Since you received the news of Samuel’s birth last Saturday 6 June, much has happened in the last 5 days and his situation continues to change day by day. We’ll attempt to bring you up to speed on the main developments concerning Samuel’s health as tests have revealed more about his condition.
On Saturday night, Francis went home to sleep as there was no bed in Shirley’s hospital room and Samuel seemed stable. Around 2am Shirley was woken up by one of the pediatricians who told her that they were concerned about Samuel’s abdomen which had expanded in size overnight. She advised Shirley to call Francis to come in and they had also called the Pediatric Surgeon to come and check Samuel. The surgeon put in a drain tube into the abdomen to drain the gas which immediately reduced the swelling. The following morning, Samuel underwent his first operation to look for the cause of the escaped gas into the abdomen, which they suspected was the result of a perforated colon. The operation took about 2.5 hours and while they didn’t discover a large hole in the colon, they did discover an underdeveloped small colon. This discovery of a small colon, together with the large and fluid-filled bladder and kidneys, suggested a different prognosis to Posterior Urethal Valve (PUV), a bladder obstruction that was originally considered to be the cause of the damaged kidneys. One of the consultant pediatricians explained that these symptoms could be the result of a very rare syndrome known as Mega-cystis-micro-colon-Intestinal Hypo-peristalsis Syndrome (MMIHS - check out this website for more info http://rarediseases.about.com/cs/mmihsyndrome/a/032704.htm), which has a 80% - 90% mortality rate in children under the age of 2 yrs. During the operation, they took biopsies of the colon which were sent away to rule out other possible diseases such as Hirschprongs, however, although there is no single test for MMIHS, all the symptoms suggest that this syndrome is the cause of Samuel’s problems.
On Wednesday, we met with a number of specialists involved in his care, and discussed the test results and where to go from here. They explained that as well as having damaged kidneys, Samuel’s digestive system is not functioning which means that the only way he can receive nutrition is to be fed a solution intravenously into his veins using a technique called Total Parenteral Nutrition (TPN - http://en.wikipedia.org/wiki/Total_parenteral_nutrition ). Only about 7 children in New Zealand are on TPN, and it requires weeks of training for parents before they are able to take the child home. Also, Samuel’s bladder is large and floppy and the muscles are unable to contract to urinate so he will have to use a catheter. Unfortunately, kidney dialysis is not possible if there is no functioning bowel, so Samuel is dependant on his damaged kidneys for the near future. They are monitoring his kidney function by blood tests, and while there is evidence of significant damage, they seem to be still partially functioning. They suggested Samuel would need to remain in hospital for about 2 months before he would be ready to go home, and before then we would have had to have undergone the TPN training and would receive ongoing home-based support by specialist nurses. Longer-term, Samuel would be reliant on TPN for all his nutritional needs for the rest of his life, unless it was possible to perform a bowel transplant, a very complex procedure with less than 50% chance of success and only performed in US or Europe.
On Thursday, Samuel had a big day with two trips out of intensive care across to the Starship Children’s hospital, firstly for a bladder x-ray using a dye solution that was injected into the bladder in order to see if the urine is flowing back (reflux) into the kidneys (it isn’t). We’d only just returned to his home in intensive care when he was whisked away downstairs again to the operating theatre where the Pediatric Urologist wanted to carry out a cystoscopy (camera up through urethra into the bladder to find and remove any valves that could have caused the bladder obstruction that damaged the kidneys, as well as to insert tubes up into the fluid-filled and oversized kidneys to dry and drain them. No valves were discovered (further evidence of MMIHS and discounting Posterior Urethral Valve that we first suspected was the cause) and unfortunately the surgeon was unable to find the holes out of the bladder that lead to the kidneys so was unable to drain the kidneys using that technique. He explained that the only other option was to use what is called a ‘perk’, a drainage tube that is inserted directly into the kidneys from the back but that technique would increase the risk of infection.
We have only held him once when he was about 12 hours old, as he has mostly been sedated and has wires and tubes coming out of his feet, hands, umbilical cord, penis, nose and mouth! But we are hoping that some of these may be removed tomorrow and we will again be able to cuddle our precious little boy. He does have a strong heart and lungs and so far findings show his liver is in good condition. The girls come up most days to see him and they are amazing how they just take him as he is – not flustered by the wires and beeps – both happy to touch and kiss him and have even helped wash him. It is very special to see their love for him even though they have never held him or even heard him cry or seen him with his eyes open.
We have nothing but praise for the wonderful team of Doctors, specialists, surgeons, nurses etc that have been involved in his care. He has a nurse with him solely, every minute of the day, monitoring and recording his various machines and making sure he is as comfortable as he can be.
We will try to update this blog as often as possible rather than sending out email updates, so we hope you can log on to www.samueldavy.com to keep track of Samuel's progress over the coming weeks.