Chloe is 1.

Beautiful Pulmonary Arteries

Isn’t it great, when on your birthday your very first compliment is ‘beautiful pulmonary arteries’? Yesterday morning we had to take Chloe for her 3 monthly cardiac checkup. Her cardiologist is very happy with Chloe’s progress and general health.

When she goes for a checkup her OBs are done. Weight, blood pressure, ECG and her SATs. Sometimes I feel that the nurse who works with our Cardiologist is a bit disorganised. Lucky I have my own SATs monitor due to the fact that she forgot to bring the cable that connects the SATs monitor up. I just roll my eyes. I get a lot of criticism over having my own SATs monitor, but I was able to tell them what her SATs generally sit at. Also, the nurse couldn’t be bothered checking her weight and asked me if I had done it recently. They asked me what her SATs were the last time I checked, but I made her doctor guess…He said he though about 85, I said ‘ooh very close!’ They were 84 last week. ‘

I find this quite frustrating at times, as early on it was all about ‘what are her SATs?’ and how her weight was going. Sometimes it seems like this nurse doesn’t care. In the past she has downplayed many of my concerns with aspects of Chloe’s health so I actually don’t like her much to be honest. However, I think her cardiologist is excellent and I trust him completely. He is also quite knowledgeable about SLO and has treated 2 other children in his career with it. It might not seem like a lot, but here in Queensland that is a lot, it’s not a common syndrome.

Anyway, Chloe had her usual Echo and he is extremely happy with her heart function. He would have expected her Pulmonary Artery to be more compromised but he said it was ‘beautiful’. He always shows me the blood flow on the screen and I find it quite amazing seeing how the blood now flows from the Left ventricle directly into the artery and the right side no longer pools blood. Best way I can explain it.

Then he says see you in 6 months! SIX months! Woo hoo! Great news.

The only ongoing problem we have and will most likely continue to have is Chloe sleep disorder. I have to admit I am getting very tired of the comments about sleep I get… especially the one that goes a bit like this…’welcome to parenthood! You won’t ever sleep again!’

I could take this comment if it came from another parent of a special needs child, but funnily enough, not one parent of a special needs child has ever said this to me. Now I don’t have other children, Chloe is our one and only, but I will guarantee that although many healthy children go through terrible sleep patterns, or struggle with sleep, it is NOT the same thing. Sleep deprivation sucks, but add to that the sleep deprivation that is on top of medical conditions, and knowing that although it might get better, it will most likely always be difficult, is not the same thing as the sleep deprivation that comes with ‘normal’ baby stuff.

Chloe has always been a bad sleeper. Whether it’s been due to the need for feeding or comfort after surgery, she has never really slept through the night. But the one thing that has always been predictable has been Chloe’s bedtime. Since she was post her first surgery, I have always been able to give her a bath, read her a story, give her a bottle and she’s gone down to sleep. That’s all changed recently!

Smith Lemli Optiz syndrome is a disorder that is related to how the metabolism works. It is a spectrum disorder, so you never really know when they are this young how the syndrome will manifest itself in the coming years. Many, if not ALL children who are diagnosed with SLO struggle with sleep. I’ve read several papers and research about how these children struggle with sleep disorders. One paper I read stated that in the study (keeping in mind that the study is based on a small group due the fact that it’s not common) over 75% of the children struggled with sleep. Some of the issues that present are:

• struggling to fall asleep
• struggling to maintain sleep
• Sleeplessness
• Not needing the same amount of sleep as healthy children
• Excessive sleeplessness during the night and excessive sleepiness during the day
• unsettled wakefulness.

Chloe definitely falls into these categories. Recently she started struggling to fall asleep at night time. She hasn’t been particularly sick, or needy and I believe I have done all the ‘correct’ things the experts say in order to develop good sleep patterns for your baby. But Chloe doesn’t march to the drum of the normal stuff.

She won’t cry it out, she’ll just cry hysterically, for hours. Without stopping. She doesn’t want to be rocked to sleep, or shhhhed…or patted or tapped. She doesn’t want the light on, or white noise or lullaby music. She just wants to get out of bed! Even though she is tired.

