To Blog or not

Let me be frank for a moment. I hate writing. Hate it, detest it even. It's painful, coming up with the right word for the right context is hard. The thesaurus is my best writing amigo, opened so much that, if it were an actual edition, the spine would be bent in dozens of places,  falling apart at the non-virtual seams. I have times I'm more verbose and coherent but I don't always think to write, and then about what? Life is a many-faceted gem and I'm not always inspired. So to write about this dilemma of mine is but an agent to put off writing about something else that might be more deserving of the keystrokes. Ha! I did it again, pass off this writing assignment like it was sophomore biology. I also hated biology, ironically enough, given my knowledge about this stupid disease. That was more my adolescent brain than intellectual ability. But to the task at hand, I pull the cloak of defeat across my shoulders and take a bow, backing into the liquid darkness of night, and breathe a sigh of relief. 

This is ALD

I wrote this as a piece for a Facebook friend, Jesse Capello Torrey, who asked for ALD stories to feature on her blog, "Smiles and Duct Tape."

I'm 47 years old and an ABCD1 obligate, symptomatic, carrier. Out of my three sons, only my middle son, Andy, has inherited the gene. My sister is Jamie and she's a carrier. She passed the gene to both of her kids, her daughter Candice, and her son, Bradley, who passed away last July at the age of 21. Candice gave birth, with a little medical assistance, to a beautiful girl, Aurora “Rory,” on July 1, 2017, and who is not a carrier. I think it's a wonderful and beautiful story!

My mom is Paula, she's a 72 year old symptomatic carrier. She is Jamie's biological mom also. (Jamie was given up for adoption at birth by our mom. She was reunited with Mom and met me on November 3, 1999. That's when she learned of the family history of ALD.)

Mom and I suspect that the ABCD1 gene originated with my great-grandmother, Ethel.

Ethel was born in July 1895 in Kingman County, Kansas, the only child of Harry and Pearl. I'm not certain if she was brought up Mennonite, but that is the community in which she and her husband Pete, who was raised Mennonite, raised their family, on a farm close to Pretty Prairie, Kansas.

Ethel gave birth to 12 children, 7 of whom survived to adulthood. Their first, Raymond Bell, “Buddy,” was born on June 25, 1916. He passed away on July 31, 1922 after “a short illness,” and/or “summer complaint.” This raises all sorts of red flags for me, looking back at family history, not the least of which is the age, 6 years old, at which he passed. Ethel and Pete had four babies that did not live past a year old; Lawrence lived two months, Shirley lived 10 months, Norma lived a day, and Deanna died shortly after birth.

My mom does not remember any of her cousins presenting any ALD symptoms, but my grandmother's siblings spread far and wide across the United States, and some family members Mom has only met a handful of times. So that leads us to believe that the only unfortunate bearers of this stupid disease were Buddy and my grandmother, Leah.

Leah was born on April 17, 1925 in the farmhouse (as were all of the babies, the Mennonite community likely had a midwife or two to assist with the births.) She tired of the farm life and longed to live somewhere else. I don't know the circumstances that led her to be swept off her feet by John, but she was wed to him, and then had my mom on May 21, 1945. Within two years after Mom was born, my grandmother grew dissatisfied with her marriage and got a divorce from John.

She then met a veteran from WWII, Harris in/near Hutchinson, Kansas. He accepted Mom as his own. Harris and Leah went on to have 4 boys: Bill in 1950, Stuart in 1955, Mathew in 1957, and Scott in 1961. Stu died from “Schilder's Disease” when he was 6. Bill was for years misdiagnosed as having Multiple Sclerosis. When a new neurologist connected his symptoms with Stu's diagnosis, he was confirmed to have AMN. He was wheelchair bound by the end of his 30’s. He committed suicide in 1997 at the age of 46. I personally don't think that Matt had the ABCD1 gene. However, he contracted HIV in 1987 around age 30. He died from AIDS in 1993 at the age of 35. Scott had mobility issues starting in his late 20’s. He died from complications from AMN in 2001 at the age of 39.

I have three sons, Patrick, 19, Andy, 15, and Alexander, 2. Of my boys, only Andy has inherited the ABCD1 gene. We found this out when Andy was 5 months and Patrick was 4 years old. This was after much inner conflict and distress on my part. I'm glad I did, however, because it gave me time to research all possible avenues of medical therapy.

