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!