I thought I would post a quick update because I’ve had a few people email me to enquire about Ali’s cardiology appointment yesterday. Thanks for the emails by the way, I really appreciate everyone’s concern for Ali.
First of all, Ali has Strep infection at her G-tube site. She’s on antibiotics currently (which don’t seem to be doing much) and we are treating the granultion tissue around the area every three days with silver nitrate. We had her looked at yesterday and I guess the granulation tissue is really excessive so we have to get rid of it. We are doing everything right but we are loosing the battle which is VERY FRUSTRATING! Hopefully once we get the strep under control the granulation will follow. Strep infections at G-tube sites are fairly rare and very difficult to treat so we may have to do a second course of antibiotics.
We got some very dissappointing and scary news yesterday at our cardiology appointment. Ali has developed Hypertrophic Cardiomyopathy. Hypertrophic cardiomyopathy (HCM) is a disease in which the heart muscle becomes abnormally thick. This can make it harder for the heart to pump blood and can lead to sudden death. Obviously this is really worrisome and to be honest I am trying to forget about it for now. The best case senario would be that she goes on beta blockers (medication)and that the hypertrophy is kept under control for many years. The worst case senario would be that she eventually needs a heart transplant which she won’t get due to tumor risk. Over 70% of people with Costello Syndrome have this condition and it varies from mild to severe. Hypertrophy and tumor risk are the main reasons why people with Costello Syndrome don’t live long lives. We knew that this would probably happen but it’s still a big slap in the face. So now I’m going to forget about it (sometimes I bury my head in the sand for self preservation) and worry about the Strep infection because that’s something that I can fix (I think).
We had to take Ali back to the emergency room again today because her G-tube stoma is really infected. She’s been really sensitive about having the area touhed or cleaned ever since she had the MicKey put in so we weren’t really surprised that it became infected. We’re really sad for Ali. It’s been almost three weeks now since she had her procedure to have the original MicKey inserted and she’s still really struggling. It’s so unfair and it’s so hard to watch a little person like Ali scream and cry every time we try to touch her tube. This is a nightmare.
Other then the G-tube, I think she’s doing really well. She has a new found love for Peek-a-boo and she’s started to open her mouth sometimes when I try to spoon feed her! She really enjoys tasting some of the commercial baby purees and yogurt. We are pushing really hard right now for her to sit. Her OT thinks that she could sit if she wanted to but she really doesn’t care to try.
Ali is not sleeping well at night. She wakes frequently and cries out in pain, I’m hoping that it’s because of the G-tube and not some more severe issue. Once we get this G-tube sorted out then we’ll have to see if it’s still happening and do some further investigation if needed.
Next week Ali has her regular Cardiology appointments. She’ll be having an Echo, ECG, holter monitor and cardiac check up. Please keep your fingers crossed that her heart defects have remained stable.
On Monday we took Ali to the hospital (again) and she was remeasured for her Mickey. We found out that the original Mickey that was put in was way to short. She was originally meausured at 1.7cm but the actual measurment was 2.0cm. That’s a really big difference in a tiny little body. The length of the Mickey was probably responsible for the the Mickey coming out over the past couple of weeks and for all the pain and discomfort that poor Ali was experiencing around her little tummy. By the time we figured out what was going on, some damage had already been done.
We had to put in a new Mickey that was not only longer but narrower. The narrower Mickey means that it may be more difficult to feed the thickened formula, although to be honest I haven’t noticed a difference yet. The reason for the narrower Mickey is that over the weekend Ali’s g-tube stoma started to heal around the emergency feeding tube that we had inserted on Friday. When we went to reinsert the original Mickey, it no longer fit. Ali was screaming because the process was so painful due to all the irritation that the short Mickey had caused so we went with a narrower one to eliminate some of the pain associated with reinserting it. There comes a point when you have to cut your losses and that was it.
The other problem is that the extreme irritation around her g-tube site caused a huge amount of granulation tissue to form around her stoma. Granulation tissue leads to poorly fitting Mickeys and has to be treated. The only way to treat it is to use silver nitrate to burn it off. So once a week we are going to have to go through that lovely process.
The past couple of weeks have been very difficult on Ali and on us but I am happy to say that I think she is feeling much better now. Today while Madi was at playschool, I took Ali for a walk downtown and she was a very happy girl. She had a couple of nice visits with some friends who stopped to say hello and she showed off her goofy grin! I love it when people admire my peanut!!!!!!!!!!!!! We then came home and she had a “bounce” in her jumperoo and had a fabulous time. Things are looking up!
Happy Remembrance Day to everyone. Let’s all take a minute on November 11 to remember the great sacrifice that so many men and women have made so that we can live life as we do in Canada. My grandfather fought in WWII and I’ll be thinking about him.
The last time I updated was after our exciting adventure with Ali’s MicKey coming out. Since then our life has been hectic. Ali’s MicKey has been nothing but trouble and I’m starting to resent that stupid thing. I am wondering who I have to speak with to get a break for my little peanut. I think she’s been through enough.
All week we’ve been struggling with the MicKey. It’s been leaky and irritated and obviously causing her a lot of pain. This darn thing has come out not once but twice and according to her feeding team normally they don’t come out. After many sleepless nights, we decided to take her into the city yesterday to have it looked at and we were told that it was fine. She had a little bit of granulation around the stoma but it looked okay and so we came home with what we needed to stop the progression of the granulation. This morning we got up after another sleepless night and I looked at it and it looked like it was about to rip through her stomach wall. I called the feeding team and I emailed pictures and the verdict is that it is probably not a good fit. It may be too short so she was probably measured incorrectly. So this afternoon I took the MicKey out so that we could replace it with a temporary feeding tube until we can get into the city again on Monday to have her measured. I couldn’t believe what the stoma looked like, it is so irritated. I couldn’t replace the tube. I knew she’d scream so I passed the temporary one to Jason and I left the room. Jason put the tube in like a pro! The stoma still looks pretty scary but the leaking has slowed down quite a bit and she seems more comfortable.
In the past 10 days my baby has had a PEG tube stuck in her throat, severe croup and a badly fit MicKey being inserted and removed from a badly irritated hole in her stomach numerous times. I think she’s due for a break.
This poor little baby seems to have to fight for everything. I wanted this one thing to be easy but it seems like nothing ever is.
