Cheap wooden dummy: Amazon.com: Wing Chun Wooden Dummy with Base Walnut Color with Form and Cover : Sports & Outdoors

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Wing Chun Dummy, Mook Jong Wooden Dummies

 

Our Gratitude

Warrior Martial Art Supply is proud to be serving the Wing Chun and JKD community for over 16 years now. All of our dummies are hand made to order right here in the United States. We specialize in custom Engraved and Airbrushed dummy trunks and are unsurpassed in our quality and designs. We would like to take the time to thank you for your business and growing support.

Build Time

 Quality work takes time and we usually have a waiting list of 8-10 weeks, depending on demand wait times can vary. We currently produce between 4-6 dummies per week. Contact us for a current build time for a more accurate estimate. 

Shipping Rates

We ship our products worldwide and shipping rates may vary. Shipping prices on our site only pertain to continental USA only. Contact us for all other international shipping rates as well as Alaska, Hawaii, and Puerto Rico.

Dummy Styles

  We manufacture 4 different dummy body styles all of which are constructed with our 9 board laminating process and come with a lifetime warranty against major cracking or splitting. All of which are interchangeable with all 6 of our dummy stands. Any engravings we offer as well as any custom artwork you provide can be added to all dummy styles.

 

 

   Warrior’s    Wing Chun Dummies are a 9″ diameter 60″ long Laminated trunk engineered to last with choice of wooden and spring arms. 

 

 

 

ARMS:  Traditional Wing Chun Dummy arms with 1.5″ x 1.5″ arm peg. The two upper arm pegs are offset to the arm to allow user to flip and change from offset upper arms to level upper arms. The middle arm is centered to arm peg. 

 

Jeet Kune Do Dummy

Jeet Kune Do Dummy is a    9″ diameter 60″

long Laminated trunk engineered to last with two types of arm styles available.  

 

ARMS:  Traditional Wing Chun Dummy arms with 1.5″ x 1.5″ arm peg. The two upper arm pegs are offset to the arm to allow user to flip and change from offset upper arms to level upper arms. The middle arm is centered to arm peg.

 

 The Octopus Dummy

 

The Octopus Dummy designed by Sifu Randy Williams is a  9″ diameter 60″ long trunk. This dummy has a total of 8 limbs including 7 arms and one leg. The Octopus comes standard with the “CRCA” and “Bot Jau Yue” (Eight Talon Fish) logos engraved and painted standard.

 

  

    The Compact Dummy 9″ diameter 48″ long and is half of a cylinder. Our laminated trunk engineered to last a lifetime

ARMS:  Traditional Wing Chun Dummy arms with 1.5″ x 1.5″ arm peg. The two upper arm pegs are offset to the arm to allow user to flip and change from offset upper arms to level upper arms. The middle arm is centered to arm peg. 

 

 

 

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A Wing Chun wooden dummy like in PVC

A low-cost wooden dummy substitute in PVC

As every Kung Fu practitioner knows (especially who study Wing Chun or Jeet Kune Do), the wooden dummy (Muk Yan Jong or Mu Ren Zhuang) is a fantastic and fundamental training tool but in most of the cases is very expensive. It is for this reason that we have loved the idea of a PVC made wooden dummy’s substitute (for beginners)

  • It has a very low cost accessible to everyone
  • It is relatively easy to build

A note by Master Kongling – These kind of training dummies are good for every martial art (from Wing Chun to 6 Dragons Kung Fu, Karate, etc. ), so we have decided to write this “review”, to have a “deeper” look at advantages and disadvantages (the original idea was on kungfu2u.com but the website, unfortunately, has disappeared).

PVC version of the wooden dummy: advantages

Advantages:

  • It is good to get familiar with the basic movements (not with the feeling)
  • It is absolutely low-cost (it is impossible to spend less than this)
  • It is waterproof (it can be installed outside of a covered area, for example in a meadow)
  • Easily portable / removable (it can be assembled and disassembled wherever)
  • No need for special skills or technical tools (it is assemblable almost by anyone)
  • It can be replaced / repaired easily (if it is damaged it is not a serious problem)
  • The compositive parts can be found by anyone (in any construction warehouse or with recycled parts)

Not a professional tool: the disadvantages

Disadvantages:

  • It is very light and then slightly unstable (in each of its parts, arms, core structure, etc. )
  • It does not simulate the weight-resistance of a human body (this is why the wooden dummy is made of wood)
  • It is not suitable for those who want to work on weight exchange, the inertia of movements, etc. (for example in Wing Chun, in most cases, the idea is to push away the opponents and not limiting to play with his limbs)
  • It is not suitable for those who are able to hit solid surfaces at high power and want to work on advanced conditioning (read Conditioning check for the impact)
  • It has extremely limited longevity (the holes tend to rapidly be damaged)

A few ideas to improve this PVC version of the wooden dummy

Let’s see some possible improvements:

