One Year

With each day that passes, I’m more acutely aware that Scott and I have been actively trying to conceive our child for officially a year now. Perhaps the thought of “a year” makes it seem longer than it has been in reality. Twelve months. One year.

I cannot believe what this year has held. Countless prescription hormones. Oral, injections, patches, suppositories. The myriad of side effects: hot flashes, insomnia, migraines, dehydration, bloating, chills, pain, mood swings, crying spells, bruises, nightmares, 30 pounds of weight gain, and probably more that I’ve forgotten with time. Literally dozens of early morning drives to our Beachwood clinic and a couple even further out to Avon to make sure my specific doctor was the one doing any needed tests. Labs and more labs, scar tissue building under each antecubital vein. Oh and transvaginal ultrasounds – literally dozens. They used to make me feel violated but now knowing that every tech in the office has seen my female anatomy, external and internal? It just makes me feel even more numb.

The Beachwood office is grey and drab but the secretaries and nurses add bits of color. This place is where I watched all of my follicles grow, it’s where my embryos rest, frozen in time, and it’s where I was promised hope. It’s where both of my intrauterine inseminations were cancelled, where we found out I wouldn’t be having a 5-day transfer, where over a dozen follicles were extracted from my ovaries while propofol kept me sleeping and as the nurse anesthetist put it, fentanyl kept me from writhing in pain. It’s where my husband’s sperm was injected into my eggs and the cells in our embryos later multiplied into blastocysts. It’s where I woke up after IVF, tears streaming from the months of pain before combined with the screaming, acute pain of the needles that had just pierced my vagina and ovaries fifteen times. I prayed to God that morning and silently hoped that all of this was worth it, that every tear, every procedure, every hope and disappointment, every check written would soon be worth it.

A year probably seems so insurmountable because of the questions that never leave my mind. Was a year a fair deadline, or was it merely a super-imposed notion? If we are not pregnant at a year, would that mean our chances were higher or lower that we would soon – or ever – meet our child? What does “a year” really mean; what does it really define?

I hope to find out soon, but for now I know that my husband and I are the 1 in 8. We have infertility, and I think we have it bad. We have given our hearts, souls, and finances in hopes of bringing a child into this world. Our families and communities have rooted for us, supported us, and prayed for us on this journey. (Thank you.) But what is a journey anyways? Does it always end at a destination? Maybe not always the one that hopes and dreams and aching hearts are made of. And that is a fear too big for me to say out loud.

I once had a dream (while on ovarian stimulating shots, where the dreams are extra vibrant, cruel, and detailed) where I had finally given birth to the perfect child, a sweet and beautiful daughter. It horrifies me that my mind remembers the graphic details during which a man with no face stormed into our peaceful hospital room past my husband and I and grabbed the swaddled bundle of joy right from my arms. The man was screaming that it had been a mistake, aggressively shouting that I did not have a baby after all. I sat there with my mouth wide open, traumatized to a point of no return. Each time I hit that point in recalling that story, my mind fades to black.

So, one year. It’s been a cruel one punctuated with hope. Yet I plead with you, Time, “Please don’t let there be a second year of this.” I’m not sure my heart could bear it. So for now, let each new day on this journey only hasten the arrival of the hope of our promise. May this territory never become our familiar.

Second wind

Go download Eve Ensler’s new book, In the Body of the World: A Memoir.  Like right now.

Profound.  I recommend this work of art to anyone who cares about or is involved in suffering, the human race, or understanding what life really means.  Eve recounts her story of having to stop her life-changing outreach work to deal with cancer, and she beautifully tells of what she learned in the process.  This book is about the big picture, and how we and all of our sufferings are all interwoven.  I read it in three days – could not put it down.  Savor these words from her book:

Having cancer was the moment when I went as far as I could go without being gone, and it was there, dangling on that edge, that I was forced to let go of everything that didn’t matter, to release the past and be burned down to essential matter.

It was there I found my second wind.  The second wind arrives when we think we are finished, when we can’t take another step, breathe another breath. And then we do.

