Monday, August 22, 2011

(Published 8/22/2011 In the Quincy Herald-Whig) Former ballerina hopes to become 12th person in world to undergo surgery to replace windpipe

Rachel and Steven Phillips, who moved from Quincy nine years ago, are hoping the necessary medical and political channels can be cleared in time for her to receive a life-saving medical procedure. Rachel, a former ballerina, suffers from a rare condition that causes her windpipe to close 90 percent each time she exhales. (Submitted photo)

Published: 8/22/2011 | Updated: 8/22/2011


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.

"It's scary."

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.


Link to the original article:

Friday, August 12, 2011

One Minute at a Time (published on the website)

August 18, 2011 By

Through Facebook, Kirsten Tynan tells the story of Steven Phillips, a man standing between his wife and her death.

Taking care of the person who means the most to you in the world when you are the only one between her and death starts to become too much to ignore any longer.
I recently read Dan Griffith’s article The Demise of the Guise of Guys discussing, among many things, men’s experiences with intimacy and social isolation. Elsewhere, I have also been following one of the most intimate glimpses I’ve had into another person’s life. 
Steven Phillips is a good man and a good husband. His wife Rachel is amazing in her own right, but I want to focus mainly on Steven’s story here. He is, for me, a living example of how strength goes hand-in-hand with vulnerability, trust, and authenticity. I invite you to read on to see what I mean. These passages are condensed from Steven’s Facebook notes. Know that what you read here barely scratches the surface.
Due to a genetic condition, Steven’s wife must have a biosynthetic trachea transplant, an experimental procedure that is neither covered by insurance nor can be performed legally in the United States. Emotionally stretched to the limit and financially devastated, Rachel and Steven now face the daunting challenge and enormous cost of the only possible life-saving therapy for Rachel. Many have come together on Rachel’s behalf, as hopefully many more people will. But more than anyone, it is Steven who stands between Rachel and death.
July 4, 2011
I took Rachel to see the fireworks last night. The park that overlooks Lake Champlain is a block and a half from our apartment. That much was a blessing as 20,000 people go to this every year. We had a spectacular view from the cliffs over the lake.
Rachel didn’t do so well in the end. She took a big tank of oxygen to make sure it would last through the event. When the show was over, she could hardly walk, and we were stuck in a crowd of 20,000 people. To make matters worse, the oxygen had run out! We inched our way back to the apartment. If we had needed an ambulance, it would have been chaos for them to get through such a crowd.
People say, “Just take things one day at a time.” For us, it is often one minute at a time. All we know to do is just to take the next step that lies in front of us. Fortunately, we always find God standing right there when our foot reaches the next step… each time, just the provision we need… just the amount of assurance to continue on or to wait a moment and take another breath.
Try this with me. Take a breath.
Now… skip the next 9 breaths you would normally take! Hold that one breath for sixty seconds. Notice how light-headed you begin to feel, how shaky, how disoriented. Your lungs begun to burn for more oxygen, and you just can’t wait for that next refreshing breath!
You are now living on a comparable amount of oxygen to what Rachel is.
Out here, the next step, the next breath, is a miracle of such wonder and grace. We wait here… waiting upon God… waiting for the pieces to fall into place… a transplant in Sweden that seems like a science fiction novel… a creative miracle… we don’t know. We only know that God is with us right here on this step we stand upon today… this minute… this breath.
Here’s to my amazing wife, Rachel… the strongest and bravest person I know.
July 14, 2011
I had to respond to a question someone asked me tonight and a good a friend of mine was here so I let him read my reply. He asked me to post this after he read it because he felt it was important for me to communicate it. I argued with him, but he said, “Steven, those people who want to help you will appreciate you sharing your heart and your needs even if it is embarrassing for you to do so again… and those who don’t will not pay it any attention anyway.” AGH!!! I just know we are facing the biggest “mountain” we have had to climb yet! I feel like some whining little boy!!! AGH!!! I wish there was some way for me to strike out against something. A very macho/alpha/male response, I know… but how do I “punch out” at a disease?
Here goes… again…
Our church is setting up a fund to receive donations for this purpose. People can donate to it at Organic Church Community; 70 South Winooski Ave. #197; Burlington, VT 05401, or online through PayPal using the email address People need to note on their donations that the purpose is to help with Rachel’s expenses, and we will send a receipt at tax time.
I now understand why “humble” is a root part of the word “humiliated”. I feel like such a beggar. I think sometimes, “God, I can’t send out another update saying our problems are overwhelming us!” He says nothing in reply to that. I think He wants to do something deeper in me, and in those who stand with us, than I can understand on this side of heaven. I just know He has made us a broken vessel. All we can do is wait for the Great Potter to mend us. WAITING is the hardest thing I have ever done!
