Your “career” as a transplant surgeon started early when diagnosed with kidney disease at the age of 8 years. What are recollections when you had become aware of the diagnosis and confronted with limitations?
SILKE V. NIEDERHAUS: To be frank, as any 8-year-old would, I hated it all. Especially the needles. My first bad (really bad) experience was when a physician promised to place an IV in my right arm, and then moved the tourniquet more distally, to place the needle in my hand. I yelled, screamed, and fought—it took 9 people to hold me down. After a 3-week hospital admission, a diagnosis of nephroureteral reflux—in addition to a poststreptococcal glomerulonephritis had been confirmed. The reflux was surgically repaired a few months later. Nevertheless, multiple months, admissions, kidney biopsies and ultrasounds later, my kidney function continued to decline, and I was sent to the “big hospital,” the University hospital in Heidelberg to be evaluated for dialysis and a kidney transplant. Initially, an attempt was made to treat my condition with plasmapheresis. At the bedside, with local anesthesia, and with my favorite song blasting louder than ever on the tape player, I got a right subclavian double lumen placed. I am not sure if my favorite song helped or if I had already been used to pain, but the central line seemed less traumatizing than my first IV.
Initially, the main limitation was that I was not supposed to swim. I may have missed out on a swimming career, but I could almost do anything else. I am still a terrible swimmer and had a chance to demonstrate this at the Transplant Olympics in 2012 in Madison. I missed school more frequently, but I had been fortunate that school was not a struggle for me. My disease came also with “advantages.” I learned other “cool” stuff: the “sounds of Korotkoff,” how to take my pulse, and how to interpret urine dip stick results. I also got good at taking medications—in liquid form—even if they tasted gross. Eventually, I learned how to swallow pills after practicing with M&Ms and other small candies.
At the age of 11 years, you started dialysis, not exactly what one envisions to be doing at that age. What have been responses and support of friends and family? How much did dialysis at that age prevent you from following age-specific interests?
SN:I started peritoneal dialysis, and while it wasn't my dream, it kept me alive! I got a 2-inch folder full of information about hemodialysis, peritoneal dialysis, and transplants, and just about memorized it all. I learned all about osmosis, countercurrent mechanisms, and sterile procedures for peritoneal dialysis. I became very good in asking questions and did not stop until I knew what I needed to know. My parents offered to be living donors but were discouraged at the time. My PD catheter was placed and I started training as an inpatient. Although bag warmers are supposed to warm the PD dialysate to body temperature, it still felt cold once the fluid had made it into my belly. Warming the bags up to more than body temperature in the microwave did the trick! I did four exchanges per day: One when waking up (Mom usually did that one), one around lunch when I got out of school, one around 5:00 PM (just before dinner), and the last run of the day just before bed-time (by 9:00 or 10:00PM, again with some family support). A strict low-protein, low-potassium, low-phosphorus diet left almost nothing exciting to eat, and my Mom made any attempt to make the best out of the permissible ingredients. Not to forget, my appetite had not been great. My friends knew I couldn't play sports, but they didn't know much else. A friend once spied the tubing wrapped around my abdomen at the playground and commented “cool belt!”, but that was about it. My disease put a burden on our family life: my mother supported me and joined me for any hospital trips, my brothers were supported by our grandparents and my dad. My brothers, I think felt somewhat left out and were jealous—at home I got everything: the big room, my own TV, and many other “special” things they didn't get (they had their health, but no 8-year-old appreciates that). They also couldn't touch/fight/wrestle with me—so basically I won every fight by definition. That was sad for me, too, because I didn't mind a little roughhousing now and then. Every time I got admitted to the hospital, three teachers helped to make-up for missed school days, unfortunately only with a focus on German and Math and I greatly missed other subjects.
I loved sports, particularly field hockey, a passion of all my siblings. Concerns that my PD catheter could get “dislodged” or, later, that my kidney transplant could get severely injured put an end to my ambitions. Instead, I was “hired” by my brothers' coach as the referee, which I loved. Attributes of quick and on target decisions and sticking to your guns may have helped with future professional demands. Being a referee overall became a good substitute to the sport and I felt that I did not miss out on too much.
At age 11 years, you received a deceased donor pediatric kidney. What are recollections from surgery and recovery?
SN: About 9 months into PD, and 3 months on the waiting list, I received a new kidney. On the first day of Christmas vacation, the phone call came at 7:00 AM (mom was in the middle of draining my PD fluid). The expectation of getting a transplant and not missing school made it even more exciting. Mom took the call: “they have a kidney; do you want it?”—this was by far the silliest question I'd ever heard. We finished the PD dialysis exchange and rushed into the hospital. The single kidney had just under 24 hours of cold time and came from a 9-month-old donor. Asked by the surgeon on my future professional career plans, I answered with confidence: “I will be a Transplant Surgeon!”. I got some chuckling as a response and the (then not yet considered) politically incorrect advice: “Then stay away from boys, because they'll ruin your career plans!” Good to see that the times have changed!
