British woman has new lease on life after lung transplant

By Pat Jones yellowad.co.uk

IT IS hard to believe vivacious Clare Hemmington was sometimes too weak to leave her bedroom three years ago. 

A rare chronic lung condition left her needing a wheelchair and stair lift to get around the house – and her parents having to help her look after her baby daughter while her husband was at work. 

Clare, who lives in Hutton, Brentwood, was a victim of Lymphangioleiomyomatosis (LAM) - a rare disorder that affects women only, and destroys lung tissue, gradually causing lung function to deteriorate. 

The only real cure was a lung transplant – and Clare, who has first been diagnosed in 2005, finally had the life changing operation in February 2009, after 10 false calls. And what a difference that has made. 

“You wouldn’t recognize me now,” she said. “I live a normal life, visiting friends, walking round shops, going on holiday and looking after my husband and daughter.” 

Clare’s experience had made her determined to help other sufferers and a wholehearted supporter of LAM Action which funds research into the disease. 

“We have lots of fundraising events. The latest is the coast to coast walk from the Irish to the North Sea. We hope many people will support that venture,” she said. 

“Paul Roylance from Hockley is taking part so it has an Essex interest.” 

Clare first noticed symptoms of the condition –breathlessness- when she was 37. 

She became pregnant and put off having tests until after Lucy was born, but she continued to get worse until she was put on oxygen 24 hours a day, as eating and sleeping or difficult and showering and dressing took ages. 

Eventually the transplant became essential. 

The fundraising walk starts on August 13 and is expected to take a week. Anyone can support it with a donation by visiting
www.justgiving.com/walking-Coast-to-Coast-in-7-days

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New therapy found for rare lung disorder, lymphangioleiomyomatosis (LAM)

News Release University of Cincinnati Health Center

CINCINNATI—Researchers at the University of Cincinnati (UC) and Cincinnati Children's Hospital Medical Center have found that the FDA-approved drug sirolimus, used primarily to prevent rejection in organ transplant patients, stabilized lung function in women with lymphangioleiomyomatosis (LAM).

The Multicenter International LAM Efficacy of Sirolimus (MILES) trial was the first randomized, controlled study designed to develop a therapy for this life-threatening illness, which currently has no cure or treatment.

These results are being reported in the March 16, 2011, online edition of theNew England Journal of Medicine.

LAM is a progressive, cystic lung disease that occurs almost exclusively in women. In LAM, an unusual type of smooth muscle cell grows uncontrollably and spreads from an unknown source to restricted areas in the body, including the lungs, lymph nodes and vessels and kidneys, limiting the flow of air, blood and lymph.

Shortness of breath and recurrent lung collapse are common in patients with LAM, and until now, lung transplantation has been the only hope for patients who progress to respiratory failure.

"LAM affects about five people per million and occurs in 30 to 40 percent of women with tuberous sclerosis complex (TSC), a genetic disorder which also causes tumors to form in the kidneys, brain, heart and other organs," says Frank McCormack, MD, director of the pulmonary, critical care and sleep medicine division at UC and lead investigator on the study.

"Sirolimus, otherwise known as rapamycin, showed promise for patients with LAM in a pilot study conducted in Cincinnati and reported in the New England Journal of Medicine in 2008, but the relative risks and benefits of sirolimus for treatment of patients with this condition have remained unclear. In this international, multicenter study, we evaluated the safety and efficacy of one year of sirolimus in stabilizing and/or improving lung function in women with LAM."

McCormack says LAM cells isolated from lung lesions have TSC mutations and exhibit activation of a key sirolimus-sensitive growth pathway. When exposed to the drug in culture, the growth of LAM cells is stopped.

Experiments in TSC animal models have also demonstrated that sirolimus shrinks tumors in the liver and kidneys.

The MILES study was conducted within the National Institutes of Health (NIH)-supported Rare Lung Diseases Consortium, led by UC and Cincinnati Children's physician and pulmonary biologist Bruce Trapnell, MD, and co-directed by McCormack, and involved 13 institutions from around the world. Data was reported using Internet-based systems connected to a centralized, Internet-based data coordinating center headed by Jeffrey Krischer, MD, at the University of South Florida, Tampa.

