#Pompe Disease
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quirinah · 10 months ago
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i! ro! ha!
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vikibro1 · 6 days ago
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Pompe Disease Treatment  Market Opportunities and Forecast By 2032
The Pompe Disease Treatment Market sector is undergoing rapid transformation, with significant growth and innovations expected by 2032. In-depth market research offers a thorough analysis of market size, share, and emerging trends, providing essential insights into its expansion potential. The report explores market segmentation and definitions, emphasizing key components and growth drivers. Through the use of SWOT and PESTEL analyses, it evaluates the sector’s strengths, weaknesses, opportunities, and threats, while considering political, economic, social, technological, environmental, and legal influences. Expert evaluations of competitor strategies and recent developments shed light on geographical trends and forecast the market’s future direction, creating a solid framework for strategic planning and investment decisions.
Brief Overview of the Pompe Disease Treatment Market:
The global Pompe Disease Treatment Market is expected to experience substantial growth between 2024 and 2031. Starting from a steady growth rate in 2023, the market is anticipated to accelerate due to increasing strategic initiatives by key market players throughout the forecast period.
Get a Sample PDF of Report - https://www.databridgemarketresearch.com/request-a-sample/?dbmr=global-pompe-disease-treatment-market
Which are the top companies operating in the Pompe Disease Treatment Market?
The report profiles noticeable organizations working in the water purifier showcase and the triumphant methodologies received by them. It likewise reveals insights about the share held by each organization and their contribution to the market's extension. This Global Pompe Disease Treatment Market report provides the information of the Top Companies in Pompe Disease Treatment Market in the market their business strategy, financial situation etc.
Amicus Therapeutics, Inc (U.S.), Audentes Therapeutics (U.S.), Novartis AG (Switzerland), Fresenius Kabi AG (Germany), Akorn Incorporated (U.S.), Teva Pharmaceutical Industries Ltd (Israel), Mylan N.V (U.S.), Johnsons & Johnsons Services Inc (U.S.), F. Hoffman-La Roche Ltd. (Switzerland), Danaher. (U.S.), B.D. (U.S.), Chembio Diagnostics (U.S.), EKF Diagnostics (U.K.), Trinity Biotech plc (Ireland), Instrumentation Laboratory (U.S.), Nova Biomedical (U.S.), PTS Diagnostics (U.S.), Sekisui Diagnostics (U.S.), Thermo Fisher Scientific (U.S.), bioMérieux S.A. (France)
Report Scope and Market Segmentation
Which are the driving factors of the Pompe Disease Treatment Market?
The driving factors of the Pompe Disease Treatment Market are multifaceted and crucial for its growth and development. Technological advancements play a significant role by enhancing product efficiency, reducing costs, and introducing innovative features that cater to evolving consumer demands. Rising consumer interest and demand for keyword-related products and services further fuel market expansion. Favorable economic conditions, including increased disposable incomes, enable higher consumer spending, which benefits the market. Supportive regulatory environments, with policies that provide incentives and subsidies, also encourage growth, while globalization opens new opportunities by expanding market reach and international trade.
Pompe Disease Treatment Market - Competitive and Segmentation Analysis:
**Segments**
- Treatment Type: The market can be segmented based on treatment type into Enzyme Replacement Therapy (ERT), Gene Therapy, and Others. - Disease Type: Pompe disease treatment market can be segmented by disease type into Infantile-Onset Pompe Disease and Late-Onset Pompe Disease. - Distribution Channel: Based on the distribution channel, the market is segmented into Hospital Pharmacies, Retail Pharmacies, and Online Pharmacies. - Region: The market is segmented into North America, Europe, Asia-Pacific, Latin America, and Middle East & Africa.
The global Pompe disease treatment market is witnessing significant growth due to the increasing prevalence of Pompe disease, coupled with advancements in treatment options. The Enzyme Replacement Therapy (ERT) segment holds a significant share in the market owing to its effectiveness in managing the symptoms of Pompe disease. Gene therapy is also gaining traction as a promising treatment option for patients with Pompe disease. The market is further segmented based on disease type, with Infantile-Onset Pompe Disease being more prevalent among children, while Late-Onset Pompe Disease affects adults. Moreover, the distribution channels play a crucial role in reaching a wider patient population, with hospital pharmacies, retail pharmacies, and online pharmacies catering to the diverse needs of patients worldwide.
**Market Players**
- Sanofi Genzyme - Amicus Therapeutics - Valerion Therapeutics - Audentes Therapeutics - Epirium Bio
The competitive landscape of the global Pompe disease treatment market is characterized by the presence of key players focusing on research and development activities to introduce innovative treatment options. Sanofi Genzyme, a prominent player in the market, offers Enzyme Replacement Therapy (ERT) for the management of Pompe disease. Amicus Therapeutics and Valerion Therapeutics are also actively engaged in developing novel therapies for Pompe disease treatment. Audentes Therapeutics and Epirium Bio are among the emerging players in the market, striving to address the unmet medical needs of patients with Pompe disease through advanced treatment solutions. The market players are collaborating with healthcare organizations and research institutes to enhance their product portfolios and expand their market presence.
https://www.databridgemarketresearch.com/reports/global-pompe-disease-treatment-marketThe global Pompe disease treatment market is poised for substantial growth in the coming years, driven by the rising incidence of Pompe disease and the continuous advancement in treatment modalities. Enzyme Replacement Therapy (ERT) continues to dominate the market as a key treatment option for managing the symptoms of Pompe disease effectively. The development of gene therapy has introduced a new era of hope for patients with Pompe disease, offering a potential curative approach that has garnered significant attention from both patients and healthcare providers.
When examining the market segmentation based on disease type, distinguishing between Infantile-Onset Pompe Disease and Late-Onset Pompe Disease is crucial due to the different clinical presentations and treatment approaches required for each subtype. Infants affected by Pompe disease may experience severe symptoms necessitating early intervention with appropriate treatment, while adults with Late-Onset Pompe Disease may present with milder symptoms that require a different management strategy.
The distribution channels play a pivotal role in ensuring broad access to Pompe disease treatments, with hospital pharmacies, retail pharmacies, and online pharmacies serving as key channels for reaching patients globally. Each distribution channel offers unique advantages in terms of convenience, accessibility, and patient education, contributing to the overall market growth and patient outcomes.
In terms of regional segmentation, North America, Europe, Asia-Pacific, Latin America, and the Middle East & Africa present diverse opportunities and challenges for market players in the Pompe disease treatment sector. Each region has its unique healthcare infrastructure, regulatory environment, and patient demographics, influencing the adoption of Pompe disease therapies and shaping market dynamics. Collaborative efforts between market players and local healthcare stakeholders are essential to navigate these regional nuances effectively and ensure widespread availability of Pompe disease treatments.
