A huge milestone for our little man. First visit to the dentist 😁. For a child with sensory issues especially oral issues this was HUGE. Jason was actually excited to go. The dentist gave his teeth a good clean and an xray. We need to go for approximately 4 more visits to do 4 fillings. What a champ he was 😊. Proud mommy moment.
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Onion and ice still the favourites but today ate half a chocolate doughnut for the first time 😁. Granny Berit Long also got him eating chocolate over the Easter weekend 😊. Myself and the kids were treated by Alberlito hospital and Netcare 911 to an ambulance vehicle ride from home to hospital for a media interview and back home. Sirens, lights and all 😃. A little treat for us. A different trip to hospital and for such good reasons. To create awareness for rare diseases ❤. Hopefully our article will create more awareness for our rare kids.
Rare Diseases Day celebrated internationally is one very important day very close to my heart. Jason has 2 diagnosed rare diseases - combined primary immunodeficiency and cyclic vomiting syndrome and may have others. I try to make this an annual fundraising event where I sign up as a Rare Activist and raise funds for Rare Diseases South Africa. I like to make this day all about awareness and the community uniting in educating and embracing our rare warriors. It's so important to create that awareness and if I can do that for one day I'm a happy rare mommy. School drop off and work arrival was so special and emotional seeing all the little one's, teachers, companies and friends embracing this day with us ❤. To our family and friends thanks for always being there for us. We are almost 3 months tube free already. Jason is doing really well, he didn't actually have any adjustment to make without having a feeding tube. He has taken it in his stride beautifully and we haven't looked back. It's so nice not to worry about infections, plasters when swimming, having to wear a shirt so we don't scare people, leaking stomachs etc. He can now walk around without a shirt if he wants to. He has this beautiful little neat scar and it's wonderful being tube free for him and for us. When it's 30 plus degrees outside he can relax without his shirt now - a perk he never had the luxury of doing before. A common question I get all the time is "wow is Jason eating so much more now". We would love to say yes but just because the tube has gone, this doesn't mean he has suddenly figured out how to eat and wants to eat anything that he is given. This is a work in progress as it has been the past few years. People don't understand that although he has had his tube for 9 years, it has taken us 9 years to get to the point where he can survive without the tube. The tube was his means of survival because he was not consuming enough in order to sustain himself. He is now eating enough to be tube free and live a normal life. It doesn't mean that he has suddenly figured "oh yes I must eat now". This is going to take time and therapy. This is something that we work on daily, he has occupational therapy at school and speech therapy at school and we have extra afternoon therapy sessions with his speech therapist who works hard on his oral tactile defensiveness. Jason has many reasons why he doesn't want to eat or drink. It's something that is difficult to explain and difficult to understand if you don't understand oral tactile defensiveness. Half the time Bruce and myself don't understand it but we go with how far Jason has come. We would love him off his pediasure formula which costs us a fortune every month and would love him to be eating dinners with us but as Dr. Shaik always said to us "in Jason's time". It has taken 9 years to get to this point and we have to remember how far he has come and how well he is doing. I trust that once he is eating properly and not so reliant on his pediasure feeds we will be able to get rid of the nappies but this is a work in progress and Bruce and myself want more than anything for the pediasure and nappies to be gone. For now we continue with the therapy that he gets and keep encouraging him to eat what he is comfortable with eating. Oral tactile defensiveness is not easy and it's not the fact that he chooses not to eat, he needs to learn to trust food and I am just happy that we have come this far!!