  • The lower part should be bigger to have better stability
  • The lower part must be ballasted with sandbags or stone blocks (at least 60 Kg)
  • The lower tubes and the middle may be partially filled with thick sand (gravel or other filling materials)
  • Holes can be protected / reinforced with hard rubber or elastic glue
  • The holes of the protruding limbs can be closed by felt pads for chairs / tables (a sort of plastic plugs)
  • The protruding limbs should cross the central body not only frontally but even from behind
  • The protruding limbs should be locked by various vertical nails (internal and external) to prevent them from moving too much
  • Some parts may be replaced or reinforced with alternative materials (even if this could mean to possess advanced working skills and / or machines)
  • Legs for chairs or pre-made tables could be used instead of PVC arms

A note by Master Kongling – An advanced practitioner cannot substitute the wooden dummy with this hand-made tool but nothing avoid him to adopt it as an alternative training option for different purposes (read The use of unorthodox methods) or as a portable version (for demonstrations, theoretical explanations, etc. ). 

In the next article of this series, we will see how to build this dummy (read How to build a Wing Chun wooden dummy in PVC).

In-depth video courses

In-depth articles

Questions

Reply in the comments and share your experience:

  • Have you ever used a real wooden dummy? What would you recommend as an improvement?

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Go to our Patreon page and choose a training plan: starting from the Practitioner level, you will gain access to all this and much more.

Inside each Premium Lesson, you will receive the same teaching (practices, tips, concepts, small secrets and corrections) reserved to the live students of Master Kongling.

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Wooden Dummy


Wooden Dummy – a unique conception of training, which is recorded in the Shaolin temple. Despite the fact that a wooden Dummy is widely used in the system of Wing Chun, it is used in many schools of martial arts.

Wooden Dummy – the art of self-expression and self-discovery. In the Shaolin Temple, the monks have demonstrated countless variations of impact on 108 wooden dummies. One of them was close to the one that is made today. Wooden Dummy appeared long before the foundation of Wing Chun as an independent style.

Many people practice their workout on WDS Wooden Dummies

All martial arts have evolved since everyone makes their own additions, throwing us farther from the source, which was originally conceived in the Shaolin Temple. There are no historical records of the original techniques used by the monks, they simply do not exist! In Shaolin there have been countless movements. Today, each of us follows only parts of those movements. Each new movement is that endless stream of skill. The essence of the wooden dummy is the discovery of one’s own knowledge.

The inventors of wooden dummies – the thinkers of the ancient east

In the time of Shaolin there were 108 unique dummies of different designs. The creators of these dummies were ancient thinkers, not setting the limits of reason. Consciousness was expanded through spiritual practice, meditation, strength of the spirit, through a deep understanding of the meaning of one’s essence, that was the nature of the sages of the ancient Orient. The process of creation and creativity was boundless. The wise men saw the true nature of things. At first the wooden dummy was conceived as a log embedded in the ground for filling of surfaces for impact. There were no restrictions for working with this device. This can be compared with a punching bag hanging on chains, are there techniques of working with it? The punching bag can be used in any variation. The same applies to the wooden dummy, only the spectrum of work is much higher, it is simply limitless. You should not limit yourself to certain a set of movements and techniques, you are able to create your own unique set.

Today, the wooden dummy is becoming increasingly popular in the circles of martial arts. Each practitioner can use the dummy in his training for the development of agility, strength of impact, shock hardening of surfaces, etc. It should be remembered that the wooden dummy generates the whole concept of yin-yang – opposite interdependence. Using a wooden dummy and practicing proper breathing, develops inner strength and spiritual practice, it can be likened to meditation as having a lack of thought and fine concentration – focus of intention. The main essence of martial arts – the formation of the equality of the mind, spirit and body. Wooden Dummy creates endless stream of practice, which is not bound by existing systems. At the beginning of the conception of WDS many techniques were analyzed, those demonstrated (Yip Man, William Cheung, Augustine Fong, David Patterson, Emin Boztepe, M.Wong, Gary Lam, Leon Ting) all essentially make one form, but all have different performance and different movements, various comments, various demonstrations, workshops and speeches. From this follows that Wing Chun is a combat system, not a style that is standardized. The wooden dummy is the beginning of reflex movements of yourself that you have trained to automatic. The main task of WDS is: the disclosure of the concept of the wooden dummy as an integral part of everyday life for any individual. The site is based on the philosophy of Bruce Lee.



I have received the first dummy! Thank you very much!

A friend of mine, whom the dummy was for, has assembled it and is very happy with it.

Kind regards,…

Well made product I use it almost daily with no problems. Thank you…

Last part was received today. Everything seems ok.

We will mount it this evening.

Thanks a lot. Very professional work and delivery.

If I need another dummy or another part, you will…





Wooden Dummy Plans — Global Wing Chun Academy

 Wooden Dummy Plans

These were some wooden dummy plans I used in 1982 to build my first wooden dummy. The original Wing Chun wooden dummy was just a log stuck into a square hole in the ground and that idea was what I used first. We all just got logs from the beach and built our own wooden dummies that way. Later I ordered a Teak wooden dummy from Hong Kong. The price was $200 Canadian dollars. However by the time it got shipped to my house it cost me $800 but it was a very good wooden dummy. These days there are many plans around and also videos to show how to make these dummies.

