Ventilator-Assisted Living©

Winter 2002, Vol. 16, No. 4

ISSN 1066-534X

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Read selected articles from this issue ...

Breathing and Pregnancy
Amy Marquez

Managing Noninvasive Ventilation: A Portuguese Experience
João Carlos Winck, MD, and Miquel Gonçalves, PhT, Portugal

My Experience with Noninvasive Ventilation
Steve White

Body Brace
Julie Levine, California

Interfaces, Ventilators, and Supplies

Noninvasive Ventilation Alternatives in Neuromuscular Disease Conference
San Diego, California, November 2002

Breathing and Pregnancy

Amy Marquez

Photo of Amy and Steve with baby Danielle.When I was in my early 20s (I am now 31), I asked my doctor, a general practitioner, about the possibility of having a child because of my diagnosis of SMA (spinal muscular atrophy) Type I, later questionable as to which type. Though surprised by the question, he responded very optimistically, reasoning that my scoliosis was not severe enough and there was enough abdominal space in which a fetus could grow. He recommended consulting a genetic counselor because SMA is a genetic condition. There was no mention of breathing difficulties that might arise.

Several years later I began to meet people outside of my social circle through the Internet, a tool that allowed people to get to know who I was as a person before they ever learned that I was disabled and used a wheelchair.

Early in 1997, I was introduced to Steve, a friend of a friend who was from Chicago, my home, but living in Maine. We began to get to know one another over the Internet, sometimes “talking” for six hours straight. In October, Steve moved back to Chicago, and soon we moved into our own apartment together. A year later, on October 17, 1998, Steve and I married and began discussing having a family.

In December 1998, we received the news that I was pregnant and would be expecting our first baby towards the end of August 1999. Although the pregnancy was unplanned, we were elated, but, due to my physical condition, concerned about what carrying a baby to term meant for both our unborn child and me. Our family doctor recommended that we seek prenatal care from a high-risk pregnancy team at a Chicago hospital.

In the days before our initial appointment, my husband and I had several in-depth and intense conversations covering all possible scenarios. We both wanted a family, we both wanted this baby, and we were both willing to make extreme “sacrifices” to ensure our baby was born healthy. We agreed abortion would only be an option if the doctors were 110% certain both the baby and I would not survive the pregnancy. If there was even the slightest chance our baby would make it, we were willing to try and needed the same commitment from the doctors involved.

The high-risk team did not offer us much encouragement. We were immediately advised to abort the fetus since there was no known record of a woman diagnosed with SMA Type I carrying a child to term. They predicted that the fetus would be born either stillborn or too premature to breathe independently and, therefore, survive. I was also warned that I would suffer severe strain and trauma to my lungs and would need to use a ventilator well before the second trimester. Furthermore, the odds of my ever being weaned from the ventilator were nearly impossible.

We decided to seek a second opinion and to find a doctor with experience in delivering babies of women with disabilities and willing to work with us. We found Eileen Murphy, MD, obstetrician/gynecologist at Northwestern Memorial Hospital and the Rehabilitation Institute of Chicago (RIC).

Although our initial conversation with Dr. Murphy was at times painfully honest in discussing compromised breathing, bed restriction, and the possibility of death for our baby and me, it was a hopeful one. We were convinced that having a successful pregnancy was a definite, but not easy, possibility. Dr. Murphy recommended that I undergo an ultrasound, and we saw our baby girl for the first time.

In early January 1999, physiatrist Kristy Kirschner, MD, pulmonologists Chris Winslow, MD, and John Parsons, MD, completed the team of RIC doctors to assist throughout the pregnancy. Every member of the RIC team was willing to listen, to work with us, and to take whatever risks were necessary to help bring our baby into this world alive and healthy.

During the first trimester, I endured serious morning sickness, but when the second trimester arrived, it was as if a switch had been flipped, and the nausea and vomiting abruptly stopped. I soon felt healthy and strong.

The team decided to take advantage of my health to prepare for occasions during the remainder of the pregnancy when I might not feel so strong.

I had undergone a tracheotomy for secretion removal when I was 5 years old due to my limited ability to cough and numerous bouts of pneumonia. My neck size did not grow “normally” as a result of SMA, and my tracheostomy tube size and style remained the same since age 5.

I never required the assistance of a ventilator prior to being pregnant, but on the assumption that I would need ventilatory assistance in the later stages, I worked with a variety of different tracheostomy tubes and ventilators to get used to having a machine breathe for me, and finally became comfortable using a BiPAP unit whenever necessary.

During the second trimester, I experienced difficulty breathing only after I consumed a full meal. Dr. Winslow monitored this and found that my oxygen saturation levels dropped significantly, so a BiPAP unit was delivered to our home, and I used it whenever I experienced any difficulty breathing. It was recommended that I eat continually throughout the day, rather than two or three full meals, thus avoiding overcrowding. Such simple advice proved to be vital in easing my breathing difficulties.

Somewhere beyond the fifth month, I began to experience physical discomfort and pain, initially mild and alleviated by a small pillow, stretching, and/or the use of a hot/cold pack. Nevertheless, the pain grew more severe, and I went into labor over the Independence Day holiday. I went into the hospital, was monitored over the span of seven hours, did not dilate at all, and was released. I later developed body shakes and an overall restlessness, regardless of my surroundings.

On July 6, 1999, Steve and I met with Drs. Kirschner and Murphy and together determined that the best solution would be to admit me to Northwestern Memorial Hospital so that the team could assist with my pain management up until the birth. As anticipated, my pain and discomfort intensified as our baby increased in size, taking up more and more of my internal space. By mid-July, the restlessness and pain became unbearable. No amount of therapy, repositioning, or pain medication would help. Finally, Dr. Murphy agreed to deliver our baby on July 19, 1999.

I hoped my body would accept an epidural and that I could be awake to welcome our child into the world. However, the epidural would not take due to my scoliosis, and all involved agreed to move ahead with complete anesthesia. I was connected to a hospital ventilator and awakened hours later by Dr. Murphy and a beautiful bundle sleeping on my chest.

Steve and I were able to spend a few moments with our new daughter, Danielle Coral (born at 34½ weeks gestation, 17 inches long, weighing 4 pounds, 10 ounces) before I was taken to the surgical ICU, where I remained for overnight monitoring. I continued to use the ventilator for several hours after surgery, and finally had it disconnected on the condition I would use it should I experience any trouble breathing. I never did.

Steve and I look at Danielle, today a happy 3½-year-old, and are thankful that we sought a second opinion. We could not have managed without the constant professionalism, compassion, and support of the RIC team. We are seriously considering another child, but only if we can have the same team of doctors by our side.

Christopher Winslow, MD, Amy’s pulmonologist states, “When Amy presented to me 10 weeks pregnant, her lung function was 80% lower than the previously reported lowest value in the medical literature for a pregnant woman with SMA. After our initial meeting, I discussed her case with a dozen pulmonologists in my group, and all (including me) predicted that she would require full-time use of a ventilator by her 20th week. The fact that her body was able to carry this pregnancy with minimal support from assisted ventilation, i.e., BiPAP®, is truly remarkable. It should prompt physicians to rethink conventional wisdom that is based on nondisabled women during pregnancy. The body seems to have a capacity to adapt that is under-appreciated. The case study of Amy’s pregnancy will be published in early 2003 in the American Journal of Physical Medicine and Rehabilitation. Women with SMA who are considering pregnancy may wish to discuss this article and others with their physicians.”

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