In recovery, I remember seeing the clock on the wall and recall the smell from the surgical hand scrub. My goal was to get out of the hospital fast, but I also felt quite groggy. I was moved to a 6-bed observation unit with curtains separating patients. My kidney made lots of urine immediately! Attempts were made on Christmas Eve to have me stand-up straight, not a good memory. Analgesics were given every 4 hrs.; patient controlled analgesic systems did not exist at the time. I recall being woken up for my on-time Cyclosporine milk shake but overall, I was just delighted to have a new kidney!
Treatments of rejections, over an almost 30-year course have changed. What have been experiences?
SN: I had 3 rejection episodes, each preceded by a slightly below normal nuclear medicine study. The first and second rejection were treated with IV steroids and Thymoglobulin for 10 days. About a month after those 2 episodes, my mom felt that I did “not look right.” I felt fine, but my Serum creatinine was up to 5.4. The worst yet, since I had received my transplant. I was given the option of taking OKT-3 or transplant nephrectomy. I picked the OKT-3 option even though potential side effects sounded like a nightmare, still I felt that things could not be worse than the pain after surgery. I was barely 12 years old, yet I understood the consequences: I knew I could die. I made quite sure I understood all the details and vividly remember engaging in all sorts of informed consent discussions, both for the OKT-3 treatment and multiple other research studies. I understood what my options were; the risks were clear; although my parents had to provide the ‘official’ signature, I had been in charge. Next, I got admitted to the ICU, got my IV pulse steroids and OKT-3 and the next 2 days were hell: high fevers, delirium, but I pulled through.
Most of the approach today in treating acute rejections is still the same. OKT-3 is now gone, although I am not sure if my kidney would still be around if I had not taken it. We certainly have many more diagnostics and the rate of rejections has decreased, but our immunosuppressive treatment remains far from optimal.
Noncompliance is the most frequent cause of graft losses during adolescence. Has noncompliance ever been an issue? How can we communicate the necessity for compliance to patients?
SN: Compliance with immunosuppressants is challenging as the effects are not experienced while side-effects are. Most patients want to take care of themselves—they want to be healthy. Think, high blood pressure meds: mostly, patients do not feel that they have the condition, so why treat it? As physicians, it is challenging to break that logic. Moreover, usually patients don't begin to understand until it's too late. After their transplant, patients have the expectation that they will take less meds, in fact, the number of meds may very well increase substantially. When being ‘educated’ prior to discharge, we tend to forget that patients are frequently still on narcotics. For myself, I must say that I can't even write a 4-letter word legibly after one single pill of narcotic. I hate them. We need better pain control options.
Noncompliance in teenagers is a complex issue with many reasons. One of the many aspects may be the imminent desire in that age group to break rules. Implementing behavioral therapies as a standard after pediatric transplantation may be one way to improve compliance.
Noncompliance for financial reasons may at least in part be an educational issue. Providers may think too often that posttransplant care is affordable. Frequently, we seem too set in our ways, and we just think patients can make the big payments work. The reality is that many cannot. Personally, I think that there is a place for good old prednisone and azathioprine to keep a graft going compared to the risk of noncompliance for not being able to afford “modern” and costly immunosuppressants.
Lastly, I think many physicians don't listen carefully enough to their patients. Or maybe they listen and are too preoccupied with other things to be able to care. This is something we can - and must - improve. When I was 15 or 16 years old, my bone density was very poor—to the point of osteoporosis. I negotiated hard to come off prednisone. My nephrology team wouldn't have it—not after all those rejections. I gave them 2 options: I can taper it, get refills on time, and check my laboratories at home with my pediatrician. You will never know what I'm doing any more. Or, you can taper it with me, be involved, and tell me “I told you so” if it goes wrong. That's how I came off steroids for 20 years of my life. In my mid-30s, my bone density was close to normal. Today, I am back on steroids, and as much as I hate it, I take it, and I do so faithfully, but I'm still negotiating. In a way, I am in a fortunate position being ‘medically educated’. With any patient, also with the “medically noneducated,” we as physicians need to assume that the chronic condition of their disease (that terminology holds true both, before and after transplantation) has educated them and we need to take their concerns seriously. It is important to listen to patients telling their physician that they cannot afford their immunosuppression or patients will be ‘pushed’ into noncompliance.
Have I been compliant? I think that I did well. It helped that I have always could understand what my providers did. Even at age 11, I was all over the effects and side-effects of the immunosuppressants that came in front of me.
You moved from Germany to the US, went to Medical School at the University of Alabama and then continued with a surgical residency and transplant fellowship at the University of Wisconsin. Currently, you work as a transplant surgeon at the University of Maryland. How much of your own experience (as a transplant patient) is guiding you in your advice and empathy with your patients?
SN:For better or worse, I was a patient long before I became a doctor. In treating my own patients today, I always put myself in my patient's situation and envision on how I would want my organ to be selected for me. I push hard for living donors. I educate my patients on how to approach a potential living donor as physicians/surgeons usually do not do that very well. Engaging in a conversation on how kidney disease has made your life miserable/complicated/difficult and telling them to help you find a (living) kidney donor gets you much closer to your goal than asking directly for a kidney.
