Maths Headaches

Gremlin's levels are up again (426) and GOSH want us to start introducing protein now. Not on 12th May as I've been expecting but now. Today. Gulp!

Michelle, the dietician, explains everything over the phone whilst I frantically scribble and try to understand. 1g of protein is equal to 1 exchange. Gremlin will have 6 exchanges per day. This will eventually replace all her regular formula bottles and enable her to have exactly the right amount of phenylalanine that she can manage.

To start we will introduce 2 exchanges during her lunch and drop her 3pm regular formula feed.

For homemade scran 1 exchange = 80g plain mashed potato, 55g avocado, 25g peas or 25g spinach.

For jars and the like = amount of protein and exchanges must be calculated according to protein in grams per 100g and the size of the jar. For example- pouch thingy contains 4.0g of protein per 100g. 100/4=25 so 25g of pouch thingy = 1 exchange, 50g = 2 exchanges etc. Pouch thingy is 125g so 50g must be weighed out precisely by digital scales.

We have already changed plans once today (for fear of passing on lurgy to heavily pregnant friend and her son) to meet Grandmamin at a cafe for lunch (we don't mind passing on lurgy to Grandmamin!). I decide to bite the bullet and go for it at lunch. I put our digital scales in the change bag, along with all the usual protein free lunch paraphenalia, and decide to see what we can find in the cafe to provide the 2 exchanges.

Once there I find an Ella's Kitchen "Chicken Something or Other" pouch which has the protein as broken down above. Gremlin has her PKU Gel first then I measure out 50g of the pouch on my scales. I can feel people looking but try to act like we are doing nothing at all unusual. The trouble comes when Gremlin appears to actively dislike the "Chicken Something or Other" and whatever I spoon in immediately comes back out again down her chin and bib. Michelle did not tell me what to do in this situation! I panic a bit (Do I find something else protein free and do the 3pm regular formula feed? But she's had some, how much has she had? Can I weigh it again? Then how much milk should I give? Aaaaah?), but persevere and eventually get Gremlin to eat all 50g. The contrast when I offer her a protein free strawberry and apple pouch for her pudding is incredible- snaffled in about 2 minutes flat- she definitely has a sweet tooth.

On the way home we pop into Tesco to buy potatoes and avocado but also to look at the other jars/pouches and that's when the headaches begin. Each jar is a different weight, and each jar has a different protein per 100g content - 3.6g, 1.8g, 4.2g, 0.6g etc etc. I'm going to be doing an awful lot of maths and carrying those digital scales everywhere .................

Parsnips Schmarsnips

I don't think Gremlin likes vegetables much. Quite ironic as her mother is a vegetarian. She enjoyed trying to pick up the parsnip but her pursed lips as it approached her mouth suggest she is not terribly keen on the taste.

In stark contrast she REALLY liked the look of the iced fingers that me, Moomin and Grandmamin ate this afternoon! I have no idea how many exchanges they are but think I will need to find out......

Oh la la

Oooo oo oo oo! Another new pouch to try! 'Blueberry, Banana and Vanilla' has 0.5g of protein per 100g and is made by Plum. This makes me happy as Moomin ate a lot of Plum ones too. Gremlin is pleasantly surprised (it really is lovely- Moomin and I try it too) and it is snaffled very quickly.


Job Done.

Party Party Party!

Today has been a bit special. For a start, it's my birthday! And second, we went to a brilliant party. Not for me, but for PKU families. We met (some for the second time) a 3 year old girl, an 18month-ish year old boy and an 8 month old girl with PKU and all their siblings and parents. It was great and I hope that they will become friends for us all, especially Gremlin when she is older.

All the children were fantastic and it was so reassuring to see that. It would have been impossible to tell who had it and who did not.

I also learnt a lot more about exchanges. 1g of protein is 1 exchange and all the children were on different amounts based on their own blood results - 10, 6.5 and 6. We don't know yet how many Gremlin will have but her dietician hinted it will be around 5. Quavers are half an exchange, Hula Hoops 1 exchange and Pom Bears about 2/3rds! That's snacks sorted anyway!

Gremlin enjoys a touch of al fresco dining

I run out of time and fresh fruit so don't get chance to prepare Gremlin any chunks of food today, but let her have her first meal outside, thanks to the arrival of our looooooooong anticipated garden furniture and our good friends round for lunch.

Also GOSH phone and tell me Gremlin's phe levels have dropped from 570 to a perfect 271.

Today has been a lovely, happy, sunny day.