Ideally they should be tailored to a student’s size. But if you have a lot of kinds of students that may not be practical for a smaller club. So hence everyone should have their own.

Here is my main teacher Dr. G.K. Khoe who was a student of Master Wang Kiu in Holland. Wang Kiu was a private students of Ip Man (Yip Man) but later moved to Holland to teach. Both have passed away (R.I.P.) The photo is when he came to Victoria from Vancouver to teach my basement group Wing Chun. Everyone was very impressed with his skill and his teaching methods.

This plan was the wooden dummy plan from one of my fellow members at the University of British Columbia Wing Chun club.

what is hard to see from these diagrams is the exact feel the dummy should have. It should not have too loose a feel but it should not have too tight a feel. A good wooden dummy makes you feel like using the dummy everyday. It is like having a good musical instrument. If you have a cheap guitar or a cheap piano or cheap violin, then you will never feel like practicing on these cheap instruments. Investing in a good dummy is important for anyone serious about Wing Chun.

One of our female members Bev Ripley in the early 1990’s made a couple of wooden dummies for our club. I was impressed with her wood working skill. She was a medical professional.

Here is a nice dummy design for kids.

This 8 year old girl is an internet sensation on Instagram.

Hayashi wooden dummy “Wing Chun” – freestanding and rotating dummy

€799.00


The delivery time is 2-3 Tage.

Bruce Lee learned the arts of Wing Chun at the age of 13. With the wooden dummy “Wing Chun” by Hayashi you now have the opportunity to study this unique Far Eastern martial arts style efficiently. Grandmaster Wang Kiu, who also trained Bruce Lee on the wooden doll, gives this unique training method great importance. The Hayashi Training Dummy is free-standing and rotatable. A sturdy steel plate, which comes with the delivery, fixes it firmly to the ground and gives you the ultimate in training fun on the 3 arms and 1 foot part. The high-quality wood and the exact processing in length, shape, position, and angle of the elements, you will quickly feel an increase in strength and speed of your movements.

Additional advantages:

  • traditional wooden doll
  • Individual
  • rotatable
  • stable floor mounting

Unfortunately this product is too big for standard shipping methods.

Orders over 31.5 kg or those with oversize items (> 110 cm) are only offered for pickup, otherwise we would be obliged to display binding shipping costs in the online shop. We will gladly send you an individual offer for shipping after your order or inform you by phone about it before.

“Wing Chun” Dummy – your guide to position, distance and angle.

Hayashi brings you the wooden dummy – also called Mook Yan Jong – the ultimate training experience. The 3 arms and the foot part are inserted in the robust stem and fixed firmly. The alignment and angle of the individual elements are precisely adapted to the exercises of the southern Chinese martial arts. Your movements will be perfected by a concentrated and recurrent training on the dummy in the aspects position finding, distance perception and the correct angle of impact.

Its strength lies in steadiness.

Due to the heavy steel plate and the anchoring on the ground, the woodman gets his firm footing. Since the dummy is free and rotatable in all directions, you can rehearse strokes, kicks, and combinations under realistic conditions over and over again. Your wooden friend is absolutely painless and easily forgives you one or the other failure.

Experience the legend of the 108 movements with the Wing Chun Dummy live.

Legends are obtained through traditions. Like the legend of the nun Ng Mui, who is said to have developed a new fighting style with Wing Chun. The “Woodman” was already used in training to harden the forearms and shins. It was also able to practice techniques that could have hurt a real partner severely. Ng Mui created a new martial art form, based on the Kung Fu traditions of the Shaolin, with her 108 movements easily rehearsed on Mook Yan Jong.

Get into the legendary world of the Wing Chun and get the “Wing Chun” wooden dummy by Hayashi straight to your home.

 

More Information
Brand Hayashi
Already discounted No
Delivery Time 2-3 Tage
Shipping oversize No

Current Language: English


90,000 10 questions about tailor’s mannequins

Do you sew and wonder if you need a mannequin? Can’t decide on the shape, color and parameters? We have collected the most popular questions about mannequins and prepared detailed answers to them.

  • How is a tailor’s mannequin different from a simple mannequin?

For simplicity, both are simply referred to as “dummies.” The difference in purpose: the usual one is needed to demonstrate things: it can be made of any material: wood, plastic, even metal.And the tailor’s one is used for sewing. It is close in proportion to the human body, allows you to see exactly how the thing will sit, make adjustments and even create designs right on it.

  • What kind of dummy do you need for sewing?

Soft, matching your measurements – that’s the main thing. The rest is details that depend on your taste and goals. Here you can find female mannequins Christina and Monica, as well as male Richard and Henry.They differ in the presence of markings, top material and design features.

  • What is the acceptable difference between the parameters of the dummy and mine?

