More story sharing: “A Wounded Healer – Amanda Goodwin”

I was recently interviewed and photographed for my college’s newspaper, the Kent Stater.  I want to thank the writer who was in contact so many times and took the time to  write such a lengthy article.  It was such an honor to publicly share my story, again, and I pray that this new audience has a chance to be inspired by the story God has blessed me with.

Unfortunately, there were several inaccuracies in the story.  I’m not sure if I didn’t describe something well enough to the writer or perhaps he took too much literary freedom, but this is the link to the article, and I will post it below with my corrections in [brackets]. If you’d like to share the article, please share it from this link where everything is 100% accurate.

A Wounded Healer: Amanda Goodwin
by Mark Oprea

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The soon-to-be bride walks around the daylight in her house, cradling her 10-pound white shichon Haylie up against her chest. She smiles with rose-colored lips. Her almond hair curls into her chin. Her dog looks up at her with beady eyes, a pocket-sized pink bow behind [her] ear.

The mother follows her with words about the wedding shower; the father quips relentlessly through his fatherly grin. Bridesmaids begin to show up in a row, letting the unforgiving cold seep in from the driveway. A five-foot-tall Eiffel Tower [set up for the wedding shower] shines with gold in the dining room. There is still a month or two for things to go wrong.

“We’re not ready to have [the wedding] tomorrow,” she said about the ceremony, “if that’s what you’re asking.”

She is happy. She is nervous. Her name is Amanda Goodwin. She is 27, and she will be married this April.

Amanda has achieved several milestones in the past few months, her latest graduating after nearly a decade in college. Last year, her boyfriend Scott proposed to her. She’s been smiling more often, her mother, Pam, said.

Ever since Amanda was 5 years old, she has had chronic liver disease.

After nearly two decades suffering from the effects of primary sclerosing cholangitis (PSC) — a disease that scars bile receptors in the liver, causing an eventual shutdown — Amanda has been through cycles of hope and despair, often buffeted by late-night phone calls from the intensive care unit [I have never in my life called the intensive care unit.  Maybe he meant my transplant team?]. As someone erudite in medicine, Amanda likes to think of herself as a “wounded healer,” someone who’s experienced firsthand what others only study. For her, it’s been an 18-year-long test.

Moving to Munroe Falls at the age of 9, Amanda spent most of her childhood indoors. She was a “book-smart, intellectual type of girl,”said Pam, the opposite of her varsity softball-star sister, Nikki.

At the Cuyahoga Valley Christian Academy (CVCA), she latched onto the interests of a straight-A student, shot for a solid 4.0 GPA and adored the arts. Even at a young age, Amanda was aware of the research behind PSC. She and her family knew very well that a liver transplant was an inevitable episode — still the only cure known for such a disease. Most PSC patients’ livers last, on average, a decade.

“My doctor said it could happen tomorrow, it could happen when you’re 60 years old,” Amanda said. “I thought I would be a grandma and have grandchildren by the time surgery would come around.”

Yet in the unmeasured meantime, Amanda lived a life bound by the limits of PSC. Some nurses who ran across her case often mistook alcoholism as the culprit of her precarious liver. (She doesn’t even drink.) She often needed 10 hours of sleep or more each day due to ongoing fatigue.  Despite that, Amanda graduated high school in 2005 looking forward to attending Kent State. She had control for the time being.

After a brief stint in the College of Business Administration — her father Keith’s go-to suggestion, owning a successful heating and cooling business himself — Amanda turned to the School of Nursing based on a gut feeling.

As a [nanny], one of the only jobs Amanda could work at the time, she admired the notion of caring for people. The remaining nudge came from introspection.

“Because I’ve been sick since I was a kid. I knew all about the health care system, and patients, and what it’s to be on the other side, being a patient,” she said. “And I thought, ‘What better way to use my journey than to help people and be a nurse?’”

So she did.

Over the next five years, Amanda plunged through Kent State’s rigorous pre-nursing program and into nursing school. She took nutrition and studied genetics in-depth (research continues on the potential for a genetic cause of PSC). Yet eight to 12-hour clinicals brought out the worst in Amanda’s fibromyalgia and fatigue, and her [family] noticed. 

But Amanda had her plan — her usual “goal-mindedness.” She knew what her body was and wasn’t capable of. Above all, she had the will. She decided to continue clinicals despite doctors’ warnings. One even told her to drop out of nursing school.