…Sometimes late in the day… a wind comes, a delicious, clean wind.  I believe in wind.  It pollinates and moves things around.  It can cool us off.  It can make electricity.  It can scatter seeds.  It can become a hurricane or a tornado or typhoon.  It can rustle the leaves.  It rises up and it can help us rise up too.

What does it mean to have a second wind, a second life?  It means screaming fire when there is a fire.  It means touching the darkness and entering it and tasting death in the earthquake scar down the center of my torso, in the first scan that announces the chances are good it’s in my liver.  I am burning because the second wind is also a fire that will burn through our fear.  We cannot be afraid of anything, not of anything.  There is no one coming but us.

The second wind is not about having or getting or buying or acquisition.  It is about giving everything up, giving more than you thought you owned, giving double what you are taking.

What is coming is not like anything we have ever known before.  Your dying, my dying is necessary and irrelevant and inevitable.  Do not be afraid, no, death will not be our end.  Indifference will be, disassociation will be, collateral damage, polar caps melting, endless hunger, mass rapes, grotesque wealth.

The change will come from those who know they do not exist separately but as part of the river.  If you want to overcome your sickness, reach out to someone who is sick.  If you want to forget your hunger, feed your friend.  You worry about germs and stockpile your herbs, but they will not save you, nor will your fancy house or gated villages.  The only salvation is kindness.  The only way out is care.  

The second wind will come from the ground, the Earth.  It will rise like a dust storm.  It will suddenly appear from the corners and the barrios, the favelas and the invisible places where most of the world lives. Because the streets are alive… this wind will take everything away. 

And those of you who can live without will survive.  Those of you who can be naked, without a bank account, a known future, or even a place to call home.  Those of you who can live without and find your meaning here, wherever here is.  Knowing the only destination is change. The only port is where we are going. 

The second wind may take what you think you need or want the most, and what you lost and how you lost it will determine if you survive.

I have lost my organs and at times my mind.  I know it is a race now between the people who are helping themselves to the Earth, to the loot, and the rest of us.  I despise charity.  It gives curbs to a few and silences the others.  Either we go all the way now or there is no more way.  Who will step off the wheel?  […] The world burns in my veins, just like chemo did only a few months ago.  I dare you to stop counting and start acting.  To stop pleasing and start defying.  I dare you to trust what you know.  The second wind is beyond data.  It is past pain.  It is found in the bloodstream and cells of the women and men who purged the poison of their perpetrators, who walked through the cancer, the nightmares.  The second wind is coming from your body, it’s in your mouth, it’s in the way you move your hips.

Every vision is necessary now.  Ever instinct must be awakened.  The wind does not turn away.  It blows through everything.  Do not be afraid.  There is no more winning and losing.  We have already lost.  Even the so-called winners feel that way.  That is why they can’t stop self-destructing.  Step off the wheel of winning and losing.  Of course there is risk.  Of course it is dangerous.  I wish I could make this easy for you.  I wish I could tell you there is nothing to lose.  Lose everything.  That is where it begins.  Each one of you will know in what direction you need to move and who to take with you.  You will recognize the others when you arrive.  Build the circles.  Listen to the voice inside.  And… stay tight in your circle.  Dance in the circles.  Sing in the circles.  Join arms in the circles.  Surrender your comfort.  We must be willing to go the distance.  We must be willing to leave the kingdom and surrender the treasures.

We are the people of the second wind.  We, who have been undermined, reduced, and minimized, we know who we are.  Let us be taken.  Let us turn our pain to power, our victimhood to fire, our self-hatred to action, our self-obsession to service, to fire, to wind.  Wind.  Wind.  Be transparent as wind, be as possible and relentless and dangerous, be what moves things forward without needing to leave a mark, be part of this collection of molecules that begins somewhere unknown and can’t help but keep rising.  Rising.  RisingRising.

Quiet

I haven’t said much lately because life has been pretty still for me. My heart has been quiet– there have been no major health scares and my loved ones are well. All is quiet here.

Yet isn’t it funny how “quiet” and “normal” to people like me means sinus infections, shingles and its post-herpatic neuralgia, carpel tunnel, anesthesia treatments, and a few ER visits.