Ideally, the parameters should match exactly if you are sewing for yourself. The difference may be 1-2 centimeters. You can adjust the measurements using overlays. If you are a designer and create items for sale, then you can safely use the standard sizes. The most popular are 42-44. Royal Dress Forms mannequins are created taking into account the real parameters of people, and not the average figures from the ceiling.So with us you can be sure that things will fit right.

  • Can’t find my size mannequin. What to do?

Correct. Soft mannequins can be supplemented with overlays that will bring the parameters as close as possible to real ones. They can be made on their own, or you can save time and order ready-made ones. The set of overlays from Royal Dress Forms is fixed with a cover that smoothes out any irregularities.

  • What is a scale mannequin?

This is a miniature replica of a large mannequin used for training and modeling with a tattoo. A scale mannequin is a replica close to 42 size at a scale of 1: 2. This allows you to transfer the simulation result to full-size models.

  • Which is better: sliding or soft?

The retractable mannequin can only be used if you do not need a fit.It is made of plastic, so it is hard inside and covered with a layer of fabric on top. It is not very convenient to pin material on it and take off or put on things. But it can be adjusted for different sizes.

The soft mannequin is devoid of the drawbacks of a sliding one, plus it can be easily adjusted to fit your measurements with the help of overlays. Our advice: take the soft one!

  • How does an expensive mannequin differ from a cheap one?

The answer “quality” will be too general, so we will decipher it in detail.The material covered by the mannequin may differ. In expensive ones, this is a fabric with protection against dust and moisture or a removable cover that can be washed at any time. In cheap ones – a non-removable cover. The second detail is measurements. The more expensive the dummy, the more accurate they are. It is important to consider the type of figure. Many take cheap Korean-made mannequins that do not match Russian figures at all.

And there are a lot of such parts: the stand and how it is adjusted, the markings, the durability. In the end, it turns out that buying a cheap dummy you pay more – because they break more often and bring less benefit.

  • Why markings on the dummy?

Marking makes it easier to design and make adjustments. It should be of a contrasting color, stand out brightly against the background. Then you will understand exactly what and where you need to move in order to make changes. You can apply the markings yourself – or, again, save time and effort by purchasing a dummy, on which the lines have already been drawn according to all the rules.

  • How will a mannequin help with sewing?

With it you can achieve the perfect fit, design products, create the most complex designs and hone your tailoring skills to a professional level. Don’t put off buying a mannequin until later: take advantage of all the benefits at once.

  • Which color should you choose?

It all depends on your taste. We have thought of mannequins in beige and black to the smallest detail. They have contrasting markings (Monica and Richard models), the coating does not get dirty or easily erased, as on the Christina and Henry models. So build on your preferences. After all, you can always buy both colors!

Now you know exactly which dummy you need? See our catalog, choose the size and order!

Mannequins

The word “mannequin” comes from the Dutch manneken “little man”, reduced.

In Ozhegov’s dictionary, “mannequin” has the following meaning:

1. A figure in the form of a human body, used when sewing or finishing clothes. Tailor’s mannequin.

2. A doll in the height of a person for displaying clothes. Mannequins in a shop window.

3. A doll with movable limbs, used by artists for sketches.

For the first time, something similar to a mannequin was found in the tomb of Pharaoh Tutankhamun, it was a wooden torso, repeating the figure of the emperor.The fact is that the tailors did not have the right to touch the head of state with their hands, but they were obliged to constantly update the ruler’s wardrobe. Paris, 1st half of the 17th century, royal court. Pandora the Big and Pandora the Little appear: dolls showing the latest fashions. At that time, French tailors were already actively using wooden models of the human body for fitting, and these very figures were exact copies of the figures of wealthy clients. With the development of industrial tailoring, they began to be used exclusively for demonstration purposes, their image was constantly improved, mannequins with hair and teeth appeared.The materials for manufacturing were still: wood and papier-mâché, in the 19th century they began to be made from wax. But they all had a significant disadvantage: a short service life at a high cost. What are modern mannequins? In the 20th century, with the advent of plastic, there is a revolution in the production of mannequins. Products made of plastic become stronger, lighter, more durable, and cheaper. Plastic mannequins were widely used and are actively used now in the field of trade and sewing clothes.What are mannequins like?

Anthropoid

Mannequins exactly repeat the shape of the human body, respectively, there are 4 types: male, female, children: boy, girl. Naturalistic mannequins – one hundred percent imitation of a figure, a person’s appearance, up to nails with manicure, makeup and hairstyles. They have a solid figure with rotating body parts. The height of female mannequins is 175-185 cm, for male mannequins – 185-195 cm. Model parameters (90-60-90) are taken for the figure.Weight 15-18 kg.

Stylized

These are abstract mannequins, often some part of the body of which is replaced by cones, squares and other figures. They emphasize the style and image of the store. Made exclusively to order.

Specialized

Mannequins for different areas of business. These include sports, intended for sports stores, clubs: boxer, running athlete, girl doing fitness. There are pet mannequins for veterinary clinics and pet shops, for children, for displaying underwear, which are made from a high quality light-colored cover with no seams.

Individual elements

This group includes mannequins for demonstrating certain types of goods: gloves, watches – hands, for demonstrating headwear – heads, in a store selling tights – legs.

Vanguard

They are made strictly to order, have unusual colors, shapes, style. The task of each owner of a retail outlet is to stand out, attract visitors, and it is the bright, extraordinary mannequins that help to accomplish this task.Blue, yellow, red, frozen in unusual poses, often with animal faces instead of human faces, they accurately fulfill the assigned task, epotage and attract customers.

Tailor’s

Used for sewing clothes in ateliers, sewing workshops. Usually such mannequins are covered with black or white fabric and it is convenient to draw auxiliary lines on them (front length, waist circumference, etc.). Sliding mannequins varying in 5-10 sizes are widespread and very convenient.As a result, the acquisition is much more economical.

Crash dummies

Used as a substitute – “probe” of the behavior of the human body in certain conditions: accident, parachute jump, etc. Such mannequins should not repeat the shape of the body, their task is to withstand the same loads that the human body will withstand in certain situations. Almost no shop window can do without humanoid figures. Revitalize, decorate, add emotion to the sales process, attract and retain a customer: these are the tasks they do well.And to solve these problems, entrepreneurs are ready to invest, often considerable ones. Indeed, in the end, the costs incurred are paid off in full. The most expensive mannequins are made of polymer resin with reinforced glass mat and mineral additives, the so-called fiberglass. Mannequins made of this material are durable, lightweight, and durable. Cheaper options for mannequins: plastic, plaster, polyester. The mannequin manufacturing process is quite lengthy, it can take up to five months of work. Their creation is a creative process, artists, makeup artists, designers, stylists work on its development.Therefore, they are so unique, sometimes funny, intriguing. A working shoemaker in the window of a shoe workshop, conveying his energy of painstaking work to the window, a tanned slender female torso in a swimwear store, the figure of an African mother in a coffee shop, a group of dancing teenage mannequins in a trendy youth clothing store, a charming skeleton in an English bar, with which I am happy are photographed. All these are mannequins created by the person himself and serving him: making the process of communication with the client more accessible, emotional, and cost-effective.

Nature (UK): gene therapy in the fight against a terrible disease | Science | InosMI

For the mother of Graevis Bakatunkanda, the symptoms of her son’s illness were all too well known: if the son suddenly lost interest in food, then soon expect a pain attack that came every week, as if on schedule. And Graevis, this shy and thin guy who then lived in the Democratic Republic of Congo, every time after an attack, he ended up in the nearest hospital, where he was injected with morphine for pain relief and was constantly diagnosed with malaria.

But the doctors were wrong: the culprit of Bakatunkanda’s disease was not malaria parasites at all, but blood erythrocytes. Some of the red blood cell cells, which should normally be soft and taut, suddenly deform and become stiff like wood chips. Then they enter the bloodstream and are transported to the vital organs – it is at this moment that severe pains occur, radiating to the back and chest.

And only in 2003, after moving to Cape Town (South Africa), the Bakatunkanda family learned that their son suffers from sickle cell disease, one of the most common genetic diseases, which had been studied by that time for more than a century.But the diagnosis is half the battle, it did little to alleviate the suffering of Graevis: he was prescribed literally a whole cocktail of drugs, but it did not help (note that each of the prescribed drugs was invented more than half a century ago, but none was developed specifically for the treatment of sickle cell anemia) …

Bakatunkand is now 22 years old. And now on the horizon are finally modern methods of treating sickle cell anemia using gene therapy. After decades of work and several major setbacks, gene therapy methods that can alter the human genome have begun to be recommended for the treatment of some rare diseases.Scientists are currently working to take advantage of the latest medical advances (including techniques that use the latest gene editing technologies) to combat sickle cell disease, which affects about 20 million people worldwide. More than half a dozen clinical trials have already been conducted; more are planned. “The trials are now literally one after the other,” said Lakshmanan Krishnamurti, a pediatric hematology specialist at Emory University in Atlanta, Georgia.”Yes, these are interesting times.”

But sickle cell disease is a major challenge for gene therapy, both ethically and technologically. The fact is that gene therapy as a method of treatment has arisen in special conditions, the prices for the use of its methods in health care start from one million US dollars. However, sickle cell disease is common in regions of the world such as sub-Saharan Africa, India and the Caribbean, where few can afford to pay such hefty bills for gene therapy.If we talk about experimental methods of treating sickle cell anemia, then here the patient also has to face great difficulties – he needs a long stay in the hospital, while doctors must be highly qualified, and such medical staff is available mainly only in large scientific medical centers. But even those patients who have access to quite expensive medical services still have to take risks – but is it worth doing?

As more and more trial data becomes available, scientists are trying to improve treatment methods, and sponsors have come close to deciding whether these methods are available.And so on October 23, the US National Institutes of Health (NIH), in conjunction with the Bill & Melinda Gates Foundation, announced that they intend to invest at least $ 200 million over the next four years to make therapies for sickle cell anemia and HIV more affordable.