***

It was right after a 2010 family trip to Disney World when Amanda went in for an annual test ordered by her [gastroenterologist], Dr. Vera Hupertz — a family friend by then —  a run-of-the-mill CT scan of her abdomen. [This was a] typical procedures ever since she was five: nothing imminent was expected.

[The next day, Amanda and her mom visited with Dr. Hupertz.] Hupertz’s voice sounded a little off as she spoke.

“I don’t know how to say this,” she said to Amanda. “I feel horrible saying this to you.”

The CT scan, she told her, showed a sizable tumor on Amanda’s liver. A transplant was vital and had to come sooner rather than later. She and her mother let tears flow. For Amanda, behind the wall were [not only feelings of loss and fear, but] feelings of joy and relief. A new liver would revitalize her body, effectively removing the chronic effects of cirrhosis her “malfunctioning” organ claimed.

“Still, we were honestly shocked,” Amanda said, “because it was the last thing on our minds. Also, we had the fear of whether or not I would make it through surgery or not. It was a very sobering time for all of us.”

Thus began the period of waiting on the organ recipient list. She spent days indoors, diverting a wavering mind through Netflix, [crafts, reading, and] her Bible for solace against pain. She started journaling, even turning her [journey] into a purple-and-green scrapbook. What paired with the laundry list of CT scans, chemotherapy and endoscopies was a deep plunge into the world of transplant survivors[, joining support groups, and learning from them. After [becoming a volunteer] with LifeBanc, she soon had others putting on shoes for her. A “Walk for Amanda” [during Lifebanc’s annual Gift of Life Walk and Run] was organized in mid-August.

For [three] months, Amanda’s transplant liver was still somewhere out there, waiting for her. She was at a moral crossroads. For Amanda to live, she had to wait for someone to die.

***

About 1 a.m. on August 31, 2010, the phone rang again and the family crowded around the receiver. It was her coordinator at the [Cleveland Clinic]. She told Amanda resounding news: they found [a liver] her size.

All Amanda knows about her organ donor was that he was a teenage male who passed away in an “unspecified accident,” a boy still without a name.

With more excitement than anxiety, the Goodwin family nearly “flew” to the Cleveland Clinic. They knew well the [85] percent [three-year] survival rate. They said a prayer and Amanda [was admitted into the hospital] sometime around 3:00 a.m. This was it, she thought. This was the goodbye to PSC.

“This should be a perfect match for me,” Amanda wrote in a blog entry right before her surgery. “I am so close to a new life. Being healthy is on the horizon!”

Lying on the hospital bed that morning, Amanda thought about her circumstances. She felt lucky and blessed – and not just for herself. She found out from the procurer that the new liver was not only saving her life: ¼ of it was destined for an infant.

The sun shone through the blinds in the windows as Amanda’s [parents and close friend watched the nurse wheel] her hospital bed away [towards the operating room.]

Keith remembers last seeing Amanda before her bed left the elevator, waving goodbye alongside Pam [and Amanda’s friend Jen] as she headed to the operating room. It was around [6:00] that evening when the team of doctors finally assembled.

“The thing was, we didn’t know if we were going to be seeing her again.” Keith said. “That’s what was on my mind the whole time.”

The surgery lasted eight hours. Her family was present the entire time.

By 1:30 a.m. the next day, Amanda was out of the operating room. Doctors were surprised at how well the operation went.

***

She was a new person. She was strong. She missed her dog most of all.

The pathway to recovery, Amanda knew, would be lined with tubes administering pain medication — [Fentanyl and] Morphine — others feeding a liquid diet [or breathing for her].  She looked down at her abdomen: 50 staples assembled in the shape of a chevron (a Mercedes-Benz logo, as Amanda puts it). The pain was telling and overwhelming. She gained 30 pounds in fluids alone that week.

As soon as she regained consciousness, Amanda’s logic kicked in. Her education was, at the time, lifesaving.

“Especially with my nursing background,” she said, “I knew that if I didn’t get out of bed and move my body somewhat, I wouldn’t be on my way up.”

She started walking slowly up and down the halls of the Cleveland Clinic. It seemed like a race to Amanda — an “Olympic sport” — and she ran as if she had been preparing her whole life. She mastered her medication intake and lost 10 pounds in one day. She knew every doctor and nurse by their first name, as they were like her. She imagined herself in their places.

