Some Good Some Bad

Hi, well it has been eventful since our last post, back and forth to the hospital. Jake was seen again and his blood has dropped by a full 2 points in a week, which is a big drop, but on a plus note he doesnt seem to bad in himself, he is sleeping loads more, and feeling pain in his body now when resting if he over does things, and trying to get him to slow down is impossible, as when he gets a burst of energy he wants to use it all straight away. So as for now jakes predisolone isnt being reduced anymore. We thought we were doing so well too. but we have had a good 6 weeks where he has maintained so that was all good. Jake has had his x-ray on his tummy and there is no blockage there its all nice and soft, but yet he is still only going to the toliet in his sleep in his big boy pants, and yes it is still just runny, It has now been 35 days since Jake has actually been to the toliet properally. So now we are at the stage where we are waiting about to see what happens they have reduced the movicol to 6 a day to try and let it thicken up, but if he stops going completly then we have to up it to 10 a day. Very confusing, I know, but the lady who we have seen over this has been very helpful, and is only at the other end of a phone. She is also going to speak to the original surgeon we saw when jake was first admitted for the prolapse, so hopefully when we go to Jakes next appointment on the 1st nov 2011 we will have a clear idea of how things are going to proceed. We feel like were back in the limbo stage where we dont know whats happening, and its a horrid feeling to have. So for now there isnt really much to report, other than keep fingers crossed his blood goes up, And his ezcema is back alot worse too, I dont think its something we have mentioned before in his posts as we have had it under control and maintained during alot of the time, It always flares up with his transfusions, and it has been easy enough to handle, but its now all the time, he is so itchy and sore, so we are now back on with with the full on treatment, for that creams 8-10 times per day, and wraps during the night, which is difficult with the pooing at the moment. His ezcema was a full time routine day by day, and worked into a daily rountine for him, and he doesnt mind so much having his creams on, luckily for us. Plus we didnt stop his routine at all even when it was looking clear, except for the wraps on a night. So it isnt really an adjustment we need to make. I just wish that I could take it all away for him, I hate having to watch him get so upset. But for now all I can say is thank you all for your support and will update when we have more news.

Bee Happy

Jake and all that goes with it

To start hi to all my followers and friends who keep up to date with this. We thank you for following. Well since our last post we were off to see the surgeons. And I think we had mentioned that Jake had been to the toliet with dihearrea and he has the prolapse again. Well it wasnt dihearrea at all it what they call fecal overflow. And jake has it bad, and the smell is horrid, it smells like sewage back up. So Jake hasnt actually now been to the toliet for 26 days. The surgeon that we saw as an emergency just dismissed us, he had'nt even read Jakes file, he was uncertain why we were there, and dismissed us saying will see him in 8 week see how he goes, and then told us to explain to Jake the it will happen again its just one of them things. Well as you can understand I wasnt impressed not at all, We were treated like rubbish, after all we had been told while is was in hospital about it being rare to happen to children and that it would need correcting if it happened again, then to see this person to be told yeah it will need seeing to but hey leave him 8 week see how he goes, it made my blood boil. So we went to see Jakes doctors who were great and very understanding about it all. They examined Jake and said they could feel the blockage, and they really needed him to go to toliet before anything else could be done. So they increased his laxativs again to help him get the flow going. But Have advised us that if by wednesday he hasnt gone then they will have to do something, but because Jakes not in pain or vomitting from it yet we have time to play with to get him to move his motins himself. So thats that for now, As for his bloood count he is still maintaining so the meds are working well for now so that is good. They have reduced his dose again we are now down to 5mg aday of predisolone, and his weight is coming down quickly. just fingers crossed he keeps maintaining. So this time its good mixed with the bad Im afraid to say. Jake was rushed back in to hospital on saturday evening as he couldnt cope with the pain any longer, they say the blockage is causing the pain, so they told us to keep on top of giving him pain relief, and to double his dose of laxativs everyday. To help him. They said for us to stay in and he can be monitored, but we were also told we could take him home, so home we came, he is more comfty at home and may even go to the toliet. So today Jake is on 9 laxativ's 8 yesturday and 6 the day before and that isnt even including the other laxativs they gave him last week which he takes 10 mls of too. And still no toliet action. Tomorrow we are back at hospital so who knows what will happen. But I hate having to listern to my baby cry from the pain, the terror in his face as he knows what may happen. I wish I could have it all for him., He should be in school making friends and doing the same as everyone else. But he is not and the bestest friend he has his big brother who, throughout all this has been strong and grown up, and a wounderful brother to Jake. Me and his dad are the proudest parents in the world to have such a caring and thoughtful young man as our son. Who maybe sometimes seems overlooked because he has been so grown up about all of this, he is my saviour, And I feel so lucky to have my boys and wouldnt change them for the world. Times like this show you the true colours of people and family that will always be there. Not only for me and my husband but for our eldest son too. Because these people are the ones that laugh at you when you fall over, but are the first to hold their hand out to help you up. And there isnt enough ways to say thank you. You keep us strong and you help make everything bareable. You hold our heads up when we cant. Our whole world had been turned upside down, and yet your still standing there with us. So I thank you again for truely being you. xx