Bakatunkanda founded a support group for people with sickle cell disease. He is confident that gene therapy, if it suddenly turns out to be really effective, despite its high cost and incredible complexity, will someday reach his homeland.“I’m sure it will, because South Africa is on the rise,” he says.

Still, he and other people suffering from this disease need to remain realistic. “My patients have internet access and now they come up to me and ask, ‘Can we get gene therapy?” Says Dipty Jain, a pediatrician at the State College of Medicine in Nagpur, India. . – I answer them: “So far this is not for you.”

El País

Illustrerad Vetenskap

New Scientist

Medical Revolution

Sickle cell anemia is characterized by elongated erythrocyte cells with an unusual shape. They were first found in 1910 in the blood of a young dental student Walter Clement Noel, a native of Grenada in the West Indies. Forty years later, the mechanism underlying this disease began to be gradually clarified when biochemist Linus Pauling, together with colleagues, reported that changes in the structure of hemoglobin – i.e.That is, the oxygen-carrying protein found in red blood cells – lead to a change in the shape of blood cells.

In this publication, scientists have been able to trace the consequences of this genetic disorder at the molecular level for the first time. Pauling named the cause “molecular disease.” Several years later, researchers determined that this genetic disorder is due to changes in the β-globin protein. It is the mutation that occurs in both copies of the gene encoding this protein that leads to sickle cell anemia; one mutant copy correlates with fewer symptoms and protects the wearer from blood-borne parasites such as those that cause malaria.This is partly the reason why sickle cell disease is especially common in malaria-endemic regions of the world. “This is really the basis from which everything we know about human medical genetics today was developed,” explains Ambroise Wonkam, a geneticist at the University of Cape Town.

Hematologist Olu Akinyanju, founder and first chairman of the Sickle Cell Foundation (based in Lagos, Nigeria), seventy years after Pauling’s discovery, continues to be underdiagnosed in many African countries, according to hematologist Olu Akinyanju.However, early diagnosis is a lifesaver. More than 300,000 people are born each year with sickle cell disease, and most of them will not live until the age of five unless they take prophylactic antibiotics and vaccines that suppress other infections. And those who survived face the risk of pain attacks; in addition, they are at risk of stroke or infection.

The close association of sickle cell disease with low-income countries means that the disease has received little attention in the past from pharmaceutical companies and governments in rich countries.Many African nations already face such acute public health challenges that sickle cell disease is not yet a priority for them, says Akinyanju, who has been trying for decades to get African governments to move towards developing treatment plans for the disease.

However, over the past ten years, Akinyanju and his associates have noticed positive developments. Patients with sickle cell disease are now living longer as doctors and other activists push for newborn screening and early medical care.Now the situation is not as dire as it used to be. Akinyanju proudly mentions the names of his friends who have been diagnosed with sickle cell anemia – all of these people have lived to be 60 years old and have become doctors, judges, and travelers.

The World Health Organization and the American Society of Hematology also played a major role in attracting scientists and pharmaceutical companies to research on the disease. “People like Bakatunkanda and other immigrants have also contributed to the increased awareness of this blood disorder in richer countries,” added Wonkam.Apparently, such attention to the disease has not been in vain. And on November 25, the US Food and Drug Administration (FDA) approved a drug for the treatment of sickle cell anemia, which should reduce the aggregation of hemoglobin molecules.

It would seem that for this perhaps one of the world’s most famous genetic diseases, the natural remedy is gene therapy. But here many problems and failures arise. The first attempts to use gene therapy were overshadowed by the high-profile death of Jesse Gelsinger in 1999, who participated in one of the first clinical trials.The trial used a procedure to replace immune system genes in blood stem cells, but the procedure caused leukemia in several participants.

According to hematologist David Williams of Boston Children’s Hospital in Massachusetts, after the death of Jesse Jelsinger, some experts began to believe that it was somewhat premature to use gene therapy for sickle cell anemia. “Sickle cell disease is not a fatal disease,” adds Williams.”And therefore, in a sense, it would be immoral to treat people suffering from it with very risky experimental techniques.”

In addition, the instruments used are still not up to the task, says Donald Kohn, a pediatric bone marrow transplant specialist at the University of California, Los Angeles. Now, if scientists were able to switch to the normal hemoglobin gene and increase the production of non-pathogenic protein, this would be a good way to neutralize the negative effects of sickle-shaped erythrocytes.“In the past, gene therapy technologies could not express genes at such a high level in human cells,” explains Kohn.

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But despite the setbacks, some gene therapy researchers insisted on developing safer and more efficient ways to transfer genes into cells. The success was achieved in 2016 when the European Commission approved gene therapy for the treatment of severe combined immunodeficiency (ADA-SCID), a rare immunological disorder that sometimes causes babies to miss their first birthday.Then, in 2017, the FDA approved gene therapy to treat one of the rare forms of blindness.

