Hi there Charli

Remember Charli? She also has Jacobsen Syndrome and is now almost a year old. She had to have a huge operation recently to change the shape of her head. Here is an extract from the letter her mom sent me. We have been keeping in touch as I am really interested in seeing how Charli develops. I think it will give me a bit of an idea of how Kendra may have developed. Although I think developmentally they were/are quite different. Initially I was also able to give her mom some support/guidance or basically share experiences but now I think she has progressed beyond needing that as Charli will soon be older than Kendra got to. Isn't she gorgeous?

Before the operation.

After the operation.

Charli had her operation on Wed 8/11 to correct her craniosynostosis. It was a success and she is on the mend and we got to come home yesterday so she was out after 5 days but it was possibly the hardest thing I have ever been through. It was just so heartbreaking to see her so helpless and vulnerable and there was nothing I could do. Charli was admitted the day before and given platelet transfusions, then more during the surgery and afterwards. Besides a bit more than average blood loss (she lost 1L and was given 2 transfusions) it all went really well. She still has a lot of swelling and bruising but has good colour and no temps anymore. I was prepared to see a lot of swelling and bruising but nothing could have prepared me for how white she was, she was almost translucent. It gave us a big fright but that's all over and done with now.

I will send photos of her new little head. Originally they were just going to remove the strip of fused bone down the centre and 2 strips of bone from both sides of her head so that her brain would have more room to expand out. This would have meant that it would be a long time to see a result as her skull would have just grown around her brain shape. But amazingly the day of her surgery they decided to do the major op involving leaving the strip of fused bone and taking the 2 side plates and front plate out moulding it to a rounder shape then reattaching it (it took 5hrs). The plastic surgeon apparently did the same surgery on another Jacobsen's child a month ago and it was a success so they were confident Charli could be kept stable.So I guess the operation being postponed in August happened for a reason. Doing that op meant that Charli has the round result straight away and better still no more operations are needed. But I have to say after seeing what she went through I don't think I would subject her to more surgery anyway.

She is doing really well and is the strongest little girl. I tried to stay positive but it was always in the back of my mind that there was a chance of losing her and I just don't know how I would have coped. But you know what if we had have lost Charli I would have known she would have had a little friend named Kendra waiting for her in Heaven and that is comforting.

I was a little worried that Charli would lose a lot of weight and perhaps forget some of the things she had learnt but she is back to doing all her old tricks and only lost 20 gms she weighs approx 7.5kg. Charli has been sitting for a few weeks now without support, rolling, weight bearing and has 3 teeth. She loves her solids an prefers them to her bottles.

I was hoping you might if possible mention something on your blog site just so that other people with Jacobsen's kids know that with platelet transfusions it is possible to keep the kids stable for any surgery that is needed. The docs were very unsure of how to deal with Charli's situation because it is not very common.

Edited to add: Ok, I cannot get the captions to appear next to the proper pictures. Blogger is not that wonderful sometimes. But I think you get the idea.