Thursday, May 14, 2009
Medical Jargon
Müllerian Anomalies (MA): The uterus, fallopian tubes and upper vagina are made up of two partially fused tubes, which, in embryo development, are known as müllerian ducts (ovaries and lower vagina are not derived from mullerian ducts). These ducts are first present in embryos of both sexes.
In the male embryo, the presence of anti-müllerian hormone (AMH), causes the müllerian structures to disintegrate during early development. They persist in the female because she does not produce AMH.
In females, these ducts run down vertically from flank to pelvic floor in the young embryo and eventually fuse into a double-barreled tube with two loose ends, known as the uterovaginal primordium, or UVP. The double UVP will eventually merge into a single-barreled uterus, cervix and upper vagina, while the loose ends develop into the fallopian tubes. In adulthood, these organs are referred to as the müllerian tract and congenital malformations of this tract are called müllerian anomalies, or MAs.
There are 7 different types of müllerian anomalies. They include agenesis and hypoplasia, bicornuate uterus, unicornuate uterus, uterus didelphys, septate uterus, arcuate uterus, and DES related uterus. I was diagnosed with a unicornuate uterus (UU).
The cause of these conditions/anomalies is fairly unknown. To date, there is no singular cause for müllerian anomalies. Some may be hereditary, others result from an insult to the fetus while in the womb (the T-shaped uterus of fetuses exposed to the DES drug, used to prevent miscarriage between 1940-1970, is the only proven case of this situation), but most cases are simply attributed to random mutation.
Unicornuate Uterus (UU): A unicornuate uterus takes place when one müllerian duct is underdeveloped or fails to develop, causing a banana-shaped half-uterus to form. It may or may not be accompanied by a rudimentary horn, and that other horn may or may not have an endometrial cavity or communicate with the main uterine cavity. A missing kidney or other kidney problems accompany this asymmetric anomaly more than they do other MAs. Adverse pregnancy outcomes are common with UU.
The Statistics: About 2%-4% of women are thought to have some sort of congenital uterine anomaly. Of those 7 anomalies, a unicornuate uterus makes up about 4% (statistics vary) of the total number of diagnosed cases, making it the least common of the uterine abnormalities. About 65% of women diagnosed with a UU will have a rudimentary horn of some kind.
Having a unicornuate uterus unfortunately brings a significant risk of both pregnancy loss and preterm labor, as well as ectopic pregnancies. Of all the MAs, it has shown the highest rate of primary infertility (15%) and the poorest fetal survival (40%). Estimates vary by specific study, but one literature review found pregnancy outcomes in women with unicornuate uteri to be miscarriage in 37%, preterm birth in 16%, and term birth in only 45%.
Doctors believe the miscarriage rate is attributed to abnormalities in the blood supply to the uterus. Per-term labor is thought to be because of space restrictions in the half-uterus and often result in a failed cervix. Doctors may recommend cerclage for women at risk of preterm labor in this situation. It is also found that most babies convieved in a unicornuate uterus will be born breached because of the inability for them to turn properly in preperation for delivary in such a small space.
Kidney System Abnormalities: In the embryo, the müllerian ducts act as scaffolding for the mesonephric ducts, which give rise to the kidneys. Because of this parallel structural relationship, it is common for a kidney or other urinary anomaly to be present with a müllerian anomaly. As mentioned earlier, a missing kidney or other kidney problems accompany this asymmetric anomaly of a unicornuate uterus more than they do other MAs.
Ectopic Ureter: An ectopic ureter is a medical condition where the ureter which carries urine from your kidney to you bladder, terminates at a different site than the bladder wall. In females, the ureters may terminate at the bladder neck/urethra (35%), vestibule (30%), vagina (25%), or uterus (5%). It can be associated with frequent urinary tract infections (UTI), urinary incontinence, and decreased kidney function. Ectopic ureters occur in 0.025% of the population and therefore are extremely rare and often hard to diagnose.
