World Trade Week NYC Small Business Global Award to Target Health Inc.
Target Health is very pleased to announce that it has been selected to receive the Small Business Global Award at the Annual International World Trade Week NYC Awards Breakfast being held on the 16th of May 2016. This is an award from the great city of NY so this recognition is quite an honor. The award will be listed after the breakfast. It should be noted that without all of the heroic efforts of the great and loyal staff at Target Health, this could never have happened.
World Trade Week NYC is an active network of more than 40 organizations in the New York metro region working together to underscore the importance of international trade, logistics and port operations on the region’s economy and to use their collective expertise to help the region’s businesses grow through international trade. Every year, the President of the United States declares the third full week of May as World Trade Week. Trade organizations, businesses and other stakeholders come together both nationally and locally to promote and facilitate international trade in the U.S. economy. The New York tri-state region’s trade and transportation community celebrates World Trade Week throughout the month of May, offering a full agenda of educational seminars, global business networking events and the International Trade Awards Breakfast that recognizes the exemplary achievements by practitioners in the field.
From our good friend and colleague James Farley, photographer extraordinaire from the great state of North Carolina.
This moth landed on a tree at our home, Friday evening. I have not seen a Luna Moth in twenty years and this was my first opportunity to photograph one, so this was quite a treat! These photos have zero crop, with the exception of changing dimensions from 8×12 to 5×7. The colors are real. Shot on my Canon 5D Mark III with 100mm Macro f2.8 lens at f6.3.
Luna Moth ©JFarley Photography 2016
ON TARGET is the newsletter of Target Health Inc., a NYC – based, full – service, contract research organization (eCRO), providing strategic planning, regulatory affairs, clinical research, data management, biostatistics, medical writing and software services to the pharmaceutical and device industries, including the paperless clinical trial.
For more information about Target Health contact Warren Pearlson (212-681-2100 ext. 165). For additional information about software tools for paperless clinical trials, please also feel free to contact Dr. Jules T. Mitchel or Ms. Joyce Hays. The Target Health software tools are designed to partner with both CROs and Sponsors. Please visit the Target Health Website.
Joyce Hays, Founder and Editor in Chief of On Target
Jules Mitchel, Editor
A positive wrist sign in a patient with Marfan syndrome. In case of a positive wrist sign the thumb and little finger overlap, when grasping the wrist of the opposite hand. Photo credit: Staufenbiel I, Hauschild C, Kahl-Nieke B, Vahle-Hinz E, von Kodolitsch Y, Berner M, Bauss O, Geurtsen W, Rahman A – http://www.ncbi.nlm.nih.gov/pubmed/24165013, CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=42107027
Marfan syndrome (also called Marfan’s syndrome) is a genetic disorder of connective tissue. It has a variable clinical presentation, ranging from mild to severe systemic disease. The most serious manifestations involve defects of the 1) ___ valves and aorta, which may lead to early death if not properly managed. The syndrome also may affect the lungs, eyes, dural sac surrounding the spinal cord, the skeleton, and the hard palate. People with Marfan syndrome tend to be unusually tall, due to the disorder causing people with the condition to have long limbs and long, thin fingers and toes. Marfan syndrome is caused by the misfolding of fibrillin-1, a glycoprotein which forms elastic fibers in connective tissue and contributes to cell signaling activity by binding to and sequestering transforming growth factor beta (TGF-Beta). This results in an accumulation of excess TGF-Beta in the lungs, heart valves, and aorta. This in turn causes abnormal structure and function of vascular smooth muscle and reduced integrity of the extracellular matrix, which weakens the tissues and cause the features of Marfan 2) ___s.
Named after Antoine Marfan, the French pediatrician who first described the condition in 1896, the disease is an autosomal dominant disorder. Management often includes the use of angiotensin II receptor antagonists (ARBs) and beta blockers. Connective tissue provides strength and flexibility to structures such as bones, ligaments, muscles, blood vessels, and heart valves. The signs and symptoms of Marfan syndrome vary widely in severity, timing of onset, and rate of progression. The two primary features of Marfan syndrome are vision problems caused by a dislocated lens (ectopia lentis) in one or both 3) ___ and defects in the aorta that distributes blood from the heart to the rest of the body. The aorta can weaken and stretch, which may lead to a bulge in the blood vessel wall (an aneurysm). Stretching of the aorta may cause the aortic valve to leak, which can lead to a sudden tearing of the layers in the aorta wall (aortic dissection). Aortic aneurysm and dissection can be life threatening. Many people with Marfan syndrome have additional heart problems including a leak in the valve that connects two of the four chambers of the heart (mitral 4) ___ prolapse) or the valve that regulates blood flow from the heart into the aorta (aortic valve regurgitation). Leaks in these valves can cause shortness of breath, fatigue, and an irregular heartbeat felt as skipped or extra beats (palpitations).
