What People Should Know About Pulmonary Hypertension - Pulmonary Hypertension News
What People Should Know About Pulmonary Hypertension - Pulmonary Hypertension News |
- What People Should Know About Pulmonary Hypertension - Pulmonary Hypertension News
- Tiny RNA Molecule, MicroRNA-483, May Help Ease PAH, Early Study Finds - Pulmonary Hypertension News
- How to Become Obsessed with Survival - Pulmonary Hypertension News
- Pulmonary Artery Denervation Helps to Treat PAH, Trial Suggests - Pulmonary Hypertension News
| What People Should Know About Pulmonary Hypertension - Pulmonary Hypertension News Posted: 01 Jun 2020 12:00 AM PDT Since I was diagnosed with pulmonary hypertension (PH) in 2017, I have struggled with people in my life who don't understand what my illness is or how it affects me. PH is a rare disease that not many people have heard of unless they personally have come across it, so I'm often tasked with explaining it. But doing so can be tricky. Many people have misconceptions and misunderstandings about PH. One question I often hear is whether PH is like high blood pressure. This is an understandable mistake given the name, but of course, pulmonary hypertension bears little resemblance to high blood pressure. When others have heard me talk about my heart, they have asked if it's like a murmur. When I mention that my illness makes me breathless, people sometimes ask if my condition is similar to asthma. People always want to compare it to something they understand, yet PH and its symptoms are different. The biggest problem I've had is people underestimating the severity of my illness. This has been particularly challenging for me in the workplace, as my colleagues are not always sensitive about how my condition affects me each day. Occasionally, I have had to take time off with a cold because minor illnesses completely wipe me out. Other times, I've suffered bad side effects from new medications and had to go home. In these instances, I sensed that people thought I am flaky or overreacting, when in reality I am battling every day to live a normal life. People with chronic health problems silently endure so much each day. At times, I may have seemed distracted in a meeting, but inside I was panicking because I had just taken the stairs and realized that I was more breathless than usual, which made me worried that my illness had progressed. Sometimes I wish that people would Google the name of my condition on their own. Of course, people want to ask questions, but it can be an emotional burden to repeatedly discuss the implications of an illness. I would appreciate it if people would do their own research and ask follow-up questions instead, such as, "What does this diagnosis mean for you?" Or, "What do you struggle with the most?" Simply put, I want people to know certain things, but I don't necessarily want to always have to say it. One example is the fact that currently there is no cure for my condition. Another is that it is a progressive illness, meaning it gets worse over time. This is painful for me to repeatedly explain, but it's also important for people to understand to gauge how much my diagnosis impacts my life. Beyond being as communicative as possible with the people in my life, working to raise awareness is the answer to the struggles I face. Ultimately, if there is a greater understanding of illnesses such as pulmonary hypertension, it makes the lives of those living with rare conditions infinitely easier. Not to mention that raising awareness contributes to the fight for new therapies, and hopefully one day, a cure. *** Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, BioNews Services, and are intended to spark discussion about issues pertaining to pulmonary hypertension.  |
| Tiny RNA Molecule, MicroRNA-483, May Help Ease PAH, Early Study Finds - Pulmonary Hypertension News Posted: 22 Jun 2020 08:00 AM PDT Increasing the levels of a tiny RNA molecule known as microRNA-483 enhanced the function of endothelial cells and eased the symptoms of pulmonary arterial hypertension (PAH) in rat models of the disease, a new study shows. The results support the potential of microRNA-483 as a potential therapy for PAH. The study, "MicroRNA-483 amelioration of experimental pulmonary hypertension," was published in the journal EMBO Molecular Medicine. The improper functioning of endothelial cells — those lining the inner wall of blood vessels – is characteristic of PAH progression. Compromised endothelial cells produce higher-than-normal levels of pro-inflammatory molecules called cytokines, and of molecules that constrict blood vessels (vasoconstrictors), while lowering those that open (dilate) blood vessels, called vasodilators. This results in increased blood pressure in the lung's vascular system. There are medicines that can target impaired endothelial cells, but their efficacy is limited. MicroRNAs are a special class of short RNA molecules capable of regulating gene activity. They have been increasingly recognized as potential therapeutic targets. One such short RNA molecule is MicroRNA-483 (miR-483), which contains two strands, called miR-483-3p and miR- 483-5p. In a previous study, researchers at the Xi'an Jiaotong University Health Science Center, in China, observed that miR-483 enhanced the function of endothelial cells by