As market players, including Sanofi Genzyme, Amicus Therapeutics, Valerion Therapeutics, Audentes Therapeutics, and Epirium Bio, continue to invest in research and development initiatives, the global Pompe disease treatment market is poised for groundbreaking innovations that could revolutionize patient care and outcomes. By leveraging strategic partnerships, expanding clinical trials, and engaging with patient advocacy groups, market players can contribute significantly to advancing the field of Pompe disease treatment and addressing unmet medical needs effectively.
In conclusion, the evolving landscape of the Pompe disease treatment market offers promising opportunities for market players to make a meaningful impact on patients' lives while driving sustainable growth in the sector. With a focus on collaboration, innovation, and patient-centricity, the future of Pompe disease treatment looks poised for transformative change and improved clinical outcomes globally.**Segments**
Global Pompe Disease Treatment Market, By Type (Classic Infantile Form, Non-Classic Infantile Form, Late-Onset Form, Others), Diagnosis (Blood Test, Genetic Test, Prenatal Test, Others), Treatment (Enzyme Replacement Therapy, Supportive Therapies, Others), Route of Administration (Oral, Parenteral, Others), End-Users (Hospitals, Homecare, Speciality Centres, Others), Distribution Channel (Hospital Pharmacy, Online Pharmacy, Retail Pharmacy) & Industry Trends and Forecast to 2029.
The Pompe disease treatment market is segmented based on various factors that influence patient management and market dynamics. The classification into different types, such as Classic Infantile Form, Non-Classic Infantile Form, Late-Onset Form, and Others, considers the different clinical presentations and progression of the disease, leading to tailored treatment approaches for each subtype. The diagnosis segment encompassing Blood Test, Genetic Test, Prenatal Test, and Others highlights the importance of early and accurate detection for initiating appropriate interventions. Furthermore, the distinction in treatment options like Enzyme Replacement Therapy, Supportive Therapies, and others provides a comprehensive approach to managing Pompe disease. The route of administration segmentation considers the ease and efficacy of treatment delivery, with options like Oral, Parenteral, and Others catering to patient needs. End-users such as Hospitals, Homecare, Speciality Centres, and others play a critical role in providing comprehensive care to Pompe disease patients. The distribution channels including Hospital Pharmacy, Online Pharmacy, and Retail Pharmacy ensure broader access to treatment options, enhancing patient convenience and adherence to therapy regimens.
**Market Players**
- Amicus Therapeutics, Inc (U.S.) - Audentes Therapeutics (U.S.) - Novartis AG (Switzerland) - Fresenius Kabi AG (Germany) - Akorn Incorporated (U.S.) - Teva Pharmaceutical Industries Ltd (Israel) - Mylan N.V (U.S.) - Johnsons & Johnsons Services Inc (U.S.) - F. Hoffman-La Roche Ltd. (Switzerland) - Danaher. (U.S.) - B.D. (U.S.) - Chembio Diagnostics (U.S.) - EKF Diagnostics (U.K.) - Trinity Biotech plc (Ireland) - Instrumentation Laboratory (U.S.) - Nova Biomedical (U.S.) - PTS Diagnostics (U.S.) - Sekisui Diagnostics (U.S.) - Thermo Fisher Scientific (U.S.) - bioMérieux S.A. (France)
The market participants in the global Pompe disease treatment sector play a crucial role in driving innovation, research, and access to care for patients affected by the disease. Amicus Therapeutics, Inc. and Audentes Therapeutics are key players focusing on developing novel therapies to address the unmet medical needs of Pompe disease patients. Novartis AG and Fresenius Kabi AG contribute to the market with their commitment to advancing treatment options and ensuring quality healthcare delivery. Akorn Incorporated, Teva Pharmaceutical Industries Ltd, and Mylan N.V are actively involved in providing therapeutic solutions for Pompe disease management, enhancing patient outcomes worldwide. Johnsons & Johnsons Services Inc, F. Hoffman-La Roche Ltd., Danaher, and B.D. are prominent companies engaged in research and development activities to introduce advanced therapies for treating Pompe disease effectively. Chembio Diagnostics, EKF Diagnostics, Trinity Biotech plc, and other diagnostic companies play a critical role in enabling early and precise diagnosis of Pompe disease, facilitating timely interventions and improved patient care. Instrumentation Laboratory, Nova Biomedical, PTS Diagnostics, Sekisui Diagnostics, Thermo Fisher Scientific, and bioMérieux S.A. contribute to the market by providing diagnostic tools and technologies that support healthcare professionals in managing Pompe disease effectively.
The collaboration and competition among these market players drive the Pompe disease treatment market's growth, leading to enhanced therapeutic options, improved patient outcomes, and increased awareness about the disease globally. As these companies continue to invest in research, innovation, and strategic partnerships, the industry is poised for significant advancements in Pompe disease management, paving the way for a brighter future for patients with this rare genetic disorder.
North America, particularly the United States, will continue to exert significant influence that cannot be overlooked. Any shifts in the United States could impact the development trajectory of the Pompe Disease Treatment Market. The North American market is poised for substantial growth over the forecast period. The region benefits from widespread adoption of advanced technologies and the presence of major industry players, creating abundant growth opportunities.
Similarly, Europe plays a crucial role in the global Pompe Disease Treatment Market, expected to exhibit impressive growth in CAGR from 2024 to 2032.
Explore Further Details about This Research Pompe Disease Treatment Market Report https://www.databridgemarketresearch.com/reports/global-pompe-disease-treatment-market
Key Benefits for Industry Participants and Stakeholders: –
Industry drivers, trends, restraints, and opportunities are covered in the study.