In the words of Jakey Bee Happy

will update when we can. xx Thank you all for support.

Appointment catch up

Hi all, Well wednesday has been and gone so that means Jakey has had his appointment. Jakes blood has dropped to 10.8 in the 5 days since he has been out of hospital. But this is still a good number for Jake as we all know by now that he does'nt usually go higher than 8. He has had a late response to the predisolone which is what is keeping Jake maintained. So on the blood front he seems to be doing well. He is a little more tired than usual and soesnt want to do much but he is holding on. As for the other situation well thats a different matter. On wednesday Jake was on day 6 of no poo action, So between Jakes doctors and the surgeons they increased his laxativs, to help get him to go.But if he had'nt been by Monday to call and see what the next step would be for him, as the surgeons would want to see him. So Jake has been on the twice aday laxativ, and on saturday Jake started with the runs and all was clear, he was so scared to go to the toliet we were letting him go in his big boy nappies which we got for him. And he seemed fine, Then as the runs started to increase more we managed to get him sat on the toliet. We sat with him and read his books and talked loads so he didint feel so scared, And we thought as it was runny it would be so much easier. But we were wrong, it has happened again,  So Jake was admitted back in to hospital on saturday. Jake was terrified he even struggled to have his blood taken, which he does now with ease every week with his fav lady. They said as the bleeding had stopped and if Jakes bloods were okay we could take him home. And luckily his bloods were fine they were 10.7. So we were allowed home, and now were waiting on the surgeons decision on how he wants to proceed. The worst part of all this is Jake was so scared to go to the toliet, And I told him not to worry that everything was going to be fine, and I was wrong but we needed him to empty his bowels and he was holding it in, his brain switched his body off from going but the laxativ's won through and in the end 9 days later they won. So we are back to square 1. So hopefully we will have a decision today on what they want to happen with Jake, and not leave him too long.

So in the words of Jakey
Bee Happy

And will update as I can. xx

Well what a week

Well what a week it has been for us all. Thursay Jakey went to school after having time off with feeling exhausted, so we were so happy that he was able to go back and play with the other children at school especially as they have only really just started full time reception class and he has been off more than he has been there. He had a great day, we even walked home with very few rest spots he talked and talked about school and how much fun he had. And then at home he sat and played with his big brother before he went to get in the bath, and thats when everything went wrong. Jakey started bleeding heavily from his back passage, the blood was so heavy it looked like he had opened a vein, it was everywhere. When I checked his bum there was ahuge sac hanging there, horrible to explain I know but there is no other way, So we phoned the hospital and they told us to take him through. So we rushed into the car and off we went, on arrival the on call doctor had a little look a Jakey but he wouldnt let her no where near him, But the sac had gone back up his bottom. So he was admitted. He was monitered through out the night and his obs were stable, which was great. The following morning the specialists who deal with Jake came to see him, and had a chat and a look at him, and they said it maybe possible it is hemorroids, but as jake isnt suffering pain or any other signs that you would get with hemorriods they also think it could be a rectal prolapse. They specialists who saw Jake decided he needed to see the surgeon. Who came and explained that hemorroids are extremley rare in children, and that rectal prolapse is usually associated with aging. But as Jake suffered a high blood loss, the sureon wanted Jake to be seen by the lead surgeon, Who came and was just lovely, made Jake feel really relaxed, and he explained at the moment, he agreed that in both possible cases it is rare in a child, but as the blood sac as gone back in to the bottom, with surgery he wouldnt be able to get a clear picture. And as the bleeding has stopped, he wanted to wait untill Jake can open his bowels to see if there is any more blood loss. And if there is Then Jake will have to have surgery. They kept Jake in hospital untill saturday night, but he had'nt been to the toliet. We decided maybe he felt to much pressure from everyone to go and was'nt relaxed enough so we said could we take him home, and as we able to get back to the hospital they allowed us to fetch him home. So we were given laxativs for him to take to help soften his stools so he would feel easier going, But we are now 08.36 monday morning and he still hasnt been. He is terrified of going, he keeps crying and everytime he even pass wind he wants me to check for blood. Im hoping that today we have some luck at getting him to go. And that it is all okay when we do, as it will make Jakey feel alot easier. So as for now Jakey is house bound untill futher notice.

