective of background therapy for pulmonary arterial hypertension. 
ABOUT THE SAFETY AND TOLERABILITY PROFILE
Opsumit is contraindicated in pregnancy because it may harm the developing fetus. Females of reproductive potential should be counselled on the use of reliable contraception and have a negative pregnancy test prior to initiating therapy and monthly thereafter.
Other ERAs have been associated with elevations of aminotransferases, hepatotoxicity, and liver failure. Liver enzyme tests should be obtained prior to initiation of Opsumit® and repeated during treatment as clinically indicated. If clinically relevant aminotransferase elevations occur, or if elevations are accompanied by clinical symptoms of hepatoxicity, discontinue Opsumit®.
Decreases in hemoglobin concentration and hematocrit occurred following administration of other ERAs and were observed in clinical studies with OPSUMIT. The decreases occurred early and stabilized thereafter. Decreases in hemoglobin seldom require transfusion. Initiation of Opsumit® is not recommended in patients with severe anemia. Hemoglobin should be measured prior to initiation of treatment and repeat during treatment as clinically indicated.
Should signs of pulmonary edema occur, consider the possibility of associated PVOD. If confirmed, discontinue Opsumit®.
Other ERAs have been associated with adverse effects on spermatogenesis. Men should be counseled about potential effects on fertility.
The use of Opsumit® with strong CYP3A4 inducers or inhibitors should be avoided.
The most common adverse reactions (more frequent than placebo by 3% or more) observed in patients treated with Opsumit were anemia, nasopharyngitis/pharyngitis, bronchitis, headache, influenza, and urinary tract infection.
ABOUT OPSUMIT® (MACITENTAN) SUBMISSIONS TO HEALTHCARE AUTHORITIES
Approval of the new drug application for Opsumit® (macitentan) was issued by the US Food and Drug Administration (FDA) on 18October 2013 for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to delay disease progression. Disease progression included: death, initiation of intravenous (IV) or subcutaneous prostanoids, or clinical worsening of PAH (decreased 6-minute walk distance, worsened PAH symptoms and need for additional PAH treatment). The need for PAH hospitalization was also reduced.
Regulatory reviews are ongoing in Europe, Canada, Switzerland, Australia, Taiwan, Korea and Mexico.
ABOUT PULMONARY ARTERIAL HYPERTENSION [9, 10]
Pulmonary arterial hypertension (PAH) is a chronic, life-threatening disorder characterized by abnormally high blood pressure in the arteries between the heart and lungs of an affected individual. The symptoms of PAH are non-specific and can range from mild breathlessness and fatigue during normal daily activity to symptoms of right heart failure and severe restrictions on exercise capacity and ultimately reduced life expectancy.
PAH is one group within the classification of pulmonary hypertension (PH). This group includes idiopathic PAH, heritable PAH and PAH caused by factors which include connective tissue disease, HIV infection and congenital heart disease.
The last decade has seen significant advances in the understanding of the pathophysiology of PAH, which has been paralleled with developments of treatment guidelines and new therapies. Drugs targeting the three pathways that have been established in the pathogenesis of PAH are endothelin receptor antagonists (ERAs), prostacyclins and phosphodiesterase-5 inhibitors. PAH treatments have transformed the prognosis for PAH patients from symptomatic improvements in exercise tolerance 10 years ago to delayed disease progression today. Improved disease awareness and evidence-based guidelines developed from randomized controlled clinical trial data have highlighted the need for early intervention, goal-oriented treatment and combination therapy.
In PAH, survival rates are unacceptably low and PAH remains incurable.
Actelion Ltd is a biopharmaceutical company with its corporate headquarters in Allschwil/Basel, Switzerland. Actelion’s first drug Tracleer® (bosentan), an orally available dual endothelin receptor antagonist, has been approved as a therapy for pulmonary arterial hypertension. Actelion markets Tracleer through its own subsidiaries in key markets worldwide, including the United States (based in South San Francisco), the European Union, Japan, Canada, Australia and Switzerland. Actelion, founded in late 1997, is a leading player in innovative science related to the endothelium – the single layer of cells separating every blood vessel from the blood stream. Actelion’s over 2,300 employees focus on the discovery, development and marketing of innovative drugs for significant unmet medical needs. Actelion shares are traded on the SIX Swiss Exchange (ticker symbol: ATLN) as part of the Swiss blue-chip index SMI (Swiss Market Index SMI®).
For further information please contact:
Senior Vice President, Head of Investor Relations & Public Affairs
Actelion Pharmaceuticals Ltd, Gewerbestrasse 16, CH-4123 Allschwil
+41 61 565 62 62
+1 650 624 69 36
The above information contains certain “forward-looking statements”, relating to the company’s business, which can be identified by the use of forward-looking terminology such as “estimates”, “believes”, “expects”, “may”, “are expected to”, “will”, “will continue”, “should”, “would be”, “seeks”, “pending” or “anticipates” or similar expressions, or by discussions of strategy, plans or intentions. Such statements include descriptions of the company’s investment and research and development programs and anticipated expenditures in connection therewith, descriptions of new products expected to be introduced by the company and anticipated customer demand for such products and products in the company’s existing portfolio. Such statements reflect the current views of the company with respect to future events and are subject to certain risks, uncertainties and assumptions. Many factors could cause the actual results, performance or achievements of the company to be materially different from any future results, performances or achievements that may be expressed or implied by such forward-looking statements. Should one or more of these risks or uncertainties materialize, or should underlying assumptions prove incorrect, actual results may vary materially from those described herein as anticipated, believed, estimated or expected.
