On the night of 3 January 2011, 10-year-old Natalie Cohen woke up with severe chest pain. A day later she received emergency surgery after being diagnosed with an extremely rare form of bone cancer, called chordoma.
Until that fateful day, Natalie was a normal, fun-loving Grade Fiver at Camps Bay Primary School in Cape Town; loving animals, nature, books and all things arty and spending all her free time with her brother Kola and her best friend Ella - the three of them are better known as “the Rats”.
Chordoma is a relentless bone cancer that occurs in the head and spine in people of all ages. In Natalie’s case, the tumour is growing from her thoracic spine to her breast plate. The cancer has also damaged five of Natalie’s vertebrae.
“It seemed inconceivable that our little girl who appears so healthy, pain-free and perfect, carries an absurdly rare chordoma tumour in her chest,” Natalie’s mother, Shirley Lowe writes on her blog. “I’m not sure when, but at some point over the last weeks, surreal became our new reality and life BC (Before Chordoma) seems a million years ago.”
Natalie underwent emergency surgery on 5 January to relieve the pressure of the tumour on her windpipe, and in March she travelled with her mother to Boston, US, where a multidisciplinary team of world class doctors at Massachusetts General Hospital had developed a plan to meet Natalie’s unique medical needs and to remove the tumour.
“The surgery is very complex, and involves a multidisciplinary team of top surgeons. Very few surgical teams globally have this level of skill and experience,” Shirley explains. “After much research and soul-searching we decided to travel to Boston.
"The surgery and treatment are very expensive - in the region of R2-3m - and we are deeply grateful for all the financial and emotional support we have received so far from family, friends and strangers around the world,” Shirley adds.
Embryonic notochord remnants
According to the Chordoma Foundation, an international multidisciplinary research effort to develop effective treatments and ultimately a cure, chordoma is a malignant bone cancer that develops from remnants of embryonic notochord in the spine and head.
During the early embryonic phase, the notochord acts as a flexible rodlike structure from which the spinal column develops. As the bones of the spine and skull form, the notochord gradually disintegrates, and by birth only remains as scattered cells in the centre of the intervertebral discs. In most people, these scattered cells reduce after birth, and disappear all together by adolescence. However, one in five people, have small remnants of notochord in their spine and/or head that can persist throughout life. It is believed that these remnants undergo a malignant transformation which causes uncontrolled growth of cells, and ultimately leads to the formation of a chordoma.
Chordomas account for about 3% of all bone tumours. In the US, there are around 300 new cases of chordoma diagnosed each year - that is approximately one new case per million people per year. Chordomas tend to return and spread in the areas adjacent to the original tumour location and currently the average survival for a patient with chordoma is about 7 years. Complete surgical removal of the tumour offers the best chance for long-term survival.
radiation and undergone two complicated surgical procedures on 20 and 26 April respectively.
Since arriving in Boston on 21 March, Natalie has received pre-operative Proton Beam Therapy (PBT), a specialised form of
“The first 10-hour-long spinal surgery procedure was primarily focused on stabilising Natti’s spine with screws and titanium rods ahead of the tumour removal on 26 April,” Natalie’s father, Jonny Cohen explains.
“The next operation involves the removal of the five damaged vertebrae, and then fusing [a piece of] Natalie’s fibula [calf bone] into her spine. The task of removing the tumour will happen in the same procedure.”
Natalie already has undergone this complicated 15-hour-long second operation to remove the tumour and currently is recovering in ICU. “In terms of the complexity of the procedure and what was done, one of the surgeons told me this morning that it was ‘unchartered territory…off the charts’,” Jonny reported immediately after the surgery.
“Natalie is a true warrior princess with an immense spirit,” Jonny adds. “I watched as they temporarily ‘de-sedated’ Natti for the neurological tests, and even though she could not respond with all the tubes ventilating her, she squeezed my finger in acknowledgement with one hand. Then they tested her feet, and when she wiggled her toes, I could not hold back the tears.”
Once Natalie is rested and has recovered from the operation, she will receive further radiation therapy. Only time will tell if the surgery and treatment were successful in eradicating the chordoma permanently. Hopefully she will be able to return to her normal life soon and fulfill her dream of swimming with dolphins.
Visit www.nataliescircleoflove.org to follow Natalie’s journey. If you would like to contribute to her surgery costs, click here.
To learn more about chordoma, visit www.chordomafoundation.org.
- (Birgit Ottermann, Health24, April 2011)
Artist James Durno and The Bridge have produced this animated short film to explain Natalie's illness and treatment: