Medical Jargon

Now that everything has pretty much been diagnosed, I thought I would take this opportunity to "spell it all out" for you. There have been multiple questions from friends and family on my condition and I hope this will clarify my situation and diagnosis in better detail than I have previously done. It's a good lesson in female development that every female should know and understand. It is also my hope that one day this information might find someone who has a similar situation as me to bring them peace to years of unanswered questions and health problems.

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.

Sick and Tired...

It's official... I am "sick and tired" of being "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...

Pieces

My surgery to remove my right uterine horn (which had no communication with my actual uterus and was suspected to have endometrosis) finally arrived last Tuesday. Everything went well and as planned and I am recovering. This surgery has left me with feelings and emotions that I don't typically allow myself to have. It is as if the surgery just sucked all of the positivity and optimism right out of me. The reality that my body will never allow me to conceive a child naturally has set in more than ever. The reality of how I was born, and what has been taken and removed, and how it simply cannot be fixed or replaced is clear.

I returned to work this Wednesday and to the love and concern of the people with whom I work with. The other designer I work with asked for a better explanation than I had previously given her of just what exactly they did. The best way I knew to explain it was by drawing a picture. I pulled out my sticky notes and drew what a normal uterus looks like and gave a brief explanation of the parts (uterus, fallopian tubes, and ovaries). Then, right below that image, I drew what my anatomy looked like when I was born. That image was followed, by how my anatomy looks now (after two surgeries). When I glanced down at my own drawing, I realized just how pitiful it looked. And not just because of my artistic inabilities, but rather that there really wasn't much there. It was all just a bunch of PIECES, none of which do what they are suppose to do.

I am left with two fabulous ovaries. The right has a fallopian tube to communicate with, but no uterus communication. The left side contains my unicornuate uterus (half the normal uterus), but does not have a fallopian tube to make communication between my uterus and left ovary(my left fallopian tube was removed as a child). Simply put... a bunch of useless PIECES, a scar covered tummy, and no baby to hold and call my own.

Part 2 of My Story

(If you haven't read Part 1, please click here)

Now that I have finished school, the reality that I am not a mother has set in harder than it had previously. When we moved to Arkansas I decided that I was ready to pursue more options and try and find solutions. This past year has been exceptionally hard as I have been faced with more UTI's than in the past and my body hasn't responded the same to the antibiotics as it has before.

Little Rock has a wonderful university medical center that I felt very good about and so I began the process of getting answers. I gathered and requested all of my medical records from the past 13 years of my life so that I would be able to give the doctors a better understanding of my situation. I set up a family care doctor so that I could get referrals to see the doctors at UAMS. At this point, I was finally able to make my appointments. When I did so, I could have never anticipated the wait that would follow. I made appointments with 2 doctors, a urologists and an OB/GYN. The earliest that I would be able to get into either doctor would be over 2 months. A week before my first appointment with the OB/GYN I received a letter in the mail informing me that they had pushed back my appointment for another month and a half. I was so frustrated after waiting so long to find out that I would have to wait even longer. Not to mention the fact that I had been bleeding for over 3 months and I was unsure why. As I explained this situation to the nurse who rescheduled my appointment, she simply said, "well, you have your appointment with the urologist in a few days, see what he says about it". Then to make matters worse, I received a call the day before my urologist appointment to inform me that they would need to move my appointment back one more month. And so the waiting process began again...

When the urologist appointment finally arrived they meant business. It was a long wait to get in to the doctor, but once I was in, he wasted no time in running test, after test, after test to try and get to the root of the problem and to get a clear understanding of my anatomy. He then confirmed, as my childhood urologist had already done, that my urethra was about 1 cm off from where it was suppose to be located. This in turn, directs a small amount of urine into the vaginal cavity and causes a significant amount of urinary tract infections along with a myriad of other issues. He then said that in his 35 years of practicing, he has never seen this situation before and wasn't sure if it would be able to be corrected or not. I then received a referral to see another urologist, as he attempted to comfort me by saying, "if there was a doctor who would have a solution it would be this new doctor". We then went to make the appointment only to be informed that there would be another two month wait to get into see him. This appointment will be December 12th, and I am very hopeful he will be able to repair and correct my urethra.

