a good day

The increase in medications seemed to do the trick. Monday night he bombed and slept great. He
was only up twice. The first was brief and the second included sitting in the ensuite for 30mins but overall we all got some much needed sleep.

On Tuesday Ethan was in a great mood. He was happy and in a good place. It was the best day he's had in a couple of months. It seemed the increase in medication had worked. He could focus and concentrate on tasks. He was happy with the girls and visitors. Tuesday night was a complete different story though. He didn't bomb with the night sedation and he was up more than 10 times during the night. A break through dose at 4:30 made him crash and he was only awake once between then and 8:30.

Today he has been cranky and sleep deprived. He was back to telling everyone to shut up and go away. After a long day he crashed with tonights sedation so fingers crossed it continues into the wee hours of the morning.

sleep deprived

The weekend was a shocker. Ethan was very agitated and cranky. I gave breakthrough after breakthrough but nothing calmed him. He just kept on going. I thought he would crash Saturday night but he didn't. The longest sleep he had was 2.5hours.

Sunday's nurse stayed for ages. She was unsure how she could help him or us. Many scenarios were thrown around between her, the doctor, Kristen and I. These included medication increases and changes, catheters and hospital/Very Special Kids admissions. Luke had taken the girls to a swim meet and I didn't want to make any major decisions with out him. In the end, we decided to increase the pump and be reviewed today.

No surprises, the increase in medication didn't really do anything. The review today resulted with another increase in pump medication and a big increase in night sedation. If this doesn't work, the Dr advised us to admit Ethan to Very Special Kids before Saturday to prevent carer burnout. He didn't think Ethan had urine retention, so a catheter wasn't inserted. So glad we didn't do that yesterday! The pump is now at Nozinan 20mg, Morphine 50mg, Midazolam 30mg and Dexamethasone .5mg. The night sedation includes doubling the Melatonin and increasing the Clonazepam by half. Hopefully this works and he can get some well needed sleep.

Today, Ethan has remained agitated and cranky with intermittent pain and is still eating non stop. He is a remorseless eating machine that will eat anything. He reminds me of Augustus Gloop. By morning tea time, he had eaten a full days worth of food. I never thought I'd see him eat like that.

fat guts

Ethan remains tired and continues to eat non stop. The increase in food with the side effects of the pump medication led to some serious constipation. He was having a lot of trouble sitting and pushing as he would fall asleep. An increase with the laxatives and 1L of prune juice has him running to the toilet every 5 mins, holding his bum yelling "poo poo"! It's actually very funny to watch. He's gone from one extreme to the other. Lucky Poppy Syd bought 50,000 rolls of toilet paper!!

His abdomen is very swollen. It looks like he has a beer pot. The needle site needs to be changed more often as the extra abdominal pressure blocks it. After the stuff up on Tuesday, I am now fully aware of the effects of this and can get on top of things early. The site itself isn't red, but the medication hasn't gone through. Once it is removed you can see the needle is flattened or bent from the pressure. Ethan still cries and gets upset with the needle site changes but today he thanked the nurse for helping him.

Ethan also has bad leg pain. His little legs are swollen and he has cankles. The added pressure in his legs as well as the extra couple of kilograms he is carrying is causing some horrific pain. Sometimes he just collapses to the floor holding them, but today he has started punching them and kicking his legs uncontrollably.

Ethan is more accepting of his condition now. When he first went into hospital he was very accepting, telling me he was sick and never going to get better. He made me ring people and tell them to come to the hospital as he was never going home. He then went through a stage of telling us he wasn't sick. He is now accepting of his condition again and is admitting he is very very sick. This is a tough thing to deal with. He can't speak with a counselor as they wouldn't understand him. Not that he would anyway. I have managed to have some pretty in depth conversations about death, dying, sleeping, heaven etc. No parent should ever have to go through that. It can really play with your head. I have had a lot of trouble keeping up to date socially as it seems everyone is either whining about nothing or bragging about life. It is tough to stay positive when everyone else is moving on and my life seems to be at its lowest point. But then I remember things could be worse.

I am looking forward to the next couple of weeks with the girls on school holidays but am also worried how they will manage seeing the ups and downs more often. We have had a lot of offers for sleep overs and play dates if the girls need to get out and about. They have also been invited to birthdays and have holiday activities planned but we need to be mindful they are not too far away or doing to much during their break. Our annual camping trip has been cancelled this year.

Challenge Visit

The cool people at Challenge came out to visit Ethan today with Baxter. Baxter is a dog Ethan has met numerous occaisons on Challenge camps. He asked to see Baxter and Challenge responded!