Yes you heard that right. Jason is TUBE FREE whoo hooo. After 9 years and 2 months our dream finally came true. It has been a long, long journey but we are finally tube free and we are all on top of the world. Bruce and myself have been talking about the tube removal for months now. We have discussed the tube removal with his doctors and we met with his surgeon on the 1st November. The surgeon advised us to remove the tube a week after octagam so that his immune system is up and if the need arises and the hole does not close he will surgically close the site when the next octagam is due. We removed the tube on Friday the 10th November. Its very difficult to put it into words how we are feeling. How does one explain 9 years with a feeding tube? Well I guess I don't have to. Most of you have been following my blog since I started it 7 and a half years ago. Jason's feeding tube has kept him alive all these years. We have no regrets inserting his feeding tube, it has been his lifeline. Watching our little man grow from a tiny 1kg little baby, struggling his whole life with endless medical issues, being immunodeficient, not being able to eat for various reasons from being born premature, having a naso gastric tube from a few days old, developing severe silent reflux and totally stop eating because your mind and body won't allow it. The mind is a powerful organ, I have learnt this over the years. I have had to play my own mind games battling through watching my son suffer and almost die a few times but if I think of Jason and Jason's mind, Jason's mind has pulled him through this. He is one tough kid and he has never let his difficulties in life stop him. As the saying "mind over matter' goes, Jason has such willpower to overcome his physical problems, it's unbelievable. So how am I feeling?? I am absolutely proud!!! I am so proud of my little man. The things he has gone through in his little life I wouldn't wish it upon anyone yet he goes through life with a smile on that face, he has such trust and faith in myself, Bruce, his teachers, therapists and his medical doctors. BUT he's the true hero of his own story!! I am so so proud!! I am ecstatic! It's time for the next chapter for us. Since removing the tube, we have had quite a bit of leakage which is normal. I'm sure most of you are wondering how the tube was removed. Well the tube has a balloon on the one end and once in the tummy, the balloon is filled with 5mls of water. This 5mls amazingly enough keeps the tube in the tummy and to remove the tube you simply remove the water to deflate the balloon and take the tube out. The body recognizes that there is noting in the way and preventing the body from healing and the skin starts to seal the hole. This doesn't take long at all. In Jason's lifetime we have had the tube come out twice in the night and when we got to Jason the skin had already closed. The skin will not always totally heal and seal properly and we have been dealing with granulation tissue for a few months. Granulation tissue is actually connective tissue and blood vessels that form on surfaces in the healing process. So this granulation tissue has been trying to heal and seal Jason's stoma site for some time. I think the granulation tissue is complicating the closing of the stoma site but I am hoping that this will heal and close completely and we won't have to surgically close the stoma BUT if we have to, we have to. How is Jason dealing with all of this. Well we have been preparing him for months as well. When I first started talking about taking the tube out months and months ago and asked him if we could take his tube out his response was often NO. It has only been in the last I would say 3/4 months where his answer has been yes! Once I heard his first yes I knew he was ready. So the last week we have been doing a countdown of sleeps preparing for the big day. The day of removal he was extremely excited. When he woke up Friday morning I asked him "how many more sleeps". His response was a sequel of delight and "no more sleeps". We decided to remove it on a Friday afternoon so that we didn't have any issues with going to school. I waited for Bruce to get home from work, we popped the tube out and that was it, like it was just another day for Jason. We haven't dealt with any fears, attachment issues, nothing. He is just Jason - happy!!
He is dealing well with the leaking, constant on and off of plasters. He understands what is going on and he just takes everything in his stride. We had no leaking Friday night, probably because we fed him before bed and he was lying still. Saturday we had a lot of leaking and Sunday (today) we had a bit as well but not as much as yesterday. We are going through plasters galore and linen savers have become my best friend saving the bedding from getting wet. Our little man is happy, I have only given him pediasure as I have been concerned about food in the stoma but he did have some pizza yesterday and was eating the mushrooms off the leftover pizza today. Tomorrow he will go to school with his normal school lunch - a sandwich, fruit and yoghurt and we shall see how that goes but I feel that he will be fine and maybe some solid foods will be good. The one thing I noticed today was how much he is talking - gosh he was this little chatter box all day today. I had to ask him to be quiet a few times which is so unusual. Has he found his inner voice finally? :-). I've been really terrible with my blog updates - so sorry for those of you that aren't on Facebook! I regularly update the Facebook group mainly because it is quick and easy. Polygam and Octagam For those of you that don't know what polygam is. Polygam is a medication used to strengthen the body's natural defense system (immune system) to lower the risk of infection in persons with a weakened immune system. The medication is made from healthy human blood that has a high level of antibodies which help fight infections. Jason has polygam treatment every 3 weeks. We find if we go over the 3 week mark he does catch