Hannah & Josh Rainsforth’s Story
Hannah & Josh Rainsforth
Parents Name:Michelle & Adam Rainsforth
Childs Age: 6 & 4
Childs Condition: VARTER & AP Window
Story:
Hannah was born on Wednesday 11th March 1998 in Darwin Private Hospital .
We were expecting a normal, healthy baby so when she was delivered and, being a bit blue, required oxygen immediately we were all shocked. Then during all the chaos, my husband noticed her right thumb didn’t look quite right. He told the obstetrician he thought Hannah had a broken thumb. After a quick glance at the thumb by the doctor the paediatrician was paged immediately.
The Pediatrician arrived shortly after and checked Hannah over thoroughly. She was then taken away to the special care nursery where she spent two weeks being X-rayed, having bloodtests and ultrasounds.
During this time there were a few suspicions about what the problem was. It was expected she may have Persistant Foetal Circulation where the babys’ body takes a little more time than usual to adjust to breathing and life on the outside. Though there was also the matter of the skeletal deformities and Floating Thumb.
The ultrasound tape and other information was sent to Adelaide’s Womens and Childrens Hospital for a review by specialists while we anxiously waited for something to happen. We were also transferred over to the public hospital so other doctors could review Hannah and so we would be ready if word came to leave for Adelaide.
It did and we had 2 hours notice. Lucky I had a suitcase packed in the car just in case.
With a nurse and Hannah in a humidicrib for oxygen we were greeted at the airport by staff ready to load us onboard and leave as they held the flight back for us.
We arrived in Adelaide and spent yet another week going thru a battery of tests including a catheterisation which confirmed Coarctation of the Aorta, ASD and VSD.
The overall diagnosis was VARTER (or VACTERLL) Association. No known cause and it wasn’t genetic.
Two days after the catheterisation we were on our way to Melbourne to the Royal Childrens Hospital.
The next day, she was in surgery. We were warned of the risks and complications as we all are but weren’t really expecting them.
Hannah came back from surgery to ICU with her chest open which was closed two days later. Five days after the surgery she left ICU to go to 7 West, the cardiac ward. The next morning I walked into the ward after breakfast just as she was surrounded by countless doctors and nurses waiting to be taken back to ICU. Her right lung had collapsed because of damage to the Phrenic nerve which operates the diaphragm. Once back in ICU Hannah spent another four days on the respirator to reinflate her lung. The next day we were back on 7 West again. We were told if it would happen again it would be within less than a week.
So we waited and just as we thought we were going home an echo showed up an 8mm blood clot in her heart. W weren’t going anywhere until doctors were certain it wouldn’t move and it started to reduce in size. It didn’t move and it began to shrink then her lung collapsed again. A week after coming back to the ward. She had a bad day feeding and her sats were going up and down all day. By this stage I was suspicious that something else was happening. The next morning I found her on another drip. Her lung had collapsed again and we were waiting to go back to ICU for the third time. After three days on the respirator it was decided she needed a diaphragm plication to her lung reinflate and expand properly .
The morning after she smiled at us. Everything was finally going on track. We finally left for Adelaide, then two days later, back to Darwin. We had to spend another week in hospital there before we went home as we set up the home oxygen supply and organised equipment supplies.
Hannah spent the next six weeks being weened off the oxygen. She gradually lost here swallow completely over a couple of weeks from a paralysed vocal cord and was fed through a nasogastric tube for 11 months before having a PEG inserted at PMH.
We had moved back to Perth when she was 6 months old to be closer to specialty facilities for her . Hannah needs three monthly orhtopaedic checkups, feeding team, has had plastic surgery to remove the floating thumb and is now waiting for speech therapy.
Hannahs PEG was removed in June 200, she eats and drinks everything now.
Hannah has 6 monthly spinal checks which have recently shown her neck is getting worse at 41 degrees. This could mean spinal fusion surgery in the next few years.
