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

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• 07-21-2011
 
• By Jennifer Nachbur

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."

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 me...it's my fault. Thanks for the encouragement. (I'm leaving off your name to spare you the embarrassment.)

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.

Making a trachea from scratch

www.latimes.com

Andemariam Teklesenbet Beyene left a Stockholm hospital Friday, breathing through a man-made trachea that was built with his own stem cells.

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!).

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?!?!

Another drs appt then get to see my amazing Sis Jenni Demont!! Soooo excited to hang out with her. :) Gonna be a low key, o2 filled afternoon.

I never foresaw having to undergo a transplant to save my life in all the things that I've thought, hoped, or dreamed might happen in my life - and certainly not at my age. One thing I've learned is that most of us take our health for granted, until we're no longer healthy. I'm trying to get my head around the whole idea of a transplant but I'm not sure how you really prepare yourself for something like that.

I'm sharing the official report of my recent exploratory bronch from Boston. It's worse than we had thought. Kind of nerve-wracking. You can read the actual report below.

Instead of rewriting all that's happened in the past week or so with my health and new info that we've received, I've decided to save my energy and repost what Steven Phillips (my husband) wrote as it explains everything as well as it possibly could be. Suffice it to say, it's not been an easy week - or a fun one, and I'm trying to come to terms with what all of this means for my life and my future.

The Update I Didn't Want To Write

by Steven Phillips on Saturday, June 18, 2011 at 2:24pm

Rachel and I have returned from several days in Boston for extensive tests regarding Rachel's medical condition.  It has taken a few days for us to be able to process the results we found enough to be able to deal with the information.  One of the tests she had was very long exploratory bronchoscopy (the longest out of 28 this year - she was in OR for three hours).  When the doctor and his assistant came out to see us, you could tell they were both really visibly shaken.  The first thing the doctor said was, "Well, are you ready to head over to Europe to see about the transplant possibilities?"  (Dr Ernst is a personal friend of Paolol Macchiarini - the only doctor in the world who has successfully grown a trachea and bronchial branch from a person's stem cells and transplanted it back into the patient.)  When we asked, "Why?" he said that, nine months out from the surgery Rachel had in September to place mesh on her airways to help hold them open, there was no evidence whatsoever that she had ever had surgery in terms of how badly her airways are now collapsing.  In other words, the surgery was a total failure - Rachel's airways are collapsing as badly now (or worst) than they were before the surgery.  This means that all of the surgeries, stent placements, medications, etc. of the past two years have come to nothing.  A hard "pill" to swallow!

Dr Ernst and his assistant spoke at length with us regarding the lack of other options.  The tracheotomy procedure that had been proposed earlier will not work for her because of the extent of the collapse she is having and how far down it collapses in her airways.

This is an extremely dangerous position for her to be in because it appears to be, in fact, progressively getting worse given the fact that immediately following the surgery she seemed to have some less collapse.  The problem is, if she should get much worse they would have to put her on life support to keep her breathing and then we'd have to figure out if there was some way to get Dr. Macchiarini involved -  which would be extremely difficult to do at that time given that his procedure has not yet been given FDA approval in the US.  Also it takes several months to grow the new tissue after all of the preparations are in place - meaning the soonest we could hope to have this done is at least six months away.  We would probably need to have the airways grown in Italy and the surgery done in Spain (Macchiarino has offices in both places), meaning we would have to be over there for several months and, of course, our insurance would not pay for the procedure or any of the other expenses.  We would have to try to get medical emergency grants, "mercy flights" for the tickets, some kind of living arrangements over there while maintaining our place here, somehow, etc.,  - to say nothing of the fact that we will have to convince Dr. Macchiarini to actually take the case

DR. Ernst is now calling Macchiarini to see what they can work out.  This means Paolo is now hearing about Rachel's case from both Dr Weiss (our pulmonologist here in Burlington) and Dr. Ernst in Boston.  Both of these doctors are on the cutting edge of this technology and personal friends of his, so, we could not have better advocacy.

You are probably wondering how we are doing with all of this.  Oddly enough, we both had a strange "peace" come over us when we were told the news in Boston.  Rachel said that, for one thing, it means she is not crazy or some kind of  hypochondriac.  She has been telling them that she could still feel the airways collapsing all the time and that it was getting worse as time went on.  Obviously, there are the doubts about the "unknown" and the fears that try to grab you when your body is in a serous medical situation.She is still in pain daily from the unrelenting headache that began almost three years ago. She has had a great deal of stress put on her body from the move which was not needed at such a critical time.So her shortness of breath and coughing have become obviously worse.

