Posts
15 - Tales From My Grandmother
Why Grandmother, what sharp teeth you have!
This post isn't about Porcelain, but about her grandmother. She learned the difficult struggle that her grandmother, Amalea, had led. She was also born with EPP, not that any in the 1920s knew what that was, much less how to treat it properly. Her future would prove to be a long, and bumpy road..
Amalea was 3 years old when the first reaction happened. Her family knew what was going on, and took precautions immediately. The hellish experience really began about the time she started school. Teachers either wouldn't listen or didn't care. She would often end up in the hospital, and catch every cold she came in contact with because her immune system kept dwindling from sun exposure. Her childhood was riddled with doctors visits and constant misdiagnoses. Her family tried to be advocates, but in the 20s, not a lot of doctors really cared what parents thought.
At the age of 12, Amalea left school as did most girls who didn't have aspirations of higher learning. It was common for girls to not go to high school or hold many business jobs higher than secretarial work. She stayed at home helping her mother clean and care for her younger siblings. Amalea felt relief in not having to deal with the outside world much and was perfectly happy learning to be a homemaker. Through the years, she would also be misdiagnosed with UTIs when she was having kidney and gallbladder problems, a fatty liver when the porphyrins built up in the liver, and elevated cholesterol. All of these were, of course, caused by sun exposure. Doctors had often told her parents it must have all been in her head, and she had a psychological disorder.
Then she met him. Porcelain's grandfather, Gregorio. “He was the most handsome man and walked with the confidence of a leader.” A very dapper Italian with his hat and cane, though he didn't need one - it was for looks. Amalea was also quite psychic and had dreamt of meeting him, immediately knowing who he was when she first saw him. Little did the two know, their families set them up. It was customary at the time to have arranged marriages. However, Porcelain's family didn't like forcing people together. They prefered to play matchmaker! It was so much more fun!
The two hit it off and had their first date at a USO. Gregorio was an officer in the U.S. Army. Amalea was thoroughly impressed but more so, quickly fell in love with his sarcastic humor. It was a success! He loved Amalea's attitude and emotional strength. He felt it was love at first sight and had found his one. Their family was quite good at pairing couples! They became engaged after courting for about a year, as was customary for her family. Couples must court for a year, be engaged for at least a year, then they could marry. It was to make sure the relationship was going to last. Neither family didn't like divorce, unless it was completely necessary.
Then WW2 interrupted their whirlwind romance. This time Gregorio was being sent straight to the most dangerous parts. Amalea feared for his life, but coming from a military family herself, she understood the dangers and told him she would “wait for him until the end of time”. They kissed goodbye and off he went. As you already know, the Allied forces won. Gregorio came back to his Amalea and they were married at once!
During his time away, Amalea began working in a factory to help. She learned very fast that the heat was affecting her and she passed out quite a bit in the summer. The doctor diagnosed her with high blood pressure, even though she knew that wasn’t the problem. There wasn't a medical term yet known in the US that could describe her reactions and why she didn't sweat. She was prescribed pills, but didn't take them. She knew better. Unfortunately, they also began diagnosing her with all sorts of things as she grew older, which led to major health problems and mini-strokes that went unnoticed until it was too late...
She settled back down into the homemaking role as fast as she could. Over the years, they had 7 children, although they didn't come easy. Amalea wanted a large family, but had several miscarriages. She was able to have a child about every 5 years. It took a toll on her body, but she was the happiest mother in the world. Unfortunately, when she finally hit menopause in her late 50s, the doctor said she was going to have a heart attack if she didn't start taking medicine for her blood pressure. Wanting to be around for her kids, she finally gave in. It was a horrible mistake.
Amalea died at the age of 63 years old. She looked as if she were 50 with long, silver hair. She had decided to stop taking the medication after Gregorio passed away 3 years prior. It hadn't helped and she was still having issues regardless of all the extra medicine the doctors kept putting her on. She had so many pills that made her sick, but she was trying to listen to the doctors. In her heart, she knew it was all wrong.
Porcelain knew something was wrong. She had a dream that someone important was missing and she felt it was her grandmother. She told her this, and Amalea told her not to worry, when it was her time to go, she would be with Porcelain's grandfather and happy again. All her children were adults and had families of their own. She had done her job. Amalea had a heart attack the next night. Porcelain ran to her side and held her hand. Amalea told her, "Do not fret child, I will see you in the next life."
Life can be very hard. My grandmother had a wonderful life with my grandfather, and 7 amazing children. She grew up in a time where no doctor really understood anything about porphyria. Today, that hasn't changed much. There are experts out there, but not nearly enough. For rare blood disorders, such as 8 different types of porphyria, there's only a handful of doctors that have studied it, and you often have to travel to them. The regular doctors are mostly clueless; you’ll be diagnosed with a thousand things before they even consider porphyria. Every porphyria has its own test. EPP has 2 distinct blood tests - urine isn't required. EPP & X-Linked porphyrias are the rarest of all.
Is it any surprise we are the most misunderstood?
Lesson for my readers: You do not have to accept every diagnosis the doctor tells you. Always get second and third opinions. There are many pharmaceutical drugs people with porphyria cannot take. Sulfites & Sulfa drugs are one of the biggest no-nos. I will include the "no-no" list from the American Porphyria Foundation for you. It might just save your life one day.
Links for your viewing pleasure:
*I have a link for tests on the blog already*
The “no-no" list of drugs for all the porphyrias: *There's a lot*
https://porphyriafoundation.org/drug_database/print.php
I made a separate blog post for all the diseases that Porphyria can mimic. It's a long one.