Individuals with Marfan syndrome are usually tall and slender, have elongated fingers and toes (arachnodactyly), and have an arm span that exceeds their body height. Other common features include a long and narrow face, crowded teeth, an abnormal curvature of the spine (scoliosis or kyphosis), and either a sunken chest (pectus excavatum) or a protruding chest (pectus carinatum). Some individuals develop an abnormal accumulation of air in the chest cavity that can result in the collapse of a lung (spontaneous pneumothorax). A membrane called the dura, which surrounds the brain and spinal cord, can be abnormally enlarged (dural ectasia) in people with Marfan syndrome, causing pain in the back, abdomen, legs, or head. Most individuals with Marfan syndrome have some degree of nearsightedness (myopia). Clouding of the lens (cataract) may occur in mid-adulthood, and increased pressure within the eye (glaucoma) occurs more frequently in people with Marfan syndrome than in those without the condition. The features of Marfan syndrome can become apparent anytime between infancy and adulthood. Depending on the onset and severity of signs and symptoms, Marfan can be fatal early in life; however, the majority of affected individuals survive into mid- to late adulthood. Marfan sundrome is inherited in an autosomal dominant pattern, which means one copy of the altered 5) ___ in each cell is sufficient to cause the disorder. At least 25% of Marfan syndrome cases result from a new mutation in the FBN1 gene. These cases occur in people with no history of the disorder in their family.
Marfan Syndrome Foundation
About 1 in 5,000 people have Marfan syndrome, including men and women of all races and ethnic groups. About 3 out of 4 people with Marfan syndrome inherit it, meaning they get the genetic mutation from a parent who has it. But some people with Marfan syndrome are the first in their family to have it; when this happens it is called a spontaneous mutation. There is a 50% chance that a person with Marfan syndrome will pass along the genetic mutation each time they have a child. Knowing the signs of Marfan syndrome can save lives. People with Marfan syndrome are born with it, but features of the disorder are not always present right away. Some people have a lot of Marfan features at birth or as young children – including serious conditions like aortic enlargement. Others have fewer features when they are young and don’t develop aortic enlargement or other signs of Marfan syndrome until they are adults. Some features of Marfan syndrome, like those affecting the heart and blood vessels, bones or joints, can get worse over time. It’s very important for people with Marfan syndrome and related disorders to have ongoing monitoring, especially for life-threatening aspects of the condition like aortic enlargement. An accurate and early diagnosis helps to ensure proper treatment.
Marfan Syndrome Foundation
Every person’s experience with Marfan syndrome is slightly different. No one has every feature and people have different combinations of features. Marfan syndrome can affect many parts of the body, and each person is affected differently. This is called variable expression. Features can even vary among people in the same 6) ___ who have the condition. More than 30 different signs and symptoms are variably associated with Marfan syndrome. The most prominent of these affect the skeletal system and are found in numerous other diseases (see Differential diagnosis, below). Therefore, distinguishing Marfan syndrome from other marfanoid syndromes requires the assessment of non-skeletal clinical and laboratory findings?especially of the eyes, aorta, and heart. Complicating the physical assessment of such persons, considerable clinical variability occurs within families carrying an identical DNA variant. Most of the readily visible signs are associated with the 7) ___ system. Many individuals with Marfan syndrome grow to above-average height. Some have disproportionately long, slender limbs with thin, weak wrists and long fingers and toes. Besides affecting height and limb proportions, people with Marfan syndrome may have abnormal curvature of the spine, abnormal indentation or protrusion of the sternum, abnormal joint flexibility, a high-arched palate, malocclusions, flat feet, hammer toes, stooped shoulders, and unexplained stretch marks on the skin. It can also cause pain in the joints, bones and muscles. Some people with Marfan have speech disorders resulting from symptomatic high palates and small jaws. Early osteoarthritis may occur. The health of the eye can be affected in many ways but the principal change is partial lens dislocation (the lens is shifted out of its normal position). This occurs because of weakness in the connective tissue strands which suspend the lens within the eye. Nearsightedness and blurred vision are common, but farsightedness can also result. Other problems are a greater risk of retinal detachment, an earlier onset of cataract formation and a higher risk of chronic open angle glaucoma. The most serious signs and symptoms associated with Marfan syndrome involve the cardiovascular system: undue fatigue, shortness of breath, heart palpitations, racing heartbeats, or chest pain radiating to the back, shoulder, or arm. Cold arms, hands and feet can also be linked to Marfan syndrome because of inadequate 8) ___. Commonly associated with Marfan syndrome is mitral valve prolapse, aortic regurgitation). However, the major sign that would lead a doctor to consider an underlying condition, is a dilated aorta or an aortic aneurysm. Sometimes, no heart problems are apparent until the weakening of the connective tissue causes an aortic aneurysm or aortic dissection, a surgical emergency. An aortic dissection is most often fatal and presents with pain radiating down the back, giving a tearing sensation.
