It’s remarkable that Alex Clementson is still alive. For the past two years, he’s been battling with a rare and life-threatening condition which has destroyed half his jaw bone. It took months to diagnose. Alex was eventually diagnosed with Gorham-Stout Syndrome or Vanishing Bone Disease, an inflammation which stimulates cells inside the bone to eat itself away. Only 200 cases have ever been recorded.

Alex Clementson was referred to Great Ormond Street Hospital shortly after his diagnosis. In her 25 years of paediatric medicine, consultant Penelope Brock has never seen a case like his before.

Dr Penelope Brock

Cranial Scan Gorham Stout Syndrome
Cranial Scan

Dr Brock explains “This is a 3D CT scan of Alex when he first came to Great Ormond Street and I think what is really striking is that you can see that his jaw is missing. The jaw had literally been dissolved by the disease process. In the skeleton picture, the bone is simply not there and the teeth are completely floating and the part of the bone under the teeth has disappeared”.

Doctors had to act quickly. Six months ago, in a pioneering eleven-hour operation they removed the remains of Alex’s diseased jaw bone. They rebuilt it using a metal frame and bone grafts from his leg. The surgery gave Alex a new and functioning jaw bone but, tragically, it failed to stop the disease from spreading. Three months ago, it struck in a far more deadly form, attacking the soft tissue in the face causing it to swell.

Gorham-Stout Syndrome

Alex’s consultant, Dr Penelope Brock is a leading paediatric cancer specialist. Despite managing some of Europe’s most cutting-edge medical research trials, coming up with a treatment for Alex was a challenge. One of the articles Dr Penelope Brock found highlighted similarities between a cancer-like disease called Langerhans Cell Histiocytosis (LCH) and Alex’s Gorham-Stout syndrome. Based on her findings she decided that a treatment normally used for LCH might work.

It was now possible that it could spread up into the brain or down into the lungs and heart which would almost certainly prove to be fatal. Dr Penelope Brock had no option but to take a step into the unknown. Five weeks ago she put Alex on a pioneering drug treatment, a combination of chemotherapy and steroids, which had never been tried on Vanishing Bone Disease before. The initial results have been dramatic.

1950s Dresses

Back home in Burton-on-Trent, the swelling in Alex’s face has gone down, but it’s still early days. He has a punishing daily routine which starts first thing in the morning with seven different types of medication. When Alex had his jaw replaced, surgeons connected a tracheotomy to his windpipe to help him breathe. The dressing needs to be changed every day.

Vanishing Bone Disease

Alex Clementson has been coming to Great Ormond Street every other week since he started his treatment, but it’s the first time Dr Penelope Brock has seen him since the swelling in his face came down. “It’s great to see Alex now the swelling’s come down, but I’m not lax about it. I don’t know what’s going to happen is the next few weeks. This sort of disease can relapse very quickly”.

Dr Penelope Brock
Dr Penelope Brock

Dr Penelope Brock is happy with his progress, but she needs to examine some exposed bone in his mouth that’s giving cause for concern. She decides, for now, that it’s too risky for Alex Clementson to go ahead with his chemotherapy. Until he’s been seen by his craniofacial surgeon his treatment will be put on hold.

Alex Clementson is at a critical stage in his treatment, a major delay to his chemotherapy could seriously jeopardise his progress. David Dunaway, consultant craniofacial surgeon, examines Alex’s jaw and decides it is safe to proceed with the chemotherapy.

Most of his medication is delivered intravenously, so his tubes need regular cleaning to prevent infection. This is very painful for him.

Alex Clementson is on very powerful medication so, after six weeks, Dr Penelope Brock tried reducing the dose, but his face has started to swell again. This is a major setback for the family. It means Alex will need to check back into hospital for another intensive six week course of chemotherapy.

Alex Clementson

It’s been a tough year for Alex and his family and they are anxious for some good news. After his course of chemotherapy, Alex Clementson has another MRI scan. The scan shows that the vanishing bone disease is still active but the treatment is clearly keeping it at bay. Dr Penelope Brock decides it is now safe to reduce Alex’s medication.

Alex Clementson
Alex Clementson

From now on on Alex’s treatment will be less intensive. The chemotherapy will be administered orally, so he can spend more time at home.

He’s recently had the tracheotomy removed, which is a huge step forward, and he’s doing well on the reduced medication. Alex is by no means cured of the disease but his treatment is going better than anyone could have expected.

Another young boy with an extremely rare disorder is Petero Byakatonda and he is living with Crouzon’s Syndrome.

CREDITS: All of this information came from the UK Channel 5 “Child in a Million” documentary series

References

Gorham’s Disease – Wikipedia Page

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