Lizard Brain is a shared blog about Science Fiction and Fantasy from Daniel Abraham and Ty Franck.

Rachel Phillips

10.17.11
by Daniel Abraham

I’m about to break a rule.

So yesterday, I spent part of my day working up a draft press release for something called Help Rachel Breathe.  I don’t know Rachel or Steven Phillips.  They’re religious, I’m not.  She’s a former professional ballerina, I’m essentially dance-blind.  There are a lot of people in the world who are in trouble.  But much as I chose not to care about Charlie Sheen, I’ve chosen to care about Rachel.

Rachel Phillips

I don’t talk about my family on this blog.  I don’t talk about my personal life.  It’s a boundaries thing, and it’s a rule I feel comfortable with most of the time.  I’m making an exception here.  The thing that’s killing Rachel is called Ehlers-Danlos Syndrome.  It’s a problem with how her body builds connective tissue, and because of the way it’s expressing in her, her trachea’s collapsing.  There are a bunch of other ways that Ehlers-Danlos can present.  Some folks, their joints spontaneously dislocate.  Some folks scar badly from even very minor injuries.  Some people, their heart valves blow out.

For my wife, it presented as a particularly vicious gastric problem.  Basically, the bits of her esophagus that keep the food and acid in your gut didn’t work so well.  If she got a bad cough, it forced stomach acid out onto her vocal cords, which made her cough, and before long we were in the ER.  After the Darling Child showed up, things got worse.  For about a year and a half, my wife couldn’t eat without feeling profoundly ill.  She couldn’t eat breakfast or lunch because she’d be too sick to work, so she made it through the day on a witch’s brew of Ensure, esspresso, chocolate syrup and ice, then came home for a small, healthy meal and sat on the couch in misery until bedtime.  She dropped almost 40 pounds that she didn’t have to lose.  The gastroenterologists at the hospital where she works misdiagnosed what was going on — we didn’t know then that Ehlers-Danlos runs in her family.  We wound up at the Mayo Clinic in Rochester, Minnesota, where a doctor named Michael Camilleri essentially saved her.  But for about a year, I was watching my three-year-old daughter watch her mother starve to death, and I couldn’t stop it.  It’s possible that my kid has it too.  If she does, we don’t have any way to know how it’ll present in her.

Ehlers-Danlos and things like it aren’t fatal.  That’s a fiction.  Rachel isn’t dying of Ehlers-Danlos, she’s dying of a collapsed trachea.  My wife wouldn’t have died of Ehlers-Danlos.  She’d have died of some other thing brought on by malnutrition.  But we got lucky.

Honestly, I don’t make Rachel’s chances as great.  The surgery she’s looking at has been done 11 times before, with ten successes and one that died from complications.  That’s actually pretty good odds.  But she can’t get it done in the US because it involves harvesting her own stem cells, and we don’t do that here.  Insurance also won’t help with it.  Getting her to Sweden and getting the money to cover it look bad to me unless someone with really deep pockets is moved to write a really big check or thousands of people decide it’s a better use of $10 than the morning latte and muffin.  I’m doing what I can.  A lot of other folks are taking on the burden.  We’re trying.  There’s not a lot of time, and it is a lot of money.

But she may get lucky too.  I hope she does.

If you feel the urge, go read about what Rachel’s up against.  Throw a couple bucks her way if you’ve got ’em to spare.  Tell your friends about it.  Tweet, post, talk about it over coffee at the office.  Whatever.

If you don’t, I get that too. There’s a lot of tragedy in the world, and we could all bankrupt ourselves trying to fix it. We all have to pick our battles, and if this isn’t yours, it isn’t yours.

It is mine, though. Doomed or not.

EDIT:  My Darling Wife is doing a fundraising gig for Rachel which makes it easier to both get funds to Rachel and also significantly alter my immediate aesthetic experiences in the morning.

7 Responses »

  1. I’ll take a swing at it with you. Donated.

  2. Daniel ~

    Thank you for a great blogspot about EDS, I have become friends with your wife due to EDS (I have it too) and am so proud to know how supportive you are of her and this issue. You’re right, no, EDS won’t kill us, but the complications that arise from having EDS could. Any help Rachel gets is not only going to help her but help the rest of us as well. The research that they are doing with her stem cells before they can do her transplant are groundbreaking and with that research they might be able to help more of us that suffer EDS. Keep up the great writing and again THANK YOU for bringing attention to this very important and dire situation! I hope everyone visits Rachel’s website at http:helprachelbreathe.com and donates!!!

  3. Well, now I’m glad I bought The Dragon’s Path new.

    (I wouldn’t have touched something that looked that much like bog-standard sword fantasy if not for the recommendation of Jo Walton, by the way. And god damn if that first chapter isn’t utterly fascinating.)

  4. When I came upon this message I was actually looking for a link between laxity of trachea and ehler-danlos, as my 20yr old daughter has just undergone testing for her breathing issues as well as genetic testing for ehler-danlos and marfans, which her doctors believe kat has both. Our thoracic specialist has discovered that kat’s trachea collapses when she has coughing fits, this is due to a combination of problems, allergies, gastric reflux, and post-nasal drip, as well as environmental issues. We also found that due to autonomic dysfunction issues kat also has additional issues with oxygen depravation for her brain at times, and the cardiologist is looking at the necessity of either a pacemaker or a defibulator to help keep her heart functioning at a better rate, as body temperature, humidity, and postural hypertensions issues also complicate her case. We are still having to undergo further testing to see if there is anything else that can be done to suppress the coughing as kats trachea collapses. So I understand where you are coming from. However my concern is about the treatment you are looking at. As you mentioned that they need to harvest her stem cells, and my understanding of my own daughters condition is that stem cells therapy of any kind is not appropriate for treatment, as the collapsing trachea is due to cartlidge laxity, which is caused by a defect in collagen. This means that they will regrow the same defect. I may be wrong but please check it out by seeking another medical opinion. We were told that in my daughters case, she will eventually require ligament replacements through-out her body, but that she would not be a candidate for stem cell therapy, current artifical ligament therapy, and operating to tidy up the ligaments will just cause further damage, so she is now in a wheelchair due to ligament laxity and muscle fatigue. I hope that I am wrong in your case, and that this surgery will help save your wife. I understand your delemma and have empathy for your and your familys situation. I am also glad to find someone else in the world with part of my daughters condition, as it has been quite difficult to deal with on my own.

    • That issue is one that I believe Rachel’s doctors are talking about (among *many* others). The stem cells are being grown on an extracellular matrix that’s a little more solid than anything Rachel could grow herself. That said, Rachel has a team of doctors discussing and weighing her options who I really hope know more than I do. 🙂

      I was wondering if I could forward your email address to my wife? She’s involved in several Ehlers-Danlos support groups that might be useful to you.

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