News Release Intended for U.S. Media Only
FDA Approves Bayer’s New Class of Drug Adempas® (riociguat) tablets to Treat Adults with PAH and Persistent, Recurrent or Inoperable CTEPH First and only drug approved in U.S. to Treat Two Forms of Pulmonary Hypertension (WHO Group 1 and 4)
Whippany, N.J., October 8, 2013 – Bayer HealthCare announced today that the United States Food and Drug Administration (FDA) has approved Adempas® (riociguat) tablets for: (i) the treatment of adults with persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) (WHO* Group 4) after surgical treatment or inoperable CTEPH to improve exercise capacity and WHO functional class; and (ii) the treatment of adults with pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity, improve WHO functional class and delay clinical worsening. In PAH, efficacy was shown in patients on Adempas monotherapy or in combination with endothelin receptor antagonists (ERAs) or prostanoids (inhaled, oral or subcutaneous). Studies establishing effectiveness included predominately patients with WHO functional class II-III and etiologies of idiopathic or heritable PAH (61%) or PAH associated with connective tissue diseases (25%). Adempas is the only treatment approved in the U.S. for use in two types of pulmonary hypertension (WHO Group 1 and 4). It is the first and only FDA-approved drug therapy for persistent/recurrent CTEPH after surgical treatment or inoperable CTEPH. It is also the only approved oral therapy in PAH with efficacy shown in monotherapy or in combination with ERAs or prostanoids. For all female patients, Adempas is available only through a restricted program called the Adempas Risk Evaluation and Mitigation Strategy (REMS) Program.
“CTEPH and PAH are serious and life-threatening diseases,” said Nick H. Kim, Associate Clinical Professor of Medicine, Division of Pulmonary and Critical Care Medicine; Director, Pulmonary Vascular Medicine; Director, Fellowship Program; University of California San Diego. “The approval of Adempas equips physicians with a new approach to treating PAH patients, and it gives us the first approved drug treatment for patients with inoperable CTEPH or with persistent/recurrent CTEPH after surgery. While surgery should always be considered as the first treatment option for CTEPH, the fact remains that up to forty percent of CTEPH patients are not eligible for surgery, and ten to thirty-five percent of CTEPH patients have disease that persists after surgery.” PAH is a disease characterized by elevated pressure in the pulmonary arteries. CTEPH is a form of pulmonary hypertension in which blood clots and thromboembolic occlusion of the pulmonary vessels leads to increased pressure in the pulmonary arteries. The standard treatment for CTEPH is pulmonary endarterectomy, a potentially curative surgery that clears clots and scar material from the blood vessels of the lung. “Bayer is deeply committed to bringing new treatment options to patients with life-threatening diseases. Adempas is an excellent example of this commitment, because it is the result of years of dedicated research in our Bayer laboratories into a new way of treating two forms of pulmonary hypertension,” said Pamela A. Cyrus, MD, Vice President and Head, U.S. Medical, Bayer HealthCare Pharmaceuticals. “We are pleased to bring this new class of treatment to patients with PAH or with inoperable CTEPH or persistent/recurrent CTEPH after surgical treatment.” Rino Aldrighetti, President and CEO, Pulmonary Hypertension Association added, “From a patient’s perspective, living with pulmonary hypertension remains difficult. We know that not all treatments work for all people. We get excited when there is a new treatment option for PAH patients, and we are thrilled there is finally an approved drug treatment for people living with persistent/recurrent CTEPH after surgical treatment or inoperable CTEPH.” Adempas, a stimulator of soluable guanylate cyclase (sGC), represents a new class of drug now available in the U.S. Pulmonary hypertension is associated with endothelial dysfunction, impaired synthesis of nitric oxide (NO) and insufficient stimulation of the NO-sGC-cGMP pathway. Adempas sensitizes sGC to endogenous NO by stabilizing the NO-sGC binding. Adempas also directly stimulates sGC via a different binding site independently of NO. Adempas restores the NO-sGC-cGMP pathway and leads to increased generation of cGMP with subsequent vasodialation. The most common adverse reactions occurring more frequently (>3%) on Adempas than placebo were headache (27% vs 18%), dyspepsia/gastritis (21% vs. 8%), dizziness (20% vs 13%), nausea (14% vs 11%), diarrhea (12% vs 8%), hypotension (10% vs 4%), vomiting (10% vs 7%), anemia (7% vs 2%), gastroesophageal reflux disease (5% vs 2%), and constipation (5% vs 1%). Other events that were seen more frequently in Adempas compared to placebo and potentially related to treatment were: palpitations, nasal congestion, epistaxis, dysphagia, abdominal distension and peripheral edema. About Patient Assistance Program Bayer offers patient assistance through the Adempas Aim Support Center program, which will assist with obtaining coverage and patient support of Adempas. Patients and providers may contact the program at 1-855-4ADEMPAS for additional information. IMPORTANT SAFETY INFORMATION
WARNING: EMBRYO-FETAL TOXICITY Do not administer Adempas (riociguat) tablets to a pregnant female because it may cause fetal harm.
Females of reproductive potential: Exclude pregnancy before the start of treatment, monthly during treatment, and 1 month after stopping treatment. Prevent pregnancy during treatment and for one month after stopping treatment by using acceptable methods of contraception.
For all female patients, Adempas is available only through a restricted program called the Adempas Risk Evaluation and Mitigation Strategy (REMS) Program.