Banana(wo)man

Success. Lots of gagging but a very happy Gremlin and I'm pleased to have found a way to make eating a bit more fun for her at the moment.



See www.babyledweaning.com for more advice on this sort of eating.

Gremlin weaning cannot be fully baby-led but a bit here and there goes a long way!

Hey Mango Mango Italiano......

Gremlin is 27 weeks now and a bit bored. We are still sticking to our short vegetable and fruit list:

Banana, Mango, Apple, Pear, Peach
and
Green Beans, Swede, Sweet Potato, Parsnip, Carrot

as well as the prescription rusk, porridge and various jars/pouches of mainly fruit.

In a bid to liven things up a bit I cut up a large chunk of very ripe mango and let her play with it. She picks it up and starts sucking. None of the mango breaks off or is eaten but she has enjoyed a more participatory lunch and I will see if I can do similar with something else from our list tomorrow.

25 weeks - The first bottle starts to drop too

I am thrilled that Gremlin is really enjoying eating. She is now eating 3 meals a day. A typical menu would be:

Breakfast- 'Hot Breakfast' porridge with mushed banana
Lunch- Aminex Rusk with warmed PKU Start milk followed by half a jar of fruit puree
Dinner - 4/5 ice cubes worth of pureed vegetable then remainder of fruit puree jar

In addition she is still having 4 sets of bottles a day. More often than not she takes no PKU Start milk in her second bottle at all. I am now giving her 2 sachets of gel per day and offer some before each meal.

However it seems she has almost taken to food too well as this week she starts to refuse to finish her first bottle. This bottle contains regular formula and is essential to give her the small level of phe she needs and can manage. Eventually measured amounts of food containing protein will provide this phe. They are known as 'exchanges' but we are not due to learn about them until our Stage 2 weaning lesson at GOSH on 12th May. Although it is a struggle, we do manage to get all the necessary formula into her by adding to later feeds/extra night time bottles but I phone GOSH to ask what we should do if we cannot one day, as the 12th May still seems like forever away. They reassure me that should it come to that we can introduce some simple exchanges which they can teach us about over the phone. Phew and roll on 12th May.

I love Ella's Kitchen

It soon becomes very clear that Gremlin does not appreciate my hard work and much, much prefers shop bought food. Humph.

With 2 boisterous girls I do not get much time to stand still in supermarkets and read labels but in the odd snippets of time that I have I find that there are lots of Stage 1 fruit purees which are 0.5g of protein or less per 100g. Her favourites turn out to be red ones - blueberries, strawberries, raspberries included. Top prize goes to Hipp Organic - Red Fruit and Apple Compote.

I keep trying to find savoury options to no avail. Then I spy a bright orange pouch in the corner of the baby food aisle. Ella's Kitchen 'Carrot, Apple and Parsnip' contains 0.4g of protein per 100g. We can now go out for lunch! We put it to the test at a soft play with friends in Enfield. Whilst Moomin is off playing, I am able to prepare and serve Gremlin's gel for her starter, Ella's pouch for her first course then Hipp jar for her pudding. Bless her she takes them all at room temperature and things again seem manageable.

Adjusting the milk and starting the gel

Gremlin has dinner 3 days in a row before we start to see an effect. She has more carrots (hmmm... still not sure) and parsnips (ok!) in this time. On the fourth day she takes much, much less of her second bottle, the PKU Start (the one with the phe removed but containing lots of other essential nutrients that she needs), than she has since she was a newborn.

On Day 5 she has lunch (a special rusk mixed with warm PKU Start- yum) and dinner (sweet potato and parsnip- ok) and drops even more of the second bottle throughout the day. This continues and by Day 7 I am worrying. I phone GOSH and they tell me I need to get her to take her gel, as this will eventually replace the PKU Start milk and provide her with everything she needs minus the phe.

I need to aim to get 1 -2 sachets into her each day. We've been given 'unflavoured' sachets as the flavoured ones are not licensed for infants under 12 months. To her eternal credit Gremlin gives the unflavoured unflavoured sachet a go. Maybe 2 spoonfuls go in. After that she pulls a face which I now know to mean "Yuck and No". I try the unflavoured unflavoured gel and pull the same face. It is almost peppery and very unpleasant. We try to disguise it the next day in fruit puree. The fruit puree flavoured unflavoured gel is more successful but not much. I phone GOSH again and am told about a great secret. Nesquik. I can flavour the unflavoured gel with banana or strawberry nesquik! Immediate success! The nesquik flavoured unflavoured gel is a real hit and I worry a little less for a while.

