“She had the best care possible — a loving family at home…”
Maria Sircello, D Trisome, June 11, 1961 to February 3, 1964, Ivine, CA
Maria was born when my husband and I were in Hamburg Germany, where he was studying under a Fulbright grant. My doctor was on the staff of the University Hospital, and was puzzled about how small my baby seemed, and why I seemed to go to 10 months pregnancy. Normally in Germany at that time, midwives were used in even in the hospital at birth, and husbands did not even have a waiting room, but went home, and then came back later. We didn’t have a phone. My husband was told it would be at least 12 hours, so left for home about a half hour away.
I was left alone in a large room, and the midwife was quite angry when I screamed out for her. However, when she saw two little malformed feet protruding, she became very frightened, and the next thing I was aware of, very dimly, was being in a room with maybe 10 people, including doctors, crowded around me. When I woke up in a semi-private room instead of the normal ward, a doctor finally came in to tell me that the baby had been taken to a special hospital elsewhere on campus and that she had a “little cleft lift.” My husband was taken later to see the baby through a glass in a new, modern neo-natal hospital building, and seemed shocked when he came to see me. When we questioned the doctor the next day about the “little cleft lift” he acknowledged she also had a cleft palate. We kept revising our talks about her future, now adding a more serious defect, with a later operation, speech therapy, etc. We decided to give her, rather than the name we had planned, “Maria — the most beautiful name in the world” after the song in West Side Story.
More than a week later, when I was finally discharged (I pumped milk for Maria each day and we took it to the hospital, but we only saw her through a window while standing outside the building), we would ask questions about what we could see. Why did her eyes roll back in her head? Finally, we were told she was blind, because she had no retinas. After noting that her outer ears appeared to look strange, we were told she had no inner ears and was deaf. Next came the fact that her head lolled to one side: she had torticollis. Every week brought a new disability. Finally we were told she had five separate heart defects, with small details like club feet, dislocated hips, deformed fingernails. Her doctor was actually a very famous geneticist who had taken part in the discovery of the results of taking thalidomide.
It was a little like having your arm amputated one inch at a time.
Finally, after a delay of about two months in our expected trip home to the United States, she was discharged from the hospital because of weight gain, and we were taught to feed her with a tube if it became necessary on board ship. However, it turned out that wasn’t necessary, and we fed her breast milk using a bottle with a special nipple with a large hole on the side, which we squeezed between our fingers at intervals. She had to be fed every two hours, day and night.
The worst event was when I took her into St Luke’s Hospital near Columbia University in New York, where the Crippled Children’s Division sent her to be evaluated for cleft lip surgery. My husband and I had initially said not to have the surgery, because we saw no point in causing her the pain and discomfort of the operation and being away from home, where we loved and cared for her. However, they explained it would improve her quality of life, and also eliminate our worst experiences when we took her out in her buggy, where people would insist on looking into the buggy, cooing, and then recoil in horror. Her cleft lip and palate were bi-lateral and extremely large, so that a very thick piece of her palate was attached to the end of her nose, and protruded.
A young doctor with absolutely no sense, confronted me in the hallway as I held her, stating crudely that I should put her in an institution and forget about her, since she was “an idiot.” We had always held out the hope, since that was the one topic the doctors had never mentioned, that her brain was normal, and that we could reach her through her blindness and deafness. I had already contacted both the Lighthouse for the Blind and the Deaf organization to begin learning how we could help her. Luckily, there was a nurse standing there, because I immediately fainted, and she would have dropped to the floor if the nurse had not caught her.
The operation did make a big difference not only in her appearance, but in her ability to be fed not only milk but cereal and some fruit. She eventually got to 13 pounds, and everyone in the family (we had a family of four foster children and then had another baby in 1963) and all our friends loved her and loved to dance around the room with her, since it made her smile.
