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 name is Tania, I am 16 yrs old and my doctors believe I have LHPS

My name is Tania, I am 16 yrs old and my doctors believe I have LHPS (although they are still checking for other possible diseases). I live in a private athletic boarding school miles away from my family and am finding it extremely difficult to get through this health issue by myself. Although I know my parents don’t know what I’m going through neither does anyone at my school, my friends, my roommate, or any of the staff members. I feel very lonely and alone at school. The pain is so unbearable and I am always scared for it to return. I’m tired of being curled up on my bed in pain or on the bathroom floor throwing up. I have been to the hospital many times and sometimes the reason for my pain is very obvious (kidney stones) but other times there seems to be no reason for all the pain, and this is when I start thinking I’m crazy or that it is all in my head.

I don’t want to let people know what is going on, although it is hard to hide it. I feel bad for them when they see me sick or and pain and are unable to do anything, they are unsure what to do to help (there is nothing they can do). I have let this disease run my life, not because I want it too but the pain limits what I can and cannot do. And I don’t want to involve my friends because then I’m dragging them into a problem that’s not theirs and a burden that they should not be carrying. But at the same time I wish there was someone there to hold me when I shake and cry in pain.

ABSOLUTELY NO ONE UNDERSTANDS THIS. My friends don’t understand, they’re telling me to be more positive but it’s so hard to be positive when you’re in so much pain and have gone through so much. If they want to try my life and see if they can be positive at all then they can switch lives with me. I try very hard to be positive all the time about my health situation but sometimes its too overwhelming and I break down and cry. I just wish there was someone who understands. Can you help me?

Kids with LPHS

Hi,

I live in NY and have an 11 year old in the process of getting diagnosed with LPHS. Her doctors are great but they do not know much about the disease and she is in constant pain and in and out of the hospital for pain management.

She misses a lot of school and it does interfere with her social life. It is very frustrating as a parent to see your child in chronic pain! Does any know of any doctors in NYC that are familiar with LPHS?

For parents whose kids are diagnosed with this, what have you been doing to support your child?

21 Years with LPHS

My Name is Craig Silko. I have been suffering from LPHS since I was 12 years old. I was actually diagnosed when I was 17 because it took so long to rule out every other possible medical reasoning behind the constant pain and blood in urine. I have been treated with many medications but none actually rid the pain completely.

Under the advise of my first surgeon (Dr. John Marks, NYC), I went to a surgeon (Dr. Zelik Frischer, SUNY Stony Brook, Long Island, NY) to undergo a Renal Autotransplantation. Dr. Frischer performed the surgery in May of 2000 when I was 13 years into the syndrome.

He was still optimistic, even though it was most effective if performed before 5 years, and I agreed at this point what did I have to lose. The surgery didn’t go as planned and I had many complications. I really don’t want to relive that moment, so I am not going to write about it. The surgery ended up being a success for about two months. Two months of complete relief and I was in heaven.

It came back just as it started many years before that, this time only pain where my kidney was transplanted, right side-lower abdomen-just below my right kidney but in front. We thought that it could be my other kidney developing the condition but we didn’t want to take any chances with scoping, the so far symptom free, kidney. Till this day, now age 33, I do not have pain where my right kidney is, just in my lower abdomen in the transplant location.

I am still treated with pain management services, which my doctor (Dr. Robert Vincent, SUNY Stony Brook)is my angel. Since 1998 when I started going to him, he has developed a plan which keeps me out of the emergency room. On a side note, I don’t know how many of us out there who have gone to the ER have been treated like drug addicts and disrespected; due to the fact that LPHS sounds like a made-up disease.

I ended up filing many complaints about the staff of my local ER. I hope that many of you never had to go threw that. Well back to pain management.

I am able to work and live a somewhat normal, well my normal, life. My pain management Dr’s plan helps me get threw my day without the extreme pain but a liveable amount of pain. I am so use to it that I can ignore it. Its just when I get, what I refer them to as, attacks (Severe untreatable pain) that last sometimes a day to my longest of two months that it gets harder.

Believe it or not, I still work when I am like this. I treat it the best I can, because I know what it is. I do not do more than I can, I don’t go to the ER, I just wait it out. I guess to some my story up is I understand how everyone feels. I don’t recommend getting an Auto Transplantation unless it is your last resort, even then I still wouldn’t recommend it. My surgeon wont perform them anymore because he feels it not the cure all that it was suppose to be.

My one question I pose to any of you, Does anyone have any other ways they control the pain, or the anger that comes with the pain?

If you do please write me at [email protected]

Living with LPHS

I am a 43 yr old female with a long history of kidney stones going back 18 yrs. 2 yrs ago I started having chronic left sided kidney pain with the same type of symptoms as the stones however no stones. It has taken me the full 2 years to convince the medical community I am not a drug addict nor am I suffering from a mental disorder of any kind. I am currently being treated by the pain clinic in my area and along with oral narcotic medication I am getting injections into the nerve area around my kidney with long acting anestetic medication. I am not free of pain and still have much breaktrough pain. I feel at a loss here as no one seems to have a long term solution. I would welcome any help anyone can offer.