promoting an anti-scarring (fibrotic) effect. Now, the team investigated whether miR-483 plays a role in PAH. The scientists first assessed whether the levels of miR-483 in the blood differed between animals with PAH and healthy ones (control group). Compared with the controls, those with PAH had significantly lower levels of the two miR strands that compose miR-483 — especially those with more severe disease. Levels of miR-483-3p below 27% were able to identify PAH with a sensitivity of 88.4% and a specificity of 56.8%, the results suggested. Meanwhile, levels of miR-483-5p below 26% did the same with a sensitivity of 82.1% and a specificity of 48.9%. Of note, a test's sensitivity is its ability to correctly identify those with a given disease. Specificity refers to correctly identifying those without the disease. After dividing the PAH animals according to their mortality risk, the researchers then saw that those with an intermediate or high risk had lower miR-483-3p levels than those with a low mortality risk. To assess if these lower miR483 levels were in fact associated with endothelial cells, the team isolated extracellular vesicles, called EVs, from the blood of both those with PAH and healthy controls. To identify EVs originating from endothelial cells they used a marker called CD144. The results showed that EVs from the PAH animals had lower levels of miR-483 compared with healthy controls. Of note, EVs are an important mode of intercellular communication, and are known for their capacity to carry proteins and DNA and RNA molecules, including microRNAs. They are increasingly recognized as potential biomarkers for diagnosis. "The lower levels of miR‐483 found in circulation and CD144‐enriched EVs of IPAH patients suggested that the expression of miR‐483‐3p/‐5p might affect genes and pathways involved in PAH," the researchers wrote. To better understand the role of miR-483 in endothelial cells, the researchers then increased the levels of this microRNA in human pulmonary arterial endothelial cells grown in the lab. Those results showed that cells altered their behavior, with changes in several pathways, including a lower activity of processes related to cell adhesion, programmed cell death (apoptosis), metabolism, inflammation, migration, and proliferation. A bioinformatic analysis identified several of the molecular players targeted by miR-483-3p and miR-483-5p. All of them played a role in the altered pathways that had been identified. Next, the researchers developed a genetic rat model, named EC-miR-483-Tg, in which endothelial cells had stable levels of miR-483. The compound monocrotaline (MCT) was used to induced PAH in this rat model and also in control animals. After MCT administration, the levels of PAH-related genes were higher in control animals than in EC-miR-483-Tg engineered rats, the results showed. At a physiological level, the mean pulmonary artery pressure (mPAP) and Fulton index (a measure of heart enlargement) were alleviated in the engineered group of rats treated with MCT. Moreover, the rats had less thickened blood vessels and less permeable endothelium compared with control rats. Another animal model was then tested, in which Sugen 5416 (SU5416, a blocker of endothelial growth factor receptor) and chronic hypoxia were used to induce PAH. Compared with controls, this rat model also had a reduced increase of mPAP and Fulton index, the researchers found. The researchers then tested whether the administration of miR-483 could ease the symptoms of PAH. They delivered miR-483 using harmless fluorescent viruses administered directly into the trachea. Because the viruses were fluorescent, the researchers were able to confirm that the miR was successfully delivered to the lungs. PAH induction using MCT in these rats showed that this miR-483 therapy suppressed PAH-related genes, reducing the effect of the disease, compared with controls. "Overexpression of miR‐483 in endothelial cells (EC s) inhibits inflammatory and fibrogenic responses and leads to ameliorated pulmonary hypertension phenotypes in rats," the researchers wrote. In addition, miR-483 reduced heart enlargement and the thickness and blockage of blood vessels. It also improved the survival of MCT-treated rats. Taken together, the findings suggested that miR-483 constitutes both an useful biomarker and a therapeutic target for PAH. "Our findings herein demonstrate the potential use of … administered miR-483 or agents that elevate endogenous [internal] miR-483 to maintain functional endothelium, at least during the early stages of PAH," the team concluded.  Patricia holds her Ph.D. in Cell Biology from University Nova de Lisboa, and has served as an author on several research projects and fellowships, as well as major grant applications for European Agencies. She also served as a PhD student research assistant in the Laboratory of Doctor David A. Fidock, Department of Microbiology & Immunology, Columbia University, New York. × Patricia holds her Ph.D. in Cell Biology from University Nova de Lisboa, and has served as an author on several research projects and fellowships, as well as major grant applications for European Agencies. She also served as a PhD student research assistant in the Laboratory of Doctor David A. Fidock, Department of Microbiology & Immunology, Columbia University, New York. Latest Posts  |
