Possible trouble for me

Okay, it’s March 16, 2008 and I’m beginning to get worried. I’m afraid my weight is creeping upward again (due to edema) and I’m not happy about that. I’m up to 149, and that’s as high as it has been since mid January. I’m noting some increased pugginess in my abdomen, and I can palpate some more notable pitting edema in my shin bones. I think my weight has been inching upward since Thursday when I went to Jackson for a doctor appointment. I weighed 149.5 on their scale wearing blue jeans, tennis shoes, and carrying my billfold and keys. Here at home today my weight of 149 is in shorts only.

Also, I’ve had notably softer stools as of late.

My plan for the near term is to be ever vigilant; taking my medication ‘on time’ every day.

Well, I've been back from the hospital for about 2 weeks. My spleen is is gone now, and I've had a hernia repair.

My PLE Hard times

Let me tell you, I know first hand how low serum protein can effect a person. You see, protein plays an important role in fluid equilibrium. Think of protein as little grains of sand that soak up water. Protein doesn’t really soak up water, but through atom polarity on a molecular level, protein holds H2O close to it (within the cells). When a large percentage of a person’s protein molecules are missing, it upsets normal osmotic pressure of the cells, and water simply runs into the tissues between the cells. This is often called, “Third Spacing”.

I’ve had as much as 60 pounds of fluid (water) third spaced at a time. Let me tell you it really sucks!

How I dealt with it? Well, I had a tough time.

I was unhappy with looking like a little fat guy. I had a hard time cleaning my self, and at one point the skin on my legs was stretched to the point of causing much pain. I had a hard time walking and could hardly bend my knees. I could barely pull my pants up from the floor, and I often had to lay with my feet and legs propped up in the air even during “fun” family events like Easter, the fourth of july and so on.

How I copped? I took it one day at a time. I cut down, way down on my salt intake. This didn’t help me as much as my doctors felt it should have.

I put my shoes on in the morning because this is when I found my fluid there to be at its lowest.

I used lotion on my legs to help allow my skin to strech.

I complained… that is, I vented my frustrations to my wife which helped me face my emotions about my condition. I didn’t want to go anywhere because I looked so bad.

I tried Beneprotein soy powder as a protein supplement. This helped my loose stools, but did little for my edema.

My doctor agreed I was in a bad way and began me on intravenous Immunoglobuluns (ivig), IV albumin, and Iv lasix. This helped some. It helped boost my immune system, helped get some of the fluid off me, and raised my albumin level if only very briefly. I was placed on a drug called Methotrexate. I was prescribed a subcutaneous injection every week at .4 mg. After the 2nd week, my doctor said to give myself an additional .4 mg which I did. That was on a Friday. By Monday, I was caughing and hacking because I suddenly developed a pneumonia like condition called methotrexate lung. The lungs fill with some weird nodules, and got really sick.

The funny thing is, about 3 weeks after that first methotrexate injection, my protein levels shot up to just about normal. This lasted for about 3 weeks and I gradually began packing on the edema again as my protein levels started falling.

I subsequently took it upon myself to try a single dose one desperate afternoon just to see what would happen. 3 weeks later my protein again shot up. But I began to develop infection like symptoms in my chest so I told my doctor what I did. Let me tell you by the time she got done with me I was promising not to do that again.

I didn’t end up with methotrexate lung again, but I did have me a scare.

Then I went to the mayo clinic. Iwas eventually diagnosed with Menetrier’s disease and was advised to try octreotide. My doctor prescribed it eventually, and a few weeks after my protein began to rise. Albumin went from less than 1 to 2.0.

My total protein went from 1.9 to 4.8.

These numbers may sound like small changes, but believe me they make a world of difference in the edema department.

The skin on my legs was so tight at one time that a shard of glass falling from counter level cut my leg with a very light glancing contact. My leg then dripped water for over a month. Every pair of pants I put on soon had a soaked leg from the shin down..

Well, this is good for now. Any questions or any stories of your own, leave them in the comments please.

Thanks,

Daniel

Your Story

If you or someone you know has been dealing with this condition, and you are tired of hearing, "There's just not much information out there on PLE, then please tell us your story in the comment section. I'll soon have a guestbook here for you to sign, as well.

You can visit my other blog, "Forward observer: Peering From a Fox Hole" for a wide variety of content. The link is www.dtaverne.blogspot.com

I wrote most of the articles there. There are also links to many funny, and/or military videos.

Thanks, and visit here often.

Protein Losing Enteropathy (PLE): My Story

By Daniel Taverne



This article, written by me, was origionally published in the American Chronicle on March 6, 2006. There have been considerable developments since then that I will post here soon.

If you have any comments, questions feel free to e-mail me or use the comment link below this post. If you’d like, I will post all of your comments in this forum as a way of shedding light on this rare condition.


