Would you put your daughter through years of agony to make her taller That was the dilemma faced by 9-year-old Robyn’s parents when she was born with dwarfism
Robyn Fellows was diagnosed with dwarfism
Sitting beside her daughter’s bed, night after night, watching over her feverish, fitful sleep, Jane Fellows wonders whether she made the right decision.
Sometimes the little girl, Robyn, wakes, crying out in pain. The heavy steel frames that encase each of her legs in a web of pins and screws tangle, and tear the bedsheets.
Jane spends hours rubbing her daughter’s sore limbs better. And by the morning, her doubts are lifted and she reminds herself once again that the difficult choice to put her child through four years of agonising surgery will give her a better future. Robyn, nine, was born with a type of dwarfism in which most sufferers are unlikely to grow taller than 4ft. She was small even for someone with her condition and would have been much shorter.
When she was five, her parents, Jane and Simon, agreed to put her through a series of four operations to lengthen her legs. The procedure involves her legs being broken and then pulled apart, supported by the steel frames, as they heal.
The three operations Robyn has had so far have added six-and-a-half inches to her height – she is 3ft 9in, 2in shorter than the average for her age – but because the agonising procedures are not deemed medically essential, Robyn’s parents feel responsible for the pain she has had to endure.
‘You’re taking a healthy and well child into hospital and making her ill,’ says Jane, her eyes brimming with tears. ‘Ninety-nine per cent of our decision to go ahead was based on health reasons – to alleviate leg pain, leg bowing and spine curvature. If I’m honest, one per cent was because we didn’t want our child to go through life being different.’
Jane had no idea that anything was wrong with her daughter before she was born on January 4, 2003. The prenatal scan she had privately did not show that Robyn had achondroplasia, a type of dwarfism that affects one in 20,000 babies.
This bone-growth disorder means the torso grows at a normal rate, while the head grows larger than average and the arms and legs remain stunted.
It means that everyday tasks – switching on a light, posting a letter, using the toilet – present difficulties. It also causes health problems. The torso weighs down heavily on the smaller, weaker legs, causing them to bow, the spine to curve and the legs to be left in crippling pain that only worsens with time. Robyn was born by Caesarean, nine days after she was due, weighing a healthy 7lb 9oz and – aside from a slightly larger head – looking no different from every other baby on the ward.
It was only the following morning that Jane realised something was amiss when a paediatrician visited and began pulling at Robyn’s arms and legs. A second doctor confirmed the paediatrician’s suspicions. When Jane was told, she was devastated. ‘I went into shutdown,’ she says. ‘I couldn’t see or speak to anybody. I wanted it to be just me, Simon and Robyn,’ she says.
At first, Jane was convinced she was to blame. She is just 4ft 11in, while Simon is an average 5ft 9in. They are both now 46, and were concerned their heights or their age might be the cause.
But achondroplasia is caused by an additional gene developing at conception, and in 75 per cent of cases there is no history of dwarfism in the family.
Robyn gets a kiss from mother Jane while in hospital in 2009
Five days after Robyn’s birth, the family returned home to Haverfordwest, Wales, refusing to see visitors and living in what Jane calls a ‘bubble’.
While Simon continued his work as a cabinet-maker, Jane obsessively charted her daughter’s growth.
‘When I showed Robyn to people, I’d say she had a few problems,’ Jane says. ‘When she was a toddler, a woman pointed at her in the supermarket and asked, “What’s wrong with her” I pulled Robyn from the trolley and ran away crying.
They reasoned the pain Robyn suffered as a
result of the surgery would limit the pain she will feel in the future,
and would hopefully mean she was less likely to be a target for
‘Simon’s reaction, was “These are the cards we’ve been handed, let’s deal with it,” but I couldn’t understand how he was so positive. I saw only the negative, the future bullying.’
With time, Robyn’s height lagged well below average, and by the age of five she was wearing clothes designed for a year-old baby. Today, she wears clothes for five-year-olds.
‘She had leg cramps as soon as she started to walk at 13 months. Immediately, her legs bowed,’ says Jane.
When Robyn was nearly two, a midwife put the Fellows in touch with another local family whose nine-year-old daughter, Amy Sibley, also lived with achondroplasia. The Sibleys told them about the leg-lengthening.