During pregnancy, even in the absence of preconception cardiovascular abnormality, women with Marfan syndrome are at significant risk of aortic dissection, which is often fatal even when rapidly treated. Women with Marfan syndrome, then, should receive a thorough assessment prior to conception, and echocardiography should be performed every six to 10 weeks during pregnancy, to assess the aortic root diameter. A transgenic mouse has been created carrying a single copy of a mutant fibrillin-1, a mutation similar to that found in the human gene known to cause Marfan syndrome. This mouse strain recapitulates many of the features of the human disease and promises to provide insights into the pathogenesis of the disease. Reducing the level of normal fibrillin 1 causes a Marfan-related disease in mice.
Diagnostic criteria of Marfan syndrome were agreed upon internationally in 1996 but were recently revised in 2010. A diagnosis of Marfan syndrome is based on family history and a combination of major and minor indicators of the disorder, rare in the general population, that occur in one individual – for example: four skeletal signs with one or more signs in another body system such as ocular and cardiovascular in one individual.
Steinberg’s thumb sign (Marfan’s syndrome) – a flexed thumb grasped within a clenched palm protrudes beyond the ulnar border of that hand.Credit: Goopsmirk – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=32430111
The thumb sign (Steinberg’s sign) is elicited by asking the patient to flex the thumb as far as possible and then close the fingers over it. A positive thumb sign is where part of the thumb is visible beyond the ulnar border of the hand, caused by a combination of hypermobility of the thumb as well as a thumb which is longer than usual.
The wrist sign (Walker’s sign) is elicited by asking the patient to curl the thumb and fingers of one hand around the other wrist. A positive wrist sign is where the little finger and the thumb overlap, caused by a combination of thin wrists and long fingers. There is no 9) ___ for Marfan syndrome, but life expectancy has increased significantly over the last few decades and is now similar to that of the average person. Clinical trials are also under way for promising new treatments. At present, the syndrome is treated by simply addressing each issue as it arises and, in particular, preventative medication even for young children to slow progression of aortic dilation if such exists. Since angiotensin II receptor antagonists (ARBs) also reduce TGF-beta, these drugs have been tested in a small sample of young, severely affected people with Marfan syndrome. In some, the growth of the aorta was reduced. However, a recent study published in the NEJM demonstrated similar cardiac outcomes between the ARB, losartan, and the more established beta blocker therapy, atenolol.
Marfan syndrome is expressed dominantly. This means a child with one parent a bearer of the gene has a 50% probability of getting the syndrome. However, as the gene causing Marfan syndrome is known, arduous genetic techniques are able to circumvent this. In 1996, the first preimplantation genetic testing therapy for Marfan was conducted; in essence PGT means conducting a genetic testing on early stage IVF embryo cells and discarding those embryos affected by the Marfan mutation. Regular checkups by a cardiologist are needed to monitor the health of the heart valves and the aorta. The goal of treatment is to slow the progression of aortic dilation and damage to heart valves by eliminating arrythmias, minimizing the heart rate, and minimizing blood pressure. Beta blockers have been used to control arrythmias and slow the heart rate. Other medications might be needed to further minimize blood pressure without slowing the heart rate, such as ACE inhibitorsand ARBs. If the dilation of the aorta progresses to a significant diameter aneurysm, causes a dissection or a rupture, or leads to failure of the aortic or other valve, then surgery (possibly a composite aortic valve graft or valve-sparing aortic root replacement) becomes necessary. Although aortic graft surgery (or any vascular surgery) is a serious undertaking it is generally successful if undertaken on an elective basis. Surgery in the setting of acute aortic dissection or rupture is considerably more problematic. Elective aortic valve/graft surgery is usually considered when aortic root diameter reaches 50 millimeters (2.0 inches), but each case needs to be specifically evaluated by a qualified cardiologist. New valve-sparing surgical techniques are becoming more common As Marfan patients live longer, other vascular repairs are becoming more common, e.g., repairs of descending thoractic aortic aneurysms and aneurysms of vessels other than the aorta. The skeletal and ocular manifestations of Marfan syndrome can also be serious, although not life-threatening. These symptoms are usually treated in the typical manner for the appropriate condition, such as with various kinds of pain medication or muscle relaxants. It is also common for patients to receive treatment from a physiotherapist, using TENS therapy, ultrasound and skeletal adjustment. This can also affect height, arm length, and life span. A physiotherapist can also help improve function and prevent injuries in individuals with Marfan syndrome.