Carolyn

lost in pain

hi I’m 21 years old and 5 weeks ago i was diagnosed with lphs. it took the doctors 5 years and 17 ops to finally diagnose me.

i don’t see how doctors can leave a human being in pain as its against there Hippocratic oath. if i was an animal they would put me down as I’m a human i have to live with the pain but i don’t think i can.

i am looking for people who know what i am going through because i need to talk to them . right now there is no light at the end of the tunnel.

LPHS and Acupuncture

Hello everyone,

I was wondering if any of you have tried acupuncture for the treatment of pain and bleeding in regards to LPHS. If you have, I would like to hear from you on whether you had success or not. I have had 5 treatments where is she trying to focus on the kidney itself. It is still hard to tell if it is working or not. Any help would be greatly appreciated.

Hoping to hear from someone who could be helpful.

Helene

Loin Pain Hematuria Syndrome (LPHS)

Loin Pain Hematuria Syndrome (LPHS) is an extremely rare disease, where there is unexplained flank pain and Haematuria. Often misdiagnosed as a hypermobile kidney has the same symptoms. This is poorly understood condition for which specific diagnostic criteria are lacking.

Here are some facts about LPHS:

  • LPHS was first identified in England in 1967 among women who were taking birth control pills.
  • Approximately 90% of the reported cases of LPHS are female.
  • LPHS was first noted in men in the 1980’s.
  • LPHS was first noted in the USA in the 1980’s.
  • The first renal autotransplatation for LPHS was done in 1982.
  • Cases of LPHS have been reported from England, Canada, USA, India, Australia, Ireland, Scotland and Germany.
  • The age range for LPHS is from 6 to 50 years.
  • There are approximately 200 cases of LPHS in the world; no doubt, there are more undocumentated cases.
  • Pain attacks due to LPHS can occur several times per week, every few weeks, or every few months. These pain attacks can last from hours to weeks.

    Here are some guidelines to help you decide if you think you need to investigate this problem with your doctor.

  • The condition is mainly characterized by intractable pain involving the kidney and abdominal areas.
  • Microscopic blood or blatantly obvious amounts of it can be seen in the urine. Clot retention can also occur.
  • It is extremely important that physicians recognize the need for adequate pain medication for this disorder.
  • Forcing fluids by mouth or I.V. are very helpful in decreasing the hematuria and in reducing the risk of clot retention.
  • Be prepared to see several doctors and yield to psychiatric exams before you get a correct diagnosis. This is common with any disease that is not common, such as diabetes. LPHS has only been diagnosed in a few hundred people around the entire world, so this is one disease that is not likely to pop into a physician’s head.

    related articles

    Department of Surgery, Royal Prince Alfred Hospital, Sydney, NSW, Australia. [email protected]

    Some patients with the loin pain/hematuria syndrome suffer incapacitating flank pain. No effective therapy has been reported. Uncertainty persists concerning the authenticity of the pain and the role of surgery in treatment.

    Forty-six patients with loin pain/hematuria syndrome and intractable pain were evaluated following treatment either by renal autotransplantation (30 patients, 10 bilaterally) or by renal denervation (20 patients, four bilaterally) over a 13-year period.

    All patients had concomitant renal nerve excision and ligation and capsulotomy. There were 37 (80%) women and nine men aged 18 to 61 years (mean age, 33 years).

    Excretion urography and angiography were normal in all patients. Nineteen of 25 (76%) patients in whom renal autotransplantation was successfully accomplished and who completed a follow-up questionnaire were free of pain, including eight of 10 with bilateral procedures.

    The follow-up periods ranged from 1 to 13 years (mean, 8.4 years). Six patients have been free of pain for 10 to 13 years.

    Of 18 patients treated with renal neurectomy who were available for follow-up examination, 12 (67%) developed recurrent renal pain, including four who had pain relief on the other side following previous renal autotransplantation.

    The follow-up period for these patients ranged from 6 to 9.9 years (mean, 8.0 years). Three of four patients with recurrent renal pain following neurectomy were treated successfully by renal autotransplantation.

    The loin pain/hematuria syndrome is a rare cause of incapacitation, predominantly of relatively young females. The pain of the syndrome is organic.

    Renal autotransplantation achieves pain relief in three quarters of patients, but the procedure is often (30%) required bilaterally and has significant complications.

    Renal neurectomy is followed by an excessive incidence of recurrent renal pain.


    What is Loin Pain Hematuria Syndrome?

    by Marc F. Brazie, MD
    Division of Nephrology
    University of Maryland Medical Center, Baltimore, MD

    What is Loin Pain Hematuria Syndrome (LPHS)?