And isn’t it funny how when you’re living your love, you just don’t notice as much?

I’ve spent so much time with my kids, my family, and my friends, and I’ve been so busy with my classes, commitments, and trying not to push my body off the deep end.

But I have to give the most credit to my kids. They are the reason I get out of bed each day. They are the people who say the funniest things and make me smile from ear to ear. They give the best hugs and show me how important I really am and affirm my love is mutual.

This is the quiet balance that keeps my life running and my joy before my pain.

For those wondering, up to bat after Christmas is Botox treatments for my migraines, a consult with an orthopedic surgeon about my carpal tunnel, my biannual tumor markers/CT scan in a few months, and maybe even abdominal reconstruction surgery. Those are my possibilities and I pray I get through what comes with grace and quiet. To some, that may sound scary or daunting, but when you have pain or issues, peace is getting it fixed. So with the best things present in my life, I will proceed with my plans. And with my family, friends, little loves, and Lord – I will transcend.

Praise Jehovah Jireh!

Always hope

Today, I’m low on words.

Two years ago tonight, I received a new liver, and my life was forever changed. I am so thankful for my donor and these 730 days of new life.

Each day is a joy and a challenge, a battle and a dance, yet full of grace to last until the next.

Always hope, loves.  Always.  Each day is beautiful; each day is a brighter day.

Amanda

You gain strength, courage and confidence by every experience in which you really stop to look fear in the face. You are able to say to yourself, ‘I have lived through this horror. I can take the next thing that comes along.’ You must do the thing you think you cannot do.

Eleanor Roosevelt

You are not alone

I truly hope that regardless of where you are on your own journey: at the beginning of an exciting adventure, in the middle of the most difficult time in your life, or nearing the end of a dark season and seeing light and hope, that you will remember this simple fact:

You are not alone.

It may feel like it. You may feel thousands of nights pass and never truly feel connection or purpose but I promise, those nights will end. There is hope for all of us. It may be far, far away from you right now. Please take comfort in knowing it is there. And when the time is right and it drowns you in every rich drop, your life will completely change. From someone who has been to the valley of death and has returned with an abundance of undeserved life, there is hope.

Anne Jackson

 

Liver Mamas

“My name is Kai.  My last name is Fairy Princess!” she’ll tell you.  She’s a happy, spunky preschooler.  You’d never know that when she was 4 months old, Kai received a liver transplant because she had biliary atresia.  Her daddy donated part of his to her, saving her life.  Read About Kai on her mom’s blog to get a picture of what a little girl with a life illness is like.  Seemingly healthy on the outside but living a life just like I did at her age – adjusting normal to mean meds, hospitals, tests, procedures.  Growing up thinking that is normal.  Oh, to be that innocent again.  To be too young to realize that your normal is not normal.  Stay young, sweet Kai.  Stay young.

Jasmine, Kai’s mother, keeps a beautiful blog chronicling her daughter’s journey.  I asked if I could share one of my favorite posts of hers, and she gave me permission.  I think everyone needs to understand what mothers/fathers/parents of “sick kids” have to go through.  Now at 24, I’m really realizing that it wasn’t only me suffering for all of these years.  It must have been hell for my parents.  I wonder if some days still are hell for them.  None of us signed up for this, but together, we draw strength, and we get through whatever is coming up next.  I hope you enjoy this post by Jasmine and visit her sweet blog.  I would love to meet the Hollingsworth family one day and give Kai a big hug.

Watch this sweet video of Kai…

Now without further ado, here is “Once a Liver Mama, Always a Liver Mama” by Jasmine Hollingsworth.  I dedicate this to my Liver Mama – you know you’re not alone.  Thank you for always fighting for me and for never leaving my side.  Only a few months until you’ll be a Liver Mama of 20 years.  I love you, Mom.  I wish you could be some other kind of mama, but I’m glad I was blessed with you.  I can’t imagine going through these years with anyone else by my side for every single moment of fear, all of the pain, and even the bright beacons of hope.  We have a unique relationship because of the battles we’ve fought hand in hand for 19 years, and I treasure you all the more for it.

