ETHAN'S STORY by Jennifer

Rough Start

When the nurse practitioner announced that we had a very sick little boy, I silently thought that was a major understatement since our son had been born a little over three months prematurely. Looking at the jumble of tubes and wires monitoring our son and keeping him alive, I could not get over how beautiful and perfect he seemed. I stumbled back to my room tired of trying not to cry in front of the constant audience surrounding me at my son’s bedside. Though I understood the implications of everything occurring, I still couldn’t understand how or why it all happened.

During my pregnancy while teaching middle school classes, I dreamed about what our delivery would be like. I voraciously read through a mountain of books expounding the wonders of gentle exercise, healthy food, and correct sleeping positions. All the books about childbirth and delivery cannot prepare you for the shock of having a premature infant, especially one so extremely early. When the doctor announced that the back pains prompting a visit to his office were active labor, the idealism of my easy pregnancy quickly fell apart.

I never suspected as I taught remedial study skills the morning of October 29, 2001, that I would so quickly become a struggling novice myself. I was bombarded with medical jargon of increasing complexity as our son’s diagnoses became increasingly worse. Because he was diagnosed with septo-optic dysplasia, a malformation of the brain that affected his adrenal and pituitary functions as well as his sight, we had consultations with endocrinologists, geneticists, neurologist, respiratory therapists, and last but not least the team of neonatalogists. A week later, the doctors also diagnosed lissencephaly, which means smooth-brained, limiting his chance of survival to a year and his intellectual development to that of a six-month old.

Despite all this, every time I looked at my son, I was flooded with love and touched with awe at his beauty. My faith was unwavering as I waited to see just what God was doing though my hopes felt as if they were uncertain where to tread. Within hours of the birth, my husband and I sent out the news that we needed prayer in abundance. We set up a website with pictures and daily updates on Ethan’s progress where hundreds of people signed on to say they were praying. We received visits from clergy and friends wanting to pray over his isolette. Friends from the choir arranged to bring us food so that we could focus on staying with our son. I was touched by their generosity and thoughtfulness in accommodating our preferences. So many stories of people’s private struggles with their own premies came filtering in to us. Even in the midst of this intense and emotional ordeal, God was proving his constant Presence and allowing us to see hints of the possibilities of good that come out of a difficult situation. Ethan’s fragility became a foil for the strength of everyone’s faith.

Watching our son struggle through seizures and hearing reports of his fluctuating cortisol levels, we tried to comfort this child we could not touch or hold too often since it desaturated his blood oxygen levels. Over the course of five weeks, Ethan received five transfusions. When we received news from a consultation with Boston Children’s Hospital that Ethan did not have lissencephaly or any further evidence of septo-optic dysplasia, we were humbled and elated. We felt a kind of miracle occurred, though there was still evidence of basal-ganglion damage and continued seizure activity.

Though we eventually transferred from EMMC and our hometown, Bangor, to Portland’s MMC for two-and-a-half months because of the need for a surgical ligation of Ethan’s heart, the grace of God continued to surface in the generosity of the volunteers at the Ronald McDonald House, in the numerous gifts and prayers from friends, relatives, and even strangers, and in the kindness of the medical staff responsible for his care. Ethan’s seizures stopped the day of his transfer; the forced surgical closing of his heart’s valve cleared up the fluctuating blood pressure and largely stopped his respiratory problems.

When Ethan had gone for over a week without apnea or bradycardia (slowed heartbeat) and demonstrated that he could take nourishment without tubal feedings (100 days later), we joyfully and rapidly collected him to go home. Holding him in my arms as Ethan attended church for the first time, I cried feeling blessed with an outpouring of love and support from our church family.

Coming Home

Coming home was a strange dream. We got a pediatrician who had a background in pulmonary medicine since we thought with his bronchial pulmonary dysplasia that Ethan may have breathing difficulties or asthma from the prolonged use of a respirator. The first year he was hardly sick. We stayed inside except for church and grocery store visits, a sort of self-induced quarantine or recovery period.

About a year after Ethan was born, he stopped eating/breastfeeding. This was not just a case of fussy eating or diminished appetite but a complete fast. After ten days of no food and water and patronizing responses from the medical community, we admitted him to the hospital for immediate help. He was given a naso-gastrostic tube. After eleven days of testing for blockages, evaluating barium swallow studies, performing an endoscopy, and enduring an emptying study, the feeding team had nothing further to offer us except therapeutic suggestions (none of which worked). So, we were released from the Barbara Bush Children’s Hospital at Maine Medical Center in Portland.

We waited six months hoping his appetite would return fighting with him to keep the tube in his nose which we taped to his cute cheeks in an attempt to deter his removal. Finally, we had a g-tube surgically placed. What we thought would be a simple procedure became complicated when Ethan screamed for three days straight every waking minute despite an abundance of pain medications. He would not even roll over. Then the vomiting began, and for the first of many times I wondered if we had made a bad decision not to do a fundoplication (wrapping the esophagus closed so he could not vomit).

After eleven days we were released though the emesis continued in profuse amounts multiple times a day for the next year. Keeping weight on was a constant struggle. We kept hoping with developmental advances, he would eventually outgrow his need for a g-tube with adequate interventions. This happens with many premies who seem to work through this issue within a few years.

