Our Story

A Smile
By Nicole Read Grubbs

I used to judge whether a mother did a good job based on how often her baby smiled. I thought smiles and laughter were the only ways to gauge a child’s happiness.

I used to unfairly criticize mothers who seemed to have chronically “unhappy” children, never taking colic into consideration and assuming the mother just wasn’t trying hard enough. I believed this well into adulthood. Then, I had a child who couldn’t smile.

Of all the things young girls aspire to be, I was always sure I would be a great mother. After all, babysitting was my favorite Friday and Saturday night activity and I was every neighborhood kid’s favorite sitter.

My friends told me lots of things I didn’t believe. They said that I was pretty, smart and cool. But, I believed them when they told me I was going to make a good mom. Many adults, including several of my teachers, complimented me on my nurturing abilities. I loved children so much, I even hoped to have eleven of my own.

Amazingly, I got through those teen years, went to college and fell in love. When the time came for me to marry, I did everything right. I married Michael Christopher Grubbs on July 29, 2000. He was the perfect man. He had been my best friend for four years. He was loving, intelligent -- he eventually became an attorney – and, of course, he would make a great father.

We waited the right amount of time to think about having a baby. We had been married more than four years, I had earned a bachelor’s degree and worked as a reporter at a large newspaper. I knew I wanted to stay at home with our child and didn’t want to have any regrets about receiving a degree I never used. I also wanted to have something to fall back on, in case my life didn’t turn out to be a fairy tale. I took perfect care of myself, watching what I ate, never consuming alcohol, drugs or cigarettes, exercising and taking prenatal vitamins and folic acid for two years prior to conception.

At 20 weeks along, the day of our 5th year anniversary, we found out we were having a boy. My husband was overjoyed and I was also very excited. Everything on the ultrasound looked “normal” and we were relieved.

My pregnancy went well, with only minor problems, including a lot of heartburn and Braxton Hicks contractions that occurred throughout half of my pregnancy.
The momentous day came. I knew it was going to be perfect. I hadn’t even gone as far as to consider what I should do if I had to have a C-section. I just didn’t expect any problems. After all, I was made to be a mother.

I was due on the 20th of December, 2005, but that day came and went, as did the next and the next and the next. On December 24, my husband and I went to the hospital, so they could perform a biophysical exam to monitor the baby’s respiration, heart rate and measure the amniotic fluid surrounding him. I was admitted right away when it was discovered there was only a little over 2 cm of fluid. A woman is automatically induced when there is 5 cm or less. Low levels of amniotic fluid increase a woman’s risk for complicated labor, but more importantly, the baby’s movements and labor contractions can cause compression of the umbilical cord.

The nurse started my labor by using a gel to soften the cervix, and later I was given pitocin through an IV. I really wanted to have the baby without pain medication, but after six hours of intense contractions that were 1 ½ minutes apart, I had only gone from 2 to 4 cm. I relented and accepted the epidural.

I slept a little bit, and was able to relax, which allowed my body to do everything it needed to get ready for the birthing process. I was ready to have my baby. My water broke at 4 a.m. on Christmas morning, but I wasn’t sure it had because no fluid came out. My nurse said because there was little to no fluid left, I would only feel a popping sensation, not a gush, like some women do. I was ready to push at 5:30 a.m., but the doctor on call wanted me to wait until my doctor, who would be on call at 6 a.m., came to the hospital. When my doctor finally arrived at about 7 am, he told me to start pushing and at 8:08 a.m., 26 hours after being admitted to the hospital, I received my Christmas gift: little Aulton Joseph[HAC1] .

First, I spotted all of his dark hair. Then, I saw the look of shock on my doctor’s face as he clamped the umbilical cord with his hand. Aulton was probably lying on the cord during part of the labor and as I pushed him out, the cord ripped. I kept waiting to hear his cry. It never came.

