World CDG Day

16 May

I can’t believe how fast one year has gone by.  I remember writing about the very first CDG Day last year, a few things have changed some good – some not so good. The one constant is that my little Ladybug is one fierce little girl.

Since Ladybugs two emergency surgeries and summer hospital stay, she has struggled to stay healthy. Each cold and flu that she caught seemed to take longer for her to recover from.  She has missed the majority of the school term. The smiles and happy jibber jabbers have been few and far between, but are slowly returning. Though she be but little, she is fierce. I keep reminding myself of this when I get a little sad about how fragile she is.  She will get better, and I’m hopeful her loud chatter will return soon. Until then, Ladybugs little brothers more than make up for it.

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This photo was taken on Mother’s Day (May 2018). 

CDG has been a massive rollercoaster in our lives, Ladybugs fragile little body is fighting and we will fight for her. It is a disorder to which there is no cure, and that is heartbreaking. I have to say though – it isn’t all bad. If it wasn’t for Congenital Disorders of Glycosylation, I would never have become a part of the most amazing tribe. I’ve grown so incredibly close to families that I’ve never met in person, we chat online, on the phone or FaceTime. Usually to ask questions about treatment options, questions about different ailments, etc., but it’s also just to talk to another mama who gets how rare and frustrating CDG can be. There is one mama in particular – she gets it! When we FaceTime I feel like I’m talking to a close relative or friend that I’ve known my entire life. It helps that our daughters have the same subtype too.

Last summer we were planning on heading to the USA for a family road trip with our Airstream “Juniper”, to see Mt. Rushmore and Yellowstone National Park. (Due to Ladybugs hospitalization we weren’t able to go) We were also going to meet Ladybugs CDG SLC35A2 sister. This sweet little American love bug is the 2nd girl in the world (Ladybug being the 1st) to be diagnosed with the same CDG subtype that my princess has. I’m so unbelievably excited that we will now be making that trek this summer. On the bright side of last summer, I had a lot of spare time while in the hospital, so all of the packing lists and must see tourist attractions like the Worlds Largest Ball of Twine list are already complete. Like last year I will be documenting our travels on a separate blog which I’ll post when I have it all up and running.

It’s PURPLE DAY!!!!

26 Mar

Purple Day 2013

Today, March 26th marks the international day for Epilepsy Awareness. My very first post about today was back in 2013. I always find it interesting to look back on my previous posts and see how far we have or haven’t come and what has changed.

The biggest change is that we’ve discovered Ladybug is seizure medication resistant. We have tried all medications that are safe for her to take and none have had any positive effects on her seizure activity, especially since she’s been diagnosed with Lennox Gastaut Syndrome. The other thing that made me chuckle, but in a bit of sad way, was how I was so excited that Ladybug was a whopping 20 pounds. 5 years later she is now only 23 pounds (10.5 kg). Although, she is taller so that is a big win – she has grown from 71cm to 84.5 cm (28″ to 33″). That is 1″ a year.

In 2015 we talked about starting her on a Ketogenic blenderized diet. This never came to fruition. Given Ladybugs reflux and tummy issues, in order for us to get her on a keto diet she would need a GJ feeding tube. This bypasses her tummy and goes strait into her intestines. It would also mean continuous feeds and would impact her school time, and could still potentially cause an increase in reflux. The cons far outweighed the pros at the time so we opted to put that idea on hold.

After quite a bit of research we were able to get Ladybugs neurologist on board with trying CBD oil. Although it was very helpful in her overall health and wellbeing, it had no impact on her eeg results and seizures.

So here we are March 26, 2018, a lot has changed in 5 years. I’m still very hopeful that there will be some kind of treatment we will come across that will help with Ladybugs seizures. Perhaps now more than ever as the type of seizures she is having is slowly increasing. Last week Ladybug had her first absence seizure. They should typically only last a few seconds but her lasted over 2 mins, then this morning what started as a typical auditory triggered myoclonic seizure turned into an almost clonic seizure where her arm started shaking. Thank heavens she returned to baseline quickly afterwards. I don’t know what we will try next treatment wise – maybe a different strength of CBD oil, or perhaps there is a new pharmaceutical that might show positive changes in kids with LGS. I do know that I will NEVER stop trying to find treatments to help my little bug live the best life she possibly can.

Here is the link to the 2013 post that gives more of a background on Purple Day.

 

It’s New Ear Tubes Day!

5 Feb

Hooray – it’s new ear tubes day!!!! Finally after far too long of a wait, Ladybug is getting new ear tubes put in as I write this. We’ve been incredibly fortunate to keep her healthy and germ free beforehand so we didn’t have to cancel the procedure. I was really worried especially given the new Pre-Op set up Hamilton Health Sciences has. I must admit I really don’t like it. You walk into a very large space, one side is an urgent care clinic that was filled with people coughing, sneezing, and a child threw up twice while we were waiting on our side. The two sides are split by little glass dividers. Every time someone on the other side sneezed or coughed I just envisioned all those germs floating over to our side of the waiting room. Whoever decided that it was a good idea to share a waiting area for people needing surgery (many of whom are medically fragile), with phlegmy, snot filled, puking people, needs to definitely reevaluate things. Don’t even get me started on the 3 1/2 hour wait to see a nurse and anesthesiologist. There has GOT to be an easier way for kiddos like Ladybug to go through pre-op. Lets just say a trip to Starbucks after was definitely needed by the time we got out of that place.

