New Zealand Organisation for Rare Disorders
PO Box 38-538,
Wellington Mail Centre
Phone: +64 4 471 2226
Charities Commission Registration CC22512
Saving Lisa - One woman's fight against LAM
The New Zealand Herald
By CARROLL du CHATEAU
Even on the blurry video screen, Bronwyn Gray could see her daughter moving through the arrival hall at Auckland International Airport unusually slowly. Leaning slightly on the trolley with its stack of suitcases, yellow duty-free bags and bottles of French merlot, the young woman with the bouncy chestnut curls and wide smile was inching forward.
"She looked really little," Gray recalls. "She'd just arrived back from her two years in London, flown home through the US to see her AFS friends, and she looked really, really tired." Extreme tiredness was part of the picture for Lisa Gray. Just short of her 29th birthday, the accomplished family lawyer had suffered exercise-induced asthma for several years. Sometimes she woke so short of breath she almost choked. A couple of coughing fits had ended in blood-sprayed tissues. This time, her mother sensed Lisa was worse. In the early morning light her skin looked papery. "I suggested she went straight to see her GP, Dr Christine Forster, in Freemans Bay."
Two weeks later, in February 1997, after a course of antibiotics, Lisa was still coughing, still tired. For the second time in three years, Forster sent her to Green Lane Hospital's respiratory department. This time the bronchoscopy was followed by a CT scan. "I got a phone call from the doctor at Green Lane asking us to come and see him," says 56-year-old Gray. "It was a Wednesday morning. Lisa was at work." The doctor said: "All I can tell you is that it's not cancer." The day stretched out like a bad dream. Gray didn't call her daughter at work, but when Lisa arrived home she was waiting. "We have to go to see the doctor," she said. "Then we went and met this man, this lung specialist, in his office. And he gave us this radiology report."
Gray hands me a sheet of waxy, old-fashioned fax paper, filled with the words that dissolved before her eyes that evening. Underneath a chunk of technical analysis is a final sentence: "The appearances are typical of lymphangioleiomyomatosis." "We said, 'What does this mean?"' The doctor leaned over and underlined the word that was to haunt Bronwyn Gray forever. "You have lymphangioleiomyomatosis." (It's pronounced lim-fangio-lymyo-matosis and known by the abbreviation LAM.) "We hardly know anything about it. What I can tell you is that it's unlikely you'll grow up to be an old lady." Mother and daughter sat there, stunned. "Are you absolutely sure?" they asked. "Yes, I'm 101 per cent sure that you have lymphangioleiomyomatosis."
Bronwyn Gray is not the kind of woman to take a death sentence lying down, certainly not when her beloved daughter is involved. "We walked down a long, bleak corridor, holding each other's hands. Every noise was magnified - the sound of my shoes on the lino sounded like crunching ice. It was a nasty night, cold and miserable. It wasn't until we got into the car that we burst into tears." At first, Gray was "paralysed with grief and the enormity of this thing". Then she moved into action.
"The terrible thing was that we didn't know anything about the disease. We'd thought Lisa had asthma. I needed to know." First call was to her brother-in-law, John Gianoutsos, who put her on to his Sydney-based brother, Peter, a respiratory physician . When Gray told him Lisa's diagnosis there was a long, long silence. Then: "Oh, Bronwyn, I'm so, so sorry." The next day, information started snaking from the fax machine. LAM is rare, affecting one person in a million. (Gray now disputes that figure. "I know it's at least four to five times that, 13 women at least in New Zealand.")
It is not familial. There is no cure, no treatment. It attacks only women, normally in their 20s and 30s, covering the inside of their lungs with hundreds of water-filled "blobs" or polyps that sometimes break, filling the lungs with water. This is accompanied by fibrosis in the lobes of the lungs. The resulting hardening and loss of elasticity gradually stops these young women from taking up oxygen. The disease is progressive. In some women it moves slowly, in others much faster, and it can go into overdrive at any second. In everyone it inexorably leads to progressive destruction of the lungs. The only possible hope is a lung transplant.
"I didn't let her read it," says Gray, even now blinking back the tears. "The first paragraph he sent me started, 'Almost all women die within 10 years of diagnosis."' Then there was a PS: "You might try the LAM Foundation in Cincinatti, Ohio."
March 1997, one week after initial diagnosis: "Anna from across the road" was insisting that the person Lisa needed to see was Auckland associate professor of respiratory medicine, John Kolbe. "Anna called him, and this brilliant respiratory physician with a three-month waiting list, saw her within 24 hours. We found out then that he had some experience with this condition," remembers Gray with a tremble of excitement. "He told us this disease had no rules. It was fast in some people, slow in others. He also said he'd like to be benignly interested. He didn't want to do anything except lung function tests. And he was the best that we could hope for ... "
At least Gianoutsos' postscript gave Gray something to do. A former English and history teacher at Auckland Girls' Grammar under Charmaine Poutney, she had excellent research and people skills. But even she was stymied by this one. How to start? "We didn't even own a computer. So Lisa wrote a lovely letter to the LAM Foundation's street address. Could they help with any information?" Ten days later, a fat A4 envelope arrived from the US. Sue Byrnes had formed the US LAM Foundation in 1995, two years after her own daughter was diagnosed. She was hugely hopeful that one day the research that scientists were doing into the disease might be helpful. Her letter ended: "We had to accept that Andrea had this disease - but we could not accept that we could do nothing about it." Neither could Gray.
