Category Archives: LPHS

Real stories of people – who are now living with LPHS. Loin Pain Hematuria Syndrome is a rare, poorly defined kidney disorder characterized by recurrent, persistent loin pain and blood in the urine.

My Story, Gabe 16

Hey Guys, My name Is Gabe, I’m a 16 male from Pennsylvania. I was diagnosed with Loin Pain Hemoturria Syndrome about 2 months ago. It all started last November when I started having the worst lower back pain in the world. It was the worst pain I had ever felt. I told my mom about it thinking it was nothing so we let it go. After about 2 months of this I went to an Orthopedic doctor who thought I had a hairline fracture in my spine somewhere. I got a bone scan along with several other texts for the Orthopedic’s. Unfortunately I had no bone damage. After that appointment trying to figure out what to do next I started urinating dark brown, that night i was checked into the ER. I got to leave that night diagnosed with an UTI, wrong again. Then we were referred to an Urologist. By now it was about March or April, the entire time i had been in daily pain with no pills giving me any relief. The urologist then sent me to the nephrologist . Our first appointment she scheduled tests for me and sent me to the Chronic Pain Clinic in my area. The doc gave me a steroid injection of some kind. All it did is put me in worse pain. He sent me on my way until 2 weeks later. By now I had gotten annoyed because it wasn’t until late May that I got an appointment to see the nephrologist again . I had several tests done by her, one involving them putting a catheter down my neck to check the pressure levels on both sides of my back/ kidneys. That was the first test that had showed any indication there was something actually wrong with me. My doctor then called me back in to tell me the news. I then found out I had LPHS, I had no idea what that meant, and I didn’t care. She said she would talk to a cardiovascular Surgeon and give me a call back when she had everything figured out. By now it was about Early July. Between this time i had been given Vicodin and that did nothing for me, some sort of a muscle relaxer, and Tramadol. Soccer season was coming up my appointment with the surgeon was for late August, another month of waiting. Shortly before the appointment the Chronic Pain doctor gave me an anti-seizure medication called Gabapentin, apparently it was supposed to work. All that did was put me in a very bad mood. I had stopped taking it and I was just waiting for my appointment with the cardiovascular surgeon. The day came and I met with Doctor. Atnip of Hershey Medical Center, apparently this guy was one of the best. When I got there a resident nurse came in fascinated asking me so many questions about LPHS, it was like I was a science marvel. Then Doctor Atnip finally came in with another resident who just stared at me in awe. Then he told me all my options to possibly fix this, waiting was the first thing he said. Which was not an option for me. After just a year of this i had worn on me so much and I just need it to be done. He talked to me about doing some sort of bi-pas by taking part of a vein from my leg and bi passing the compressed vein of leaving my kidney. He was against putting a stent in the vein that needed to be fixed. So as of today September 18th I just got a vein mapping done to see if my veins would even work for the surgery. When the nurse said they would I felt some hope finally. Now Doctor. Atnip had talked to my nephrologist and they both feel this is the best option, and apparently due to my body weight and type this surgery had a good chance of success. After reading up on LPHS a bit i discovered what other patients were taking to manage pain, so on the 20th i have another pain appointment, hopefully to actually get something that works. The only thing that worries me is it seems like no one actually gets rid of this or fixes this. But I’m hoping for the best. If anyone has any advice of surgical experience, pain advice, anything i would be more than happy to hear.

