What an amazing and rewarding three months it’s been. We wondered if medications would be an issue because she takes several medications and they don’t taste great. Right on that very first day, we realized that meds were not going to be an issue. Very quickly and very easily Ali learned to take her medication orally from small syringes. It was messy for a few days but within the first week she had mastered it. We carefully tracked calories and fluids for the first two months to get a feel for what she was eating and to make sure she was eating enough and getting enough to drink. Over those two months Ali slowly increased her fluid intake and she slowly learned to tolerate new textures and tastes.

I took another huge step and I stopped counting calories and fluid volumes. It was tough to just let it go after 4 years of calorie counting and worrying about Ali’s fluid intake. I’ve written here before what a huge part of our lives it was to count everything that Ali ate either via tube or orally.  It was an absolute necessity and it was how we kept her alive. I hated counting and it was stressful to try to meet her goal every day and the day I stopped was like a huge cloud lifted off my shoulders.

In the past months Ali has made more progress then we every could have imagined. In 7 months Ali went from being %100 tube dependent to not using her tube any more. We had recently been told that we had a very long road ahead of us in regards to oral feeding. I was devastated that day and that was less than a year ago. She still doesn’t chew or bite but she manages great with all purees and small pieces. She eats meat (cut small enough that she doesn’t need to chew), pasta (cut small), cottage cheese, fork mashed veggies, small pieces of fruit, cheese and small pieces of bread. She devours yogurt, apple sauce and other purees. Her favourite two meals are tomato soup with crushed gold fish and small pieces of cheese and fork masked Mac and Cheese. She also drinks from a straw and from a cup and can now drink an entire juice box in one sitting.

So, where do we go from here? We keep working on chewing and biting but in the meantime we can go ahead and look into removing Ali’s G-tube.  We are planning to talk to her doctor about it next week. The feeding team wanted her to go three months without needing it and she did. I honestly never thought we would see this day. Throughout the past four years, the eating has always been the big battle in my eyes. Aside from the cancer risks, the G-tube has always been the hardest part of having a child with Costello Syndrome to me. I know that having a G-tube was a necessity. Never once have I regretted putting it in. It kept her alive and because it was “permanent” it was a lot easier than living with some of the less permanent options. Ali would certainly not be where she is today without having had her G-tube. But to think that soon, she won’t have it anymore is almost unbelievable to me. It proves that the best things in life are worth really fighting for. I am so proud of my steak eating, cake loving, juice box devouring little girl. She did something that I honestly thought she might never do and she did it faster than I ever thought was possible. So, hopefully we have a big day coming up soon and that would be the day we go tube free! I’ll keep you posted!

From the day Ali was born, I knew that something wasn’t right. I’m her mom and I think the instincts of a mother are incredible. When she was in the hospital for those first 6 weeks and everyone was waiting for her to recover from her overdose of dijoxin, there was a part of me that wanted to scream that there was more. In my heart I knew there was more.

Eventually we went home. I don’t think many people know what we went through in those first months. We didn’t know what was going on with her and it was such a steep learning curve. We suddenly had feeding tubes to deal with and there were medications and medical supplies. There were nights when I used to be up with her all night wondering if she was going to suffocate from all the fluid in her lungs and times when Jason and I were both up trying to decide if she needed to go to the hospital. There were days when she threw up everything that she got through her feeding tube and she used to retch and gag and choke. I hated Fridays because we couldn’t reach our doctors or support teams on the weekend and when we took her to the ER they were nervous about dealing with her. We eventually closed all the curtains, laid her on a pillow on the floor, surrounded her with burp cloths and made sure that Madi and the dogs weren’t around to make noise. We simply didn’t touch her. She hated to be touched and her reaction to being touched was physical. It was like it was hurting her.

Then came the week that I think was our absolute lowest point. We found out that my instincts were right and that Ali had Costello Syndrome. It was devastating. Actually devastating doesn’t describe it, it was worse. I literally wanted to die. My heart was broken, my life was forever changed and my baby would never be “normal”. I’ve had many people tell me since then that they totally understand how it felt but unless you’ve been through it, you absolutely can’t understand the depth of the pain that a parent feels when they get a life limiting, life altering diagnosis for their child.

On February 27th, 2010 I found out for the first time how it feels to have your child do something that you thought she may never do. Ali smiled at me. I felt like my heart was going to explode and learned that day how very sweet the milestones are when you’re not sure if they’re going to come! You have to savour it, you have scream from the roof tops what your baby did because you may not have many of those opportunities. Luckily for us, we did. First she smiled and then she rolled over, she learned to sit, she learned to pull up, she learned to walk. The fog started to lift and after some horrible, scary years Ali started to show us what a happy, social and smart little girl she is and she was meeting milestone after milestone. Now, don’t think that those milestones came easily. We’ve worked with Ali every single day of her life to help her meet those milestones. There’s been OT, PT, SLP, vision therapy and feeding therapy every day. Ali is such a brave little girl and she’s very determined. She met those milestones because she wanted to and because a whole team of people worked with her every day to help reach her goals.

Even with all the progress, there was always that one goal that we didn’t seem to be making progress on and that was eating. I started to think that I would just be thankful for all the progress and for how amazing she was and that if she never ate, I’d have to deal with it. I started to just accept that she may never eat and that was hard. I was so frustrated and I didn’t think that I could spend another day doing feeding therapy and blending tube feeds.  I wondered if maybe that was the milestone that she would never reach and then she did! I sat on the floor and I cried. I think Jason thought I was nuts as I sat there with an empty yogurt tube and sobbed like a baby. That was the big one. That was the thing that made life the most difficult and for the first time there was progress. Then it stopped and honestly I almost lost my mind. I was devastated and angry and I wanted to quit and actually I did for about a week. After about 3 months of not eating, Ali started again and 6 weeks later we were able to stop using the tube for food and only gave her water via tube. She literally left her therapists and doctors shaking their heads in disbelief, what an incredible amount of progress in 6 weeks. During that time she also got her words and now, she’s putting words together. Today she was angry because we needed to leave the house and Madi was hiding. She took me by the hand and scowled and led me to my room. She pointed to Madi and then crossed her arms and said, “Madi hide”. She’s a funny kid. While she’s been learning to talk, she’s also been learning to drink and again she blew everyone away with how fast she learned that skill.

So that brings me to today. Our appointment wasn’t so much an appointment but a good bye. Today I was told to stop using Ali’s G-tube. She’s ready to be 100% orally fed, they feel that she can get enough fluid and calories orally now and I agree. So tonight, I gave Ali all her medications orally and as I was giving her a flush through her tube, I explained to her that that was it, “No more Tubie”. We’re done. WE’RE DONE! So what now? The tube will still be in place for 3 or 4 more months in case Ali gets sick or can’t sustain her oral feeds. After that time, if we haven’t used it and we’re all ready it comes out. So here we are, at the end of one of the most difficult journeys of our lives. Sure, there’s still lots to work on. We need to teach Ali how to chew and we need to get her more comfortable with textures. Currently she only drinks from a straw so we need to teach cup drinking and we still have a long way to go with her speech. We still have the health issues, the orthopedic concerns and the developmental stuff but to me, the G-tube has always been the big battle. But not anymore. This is the biggest milestone yet and I am so proud of my incredible little girl.