I am only a block and a half away, but the thoughts start to increase in intensity. Was she breathing? Was her oxygen all right? What kind of a husband are you, Steven? You didn’t even bother to check her color before you left.
July 15, 2011
Rachel is in ICU. She will be sedated all night and she is intubated on a ventilator into her airways for her to breathe. They can’t bring her off sedation because every time they have tried, the coughing has resumed. Dr. Wiess told me they will try again tomorrow, but he said if they can’t do it any other way, they will keep her intubated until Dr. Macchiarini comes on the 25th.
They asked me not to try and stay overnight at the hospital because they said she would not be awake at all and I have to look after Siena [Rachel's service dog] the whole time. They were very understanding about her and let her right along bedside in ICU. Siena really got worried when she licked Rachel’s hand and there was no response. Anyway, I’m only one mile away, and they said I could come back anytime in the night if I wake up and want to come back to see her.
This is a hard time. I couldn’t sleep last night because I was trying to imagine any kind of life without Rachel. I know everyone faces that some day… but not everyone faces it with a wife of only 34 years old. This will be the hardest night I’ve ever had yet. I’m holding on, Lord. Now You have to hold on to Rachel!
July 23, 2011
When you are caught in a serious medical problem, you find yourself somehow strangely detached from it at the same time. You do this to try to protect your inner core from the fear, the sense of helplessness, and the worry that “this could be it” that nags at your mind.
Then all of a sudden, the time for making choices that will affect the rest of your life is upon you. One day is all we have left before that day is upon us. We think, “You should be feeling something, doing something… anything.” All we can do is let the minutes tick by and wait. It’s like the calm before a tornado, eerie and hushed. Are we doing the right thing? What else can we do? Have we done everything we should have done? Oh God! Hear my lonely, trembling cry. I believe… help me with my unbelief. Why is it so quiet?
Is this how Jesus felt that night in the garden?
No answers… only loving friends waiting with us.
Silent weeping.
July 31, 2011
I walked down to the park overlooking Lake Champlain this morning. Beautiful! I took Siena with me because she needs a life also. She can’t be a service dog all the time. By the time she does her “morning stuff”, we run into a couple visiting Burlington for the weekend. They watch us for several minutes before they come up and ask about the “the amazing dog” they have been talking about.
A few minutes more and they know about her “service dog” abilities. She can tell every time Rachel’s oxygen level drops below 95% and comes to warn us. “Wow, I’ve heard about dogs like that before but I’ve never really met one before. I work with kids with pulmonary issues. What’s the medical condition affecting your wife?” Fifteen minutes more and they are almost in tears. “We’ll keep her in our prayers,” they say as we part company. This happens at least a dozen times every day.
Siena and I try to get five minutes to ourselves where she is just a dog and I am just a guy in the early morning mist. But a nagging feeling starts to niggle at my thoughts. I didn’t check Rachel before I left with Siena this morning. I am only a block and a half away, but the thoughts start to increase in intensity. Was she breathing? Was her oxygen all right? What kind of a husband are you, Steven? You didn’t even bother to check her color before you left. I’ve been gone less than a half-hour, but the pressure of taking care of the person who means the most to you in the world when you are the only one between her and death starts to become too much to ignore any longer.
Siena and I start home at a quicker pace than we used to head out this morning. I have to make sure she is OK. On Thursday, Paolo [Dr. Macchiarini] said, “Your job, Rachel, is to make sure you stay alive to October. You have to do whatever that takes to make sure you do!” Those words awaken me every night. These thoughts are every day of our life. I never have fifteen minutes without these concerns coming in. I never wake at night without listening, very quietly, to see if she is still breathing. This is “life” as it is today… and everyday. This is why I write you guys so often. Without the chance to get some of this stuff off my chest… I think I’d go mad. Thank you for caring enough to listen.
August 15, 2011
I know many of you are praying for Rachel. Just know that her airways collapsed SEVEN times this weekend alone. Seven times I had to pause and hold my breath to see if she would be able to get them to open again or run for the car to make a flying trip to the emergency room.
We are now talking with our doctors about trying to raise the money to just go to Sweden and have the surgery because each one of these events is now life threatening. To date, we have raised about $3000, for which we are truly thankful. That represents only about 1% of what we will need… but it is a start… and God is BIG.
Please keep us in prayer. We have some tough decisions to make soon. It’s nerve-racking to “camp” this close to the “fault line” of an earthquake. Pray for endurance and sanity.
This story continues to unfold in Steven’s Facebook Notes, which you can read if you are logged into Facebook, and on Steven’s and Rachel’s blog Breathing Companions. I also want to mention that in addition to the information on how to make a tax-deductible contribution in the July 14 passage above, through the end of August I am offering my self-esteem, disarming good looks, and dignity at a rock bottom price to help out. See the challenge in the sidebar on the website Rachel Reinspired.

