One of the goals I set myself this year was to have a better understanding of Zak’s needs, condition and get a clearer diagnosis. As we are edging ever closer to that I seem to have gained a wealth of information and yet still have so much more to learn.
Cerebral Palsy is the most common disability in children but still most people don’t know a lot about it and I hope this posts helps at least with some basic understandings. I certainly didn’t know much before Zak!
There are 4 main types of Cerebral Palsy:
- Spastic - This is high muscle tone which often results in stiff, jerky movements. Typically patients suffer with stiff muscles and spasms
- Ataxic - damage to cerebellar structures & patients with ataxic cerebral palsy experience problems in coordination, specifically in their arms, legs, and body. Ataxic cerebral palsy is known to decrease muscle tone. The most common manifestation of ataxic cerebral palsy is intention (action) tremor. Ataxic cerebral palsy is observed in approximately 5-10% of all cases of cerebral palsy, making it the least frequent form of cerebral palsy
- Dyskenetic (sometimes abbreviated DCP) – means struggle to control muscle movement, have twisting abrupt movements and can often affect balance.
- Mixed - Mixed cerebral palsy has symptoms of dyskinetic, ataxic and spastic CP appearing simultaneously, each to varying degrees, and both with and without symptoms of each. Mixed CP is the most difficult to treat as it is extremely varied and sometimes unpredictable in its symptoms and development over the lifespan.
Cerebral palsy is defined as a group of permanent disorders of the development of movement and posture, causing activity limitation. It is characterised by abnormal muscle tone, reflexes, motor development and coordination.
While movement problems are the central feature of CP, difficulties with thinking, learning, feeling, communication and behaviour often accompany the condition. Associated disorders include intellectual disabilities, seizures, muscle contractures, abnormal gait, osteoporosis, communication disorders, malnutrition, sleep disorders, and mental health disorders, such as depression and anxiety. In fact the following stats show this:
28% have epilepsy
58% have difficulties with communication
42% have problems with their vision
23–56% have learning disabilities
Pain is also a common side effect for CP sufferers and may result from the inherent deficits associated with the condition, along with the numerous procedures children typically face. When children with cerebral palsy are in pain, they experience worse muscle spasms. Chronic pain is under-recognized in children with cerebral palsy, even though 3 out of 4 children with cerebral palsy experience pain. It is also worth noting that CP sufferers burn 3-5 times more energy compared to non CP sufferers.
So what causes Cerebral Palsy? It’s caused by an injury to the brain either during pregnancy, birth or within first 3 years of a child’s life. Premature babies are at higher risk of CP as they are still developing but the injuries are caused to the brain by not getting enough blood, oxygen or other nutrients to the brain, before or during birth.
Symptoms of CP may appear or change as a child gets older. Babies born with cerebral palsy do not immediately present with symptoms. Classically, CP becomes evident when the baby reaches the developmental stage at 6 to 9 months and is starting to become mobile, where preferential use of limbs, asymmetry, or gross motor developmental delay is seen.
As of 2013, it was unclear how much of a role birth asphyxia plays as a cause. It is unclear if the size of the placenta plays a role. As of 2015 it is evident that in advanced countries, most cases of cerebral palsy in term or near-term neonates have explanations other than asphyxia.
Infections in the mother, even those not easily detected, can triple the risk of the child developing cerebral palsy. Infections of the fetal membranes known as chorioamnionitis increases the risk.
CP is not a progressive disorder (meaning the brain damage does not worsen), but the symptoms can become more severe over time. A person with the disorder may improve somewhat during childhood if he or she receives extensive care. The age when CP is diagnosed is important, but medical professionals disagree over the best age to make the diagnosis. The earlier CP is diagnosed correctly, the better the opportunities are to provide the child with physical and educational help, but there might be a greater chance of confusing CP with another problem, especially if the child is 18 months of age or younger.
The ability to live independently with CP varies widely, depending partly on the severity of each person's impairment and partly on the capability of each person to self-manage the logistics of life. Some individuals with CP require personal assistant services for all activities of daily living. Others only need assistance with certain activities, and still others do not require any physical assistance.
So where does Zak factor into all this? Zak currently falls into the Cerebral Palsy bracket but it’s too early to say what type. At the moment he is showing signs of mixed cerebral Palsy as he has symptoms for both Dyskenetic and Spastic CP. His symptoms include tone issues in his arms, stiff hands, using the wrong muscles in his hands and his balance is not great. Zak’s Cerebral palsy is a direct result of what happened in my labour. I had an infection during labour which was missed, this turned to sepsis and I passed this onto Zak, he was so stressed he pooed on his way out and swallowed meconium and as a result of the 2 was born not breathing. This meant he was starved of oxygen for 11 minutes and had 2 seizures and ultimately suffered brain damage from the lack of oxygen. The name of his injury is caused Hypoxic Ischaemic Encephalopathy (HIE) which is a brain damage caused by oxygen deprivation ant limited blood flow in newborns and is always a birth injury.
