Paul Quinton

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Paul M. Quinton is an American physiologist.

He is a professor of pediatrics at the University of California San Diego and a professor of Biomedical Sciences at the University of California Riverside. [1]

Contents

Early life and education

Quinton was born in 1944. He grew up in Southeast Texas. As a child, he suffered from chronic coughing and often had unusual salt stains on his clothes from excessive sweating. His mother would cover him with Vicks VapoRub to ease his cough. [2]

In college at the University of Texas Austin, after coughing up blood, Quinton began investigating his possible condition looking into bronchitis and bronchiectasis. He noticed a footnote that referenced cystic fibrosis. Its definition gave him chills as it was so similar to his symptoms. His CF was confirmed by a sweat test. After his diagnosis, he committed to find the underlying causes of the disease. [2]

Quinton received a PhD from Rice University. [3]

Career

Quinton focussed his research on sweat glands, exploring how salt is transported in and out of cells. Sweat glands were easier to study in CF patients as most of the other organs of the body are heavily damaged by the disease making analysis difficult. In CF patients, sweat glands are not destroyed, they just produce a high concentration of salt in the sweat. He removed his own sweat glands to study. He discovered that in CF patients, the cellular mechanisms responsible for reabsorbing salt during sweating were dysfunctional, leading to the characteristic symptoms of the disease, including thick mucus buildup that can cause lung failure. [2]

In the early 1980s, Stanford University psychologist Jeffrey Wine, an expert researcher of invertebrate nervous systems, joined Quinton's lab after his daughter was diagnosed with CF. He would go on to found his own CF laboratory at Stanford. [4]

In 2015, Quinton and a group of prominent cystic fibrosis doctors, after three years of private dialogue, publicly criticized Vertex Pharmaceuticals for overcharging for its breakthrough CF drugs. The group went public with their objection three weeks after the company won US approval for Orkambi which would help around half of the 30,000 Americans with CF. The group was disturbed by the $259,000 per patient annual price the drug, which is more than five times the annual salary of the average American family. Quintion called the price "egregious." [5]

Vertex defended its pricing by citing the substantial investments made in drug development and the necessity of generating revenue to fund further research. It acknowledged the need for sustainable profits to continue its work in expanding treatment options for cystic fibrosis. Quinton noted the substantial financial rewards granted to Vertex executives, including the board awarding more than $53 million in one-time bonuses to twelve senior executives. He questioned the ethics of such compensation when the drugs are financially inaccessible for many patients. The group made efforts to seek a transparent dialogue regarding drug costs, including proposals for public discussions, but their concerns were largely ignored. [5]

Awards and honors

In 2000, Quinton was inducted as a Fellow of the American Association for the Advancement of Science. [6]

Personal life

Quinton is married. He and his wife adopted a son. [3]

Related Research Articles

<span class="mw-page-title-main">Cystic fibrosis</span> Genetic disorder inhibiting clearance of mucus from the lungs

Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive manner that impairs the normal clearance of mucus from the lungs, which facilitates the colonization and infection of the lungs by bacteria, notably Staphylococcus aureus. CF is a rare genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. The hallmark feature of CF is the accumulation of thick mucus in different organs. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males. Different people may have different degrees of symptoms.

<span class="mw-page-title-main">Bronchiectasis</span> Permanent enlargement of the lung airways

Bronchiectasis is a disease in which there is permanent enlargement of parts of the airways of the lung. Symptoms typically include a chronic cough with mucus production. Other symptoms include shortness of breath, coughing up blood, and chest pain. Wheezing and nail clubbing may also occur. Those with the disease often get lung infections.

<span class="mw-page-title-main">Cystic fibrosis transmembrane conductance regulator</span> Mammalian protein found in humans

Cystic fibrosis transmembrane conductance regulator (CFTR) is a membrane protein and anion channel in vertebrates that is encoded by the CFTR gene.