Keeping yourself healthy on dialysis is important as well: I am surprised, at times, on how little patients know about saving their good veins. About planning ahead for a lifetime of repeated episodes of transplant and dialysis, in a cycle. It is surprising, on how little of that is taught to phlebotomists, nurses, and even in medical school. Dialysis access is the patient's life line before the new kidney and again once the transplant fails!
I feel strongly about getting back to work after the transplant and have a low threshold in accepting “you don't know what it's like”. That's when I sit down with them and tell them my story.
Five years back, you gave birth to a healthy baby. How has your experience in transplantation, the exposure to many patients over the years helped you in deciding to get pregnant?
SN:Other than being a transplant surgeon, I always wanted to be a mom. I knew it was possible, although not risk free. The Transplant Games were very helpful in that I met several other mothers who had babies after transplant. At the same time, I met some patients who had lost their grafts after pregnancy, so I knew I was taking a risk, but one I thought would be worth it. The planning took some time, adjusting medications a few months before trying. The pregnancy was easy overall, and my son arrived 3 weeks prior to the expected date. The most emotional moment came when he turned 9 months, and I realized that I was alive because a little boy like him died and gave me his kidney 26 years back. If I knew the donor family, I think they would be very proud to consider my son their “extra” grandson - but they don't even know he's there. He's the best: loves to ask random questions at night, like “Mommy, how does the liver work? Why are the leaves green?” My kidney did take a hit 6 month after delivery, oddly, but then again, it's also almost 30 years old now, not bad for an organ that was given a 50/50 chance for 1-year survival in March 1989.
Clinical research is greatly enhanced by the input and advice of patients and many grant applications strongly suggest patient input. You have a unique experience and can contribute in a unique way? Have you taken advantage of your special experience in advising or participating in clinical trials?
SN: I am happy to provide input every day and would be thrilled to advice as a patient in clinical studies. I do like research a lot, but I discovered during residency that my first love is patient care. Right now, I work part time to accommodate for my compromised kidney function. I jokingly say that I should work the hours that equal my GFR. As a patient, I participated in many clinical trials, starting at about age 10. I remember the experience as being a lot of fun, with a fair bit of interaction with young physicians, who would take time talking to me. I continue to be very interested in clinical trials and try sign up if they seem to fit.
You have a research interest in an optimized utilization of organs for transplantation. Where do you see the most promising research in this area?
SN:It just breaks my heart to think of the waiting list. I work on optimizing what organs we currently have, but ultimately I don't think that this will solve the organ shortage. I dream about a day when organs will grow on trees, or in laboratories, when the waiting list will go away, when surgeons can work implanting kidneys Monday-Friday, and only round on weekends. No nighttime cases, no weekend cases. 3D printing, or a stem-cell based organ, or a xenotransplant, all seem wonderful opportunities, still far from clinical reality. From a patient's perspective, the progress in research is disappointing. The discrepancy between reported research progress, and the potential for clinical application is often difficult to comprehend. Moreover, everything takes forever before it can be trialed and regulatory barriers slow things down further. I think patients should have much more decision-making ability when it comes to what research should be offered.
Combining clinical, research and private responsibilities is always a challenge. Is the well-being of your transplant an additional task?
SN:Indeed, our individual wellbeing of all of us should be a task we prioritize. As surgeons, we're trained to work not just to, but through, our exhaustion, putting the patients and other providers far before our own needs. We work very hard, much harder than most people can appreciate. And we do not ever show weakness. We just keep on going! It took me a long time to realize that I must be different, and I must do what is right for me first. I worked full-time throughout medical school, residency, fellowship, and the first few years being on faculty. I kept ‘mini-pharmacies’ in my locker or my office in case I had to stay late - it's always good to have some spare immunosuppression nearby for those unpredictable hours. Until my GFR dropped. Even then, I continued full-time work, until I finally was so exhausted and just close to quitting altogether. Thankfully, I have awesome coworkers, bosses, and mentors who advocated for me and allowed me to go part-time. I tell you, I've never felt more awkward and grateful in my entire life. I had to admit, and worse, come to peace with it myself, that I was not going to be a 110% transplant surgeon - at least not for right now. That was hard as doing anything half-way does not fit to my personality. But, I finally realized that it's better to take care of myself and my family first. I think it's time for all of us to wake up and learn that lesson (hopefully sooner than later) - and I think my kidney (disease) has helped me do just that!
Your life is certainly very busy. Is there any time for hobbies?
SN:With a 5-year old who (thankfully) loves sports as much as I do, we like to watch and play baseball, soccer, Alabama football, and many other sports. We go to the pool in the summer, where I don't swim much. I have started coaching again, but now it's U6T-ball and soccer (not field hockey). I teach Sunday school year-round. The month of December is very busy with baking literally hundreds of German Christmas cookies. We try to go skiing as much as possible in the winter. When it's just me, I like to read sci-fi, fantasy, or child development books. I also have finally gotten a sewing machine, but I don't know what I'm doing with that. Kidneys are much easier than sewing a copy of George Washington's coat.