Reba B. - Owensville, IN 2020
Ichthyosis: Blessing or Curse
In 1937 Mrs. Floral Emerson birthed a very normal red-headed baby girl except for her skin, the largest organ of the body. She was not only red-headed, but abnormally red-skinned, which later developed water blisters and the beginnings of a life-long care of skin, which is definitely the cursed side of ichthyosis. Primary doctors and dermatologists practicing in 1937 knew little about the disease, possibly not even the medical term for it. The little girl was named Reba Carol and her paternal grandfather said she looked exactly like him – NO TEETH and NO HAIR (except for a dark auburn curl at the back of the neck.)
My wonderful, loving mother (blessing) had a very mild case which doctors called “dry skin” and she ignored it in the 1920’s, except for use of lotions and creams until her only child was born. Then we became researchers for dermatologists who knew little about our skin, only the term “ichthyosis.” Most doctors were very interested in learning more about our skin, but some admitted they weren’t knowledgeable about it.
I was so blessed to have a wonderful mother who encouraged me to be “normal” in everything. She spent hours with baths, white lotion that exfoliated my dry, ugly skin and made my skin look somewhat normal. It was rather difficult for mother and me in primary school when the kids preferred not to hold my not-so-soft hand when playing games, but they liked me and I understood their positions – we were different only on the outside. Thankfully, mother did not limit my social activities or childhood normal play activities. When I fell on the gravel playground, a normal floor burn was a raw flesh wound. The teacher called the nurse, the principal, other teachers and everyone became quite concerned. The next day this would be a neat little scab and I was ready to play again. The school staff was amazed. (Another blessing of ichthyosis – we heal very quickly because of the rapid production of skin.)
I graduated from eighth grade and life was not too difficult. I had church friends that knew me and my skin and school friends for whom my grandmother and mother cooked many wonderful farm meals and we had fun at my home usually overnight.
When I went to high school, I was not a happy student. I felt that my skin was a deterrent and the majority of the classmates were “city kids” and from another grade school. I joined the marching band and my dear family purchased the instrument and always taught perseverance, so I felt I must “stick-it-out.” Needless to say, marching and practicing on very hot days was not a plus for me and I was not going to admit it was my skin. After two years of high school in that large school, I changed to another school because we lived on the township line and I could go there with my church friends. I did not change because of low grades or some of my former school friends, but because I was very unhappy.
At the smaller high school, with people who I understood and they understood me, I was very happy. The music teacher even asked me to show some marching maneuvers for the band. I was in all the theater productions, sang solos for between acts, was in most of the organizations and graduated with good grades and feeling very normal.
What about my future? My church had a liberal arts college about thirty miles from my home, but if I went to college, I wanted to stay on campus. In the year 1955, I enrolled in Oakland City College, now Oakland City University, not having any idea what I wanted to pursue. Thanks to my dear mother for working telephone switchboard, so that her daughter could get a college education.
When Mother moved me into the dormitory, there were junior and senior guys helping move in freshmen. God did bless me with nice auburn hair and with much care my skin was not too noticeable, but I feel the good training at home and development of a real care for others helped my personality. The one junior guy that helped unload my clothes phoned me on the one phone on my floor of the dorm. (All the girls gathered around when you received a call.) This call was from Johnny asking me for a date. (That’s what they did in those days.) The reason for this explanation is that I dated him for two or three years and he did not once mention my ugly skin! (Good cover-up!) He encouraged me to join every organization on campus – theater, choir, Student Christian Association, Alpha Phi Gamma, Alpha Psi Omega and others. With his encouragement during my freshman year, I tried out for cheerleader and made it all four years. What happened to Johnny? He asked me to go to the mission field with him and this was not my calling.
I decided to major in education and became a teacher. I had a major in English, Physical Education and a minor in Music. I would be licensed in Middle School and high school, but I thought I’d like to be able to teach in elementary. So, with a few more credits, I could be licensed in elementary education and I did that. I graduated cum laude and went on to pursue my master’s degree in education.
I taught a total of thirty-two years and retired in 1998.
I’m writing all this to say: We ichthyosis skin kids can lead a normal life. It is sometimes a bit difficult and requires much more skin care, but it can be done!
