Erdheim-chester.orgLightheadness, Kidney issues in 1990's dx'd as multi cystic dysplastic kidney disease, controlled by meds. Chronic sinus Lesions on the brain, mass behind eye, problems. MRI showed masses behind the eyes. Surgery revealed a benign scar tissue type mass.
kidney, long bones of leg.
1980's first symptoms were high blood pressure dx'd as renal artery stenosis, profuse perspiration, and itching/burning sensation after a shower. In 1999 had pain in knees, shins, lower back and flank dx'd as age related. In 2001 had night sweats, muscle cramps, anemia dx'd as myelofibrosis. In 2004 had extreme weight loss, extreme fatigue, nausea, numb upper lip, back rash, small, longstanding skin lesion bx'd, congestive heart failure, brain lesions, kidney failure, dialysis, fevers of unknown origin, kidney transplant. After transplant (2005 – 2007) had balance issues, slurred speech, loss of tooth material, Kidney, brain, adrenal gland, heart, lungs, extreme and urgent need to urinate, extreme pain in various parts of body, shortness of breath, double vision, excessive bone, peritoneal area, testes, teeth, virtually mucous in throat, swallowing issues, many infections. Surgery to remove mass on adrenal gland was aborted due to benign entire body.
scar-like tissue around the vena cava. Adrenal gland biopsied 2 separate times. In late 2005 required feeding tube due to inability to swallow and loss of appetite. At the same time a superpubic catheter was placed due to inability to completely void bladder, dx'd as neurogenic bladder. In 2006 required stent in ureter of transplanted kidney due to stricture thought to be result of chronic UTI infections. Complete workups at Methodist in Houston, M.D. Anderson and Duke with no diagnosis.
Bone pain in right femur, diabetes insipidus. Diagnosed with LCH in 1995 after lesion removed from right femur. Inability to Head (pituitary gland, temporal lobe), eyes, have children due to drugs. Lesion removed from skull in 2000. Kidney involvement in 2006. New biopsy of left pelvic bone kidney, bones, adrenal gland, high blood led to ECD diagnosis. pressure, tonic-clonic seizures Earliest symptoms, night sweats, fever, WBC High, RBC Low, overall cholesterol levels low, LDH high; C-reactive protein level from lowest of 57 to high exceeding 100. Thigh bone, knee, ankle pain, tired easily, w/ general feeling of malaise. Just prior to dx 4 yrs from onset of symptoms, had lost 25 pounds, was in Congestive Heart Failure, Fluid both lungs, weak, very anemic, in constant pain, fever, legs, ankles swelling. Had, MRI showed excessive lymph nodes, which led to abdominal surgery to take biopsies f/u with CT and CT Pet. Platelet issues required transfusions prior to surgery, steroids. Continues w/ Leg pain, entire skeleton, back pain and High blood pressure. Osteoporosis, lower spine. Recently developed itchy spots.
Update: 2010Continues w/5MUI 3x/week Interferon; no new sites of involvement in last PET Scan (5-10); Pain on daily basis has been Kidney, abdominal aorta, bone in lower back, greatly reduced; continues with bad day following shot; pain comes on quickly over entire skeleton following stressful incident neck and legs, mandible.
or day; hot weather has worsening effect on condition; itchy legs (red spots) increases, dehydrates quickly, loses energy or has none to begin with and spends most days "napping" after 10 hours of sleep, needs additional 4 hours during day. Caregiver has noticed memory loss as it relates to things happened in past 10 years or shorter time-frame.
Since stabilizing disease, he has been able to reduce some medications, i.e., blood pressure. Now on low-dose Topral as a precautionary measure.
Only sees doctor every three to six months as he is considered at "maintenance" levels. They are all too busy to look further into ECD.
YOB Treatments Tried Current Treatment Tamoxifen. Doctors are suggesting cytoxan because of past success with this drug. Patient hesitant to switch due Tired from tomoxifen. Night sweats sometimes.
to desire to have children.
Kidney transplant in 2004, anti-rejection drugs (prograf, cellcept, 1940 prednisone) from 2004 until 2007; inpatient methylopredisonole 1g/day x 5 day steroid treatment in 2005, IVIG treatment in 2007 Surgery to remove lesion in right femur (1995). Titanium rod placed in femur at that time to support the bone. (DDAVP for Balance & speech problems (more pronounced Diabetes Insipidus)? Surgery to remove tumor on skull during lower barometric pressure). Short term (2000).Steroid injections and radiation to remove tumors behind memory loss. Fatigue. Anxiety. Tonic-clonic the eyes (2001, 2002). Radiation for pituitary tumor seizures this summer.
(unsuccessful). Interferon (stopped in 11/06 after 8.5 mos. as he Anakinra, anti-seizure medications did not respond well to it and had severe side effects if he took Anakinra has taken away the bone pain. Able to other medication), chemotherapy (Vinblastine & prednisone - 3 stop narcotics and only use ibuprofen every so treatments) helped shrink a bone tumor, but side effects were bad. Tried Methotrexate and Remicaid in 2008 but quit treatment due to side effects. Low dose Interferon again for 1 year. Now just Anakinra.
Doing well, disease slowed down on interferon. CT PET shows no more affected sites. Previous Interferon 5 MUI 3x week since diagnosis, plan is to remain on this long term; prednisone 5mg; Toprol XL & Altace sites show no activity. Very tired from interferon, for Blood Pressure; Prozac to negate side effects of Interferon (can be rage, suicidal) especially the day following the shot. Zero 1956 Current only.
energy, takes lots of naps.