19 Year Old Suffering for the Last Seven Years

For the past seven years I have been in and out of hospitals, tests done, specialists seen, and surgeries done to help me with pain. It took me about 5 years to actually be diagnosed with LPHS. I was diagnosed my senior year of High School, along with RTA (Renal Tubular Acidosis), they say this is a primary disease to my LPHS but who knows if they really know what they’re talking about. I was involved in absolutely everything you can imagine in school. I was a two year dance officer, yearbook and newspaper writer, academic UIL team, national honor societies, and even homecoming queen ect. My pain got so bad that I was hospitalized for a week during New Years of 2011. With the grace of God I was able to push through and actually graduate high school in June of 2011. I couldn’t have done this without my amazing pain specialist Dr. Jeffery Charnov in Houston, TX. He made me feel that he knew I wasn’t making up my pain. I took a pain patch to be able to attend school. The pain patch (Fentyal) slowly went into my blood stream and maintained a constant level of pain medication to where I was able to function everyday, although I was still in pain. The pain patch takes the edge off of the pain and still allowed me to drive. Other pain medication I took would drain my energy and I couldn’t function day to day. I then found out I could get a nerve block (Celiac Plexus Block) which is a procedure done under general anesthetic, where my pain specialist injects a local anesthetic mixed with a small dose of steroids. This procedure is an outpatient one, and I can usually function after a couple of days. The pain afterwards is due to swelling in the back and you receive a series of injections to allow the affects to last longer. I usually receive two injections back to back within two weeks of each other. This is the only treatment that has given me significant relief. The longest I went without pain medicine was about two months. Over the past year I have received eight injections and I just had my eighth injection a few days ago. The injections usually lessen my pain from about an 8/10 to a 5/10. I also get kidney stones too which just makes the pain that much more excruciating. I had a Lithotripsy surgery done two months ago to break up a stone I had. I just finished my freshman year of college although it was hard to balance school with my pain, I managed to not give up and not let LPHS Get the best of me.

Keep fighting! Don’t ever give up, we’re too strong.

If anyone would like to chat, I’d LOVE to! You’re not alone and you need someone who can understand where you’re coming from!

Most Helpful Treatments

For most of my years with Loin Pain Hematuria I kept ending up in the hospital because of uncontrolled pain. I was probably prescribed every narcotic possible … tried many different types both pills and patches. It was not until my regional hospital started a subcutanous IV lidocaine pain program , combined with methadone that I finally found life manageable three years ago. Lidocaine is used to treat many different types of pain. Every person is different. Some can stop using narcotics, others can reduce their amounts. For me it meant that I stopped using short-acting narcotics on a regular basis, instead only an infrequent as need basis. I am on 18mg of methadone. I was on 1080ml of lidocaine. I have been able to raise my 3 children. But ….. chronic pain is always a series of trade offs.

My hair has started to fall out and in the past 18 months I have been exhausted. The exhaustion is deep and debilitating. I have been working with my family doctor, naturopath, pharmacists, nurses at the pain program, ob/gyn, and dermatologist to figure out any answers. But my own research has proved to be very important. I have learned that lidocaine is being researched in cancer studies. It is an EGFR inhibitor. This is the same type of drug used in chemotherapy that causes hair loss. It works on very basic cellular production. Then I found out that methadone is more effective than other narcotics because it works on all 3 pain receptors, rather than just 2 two. But it will cause chronic adrenal fatigue, hence the exhaustion. Working with my naturopath, hopefully I can feel healthier again with supplements, nutrition and reductions in Rx dosages It is very important to use the Internet to research all medication recommended to you.

LPHS Journey

I am 47. My first signs of a problem was at age 19 with high blood pressure. Then at age 27 with blood in my urine thru-out my pregnancy. Next at age 33 at conception of my third pregnancy with a very dangerous bacterial strep infection and pain mostly in my back. Then at age 37 I had to have emergency surgery; my kidneys could not handle it. I got a kidney infection. And then all kinds of pain and blood in my urine. My blood pressure was out of control. A ureter stent was put in, but something went wrong because it caused agonizing pain. It was taken out. I required more surgery that found shards of the stent in my ureter. That relieved some acute pain, but I still had kidney pain and blood in urine. By the time I was dealing with the shards of the stent about 20 months of agonizing pain had occurred – I was ready to die. The only thing that stopped me was my three children. My mind could resolve that my husband would want me at peace, but I could not reconcile how my children could live without me. As I researched suicide methods I found 1 website for LPHS & a friendly voice in Ohio helped me. She connected me to doctors at Ohio State University. I also went to a teaching hospital in the largest city in my province. (Canada) The teaching hospital was a smart idea! This type of hospital loves hard to figure out medical problems. I finally got good care & thorough diagnosis + start of a pain management plan. I also went to Cleveland Clinic in Ohio because it is considered the #1 Urology & Nephrology Centre in the US. It cost $5000, but was worth it. When we add up all lost wages in/out of Cdn hospitals with out diagnosis, this was cheap. Now there is more knowledge about LPHS.