How This Relates To Me: At the beginning of 2009, I underwent an HSG test, which resulted in the diagnosis of the rare anomaly of a left unicornuate uterus. After laproscopic surgery, they found a right (non-communicating) rudimentary horn , which after testing, proved it contained entometerial tissue. This means I was menstruating not only from my left unicornuate uterus but also from my right rudimentary horn. The horn had no outlet, as it was closed off from my uterus, thus my body had been re-absorbing the fluid for roughly some 13 years (I am fortunate it did not result in a rupture).
My left fallopian tube was removed as a teenager (at the age of 12) due to blockage of menstruation upon menarche caused by a vaginal septum. Because my unicornuate uterus is on my left side which no longer has a fallopian tube, there is no communication between my ovary and uterus. Thus, when an egg is released from my ovary it has no path to my uterus to be fertilized (the right ovary and tube are also considered useless since they do not attach to my actual uterus). This results in complete infertility outside of the means of IVF (which then takes into account the risks of the above pregnancy statistics with a UU).
As it is very common for patients diagnosed with a UU to also have an abnormality of the renal (kidney) system, such was my luck. In March of 2009, they discovered an ectopic uterter which had been misdiagnosed for some 13 years. In my case, my left ureter drained into the lower portion of my uterus, which if you read the statistics above, is the most rare of the insertion sites with the presence of such a condition.
Prior to the removal of my above mentioned vaginal septum (at the age of 12), my body had also been re-absorbing the urine drained from my left ureter into my uterus (sounds healthy-huh?). During an extensive surgery, they reconstructed the size of my left ectopic ureter and implanted it back into my bladder (thank goodness for modern day technology). Attributed to years of damage from this condition being misdiagnosed and years of battling infections, I am left with 40% function of my left kidney which will not likely improve.
After a visit to my urologist recently, a nurse at the front desk asked if I was Kara. As I responded yes, she proceeded to tell me, "my goodness...you are one for the history books". I have become quit use to being the "science project" and "show-n-tell" of many doctors. I even jokingly told my urologist that if he wrote about me in a medical journal, I wanted a copy of the article. I try and remind myself that we all have our trials in life...this was simply the one I was blessed to overcome. It brings me tremendous comfort to know that in the life hereafter my broken and somewhat pieced together body will once again be made whole and perfect. I can't wait...
For more information on Mullarian Anomalies, please click here.
Monday, May 11, 2009
Be Careful What You Wish For
Thursday would be just shy of three weeks, so I was desperately wishing that he would be ready to remove it. Dave left work to come and pick me up for the appointment. In preparation for the appointment, I took my strongest pain killer before leaving the house. If he was going to be removing the catheter I wanted to make sure I was drugged up for the experience! Even though he had reassured me that it wasn't going to feel anything like the removal of my incision drain...I secretly didn't believe him.
He came into the room and agreed that because I had an infection and had been experiencing pain around the site of the catheter that it was time to remove it. I questioned him one more time as to whether or not it would hurt like the drain. He attempted to comfort me saying that it would not. He informed me that he never tells anyone how painful the drains are to remove otherwise no one would ever let him take them out. Those words were very reassuring (sigh) because I was beginning to think that I was just the biggest wuss in the world!
The nurse came in to remove it...snipped out the stitches, and told me to take a deep breath. At this point it was all sounding much like what they said when they removed my drain (if this is not making sense, please refer to Wednesday under "My Five Day Stay In The Hospital"). I took my deep breath, somewhat clinching the bed in expectation of sever pain as the tube was pulled from my abdomen. A slight sting was all I felt...you can imagine my relief! She then informed me that the whole in my abdomen would close up within 24 hours.
The doctor returned and instructed me to empty my bladder "often". We then proceeded to discuss how I was recovering. I BRAGGED at the fact that my stent had not really been bothering me, contrary to what I had heard about the PAIN they can cause. This is the point in which I took a massive bit of "humble pie". He proceeded to inform me that I may experience "more pain" from the stent now that I would be urinating normally.