Neutral perspective on the Pompe Disease Treatment Market scenario
Recent industry growth and new developments
Competitive landscape and strategies of key companies
The Historical, current, and estimated Pompe Disease Treatment Market size in terms of value and size
In-depth, comprehensive analysis and forecasting of the Pompe Disease Treatment Market
Geographically, the detailed analysis of consumption, revenue, market share and growth rate, historical data and forecast (2024-2031) of the following regions are covered in Chapters
The countries covered in the Pompe Disease Treatment Market report are U.S., Canada and Mexico in North America, Brazil, Argentina and Rest of South America as part of South America, Germany, Italy, U.K., France, Spain, Netherlands, Belgium, Switzerland, Turkey, Russia, Rest of Europe in Europe, Japan, China, India, South Korea, Australia, Singapore, Malaysia, Thailand, Indonesia, Philippines, Rest of Asia-Pacific (APAC)  in the Asia-Pacific (APAC), Saudi Arabia, U.A.E, South Africa, Egypt, Israel, Rest of Middle East and Africa (MEA) as a part of Middle East and Africa (MEA
Detailed TOC of Pompe Disease Treatment Market Insights and Forecast to 2032
Part 01: Executive Summary
Part 02: Scope Of The Report
Part 03: Research Methodology
Part 04: Pompe Disease Treatment Market Landscape
Part 05: Pipeline Analysis
Part 06: Pompe Disease Treatment Market Sizing
Part 07: Five Forces Analysis
Part 08: Pompe Disease Treatment Market Segmentation
Part 09: Customer Landscape
Part 10: Regional Landscape
Part 11: Decision Framework
Part 12: Drivers And Challenges
Part 13: Pompe Disease Treatment Market Trends
Part 14: Vendor Landscape
Part 15: Vendor Analysis
Part 16: Appendix
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surkuted · 1 year ago
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https://biiut.com/read-blog/59398
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roseaa11 · 2 years ago
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The global pompe disease treatment market is projected to witness significant growth during the forecast period. In U.S., projected occurrence are approximately 1 in 40,000 births. Males and females are equally affected by the diseases. Medical advancements that involves ongoing drug discovery, and government and non-government organizational initiatives are what that boosts the market growth.  
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sravanthiiarc · 2 years ago
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thecreaturecodex · 2 months ago
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Sangubrax, the Smile in the Shadows
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"Nightmare--Revenge" © ArtStation user Midnight, accessed at their gallery here
[Sponsored by @tar-baphon, who wanted a CR 15 herald, suitable for greater planar ally spells, for Nyarlathotep, specifically in the Bloody Tongue mask. The Bloody Tongue is one of the widest influences of the Call of Cthulhu game on greater views of the Mythos; Nyarlathotep was not associated with being a tentacle monster before that point, and was usually depicted as being mostly human, as he appears in Lovecraft. Hence, my feeling it was appropriate to make this herald appear pretty humanoid. The epithet and CR were provided for me, but I was allowed free reign otherwise.]
Sangubrax, the Smile in the Shadows CR 15 CE Outsider (extraplanar) This humanoid creature appears to be a flayed knight made of blood and gore. There is a visor covering where there should be eyes, and his face is split in a mad ear to ear grin. His arms end not in hands, but in baroque and lethal weapons. He moves with liquid grace.
Sangubrax is the Smile In the Shadows, a supernatural murderer with a penchant for targeting nobility, royalty, the powerful and influential. He is one of the Heralds of Nyarlathotep, being devoted specifically to the Mask known as the Bloody Tongue. The Bloody Tongue seeks to destabilize societies through sacrificial murders, and Sangubrax is called upon to strike at reformers, benevolent leaders, and anyone who might try to make a city, province or nation safer and more supporting of its citizens.
Sangubrax is a consummate assassin, but he has a penchant for the theatrical. Murders he commits vary in tune with the societal importance of the target. A beggar that gets in his way will merely have his throat slit, but a monarch will be murdered in broad daylight with much pomp and ceremony. His arms can become all manner of weaponry, but he prefers sword-like blades, the better to spill plenty of blood. Creatures he strikes bleed more and more until their entire life comes gushing out, and he has an extensible, chameleon-like tongue for dragging fleeing victims closer and ensuring they cannot even magically escape.
The Smile in the Shadows has a mocking, sardonic personality, even to his summoners. He believes that mortals are fundamentally incapable of ruling themselves, and that the return of the Old Gods will be a time of glorious and violent anarchy. He serves in the Prehistoric Temple in Leng, acting as bodyguard and majordomo for the High Priest Not to Be Described. Although he performs such duties loyally, he is always excited to be summoned in order to commit murder and mayhem on another plane.
Being a semi-liquid creature, Sangubrax has no set height, but weighs 200 pounds. Although he has no eyes, he can see perfectly well.
Sangubrax CR 15 XP 51,200 CE Medium outsider (chaos, evil, extraplanar) Init +7; Senses lifesense 60 ft., Perception +28, see in darkness Aura frightful presence (60 ft., Will DC 25)
Defense AC 30, touch 18, flat-footed 30 (+7 Dex, +1 dodge, +12 natural) hp 225 (18d10+126) Fort +13, Ref +18, Will +18 DR 15/lawful or good; Immune bleed, curses, death effects, disease, poison; SR 26 Defensive Abilities amorphous, evasion, improved uncanny dodge
Offense Speed 30 ft., fly 30 ft. (perfect) Melee 2 morphic weapons +24 (2d10+6/19-20 plus exponential bleed), tongue +19 touch (pull plus no escape) Ranged morphic weapon +24 (2d10+6/19-20 plus exponential bleed) Space 5 ft.; Reach 10 ft. (30 ft. with tongue) Special Attacks pull (10 ft.), sneak attack +4d6 Spell-like Abilities CL 18th, concentration +24 At will—deeper darkness, detect good, detect law, invisibility, waves of blood (DC 19, CMB +25) 3/day—cruel jaunt, mass fester (DC 22), quickened true strike, empowered vampiric touch 1/day—decapitate (DC 22), greater shadow evocation (DC 24), regenerate
Statistics Str 23, Dex 24, Con 25, Int 20, Wis 25, Cha 22 Base Atk +18; CMB +22; CMD 40 Feats Combat Expertise, Combat Reflexes, Dodge, Empower SLA (vampiric touch), Improved Critical (morphic weaponry), Mobility, Quicken SLA (true strike), Spring Attack, Whirlwind Attack Skills Acrobatics +28, Bluff +27, Escape Artist +36, Fly +33, Intimidate +27, Knowledge (nobility) +23, Knowledge (planes) +26, Perception +28, Sense Motive +28, Stealth +36, Survival +28; Racial Modifiers +8 Escape Artist, +8 Stealth Languages Aklo, Abyssal, Common, Protean, Shadowtongue SQ compression
Ecology Environment any land or underground (Leng) Organization unique Treasure incidental
Special Abilities Exponential Bleed (Su) Whenever Sangubrax hits a creature with a morphic weapon, it deals 1 point of bleed damage. If it hits that creature again, the bleed damage doubles, to a maximum of 16 points of bleed a round for 5 hits. This doubling effect occurs even if the creature stops bleeding, such as through a Heal check or healing magic, but expires 24 hours after the last time Sangubrax struck it. In an area of antimagic or similar effect, Sangubrax’s morphic weaponry still deals 1 point of bleed damage. Morphic Weapons (Ex) Sangubrax can manipulate his body to mimic a wide array of potent weaponry, or revert to having hands, as a swift action. While the weapons may look different, they are considered natural weapons and they all do the same amount of damage. Morphic weapons deal bludgeoning, piercing, and slashing damage, and are treated as magic for the purpose of overcoming damage reduction. Sangubrax can also attack with a morphic weapon by hurling a portion of his body as a thrown weapon with a range increment of 60 feet (he swiftly regrows thrown portions of its body after using this attack, while the hurled portion melts away into a bloodstain after dealing damage to a target). No Escape (Su) A creature touched by Sangubrax’s tongue must succeed a DC 24 Will save or be affected by both a slow and dimensional anchor spell that lasts for 1 minute. The save DC is Charisma based.
