We had a great experience taking Braxton to the sensory room at the Flames Rotary House. The Flames Rotary House is a hospice that serves families caring for a child with a progressive life-threatening or life-limiting condition (Learn more at http://www.rotaryflameshouse.ca). It is a beautiful facility, where Braxton and I will be staying when I need some respite, and visiting for events and parent group nights. One of the rooms in the house is the multisensory room. Most of the elements in the room stimulate more than one sense, for example there is a bed that plays music, and a wall that lights up when you talk into a microphone. There is lots for kids to explore tactile, visual and auditory experiences. Braxton really liked the fiberoptics, drum and music bed. We already knew that Braxton is partial to the colour green, but we learned that he also likes firey orange glowy colours as well. Braxton didn't like the bubbles as much as the fiberoptics, he likes to be able to touch the light. Braxton really liked the drum in the room, and he liked to feel the bass through the music bed.
What is Dandy-Walker Syndrome?
Dandy-Walker syndrome is a condition that affects brain development, primarily development of the part of the brain that coordinates movement (the cerebellum). The central part of the cerebellum, known as the vermis, is absent or very small. Cysts form in the fourth ventricle, which is a fluid-filled cavity between the brainstem and the cerebellum, causing the ventricle to enlarge. The part of the skull that contains the cerebellum and the brainstem, called the posterior fossa, is abnormally large. These abnormalities often result in problems with movement, coordination, intellect, and other neurological functions.
In 80 to 90 percent of individuals with Dandy-Walker syndrome, signs and symptoms caused by abnormal brain development appear within the first year of life. Most children have a buildup of fluid in the brain (hydrocephalus) that causes increased head size (macrocephaly). Affected individuals typically have intellectual disability that ranges from mild to severe, although some have a normal intellect. Children with Dandy-Walker syndrome often have delayed development, particularly a delay in motor skills such as crawling, walking, and coordinating movements. People with Dandy-Walker syndrome frequently experience muscle stiffness and paralysis of the lower limbs (spastic paraplegia), and they may also have seizures. Hearing and vision problems are sometimes a feature of this condition.
Less commonly, other brain malformations have been reported in people with Dandy-Walker syndrome. These malformations include a failure of the brain to divide properly into the right and left hemispheres (holoprosencephaly), underdeveloped or absent tissue connecting the left and right halves of the brain (agenesis of the corpus callosum), a sac-like protrusion of the brain through an opening at the back of the skull (occipital encephalocele), abnormal slits or clefts in the brain (schizencephaly), or a type of birth defect that occurs when the protective covering around the brain or spinal cord fails to close during development (neural tube defect). These additional brain malformations are associated with more severe signs and symptoms.
Dandy-Walker syndrome is estimated to affect 1 in 25,000 to 30,000 newborns. It is estimated that 20 percent of individuals with Dandy-Walker syndrome survive past the first year of life. The most common causes of death in people with Dandy-Walker syndrome are problems related to hydrocephalus or complications of treatment.
How Dandy-Walker Syndrome Affects Bubbe
At first the diagnosis was both scary and abstract. No one could tell us how the brain abnormalities would contrite to Braxton's future. The first and most obvious symptom is Braxton's muscle tone. He had a very strong back arch, he could hold his head up almost immediately after birth, which was his hyper-tone, not strength as we originally thought. He could push up from his stomach early on as well. However this tendency to extend started to affect his ability to nurse and remain in a seated position. He would push back and away constantly. When he was three months old Braxton developed seizures, the second major symptom of his brain abnormalities. As he grew there were more problems with his muscle control and coordination, many of these problems were probably worsened by his seizure medications, which had the side effect of making him sedated and "floppy". Instead of being able to push up, he became a rag doll, and he can no longer hold up his own head or roll over.
Braxton doesn't have hydrocephalus and instead of the typical larger head, Braxton has a tiny head, microcephaly. This means that everything associated with his head is tiny, his eyes, his throat, his airway. This has caused some complications when Braxton has been admitted to the hospital and doctors expect him to be a certain size, especially when he has needed breathing and feeding tubes.
Braxton has many vision problems, which may or may not be related to his brain abnormalities, which I have outlined on the vision page (http://fortheloveofbubbe.weebly.com/vision.html). He also has the related abnormalities of agenis of the corpus collosum and simplified folds of the brain. We don't yet know how this might affect Braxton, although there is some thought that it can lead to autistic behaviors.