About Kirsten Tynan
Kirsten Tynan lives in a small town in Montana with her dog Pepper Ann Delbarco. Her superpowers are overanalysis and bed covers hegemony. She believes that doing the impossible makes us mighty.


  1. Iris says:
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    I can’t even begin to describe how touching this is.
    I wasn’t gonna leave a comment, but as I tried to access their blog, the links weren’t working. I had to google the blog’s name to find it. I just want to point this out, ’cause I think it is important that people can access Rachel Reinspired, specially so they can donate too.
    • Kirsten (in MT) says:
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      Thank you, Iris. I think the links have been fixed now.
    • Chrysti Gilbreth says:
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      Steven and Rachel have been dear friends for many years. Thanks for taking the time to read their story. What amazes me is that this is only a fraction of their story.
      Again, thanks for your kindness!
  2. Daddy Files says:
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    Man…sometimes there are no words.
  3. Andrew says:
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    Speaking as someone who is personally acquainted and peripherally involved with Steven and Rachel, I can only confirm what Kirsten says: “Know that what you read here barely scratches the surface.”
    Thank you for writing this.
  4. Thumb up 3 Thumb down 0
    I must confess as I read this, I almost felt like, “Who is this guy they are talking about?” I’m no hero. Just trying to put one foot in front of the other and love the girl of my dreams the best way I know how. Rachel is 24 years younger than me and a former ballet dancer who was once with the Royal Ballet in London. She’s only 34 now and it is heartbreaking to watch her this physically devastated. Thanks for posting the story. (If people want to know more they can go to
  5. William Hurst says:
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    I have known Steve and Rachel for over ten years. I have followed their lives closely over the years, having visited with them personally before this began and talked with Steven on the phone. Every bit of what Kristen has gathered is true, and she is right, “it hardly scratches the surface. Please help them in this battle with support, both financially and dropping Steve a line of encouragement from time to time. I love these guys!
  6. Eleanore Hurst says:
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    I’m so thankful that Jesus is the great intercessor. Rachel and Steve your name is continually 24/7 before the throne. We love you. :’(

If you'd like to see the original link, you can access it here: - Steven Phillips