When we moved to Portland for my husband to teach at Reed, our alma mater, we were able to take her to physical therapy, and after many sessions, she was able to turn over by herself. This was a huge improvement in her life, since she didn’t have to wait for someone to turn her. She would tend to whimper if she got uncomfortable in the same position too long. We had a twin stroller, and she went everyplace with us on family outings.
However, the time came when the children, who had come from an orphanage in Germany, had to be vaccinated for small pox. The doctor at Kaiser thought she would be OK, and that if they were vaccinated, it was safer for her to get it as well. However, soon afterwards, when I was home alone with her, she appeared to have gotten perhaps a cold. I was holding her, and she sighed softly and died in my arms.
When my husband came home a few minutes later he rushed her to the nearby hospital, but she had died. Unfortunately, since we were young and had no counseling from anyone, we allowed her body to be taken away to the Medical School, where we donated it to science, thinking that would be the best gift we could give, and that she could give. At that time, the doctors considered her case to be extremely rare, and told us they didn’t know of another child with all those defects who had lived to that age. What I have regretted ever since is that we did not go to see her before they removed her body, to say good-bye one last time.
Later, I had nightmares over years, where she cried out for me that she was cold, and I pictured her somehow on ice at the medical school. However, I do hope that learning more about her body was of some assistance. We recieved her ashes in a tiny box, and put them into a specially ordered hand-painted music box we ordered from Austria.
Over the years, I have wondered many times, and still do, at the age of almost 80, what I would have done had I known about her chromosome defect. Would I have chosen an abortion. Everyone who knew her was enriched by her short life. It taught my husband and me a valuable lesson. We learned, for one thing, that even though we were both considered highly intelligent people, both recipients of prestigious scholarships and awards, that we were “good” people who followed a high ethical standard in our personal lives, and not physically unattractive, but normal in appearance, absolutely none of that mattered. We were not “entitled” to have “perfect” children. Our children were each individuals, and none of them were rewards to us, but they were gifts to us.
So, the question I ask myself is, since she was a gift, and a gift not only to me and my first husband, and to our children, but also to our friends, was that reason enough for her life, or is that selfish on our part. After all, her life was mostly a life of pain or at least discomfort. I don’t think it had a lot of what we would call “quality of life.” All she could do, other than smile when stimulated by being moved around in someone’s arms, was lie supine until someone tended to her. You had very few cues from her as to when she was hungry or uncomfortable. So were those moments when we waltzed her around the room in our arms, and cuddled her, and fed her and stroked her while cleaning her or changing her diaper, or the relief she got when she finally was able to turn over in her crib worth her life the rest of the time?
I just stumbled on this site while looking for something completely different about blindness. One of the gifts that Maria gave was extra knowledge for me about the profession I have adopted. I had always been interested and involved in disability, many years before her birth. I might have done what I do now even without her, but certainly she has been an influence in my middle-age career as both an advocate for disabled access, and a specialist in it. I am a member of the American National Standards Institute Committee that writes all the architectural standards for disabled access to buildings and sites. I am considered the main specialist on the committee for access by people with communications based disabilities, and I represent the Hearing Loss Association of America as a voting delegate. People call me the “ADA Sign Lady.”
I am amazed to know that there are children out there whose defects are obviously not as severe as hers, who go on to live much longer and probably have more fulfilling lives than hers was to her. I am so happy for them, and for their parents and families.
Because so much less was known back when she was born about genetics, I did not hear the exact designation “d-trisome” for some time, in connection with other pregnancies, when I always had counseling. No cause was ever determined for Maria’s condition, and I was told it was probably just a mutation and that I should not worry about other children. That has turned out to be correct. One of my daughters did have a defect that caused her problems when she neared fifty, of very enlarged ureters, but other than that, my three natural children are all very well, and are all highly intelligent. Their problems — and they do have them — are all common in our family, such as depression and near sightedness.
I wish we had known then what parents know now, although it would not have saved Maria. She had the best care possible — a loving family at home, and undoubtedly had the smallbox vaccination not claimed her, some other minor illness would have.