I walked out of the office feeling like I was on cloud 9 (even though I actually threw up on the drive home and several times when I finally arrived home...but that is beside the point). When I returned home, I went pee for the first time in three weeks...whoohoo! It was a little painful, but I figured that was expected since it had been so long. I was exhausted after the big outing and from getting sick therefore I laid down for a nap. Upon waking up my whole world had suddenly changed.
I went to the bathroom again, and again, and again...each time it became more and more excruciatingly painful. I would sit on the toilet shaking in pain as my eyes would water up. I suddenly was very much AWARE of my stent! For three weeks, I had wanted nothing more than for my catheter to be removed and now I could think of nothing I wanted GREATER than to have it back in! And so the saying goes, "be careful what you wish for".
During the day I have to go to the bathroom about every 30 minutes to an hour and at night I have to set my alarm to wake me up every few hours to get up and go to the bathroom. Each time I go, the pain is agonizing, and nothing seems to take the edge off. I have two more weeks with the stent in before it is removed. I have been very humbled by this whole experience. It is ever so clear that now matter how bad something is...there is always something worse.
Sunday, May 10, 2009
Beneath It All..
After missing almost a month of church from surgery, I was finally able to attend on a personal level what seems to be the most dreaded Sunday of the year. Mothers Day is always a harsh reminder of what I am lacking despite our efforts. I have been attempting to emotionally prepare myself almost the entire week for the day filled with gratitude and joy. All in dedication to the most blessed role we can hold as women and that I simply cannot claim as my own.
Saturday, May 9, 2009
Stop This Train…I Wanna Get Off
"To get through the hardest journey we need take only one step at a time, but we must keep on stepping" ~Chinese Proverb
This experience has tested my strength both physically and spiritually more than a few times. I don't know how many times along this journey I have wanted to say, "stop this train, I wanna get off". I have felt on more than one occasion so completely inadequate to keep going; so trodden down to pick myself back up. Each time I reach a new destination, or in other words, overcome another trail...there is this part of me asking, "This is it, right?", "I will be done after this one?". Funny thing is that it never seems to be "it". I haven't finished a race, I can't throw in the towel, and I am certainly not done...
I have come to realize that so often our trials are simply a means to prepare us for...you guessed it, even bigger trials. I know it isn't exactly fun to think of it that way, but I imagine if I were to look at it like this more often, I might have less disappointment and sorrow. I have also found it important to never assume that things can't possibly get any harder, because they inevitably will.
Life is full of surprises, which I am sure isn't news to anyone. It's full of ups and downs, highs and lows. Now speaking for myself, I would say I have had my fair share of both. At times it has been filled with nothing but those "highs", and more recently it seems to be filled with nothing more than the "lows". I am praying that my opportunity for some "highs" is soon in coming. So what do I do till they arrive? I am trying ever so hard to be patient… because there is one thing I know, and that is through adversity comes strength, courage, and blessings. Strength that I am certainly lacking, courage that I desperately need, and blessings that will fill my hearts desires.
After a trip to the ER Monday evening, after complications with my stent and a potential infection, I laid on the hospital bed in excruciating pain. I turned to Dave with my eyes full of tears and simply said, "I can't do this anymore…it is just too hard". His reply was simple, "yes, you can…you already are".
Throughout the course of the past several months, I have reflected a great deal on the Saviors Atonement. Most of my thoughts have been directed towards the peace of knowing that because of His infinite Atonement, he knows every pain my body has relentlessly endured and every sorrow that my heart has felt. More recently, I have turned to those sweet words He uttered to His Father in Heaven in the Garden of Gethsemane, saying "Father, if thou be willing, remove this cup from me; nevertheless not my will, but thine, be done" (Luke 22:42). Even the Savior Himself asked for deliverance from such hardship. But He, knowing what had to be done, suffered and atoned for each one of us.
