Joni's Journey

Our baby and her journey so far

Surgery!

So, as promised, here I am with details of Joni's surgery. You might recall that we were shocked recently with the news that Joni-Mai would be needing a hip replacement. Last Thursday, we had the chance to sit down and discuss with Chris Henry - a specialist sarcoma nurse - the surgery, procedures and implications on Joni-Mai's life.

I will put everything I think relevant to the readers and friends and family in a bullet point list. It makes it easier for me and for you to understand. I will also include some of the questions we asked, so you know exactly what was said.

• How long is the operation?

The operation itself will only take an hour and a half (estimated) but she will be in theatre for about 4 hours. This includes opening Joni-Mai up and stitching her back together after the operation.

• How long is recovery?

Joni-Mai will have to spend 6 weeks in either a hip brace or a hip spica to help set her new hip. It is believed - due to her age and size - that she will need to have a hip spica. Recovery after removal of hip spica depends on Joni-Mai. If she is willing to participate in physiotherapy sessions, she will recover quickly. If she isn't so willing, it will take longer for her to get better with movement and maneuvering with her new hip.

• How will replacing the ball part of the joint affect the socket part of the joint?

In the immediate future, there will be very little - if any - noticeable difference on the wear on the socket. Because she is so young and her bones are still growing and developing, there is nothing that they can do to protect the socket without damaging it further. In time, when she is older, she may be able to have surgery to place something in the socket to protect it. At the moment, the only thing that could happen would be for the ball part to pop out of place - which would cause some discomfort - but in time, there are things that could be done to minimise this happening. For now, Joni-Mai has to get used to using her new hip as naturally as possible.

• How will the new hip affect maneuvering things like stairs?

To begin with, she may be wary of things like stairs but, with practise, she will manage them just fine. She is determined enough to get on so she will be just fine. Physio will teach her how to use stairs in a way that she finds comfortable and as time progresses, she will do them as she always has done. She may walk with a slight limp but this is unavoidable.

• What kind of growth mechanism will Joni-Mai have in her prosthetic bone?

Because of the size of the bone in Joni-Mai's leg, it is likely that she will have an invasive growth mechanism - whereby, to lengthen the bone as she grows, she will need to go into surgery and have it lengthened by an alan key mechanism - as opposed to a non invasive growth mechanism. Joni-Mai can, in time, have her hip replacement replaced with a non invasive mechanism, this will mean taking away everything that is in there already and replacing the whole lot. This is possible and will mean more intense surgery but in the long term it will probably be more beneficial for her to do this.

• Will repeated surgery cause damage to the remaining bone?

In the short term, no. In the long term, possibly. But as we age, science and technology progress so we might be able to do something before anything occurs.

So, as you can see, we have a long term issue but, it is better than any other alternative. Joni-Mai will only get about 5CM of growth from the new bone they are putting in, so we expect to be back in to replace the head in about 18 months time. This means more surgery but we will deal with this when it occurs. In the long term, Joni-Mai isn't allowed to play on bouncy castles or trampolines. This is indefinitely. Joni-Mai will also not be allowed to play contact or high impact sports. We are to discourage any ideas of ice skating and if she wants to take up horse riding as she gets older, we will have to go into discussion about it.

In short, she's going to have a restricted childhood - in my mind - but it is in her best interest. I'm sure there are things I have missed and if there's anything you want to know, please leave a comment and I will get back to you!

Mummy Stevens x

VIDE 5

Monday 9th January - Friday 13th January

So this week saw course 5 of 6 pre-surgery chemotherapy. It was our hardest yet because of all of the different things that hit us, new bumps in our road.

As part of Joni-Mai's treatment, she needs to be monitored for changes in her body. This includes checking her heart and other organs in her body to see that they are functioning properly. We already noticed that there were possible issues with her hearing - which, after being checked seemed nothing for us to worry about. Joni-Mai also seems to have had some hair regrowth which is easily explained - the chemotherapy drugs affect ONLY the cells that are working at the time of being administered. Thus, if her hair is growing at the time of receiving chemotherapy, her hair will fall out again. If her hair is not growing at the time of treatment, it will not be affected and therefor can grow the week after chemotherapy.
The main thing they need to keep an eye on, is the heart. Joni-Mai, before VIDE1, VIDE4, VIDE5 & VIDE6 has to have ECHO scans on her heart to make sure that the medicines aren't having a detrimental effect on it. If there are signs of problems then they can act and prevent extra problems occurring.

