My history:
I was born in 1970, and, when I had not begun walking by the age of 2, I went through extensive testing. Specialists diagnosed me as having Werdnig-Hoffman's Disease, also known as Spinal Muscular Atrophy Type 1. This is the most severe form of the disease, and the doctors told my parents I would not live past my second birthday. In my younger years I often caught the flu and was hospitalized nearly a dozen times because of severe dehydration. But I avoided the respiratory infections that seem common to children with SMA I. At the age of 16 I had metal rods put in my back to partially correct my severe scoliosis. I was told later by my surgeon that if I had not had the surgery I would have likely died within the next six months. My recovery went fairly well, although one of the lobes of my lungs had been collapsed due to my curve, and when it was opened up again, I developed my first case of pneumonia. Eventually I graduated from high school with honors and went away to college. When my first daughter was two, I was rediagnosed as having SMA II, a slightly less severe type.
Society's reactions to my sexuality:
As a teenager, boys were attracted to me, but most felt they could not handle the additional responsibilities of dating a person with a disability. Many girls my age were downright cruel. When one classmates found out I was going to my junior prom, she wailed, "Even she is going to the prom!" The college I attended was well known for its accessibility to the handicapped, so I was not as much as an "oddity". I dated several men, and eventually met my husband Lee, during my junior year. A few people, especially many of Lee's ex-girlfriends, expressed displeasure with Lee's choice of a spouse, but for the most part, peers were very accepting and supportive. Lee's family seemed happy to include me into their circle. My parents were more dubious about our relationship.
Working out the "mechanics":
It took us awhile to figure out different positions and such, but once we got the basic "mechanics" of intercourse down, I became pregnant with our first child very quickly. I know that many people with SMA have trouble with contractures in their hips and knees that make lovemaking more of a challenge, but I did not experience these problems to a large degree. My biggest hindrance was that I was very sexually naive prior to marriage because nobody felt it necessary to enlighten me about the "nuts and bolts" of sex, figuring, I guess, that I would never have to use this information.
My first "symptoms" of pregnancy:
I think I started feeling run down literally the day after conceiving my daughter. I have never been very strong, and have always relied on others for my daily care. I could pick up objects up to a pound or so with effort. But after I became pregnant, everything seemed to weigh five times as much. A computer disk then felt like it weighed a pound instead of a couple ounces. I was also constantly exhausted. During the first trimester, I woke up around 8 o'clock, went to work for 4 hours, slept until dinner, ate, and then went to bed for the night! I liken it to a battery being over-extended or an over-used circuit. If you plug a lamp into a circuit that is being over-taxed, the light it produces is dimmer and erratic. My internal energy was being drained not only by my daily activities now, but also by a growing baby. If I had been able to dress myself and transfer prior to this, I think I would've needed help with those things during my pregnancies, especially as my tummy got bigger. Weirdly, though, my sapped energy and strength seemed to return to normal almost immediately after giving birth, especially with my second child, with whom I avoided major medical complications. In some ways I even felt stronger post-pregnancy.
The medical establishment's reactions to my pregnancy:
It took the intake people at the clinic, I went to while pregnant with my first daughter, several months to recognize that I was there because I was pregnant, and not because I needed birth control. The doctors there had no idea what to make of me. I was told correctly that my pelvis was too small to deliver vaginally, so they began mentally preparing me for a c-section. I always thought this would probably be the case because, although childbirth and contractions have a lot to do with involuntary muscles that are not affected or less affected by SMA, I was pretty sure I'd need stronger voluntary muscles to push the baby out.
My worst experience centered around my first ultrasound. The technician was very nice and excited for us, showing us the baby's fingers and such on the screen. But when the doctor came in, he took one look at my wheelchair and my chart, and stormed out, refusing to further examine the ultrasound results. He was irate that I had an ultrasound prior to receiving genetic counseling, where he presumably thought I'd be advised to terminate the pregnancy.
When I did go to the genetic counselor, I explained the recessive nature of SMA genes, and explained to her why it was statistically unlikely that my offspring would be infected with SMA because there were no known occurrences of it in Lee's family. I also explained that I realized my children would be carriers and planned to advise them accordingly, as they grew older. The geneticist said that I knew more information than most of her patients and even some genetic counselors, and gave me her professional "blessing".
I've often fantasized about tracking down that ultrasound doctor and mailing him a picture of my daughter with the note, "Here is the little girl you wanted me to abort."
Breathing:
Shortly after this experience, I was sent to get testing done on my lung capacity. I was hooked up to a machine with a digital read-out of my breathing volume. It looked like they were running a test on a gopher instead of me. The technician looked at me like he was amazed I was alive. Despite my wimpy gopher lungs, my breathing remained fine through both of my pregnancies. I never needed bi-pap before, during or after. Doctors were concerned that as the babies grew in my womb, cramped by my residual scoliosis, they would push up into my chest and decrease my lung volume further. This never happened. Both my children seemed to grow way out instead of up. My second daughter in particular seemed to sprout out of my left side. They both found room when they needed it.