Prior to modern cardiovascular surgical techniques and drugs such as losartan and metoprolol, the prognosis of those with Marfan’s syndrome was not good: a range of untreatable cardiovascular issues was common. Lifespan was reduced by at least a third, and many died in their teens and twenties due to cardiovascular problems. Today, cardiovascular symptoms of Marfan syndrome are still the most significant issues in diagnosis and management of the disease, but adequate prophylactic monitoring and prophylactic therapy offers something approaching a normal lifespan, and more manifestations of the disease are being discovered as more patients live 10) ___.
ANSWERS: 1) heart; 2) syndrome; 3) eyes; 4) valve; 5) gene; 6) family; 7) skeletal; 8) circulation; 9) cure; 10) longer
Two Greats with Marfan Syndrome: Niccolo Paganini and Michael Phelps
Paganini became one of the world’s great virtuoso violinists. But what is especially fascinating is that part of Paganini’s great success came as a result of a rare physical ailment.
Niccolo (or Nicolo) Paganini (1782-1840), an Italian violinist, violist, guitarist, and composer, was the most celebrated violin virtuoso of his time who left his mark as one of the pillars of modern violin technique. His 24 Caprices for Solo Violin Op.1 are among the best known of his compositions, and have served as an inspiration for many prominent composers.
Paganini was born in Genoa, then capital of the Republic of Genoa, the third of the six children of Antonio and Teresa (nee Bocciardo) Paganini. Paganini’s father was an unsuccessful sea trader, but he managed to supplement his income through playing music on the mandolin. At the age of five, Paganini started learning the mandolin from his father, and moved to the violin by the age of seven. His musical talents were quickly recognized, earning him numerous scholarships for violin lessons. The young Paganini studied under various local violinists, including Giovanni Servetto and Giacomo Costa, but his progress quickly outpaced their abilities. Paganini and his father then traveled to Parma to seek further guidance from Alessandro Rolla. But upon listening to Paganini’s playing, Rolla immediately referred him to his own teacher, Ferdinando Paer and, later, Paer’s own teacher, Gasparo Ghiretti. Though Paganini did not stay long with Paer or Ghiretti, the two had considerable influence on his composition style. By the time Paganini was 18 he was well known around Genoa and Parma, and a decade or so later, the violinist had made a name for himself throughout Europe. When he died in 1840 in Nice, France, Paganini had established himself as one of the great masters of the violin. In his relatively short life, Paganini dramatically increased the technical possibilities of the violin. He could do what no one had ever done on the instrument. The virtuoso made left hand pizzicato and harmonics hallmarks of his style, and was even said to be able to play three octaves of notes across the four strings. Most violinists consider this impossible today.
Scholars have pondered how Paganini was able to do these miraculous feats on the violin, for more than a century and a half, and many have come with the conclusion that the violinist had a little known medical condition called Marfan syndrome.
Drawing of Paganini’s Hands
Marfan syndrome is a genetic disorder that affects a person’s connective tissue, often making them unusually tall with lengthened limbs and long, thin fingers. Observers of Paganini frequently commented on his unique hands. In 1931, his personal physician wrote, Paganini’s hand is not larger than normal; but because all its parts are so stretchable, it can double its reach. For example, without changing the position of the hand, he is able to bend the first joints of the left fingers which touch the strings sideways, at a right angle to the natural motion of the joint, and he can do it with effortless ease, assurance, and speed. Essentially, Paganini’s art is based on physical endowment, increased and developed by ceaseless practicing.
An anecdote of Paganini’s unheard-of ability is especially telling. One night, a rich gentleman asked the virtuoso to serenade his lady friend. The air was quite damp, and the violin strings of the day did not respond well to this kind of humidity. First the E string broke. The violinist was not fazed. Then the A and D strings snapped. The older gentleman was instantly worried and feared that the serenade for his friend would be ruined. What did Paganini do, now that he only had one string to play on? He simply smiled and continued to play on one string just as if he was playing on all four. The serenade was a success after all, thanks to the virtuoso’s amazing ability.
Poster for a Paganini concert.
Having Marfan syndrome also created a certain mystique for Paganini. People called him Hexensohn (Witch’s Child) because of his seemingly superhuman ability. Some claimed that he had made a pact with the devil to play as well as he did. Reports of his demonic possession were enhanced by the medical condition which made him appear unusually thin and pale. Paganini loved all this notoriety and had fun with it. To accentuate the rumors, he would dress completely in black and sometimes arrive at a concert in a black carriage pulled by four black horses. And when he lost his teeth in 1828, his face looked even more ghostly. Of course, people flocked to his concerts. Some have even called him music’s first rock star. There were times when he dramatically entered the concert hall from the back of the audience, playing his violin as he sauntered up the aisle, enjoying the delight of his admirers, as he passed by the sold-out seats. Medical ailments are often viewed as things to overcome. With Paganini, Marfan syndrome actually enhanced an already considerable talent to help him become one of the world’s finest instrumentalists.