As I sit and keep a distant, online vigil for a baby and a family I have never met, I am forced to reflect.
 
Liver Mommas: We support each other. We share our stories, our joy, our pain, our disappointments, our triumphs, our hope, our advice… But, most of all, we share the experience of having a child with a life-threatening liver disease, more often than not, leading to transplant and the life-long trials and complications that come with that.
 
When someone announces “THE call has come; a match has been found!” We rejoice. Those of us who have been through transplant feel the excitement, the anxiety and the rush of conflicting emotions that we remember from our own experience. Those who have not, I imagine, feel the excitement and hope, fear and longing that goes with the waiting and watching your child grow more ill. 
 
When we hear “There are complications, we need prayers!” We bow our heads and fervently whisper words of love and hope to be carried to heaven, knowing the fear and the way time freezes as you wait to hear that, hopefully, everything will be okay.
 
Sometimes, with a profound sadness that cannot be expressed, we receive the devestating news that a tiny spirit was too great for this earthly world and was called to heaven. Those of us who have never experienced this have a mixture of emotion… grief for the life lost and the family, a desire to reach out to comfort where we know there can be none, a gripping fear in the knowledge that our story could have followed the same path (and maybe still could), guilt that we were “the lucky ones”, and a renewed appreciation for the life of our own children and each day we are blessed to spend with them.  
 
To those of you waiting, we know it seems endless. We remember the hope and we hope with you. To those of you recovering, it’s a long road, but you and your child will get there. One day you will look back at how far you have come and marvel at all that has been accomplished. To those of you who have come out pink and rosy on the other side, we celebrate and cherish each day with you, knowing that tomorrow could bring new challenges and fears. To those of you who have experienced the loss of your precious baby, I have no words of comfort; saying that I’m sorry for your loss does not even begin to adequately cover the depth of my empathy. 
 
I can say this, though: Once a Liver Momma, always a Liver Momma.

The Case of the Disappearing Liver Disease

This story from Stanford University School of Medicine is the most personally hopeful account of a medical observation I’ve heard in my lifetime.  As a patient who was forced into a liver transplant due to Primary sclerosing cholangitis and having met friends along the way with identical stories, this article ought to inspire a lot of us.  There is no cure for our disease – none but transplant itself.  But sometimes, by the point a patient is sick enough to get to that point on the list, he or she is too sick for transplant.  Not to mention the ease of a transplant compared with the ease of well… read on and see what this physician noticed is “curing” PSC.  Even though what this doctor at Stanford has discovered perhaps may not necessarily be a medical breakthrough, or not quite yet at this point, it is to us!  To the families this treatment has impacted already, to each patient it’s prevented from transplantation, to each patient whose life it has saved, it most definitely is.

To those of you who may be reading with PSC right now, print this out and take it to your doctor to read over.  I’m sure Dr. Cox would be very interested to know from your physician how your case ended out.  I pray this saves you from a transplant and helps us find a cure so that one day, PSC will only be a term for a disease that used to be.

The Case of the Disappearing Liver Disease
by Erin Digitale; Spring 2011 issue of Stanford Medicine Magazine

IT WAS THE TYPE OF CASE THAT MAKES DOCTORS FEEL HELPLESS.

The 15-year-old boy’s lab tests indicated his liver function was badly impaired. He had a double whammy of two serious gastrointestinal diseases, both lacking cures. On top of it all, his colon was infected with an aggressive bacterial strain, Clostridium difficile.

Although pediatric gastroenterologist Kenneth Cox, MD, had little to offer for the teen’s other problems, he could at least treat the infection. He prescribed the antibiotic vancomycin.

And something very strange happened. The liver-disease symptoms vanished.

“At first I thought it was a coincidence,” says Cox, now chief medical officer at Lucile Packard Children’s Hospital, recalling the moment in 1993 when he saw the first hint of improvement. Maybe he had misattributed symptoms of infection to liver disease, he thought. “But then I stopped the antibiotic, and the liver disease came back, even though the infection was gone.”

