Wednesday, 31 October 2018


This is what an angel looks like, right? 

How about this? Is the above a picture of an angel?

Actually, it is. This is the only known picture – or drawing – of the angel who named my disabled son, or at least, she named the condition with which he was born.  

Her name was Cornelia de Lange. Cornelia means wise or strong. De Lange means one who lives at the sign of the angel. This woman was a wise and strong angel to thousands of children. 

Way back in 1897, Cornelia Catharina de Lange graduated from the University of Amsterdam. This is pretty amazing because in the United States, it was believed that women who went to college, wrote the Harvard gynecologist Edward H. Clarke in 1873, risked “neuralgia, uterine disease, hysteria, and other derangements of the nervous system.” (example of misogyny)

Cornelia de Lange was only the fourth woman in the Netherlands to write a medical thesis and become a doctor. After graduation she moved to Switzerland where she became a pioneer in a new field of medicine: pediatrics. 

By the late 1920s, Dr. de Lange achieved respect and acclaim for her work in pediatric medicine. In 1933, within weeks of each other, two unrelated children were admitted to the hospital. One was seventeen months old, the other was six months old. They had similar medical conditions and they resembled each other. Dr. de Lange recorded the similarities in minute detail and reported on these two remarkable children. 

In 1941, she presented a third child with the same medical and facial similarities to the Amsterdam Neurological Society, and the Cornelia de Lange Syndrome was defined.
Since Cornelia de Lange Syndrome (CdLS) only shows up in 1 in 10-30,000 children, can you imagine how many children Dr. de Lange cared for? Lucky kids. Devoted doctor. 
Frequently Asked Questions and Answers:
Question: What is Cornelia de Lange Syndrome?
Answer: A syndrome means there is a collection of recognizable signs and symptoms. Cornelia de Lange Syndrome is a very rare spontaneous genetic disorder with a specific and repetitive collection of recognizable signs and symptoms.  

Question: Okay, what does that mean in English? 
Answer: Similar to Down Syndrome, no one really knows why it happens. Neither can be predicted or prevented. 

Question: Can this syndrome be tested for?
Answer: Sometimes, after conception, Cornelia de Lange Syndrome can be tested for with chorionic villa sampling. That involves pinching off a tiny tissue sample of the placenta within the first weeks of conception. What is true is that this procedure may put the pregnancy at risk. Later on during the pregnancy, Cornelia de Lange Syndrome can often be seen on ultrasound. 

Question: What are the characteristic features of Cornelia de Lange Syndrome?
Answer: People with the syndrome have varied features as part of their heritage. These may range from mild to severe:
long and/or thick eyebrows,
short nose,
concave nasal ridge and/or upturned nasal tip,
thin upper lip and/or downturned corners of mouth,
missing fingers or toes or complete hands,
small hands and feet, and
small prenatal and birth size/weight.

Question: What are the health challenges of people with Cornelia de Lange Syndrome?
Answer: These may also range from mild to severe and may include:
respiratory illness,
heart defects,
hearing impairment,
vision abnormalities,
gastrointestinal abnormalities, reflux, diarrhea,
muscoskeletal problems,
cleft Palate,
feeding problems,
poor immune system, and
congenital diaphragmatic hernia.

Question: What is the treatment for Cornelia de Lange Syndrome?
Answer: An interdisciplinary approach is needed. A team for promoting the child's well-being often includes speech, occupational and physical therapists, teachers, physicians and parents. 

Question: Are intellectual delays present?
Answer: Yes, but the severity varies as much as the physical disabilities do. 

Question: Are there behavioral issues associated with Cornelia de Lange Syndrome?
Answer: Some parents and caretakers of children born with Cornelia de Lange Syndrome report autistic-like behavior such as aggression and self-injury.
Sometimes Adam displayed repetitive behaviors when he pulled at his single digit finger but generally, Adam was a happy roly-poly bundle of chuckles and joy. 

Question: What is the life expectancy of people with Cornelia de Lange Syndrome?
Answer: That’s a tough one. When Adam was born, his life expectancy was: ZERO. I was told to institutionalize and I would be notified when he died. This advice created extreme fear and caused me a lot of grief. But that was then. Now? Parents today have a realistic hope that most children with Cornelia de Lange Syndrome will survive into adulthood, however, each child must be evaluated for life-threatening conditions. Life expectancy for affected individuals is lengthening with better medical care but it would be fair to estimate their life span is less than the general population. 


This cute little kid’s pixie smile was irresistible. He could make me both laugh and cry with his antics. His tiny size, and delicate fingers say: “I’m a living, breathing doll. Love me, play with me, hug me.” Of course, I am prejudiced. I was and will always be his mother. 
My little boy was born with Cornelia de Lange Syndrome. He needed all the love and medical help plus all the intervention of angels I could find for him. He was worth every battle, every prayer, every worry, and every minute I had with him. 

The most difficult question of all: Would Adam be alive today if he hadn’t been murdered?
Answer: I will never know because a monster decided to abuse and murder my little boy when he was six-years old. 

“...I believe that the pediatrician will...flutter with butterflies, frolic with lambs, coo with babies and retain deep inside something of eternal youth.”
Dr. Cornelia de Lange, 1932


As Long As I Breathe is dedicated to:
survivors of emotional, physical, spiritual, or sexual abuse,
those who have had to bury a murdered child,
former members of a religious cult with a culture of misogyny, 
children born with Cornelia de Lange Syndrome,
and anyone who was falsely accused of a crime.


Joyce A Lefler is a true crime survivor and the author of
From Miracle to Murder: Justice For Adam.
She is a facilitator for Parents of Murdered Children,
a bereavement counselor, registered nurse,
and an advocate against abuse.

Connect with her:
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