Little Ladybug Hugs

It’s the most wonderful time of the year. Excitement for the upcoming holiday is building, people are feeling a wee bit more charitable, the air is crisp, and there are leaky children everywhere spreading the holiday germs.

I knew it was inevitable that our little ladybug would catch something this cold and flu season, especially since we’ve been attending birthday and Christmas festivities all month.  Sure enough last Monday in the middle of the night I woke up to what I can only describe as the sound of a little baby seal in the nursery.  Still half asleep I dug through the storage closet and grabbed the cool mist humidifier and the flowing vapour fan and prepared myself for a very long night.

Knowing that there isn’t much one can do medically for croup I took to the advice of seasoned moms and tried nearly every suggestion that was given to alleviate some of my daughters discomfort.   By Friday things weren’t improving so we made the trek to the doctors, and came home with a bronchodilator and the plan to continue on with what we had been doing before.

After a week of what seemed like endless nights, fevers spiking then breaking, and continuous cuddles, my little ladybug is beginning to sound less like a baby seal and more like her adorable happy self again.

I’m quite sure this is only the beginning of many more germ filled events to come.  After all, there are trips to the hospital for appointments, parties, play groups, oh and who could forget school. At least I won’t have to worry about the latter for a few more years.  CDG kids tend to be more susceptible to travelling germs and can have a very difficult time fighting them off.  However, in the end every sniffle and cough she has now will help build up her immune system for the future.  (at least that’s what I keep trying to remind myself.)

Wishing all of our friends and family who follow Little Ladybug Hugs a very happy and healthy holiday season, and a wonderful new year.

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http://www.cdgfamilynetwork.org

Continuing on with the milestone theme – our little ladybug just celebrated her 1st birthday.

Although there were some twists and turns along the way, every single day of this past year has been a blessing. It’s so hard to believe that my daughter is 1-year-old already, I can still remember the moment she came into this world with such vividness.

In true princess style there was never any doubt in my mind that we would throw her a fantastic 1st birthday bash – and that we did. There was a giant nutcracker, build-a-bears, a dress up centre and of course my personal favourite and I’m sure all the kids as well – The Land of Sweets candy buffet.  (Although the parents might disagree just a little) But hey, that’s what being a kid is all about – having fun!

Some might say (and have said) that it’s a bit overboard for a 1st birthday party, but why shouldn’t it be? After all we had no idea how things would turn out when I was pregnant and now here she is going strong against all odds and surprising everyone along the way.

I truly can’t think of a better reason to throw a BIG 1st BIRTHDAY BASH!

The day of the party was a wee bit of a blur, as the hostess it was my job to make sure that the children were all having a blast, and that their parents and all the other adults were  enjoying themselves as well. It really was a magical few hours. The hall was filled with the candy induced laughter from all the kids, happy chatter from the adults, and smiles were found on everyone. The most important thing is that our family and friends were all there to celebrate our ladybugs 1st birthday with us. It was their love and support that helped us through the twists and turns this past year, and I couldn’t think of any other people I would want there.

After months of planning, everything went off without a hitch. The day was absolutely perfect.

Ladybug has nearly been home for 1 month.  I won’t sugar coat it – the first couple weeks home were so very difficult.  I could think of a few other words to describe that time, but will be polite for all the virgin eyes out there. However, day by day she slowly started getting better and I was slowly starting to regain my sanity. Once I had decided to change her feeds to continuous over night things have soared. Sleeping through the night is now an everyday occurrence in our household – for the most part.  One thing I have learned in my almost 1 year of motherhood is that our children pick up on our emotions, when mummy is happy and relaxed so is baby.

The other day I was in town picking up a few household items with the munchkin, and a stranger came up and asked how old my baby was.  Not a huge shock – it often happens.  The woman proceeded to ask if she was crawling or beginning to walk yet.  I answered no and hoped the conversation would end there and I could continue on with my shopping. Then she told me that she has a niece that was 2 months younger than my princess and already crawling and nearly walking.  She was very sweet about it and it was obvious that she was just a very proud aunt, however I couldn’t help but be really irritated by the whole conversation.  I found myself feeling very protective of my daughter and even felt a hint of sadness that she hadn’t reached these milestones yet.