"The trial included a screening visit and a year-long double blind, placebo-controlled treatment period, followed by a year of observation," says McCormack.

All eligible participants were female, aged 18 years or older and had a confirmed diagnosis of LAM with abnormal lung function.

Patients meeting the criteria were randomly assigned to either receive an initial dose of oral sirolimus at 2 milligrams per day or a matched placebo, and sirolimus levels were measured at each follow-up visit. Results were revealed only to an independent medical monitor who made dosing recommendations to maintain serum levels within a pre-specified target range.

A total of 89 patients with LAM were enrolled in the United States, Canada and Japan. Patients underwent baseline lung function testing, and lung function and exercise outcomes were measured over the course of six visits in the first year. Participants were also given questionnaires to determine how their symptoms changed throughout the course of the study.

Researchers found that sirolimus stabilized lung function and was associated with improvement in measures of functional performance and quality of life.

Sirolimus also reduced levels of serum vascular endothelial growth factor-D, or VEGF-D, a protein that is known to be elevated in LAM. VEGF-D promotes the growth of lymphatic vessels and can be involved in the spread of cancers.

"After discontinuation of sirolimus, lung function decline resumed and paralleled the placebo group," McCormack says. "Adverse events were more common with sirolimus, but the frequency of serious adverse events between the groups was not different."

McCormack says that these results suggest that sirolimus may be useful as therapy for moderately severe LAM-related lung disease.

"LAM is typically slowly progressive, and sirolimus therapy has risks, so treatment decisions should be individualized. Care must be taken in generalizing the results to those with milder or more severe lung disease due to LAM," he says. "Also, additional trials are needed to determine the optimal dose and duration of treatment with sirolimus. Given the toxicity profile of the drug during a one-year period, the long-term safety of this approach over an extended course must be carefully evaluated."

The trial involved efforts by the LAM Foundation that lobbied for the NIH's attention, organized and recruited patients and supported pivotal basic and clinical research that formed the scientific basis for the study.

###

This study was funded by the NIH Office of Rare Disease Research, the Food and Drug Administration, the LAM Foundation, the Japanese Ministry of Health, Labour and Welfare, the Canadian Institutes of Health Research, Cincinnati Children's, UC, the Tuberous Sclerosis Alliance and Vi and John Adler and the Adler Foundation.

Pfizer, maker of sirolimus, provided the drug and financial support but had no role in study design, conduct, analysis or reporting. McCormack cites no conflict of interest.

Regulatory oversight was provided by the National Center for Research Resources of the NIH. Participating centers included UC; Cincinnati Children's; the University of Toronto, Canada; the University of Texas Health Science Center at Tyler; the University of California at Los Angeles; Oregon Health and Science University, Portland; Niigata University Medical & Dental Hospital, Japan; National Hospital Organization Kinki-Chou Chest Medical Center, Osaka, Japan; the National Jewish Medical and Research Center, Denver; the National Heart, Lung, and Blood Institute, Bethesda; Medical University of South Carolina, Charleston; the Cleveland Clinic Foundation; and Brigham and Women's Hospital, Boston.

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Has your life been saved by an organ transplant? "Pay it forward" and help spread the word about the need for organ donation - In the U.S. another person is added to the national transplant waiting list every 11 minutes and 18 people die each day waiting for an organ or tissue transplant. Organs can save lives, corneas renew vision, and tissue may help to restore someone's ability to walk, run or move freely without pain. Life Begins with You

Woman whose lungs collapsed 15 times wins gold at transplant games

Justine is thrilled by her haul of medals from the European Heart and Lung Transplant Games which were held in Sweden
and will compete in British Transplant Games in August

By Daily Mail Reporter at daily mail online

A former fitness instructor who defied death after a one-in-a-million lung disease put her in a coma, has gone on to win gold at an international sports event.

Justine Laymond battled back from the brink after her lungs collapsed 15 TIMES.

She became a track and field star after receiving a life-saving double lung transplant.

Justine took home six medals at the European Heart and Lung Transplant Games last month.

She also competed in the badminton, squash, as well as sprinting, long jump and the relay. And now she plans to compete all over again this month.