Prednisone between 5-7mgs /day. Topral low dose; Paxil, antidepressant (anti-anxiety) Update:2010Low or no energy; exhaustion;fevers; pain intermittant (following stress) Poor balance and some slurring of speech and itchy spots. Had one major epileptic fit and some partial seizures so not allowed to drive. Epileptic seizure provoked more investigations. Previously had double vision, slurred speech and wobbliness. Brain MRI & removed longstanding skin lesion indicated EDC. Some night sweats. Lost weight while hospitalized in 2007. Odd sensations in the front part of the soles of feet (like numbness after having a jab although they aren't numb when they are touched). Problems with bladder needing to pass urine often and in a hurry (not believed to be diabetes insipidus).
A PET scan after the last cladiribine treatment had finished in Jan. 2009, showed a reduction in the activity of the disease. The PET will be repeated in 3 months. Going to Paris in early March to see Dr Haroche for assessment and for any advice he can Brain, chest, skin, eyes and retroperitoneal offer, and to be part of his research project.
Whilst attending Dr Tony Chu in London Cladiribine was suggested. Got in touch with Professor Pete Hillmen in Leeds as it was much closer. Had subcut Cladiribine for 5 consecutive days each month for 6 months. Well tolerated but Pet scan did not show improvement. Bad chest problems Winter 2009 (consolidated lungs > steroid treatment; respiratory syncytial virus pneumonia).
Steroids continued for 6 months. Started low dose methotrexate (40mg once a week). PET scan shows improvement of most areas.
2 months of sudden and intense illness. Initially thought food poisoning or stomach flu. Remained nauseated, began losing weight, gray colored stools. Three weeks later developed 104 degree fever. Extremely jaundiced. Then thought he had Liver, kidney, lung, heart.
hepatitis. Entered into hospital via ER for 1 month hospital stay with liver, kidney, lung and heart failure. Lung biopsy resulted in major bleeding issues due to low platelets. Night sweats, but they were while on steroids. 2001-Diabetes insipidus, excessive urine output and unusual, constant fatigue. DDAVP, one prescrip, solved Diabetes insipidus.
Brain surgery recommended, but refused.
2004-Onset of severe leg pain involving shins. Shrugged off pain as fatigue from constant walking on concrete & asphalt One growth, the first detected, begins surfaces at work.
surrounding Pituitary gland, continues down 3/2007-Unusual fatigue returns enough to cause concern.
around Carotids and reaches back up into the 3-4/2007-Sought Family Physician's advice about leg pain and fatigue. Suspicious due to lack of follow up concerning Pituitary orbits.
Xanthum, He referred me to a Neurosurgeon. MRI reveals the growth has increased. Neurosurgeon referred me to an Endocrinologist to explore effects of the growth on hormone levels. Testosterone is found to be dangerously low, and a A shadow is seen in (right?) kidney.
hormone replacement gel is prescribed. A Transnasal biopsy is attempted, but fails due to depth of growth in brain. Neurosurgeon referred me to a Neural-Ophthalmologist, who successfully gains biopsy after Optical Ultrasound locates growth Xanthum detected around the aorta in the grazing Optic Nerve. Peritoneal (intestinal) cavity.
Local lab in NC was unable to identify biopsy. Tissue sent to Washington University in St. Louis, MO. Growth ID'd as Xanthogranuloma.
Area of the lower Femurs and entirety of Tibias Referred by Neural Ophthalmologist to Oncologist, who stated Xanthums rarely grow in one area, and a Full Body Bone Scan are found to be involved.
is scheduledXanthums discovered in numerous areas from the brain to the Tibias.
Upon images being examined by Oncologist, a diagnosis of Erdheim-Chester Disease was made.
1997 noticed initial dizziness when climbing up high. Developed loud snoring in 2003 but not sleep apnea. Testing at that time showed high blood pressure. 2004: worsening balance & dizziness, slurred speech and emotional incontinence. CAT scan showed growth in kidney and puffiness around kidneys. Bone scan indicated ECD. Biopsy of kidney growth confirmed Cerebellum-pons-pituitary area of brain, kidney ECD. Currently chronic constipation, sexual problems. Diabetes insipidus since childhood due to injury to head. Stamina 'halo' and lesion, femurs and strength have decreased, but mostly that is from the lack of physical activity. Balance and speech issues continue, but have adapted to them. (12/08 – speech and cognitive issues seem to be getting worse, but no pain issues) Swollen eyes, achy joints, slurred speech, wobbly walk.
Lungs, eyes, nodes under jaw.
YOB Treatments Tried Current Treatment Retired at 46. Generally improved. Less slurry; Has been on (1) Interferon, then (2) azathioprine and then (3) only use sticks when I go out, stair lift only cyclophosfamide and then back to (4) only interferon 3mu every Lamotrigine 100mg bd (for epilepsy) occasionally (after too much slurp); only one pair other day and then (5) subcutaneous cladiribine subcutaneously Warfarin 7mg od (for pulmonary embolus) of glasses needed for reading and distance; for 5 days consecutively at intervals of one month, for a total of 6 Co-valsartan od (for blood pressure) swallowing usually OK so the weight is piling on!; months beginning in July 2008 due to activity seen in bones for Fluoxetine 20mg od (for depression) still coughing quite a lot. Restarted regular Methotrexate 40mg once weekly trumpet lessons, and doing voluntary sessions in Prednisolone (reducing over 6 months) local neurology ward and a local "resource centre Methotrexate 40mg once a week for those with chronic neurological conditions". Also making bread, cakes, and jam at home.
Feels worn out from interferon, feels like he had Interferon 6 MIU 3x week. Prednisone 4 mg (tapering off). Plan to taper interferon to 3 MIU when off prednisone. the flu, but not too bad. Stable. On blood 1964 Steroids, interferon.
Bone marrow stem cell transplant on hold because ECD is stable and risk of transplant.
pressure medicine. Anemic. Has numbness in right leg, strange sensations on foot.
Intron A injections- 3 million units (.5 ml), 3 times per week.