I had to remember what a very good friend once told me – our babies may be smaller than other babies but it’s a blessing, because we can do what the majority of other parents can’t – we can enjoy the precious innocent baby stage just a wee bit longer.

Later that week my little ladybug had just woken up from a nap and was smiling so big that both her top teeth were in clear view.  I decided to do the gum check just to see if there were any more.  Sure enough I felt a sharp little point on her bottom gum.  It was so exciting.  I immediately called my parents to let them know, then posted it on FB so my friends and extended family could share in my joy.  (Little side note – not everyone will be as excited as you over a tooth) Her third tooth had arrived – what made it more special was it was her 1st bottom tooth.  Even now as I’m recalling this I find myself filled with happiness.  It made me realize that deep down I really didn’t care if my daughter wasn’t crawling YET. She was still reaching milestones and each and every one of those milestones is just as important as the big ones that most other parents look forward to only to forget about in time.

In fact one of the most monumental milestones that I can recall thus far happened while my little princess was still in the hospital.  It was around 2 weeks post surgery. We were waiting for the port line to be changed over and the nurse gave us the okay to walk around the ward IV and heart monitor free.  That in it self was fantastic, but that’s just the beginning.  As we were walking I began bouncing and noticed that she was smiling so I began putting more umph and sounds into each bounce (I even got some crazy looks from other parents) just as I looked down at my happy little ladybug she giggled.  I stopped in the middle of the ward and started crying.  The nurses came rushing over to make sure we were okay and I had to try to explain that they were happy tears.  My little ladybug had giggled because of an action for the first time in her life.

One of the CDG moms had emailed this quote on the CDG Family Network to another mom who was having a difficult time – it was a turning point in the way I viewed my daughter, her health, and development. “Struggles only make triumphs that much sweeter.”

Whether it be eye contact, a new tooth, giggling, or the many milestones to come – each and every one will be monumental and I will continue to celebrate them all.

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I’m happy to announce that our little ladybug is now home.  It has been a very difficult few days adjusting to a different setting, especially since she was having horrible side effects from the iron.  Screaming and not sleeping for 3 days straight definitely took its toll on all of us. We have since put the iron meds on hold to regain a bit of sanity and find a new way to help rebuild all the hemoglobin that has been lost these past few months.

I know that I haven’t written as much as I did when I first started this blog.  Truth be told, we finally got a diagnosis that ties everything together and it has been extremely hard to take everything all in – let alone try to explain it to others without breaking down. When our little princess was born we knew there would be a special secret that put all of her little quirks into one itty bitty, cute, and adorable package. At the time I would wish we knew what was going on, although it is nice to finally have answers it doesn’t make it easier.

Anyone who has ever received a life changing diagnosis for the ones you love – whether it be a child, spouse, parent, or friend, know that as soon as the doctor tells you those words your entire world flips upside down in an instant.  Any hopes or dreams for them just seem to float up in the air and hover, because even though you may finally have a diagnosis there is no way of knowing how things will play out.

This has been my life for the past two weeks.

Our little ladybug was diagnosed with an extremely rare metabolic disorder known as CDG (Congenital Disorders of Glycosylation). It means that her body can’t transfer the sugars needed, which means that she is unable to absorb protein. CDG causes many different disorders that effect several systems in the body. There are approximately 500 cases of CDG worldwide, most of which are type I, only a very tiny handful have type II.  The latter is where my princess falls.  Although there are 500 cases of CDG worldwide there are less than 4 cases I’m aware of where the child has type II (although I’m sure there are more).  Type II has defects in the enzymes that remodel the sugar chains after they have been added to the proteins.

I’m still trying to learn about this disorder, I’ve even bought a biochemistry book to try to grasp the science behind CDG.