I guess, for me it means some clarity of where we stand.  We either need a complete "restorative miracle" or we will need a miracle to get all of the pieces to fall into place for the surgery. Please don't "preach at us" right now about the first option.  We know that restorative miracles happen and I have had them happen myself, as well as seen them take place during our ministry.  But, at best, they are notably few and far between.  Sometimes it is hard when people rush to try to get you to depend  upon that option alone when you know that they have never seen one, let alone had to believe for one for themselves.

I also don't see Peter asking advice from the guys in the boat when he began to sink when he was walking on the water with Jesus.  The fact is, faith is a "gift" and there is only One who can help us to trust Him more. Believe me,  we are trusting God, the best way we know how, right now.  We'd both be dead by now if we weren't.  But we are also amazed at how God has set things up for us, so far.  The three pulmonolgists involved in the case are from different sides of the globe, they know each other well, and they have all worked in this technology (DR. Wiess is working on growing a complete set of lungs, the cardiologist Rachel was referred to for another issue turns out to be working with Dr. Wiess to figure out how to hook the new lungs up to a heart when Wiess finally gets it done, Dr Ernst has been a part of regrowing airways and transplanting them into sheep in Boston, Dr Macchiarini has actually done the procedure three time now on humans and... HE IS SCHEDULED TO SEE RACHEL HERE IN BURLINGTON IN A LITTLE OVER A MONTH!!!  What "Masters Hand" could have brought all of that together?????)

The biggest problem that I face is the fact that my young wife - the love of my life - is suffering and no one knows how to fix it.  Having to think about life threatening issues with someone you love is extremely wearing... to say NOTHING about how wearing that is on her!  Our lives have diminished... but hopefully so that HE might increase.

Last Tuesday, setting in the waiting room down in Boston, the chaplain's assistant came over to speak with me because she had seen me waiting there for so long.  As I told her the story, and the fact that this was the 28th time I had sat waiting in a similar situation the past year, I noticed the whole waiting room began listening in.  I said that we had found that we had been required to rethink what church/faith/success really meant.  I said that, at one point through the past two and a half years, I had become very depressed because I saw my life's work completely dissolved.  But then some strange things had been happening.  For example, the "blog" that Rachel had put up to sell some of our stuff before the move had received over 19,000 hits in two weeks!!!... and, it was only published in our local area!!!  Some people came to our house in tears. ( Rachel had published our story on the blog.)  A lot of them didn't buy anything... some of them had driven two hours just to buy a $20 item!  They stayed and talked... exchanged email addresses and phone numbers.  Some of them talked all evening long with us... most left saying they would be praying for us and many have been in contact with us since!  People all over the world are praying for us because of these updates.  Now, almost the entire apartment building we are living in has told us they are praying for Rachel after our only being here for a couple of weeks!  I told her that, at our lowest point in our ministry work, we find ourselves "ministering" to more people than we ever had before. I said that we had redefined "success" as just being here, now, and just being here with God... MAKING JUST THAT, OK. 

I said, "All my life I have been looking for a new definition of "church" and now I fine myself living in one that has NOTHING to do with a place or a format... just being in the midst of a collective group of people who are sharing their lives together... The struggles, and the joys, and the tears, and all of it. When I finally stopped talking with her, I noticed several people in the waiting room had been crying.  One guy said, "Sir, I would be happy to take care of her service dog (Siena) while you go back into the Recovery Room with Rachel."  The chaplain's assistant gripped my hand with tears in her eyes and thanked me for sharing with her. 

God still seems to be at work in us and through us, through all of this.

You may ask, "What do you need?"  The answer is, simply, "In many ways, we don't really know, right now."  But we know we need friends through the process.  Somehow, when we have a need, what needs to happen, happens.  You have been a wonderful part of that process many times.  Right now, we just need the strength to keep putting one foot in front of the other.  The trouble is, we are on a stairway to heaven... and we can only see one step at a time.  Until He shows us the next step, we just have to wait and trust.  We're trying to not rush ahead of Him or lag behind... but just to be... here... now.

Thanks for waiting with us... This stair would be mighty lonely without you.

God Bless,

Steven and Rachel

Pain, just pain.