She is also having speech therapy, occupational therapy for developmental delays and physio therapy for balance and strength. In a couple of years Hannah will need to wear a dental plate to widen her top jaw aswell. Given all of this, we are having eye tests done soon to make sure her eye sight is ok. I have learned to go to new appointments expecting a problem, so if there isn’t one I am relieved.
Hannah started kindergarten this year and loves it. She is doing well, better than expected. Learning her numbers and colours so quickly now. Her speech is still a work in progress. Once Hannah is in pre primary and year 1 she will probably need some assistance with learning reading and writing but I am sure she will love it also. Hannah has persistance and drive that is amazing. She goes non stop all day.
Hannah now also has a little brother. Joshua.
Joshua was born on the 31st of March, 2000. We had a foetal echo at 19 weeks during pregnancy and everything looked good. Still, when he was born we requested an echo just to be sure. We were told by the pediatrician if he couldn’t hear a murmur he wouldn’t do an echo. No murmur was detected so we went home happily with our “healty” baby boy.
What followed was a few weeks of unsuccessful breastfeeding with Joshua loosing weight so he was put onto formula. He still didn’t gain much weight though and was constantly at the local doctor for coughs and colds.
He also had an Xray which showed a mild case of “pnuemonia” with afollow up Xray after the course of antibiotics. He still had a persistant dry cough and was still not gaining weight. After seeing another doctor at the surgery we received a referral to a pediatrician to investigate further.
Two weeks later we were at the pediatricians. He looked at Joshua, listened to him then turned to me and said sit down and we’ll have a talk. My stomach flipped and my heart sank. That must mean something was wrong. Five little words broke my heart. “There’s a very loud murmur.” I could not believe we were here again. I called it a time warp. While the world continued at it’s normal place, mine came to a screaching halt. Then began again at a snails pace. Every minute seemed like an hour. “He needs to be in hospital” “Now?” “Yes.” “PMH?” “YES.” “We’re on our way.”
I called my husband from the doctors’ phone and said, “Joshua needs to be in hospital. Be ready, I’m on my way to get you.” Nothing else. The pediatrician made me sit and calm down for a few minutes before leaving. It didn’t work. I balled my eyes out all the way.
Once we arrived at emergency at PMH we were greeted by Hannahs pediatrician which saved us a long time of explaining the family history. They did an ECG and Xray then admitted us to the infants ward where we spent a week being monitored, having tests and having the catheterisation ready for surgery the following week. The results showed an ASD, VSD and a suspected PDA or AP Window. AP Windows are rare and very difficult to find even during surgery but the problems Joshua was having did not correspond with just the VSD and PDA. The surgeon was told of the possibility of the AP WIndow. It is a hole between the Aorta and Pulmonary Arteries where they haven’t seperated properly during development. It had caused Pulmonary Hypertension and could cause problems with his lungs after surgery as the pressures dropped. Spasms that could stop the lungs from working.
Surgery was on Tuesday the 15th of August, 2000. Six and a half hours waiting. There was a big AP Window. Hold up your pinky finger and thats the Aorta. The hole was 2cm. It was patched along with the ASD and VSD. The next few hours were even worse with Joshua bleeding everywhere. He had two episodes when he required cardiac massage. Joshua went back to theatre at 7.45pm that night and came out at 10pm. There had been a couple of tiny stitch holes that should have seeled over but due to high blood pressure they hadn’t and had kept bleeding. With some reinforcing stitches and another drain in place he recovered quickly. None of the expected complications arose and he was out of I.C.U. in less than 48 hours.
We went back to 5A began feeding well. A huge relief after our experience with Hannah. Joshua came of the oxygen after a couple more days and we were sent home just seven days after surgery.
Joshua is now strong, and feeding well. He is gaining weight and very happy.
Even though we have two heartkids, they are both well and happy and the future is bright with Hannah now chewing. Hannah may need more surgery when she’s older but only if her aorta reconstricts. For now, Hannah and Joshua are very healthy and happy.
Best wishes to you all.
Michelle Rainsforth.
Proud mum to Hannah and Joshua. My little miracles.