Currently I feel great. My pain level is nearly non- Testim testosterone replacement gel- 50 mg per 5 g tube, 4 times per week.
existent. I do, at times, have trouble w/ balance Fentanyl, 12 mcg patch- replace patch every 72 hrs.
and "steering", ie I often run into walls turning *25 mcg patch replaced w/ 12 mcg due to pain relief lasting beyond 84 hrs.
corners and veer right a bit when walking. I'm Bupropion 150 mg tablets- one per day (AM) for depression.
very far sighted and, more often than I'd like, fall Clonazepam 1 mg tablets- one per day (PM) for leg spasms.
asleep quickly during the day, sometimes for 2-3 Loratadine 10 mg- One as needed to relieve itching in shins which began after Radiation treatments used to shrink hrs. This is very unusual for me.
Xanthums in that area.
I walk for 30-60 minutes 5-7 days per week, If symptoms return, I have qualified for financial assistance from Johnson & Johnson to aquire Leustatin, which work out w/ 20 lb dumbbells 3-5 days per wk, do has shown promise in treating the Xanthums.
Androgel testosterone replacement gel, 5 g tube, did not absorb a number of abdominal exercises, push-ups, as quickly as Testim, per Endocrinologist.
flexibility routines, use an ellipse machine for 10 Ester-C- 1000 mg- One per day to boost bodies immune system.
min.'s after walking 4-6 days/wk. It takes me 2-3 Vit. D w/ D3- One per day for energy during Winter and to support prostate health.
Vicodin (e) - Did nothing for leg pain. Caused drowsiness.
days. But, I push mow our roughly one acre, Saw Palmetto 320 mg ( per 2 capsules)- Two per day for prostate health. Concern due to Testosterone mountain yard and am working on an aesthetic replacement gel's side effect of Prostate enlargement.
plan for our yard.
Fish Oil 1000 mg- 2 per day for brain health.
I may need rest regularly to get through the Folic Acid 400 mcg- Helps w/ energy level and cell production, such as in the stomach, to aid w/ digestion.
day. But, I will NOT give in and let someone else Calcium, Magnesium & Zin (Cal.-600+mg, Magn.- 250+ mg, Zinc- 10 mg)- Lab work showed Calcium levels to be handle my responsibilities, and this disease low. Zinc is involved w/ eye health.
doesn't have what it takes to damage my faith. Magnesium 250 mg- 2 per day to ease constipation caused by Fentanyl.
NOBODY/NOTHING wins in a fight against God! Men's Multivitamin.
He's in my corner. Thus, we win. ECD loses! Silicea terra- To relieve unusual body odor, from combination of meds and supplements, I assume.
Stable since 2005. Some good days, some bad. Avapro & Norvasc for high blood pressure. Nortriptylene for emotional incontinence. DDAVP for diabetes incipidus. 1963 2CDA in 2005; stable since then.
Mayo clinic in April, 2008, showed no changes in CPAP for sleep problems.
scan for last 2 years.
M 1967? Remicaid and Methotrexate along with Cellcept None since 11/06.
In remission since 3/08.
(incomplete history) Night sweats and leg pain. Tired and cold with intermittent fevers. Internal pressure inside head and optic nerves, treated with LPI to release pressure immediately and meds of Umerux and Prednisone .After 6 months of treatment pressure Kidney, bone, brain.
disappeared. Diabetes insipidus 3 years after onset of symptoms. From 2006 - 2008 ears plugged with yellowish discharge that must be removed once a month.
Increased thirst (Nov 1995) 4 plus gallons of water per day. Low blood pressure, swelling (legs, face), Low grade fever (for 3-1/2 years) (1996). Shin pain (1997). Bone pain (1998). Slight reduction of tissue (abdominal, kidneys) after off of morphine. Skeletal, Central diabetes insipidis, Last 7 years I've remained stable (no increased tissue). Learning to deal with fatigue through better nutrition. Control pain with brain wave stimulation. Began with pain in the long bones of the legs in about 1989. In 1/04 stomach began getting bigger and would feel full after eating only a few bites. In 12/03 began having chills and fever every evening. In 12 01prior to knee surgery an MRI revealed what was thought to be prostate cancer. (Prostate had been removed in 1990. Prostate cancer was retreated in 1998 with radiation therapy and PSA was normal one month prior MRI of knee so return of prostate cancer was not considered a correct Masses in stomach, chest, and Behind eyes, diagnosis.) Knee surgery in 01/2002 resulted in infection of the knee. In 04/02 began having problems with legs again and involvement in bones skull, shoulders, arms orthopedic surgeon wanted to do a biopsy of Rt. leg above and below knee. This resulted in having a bone biopsy of hip which resulted in an infection. In 03/04 fatigue and shortness of breath resulted in a consultation with an infections disease doctor who ordered a CT of the pelvis, abdomen and chest. Within two days 2200 cc of fluid was removed from the sac surrounding the heart. 8 biopsies of stomach tumors were done with no definitive diagnosis except none malignant. A month later finally got a diagnosis.06/04 1995: (possible sx's): large fibroids in uterus necessitating hysterectomy 1995, no dx of fibrosis at the time remembered: 1999: (probably ECD related?): severe ‘heartburn' pains: dx'd as narrowing of the coronary artery supplying blood to the right atrium - a stent was implanted 1999 – an unusual cholesterol/ triglycerides ratio was noticed (very low HDL giving HDL/Triglycerides ratio that indicates very high likelihood of coronary artery disease – the material blocking the artery was said to be cholesterol but no biopsy was done. 2001: Definite ECD symptom: fluttering / waviness in peripheral vision – biopsy taken: dx: Acute Idiopathic Orbital Inflammatory Syndrome – fibrotic tumour growth around both optic nerves. The biopsy material was retained and used in later analysis. Treatment with cortisone (75mg/d for three months reducing to 10mg/d over three months before coming off) slowed growth but did not reduce size of tumours. Radiation treatment on both Retro-ocular area surrounding both optic eyes had no effect. Treatment with Imuran reduced the size of tumours somewhat relieving pressure on the eyes – by 2004 nerves; retroperitoneal area with two visible the growths stabilised and vision returned to a reasonable level however glasses are now needed permanently and cortisone growths around renal arteries, probably fibrotic led to the onset of Type 2 diabetes. 2005: ‘galloping in the ears' / very high blood pressure: scans showed a growth in the tissue related to that biopsied from th.e retroperitoneal area, adjacent to but not enclosing the aorta and invisible but probably enclosing and compressing both renal retroperitoneal area. Density changes/ arteries (hence the high bp). Biopsy of retroperitoneal mass confirmed fibrotic tissue. A stent was inserted into the right renal lesions to all long bones (arms and legs).