Hard to say today but in the words of Jakey Bee Happy

Our Little Super Hero

Hi everyone, well as you know its wednesday and Jakes usual trip to hospital, Well we got some more good news today, Jakes blood was 11.4 which is amazing. His hb has only dropped by 0.2. So we are now on week 3 without a transfusion, which feels amazing and a really nice break for Jake too. Meaning they are holding off on taking his bloods for more tests untill he is ready for his next transfusion, so then he is only needing 1 canular fitted. They have indicated today that Jake maintaing this week could be a late response to the meds, which would be great, but they have lowered his dose of predisolone from 17 and half down to 10mg a day. But they also mentioned that it could be that the transusions are maintaining him longer, which would also be good. This weeks appointment was a nice and short one, it was great to be and out, alot quicker than usual.
Jake is in his second week of school, and seems to loving it, he seems to settling in well, he is exhausted and sleeping loads once home, but for now he is enjoying it. Its nice that he is getting that normality in his life too.
He is still nice and chubby and his appitete does'nt seem to slowing down at all, yet. But they do say once he is weaned off the predisolne his appitete will diplete. And his weight will go back down. But for now he is loving his food.
We have noticed tonight though that he is looking a little yellow, which he didnt this afternoon. So will be keeping an eye on that. As we know how quickly Jakes levels can drop. So for now thank you for following Jakes journey and will update soon.

In the words of Jakey
Bee Happy

Sorry for the late update everyone

Hi well as you all know jake was back in hospital and had another transfusion, And then as always we go for his blood check up, and on wednesday last week it was good news, YES I said good news, His levels were good they were 11.6 the highest they have been in 4 months. I know they are still not within the normal range but we were close. So they have given us 2 week off, Before Jakey has to go back. So then they have started to slowly decrease his meds predisolone he has been drooped by 2.5 so he is now on 17.5 a day instead of the 20mg. And will keep reducing the dosage till Jake is off fully. As this happens his hungar will decrease too and the amount he drinks, so instead of our little chubby boy who looks really healthly at the moment, we will start to see Jakes weight decrease too, and his colour fade. Well we are back on the 14th and he will have another transfusion, if his levels have dropped low again, and he will be having a new lot of bloods taken. They discussed at the last appointment about Jake having his kidneys checked, and booking him for another bone marrow test. They want to keep on top of everything. They are really good and very supportive of us all. And its really nice to know. He is starting to look alittle tired around the eyes but he is holding up really well.

On another good note, YES I said another good note, Jakey got to start school yesturday and he loved it. The school are great too, and Jakeys TA seems really nice. He looks really grown up in his little uniform,  and loved telling me all about his day, he even drew my a picture for the fridge which was lovely.
I must admit it is really hard letting him go, I know it will be good for him as he gets that interacting with other children, and it brings some normalitilty in to his life too. But it wont stop me worrying.
Jake didnt have the best night, after his first day, he was shattered like most children after there first day, he was asleep early and kept waking throughout the night needing cuddles and saying his legs and arms hurt. And was extremly warm and clamy too, It found it very hard to settle fully. But it could have been worse. All in all Jake is amazing, and very strong and I hope he loves school, no matter what weather he can make it 2 days aweek or 4. And no matter what its nice to know that the school are there for us aswell as Jake.
So I will update next week when have had his appointment. unless before he needs to go in. But we have had a good week, fingers crossed for another one. xx

