Julia Kennerley
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BEGINNINGS
Julia was born on a still, muggy Saturday evening in December 1995 at our local hospital in Wellington, New Zealand. To my husband, Brent, and me, she seemed like a precious and perfect Christmas gift.
We were delighted when she was awarded an Agpar score of 9 out of 10, even though she weighed only six pounds. My pregnancy had been difficult with morning sickness, and later high blood pressure, pre-eclampsia and bed rest. Julia was induced when she was ten days overdue, and the labour was short but painful, ending with a difficult forceps delivery. So we were extremely thankful to learn she was well.
However by morning our happiness had evaporated. Julia was refusing to breastfeed, and had vomited up all of the tiny amount of formula which she would tolerate. Her rosy colour had faded to a pale sallowness, and she was floppy and lethargic. Then to our horror, twelve hours after her birth, she began to vomit green bile and meconium.
The next few hours were a blur. We were transferred to the neonatal unit. I remember the young doctor on Sunday night duty telling me repeatedly and unhelpfully "it's not your fault", but failing to answer my questions about what was wrong. Suggestions of a malrotation, bowel obstruction, or a meconium plug. Consent forms to sign for invasive exploratory tests. Seeing our beautiful little girl lying naked and vulnerable under the lights in the intensive care unit, beginning life surrounded by strangers. Praying that she wouldn't die. Grieving. Finally, the results came through - "just a meconium plug" which could be cleared with an enema. "Is that all?" we said with great relief. But our relief was shortlived, as we learnt that the meconium plug was probably caused by a medical condition, most likely cystic fibrosis, but possibly also Hirschsprung's Disease.
LATE DIAGNOSIS
Life became a nightmare. I was discharged from hospital, and we came home without our longed for first baby. Everywhere we looked, people were enjoying Christmas celebrations, but the happiest time of the year had turned into one of the saddest times of our lives. Initial tests on Julia were inconclusive, but we believed instinctively there was an underlying problem.
Finally, three days before Christmas, we were allowed to bring Julia home. She was still refusing to breastfeed, but was tolerating small amounts of formula. But at home her condition soon deteriorated. It would take an hour or more to get her to take just 30 mls of formula, most of which she then vomited back at us. Her bowels were again locked. On Boxing Day, we were back in hospital with Julia bilious vomiting and dehydrated. This was to be the pattern over the next six weeks - in and out of hospital. Julia would scream; refused to feed; vomit bile; be severely constipated or tip over into haemorraghic colitis; and then spontaneously recover enough to return home. After six weeks of waiting, cystic fibrosis was thankfully ruled out, and an early positive test for hypothyroidism was proved inaccurate. Finally, when Julia was 6 weeks, we were told Hirschsprungs was the most likely diagnosis and prepared for an emergency colostomy. On the day the rectal biopsy results for Hirschsprungs were due, Brent and I drove to a nearby beach and prayed for strength and that Julia would be spared the surgery. Our prayers were answered, for much to everyone's amazement, a single ganglion cell was found, meaning the results were inconclusive and Julia was discharged yet again without a diagnosis.
Then Julia turned a corner. She began to breastfeed, to smile and be happy. She alternated between constipation and diarrhoea, but somehow still grew and developed. We knew something was wrong, but nobody could tell us what. There were days of fear and despair, when Julia would refuse to feed, and was quiet and in pain. But at other times she was well and active, and we felt like a normal family. We began to feel people were treating us as neurotic parents, and we were told constantly that Julia would outgrow her problems, that we should be more confident in our parenting, and to stop worrying.
But problems resurfaced in earnest when Julia started solids at six months. Her bowel movements just stopped. We began again on the round of visits to medical experts. X-rays revealed a megacolon, and it took huge quantities of laxatives and enemas to make Julia pass any motion at all. We were told her barium enema results were highly unusual, but not typical of Hirschsprungs.
SHORT SEGMENT HIRSCHSPRUNG'S DISEASE
Finally and thankfully, we were referred again to a local paediatric surgeon, Kevin Pringle, who had performed Julia's first rectal biopsy. He took one look at her notes and x-rays and said, "Julia has HD". By now sceptical of medical experts, we demanded a repeat rectal biopsy. This time there was no doubt about the results. We were shocked, but deep down relieved to have an answer. Only a very small segment of Julia's colon was affected, and this, combined with her amazing ability to thrive despite everything, contributed to the late diagnosis.
COLOSTOMY SURGERY
Julia had a colostomy formed at 9 months. She suffered a life-threatening bout of enterocolitis immediately after surgery, but responded to IV antibiotics. Her bowel was so distended that she was expected to need further surgery to make a new stoma before her pullthrough operation, which was not scheduled until she weighed 10 kilograms and her bowel had healed. Sure enough, she began prolapsing within three weeks of surgery. Determined to keep out of hospital, and with the support of a wonderful ostomy nurse who called at our home every week, we got very proficient at reducing the prolapses ourselves. But, every time Julia cried for long, or got too excited, a prolapse would occur. We lived on tenterhooks but grew more confident as time passed. Most importantly, Julia thrived. Our little girl who had always been so interested in food suddenly developed an insatiable appetite and grew! We had a very happy Christmas and summer, so different from the previous year.
PULLTHROUGH AND BEYOND
At 16 months, Julia underwent a Soave pullthrough operation and colostomy closure. Two days later, she passed that first miraculous bowel motion, and we began coping with diaper rash and frequent stooling. It is now over a year since Julia's pullthrough, and we are making real progress. Julia has fought several bouts of enterocolitis, and will probably continue to - a legacy, in part, or her late diagnosis. She also still has a tendency to frequent stooling, but careful juggling with her diet is helping control this. Natural remedies like aloe vera juice and acidophilus have been very beneficial. We have yet to experiment with laxatives and enemas, holding these in reserve for any future continence problems. But a few weeks ago, Julia initiated her own toilet training, and is making good progress.
Despite (or perhaps because of?) all she has been through, Julia has a sunny, confident personality. People learning her history for the first time are always surprised because she looks so healthy. She lives a very "normal", busy life.
I will never forget the fear and grief I experienced when I first learnt that Julia had special needs. Yet both Brent and I have grown from this experience, found we have depths of which we never dreamt, and have truly learnt the meaning of unconditional love. We feel forever blessed to have been given the gift of sharing our lives with this very special little girl.
Jennie Kennerley
Wellington, New Zealand
September 1998