artery, eliminating the blood pressure problem and treatment with Tamoxifen & Imuran has stabilised the tumours (no growth, no shrinkage) has been seen in over two years. 2006: Ophthalmologist did research work and initiated a review of all the available material including carrying out scans of the long bones: a firm dx of ECD was made, confirming the treatment regime as appropriate. Re-evaluation included both biopsies which were now interpreted as characteristic of ECD 2007: scan done which showed no change in the size of the tumours in 4 years even though the specialist in looking inside the eye had expected significant improvement. Lifelong (since 16) hypothyroidism. Lifelong difficulty with metabolising fat. Enamel on front teeth "breaks off." Initial problem was a small growth on pituitary gland discovered around 1992. Later when dizziness became an issue the growth on the pituitary gland was removed and as a result blindness occurred in right eye. Began taking testosterone injections and enjoyed a period of feeling good. In 1996 while in Michigan the symptoms returned and had another surgery to Eyes, lung, kidney, brain remove tissue from the brain stem and optic nerve. This resulted in balance issues, reduced sight in the left eye. The doctor did not understand what he was seeing and put the case on the computer. A doctor from Korea actually came up with the diagnosis. Previously had issues with colon disease, but now temporary colostomy has been removed.
YOB Treatments Tried Current Treatment Initial treatment was Vinblastine 1 x week, Aredia once every 3 1972 weeks both for 7 months. Naxin (pain med) 2 x day, Calcium w/D For last 6 months treated with 1X9mil + 1X6mil per week . Week ago reduced the dose to 2X6mil per week as result for 3 months. Symptoms were improved. Prednisone for 8 of bad quality of life .
months beginning at 100 mg/day stepping done to 0 mg.; Uramux - 750mg per day to resolve internal head and eye pressure .
Interferon as high as 9 mil x 5/week in 2008 to stabilize the Vinblastin - 1X60ml once in 8 weeks Stable, but not feeling well. disease. Had LPI 3/08 to decrease head internal pressure.
Aredia - once in a monthMinirin spray - sniffing 1-2 times per day In 2010 Interferon (9 mil) 2 x week; Vinblastine once every 3 Nerocin - for pain weeks; Aredia 1 x month, Naxin 1 x day. Infusion of pamidronate every 30 days. Shiatzu and acupuncture.
Three rounds of chemo, vinblastin, 2cda, didn't help. 2 radio active injections of Samerium 265 (or 285) didn't help. On Pamidronate every 60 days to prevent bone break down.
Osteoprosis in my lower back.
morphine for 33 months. highest level 400mg per day, off in 11/2001 with the help of brain wave stimulation.
No new Masses since 09/02; lots of leg pain, Round 1- 24/7 of 2cda chemo; 7/04 Round 2 was 1 treatment Eyes, Leg Weakness. Lower back or left hip 1940 each for 4 days; 9/04 Round 3 was 1 treatment a week for 6 Prednisone 10 MG per Day has pain along with legs. But patient is glad to Condition remains stable (2009) but no indications the ECD growths are reducing. Lots of walking to maintain mobility. Pilates & ECD meds are combination of Imuran (for eyes) and Tamoxifen (for retro- peritoneal growths). Other drugs at Osteopathy to maintain flexibility. Muscle For Eyes: Cortisone; Radiography; Imuran Retroperitoneal present: Oroxine: for thyroid; Amaryl / Diaformin: for diabetes; Cartia / : for coronary artery disease; Lipitor: for spasms / strains / pain happens frequently and 1955 growths: Tamoxifen. cholesterol control; Atenolol & Karvezide for blood pressure; Panadol if needed for pain relief (avoided when easily. Bone and muscle pain is present possible);Tea Green for removal of radicals/ toxins; Fish Oil for arthritis continuously but at variable levels of intensity. The use of a Homedics Percussion Massager (model PA-MH) reduces incidence of muscle cramps, especially those occurring during sleep Chemo - vp16 topicide – 3 days of treatment in a row each month (did for 3 years, stopped for summer of 2008 due to side effects); DDAVP (for diabetis insiidus); Synthroid; Prevacid; Lopressor; Lasix; Lovastatin; Humalog; Nutrophin; Testosterone Died March 7, 2011 No new tumors, old ones are dying; Hydrotcort; worried about injection; Lyrica; plnng to start interferon on Oct 13, 2008 losing eyesight as of 9/20/2008.