The Father knows my heart. As much as I would like to be relieved of such physical pains from surgery and the emotion pains of infertility, it simply isn't up to me. He knows what will strengthen me. He knows what will prepare me for what the future holds. He knows what blessings are in store so long as I submit to his will and not my own. Every part of me wants to stop this train, every part of me wants to get off and try my hand at something a little seemingly easier of a ride. But I WILL keep going… one day at time…step by step.
Tuesday, April 28, 2009
A Five Day Stay in the Hospital…
Here is an account of my experience in the hospital during my last surgery. It is long and detailed, so if you not interested, please ignore it. I wrote it in detail because I knew I would forget otherwise (especially considering all the meds I was on). During the procedure they reconstructed my left ectopic ureter (ureter-the tube draining urine from the kidney to the bladder) and re-implanted it into my bladder wall. Over the course of my life time it was previously draining urine into my uterus. This obviously creates a myriad of health issues which have finally been resolved. I feel tremendously blessed to have found a doctor who could finally diagnose the problem and to my Heavenly Father for cutting me a break. I am looking forward to a healthier life and for the chance to begin our family by some means undecided by us and the Lord. If you are interested in the details of the most challenging physical, mental, and emotional struggle I have ever faced, please read on….
After 5 days in the hospital, 15 shots, 3 IV's, 3 new incisions, 2 suppositories, 2 catheters, 1 epidural, 1 stent, and lots of medication… I have finally returned home. This experience has been the most challenging physical trial I have ever been through. I went into the surgery prepared for what I knew would be the hardest recovery I would have to face thus far, but nonetheless, I was optimistic! I was optimistic that I was young and healthy and that my recovery would surely be quicker than most. I had no idea just what kind of wake-up call I was in for.
Everything done surgically went just as planned; it was the recovery that would prove differently. Moments before I was taken into the operating room, my doctor strongly recommended that I get an epidural to control my pain during recovery. I am not typically afraid of needles or shots, but I have to admit I was completely beside myself with fear! They gave me a sedative to calm my nerves before giving me the epidural, but I don't think I ever really calmed down until it was done! Dave commented that I wouldn't shut-up and I kept making random comments. The nurse assured him I probably wouldn't remember any of it when I woke up. It wasn't nearly as bad as I think I had made it up to be in my head (the IV's were definitely more painful than the epidural).
My five day hospital stay seemed never ending. My nurses during the day were less than great. The night nurse's seemed to be the most prompt with my care. They came in every few hours to check my blood pressure, pulse, and temperature, so as you might imagine, sleep was seldom and interrupted. Saturday was a difficult day as I couldn't keep anything down. Vomiting is never fun… but vomiting with a large incision in your stomach and 4 tubes hanging from you is horrible. Dave left me for a few hours to go home and take a shower and get some food. During that time they brought me a "wonderfully" prepared hospital meal (no sarcasm intended). It was the first bit of solid food since the operation and I was pretty excited about it. I took it easy but about half way through the meal I started to feel sick. With practically no warning, I threw-up all over my lunch tray. I pressed my nurse call button and informed the nurse's desk I had thrown-up. I figured this would result in someone being sent to help. After waiting for about 10 minutes and trying every attempt possible to not vomit again, I paged the nurse's desk a second time. This time I was in tears and informed them I needed something to throw-up in immediately. After waiting a while they finally came with something for me to throw-up in besides my lunch tray (not that I was planning on eating any more of it anyways). My mom arrived late that evening and dropped by for a quick hello before I fell asleep.
Sunday was the best day I had during my stay in the hospital. Maybe it was because the Lord knew I needed a break since it was the Sabbath. I never threw-up and I was alert and talkative for most of the day. Monday I woke up to more nausea and vomiting and the discouragement that I would not be going home yet. Before the surgery the Doctor said I would have to stay in the hospital for a minimum of three nights but he would judge my stay according to my progress. In the back of my mind, all I heard was three nights, and that is all I had prepared myself for. On the bright side, the doctor ordered my 2nd (urethra) catheter to be removed, which meant one less tube attached to me! The urethra catheter was very uncomfortable, so its removal brought me a much needed smile.