Mothers Day Thoughts From a Different Perspective

Mother's day can be a mix of emotions for many of us. For me, it's a real struggle. I'm a mother of 5 kids, all of whom I lost before I ever had the chance to meet them. I know there are many others like me who are mothers to children they've never met or to children they lost. And for many of us, we have, at best, challenging if not impossible relationships with our own mother, mothers that didn't live up to basic expectations of what a parent should be, or have lost our mom in one way or another. Please remember with me, the kids that aren't here with us, and for those of us who've lost our mom (however that may be), remember that you've survived and become the amazing person you are because of the challenges. Happy Mothers and Kids day.

Gabbi

Today we had to say a final goodbye to one of best friends ever - our constant companion who loved us no matter what through thick and thin, through lots of moves, traveling North America and was always happiest when she was closeest to us. She never met anyone she didn't win over. Gabbi lost her battle with cancer this afternoon. She was beginning to visibly suffer so much that allowing her to go was the last act of love we could do for her. Our vet gave us a poem that meant a lot to her when she lost her dog, and it meant a lot to us as well. "Cut the leash that holds me here, dear friend, and let me run. Once more a strong a steady dog, my pain and struggle done".

So many of our friends knew and loved Gabbi, and we wanted to give you the chance to share in our tears and remembrance of her tonight. Gabbi, Mommie and Daddy love you so much and have a huge hole in our hearts tonight...goodbye our sweet puppy. Gabriella Noelle Phillips - November 1998-April 30, 2011. Please feel free to post any stories or memories you have of her. We uploaded pictures of her from puppyhood to now, if you'd like to see them.

The Grind of Healing

I need some sort of a mantra or perhaps a set of them.  Healing from a surgery is always difficult for me at this stage.  I'm past the initial couple of weeks, when to be honest, I think adreneline sort of kicks in and helps you get through.  But this stage is sort of flat.  I'm still dealing with post-op pain, can't figure out how to sleep with my "new" neck, ie with the fusion of C4-C5 and that's been more of an adjustment than I would have thought.  To be honest, everything happened so quickly with the surgeries that I wasn't really prepared for the recovery period and what to expect.  So, I've yet to hit a time when I'm feeling really good, though I've been trying to put on a good face for most people.  I think Steven really knows I'm having a difficult time, though we've not talked too much.  He gave up literally his entire month in January just so I could get medical treatment and I know he's "medicaled out" so I've tried not to make tons of conversation about it, though it's almost impossible to avoid the topic.  So, I'm back to the mantras, something I can recite to myself to get me through the times when I'm feeling depressed or drained or in pain or all of the above.  Something to get me through this recovery time and on to the next phase, which will be 3-6 weeks away.  I don't know what I want to be doing, seems like something other than what I am, though.  I'm trying to remember that the most important thing I can do right now is to allow my body to heal as much as possible and recover from the surgeries.

I want to say both to myself and anyone else who reads this that despite my complaining and struggling through this time, I'm SO incredibly grateful that I was able to have the surgeries!  I know that it was literally a miracle that it all happened and I have had SO many people praying and physically working on my behalf to make those surgeries happen (special thanks to Anne, my dr and Sheila, part of her team, without whose epic amount of work, those surgeries wouldn't have happened).  I've been wondering lately if over my life I've done things for other people that in the Universal flow of things somehow have come round to bless me with so many wonderful people who have been willing to help me, or if I have years and years ahead of finding ways to pay back good things to others for all that's been done for me.  I hope, to be honest, that it's some of both...though I have no idea at this point how I could do that.  But those thoughts need to be saved for a later time in order to preserve current emotions which have been a bit internally volatile (understatement).

So back to my mantras.  What could those be?  I will get through this.  I will heal from these surgeries.  This healing period will pass. It's just a few more weeks.  It's OK to feel emotionally variable through this time.  The pain will ease.  My body will acclimate to the new changes that have been made in my body.  These changes will be good and will help.  My TBM has been more manageable for the past couple of weeks.  My HA isn't better yet, but we have time and the new meds on our side to try to help that.  It will be interesting to see how the Diamox will help with the Intracranial Hypertension which may help the HA.  I have friends and family who are helping and supporting me through this time.  With my drs' help, we'll figure out how to deal with the nightly horrendous, gut wrenching, waking up in tears nightmares that happen EVERY night.  And somehow, with their help, we'll figure out how to get that better.

I guess that's enough pressure let out of me for now and maybe a few good thoughts to hang onto in the midst of this lull that's hard to get through.  Problem is, no matter how much help I have, it's still me that has to live out every minute of every day and that's a daunting prospect.  But I'll do my best to not look ahead to that and just focus on now and let the future be just that...the future.