First symptom was at least ten years of severe hip/leg/knee pain. Can't remember exact date of onset. Was diagnosed during this time with fibromyalgia, chronic fatigue syndrome, lymphoma, arthritis. Diabetes Insipidus onset in 1990. Hyperthyroidism onset in 1998. Routine abdominal CT in 2000 showed retroperitoneal mass with extensive arterial involvement. Histiocytes blocked ureters and infiltrated kidneys causing level 3 CKD and necessitating a bilateral nephrostomy in 2003. It continues to manage the situation. Had one round of experimental chemo iwith Fludarabine in 2000 but it had no effect on mass. Continue to have aching in lower spine, weight bearing bones and more severe pain in feet, which tends to come in cycles. Started having problems with one eye in December 2006 that was diagnosed as blephoritis. Noticeable bulging in early 2008 and CT showed orbit was filled with mass. Vision is not impaired as of yet. Histiocytes around port-a-cath caused superior vena cava syndrome in 2008 which was relieved by angioplasty. In 2011 was diagnosed with inferior vena cava syndrome due to abdominal mass, which has flattened inferior vena cava "like a pancake" and causes severe edema in lower body. Took Interferon Alpha 2-B from March 2008 until September 2010, when insurance would no longer cover costs. A doctor at MD Masses in abdomen, kidneys, lower spine, Anderson, who has done research on ECD, believes that ceasing Interferon treatment may cause disease to kick into "over hips, legs, knees, feet, orbit and pituitary drive." This theory could explain sudden massive growth of histiocytes in abdomen. Masses in abdomen, kidneys, lower spine, hips, legs, knees, feet, orbit and pituitary. March 27, 2013 Update: The large mass in my abdomen (which was what led to diagnosis) has grown so big that it has completely compressed my inferior vena cava flat. This leads to little or no circulation and fluid retention below the waist. I have been able to miniimize swelling below the knee with support hose, but the thighs and abdomen continued to get bigger and bigger. This severely affected my balance - thus a broken wrist - and made walking distances impossible. Two weeks ago the fluid reached the breaking point and ever since I have been housebound because of profuse weeping below the waist. I can't move from one room to another without getting off balance and light-headed. I spend 24/7 in a recliner with a constantly changing layer of towels padded around me. My doctors have conferred several times and consulted others in their fields but have no answers. My hematologist even makes house calls since I can't come in. it is dangerous for me to try to get into the car and we are waiting for an electric stair lift to be installed.
Bone loss in lower jaw. (waiting for rest of information from patient) (waiting on information from patient) First symptom was double vision. After repeated attempts to correct the problem with glasses it became obvious something bilateral. xanthogranulomas in her eyes; a might be really wrong. August 2008: Scans showed no change in eye, abdomen, or mass at base of skull. First skeletal mass in her abdomen; a mass at the base of survey so done and bone involvement was seen. The eye has had noticeable improvement in the protrusion, but is not her skull in the spinal canal; showing areas on documented by the radiologist as the initial scan in fall 07 and the more recent one in Feb08 were done at two different her proximal tibias • In January 2007, patient complained of right ankle ailment: slightly swelling at time; still remnants of "black and blue" from a softball injury sustained four to five years earlier when stepping on a base and catching on back edge with full weight. This led to a comprehensive battery of tests and exams (metabolics; chest and pelvic x-rays; Thoraic, abdominal and pelvic CT scans; and a venous extremity with doppler of the right leg. All results were normal - except the CT scan of the pelvic/abdominal region indicating two soft tissue masses surrounding both kidneys.
• Other than discovery of the two tissue masses, no other symptoms experienced. A succession of five medical specialists (hemotology, onocology, radiology, urology, and nephrology) at two major hospitals and two biopsies resulted in a preliminary diagnosis of retroperitoneal fibrosis (RPF). Two biopsies (1st - local left renal; 2nd - laparoscopic left renal) were conducted in succession over two months; each proved inconclusive. A review of CT scans and xrays in pelvic/abdominal area reveals NO soft tissue around the aorta in this region (clinical cases define RPF as having such soft tissue in this region. A bone scan with TI-99 led to a change in diagnosis to Erdheim-Chester disease in September 2007. Patient during this period was frequently asked by medical professionals how he felt. He always responded "Great! It's only you doctors who tell me something is amiss."• Other than the two tissue masses surrounding the kidneys and the "long bone evidence" in both legs and arms, there is no other involvement of any other organs, to include the brain, eyes, lungs, etc. These organs are CT scanned periodically (every 9-12 months) monitoring any change. Metabolically, all organs were performing within their norms and these are checked monthly.
If I could offer any encouragement to others, it would be these two adages: "Dream/Plan as if you will live forever; live as if you will die tomorrow." and "Forget the potholes in the road of life. Celebrate the journey."• Be strong! Keep the faith! Long bones, heart, brain, pituitary gland, and my optic nerves were crushed causing me to lose my sight.
Liver enzymes elevated; red spots on skin; elevated sed.rate (100); chest x-ray showed unusual spots in lungs; sinus problems. Occasional joint pain, fatigue, anemia, CT scan showed unusual material in abdominal area, vision problems, Lung, eyes, abdominal, thyroid thyroid deficiency. Lung biopsy at Mayo in 2003 showed ECD. Consulting doctor knew nothing about ECD.
Leg, knee & ankle swelling, joint pain,sleeplessness, weight loss(this was prior to treatment, symptoms have improved some Legs, kidney, heart, lungs YOB Treatments Tried Current Treatment On disability as of 5/31/2003, able to continue with all normal activities except for walking distances. Self care for nephros-tomy which Interferon Alpha 3MIU 3x/wk since 3/31/08, pain medicine as needed, Prednisone, 10mg, daily, BP medicine needed sometimes needs bandage changes as often as 1950 One round of chemo, otherwise just treating symptoms as a result of Prednisone every two hours. CT shows no change 3 mos. after starting interferon treatment. Very low energy, hip/leg/foot pain and loss of flexibility in lower extremities.
Dec 2007: Tried 1 month steroid burst which showed mild improvement but with severe side effects. June 2008: Began interferon at 6mil. Aug 2008: Interferon lowered to 3mil due to Interferon 1MIL 3xweek adverse side effects. Sept. 2008: Again reduced the interferon dosage down to 1 MIL 3Xweek due to side effects of exhaustion, malaise & weight loss I was on Gleevec for 5 months, Nov. '10 to April '11. It didn't help with my illness & after 1-2 months ended up with some bad side effects. Mainly debilitating fatigue, weight gain, & double vision. I've been going through a couple experimental treatments as of late. No success yet, but I'll keep you posted.