LPHS and Endometriosis

Yesterday I was diagnosed with LPHS as a 28 year old female. In 2007 I was also diagnosed with endometriosis and have since had 4 surgeries to remove nerves and the endometriosis that has grown rapid in the right side of my body. I always had kidney pain, on the right side, that I just figured was pain that radiated due to the endo. However, after having had another surgery in October, I ended up back in the hospital with severe kidney pain. Again no stones or signs of a UTI, so the easy answer for my doctors was to blame the endo, put me on meds and send me home. This time the pain has gotten worse and even the medications are useless. I just got back to work from having been on leave and I was in tears yesterday from all the pain in my back. I finally went back to my primary doctor who also specializes in neuphrology and due to my urine cultures and symptoms was diagnosed with LPHS. My question to him was, what do I do? The look on his face was very alarming as there isn’t much to be done. He knew of some patients that were placed on ACE inhibiters but my blood pressure is super low so I’m not eligible for such option. I’m looking for guidance as I want to know if anyone else has the combination of LPHS and Endometriosis (Stage 4). Also, suggestions on what to do and how to make my quality of life better. I’ve been on narcotics before for the severe endo pain and I really don’t want to go down that same road. I feel as though my pain reacts to nothing, so why bother being on something when I’m going to be in pain anyways. I’m also scared that since I’ve been in the hospital so many times I may be labeled a “drug seeker” as when I enter the first thing they do is give me dialudid through the iv to try and take the edge off, which is prob the only thing that helps but I can’t live on that! AND the iv works but when I took the meds, it helped very little and keep in mind this is extremely heavy narcotics. I’ve also gained 40 lbs in the last year, I was very skinny to begin with, but both diseases have made me feel self concious and I barely eat because I’m so very nauseated. I feel I’m way too young to give up. I’ve dealt with the endo and will deal with this just as well. I’m just at a loss on what to do next, some said (including my doctor) a possible kidney biopsy, pain management, accupuncture, excersie but I’m just exhausted and don’t know how to handle all of this. HELP!

Warm Regards,
J

My 18yr old Son Diagnosed with LPHS

Since he was about 14 years old we have been trying to find out what is wrong with our son. He is now 18 years old and we have finally found out what is wrong. He is an athlete and favorite sport is football. He has been living with this severe, chronic pain for soo long. Many nights i cry myself to sleep with worry. Right now we have not found relief. Ultram will sometimes help with the pain but not always. Our nephrologist is sending us to Dallas to see a doctor with more experience with this syndrome. I can only pray that we have some luck and find a way to end my son’s constant agonizing pain. Any suggestions on dealing with this chronic disorder would be very appreciated.

Caretakers -Parents Perspective Living with Life in Pain

Several years ago our daughter Sarah began to consistently complain of stomach pain which radiated to her back. Doctors were unable to diagnose it, and eventually dismissed her pain as a teenager’s attempt to get attention from her parents.

Sarah went to several doctors before we found Dr. Henry Rosas. After one careful examination, he found that she had several symptoms that indicated her gallbladder was not working. This confirmed the results of a Hida Scan that had been administered following an emergency appendectomy during Easter a year prior. The scan showed not only that Sarah’s gallbladder was not working but also what looked like a large mass of what might be cancer. When our daughter was taken in for surgery, Dr. Rosas noticed the previous doctor had not sealed an area of her appendix. This had compressed several inches of her small intestines into a large ball. He decompressed the tissue and reattached what should have been done a year prior.

Sarah was fine for a few short months, but gradually her pain began to increase and her blood test showed that she may have an immune deficiency. Dr. Rosas requested for Johns Hopkins to see her, but approval for the appointment was denied by the head of the Autoimmune Disorder/Deficiency Department.. He felt that there was not enough evidence in Sarah’s medical records to warrant an appointment with their world renowned doctor.