infections very easily. We have had twice it where polygam has been delayed and he has caught infections. In August he had bronchial pneumonia and was in hospital for 3 days and in October we had to stay for 3 days because of RSV and Streptococcus pneumoniae (bacterial infection). In November our medical aid yearly authorization for polygam expired. I am very pleased though, Jason's pediatrician motivated for octagam which is the imported version of polygam. Octagam is so much better. The bottles come pre-mixed therefore no need to mix and wait for the bottles to de-bubble, there are only two 100ml bottles as opposed to two 400ml bottles of polygam and the infusion time is only about 4 hours as opposed to 12 hours. Our last treatment went very well. We got admitted in the afternoon at around 2:30pm and we were home by 9pm. Polygam we are normally there for the night and the infusion usually only ends around 5am. School and therapy Jason is doing really well at his school. He goes to Bloom Centre for Special Needs which he has been going to since they started in 2013. This school has been the best thing for Jason and he has grown from strength to strength. He is in the primary class where they are basically learning grade R. Jason is learning to count, learning sight words, has learnt how to write his name and surname. He is battling with a few letters of the alphabet which makes him struggle with reading but he is getting there. We have noticed though when in shops or watching T.V. he can actually read a lot so we are not 100% sure how much he is learning but he's learning a lot. I think more than we are aware of Bruce and myself are just happy for him to go at his own pace. We never thought he would learn to read or write so we are very excited with his progress. He goes for speech therapy, occupational therapy and aqua therapy at school, which is so much easier having everything under one roof. His speech therapist has been working extensively with Jason focusing on oral stimulation, desensitizing his mouth and assisting with feeding and chewing. We had Jason's IEDP meeting before term 4 and we are all pleased with Jason's progress given the difficulties he has had to face. Kidneys We test Jason's urine protein creatinine ratio every time we do polygam. Unfortunately the last few results have been very high but he has had a few infections that could make the levels elevated. The doctors are still scratching their heads as to what to do here as all the other kidney tests have been normal so it's a bit of a mystery at the moment. We do find when he is better hydrated his results are a lot better. Jason's nephrologist would like to see us soon and would like a few more tests done. So this is something that is always a big question mark. Stomach issues and gastroenterology Since seeing the gastroenterologist a few months ago we haven't had much progress as to why we are dealing with such bad digestion and tummy issues. I'm glad we went through with the colonoscopy and endoscope. We had to go in and see if there was possibly anything. Nothing really was found besides inflammation. All tests have come back clear and there is nothing to report. I have had a few dealings with the gastroenterologist through email. In her last email she advised that she would like to do some expensive tests for Primary Immune Deficiency genetically and suggested we speak to a geneticist and/or immunologist. She has had a few patients who have had similar scope findings that have ended up having the testing with genetics coming back positive for an enteropathy. She has advised that the tests are quite expensive, they exceed R17,000.00. Which of course is money we don't have. Although it is always good to do these tests and find out a name or answer, this won't necessary give us a treatment plan. Jason is already being treated for Primary Immune Deficiency due to his IGG levels and he is doing pretty well on treatment. Eating Jason is doing really well with his eating. I can't even remember when we last used his feeding tube. He is not chewing 100% yet but everything that he is having is orally and he has been able to sustain his weight for quite some time now. A lot of his feeds are still pediasure which does help him retain that weight that he needs. He is eating all his lunch at school and after care. I love unpacking his lunch boxes seeing all the empty tupperware boxes. With his lunch, he actually eats better than Nicole his 5 year old sister. We are still trying to get him to have proper dinners but the chewing and swallowing is still a big problem. He doesn't like chunky food, can't chew meat or anything that requires too much chewing. For now we are doing mashed potatoes, butternut, gems etc. all the soft foods. His favorite foods are still onion, mushrooms and tomatoes. If I am cutting onion, he races over and starts eating. Just look at this face... I have been prepping Jason to take all his medication orally for a few years now. Friends of ours battled with this with their son who was tube weaned and this was one very important lesson I learnt. We are now able to give Jason all his medication orally which includes tablets. This is a huge battle won and a massive step forward for us.
So although Jason isn't eating meals, he is eating enough and everything that he is eating is going in orally. We couldn't be happier!! Today was a pretty smooth day. The plan was to see the doctor and get discharged by this afternoon. But believe it or not this week is polygam week and polygam was due tomorrow. I phoned our hospital close to home but there were no isolation rooms and the ward was full. It didn't make sense to get discharged waiting to be readmitted if there were no isolation rooms. We had a room, Jason had an IV line, all we needed was the polygam. I phoned our hospital pharmacy where we live to ask whether the polygam could be transferred to us here. This was not possible but the pharmacist phoned pharmacy here and miraculously they had enough polygam on the shelf for Jason's infusion.