In 1801, the 18-year-old Paganini was appointed first violinist of the Republic of Lucca, but a substantial portion of his income came from freelancing. His fame as a violinist was matched only by his reputation as a gambler and womanizer. In 1805, Lucca was annexed by Napoleonic France, and the region was ceded to Napoleon’s sister, Elisa Baciocchi. Paganini became a violinist for the Baciocchi court, while giving private lessons to Elisa’s husband, Felice. In 1807, Baciocchi became the Grand Duchess of Tuscany and her court was transferred to Florence. Paganini was part of the entourage, but, towards the end of 1809 , he left Baciocchi to resume his freelance career.
1831 bulletin advertising a performance of Paganini
His international fame started with an 1813 concert at La Scala in Milan. The concert was a great success. As a result, Paganini began to attract the attention of other prominent, musicians across Europe. In 1827, Pope Leo XII honored Paganini with the Order of the Golden Spur. His fame spread across Europe with a concert tour that started in Vienna in August 1828, stopping in every major European city in Germany, Poland, and Bohemia until February 1831 in Strasbourg. This was followed by tours in Paris and Britain. His technical ability and his willingness to display it received much critical acclaim.
Throughout his life, Paganini was no stranger to chronic illnesses. Although no definite medical proof exists, he was reputed to have been affected by Marfan syndrome or Ehlers-Danlos syndrome. In addition, his frequent concert schedule, as well as his extravagant lifestyle, took their toll on his health. He was diagnosed with syphilis as early as 1822, and his remedy, which included mercury and opium, came with serious physical and psychological side effects. In 1834, while still in Paris, he was treated for tuberculosis. Though his recovery was reasonably quick, after the illness his career was marred by frequent cancellations due to various health problems, from the common cold to depression, which lasted from days to months. In September 1834, Paganini put an end to his concert career and returned to Genoa. Paganini devoted his time to the publication of his compositions and violin methods. He accepted students. In 1835, Paganini returned to Parma, this time under the employ of Archduchess Marie Louise of Austria.
Tomb of Paganini in Parma, Italy; Wikipedia commons
In 1836, Paganini returned to Paris to set up a casino. Its immediate failure left him in financial ruin, and he auctioned off his personal effects, including his musical instruments, to recoup his losses. At Christmas of 1838, he left Paris for Marseilles and, after a brief stay, travelled to Nice where his condition worsened. In May 1840, the Bishop of Nice sent Paganini a local parish priest to perform the last rites. Paganini assumed the sacrament was premature, and refused. A week later, on 27 May 1840, Paganini died from internal hemorrhaging before a priest could be summoned. Because of this, and his widely rumored association with the devil, the Church denied his body a Catholic burial in Genoa. It took four years and an appeal to the Pope before the Church let his body be transported to Genoa, but it was still not buried. His remains were finally laid to rest in 1876, in a cemetery in Parma. In 1893, the Czech violinist Frantisek Ondricek persuaded Paganini’s grandson, Attila, to allow a viewing of the violinist’s body. After this bizarre episode, Paganini’s body was finally reinterred in a new cemetery in Parma in 1896.
Though having no shortage of romantic conquests, Paganini was seriously involved with a singer named Antonia Bianchi from Como, whom he met in Milan in 1813. The two gave concerts together throughout Italy. They had a son, Achilles Cyrus Alexander, born on 23 July 1825 in Palermo and baptized at San Bartolomeo’s. They never legalized their union and it ended around April 1828 in Vienna. Paganini brought Achilles on his European tours, and Achilles later accompanied his father until the latter’s death. He was instrumental in dealing with his father’s burial, years after his death. After giving a recital when one string after another broke, first the E, then the A, and finally the D, leaving him only with the lowest, the G string, refusing to stop playing (or to re-string), he improvised on his one remaining string. Seeing the drama in such an event, at future concerts, he sometimes broke strings, just to awe his audience further, as he continued playing with only the one remaining string. And, in fact, Paganini did write his Moses Fantasy solely for the G string, offering tremendous difficulties that strike fear into the hearts of violinists and ensure that the music is rarely played. At any rate, restriction to the G string gives even the high notes a dark coloring that can lead unwary listeners to believe they’re hearing music for the viola.
The violinist, David Garrett, plays Niccolo Paganini in a wonderful recent film, The Devil’s Violinist, which gives authenticity to the colorful style of Paganini as he toured through Europe to great acclaim. This piece, by Paganini is called, Caprice 24
Paganini composed his own works to play exclusively in his concerts, all of which profoundly influenced the evolution of violin technique. His 24 Caprices were likely composed in the period between 1805 to 1809, while he was in the service of the Baciocchi court. Also during this period, he composed the majority of the solo pieces, duo-sonatas, trios and quartets for the guitar, either as a solo instrument or with strings. Paganini’s exceptionally long fingers enabled him to play three octaves across four strings in a hand span, an extraordinary feat even by today’s standards; no doubt a result of Marfan syndrome. The Paganini Competition (Premio Paganini) is an international violin competition created in 1954 in his home city of Genoa and named in his honour. A minor planet, 2859 Paganini, discovered in 1978 by Soviet astronomer Nikolai Chernykh is named after him.