23 weeks - Carrots

By 23 weeks she is very, very interested in what we are eating and sitting up well. The time has come to begin the daunting PKU weaning process.

Moomin's first taste of food was carrots, so I think it only fitting that Gremlin has the same. She's chuffed to bits to be sitting at the table and knows exactly what to do as the spoon approaches. She still looks pleased as the carrots go in, but then they are considered and she seems rather unimpressed....

.

Let the food fun begin- Stage 1

So we bumbled along for several months. Levels going up. Levels going down. Worrying endlessly despite being told not to. Altering and correcting formula levels. Writing down everything Gremlin drank. Overall though we got used to our own little routine and started to feel, dare I say it, confident.

Then at 19 weeks we were called back to GOSH and told to start weaning.Gulp.

For a child with PKU, there are 4 stages to weaning. Stage 1 is the introduction of protein free foods. We are told that Gremlin can eat banana, mango, apple, pear, peach as well as carrots, green beans, parsnips, swede and sweet potato. We are also given special rusks and breakfast porridge which are protein free and available on prescription. The final part to learn is her medicine. She will need to get all the other nutrients she needs from a special gel.

In addition she can have shop bought jars/pouches so long as they contain 0.5g of protein per 100g or less. These items count as protein-free foods. My newest obsession for analysing labels begins.

We head home and I start steaming, pureeing and freezing ice cubes of the aforementioned fruit and vegetables in earnest. But Gremlin just isn't ready and I hold off for another 4 weeks.

Treatment

It's all diet.

So, for a teeny tiny baby it was controlling her milk intake. It is perfectly possible to breastfeed a PKU baby (supplemented by PKU special formula) but as we were already having huge weight loss issues (over 15% of bodyweight despite very best efforts) we changed to bottle feeding as I felt at the time I just couldn't cope with the weight loss and the PKU.

She would have to have 2 bottles per feed. This is because she needed to have a little phenylalanine (or 'phe' to those in the know) to enable her to grow and develop but not too much that w0uld cause her damage. So the first bottle contained a specified amount of regular formula (with it's bit of 'phe'), to be followed by a second bottle with a special formula which had had the phe removed. She could take as much of this as she wanted. The amounts would be specified by GOSH dieticians and based on the result of her blood tests to check her phe levels. We would have to perform heel prick tests on her twice a week ourselves and send them in the the lab for analysis. If her levels went too high, the regular formula would reduce. If they dropped too much, the regular formula would increase.

That sounded manageable. Just.

First trip to GOSH

We drove up on the Saturday, leaving Moomin at home and confused with Grandma, to see Dr Lara and start treatment. I cried again when we saw the GOSH entrance. We found her in Rainforest Ward.

She was wonderful and very reassuring. She explained that PKU is an inherited metabolic disorder and, in very very basic terms, Gremlin cannot process protein effectively. She would have to follow a protein restrictive diet (advised for life) but that if she did she would develop healthily and live a 'normal' life. If she did not, a particularly amino acid found in protein called Phenylalanine, would build up in her body and cause 'brain damage'. There were those words again. She also said, jokingly and in another reassurance effort, that if you were going to get a metabolic disorder, PKU was the best to get.

Wow.

The list of what she wouldn't be able to eat was quite overwhelming at first. Meat, fish, dairy, wheat, soya......

What on earth could she eat? More of that later.

A bit of history about us- diagnosis

I had never heard of PKU before. I mean I had 'heard' it in as much as the midwife explaining what the heel prick test was checking for when Gremlin (and Moomin too for that matter) was 5 days old but I hadn't really been listening in all honesty. I first properly listened to the letters PKU when Gremlin was 2 weeks old and we had a knock at the door late on Friday night from the on-call midwife. She explained that Great Ormond Street Hospital (GOSH) had been trying to call us all day and had been unable to reach us, so this poor gentle midwife had been sent out to deliver the news in person.

I cried. Daddymin's eyes went very wide.

He phoned Dr Lara at GOSH there and then on the midwife's instruction. She explained a bit about Phenylketonuria and that we needed to get Gremlin to GOSH the very next day to start treatment. She told us that we really shouldn't but she knew we would google the minute we got off the phone, and that if we did we should absolutely not worry about the words 'brain damage'. Of course we googled, found the words 'brain damage' and worried a lot.

Getting to grips with blogging

Ummmm.... I am a technophobe. I have never blogged before but, having been inspired by another blog, thought this might be a useful tool as a record for us but also to other Mums freshly facing the world of PKU and feeling a little daunted at times.

Bear with me.......