"I couldn't sit round bawling my eyes out because my daughter had been given a death sentence," she says. "I telephoned Sue: 'Please send me anything relevant. How did you get this information? How did you get in contact with the doctors and researchers? What can I do?"' While Sue Byrnes in middle America had started her search with her family minister, Gray began with her lawyer, the equally formidable Sandi Anderson of Ponsonby feminist fame. By then Gray's aim was to set up a non-profit trust from which she could help to fund local research.
"Then I rang Helen Clark and asked if she'd be prepared to be our patron." At the time, Clark was leader of the Opposition. Gray had worked on her various campaigns and knew her personally and Clark had met Lisa as a child. Having suffered asthma as a teenager, Clark also understood the terror of difficult breathing. She said yes. Gray was on a roll. Her chutzpah makes me gulp. When Professor Lord Winston, one of the world's most famous geneticists, was in town for the Knowledge Wave conference, she bowled right up to him: "You're a Jewish father, I'm a Jewish mother. Now, can you help us?" He wished her well.
The trust was beginning to take shape. "It took me two minutes to put together a list and then eight phone calls in about eight minutes to organise a board."
As so often happens, it all came down to contacts. First call went to John Gianoutsos in Wellington, who Gray had called the night Lisa was diagnosed. Then there was her cousin, Wally Hirsh, the former Race Relations Conciliator. Both agreed without hesitation.
Next on Gray's list was Jennifer Shieff, an old friend working as a policy analyst with the Ministry of Health; Adina Halpern, a legal specialist in health and partner at Buddle Finlay; Carol Hirschfeld of TV3; Forster, the GP who had always known that Lisa's illness was more serious than asthma; Dr Hetty Rodenburg a close friend and GP who specialised in the management of people with life-threatening diseases; Dinah Morrison Bradley, Lisa's respiratory physio; and Janet Barrett, a registered nurse.
Gray finishes rattling off her list with its preponderance of Jewish names. "There are three GPs, an OBE, a few doctors and nurses, but they're all mates, all friends I've had strong connections with over the years." She needed all the names to put to "the men in grey cardies in the Henderson IRD office so I could have charitable trust status". On March 29, 1999, two years after Lisa's initial diagnosis, the LAM Charitable Trust came into being.
From Cincinatti, Byrnes was agitating for Gray to "get yourself a science and medical advisory group". Again, Gray picked up the phone. Again, she was astounded when people such as Kolbe said they would be delighted to help.
The next name forward was Professor Ingrid Winship, clinical geneticist at Auckland University, who had experience in tuberous sclerosis, sometimes associated with LAM.
"I sort of forced her to come and have coffee with me," Gray says with refreshing self-awareness. "It has to be done. I'm relentlessly positive and factual. People can't say no."
And Winship didn't. Neither did Associate Professor Innes Asher, a paediatric respiratory specialist at Starship, or Associate Professor Mervyn Merrilees, the head of anatomy and the foremost researcher into heart smooth muscle proliferation at Auckland University.
By this time, even Gray was surprised by the reaction to her request. "When I asked them there wasn't a single split-second of hesitation."
Over the months, things fell into place. Gray's pamphlet had been donated by a printer friend. When Gray decided to call a meeting of the science advisory team she was offered the medical school meeting room. And when the "wonderful" Merrilees turned up and Gray had to address the meeting, she didn't collapse in fright. It was November 13, 2000. Gray knew she had only one hour. Her question to the group: "Is it feasible that we can do something in New Zealand so that we can be part of international research? Do you think it's possible?"
As they sat there thinking it over, she added a rider: "And if it's a matter of money, I'll get that money." An hour after she arrived home there was an email from John Kolbe. "I think Merv Merrilees was really interested. He said he's never heard of this [disease]."
Summer, 1999: After three consecutive games at a touch rugby tournament on Waiheke, the young woman feels a strange "roly-poly" feeling in her chest. Her doctor suggests an x-ray, which is carried out next morning at Auckland Hospital. "It showed a minor lung collapse," she says. "Only minor, but it brought it home to me that I have to be sensible." And what does sensible mean? "That I don't go scuba diving or bungy jumping." Within months, Merrilees and Gray were en route to Cincinatti and the International Conference on LAM.
As Gray had promised, she had found the money. Merrilees' fare came courtesy of the Asthma and Respiratory Foundation in Wellington, Gray's via a typically more creative route.
"Around that time the New Zealand Herald ran a story about a New Zealander born in the backblocks named Jilly Evans, who worked as a research scientist in Philadelphia for Merck Sharp and Dohme" she says. "She sounded a brilliant woman." Gray sent her a fax (the hardest letter she had ever written) explaining her daughter's predicament. At 5.45 next morning she was rewarded by a buzz on the fax machine. Like so many people, Evans had never heard of the disease but she got on to the American website and contacted Dr Lisa Henske, who had found the gene that connected LAM with tubular sclerosis.