She said: 'I used to be really into fitness and I worked in a gym. It was awful to know that my body was wasting away and there was nothing I could do.

'By July when I had my transplant I was close to death. I had nothing left to give and doctors were amazed that I had survived so long.

'The double lung transplant could not have come soon enough for me. But after the operation the difference I felt in myself was incredible. For the first time in several years I could breathe without effort.

'It took me a while to get back to peak fitness but now I feel on top of the world.

'I'm really proud of my achievements and especially my gold medal. I can't wait for the next contest.'

The feisty 37-year-old started to have problems with her lungs when she was 21 and suffered two lung collapses in her early twenties.

It took ten years and a further lung collapse before doctors diagnosed the incredibly rare condition Lymphangioleiomyomatosis (LAM) in 2005.

Soon after the diagnosis Justine took a turn for the worse. Within just eight months her left lung collapsed a further 13 times.

Eventually Justine was hospitalized for six months in February 2006 while she waited for a transplant.

Unable to eat, walk, drink or eat, her body was deteriorating fast. She was on oxygen 24 hours a day and was put into a coma for three weeks.

Finally, in July 2006, Justine received a new pair of lungs from a donor and has been rebuilding her life ever since.

Her journey back to health culminated in her bringing home gold for Britain in the European Heart and Lung Transplant Games which were held in Sweden.

Justine said: 'I know because of my illness I can't take life for granted. I've been on such a rollercoaster journey and I now I try and live every day to the fullest.

'I started to have certain symptoms when I was around 20 years old. But at the time I had no idea that there was anything seriously wrong.

'It wasn't until my left lung collapsed in April 2005 that I had a CT scan. I expected that something was seriously wrong, but nothing could have prepared me for what I was told.

I was told that I was in the 'end stage' of the disease Lymphangioleiomyomatosis, and my world just fell apart.

'I listened in disbelief as the doctors told me that my right lung had completely failed and my left lung only had about 30 per cent lung capacity.

'I was in an incredibly dark place and that time still haunts me to this day. I knew I had to stay alive though- I never gave up.'

Now Justine's future looks bright with a number of planned athletic events.

She said: 'I'll be competing in the British Transplant Games in August playing squash, as well as sprinting, badminton, long jump and the relay. I can't believe how far I've come.'

LAM is an extremely rare condition that affects around one person in a million.

The disease affects a certain type of muscle cell and only occurs in women. Just 60 people in the UK are known to have it.

The condition causes an overgrowth of a certain types of cell. This overgrowth occurs around the airways and also around the blood vessels and the lymph vessels, which usually drain excess fluids from the lungs.

The cells lead to cysts developing in the lung which can be detected by taking a CT scan of the lung.

For more information visit the LAM Action website

“You Have the Power to Save Lives – Register to be an organ and tissue donor & Tell Your Loved Ones of Your Decision”

Register to be a donor in Ontario or Download Donor Cards from Trillium Gift of Life Network. NEW for Ontario: recycleMe.org - Learn The Ins & Outs Of Organ And Tissue Donation. Register Today! For other Canadian provinces click here

In the United States, be sure to find out how to register in your state at ShareYourLife.org or Download Donor Cards from OrganDonor.Gov

In Great Britain, register at NHS Organ Donor Register

In Australia, register at Australian Organ Donor Register

Your generosity can save up to eight lives with heart, kidneys, liver, lungs, pancreas and small intestine transplants (see allotransplantation). One tissue donor can help 75 to 100 other people by donating skin, corneas, bone, tendon, ligaments and heart valves

Has your life been saved by an organ transplant? "Pay it forward" and help spread the word about the need for organ donation - In the U.S. another person is added to the national transplant waiting list every 11 minutes and 18 people die each day waiting for an organ or tissue transplant. Organs can save lives, corneas renew vision, and tissue may help to restore someone's ability to walk, run or move freely without pain. Life Begins with You

Lung transplant not a good match

I know many patients who have had several 'false alarms' after being called in for their lung transplant. It is a devastating experience after going through the exhilaration and excitement of receiving the call to rush to the hospital for a second chance at life. I've been through it and fortunately my donor lung was suitable for transplant. I know of one patient in England who had 11 false alarms before he finally got a suitable pair of lungs. Let's hope that Holly Bergo gets another call soon.