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b-does-the-write-thing · 7 months ago
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general incivility, chapter four
                              - a brienne x jaime pride & prejudice retelling -
chapter one l chapter two  l chapter three l chapter four
The Stormlands were, like most of the families who had settled there, rather unremarkable. The rocky region lay nestled between the more wealthy Crownlands to the north and the more fertile Reach to the west. Most ever bothered to cross the mountain range that separated the regions, and to the south was nothing but the Dornish desert, as inhospitable as its people from what Septa Roelle had taught her. 
It was a rugged land, as large and sprawling, pitted and scarred as she was. It would never be considered beautiful but it was what it was. One either learned to love it or they hated it and Brienne had adopted the same practice to her own life. 
Many a day, Brienne went riding. Usually to train, but other days, just as an excuse to stretch her legs and clear her mind. But today, fresh from a wash after her morning bout with the Colonel, Brienne set out on the path towards the closet manor home, Storm’s End, to pay a call upon her most intimate of friends—her only friend to speak plainly. 
Storm’s End was perched upon Durran’s Point, the southernmost stop of the King’s Road. Here, the elder of King Robert’s younger brothers had made his home nearly a decade ago now. Brienne had been too young to attend but her father had told her stories of the great retinue that had arrived with Stannis Baratheon and his young wife and how all of the Stormlands had celebrated for seven days straight before King Robert had returned north to his iron throne. 
If the people of the Stormlands had hoped for a lively royal in their midst, they were sadly disappointed. Stannis Baratheon lacked the love of pomp and party that his two brothers had inherited, preferring the solemn and dreary coasts of Storm’s End to the other manors he may have claimed for his own as the King’s brother and heir. 
As they arrived at the manor’s gates, Septa Roelle turned her nose up. “Oh, Lady Selyse is at home,” she remarked in the same tone she pointed out mice droppings. Septa Roelle liked few people but she actively disliked even fewer, but somehow Lady Selyse Baratheon had never risen high in the Septa's mind. 
The Bartheons only had the one daughter, an intelligent, sweet young debutant of fifteen who though Brienne's junior, was more mature than any of the other ladies in the region.  She was also, like Brienne, no stranger to cruelty at the sake of her appearance.
As tall and thin as both her parents, Shireen had the Baratheon bold blue eyes and the equally strong, jutting jaw which may have made her handsome if not partnered with her mother’s large ears and aquiline nose. 
Fate had taken a hand in Shireen’s appearance as well. While still in the cradle, Shireen had been afflicted with grayscale. While she had recovered, she had been left with gray and black mottled scars all across her left cheek and down to her neck. 
The more superstitious families avoided the Baratheons, believing the disease lay dormant in the skin and could be reawoken with a single sneeze. Folly, according to all the maesters but still even the more opportunistic fortune hunters steered clear of the young Lady Baratheon. Septa Roelle had also been conflicted. On the one hand, her charge rubbing elbows with royalty, and on the other, a disease so deadly that its mere name was considered dangerous.
Thankfully, royal blood, diseased or not, won out in the end, and the two unfortunates became fast friends. Shireen liked the loyal and true Brienne Tarth, finding her refreshing and more intelligent than any of the other ladies her mother tried to foster upon her, while Brienne liked the quiet solitude of Shireen’s company. Shireen never stared or ogled or winced and, on numerous occasions, put herself pointedly between Brienne and her tormentors at assemblies so no one would jokingly ask Brienne the Beauty for a dance. 
Shireen had been in attendance at the assembly though her mother had kept her occupied with both Lannister brothers. “Brienne tells me you were the first to dance with Mr. Lannister,” Septa Roelle praised Shireen as they all sat down in the sitting room over a cup of tea. 
Lady Baratheon beamed at the accomplishment, but Shireen was quick to deflect any praise. “Yes, but he danced with nearly every lady present. Though, he did ask after you, Brienne.”
Septa Roelle perked up at this. “Oh?”
“Yes, but it was later in the evening, and poor Mr. Tarth had already called for the chaise. Mr. Lannister did seem rather disappointed.”
“My departure, if truly noticed, was nothing but a slight inconvenience, if that.” Brienne insisted lest Septa Roelle get the wrong idea. 
“Oh, but he was most sincere,” Shireen protested. “He mentioned you had spoken for a moment and while he was rudely whisked away, he had great hopes of the two of you finishing the conversation before the night came to an end.”
“I dare say he’ll have to get used to disappointment,” Brienne murmured into her cup. 
Shireen heard it and gave her a knowing smile, but Septa Roelle had already moved on to the next eligible bachelor. “Is there any truth to this rumor that the elder Lannister is set to marry the cousin that was in attendance?” 
“It was obvious to even that blind old bat Whent,” Lady Baratheon confirmed. “Ms. Lannister did deign to dance with some of the local gentlemen but never more than once. As for the Lion of Lannister, he was most disagreeable. I was in his company for nearly the entire evening, and he barely uttered a word. Most unfortunate. Good breeding does not always result in good manners.” 
Brienne could only imagine what Jaime Lannister had thought of Selyse Baratheon shadowing his every move. His unpleasant mood became less of a mystery. 
“The youngest Mr. Lannister told me his brother is not much for conversation among strangers but is a remarkably agreeable fellow among his intimate acquaintances,” Shireen contributed.
“Simply making excuses for his family,” her mother replied. “It was clear to the whole assembly that he is a sinfully prideful man. Comely or not, I’ll be happy to see the back of him.” 
“I do not see why he should not be proud. He is the most handsome man I have ever seen, and his family is reported to have more wealth than even Uncle Robert’s treasury. If our roles were reversed, I would surely be proud, wouldn’t you, mother?”
Selyse Baratheon bristled at the suggestion. “I certainly would not. I am not so vain as that, child!”