Sources: NIH.gov; Medline.org; Wikipedia; http://inmozartsfootsteps.com/1032/paganini-violinist-helped-by-marfan-syndrome/
Michael Phelps, After Winning His 8th Gold Medal
Photo credit: Bryan Allison – CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=4679646
Michael Phelps, Athlete Extraordinaire
The most celebrated Olympian and Swimmer Michael Phelps has been regarded the most incredible swimmer in the world. He is known for his extremely tall and thin physique, great flexibility, speed in water faster than any other human and a grand wingspan.
Michael Fred Phelps II, born June 30, 1985), is an American competition swimmer and the most decorated Olympian of all time, with a total of 22 medals in three Olympiads. Phelps also holds the all-time records for Olympic gold medals (18, double the second highest record holders), Olympic gold medals in individual events (11), and Olympic medals in individual events for a male (13). In winning eight gold medals at the 2008 Beijing Games, Phelps took the record away from fellow swimmer Mark Spitz (7) for the most first-place finishes at any single Olympic Games. Five of those victories were in individual events, tying the single Games record. In the 2012 Summer Olympics in London, Phelps won four golds and two silver medals, making him the most successful athlete of the Games for the third Olympics in a row.
Apart from his achievements and efficiency he has been in news because of his curious physique associated with Marfan’s syndrome. The rumor became a truth when Phelps himself confirmed having Marfan’s syndrome although he tested negative for it but he still meets the criteria for the syndrome which a unique connective tissue disorder is affecting about 1 in every 20,000 people caused mostly due to a pathological condition of the protein fibrillin-1, a structural protein. The ones suffering from Marfan’s are extremely tall and have longer bones, limbs and fingers. Their detached retinas, larger aorta, and leaky heart valves make it a fatal disease and a rare genetic disorder. Michael Phelps, according to critics has been helped by this disorder helping him in his athletic pursuits because of the height and flexibility which is generally an advantage for the athletes. However this fatal disorder can be seen more in the context of posing threat to his life. In one of his training sessions his heart pace was alarmingly high that made him undergo tests for the fear of this syndrome. There is no doubt that he has the classic symptoms of Marfan’s syndrome with an elongated stature of about six feet and five inches and an arm span of six feet and seven inches (more than his height) an unusual clinical cut off. According to his mother, he grew unevenly with extremely long arms and unusual ears.
In one of his interviews after his autobiography was released he said If you reach out your arms and form a T and your wingspan is longer than your height, you can be at risk. In my case, those measurements have always been very close.
Phelps goes for an annual checkup and has maintained stronger tissues with a clear aortic rout and a heart in good condition. Marfan’s Syndrome has been known for causing abrupt death of athletes, especially after the event. Phelps however has the best doctors taking care of this condition and monitoring him time to time but that doesn’t really minimize the risks which can actually increase with his limits. Phelps has been more in the news because of his unusual conditions than his achievements as a fascinating subject of study among sports scientists, psychologists and physicians. Phelps also contains only two-thirds the lactic acid in the muscles as compared to the other swimmers and has an intake of food that is six times more than an average adult male.
Antiarrhythmic Drugs Beneficial When Used by EMS Treating Cardiac Arrest
Sudden cardiac arrest is a condition in which the heart suddenly or unexpectedly stops beating, cutting off blood flow to the brain and other vital organs. More than 300,000 people are treated for out-of-hospital cardiac arrest each year, with the vast majority occurring at home, according to the American Heart Association. Studies show that nationally only about 10% of people who suffer cardiac arrest outside the hospital survive. Effective treatments, such as CPR and defibrillation, can greatly increase a victim’s chance of survival.
EMS (emergency medical services) providers commonly use antiarrhythmic drugs for out-of-hospital cardiac arrest that is not responsive to defibrillation shocks to the heart for restoring its normal rhythm. However, doctors remain unclear whether these drugs have proven survival benefit or if any benefit might be undone by possible drug side effects. As a result, use of these treatments by paramedics varies.
As a result, a study published online in the New England Journal of Medicine (4 April 2016) has confirmed that certain heart rhythm medications, when given by paramedics to patients with out-of-hospital cardiac arrest who had failed electrical shock treatment, improved likelihood of patients surviving transport to the hospital. The study was and helps answer a longstanding scientific question about the effectiveness of two widely-used antiarrhythmic drugs, amiodarone and lidocaine, for treating sudden cardiac arrest. The study followed the patients from hospital admission to hospital discharge. Although neither drug significantly improved the overall rate of survival to hospital discharge, amiodarone showed a favorable trend in that direction. Survival to discharge is the point at which a patient is discharged from the hospital.