A Long Road

Because of Ethan’s severe prematurity, he had numerous delays. Our life slowly started to revolve around therapy. We had started Physical Therapy at six months of age which soon prompted into Occupational Therapy which soon led to Speech Therapy and then Developmental Therapy. All progress made was slow and not always sure. I remember wondering as we approached his second birthday if he would ever walk.

After going through two winters getting RSV shots every month, we thought we were in the clear. So in the third winter, he got a bad cold and we were told it was RSV. Ethan was hospitalized for four nights with a CPAP to help keep his oxygen levels up. This hospital visit felt kind of like a non-event since we were so close to home and not much exciting happened. I just ordered pizza and waited until he got better while reading magazines and watching TV in an isolation room.

Consultations Continue

Ethan has seen numerous specialists for evaluations. We consulted with an endochrinologist to see whether his adrenal and pituitary function had been affected; they were all within normal ranges. After a few feeding evaluations conducted in Maine which gave little help or insight into Ethan’s strange feeding behavior (or lack thereof), we consulted with a feeding specialist at Children’s Hospital in Boston who told us about sensory-integration disorder and felt a sensory component and control issues were evident in his feeding aversion. We went to Portland several times for long interviews with a developmental pediatrician when Ethan was 18 months who did not think Ethan necessarily had autism but rather sensory-defensiveness and developmental delays due to prematurity.

THE Diagnosis

Ethan was finally diagnosed with autism by a psychologist when he was almost three years old. We later consulted with a developmentalist in Waterville who told us Ethan may benefit from some medications. A behavioral psychologist met with us a few times to address some of the more difficult behaviors with a clear plan that was to be implemented in-home by a team of behavioral therapists that never did quite actualize despite our efforts. The most progress is being made at his special-purpose school that implements applied-behavior analysis techniques. With much of the reading we have done, it is clear that a nutritional/dietary component is present. We have begun some rather intensive and often expensive supplementation directed by a doctor who specializes in chelation and alternative therapies in Portland.

Another Surprise

During one of the periodic visits with an orbital surgeon (a specialized opthamologist of sorts) in Portland referred to us after seeing a pediatric opthamologist concerned about the proctosis (bulginess) of Ethan’s right eye after a routine check of his vision, we discussed one of the MRI’s of Ethan’s head. The doctor had ordered the films to determine whether surgery was necessary to remove a hemangioma causing pressure on the optic nerve. Though he felt we had about a millimeter of bargaining room, the larger concern was the mass in the back of his head that indicated either massive bleeding or a tumor.

We immediately went to Boston’s Children’s Hospital to have an angiogram during which an arterial venous fistula was embollized by the chief of neurosurgery. The pediatric radiologist asked us if we had had any genetic testing done since she felt Ethan had some markers indicating a rare disorder called Bannayan-Riley-Ruvalcaba Syndrome. An evaluation with a geneticist was inconclusive. The following spring we went back to check out the plumbing and we were told that he looked good despite having no jugular vein on the right side of his neck.

Dental Travel

Despite being five minutes from a Level-3 NICU, we have had to travel some distance in the state to get to all the specialists Ethan requires. We are used to the 1-hour, 2-hour, 5-hour drives. We never thought finding a dentist would prove difficult. After an attempted visit to my dentist who tried nitrous-oxide on Ethan who kicked and single-handedly overturned the contents of an entire room (while the children of a close friend sat next door pleasantly cooperating with their cleanings), I started what proved to be an impossible search for a sedation dentist anywhere nearby. So many dentists had excuses-they didn’t take children; they only did extractions/removals; their client-loads were full. A suggestion from a relative landed us on a simpler (of sorts) solution; to go to Pennsylvania to have his teeth cleaned. Calling the dentist down the street from my grandmother’s house, we discovered the simplicity and ease of finding skilled folks to help us. He gets better and requires fewer clamps in his mouth to assist with the cleaning and examination each time.

Hunger Strike Continues

Although Ethan is making progress such as brushing teeth without vomiting, repeating some words spoken to him, he still does not eat much more than periodic snacks and even then from a very limited food group (mainly chips). With years of speech therapy (which has a feeding/oral-motor skills component) and consultations not making a very discernable difference in eating/drinking behaviors, our pediatric gastroenterologist told us he felt Ethan would not outgrow it at this point but would require some dramatic intervention. He explained that there are not many intensive in-patient long-term feeding institutes in the United States that could deal with the level or extremity of feeding aversion Ethan possessed. He suggested we go to Kennedy Krieger Children’s Institute (affiliated with Johns Hopkins) for an evaluation and in-patient stay. Then the fun began...

We began months of advocacy work trying to get the state on board with prior authorizations to cover expenses incurred during a long hospital stay. Even with two case managers lending support, the hours spent trying to get medical records sent from several doctors and therapists to both the hospital and state as well as trying to discover how to overcome billing denials and lack-of-contract difficulties were proving exhausting. I called my state representative to get some answers while contemplating taking legal action. The apathy towards our situation and ignorance of the system was unbearable. Numerous phone calls and meetings were not getting us anywhere.

To be continued...

Good Things Come