A mother of five lived 99 years with a “completely retarded” body, doctors have revealed.
Rose Marie Bentley, from Molalla in northwestern Oregon, turned out to have situs inversus with levocardia.
This caused her liver, stomach and pancreas to develop on her right side instead of in their normal more ‘left’ position.
Mrs. Bentley was not aware of her bizarre anatomy, where her condition only came to light after she had donated her body for medical examination.
The mother – who died in October 2017 – is considered the oldest known person with the condition. Doctors say that the chance of survival after childhood is as small as one in 50 million.
Although most patients die from heart failure, Ms. Bentley’s family claims she led a healthy life, with only symptoms such as arthritis and acid reflux.
Rose Marie Bentley (photo) died on October 11, 2017 at the age of 99. She is thought to be the oldest known person with sitoc inversus with levocardia, causing her liver, stomach and pancreas to develop on her right side instead of in their normal left position
Ms. Bentley (pictured as a younger woman), born in 1918, was unaware of her bizarre condition and never suffered any health issues except arthritis and acid reflux. She is one in 50 million with the condition that survives into adulthood and unusually has no heart defects
Medical students encountered Ms. Bentley’s condition in March last year after she gave her body to the Oregon Health & Science University (OHSU) donation program.
Cam Walker, assistant professor of anatomy at the university’s anatomical service center, said: ‘I knew something was wrong, but it took a while before we found out how she was structured.
“I think the chance of finding another person like her can be as far as the 50 million. I don’t think any of us will ever forget, to be honest. “
WHAT IS SITUS INVERSUS WITH LEVOCARDIA?
Situs inversus is a genetic disorder that occurs when the internal organs are arranged as a ‘mirror image’.
It is thought that it affects one in 22,000 people. Singers Enrique Iglesias and Donny Osmond are known to have it.
The condition causes the most important abdominal organs – such as the liver, pancreas and spleen – to shift to the right instead of being in their usual ‘left’ position.
Situs inversus alone rarely causes complications, with most patients not knowing they have it.
However, problems can occur if the heart is the only organ that develops on the right side of the body, known as situs inversus with levocardia.
This can lead to a fetus developing heart defects that can lead to cardiovascular failure later in life.
It is estimated that the chance that a patient with situs inversus with levocardia lives in adulthood is only one in 50 million.
Situs inversus patients must wear a medical identification tag to alert emergency medical services that their internal organs are reversed so that they can act accordingly.
For example, by listening to a heartbeat on the right side instead of the left side of the chest.
The medical students had to judge the hearts of the cadavers.
Those who examined Ms. Bentley quickly saw that she was “missing” her inferior vena cava – the great vein that transports oxygen-poor blood from the lower part of the body to the heart to “absorb” oxygen.
Student Warren Nielsen, who is now in his second year in medicine, has called on the professors: ‘That is the moment when the crowds begin.
“They are like” oh my God, this is all the way back! “
Upon closer inspection, doctors noticed that Mrs. Bentley’s abdominal organs were like a mirror image.
“Instead of having a stomach on the left, which is normal, her stomach was on the right,” Professor Walker said CNN.
“Her liver, which normally occurs mainly on the right, was mainly on the left.
“Her spleen was on the right instead of the left.”
And that was not the end of Mrs. Bentley’s anatomical abnormalities.
Her inferior vena cava was not missing, but ran to the left of her vertebrae instead of to the right.
The veins that usually run in the liver or chest were missing or in an unusual place.
Her right lung only had two lobes instead of three. And the right atrium of her heart was twice his normal size.
Situs inversus with levocardia affects one in every 22,000 births, with most patients suffering from life-threatening heart defects.
According to the OHSU team, only five to 15 percent of patients live at the age of five.
They estimate that a patient’s chance of survival into adulthood is one in 50 million.
On the right you see a diagram of Mrs. Bentley’s ‘mirrored’ belly, with her stomach, spleen and pancreas all ‘shifted’ from their normal position. The only vital organ that developed in the right place was her heart. The left shows that she only had two lung lobes, instead of the usual three
I WILL ALWAYS LIVE: THE POEM THAT MRS BENTLEY INSPIRED TO KNOW HER BODY IN SCIENCE
The day will come when my body is lying on a white sheet, neatly stored under four corners of a mattress in a hospital; busy with the living and the dying.