Contraindications. Adempas is contraindicated in:
Warnings and Precautions Embryo-Fetal Toxicity. Adempas may cause fetal harm when administered during pregnancy and is contraindicated for use in women who are pregnant. In females of reproductive potential, exclude pregnancy prior to initiation of therapy, advise use of acceptable contraception and obtain monthly pregnancy tests. For females, Adempas is only available through a restricted program under the Adempas REMS Program. Adempas REMS Program. Females can only receive Adempas through the Adempas REMS Program, a restricted distribution program. Important requirements of the Adempas REMS program include the following:
Further information, including a list of certified pharmacies, is available at www.AdempasREMS.com or 1-855-4ADEMPAS. Hypotension. Adempas reduces blood pressure. Consider the potential for symptomatic hypotension or ischemia in patients with hypovolemia, severe left ventricular outflow obstruction, resting hypotension, autonomic dysfunction, or concomitant treatment with antihypertensives or strong CYP and P-gp/BCRP inhibitors. Consider a dose reduction if patient develops signs or symptoms of hypotension. Bleeding. In the placebo-controlled clinical trials program, serious bleeding occurred in 2.4% of patients taking Adempas compared to 0% of placebo patients. Serious hemoptysis occurred in 5 (1%) patients taking Adempas compared to 0 placebo patients, including one event with fatal outcome. Serious hemorrhagic events also included 2 patients with vaginal hemorrhage, 2 with catheter site hemorrhage, and 1 each with subdural hematoma, hematemesis, and intra-abdominal hemorrhage. Pulmonary Veno-Occlusive Disease. Pulmonary vasodilators may significantly worsen the cardiovascular status of patients with pulmonary veno-occlusive disease (PVOD). Therefore, administration of Adempas to such patients is not recommended. Should signs of pulmonary edema occur, the possibility of associated PVOD should be considered and if confirmed, discontinue treatment with Adempas. Most Common Adverse Reactions The most common adverse reactions occurring more frequently (>3%) on Adempas than placebo were headache (27% vs 18%), dyspepsia/gastritis (21% vs. 8%), dizziness (20% vs 13%), nausea (14% vs 11%), diarrhea (12% vs 8%), hypotension (10% vs 4%), vomiting (10% vs 7%), anemia (7% vs 2%), gastroesophageal reflux disease (5% vs 2%), and constipation (5% vs 1%). Other events that were seen more frequently in Adempas compared to placebo and potentially related to treatment were: palpitations, nasal congestion, epistaxis, dysphagia, abdominal distension and peripheral edema. For important risk and use information, please see the full Prescribing Information, including Boxed Warning, at www.adempas-us.com. About Bayer HealthCare Pharmaceuticals Inc. Bayer HealthCare Pharmaceuticals Inc. is the U.S.-based pharmaceuticals business of Bayer HealthCare LLC, a subsidiary of Bayer AG. Bayer HealthCare is one of the world’s leading, innovative companies in the healthcare and medical products industry, and combines the activities of the Animal Health, Consumer Care, Medical Care, and Pharmaceuticals divisions. As a specialty pharmaceutical company, Bayer HealthCare provides products for General Medicine, Hematology, Neurology, Oncology and Women’s Healthcare. The company’s aim is to discover and manufacture products that will improve human health worldwide by diagnosing, preventing and treating diseases. Bayer® and the Bayer Cross® are registered trademarks of Bayer. Intended for U.S. media only U.S. Media Contact: Marcy Funk, Communications, Bayer HealthCare Telephone: (862) 404-5385 E-Mail: email@example.com Forward-Looking Statements This release may contain forward-looking statements based on current assumptions and forecasts made by Bayer Group or subgroup management. Various known and unknown risks, uncertainties and other factors could lead to material differences between the actual future results, financial situation, development or performance of the company and the estimates given here. These factors include those discussed in Bayer’s public reports which are available on the Bayer website at www.bayer.com. The company assumes no liability whatsoever to update these forward-looking statements or to conform them to future events or developments.
News Release Intended for U.S. Media Only
FDA ADVISORY COMMITTEE UNANIMOUSLY RECOMMENDS APPROVAL OF BAYER’S RIOCIGUAT IN TWO PULMONARY HYPERTENSION INDICATIONS
If approved by the FDA, riociguat will be the first treatment for inoperable CTEPH or persistent/recurrent CTEPH after surgery and a new treatment for PAH
Whippany, N.J., August 6, 2013– Bayer HealthCare today announced that the U.S. Food and Drug Administration’s (FDA) Cardiovascular and Renal Drugs Advisory Committee recommended approval for investigational riociguat, proposed trade name Adempas™, in two forms of pulmonary hypertension. The Committee voted 11 to 0 that riociguat should be approved for the treatment of pulmonary arterial hypertension [PAH] of WHO1 Group 1. The Committee also voted 11 to 0 that riociguat should be approved for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) of WHO Group 4. In February 2013, Bayer submitted a new drug application for riociguat in two indications: (i) the treatment of PAH (WHO Group 1) to improve exercise capacity, improve WHO functional class and delay clinical worsening; and (ii) the treatment of persistent/recurrent CTEPH (WHO Group 4) after surgical treatment or inoperable CTEPH to improve exercise capacity and WHO functional class. “We appreciate the Committtee’s discussion today around the safe and appropriate use of riociguat and are pleased with the outcome of the votes,” said Pamela A. Cyrus, MD, Vice President and Head of U.S. Medical Affairs, Bayer HealthCare Pharmaceuticals. “If approved, riociguat will offer a new treatment option for patients with PAH and will also provide the first approved non-surgical treatment option for CTEPH patients who are inoperable or who have recurrent or persistent disease. We look forward to continued dialogue with the FDA in order to make riociguat available to patients.”
PAH and CTEPH are both life-threatening forms of pulmonary hypertension that cause significantly increased pressure in the pulmonary arteries. Riociguat is an investigational, oral medication for the treatment of adult patients with PAH or inoperable or persistent/recurrent CTEPH. If approved by the FDA later this year, it would create a new class of therapy available in the U.S. PH is associated with endothelial dysfunction, impaired synthesis of nitric oxide (NO) and insufficient stimulation of soluble guanylate cyclase (sGC). Riociguat stimulates sGC independent of NO and increases the sensitivity of sGC to NO. Data presented at today’s advisory committee meeting included results from the global Phase 3 clinical program, which enrolled 704 patients across two Phase 3 studies. Both studies met their primary endpoint by demonstrating a statistically significant improvement in the six-minute walk test (6MWT), after 16 and 12 weeks respectively. Riociguat was also associated with improvements across multiple, relevant, secondary endpoints in the studies. The most common treatment-emergent adverse events with riociguat were headache, dizziness, dsypesia, peripheral edema, nausea, diarrhea and vomiting. The advisory committee’s vote will be taken into consideration by the FDA when making its decision on the approvability of Bayer’s NDA for riociguat, which was submitted in February 2013. After acceptance of the NDA, the FDA granted riociguat priority review designation, which is given to drugs that have the potential to offer significant improvement in treatment or provide a treatment option where no adequate therapy exists. About Pulmonary Arterial Hypertension (PAH) In PAH, a rare and life-threatening disease, the blood pressure in the pulmonary arteries (the arteries that take de-oxygenated blood to the lungs from the heart) is significantly increased. PAH is characterized by morphological changes to the endothelium of the arteries of the lungs causing remodeling of the tissue, vasoconstriction and thrombosis-in-situ. As a result of these changes, the blood vessels in the lungs are narrowed, making it difficult for the heart to pump blood through to the lungs. In most cases, PAH has no known cause and, in some cases, it can be inherited. About Chronic Thromboembolic Pulmonary Hypertension (CTEPH) CTEPH is also a rare and life-threatening disease in which it is believed that thromboembolic occlusion (organized blood clots) of pulmonary vessels gradually lead to an increased pressure in the pulmonary arteries, resulting in an overload of the right heart. CTEPH may evolve after prior episodes of acute pulmonary embolism, but the pathogenesis is not yet completely understood. The standard treatment for CTEPH is pulmonary endarterectomy (PEA), a surgical procedure in which the blood vessels of the lungs are cleared of clot and scar material. However, CTEPH is inoperable in an estimated 20 to 40 percent of patients, and, in some cases, the disease persists or reoccurs after surgery. About Bayer HealthCare Pharmaceuticals Inc.Bayer HealthCare Pharmaceuticals Inc. is the U.S.-based pharmaceuticals business of Bayer HealthCare LLC, a subsidiary of Bayer AG. Bayer HealthCare is one of the world’s leading, innovative companies in the healthcare and medical products industry, and combines the activities of the Animal Health, Consumer Care, Medical Care, and Pharmaceuticals divisions. As a specialty pharmaceutical company, Bayer HealthCare provides products for General Medicine, Hematology, Neurology, Oncology and Women’s Healthcare. The company’s aim is to discover and manufacture products that will improve human health worldwide by diagnosing, preventing and treating diseases. Bayer® and the Bayer Cross® are registered trademarks of Bayer.