According to the authors, the trial shows that amiodarone and lidocaine offer hope for bringing patients back to life and into the hospital after cardiac arrest, and that while the overall increase in survival to hospital discharge of about 3% with amiodarone was not statistically significant, it came very close. Importantly, there was a significant improvement in survival to hospital discharge with either drug when the cardiac arrest was bystander-witnessed. A bystander-witnessed cardiac arrest is one that is witnessed by another person.
The three-year study began in 2012, and randomized 3,026 patients with out-of-hospital cardiac arrest caused by ventricular fibrillation andventricular tachycardia, life-threatening rhythms of the heart’s lower pumping chambers that are often resistant to electrical shock. Patients were assigned to treatment with amiodarone, lidocaine, or neither drug (a saline placebo) by rapid injection, along with all other standard resuscitation treatments. The study was conducted by the Resuscitation Outcomes Consortium, which includes clinical sites in the United States and Canada.
Results showed that survival among the approximately 1,900 patients in the study whose cardiac arrest was witnessed by a bystander was improved significantly, from about 23-28%, by either drug. With better than half of study participants fit this bystander-witnessed category. This suggests treating patients as soon as possible after their collapse might be a critical determinant of whether drugs like amiodarone or lidocaine will have a significant clinical effect.
Bypass Surgery Extends Lives of Patients with Heart Failure
Coronary artery bypass graft (CABG), a surgical procedure to help improve blood flow to the heart by bypassing arteries clogged with cholesterol plaques, was thought to be too risky for patients with the long-term effects of coronary artery disease: left ventricular dysfunction (when the left side of the heart is unable to pump normally) and heart failure. Studies of the safety and effectiveness of CABG in the 1970s excluded most patients with these two conditions. The procedure was typically used to relieve angina, or chest pain.
According to a study published in the New England Journal of Medicine (3 April 2016), a five-year global, randomized controlled clinical trial, called the Surgical Treatment for Ischemic Heart Failure (STICH) study, and a five-year extension study (STICHES), which was performed to evaluate whether CABG plus guideline-directed medical therapy had a durable benefit over medical therapy alone for patients with coronary artery disease and left ventricular dysfunction.
From July 2002 to May 2007, a total of 1212 patients with an ejection fraction of 35% or less and coronary artery disease amenable to CABG were randomly assigned to undergo CABG plus medical therapy (CABG group, 610 patients) or medical therapy alone (medical-therapy group, 602 patients). The primary outcome was death from any cause. Major secondary outcomes included death from cardiovascular causes and death from any cause or hospitalization for cardiovascular causes. The median duration of follow-up, including the current extended-follow-up study, was 9.8 years.
A primary outcome event occurred in 359 patients (58.9%) in the CABG group and in 398 patients (66.1%) in the medical-therapy group (P=0.02). A total of 247 patients (40.5%) in the CABG group and 297 patients (49.3%) in the medical-therapy group died from cardiovascular causes (P=0.006). Death from any cause or hospitalization for cardiovascular causes occurred in 467 patients (76.6%) in the CABG group and in 524 patients (87.0%) in the medical-therapy group (P<0.001.
According to the authors, the results are particularly important because the prevalence of left ventricular dysfunction and heart failure is expected to increase to approximately 8 million individuals by 2030 in the U.S. alone. The increase in the projected prevalence is a result of advances in the management of cardiovascular disease and its risk factors, increasingly transforming coronary artery disease into a chronic disease with long-term effects such as left ventricular dysfunction and heart failure.
Innovation at FDA: Helping to Speed Cures and Treatments to Patients
Target Health congratulates FDA on their innovative approaches to help deliver safe, and effective treatments and cures to the patients who need them as quickly as possible. To achieve this goal, FDA has implemented a variety of expedited review programs and are working to help shorten the development time before a product is even submitted for FDA review. In addition, FDA has issued far reaching guidances on use of eSource records, risk-based monitoring, and electronic informed consent, which when properly implemented, can easily reduce the cost of monitoring clinical trials by 50%.
As a result of these innovative efforts, in 2014 alone, FDA approved 51 new molecular entities and biological products (41 by CDER and 10 by CBER. These approvals included major therapeutic advances in the treatment of cancer, hepatitis C and type-2 diabetes. They also included vaccines for meningococcus type B, and more new orphan drugs for rare diseases than any previous year.