So Cox, who is also associate chair of pediatrics and senior associate dean for pediatric and obstetric clinical affairs at the Stanford School of Medicine, gave a second round of vancomycin. Once again, the teenager’s appetite returned, his pain disappeared and his liver tests normalized.

Cox tried vancomycin in a handful of other patients who shared the teen’s liver and colon diagnoses but had never had C. difficile. These kids had been told that their liver disease, primary sclerosing cholangitis, was untreatable. Even a liver transplant was not a guaranteed cure — the disease could recur and destroy a new organ. Yet with vancomycin, the PSC disappeared.

The discovery left Cox in an unusual position. A coincidence — a serendipitous colon infection, of all things — left him holding a potential silver bullet for a devastating and poorly understood pathology.

“The problem is that I’m dealing with a very small group of kids with an unusual disease,” he says. “How do I get the science to prove that vancomycin works, so that all of my colleagues would say, ‘This is the therapy’?”

UNEXPLAINED DESTRUCTION

PSC starts in the “biliary tree,” the tree-shaped network of tubes that carry newly manufactured bile from the liver through the bile duct to the intestine, where bile aids digestion and absorption of dietary fat. In PSC, for reasons no one understands, the tubes become blocked by inflammation. So bile backs up, destroying liver cells and eventually causing cirrhosis.

The rare disease, which occurs in about 10 people of every million, leaves patients feeling severely unwell, with abdominal pain, itching, jaundice, poor appetite, deep fatigue and signs of malnourishment. It can hit people of any age. About three-quarters of PSC patients — including the 15-year-old who started Cox’s research odyssey — also have the more-common diagnosis of inflammatory bowel disease, another poorly understood condition, which is characterized by inflammation and ulceration of the intestine, diarrhea, abdominal pain and a host of other problems.

Cox and his Stanford collaborators believe that if they can figure out how vancomycin alleviates PSC, they’ll solve two mysteries at once. Not only will they have the evidence to convince other physicians that vancomycin is a good PSC treatment, but by finding out how the drug works, they may also learn how PSC begins — which may open doors to better therapies.

Although the research task is daunting, beneath Cox’s caution about its challenges is a definite sense of excitement.

“Most discoveries come by careful observation. I feel lucky that I’ve made this observation,” he says. “The remarkable part is, not only do the liver tests get better, but the children also feel so much better. If you take a look at these children before and after therapy, they don’t look like the same child.”

RESCUING A TODDLER’S LIVER

One of the most dramatic vancomycin-induced transformations came in 2005, after Cox’s team suggested the drug to Lyn Woodward and Melissa Hartman. Their little girl had been through the diagnostic wringer.

Things began to go wrong for Ellery Woodward-Hartman at 8 months of age, when her growth started to lag behind that of her twin brother, Robert. Her liver function gradually worsened; no one could figure out why. By the time she turned 2, Ellery’s liver was scarred with cirrhosis and she was badly jaundiced. Before Cox saw her, other physicians had tested Ellery for everything from cystic fibrosis to lymphoma to HIV. None of those diagnoses fit, and her liver was getting worse. Woodward and Hartman were told to anticipate a liver transplant.

“I thought, this can’t be happening,” Hartman says.

In late October 2005, Cox’s pediatric gastroenterology fellow, Anca Safta, MD, read Ellery’s chart. The symptoms lined up with PSC, Safta and Cox agreed. Safta proposed vancomycin treatment to the family.

“She said, ‘I know about Ellery; we have something that can help her,’” Woodward says, recalling her first conversation with Safta.

“There’s a line from Emily Dickinson: ‘Hope is the thing with feathers,’” Hartman says. “I thought of that poem. It was such a relief.”

 ‘MOST DISCOVERIES COME BY CAREFUL OBSERVATION. I FEEL LUCKY THAT I’VE  MADE THIS OBSERVATION.’Cox wasn’t sure a child as sick as Ellery would benefit from the therapy; his other patients had mostly been at earlier stages of PSC illness. Maybe the antibiotic would at least give her a few months to get stronger before a transplant, he told Woodward and Hartman. Cox wrote the prescription and told the family he would follow up with them soon.

