HISTORY OF SICKLE CELL DISEASE


June 19 is observed as “World Sickle cell day” every year.

On 22nd December 2008, The United Nations General Assembly decided to celebrate “World Sickle cell day” on June 19 every year to raise awareness about Sickle cell disease. Sickle cell disease is recognized as a public health problem and ‘one of the world’s foremost genetic diseases’.

In this post, lets see the history of sickle cell disease (SCD).

SCD was first discovered in 1910 in medical history. But it was known in Africa even atleast five thousand years before and was called by various names.

Here it goes, how the SCD came into light in medicine.

A young dental student, Walter Clement Noel from the island of Grenada, was studying in Chicago. He had complaints of pain and symptoms of anemia, for which he visited a cardiologist Dr. James B.Herrick. Noel was seen by Herrick resident Dr.Ernest Irons as Herrick was not interested in this case.

                                                     Dr.James B. Herrick and Dr.Ernest Irons. 

And now the surprise came to everyone. Irons saw Noels blood under the microscope and he saw the red blood cells having the shape of sickle cells. Herrick saw this in the chart and got interested as he thought this might be a new disease. He subsequently published a paper and described it as sickle shaped cells. The mystery of this disease was getting deepened as more cases emerged. One thing was clear, it occurred primarily in persons of African origin. In 1922, Dr.Verne Rheem Mason, medical resident at Johns Hopkins, named the disease as Sickle cell anemia, based on the description of Ernst Irons.

                                                                  Sickle

In 1927, Hahn and Gillespie discovered that red blood cells from persons with SCD could be made to sickle by removing oxygen. They also found that the relatives of this SCD patients had this trait of sickling when deprived  of oxygen but had no disease. This condition was known as “sickle trait”.

Many people were working on this SCD which led to publication of two significant articles in 1949. One was by Col.E.A. Beet, a military doctor from Portugese East Africa, now Mozambique in African Medical journal and the author was by Dr.James V. Neel, Chairman and founder of the Department of Human Genetics at the University of Michigan in American journal Science. It showed that SCD was inherited. It can be either homozygous (SS) or heterozygous (carriers or AS).

In 1951, Dr.Linus Pauling, the famous Nobel Prize- winning chemist and his colleague, Dr.Harvey Itano, discovered that the patients with SCD had a different chemical structure in their hemoglobin. Hence, Dr.Pauling coined the term “molecular disease” for disorders which resulted from proteins with abnormal chemical structure. In 1951, SCD was the first disease in that group, though thousands of diseases are known now. The actual amino acid substitution was discovered by Dr.Vernon Ingram in 1956.

In 1970s, more details were revealed about this disease and better tests for detection were developed. In the following years, better ways of treatment emerged which improved the quality of life of patients. Genetic counseling became an important measure. Great progress has been made in understanding the disease and its treatment.

A disease, which can be traced back to more than five thousand years in Africa, discovered only in 1910 in America, by a medical resident and the disease being named after an agricultural implement owing to the shape of red cells is Sickle cell disease.

References:
1.     www.sicklecell.howard.edu.
2.    Images taken from Google images.

                                                                                      Written by Dr.Priyavadhana.






Comments

  1. Wow. Very interesting. No one have came this history before. We only read what is mean by sickle cell nd findings. But u have explained the history behind it . Thank you

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