    LPHS is a rare kidney disorder causing attacks of severe flank pain and blood in the urine.

    Who gets LPHS?

    LPHS was first reported in three patients in 1967 who were found to have recurrent attacks of severe flank pain and blood in the urine (hematuria) in whom no other explanation could be found [2]. Since that time, it has been reported in several hundred people worldwide, although it is likely that there are many more unreported cases. It is more common in women (70% of all cases) [3-5], and has been seen mostly in the United States, Great Britain, Australia, and Canada, suggesting a white predominance. While it can start at almost any age, most patients will first develop symptoms in their 20’s [6].

    How do I know if I have LPHS?

    The predominant features of LPHS are recurrent flank pain and blood in the urine. The pain can occur on only one side or both, and may radiate to the abdomen, groin, or inner thigh. Pain episodes may be associated with low-grade fevers and a burning discomfort with urination. The blood in the urine may be overt (called “macroscopic hematuria”), or only detectible on a urine dipstick test or by examining the urine through a microscope (called “microscopic hematuria”). Up to 47% of patients with LPHS also have a history of kidney stones [7], but stones must be absent during pain episodes for LPHS to be diagnosed.

    What causes LPHS?

    As LPHS is a rare disorder, not much is known about the underlying causes. It is thought that there may be a problem with the glomerular basement membrane (GBM), which is the blood “filter” in the kidney, causing bleeding into the kidney tubules. These tubules can then become blocked by the blood cells, causing swelling and increased pressure in the kidney. This in turn leads to stretching of the outer layer of the kidney, called the “capsule,” and pain. Indeed, studies examining biopsies of patients thought to have LPHS have shown that in over 50% of patients, the GBM is abnormally thick or thin [7].

    What should I do if I think I might have LPHS?

    There are many disorders that can cause flank pain and blood in the urine, and as there is no specific test to confirm LPHS, it is considered a diagnosis of exclusion. It is very important to have a thorough evaluation by a kidney doctor (nephrologist) to be sure there is no other cause for the symptoms. This evaluation may involve laboratory tests, radiology studies, or even a kidney biopsy.

    What can be done for LPHS?

    Again, as LPHS is a rare disorder, it is not clear what the optimal treatment is. What is known is that patients with LPHS tend to have normal kidney function, which does not deteriorate over time. Thus, treatment is aimed at pain control and improvement in quality of life. Multiple therapies have been tried, all with mixed results. These include:
    • Medications such at angiotensin-converting enzyme inhibitors (a family of blood pressure medicines which reduce the pressure in the kidney and have been shown to be of benefit to some patients [8]), non-steroidal anti-inflammatory drugs (such as ibuprofen, naproxen, or ketorolac), and narcotic pain medications.
    • Nerve block procedures to decrease the pain signal from the effected kidney, and
    • Surgery to strip the nerves from the kidney, “autotransplant” the kidney (in which it is removed from its normal position and reinserted in the lower abdomen – this has been shown to relieve pain in up to 70% of patient in some reports [3,4]), or in extreme cases, remove the kidney altogether.

    It is best to discuss all the treatment options with your doctor, and ideally as part of a “multidisciplinary” approach involving primary care doctors, nephrologists, psychiatrists, and chronic pain specialists.


    References
    1. Dube GK, Hamilton SE, Ratner LE, Nasr SH, Radhakrishnan J. Loin pain hematuria syndrome. Kidney Int. 2006; 70: 2152-2155.
    2. Little PJ, Sloper JS, deWardener HE. A syndrome of loin pain and haematuria associated with disease of peripheral renal arteries. Q J Med. 1967; 36: 253-259.
    3. Sheil AG, Chui AK, Verran DJ et al. Evaluation of the loin pain/hematuria syndrome treated by renal autotransplantation or radical renal neurectomy. Am J Kidney Dis. 1998; 32: 215-220.
    4. Chin JL, Kloth D, Paulter SE, Mulligan M. Renal autotransplantation for the loin pain-hematuria syndrome: long-term followup of 26 cases. J Urol. 1998; 160: 1232-1235.
    5. Greenwell TJ, Peters JL, Neild GH, Shah PJ. The outcome of renal denervation for managing loin pain haematuria syndrome. BJU Int. 2004; 93: 818-821.
    6. Weisberg LS, Bloom PB, Simmons RL, Viner ED. Loin pain hematuria syndrome. Am J Nephrol. 1993; 13: 229-237.
    7. Spetie DN, Nadasdy T, Nadasdy G, et al. Proposed pathogenesis of idiopathic loin pain-hematuria syndrome. Am J Kidney Dis. 2006; 47: 419-427.
    8. Hebert LA, Betts JA, Sedmak DD, et al. Loin pain-hematuria syndrome associated with thin glomerular basement membrane disease and hemorrhage into renal tubules. Kidney Int. 1996; 49: 168-173.