Most recently Braxton has developed problems with feeding and breathing. Tests show he is aspirating his own secretions. We suction his mouth when he has a build up of saliva to help. He is needing a g-tube to feed to reduce the risk of aspiration. We don't know if this sudden change was caused by a seizure, a reaction to his sedating medications, or is a symptom of his brain abnormalities. He is having sleep apnea, which is caused by his brain not recognizing the need to breath. Currently he has very short spells where his oxygen levels drop, but he recovers quickly, so doctors don't think it is causing harm. However these regressions are cause for concern, we want him to grow and progress, lately his development is slowing instead.
We feed Braxton a ketogenic diet. Each day we mix up water, ketocal formula, oil, beneprotein, and similac formula according to a recipe from the nuero dietitian. Once mixed we keep the feed in the fridge, then use it all in Braxton's six feeds. We also keep his pump bag in the fridge in between feeds. Braxton has "bolus" feeds during the day, he has 110 ml over one hour, every three hours. At night he has a "continuous" feed of 220ml over six hours.
Here is how we feed our baby:
1) Measure the feed from the daily mixture. (We use only glass measuring cups as less of the feed sticks and when we heat the formula we don want chemicals leaching into his food.)
2) Boil water and heat the feed with the water. (Don't heat formula with a microwave)
3) Add supplement(s) to the feed. (Each feed has different supplements scheduled by his dietitian)
4) Take pump bag out of the fridge. Once bag and feed are warmed, carefully pour the feed into the pump bag.
5) Remove as much air as possible from the bag then close the lid. Make sure the lid is closed evenly all the way around, if there are any edges still up, press down to snap them into place.
6) Gently squeezing the "rain drop" to open the valve on the hose of the pump bag to remove any remaining air and move the feed all the way through the bag's hose. This is known as priming the hose.
7) Hook the circle part of the hose into the pump.
8) Gently pull the ends of the hose until the cartridge fits into the pump. Push down on it to make sure it's in all the way.
9) Close the pump's door. (If you forget this step the pump will beep at you, which it does to me all the time)
10) Velcro the pump bag and pump into the pump backpack. (The backpack make it easy to carry and hold baby while he's being fed. When hes older he could wear the backpack and eat while he plays).
11) Hold the on/off button to turn on the pump.
12) Check the rate and dose, for example our bolus dose is 110ml and rate is 100ml/hr while our continuous dose is 220ml and rate is 37ml/hr.
13) Make sure the volume is cleared to zero.
(The pump will count the volume it pumps as it goes, and will say "dose done" when it has completed. If you didn't add enough feed to complete the volume you programmed it will beep and say "no feed". It will also beep and message if there is a kink in the hose or an air bubble.)
14) If there is still air in the hose, you can hold down the "prime" button to push the feed through the hose.
15) Make sure the Gtube clip if closed (so nothing from the stomach can come out) and open the big end of the Gtube and insert the red tip of the hose securely into the Gtube. (Don't push the tip in too far or you will stretch the tube.)
16) "flush" the Gtube by syringing water into the small hole in the Gtube. We flush 10ml of water before and after each feed to make sure Braxton gets enough water throughout the day.
17) Unclip the Gtube.
18) Press the "run" button on the pump and watch your baby eat! And the best part....he can eat while he sleeps. Thats one calm, happy, baby :)
We have been home almost two weeks now. It’s amazing how much faster times goes at home than in the hospital. We have had lots of visitors, mostly from Alberta Health Services. We are starting to find a routine, a new normal, but it is hard not to feel like we are being suffocated by our 8.2kg bundle of joy. Our big discussion with AHS and FSCD is what sort of supports we need in the house. Braxton is now a nearly ten month old with the development of a newborn to one month old. Nine months of having a newborn is exhausting. He still cannot hold up his head, roll over, or express his needs in anything other than a cry. I know other nine month olds aren’t talking, but most can at least move their hands to scratch an itch or rub there stomach or put their hands in their mouth. We always have to guess what is bothering our little man. Lately he seems to be getting sore muscles from not being able to move then on his own accord. Just one more thing to check on the long list of us trying to sooth a tired, teething, post-surgery, baby.
I have only attempted to leave the house once. Pretty much need a suitcase to haul all of the stuff Braxton needs to go anywhere. There is the typical diaper bag (with everything needed for a messy poopy baby, plus tape, syringes of different sizes, emergency meds, emergency gtube, emergency apple juice), his oxygen tank and mask, his suction machine (along with catheters and water), feeding pump bag (with pump, feeding bags, and charger), and finally a cold bag (with his ketogenic formula, meds, and flush water). I still have to figure out how I’m going to be warming his formula on the go, probably also have to pack a snap warmer pack if I am not somewhere where I can access hot water.