By that time, some researchers, armed with more sophisticated tools and relying on the achievements of the biotechnological industry, again turned their attention to sickle cells. Various approaches are currently used in experiments. To fight sickle cells, Cohn tries to insert a copy of the modified β-globin gene.The Cambridge, Massachusetts-based Bluebird Bio is trying to do the same. According to Yaron Werber, a biotechnology analyst with financial services firm Cowen in New York, Bluebird Bio appears to be the first to get approval to market its therapy to the US market.

Other scientists propose to introduce modified copies of genes encoding fetal hemoglobin (ie, a protein that is produced in the developing fetus and is usually turned off shortly after the birth of a baby).Fetal hemoglobin is a tempting thing, because it acts as effectively as its analogue – ordinary hemoglobin circulating in the adult body; fetal hemoglobin prevents the aggregation of defective hemoglobin molecules.

A third approach is to block the mechanism that turns off the production of fetal hemoglobin after birth. In this case, the BCL11A protein acts as a “switch”, and its suppression in mice suffering from sickle cell anemia is able to maintain the level of fetal hemoglobin at a high level even after birth, as well as prevent the symptoms of sickle cell anemia.In Boston, Williams licensed Bluebird Bio’s technology, which uses a so-called RNA interference technique to reduce the expression of the gene encoding BCL11A in blood stem cells. Richmond, Calif., Sangamo Therapeutics, in partnership with Paris-based Sanofi, is using gene editing tools called zinc finger nucleases (ZFNs) to create mutations that disable the gene. And Boston-based Vertex Pharmaceuticals is working with Cambridge, Massachusetts-based CRISPR Therapeutics to do the same using the CRISPR-Cas9 gene editing technique.In all three approaches, hematopoietic stem cells are removed from the body, then genetically modified (often with the help of a virus) and then reintroduced into the bone marrow. Before cell replacement surgery, patients usually undergo chemotherapy with busulfan; this procedure is aimed at destroying the remaining diseased stem cells and is designed to promote the proliferation of newly introduced, genetically modified cells.

This method of therapy is risky, as the patient may develop an acute and even severe form of anemia.When using this therapy, leukocytes are destroyed and a blow is applied to the intestinal mucosa, while the body becomes dependent on intravenous nutrition. Many patients will have to stay in the hospital for a month or more. In addition, chemotherapy causes infertility and, later in life, cancer.

This means that gene therapy is likely to be used only for those patients who are diagnosed with the most severe forms of sickle cell disease.But many of these people will continue to experience side effects in the form of heart, kidney or liver disease, which is why chemotherapy should be considered too dangerous.

There are other reasons why sickle cell disease is difficult to treat. In many cases, when doctors collect bone marrow, patients must first receive a drug to help collect blood stem cells. But this operation is too dangerous for people suffering from sickle cell disease, as it increases the risk of pain attacks.And because defective red blood cells die faster than healthy ones, stem cells in a person with sickle cell disease must work harder to make more red blood cells. As a result, patients sometimes need blood transfusions just prior to harvesting the bone marrow to relieve the stress on the stem cells. But as soon as the first signs of success appeared, they were trumpeted everywhere. So, in one of the patients subjected to RNA interference in Williams’ laboratory, no complications were observed for a year.And the first patient to receive CRISPR therapy left the hospital after completing a grueling course of treatment. And on November 19, Vertex and CRISPR Therapeutics announced that the patient had no episodes of pain; however, his fetal hemoglobin level remained high for four months. Both clinical trials have generated media enthusiasm, sometimes too much. “Now I find it difficult to accept the overwhelming enthusiasm with which people react to the fact that one of the patients was discharged from the hospital.As if it were tantamount to treatment, ”says Alexis Thompson, a hematologist at Northwestern University in Chicago, Illinois. “This is not a great achievement.”

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Still, there is reason for cautious optimism. So far, none of the clinical trials we mentioned have been stopped for safety reasons.Bluebird Bio reported that 13 people were undergoing therapy, some of whom were followed up for a year after discharge, without any serious pain attacks. In June, the gene therapy used was approved in the European Union for the treatment of some patients with another genetic blood disorder, β-thalassemia.

But the main problem for many people is the cost of treatment. For example, treatment for β-thalassemia costs about US $ 1.8 million, excluding hospital stays and other related costs.

And yet, in fact, it’s cheaper than the amount a patient would have to shell out for a lifetime, says Mani Foroohar, an analyst with investment bank SVB Leerink in Boston, Massachusetts. In addition, Bluebird Bio has established an unusual payment method: the payment schedule is extended for five years and if the effect of the treatment suddenly disappears, then payments can be stopped. However, Forouhar says it is unclear if this payment model will be possible in other regions of the world.

Such gigantic sums required for a course of treatment are certainly not affordable for most patients living, for example, in central India, who go to the hospital to Deepti Jane, because they cannot afford to shell out only about three dollars a month for the usual medicines. Even in the United States, access to gene therapy may not be affordable for everyone. This is especially true for black Americans, who tend to have more limited access to health care than white Americans.Although clinical trials are still at an early stage, Krishnamurti encourages interested people to raise the issue of the availability of therapy now. “This is a huge ethical issue,” says Krishnamurti (and he has to counsel patients suffering from sickle cell disease on a weekly basis at his Atlanta hospital). “In my community, I constantly write: ‘You had better say your weighty word, otherwise decisions will be made without your participation.’”