Intended for U.S. media only Media Contact: Marcy Funk, Communications, Bayer HealthCare Telephone: (862) 404-5385 E-Mail: firstname.lastname@example.org
Forward-Looking Statements This release may contain forward-looking statements based on current assumptions and forecasts made by Bayer Group or subgroup management. Various known and unknown risks, uncertainties and other factors could lead to material differences between the actual future results, financial situation, development or performance of the company and the estimates given here. These factors include those discussed in Bayer’s public reports which are available on the Bayer website at www.bayer.com. The company assumes no liability whatsoever to update these forward-looking statements or to conform them to future events or developments.Posted in About Us, Diseases, Featured | Tagged Adempas, Amy Magro, antiepileptic, Bayer, Bayer HealthCare, blood clots, child treatment, chronic, complex partial seizure, complex partial seizures, connective tissue diseases, CPS, CTEPH, disease, drug, drugs, dyspepsia, endothelial dysfunction, endothelin receptor antagonists, Epilepsy, Epilepsy Foundation, ERA, FDA, Food and Drug Administration, gastritis, Healthcare, Lundbeck, macitentan, Marcy Funk, Matt Flesch, monotherapy, News release, opsumit, partial seizure, partial seizures, Patient Assistance Program, PH, PHA, Pharmaceutical, Philip Gattone, prostanoids, pulmonary endarterectomy, Pulmonary Hypertension, Pulmonary Hypertension Association, Pulmonary Veno-Occlusive Disease, rare, refractory, refractory CPS, REMS, Rino Aldrighetti, riociguat, Risk Evaluation and Mitigation Strategy Program, Sabril, seizure, seizures, sgc, SHARE, SHARE program, soluable guanylate cyclase, thromboembolic occlusion, thromboembolic pulmonary hypertension, treating children, treatment, University of California San Diego, vigabatrin, vision loss, World Health Organization | Leave a comment August 20, 2013
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Rino Aldrighetti was hired as the first part-time professional staff member of the Pulmonary Hypertension Association. In the years since, he’s assumed the title of President of the organization, enhanced PHA’s scope, built a full-time staff, and advocated tirelessly to increase awareness about pulmonary hypertension. Here, Community talks to Rino about where PHA has been, and where it’s going.
Describe the journey in becoming President of PHA.
In 1998, seven years after PHA’s founding, the organization’s volunteer leadership decided to build a staff. Their first step was to advertise for a part-time executive director. They put an ad in the Chronicle of Philanthropy, running it once.
At the time they were doing this, I was a non-profit consultant. I had just finished a five-year project for one of my larger clients and was getting ready to do what I always did when I was preparing to think through a new direction, begin a three-day retreat. The day before I was to begin, I picked up the Chronicle of Philanthropy and saw a brief two-line ad: “Small rare disease association looking for part-time executive director”.
I responded and was invited to meet with an extraordinary person, Bonnie Dukart, who was PHA’s president. Bonnie, who had been diagnosed with PH shortly after graduating from college, explained to me that the goal was to bring on a person who could increase PHA’s budget so that the organization could do more. Following that meeting, I had the opportunity to meet the board and quickly became aware that I was in the presence of heroes.
I soon accepted the board’s offer and became PHA’s first (part-time) professional staff person in 1999. By 2001, we had increased PHA’s income from $132,000 to $1.1 million and built a small part-time staff. The board then invited me to lead a new full-time staff. The work has always been a privilege.
What are some of the challenges you face when trying to increase awareness or support a rare disease like pulmonary hypertension?
Our greatest challenge in raising awareness about PH is our relatively small numbers. A rare disease in the U.S. is defined as one with 200,000 or fewer patients. PH has 20,000 to 30,000 diagnosed patients.
Given that reality, each person’s decision to make a difference is of enormous importance. At PHA, our mantra is that any person whose life is touched by PH has the right to fight back as much or as little as health and interest allow. Over and over again we have seen what the ability to influence positive change means in people’s lives. We may have 30,000 patients living with this disease, but each has family members and friends, neighbors and medical professionals who care and are ready to help. Harnessing that energy with a strong community is what makes a real difference.
What are some of your goals for PHA for 2013?
In this economic and political environment, organizations that cannot adapt will ultimately decline. At PHA, we have been working hard over the past year to introduce new ways to support our research and patient-serving programs. In December, we opened PHA’s first three chapters – in New York, Chicago and San Francisco. This is a pilot for us to build a professional events structure that will develop a larger population of supporters for our core programming in communities around the country. If we are successful in our first year, we will implement a five-year program to expand the network. It’s an ambitious effort to not only assure our sustainability, but to make sure that our ability to serve the needs of our community is not limited by funding restrictions.