Unfortunately, Dr. Rosas died from cancer. Sarah was referred to a female doctor at GBMC, but we were met again with questioning looks and an overall lack of information about the cause of her health issues. She was in an out of the ER so many times, each time with the same pain near her kidney, and yet none of her doctors referred her to a nephrologist. As parents, we were fed up. It made sense to me to send her to a nephrologist, and if her physicians were not going to recommend someone, then I would find one myself.

Our prayers were answered when God sent Dr. Marc Brazie to our rescue. Dr. Brazie was dedicated to Sarah and took the time to understand her, support her and most importantly believe in her. He did his own research, own blood work, own scans, kidney biopsies etc. After examining Sarah’s myriad of symptoms–hematuria in her urine and renal tubes, stabbing pain in both kidneys (she described it “as if many snowflakes were poking around”) passing two kidney stones, and a double biopsy, he diagnosed her with LPHS – Loin Pain Hematuria Syndrome. We were happy for a short while just to know what we had, but the rarity of the disease was overwhelming.

I contacted the Rare Orphans Disease Foundation in Bethesda only to be told there was no research, no funding and no interest in LPHS because there were less than 1,000 patients with this disease. After countless calls, we pleaded with our friends to find a doctor that would be able to lend a hand. Finally, our friend Ellen Potapane told us how their physician, Dr. Kevin Ferentz, had helped her husband, Frank, when he had been diagnosed with a rare form of cancer. At the time of diagnosis, Frank Potapane was a neighbor and good friend of ours. We had joined forces with them during their health crisis and Frank had survived; we were grateful that now they were referring Dr. Ferentz to us in our time of need.

Dr. Ferentz is currently one of the heads of the Department of Family Medicine at the University of Maryland, and has been part of our family now since the beginning of our daughter’s diagnosis. He cares about the individual patient, believes in them and works with the whole family. For the past several years, his team of doctors and students have been learning about this rare disease, and I am thankful that God sent him to us.

Unfortunately, not all students are as eager to research LPHS. Some take the time to learn, while others question the disease because of the lack of information available. While Dr. Ferentz has been amazing through his efforts educating medical students, the bottom line is that students have to be willing to take the time to learn or no progress in LPHS research will be made.

In my mind, a lack of interest is a major problem affecting medicine today. Students and doctors need to take the time to learn and support their patients with sincerity. They need to be less worried about their own credibility and more supportive of their patients themselves. Dr. Kevin Ferentz and Dr. David Stewart are two doctors that truly support their patients. Our hope is that the others will learn by their example.

As happy as we were to finally have caring physicians, Sarah’s medical problems weren’t solved. As a Mom, “no answers” is not a phrase in my vocabulary. “Can’t” doesn’t exist and neither does “there is nothing more we can do”. So instead of sitting around doing nothing, our family held a fundraiser at the Engineers Club in Baltimore. We were fortunate to have many supportive business associates, friends and family to help raise money for the Dept. of Family Medicine – Community Medicine at the University of Maryland. Our hope was that this funding would help the department to fund LPHS research, which in turn would help our daughter. At the time, there was no LPHS research being conducted at the University of Maryland at all.

In the meantime, Sarah was still in and out of the hospital. She had started seeing a pain therapist, Claudia Cameron, an amazing woman that is always there for Sarah. Claudia helped Sarah through her crisis and supported her in spite of some individuals that didn’t believe in her because of the lack of information about LPHS. Through her wonderful support and friendship, Claudia has helped our entire family adjust to Sarah’s illness. Through networking, I also had the good fortune to meet Bella Folksman, an acupuncturist in Owings Mills Maryland who has also been a major part in Sarah’s pain management. Sarah tries to see her once a week…unless she is in the hospital. From a parental perspective, Bella is a hands-on therapist that treats Sarah with an attitude of true sincerity and caring.