A typical night in the last month or so for us has looked a bit like this.

Bath and bedtime routine starts at 6ish. She is usually in bed by 7pm. Cue the crying. Mike or I have attempted for more than hour, sometimes 2 or 3 to get her to settle to sleep, but have given up and put her in the swing we have, which calms her and she watches us eat dinner! Then we try again. She is usually asleep by 11pm. She might get 2-3 hours sleep, but will often wake by 1 or 2am crying. Hysterically. And remember at this point she has a heart condition, so her heart will race the more hysterical she gets, and she will start to turn blue and gasp. Sometimes she doesn’t go back to sleep until  4 or 5am. Then she might sleep til 630 or 7 but will be awake and ready to party! Some nights I haven’t even managed to get to bed by 4am. Sometimes I sleep on the floor in her room as my presence seems to help.

This is very common amongst SLO parents. We are lucky that we have a great paediatrician who helps when I email him and we have started trying melatonin in the last fortnight. If you don’t know what melatonin is, it is simply the hormone that we release in order to help our natural circadian rhythms to get ready for sleep. We dose Chloe at 0.02mls! It’s not much! It has to sit under the tongue for about 30 seconds (which can be interesting) as it is better absorbed this way than digested. Some nights are hit and miss, but we do believe it is helping. She has slept through from about 10pm til 5 or 6am about 4 nights since we started.

So the sleep things continues to be a work in progress. Sometimes I get frustrated, and desperately want sleep, but mostly, I just want to see Chloe be settled and happy. I do get frustrated at the criticism Mike and I get about not attending things, especially church or Bible study. Sometimes getting out and about when you’ve had 2 hours sleep is not easy, or sometimes events fall into the ‘nap time’ category. And missing one nap, when your child doesn’t sleep just doesn’t work! I also get sick of the constant comments by strangers…. ‘oh she looks tired!’ ARGH! I’m contemplating getting a shirt made…or perhaps firing back a comment about the stranger looking tired or unwell.

I’m not writing this as a cry for help, but wanting to have people be aware that sleep is a huge issue for children who have SLO. It’s a huge issue that cannot be modified through behaviour or sleep school (though we might try it even if it gives me sleep while the nurses care for Chloe!) However, I have found one solution that seems to help Chloe settle. Cloudbabies! Cloudbabies is a BBC show, a cartoon for little kids. We have the DVD playing on loop now in her bedroom. It’s like she needs them for comfort! She’ll wake, let out a cry, realise it’s playing and watch for a bit, then fall back to sleep. It might not be a long term solution but it works for now!

My top ten…from both sides.

Wow… I cannot believe how time can fly and yet also seem to drag at the same time. There must be a word for that? 

This time last year Mike and I were at hospital and the process of an induction to bring Chloe earthside had started. I of course was very anxious. About a multitude of things, and I’m sure Mike will tell you he was too.

The afternoon before heading to hospital Mike and I relaxed, set up the Christmas tree and packed. Okay, let me rephrase that, I packed, Mike procrastinated. And when it was time to leave, he still wasn’t ready, but I had been ready for ages! 

Anyway, that night in hospital I was told to rest and sleep. I had a cup of tea at around 8pm and hoped and prayed that Chloe would surprise us all and come overnight! I spent most of the night worrying and listening to Mike’s snoring! (How do men do that? Just sleep and not worry!)

Alas, nothing happened overnight and by the morning I was sent down to the birthing suite to get things moving. I’ll leave that story there and fast forward to her finally being here. She was delivered by c section after 5pm in the afternoon, I asked over and over, ‘Is she okay? Is she okay?’ I needn’t have bothered, she showed the world right then that her lungs were strong and working properly! (Hasn’t stopped since!)

Since that point (well probably before that) Chloe has turned our lives upside down. Like most babies do! It’s been one busy, stressful and hectic year.