First of all, let me explain why I’m writing this, and why I’m disclosing so much of my personal history. You see, as my doctors searched for the cause of my condition, I can’t tell you how many times I listened as they said, “There is just no literature on your condition”. In addition, doctors who were introduced to me in different areas of the hospital would say stuff like, “I’ve heard of you! You’re the interesting case.” Or, “You hit the lottery with this condition because you’re case is so unique.” This is where my reasoning for this disclosure comes in.

I don’t want any other person hearing that no literature exists on this condition. This, therefore, is my effort to begin some discussion on this idiopathic protein losing enteropathy (PLE). Maybe someone else will be helped more quickly than I had been.

In the spring of 2002 I began experiencing severe muscle spasms of just about every part of my body you can imagine. Spasms would render my hands useless at work, and they regularly paralyzed me with pain in my abdominal area as well. Even my scalp and facial muscles would involuntarily contort my face into some interesting yet painful positions. I didn’t have to do anything strenuous to experience them, and regularly woke from naps on the recliner to sudden severe and painful abdominal spasms that would last from 10 to 45 minutes..

Then, late summer 2002, I had an episode of swelling in my lower extremities as well as some scrotal edema. This prompted me to seek out medical evaluation where I soon discovered my serum protein was very low. Before the doctor explained anything to me, she asked me, “Do you eat?” Of course I said, “Yes.” I then listened closely as she informed me of how I had “the protein level of a child in an impoverished country”. She explained about my low albumin and its importance in regulating fluid equilibrium. Even if her scientific explanations didn’t sink in, I soon discovered for my self how important it, and other proteins in the body are.

Within a few weeks, I began feeling tearing sensations within my groin area where I was beginning to feel an inguinal hernia forming. I also felt pressure around my umbilicus as I developed some umbilical hernias. As this was happening, I began to experience pain in my toes that pegged the pain scale. This pain felt to me as if someone had a hold of my toes with a pair of pliers and was squeezing them like crazy. The smallest toe on my right foot began to necrotise.

I felt full all the time, and could only eat small portions of food at a time because fluid in my abdomen (acites) was interfering with my ability to breathe properly. I fought off bouts of cellulitis and bronchitis as well as some yet to be explained severe joint pain in my hip and knee.

I felt exhausted most of the time, and being a brick layer, I had to force my self (amid all these problems) to go to work. That’s right , even when I felt the tearing in my groin and my feet hurt like hell I still showed up to work and did the
best I could.
Soon, I referred myself to Sonny Montgomery VA Medical Center for further workup. Test I’ve had are numerous and consist of dopplars, cat scans, endogastric duodenoscopy (with biopsies), colonoscopy, interabdominal exploratory surgery with several full thickness biopsies of small intestine, heart cath, CT of entire digestive tract, tested for polyarteritis nodosia, ciliac sprue, you name it. To no avail, my doctors tried and tried to find what was causing this. I had a fecal test that showed alpha-1 antitrypsin which indicated I was losing the protein through my bowel.


Secondary to the Protein losing enteropathy (PLE) was the fact that I had hyperlipidemia, meaning that my cholesterol and triglicerides were through the roof. I additionally had a very low testosterone level which requires biweekly injections.

Over the next 2 years, I tried a number of medications, including high dose prednisone, cytoxan amuran, as well as one or two others none of which made any significant improvement. Fortunately, my doctors decided to try the drug adalimumalb which seems to have done the trick. My albumin has gone from .8 to 3.5 and my total protein has risen significantly as well.

Unfortunately, I soon discovered another problem. It was discovered that I also have osteoporosis when I reported to the emergency room for wrist pain. The doctor who looked at my wrist x-ray told me my ulna looked like I had osteopenia. It was after I had received a lumbar compression fracture while attempting to help up a fallen elderly man that I was given a bone density scan. This scan indicated severe osteoporosis.

Another problem that crept up on me my vision was diminishing. The diminishing of my vision seemed to coincide with my prednisone treatments. I had begun this prednisone treatment 3 separate times, and each time my vision suffered. The first time, my vision began to falter I took myself off the drug and my vision came back. This was the time that a Dr. McMurray of Sonny Montgomery VAMC prescribed it to me at a dose of 50 mg/day for 5 weeks.

The second time, I kept a running log of my visual changes that indicated a decline of my vision. This time I was taken off the prednisone because the doctors were considering laproscopic exploratory surgery and didn’t want my immune system weakened.

Soon, my vision began showing some improvement, though I was still legally blind. After the surgery, the 3rd round of prednisone began with IV pulse doses of 1000 mg for 3 days, then I was put on 60mg/day by mouth. My vision almost immediately started turning for the worse.

I am currently working on improving my bone density through a combination of injections and vitamins. I’m scheduled to take the injections for 1 year due to its associated risk factor of bone cancer.

Finally, my quality of life has improved much over the past 2 years although my poor vision keeps me dependant on others for transportation. I’m taking a number of drugs, in addition to a number of fat soluble vitamins such as ADEKS in multivitamin form. I have gained about 60 pounds while taking prednisone and now that I have had the dose decreased to 2.5 mg every other day, my weight seems to have begun a downward trend.