“Vanity is something different entirely,” Septa Roelle corrected. “A person may be proud without being vain. Pride is our opinion of oneself; vanity is what we would have others think.”
The two older women soon fell into debate on the subject, with Septa Roelle taking the high ground of her faith and Lady Baratheon that of her education.  Shireen scooted closer under the guise of rearranging her skirts. “All this talk of pride and vanity, but you never said, what did you think of Mr. Tyrion Lannister, my dear Brienne?”
Ensuring Septa Roelle was caught up in the debate, she confessed, “I found him to be an odd sort of fellow.”
“How so?”
Brienne shared what she had overheard, speaking low so as not to be overheard. By the end, Shireen was clearly amused. “He ought not to have said those things,” she conceded, though it was unclear if she was speaking of the younger or elder Lannister.
“It was not gentleman-like,” Brienne conceded,” but neither of them were wrong.”
Shireen lay her hand upon Brienne’s to administer a gentle squeeze. “You are too kind to others and much too hard on yourself,” she admonished. 
“Careful, lest you make me too vain of my so-called good nature,” Brienne teased.
“Never. I can only attempt to make you proud of it,” Shireen rallied back.
“Girls? What are you two whispering about?” Septa Roelle demanded. They quickly echoed platitudes about the weather and the rest of the visit was spent discussing the health of Lady Whent. 
--- Just some general story establishing today, folks. Next chapter, everyone is back.
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masshu-art · 4 days ago
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Not finished yet but in time I ran out of paint 🎨 🤣 but the start of Leo's room whilst he's in Claire house pretty happy with the look so far inspiration from Leo's first book I illustrated and wrote the floppy slothy :)
https://pompe.uk/publications-library/leo-and-the-floppy-sloth want to do something amazing this year by even sharing this it's spreads the word about infantile pompe disease a disease that has no cure and uncertain. This book is a child friendly positive life experience I share with my son who's fighting infantile pompe disease if you wish to donate to this charity or even share to spread the awareness maybe one day we will have a cure #cure #pompe #infantile #nhs #lifelimiting #awareness #health #donate #share #facebookviral
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aceofcupsbiggestfan · 10 months ago
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Dionysia ta Megala
~ 10-17 Elaphebolion ~
Dionysia ta Megala translated to Greater Dionysia (or Dionysia ta astika/City Dionysia), was the second most important Dionysian Festival of the year.
City Dionysia celebrated the end of winter, as well as the crop harvest of the year. Nearing around the Spring Equinox, you can also celebrate as a welcoming of spring.
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The festival has it origins in Eleutherea, a town north of Attica. A myth came about that when the Athenians rejected the Dionysian cult, he cast upon the men a disease on their genitals.
This lead to the Athenians hosting Dionysia ta Megala, as honoring Dionysus after their misfalling. Because of this particpants carried a large phallus across the polis durng Dionysia.
Several things were done every City Dionysia. Starting with the pompe, citizens continued with komos after.
Modern Hellenes can celebrate City Dionysia by following the pompe and the komos.
Plan your own route to walk, talking time to celebrate Dionysus by carrying a statue or a reminder of him.
Find a City Dionysia festival or group to join! Sing loudly and go out with friends to raucous parties. Have a feast, holding a space for Dionysus. Watch theatre, whether modern or ancient and sing hymns!
Traditional Offerings:
-phallus shaped objects
-oboloi
-wine
-water
-statues of Dionysus
Traditional Acts:
-party
-sing hymns
-hold a feast
-watch plays
-drink wine
-parade through the streets
-role-reversing
Khaire Dionysus! 🐆🍷🍇
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brinefathomcaves · 3 months ago
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Nov 9: The Wheel of Fortune
The walls of this chamber are hung with banners depicting humanoid body parts: Eyeballs, skinless hands, anatomical hearts. The banners are always moist. On the raised platform, 10’ of stairs above the rest of the room, a flayed humanoid corpse is nailed to a wheel. Once per day, a creature can spin the wheel to receive the following:
d10
Disgust: Gain one level of exhaustion as the corpse spits in your face.
Confusion: Be under the effect of the confusion spell during the first round (and surprise round, if applicable) of each combat for the next 24 hours.
Ridicule: The corpse looks the creature in the eye and laughs. No game effect.
Pomp: A dramatic puff of sulfurous smoke, after which nothing happens. No game effect.
Betrayal: Grow devil horns, cloven hooves, or another signifier of an infernal nature. No game effect.
Foresight: For the next week, gain the ability to cast augury once a day.
Quiet: For the next week, gain the ability to cast silence once a day.
Vision: Gain the ability to see 120’ normally  in mundane and magical darkness for 24 hours.
Fortitude: Gain resistance to cold and fire damage for 24 hours.
Vigor: Immediately healed to full health. All diseases, poisons, and curses on the creature are cured.
If the corpse is removed from the wheel, it crumbles to dust, and spinning the wheel has no effect until another corpse is skinned and nailed on.
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eliotrosechild · 3 months ago
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Pigeon Pomp
The park and bus platforms are, at midday, same
as a walk by the seaboard rushed with breeze,
or the cranny grandstands hours after a game.
There, where hunger begets the fever for seed,
you find my head, sullen, with social ease,
chomping, stomping, pigeon pomping through need.
Man asks Pigeon: How does pomping suit your need?
Pigeon tells Man: Suits are pompous, needs are same.
You and we, bound by blood, cloaked with disease,
envy and pride, form graceless chords to the breeze,
mar the tokens of Regality’s seed,
and rake life’s contract with our pompous game.
By our sails, we are greatness in sport, a game
we love and hate. We fly and fie by need.
We take by want. We rape to spread our seed.
Dear murderous man, our pomp suits you the same.
Yet we are innocent when we yield to the breeze,
Juxtaposed, fat bellies of crumb fed ease.
If these pigeon thoughts offend your Gentle ease,
perhaps you ignore the pomp of man’s game,
where pride, envy and other chords hang the breeze:
man wakes to tame the day, anticipates need
and takes without remorse or shame. This same
taking is as common to hand as beak to seed.
We come to the ripe ground to eat the seed
en masse. Tireless at work, but sporting an ease,
in Congress we hoot, peck, hoot. While sesame
slips from our tongues, we’re coy to rethink this game—
“Could life, without competing, unscratch our need?”—
but the words taste dry as cotton-curled breeze.
Will we forever fly in a waking breeze?
Will we yield to the best the ripest seed?
Are we damned to hoot and peck the pomp of need?