At the Children’s Hospital in Cincinnati, Ohio, hematologist Punam Malik wants to bring the day when gene therapy is cheaper and simpler.Malik received her medical education in India, where she had the opportunity to see many people suffering from sickle cell anemia and related diseases. When Malik immigrated to the United States about 30 years ago, she vowed that she would do her best to ensure that the fruits of her research would help people in poor countries.

Malik is currently conducting a study involving the introduction of stem cells that produce fetal hemoglobin. Scientists inject low doses of a drug called melphalan, which removes diseased cells from the bone marrow, which should make the treatment less toxic than busulfan.Malik hopes that thanks to this technique, patients will be relieved of the need for long hospital stays. In addition, this technique, in her opinion, will reduce the cost of treatment, making it safer and more effective.

However, this technique has been criticized by other scientists, who reason as follows: if you use small doses of the drug, then some cells will remain defective, and the therapy will not be as effective. “It’s too daring,” says Stuart Orkin, a blood specialist at Boston Children’s Hospital.

Malik believes that once a high dose is considered effective, it is difficult to reduce it. Malik draws an analogy with cancer chemotherapy: for some cancers, for example, doctors reduced the dose of certain drugs and found that their effectiveness remained at the same level as when using the higher doses initially administered. But it took decades for oncologists to accept this conclusion.

Malik’s attempts have collided with the practice of exporting gene therapy methods to poorer regions of the world.Malik’s team of doctors has secured support from the US Food and Drug Administration, which has authorized clinical trials only at Cincinnati Children’s Hospital. But after Malik made a presentation at a conference held in Jamaica, one of the visitors, suffering from sickle cell anemia, approached her with a request for help and said that during painful attacks he often had to stay in hospitals.

After that Malik began to develop cooperation with Jamaica. “I was convinced that it was necessary to do this,” says Malik. It took her and her colleagues about two years to get all the necessary permits and funding. However, after that, doctors in Jamaica faced another problem – a lack of high-quality blood for transfusion.

Already in April, the team of doctors reported that the first patient experienced only two pain attacks in the next 18 months after discharge and had a high hemoglobin level.According to Malik, doctors have now begun treating a second patient; there are two more in line.

The problem lies not only in the cost and methodology, but also in the availability of experienced clinicians and medical institutions who are able to perform stem cell transplants. In rural areas, the problem of providing patients with hydroxycarbamide, a relatively cheap drug that reduces the incidence of pain attacks, is already being addressed. “I don’t think there will ever be a specialist in these areas who can follow up long-term gene therapy patients,” says anthropologist Duana Fullwiley of Stanford University in California.

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At the same time, some believe that it is too early to think about such problems. “If we improve the technology, then someday it will become available,” says Ambroise Woncam. – But it seems to me that the price is not the main problem now. The emphasis should be on the effectiveness of therapy. ”

However, other experts believe that now is the time to think about the problem of global access to advanced treatment methods.If this is not done now, then, according to the director of the National Institutes of Health (NIH) Francis Collins (Francis Collins), this state of affairs “should be considered immoral.”

Collins believes that the key to completing the NIH project with the Gates Foundation will be finding ways to deliver corrected genes or gene editing tools directly to bone marrow stem cells without first removing those cells from the body. This method will reduce the cost of therapy and simplify the delivery of genes.The task is too ambitious, Collins said, so it’s understandable that it is sometimes met with skepticism. “At times, when I had vague doubts, I would say to myself, ‘Boy, you went beyond reality,’” adds Collins.

According to Donald Cohn, the following ideas are already being put forward: the same viruses that are usually used in laboratory conditions to transfer genes into cells placed in a Petri dish can first be modified, and then with the help of these viruses the genes can be inserted into hematopoietic stem cells directly into the human body.“It’s too difficult a task,” Kohn adds. “But science is also developing rapidly.”

For Bakatunkanda, the salvation from sickle cell anemia was not medication at all, but the aging of the body. According to him, some people suffering from sickle cell anemia tolerate this disease much worse in childhood than in adulthood, and Bakatunkande thinks that he is just one of those people. No, no, he has not completely got rid of the bouts of pain, but they pester him not as often as in childhood.And in recent months, he even began to lead a more active lifestyle – now he goes hiking, is engaged in bodybuilding, and after all, once all this was contraindicated for medical reasons. “Now I know I can do a lot,” he says.

Nevertheless, Bakatunkanda would prefer to live without the constant fear of waiting for the next attack of pain and seizure. He knows that gene therapy has a great future. But whether gene therapy will be able to create the necessary medicine – there is still no clear answer to this question.And Bakatunkanda knows about it. According to him, he, of course, would not refuse gene therapy, but today it does not give firm guarantees.

InoSMI materials contain assessments exclusively of foreign mass media and do not reflect the position of the InoSMI editorial board.

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