Young Kyle’s condition, CD40 ligand deficiency, is found in only one in a million male births. It makes him highly susceptible to infection, and his lungs and liver have already been damaged. But Great Ormond Street now offers the hope of a cure, in the form of a bone-marrow transplant. A matching volunteer donor has been found and hopes are high, but there is no getting away from the fact that this will be a risky procedure. “We’ve given him a 50% chance of success, which is not good,” admits consultant Paul Veys.

Before the transplant, Kyle must undergo chemotherapy to prepare his body. A new therapy using two relatively gentle drugs will mean chemo can be kept to a minimum, but Kyle’s immune defences will be weakened to prevent the rejection of the new cells. This means he will be extravulnerable to potentially life-threatening infections. “Even though you know it needs to be done, you can’t say you’re ever ready for it,” says distraught mum Gloria as she sees Kyle in pain and throwing up.

After 24 hours, Kyle is moved to a specialist isolation ward where he and his mother will live for a month, keeping a video diary to chart their feelings and experiences. Gloria says that she and her husband are always very honest with Kyle about what his treatment will entail. “We couldn’t put him through all this without him knowing what was involved,” she insists.

Finally, it’s the big day when the bone marrow is introduced into Kyle’s blood via a drip. “It’s a very big fight for me,” Kyle realises. He is right, since this stage of the treatment brings new risks: the donor cells could fight Kyle’s own cells and attack his weak liver and lungs It will be two weeks before tests reveal whether the new cells have taken. Meanwhile, there is the constant risk of infections, so Kyle is confined to the isolation ward.

Despite the family’s optimism, the results of the lab tests are disappointing. “We are not seeing any donor cells at the moment,” admits Paul Veys. It’s left to a tearful Gloria to tell Kyle that they are back to square one. She is wary of giving the gruelling procedure another go, so all that remains at the moment is for her and her husband to help their spirited son live life to the full.

Tonight’s programme also follows 17-year-old Leanne, who suffers from pulmonary hypertension. This rare condition affects her lungs and puts pressure on her heart. Left untreated, it would probably have killed her within three years. Now, she is fighting a daily battle to stay alive.

Up until the age of 13, Leanne was the picture of health. “I was such an active, super-fit person,” she says. “To wake up one morning not being able to breathe or climb the stairs… Wow, it was a shock.”

There is no cure for Leanne’s condition, although a range of treatments help her manage it. She may need a lung transplant in the future, but this is a high-risk operation. For now, she follows a strict regime of tablets. In addition, the medication needed to open up the blood vessels in her lungs must be administered to her system directly throughout the day. A small pump pushes the medication through a tube that enters Leanne’s body via a needle stuck into her stomach tissue. Every ten days, Leanne must reposition the needle to keep the drug diffused throughout her body – a painful procedure.

But administering drugs this way seems to be getting less effective for Leanne, and her health is declining. Luckily, another visit to Great Ormond Street throws up a whole new procedure that promises to improve the quality of Leanne’s life…

Channel Five, Wednesday 7th March, 8PM

View the original post Child In A Million: Stories Of Kyle And Leanne on Unreality TV

London’s Great Ormond Street. Filmed over eight months, the series follows 12 children and their families at home and in the hospital, as doctors diagnose and treat their conditions using cutting edge techniques. In this programme, we follow six year-old Alex, who has a bone disease so rare only 200 cases have ever been recorded, and 11 yearold Matthew, who has flown in from New York for life-saving heart surgery.

Doctor Penelope Brock has been treating Alex since his diagnosis and referral to the hospital. He has Gorham Stout syndrome, or ‘vanishing bone disease’ – an inflammation inside the bones which causes the destruction of normal bone tissue. He has already undergone surgery to remove his lower jaw, and has been given a tracheostomy to ease his breathing. The next steps, however, are less clear.

In her 25 years as a paediatrician, Dr Brock has never seen the condition before, and she is forced to return to her medical books in an effort to find the best course of treatment – there is no conventional cure. Comparing the condition to some forms of cancer, she decides that a combination of steroids and chemotherapy will give Alex the best chance of survival. “He’s a guinea pig really,” says his mother, Tammy Stoakes. “If he’s going to be my little miracle, then he’s in the right place.”

Dr Brock remains optimistic but highlights the desperation of Alex’s case. “We had to do something to stop the progression of the disease or it would become life-threatening to him,” she explains. Alex already endures a punishing daily routine involving regular dressing-changes and vast quantities of medicines, but he is now admitted to hospital for chemotherapy. On his arrival, however, there is a worrying discovery. A large piece of bone has become exposed inside his mouth, considerably raising the chances of infection.