Prednesone was utilized for the first four months in concert with CellCept. Starting month: 40 mg/day. Second month: 30 mg/day. No visible symptoms. Fairly healthy and 1945 Third month: 20 mg/day. Fourth and last month: 10 mg/day. CellCept 1000 mg x 2 per day.
physically active throughout life; accomplished CellCept was taken from the first day/first month at the dosage of many goals and still has a few more to tackle 1000 mg, twice a day and continues.
I am in remission. I use a blind cane to get 1979 Radiation, cladribine, interferon Am concerned about anemia, lungs, eyes, plus whatever may be a problem in the future that is not currently affecting thingsCurrently I am blessed, still able to work fulltime & do everything I did before. I do have constant 180 mcg of Pegasys weekly injection. Was at 150 mcg for 18 months, but have seen slight enlargement in lymph leg pain, but nothing I can't tolerate. Take Tylenol 1968 No other treatments tried nodes so Dr increased injection dosage to see if this helps.Will see dr in October 2010 to see if there is when needed. I have lost a great amount of improvement. No other sites increased in size. If there is no imporvement Dr. will try Gleevec.
weight as a side effect form the pegasys/interferon. Am nervous about the possibily of taking Gleevec.
Kidneys (both surgically removed and subsequent live-donor transplant) Initially: fatigue, swollen ankles, night sweats, and high blood pressure. Spinal cord (debulking surgery to remove Currently: fatigue; pain in shins, thighs, and upper arms; fevers; night sweats; headaches; muscle weakness growths from thoracic spine)Eyes (growths on both optic nerves -- the right more pronounced than the left. No additional growth in the past 8 months) First sign was double vision, but the neurologist couldn't find the cause. In 1998 more problems with eyes. Vision was not stable and left eye was bulging. Diagnosis was given as Graves disorder. A CT-orbita showed tumors behind both eye-balls in the orbita. In January 1999 a biopsy was taken, a lipid deposit called xanthogranulomas was found. A lot of scans (thorax, abdomenal, skeleton, hollow pipe-bones, MRI, SMS), biopsy of a kidney, bone biopsy, bone marrow biopsy and tests (blood, thirst) followed and in March 1999 a team of internal medicine doctors were convinced of ECD diagnosis with a beginning diabetes insipidus. The disease had spread out in my body: xanthogranulomas is behind my eye-balls, spots of lipid deposits are found in the bones (hollow pipebones above knees, in hipbones, shoulders, chest, skull, lungs, around the kidneys, around the aorta and cerebral membrane (along the tentorium).
Eyes, bones, lungs, kidneys, heart, brain, In an attempt to save the optic nerve, Cytostatica Solu-Medrol iv followed by another cytostatica called Endoxan iv were tried chest, pituitary gland with no sustainable effect. June 1999 a decompression of the orbita was tried where bones in eyesockets and cavity of nose were removed to make more space in the orbita with no sustainable effect. 18 sessions of Radiotherapy with a temp. improvement in vision. Jan. 2000 a „debulking‟ surgery (removing lipid deposits out of the orbita behind left eyeball) was successful and sight was regained in left eye.
I have learned that it is important to believe in the good things of life, enjoy each day and make each day a good one with my wife and kids, my family and friends.
I discovered my sources of energy and fun, and they brought new and unexpected dimensions, which surprised me so much.
For example, I found my guitar again. Making songs and music give me power, satisfaction and a lot of fun.
Edema, Shortness of breath and on oxygen. Tired all the time.Alawys aching.
Left Plueral Area 3 years of bi-yearly follow up with bone gammagraphy she was found to have more lesions in the ribs, liver and spine.
Ribs, liver, spine January 2000 developed Pleuritis. After nine weeks of rest he got sick again with Pneumonia. Recovered, but had trouble breathing normally when exercising. Test showed lung capacity was decreased to 55%. Underwent multiple blood tests and biopsies. No diagnosis means no treatment. Physical condition slowly decreased and treated with antibiotics or prednisone. Lungs, heart, brain In July 2002 underwent an open lung biopsy. Biopsy showed no cancer or asbestos. January 2003 was given one gram of Prednisone treatment. birth? Or Knee pain since I was 17. Poor balance and occasional slurry speech at age 43. Low kidney function, Higher level of thirst Balance and speech, kidney function, and since I was 26.
Loss of alveolar bone (supports teeth) in mandible (lower jaw) - began in 1999. Have lost 5 posterior teeth in lower jaw since bones, lower jaw, behind eyes 2001; occasional leg pain since 2000; hole in left hip DX in 2002; bulbous eyes, headaches, swollen optic nerves (10-08).
Symptoms first began as swelling in legs, fatigue/no energy, severe cough, shortness of breath, feeling of fullness in chest, bruising and pain of the legs, changes in breasts. In July 2009 after several doctors could not diagnose and symptoms had gotten much worse, we went to Mayo Clinic where 1700 cc's of fluid were drained from the pericardium of the heart after Retroperitoneum, both eyes, jaw, ends of leg discovery via echocardiogram. Fluid continued to be drained every 4-6 hours via permanent drain. Other symptoms had bones, shoulders, sternum/chest cavity, developed such as back pain, tingling in extremities, pain in jaw, fluttering of peripheral vision, some difficulty sleeping. pericardium with some infiltration into heart.
Diagnosis was made via biopsy of mass surrounding kidneys (retroperitoneum) that was discovered. A pericardial window was placed on July 14, 2009, which fairly quickly stopped the fluid buildup and alleviated the severe chest discomfort.