 

Ethan with Baxter, Sonia and Jamie

 

Ethan last November in Ballarat on the 'Cops and Kids Camp' run by Challenge

 

Ethan with Baxter in Ballarat

nozinan effect

Over the weekend we continued to play with medication doses. The pump has remained on Nozinan 12.5mg, Midazalom 20mg and Morphine 40mg since Sunday. This seems to be the right dosing. The change in medication hasn't improved things but we can now increase the pump doses as he deteriorates. He is still having the odd breakthrough and not settling well during the day. The last 24 hours have been even more difficult as he missed out on most of his medication due to the needle being bent, poor nursing skills with that needle insertion! The pump is checked regularly to make sure it is working however I must admit I have been slack. You can see the light flashing through the bag and hear it so I didn't take the syringe driver out, if I had I would have noticed the lack of movement. Lesson well and truly learnt. He has been a nightmare today but I know tomorrow will be better!

Our biggest issue is his fighting of sleep. He refuses to sleep during the day as he's 'not a baby'. He is a walking zombie. He eats non stop to stay awake. He also has edema in his abdomen and it is very tight and full. It is the first time he has had a little pot belly and is heavy to pick up. The added pressure and volume in his abdomen is causing his breathing to be quite fast and shallow. He also has facial edema causing his face to be very full and tight and shiny in appearance.

He is that tired he falls over. I have caught him falling from the dining table and toilet. But there have been times we haven't been as lucky. He has had a few falls while walking. He fell walking into the toilet, luckily he didn't hit his head on the porceline on the way down! He is very stubborn and pig headed so will not let you help him.

The girls are exhausted. They are having issues with the amount of visitors. We have created a 'quiet space' which no one is allowed to go in. I have explained that Ethan and I are house bound, we have no contact with the outside world. The only communication and interaction we get is from visitors so visitors are welcome. But we need to be aware they are trying to keep a normal routine, learn new things and be kids. They are dealing with a lot and need to come home and relax. So far the 'quiet space' is working and most people are understanding of this.

Ethan did manage an outing yesterday. The 4th one since this all started. He went over to Glenallen for a friends birthday. It was a nice outing. We managed to draw it out to 40mins!

Some good friends are organising some landscaping for us. The details are being kept from us but a lady came today to talk to us about plans and get our input. They should get the detailed plans in a couple of weeks. We are on a need to know basis and I am starting to feel that most of you might already know about this.

The girls dreams are definitely going ahead. I realised the other day when a friend asked, I haven't mentioned what they wished for. Erin wanted a pink stretch limo ride with all of her friends to the Hilton for lunch; a girly day to share with her family and friends. Adele wanted a BMX. We are also staying at the Hilton for the night as an added extra. Little Dreamers are a new charity that grant young carers or siblings dreams. Little Dreamers Online

Ethan will go on his own holiday to VSK as he is now too sick for friends and family to mind. They are the best people to care for him at this stage with the added bonus he loves it. He is staying 2 nights but he cracked it at me today as he wants to stay 6 nights!

drowsiness

Over the last week Ethan's behavior has been much the same; wanting to watch a DVD, then 2 mins later wanting a PS3 game on, then wanting to go to the toilet, then wanting to lay down, then wanting to eat etc etc. To add to this he is still coughing blood and is physically exhausted. He is now a danger to himself. We can not let him do anything by himself in case he falls asleep and falls. This means sitting at the computer, dining table, on the toilet etc. I have already caught him falling off the toilet once. We had one day last week where he was that agitated he ended up with 6 different break through doses and was still up yelling with his eyes closed, when he did finally go to sleep for the night he was up 6 times. We don't know why this is but it is physically and emotionally exhausting.

We have had a few changes in medication to try and help. The Haloperidol was increased to 3mg at one stage. This calmed him a lot. He was no longer yelling, he was tranquil but still agitated and pacing around the house changing from thing to thing. The Haloperidol has since been ceased, the Midazolam has been decreased to 30mg (from 40mg) and a new drug, Nozinan, has been commenced. At the moment there is no real change in his behaviour or sleepiness. Over the weekend we are slowly increasing the Nozinan and hopefully decreasing the Midazolam some more. It is going to be a tough weekend trying to get the medications right. But we may never achieve this. While we are trying for the correct doses, his heart disease and cardiac failure is worsening, he is still coughing blood and therefore becoming anaemic, his kidneys and liver are under attack from all the medicine. It is a tough and vicious circle that can only end one way.

The girls are coping extremely well considering what is happening. I am always amazed at their strength and ability to function. They are carry a huge weight on their shoulders but just suck it up and get on with it. They will get their dreams soon so that will be exciting for them and us. I haven't told them, I am trying to keep it a surprise. They are gorgeous girls that deserve so much more.