During my senior year of college, a transfer student from our capitol city, Indianapolis, enrolled as a junior at Oakland City College. He immediately became known as the guy with the beautiful voice and extreme talent musically. He was the item of attention at every gathering, especially if a piano was near. He could play anything and did. He asked me for a date after choir one day. We had little in common because he was from the big city and I was from the farm, but we both loved music and I was very impressed with his voice, musical talent, and good looks. We sang duets often and he accompanied. During our three years of dating, I had two or three severe skin infections. They made me sick and I had to get antibiotics (penicillin, then) immediately. He was very understanding about this. We were married after my first year of teaching. We lived in the beautiful farmhouse that my paternal grandfather gave us and we worked together to remodel and restore. He became a teacher of music education and band director at a small school in our area and then he was hired as a choir and music education teacher at the large high school that I attended for two years. He was also Minister of Music for a church located near our home.
After five years of marriage, I became pregnant and my pregnancy was so easy, but the whole nine months, I was trusting and praying that I would be fortunate and not have a child who would have to care for her skin as I had. Also, the thoughts of all the loving care my mother mad given me because I knew the statistics concerning my chances.
On April 9, 1966 a beautiful baby girl with blond hair and beautiful brown eyes was born to us. She was immediately put into isolation and we viewed her through glass. They told me she had a small blister on her little bottom and until a specialist could see her, she would remain in isolation. The medical staff did not know what was wrong, but her mother knew exactly. They called a dermatologist. I don’t think that he knew what to call the condition (wish he’d asked me!)
After we were home from the hospital about four days, my husband had a bicycle trip with his Madrigal singers, but my dear mother was with us. The dermatologist told me when leaving the hospital not to use any water, except her diaper area and put her in a zip-up gown, covered completely. She developed a crust-like growth at the top of her head and it progressed down and was near her eyes. The doctor rushed her to Riley Hospital in Indianapolis, Indiana which is three hours away. I finally contacted her father and we were Indianapolis bound with bassinet in back seat of the Volkswagon. We got into road repair and my husband took to the median. A police stopped him and he looked into the bassinet and immediately gave us a police escort to Riley Hospital. Within hours, the staff at Riley had all the crust (for better word) removed and she looked beautiful. She remained in isolation at Riley for about five days. How they removed this, I do not know nor do I want to know. Many thanks to Riley Hospital because she looked so good. The beginning of another journey with ichthyosis!
Becca’s mother had a very rough time accepting the fact that her beautiful little girl had to be cursed with this rare skin problem too!
I started my trek to locate a doctor doing research on medication for ichthyosis.
I called NIH, National Institutes of Health, Bethesda, Maryland. They referred me to Dr. Phillip Frost in Miami, Florida. He was the only doctor doing any research in ichthyosis at that time.
We put the bassinet in the little Volkswagon and we were Miami bound! In the meantime, I contacted with Marjorie Locke, who grew up in Carmi, Illinois, just across the river from Indiana, and she worked for a doctor in Miami. She had gone to college in Miami because of her skin problem, ichthyosis, thinking the salt water, warm climate, etc. helped her skin. She married a Floridian and they still live in Florida. When we reached Miami we immediately got a motel and my husband watched the baby and I drove across Miami to talk about our ichthyosis and Dr. Frost. (A most informative trip, but rather dangerous for a small-town farm gal to travel across the city of Miami alone.) The next day was our appointment with Dr. Frost. He informed us that his research was in classifying the types of ichthyosis and after a bit of research and testing both Becca and myself, he diagnosed us with congenital Epidermolytic hyperkeratosis ichthyosis. Nice to know, but I wanted a treatment or cure.
Dr. Frost saw us about every six months for a while and at two years of age, Becca spent about three weeks with two other children with her problem in St. Joseph Hospital in Miami, testing vitamin A acid creams etc. on three little girls, Becca being the youngest. (She was a very good two year old patient.) This location was where we met a friend, Jana Silfe and her mother, Dean (very nice people from Alabama.)
I, too, tested vitamin A acid creams, etc. for Dr. Frost. (We killed two birds with one stone.) Then Dr. Frost’s government grant was discontinued and his research stopped. This testing was later called Retin-A.