| How to Become Obsessed with Survival - Pulmonary Hypertension News Posted: 22 Jun 2020 07:00 AM PDT Among the many challenges of living with a life-threatening condition are the daily demands it places on both the patient and the caregivers. A life-threatening illness isn't something one can push to the back of the mind. Every day, people face the need to assess and address myriad symptoms, side effects, and anything out of the norm. To an outsider, this daily evaluation process, combined with frequent doctors' appointments, emergency room visits, hospital admissions, and diligent medical research, might seem obsessive. For my son Cullen and our family, a persistent battle with germs, especially after Cullen's heart and double-lung transplant, has probably made us appear neurotic and overprotective. We were the hand-washing, sanitizer-toting, stay-away-from-us-if-you're-sick, mask-covered people long before it became the norm. Believe it or not, for many years I was blissfully free of medical worry. My children needed a doctor only for wellness checks, immunizations, and occasional ear infections. I vaguely remember the day when a 6-year-old Cullen said, "Mom, I sometimes feel like I can't breathe." But at the same time, he was a healthy, rambunctious child who enjoyed roughhousing with his brother and playing sports. Just watching him made me feel out of breath, so I dismissed his comments while assuming he was breathless from being a highly active little boy. As time went by, it became apparent he was having issues. Cullen's doctor considered asthma as a possibility, so he prescribed an inhaler and gave Cullen permission to resume his normal activities. And so he did — until he started experiencing chest pains. We escalated from seeing a pediatrician to seeing a cardiologist. During the family history portion of the appointment, I shared that I have long QT syndrome. The nervous mom in me asked, "Could Cullen possibly have it, too?" Unfortunately, test results confirmed the condition, but Cullen's was much worse than mine. They placed him on a beta-blocker and told him he should no longer participate in sports. Though it was difficult, Cullen complied, but his symptoms continued to worsen. Soon, he developed a recurring, barking cough that his doctors believed to be croup. Although he was still young, Cullen started advocating for himself by insisting that he was sicker than we thought. This led to the suggestion that Cullen might struggle with anxiety or hypochondria. For two years, we rode this asthma/long QT/anxiety/hypochondria roller coaster, but his dad and I suspected Cullen was suffering from something not yet diagnosed. We became "those parents" who insisted on the power of their instincts. Luckily, Cullen had an excellent pediatrician and cardiologist who didn't feel threatened by our concerns. They recognized that Cullen's symptoms warranted further testing to find the elusive root cause and finally put our worries to rest. An echocardiogram and right heart catheterization eventually confirmed our fears and identified the underlying cause as pulmonary hypertension (PH), a rare, incurable, and life-threatening condition. It was a bittersweet "victory." After struggling for so long with the unknown and Cullen's deteriorating condition, finally putting a name to his disease was almost a relief. Sadly, we now became even more focused on Cullen's health and the health of everyone around him. Exposure to anything contagious, even the common cold, could have led to a serious PH crisis and hospitalization. At the risk of sounding paranoid, we had to educate others. At the beginning of every school year, we met with Cullen's principal, teachers, and staff to update them about his condition and address concerns. We also made a point of making sure Cullen's classmates and their parents were PH-aware, too. As self-conscious as advocating sometimes made us feel, our efforts were not in vain. People became more understanding of our obsessive attention to Cullen's health. But I know it didn't go unnoticed how often Cullen was absent from class or how frequently he visited the emergency room for seemingly minor concerns. Whether symptoms were big or small, we couldn't ignore them. A cough, change in appetite, fatigue, insomnia, edema, aches and pains, skin discoloring, and everything in between often were indications of something that needed to be addressed. It is a common misconception to think Cullen's transplant meant an end to our obsession about his health. Thanks to a healthy heart and new lungs, concerns eventually became less frequent. But the importance of follow-up has once again intensified, this time because of kidney disease. My advice to patients and caregivers witnessing worsening symptoms: Ask doctors to consider the medical zebra and not the common horse in the hoofbeats. For the newly diagnosed with PH or another rare condition: Assiduous self-advocacy will help you maintain a better quality of life and hopefully provide you with more time on this earth. Stay obsessed with surviving. *** Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, BioNews Services, and are intended to spark discussion about issues pertaining to pulmonary hypertension.  |