Deep breath, and sigh.

Good night.  I really hope it is one. :)

Life after untethering - 6 days post op

Symptoms are changing around from day to day, which I expected but the surgical pain in still very much present.  I forget I can't do simple things like pull up my underwear in the bathroom, which is why, I suppose they invented the infamous "sexy" johnny hospital gowns.  They're not the most comfortable things in the world but they are functional.

I try to spend a little time up on my feet (with help, as I find I'm a little wobbly with the meds...) to get my "sea legs" back.  This is the tightest my back has felt in my entire life!  Funny, you un-tether the spinal cord so it's finally NOT under strain...and my lower back feels really tight.  It's just the surgery itself.  I know it will get better and resume it's hypermobile self in time.

I'm a little scared as I've had a few coughing spells today.  So far, still better than before but it hurts SO badly to cough because of the cervical discectomy that was done that it's miserable.  I can't imagine the pain if I couldn't stop the coughing!  I have moments when the ringing in my ears stop and for the first time in my life I hear - silence.  But I also have times when it comes back so I'm trying to remind myself that this is a major process of my body rearranging the nerves and relearning what it's like to live without the spinal strain and brainstem compression.  I think it's going to take several months for things to "refind" their way but the time will pass.

I had some issue with one of my ears bleeding after surgery and it's been "popping" strangely today but since this is mostly a place to write down some of the weird symptoms as they happen, just to keep a record, I figure it's worth mentioning.

Keeping fingers crossed that the meds allow me to sleep tonight. :)

Giving over, not giving up

I've come to a place of surrender.  I've always hated that religious term because it holds such difficult and negative connotations.  No one wants to "surrender", wave the white flag, or not "get their own way".  But I'm realizing that those aren't really the true meaning of the word.  I'm believing that it means instead of "giving up" or "not getting my way" that it means I can give these concerns "over" to God because he is well able to keep them for me.  So, I'm attempting to hold these surgeries lightly in open hands before God. What he chooses to do, I leave with him. If there's supposed to be a delay, he still knows best. I have to admit to still hoping he works all out so that miraculously, I could still have the surgeries this week and I still plan to do my part and follow through on any work I need to do to accomplish that. But if he wants me using a walker for a season, that's OK. If he wants to allow me a season in a wheelchair, there must be a reason. So many people have seen his miracles in my life through this, often in working things out quickly for me. But I want that to continue. I don't have the strength to go on and to do this...but he does and my extremely limited strength is only an opportunity for his limitless strength kick in and get me through. I have no idea what this week will hold for me. I still am in need of treatment and surgeries but God knows this so much better than I do. And I'm so grateful for the information I've learned about my genetic conditions and how're they're affecting me and the expertise and kindness I've experienced from the drs I've seen while being here in Maryland. As a posted earlier, a very good friend suggested I "trust the process". Throughout my life, God has often come through for me "last minute" and I was reminded by this friend that, while it may be difficult, it IS a recurring pattern in my life and if I go with it instead of fighting it, I'll be much more at peace. And she was right. :)

I'm remembering that even though I truly believe that healthcare is a basic right for all people, it's also a privilege that I went without for many years.  I'm trying to remember how far I've come because of all the doors that have opened for me.  Without the intervention time and again from a higher source than any of us, I can't even imagine where I'd be.

And, I have a wonderful family - intentional family, who have supported me and loved me through thick and thin when times have been awful and I've been a pain to be around.  I'm so grateful for all of you and for the many of my medical providers who I count among that group.  To have such a family, is another miracle in my life.

A different day and more tests needed...

Repost from my husband: @Steven Phillips

THE GOOD NEWS: Doctors down here think they may have isolated three specific possible conditions that may be the reason for Rachel's problems. THE BAD NEWS: we are stuck here waiting on approval from the insurance before we can proceed with testing to confirm findings. ARAUGH!!! Can't go home - can't proceed! PLEASE PRAY NEED MIRACLE RIGHT NOW!

Couldn't agree more but I'm feeling kind of off kilter with the new info as no one has really shared all that it means or could mean for me. Plus, it's been an awful pain day and actually a really bad cough day, too. My team of drs back home are working overtime to try to help get things sorted out but we really do need prayer for all of this to work out OK. I guess I got the two appts back to back to begin with, which is why I'm here so I'll try to trust for similar openings going forward.