YOB Treatments Tried Current Treatment When first diagnosed, doctors believed (or hoped!) that the Rx'd 1972 immuno-suppresant & Prednisone might have a positive effect on the ECD. However, only 2 months after the transplant his spinal Walks unaided, although has transient spells of cord was attacked, again by ECD growths. Then for a year no weakness that causes him to hold onto new growths made their presence known. Personal MD something stable. communicated with Dr. Vaglio in Parma, Italy, and the Prograf was Prednisone 10 mg/day (originally prescribed as part of the transplant immuno-suppresant) Feeling OK some days; weakest at beginning of 1958 switched to Rapammune. The Rapammune is an immuno- Rapammune 3 mg/day (immuno-suppresant for kidney transplant, as well as potential anti-proliferative properties) the morning then usually improves as the day suppresant that (hopefully) will also have anti-proliferative For pain: Oxycodone & Fentanyl patches progresses. On a scale of 1-10, pain is often 8.5 - properties. An incidental finding on a head CT revealed the 9; however, pain meds make it possible to be growth on the optic nerves; he retains full field of vision in both eyes. In a repeat CT 8 months later, no further growths on the optic nerves. We are encouraged that the Rapammune is working. :) Drug cocktails over the past 10 years have included:prednizon-cyclosporine-octreotide-minrin-renitec-fosamax 10 mg prednizon/day 100 mg azathioprine/day Stable. No pain, but energy has limits, yet 30 mg APD iv/ 3 months "energy enough to do enjoyable things.
5 mg enalapril maleaat/day 1000 mg calcium/day In 2001-2002 an experimental treatment with Lutetium 77 (an internal radiotherapy was tried.) On oxygen, pain level of 7, using walker to get 1938 Prednisone….Somewhat helpful Prednisone 10 mgs twice a day Experiencing bone pain.
1955 Antibiotics, prednisone working full time as a Social Worker, still driving. Living a normal, but altered life. Since on Gleevec 400mg (Improved energy, no current pain) Gleevec there has been a slight improvement of the disease and an increase in energy with only slight GI side effects.
In 2003, I took Fosamax (1/wk) for 5 yrs - discontinued 2 yrs ago. Continue to experience occasional leg pain; Surgery 8/09 for decompression of left eye; 9/09 for unexplained, occasional joint pain ; double vision decompression of right eye.
5 MIU Interferon alpha-2b injections 3X/wk since 12/09 in evening if watching TV; fatigue from Interferon. 5 MIU Interferon alpha-2b injections 3X/wk since 12/09 Nsaids as needed for pain I still work as a psychotherapist part time and Nsaids as needed for pain work on remaining positive, which I believe has helped me stay relatively asymptomatic.
Still have fatigue, needing 10 hours of sleep per night, plus frequent naps. Leg pain continues, Currently, only taking natural supplements and vitamins under the direction of a homeopathic physician, and some days worse than others. Also, arm pain Ibuprofen daily for pain. Went through 6 rounds of Cladribine chemotherapy as prescribed by Mayo Clinic which and sensitivity/tingling. Difficulty walking or appeared to have significnatly shrunk the masses in the chest/heart, and retroperitoneum, less improvement behind standing for very long. Overall weakness, loss of 1955 Stronger narcotic pain meds had negative side effects.
eyes and other areas but no worsening. Now take Ativan for sleep, and Thyroid medication for many years. Will strength. Residual bruising of lower legs, minimal have new sets of scans done soon. Latest echocardiogram showed heart/fluid is stable, actually improved since last swelling. Sensitivity to heat. Skin changes: small echo 6 months ago.
white raised "bumps" on face, arms. Occasional cognitive difficulty with memory, organization skills.
Slightly damaged pituitary gland confirmed by scans in July 2009.
2004 – Xanthalamus on eyes, high cholesterol (may be unrelated to ECD) Xanthalamus in eyes shown to contain some 2006 – slight diabetes insipidus (increasing in subsequent years) then stabilizing in March 2010.
Touton cells related to ECD. Surgery in Dec 2007 – lowered testosterone levels, mood swings, low libido etc. 2009 to remove yellow / Xanthalamus patches 2009 – leg aches, discovered increased prolactin levels.
was successful, Xanthalamus have not returned as of Aug 2010. Classic ECD scan when bone scan completed in July 2009.
retro-peritoneal tumor that presses on Fatigue, nausea, abdominal pain, bone pain, headaches, frequent urination kidneys/ureters, femurs, hips, lumbar and cervical vertebrae, sinuses 1981, age 33, Iwas dx with Diabetus Inseptus cause unk. Went on DDAVP.;Late eighties passed out at church, went to hospital,checked out ok.
1999 Had cateract surgery on right eye, Had fluid in heart sack had to be removed.
2000 had cateract removed from left eye.
2001 went to the V A Hospital to be checked for effects of agent orange, C T scan showed what they called fatty tissue around my liver. nothing done.
1995 Dx with type ll diabetus, treated with pills under control2000 started haveing trouble breathing (short of breath all the time). My wife commented several times about my breathing.
2004 Got sick had pain and discomfort in stomach, thought it was my galbladder, went to the VA emergency room, they said I didn't have classic symptoms, gave me some pain pills, and sent me home.
April, 2004, a week later same problem returned, went back to E R. did a C T scan said I had kidney stone, July 2004, I woke up with very dry mouth and a temp. of 104. Went back to E R . They did another C T scan saw the fatty tissue and admitted me. For the Next three weeks they did many test,C T, Blood work, skeletal x-ray, pet scan, heart test, lungs, GI track testing.
The end of July 2004, they took out my gallbladder, they were supposed to do laporscopic surgery. When I woke up I had a 12 inch incession. They told me they could not get it out with the small surgery, and hade to do major. More tests followed.
