I just never know anymore how to feel. I get so darned tired so often that in some respects it is just too much trouble to get dressed, make a plan, leave the couch and go out the door.
In addition, as the head of my household, I run a business, too. My business requires I do everything.
So I keep pushing myself in the hopes that someday all this disorder will go away. Lots of luck I know. Enzyme mutation studies are only at the plant level and animals studies have yet to begin. But I can continue to dream. Not really reality, but it gets me through some days.
And then I know that I am getting worse by the year and no one is holding out hope that it will get better. All in all it has been 27 years since I became acute.
All I can do at this point is put one foot ahead of the other. I got through many activities in life because I have too, although I get so tired it is so hard for me to imagine that I have actually done so.
My life with porph is hell. SImple and to the point. I knew there was something wrong when I was in high school because other girls did not have the horrible time I did before menses.
In college there were a few times when I was so sick I wished that I could have died. Once was when the university was busy sprucing up the campus just before the opening of the new quarter. New asphalt was laid in every parking lot. New tar was being pour on all of the roof tops. Inside the building where I was a work study student new tile were being laid with sticky glue which caused me to literally vomit all over the hallway before reaching a restoom.
Again I went to the hospital and spent five days. And once again it was considered psychological. Sure it was!
In the hours and days that followed my skin blistered and itched and burned to no end. Scars were visible for months afterward.
I ended up having to go an extra semester in order to graduate because I was sick so much and could not get papers finished on time.
My fiance and I went on a fraternity picnic and it was on the country near irrigation canals. While there the county road crews were busy spraying for weeds along the road. Well the spray drifted our way and the picnic was all over for me.
I ended up in the hospital and after a week was released and told that there was nothing wrong with me. Northing, my eye!!!
The next time was when I got married. It was a beautiful affair and at the reception I downed a glass of champagne. DOesn;t every bride?
Two hours later my groom had me in an emergency room as I was vomiting, having seizures, and hallucinating. They ran electrolytes and glucose for four days and then released me. Again they said there was no medical reason for my condition.
We had three wonderful babies over the next five years. The sixth year I was pregnant again and 5 months and two weeks into the pregnancy I had massive seizures, bleeding, and respiratory paralysis and was placed on a respirator. The baby was spontaneously aborted.
Then started the ping pong of depression and mood swings. The psychotherapist said that I seem perfectly normal except just prior to menses. Because of complications from the fourth pregnancies I was advised to undergo a hysterectomy.
In additon the psychotherapist advised my personal physician to place me on anti-depressants because she felt I had a chemical imbalance. Again a few days of the medication and I was in "outer space" vomiting, hallucinating, and with severe ab pain. The pills were tossed and immediately I was much improved although still with slight depression before menses.
I had the hysterectomy six months later and then the OB-GYN decided that because of my youth I needed to be on estrogen replacements.
I was started and immediately had another series of attacks along with deep vein trombosis and pulmonary emboli as a result of the blood clots stemming from the Premarin.
The next thing was some long overdue dental work I needed to have done. I went to the dental surgeon and was given the anesthetic to prepare for the oral surgery. The root canal would be long and tedious. The drug used however put a stop to the whole thing. I hallucinated in the chair, vomited all the way to the restroom, grabbed my gut which agonized in extreme stabbing pain. An ambulance was called as I became incoherent.
After five days of glucose and observation along with more tests which included the urinary porphyrin tests, I was released and told that "it was all in my head".
Five years later will driving the kids to school a car failed to stop at a red light and I was side-swiped landing me in the hospital. I was in and out and unable to tell them anything about my past medical history, not that I had a name for the porphyria at that time. I was given a sleeping pill and it had quite the opposite effect. I was fully awake, screaming in pain, vomiting, and grabbing my gut. Of course doctors exrayed and scoped and could find no cause for my pain. Once stablized I was sent home and again told that I should seek psychological help.
This repeated abuse by doctor by telling me it was psychological was beginning to take a hold on me. I felt my self worth, my self esteem, and abilities all to begin to dwindle. My depression worsened.
Then I was at the Farmers Co-op one day getting lawn seed and some men were loading farm chemicals. One container erupted when it accidentally rolled off of the loading platform. I was severely exposed to the chemical and immediately had reaction.
I went home however and called my own doctor who had my husband come and get the container. I finished the 24 hour test and was still having problems. My doctor then called me and told me to have my husband take me to the hospital. They began the glucose and some of the nurses remembering me asked if I was not on the wrong floor as I would probably do better in the psych ward. Activities there would keep my mind off of my imaginary illness.
I was fuming mad and could hardly wait for my doctor to put in an appearance. However instead of me jumping on him at his arrival, he told me a spot test proved the porphyria and the 24 test would be ready within the next day. It was not in my head.
I felt validation and wanted o grab him and hug him to no end. He said that the psychotherapist felt that it was a physical condition but was unsure of what. Now we knew.
I thought I was done with the therapist, but the doctor sent her in to see me. She said I needed to plan for the future with this horrible disease and that I needed to be strong, straightforward and not afraid to speak my mind. That like most patients of rare or little known diseases the patient has to be their own educator, spokesperson, and fight the system for health care coverage, and for the health care itself. She was so right. Doesn't this all sound so familiar to you?
Since then I have been hospitalized 32 times. I am in an active support group of four. We live about 20 miles apart, but we plan get togethers to share our stories and our ups and downs and any new revelations we may have come upon.
Trips to the supermarket are like trips to hell because of the central location of the laundry supplies. The soaps are verytoxic to me. The next aisle has the Rubbermaid and like products. I can not go in that aisle because nausea will be instant and the skin will itch and burn.
Many stores I can not go into because of the lighting upon the ceiling. My skin will instantly burn and itch until I leave the store and the lighting.
The finer department stores with their cosmetic counters are almost death for me. Each time I have been hospitalized for having passed through a cosmetic area of a store in a mall.
At church and social gatherings the women with heavy perfume are toxic to me and again nausea will begin. Men was heavy aftershave are equally offending to me.
When I am fortunate enough to leave home I have the problem of bleaches and laundry soaps used on bed linens. Nighttime sleep is impossible. Restlessness and heavy sweating and a rash will appear, along with the skin burning and itching.
My support group is my extended family here in Missouri. I would not trade it for anything. My doctor is my hero and has become like family as well.
I have progressed in symptomology. I am now fairly well restricted in movement of my limbs. I have much numbness and I have a tank of H2o everyway I go. One certainly can not be shy with porphyria. You have to garner everything you've got and continue on. Your have to for yourself, for your family, and for other porphyria patients.
And I still have the dream. Maybe tomorrow I will wake up and it will all have gone away. But then tomorrow comes and I think...well maybe tomorrow's tomorrow!
Mary Kay Adams HCP/MCS /
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