But being immunosuppressed as a result of organ [transplantation], doctors told Amanda true body regularity would take months, even a year. She walked and walked despite the time ahead of her. She left the Cleveland Clinic on a Saturday morning. Her mother drove her back to Munroe Falls on an afternoon without a cloud in the sky.

At home, Haylie was waiting for her [at the door].

“Seeing her was proof that I was home,” she said.

What was supposed to be a new life for Amanda was merely another side of the same coin. Adjusting to her new liver meant repeated trips back to the “Liver Clinic” for CT scans (to check for any signs of a returned tumor [or issues with blood flow]), redressing surgical wounds and intake of pain [and anti-rejection] medication. Her body, as she knew, would take [some time to get past the time of the highest chance of] organ rejection. Or as Amanda puts it, “my body was attacking itself from the inside.”

Problems became so frequent that Pam learned how to dress and clean Amanda’s “cratering” wound herself — knowing, just like her proto-nurse daughter, how to attend to it tactfully. Her father had to readjust Amanda’s bed so she wouldn’t have to climb up to sleep in it. She would lie awake late at night examining with her fingers the 90 or so swollen bumps on her abdomen. More tears came. This time, those of exasperation.

“The stamina just wasn’t there for her,” Pam said. “After the transplant her immune system was shot, and the medication she was on was just making it worse.”

After a month and a half, the girl with the incision was starting to show healing signs. She was weary from the side effects of immunosuppressant drugs and steroids (she recalls restaurant menus “shaking”) but began to live somewhat of a normal life. She resumed [nannying] and her work with LifeBanc, but most important of all was the plan to return to nursing school the following spring.

The problem was that Amanda, even after transplant surgery, was able to handle clinicals even less than she was pre-operation. Doctors told her that even if she did make it through nursing school, her suppressed immune system would prevent her from working around ill patients. [For example,] caring for a sick 7-year-old with mono, could mean, for Amanda, a month in the hospital. “Fighting tooth and nail” to continue her dream of becoming a nurse wasn’t enough. She had to look elsewhere.

Her answer laid in Kent State’s College of Public Health, where she picked up online classes in the fall of 2011. Through several [additional abdominal] surgeries, [such as] a splenectomy [and reconstructive surgery], Amanda [succeeded] through courses in the college, even traveling to the World Health Conference in Geneva in 2013. She met her soon-to-be fiancé Scott the following July. He asked her out on a coffee date, and Amanda said, “we just sort of fell in love.”

In August 2014, Amanda graduated from Kent State with a focus in Education and Promotion, nearly four years after her transplant surgery. She lists it as one of her most noted accomplishments to this day, one drenched in trials and tribulations.

The “wounded healer” had finally made her mark. The surgeon’s knife had only cut so deep. The lessons of life continue to pour from her endlessly like the love she transfuses to others, her dog Haylie included — and maybe most of all.

“You can be at the end of your rope, you can be where there literally is no hope,” she said, “and you can still be able to pick yourself back up.”

It was in the fall of 2014 when Amanda’s doctor at the Cleveland Clinic sat her down after analysis. Future warnings aside, he smiled with good news.

He told her, “You can go on with life now. You can start to live.”

***

It will be a small wedding, she says. Roughly two dozen people, no more. [A large 300-people will come two weeks later.] The “wedding explosion” in the Goodwins’ basement will disappear come the Saturday of the reception.

“And then afterwards,” Amanda says, “we can all finally rest.”

She cradles and kisses Haylie behind her ear, talking about her and Scott’s house hunt, their plans to settle in the area by the fall. She wants kids. She wants to travel to France again, along with Italy. All this, she says, comes with time.

An end to Amanda’s journey isn’t finalized. She still returns for clinic checkups every so often, and even spent two weeks in the hospital in December after she became ill. (“I’m not bad,” she admits, “just unstable sometimes.”) She continues to volunteer for LifeBanc and hopes to work for them professionally one day. And to forget her donor would be to forget where she’s going and where she’s been. It’s [part of] what makes her story her “gift.”

“I’m just happy to be living life,” she writes in a recent journal entry. “Aren’t you?”