PHA’s early diagnosis program, Sometimes It’s PH Campaign, was launched at our 2012 International Conference and has generated early excitement in the U.S. and other nations. It came about following research indicating that despite all the increased visibility for PH during the past 20 years, the time from onset of symptoms to point of diagnosis has not reduced. This may not have been a problem two decades ago when there were no treatments; however, today with nine treatments and more on the way, getting patients diagnosed so that they can take advantage of these treatments is hugely important.
Why should a PH patient join PHA?
PHA is more than an organization. It’s a community, a place where people understand and help each other get through the challenges of this difficult disease. The strength of the community has value for the individuals who choose to join. It also has value for the whole, for all who live with the disease through our collective ability to develop public awareness, drive advocacy and sustain helping networks. These are things we can only do together and that will create opportunities for better tomorrows for everyone.Posted in Diseases, Featured, Media Center | Tagged PHA, Pulmonary Hypertension, Rino Aldrighetti | 1 Comment November 28, 2012
Whether you’re newly diagnosed with a condition, or have been living with it for years, getting the support you need is crucial to maintaining your best healthy life, both physically and mentally. There are tons of ways to participate in support groups, whether it be through an actual physical get-together, online meetings, or even actively participating in message boards.
The benefits of support groups are obvious – being in an environment in which other people understand you and can share helpful advice and motivation is always going to be an aid, however it’s important to see how this engagement can benefit your personal relationships. It can be truly difficult for someone to give the support they want to a person with a chronic illness, because they don’t truly understand or know what to say. And it can be equally hard for the person affected to really share in words what is going on in their minds and with their bodies. Going to an outside source of support can create a better bond between the caregiver and the person who is ill.
Often times, people are hesitant to go to support groups because they feel what they are going through is too personal or intimate to share with a room full of strangers. So, if you’re feeling hesitant, think about the benefits of just going and listening. It can be more beneficial than you may believe to just be silent for an hour and soak up what others are saying. And if going to a support session seems like you are relinquishing some of your independence, just remember that everyone needs a helping hand sometimes, just don’t be afraid to take it.
Below are some great resources for support groups from some of our friends in the rare and chronic disease community. Also, don’t hesitate to ask your doctor or medical professional where to look.
Pulmonary Hypertension Association
Pulmonary Hypertension Central
MD Junction Pulmonary Hypertension Online Support Group
Daily Strength Pulmonary Hypertension Online Support Group
Pulmonary Hypertension South Africa
Pulmonary Fibrosis Foundation
Daily Strength Pulmonary Fibrosis Support
Coalition for Pulmonary Fibrosis
MD Junction Pulmonary Fibrosis Online Support Group
Daily Strength Narcolepsy Online Support Groups
Narcolepsy Online Support Groups
Huntington’s Disease Society of America
Daily Strength Huntington’s Disease Online Support Groups
Huntington’s Disease Lighthouse Families Online Support Groups
Huntington’s Disease Advocacy Center Message Boards
Huntington’s Disease Youth Organization
Are there support groups which you have found helpful? Please share with us!Posted in Caregivers, Diseases, Featured, Media Center, Uncategorized | Tagged chronic illness, Infantile Spasms, narcolepsy, Pulmonary Fibrosis, Pulmonary Hypertension, Support, Support Groups | 3 Comments October 19, 2012
I have had a long history of coronary heart disease so it is not uncommon for me to experience angina on a regular basis. However, in November 2009 it became stronger than usual and my doctor ordered first a stress test, then a heart catheterization. The doctor preforming the cath found no real changes in the left side, so he decided to do a right sided cath and check the pulmonary artery pressure, which was elevated but not too high. He told me that I was begining to show signs of pulmonary hypertension.
I was not familiar with this and when I went to the follow up with my doctor he put me on a new medication which I assumed was for the PAH. It wasn’t, so in September 2010 I had to go to the ER due to severe angina again. Another heart cath was done including a right sided one to further assess the pulmonary pressure. It had gone from mild to severe in less than a year with a pressure of 80.
I discussed this with my doctor and asked why the new medication had not worked and I was then told that the medication he put me on was not for the pulmonary hypertension. I asked why he had not addressed the PH and he shrugged and left the room. It was at that time that I found a new cardiologist as well as a pulmonologist who put me on PH meds. I was on the lower dose for a year and a half and there were episodes of shortness of breath and I asked about possibly increasing the dosage, but the doctor did not want to do that. I had planned a trip out of the country for vacation and the doctor did a HAST test and said I was fine to fly without oxygen.
He was wrong. I thought I was going to die the enitre trip. I became very sick and really could not participate in my own vacation. By the time I got home I was even sicker. I went to the doctor to explain what had happened and he said it was not from the PH and that I should talk to my cardiologist. It was at that point that I looked for a PH specialist that my insurance accepted and found one in my general area. He is still in the process of adjusting my medications and has done two cardiac caths, one right side the other left side. I have still not been able to return to what I was able to do before the trip and the doctor isn’t sure if I will ever be able to.
When I was put on the PH meds, I had no idea how expensive the medication was and when they told me what my co-pay was I said, “Well they might as well shoot me because there is no way that I can afford that as I am on disability.” It was then that the speciality pharmacy referred me to Caring Voice. They made everything so easy and within only a couple of days had me approved for the balance of that year as well as the following year. I cannot thank them enough for making such a difficult time easier as I don’t have to fight to get my medication.
So that’s my story so far. I am still working on getting back to where I was before the trip and I am determined that I will do so. I will just have to take baby steps, but I know myself, and I will get there.Posted in Diseases, Featured, How We Help, Media Center, Uncategorized | Tagged Angina, Coronary Heart Disease, Hosiptal, Misdiagnosis, Pulmonary Hypertension, Share Your Story, Swollen Ankles | 1 Comment October 12, 2012
I am a spouse of someone that was diagnosed with Pulmonary Hypertension almost 6 years ago. Although the therapy medications worked and helped for 5 years it came to a point where the medication stopped working for him and the only thing that could save him was a double lung transplant.
He was put on a transplant list at UCSD in August of this year. Just two months later he got the call this past Monday that they had a potential donor. He cried when he heard the news. Two hours later from having had received the call we were at UCSD waiting to hear if the lungs were a match. At 11:00p.m. that night the doctor came out to let me and his siblings know that the surgery was a go. On October 9, 2012 my husband received a second chance
at life without PH. Never give up and always keep the faith.
