The end of the line...(April - June 2012)

After the near catastrophe in March with the septic shower episode (see previous blog), the following two and a half months were a precarious time for Max. The pattern of being home for a few days before spiking another temperature and being trundled off to hospital for more I.V. antibiotics showed no signs of breaking. Also, Max’s skin, particularly around his right hand and thumb had broken down quite badly and was showing no sign of recovering.  The aim of the photophoresis treatment was to help him in fighting off the Graft Versus Host Disease (GvHD) of his skin, and yet there were no clear indications that photophoresis was working as hoped, and the intravenous line that was facilitating the treatment was also the number one suspect as the entry point for the barrage of infections he was regularly succumbing to. Something had to give.

Max made it home for Easter. Fortunately he was able to be administered the remaining doses in his course of I.V. Bactrim in the comfort of his own house, thanks to the RCH @ Home service.  Often, the only thing keeping Max in hospital, after the initial fever had subsided, was the need to have the remaining course of antibiotics delivered via an I.V. pump. In particular, if he needed to have the antibiotic more than once a day, it was not feasible to make multiple trips to the hospital, hence the reason for staying. Thanks to the RCH @ Home service, he could have his medicines lying down on his brand new couch, in front of his big screen T.V.

Max was back in hospital on April 12^th with another fever and back out on the 16^th after a short weekend stay. 

Not a happy camper...

Outpatient visits are much more fun than inpatient admissions!...Knowing the right people also helps!!!
Thank you to Max's Music Therapist Beth.

Max returned again to the hospital on April 27^th, this time for a more extended stay of two weeks, after registering a temperature of 39.9 C. This was a particularly frustrating time for us all as, much like the previous September, the doctors were unable to specifically identify the cause of the fever: was it related to the poor condition of the skin on his thumb, was the central line the culprit or was it just down to his GVHD? Max was pretty dispirited by this stage, and, because of the fevers and the general state of his skin, he wasn’t really interested in doing much of anything. This was a hard time all round.

Was it his thumb that was making him sick? His thumb had been rather manky for sometime. Max would be overly protective of his thumb, and tend to keep it enclosed in his fist. This might of kept his thumb out of the way but it also meant that he was not using his right hand at all, and now the entire palm of his hand was beginning to deteriorate. During our previous hospital visit, I (Bee) had mentioned the fact that there was a sore on his thumb that just wasn’t healing. I was just told to ‘keep an eye on it’, but no treatment was suggested or offered. By this time, his thumb had turned black. This was now a major cause for concern. Many departments from across the hospital were now involved in trying to help fix this issue: Infectious Diseases were involved to get the fevers and antibiotics under control, Plastic Surgeons were on board to determine whether surgery was needed to 1) open up his thumb to clean out any junk, should any be in there – and 2) to determine if infection had spread to the bone, potentially causing bone death, and Hand Therapy to manage the appropriate dressings and cleaning of his hand.

3rd May

11th May

Multiple cleaning procedures each day were a real battle. 

To attempt to ascertain if an infection was present in Max’s thumb bone, he was required to have a MRI scan, which for kids of Max’s age is usually done under sedation. We explained that Max had had one before without sedation and that he would be fine. Sedation would have required fasting and to be honest we didn’t want him to have more drugs in his system than was necessary. As protocol, Max needed to have a ‘practice run’ in a Mock MRI room. As expected, he blitzed it! 

Mock MRI

So time now came for the real thing. All systems were go until the staff pulled out a box, absent during the mock trial, that Max was instructed to put his hand inside in order to keep it still during the test. There was NO WAY Max was going to put his extremely sensitive hand, which he had protected with his life for so long, into a tiny solid box that would be pressed firmly onto his hand to keep it still. I (Bee) was totally gutted. Why did they not mention this during the mock procedure???? As a consequence, Max could not have the MRI that day, and when he eventually did, he was required to be sedated. We were put on a waiting list for the procedure. First time, we had to wait the WHOLE day, with Max having to fast from 5am to 5pm – only to be told it could not be done that day…..very angry! Second time lucky, and the results of the MRI showed no indication of bone involvement…relief!

