Blues Guitarist Johnny Winter Has Died

Blues Guitarist Johnny Winter has died at the age of 70. He was found in his hotel room in Zurich, Switzerland, where he was on tour. No cause of death has been released at this time.

Known for his high-energy blues-rock albums in the 1960s and ’70s, Winter also produced three Grammy Award-winning albums for blues legend Muddy Waters.

In 1988, he was inducted into the Blues Foundation Hall of Fame and in 2003, he was ranked 63rd in Rolling Stone magazine’s list of the “100 Greatest Guitarists of All Time.”

His spokesperson, Lori Haynes, released a statement saying:

“His wife, family and bandmates are all saddened by the loss of their loved one and one of the world’s finest guitarists.”

Johnny’s brother, Edgar Winter, is a rock and blues musician who is known for his 70’s band, The Edgar Winter Group, and their popular songs “Frankenstein“and “Free Ride“. Both Johnny and Edgar were born with albinism.

What is Albinism?

melaninAlbinism is a defect of melanin production that results in little or no color (pigment) in the skin, hair, and eyes. Melanin is a natural substance that gives color (pigment) to hair, skin, and the iris of the eye. It is produced by cells in the skin called melanocytes. Melanin helps protect the skin from the UV effects of sunlight.

Albinism, also known as oculocutaneous albinism, is a group of conditions that affect coloring (pigmentation) of the skin, hair, and eyes. Affected individuals typically have very fair skin and white or light-colored hair. It is considered a genetic disorder-i.e. it runs in families.

Overall, an estimated 1 in 20,000 people worldwide are born with oculocutaneous albinism. The condition affects people in many ethnic groups and geographical regions. In the United States, most people with albinism live normal life spans and have the same types of general medical problems as the rest of the population.

albinismThere are four forms of oculocutaneous albinism, designated OCA1 through OCA4:

  1. Oculocutaneous albinism type 1 is characterized by white hair, very pale skin, and light-colored irises.
  2. Type 2 is typically less severe than type 1; the skin is usually a creamy white color and hair may be light yellow, blond, or light brown.
  3. Type 3 includes a form of albinism called rufous oculocutaneous albinism, which usually affects dark-skinned people. Affected individuals have reddish-brown skin, ginger or red hair, and hazel or brown irises. Type 3 is often associated with milder vision abnormalities than the other forms of oculocutaneous albinism.
  4. Type 4 has signs and symptoms similar to those seen with type 2.

Types 1 and 2 are the most common forms of this condition. Type 2 occurs more frequently in African Americans, some Native American groups, and people from sub-Saharan Africa.

What are the manifestations?

Skin:

Because those with albinism are unable to make sufficient amounts of melanin, their skin not only looks light, but it also lacks the natural protection melanin provides for the skin. Affected individuals are unable to tan and are at greatly increased risk of sunburn. Long-term sun exposure greatly increases the risk of skin damage and skin cancers, including an aggressive form of skin cancer called melanoma.

Eyes:

eye_anatomyAlbinism reduces pigmentation of the colored part of the eye (the iris) and the light-sensitive tissue at the back of the eye (the retina). People with this condition usually have vision problems such as reduced visual sharpnes; rapid, involuntary eye movements (nystagmus); and increased sensitivity to light (photophobia).

How do people inherit oculocutaneous albinism?

Each of the four types of oculocutaneous albinism is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. Most often, the parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but do not show signs and symptoms of the condition.

Treatment

There is no specific cure for albinism. The goal of treatment if to relieve symptoms and is directed toward protection of the skin and eyes:

  • Avoiding the sun, using sunscreen and covering as much skin as possible with clothing
  • Sunscreens should have a high SPF factor.
  • Sunglasses (UV protected) may relieve light sensitivity and protect the eyes from UV damage.
  • Glasses can correct some vision problems.
  • Occasionally eye muscle surgery is done to correct eye position (strabismus) problems and nystagmus.
Michele R. Berman, M.D. was Clinical Director of The Pediatric Center, a private practice on Capitol Hill in Washington, D.C. from 1988-2000, and was named Outstanding Washington Physician by Washingtonian Magazine in 1999. She was a medical internet pioneer having established one of the first medical practice websites in 1997. Dr. Berman also authored a monthly column for Washington Parent Magazine.

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