This post isn't about Porcelain, but about her grandmother. She learned the difficult struggle that her grandmother, Amalea, had led. She was also born with EPP, not that any in the 1920s knew what that was, much less how to treat it properly. Her future would prove to be a long, and bumpy road..
Amalea was 3 years old when the first reaction happened. Her family knew what was going on, and took precautions immediately. The hellish experience really began about the time she started school. Teachers either wouldn't listen or didn't care. She would often end up in the hospital, and catch every cold she came in contact with because her immune system kept dwindling from sun exposure. Her childhood was riddled with doctors visits and constant misdiagnoses. Her family tried to be advocates, but in the 20s, not a lot of doctors really cared what parents thought.
At the age of 12, Amalea left school as did most girls who didn't have aspirations of higher learning. It was common for girls to not go to high school or hold many business jobs higher than secretarial work. She stayed at home helping her mother clean and care for her younger siblings. Amalea felt relief in not having to deal with the outside world much and was perfectly happy learning to be a homemaker. Through the years, she would also be misdiagnosed with UTIs when she was having kidney and gallbladder problems, a fatty liver when the porphyrins built up in the liver, and elevated cholesterol. All of these were, of course, caused by sun exposure. Doctors had often told her parents it must have all been in her head, and she had a psychological disorder.
Then she met him. Porcelain's grandfather, Gregorio. “He was the most handsome man and walked with the confidence of a leader.” A very dapper Italian with his hat and cane, though he didn't need one - it was for looks. Amalea was also quite psychic and had dreamt of meeting him, immediately knowing who he was when she first saw him. Little did the two know, their families set them up. It was customary at the time to have arranged marriages. However, Porcelain's family didn't like forcing people together. They prefered to play matchmaker! It was so much more fun!
The two hit it off and had their first date at a USO. Gregorio was an officer in the U.S. Army. Amalea was thoroughly impressed but more so, quickly fell in love with his sarcastic humor. It was a success! He loved Amalea's attitude and emotional strength. He felt it was love at first sight and had found his one. Their family was quite good at pairing couples! They became engaged after courting for about a year, as was customary for her family. Couples must court for a year, be engaged for at least a year, then they could marry. It was to make sure the relationship was going to last. Neither family didn't like divorce, unless it was completely necessary.
Then WW2 interrupted their whirlwind romance. This time Gregorio was being sent straight to the most dangerous parts. Amalea feared for his life, but coming from a military family herself, she understood the dangers and told him she would “wait for him until the end of time”. They kissed goodbye and off he went. As you already know, the Allied forces won. Gregorio came back to his Amalea and they were married at once!
During his time away, Amalea began working in a factory to help. She learned very fast that the heat was affecting her and she passed out quite a bit in the summer. The doctor diagnosed her with high blood pressure, even though she knew that wasn’t the problem. There wasn't a medical term yet known in the US that could describe her reactions and why she didn't sweat. She was prescribed pills, but didn't take them. She knew better. Unfortunately, they also began diagnosing her with all sorts of things as she grew older, which led to major health problems and mini-strokes that went unnoticed until it was too late...
She settled back down into the homemaking role as fast as she could. Over the years, they had 7 children, although they didn't come easy. Amalea wanted a large family, but had several miscarriages. She was able to have a child about every 5 years. It took a toll on her body, but she was the happiest mother in the world. Unfortunately, when she finally hit menopause in her late 50s, the doctor said she was going to have a heart attack if she didn't start taking medicine for her blood pressure. Wanting to be around for her kids, she finally gave in. It was a horrible mistake.
Amalea died at the age of 63 years old. She looked as if she were 50 with long, silver hair. She had decided to stop taking the medication after Gregorio passed away 3 years prior. It hadn't helped and she was still having issues regardless of all the extra medicine the doctors kept putting her on. She had so many pills that made her sick, but she was trying to listen to the doctors. In her heart, she knew it was all wrong.
Porcelain knew something was wrong. She had a dream that someone important was missing and she felt it was her grandmother. She told her this, and Amalea told her not to worry, when it was her time to go, she would be with Porcelain's grandfather and happy again. All her children were adults and had families of their own. She had done her job. Amalea had a heart attack the next night. Porcelain ran to her side and held her hand. Amalea told her, "Do not fret child, I will see you in the next life."
Life can be very hard. My grandmother had a wonderful life with my grandfather, and 7 amazing children. She grew up in a time where no doctor really understood anything about porphyria. Today, that hasn't changed much. There are experts out there, but not nearly enough. For rare blood disorders, such as 8 different types of porphyria, there's only a handful of doctors that have studied it, and you often have to travel to them. The regular doctors are mostly clueless; you’ll be diagnosed with a thousand things before they even consider porphyria. Every porphyria has its own test. EPP has 2 distinct blood tests - urine isn't required. EPP & X-Linked porphyrias are the rarest of all.
Is it any surprise we are the most misunderstood?
Lesson for my readers: You do not have to accept every diagnosis the doctor tells you. Always get second and third opinions. There are many pharmaceutical drugs people with porphyria cannot take. Sulfites & Sulfa drugs are one of the biggest no-nos. I will include the "no-no" list from the American Porphyria Foundation for you. It might just save your life one day.
Links for your viewing pleasure:
*I have a link for tests on the blog already*
The “no-no" list of drugs for all the porphyrias: *There's a lot*
https://porphyriafoundation.org/drug_database/print.php
I made a separate blog post for all the diseases that Porphyria can mimic. It's a long one.
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