In the words of Jakey Bee Happy

Just a quick update, we did'nt get far into jakes week off, but we made a good start we mananged to go swimming saturday which jake loved. But looked so washed out after. And then sunday he seemed to really be tired and extremley pale, monday came round and he looked like he was auditioning for casper, so took jake for a check up at the hospital. And his blood had dropped again by 1.2 in 5 days of last being there. So my poor little man has had to have another transfusion.
I just hope his blood goes up enough for his appointent next week, so he doesnt have to have another transfusion then too.
His transfusion went went well enough, except he cried alot throughout it, as he does'nt understand why he has to have his orange juice. But he seems alot brighter this morning so fingers crossed. I have 1 brave little man, and he keeps is mummy strong. x
Interesting fact of the week Jake has had  the equilevent in blood transfuions to a large bottle of pop.

Bee Happy

Appointment update for jakey

Hi all, Jake had his appointmet on wednesday, and I wish I had better news, but I dont. It seems like the drugs are'nt working for him, As his blood has dropped by 1.3 this time. We were so hopeful as they had gone up by 0.2 last time. And I know it wasnt much but it gave us the hope that they were working, and the hope it would make jakes life easier for him. But seems not to be the case. Well for the next 2 weeks jake will stay on the high 20mg dose of predisolone and then they will start to wean him off by reducing the doseage, each time. Jake will need another transfusion when we go back for his appointment and they will take more bloods, and do more tests to see if there is anything else under lying. But for jake his next step is having to go on blood transfusion therapy, which is going to be long hard and gruling for him. He hates having to have the canular fitted, for his orange juice (blood), he hates having to sit there for hours after for it to be done, and all the waiting around. But how do you explain to a 4 year old, that this your life. The hardest thing is the hoping, but we still do it, and I hope that when we go back ,some miracle has happened and the meds work. I know they try and prepare us for the following week to come, and what must be done, And they are amazing at there job. But without the hope what else do we have. Well they have given jake next week off for some well earned hospital free time, so I will update soon with more on jakeys journey, and his progress soon. And will add as much information on blood transfusion therapy and what it all means for him, when he starts on the 31st August. Thank you all for your support.

In the words of Jakey

Bee Happy

A little information on Diamond Blackfan Amiema & Jakey

Diamond Blackfan Anemia, affects between 600-700 people in the whole world and is named after the Dr Diamond and Dr. Blackfan in 1935. The condition is a congential disease or can be caused by mutation in the gene encoding ribosomal protien S19 on chromosome 19 or chromosome 8. Most cases of DBA occur randomly. 10% are autosomal dominant or (less frequently) autosomal recessive.
Patients with DBA are at a higher risk rate of Leukemia and hemolytic malignanicies than that of the general population.
Most patients with DBA are diagnoised by their fisrt birthday. A nd in rare cases this can go undiagnoised untill it manifestes itself in early childhood.
DBA is where no or little red blood cells are produced by the bone marrow. The symptoms are hard to diagnosis due to the rare illness, Children will appear pale, fatigue, irritability, and even fainting. All common signs of everyday Anemia.

What have written is in no way to be used for medical reference.

Jake still has a irregular heartbeat due to the heart trying to keep thr oxygen moving throughout his body, but this is not as bad or as life threatening as it was when first admitted, And he has great doctors and nurses who are always checking him every week we are there.

Jake has been started on his first course of treatment, Which is predisolone at 20mg per day they started jake on this when his levels reached 8, as they suggested this would be the best time to try him, as he his Hb drops so quickly, and they didnt think he would reach 10. He has now been on the course for 5 weeks and as of yet it hasnt made any sign of working, and he has still had to have a transfusion, to push his levels up. But to say at his last appointment it had gone up by 0.2, which his specialist said wasnt enough to say it was working, to me Im holding onto hope that it is. But this wednesday coming the 17th we will know 1 way ot the other, if its working then we have a long wait to see if he can substain on it, and then battle all the side effects. Jake is having to have weekly gulcose tests to check for diabetes as this is a side effect to the drug. His levels go and down, but so far so good. And he is also piling the weight on, he is getting what you would say a moon face. x

Jake has to weighed and measured frequently as another side effect is weight gain and can stunt maturity growth. Another side effect jake has but I would'nt say serious is hungar, he is hungry all the time, we are lucky that jake likes healthier food he isnt as interested in sweet things, except skittles but only the purple ones. x

So I hope this helps a little to understand a little more about Diamond Blackfan Aniema and alittle more about Jake. I will add updates and more information as we get it. I also think this is helping me understand more too. So thank you for letting me share.