Dec 2004 they took samples of the tissue that was growing inside of me. In June of 2005 sent samples to Ohio State University for second oponion. O S U made a dx of ECD.
weakness, loss of strength, balance issues, unsteady gait, slurred speech, tremors in hands/arms, diabetes insipidus, retro-peritoneal brain, pituitary gland, kidneys, long bones bones, pituitary, paransal sinuses and some lymph My symptoms were bone pain, diabetes insipidus and other hormonal deficiencies in addirtion to chills and profuse night sweats YOB Treatments Tried Current Treatment A healthy diet avoiding saturated fats, high sugar and high meat 1972 intake. I eat fish regularly and lots of vegetables. I am not doing enough exercise currently. I am taking various herbal / naturopath recommended items for general health: I feel good apart from slight leg aches. a) Turmeric extracts BCM-95 for anti-inflammatory effect My well-being is so much better since the b) "adpatogen" herbal treatment for adrenal stimulation. (liquorice testosterone / ADH treatment stated in early c) Fish oil (2 x 1000 mg pills per day) with EPA:DHA ratio.
Jan. 2011 Update: d) Specialised Multi-Vitamin pills to give daily intake of 1) Perhaps unrelated to ECD, but I have cured a. Vitamin A – 15,000 IU; b. Vitamin C - 850 mg 1) Daily 50 mg / day Testogel (testosterone supplements) my asthma in the last 6 months by rigorous 1961 c. Vitamin D3 – 1000 IU; d. Vitamin E – 400 IU 2) ½ pill (0.5 mg each) twice a week of Cabaser (cabergoline) to reduce prolactin.
avoidance of preservatives (the main culprits e. Thiamine – 40 mg; f. Riboflavin – 11 mg 3) Daily ½ pill (100 µg) of Minirin (desmopressin) to supplement lack of anti-diuretic hormone. Reduce effects of DI.
being soft drinks ("pop") and cider. g. Niacin – 160 mg; h. Vitamin B6 – 10 mg 2) My eye xanthelasma have disappeared which I i. Folate- 100 mcg; j. Vitamin B12 – 450 mcg believe strongly is due to avoidance of dairy fats k. Biotin – 400 mcg; l. Pantothetic acid – 413 mg (and replacement with oil fish and nuts- almond m. Calcium – 300 mg; n. Iodine – 225 mcg etc., and no peanuts) o. Magnesium – 285 mg; p. Zinc – 15 mgq. Selenium – 200 mcg; r. Manganese – 6 mgs. Chromium – 20 mcg; t. Molybdenum – 100 mcgu. Potassium – 90 mg; v. Boron – 3 mgw. Choline Citrate - 100 mg; x. Vanadium – 100 mcg A tumor has caused the left kidney to fold in half Prednisone, 40 mg./day that tapered off after 6 months. Felt and the kidney is failing. The right kidney is energetic on it, but had terrible withdrawal from it for 2 months. Started vinblastine on 8/17/10. Initially treated once a week, but had to cut back on the frequency due to a low white functioning, but the tumor is beginning to press 1971 Interferon, 3 injections/week for 5 months during which tumor grew blood count. Stents for the kidney were placed on 10/1/10. Also taking oxycodone and lorazepam for pain as on it causing it to loos function. Always tired, and disease spread to cervical vertebrae. Felt okay on Interferon needed. frequent abdominal pain, occasional bone pain. other than flu-like symptoms the day after injections.
Some days are good, some days are bad. Status can change in a matter of minutes.
All symptons have remained, as well as extreame In 1981 began DDVAP.
fatigue, shortness of breath, A strong feeling of In Nov 2005 started on interfuron, been on it since.
weakness, no strength, or ambition.
Tolerating Gleevec well, but all issues still present. 2nd PET scan in 2 months. Battling swelling from Gleevec. prednisone tried for retroperitoneal fibrosis (before ECD diagnosis) and Currently in rehab center recovering from 2 month long Started Gleevec January 2011. Also, desmopressin for diabetes insipidus.
no changes seen. Tapering off presdisone in early 2011.
hospital stays (Oct 2010 and Dec 2010/Jan2011). Walking with assistance and walker. Hoping to come home soon.
Have been taking Kineret since first diagnosed. Tested positive for BRAF mutation, but will stay on Kineret as follow ups with the BRAF-inhibitor treatments is too difficult logistically.
Kidneys, Liver, Pancreas (Surgeon who got final Biopsy said that the growths were the size of his fist on each organ), around Aorta, on two heart valves, Smooth pleural thickening to lungs plus …ECD Shortness of breath involvement – not sure what that means, he sprung that one on me at this last meeting. Lung capacity Diabetes Insipidus severely reduced. Lytic lesions to ribs, left shin, hardening of bones in pelvis and lower spine, bone marrow has gone fibrotic there too.
YOB Treatments Tried Current Treatment I have been retired from work on the grounds of ill health. Lethargy makes working a normal pattern impossible as I constantly feel tired. The need to sleep is random/unpredictable and feels like "passing out". Methotrexate – 30 mg weekly now also take folic acid on 5 days of the Shortness of breath caused by damage to lungs means cycle as it was affecting my liver I get breathless when talking or moving. This is the main outward sign of the disease, people say I look really well… Previous medical history
I was in a lot of pain- aches in knees, ankles, wrists. Testicular cancer – seminoma at age 41, treated with orcheoctomy and Stabbing pains in chest, lower back and abdomen radiotherapy (to lymph nodes in similar area to ecd organ involvement).
As listed in "Treatments Tried." This may be reviewed if lung function is thought to be decreasing rapidly caused by compression from the Histio masses? These Epstein Barr Virus at 43.
have largely gone away with treatment. Previous work/environment factors
I still drive, but find it very difficult to do chores/tasks Craft Design Technology teacher for 30 years, used Toluene based that before would have done in minutes – I move very products in the workshop. Exposure to wood dust and welding fumes.
slowly, it's not a conscious thing just seem to be living Live in an area that although rural has three major coal fired power life in slow motion.
stations within six mile radius.
Emotionally/mentally I feel scared by the unknown and by the future. I also feel inadequate as I cannot do things that would have posed no problem.
ECD has totally changed my life, I have gone from having a very successful career and being very active to being a pensioner who can do very little without falling
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