In The Beginning...

Ok.. Here is a little background on my PKD Journey and an update for everyone who has been asking.. (Originally posted on my Myspace page November 2007) Christmas 2005 changed my life forever.. I was diagnosed with Polycystic Kidney Disease. Yeah yeah, whats that right?! Well it is a disease in which cysts grow on your kidneys and eventually they take up all the good tissue and cause the kidneys to stop working. It is the most common life threatening genetic disease out there, yet nobody seems to know much about it. Most people are diagnosed in their 30's and 40's when symptoms start to appear (most commonly high blood pressure) and extensive testing is done to find the cause. It is a hereditary disease so people have this from birth but generally it does not cause any problems, hence why it goes undiagnosed for years. Go figure with me it would cause problems.. I was diagnosed after having a fever, lots of pain and a high white count for 5 days straight with no relief from IV antibiotics. My doctor thought it was appendicitis so a CT scan was ordered.. Much to our surprise, my appendix was fine! But I had a huge cyst on my right kidney that was infected (it later ruptured). Come to find out there were a lot of large cysts, on both kidneys! I was immediately sent on to a specialist for the initial tests. My BUN/Creatnine levels looked good which meant my kidneys were functioning well! Great news! I was told that it was a slow progressing disease and we would just follow up every year with an ultrasound and lab work to watch the progression but no worries! I would follow up as needed in between when signs of infection were present.. I went on for almost 2 years with hardly any problems. An occassional infection that antibiotics cleared right up. I had occassional pain that became very tolerable over time. Now back in August I started having problems with my heart rate.. I was constantly tachy (faster than normal heart rate). I also was having super high blood pressure.. Now I normally (even during pregnancy) ran 118/68 which is great! I was now running 160/110 sometimes higher! On top of it I was having constant headaches and hot flashes. After talking to a few doctors and having blood pressure meds not kick my symptoms I was referred to Mercy DSM to Dr Smith.. An amazing nephrologist! My first appointment with him went wonderful, for the first time ever I had a Dr who knew about PKD and had taken care of several people in the past that have it as well! What a relief that is.. His initial plan was to do a full work up, bloodwork, CT, Ultrasound, etc.. And because my symptoms were coming in spurts and not constant.. He decided to check my catecholmine levels (a test that is rarely ordered). It came back at 2400!!! The normal range is like 60-400.. Holy Cow!! He very quickly diagnosed me with a Pheochromocytoma (this thing only occurs in about 10% of the population).. A tumor that releases catecholmine hormones causing high bp, fast hr, ha's, hot flashes, etc! My kidneys were ok!! And this tumor.. once found, you can surgically take it out and all the symptoms are gone! No more blood pressure meds!! Wahoo! Of course this all sounds too easy, and we all know I'm not that lucky :) I went to Des Moines for 4 days.. I was injected with radioactive dye and multiple scans (MIBG scans) were done to locate this problem-some tumor! It came back negative.. They can't find it. My labs were repeated and still elevated so the diagnosis remains but now we just wait.. The plan is to repeat the scans in a few weeks hoping the tumor grows and is visible on the scans. In the meantime, I am going to see a cardiologist to make sure nothing is wrong with my heart and we didn't miss something. In patients with PKD a Mitral Valve Prolapse (a valve in your heart) has been noted.. The problem with the uncontrolled high blood pressure and fast heart rate is that it kills your kidneys, now when you already have other things killing your kidneys you don't want to add to it.. It is terrible for your kidneys, heart and many other organs! Scary stuff when you are 20.. So I had a follow up appointment this week with my nephrologist and everybody is asking for updates.. We changed my meds up.. Instead of Metoprolol.. I am now taking Coreg (It is working GREAT so far), it will hopefully block the large amounts of Epinephrine in my body and thus reduce the strain on my heart. We are also discussing an experimental drug that is showing to slow the progression of PKD but of course we can not start this until my blood pressure and heart rate are under control. I am hoping to start it around Christmas.. He has started to mention transplant options but feels this drug will be very effective and no action will be needed towards that long process.. That in itself is a scary thing to start thinking of, initially I was told I would be 60+ before that decision needed to be made. But my PKD is progressing quicker than most.. I am now just praying to make it another 10 years AT LEAST before that is in the treatment plan.. So far though, kidney function is ok and we just have to treat symptoms as they come.. So there is your update, not a lot of new information.. still waiting on tests but definately can see a light at the end of the tunnel.. At least for this phase of the PKD.. I found a great website for PKD patients.. www.pkdcure.org I have been talking to other people that have the disease and am looking forward to getting involved with the organization. It is important to raise money for reasearch (as there is no cure for PKD Yet, treatment and dialysis is in the future of every person diagnosed) and also raise awareness to the disease itself! It affects alot of people.. It is genetic and does not skip generations.. Therefore if you have a history of High blood pressure, frequent UTI's, kidney stones, etc, Or if you know someone in your family has the disease, you need to get checked out! The testing for the disease is easy.. All it takes is a renal ultrasound and a simple Bun/Creat lab test. PKD has changed my life in so many ways but I am able to stay on top of the disease. Lots of research is being done towards a cure.. Until then we just have to keep fighting.. :)