When we first saw the geneticist she had mentioned a carbohydrate disorder (CDG) that is rare and probably not the cause but they would test for it just in case.  I immediately went to the McMaster University’s library and tried to find as much information as I could about CDG. (There isn’t much) I did manage to find a FANTASTIC website for families of other children who have CDG.  The parents have the opportunity to share their stories, and join an email network that connects everyone.  This CDG Family Network http://www.cdgs.com has been a lifesaver for me.  Although we hadn’t even had a confirmed diagnosis, and CDG is rare, I felt in my heart that everything fit.  The families were there to answer any questions I had, but most importantly were there for me as a support system while my ladybug was in the hospital.  Every parent on the network had been through exactly what we were going through to one extent or another.

CDG symptoms vary from child to child.  To sit here and list all of the symptoms  of CDG would take forever, but for all of you to get a better understanding I will list the most common.  There are over 15 known sub-types of type I, and over 5 known sub-types of type II.

Some symptoms will become increasingly prominent as the child grows older.  The first 3 years in the child’s life can be the most serious and sadly in some cases become fatal.  Along my journey on the CDG Family Network I have talked with several parents whose  children have become precious little angels.  This was and still is very hard to deal with. I know that these beautiful angels are now watching over my sweet daughter and all the other children across the world that have CDG.

We are now in yet another stage of waiting to see if a sub-type can be found.  Both type II children that I’m aware of are still waiting and its going on 3 years. So we may never know, or she could have a whole new sub-type.

For a better understanding of CDG I encourage you to check out the CDG Family Network site at http://www.cdgs.com.

As of now there is no cure for CDG, it’s a matter of treating the symptoms as they begin to present themselves.  In our ladybugs case, putting in the g-tube to help with feeds, being followed by GI to keep an eye on her tummy, Neurology to follow the Hypsarrythmia and watch for seizures, Haematolgy to keep an eye on the coagulation of her blood.  It also means that Ibuprofen and Aspirin are out of the question because they could cause bleeding, Tylenol has to be given in strict moderation because it could affect the liver, and even the common cold could cause bigger problems.  HOWEVER, I will not become one of those mothers who is afraid of their child living their life for fear of getting sick.  I guess it just means I’ll be more aware of her surroundings, so no chicken pox parties, or group play with a bunch of runny nosed munchkins during RSV season.

As hard as it was to get this diagnosis, it will never change how much we love our sweet little princess.  She will always be perfect in our eyes.

I want to thank everyone who helped me get through the past few months. First and foremost my wonderful partner and baby daddy who keeps me calm and reminds me to breath when I get worked up over things, our families for being so supportive, my bestest friend in the whole wide world – poppet I love you and am so thankful to have you in my life.  My CDG family for answering any questions I might have and openly offering advice,  the mommies of ladybugs favourite roommates (C, H and A), Little A.S. for always making us smile. Finally all the wonderful, fantastic nurses at MAC who were always there to give hugs when I’d break down in tears or needed to vent – Heather, Jill, Tracy, Pam & Natalija (and so many more to list).

The unknown, yet exciting adventure of mommyhood in the CDG world is only just beginning. I know there will be bumps in the road along the way – it just makes all her milestones that much more precious.

http://www.cdgs.com

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I hope that all my Canadian friends and family had a wonderful Thanksgiving.  Autumn is by far my favourite time of year, especially going for long walks in the crisp air with the smell of freshly fallen leaves.  Hopefully my little ladybug will be out of the hospital before winter begins.  It’s looking promising.

Like I mentioned above my princess is still in the hospital, we are most definitely on the home stretch though.

Today a friend asked me how I can handle being at the hospital and away from home day after day, week after week, and now month after month.   Truth be told the hospital feels more like home right now than my own house.  The nurses have become our extended family and love my ladybug as much as any other family member would, in fact there are a few nurses who have gone above and beyond their duties and have become close confidants for me. The house I go home to some nights is just plaster walls and a roof. Once my daughter is discharged from the hospital it will become a home again.

