Introducing "Miss Erinne"
Erinne Rose Williams is an angel-on-loan. She is a gift from God. I feel very honored to have been chosen to be her Mommy.
When Erinne was born on May 16, 1997, she appeared perfectly healthy in every way -- 10 fingers, 10 toes, 2 beautiful blue eyes and one cute nose. I had genetic testing done while I was pregnant with her because I was considered an "older mother" at age 36. Every test came back normal. (However, I now know that they can't test for every single disease or condition. It is just not feasible. Since there was no history of neuromuscular disease in either side of the family, they had no reason to check for it.) Erinne had a little bit of a problem coming down the birth canal and fractured her clavicle on the way out. She did not spontaneously breathe at birth, and had to be resuscitated. The docs surmised it was because of the pain of the fractured clavicle -- but now I wonder.
Erinne's growth and development seemed right on track for the first 7 months of her life. But then I started noticing that she was missing some important gross motor milestones -- pulling up to standing, cruising along furniture and walking come to mind. Then she started losing some skills she had mastered - crawling normally (as opposed to a belly crawl) and then getting to a sitting position by herself. At first I was told I was expecting too much and measuring her against her siblings who walked very early (Claire at 9 mos and Kevin at 8.5 mos) but I knew that was not it. I have worked with young children all of my life and I know what is normal and what isn't. And Erinne wasn't. We made the rounds of various specialists for a couple of months. I was told that an MRI showed she had tumor on her spine which was causing the problem, and she needed surgery to correct it. I took her to other hospitals for a second and third opinions which all said the same thing. Then we went back to the first surgeon and I told him I did not believe Erinne's problem was surgical and I wanted more tests done. So then he sent us to a pediatric neurologist who diagnosed her -- after just a few minutes of watching her -- with Spinal Muscular Atrophy What the heck is that? The doctor sent her blood off to a lab for confirmation and also did an EMG and a nerve conduction study (VERY PAINFUL TESTS).
It all came back positive for SMA. I left there, stunned. I had no idea what SMA was, and what her prognosis was. I was told there was no treatment available, nothing could be done and I was just to take her home and love her for however long I would have her. I later learned that SMA is a motor neuron disease much like Lou Gehrig's disease (ALS) only for little kids. I also learned that she would probably die before she reached her 8th birthday. Someone recently asked me how I felt after that, and what kinds of emotions I had and I had to honestly say I don't remember any of it. I was in a fog and everything was, and is, a blur. But one thing I do remember is that I was never angry at God for giving me Erinne. I honestly never asked, "Why me?" My life has been so blessed by her. I look at life a whole lot differently through her eyes. Accomplishments I never fully appreciated with Claire and Kevin - and in fact, took for granted -- I now treasure with Erinne.
Erinne has certainly had more than her fair share of struggles, trials and tribulations. She has had 21 pneumonias, 14 surgeries and 25 hospitalizations. She spent the summer of 2001 (May - September) on life support due to unforeseen post-op complications and was not expected to survive. During that time, she was hospitalized for 101 days and was on a ventilator for 85 of those days. She 'coded' (arrested) 5 times during that period. She failed 3 times to come off the ventilator. She was in multiple organ failure and her body was shutting down. She was just 4 years old. I was told that I needed to accept reality and that my daughter was never going to come back as the child she once was. I was told to let her go. But I couldn't -- I KNEW it wasn't her time. I knew that she still had so much yet to accomplish on this earth. Her work wasn't done. I knew that. I never left her side. I was afraid that if I did leave, then she would leave too, and fly home to Heaven. So I physically kept my hands on her, keeping her there -- with me. Many times her survival was hour-to-hour. Some times minute-to-minute. To this day, I don't know how we got through it. I look back at some of the journal entries I made during that time and it is difficult for me to read them. So much pain and anguish in those words.
In September 2001 I was finally able to bring my daughter home. Erinne was 4 years old, and a very frail 26 pounds. She did not have the strength to raise her finger to scratch her cheek. She could not hold her head up or sit up in her wheelchair anymore. In February of 2002, I started her on a very specialized tube feeding diet created by other moms of SMA children -- called the Amino Acid diet. I started her on a specialized respiratory protocol called NIV or non-invasive ventilation, instituted by Dr. John Bach in New Jersey. Both of these were not considered to be part of mainstream medical treatments. I took a lot of flack from some members of the medical profession (not her pediatrician or pediatric physiatrist, though) for putting Erinne through these unorthodox (?) treatments. Just 22 months later, I had a healthy, vibrant 6 1/2 year old who now weighed 56 lbs (a gain of 30 lbs!) and grew 12 inches in that 22 month period of time. All because of the AA diet and NIV protocol. The before and after pictures of her are shocking. Erinne got stronger and stronger and never looked back.
