Thursday, July 28, 2011

A Compelling Story

To Whom It May Concern,

I have what I believe will be a story of interest to you and your public. A few weeks ago, international news media carried a story concerning the the first synthetic organ tissue transplant performed by Dr Paolo Macchiarini (see First Successful Transplantation of a Synthetic Tissue Engineered).

Dr. Macchiarini is a newly appointed Professor of Regenerative Surgery at the Karolinska Institute in Stockholm, Sweden and also works in Florence, Italy and Barcelona, Spain. He performed the historic surgery last month in Stockholm transplanting a trachea which had been grown on a bio-synthetic matrix using the patient's own stem cells.  Macchiarini and his team have performed ten such operations in the past two years using stem cell regeneration, but all previous operations have used a matrix or "scaffold" from a human donor.  The successful medical breakthrough of the use of a bio-synthetic matrix means that not only will doctors in the near future be able to reconstruct ailing body parts using the patient's own stem cells, and thereby greatly reducing the possibility of rejection of the transplanted tissue, but they will also be able to precisely construct those parts using a computer to create a matrix that will exactly match the structure of the patient's original tissue.

Dr Macchiarini is in Burlington, Vermont this week speaking at a conference on Regenerative Medicine at the University of Vermont. However, most people are not aware that a part of the reason for his visit was to evaluate my wife, Rachel Phillips, a Burlington resident, for this surgery. Rachel is 34 years old and a former dancer with the Royal Ballet of London. She also danced at the Kirov in Russia, the Nashville Ballet, Ballet West in Salt Lake City, Utah and other dance companies both here and abroad. Rachel and I moved to Vermont originally to start a performing arts school. Unfortunately she developed a serious medical condition that has caused her to put her life on hold for the past several years. Her airways are failing from severe tracheobronchomalasia (TBM), a condition that causes trachea and bronchial airways to collapse. The underlying condition that brought about this problem in her case was Elhers-Danlos Syndrome (EDS) - a genetic, connective tissue disorder that affects the collagen in the body.

Over the past two years, doctors have tried numerous surgeries, tests, and other procedures - including over 30 bronchoscopies in the past twelve months alone - in an attempt to try to find a solution for her. However, recent test have verified that she is currently collapsing approximately 90% on normal exhalation - a condition which requires her to wear a special, portable CPAP unit with oxygen support to be able to keep her O2 levels acceptable. She also has a special service dog, named Siena, a four year old Labradoodle who is specially trained to detect, by scent, a decline in her O2 levels and warn her when they fall below acceptable limits. Last fall, Rachel had a tracheobronchoplasty surgery in Boston to try to hold her airways open using a surgical mesh to strengthen the back of the tissue. However the procedure has proved unsuccessful.

Last month we met with Armin Ernst, MD - Caritas Christi Health Care in Boston, MA who has been advising us on the case for over a year now. After his bronchoscopy and review, we were told that there were no other options that they could offer us in the US and that the collapse has rapidly worsened since the operation last fall. Dr. Ernst recommended that we contact Dr. Macchiarini to seriously consider the regenerative transplant option.

We initially met with one of Dr. Macchiarini's associates who was in Burlington, Vermont last month for a review of her case. At that time we were told that they would likely be able to offer the surgery to us, pending additional tests and a final review of the case. Earlier this week, we met with Dr. Macchiarini at Fletcher Allen Medical Center in Burlington, Vermont for an extensive evaluation of her condition, including, yet another bronchoscopy. After looking at the results, Paolo offered to take our case and is currently preparing the paperwork to allow him to operate on Rachel to attempt to repair the failed surgery. He will be taking stem cells from Rachel to grow a bio-synthetic airway replacement to be able to transplant immediately if the surgical repair is unable to correct the situation. We were also told that we have exhausted all other options and, without the surgery, Rachel will die from the condition. The extent of the collapse has progressed so rapidly that waiting as much as a year is no longer an option.

Dr. Macchiarini is making tentative plans to do the surgery sometime in October. At this time, it is uncertain if the procedure will be done in Europe or here in the US, which may be more problematic than the European option due to regulatory issues. My wife and I are currently in the process of trying to find funding options to offset the costs of the procedure which, we are told could run up to $300,000.00, not including our cost for an extended potential stay oversees for the procedure and recovery period, if it cannot be done in the States. This is in spite of the fact that Dr. Macchiarini has offered t do his part at no cost to us. None the less, the financial issues represent a huge mountain for us to climb.

Macchiarini is also an outspoken advocate of health care reform. He told us he believes it to be “criminal” for patients in the US to have viable lifesaving treatment options which are not available to them due to the lack of funds. On this subject he brings his considerable expertise to bear in very practical terms. He quickly compared for us the on going cost of over 30 bronchoscopies and numerous hospitalizations that could be more than offset, in Rachel's case, if stem cell regenerative surgery could resolve the problem. When you add the additional costs of numerous Emergency Room visits, and several extended stays in the Intensive Care Unit, to say nothing of the medications and ongoing visits to our primary care physician and other specialists, it is easy to see what the potential saving could be.