Looking back to when she was first admitted we have come so far. A 10 cm portion of her small intestine filled with ulcers was removed, a milk protein allergy was found to be the cause of a lack of peristalsis, an EEG test confirmed a hypsarrythmia in the brain waves. Not to mention countless tests, and 5 blood transfusions in nearly as many days. Today my beautiful 10 month old princess is tolerating her feeds, beginning to gain weight again and is no longer in pain – aside from the *2nd* tooth that broke through today.  YAY!!!  I even went out last night to buy her first toothbrush.  Brought tears to my eyes – milestones like these are amazing but a teeny bit sad because my  little ladybug is starting to grow up.

Last week the wee princess had her 2nd EEG to see how well the Vigabatrin was helping the hypsarrythmia.  Yesterday, the Neurologist along with his entourage of mini doctors came in to discus the results.  The hypsarrythmia was still present on the EEG ( I had a feeling) however, I was not prepared for what he would tell me next.  It hasn’t decreased.  Which means that the Vigabatrin had failed.  Aside from a Steroid type injection (which has a list of side effects that could wreak havoc on a healthy persons system let alone a wee baby with medical issues), at this time there is nothing else they can do. For the first time I was speechless – what are you supposed to say at that point?  I knew when we started this medication it may not work and the other option was much worse.  Even still – I really felt like it would help. Now it’s a matter of waiting to see what long-term effect it could have, and in the mean time hitting the books and medical journals to see if there are any other possible treatments aside from the steroid injection or the diet.

At one time it was thought that my ladybug had a form of infantile spasms – this has been ruled out.  There is a type of special ketogenic diet that has been linked to helping children with seizures, and was suggested as a preventative measure. However at this point with finally finding a formula that LB can tolerate I’m not ready to start experimenting until her tummy is completely 100% back to normal.

Today….we almost had the MRI.  It was an incredibly anxiety filled day waiting and waiting.  Alas, it never happened.  As of now we have one booked for tomorrow at 12:30.  I’m not going to hold my breath, but at least we are getting closer.  The current pediatrician who is pregnant came in after the huge cluster mess of today and mentioned that if the MRI isn’t done by  tomorrow she is going to start spitting fire.  Anyone who has ever been pregnant or even around a pregnant woman when they are pissed off and on a mission knows that it won’t be pretty.

I’ve no idea when I’ll get the results from the MRI, but will update as things come along.

Happy Wednesday!

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It’s so hard to believe that we’ve been in the hospital two months now.  The days tend to mesh into one another – half the time I never even know what day it is.

I was really hoping to have my little ladybug home by thanksgiving, however to my surprise tomorrow is October 1.  (It’s CRAZY.  What happened to August?) Unless the teams can get their butts in gear and coordinate the MRI/Echocardiogram next week I don’t think we’ll be home.  I mentioned it again today to the wee intern, that it must be done before we get discharged or I’ll form a sit in and chain myself to the crib.  He looked at me like I was joking.  I guess he’s yet to experience such a vigilant mom in his young career.

My princess is nearly up to full feeds with the new formula Neocate, everyone myself included are still very precautious but she is tolerating it quite well. (knock on wood)

This past week has been rather difficult with tests, awaiting the results, and numerous meetings with numerous doctors.  I’ve decided to take a wee little blogging break  to regroup my thoughts, and do some research to prepare for my return after the CDN Thanksgiving. (unless there is a huge update then I’ll add another little note to keep everyone in the loop)

Thank you all again for all your support.  See you soon!   xoxo

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Along this journey there have been quite a few ups and downs and bumps in the road.  It has been a long road  and we’ve got a wee bit longer to go, but the sun is finally begining  to shine on the horizon.

The most repeatative bumps are feeds.  They go great for a day or two then have to be stopped because she isn’t tolerating them.

Like I’ve said many times a parent knows their child better than any doctor ever could.  So I had to laugh when one doc said there was nothing wrong with my princess and that she was just a fussy baby.  After getting over the initial shock of that comment I asked the doc to explain to me why every time the feeding pump sends more food through the g-tube into my daughters tummy, she cries in pain.  However within an hour after stopping feeds completely she goes back to being her happy content self.

Needless to say – they couldn’t.

Another GI test was done on Friday, this time they put the solution through the g-tube to follow the exact path her food has been taking.  Within 10 minutes the doctor monitoring the x-ray found the problem causing all my daughters discomfort – probably since she’s been born.