Holly, who is in her late 30's, has Lymphangioleiomyomatosis (LAM), a progressive disease that strikes women in the prime of their lives. It unleashes abnormal muscle cells on patients’ lung, where they form cysts and bore holes in healthy tissue. Read her story in my June 30th post.

June 9th - (WDAY TV) - Disappointing news tonight for Holly Bergo, she got the call today from the Mayo Clinic in Rochester.

A match had been found for a double-lung transplant, but tonight, friends of holly tell us it's not a good match.

Holly is battling a rare, incurable lung disease. Her only chance at survival is a transplant.

“You Have the Power to Save Lives – Sign Your Donor Card & Tell Your Loved Ones of Your Decision”

Register to be a donor in Ontario or Download Donor Cards from Trillium Gift of Life Network. NEW for Ontario: recycleMe.org - Learn The Ins & Outs Of Organ And Tissue Donation. Register Today! For other Canadian provinces click here

In the United States, be sure to find out how to register in your state at ShareYourLife.org or Download Donor Cards from OrganDonor.Gov

In Great Britain, register at NHS Organ Donor Register

In Australia, register at Australian Organ Donor Register

Your generosity can save up to eight lives with heart, kidneys, liver, lungs, pancreas and small intestine transplants. One tissue donor can help up to 100 other people by donating skin, corneas, bone, tendon, ligaments and heart valves

Has your life been saved by an organ transplant? "Pay it forward" and help spread the word about the need for organ donation

A life interrupted: Moorhead, North Dakota nurse waits for double lung transplant

I have a very good friend who will be celebrating the 7 year anniversary of her double-lung transplant this summer. She had the same diseases as Holly Bergo who is profiled here. Holly has Lymphangioleiomyomatosis (LAM), a progressive disease that strikes women in the prime of their lives. It unleashes abnormal muscle cells on patients’ lung, where they form cysts and bore holes in healthy tissue. My friend's life has been transformed by her transplant and she is now full of life with boundless energy. Hopefully Holly Bergo will experience the same "gift of life" when she receives her transplant. You can help with her medical and living expenses by clicking on the links below. Merv.

By Mila Koumpilova INFORUM

Holly Bergo’s new pulmonologist had one word for her after poring over her CT scan: impressive.

He had never seen lungs so ravaged by illness, with an owner who had clung so gamely to her lifestyle.

That was in 2007, and for more than a year, Bergo kept going: She remained the roving public health nurse known among clients as “Jolly Holly” and the former cheerleader her many friends deemed a “spiritual picker-upper.”

But over the past year, a rare, incurable lung disease has slowly dismantled that life. These days, Bergo is mostly confined to her Moorhead apartment, with only two tasks: Wait for a phone call from the Mayo Clinic summoning her for a double lung transplant surgery and keep breathing.

Well, there’s also comforting the occasional teary friend and joking about meeting a cute doctor at Mayo.

“Holly hasn’t lost her humility and her grace and her humor and her beautiful, outgoing personality,” said Michelle Eldredge, who along with other friends is organizing a July benefit for Bergo.

Not breathing easy

For years, Bergo thought she had asthma. She was misdiagnosed after shortness of breath started hounding her constantly. The meds seemed to help.

Shortly after, she went to get a second bachelor’s in nursing at North Dakota State University, where she’d once been a Bison football cheerleader. When she graduated, she got a job at Cass County Public Health. Climbing the two flights of stairs to her office became an increasingly breath-robbing ordeal.

She kept telling herself she needed to get in better shape. But the breaks she had to take after the first flight of stairs grew longer.

In spring 2007, her doctor ordered a CT scan and, concerned, sent her to a MeritCare pulmonologist. He examined her CT scan – her lungs pocked by cysts like honeycomb, down to 50 percent of their function – and sprung to action. He lined up oxygen tanks and an appointment at Mayo.

Bergo had lymphangioleio-myomatosis, a progressive disease that strikes women in the prime of their lives. It unleashes abnormal muscle cells on patients’ lung, where they form cysts and bore holes in healthy tissue.