In the words of jake Bee Happy
Jakes biggest strength is his happiness, he can always smile no matter what.

Today was the day

Today was the day I felt the urge not to cry, and decided to start a blog about my son. So where to start.
 On the first saturday of may was like any other day. Except for us it was'nt Our son jake had been looking rather pale. Paler than usual, to anyone who knows jake he has always been fair, but this was different his lips and eyes just were disapearing into his skin. And his temp was soaring up, So we took him to our local A&E. With in minutes we were seen, and within the hour we were moved up stairs. They told us he needed to be seen by a doctor on the peadiatric ward. We were given a private room after he was examined and told he needed to have some bloods taken. As he had a heart mumar, the doctor told us this was due to the fact his heart was working overtime, to keep him alive. After the fitted him a canualar and took the bloods they said it was going to be awhile before his results were back. It only felt like minutes when the nurse came in and told us the doctor needed to talk to us. Thats when it hit, this wasnt an infection. They proceeded to tell us jakes heamoglobin level was 3.9, (normal heamogloin levels should be 13-15) and we were very lucky that we brought him in when we did. They told us we would be transferred to a special childrens hospital were jake would be looked after. That night seemed to have lasted a week. But sunday morning came and we were taken to the childrens hospital, where we greated by a lovely nurse who showed us to our room. A doctor came and spoke to us and told us jake needed a transfusion straight away, so we consented with no questions asked. We sat and we told jake that he needed a special drink to help him feel better, the transfusion took 4 hours. And we watched jake go from white to a slightly pinker colour. The following morning  a lady came to take jakes blood, to do a full blood count. Jake wasnt impressed and cried and fought her as much as he could. ( jake and this lady are now really good friends). Later that day the doctor came back to tell us jake needed another transfusion as his Hb wasnt high enough. So we agreed and another 4 hours later jakes had his second blood transfusion. Then we get the news that jake needs a bone marrow aspiration and biopsy as his white cells and plateletes have dropped. We were allowed home that wednesday afternoon, but only untill thursay morning and had to be back for his operation. The operation its self took 45 minutes, but seemed like hours. When we thought we were going to have answers we were told we had to wait 2 weeks. The weeks came and went, and still no news and in the mean time jakes Hb levels were still droping so he was still having to have blood transfusions. Which jake has now named his orange juice. At every appointment they told us little bits about what they were looking for, And then they told us they were checking for a condition called Diamond Blackfan Aneima and that it was very very rare so it was unlikely, because it usually comes with disformaties but jake does'nt have any, but they just wanted to rule it out. So we certain jake was going to be fine, And then thats when they tell you the 1 thing you don't as parent want to hear, your son has diamond blackfan aniema a condition basically  were the bone marrow does'nt produce red blood cells, So okay I tell the doctor, so how do do cure it, We don't there is'nt a cure.
The hardest words in the world to hear, a moment where it takes your breath away and you can't breath everything around you vanishes. And your whole life has just changed. So where have been since this? more blood transfusions yes. And now his blood has gone up with the transfusions they said it was the best time to try jake on the Prednisolone at 20mg which is extremely high, but if it works it will stimulate the bone marrow and produce red cells for him. Jake has still had transfusions while on the treatment, but were hopeful. If it doesnt work, then it will blood transfusion therapy all the time. They have explained that the treatment may not work or will just stop working, and then we will be back on transfusion therapy. And the we will be looking for a donor for a bone marrow transplant. We take everyday as it comes and watch jake as he smiles and how he just gets on with it. I will post with updates as I can, and on the 17th august we will know if the prednisolone is working. x I hope you will follow us as jakes journey continues, through the good times and the bad. xoxo