He currently has no other disorders alongside his CP but he is being investigated for his swallowing as on average once a week Zak is sick whilst eating, caused from choking/gagging. His eyesight and hearing are perfect and he can currently say around 120 words so from a communication perspective he’s exactly where he should be. His speech is really coming on and he’s communicating more and more, he seems to be picking up at least 2 new words a week and is starting to connect words like ‘I walk’ or ‘I catch’. In some areas like social skills he is actually measuring ahead – Zak has always mixed with adults and children and I am a big believer that children shouldn’t stop you doing things (you just have to make a couple of adjustments) so everywhere I go Zak goes, he is used to eating out, travelling, day trips, friends’ houses etc. This makes him really adaptable and very sociable, he has no fear and chats to anyone he sees. I also always tell Zak what we are doing every day, what order we are doing it in and who is seeing etc. I have always spoken to him like an adult and he even though he’s adaptable he is most comfortable in routine. (he definitely gets that from me)
As swallowing and sucking can be a challenge with CP we just need to eliminate there’s not an underlying issue. Zak struggled to feed when he was first born, he would latch on me but not get much milk and there certainly wasn’t a problem with my supply cos I would be dripping it all over his face trying to get him to latch! He was tube fed initially when he first came out of his coma, as it took him a long time to shift the sepsis so we had to keep giving him the fuel to fight it, meaning he had formula top ups alongside my milk. I was so desperate to breast feed it was the one ‘normal’ thing I was clinging to but after 2 weeks of really trying I accepted defeat and just gave into pumping and he was bottle fed, the important thing for me was he was still getting my milk and I managed to keep this up for 3 months (with formula top ups) – because fun fact your breast milk helps the baby fight infections, its full of all kinds of goodness and if the baby is sick your body produces antibodies to help fight the babies sickness, you can’t get that in formula so for me I was so adamant to at least give him 50% of it. Tom and I did disagree over this a little as Tom was keen to push him onto a bottle just so we could get him home and I could see his point but I was just so desperate to try, having already felt like I failed Zak. I know feeding is everyone’s choice and trust me no judgement here after all fed is best, but this was just something I was really passionate about. All that being said Zak also struggled with a bottle initially, hence the tube staying, for him to still get what he needed. He just couldn’t seem to latch even on a bottle but he did get there after a few days and the tube soon came out. They did test his suck and he seems to have a very strong suck, in fact almost over sucks – even now when he has a bottle it sounds like he’s sucking the top off the bottle and like there should be no milk left but he still finishes a bottle in the normal time of 10 minutes. He never got on with a dummy (a relief of mine really as I am dead against them) and he would over suck it like a bottle or just drop it out of his mouth all the time, he’s never even sucked his thumb – just likes to chew his fingers when he is teething!
He suffered with colic and reflux as a baby and so was sick often. I tried all the tricks of tilting the cot, walking up and down the stairs, feeding him sat up but trying to wind him was a nightmare, I felt like I was beating it out of him a lot of the time! As he got bigger he seemed easier to wind but it wasn’t until he was sitting at 11 months that the hourly puking seemed to ease. The doctors had expected this to ease once he sat and also once a child turns one their oesophagus seals over and is fully developed which helps ease the reflux.
So rather than puke after every feed it was only like once a week. I started weaning him at 4months under the paediatrician’s instruction as he was such a hungry baby and he took to it really well and within in a month we were on 3 meals a day and just 2 bottles. As he couldn’t feed himself he struggled with baby led weaning so we did a real mix of puree and finger foods but he seemed to eat everything and still does! He eats what I eat not only in variety but often in quantity too. Like most kids he can be a bit fussy with textures when he is teething and he can be a lazy eater so things he doesn’t have to chew much, like mashed potato, yoghurt, porridge etc. are his faves cos he can just inhale them!