My husband-at-the-time and I decided to get Andy into the Lorenzo's Oil Study at Kennedy Krieger Institute. We had to wait until he was 18 months old before we could get him in. We went to Baltimore in July 2004 for the first time. What a crash course in how to cook and how to negotiate through the grocery store! Andy stayed with the study until 2012, 8 years in all. Kennedy Krieger's funding kept getting cut, first the MRIs got cut and I had to arrange for them in town, then the neuropsych testing got cut and it wasn't something I could get done on my own, so we made the decision to stop being in the study.

Life outside of the study has been interesting. I've been trying to teach Andy how to negotiate what he eats on a daily basis, to get him to choose lower fat options when available. He’s 15, so only so much of what I say gets heard. He gets yearly MRIs, and continues to have clear ones at that. I like to think that the L’Oil has something to do with that, but I can't be entirely certain ever. It's as if we are eternally fated to be Damocles, with the sword of ALD hanging by a mere horsehair above us, never certain when, or if, the hair will break and life as we knew it would end and our new life with ALD begins.

Orthotics!

I got them!!
I got them in January and have been trying to get used to them. It's been a challenge. But I can walk!

My resolve has slipped...

I know I've written more about my migraines than my AMN, but I am used to my horrible affliction, and the migraines come and go. The stupid headaches are my Achilles heel, the great flattener of my life. I've had 2 puking migraines this week and for the 2 days after, I've been so sore and miserable. It seems like it's taking my body longer and longer after each headache to recover, and having 2 nasty ones in a row has been hell.

So, as the title of the post suggests, I've unfortunately let myself think that I wish I was normal and healthy. I get told frequently that I have a great attitude about life in the face of my health problems, and I almost always do. I have my weaknesses, and this week I've gone over that tipping point and gone to the dark side. What if I didn't have AMN, depression, or migraines? What if I could run and jump and skip and ride a bike? What if I didn't have chronic pain and get tired easily? What is life without migraine like? I've never known what a normal life is like. I don't say that for pity, it's just a fact of my life. I don't know why God has chosen me to carry certain burdens; it's not mine to wonder why. Tis mine yet to do or die, I guess. I don't have a death wish, so on and on I go, stiff, sore, and sometimes grouchy. So please understand, I'm only human.

My body image has drastically changed in the past few years also. I'm no longer a size 2, I no longer have an hourglass figure. Because of the pump, my measurements are 35", 35", 35". I don't have to unbutton my jeans to pull them off; in fact, I have to pull them up when I stand up. I gravitate towards clothes that have a distinct waistline, then I have to remember that I don't have a distinct waistline myself. Ohhh... It used to be like a punch in the gut, but now it just makes me sigh...

My Bayard is truly a blessing to me. He accepts me as I am, health problems, sons, and all. I am lucky. I am loved.

Happy new year! (And happy news!)

Yes, I've been neglecting my baby blog the latter part of 2014, but I've been living quite the interesting life, in my opinion, as of late... On January 3, 2015, I married my best friend, Bayard Bray!

Physically, however, I reached my Medicare limit for physical therapy in October, so I was exercising with the physiotherapist, which I really didn't find to my liking. I liked working with my physical therapist, Erin. Then Bayard and I started seeing each other and fell in love rather quickly, so my time got preoccupied and I never got around to rescheduling my appointments. I hope I can go back and see Erin again. Bayard's a trained massage therapist, so he works on my neck when I need it, but it's nice to get out of the house(!)...

My darling sons are each having their own issues, not necessarily related to Bayard's being around. As a mom, I worry about them anyway, but guiding them through adolescence has seemed daunting, until now, having been a single mom. Bayard doesn't have kids of his own, so it's mainly the four of us, plus the 2 kitties. Ooh blah dee, ooh blah da, la-la-la-la-life goes on.

ANDY GOT APPROVED FOR A MAKE-A-WISH!!!!!

He wants to go to Legoland in California, the beach, and Sea World. So far, so great!


Okay, found some more information about ALD on the web:

http://www.ninds.nih.gov/disorders/adrenoleukodystrophy/adrenoleukodystrophy.htm

I have realized this sobering fact lately: that my uncle Bill committed suicide when he was 46, two years older than I am now.