Satdy

Overall, friday night was a pretty good night, Ethan got up a couple of times for the toilet, but otherwise slept. He was up at around 9am this morning and in a decent mood for most of the morning, he even chatted to the SEPC Nurse instead of telling her to piss off. But as the day wore on, Ethan started going a bit crazy, wanting to watch a DVD, then 2 mins later wanting a PS3 game on, then wanting to go to the toilet, then wanting to lay down, then wanting to eat etc etc. This cycle continued until he started coughing up blood again and then collapsing on the ground because his legs had given way.

He was a struggle to deal with. At one point he was trying to cough up the blood in his chest, but seemed unable to, his eyes rolled back into his head, but then he eventually did a big cough. His chest still sounded gurgly. It was basically a repeat of yesterday afternoon.

Even though he was totally knackered, had extra morphine and midaz on board, he was still fighting going to sleep after this. We ended up darkening the room, telling him it was night time and sending him to bed. And he did finally go to sleep at about 6pm. Phew!

Not looking good for tomorrow, if this pattern continues, but no-one said this was going to be easy.

leg trouble

The haloperidol was increased to 1mg yesterday. This seemed to take the edge off the mood swings. He seemed more content between the ups and downs. Things seemed to be better but then today happened.

The nurses came and they noted he was more settled. They came with a drug order in case Ethan has a catastrophic bleed. We have this drawn up ready to go just in case. It's a horrible scenario and I'm hoping it doesn't happen. The nurses had barely closed the door when things went pear shaped. He lost the ability to use his right leg. He was staggering in circles and crawling on the floor. When he did have the ability to walk his leg would give way and he'd end up the floor. This happened several times this afternoon. He then started having trouble going to the toilet. He was getting very agitated and angry. During all of toilet visits he started coughing blood. I'm not too sure what happened next but he seemed to forget how to cough, swallow and smile. He had a mouthful of blood which I had to scoop out. He sounded horrible. I wasn't sure what to do, what medications to give etc. I made a few phone calls to the palliative care team who were supportive. We finally got on top of things and he is now fast asleep. I wish and hope nothing like this will ever happen again but chances are they will.

I'm hoping all the extra medicine will help him sleep all night but I have this feeling he'll need to go to the toilet.

dexamethasone

The doctors came out to the house yesterday to review Ethan. It was decided to keep all dosages the same. I'm not too sure how effective the haloperidol is. He seemed a bit happier yesterday but he also had a really good sleep the night before. He still had episodes of yelling and screaming but got over them quicker. I am hoping this is the effect of the new medicine and today will only get better.

He is still spending his days watching DVD's. He likes to forward them to the funny bits and then rewind them to the funny bits. Finding Nemo was played backwards yesterday. He demands someone sit with him while he does this until Uncle Colin gets home and then it's his turn until bed time.

He is still coughing blood. These coughing fits are happening more often. There is nothing we can do other than sit and comfort him and hope the next cough doesn't produce a massive amount of blood. There was some talk about changing antibiotics as infection can cause him to cough blood, but with no other signs or symptoms this seems very unlikely. The most likely cause, and the one we believe to be true, is that the increased pressure in his pulmonary artery is causing the blood vessels in his lungs to burst. We have dark towels strategically placed around the house so that when blood does splatter out it doesn't scare him or the girls. Blood on white stuff always looks like more than it is.

The needle sites were lasting a week but now we are lucky to get 2 days from them. We are trialling a small amount of dexamethasone inserted into the cannula to see if this will prolong the site. This works well with the elderly but the doses aren't as high. Fingers crossed this works. Changing the needle is horrible. He gets so worked up during it, putting a lot of pressure on his heart.

The girls have their moments. Sometimes good, sometimes bad. We have had a counselor come and visit them. They liked her visit but she didn't get much from them. They both expressed trouble talking with friends. The last time Lucy came the girls were very open. She is coming back again so I'm hoping it will be more productive next time.

haloperidol

Ethan had the Dr review at Monash as planned. After many discussions between 4 different units, it was decided to keep the morphine and midazolam at the current dosage and add haloperidol. This wasn't a decision taken lightly as haloperidol has major cardiac effects. It has been commenced at a low dose and can be increased if needed. Yesterday afternoon was again very trying and he required breakthrough medicine last night but he has since slept very well for him. It'll be interesting how today goes.