<span class="mw-page-title-main">Vertex Pharmaceuticals</span> American pharmaceutical company

Vertex Pharmaceuticals Incorporated is an American biopharmaceutical company based in Boston, Massachusetts. It was one of the first biotech firms to use an explicit strategy of rational drug design rather than combinatorial chemistry. It maintains headquarters in Boston, Massachusetts, and three research facilities, in San Diego, California, and Milton Park, Oxfordshire, England.

<span class="mw-page-title-main">Dorothy Hansine Andersen</span> American physician (1901–1963)

Dorothy Hansine Andersen was the American physician and researcher who first identified and named cystic fibrosis. During her almost thirty year tenure at Babies Hospital of Columbia-Presbyterian Medical Center, Andersen not only identified CF and its inheritance through a recessive gene, she was also at the forefront of developing diagnostic tests and life-extending treatments for the disease. Andersen was also active in researching other diseases that are diagnosed in children. She was the first to describe Glycogen storage disease type IV, which, in recognition of her contributions, became known as Andersen's Disease. Her research on heart malformations informed the development of open heart surgery and the training of new surgeons.

The sweat test measures the concentration of chloride that is excreted in sweat. It is used to screen for cystic fibrosis (CF). Due to defective chloride channels (CFTR), the concentration of chloride in sweat is elevated in individuals with CF.

<span class="mw-page-title-main">Allergic bronchopulmonary aspergillosis</span> Medical condition

Allergic bronchopulmonary aspergillosis (ABPA) is a condition characterised by an exaggerated response of the immune system to the fungus Aspergillus. It occurs most often in people with asthma or cystic fibrosis. Aspergillus spores are ubiquitous in soil and are commonly found in the sputum of healthy individuals. A. fumigatus is responsible for a spectrum of lung diseases known as aspergilloses.

<span class="mw-page-title-main">Cystic Fibrosis Foundation</span> American non-profit organisation

The Cystic Fibrosis Foundation (CFF) is a 501(c)(3) non-profit organization in the United States established to provide the means to cure cystic fibrosis (CF) and ensure that those living with CF live long and productive lives. The Foundation provides information about cystic fibrosis and finances CF research that aims to improve the quality of life for people with the disease. The Foundation also engages in legislative lobbying for cystic fibrosis.

Pancreatic diseases are diseases that affect the pancreas, an organ in most vertebrates and in humans and other mammals located in the abdomen. The pancreas plays a role in the digestive and endocrine system, producing enzymes which aid the digestion process and the hormone insulin, which regulates blood sugar levels. The most common pancreatic disease is pancreatitis, an inflammation of the pancreas which could come in acute or chronic form. Other pancreatic diseases include diabetes mellitus, exocrine pancreatic insufficiency, cystic fibrosis, pseudocysts, cysts, congenital malformations, tumors including pancreatic cancer, and hemosuccus pancreaticus.

<span class="mw-page-title-main">Epithelial sodium channel</span> Group of membrane proteins

The epithelial sodium channel(ENaC), (also known as amiloride-sensitive sodium channel) is a membrane-bound ion channel that is selectively permeable to sodium ions (Na+). It is assembled as a heterotrimer composed of three homologous subunits α or δ, β, and γ, These subunits are encoded by four genes: SCNN1A, SCNN1B, SCNN1G, and SCNN1D. The ENaC is involved primarily in the reabsorption of sodium ions at the collecting ducts of the kidney's nephrons. In addition to being implicated in diseases where fluid balance across epithelial membranes is perturbed, including pulmonary edema, cystic fibrosis, COPD and COVID-19, proteolyzed forms of ENaC function as the human salt taste receptor.

Venture philanthropy is a type of impact investment that takes concepts and techniques from venture capital finance and business management and applies them to achieving philanthropic goals. The term was first used in 1969 by John D. Rockefeller III to describe an imaginative and risk-taking approach to philanthropy that may be undertaken by charitable organizations.