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What Ebola prevention means for immunocompromised transplant patients

Ebola can be scary, especially for an immunocompromised transplant patient! (Especially in Cleveland/Akron where an Ebola patient traveled immediately before being diagnosed!) The media is going crazy with every lead they can find, and people are blowing things out of proportion on social media.

Instead of being consumed with fear, I decided to get some answers.

On my quest of separating facts from undue overreaction, I’ve generally avoided the media and stayed in-tune to the CDC and live airings of our local health departments, doctors at our top Cleveland hospitals, and government officials.

I’m speaking to you both as a fellow transplant patient and as a person experienced in nursing and holding a Public Health Education degree.

First of all, Ebola is not contagious unless someone is showing active symptoms. Second, Ebola is not airborne which means you won’t get it from sharing a bus or plane with someone who is showing symptoms. The only way to catch the virus is to handle bodily fluid of an Ebola victim and somehow get it into your body. This could be due to a paper cut on your finger that it seeps into or from transferring the substance to your face/mouth. And lastly, remember that anyone in contact with the virus may not show symptoms for 21 days. I’ll talk about that later.

As transplant patients, hand washing and avoiding sick people is drilled into our brains.

Believe it or not, that’s really all that applies with Ebola, as well.

Avoid anyone who was in contact either with an Ebola patient or anyone in quarantine. Wash your hands after going anywhere, before entering your home, after using the restroom, etc. Just use common sense.

I talked with my transplant surgeon who is renowned in several states around me, members of many boards, and has received training around the world. I have trusted him with my life for several years, and he is the best of the best. His exact words:

Just follow all the protective measures as you have been doing. No need to wear masks or surgical gloves when you go to the shopping mall or church or similar places. No need to cancel your travel plans if you have any. Just follow the general precautionary measures and don’t make life hell for yourself. No need to be in masks and gloves like what was seen frequently yesterday in airports and in planes.”

Knowledge is power, so go over to CDC’s website, and read up. Ask your doctors any questions you may have. They are all receiving constant training on any changes in the disease and are there to help you. You can also call 1-800-CDC-INFO, however if you are a transplant patient, I would start by calling your coordinator/surgeon.

Let’s believe in God to keep His children safe and rest in the knowledge we have of this virus.

Great links:
CDC Press Releases

CDC’s detailed pages on Ebola

In recent news: Liver Preserver Holds Promise for Transplant Patients

Saw this in the Wall Street Journal, Weekend Edition March 16-17, 2013.

Liver Preserver Holds Promise for Transplant Patients

Scientists from Britain’s Oxford University have invented a device that preserves human livers outside the body for up to 24 hours, an advance that could potentially make more of the organs available for transplant at a time when demand is climbing.

This has huge potential.  The future of medicine is so exciting for transplant patients.

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.”

Important: Moving

Hi everyone,

Please change your bookmarks and feed subscriptions as I am moving this site to a new address.  It will be under construction for a little bit and will have a new address, but all of the content will still be there, and it will hopefully be a little bit more organized.

You can always use http://www.crazymiracle.com to get to the site, and its official address is now https://crazymiracle.wordpress.com  Unfortunately, Amanda’s Journey was not available.  😦

I hope to deliver the same content you’ve come to expect, as well as more insight into my life dealing with chronic illness and how God has moved in different ways.  I really want good to come out of the bad, and I want people to somehow find encouragement out of this mess.  If I encourage one person, all of this pain will be worth it.

I am moving to WordPress for a couple of reasons:

1. It’s much more organized.  You (and I) can view posts by searching, tags, and dates without aimlessly clicking the ‘previous posts’ button.

2. Comments will finally work.  They’ve never worked on this Tumblr site, and I’m not exactly sure why.

3. I’m printing a book of my entries sometime in the near future, and WordPress will make this much easier and more automated than Tumblr ever would.

That’s really all.  I’m working on a new post or two to get up shortly.  The new site isn’t very pretty at the moment so please pardon the dust.

See you over at WordPress,
Amanda

8 Months – News & Prayer Requests

Hi everyone,

Yesterday marks my 8-month transplant anniversary. In important news, this year’s Lifebanc Gift of Life Walk & Run is going to be Sunday, August 13 at 7:30am I don’t have my leaders’ guide yet, but I will be leading a team. I hope to have double the participants as last year so write it on your calendar, invite a friend, and stay tuned for an information statement here – probably in a month or so. Here is last year’s campaign page – Gift of Life Walk & Run! as well as photos/video about last year’s event – if you’d like an idea of what this is all about. This year I will have 365 more things to be grateful about, and a donor to honor, so it’s my dream and goal to get double participation.

