“Gotham City Sirens”
~ by Alex Garner
Today is Loin Pain Hematuria Syndrome Awareness Day.
These are my fellow patients living with this disease. We support each other and do research, since this is an orphan disease. Only about 1,000 people in the world have it. Here is some more information:
Loin pain hematuria syndrome is a rare syndrome. To help illustrate this rarity worldwide there is currently only a couple hundred documented cases. It is believed that since it was first discovered in 1967, there have been an estimated 1,000 documented cases and several hundred unofficially documented cases. It is more common in women then in men; about 70% of cases are women. Theses documented cases are mostly in the United States, Great Britain, Australia, and Canada. 94% of cases are White/Caucasians. Due to its uncommonness and that it was only discovered approximately 40 years ago, there isn’t very much information available regarding this syndrome. The doctors have acknowledged that the pain is not psycho-somatic and that there is blood excreted in the urine (Hematuria), but we don’t know why this happens, where it comes from, or what causes the condition. For this reason, it is extremely difficult to treat a patient with loin pain hematuria syndrome. It is often very difficult to treat something if root of the problem is unknown. LPHS is considered to be idiopathic.
The patients experience pain in the loin area, which is another term for the kidney area. It can also be described as flank pain. It can be felt on one side or on both sides; the medical terms for this are unilateral and bilateral, respectively. This pain can sometimes radiate to the abdomen, the groin ,and the inner thigh (sciatica). This pain is often chronic and excruciating. It can incapacitate the patient. I myself have fainted many times for simply experiencing too much pain. The fainting is a variation on the condition “shock”. The pain can be described differently by different patients. Some feel a sharp stabbing while others feel a dull throbbing. I compare LPHS pain to a kidney stone: having LPHS is like constantly passing a kidney stone. The pain never goes away.
Most patients that experience the pain also have blood in their urine. The blood can be microscopic or it can be macroscopic. The term macroscopic is a synonym to gross and overt; both of these terms are frequently used to name the blood in the urine when it is visible. Blood in the urine is called Hematuria.The blood loss can cause little to no complications to some. On occasion some patients require blood transfusions to make up for the loss. Most develop anemia. It is important to know that on rare occasions someone can be diagnosed with loin pain hematuria syndrome even if they do not have blood in the urine. Diagnosis of LPHS is a diagnosis of exclusion, meaning you test for everything and you come up with nothing, you’re a likely candidate for having this disease.
Often, when the pain worsens the hematuria also worsens and vice versa. The pain can be constant in some patients and come in waves for others. A large percentage of patients feel the pain daily. The same goes for the visibility of the hematuria or its’ comings and goings. Flare-ups can occur often. Flare-ups are how patients and doctors refer to periods of heightened pain and/or hematuria. Some patients feel the pain of flare-ups on a daily occurrence and others on a weekly, monthly or annual occurrence. When a flare-up occurs, they can last from minutes to days. It can calm down simply for it to flare back up in an hour. The intensity of the pain does not seem to follow a pattern.
Some secondary symptoms associated to loin pain hematuria syndrome can be a low grade fever, painful or discomfort during urination, loin tenderness, muscle spasms, dizziness, and tenderness. Some patients lose the ability to urinate all together. I myself must use a straight catheter.
Most patients do not develop kidney stones. If they do, it may not directly be linked to the previous diagnosis of loin pain hematuria syndrome. The kidney function remains optimal and there is no deaths caused by the condition. In my case, I have passed 31 kidney stones, and do have Stage II Kidney Disease.
The age group of people who can have this condition is large; it is between 13 and 70 years. There was one case where a 6 year old child was diagnosed with loin pain hematuria syndrome. Most patients develop their first symptoms in their 20’s. Some doctors are lead to believe that patients eventually grow out of this diagnosis, but that is yet to be consistently proven. I do not know of a single patient who has “outgrown” their LPHS.
Most doctors have never heard of loin pain hematuria syndrome because it is so rare. The symptoms are similar to kidney stones or hyper-mobile kidney. But after many tests are run, everything comes back normal and doctors are left baffled. Due to this, patients often see many doctors before one finally remembers hearing about a certain condition, a facinoma called LPHS. Many tests have to be done to eliminate all other possibilities besides LPHS. This is a diagnosis of exclusion.
The most common things the doctors check for is the functioning of the kidneys. Then they check to see if there are any stones, urinary tract infections, genetic disorders, tumors, viruses, bacteriophages, parasites, trauma, somatoform disorders, and autoimmune disorders (it’s never lupus). Blood tests for all kinds of problems, urine tests, ultrasounds, angiograms, Foley catheters, X-Ray scans, CT scans and MRI scans are used to check for stones or abnormalities. Most patients go through a renal biopsy if there is reason to believe that the patient may have some inflammation in the kidney or problems with the blood vessels surrounding the kidneys, such as thin or thick glomuler membrane, or IgA nephropathy.
