Multicystic Dysplastic Right Kidney.

So, as many of you probably already know, I am a perinatology sonographer during my daytime hours...(gee, sorry for all the shop talk tonight. Perinatology is high risk obstetrics, and sonography is ultrasound. Essentially, I do ultrasounds on babies or mother's with very high risk factors. I also refer to myself as a prenatal photographer, but that's not a very medically accepted term:) As you might guess by the context of my job, I see all kinds of various conditions that babies can be born with. I have learned that most things are not quite as scary as they'll sound, and though there are textbooks full of random things that can go wrong in fetal development, you'll be surprised to hear that they rarely do. And in the off chance that a child does have a flaw in their development, it's usually something very livable. My job, as a prenatal photographer, is to identify all the normal aspects of a baby fetus, to identify the abnormal elements of the fetus, and to help the parents understand and visualize what is developing abnormally when possible. (Okay, so it's a whole lot more complicated than that of course, but it's the gist...) The trick to my job is doing all of this in a way that is as reassuring as possible for the parents to be, while still being honest and not sugar coating a potentially upsetting condition. Lucky for me, I have a good idea of what it feels like to be the panicked person on the table, and so I am well suited for this job.

One of my favorite anomalies to talk with parents about is called Multicystic Renal Dysplasia (MRD). Interestingly, this condition has weaved its way in an out of my life more than once. I first ran across MRD when I was an ultrasound student. At that time, I was pregnant with Jake, and dealing with renal hydronephrosis. That meant my own kidney was really swollen as a result of a heavy uterus and baby squishing the tube that allows urine out of the kidney. Other than a lot of backaches and a few visits to a less than honest or polite urologist,my hydronephrosis pretty much caused little or no long term damage to my body or my pregnancy. It did, however, spark my interest in renal disorders. At the time I was learning about my own renal complication, I was assigned a research project for my clinical study. Aptly, I chose renal hydronephrosis as my topic of research. That lead me to researching a condition known as Multicystic Renal Dysplasia, because these two conditions can look really similar in the womb, but have much different implications at birth, so it was important to know and highlight the difference. And so, during that time of my life, I thought I had become an expert on MRD.

Little did I know, that was just a taste of what I was about to find out. Imagine my surprise when I was poised on an exam table, pondering why my good friend and fellow sonographer, Ms. Mandy suddenly became very quiet. For those of you who don't know, a quiet sonographer is usually a bad sign, and if you know Mandy, it is a VERY bad sign, because an exam with Mandy is like front row seats for a comedy stand up act. (Or sit down act, as she often jests.) When I looked up at the screen, I very clearly remember saying, "Holy hydro!" And, my initial impression would've been wrong, which brought everything I had studied during the research project flooding back to my memory. 

Upon closer examination, I realized that what I was starring at wasn't just a swollen kidney, but a multicystic dysplastic right kidney. My initial response to this anomaly living in my child was very similar to the response I'd have with any child. I studied it, looked at the cysts and tried to determine if they connected, if the fluid around Ben was adequate (indicating renal function), and looked for a normal kidney on the other side... These are all the steps I would take with any other fetus, and I followed each of them in my mind while Mandy worked my job. But, all at once, it occurred to me that this was not just any other fetus. This was my son. This was my son with this hideous looking kidney taking up more of his abdomen than I could fathom would be possible, and before I could push that idea away, my eyes stung hot with tears and Mandy became one of the few people to see me cry in my adult life.

Now, this reaction, with any other parent would seem completely appropriate to me. But from me, it was a completely silly set of emotions which were not well founded. I knew that MRD could only be fatal if it occurred in both kidneys, and yet, I could clearly see his left kidney was well formed. I also knew that if the left kidney was formed well but not working properly, there would be no fluid, and I could assess that the fluid was completely perfect with one glance. I knew that babies with this condition often were born and the ill formed kidney would atrophy away, leaving them with just one normal, functioning kidney, and a pretty normal life. So, what on Earth was I crying for? I knew this was not a fatal disorder, nor was it a disorder which would profoundly change the lifestyle that we had become accustomed to, nor would we have a child who couldn't function cognitively. In the grand scheme of things, most adults who find out that they have only one kidney probably had a multicystic dysplastic kidney and didn't even know it until it was discovered on accident years later.

So, I cleared up my crying, thanked my lucky stars that my son had no other indications of a larger problem, and agreed that I would see a geneticist. The geneticist, (who interestingly now works within my building at times and has allowed me to assist him on procedures from time to time) agreed with what Ms. Mandy and I had diagnosed, and further sent me on to meet a pediatric urologist. Now, another stroke of luck, because it just so happened that the pediatric urologist we went to visit was MUCH better than the urologist I had as an adult. And, after a visit with the pediatric urologist, I found that I had even less to worry about than I had once thought.

I was concerned that my son would not be able to play sports. I have no idea why this is such a common misconception about children with one kidney, but Ben's urologist assured us that there is no statistical reason to believe that children actually injure kidneys in sports, even contact sports  Although, he would advise us not to let any of our children skateboard because that is a statistically dangerous activity for children under the age of ten. (Um, so of course I would later go on to play a contact sport on skates... go figure...) I was reassured that Benjamin would be able to take any medicines prescribed to him, that he would be able to get his immunizations as any other child would, that illness would affect him no differently than it would any other healthy child. I was assured, in fact, that Benjamin was a healthy child, because really, you only need one good working kidney to survive. 

Ben's doctor did suggest that we keep a very close monitor on Ben's blood pressure, as at any age, elevated blood pressure can be associated with only one kidney and can also be damaging to a kidney. He suggested a few tests to be done early in Ben's life to assure us that his good kidney would function properly, which it did. He suggested that we try to establish healthy eating habits and good exercise routines early in life, to ward off conditions such as diabetes which can be devastating to kidneys later in life. Finally, Ben's urologist suggested we follow the poorly developed kidney with ultrasound to be sure it atrophied as it was supposed to. The plan was to remove it surgically if it did not resolve on it's own, as there is a small but significant link to renal malignancies later in life when they do not resolve. We would watch every year until Ben was four, though we could not identify any cysts after the age of two. 

Ben's extraordinary pediatrician helped us implement these things and also added that the most common reason, statistically, to lose a kidney as a child is in a car accident. He suggested we simply be sure that Ben's good kidney always points towards the inside of the car. Ben's urologist was impressed by that tidbit and now bestow's it upon his patients as well.

So, flash forward to today. I have a very healthy nearly 5 year old boy who doesn't ever really stop moving. Though I plan to encourage him to be a golfer, or a tennis player, or any other low impact athlete, he seems to want to follow in his parents footsteps. He likes to climb. He likes to fall, and has already dislocated his elbow. He collects bruises and scrapes, just like most other boys. He likes to go fast. He can't wait to start playing soccer very soon. He has become my empathy for parents facing the anxiety of a fetal anomaly of their own. More importantly, he has become my reassuring story of success to share with parents who are faced with an anomaly of their own.