Tuesday would prove to be my most challenging day. After a very sleepless night, my Doctor came by for a visit at 5:30 in the morning. He informed me that it the end of the time clock with what had become a dear friend, as they would be removing my epidural. The rest of the day was a bit of an awaking as I don't think any of us quit understood how much GOOD the epidural was doing at managing my pain. After its removal, it was very clear how much pain it had been covering up. The pain made me vomit and the vomit created more pain! Most of the pain was resulting from bladder spasms. I was warned prior to my operation that they were very painful and similar to contractions. I was having several problems with my super-pubic catheter draining properly as well. They wheeled me down to radiology to do an x-ray of my abdomen. This was to make sure there were not any twists or kinks in the catheter. The doctor also wanted me to try taking my pain medication by mouth in preparation for my IV removal. It was all I could do to try and keep my pain meds down and the anti-nausea medicine never seemed to help.
Wednesday's early morning doctor's visit was filled with many mixed emotions. I received the exciting news that everything looked as though I would finally be able to return home. He ordered my fluids in my IV to be reduced and removed my JP (incision drain) in preparation for my release. The JP drain is like a suction bulb with a LONG tube attached to the end of it. That tube is inserted into my abdomen to drain fluid from the incision site. The bulb is drained several times a day to measure the amount of fluid the incision is producing. The doctor snipped a few of the stitches around the JP which held it in place. He then proceeded to tell me to take a deep breath...
What took place after that deep breath became a sudden blur of some of the worse sudden pain I have ever experienced in my life. I literally screamed in pain during the removal (I am sure I woke up the entire floor at 6 in morning). The tube felt never ending as he pulled and pulled and pulled. I know this maybe a little graphic, but since this is my way of journaling my experience I will share it. As he pulled I could feel chunks of my flesh coming out with the tube. In that moment, I felt as if my insides were being ripped out and every part of me was ready to call it quits. I laid there in complete tears as he assured me that the removal of my super-pubic catheter would feel nothing like that experience. It wasn't too long after he left that the nausea and vomiting returned. I am sure it was due to the state of shock that I was in after the removal of the JP drain. The rest of the day seemed fairly uneventful until my discharge late that afternoon.
The wheelchair ride to the car and car ride home were both "bumpy", but I survived. Coming home was a huge relief, but also an enormous challenge. I then had to adjust to my "stuff" with my new equipment. I had grown to love the adjustable bed at the hospital (it was really more of a love-hate relationship). It made getting up and down, in and out, much easier. The hospital bed was also equipped with adequate areas to hang my catheter bag (or as I like to call it, my pee bag). At the hospital, I didn't have to worry about getting any "gross stuff" (I will leave that up for interpretation) on my furniture, floor, rugs, ect. I have my post-op appointment next Thursday, and I am really hoping the will remove my super pubic catheter (aka: the "un-super" pubic catheter).
We have been so grateful to everyone who has kept us in their thoughts and prayers. We have appreciated the generous meals and visits. I still have a long way to go, but things seem to be healing. My mom has been a tremendous help and I have especially enjoyed her company. I hope it is all downhill from here…
Thursday, April 16, 2009
Life's pivotal moments
Over the course of my life time, I have had many pivotal moments, many new chapters in my life's book. The past year seems to fill my personal pages with such moments. There are so many changes taking place, not only spiritually, but physically, mentally, and emotionally.
After 12 years of being sick (more severely the past 5 years), I finally have answers, but more importantly solutions! My exploratory surgery, done on the 27th of March, revealed just what the doctor was suspecting. He found a left ectopic ureter (please read previous post for further explanation). Proceeding the surgery, they rushed me over to radiology to run tests on my kidney. The function of my kidney would determine what the best surgical option would be for me. The good news... it functions! The bad news... only about 40% of what is should.