Despite the risk, craniofacial consultant David Dunway decides that the treatment must continue. After six weeks of intensive treatment, Dr Brock decides to reduce Alex’s medication – but he doesn’t react well. His jaw begins to swell and he is readmitted. Alex’s family then awaits the results of a new MRI scan, which will reveal if the disease is back under control. The results are mixed: the disease is still active, but Dr Brock’s medication appears to be keeping it at bay.

Another child requiring the expert attention of Great Ormond Street’s doctors is 11 year-old Matthew, born without a pulmonary valve and now requiring urgent surgery to replace it. His parents have re-mortgaged their house to fly with him from New York, where the only treatment available is a painful operation with a lengthy period of recovery. Time is running out for Matthew, whose father explains why the failing valve is so dangerous. “If we don’t do something now,” he says, “the heart could deteriorate to the point where blood would flow back up into the lungs.”

At Great Ormond Street, however, an amazing technique has been developed that will spare Matthew any painful surgery. Paediatric cardiologist Professor Philipp Bonhoeffer has designed a revolutionary method of replacing the pulmonary valve through the patient’s leg. Using a stent and a small balloon, the valve is inserted into a vein in Matthew’s thigh and guided up to the heart during the operation. A few months later, Matthew is back home in New York playing baseball with his older brother.

Alex, meanwhile, is doing well on a reduced level of medication. He has had his hated tracheostomy removed, and the doctors of Great Ormond Street have given his family hope.

Tonight Channel Five At 8PM

View the original post Child In A Million: Story Of Alex And Matthew on Unreality TV

Filmed over eight months, the series follows 12 children and their families at home and in the hospital, as doctors diagnose and treat their conditions using cutting edge techniques.

This opening programme features Molly who suffers from a rare form of cancer, and Tahlia, whose windpipe is so narrow she can barely breathe.

Four-year-old Molly has a form of kidney cancer called Wilms’ tumour. The condition is normally found in younger children and is relatively easy to treat, but the cancer has gone unnoticed in Molly for so long that it has spread to her chest. Having already been through months of chemotherapy and had one kidney removed, Molly now has three life-threatening tumours which surgeons must remove.

But none of this seems to daunt Molly who remains happy and talkative at all times. “As ridiculous as it sounds,� says Rob, Molly’s dad, “you get by through the strength of your child.�

Molly’s consultant at Great Ormond Street is Gill Levitt, a specialist in Wilms’ tumour who has worked at the hospital for some 20 years. She explains that the tumours in Molly’s chest may still be active, but surgery to remove them would be life-threatening. Due to the proximity of one of the tumours to the heart, in fact, surgery may not even be possible. Levitt hopes that a new scanning technique – only recently being tested on children – will reveal if the tumours are active, without the need for invasive surgery.

Two days after Molly’s scan, it seems that Levitt’s technique has worked, but the news is not good. The cancer in Molly’s chest is still active, despite the chemotherapy, so she is booked into surgery with Professor Martin Elliott, one of the hospital’s top surgeons. Elliott and his team plan two operations to remove the tumours, but they are well aware of the risks involved. Because one of the tumours is so close to Molly’s heart, it may be too risky to remove it. The only way to find out for sure, however, is to go ahead with the operation. “It remains one of those rare bits of surgery in the modern era,� Elliott explains, “where you can only find out by doing it – which is not an ideal situation.�

As the surgeons prepare for the first of the operations, it is a very stressful time for Molly and her parents. Rob and Emma, Molly’s mum, try to remain strong for their daughter, knowing all the time that her life hangs in the balance. Dr Levitt remains positive, but admits that if the surgery fails, “we’re in serious trouble.�

Great Ormond Street does not have an accident and emergency department, so when a child arrives by ambulance, it can only be a very serious case. One-year-old Tahlia has been rushed to the hospital with breathing problems, and is taken immediately into the critical care unit while her parents look on.

It is only now that Alison and Paul discover that their daughter suffers from longsegment tracheal stenosis – a condition that means her windpipe is very narrow in places. The disorder, which can be present at birth or be acquired through trauma or infection, is so rare that only one in five million people suffer from it.

Many children in the past have died from Tahlia’s condition, but Professor Elliott and his team changed all that with a revolutionary new surgical technique that shortens and widens the trachea.

Tahlia’s situation, however, proves a challenge even for Elliott’s team. As she is rushed into theatre, the surgeons discover that Tahlia’s windpipe is less than a third of the size of that of a normal child. With a tube just 1.8 millimetres thick through which to breathe, Elliott reflects, “it’s incredible this child is alive.�

Sadly, after successful surgery to remove Molly’s tumours, the cancer returned and she died at the end of 2006. Her parents have asked for the programme to be shown as a tribute to her life.

This episode of Child In A Million will air on Channel Five on the 21st February from 8-9pm

View the original post Child In A Million: Molly and Tahlia’s Story on Unreality TV