I guess it's dawning on me that if the problems they are going to be looking for on the next round of studies/testing are actually conditions they find I have, I'll be looking at likely two surgeries. One I'm not overly nervous about, but the other would require surgical correction of the area in my mid brain where the brain stem is (where the spinal cord connects to the brain). I didn't realize that would be what would be needed and any surgery around the brain stem (controlling all autonomic nervous system functions) has a potential for some serious complications. I'm trying to just be at peace since it could be found not to be a problem but there is definitely a malformation of one of the bones in my mid brain according to the geneticist yesterday.

I need sleep so much, it would help the pain and the ability to cope and process all that's going on. I'm SO grateful for the new information and am trying not to get my hopes up (too much) that this could help because it would be absolutely awful to hear now that they know I have some malformed bone in my brain BUT they can't do anything about it (sort of a "sucks for you" type of thing).

Enough rambling for now. I'm not even sure I'm making sense now but I'm so grateful for my friends/family and I'm so grateful for your love and support. I'm feeling and acute need for it right now so, please know it's valued and appreciated.

Sleep well all, hopefully with wonderful dreams.

1st Appt in Maryland with my geneticist

Today was overwhelming and I'm really tired & hurting. Looking back, I'm not sure exactly what I expected but the appt with the geneticist certainly was good. She is AWESOME & we definitely loved her & her approach immediately. She was SO thorough, did a lot of physical examination, measuring, etc. I have much more connective tissue disorder involvement than I had realized but we need to do DNA testing to (hopefully) rule out a couple of possible types of heritable connective tissue disorders that are life threatening. We're praying that the insurance will spring for the testing (it requires a pre auth). She's never seen a patient with a CT disorder with the level of respiratory involvement that I have but she's willing to help as much as possible. She (and we) are really looking forward to the neurosurgery appt tomorrow and what the neurosurgeon will have to say.

The weather is bad down here...especially for down here! It's snowing and icing so we're also hoping and praying for safety getting to appts for us and the drs.  But, we both have peace that the weather might open possibilities that wouldn't be there otherwise.  We'll see how it goes.  I'm so grateful to be getting the chance to see these drs and for all the work that MY drs back home have done to talk to these specialists and have them accept my case. Going to try to sleep now...thanks everyone for all the love, energy, prayers and friendship.

A Very Different Set of Resolutions

As 2010 comes to a close, and 2011 dawns, I find myself as I do every year contemplating resolutions that I'd like to make for this new year.  But this year, my resolutions are quite different than in years past. This year, the overall goal is peace. Seeking that peace, searching for it and finding a balance in it is my overall goal. But to achieve this, I have some specific things that I want to aim for as I endeavor to make this peace a reality in my life.

I want to "be" more and "do" less. I want to accept more and judge less.  I want to love more and strive less.  I want to communicate more and assume less.  I want to be content with simplicity, and be satisfied with less.  I want to be able to express myself in ways that make me understood.  I want to let go of perfectionism and allow what is to be enough. I want to turn my thinking and the unhealthy thought patterns that have been ingrained for years upside down and accept and explore the person that I am. I want to give myself permission to be authentic with everyone, regardless of the outcome. I want to learn to be gentle with myself, kind to myself, and as understanding with myself as I try to be with those that I love.  I've missed something important throughout my life and it's a very simple but difficult concept to actually put into practice on a day-to-day basis.  There's so much in the statement, "love your neighbor as yourself". First of all, I need to put that in 21st-century context. To me, that means I should love my family (itentional or origin), my friends, my community, those people I share my life with as I love myself. But the truth is, I've spent the greater portion of my life disliking and at times hating myself so that has to hamper my ability to love those around me if I can't love myself. I'd like to make peace with myself this year. So that's why, in this strange way, my New Year's resolutions are as much about "doing" less and "being" more.  Maybe, if I can learn to treat myself differently, to treat myself gently, I can come to understand what it means, and find the value in myself "human being", rather than a "human doing".

Normally, I'd probably post something like this on my private blog but my pain therapist has been encouraging me to share more of my journey with people - regardless of what that might look like.  So, in order to try to get my head and heart back together and find some sort of equilibrium again, I thought I'l write.  I wish I could use my dictation software but unfortunately, my cough and the acid/vomiting has been back again tonight with a vengeance so I'm on CPAP with all the anti cough meds I can take.  So, the face mask nixes the ability to use the voice software.