We have had a lot of people ask us whether he is in hospital or home. We have decided to nurse him at home until he passes away. If we wanted him in hospital we would still be there from our admission in January. Any review at Monash is just that, a review and home again. It is easier going to Monash instead of the GP and paediatrician etc as it gets done in one go rather than the running around between doctors and the chemist and then waiting on nurses to come etc.

haemoptysis

Ethan was awake all day yesterday. He was exhausted and his behaviour was all over the place. He yells and screams and wants you to sit with him one minute and then he's telling you to bugger off the next. He gets very angry and slams doors and throws things. It was a long afternoon. I was hoping he would have a good sleep and sleep most of the morning but he was up a few times and he woke early this morning.

By lunch time the same pattern of behaviour had started. It was horrible. Uncle Colin was flying back and I stupidly offered for him to go to the airport. He said yes and about 5 minutes later I was regretting opening my mouth. We loaded the car and set on our way. A minute into the trip I was going to turn around and take him home. He was confused and crying but it seemed he would fall asleep. He fell asleep 10mins into the trip. Finally he could get some rest but 2mins later he was wide awake. When we got home he spent 3 hours yelling at me. Telling me he hates me, that I'm stupid, go away, come here etc. I'm not too sure what they can do for this but something has to be done or we'll all end up at a loony bin. He has had 3 extra midaz break throughs this arvo and he was still up yelling at me. I was pulling my hair out. He has also had a major coughing fit this evening and is now coughing small amounts of blood. Lets hope this doesn't get worse. He finally fell asleep exhausted at about 7:30.

I paged the palliative care nurses. After discussing the issues with the Dr, we have a plan. Hopefully Ethan has a settled night and we will go to Monash tomorrow morning for a review and a change in pump medication. If he is unsettled we are to give morphine and midaz. Hopefully that will get on top of things until the morning. If not we will be going to Monash overnight.

Hopefully this upcoming review will get on top of things. I'm not to sure how long I can keep going like this. It is taking it's toll on all of us.

Still fighting

Over the last couple of days Ethan has been extremely restless and agitated. The pump has been increased to 40mg of midazolam to help ease this. (The morphine has remained the same). These doses are extremely high, especially when you add the break through doses. A lot of adults don't handle these levels but Ethan is still up fighting. His strength is amazing. He was awake a lot last night. I couldn't believe it, after a total of 50mg of Midazolam he wanted to play. I didn't appreciate the car and tractor noises on the side of the bed! He was then up to the toilet constantly. When he finally got to sleep, I thought he would sleep all morning but he was up early. By lunch however he was completely buggered and has rested and slept the day away.

We are now on top of his toileting trouble but he has developed fluid retention. His abdomen and torso are very tight and his little ankles and wrists are showing signs of swelling. His diuretics have been increased to help ease some of this pressure but it hasn't helped.

Toilet Trouble

Ethan had the needle site changed yesterday. This is now positioned in his right arm. He has had a few changes since the syringe started. His stomach has big red inflamed lumps and so it has been moved to his arm. Originally they were lasting a week but now with the increase in meds they are only lasting a couple of days. The pump is now at 50mg morphine with 7mg breakthroughs and 27mg midazolam with 5mg breakthroughs.

He slept a lot yesterday and when he was awake he was irritable and very demanding. He jumps from thing to thing and can't focus. Lucky for us we had a few visitors to help sit with him. This is the trend with all awake time now. This plays havoc on the girls.

Toilet time is now a big issue. He can spend hours trying to wee and can't poo. Morphine causes urine retention. He can spend hours in the ensuite trying to wee. He can no longer stand for long periods so ends up sitting, but then falls asleep. We have tried everything. Luckily for us he has always managed to go before a catheter is needed. We hope this continues. Morphine also causes constipation. Ethan has had a lot of trouble with this as well. When he finally gets the urge to go he pushes until he falls asleep. Lastnight I spent hours in the bathroom waking him up, telling him to push. I think at one stage we were both asleep in the ensuite! It was a shocker of a night based around trying to wee and poo.

Today Eth's class come for a visit on the bus. Ethan was excited. He even got on the bus and went to the Rocket Park with them. I drove behind just in case. He lasted 35-45 mins before he was completely buggered and had to come home. He had a ball with his friends but we paid for it for the rest of the day. He was a very angry and upset for the remainder of the day.

Pix

 

 

 

Sleepy

Ethan didn't have a great day yesterday (Sun). He was irritable and cranky. He required 3 breakthroughs, so the SEPC Nurse increased the syringe driver to 50mg of Morphine today. Midaz was kept the same. The breakthrough doses have been increased to 7mg.

He has slept all day so far and this may continue for a couple of days so he can catch up on lost sleep.

Hopefully we can again get on top of his pain.