Dr. Dan Weiss (UVM College of Medicine) is our pulmonologist in Burlington. He is involved in stem cell research at the University of Vermont and was instrumental in bringing Macchiarini to the Regenerative Medicine conference. Dr. Weiss is the person responsible for putting us in contact with Dr. Macchiarini over a year ago. Together with our primary care physician, Dr. Anne Knott (see Winooski Family Health), the two doctors have given selflessly of their time over the past two and a half years to advocate for Rachel. For example, Dr. Weiss has been present at all of the more than 30 bronchoscopies performed on Rachel, even though another physician performed the procedure. He has done this without regard to the fact that these are “un-billable” hours simply to keep himself apprised of her situation and to reassure her that someone is keeping tabs on her case. Dr, Knott has advocated with doctors, clinics and hospitals here, in Boston, in Maryland, and in the Washington, D.C. area to help find viable treatment options for us. The endless forms these doctors and their staff members have had to complete for us and dozens of phone consultations that they have made have not been something they could bill to our insurance. But without their tireless support, none of this would have been possible nor would we have had the current opportunity with Dr. Macchiarini.

It is our understanding that ABC News has expressed interest in doing a documentary if Dr. Macchiarini does, in fact, choose an American for the new surgical procedure. For this reason e specifically gave us permission to discuss our case with the news media.

It was my thinking that, given the the fact that this represents an American and a resident of Vermont who was being evaluated for this ground breaking medical procedure within weeks of the first transplant hitting the news, you would doubtless appreciate the local and perhaps national news interest in such a story. In addition, the fact that it also involves stem cell research being done in the US, the fact that the patient is a former London Royal Ballet dancer, the fact that she has a special trained "O2 Sniffing" service dog to assist her, etc., there would be numerous additional reasons this story would be compelling to your audience. Should you or your staff wish to be in contact us and/or Dr. Macchiarini you can reach us at this email address, or by phone at 802-735-2211 or by cell phone at 802-503-1211.

For photos of Rachel at the Royal Ballet as well as several pictures showing the service dog and Rachel's current condition please visit:

For a brief clip about the story done on our local ABC TV station please visit:

Thank you for your consideration.
Very Truly Yours,

Steven and Rachel Phillips

Wednesday, July 27, 2011

On the News

Paolo Macchiarini recommended that we get our story out to the media so that we can find a way to pay for the surgery.  Here's the video from our first TV news story:

Sunday, July 24, 2011

Reality check through a fog

I'm on the official countdown now until I see Dan and Paolo. In a little less than 30 hours Steven and I will be sitting talking with them, finding out the particulars of the surgery, finding if he'll take me as a patient. I just had to write a little tonight because the feelings you have before something like this are never easy to remember after the fact. Usually, clarity comes in once you have clarity and answers the misty fog that previously encompassed you is hard to remember. I have this feeling that it won't be exactly like I expect - and that looking back, it will make perfect sense and seem as if the meeting when exactly as it should and exactly as I should have expected. I was looking through some writing from our trip to Maryland back in January and it was just like that. My only worry is that I am putting too much hope in this meeting, and that my expectations are too high. I pray it isn't so, but anything that would devastate me would definitely rock the boat of my psyche right now.

There are so many things I wonder. I wonder if Paolo will take my case, I wonder if he can grow a large enough segment of my airways to eradicate the effects of the collapse, I wonder if the surgery I had in September will somehow mess up my chances for this. I wonder how we will get to Sweden, I wonder where we'll stay where there, I wonder how soon this will happen, or how long I'll have to wait. I wonder if I can make it that long. I wonder who will keep my service dog, I wonder if we'll have access to the funds we need to pay for all of this. I wonder what they'll do with my trachea, the one they'd be taking out, can they carry out research on it and find out what caused this terrible problem?  Can my geneticist be in on that research? Will I be able to help others if I go through with the surgery? I wonder how Steven will manage this. Alone in a foreign country, his wife in the hospital for numerous weeks. No friends to visit, no family around, not even our beloved Sia. How lonely that will be.I wonder if all of it the transplant. Steven and I had to have the uncomfortable conversation tonight about what he would do with my body if I die Sweden. Could we afford to bring me home? These are the questions that play in my mind, that plague my mind at all hours of the day and night.

Sometimes I get moments of clarity where realize the true gravity of my health situation and how fragile, and precarious life i has become. I had one of those moments today and my eyes were opened to the stark reality that I could die, and that really, death is only a few moments away from me at any given time. I'm not particularly frightened of the thought, though asphyxiation was never high on my list of ways I'd like to die. I wonder with the moment is like when your consciousness leaves this earth and moves to another place.  I wonder what you do and don't feel, physically and emotionally. I imagine it is a peaceful event emotionally, though I always wonder about the physical pain. Billions of people before me have experienced this and it certainly nothing new. But I don't know how many people are in a situation where these unanswered questions are always so visible. I really don't worry about me, but I do worry about Steven, a lot. We've been through so much together, to be together, to have a life together. I don't think he'll easily face a life on his own.