Well that’s my little rant for now. All things considered, we are incredibly fortunate to have such a great team though. On a super awesome bright note, our favourite Mac Kids PSW Liz stopped in to see us while we were waiting for Ladybug to go in. It was such a great little visit, and just in her magical Liz way, she immediately had Ladybugs attention just by the sound of her voice.

I’ll do a quick update on the Little Ladybug Hugs Facebook group once my little princess is out of recovery.

The picture is one I was able to capture of Ladybug relaxing and enjoying a snuggle with mommy. It didn’t last long as she was quite unhappy that she wasn’t getting water when she signed for it.

Picture Description:

Ladybug, a teeny tiny 8 year old girl is snuggling in her mommy’s arms. Mommy is wearing a beige cardigan and Ladybug has her hair braided and is wearing a white kids hospital gown with a green ribbon by the neckline and puppy dogs, bees, and butterflies on it. She has her eyes slightly open and her left hand partially open and resting on mommy’s chest.

Finding Our Groove

19 Sep

This update is a little late but it has honestly been very hectic these past couple weeks.

Ladybug was discharged just before school started, although I kept her home for a week to get used to being out of a hospital setting. A couple days after she was discharged there was a critical family emergency and our 3 year old niece came to stay with us.   Within a day the teacher in me kicked in and all 4 kids were on a set routine and things ran like clockwork. Except for laundry and keeping things tidy. Truth be told there were days the place looked like we took all the books and toys and just threw them in the air and left them wherever they’d land. The organizational freak who had a perfect place for everything in our hospital room had to learn to let it go, and have tidy up parties at the end of the day or every other day.

The entire summer seems like a dream now. 2 months of absolutely no sleep, endless IV alarms, 07:00 doctor rounds – running off of that primal instinct to always be there to advocate for my daughter because if I don’t no one else will. Despite all of the not so wonderful things that happened, I’m focusing on all the good things. The good things certainly come out on top – I’ll write more and share some pictures of our summer soon.

I’m eternally grateful to our family and friends who stepped up to help us out with the boys, checked in via text or messenger to see how Ladybug and I were doing, dropped off snacks and uplifting books, or just popped by to give me a great big hug. It gets very isolating at times, so each and every one of these were greatly appreciated.

Ladybug had a follow up appointment with our fantastic surgeon yesterday and everything looks great. We have an unrelated test at Sick Kids Hospital in Toronto this Friday – Ladybug will have to be sedated but we should be able to go home that same day.

Here is a picture of her on her first day of school.

Slow and Steady

17 Aug

Things are slowly getting better here – Ladybug is healing nicely from her Surgery a week ago, the incisions look impeccable, and according to the last contrast study there are no more obstructions. She does have very slow moving intestines (little to no peristalsis), so the process of getting her back on feeds will be long. The goal before she can start Pedialyte through the g-tube is for Ladybug to tolerate having her tube clamped for 24hrs.  This tests whether everything is able to move through nicely or not. Each day we add another 2 hours to the time. Today we will try 12 hrs and I’m sure she will do great. Providing everything continues to go well, Ladybug should be back on formula and out of the hospital in a couple weeks. 

Slow and steady wins the race – if this is the case Ladybug is going to smash that racing record. 

This morning we ventured to the lounge for the Wordworking with Home Depot activity. We worked together and built a fire truck to give to the 2 little monkeys at home. 

A Very Long 4 Hours

5 Aug

Ladybug was brought in to the OR for her surgery at 08:45 this morning. By 10:45 I was hoping for someone to come get me – but no one did. 11:45 – still no word. Finally at 12:45 the surgeon came out and said that the surgery went well.  They had found a stricture which is essentially a narrowing of the intestines. Because of this narrowing it was nearly impossible for food to get through which caused the 2nd obstruction.

The stricture was resected, and the small bowel(intestine) was stitched back together. She is now rocking a pretty large scar across her tummy that is held together with a topical skin adhesive known as Dermabond. This was new to me as her two previous surgeries she had steri-strips. I guess it is used more often in the USA as opposed to Canada – as it is rather pricey. (Her Surgeon was trained here at McMaster and then went to work at St. Jude’s Children’s Hospital in Memphis TN. She is now, thankfully, back at Mac Kids).

Her hemoglobin levels were low before the surgery so Ladybug was given a blood transfusion to help get her back to her normal, she was also given an epidural for pain management. This will stay in for a few days.

Around next Thursday they will do another contrast test to make sure that the resection site has healed nicely and there is no leakage. If all looks good they will very slowly start her on a continuous feed of Pedialyte then move on from there.

She is now back in our room and resting nicely, hopefully I will be able to do the same tonight.

 

Though She Be But Little, She Is Fierce!

3 Aug

This morning, Ladybugs surgery team had a big meeting with all of the surgeons from the children’s hospital, and 2 from out of town. They went over her tummy x-rays, ultrasounds, and the CAT scan. They were all of the same opinion that there is another obstruction – this time it is further up in the bowel (Upper Left Quadrant).

She will be going in for another surgery tomorrow (Friday) sometime. It is pretty unheard of to have a second obstruction after just having one fixed so there will be a couple surgeons on call to give some fresh eyes if need be.

You would never know that she is unwell to look at her. In fact, she has become the biggest little diva I’ve ever seen. She could be fussy one minute, but as soon as a nurse or doctor walks in she turns on the charm and starts smiling, chatting, and staring up at them with those gorgeous big eyes. Ladybug has definitely won the hearts of everyone here.

I’m thinking I need to make a sign for her hospital crib in big sparkly letters saying – ‘Though she be but little, she is fierce!’. This little princess has been through and is still going through so much – but she is a fierce little diva and will get through this too.

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