FDA has also made strides with medical devices. As a result of activities coordinated by CDRH Innovation, and programmatic improvements and innovative use of our existing approval and clearance pathways, many devices investigated in the United States now reach the market a full year sooner than they did at the beginning of this decade. Products recently approved or cleared by FDA include the BrainPort V100, a first-of-its-kind wearable device that can help orient profoundly blind individuals to their physical surroundings; Watchman LAA Closure Technology, a permanently implanted device that prevents certain clots from entering the bloodstream and potentially causing a stroke; and the Maestro Rechargeable System to treat obesity in certain adult patients (it targets the nerve pathway between the brain and the stomach that controls feelings of hunger and fullness).
Brussels Sprouts Roasted with Garlic, Oil & Aged Balsamic Vinegar
After years of never hitting it off, with Brussels sprouts, I finally got it and we all benefit! ©Joyce Hays, Target Health Inc.
Coat a heavy pan with 5 Tablespoons extra virgin olive oil
15 to 20 fresh garlic cloves, sliced but not thin
1 pound of Brussels sprouts. If box is sealed and it’s more than one lb, that’s fine.
Pinch black pepper
Pinch chili flakes
4 to 5 Tablespoons aged balsamic vinegar (5 years or more)
After years of trial and error, trying to get the bitter out of Brussels sprouts, what finally made this recipe perfect was simplicity and a few excellent ingredients with outstanding flavor characteristics. One of these is a well-aged balsamic vinegar. I have a few of them in my collection and one of them is made with figs. As long as you use a well-aged balsamic vinegar, the recipe will be just fine. Be prepared to spend more for aged, than for a typical bottle of it. However, for this recipe to be at its best, go ahead and splurge a little. The other ingredient is lots of roasted garlic. Finally, I discovered that the way to bring the best out of the Brussels sprouts, is to roast them; nothing else will do, as well.
After years of ignoring this little veggie because it never tasted that good, it has become one of my favorites, just because of the way I’m cooking it now. There’s an extra plus here – it’s very low in calories and it’s one of the top ten healthiest veggies. ©Joyce Hays, Target Health Inc.
Here’s the garlic just before slicing it. ©Joyce Hays, Target Health Inc.
Slice the garlic in nice big chunky slices. ©Joyce Hays, Target Health Inc.
Preheat oven to 400 degrees.
Trim Brussels sprouts, discard any discolored outside leaves, and cut each in half, vertically, from top to bottom, not from side to side.
Simple preparation: remove any outer discolored leaves. If the very bottom of a sprout is discolored, cut and discard a thin slice from the bottom. Then, cut each sprout in half, lengthwise. ©Joyce Hays, Target Health Inc.
Heat extra virgin olive oil in a cast-iron pan, if you have one, over medium heat, until the oil shimmers.
Put the Brussels sprouts into the pan with the cut side down.
Only cook them in one layer in pan. If your pan isn’t big enough, cook the sprouts in stages.
Put in the garlic slices, and sprinkle with salt, pepper and chili flakes.
Cook, without stirring, until sprouts begin to get brown on the bottom.
Cook the sprouts and the garlic in extra virgin olive oil and the salt, pepper, chili flakes. This step softens up the Brussels sprouts, so they absorb the flavor of the extra virgin olive oil, salt, pepper, chili flakes, before blasting them with heat. ©Joyce Hays, Target Health Inc.
Next, transfer the pan to the oven or with a spatula, transfer the sprouts, garlic and the oil with spices, to a baking sheet.
Scrape every last bit of the oil, over the sprouts.
Roast, the sprouts.
Every 5 minutes, shake the pan or stir the sprouts on the baking sheet.
Do this shaking and/or stirring, until sprouts are quite brown and tender.
This should take from 10 to 20 minutes, depending on your oven.
I used a flat baking pan, here, because I don’t have one of those heavy black iron pans. The sprouts and garlic are partially done, now in the oven, the finishing touch only achieved by roasting. ©Joyce Hays, Target Health Inc.
When the sprouts are nicely browned, stir in the balsamic vinegar, and serve right away. Sprouts cooked this way are good warm or cold.
For dessert we had this simple almond cake with marzipan filling, served with a glog of rum, which the sponginess of the cake soaks up right away. I’m still experimenting with this cake, but will share in the future; perfect for Passover. ©Joyce Hays, Target Health Inc.
We started dinner with chilled Stag’s Leap Cellars’ Aveta Sauvignon Blanc
This weekend we saw Fiddler On The Roof, a beautiful production, directed by Bartlett Sher and starring Danny Burstein doing a great Tevye. We think this terrific Fiddler revival resounds because of Burstein, who carries the show, and very fine choreography, with unusually great dancers. This show always reminds me of my roots. I wasn’t born with a silver spoon, but I was born with a Klezmer soul!
After seeing Fiddler, we went to Sardis, where we’ve been going for decades, and never get tired of it. See below, what we shared for dessert.
From Our Table to Yours !