Nowadays he is sick on average once a week and there doesn’t seem to be any rhyme or reason to it as it doesn’t seem to be texture related. His sickness is always when eating and I suspect it might be because he crams too much in his mouth and then gags, once he gags he can’t stop so he is ultimately sick. The gagging is definitely the cause of the sickness but the gagging can be from milk, rice cake, cereal, shortbread or banana, so a whole host of textures. So he has been referred to the SALT team (speech and language therapists) for a virtual assessment in December where she basically watched him eat as I gave him a whole host of different textures to try. I had already had a long phone assessment with her and we talked through everything and she asked lots of questions for things I hadn’t even considered like how he breathes when he drinks from a cup – the answer is he doesn’t, he holds his breath! This again could be a concern but he has never had a chest infection so they don’t think fluid is getting on his lungs but it is unusual to hold your breath whilst you drink. The good news is she doesn’t think there is a swallowing problem, no allergy as the sickness is already from different food and it could be as simple as he had inherited my ability to puke easily! We are just going to monitor him moving forward and she wants his tongue checked by the paediatrician as she is concerned there might be a slight tongue tie there – even though he got checked twice as a baby! Main changes are limiting his portion control and how much he puts in his mouth for the time being.
Zak is left hand dominant which is unusual to be so clearly preferring one hand at such a young age, typically most children don’t choose a hand until around school age but from about 6 months old Zak has preferred his left as it is his stronger hand. As I have mentioned before his right hand is very stiff but also now they are noticing the tone in both his arms and also his left hand is not quite as it should be. Whilst the left hand remains the strongest he still uses the wrong muscles in this hand and I’ve definitely noticed it more recently. He clenches or outstretches both hands when they are not in use, like he is having spasms and he still has no pincer grip on his right hand. The tone in his forearms means he can’t turn his arms over completely and he can do some tasks a bit awkwardly – hard to explain without seeing it! This is most likely Spastic CP. We have exercises we do every night to massage and release the muscles that both Tom and I do with him. These are not the most pleasant for Zak and he will tell you when he wants to stop and ask for a cuddle but he has progressed in the sense we are now able to do them awake. It is essential that Zak is extremely relaxed and as still as possible when we do them so initially we were advised to do them when he was asleep. When doing the exercises it is very clear how stiff the muscles are and I can often feel them move.
He is behind in his milestones and has been throughout. Children should be walking by 18months so at just over 2 he is considered quite behind and we are not expecting him to walk consistently for at least another 6 months. He has always followed the typical route for milestones, so he will get there and they are sure he will walk but his core is still very wobbly at the moment. He loses his balance very quickly, particularly if he is concentrating on something so for example if he is stood independently and picks something up he tends to fall over, or if he is sat up studying something he can fall over. This is likely the Dyskenetic CP. He is choosing to walk more independently and in recent weeks he has really pushed his coordination and often asks to walk now. Nursery is the best place for him to be as he is encouraged by the other children and has a lot more space/freedom to do things of his own accord. He is up to about 15 steps independently and he does this nearly every day now. His therapists have given us different exercises and activities to try with him, like using reigns when he walks so he feels supported, any activity that involves him standing and they’ve even suggested toys to help his development.
I have just accepted he will fall a lot more than other kids and he seems to have too as he doesn’t normally react, sometimes he just needs a quick cuddle and bit of reassurance to carry on, other times he laughs it off. His therapists have helped me to understand CP a bit more in terms of Zak’s behaviours, so when he is learning a new skill expect another skill to go backwards. Yesterday for example he did lots of walking practice but then in the evening he couldn’t hold a bottle. Also when he is tired the muscle tone will win so he will fall a lot more. CP sufferers are always fighting their muscle tone but when they are well rested and had a good meal they can generally overcome it but when they are tired the muscle tone starts to win so it’s helping him not push those boundaries. When he has swimming he works really intensely for 30 minutes and loves it but that means afterwards when I am getting him changed I need to lie him down as he can’t even sit up and the he eats me out of house & home! Finding the line is definitely one the challenges we face the most at the moment as he seems to go from not hungry to starving in seconds and not tired to beyond exhausted so quickly and trying to encourage a 2 year old to rest is often challenging!
At the moment it is too soon to say about his legs and hips so when he is walking we will get a better idea. I personally think there are going to be some issues with his right leg or hip as when he walks he seems to drag his right foot a little and over rotate the hip round, the physio sees it too but it could just be him figuring it out, so it’s just a waiting game. The more he tries and pushes himself the more it becomes apparent.
Pain is a real concern for me with Zak, the kid is clearly resilient, has overcome so much, and he has a very strong pain threshold probably from all the procedures he has endured already. But he has these episodes where he kicks for no reason and sometimes he puts himself in odd positons like he needs to stretch out or something is hurting. He doesn’t cry or scream but he whines and often wants lots of cuddles. He can get frustrated and lash out and often thrashes around his cot. Recently the pain episodes seem to be more frequent and different parts of the body, last week we had 3 different occasions. When he is in a growth spurt or in pain (like teething or poorly) then is spasms are worse and ultimately the spasms are what cause the pain. He will try to say where the pain his by giving me his hand or pointing to it. Some things he can say like toes and he generally just wants me to hold the area or rub them to try and ease it. Its absolutely horrendous watching him have these episodes – a few days ago he had the worst one I had ever seen and I felt so helpless. He was screaming, kicking and ended up sobbing uncontrollably clinging to me shouting mum over and over. All I can do is try and relax the muscle and give him calpol. The crazy thing is once the episode passes (generally only lasts a few minutes) it’s like it never happened! He is also at the age where he can’t communicate clearly that and is a typically emotional 2 year old so it’s trying to pin point exactly what is the problem but I believe in mother intuition and you learn their cries/whines don’t you?! It’s something I am monitoring very closely and bringing up at every appointment so we are all on the same page. The last thing I want to do is pump I’m full of drugs but I also don’t want him to suffer, something we are going to have to figure out as we go. Again another waiting game..