CLUES FROM THE CLINIC

Since 1993, Cox has tried vancomycin on every PSC patient he’s treated, slowly accumulating evidence for the drug’s effects. In 2008, he published clinical observations of the first 14 patients, showing the drug caused improvement in blood markers of liver failure. The index patient, now an adult, is no longer in Cox’s care, but last Cox knew, he continued to do well. To date, 33 of Cox’s patients, plus a handful of others cared for by colleagues around the country, have received the drug. But it’s still largely unknown as a PSC therapy.

Funding has been one obstacle to advancing the research. So far, the work has proceeded without traditional funding sources such as NIH grants. Instead, patients’ families have financed the research via a parent-launched nonprofit, the Children’s PSC Foundation. Cox is now working to secure pharmaceutical company funding for a multicenter study to enable researchers to try the drug in a larger group of adults and children.

The Case of the Disappearing Liver Disease

Bile normally moves from the liver through the branching network of ducts to the intestine, where it digests dietary fat. In primary sclerosing cholangitis, a rare liver disease, inflammation blocks the ducts. As a result, bile backs up, damaging the liver.

FILLING THE KNOWLEDGE GAPS

In spite of the limited resources, Cox has assembled a multidisciplinary team of Stanford collaborators to figure out how vancomycin works. The scientists are starting from one important clue: They know oral vancomycin, the drug formulation Cox uses for PSC, is not absorbed from the intestine. Yet PSC’s trail of destruction starts with inflammation outside the intestine, in the tubes that drain bile from the liver to the gut. The drug must be acting at a distance — but how?

One hypothesis is that PSC arises when pathogenic bacteria in the gut backflow into the bile duct and start a destructive inflammatory response. Normally, everything moves down the duct in one direction, from liver to intestine.

“Essentially, this would be regurgitation of bacteria into the bile drainage system,” says project collaborator David Relman, MD, a professor of infectious diseases and of microbiology and immunology at Stanford.

Another possibility is that bacteria somehow escape from the gut to the blood, then travel through the blood to the bile duct and trigger inflammation.

Under these hypotheses, which Relman’s laboratory is starting to investigate, vancomycin would resolve PSC with its antibiotic action, killing gut bacteria. To determine if that’s happening, the researchers are first taking a census of the bacterial communities in healthy children’s small intestines.

“Almost everything we know so far about the usual gut microbe community is based on adults,” Relman says.

The researchers plan to compare gut microbes in healthy kids to those in PSC patients before and after vancomycin. Their major obstacle — indeed, the reason we know so little about kids’ gut microbes — is the difficulty of sampling the small intestine’s contents. It would be unethical to perform invasive endoscopy on children who have no medical indication for the procedure, so the control samples in Relman’s new study will come from kids receiving endoscopy to investigate non-PSC complaints such as chronic abdominal pain. It’s also challenging to find “control” children who have not received recent courses of antibiotics. “That we know messes with the normal picture,” Relman says.

Still, he is optimistic about the lab’s prospects for cataloguing the gut microbes of kids with and without PSC. If kids with PSC have “different” bacteria before vancomycin treatment and return to a normal bacterial profile with the drug, it would provide strong circumstantial evidence that bacteria initiate PSC. And it would be a good starting point for studies of how the bacteria incite disease.

AN UNEXPECTED MODUS OPERANDI

Another possibility, however, is that in PSC vancomycin is acting as more than an antibiotic. Though textbooks label it a bacteria-killer, the Stanford team suspects vancomycin is also changing patients’ inflammatory response.

Although the idea might seem strange at first, there’s a well-established precedent for antibiotics quieting inflammation. In the last decade, several groups of researchers have demonstrated that, for example, tetracycline’s anti-inflammatory activity contributes to its effectiveness against rheumatoid arthritis, that macrolide antibiotics reduce inflammation in chronic airway disease, and that amoxicillin lowers bowel inflammation in ulcerative colitis.