My mom, Nelda Backus, suffers from Pulmonary Fibrosis and has been kept alive only via medications. In one month, her cost of the medication escalated from $447 to $1894 per month. My dad had always handled anything to do with mom’s medical issues, but we lost him last October. My sister and I were aghast at what the pharmaceutical company had done and didn’t know what to do to help our mother. Please understand that we are talking about a widow who receives $2013 a month in social security benefits. We started calling everyone and anyone we could that might possibly be able to help, only to be turned away time and again because her medication hadn’t been traditionally approved for her illness. Then we were fortunate enough to find out about Caring Voice Coalition.
Caring Voice did everything but stand on their head to ensure that she was able to receive the medication that has kept her with us since 2003. There was a young woman there named Tye who has held our hand through the process and who fought the battle for us! Tye called me to let me know that they would be issuing a grant for my mother that would greatly offset her costs and allow her to be able to buy groceries each month!
Our family owes the Caring Voice a debt of gratitude and we can’t speak highly enough of what they did for our mom. Please get out your checkbook and donate to the cause. They help so many people who are just like my mom, living on a fixed income with no way to generate enough income to pay the rapidly accelerating costs of medicines. I truly believe that they were put in our path by a higher power and that we are blessed to have found them!
Posted in Caregivers, Diseases, Featured, Media Center, Uncategorized | Tagged caregivers, chronic illness, Coping, Medical Bills, Pulmonary Fibrosis, Pulmonary Hypertension, Transplants | 1 Comment October 5, 2012
I would have to say my situation started back in the ’90s. I was experiencing a shortness of breath and would be sick with bronchitis a lot. I decided to go see an allergist and he put me on shots, I took them for about 10 years. It did help me but I still had the shortness of breath. My internal medicine doctor back then decided to do a chest x-ray and when she saw the results she decided to send me to a Pulmonary Specialist.
They diagnosed me with mild asthma and also did a lung biopsy. They said I had scar tissue on my lungs but never really told me how bad it really was. I was treated by this doctor for a number of years with steroids, antibiotics and breathing treatments. As the years went on I became sicker more and more often. I would have periods in which when I would cough I would black out for a few seconds, however the doctor didn’t make a big deal about it.
Fast forward to 2010, I started to have a lot of weakness and fatigue. One day while driving home I began to choke on some water and I blacked out while driving. When I came to I was making my way over to the next lane on the interstate. I know that the Lord was with me that day because otherwise I would not be talking to you today. I didn’t go to the doctor until about a week or so later when I began having severe headaches and weakness. I thought it maybe was a toothache so I made an appointment with the Dentist.
On the day of the appointment, I had to climb a flight of stairs and by the time I got up those stairs I was gasping for breath. My dentist told me he would treat me for my toothache but he said I needed to see a doctor becayse he believe I had something very serious going on, pointing out that my hands were blue!
I called my Internal Medicine doctor and she ordered tests on my heart and blood tests. They would not let me go home because of the severity of my condition. They also conducted a CT scan of my lungs. The doctors came in and told me I had Pulmonary Hypertension.
They immediately started me on medicine (this was in July of 2010) and then in October of that same year I started to see a pulmonologist who put me on medicines related specifically to Pulmonary Hypertension. I am now also on oxygen pretty much all the time and I am doing Pulmonary Rehabilitation to help build up strength and endurance for my body.
It has been very encouraging and inspiring to do. I would like to say it has affected my life in many ways. It has affected my physical relationship with my husband who does not always understand and who is also dealing with his own health issues. It has affected my daughter and grandchildren as they were very worried about me. But, I am a fighter and refuse to give up because I know my Lord Jesus Christ is my healer, my rock and my shield and He will heal me.
I would like to say to all who are battling this disease or anyone who has a loved one who is battling it, to always support them, love them and pray for them and know that the Lord is with you and with them. I hope that I have been able to help or encourage someone today.Posted in Diseases, Events, Featured, Media Center, Uncategorized | Tagged Asthma, Pulmonary, Pulmonary Hypertension, Shortness of Breath | 6 Comments September 17, 2012
It was the third week that my ankles had been swollen consistently. That Wednesday, May 16, 2012 my mother and husband convinced me that an ER trip was necessary. The swelling had come and gone over the past 3 months. As a 41 year old mother of two teenagers, an asthmatic and someone who was overweight, I chalked the swelling up to being heavy. The shortness of breath had been there for years and again, I chalked that up to being heavy. Then I started to notice chest pain with the swelling and shortness of breath. That was when I realized that this might be a little more serious than I thought.
At the ER, the initial thought was that I had had a heart attack. Then blood clots then eventually, the final diagnosis… Pulmonary Hypertension. The language was not new to me or my family as my niece was born two short years ago with PH.
After a whirl of tests and a week-long hospital stay, I was discharged with little knowledge other than what I had learned from my niece. A Cardiac Cath was done and with this I was introduced to my now Pulmonary Doctor whom I love. Now the diagnosis was worse. Pulmonary Hypertension and Pulmonary Veno-Occlusive Disease.
I was told that after 5 years I would have a 50% chance of living without a double lung transplant. My world just stopped. Here we were a family of four making good money and living a great life. I had to quit my job and file for Social Security Disability. I had to deliver the news to my husband and boys that I was going to die.
Here it is four months later; I have had an open lung biopsy and have been taking medications that make my symptoms worse. The money situation is so bad, we’re making enough barely struggle by, but not enough to live. I was approved for disability but have a $5000 out of pocket deductible with my husband’s insurance. Not to mention copays and script costs. Disability wont start until December 26, 2012 and Medicare wont start until May 2014.
Stress is bad, I find myself crying all the time. I try to put on a happy face but it is so hard to ask for help, watch my husband come home tired from work and do housework and cook because I cannot.
Everyone keeps saying it will be ok, my question is ok for who? I have not yet learned to deal with my illness and pray that soon I will see that I can survive.