The past several weeks have been a whirl-wind preparing for surgery number three, in less than three months. This surgery will be the most extensive and challenging of them all! Estimated recovery time is 4-6 weeks, 3 weeks of which I will have a FABULOUS catheter (I feel like I am 90... what 24 year old should have to wear a catheter for 3 weeks!). The doctor informed me that I was not allowed to drive and I was to stay home while I have the catheter. As if it was some questions that I would be out and about carrying my lovely pee bag around to show off as a souvenir from my surgery. No, I think I am rather content at home with such a prized possession.
Thankfully, the doctor can go over my old 4"scar (from my major surgery from childhood) because I was really not looking forward to adding another to the collection! I guess you won't find me out on the beach in my bikini this year (not that you would anyways). It is hard to imagine that by the time I am recovered, it will be June. I am pretty much out of commission for the entire month of May.
I have been keeping an extensive to-do list in attempt to make sure everything is in order before I go in for surgery. It is now the eve of the big day and the last thing left on my list is to blog about this pivotal moment in my life. This is a moment I have waited, hoped, and dreamed about. I will leave for the surgery tomorrow morning with all my urinary problems and return home with brand new plumbing. It is expected that I will no longer suffer from UTI's and Kidney infections, but only time will tell. Either way, I know this is a new chapter in my book, and one that I hope will be much healthier. It is a pivotal moment that I hope will lead to a chapter on motherhood.
Thursday, March 19, 2009
Sick and Tired...
Last night I went to the Wal-Mart Pharmacy to pick up a prescription for another (sigh) UTI. As I approached the counter, the pharmacist, quickly recognized me and attempted to guess my last name. Thankful, she got it wrong, otherwise I think I would have cried (okay, so that would be a little over dramatic... but you get the point). She did reassure me that one of these days she was going to have it memorized. Just why would there be a need for her to memorize my last name... 'cause I practically live at the place these days!
On Monday I will go in for surgery number two, in less than two months! I am finally working with a urologist who is actively seeking a solution to my problems and not just treating my symptoms. It will be an exploratory type surgery to get a better look at my urinary system. It is his impression that I could potentially have an ectopic left ureter. Your ureter is what carries urine from your kidneys into your bladder (you have two, one on each side). If it is ectopic, it means that it has an insertion point other than the bladder. All of my tests, procedures and symptoms, thus far point to this diagnosis, but as I have come to learn, there is no guarantee this is what they will find.
Of all the uterine abnormalities, a unicornuate uterus is most often seen with some sort of renal (kidney) anomaly as well. For many patients diagnosed with a unicornuate uterus, it is discovered that they only have one kidney or a misshapen kidney. Thankfully, I have both kidneys, but it is sounding like I could have some abnormalities in other parts of that same system. If an ectopic ureter is found, there are two solutions. The first and most appealing, is if my kidney is found to be functioning properly; they can just re-route my ureter. The second scenario, would be if my kidney is found only producing water instead of urine. If this is the case, they would have to remove my left renal system (kidney, ureter) entirely.
I never would have imagined that the health problems I have endured would be so closely related and stem back to one simple diagnosis of a unicornuate uterus. For the majority of my life, they were treated as individual issues/symptoms. It is now clear that it needs to be treated as one whole condition with multiple medical issues stemming from it. I have adjusted to being the science project to many doctors as they observe my rare medical scenario.
I have to admit I am almost hopeful that it is an ectopic ureter. It seems a little strange to hope for such a diagnosis as this, but it would mean an answer and a solution. An answer to so many frustrating and wearing health issue. It would potentially mean no more UTI's or kidney infections. I have imagined the doctor informing me of the diagnosis and there being tears of joy rather than of sadness. Joy that I might not have to live the rest of my life being "sick and tired". Joy that we may soon be able to start IVF treatments, and thus our family, without another odd fighting against us. It seems to be a dream worth dreaming...