| Pulmonary Artery Denervation Helps to Treat PAH, Trial Suggests - Pulmonary Hypertension News Posted: 01 Jun 2020 12:00 AM PDT Pulmonary artery denervation was found to be safe and to improve the health of people with pulmonary arterial hypertension (PAH), according to results of an early clinical trial. The study, "Intravascular Ultrasound Pulmonary Artery Denervation to Treat Pulmonary Arterial Hypertension (TROPHY1)," was published in the journal JACC: Cardiovascular Interventions. PAH is characterized by high blood pressure in pulmonary arteries, and patients can experience higher activity in nerves of the lungs. Studies have shown that the targeted removal of nerves — a procedure called denervation, done to ease overactivation of nerves involved in vasoconstriction — surrounding renal arteries can reduce blood pressure in patients with hypertension (high blood pressure). Researchers wondered if using targeted denervation, removing nerves that surround pulmonary blood vessels, could potentially lower blood pressure and alleviate PAH symptoms. They enrolled 23 PAH patients (mean age of 60, range 18 to 75) in a feasibility clinical trial (NCT02835950), called TROPHY1, to investigate the safety and effectiveness of the PDN procedure in this patient population. PDN was performed using a device called the Therapeutic Intra-Vascular Ultrasound System (TIVUS), developed by SoniVie (formerly known as Cardiosonic). TIVUS works by inserting a small catheter into the pulmonary artery, which produces ultrasounds that selectively ablate (remove) the nerves that constrict the pulmonary artery without damaging the vessel walls or adjacent tissues. TIVUS was recognized as a breakthrough device by the U.S. Food and Drug Administration (FDA) in September 2019; this designation is meant to expedite the development and approval of medical equipment that can provide a greater benefit to patients than available treatments. The trial's primary goal trial was to determine the safety of the denervation procedure, determined by adverse events at one month after its use, as well as the overall proportion of patients who survived or who experienced clinical worsening of PAH symptoms over one year. All PAH patients enrolled in TROPHY1 were using at least two oral therapies. They were sedated and given an average of 10 ultrasound activations with TIVUS (minimum 7, maximum 16). The duration of the denervation procedure was, on average, 32 minutes. No serious side effects related to PDN were reported up to 30 days after the procedure — meeting the trial's primary safety goal. In total, 15 serious adverse events occurred within the first 12 months following treatment, but 11 were attributed to PAH itself. Data also showed that survival and clinical worsening among patients undergoing PDN were within the expected ranges for people with PAH. Effectiveness of the procedure in treating PAH was also examined. One measure of effectiveness is pulmonary vascular resistance (PVR), which evaluates the internal resistance to blood flow within the pulmonary arteries. At four or six months post-treatment, PVR had decreased by 17.8% in these patients compared with measures taken at the study's start, or baseline. A significant drop in average pulmonary artery pressure and right atrial pressure at the four- or six-month mark was also observed. Treated patients were also able to significantly increase both the distance that they could walk in six minutes (6-minute walk distance test) — a mean increase of 42 meters (about 138 feet) — and reported a capacity to be more active in daily life (using the emPHasis 10 questionnaire). Overall, "in this multicenter early feasibility study, ultrasound PDN was performed without procedure-related adverse events and was associated with a reduction in PVR and increases in 6-min walk distance and daily activity in patients with PAH on a minimum of dual oral therapy," the researchers wrote. Based on the results, they suggested that PDN could be beneficial to people with PAH and should be considered in the treatment plans along with other therapies. "The health-economic cost of PAH-specific therapy and clinical deterioration is high … as such, a single, or repeated-interval, [PDN] procedure may provide an alternative to therapeutic escalation or an addition to combination therapy," the team wrote. Further studies are necessary to evaluate the long-term effects of PDN and its potential benefit for other forms of pulmonary hypertension. Two of this study's 20 authors are employees of SoniVie, based in Israel.  David earned a PhD in Biological Sciences from Columbia University in New York, NY, where he studied how Drosophila ovarian adult stem cells respond to cell signaling pathway manipulations. This work helped to redefine the organizational principles underlying adult stem cell growth models. He is currently a Science Writer, as part of the BioNews Services writing team. × David earned a PhD in Biological Sciences from Columbia University in New York, NY, where he studied how Drosophila ovarian adult stem cells respond to cell signaling pathway manipulations. This work helped to redefine the organizational principles underlying adult stem cell growth models. He is currently a Science Writer, as part of the BioNews Services writing team. Latest Posts  |
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