So news about me. If you’re here, you probably are interested. 🙂 I have a few people checking in via email and social networks, and this is your update. To be candidly honest, I am not feeling that well right now and am extremely overextended with school and obligations, so please let this be your update. My inbox is so behind right now, you don’t even want to know! I love you all, I just am not physically up to all I want to do at the moment.

There is so much going on right now, I don’t even know where to start!

Since March and throughout April, I have had 4 trips to the ER, have seen 2 new specialists, 2 MRIs, 1 CT scan, and numerous other tests.

I desperately want to tell you how well I’ve been feeling and how amazing this new life is, but I can’t do that this time.

See, a lot of you – myself included – may have thought post-transplant life would have to be amazing – easy, even.  Low key.  Healthy.  Fewer meds.  More energy.  Healthy.

One day, I’ll get there.  But not today.

Sure, I have to think of where I was before my surgery.  I had so many other diagnoses along with liver disease, and a tumor to go with it, so thinking one surgery would cure my life was foolish.  Yes, I have a healthy liver now.  That’s amazing.  But I still have back pain, fatigue, and fibromyalgia, with the addition of medication side effects, a low immune system, chronic neutropenia (low white blood cell count, in my case – 1.5 – critical), and newly, degenerative disc disorder and arthritis in my back.  I have to go to the hospital with practically every new problem that arises, even if I’m just sick with a virus or dehydrated.  I get a new specialist over ever issue, because my transplant team acts like my body is a gold box housing a diamond, a donated liver, a rare treasure to guard like it’s the most important thing in the world.  I agree, this gift is priceless, but I think sometimes they go to the extreme.

Either way, this is my life now, and 8 months later, you’d think I’d be able to cope with it.  Sometimes I wish I had my surgery when I was a lot younger so I wouldn’t know what kind of life to compare it to.  Maybe this would be normal to me.  Or maybe my idea of normal life is skewed from being a victim of childhood chronic illness.  What if this is the best it ever was, or ever will get?

I’m trying to be okay with that.

The other week, I had many doctors appointments to figure out some more issues going on with my crazy body, and since then, we are still trying to get on the path to answers.

First, throw in a virus that had me to my PCP twice, ER twice, and required 2 types of antibiotics.  Not fun.

Then, the “big thing” right now – we are consulting with a hematologist/oncologist about my chronic neutropenia and thrombocytopenia (low white blood cell levels – “critical,” even, and low platelet levels).  Last week, I had several labs done for oncology and a CT scan for them.  The doctors have also ordered a bone marrow biopsy, as well, which is my absolute last choice for anything.  More on that another time.

Last week, a home care nurse stopped by to teach me how to administer pentamidine treatments to myself at home.  Because my white cell count is so low, I’m at risk for PCP pneumonia, one of the most dangerous kinds of pneumonia, so I have to get these monthly treatments now.  They taste really bad, hurt my throat, and make my voice hoarse, but at this present time, I don’t have a choice. It’s also scary why I have to get them.

All of that said, my good news is that I’m going to Disney World tomorrow – hopefully – followed by 2 weeks in Ireland with my nursing school.  I’m hoping I’ll be up for both trips.  If I can just have improved health during this month – nothing more – that’s really all I want.  I’ve been especially dreaming of Ireland for a couple years now, so please pray for my one wish to come true.  I filled out my application, turned in reference letters, interviewed, got accepted, paid, bought rainboots, started packing… I’m so close to being there!  🙂 It’s such bad news that my WBCs are still so low. I’m waiting to hear from my infections disease/travel medicine specialist to confirm whether or not I can still go on these trips – I don’t think anyone was expecting my labs to get, and stay, this low. Not only do I sleep all day and am so weak, but I don’t have much of an immune system at all right now.

Despite it all, I’m forcing myself to still get out there and enjoy life so it doesn’t pass me by. I’m making desperate attempts to keep up with all the people I love, the friends I hold so close to my heart. And it always is such a wonderful thing to see how we, at our lowest, can care the most for others who are hurting. It’s an amazing part of this human suffering, and the times during which I am low, it’s so evident – and such a blessing – to me.