When Becca was two years old, we moved about a quarter mile from our home to a new home because my husband and cousin wanted new homes. I was not teaching at this time because when I found out that I was pregnant, I was teaching physical education and I wanted nothing to go wrong during my pregnancy. So, until Becca was in school, I did not teach public school, but I taught a few night classes at Oakland City College. My mother cared for Becca while I taught to help paya for a new home. One evening before I went to get Becca at Mother’s, I stopped at home and found a note on my kitchen bar from my husband saying he wanted out of our marriage. I was very shocked and surprised because he wasn’t at home a lot, but with a musician, this is expected. After my divorce, it was necessary for me to go back to teaching in order to pay for a new home and loads of medical bills. Later, I learned he had married his accomplished organist.
I was very fortunate to obtain a position of teaching in my home school where I had taught previously. Becca and I with my dear mother’s help got along fine. I certainly admire all you fathers who take such devoted interest in your child’s problem and help the mothers in their search for help. Becca was in kindergarten at the same school that I would be teaching and this was a blessing! She probably didn’t always agree with this idea.
At about the age of two or two and a half, I taught Becca to say “congenital epidermolytic hyperkeratosis ichthyosis.” When anyone said “Honey, what’s wrong with your skin?” she told them. The answer was usually “Oh!”
Even with teaching and keeping my Becca in school, I had to have some answers about our skin. I heard of Dr. Goldsmith’s research at Duke University, so Becca and I spent three weeks there one summer, testing vitamin A acid creams, etc. This became our summer vacation with beautiful scenery and meeting some nice people.
I then read of Dr. Willliam Schorr of Marshfield, Wisconsin. He too was researching in the ichthyosis area. We wrote to him and he encouraged us to come there for three weeks of testing. We both spent another three weeks in the pediatrics wing of the Marshfield clinic and Hospital again testing vitamin A acid creams (Retin-A) Dr. Schorr saw us about every six months for several years.
One of the blessings of this adventure was Dr. Schorr’s nurse, Mrs. Brusky. During our stay she invited us to her home and took us on a tour of the dairy farms of Wisconsin and made the trip fun for both of us.
When we were with Dr. Frost in Miami, he mentioned that possibly very soon in Europe they would have a systemic medicine rather than just topical treatment which later was known as R.O.-10-9359. Dr. Frost gave me the name Dr. Klauss Wolfe, Innsbruck, Austria. After doing some investigating, I found this pill was distributed in Europe, but not in the United States, but this idea never left my mind (not so many creams, lotions etc. to care for my skin.)
Dr. Schorr and I discussed this pill and he said that he knew of it but he said, “If you were my wife and Becca were my daughter, I would not prescribe the drug because by age sixty-five you probably would need to be in a wheel chair because of bone damage. “I just briefly forgot about Dr. Klaus Wolff, but then I started thinking how wonderful it would be not to have to use all those creams bath oils etc. I wrote Dr. Wolff in Innsbruck, Austria. Then I started talking to travel bureaus and finally found a flight which had special rates and a rail pass fee. With God’s help and my mother’s approval, I found myself traveling to Europe-just the two of us.
The travel agents in the US set us up for a Holiday Inn because I did not speak any German. When our appointment time came, I showed him the address and he reacted strangely. We went one block and he said, “this is it!” I felt rather stupid but it was nice to be so close to the hospital. We met Dr. Wolff, a typical researcher, for about ten minutes. The rest of the time we met with his female assistant. We had to find her in the hospital and she brought us two handfuls of pills and our orders, but this was after blood work, etc. This was RO-9359 later named Tegison. We were checked every day for five days. We had to promise to have blood work done when we got to the US. When leaving Europe, we packed all our socks and underwear with pills and hoped and prayed that customs would not take them. We returned to the US without any problems with customs in Chicago. When my mother met us at the airport she said, “She looks beautiful. It’s a miracle.” We left every norming while in Innsbruck and carpet of skin – Becca a complete coat of skin and myself some.
Because of distance Dr. Shorr referred us to Dr. James Zolla in Florence, Kentucky to do our blood work and send results to Europe.
