By STEVE EIGHINGER Herald-Whig Staff Writer
Rachel Phillips hopes to some day be able to return to Quincy, if for no other reason than to thank all the people here who continue to hold her up in prayer.
But first things first.
Phillips, 34, a former ballerina with the world-renown Royal Ballet of London and wife of Steven Phillips, a native Quincyan, is fighting for her life with every breath she takes.
Rachel and Steven, who moved from Quincy to Burlington, Vt., about nine years ago, are attracting nationwide attention. Rachel's medical condition has drawn interest from ABC News, PBS and Reuters. Add to that numerous articles in a wide variety of other publications.
Rachel is hoping she will soon be the 12th person in the world to undergo experimental surgery that will replace her trachea, or "windpipe," which now collapses at a 90 percent rate every time she exhales.
"I'm not going to lie," she said. "I try not to think about it. I am doing everything I can to stay alive ... it is what it is."
There is only one doctor in the world who can perform the surgery, and he has agreed to attempt the 20-hour procedure. Italian surgeon Paolo Macchiarini, a professor of regenerative surgery at Karolinska Institute in Stockholm, Sweden, has pioneered this type of regenerative surgery through the use of stem cells extracted from the patient's own body. The stem cells used in these procedures are not the embryonic kind that have aroused opposition from antiabortion activists in the United States and elsewhere.
Macchiarini, who recently visited with the Phillipses in their home while on a visit to the United States, has developed a method of growing a replacement trachea through the assistance of an international team of doctors and researchers in Sweden, Spain and Italy.
The procedure, however, has yet to be approved by the U.S. Food and Drug Administration, and unless Phillips can receive a "compassionate use" exemption, Macchiarini will not be able to perform the operation in this country. In addition, the Phillipses could not expect any governmental financial assistance for a procedure that would cost a minimum of $300,000 -- and that is with Macchiarini waiving his fee.
And due to Phillips' condition, it is uncertain if she would be able to make the cross-Atlantic trip to Europe, a journey that would add even more cost to an already staggering figure for a family strapped by medical bills.
"I find myself wondering if a lot of the red tape will work out," she said. "It's strange to have your life valued in a dollar amount.
Steven and Rachel are receiving support from across the country, including Quincy.
"We are so grateful for the friends we have in Quincy, and all the prayers there," Rachel said. "I wish we could get back to visit."
Right now, though, that is not possible.
Phillips' condition is all related to a life-threatening condition called Ehlers-Danlos Syndrome (EDS), a genetic disorder that affects her body's connective tissues. Through early adulthood, her "blessing" of extreme mobility was actually a curse working against her. At around age 30, her body literally started to fall apart via numerous torn ligaments and dislocations. She lives in what is often extreme pain.
The physical trait that may have allowed Phillips to rise to the top of the ballet world now threatens her life.
Doctors have now also determined that EDS was directly responsible for five miscarriages Rachel had earlier in the couple's marriage.
In just the last year, she has spent more than two months in the hospital and had 31 bronchosopies to open and examine her trachea.
Macchiarini has detailed in published reports how the procedure works. Doctors create a matrix -- or "scaffold" -- of Phillips' windpipe, using either a plastic polymer modeled after her own trachea, or one taken from a cadaver stripped of all its organic cells. That scaffold is then placed in a liquid medium and seeded with cells extracted from Phillips' bone marrow. Within 48 hours, Phillips' stem cells grow a new windpipe over the scaffold. Macchiarini said that unlike conventionally transplanted organs, this one won't be rejected by her body or require lifelong immunosuppressant drugs.
The odds of success appear excellent. Of the 11 trachea transplants he's done so far -- 10 involving donated tracheas and one using a synthetic one -- Macchiarini said only one patient has died, and that was due to complications unrelated to the surgery. Most patients, he said, have awakened after the lengthy surgery and began breathing normally.
As a thoracic surgeon, Macchiarini is also developing regenerative therapies involving the lungs, heart and esophagus. He hopes to use the patient's body as a "bioreactor" to regenerate damaged organs and tissues in place -- without surgery.
Steven and Rachel moved to Vermont to start a school for the performing arts. A retired minister, Steven, 58, now devotes his life to caring for his wife of 12 years.
"We have been averaging 4-8 medical appointments per week for the last year," said Steven, who still has family members in Quincy. "It has been extremely draining ... but Rachel is a unique person."
Steven loves to talk about his wife's zest for life and her fighting spirit. She has personally done extensive research on her own condition.
"That same tenacity is there in everything she does," he said.
He said it is especially difficult to watch when she begins to struggle for air. There are times when her trachea struggles to open after she exhales.
"I know (at a certain point) I have two minutes to live," Rachel said.
That's when there is a mad dash to the hospital.
Steven said for Rachel to have accomplished what she did as a dancer -- beating out 14,000 other artists for a spot with the Royal Ballet -- is equivalent to winning a gold medal in the Olympics. Rachel also danced with the Kirov in St. Petersburg, Russia, the Nashville Ballet and other companies in the U.S. and abroad.
That all makes her current situation even more frustrating for the both of them, realizing how fate robbed her of a special gift and talent.
"If we had the funds, we would be in Sweden right now," Steven said. "In spite of several surgical procedures aimed at keeping her airways from collapsing, her condition has dramatically declined in the past 12 months and, we are told, without this operation, she will die from the condition.
"Obviously these obstacles are daunting. We are in the process trying to raise the funds from private sources. One church did a water balloon throwing booth where the pastor sat in a chair and people purchased chances to hit him with a water balloon. Another woman from Montana has offered in her blog online to shave her head if she can raise $5000 to help. The local news station put a link on their website for people willing to contribute."
Rachel is quick to point out that if she is able to receive the surgery, her problems with EDS will not be solved, but the procedure would solve one -- and arguably the most important -- part of the equation.
It would keep her alive.