The best and probably the worst year of my life. It sounds crazy to say it, but I think most new mums would agree, adjusting to motherhood is a pretty daunting task, and there are days you’d like to just crawl under the doona and go back to sleep for a week! On top of the regular new mum stuff (though to be honest I really have no understanding of ‘regular’ mum stuff in that sense), we had the extra worries of Chloe’s medical condition. The constant fear of the unknown always hanging around like a massive rain cloud, just teasing us to pour.

I’m not sure it ever did. We have been so unbelievably ‘lucky’ with Chloe. Throughout this last year I have listened and followed stories of other kiddies who went to be with the angels, or who had major complications and long hospital stays, but Chloe, not her. She showed everyone that she’s boss and she’ll do things when she’s good and ready! I have no idea where this stubborn streak comes from.

Over this year I’ve done many things I never thought I would do. One that tops the list is have a baby! Those who know me know what a surprise that one was! 

I’ve spent the last few days, as the 26 November approached, contemplating some of the milestones that have happened this year. 

1. Chloe was born, without drama. No medical intervention was needed, she breathed on her own. 

2. Chloe breastfed like a champion, even though they thought she might burn too many calories feeding.

3. Chloe went into heart failure at Christmas 2012.

4. Chloe was admitted to hospital on the 28 December with failure to thrive/CHF (Congestive Heart Failure). Her first ng tube was inserted.

5.  Chloe had her first heart surgery on the 31st December 2012 (we spent NYE in PICU). On the 1st January she had a few hiccups, but otherwise sailed through and the PA band did exactly what doctors hoped it would.

6.  Two weeks in hospital, we headed home.

7. Chloe was diagnosed with Smith Lemli Optiz Syndrome.

8.  Chloe developed TET spells. 

9. Chloe had her first Cardiac Cath, but bombed out after anaesthetic and was admitted to PICU. 

10. May 16 2013, Chloe had her Glenn surgery. We were home in a week.

There are other countless blood tests, doctors appointments, a few emergency trips to hospital, a couple of admissions that weren’t planned, one ambulance visit…too many echocardiograms to count, or x rays, feeding difficulties… therapy appointments… medications… honestly it’s not worth counting. I don’t want to dwell on these things. And while I am constantly amazed by how far she has come, and I don’t want to forget the obstacles, as they are an important part of her journey and who we are as a family, I like to focus on the amazing milestones…the ones that most parents look forward to! And celebrate.

And one reason I love these milestones is because the doctors are constantly surprised by how much she can do because she is doing far more than expected. 

1. Chloe smiles. She started at about 6 weeks old and hasn’t stopped bar the usual grumpiness or pain from surgery. Pull out a camera, she poses… I love her smile.

2. She holds her head up…rolls over. I was beyond excited when she did this! When she did this in front of the metabolic specialist he was genuinely suprised.

3. She talks… non stop! 

4.  She sees…she follows people. She’s alert and wants to know everything going on!

5. She got teeth! Two bottom teeth!

6.  She crawls. Fast! 

7.  She eats! Months of hard work…. and she eats! (This is a huge one!)

8.  Chloe sits up, gets down from sitting up and is starting to try and pull herself up. 

9.  She’s starting to sign. I’m certain she is trying to do ‘more’ and ‘done’.

10. She’s 1! 

So tomorrow is her first birthday. And like all parents, we celebrate the year that was. It’s been full on. I’ve become adept at many things I never thought I would be. I am a mum. I am a mum to one very special little princess. I cannot wait to see her eyes light up and smile at all the new things she is yet to experience. 

And tomorrow, it’s only fitting that she has a cardiac appointment just to see her birthday in, much in the same way she had an echocardiogram at only an hour old, she’ll have another on her first birthday! I only hope this time she stays on the table long enough to get a good picture of my little heart baby…who is no longer my heart baby but my ‘heart’ toddler….I suspect she’ll be either be itching to ‘help’ with the ultrasound, or pull the ECG cables off along with the SATs monitor… good luck to the doctor and nurse tomorrow! I might bring them a cupcake to wish them luck!