The Coles Notes verson: when food goes in the stomach it then goes through the duodenum and makes its way through the small intestine then large with a progressive wave of contraction and relaxation  until the contests are forced our of your system. The contraction and relaxation is called Peristalsis.  My little ladybugs tummy can’t do this so food just keeps building up in her tummy causing discomfort until it everutally and very slowly gets forced through.

Fussy baby my a**!

According to the GI specialist there are three causes for the absence of Peristalsis.
1. Caused by the surgery – ruled out
2. Part of NYDS (not yet diagnosed syndrome)
3. Sevre milk protein allergy

There are three treatments
1. Erythromycin (2 weeks on 3 weeks off)
2. A stronger med than the one she is on now for digestion (can’t recall the name – but could cause neurological side effects and was taken off the market 10 years ago.  However is apparently is still available in certain cases.
3. Neocate Formula for infants with sevre milk/soy/food protein allergies or intolerances.

Yesterday, for what I pray will be the only time, my princess started up on feeds again.  This time at a very low rate and with the special formula.

I truly hope that the cause is only (used loosely) a milk protein allergy and the new formula helps everything get back on track.

One day at a time.

Milstone Celebration:  My little ladybug got her first tooth (on the top) on September 8/10 and the second looks like it will be here in the next few days.  I love milestones!

I wanted to thank everyone – family, friends, friends of friends, and especially the families that know exactly what I’m going through, for being so supportive through out everything.  This blog has become my release and my way of connecting with everyone on a whole new level.  I’m very grateful for all the  on-going support.   Thank you.   x0x0

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While I was in Thailand a few years back the most popular phrase on t-shirts and souveniers was Same Same, But Different. That saying pretty much sums up this past week.

I’m happy to say that my little ladybug is finally tolerating full feeds through the G-Tube, it’s been a bit of a battle but everything seems to be on the up and up feeding wise.

The meds for her hypsarrythmia began late last week. They started off at a very low dose, every two days the dose was doubled with the hope that she would tolerate the maintenance amount. Well, in true princess style things couldn’t just run smoothly. This past weekend she was up to half strength and it was taking a huge toll on her body. All she wanted to do was sleep, and sleep, and sleep. I knew some sedation was to be expected but nothing could prepare me for watching my daughter lapse into such a deep sleep that she would begin to hold her breath. I guess they forgot the apnea section in the side effects hand-out. I thought I could handle everything thrown at me thus far – but when the nurse was trying to wake up my daughter and the only reaction was a little eyelash flicker, I couldn’t prevent my mind from going on a tangent of what could possibly be wrong. Did she have another adverse side effect to the Vigabtrin and was now in some sort of coma. Why isn’t she waking up? The only thing that kept me grounded was that her vitals were fairly stable again. Eventually – 4 hours later she began to wake up. The doctors decided to hold the dosage increase for a day just to monitor her. By Sunday morning she was somewhat like herself again, although she had a constant glaze over her eyes. She has since been on the full dose, isn’t as sleepy and hasn’t had another episode of apnea.

A little side note: I just spoke with my daughters nurse and for around 1 hour this evening she was cooing, looking around, and quite happy and content. I couldn’t have asked for any sweeter dreams tonight.

It has certainly been a busy couple of weeks since my last post.  Early this afternoon my little princess was moved from the paediatric ICU back to the children’s ward.  Such a welcome change.  Although it was very reassuring to know that a nurse is just outside the door in the PICU it was a good feeling to know that my ladybug is finally well enough to be transferred back to the ward.

Since the surgery the next item on the agenda was to change over the G-Tube feeds from Pedialyte to the actual formula. The transition went smoothly for the first couple days I even came home Friday a wee bit early to get a good nights sleep. Then when I came in the next morning the acid reflux had returned with a vengeance – screaming, arching, vomiting.  As bad as it sounds coming from a mother who loves my daughter dearly it was quite comparable to the scene in the exorcist.  All kidding aside it meant that the formula would have to stop and we would return back to the pedialyte. Two more days passed and once again things seemed to settle. Today the  non-dairy, non-soy formula Nutramagen (which by the way smells just as horrific going in as coming out) was started again.  Aside from a lot of discomfort, and arching it is being tolerated.  One day at a time.