If vancomycin is acting as an anti-inflammatory in PSC, says Kari Nadeau, MD, PhD, an assistant professor of pediatric immunology and allergy at Stanford, that suggests PSC is a disease of immune function run amok.

Scott Seki of Nadeau’s group already has some enlightening preliminary data. Regulatory T cells, the immune cells that prevent autoimmune disease by tamping down the inflammatory response, exhibit interesting changes during vancomycin treatment, he has found.

Using blood samples drawn before and after vancomycin therapy, Seki showed that the drug doubles PSC patients’ levels of regulatory T cells. Evidence from other autoimmune diseases suggests this change is big enough to cause therapeutically useful drops in inflammation — in other words, it may explain why vancomycin works. Two other experiments in Nadeau’s lab also pointed to regulatory T cells as key players in the vancomycin response. However, this information is still drawn from observations of a very small group of patients, so the team is now working to expand and strengthen their data.

If the findings about the regulatory T cells do turn out to be the PSC linchpin, Nadeau says, “We might infer that some kind of inflammatory process is turned on early in the life of these children that we should move quickly to try to regulate.”

And if the antibacterial effects of vancomycin are key, Relman says, the best approach would be to design a drug that gets rid of PSC-provoking bacteria but acts more selectively. Right now, vancomycin is probably killing beneficial bacteria that have nothing to do with PSC, he adds. “We’d rather not be using a sledgehammer if something more precise and elegant could be devised.”

SURPRISE ENDING

In mid-November 2005, Ellery Woodward-Hartman’s case was presented to the transplant selection committee at Packard Children’s. Though medical records from her pre-vancomycin days clearly pointed toward transplant, the liver-function tests performed after her first 10 days on vancomycin looked promising. The committee decided to re-evaluate her case in December.

A few weeks later, after about a month on vancomycin, Ellery and her family saw their physicians again. “Dr. Safta and Dr. Cox couldn’t believe how well she looked,” Woodward recalls. Ellery’s jaundice had cleared up. Her belly, previously swollen with fluids that accumulated when her liver function was at its worst, had returned to a healthy shape. She was still tiny in comparison with her twin but she was more energetic.

And by the time the transplant committee reconsidered her case, it was clear that the vancomycin was a success. Ellery didn’t need a liver transplant.

“With the degree of disease she had, I was very surprised,” says Safta, now an assistant professor of pediatric gastroenterology at the University of Maryland. Woodward and Hartman feel extremely grateful for the compassionate care Safta provided when Ellery was at her worst, and they still send periodic updates. “It’s just amazing where Ellery has gotten to,” Safta says. “She’s probably the only one with such severity of cirrhosis that has turned the corner like this.”

Now, after more than five years on vancomycin, Ellery is a thriving 7-year-old. Like other patients taking the drug for PSC, she continues to use it without side effects. Though her liver still bears the scars of cirrhosis, and there’s a possibility she may need a transplant at a future date, her liver-function tests are now normal. Her growth has caught up to her brother’s.

“I can’t even calculate what Dr. Cox has been able to do for Ellery,” Woodward says.

Cox sees Ellery’s case as a gratifying success, and he’s encouraged that emerging Stanford science supports the therapy he discovered by accident. This type of discovery is “one of the rewards of being an academic physician,” he says. His collaborators agree. In a project like this, “the patients talk to you through their data,” says Nadeau. “If they’re getting better, that’s what you take as real. That’s what inspires you to go back to the lab and figure out what is happening.”

But there’s one last hurdle: Cox worries that too many patients like Ellery are never offered vancomycin. More than 6,000 people have received liver transplants because of PSC, he says. Though it sounds like a large number, the disease is rare enough that many gastroenterologists never see a case — and so they aren’t reading the literature about new treatment advances. To try to bridge the gap, Cox has partnered with the Children’s PSC Foundation in hopes of helping physicians and patients’ families learn about the treatment.

At a recent foundation fundraiser, he got to meet a few children who had received the therapy from other doctors.

“Kids came up to say it had changed their lives,” Cox says. “They were so thankful. That makes me think this is the right thing to be doing.”