An unsettled beak and neck finds no ease
competing in an uncompleting game:
these pompous virtues make man and pigeon, same.
At ease, my sweet, fat, pigeon man, you need
the man-bird to study its game and learn, same
as I, how pompous seed breeds a foul breeze.
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vikibro1 · 12 days ago
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Pompe Disease Treatment  Market Trends, Opportunities and Forecast By 2032
The Pompe Disease Treatment Market sector is undergoing rapid transformation, with significant growth and innovations expected by 2032. In-depth market research offers a thorough analysis of market size, share, and emerging trends, providing essential insights into its expansion potential. The report explores market segmentation and definitions, emphasizing key components and growth drivers. Through the use of SWOT and PESTEL analyses, it evaluates the sector’s strengths, weaknesses, opportunities, and threats, while considering political, economic, social, technological, environmental, and legal influences. Expert evaluations of competitor strategies and recent developments shed light on geographical trends and forecast the market’s future direction, creating a solid framework for strategic planning and investment decisions.
Brief Overview of the Pompe Disease Treatment Market:
The global Pompe Disease Treatment Market is expected to experience substantial growth between 2024 and 2031. Starting from a steady growth rate in 2023, the market is anticipated to accelerate due to increasing strategic initiatives by key market players throughout the forecast period.
Get a Sample PDF of Report - https://www.databridgemarketresearch.com/request-a-sample/?dbmr=global-pompe-disease-treatment-market
Which are the top companies operating in the Pompe Disease Treatment Market?
The report profiles noticeable organizations working in the water purifier showcase and the triumphant methodologies received by them. It likewise reveals insights about the share held by each organization and their contribution to the market's extension. This Global Pompe Disease Treatment Market report provides the information of the Top Companies in Pompe Disease Treatment Market in the market their business strategy, financial situation etc.
Amicus Therapeutics, Inc (U.S.), Audentes Therapeutics (U.S.), Novartis AG (Switzerland), Fresenius Kabi AG (Germany), Akorn Incorporated (U.S.), Teva Pharmaceutical Industries Ltd (Israel), Mylan N.V (U.S.), Johnsons & Johnsons Services Inc (U.S.), F. Hoffman-La Roche Ltd. (Switzerland), Danaher. (U.S.), B.D. (U.S.), Chembio Diagnostics (U.S.), EKF Diagnostics (U.K.), Trinity Biotech plc (Ireland), Instrumentation Laboratory (U.S.), Nova Biomedical (U.S.), PTS Diagnostics (U.S.), Sekisui Diagnostics (U.S.), Thermo Fisher Scientific (U.S.), bioMérieux S.A. (France)
Report Scope and Market Segmentation
Which are the driving factors of the Pompe Disease Treatment Market?
The driving factors of the Pompe Disease Treatment Market are multifaceted and crucial for its growth and development. Technological advancements play a significant role by enhancing product efficiency, reducing costs, and introducing innovative features that cater to evolving consumer demands. Rising consumer interest and demand for keyword-related products and services further fuel market expansion. Favorable economic conditions, including increased disposable incomes, enable higher consumer spending, which benefits the market. Supportive regulatory environments, with policies that provide incentives and subsidies, also encourage growth, while globalization opens new opportunities by expanding market reach and international trade.
Pompe Disease Treatment Market - Competitive and Segmentation Analysis:
**Segments**
- Based on product type, the Pompe Disease Treatment Market is segmented into Enzyme Replacement Therapy (ERT) and Substrate Reduction Therapy (SRT). ERT segment is expected to dominate the market as it is the most widely used treatment for Pompe disease. - On the basis of application, the market is divided into Late-Onset Pompe Disease and Infantile-Onset Pompe Disease. The infantile-onset segment holds the largest market share due to the early onset of symptoms and higher treatment demand. - By distribution channel, the market is categorized into Hospital Pharmacies, Retail Pharmacies, and Online Pharmacies. The hospital pharmacies segment is anticipated to have significant growth owing to the availability of specialized treatment facilities. - Considering the end-user, the market is segmented into Hospitals, Specialized Clinics, and Others. Hospitals hold a major share in the market due to the presence of advanced medical infrastructure and skilled healthcare professionals.
**Market Players**
- Amicus Therapeutics, Inc. - Sanofi - BioMarin - Valerion Therapeutics - Audentes Therapeutics - EpiVax - Oxyrane - REGENXBIO Inc. - JCR Pharmaceuticals Co., Ltd. - Avrobio, Inc.
The global Pompe Disease Treatment Market is witnessing significant growth and is expected to continue its positive trajectory until 2032. The market is driven by factors such as increasing awareness about rare diseases, technological advancements in treatment options, and favorable government initiatives supporting healthcare infrastructure development. The rising prevalence of Pompe disease, particularly in infants, is also a key factor contributing to market growth.
On the basis of region, North America is anticipated to dominate the market due to the presence of well-established healthcare infrastructure, high healthcare expenditure, and increasing R&D activities in the pharmaceutical sector. Europe is expected to hold a substantial market share, driven by the growing geriatric population and rising healthcare investments. The Asia Pacific region is projected to witness the fastest growthThe global Pompe Disease Treatment Market is experiencing significant growth and is poised to maintain its upward trajectory until 2032. The market is segmented based on various factors ranging from product type to distribution channels and end-users. Enzyme Replacement Therapy (ERT) is currently the dominant segment in the market due to its widespread usage in Pompe disease treatment. However, Substrate Reduction Therapy (SRT) is also gaining traction as an alternative treatment option. The application segment is divided into Late-Onset Pompe Disease and Infantile-Onset Pompe Disease, with the latter holding a larger market share due to the early onset of symptoms and higher treatment demand.
In terms of distribution channels, Hospital Pharmacies are expected to witness significant growth, attributed to the availability of specialized treatment facilities. The end-user segment includes Hospitals, Specialized Clinics, and Others, with Hospitals leading the market share due to advanced medical infrastructure and skilled healthcare professionals. Market players such as Amicus Therapeutics, Inc., Sanofi, BioMarin, and others are actively contributing to the market growth through research and development of innovative treatment options.
North America is projected to dominate the global market, driven by well-established healthcare infrastructure and high healthcare expenditure. Europe is expected to hold a substantial market share due to a growing geriatric population and increased healthcare investments. The Asia Pacific region is forecasted to witness the fastest growth in the Pompe Disease Treatment Market, attributed to rising awareness about rare diseases, improving healthcare infrastructure, and increasing government initiatives to support healthcare development.