We finally did get into Johns Hopkins, and their nephrologist, Dr. Michael Choi, reaffirmed Sarah’s diagnosis. Dr. Choi also said he believed there maybe another underlying illness. While we were at John’s Hopkins, Dr. Choi spent 3 hours with us talking with Sarah. He wrote everything down specifically and in detail. That effort meant so much to us! He even had someone send us a video of a doctor in Montgomery County that is specializing in a program to treat patients with rare diseases that affect 200,000 people or less. Though Sarah did not get into this program, we hope that the National Instituted of Health (NIH) will have other programs and research that correlate with Sarah’s illness and may find a cure.

We are fortunate that we have a great team of several doctors and therapists that look out for Sarah; Dr. Ferentz, Dr. Brazie, Dr. Claudia Cameron, Bella Folksman, Dr. Bruce Greenwald her GI doctor and Dr. Diane Orlinsky her dermatologist. But though they are very heart-centered and caring doctors, they still don’t have the cure for her illness. We have tried everything we can think of to help Sarah: alternative medicine, contacting friends such as Adele Linsalata, Tracey Hutchins, Shari Sternberger, Kyle Brooks, Tina York – Chaplain, prayers from every denomination you can imagine all in the hopes of helping Sarah and others like her to find a cure or help her manage her pain so she can have a life.

As a parent, the most difficult area is watching your child in pain and finding that there is no cure. As parents, we have been told we are here to protect, reassure and take care of our children. To keep them from all pain, surround them with love and help them to grow into adults. To find their dreams, help them turn those dreams into reality and see it all blossom before our eyes. Though we have seen Sarah try on several occasions to fulfill her own dream of becoming a nurse, thus far it has been diminished. For a time, she was attending Medix School, located in Towson, but they had to take away her admission because of her illness. We are told we still have to pay for her admission even though she is no longer there. The school’s hope is that she will return to classes when she is able. In my heart, Sarah’s ordeal will make her very valuable nurse for any doctor, hospital and patient. She has seen good, bad and horrible nurses, doctors and staff. She has held it together and dealt with illness in a mature fashion by learning grow in her situation.

Sarah tries to understand her illness from both sides of the spectrum, which is something I am not sure many patients have done. I think some of the doctors and nurses that see her appreciate her efforts, though as a parent I will say that sometimes it is very difficult to watch. There are several quotes I share with Sarah: “Never take No for an answer”, “Live Life with a Purpose” “You can reach your dreams” “We are all here for a reason and your journey is going to help you and so many others” Sometimes she can listen and believe in these quotes too, but other times, I can tell that she is so angry because her disease is holding her back from whatever she wants to do in life. As parents, it’s difficult to see others (neighbors, friends, family, business associates) with children around the same age as our daughter. It is hard to celebrate with them on their momentous occasions; graduation, weddings, births and so much more.

I will not lie and say there have not been tears. There have been tears, screaming, anger and more…but then we try to regroup and understand that there are times that are amazing, even if they are just a few hours here and there when we can be an average normal family.

As parents, our job is to keep HOPE alive, as difficult as it can be, and to continue trying to always find one silver lining that can give hope to our daughter and all those suffering with this very painful disease.

My own hope is that by putting this book together, someone out there that will use it as a resource. Someone will gain a greater understanding about rare diseases and understand that those suffering from them are patients, not numbers. I hope that people will see that there are people with feelings; caring doctors and researchers who are not looking for the almighty dollar but who look at these patients and believe they may have the answer to help! And that patients around the world can have the support of family, friends and the medical system to care enough to help them and so many other patients that suffer with rare diseases affecting 1,000 people or less. That people will take the time to CARE! That the families of patients dealing with rare diseases will be helped, because these diseases affect the entire family — the aunts, uncles, cousins, everyone! If you allow it, disease can tear the family apart. But, if you hold steadfast and are hopeful and loving to the patient, (in our case, our daughter) a bond can be formed that cannot be broken. And that is what family is all about—UNITY!

It is with my heartfelt gratitude that I thank you for helping. Thank you for reading this book and supporting those with LPHS – Loin Pain Hematuria Syndrome. Please join with us in trying to find a cure, start research, and help so many patients and their families in need with this rare and life-altering disease/syndrome/affliction.

Thank you from the bottom of our MANY HEARTS!
Tammy, Jeff, Sarah, Josh and Zach – Maryland