After I had taken 60 mg pills for about four months, I began to lose hair and the texture was very stiff and unmanageable. I had to wear a hairpiece for about nine months, but no one knew. I lessened my dosage to 25 mg. and my hair grew back. Dr. Wolff sent pills for about a year and then he would send 200 pills and they would never reach us and finally we could not get them, whether customs or just bad mail service, we didn’t know. This (that helped us so) could not be obtained. Dr. Zolla informed me that Dr. Comissa at Ohio State was researching Acutane and we might get into this test group. We contacted him and he gave us Acutane. It helped but never as much as Tegison.
Becca was now a junior in high school and her science club planned a trip to Europe and the sponsor asked me to go too. I wrote Dr. Wolff to see if it would fit into our itinerary to visit a doctor and obtain Tegison. He sent a prescription for purchasing it in Switzerland. It was very expensive and Becca was only taking 25 mg a day and maintaining her skin (She needed 50 or 60 mg). It was free before because it was still being tested. It was then on the market in Switzerland. I took Acutane which could be purchased in the US, so that Becca could have Tegison.
Becca did very well in school. She had many friends and because water or chlorine seemed to help her skin, she and her next-door friend and cousin persuaded me to install a swimming pool. “We’ll be glad to take care of it.” Promises are easy at age 10. Mother gave in and put in an inground pool and the upkeep lasted about a month. Then it was “my baby”. It was good for her skin, but after 38 years, the pool still exists, but Becca seldom uses it and you can’t sell in inground pool. (Another lesson learned.)
Becca did well in school and had her mother as her teacher for three years (gr 6-7-8) English.
After graduation from high school, Becca had opportunities to pursue a college education, but college was not in her planning. She worked as a very good waitress, went to beauty school and graduated, did an excellent job as hairdresser, then started working at the Evansville, Indiana airport and was an often-requested agent for frequent flyers etc. She loved her job and continued there for 16 ½ . She is now working for “Meetings and Events” International sort of a travel agent for CEOs and doctors. She is a “people person” and misses the airport and its passengers.
As for our present medications – for a short time we could purchase Fegison in the United States. Becca preferred Acutane and had good insurance to purchase the high-priced medication. Tegison went off the market and I started using Acutane. Soon, long-term Acutane came off the market and both of us use its substitute with fewer side effects, Soriatane. It too, is very expensive. I purchase it from Canada via United Kingdom and take 10 mg per day. Becca’s insurance makes hers affordable and she uses 25 mg (sometimes two per day). She also uses creams and lotions after lots of bathing. Vaseline for lubrication is her favorite. She sometimes uses Albolene Moisturizing cleanser for lubrication also.
In the late 1980’s, I found my neck had very little mobility and I was not nearly as agile and active. After many bone exams-x-ray-MRI-bone density, etc. I was diagnosed with DISH, diffuse idiopathic skeletal hyperostosis, osteoarthritis stenosis, etc. of the spine and pain into my hips. Dr. Schorr’s words resound in my ears constantly, but you think of the NOW and it’s difficult to project into the future. I see two rheumatologists, and endocrinologist, a pain clinic, two dermatologists, and my primary doctor. These bone problems are things I probably will have to live with all my life. At 77 years of age, I am reasonably active and still able to live by myself. Walking is becoming more difficult, but my skin pill has been a God-send even with all the side effects.
On August 7, 1985 I was married to an old high school friend whose wife died and we were happily married for 23 years. He had two grown daughters that seemed to like my family. He passed away in 2008 and I live in my house on about 3 acres and my cousin farms the farm.
Becca’s second marriage was September 1, 2012 at Gatlinburg to Jeffrey Ott. They live in my old homeplace and very near. I’m happy that they are happy.
My skin care regimen is as follows:
- Albolene Moisturizing Cleanser – every pm over most of body, used for lubrication and moisturization
- Shower every other day as follows:
- Soap with good bar soap
- Soap with good body wash and rinse
- Apply “olay moisturinse in-shower body lotion” and rinse well
- Apply baby oil or a good bath oil
- Apply Glycerine and Rose Water
- No towel drying
- Apply a good body lotion
- Sponge bath the next morning. I haven’t taken a bath tub bath for many years due to the bone problems.