I delivered a purple, lifeless baby who was rushed to the neonatal intensive care unit after a single glance from me. My husband accompanied our son to the NICU, while I lay in the hospital bed crying. My mother, the perfect model of motherhood, lay down beside me. She didn’t say anything, she didn’t have to. We both knew that my baby, her first grandchild, was very sick. She just held me while I wondered what I had done wrong

I had to wait for the epidural to wear off a little bit before I could see my son. After about an hour, my husband lifted me out of bed and put me in a wheelchair. I was astounded at the number of wires and cords that were attached to my 6 pound, 14 ounce baby. He had oxygen tubes in his nose, an IV in his forehead and a little band on his foot to measure his pulse. Later that day, a nurse inserted a feeding tube into his nose and down his throat. That was the most difficult thing for me to watch. It was the first time I had ever heard him cry.

When I first saw him, he looked so fragile. I really wanted to hold him, so the nurses helped us move all the equipment so that I would be able to. As I gazed at this precious child, I didn’t know how it was possible to feel so much awe and fright at the same time.

The day of the birth, a geneticist came to see us and asked if we had any birth defects in our family, which we might have passed on to Aulton. There were none that we knew of. Aulton’s pediatrician, Dr. James Leiferman, also came to see us and explained that they didn’t know what was wrong. They had a few theories and would test them out. The doctors tested his chromosomes, gave him an MRI, did an ultrasound of his brain, performed an EKG and ECG, and tested every other possible thing to find out why our baby was so sick. Each day we were updated on the tests he was going to have and each day we received the same news- the results were normal. We were both relieved and confused. Most of the NICU nurses said he probably was recovering from the traumatic birth.

It seemed they were right, each day he improved, some wires were detached, and he was eventually moved to a less sterile “crib.” He started breathing on his own and had his feeding tube removed.

Aulton, like most children, was resilient and fought hard. I also fought hard to get him to come home, which he did New Year’s Day. Initially, we had a lot of doctors to see because he had been in the NICU. They monitored his weight, his hearing and his bones – some of which looked like they might have been fractured during delivery. The doctors noticed that he was not hitting important milestones like he should. He started receiving physical and occupational therapy when he was just four months old. I thought if I could just make it through these appointments, soon I would be able to be a normal mother. I would wake up in the morning and not worry what appointment we had that day, but would actually be able to play with my baby. My husband and I felt sure Aulton’s delays were a result of his traumatic birth.

As 2006 progressed, the appointments did wane. We continued to see therapists and a few doctors, including his pediatrician, who remained determined to find out what was wrong with Aulton.

In mid-October, when Aulton was only 10 months old, his neurologist guessed that he had a disease called myotonic dystrophy. That was the first time I had heard those words. It’s funny, now. I look back on that day and remember thinking that I would surely forget what it was called. I had to write it down in my planner.

It would take seven weeks for us to get the results back confirming that Aulton did, in fact, have this rare form of muscular dystrophy. In the meantime, I researched the disease on the Internet, which was probably a bad idea. I did a lot of crying.

Myotonic dystrophy is a complex disease that has varied outcomes depending on the patient and the severity. I, for instance, have it, but didn’t even know until Aulton was diagnosed and I was tested. Now, I can see how some of the symptoms are present in my body. I had always attributed them to other causes. In adults, the disease causes muscles to cramp. Sometimes, I have a hard time releasing a really tight grip on things. I thought it was a joint problem. My leg muscles always tighten and ache when I’m sick. I never knew anyone who had the same problem. I have always had really bad premenstrual cramps, likely a result of the disease.

The disease is different for babies, causing severe hypotonia, or lack of muscle tone. It not only affects muscles in the extremities, but also the organs. In fact, it can affect cognition. Sixty percent of babies born with the disease also have some form of mental disability. Many babies also have difficulty breathing, sucking and swallowing. Miraculously, those problems only lasted a few days for Aulton. However, he continues to suffer from low muscle tone throughout his body, especially in his legs, shoulders and face. He can use these muscles, but has to be very motivated.