Erinne is now 17 yrs old!! Older than anyone ever thought she would live to be. She is now a senior in HIGH SCHOOL (wow!) in regular classes and is an excellent student. Her intelligence continues to amaze me. Erinne currently plays defense on a wheelchair hockey team (over 50 some players, mostly young men and only 3 players are girls. Erinne's isn't the least bit intimidated by it either!) In the past has played in a wheelchair soccer league and Miracle League Baseball League. Erinne is a HUGE, HUGE Red Wing fan. She absolutely adores her beloved Red Wings. She could talk your ear off for hours about her Red Wings. Erinne has a zest for living and is happy just to wake up in the morning. How many of us can say we are happy we woke up this morning?
In March 2006 Erinne and I flew to Georgia to pick up her first service dog, Latte, from Canine Assistants -- an awesome organization -- check them out at their website (www.canineassistants.com). You can read about their adventures on the page marked In "Our Journal" and view the pictures from training and their new life together on the "Photos" button, pages 7 - 9.
2006, Erinne had her
first growth rod scoliosis
surgery to correct her 100
degree (sitting) curve in her spine by
the very gifted Dr. Michael Mendelow. We
were worried that she would experience the post-op respiratory compli
In November 2008, Erinne began using a ventilator to help
her breathe, as the bi-pap could no longer give her the
respiratory support she needed. But, due to the wisdom of 2 very
smart doctors - one in
In September 2009, Erinne began some very extensive orthodontic
work. Her upper jaw is markedly smaller than her lower jaw and
she has a severe underbite. Eventually, reconstructive surgery
may be required. This will be a very challenging undertaking for
both the doctors and Erinne. Please keep Erinne in your prayers.
In January 2013, the disease, which had plateaued for so long, has started progressing. Erinne has trouble breathing and is losing the strength to sit in her wheelchair all day. She is plagued by migraines. It is hard some days for her to put a smile on her face. I made the hard, hard decision to trach her (put a hole in her neck so she could breathe) and place her on a ventilator. The extensive orthodontic work continues. In June 2013, she will have major reconstructive surgery to move the center of her face, her upper jaw forward and her lower jaw back -- the result of all the years she has used bi-pap to help her breathe at night. She is scared. Please keep Erinne in your prayers.
September 2014 -- This horrible disease continues to progress. Erinne's condition has rapidly deteriorated. She has been hospitalized 6 times in the last 8 months. She has a reoccurring MRSA (staph) infection that the doctors cannot get rid of. It is located or colonized in her trachea. The infection has taken a toll on her body, causing her to lose a great deal of strength and stamina. She is exhausted all the time and, on many days, would sleep for 22 hours a day. She relies on her ventilator to breathe for her more and more. She has developed auditory and visual hallucinations, possibly from the medications, and has had thoughts of suicide. She experiences significant levels of anxiety. She had to cut back on school hours. She was afraid to fall asleep at night. It took every ounce of energy she had just to go to a doctor appointment. She has had a grand mal seizure.
Erinne has spent her summer either in the hospital or home recuperating. She was not able to join her travel hockey team this summer to play in St. Paul, Minn. She starts school tomorrow -- her senior year. I am praying she will be able to finish it and graduate. I am hoping and praying she will have the strength and stamina to play hockey this year. Right now, her neck control is not good.
I hate this disease. SMA is horrible.
Please read Erinne's Journal page for the rest of the updates.
Thank you for taking the time to get to know my daughter.
We will be launching several fundraisers to create a fund to
cover Erinne's uninsured medical expenses. We hope you will
support us in this new endeavor. The easiest was to help is to
go to any Chase Bank and make a deposit to her trust account,
Erinne Rose Williams, #2747823769. There are currently no active
fundraising drives going on. If you have a suggestion or would
like to do one, we would be most grateful!!
As always, your prayers are very much appreciated and always welcomed. If you have a moment, please leave Erinne a note.