Thank you Caring Voice Coalition for all you do everyday.Posted in About Us, Diseases, Events, Featured, How We Help, Media Center | Tagged CVC, Medicare, Pulmonary Hypertension, Pulmonary Veno-Occlusive Disease, Shortness of Breath, Swollen Ankles | 8 Comments September 7, 2012
I was diagnosed with pulmonary hypertension in 1998, but my story begins two years earlier. I gave birth to my youngest of three daughters in February of 1996. Immediately, I started feeling exhausted all the time. I would wake up in the morning, feed my children breakfast, sit them down in front of the television, and fall asleep on the couch. I’d wake up in time for lunch, fix them something quick, send them out to play, and fall asleep. At 5 p.m., I’d wake up, make dinner and wait for their father to come home, where I would hand him the children and say, I’m exhausted from my day, and fall asleep. This went on for nearly a year, until we moved to Texas.
I thought the sleeping was more depression, which I had been suffering with since age 5. When we moved to Texas, my two older girls were of kindergarten age and I sent them off to school. My youngest daughter and I would spend the day watching television and playing on the floor. During the summer after their kindergarten year, I began passing out. I didn’t tell anyone that I passed out because honestly, I thought I had fallen asleep. I thought I had woken up. But I would wake up on the floor, usually with my head throbbing, from hitting it some place.
The first time I passed out in front of someone, I was chasing my daughter around and I passed out in front of my sister and ex-mother in law. I woke up to paramedics around me taking my blood pressure, and who knows what else. I refused to go with them because I didn’t have insurance but promised to go to my doctor the next day.
I had been seeing this young, handsome doctor and for many months I kept telling him how tired I was, how I could feel and even see my heart beat, not to mention having to hear it at night when all was quiet. I told him how I was fainting. He gave me Prozac, and each time I would go in to see him, he’d up the dose. Until one day, a year later, I fainted in front of my younger sister. She ran and got my ex-husband who took me in to see my primary doctor.
Luckily, he wasn’t there so we saw his on-call doctor. He listened to my heart and actually listened to ME and said “This is not in your head. This is in your heart”. I remember that day so clearly. He sent me for an echo-cardiogram and the next day, I saw a cardiologist who gave me the news. It was a month later that I saw a specialist in Houston, Texas, Dr Adaani Frost, who, on September 6, 1998; after a Right Heart Catheterization, and waking up on meds, diagnosed me correctly with Primary Pulmonary Hypertension,
I will be celebrating my 14th year since diagnosis with a BANG! I found PHCentral.org first and then PHAssociation.org and right before my divorce was final, in 2008, I found Caring Voice Coalition, without whom, I probably wouldn’t be here, or else, I’d be in a horrible financial situation!
Thank you so much Caring Voice!!! You have been such fantastic help, not just monetarily, but emotionally as well. I don’t know what I would have done without you guys!!Posted in Diseases, Featured, How We Help, Media Center, Uncategorized | Tagged Depression, fainting, phcentral.org, Pulmonary Hypertension, Pulmonary Hypertension Association | Leave a comment August 30, 2012
Our beautiful little girl was born in August 2008 with a Congenital Diaphragmatic Hernia. This is a condition that 1 in 2,500 children are born with. She had a 50% chance of survival. Basically what happens, is that there is a hole in the diaphragm which allows the stomach, intestines, spleen and sometimes liver to move up in to the chest cavity and the lungs are not able to form properly.
We were diagnosed in utero and so we were as prepared as we could be for a situation like this. Zoe was born in Portland and within a few hours of birth was placed on ECMO ( a heart/lung bypass machine). It was touch and go several months. We were able to hold her for the first time when she was 1 month old.
Her CDH (Congenital Diaphragmatic Hernia) was repaired at 1 month old and she remained in the hospital on various ventilators and breathing machines until she was 3 months old. As her lungs got a bit better her pulmonary pressures did not. She was diagnosed with severe pulmonary hypertension at just a few weeks old. She has been on pulmonary medication since birth and at 10 months old had a central line inserted through which she receives a 24/7 infusion of medication. She is also on various other PH meds to help with her pressures.
All these medications have helped dropped her pressures slightly but they are still way higher than they should be. She is still on oxygen as needed, mainly whenever she gets sick. Medications for PH are incredibly expensive. One of the little pills she takes every day has a copay of $3000 a month, and that’s just the copay. We have really good insurance for her, but still would be stuck paying almost $40,000 a year for one medication. Caring Voice has come through for us and helped us with the copay for this medication our daughter so desperately needs.
Today, 4 years later, Zoe is an effervescent little girl who charms everyone she meets. She has come so far, you’d hardly recognize her from the baby who was surrounded by machines and doctors at birth. She still has a long way to go though. We are looking at her being on IV meds for the rest of her life. We are so grateful to Caring Voice and the support they provide to help ensure that our little girl can get the medications she needs and live the fullest life she can.Posted in Diseases, Featured, How We Help, Media Center | Tagged childhood ilness, chronic illness, Congenital Diaphragmatic Hernia, heart/lung bypass, Pulmonary Hypertension | 2 Comments August 2, 2012
My journey began in September 1998 when I was diagnosed with PPH (Primary Pulmonary Hypertension). When I was first informed about my illness, I was relived because I had always known there was something wrong with me.
I was told in such a cruel way though. I had just started working in a law firm as a secretary and it was only my third day, but I had a hospital appointment in the afternoon so I took the afternoon off. I wasn’t going to go but something that day made me! I met my mum outside the hospital and we went in. The doctor asked me about my symptoms, which included fainting, blue lips, palpitations, and always finding it hard to catch my breath. He said he wanted an ECHO so I went into the next room and had to take off my top and bra. I felt scared because they didn’t let my mum in to be with me. The nurse put a cold jelly on my chest and did the examination.
We waited another hour before the doctor told us that I would need a transplant. I was given the name of the illness but no explanation as to what it was or anything, just that it was very rare. Then I was taken into another room where there were loads of student doctors, as the doctor thought they should listen to my heart and lungs to learn. I was in shock, I felt sick, and now I was being prodded like a guinea pig. Finally, I just burst into tears, but they kept on listening. It was one of the hardest days of my life.
I was admitted and then sent onto Papworth Hospital in Cambridge, England, where I got the best care. I came to understand my illness and realize it was now a part of me and we had to live together.
But I knew I would beat it.