As for the OB/GYN, I went to that appointment and was again referred to another doctor who specializes in fertility treatment. Luckily this appointment was scheduled just a week and a half following this visit.

My first fertility appointment arrived November 10th, and I could hardly contain my excitement of the possibility that he would have an answer for me as to why I have been unable to get pregnant. Once again he wasted no time in finding a solution, as I underwent a series of tests and blood work. He discovered that I was not ovulating and that is why I had been bleeding for several months. He then ordered and HSG test to determine if there was any blockage in my right fallopian tube (since I only have one) and to get a clear picture of the shape of my uterus. During an HSG test, they shoot dye into you uterus and watch the path of the dye flow out through your fallopian tubes. It is done by taking a consecutive series of x-ray images as the dye is injected. It causes severe cramping for the point of dye injection up to two days later. I had watched a video of a normal HSG test before I went in for the procedure so I would know what to expect.

During the procedure I couldn't see the screen of the X-rays, but once they were done, the doctor sat me up and pulled the screen over to show me the results. The second I saw the images, my heart sank. I knew something wasn't right because my images looked nothing like the normal HSG test I had watched online. I left the hospital that Wednesday with a brief explanation of what the doctor thought might be wrong, but he told me that my fertility doctor would go over the results with me on Thursday. In my heart, I knew the outcome was not what I was hoping for. I spent the rest of Wednesday lying in bed because of pain from the procedure while I looked online for further explanation of what I had seen. There was alot of tears as I read through article after article describing my situation.

Yesterday was the follow up appointment for the test results. Going into the appointment, I had pretty much prepared myself for what the doctor would be telling me. He sat us down in his office and went through everything with us for almost an hour. I was born with a rare uterine abnormality known as a unicornuate uterus. This basically means I have about half the uterus that most women have. My unicornuate uterus has a left horn and a right non-communicating rudimentary horn (as seen in the diagram below, for a picture of a normal uterus please click here).




I have a very healthy right ovary and fallopian tube but there is no communication between them and my uterus. It is suggested that there are several women who have this condition and never experiance any problems from it. It is often diagnosed when a women suffers from repeated miscarriages, pre-term births, or infertiltiy. In my situation it has caused steralization. This is due to the fact that they removed the only fallopian tube that had communication with my uterus because of the damage the tube withstood as child. My uterine malformation was not discovered during my childhood operations because of the initial distortion of the organs from the blood that they held.

The doctor also suspects that I have endometrosis. He has suggested that I undergo a laprasopic surgery to remove my right uterine horn and fallopian tube and the portion still remaining of my left fallopian tube. This will resolve the pain caused from the endometrosis which has built up in my rudimentary horn and decrease the risk of any tubal pregnancies. I am waiting to see if the urologist will be able to operate on me to correct my urethra, and if so, I will have each doctor do their surgeries on the same day.

My options for motherhood are IVF (invitro fertilization), surrogacy, or adoption. If we were to go forward with IVF they would only be able to implant one egg at a time (they usually implant multiple Embryo's). This decreases the chance of the procedure being as affective. They would do this because of the risk involved if all the embryos took and I carried a multiples. Because of my uterine size, carrying just one is like carrying multiples for everyone else. I would be considered a very high risk pregnancy and the chance of miscarriage and preterm births are very high. The babies are usually breeched as well, because there is not enough room for the fetus to turn.

Amongst a lot of sorrow, there is certainly hope, but I have a significant amount of odds to fight. I know that if it is meant to be, the Lord will provide a way. Please keep us in your prayers as we continue are quest to become parents. I can't thank you enough for the immense amount of love and support that you all have shown. I will keep everyone updated as to what we find out over the coming months.