Technological advancements in treatment options, coupled with favorable government initiatives supporting healthcare infrastructure development, are key factors propelling market growth. The increasing prevalence of Pompe disease, especially in infants, is further boosting market demand. Overall, the global Pompe Disease Treatment Market is set for continued growth in the coming years, offering ample opportunities for market players to innovate and expand their presence in the industry.**Market Players**
- Amicus Therapeutics, Inc. (U.S.) - Audentes Therapeutics (U.S.) - Novartis AG (Switzerland) - Fresenius Kabi AG (Germany) - Akorn Incorporated (U.S.) - Teva Pharmaceutical Industries Ltd (Israel) - Mylan N.V (U.S.) - Johnsons & Johnsons Services Inc (U.S.) - F. Hoffman-La Roche Ltd. (Switzerland) - Danaher (U.S.) - B.D. (U.S.) - Chembio Diagnostics (U.S.) - EKF Diagnostics (U.K.) - Trinity Biotech plc (Ireland) - Instrumentation Laboratory (U.S.) - Nova Biomedical (U.S.) - PTS Diagnostics (U.S.) - Sekisui Diagnostics (U.S.) - Thermo Fisher Scientific (U.S.) - bioMérieux S.A. (France)
The global Pompe Disease Treatment Market is experiencing robust growth driven by factors such as increasing awareness of rare diseases, technological advancements in treatment options, and supportive government initiatives for healthcare infrastructure development. The market segmentation based on product type includes Enzyme Replacement Therapy (ERT) and Substrate Reduction Therapy (SRT), with ERT currently dominating the market due to its widespread use. However, SRT is gaining traction as an alternative treatment option. In terms of applications, the market is divided into Late-Onset Pompe Disease and Infantile-On
North America, particularly the United States, will continue to exert significant influence that cannot be overlooked. Any shifts in the United States could impact the development trajectory of the Pompe Disease Treatment Market. The North American market is poised for substantial growth over the forecast period. The region benefits from widespread adoption of advanced technologies and the presence of major industry players, creating abundant growth opportunities.
Similarly, Europe plays a crucial role in the global Pompe Disease Treatment Market, expected to exhibit impressive growth in CAGR from 2024 to 2032.
Explore Further Details about This Research Pompe Disease Treatment Market Report https://www.databridgemarketresearch.com/reports/global-pompe-disease-treatment-market
Key Benefits for Industry Participants and Stakeholders: –
Industry drivers, trends, restraints, and opportunities are covered in the study.
Neutral perspective on the Pompe Disease Treatment Market scenario
Recent industry growth and new developments
Competitive landscape and strategies of key companies
The Historical, current, and estimated Pompe Disease Treatment Market size in terms of value and size
In-depth, comprehensive analysis and forecasting of the Pompe Disease Treatment Market
Geographically, the detailed analysis of consumption, revenue, market share and growth rate, historical data and forecast (2024-2031) of the following regions are covered in Chapters
The countries covered in the Pompe Disease Treatment Market report are U.S., Canada and Mexico in North America, Brazil, Argentina and Rest of South America as part of South America, Germany, Italy, U.K., France, Spain, Netherlands, Belgium, Switzerland, Turkey, Russia, Rest of Europe in Europe, Japan, China, India, South Korea, Australia, Singapore, Malaysia, Thailand, Indonesia, Philippines, Rest of Asia-Pacific (APAC)  in the Asia-Pacific (APAC), Saudi Arabia, U.A.E, South Africa, Egypt, Israel, Rest of Middle East and Africa (MEA) as a part of Middle East and Africa (MEA
Detailed TOC of Pompe Disease Treatment Market Insights and Forecast to 2032
Part 01: Executive Summary
Part 02: Scope Of The Report
Part 03: Research Methodology
Part 04: Pompe Disease Treatment Market Landscape
Part 05: Pipeline Analysis
Part 06: Pompe Disease Treatment Market Sizing
Part 07: Five Forces Analysis
Part 08: Pompe Disease Treatment Market Segmentation
Part 09: Customer Landscape
Part 10: Regional Landscape
Part 11: Decision Framework
Part 12: Drivers And Challenges
Part 13: Pompe Disease Treatment Market Trends
Part 14: Vendor Landscape
Part 15: Vendor Analysis
Part 16: Appendix
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wolfcake101 · 10 months ago
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uhh facts about baldi's basics ms wc i should probably mention
i call her bbieal ms wc as to not cause confusion with myself. she goes by Wolfie though
she is made of plastic! she is 3d printed. thats why she's 3d sometimes. though most of the time i draw her 2d because its less time consuming
she IS diseased, but not contagious
those fun splotches on her are paint
she is 15 years old
mrs. pomp absolutely terrifies her
as you already know she looks up to dr. reflex a lot and likes to follow him around when possible
she ends up having to see dr. reflex a LOT because of how ill she is
also those marks on her are bruises
she likes drawing a lot!!
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lauvra · 8 months ago
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The Progress of Poesy: A Pindaric Ode by Thomas Gray
I.1.
         Awake, Æolian lyre, awake, And give to rapture all thy trembling strings. From Helicon's harmonious springs A thousand rills their mazy progress take: The laughing flowers, that round them blow, Drink life and fragrance as they flow. Now the rich stream of music winds along Deep, majestic, smooth, and strong, Thro' verdant vales, and Ceres' golden reign: Now rolling down the steep amain, Headlong, impetuous, see it pour: The rocks and nodding groves rebellow to the roar.
I.2.
         Oh! Sovereign of the willing soul, Parent of sweet and solemn-breathing airs, Enchanting shell! the sullen Cares And frantic Passions hear thy soft control. On Thracia's hills the Lord of War, Has curb'd the fury of his car, And dropp'd his thirsty lance at thy command. Perching on the sceptred hand Of Jove, thy magic lulls the feather'd king With ruffled plumes and flagging wing: Quench'd in dark clouds of slumber lie The terror of his beak, and light'nings of his eye.
I.3.
         Thee the voice, the dance, obey, Temper'd to thy warbled lay. O'er Idalia's velvet-green The rosy-crowned Loves are seen On Cytherea's day With antic Sports and blue-ey'd Pleasures, Frisking light in frolic measures; Now pursuing, now retreating, Now in circling troops they meet: To brisk notes in cadence beating Glance their many-twinkling feet. Slow melting strains their Queen's approach declare: Where'er she turns the Graces homage pay. With arms sublime, that float upon the air, In gliding state she wins her easy way: O'er her warm cheek and rising bosom move The bloom of young Desire and purple light of Love.
II.1.
