Being like other teenagers means a lot to Leanne Pannell, but being ordinary is an extraordinary achievement for Leanne. Seventeen year old Leanne has pulmonary hypertension, a condition that affects her lungs and puts pressure on her heart. If the disease had been left untreated it would most likely have killed her within three years of it's onset when she was thirteen. Now it's a daily battle to stay alive.
Leanne's treatment is under the supervision of Great Ormond Street's Professor Sheila Haworth "What happens to these patients is that the very small blood vessels in the lungs, hundreds of thousands of them, instead of being thin-walled and delicate become think-walled until many of them become largely or completely obstructed. Then the heart has to work harder".
Leanne offers her interpretation "After a while, the heart gets tired and becomes bigger and more muscular. The end result is heart failure, if you're not kept on a suitable treatment".
Pulmonary Hypertension can strike at any time without warning. Until she was thirteen, Leanne had been a picture of perfect health and fitness. She was very active and a great sportswoman.
One morning she awoke with heart palpitations. She had difficulty breathing and was changing colour from purple to blue. Leanne was rushed to hospital, but no-one knew what the problem was. Pulmonary Hypertension is very rare, just two people per million, so it's not always recognised straightaway.
After several months, the doctors taking care of her took a lung biopsy and sent it to Great Ormond Street. Professor Haworth recalls "I knew exactly what was going on on th lung, both from the physiology at the catheter and the structure in the biopsy". She confirmed it to be Pulmonary Hypertension for which there is no cure but there are many treatments available.
Leanne is now an outpatient of Great Ormond Street Hospital. She visits the hospital every three months, but most of her treatment takes place at home. Ultimately, Leanne may need a lung transplant, but that's a high risk procedure followed by a limited life-expectancy.
For now, drugs are containing the disease. Leanne's on a strict regime of tablets. The medication to open up th blood vessels needs to be administered directly to her system throughout the day.
Leanne demonstrates how it works "This is my pump and the syringe. The pump will gradually, over the course of five days, will push the syringe down and push the drug through the tube to a needle just under the skin".
The alternative is a Hickman Line, a semi-permanent catheter going in to the chest. It's pain-free but it's more conspicuous.
Leanne's choice comes at a price. Changing the subcutaneous line she is using is very painful. Leanne's needle has to be moved to a new area of stomach tissue every ten days to keep the drug diffusing efficiently. It's not just inserting the needle that's painful. Each time the site is changed, Leanne's body takes days to recover. The morning following the site change, sh is in a lot of pain. She knows that without the treatment she would probably be in a wheelchair or in need of a transplant.
Leanne's health is gradually declining She comes to Great Ormond Street to see Professor Haworth to try out a new treatment. The treatment she is using now is not working as well as it was.
Professor Haworth explains the change to her "You've had this infusion for so long that a lot of the subcutaneous tissue is a bit thick, that's why sometimes the response is unpredictable".
Her new plan is for Leanne to use a nebuliser to deliver the drugs to her system using a fine mist. It's essential that Leanne gets the technique right. Specialist clinical nurse Yvette Flynn shows her how.
Back home in Devon, Yvette Flynn pays a visit to see if Leanne's nebuliser is working. She is happy with Leanne's progress, but suggests that taking a little more time with the nebuliser will improve the effect.
Some weeks later Leanne attends a hospital in Bristol for a series of tests. For long-term outpatients, like Leanne, the Great Ormond Street team will travel to the nearest suitable hospital. Yvette Flynn wants improvements in Leanne's nebuliser technique. Using a computer model, she trains Leanne how to get the best from her nebuliser.
Leanne is coming up for her eighteenth birthday and has almost outgrown Great Ormond Street's care. So after five years it's time for Professor Haworth to say farewell. Another life threatening disease that affect the lungs is called mesothelioma lung cancer.
CREDITS: All of this information came from the UK Channel 5 "Child in a Million" documentary series