A request has been put in to finally get an MRI done while we are in the hospital.  Especially since the clerk cancelled the out-patient one we had been waiting over 7 months for. (Once again props to the “fantastic” Ontario medical system) It’s just a matter of getting the OR time and an anaesthesiologist to coincide with Neurology for the MRI  because she will have to be put under a general anaesthetic.  Oh and since she will already be sedated cardiology wants in on the festivities to do an echocardiogram.   Seeing as every team who is looking after my daughter wants this done I’m happy we are finally seeing some progress.

I guess when one spends an extended time in a children’s hospital one is likely to be recognized by many nurses – well my sweet little princess was getting a reputation for being quite the diva on the ward. In fact, when the night nurse came on she already heard how adorable my daughter and that as soon as she wet or soiled her diaper she demanded a new one be put on that instant. (I’m with the ladybug on this one I wouldn’t want to sit in a soiled diaper either) Don’t even get me started on if the soother comes out – she will be all sweet, cooing smiling and then if no one helps then the adorable little diva appears.  I’ll post some pictures down the road but she has great big eyes and long eyelashes and even for the nurses it’s hard to refuse a cuddle only fueling their diva nick-name.

My hope is to be out by end of the month – with any luck the 29th because that’s when my most recent parking pass expires and I would really hate to have to keep giving the company that owns the parking more money.  

Once the feeds are back to normal my daughter will be able to start her Vegabatrin for the Hypsarrhythmia (mild form of infantile spasms) and if those meds are tolerated we will finally be homeward bound.

4 days post surgery. My little princess is resting comfortably for the first time in weeks.

The surgery went great. It was a little over four hours but they managed to put in a G-tube, a Port and did a laparotomy. (a surgical procedure involving an incision through the abdominal wall to gain access into the abdominal cavity) During the laparotomy they found a portion of my daughters small intestine that was inflamed and looked abnormal. After consulting the GI team the decision was made to sent it to pathology so they did a 15cm resection and away it went.

Shortly after we returned to the ward the wonderful Dr. B. (surgeon) came in with a big smile and told us that the entire portion of small intestine removed was an ulcer. An ulcer that was most likely caused from stress. Crazy isn’t it – a 9 month old baby getting an ulcer due to stress. I guess a million and one x-rays, ultrasounds, poking and poking and poking for any type of vein for an IV or to get blood. Would be more than stressful to a grown adult let alone an infant.

It was a huge relief that they found and removed the cause for the bleed. No more transfusions. At least I thought.

The morning after her surgery was a nightmare. My little princess just had a major surgery for an adult never mind for a wee 9 month old. Initially I was extremely irate and was all set to go into great detail of all the mis-haps that came about that day; however after having some time to cool down I’ll just give a brief synopsis. Somehow the orders were “mis-read” “misinterpreted” “missed” (I know what happened but out of respect for the nurses that are fabulous I’ll just leave it at that.) In any case my daughter hadn’t had ANY type of pain medication for well over 5 hours. She grew very irritable which was inevitable and became tachycardic(high heart rate). Eventually everything got cleared up and she was given a morphine infusion. Once that little crisis cleared up yet another one arose. She had started bleeding through her NG suction. Dr B. just happened to be checking up on our little ladybug and noticed that someone had turned her suction to over 100 when it’s supposed to be 40. Sooooo my little princess ended loosing a fair amount of blood was moved to the PICU and needed a 5th and what I’m hoping will be final transfusion. She is being taken care of extremely well there.

Now that she is on her regularly scheduled pain meds I am finally starting to see signs of my daughter again. She is beginning to coo again and even smile. It could just be that she is stoned off the morphine – but I’ll take it. 🙂

My little princess has started a small amount of Pedialyte through the G-tube, and will eventually start on formula.

All in all our little ladybug is beginning to recover, and hopefully we will be home in a few weeks.