<span class="mw-page-title-main">Ivacaftor</span> Cystic fibrosis treatment drug

Ivacaftor is a medication used to treat cystic fibrosis in people with certain mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, who account for 4–5% cases of cystic fibrosis. It is also included in combination medications, lumacaftor/ivacaftor, tezacaftor/ivacaftor, and elexacaftor/tezacaftor/ivacaftor which are used to treat people with cystic fibrosis.

Paul di Sant'Agnese (1914–2005) was the founder of the Cystic Fibrosis Foundation and of Cystic Fibrosis Care in the United States. He was a physician, researcher and one of the first clinicians who devoted his life to understanding and conquering cystic fibrosis through his work at the Columbia University Medical Center, New York City. Undoubtedly the major advance during the Fifties was the recognition of the increased salt content of the sweat in people with CF by Paul di Sant’Agnese in 1953.

<span class="mw-page-title-main">Lumacaftor/ivacaftor</span> Cystic fibrosis drug

Lumacaftor/ivacaftor, sold under the brand name Orkambi among others, is a combination of lumacaftor and ivacaftor used to treat people with cystic fibrosis who have two copies of the F508del mutation. It is unclear if it is useful in cystic fibrosis due to other causes. It is taken by mouth.

<span class="mw-page-title-main">Jeffrey Leiden</span> CEO of biotechnology company

Jeffrey Leiden is an American physician, scientist and businessman who is the executive chairman of Vertex Pharmaceuticals, a biotechnology company based in Boston, Massachusetts. He was initially appointed to the board of directors of the company in 2009 and was CEO and president from February 2012 to March 2020.

Elexacaftor/tezacaftor/ivacaftor, sold under the brand names Trikafta and Kaftrio, is a fixed-dose combination medication used to treat cystic fibrosis. Elexacaftor/tezacaftor/ivacaftor is composed of a combination of ivacaftor, a chloride channel opener, and elexacaftor and tezacaftor, CFTR modulators.

Johanna Rommens is a Canadian geneticist who was on the research team which identified and cloned the CFTR gene, which when mutated, is responsible for causing cystic fibrosis (CF). She later discovered the gene responsible for Shwachman-Diamond syndrome, a rare genetic disorder that causes pancreatic and hematologic problems. She is a Senior Scientist Emeritus at SickKids Research Institute and a professor in the Department of Molecular Genetics at the University of Toronto.

<span class="mw-page-title-main">Cystic fibrosis and race</span>

Underrepresented populations, especially black and hispanic populations with cystic fibrosis are often not successfully diagnosed. This is in part due to the minimal dissemination of existing data on patients from these underrepresented groups. While white populations do appear to experience a higher frequency of cystic fibrosis, other ethnicities are also affected and not always by the same biological mechanisms. Thus, many healthcare and treatment options are less reliable or unavailable to underrepresented populations. This issue affects the level at which public health needs are being met across the world.

Paul Adrian Negulescu is an American–Romanian cell biologist. He is a Senior Vice President at American pharmaceutical company Vertex Pharmaceuticals. He received the 2022 Shaw Prize in Life science and medicine, together with Michael J. Welsh, for their work that uncovered the etiology of cystic fibrosis and developed effective medications.

<span class="mw-page-title-main">Isolated hyperchlorhidrosis</span> Medical condition

Isolated hyperchlorhidrosis, also known as Carbonic anhydrase XII deficiency, is a rare autosomal recessive genetic condition characterized by a lifelong tendency to lose massive amounts of sodium and chloride through sweat which leads to various symptoms.

References

  1. ↑ "Quinton Lab". pediatrics.ucsd.edu.
  2. 1 2 3 "Too Ornery to Die | Only Human". WNYC Studios.
  3. 1 2 "News & Events". pediatrics.ucsd.edu.
  4. ↑ Morell, Virginia (September 18, 1992). "Alternate careers: surviving off the beaten path". Science. 257 (5077): 1758–1762 – via go.gale.com.
  5. 1 2 "Doctors challenge Vertex over high price of cystic fibrosis drug - The Boston Globe". BostonGlobe.com.
  6. ↑ "Honors". pediatrics.ucsd.edu.
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