Jason is doing pretty well considering what he has been through this week. He was very tired tonight and was fast asleep pretty early. My little superman has been through so much this week and he has been an absolute angel through it all. Even the nurses commented how patient and relaxed he was about everything. We had to re-site the drip this afternoon as the first drip had infiltrated but he didn't complain, he just sat there and let the nurses do their thing. We don't have any results back as yet. I did have a sneak peak in the file and I can see that the doctor has done plenty tests to test for all sorts of things but 90% of the results are pending. Hopefully we will have some results tomorrow when we see the doctor. I am very relieved that all of this has been done. It's like a huge weight has been lifted off of me. I have never been comfortable with the "that's Jason" answer when I question about his gut issues and finally we have been in, checked him out and done invesigations. If nothing is found, I will accept that but at least I know that we have done everything possible to try and help Jason. So the last 400ml bottle of polygam is up. I have been waiting for the first one to finish so that I can get to bed. I am really tired. It's been a long week but hopefully a week of some results. After literally NO sleep last night I "woke up" this morning actually excited by the fact that we had a theater day planned for us. I have been waiting for this moment for a while. No theater days or anesthesia is great. It scares the living daylights out of me because Jason's oxygen usually drops. He doesn't react well. BUT I was pleased we were finally here. We would finally see inside of that tummy that has given us so much uphill over the years. So first thing we had to do after the terrible night before was an enema to do a final cleanse. Poor Jase, his bum is so raw and now he had to have this enema. The poor child has been so traumatized. He handled it like a complete champ though!! He wasn't sure of this silly little green gown that he had to put on and there was no ways he was going to take off his shorts. I had to make a game of it and tell him that he was Dr. Long. The nurses kindly played along with me and he found this rather amusing asking for his file and everything. He wasn't the only one that looked silly either. Mom had to wear a blue gown with hat and boots so that was okay!! The only way I could describe to Jason where he was going was through his ipad game called "surgery simulator". This is one of his favorite games where he is the surgeon and he does kidney, lungs and heart transplants. He kind of thought he was getting a kidney transplant - to him this was cool so we went with that and in theater he was Darth Vader when they put his mask on to gas him to sleep. He went out like a true champ! The procedures took a little over an hour and a half. The gastroenterologist did an endoscope, a colonoscopy, biopsies and bloods. A long wait but my one best friend, bless her soul ALWAYS rushes over without me even having to ask. We had tea, scons and muffins together and the time flew by. The procedure went really well. In my opinion we already have progress. The gastroenterologist noticed a few things. Jason's oesophagus has these ridges where it should be smooth and straight. This could be a reason why he has been battling with swallowing and a reason why we thought he was "pocketing" his food, downing water to get it down and eventually choking and bringing it back up! The colon is normally rough like a carpet but a lot of Jason's colon is smooth and pale and also she said that despite all the meds and enema he had the night before there was still a lot of faecal matter so he could actually have difficulty with the excretion and movement as well. I had a good nap after the procedure. Jason I don't know how he does it but he was awake all afternoon.
The doctor also ordered a swallow xray to be done which Jason had to drink this horrible tasting liquid and two hours later they take and xray and then 2 hours after that they take another xray. This will show how quickly or how slowly the fluid moves through the digestive tract. So this will also give us some answers whether his digestion is too fast or too slow. I always thought it was too fast and he wasn't absorbing but at least this test will give us some more insight into the matter. It's been a long day, Jason is still battling with watery diarrhea and he had a fever tonight so it may be another long evening for me. Can't be worse than last (touch wood) but I must get some sleep now. Let's see what tomorrow brings!! If you are reading this and would like to, please leave a comment or contact through the contact page. I would really love to hear from whose reading and following the website. Some days I have over 1000 people reading, others just a few hundred. BUT it's great to hear from you. Please drop me a small note! Night night!! It has taken us a while to get to this point where we are FINALLY doing further tests and investigations into Jason's gut issues. The gut has always been one of my major concerns but with everything else Jason has had and also with not having a specialist in our area we haven't been able to progress with getting down to the bottom (excuse the pun) of his gut issues and why he has this chronic diarrhea and why he is not absorbing much of his food. Jason has never had a solid poo despite the solid foods he is eating. We were finally referred to a pediatric gastroenterologist (there hasn't been one available in our town) and we are FINALLY doing some investigations into Jason's on going gut issues. His last endoscope and colonoscopy was when we were in Austria in 2010 for tube weaning. Back then Jason was 3 years old and we had no idea that 7 years later we would still be scratching our heads over his gut issues. BUT in Austria he was there for tube weaning, he was very ill with rotavirus, norovirus as well as pharyngitis and bronchitis. Not exactly a perfect situation and a reason to do intense investigations there and besides that was not why we were there anyway. So here we are in hospital - not our usual home away from home hospital but we are getting pretty familiar with this hospital as Jason has been admitted twice for a week each for his renal issues. I must say the nurses are very pleasant, the doctor is great and the technology is great as well. They have a vein finder here - something every pediatric ward should have. This is an infared contraption that picks up veins without having to prod and poke and guess where the veins are. Wonderful invention but I believe they are very expensive!! See picture below. I don't know what happened but the medication to clear Jason out only arrived in the ward at 4:30pm. I was extremely irate as I knew what this meant for me and for Jason. It meant we were going to be up all night! I mean we were admitted before 10am and sat the whole day when the meds could have worked during the day. Instead we were literally up ALL night. I got NO sleep and this morning I was absolutely buggered. A colon cleanse is all I can describe it as - pretty much like rotavirus - watery, bubbly diarrhea that goes on and on and on. Jason's bum is raw, raw raw despite me coating it with Vaseline. No amount of cream or barrier can protect a bum from that kind of diarrhea.
So not a smooth night at all and I was absolutely finished this morning. |
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