I was put on the transplant list in June 2002 (during the World Cup!). My PH doctor, Joanna, was the first to mention the transplant waiting list to my family and me when I was about 22 years old. The doctors estimated that I would have to wait for 1 to 2 years, and maybe even more. I didn’t want to be negative about it. Instead, I wanted to keep a positive outlook on things, so I really didn’t listen to the doctor when he was saying all the bad stuff. I thought, “No, I’m going to get better!” And in nine months my time came! I had a heart and double lung transplant on March 18, 2003.
I spent a week with my transplant team early on in the process. I had tests; from blood tests to echoes to x-rays. They explained to me everything that I should expect about having such a big operation, including taking medication and all the possible side effects. What I remember most was one of the nurses saying to me that I would get facial hair and asking if I would be okay with that. My reply to her was that that would be a simple problem compared to the ones I have now. I also saw a psychologist to help me with my fears about the transplant and everything else I was worried about.
My family was my rock during this difficult time, especially my mum! She was, and still is my rock! My friends kept their distances. I think they were scared that they were going to lose me so they chose to bury their heads in the sand. The only support I got from the hospital was from the PH team and from a Macmillan nurse by the name of Sarah. I remember telling her that “this PH will never beat me, I will fight it until the end and beat it!” My mum always said to me, “if God gave this illness to you, he also [will give] you a cure!”
What gave me hope in the midst of my journey with PPH was meeting my husband to be in 1999! He gave me hope when I thought no one would want an ill person or someone who might die soon. But he loved me unconditionally and didn’t listen to those who were telling him to forget me. He fought for me and I fought my illness.
It was St. Patrick’s’ Day, March 17th, when I got the call. I was in my room, as I was bedridden, and watching my favorite soap, “Eastenders” and I remember my grandmother coming to my room with the phone and saying that a nice man was on the line. I didn’t think anything of it because I thought my fiancé was calling. I realized it wasn’t him, but instead my transplant coordinator, Steven. He asked if I would like to get my new heart and lungs, and I thought he was joking and said, “But I’m busy watching TV!” (I guess it was the shock.) I had an hour to get ready, so my mum took me to the bath to bathe me and I remember all of my family being there and how hard it was saying goodbye because it might have been the last time I was going to see them.
I was being brave, but my emotions were all over the place. I was happy, scared, sad, sick and tearful. I felt sad that someone out there was dying to save me but I tried not to think about this too much because it was very hard to deal with on top of everything that was happening. After my transplant, I wrote the family a letter to tell them that I was doing well and how their loved one saved me and gave me back a normal life. To me, they will always be my angels who were put on this earth to save me, and who I’m grateful for each extra day I have with my loved ones.
My life has changed forever! The transplant gave me back my life. It was like I was reborn. Before the transplant I was in a wheelchair, and on 24-hour oxygen and I needed help with dressing or bathing. I came home from the hospital a month after my transplant, and it took me quite a while to adjust to doing things for myself again. I was apprehensive of everything. I was scared that I would get ill again. I felt alive, yet guilty of it. I didn’t feel the need for oxygen but still wanted to use my wheelchair but my mum put it away! I was doing exercises the physiotherapist gave me which helped me to gain my strength back.
Today, 9 years after the transplant, my life is a happy one. I’ve been happily married since 2004. I don’t want to paint a picture of a perfect life after a transplant because it’s hard with all the medications’ side effects. They consisted of putting on weight and getting excess hair on your face and body. You get an appetite so be careful! I put on so much weight and had to lose it all! Two other side effects include very bad shakes and a puffy face from the steroids. I could always tell when someone is on steroids because my own face was the same! It may not be perfect, but when I think back to how I was before, I wouldn’t change anything.
It took me over a year to fully recover from the transplant. The first year I got CMV (Cytomegalovirus) and I was scared I made a big mistake in having the transplant. But once I recovered from that, everything got better and I got used to the medication (much simpler than the ones from PH) that I had took just twice a day!
The advice I would give to people who have been diagnosed with PPH would be: Be strong. Be brave. Laugh at yourself! Make fun of the PH, that’s what my mum and me did. And still do! There were days that I just wanted to give up to die and be done with it, but that feeling never lasted long! I think I’m the sort of person who cannot stay depressed for too long. I find happiness in the smallest things. Like I love opening a new jar of Nutella the most! Also, I think my family and husband help make me who I am. They give me the strength and love I need. My faith helps me too. Praying and believing in your dreams is vital to helping a person get through obstacles. To me, it doesn’t matter what religion you believe in, just that the power of belief is stronger than anything is possible. I think (and it might sound wrong to others) that everything happens for a reason and that you must always look for the good in that reason, in order to go on with life.
On the night of March 17, 2003, an angel saved me. I’m forever grateful to you. You gave me back a life I never thought I would see. I live each day a happy one with the people I love.Posted in Diseases, Featured, Media Center | Tagged Heart Transplant, Lung Transplant, PPH, Pulmonary Hypertension, Share Your Story | 6 Comments July 13, 2012
Hi. My name is Pat and I have Pulmonary Arterial Hypertension. I was diagnosed about 9 years ago, and yes, I thought I was going to die very soon. Everything I read was very discouraging and I believed that I had no more than 3-5 years to live.
It began when I gained weight and found my breathing and walking was becoming a struggle for me. After accepting there was something really wrong, I found two new doctors: a Cardiologist, because I had elevated blood pressure for years, and I was referred to a Pulmonary Specialist. Both of my doctors have literally saved my life. They both worked with me to identify the problem and to prescribe the proper medications I needed.
My life was not as I had planned it, but with the help of my doctors and my husband, Vic, I have pushed on. It has been a journey filled with disappointment and depression, but for now I am taking it one day at a time!
Slowly though, I did realize I needed something more. I started to pray and have faith that I will survive this disease; I don’t know for how long, but I am trying to make each day a positive one.
To all those with a chronic disease, such as PAH, keep strong with faith, a cure might just be around the corner.. ”If you believe, you will receive whatever you ask for in prayer.” Matthew 21:22
Posted in Diseases, Featured, Media Center, Uncategorized | Tagged Pulmonary Hypertension, Testimonial | 8 Comments ← Previous post