The pulmonologist told her there’s no cure, and Bergo’s illness was too far along to enroll in a trial for promising new medication. She would likely need a lung transplant.

“Here’s me, 36, single and looking, wanting to have children, barely two years into my dream job and thinking my asthma is getting worse,” she recalled.

Bergo spent that Friday crying. She was one of only 1,500 women diagnosed with the disease. Experts believe as many as 250,000 go undiagnosed or, like Bergo, misdiagnosed, blithely planning out the rest of their lives.

“That first day was devastating,” she said. “That weekend I was in shock. On Monday, I decided I had to fight.”

Holly’s wait

Bergo left her oxygen tank in her car before she visited the apartments of clients, some of whom used oxygen themselves. She hid her illness. She didn’t want it to take the fun out of Jolly Holly.

She joked about her ordeal to friends. Nadine Perkins, Bergo’s nurse coordinator at Mayo, remembers her laughing and snapping pictures of staff when she came in for her transplant evaluation.

“At first I was like, ‘Why me?’ ” Bergo says. “Then I decided, ‘Why not me?’ There’s a reason God is making me go through this.”

But the disease was on the march. Bergo spent Christmas and Valentine’s Day after her diagnosis in the hospital with a collapsed lung. That spring, when she could no longer part with her oxygen for long, she had to come clean to her clients.

In the summer, her parents, Bruce and Roxy, moved to the area to help care for her. In the fall, as taking a shower became a strenuous exertion, she had to concede she couldn’t continue on her job.

“Pieces of her life were slowly being taken away,” Eldredge said. “Breathing is her job right now.”

As Bergo climbed the lung transplant list at Mayo, she retreated into her apartment. With a wheelchair and a change of oxygen tanks needed, she said, “It takes a village to bring me out.”

She keeps her cell phone close by her side. She can get the call from Mayo any day now. She has a bag packed and an arrangement to fly to Rochester, Minn., where she needs to get within hours of receiving the call.

There’s no telling how long the wait is going to be: She needs a donor who shares her petite frame and her rare B positive blood type. Her heart is already breaking for the family of the donor who’ll give her a chance to reclaim her life.

Her lung function is down to 16 percent.

She fears she might not survive the five-hour surgery or that her body will reject her new lungs. She worries that eventually her disease might attack her new lungs. But most of the time, she’s strangely calm. All she has to do is breathe.

In the meantime, her friends have rallied around her. They’ve cut her hair for free, dispatched a massage therapist to her apartment and planned a July 31 dinner and auction at the Fargo Holiday Inn.

“It’s amazing how many people she’s touched,” says Bergo’s friend and former colleague Karen Irey, who called her “a good spiritual picker-upper.” “It’s all coming back to her now in full force.”

Stephen Cassivi, Mayo’s director of lung transplantation, says if a set of lungs becomes available on time, a patient like Bergo can make a real comeback, starting with the most basic activity: “Breathing is one of those primordial acts of life we take for granted, but you really notice it when you have to work at it.”

Bergo looks forward to taking a shower without having to sit down several times, keeping up with her nephews and getting her job back: “I will reinvent myself and be better and pay it forward.”

How to help

To help with Holly Bergo’s medical and living expenses, visit http://www.transplants.org, click on “Patients we help” and type her name in the search box.

“You Have the Power to Save Lives – Sign Your Donor Card & Tell Your Loved Ones of Your Decision”

Register to be a donor in Ontario or Download Donor Cards from Trillium Gift of Life Network NEW for Ontario: recycleMe.org Learn The Ins & Outs Of Organ And Tissue Donation. Register Today! For other Canadian provinces click here

In the United States, be sure to find out how to register in your state at ShareYourLife.org or Download Donor Cards from OrganDonor.Gov

In Great Britain, register at NHS Organ Donor Register

In Australia, register at Australian Organ Donor Register

Your generosity can save up to eight lives with heart, kidneys, liver, lungs, pancreas and small intestine transplants. One tissue donor can help up to 100 other people by donating skin, corneas, bone, tendon, ligaments and heart valves

Has your life been saved by an organ transplant? "Pay it forward" and help spread the word about the need for organ donation