People you meet along the way...

I was thinking about a friend of mine and her daughter today and that got me thinking about other people we've met over the years too.



Corinna was the first person I found whose child had the same heart condition as Emily. Tis a very rare defect, around 1 in 300,000 live births, and I've not been able to find a child older than Em although I know there are some out there. It was Corinna who introduced me to a couple of online support groups and the friends I've made through them have been with us through thick and thin. Corinna's daughter Nia has been very unlucky in contracting leukaemia (again a very rare strain). She doesn't do things by halves, Nia. At some point over the next couple of weeks she's due to have a bone marrow transplant and they are on my mind alot.

The appointment.

The results of the MRI were inconclusive.



This is more than a little disappointing as it means that Emily has to have a cardiac catheter to clarify what's going on. This issue is with her pulmonary artery which is narrowed where the left coronary passes through it. On echo the pressures are high, the stenosis is graded as severe and would normally require surgery before causing permanent damage to the overworked ventricle. That said, past catheters have shown that the area is difficult to measure on echo and measurements taken once inside have been more acceptable. Just hoping that this is the case now and the cath will just show that ongoing monitoring is required rather than intervention.



I'm trying to be hopeful but my pessimistic side is currently winning the battle going on in my head.

Hello.....

I've rather neglected the blog haven't I? It took me years to start it and now it's only half told. Still, it got it out of my system for a while.

I'm back because Emily went to Birmingham for an MRI this week. It's not something she was looking forward to but she did so well. It was a very long scan, with lots of breath-holding. The machine is very claustrophobic and noisy and it's not something she wants to repeat so I hope the pictures are in focus!

The follow up appointment is in four weeks. So now we wait.......

Fast forward.....for a moment...

Ok - so whilst this is really a 'historical' blog there may be the odd foray into real-time for relevant updates. Confused?!

Today we had an appointment with Emily's orthopaedic surgeon.

Following on from the clot that I posted about previously, the blood flow in the leg was compromised and it didn't grow properly. By the time she walked at around 18 months it was obviously much thinner and a good bit shorter. She has had very weak pulses in that leg all her life. We had some physio whilst she was small to help with strengthening the muscle and she has always worn a raise on her shoe in order to maintain her back and hips in alignment. Her surgeon was always hopeful that her would be able to level her out by a procedure called epiphysiodesis. The surgery involves putting pins (big fat Frankenstein bolts) in in the leg around the growth plates at the lower end of the femur and the uppper end of the tibia/fibia. The pins restrict the growth of the bone and allow the good leg to catch up. This all went ahead when she was 12 and the pins removed 2 years later.

The upshot of today's appointment was that the wounds are well healed, she's fully grown and is as good as level. Discharged. Success!!

Escape from PICU

Finally we made it to the ward. Not far physically - PBG ward which was right next door to ICU in the old Children's Hospital - but it was a massive step forward and I did finally start to relax a little and believe that I would bring her home eventually.

I'm not sure that anyone would be interested in a blow-by-blow account of the next few months. We continued to have some bad days but they were more than outweighed by the good. Emily had had no proper nutrition during her time in ICU - only maintenance fluids as she couldn't seem to tolerate even the tiniest amount of feed. There was a short foray into TPN but that was discontinued when there was a concern about infection. So she left ICU at barely over her birth weight despite now being around 20 weeks old. She looks so skinny in this picture with Josh. Look at her stick legs! And the bald patch on the side of her head where she had a line in after all her other veins gave up the ghost.

Feeding, putting some weight on, and sorting out her long-term drugs were the next goals. She was still in quite severe heart failure and this was likely to continue for some months at least, still couldn't suck so all her feeds went down the tube and we gradually increased from 10ml an hour to something more reasonable. The feed had so many additives to increase the calories that it was as thick as custard and couldn't be gravity fed. It had to be pumped or syringed via the tube. We were patient though and she gradually gained a little weight.

A cuddle at last

Emily continued to give us some scary moments but on the whole began to improve. She did not want to be weaned from the vent though so it was slow going. Towards the end of the fourth week post surgery she finally got extubated and into a 'headbox'. It was fabulous to see her face again without the tubes although she still had her nasogastric tube which was to become almost a permanent fixture for several months. And better still she was beginning to be weaned off some of her drugs. The morphine and sedation withdrawal made her particularly miserable; unsurprisingly as she'd been on it for quite some time. She worried me for a time as seemed unable to focus on anything but that slowly got better over the weeks.

Being able to hold her in my arms was wonderful. If you've ever heard the phrase 'my arms ached for her' then I can tell you that it is almost a physical pain.

Worries averted.

It took some time for her to become settled again - she was having periods of tachycardia and desaturating. They wondered if she'd had a brain bleed and was fitting despite being on full support so there were more tests to endure; scans, ultrasound and an EEG. All proved to be clear and during the next few days she was finally listed as stable as opposed to critical. At last something to celebrate!