But the enjoyment of everything, the gratitude, are the only things keeping me sane right now. I was thrilled to be able to attend my precious cousin’s wedding a month ago as well as my little buddy’s karate testing – yellow belt!  I go out with my friends a lot and have still been able to go to church and Bible study – priorities! School hasn’t been going very well, but I can’t say I didn’t try. Fun in the works is my baby sister’s graduation party and my birthday in a few months.  I think because I’m a summer baby I have an extra passion for summer, and I’m making mental lists of all the things I want to do this year!  Last summer doesn’t count for too much but waiting on my liver.  Even though a year ago this month was the time my world was forever changed, I’m trying to focus on the good things that are all around, even if it’s just snuggling with my little Haylie, the anticipation of Ireland, or the feeling of seeing that perfect pair of shoes on the shelf. I love moments like those.  🙂  So everyone please spend the next week savoring the big and little moments, being passionately grateful, in and for your life.

So friends, this is your big update on all things in my health life. Thank you, thank you, thank you for all of your support.  Please pray I may have peace with everything no matter what happens as well as the purest form of gratitude for my new life highs and lows.

Love, love, love
Amanda

One night when I was really upset about all the confusion and pain going on around me, my mom wrote this Scripture down for me:

Be merciful unto me, O God, be merciful unto me: for my soul trusteth (taketh refuge) in thee: yea, in the shadow of thy wings will I make my refuge, until these calamities be overpast.

Psalm 57.1

7 Month Update + News

Today marks my 7- month transplant anniversary.

It’s been quite a month.  I’ve had a lot of highs and lows with my labs, a lot of new medication side effects, and my back problems have gotten so much worse, so I’ve been seeing new doctors for that.  We’re also trying new breathing treatments to prevent PCP (a dangerous type of pneumonia) since my white cell count is so low, and my doctors have adjusted several meds this month as well.   Additionally, I’ve been in the ER twice, once with back pain, and another time with something we think may be an effect from the back pain.  (The picture above is from the ER.)  I’ve started acupuncture and begun my osteomanipulative treatments, so I’m hoping for some relief.  Sometimes I wish I could just have one week without seeing any doctors or practitioners, one week without any phone calls or labs or paperwork.  Is it sad that this is my dream?

Even at that, I’m tapping my foot and twiddling my thumbs, just waiting for some kind of normal life to return. My liver is doing great, but even a slight fluctuation in my enzymes really upsets my body. I get so tired all I can do is lay in bed. Then we get them under control, and I’m better than ever! The ups and downs are very frustrating to me. As is dealing with the side effects of these meds, especially the Prograf (anti-rejection/immunosuppressant) and the Prednisone (steroids). Everyone says things stabilize after the first year, and I’m aching to get there. September 1!

On another note, I am still working on starting therapy for the post-traumatic stress disorder. I have nightmares and flashbacks, and I’ve learned this happens in a percentage of transplant or surgery patients. I suffered from this before when I initially got sick as a child, so I was predisposed to get it. Once I start therapy, I hope to get out of this state of mind.

I’ve been continually trying to share my story with anyone who will listen. I’m still amazed at what God has done for me, and that’s the bottom line. Yes, I am still adjusting to this life, and some days are a challenge, but that doesn’t matter in the whole scheme of things. I’m a walking miracle, and if you’ve followed my story, I’m sure you’ve seen it as well.

So I’ve decided that I’m not succumbing; I am rising above. I’ve made that choice.
I have so many wonderful joys in my life and so much support here for whatever may happen. I’m so grateful.

And on this high note, here is my “News” – I want to share that I am definitely going to Ireland in May.  All my doctors have cleared me, and I’m absolutely beyond excited for this 2-week trip with my nursing school.  7 months ago, we didn’t know when I’d ever be able to travel again, and now I’ve been completely cleared to go out of the country at that!

Thank you for sharing this journey with me and for your prayers. I pray for all of you, as well.  🙂

So thankful for this today…

Who shall separate us from the love of Christ? shall tribulation, or distress, or persecution, or famine, or nakedness, or peril, or sword? Nay, in all these things we are more than conquerors through him that loved us. For I am persuaded, that neither death, nor life, nor angels, nor principalities, nor powers, nor things present, nor things to come, Nor height, nor depth, nor any other creature, shall be able to separate us from the love of God, which is in Christ Jesus our Lord.

Romans 8.35, 37-39