Nutrition:
I had almost constant morning sickness with my first daughter, but I never really threw up. I dry heaved a lot and most food was completely unappealing. I tried prenatal vitamins for a bit, but they were hard to swallow and caused really bad constipation. Instead I just supplemented my diet with Vitamin B Complex, which seemed to increase my energy levels. I still take Vitamin B to help with energy.
Weight gain:
With both of my children I do not believe I gained as much weight during pregnancy as many women. In fact, the clinic workers would always nag me about eating more during my first pregnancy. They would weigh me in my electric wheelchair down in the hospital basement on the big roll-on laundry scale, and subtract out the 200 pounds added by my chair. During the third trimesters of my pregnancies, it was definitely harder for my husband to pick me up, but not impossible. The hardest part was that when he'd pick me up, in the usual "cradle carry" position, sometimes my body would "fold up" too much and press uncomfortably on my tummy.
Because of my lack of muscle tone and mobility, it was extremely difficult to get my stomach back into its pre-pregnancy form. Over the years, I've managed to lose some of the weight but I fear my tummy is irreparably stretched out of shape.
Positioning changes:
As my stomach grew, I had a harder time getting comfortable in my bed and wheelchair. Typically I sleep on my stomach most of the night, with a pillow length-wise under my torso. This has always seemed to help drain my lungs and keep my airway clear when I'm sleeping. As my tummy grew, I had to reposition pillows cross-wise under my chest and my hips, with my stomach acting as a sort of middle "pillow". My children apparently suffered no adverse effects from their nightly "squish".
In my wheelchair, I gradually had to sit forward further and further in my seat so I could lean further and further back to accommodate my expanding waistline. This completely threw off my balance, and my already floppy head became increasingly floppier. It took some time post-pregnancy to rediscover my optimum point of balance.
Anesthetics:
I was told that I could not have a c-section with an epidural because of my previous spinal fusion. My epidural spaces in my spine were fused together. It would have been very difficult if not impossible to inject the anesthetic into my spinal column. I had to have general anesthesia.
On the day of my first scheduled c-section, the anesthesiologist freaked out when he discovered I could only open my mouth a very limited amount due to contractures in my jaw. He decided I needed to be intubated while I was still awake, but that he would inject a local anesthetic into my throat to numb it and make the procedure more "humane". Nobody knows for sure what went wrong. The doctors tried really hard to blame my problems on my wimpy lungs. It is now speculated that the doctors took my weight from my chart, which included my 200-pound wheelchair, and overmedicated me with enough local anesthetic for a 300-pound person! Whatever the cause, overmedication or allergy, my throat instantly swelled shut. The doctors had to ram the respirator tube down through my nose, causing severe damage to my throat and vocal cords. It took me six weeks in the hospital and a tracheotomy before I recovered enough to leave the hospital. The trach was eventually removed, but to this day I still have additional problems swallowing and am much more prone to respiratory infections.
My experience with my second daughter was completely different. I worked with my anesthesiologist, Dr. Scott Hadaway, throughout my pregnancy. He used the topical anesthetic Dyclonine 1 % (Dyclone), manufactured by Astra USA, instead of the Xylocaine, Lidocaine, Novocaine family because a possible allergy, which resulted in the anaphylactic reaction in the past. He also nasally intubated me because my jaw contractures. He used an unique procedure to intubate me for surgery. First, he used a spray in my nose to dry up secretions, and turned my head to look towards the extreme right, as I have a better airway in this position. Then he applied Dyclonine to my nostril to numb it. After lubricating the nasal trumpets he used, with Dyclonine and a lubricating mixture, he inserted progressively larger trumpets to stretch out my nostril. He left the largest trumpet in place and took me to surgery. They put me to sleep with mild general anesthetic and intubated me through the trumpet, using a fiberscope to locate my vocal cords. Then they administered more general anesthetic. I believe they used less muscle relaxants than usual, so my breathing would not be as affected when I woke up. After surgery, they extubated me while I was still groggy, and administered steroids to prevent swelling of my throat. An Ear, Nose, and Throat doctor was available during the surgery in case I required a tracheotomy. I returned home with no ill effects in four days.
Society's reaction to my motherhood:
To this day, when I casually mention my husband or biological children to strangers, many look at me like I am severely deluded. Some think it is a horrible travesty that I would subject my offspring to the "horrible fate" of having a disabled mom. Others treat me like a saint who immaculately conceived, not wanting to acknowledge my sexuality. Children seem to be much more accepting. To many of them I'm just "Emili and Ravyn's mom." Hopefully the tide is starting to change and no one will have to deal with "professionals" like the misguided ultrasound doctor.
No more kids for me:
I was told by the obstetrician from my second pregnancy that I should avoid becoming pregnant again. My uterus is very thin, possibly due to the SMA, and there is a good chance it would rupture long before I reached term. I trust this doctor's advice, as she was very positive while working with me throughout my second pregnancy.
Last updated February 24, 2004.