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Her recovery in ICU was very slow this time, not at all like previous admissions. She bounced up and down between ICU and the ward a number of times, there was definitely something not quite right. After more incidents of formula being suctioned up it was apparent that Lauren’s muscle spasms were so strong that they were responsible for her stomach contents being forced up through the fundoplication. We decided to change her feeding routine to take pressure off her stomach. Bolus feeds were replaced with  a combination of  smaller bolus feeds and pump feeding over a longer period of time.

Another story has been unfolding behind everything that has been happening to Lauren. We wanted to have another child but we weren't prepared to take the risk and conceive another child with Lauren's problems. A number of doctors have said they suspect Lauren's condition is autosomal recessive and there could be a one in four chance of having a child with the same condition. We decided to use IVF technology with the use of donor sperm to avoid any chance of repeating what we have been through with Lauren.  After three long years of hope, anguish and frustration we were finally successful in achieving our goal. Carolyn fell pregnant and our precious baby boy, Daniel was born on 24th. May, 2002. He is healthy and well, very alert and active. He is giving us much pleasure and is adding a new perspective our lives. Lauren is getting aquainted with her new baby brother and appears to enjoy his company.

 

SUMMARY 
Lauren has experienced a range of behavioural and physiological problems which have been very difficult to control or manage with confidence.  Often she does not respond  normally to medications and treatments.  She has been hospitalised numerous times to address these problems.

CONSTIPATION:  She is very susceptible to becoming constipated.  Monitoring fluid intake and using laxatives and enemas has proved to be effective.  A number of  laxatives had no effect.

POOR MUSCLE TONE:  She is a very floppy child, incapable of supporting herself.  She cannot sit or roll, her head support is very poor.  She tends to drop her head forward when she is supported upright.    She is unable to move her legs voluntarily, they only move during muscle spasms.

MUSCLE SPASMS:  She experiences muscle spasms constantly, even in her sleep.  In the past they have been noted to occur up to 40 times an hour.  These spasms are often linked to irritability and can be distressing to her.
Lauren has had three EEG’s to determine if these muscle spasms are actually fits of an epileptic nature.  The results do not show evidence of epileptic brain patterns.    

FEEDING PROBLEMS:  Lauren appeared to feed well from mother’s breast milk for the first 10 months.  She was reluctant to accept formula or solids and there were concerns about her growth in general.  She was nasogastric fed to supplement her oral intake, but this proved to be unsatisfactory and was stopped.  Lauren seemed more comfortable when the total volume of her food intake was less than 400ml per day.  Feeding consisted of numerous small feeds per day in order to reduce her irritability.  The formation of calcium oxalate crystals in her urine led to the decision to have a gastrostomy tube inserted.  This was the only way to increase her overall fluid intake up to around 700ml per day.  The insertion of the tube has resulted in Lauren refusing to take food orally, now all her feeds go down the tube.  She has gained weight but she is more irritable and does not tolerate the additional volume well.