At a certain moment a doctor will determine that my brain is no longer functioning and that my life has been stopped for all purposes.
When that happens, don’t try to bring artificial life into my body through the use of a machine, and don’t call this my deathbed, let it be called the bed of life, and let my body be taken away from it to help others lead a fuller life.
Give my face to the man who has never seen a sunrise, the face of a baby or love in the eyes of a woman.
Give my heart to someone whose own heart has caused nothing but endless days of pain.
Give my blood to the teenager who was pulled out of the wreck of his car so that he could live to see his grandchildren play.
Give my kidneys to the one who depends on a machine to exist from week to week.
Take my bones, every muscle, every fiber and nerve in my body and find a way to make a lame child walk.
Explore every corner of my brain.
Take my cells, if necessary, and let them grow so that one day a speechless boy screams at the crack of a bat and a deaf girl hears the sound of rain against her window.
Burn what is left of me and scatter the ashes to the wind to help the flowers grow.
If you have to bury something, let it be my mistakes, my weakness and all prejudices against my fellow man.
Give my sins to the devil.
Give my soul to God.
If you happen to remember me, do it with a kind act or a word to someone you need.
If you do everything I have asked, I will live forever.
From Robert Test
Mrs Bentley did not suffer any cardiovascular problems, which the scientists owe to her long life.
When the heart is ‘mirrored’ like the rest of the abdominal organs, a patient is usually okay.
But when the heart is the only organ that does not shift its position – as in the case of Mrs. Bentley – the affected fetus typically develops defects that lead to heart failure later in life.
Before Mrs. Bentley, there were only two known cases in which patients lived in their seventies.
Speaking of the investigation, Mr. Nielsen, then 26, said: “It was pretty amazing.
‘We could not only learn the normal anatomy, but also all the anatomical variations that can occur.
“I started to appreciate how she could live as long as she did. I wondered who she was.
Through the experience I look forward to caring for patients and being able to apply what I have learned from her. ”
A poster about the state of Mrs. Bentley was presented at the annual meeting of the American Association of Anatomists 2019 in Experimental Biology in Orlando.
She and her husband James – who died of pneumonia 13 years earlier – decided to donate their bodies to OHSU after reading a moving poem about remembering our loved ones when they die.
I Will Live Forever by Robert Test reads: ‘Give my face to the man who has never seen a sunrise, the face of a baby or love in the eyes of a woman.
“Give my blood to the teenager who was pulled out of the wreck of his car so that he could live to see his grandchildren play.
“Give my kidneys to someone who is dependent on a machine from week to week.”
It concludes with the rule: “If you do everything I have asked, I will live forever.”
Their family read the same poem at the OHSU’s Service of Gratitude of December 2018, thanking donors and their families for their selfless act.
Mrs. Bentley, born Phelps, was born in 1918 in the small coastal town of Waldport, Oregon.
Mrs. Bentley was an avid swimmer, camper and fisherman. The mother of five lived in the rural town of Molalla in northwest Oregon and continued to grow vegetables for her large family until her death. The trained hairdresser also sang in her local church choir and gave Sunday school
Mrs. Bentley, the youngest of four, admitted that she was spoiled, according to her oldest child Patti Helmig, 78.
Mrs. Bentley was trained as a hairdresser and volunteered as a nurse corps during the Second World War.
The ‘excellent swimmer’ was very active and often took her children camping and fishing, according to her third child Ginger Robbins, 76.
Mrs. Bentley – who lived in the rural town of Molalla in northwest Oregon – had her gallbladder, appendix, and uterus removed during her lifetime.
However, doctors have never picked up her condition. But a surgeon did notice that her appendix was in an unusual place in their notes.
She and her husband owned and ran the Bentley Food Store, which sold farm and pet supplies.
The couple visited all 50 states and traveled outside the US after they retired in 1980.
Until her death Mrs. Bentley went to the Molalla United Methodist Church, where she sang in a choir and taught Sunday School.
She was also an avid gardener and would grow vegetables to feed her large family.
Mrs. Bentley’s fourth child, Louise Allee, 66, added: “My mother would think this was so cool.
“She would tickle pink that she could learn something like that.
“She would probably get a big smile on her face, knowing she was different, but it went through.”