The prognosis for children with congenital myotonic dystrophy is varied. Some children progress into adulthood well with the assistance of physical therapy. Others, may progress well until early childhood then their muscles start to deteriorate. No one can really determine how the disease will present itself in the future for each patient.

I couldn’t seem to get past the feeling that all of the work I was doing with Aulton might be in vain if his muscles just decided to stop working one day.

My husband has had an optimistic attitude and has worked hard at trying to pass this onto me. I marveled that he could be so happy with just having a son and not dwell on the fact that Aulton would probably have physical therapy his entire life, might need a wheelchair by his teens or consider the endless doctor’s appointments we had to look forward to.

After the diagnosis, our pediatrician gave us referrals to some specialists. He had to add more lines to the form he used because of the number of doctors he wanted Aulton to see. By the first of June 2007, Aulton had been to more than 80 appointments with doctors and therapists since the beginning of the year. That’s an average of about three each week.

After one particularly grueling week, when Aulton had five appointments, some of them painful for him, I complained to my father. I told him I just couldn’t understand why my life was so full of challenges. My father trains employees on proper work behaviors and conduct. He gave me some advice that he has given co-workers. He told me that sometimes our trials aren’t trials at all, they are just our lives. I hated that advice and didn’t even consider it, because I didn’t want this to be my life.

Everyone pretended they understood. My sister-in-law once told me about a story she read in a religious magazine about a woman who had a special needs child. The author was expecting at the same time as many of her friends, so they were all experiencing similar things, until they had their babies. I don’t know what particular challenge this woman and her child faced, however, my sister-in-law said the author compared the pregnancy and birth to a journey. She had been prepared to go to “France” with everyone else, but ended up in “Spain” instead. I know my sister-in-law meant well, but the story didn’t resonate with me. I didn’t know why until I was discussing with my friend how worried she was that her child was going to choke on peas if she didn’t shell them. I thought about my biggest worry at the time, my son’s upcoming surgery. I was worried about how the doctors were going to have to take extra precautions because of the risk anesthesia poses on a patient with myotonic dystrophy. Then I understood why the story hadn’t affected me. Instead of landing in “Spain,” I felt like I had landed on “Mars.” A place so completely foreign to everyone, myself included.

As soon as I realized that my life of endless phone calls and doctor’s visits was not going to change, I had to decide whether I wanted to stay alone on Mars, or go to Spain. I knew it would be hard because my friends would stay in France, but at least I could go and visit. I looked around me and realized that I was bathing in self-loathing and bitterness. There are mothers who deal with children that have challenges far more difficult than the ones Aulton and I faced. I knew that these women must have felt the same things I was feeling. It was time for me to deal with this disease.

I realized that the people around me – my mother with her unconditional love, my husband with his optimism and my father with his wisdom and advice – were giving me the tools I needed to raise my son. And, I have the support of my friends, other family members and his therapists who love him and do everything they can to help him progress.

I started to look at Aulton in a different way, cherishing each accomplishment, no matter how small. I appreciated his differences, instead of resenting them. I tried to stay positive, even when it seems he is regressing or when I hear bad news. I read that children, even tiny babies, can sense frustration and stress and I don’t want my attitude to negatively affect him. And, I accepted my life as it is. I am blessed to have a child and I am blessed to have support. This doesn’t mean, by any stretch, that some days aren’t still extremely difficult, but I am doing my best.

Even though Aulton has started to smile more, he often shows his happiness in other ways. He was blessed with an amazing, loving personality and demonstrates his joy through the sweet noises he makes, clapping, cuddling, “dancing,” exhibiting his content nature and hundreds of other ways that only his parents know.

I used to have this crazy notion that a mother was doing a good job based on how much her baby smiled. My child has very little muscle tone in his face and a smile is a rare and important occurrence. Fortunately, I now know that there is more than one way to tell if a child is happy and that a mother’s worthiness is not based on how much her child smiles, but by how hard she tries.















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