‘They’d been trying to make this huge and horrible decision since Amy was five,’ says Jane.
The disorder means the torso grows at a normal rate while the head grows larger than average and the arms and legs remain stinted
‘We have long discussions about this with the family and, if they’re old enough, the child. The operation improves self confidence. The world is built for taller people. It’s not a option to be taken lightly, but Robyn is an object lesson in how it can all go well.’
In the first operation, the lower half of one leg and the upper part of the other is operated on. In the second operation, a year later, the other halves are lengthened. Between procedures, the height of the knees is uneven but the gradual process minimises the trauma to the body, so the bones heal better.
During surgery, which lasts three hours under general anaesthetic, the leg bones are broken using a surgical chisel, inserted through a small incision. Then a number of pins and screws are inserted at points around the broken section. These are attached to an external steel frame. The screws can be adjusted to lengthen the frame a millimetre at a time, a process that is carried out daily by Jane at home.
This creates a tiny fracture that then heals, prompting new bone to grow to fuse the break and lengthen the legs.
The screw-turning lasts 50 days, during which physiotherapy is given to stretch the muscles. Afterwards, the leg is put in plaster for six weeks while the bones heal. The process can be repeated, and in January last year Robyn went in for her third round of surgery. She will have her fourth operation next month.
Simon and Jane have decided this will be the last surgery for their girl.
‘Putting her through more pain and interrupting months of school felt too high a price to pay as she becomes older, and there is always the risk with each operation that it won’t work. Luckily, she has responded very well.’
In theory, the legs can be lengthened almost indefinitely, says orthopaedic surgeon Brett Rocos, at Yeovil District Hospital, Somerset. ‘The limiting factor is the skin and muscle, which must grow to accommodate the new height. And after a point, the joints – which have developed to support much shorter legs – will be thrown out of alignment. These operations are a balancing act between what is desirable and what the body can do.’
In the months following each operation, many of the activities that Robyn normally enjoys are forbidden. There is no swimming, sleepovers or walks on the beach in case sand gets into her wounds. Robyn is confined to a wheelchair, looking out of the window at the sand she wants so much to run across. The house has to be scrupulously clean. ‘The biggest risk of a frame such as this is infection getting into the leg,’ says Dr Rocos. ‘The more pins, the higher that risk. If an infection does take hold, the bone can heal in a deformed way or not at all, meaning great pain and further surgery.’
Robyn, with her parents, she has already become significantly taller following three operations
One frame runs from Robyn’s ankle to her knee and the other from her knee to the thigh. Each of the 26 pins, which protrude out of the skin, needs to be turned every few hours. Jane has to go to Robyn’s school several times a day to make the turns, using a spanner to adjust the difficult pins.
‘I have timers everywhere after Robyn’s had an operation,’ she says. ‘One for medicine to numb the pain, one for turning screws, and one for physiotherapy that has to be done four times a day. It’s awfully painful for her. She’s crawling away screaming and did ask last year, “Why me, Mummy”?’
In August this year the final frames will be removed and, after six weeks in plaster waiting for the bones to heal, Robyn’s wheelchair will be replaced by a special Zimmer frame to help her walk.
‘I don’t walk, I run and my walker zooms with me,’ says Robyn. Only when she rolls up her trousers to reveal the 78 scars gouged deep into her legs is there any sign of the years of excruciating pain she has endured.
Robyn does not mention the looming surgery, and when I ask how she feels about it she says: ‘It’s fine.’
The hope is that the final operation will stretch her bones another 2in, drawing Robyn to 3ft 11in. Add to that the amount her torso will grow as she ages, and it is likely her final adult height will be 4ft, or just over.
After that, the Fellows will face their next impossible decision – whether Robyn should have a one-off operation to lengthen her arms next year.
Dr Rocos says: ‘Arm-lengthening is considered more risky than surgery to the legs, and disabling the arms for a year is a greater ordeal.’
And even after that, Jane worries for the future.
‘People stare,’ she says. ‘Now, the boys in school think her frames are interesting, like a big Meccano set. But what about when she starts secondary school’
‘New children can be mean,’ says Robyn. ‘Some shout, “Why are you so small” But my friend Millie sticks up for me.’
Life may never be plain sailing for Robyn but she is a charming and undeniably brave little girl.