So the next steps for Zak…Now he is 2 we have moved to community paediatrics so he will change paediatrician in the coming months as he is at the age where he can be discharged from Neonatal follow up clinic. He is likely to have another MRI scan in the coming months to see if we can see the brain damage in more detail as he was only 9 days old in the last one and this will likely help confirm the diagnosis of CP and what type. I am pushing for the MRI to be in Southmead, Bristol so he is sedated, rather than at Swindon. You have to be a specialist unit to sedate minors for MRI scans and they don’t do this at Swindon so rather than put Zak through unnecessary stress of the scan and trying to force him to lie still I think it’s better it’s just done at Bristol. I dread it and the thought of him being sedated makes me feel sick, it’s like we are going back not forward, I’ll never forget those images of Zak hooked up to all the machines and wires but it’s about what’s best for him.
He continues to have Physio & occupational therapy every 3-4 months and they have been a great support working with both Zak & I to find the best solutions and exercises for him. I know how important it is for Zak to be as independent as possible so I hate leaving him to struggle with some tasks but it’s so important that he tries and they are always helping me set boundaries for him. They are so positive and encouraging of both Zak & I. They understand Tom & I are separated and as a mum I always feel they listen to my concerns and hear everything I have to say, offering plenty of reassurance. They instantly lift my spirits and Zak adores them even if he does always leave the appointments exhausted!
I am in the process of trying to get Zak private healthcare. As mentioned above the better the treatment Zak gets the better the chance he has at living as normal a life as possible. I want Zak to be able to see his physio whenever he needs to or have extensive therapy as required without having to go months in between because of the NHS delay. I’ve said it before and I’ll say it till I am blue in the face, I believe in the NHS and I whole heartedly support them, they saved my little boy, they are the reason he is who he is today. But they are also the reason he suffered in the first place. The term clinical negligence is not a phrase I sit comfortably with but it’s the truth. If just one of the things that were missed on the day I was in labour had been picked up, Zak would not have been born in the condition he was. Fighting for private healthcare and ensuring Zak has the future he deserves is not about blaming anyone or pointing the finger, it’s about stating facts and making sure Zak has the best possible chance. None of this was his fault and he deserves the world. He’s proved how much of a fighter he is and I know how lucky we are because he could have much, much worse CP but the reality is none of this should have happened.
My main aim through all this is for Zak to just be Zak. I don’t ever want to hear Zak say: Hi I am Zak and I am disabled. He has a disability, he is not disabled. It is part of him and part of his story but it does not define him. Zak will have to do things differently, he may not be able to do things the way his friends do but together we will find a way that works for him. I remember them saying to us when we were in Bristol that the hardest part of CP is not knowing which the condition is and which is Zak, because he could just be a kid that didn’t walk till late anyways; after all every kid is different! For me I struggle with patience so waiting is hard, I want to know what I am dealing with so I can get on with it and make a plan and that applies to Zak too. This year I think we have taken a big step in the right direction and I am in a better position to help Zak as much as I can, because knowledge is power.
I want him to know anything is possible but he just might have to take a different approach. At the moment he doesn’t seem to notice he is ‘different’ sometimes he watches other children and he gets frustrated he can’t do things the way they can but I don’t think it’ll be until he is older that he really starts to realise and we know exactly what his challenges are. I am fully prepared to have that conversation with him and it’s something I think about often, I made a promise to myself when I was pregnant that I will never lie to him so whatever questions he has I will answer. It’s going to be a fine line between addressing his condition but not limiting him. I just want him to remain the happy, cheeky little boy he is and I think that stems from the support network he has, he is so loved and encouraged by everyone. He often does things for a reaction – like he will suddenly just stand up and walk across the room but he makes sure we are all watching first as he loves to see us all get excited and make us smile! He is such a loving boy who has no fear and I count my lucky stars every day. I don’t know anyone that’s met him that doesn’t just soak up the joy that he radiates, he really is the best mix of me and Tom.