         Man's feeble race what ills await, Labour, and Penury, the racks of Pain, Disease, and Sorrow's weeping train, And Death, sad refuge from the storms of Fate! The fond complaint, my song, disprove, And justify the laws of Jove. Say, has he giv'n in vain the heav'nly Muse? Night, and all her sickly dews, Her spectres wan, and birds of boding cry, He gives to range the dreary sky: Till down the eastern cliffs afar Hyperion's march they spy, and glitt'ring shafts of war.
II.2.
         In climes beyond the solar road, Where shaggy forms o'er ice-built mountains roam, The Muse has broke the twilight-gloom To cheer the shiv'ring native's dull abode. And oft, beneath the od'rous shade Of Chili's boundless forests laid, She deigns to hear the savage youth repeat In loose numbers wildly sweet Their feather-cinctur'd chiefs, and dusky loves. Her track, where'er the goddess roves, Glory pursue, and generous Shame, Th' unconquerable Mind, and Freedom's holy flame.
II.3.
         Woods, that wave o'er Delphi's steep, Isles, that crown th' Ægean deep, Fields, that cool Ilissus laves, Or where Mæander's amber waves In ling'ring Lab'rinths creep, How do your tuneful echoes languish, Mute, but to the voice of Anguish? Where each old poetic mountain Inspiration breath'd around: Ev'ry shade and hallow'd Fountain Murmur'd deep a solemn sound: Till the sad Nine in Greece's evil hour Left their Parnassus for the Latian plains. Alike they scorn the pomp of tyrant Power, And coward Vice, that revels in her chains. When Latium had her lofty spirit lost, They sought, O Albion! next thy sea-encircled coast.
III.1.
         Far from the sun and summer-gale, In thy green lap was Nature's darling laid, What time, where lucid Avon stray'd, To him the mighty Mother did unveil Her awful face: the dauntless child Stretch'd forth his little arms, and smiled. This pencil take (she said) whose colours clear Richly paint the vernal year: Thine too these golden keys, immortal boy! This can unlock the gates of Joy; Of Horror that, and thrilling Fears, Or ope the sacred source of sympathetic tears.
III.2.
         Nor second he, that rode sublime Upon the seraph-wings of Ecstasy, The secrets of th' Abyss to spy. He pass'd the flaming bounds of Place and Time: The living throne, the sapphire-blaze, Where angels tremble, while they gaze, He saw; but blasted with excess of light, Clos'd his eyes in endless night. Behold, where Dryden's less presumptuous car, Wide o'er the fields of Glory bear Two coursers of ethereal race, With necks in thunder cloth'd, and long-resounding pace.
III.3.
         Hark, his hands thy lyre explore! Bright-eyed Fancy hovering o'er Scatters from her pictur'd urn Thoughts that breathe, and words that burn. But ah! 'tis heard no more— O lyre divine, what daring spirit Wakes thee now? tho' he inherit Nor the pride, nor ample pinion, That the Theban Eagle bear, Sailing with supreme dominion Thro' the azure deep of air: Yet oft before his infant eyes would run Such forms, as glitter in the Muse's ray With orient hues, unborrow'd of the Sun: Yet shall he mount, and keep his distant way Beyond the limits of a vulgar fate, Beneath the good how far—but far above the great.
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droughtofapathy · 9 months ago
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"Welcome to the Theatre": Diary of a Broadway Baby
Mother Play
April 26, 2024 | Broadway | Hayes Theater | Evening | Play | Original | 1H 30M
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I am now deeply, and unsurprisingly, in love with Jessica Lange. After so many new and disappointing musicals this month, being able to sit third-row in a smart, well-written, expertly-acted play with no pomp and circumstance is a relief. Written by Pulitzer Prize-winner Paula Vogel, the play examines her own childhood with a mother who should never have been a mother. Under Jessica Lange's masterful craft, Phyllis is part-Joan Crawford, part Mama Rose. Sympathetic and deplorable, enchanting and repulsive. A prime example of a woman who had motherhood forced on her, and tried to cope, but failed at every turn. Celia Keenan-Bolger, no stranger to embodying child roles as a grown woman, is the playwright's stand-in (here named Martha, not Paula) and carries the show's narrative with the same competence as the character has in packing up a house and moving out when the eviction notice comes. Jim Parsons, also a wonderful performer as Carl, the brother who doesn't survive. The three actors are so well-connected.
The show is comedy and tragedy. Morbid in the best way. The last scene, as a middle-aged Martha takes care of an elderly mother who no longer recognizes her, carried more power and nuance than the entirety of The Notebook's handling of the same disease. Vogel trusts her audience to still have an ounce of critical thought. The biggest example of this is in the play's subtle analysis of Phyllis's own sexuality. Her children, Martha and Carl, are very openly gay. Something that horrifies their mother, who slowly comes around (sort of) as the years pass, only to heartbreakingly not in the end. Martha, the narrator, never once comes out and says "I think my mother might've been a repressed lesbian," but that idea is woven into the entire piece. Phyllis, who is so tactile with her young son, but can't bear to touch her daughter, and who avoids any contact with women. In the end, old and senile, she is bathed by a daughter she thinks is just a nurse at the home. And as Martha drags a damp cloth over her mother's neck and collarbone, across her bare shoulder and down beneath the robe over her breasts, the relief and simple pleasure Phyllis gets out of this touch reveals more than any blatant statement. Jessica Lange's expressions in this moment are mesmerizing. And Celia Keenan-Bolger's just heartbreaking.
There's also something delightful in the way Phyllis, who starts the play at 37 and continues into her seventies, is allowed to visibly age. By the curtain call, Jessica Lange is onstage with her wrinkles and lines and bags visible for all to see, and I love a woman who is allowed to be old on stage.
Verdict: Why I Love the Theatre
A Note on Ratings
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lucysweatslove · 1 year ago
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According to 23andMe, I have a genetic variant that is pathogenic for Pompe disease.
Pompe is a “glycogen storage disease” that is actually a lysosomal storage disease where you have a deficiency in an enzyme that causes glycogen pieces to get suck in lysosomes, so you don’t use glycogen as well and then the carbs stuck in your cell can also cause problems. Classically it presents in infancy and is a pretty big deal, but presentation can be varied and there is a late-onset presentation too. My variant is involved in majority of known late-onset disease.
Anyway, the testing 23andMe does can’t say if I HAVE the disease or if I’m a carrier, it just says at least one of my alleles is a likely pathogenic variant.
So now I’m torn, because some of the symptoms associated with late onset do fit some issues I’ve had (eg, increase in respiratory illnesses, muscle cramps and pains, swallowing difficulties, exertional dyspnea), but it could very easily just be “carrier status.”
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