One of the most common questions we are asked is what is our day like. In many ways our day is like any other parents. Braxton usually falls asleep around midnight and gets up around 8:00, usually with a couple diaper changes or repositioning needed in the night. We get up early to get everything rolling and ready for the day. Our day is mostly made up of feeding, diapering, and entertaining Braxton, with some ‘special needs baby’ add-ons.
Feeding: Braxton’s meals are every three hours 6am to 6pm, then he gets a slow “continuous” feed from 9pm to 3am so we can get a bit of rest. Every morning we make his daily feed. It’s a mixture of ketocal formula, water, oils, beneprotein powder, and simaliac advance powder. We have to measure or weigh each ingredient, mix, and refrigerate the big jugfull. Each day time feed is currently 110ml of this ketogentic mixture and he gets 220ml over his nighttime feed. We warm up the feed, add two supplements/vitamins to each feed (there is a whole variety of these he needs since the diet isn’t complete nutrition), pour the feed into a feeding bag, hook the bag into a pump, flush Braxton’s gtube with 10ml of water, attach the bag’s hose to Braxton’s gtube, program the pump and turn it on. His feeds take an hour to empty the bag, then we detach all the parts, flush the gtube again, clean the feeding bag, then have a two hour break before starting all over again.
Diapers: I know all parents have the joy of dealing with wet and poopy diapers. Because of the frequency of Braxton’s feeds, especially the overnight feed, I think we have more wet diapers than typical. At least it feels like it because Braxton cannot stand a wet diaper. I blame this on the fact that we have to check his urine for its level of ketones, and so we keep cotton balls in his diaper, which when wet we press onto a test strip which changes color to indicate how well his diet is working. The cotton balls are understandably cold and uncomfortable when wet. We also record all of Braxton’s bowl movements for his dietician. We were weighing all of his diapers, but luckily we no longer have to do that. A fun tidbit: Braxton cannot use most bum wipes because of their sugar/glycerin which is prohibited on his diet.
Entertaining: Probably the most frustrating thing about caring for Braxton right now is that everything has to be done TO him. He doesn’t have the ability to make the connections and developments most children learn by just observing and exploring their environment. Instead we have to bring the environment to him. We try to help him experience different things by rubbing different textures on his skin, letting him listen to tons of music and always using auditory cues, moving his body for him into new positions, bringing different smells to his nose. The reality is after Brock and I complete his feeds and diapering every couple hours we also need to do some of our own eating and self-care, and in those times Braxton is often left on his back or in his chair like a blob. Because of his vision impairment we can’t use the old standbys of TV or a mobile to entertain in our absence. That’s where I could really use help, someone to either get his feeds ready and change diapers so I can concentrate on his entertainment/development or someone who can spend time stimulating and exercising him while I care of his basic needs. He is getting heavy for me to carry so I also need to start getting some exercise so I can continue to hold all of his weight each day for the foreseeable future. We continue to do physiotherapy and vision therapy, but once a week isn’t enough. And that’s where I start getting stressed that there aren’t enough hours in the day, and many days I am just too tired and sore, to do the development work Braxton needs daily.
All the other stuff: Coming home from the hospital Braxton is much more complex than before. Meaning his needs are extensive, risk of death high, and has problems in many areas. Braxton has central apnea, meaning his brain doesn’t always tell his body to breath. This happens most often when he is sleeping, his oxygen levels will drastically drop. It also happens when he is angry or crying. Like other kids he screams out all his air and holds his breath, but he doesn’t always get the message that he is out of air and needs to breathe in. Therefore we have to have oxygen on him when we travel (if he’s sleeping or crying in the back seat I can’t help him), always when he sleeps, and as needed when he cries. Right now this is Braxton’s biggest risk, each night we have to go to sleep with the knowledge he might stop breathing in his sleep. Braxton also has little control of his swallowing, so we can no longer put anything in his mouth. That means no soothers or drinks. Occasionally Braxton makes more saliva than he can control, particularly if he is sucking a blanket or crying, and then we have to suction his mouth so that the saliva doesn’t end up in his lungs. Braxton still struggles with three types of seizures (infantile spasms, generalized and focal seizures). We are eight days since a general or focal seizure so his diet is helping, but he still needs three medications, five times a day. Any seizure activity has to be recorded, along with his ketone levels, poops, any crying or throwing up, and weekly weight and length and emailed to his neuro dietitian on a weekly basis. This information helps her to adjust Braxton’s ketogenic diet for ratio of fats and calorie intake. Braxton has to be watched 24/7 because he needs rescue meds if he has a seizure longer than five minutes. We also have to fit gtube cleaning into our daily routine. So to sum up we have very full days back at home.