Today has been a hard day.  It was clear to me that I am never quite good enough for certain people in my life (no, not Steven).  I've realized how many times I've been dropped by family and close friends because I didn't meet their expectations which again says to me, I'm still not good enough.  I feel like a leper, outcast, spurned by those who tell me they love me. What does it take to get people to stay around? How do you cope with being abandoned multiple times?  How do you manage to have any belief in yourself when you're told that genetic health conditions are your fault?  I didn't get to vote on whether or not I got this disease. I didn't get asked whether or not I'd like it. I didn't get asked if I'd be willing to have our lives shaped around. But instead, it was forced upon me, something I've had to fight and deal with this since I was a small child. Something that would cause me health problems and pain that people would dismiss.  I was ignored and left to fend for myself, and then abandoned by my family.

But then I wonder what it would be like to breathe unfettered.  It's daunting to think of going through transplant surgery only to come back to constant headache pain, dislocations, subluxations, and constant pain in my limbs, back, joints.  I wonder if solving the breathing problem will help these other issues at all, or if they will be things I will contend with for years to come. I wonder if I'd be free of carrying oxygen, free of my respirator. I wonder if I'll be able to ride my bike, camp, or go to Prince Edward Island like we've always dreamed of.  I wonder if we'll  keep the people in our lives right now, or if this will become too much for them as well.  "A friend is made for the time of adversity."  I guess that puts our list of friends is rather short. But it makes me realize the importance of having people in your life that are true friends, through good times and bad, through fun, pain, despair, levity. A few true are worth thousands of those who love to be around in fair weather but who run at the first sign of a dark cloud.

I'm probably rambling on now about philosophical gibberish, but these things are incredibly important to me as I face the challenges ahead. Monday is not the end of a road ( unless Paolo refuses my case altogether), but the beginning of a long and uncertain journey.  I hope and pray that somehow I'll be able to help others as I go through this and come out on the other side. Though the limitations that have been forced upon me because of my health are extreme, I still have dreams of helping make the world a better place.  And that's with th at hope in my heart that I count down to Monday morning.

Friday, July 22, 2011

College of Medicine: University of Vermont

Dr. M. and his trip to VT. I'll see him Monday morning before his presentation at Noon. :) Literally counting down now!

Regenerative Medicine Expert to Discuss Airway Surgery Advances

Paolo Macchiarini, M.D.. Ph.D.
Paolo Macchiarini, M.D., Ph.D., a professor of regenerative surgery, director of the Advanced Center of Translational Regenerative Medicine, and director of the European Air Institute at Karolinska Institute in Stockholm, Sweden, led the groundbreaking team that transplanted the first synthetic, laboratory-grown windpipe in June 2011.
Surgeon Paolo Macchiarini, M.D., Ph.D., leader of the groundbreaking Stockholm, Sweden-based team that transplanted the first synthetic, laboratory-grown windpipe in a tracheal cancer patient in June 2011, will present a lecture hosted by the University of Vermont College of Medicine at noon on Monday, July 25, 2011. A professor of regenerative surgery, director of the Advanced Center of Translational Regenerative Medicine, and director of the European Air Institute at Karolinska Institute, Macchiarini will discuss “Advances in Airway Surgery” in Davis Auditorium in the Medical Education Center on Level Two of Fletcher Allen Health Care’s Ambulatory Care Center. 

College of Medicine Home : University of Vermont
‎"There is a crack in everything. That's how the light gets in." -L. Cohen, "Anthem."

Wednesday, July 13, 2011

I want to scream right now. I really do. But I can't because my airways collapse. I can barely breathe because my genetic disorder airway collapse is causing me to slowly die, unless I get an experimental airway transplant. And someone just wrote me to tell's my fault. Thanks for the encouragement. (I'm leaving off your name to spare you the embarrassment.)

Friday, July 8, 2011

Check this out, it's what Steven Phillips and I have been talking about. Please keep your fingers crossed and prayers and energy coming our way. I see Dr. Paolo Macchiarini when he's in VT at the end of the month.
Andemariam Teklesenbet Beyene left a Stockholm hospital Friday, breathing through a man-made trachea that was built with his own stem cells.

Saturday, July 2, 2011

Summer has finally arrived & I can't complain since it's the July 4th weekend & if it didn't get here soon, we were gonna miss it! Having said that though, it's giving me A LOT of breathing trouble. Thank goodness for my amazing, can-do-anything husband Steven Phillips who's hooking up A/C for me (& thanks to my dr for prescribing it!).

Friday, July 1, 2011

Happy July 1! We're almost to the biggest holiday of the summer, days are already getting shorter, the swamp maples will start turning by the end of the month, we're more than halfway to Christmas, and only 25 days till I see the transplant surgeon from Europe! Where the heck has this year gone?!?!