After all these tests have been done and the results have come in, if there are still no answers, most patients undergo a psychological test to make sure they aren’t faking an illness for attention, drugs, disability payments, or to get our of school/work.The final diagnosis is placed only once all of the above is completed and the terms of LPHS satisfied. The patient is then officially diagnosed with loin pain hematuria syndrome.
After the patient has a working diagnosis of LPHS, they then normally work with their doctor to create a long term plan for treatment. The doctor will look into treating the pain the best way they can. They like to start with small, less invasive options and hopefully stay small to minimize long term damage to the patients’ vital organs. If nothing works or treatments that used to work cease to, only then will they move up to more invasive surgery. Medications and surgeries (whether invasive or evasive) are hard on the body. Some doctors will focus on the three P’s of treatment; pharmaceutical (medically), psychologically (mentally), physiology (physio therapy). Most patients rely on high-level opiod and opiate treatments, along with anti-nausea medications, antidepressants, ACE inhibitors, therapy, and close monitoring of the situations. Diet changes are usually required, and everything that goes in and out of your body, tabulated.
Many scientific specialists and practitioners have come up with many hypotheses to why this condition happens; vascular disease of the kidney, hypersensitivity, complement activation on arterioles, coagulpathy, renal vasospasm, autoimmune response, venocalyceal fistula, abnormal urethral peristalsis, psychopathology, intratubular deposition of calcium or uric acid microcrystals (kidney stones), cysts, excitotoxcity of the sympathetic nervous system, and nephritis are all major suspects.Nephritis, intratubular dystrophy, IgA nephropathy, and hypermobile kidney have been officially documented as causing LPHS, however, that type of LPHS is known as “Secondary LPHS.” Primary LPHS is idiopathic.
Here is a brief overview:
- It was discovered in 1967.
- It is a rare condition.
- It is most common in women, 70%.
- There is a white predominance, 94%
- Pain in the kidney(s) and blood in the urine.
- The pain is CONSTANT and UNRELENTING
- The cause is uncertain, there are many theories.
- There are a few secondary symptoms to the pain and hematuria.
- Difficult to treat.
- The pain is normally severe and can incapacitate the patient.
- It doesn’t affect the kidney function.
- Most do not develop kidney stones
- It isn’t deadly.
- The symptoms normally begin in the patients 20’s.
- Diagnosis of elimination
- There are a couple of hundred patients worldwide.
- There is no cure
A list of possible treatments; can be used on their own or in conjunction with others to provide some relief:
- Ace inhibitors
- Celiac plexus block
- Daily intravenous sodium thiosulfate infusion
- Estrogen withdrawal
- Intraureteric capsaicin
- Itrathecal morphine/hydromorphone pump (IDAS)
- Kidney pedicle denervation
- Major tranquilizers
- Nerve Blocks
- Pulse radio frequency application to lower thoracic dorsal root ganglion
- Renal denervation
- Retrograde infusion of capsaicin into the urethra and renal pelvis of the painful kidney
- Sympathectomy and rhizotomy
- Transcutaneous nerve stimulation
- Urethral reimplantation
- 70 % of patients are women.
- 47% of patients with LPHS also have a history of kidney stones.
- Over 50% of patients, the GBM is abnormally thick or thin.
- Autotransplantation has been shown to relieve pain in up to 70% of patient in some reports
- 95% of patients have hematuria
- 26.5% have unusually thin GBM width.
- 32.4% have unusually thick GBM width.
- 66.5% of patients with primary LPHS have an abnormal GBM width.
- 47% of patients with primary LPHS had documented urolithiasis.
- 25% of patients go into remission after 3.5 years.
- 30% of patients obtain good pain relief after an Autotransplantation.
Differential diagnosis of loin pain hematuria syndrome
- Obstructive uropathy
- Urinary tract infection
- Renal vein thrombosis
- Modularly sponge kidney
PLEASE HELP ME SPREAD THE WORD ABOUT THIS AWFUL DISEASE. PLEASE REBLOG THIS. TODAY IS LPHS AWARENESS DAY, AND AN IMPORTANT DAY FOR DOCTORS, PATIENTS, AND CAREGIVERS ALIKE.
LOIN PAIN HEMATURIA AWARENESS DAY
PLEASE HELP ME SPREAD THE WORD ABOUT THIS AWFUL DISEASE. PLEASE REBLOG THIS. TODAY IS AN IMPORTANT DAY FOR DOCTORS, PATIENTS, AND CAREGIVERS ALIKE.
HELP US FIND A CURE!!
REBLOG IF YOU HAD ONE OF THESE BITCHES
i had all of them!
I had one of the yellow ranger.
OMG I totally had the green one!!!
Wulfenite with Mimetite and Barite from Mexico
looks like nachos and cheese and hamburger meat
This is so amazing. Thank you to whomever did this!