With Evans excited by the the setting up of the New Zealand group, prospects took a leap. Merck New Zealand offered, no strings attached, to provide Gray's airfare, accommodation and fees to attend the US conference - not just in 2000 but for the next two years as well.
As she says, "Because of Jilly, because of Merck, I - who had given up my job and was working full-time on LAM matters - could get there." With her medical and research team established, Gray started to raise funds. Her system was the same as before - use friends and connections.
First idea was a paid dinner party project. "We had five dinners over five Saturday nights and charged a minimum of $50." A few months later, a close friend of Gray's, London-based New Zealand chef Peter Gordon, had a better idea. "He rang me to say he was going to be in New Zealand and what could he do to help? How about I cook a special dinner for 100 people?"
By February last year, Gray, who had previously run Pabulum restaurant in Ponsonby with her partner, Lesley, had everything organised. Point 59 restaurant in Pt Chevalier closed for the night and gave the space and kitchen free. Others supplied the food and wine. And the 100 guests happily paid up. "There's $50,000." Another $10,000 was donated by the Deane Endowment Trust (Roderick and Gillian Deane's daughter died of a rare genetic disease) to be paid in quarterly instalments. That money went directly into the LAM trust account.
Taking Merrilees to the Cincinatti conference had been a well-calculated move. Dr Frank McCormack, scientific director of the American LAM Foundation led a team that found a link between the proliferation of smooth muscle cells in LAM and some patients with tuberous sclerosis. He says that breakthrough research would probably not have been made for decades without the foundation. Merrilees, with his interest in any links between the smooth cell growth that characterised LAM-attacked lungs and his research into smooth muscle proliferation in heart disease, was "bowled over" by the way US scientists worked in tandem.
Fuelled by his new professional connections with US researchers, he and Kolbe set up a joint primary investigation at Auckland Medical School. Seven months later, masters science student Elyshia Hankin began working full-time on a project to assess possible links between hormonal changes in the progression of LAM (which, remember, occurs only in women of child-bearing age) and also on triggers that start the disease in certain individuals.
February this year, five years post-diagnosis: regular tests at Green Lane hospital show that although Lisa's lung function is decreasing, the progression of her disease is much slower than for some other women. She has acupuncture twice a week and practises yoga. Recently promoted to senior solicitor, her job with Davenports West Family Law team is going well. "Mum, I'd be so healthy if I didn't have these lungs."
The day I speak to her, Gray and Merrilees have just returned from their second LAM conference in Cincinnati. This time, while friendships and research relationships have been strengthened, Gray is sobered. This disease is a killer. Some patients were much sicker than Lisa. A video shows most of them with oxygen tubes up their noses. One, who had been waiting for a lung transplant, had died. Another, with a set of transplanted lungs, was almost unrecognisable as the anti-rejection drugs bloated her once-pretty face. All of which makes Bronwyn Gray that much more determined to keep up the pressure.
This weekend this indomitable woman stages another near-miraculous breakthrough, in the shape of the first New Zealand-Australia scientific Symposium on LAM. The list of 32 delegates gathering on Waiheke Island includes Australasia's top respiratory scientists, physicians, lung transplant experts and physiotherapists. This is no ordinary accomplishment. "The first-ever LAM conference, held at Columbia University in New York four years ago, had just this many people," says Gray. "Now they have 150 clinicians and doctors investigating and have raised US$3 million ($6.7 million) to fund research. We have raised just under $100,000."
Not a cent of the research money is being used on this conference. Once again, Gray has begged, borrowed and bulldozed people into offering everything free. Her partner Lesley is cooking the food for the 30 delegates. Friend Molly Schwarz is providing her stylish new residential food school, the Longhouse. Wine is from Goldwater wines, veges from Bhana Brothers of Ponsonby Rd, meat by Brian the Butcher of Superior Meats of Ponsonby, with help from New Zealand Beef and Lamb. Science will be by people such as Frank McCormack of LAM America. "I think we can now talk about a cure for LAM - this could happen in our lifetime," he says.
April this year: The young woman with the flyaway chestnut hair and amazing green eyes looks across the table at her mother who is, once again, glued to a cellphone.
"I think what mum's done has been phenomenal," she says. "I've gone on with living while she's done all the work on the trust. She's so astounding, what's she's doing is incredible. It's because she's put so much into it that the trust has come as far as it has ... "Even with a disease like LAM you can't just curl up and do nothing."
As I leave the house in Freemans Bay, Gray throws a handful of jigsaw pieces across the floor of her study. "This is part of the jigsaw they're doing in the States," she says. "There are no edges, there's no pattern or picture. There's not even a box. That's what we're dealing with in this fight against LAM." She smiles. "But you know, I really do believe that we can win this battle. That the research can be done - that we really will find a cure."
All I hope is that it is in time for her daughter.
A web video series about LAM, produced specifically for GPs can be viewed here.
This article shows the search for a cure continues.