After spending the morning in the clinic, we had an appointment at Great Ormond Street Hospital for an ECHO, there was a long wait and after seeing the technician and waiting for the report, we headed back to UCLH. To us all seemed fine but, before the dinner time, one of the doctors had pulled us to one side and explained that there was a problem arising with Joni-Mai's heart. The problem was to do with the left ventrical (Fractional Shortening) its numbers weren't as high as they ought to be. Before VIDE1, her FS was at 46% - absolutely perfect and very strong. Before VIDE4, her FS was at 38% which is a perfectly acceptable decline in three rounds of chemo. The problem was that VIDE4 knocked the figure down to 31%, this was the worrying thing. One course of chemotherapy knocked her heart quite badly and the fear was that if they give another dose of one of her chemotherapy medicines, it might push her heart so far down that she'd need medication to bring it back, it would also mean that there was a chance of there being problems when she was an adult. The cut off figure for medical attention is 29-30% so you can imagine our anxiety at being told this news. The doctor said that a consultant cardiologist would be assessing the images from earlier in the day and they'd make a decision regarding the medicines between them.

After a good night's sleep, the doctors had all been in contact and confirmed that the FS was indeed down at 31%, the three options were -
1) To administer the full dose of Doxorubicin (the drug causing the problem).
2) To administer a modified dose of Doxorubicin.
3) To cut the Doxorubicin completely and review after her pre VIDE6 ECHO.

They finally decided to cut it completely this time and review it before VIDE6, this would give her heart extra time to recover and then if needed, she would be referred to the consultant cardiologist to assess the damage and take steps to fix. So, for now it is a case of hoping and crossing our fingers. We want her heart to fix itself because on top of everything else, a heart problem would be rubbish!

So we began the chemotherapy on Tuesday, Joni-Mai had all of her anti-sickness medicines (a cocktail of Ondansetron, Dexamethasone, Domperidone and Nozinan) and started her pre-chemo hydration. She spent the afternoon going to school and playing in the playroom and she had her regular visitor - Uncle Eddie (she ADORES her uncle Eddie). We had some cake in the day and Joni-Mai had a few jelly babies with her Uncle Eddie - nothing too out of the ordinary for her. In the evening she started to develop a strange rash. It appeared and disappeared before our eyes, and kept doing so for the next thirty minutes, or so. I told the nurses that there was a strange rash and after watching her they decided it was an allergic reaction to something! I held my head in my hands and watched the rash come and go. She'd had nothing out of the ordinary and there was no other explanation for the rash. After some quick thinking, one of the nurses thought to check Joni-Mai's blood sugar levels. Her level was all the way up at 11.4 - FAR TOO HIGH!! So, they cut her drip off and changed her drip bags from glucose solution to saline solution with the medicine added. I can only begin to tell you the stress I was feeling at this point. After doing some reading, I learnt that the combination of the Dexamethasone and chemotherapy plus all the glucose that's pumped through her little body can indeed cause diabetes! "OH CRUMBS!!!" I found myself saying A LOT!!!!! The nurses cut the Dexamethasone from her medicine cocktail and it seemed to work in lowering her blood sugar levels.

The rest of the week went smoothly, until Thursday when her blood pressure was too low. This could have been because she wasn't eating and drinking enough, it may have been caused by the Nozinan - it makes her all droopy. By the Friday, she was fine again! So, a very eventful week.

Please accept my apologies if this post is all over the place, I had a lot to try and remember! We also had a meeting about the surgery. I will put a new post about that tomorrow.

An afternoon of school!

So, Joni-Mai has spent the last 3 afternoons going to school! Today she took sweets in for her class mates, because it's going to be another 2 and a half weeks before she goes again. A farewell, of sorts. She loves going to school, she enjoys seeing her friends and learning new things so it's sad when she's been and has to say goodbye again!

It's also nice to see familiar faces, so many of the mums and dads have gotten to know us now. It's nice to say "THANK YOU" to all the people that go out of their way to say kind words and do nice things for us.

Joni-Mai is getting ready to go to the pantomime now, so I must go and supervise what she's changing into. Otherwise it'll be A princess costume with Tshirt over the top and crocs... hmmmm!!!!

Can YOU help??

!!!WE NEED YOU!!!

A fundraising event is being held on Friday 20th January FOR THE LOVE OF JONI and WE NEED YOU!!! Can you donate anything for a raffle prize? Or even for an auction prize?? Could you provide a service to help out?? If you feel you can, please get in touch with me! You can find me on twitter @HollyJadeBear, on FaceBook or you can email me: Email HollyJadeBear


If you don't think you can help but would like to make a donation or attend the event please go to WWW.JONI-MAI.CO.UK