Max’s high temperatures were somewhat of an enigma. Without fail, they would peak at 38-39C each night, but then come morning, they would return to a more respectable 36-37C. This was not typical of the fevers Max had previously experienced where he was consistently febrile until the antibiotics had had a chance to kick in. Finally, after having thrown all sorts of antibiotics at him, the Doctors were convinced that despite the recurrent temperature spikes, Max would be much better off at home. This was a massive relief to us all as we could see that, during this stay, the longer we were in hospital the more despondent Max was becoming. As a precautionary measure, he was prescribed an I.V antibiotic (from memory, we think it was Timentin) that could be administered at home, via a ‘Baxter Pump’.

A Baxter Pump looks like a babies bottle with a full balloon inside (it was actually described to us as like a condom – whatever takes your fancy I guess?!). The antibiotic is stored in the balloon/condom and is slowly discharged over the course of a day via an I.V line to which the Baxter Pump is connected; in Max’s case, his central perma-cath line. The device was configured to release the antibiotic over 24 hours and each day a nurse would come out to our house to change the bottle over.  Max would have this Baxter pump for 5 - 6 weeks.

Max at home with his Baxter pump.

So with a Baxter pump attached to Max, we headed home. Not even 24hrs later, the RCH @ Home  nurse who came to change 'Baxter' got a temperature reading from Max of 39.7°C. Upon calling the hospital for their advice, it was strongly suggested that we take Max to the ER. We were devastated. We got to the ER and explained to the Doctor that during our two-week stint in hospital, Max got fevers each evening which settled by early morning. We were so grateful that we saw a Doctor and nurse in ER that knew Max, understood our situation, and… allowed us to go home again! I'm sure this decision was made easier, given that his temperature, whilst still febrile, was lower than what was recorded at home and that he was enjoying a 24 / 7 infusion of antibiotics - thanks Baxter! This ended up being a Finch record ER visit; in and out in less than two hours!

Although, ‘Baxter’ had facilitated getting us out of hospital, whilst on it, Max was nauseous throughout the days, gagging often and vomiting at least twice daily.  This was quite concerning as Max was not eating as a result, and losing a lot of weight. He was not a ‘happy chappy’.

Vomit bag hand at all times.

Along with his hand sores…the skin on Max’s feet had also begun breaking down, resulting in some rather nasty wounds. They were so tender that he was unable to walk, or wear socks - for fear of the socks sticking to his wounds - and shoes for that matter. As a consequence it was necessary for us to drag out Max's pram so that he could be chauffeured around during his hospital visits. As these wounds were regarded as a potential entry point for infections, it was required that we attend the dressings clinic for appropriate foot dressings and supply of an abundance of fancy pads, bandages and tapes for his feet. The preferred pad of choice was one that was impregnated with Silver - as this metal is known for it's healing properties! Max was our little King with very expensive feet!

King Max being chauffeured around the hospital 

The effort required to get off the pads and bandages each day...

It was a massive production each time we had to remove the pads/bandages. When at home - prior to his evening bath - we had the procedure 'down pat' by pre-soaking his feet in a shallow bucket to loosen the dressings. On one occassion at hospital however, when Dr KT wanted to see Max's feet, our only choice was to soak his feet in cardboard bedpans!!! Needless to say - after this mammoth hour long task of a screaming Max with bleeding feet - Dr KT took my word for it as to how his feet were tracking!

Meeting 'Giggles"

On May 24^th, Max went to hospital for his weekly photophoresis treatment. However, during preparation, a clot was discovered in the line and so treatment had to be called off for the day. The clot was able to be removed but in the process of re-locking the line with his special vancamycin lock, part of the line cracked! What was immediately thought to be a disaster actually turned out to be a blessing of sorts as it was the cracking of the line that triggered the decision to cease photopheresis. With the line cracked, it was no longer safe to be used for photophoresis and nor could it be repaired. If we wanted to continue photophoresis, Max would have to, for the third time, have a new line surgically implanted.  So weighing this up against the fact that his skin was not obviously improving and the seemingly never-ending round of infections he was enduring, it was an easy decision to make. 

We had dearly hoped since the previous September that photophoresis would be the silver bullet that would enable him to get off all of his medicines and cure him of his GVHD. We were however also well informed of the relatively experimental nature of the treatment; of all the associated risks and that there were no guarantees, only vague probabilities of success.

During the course of the treatment, we had genuine hope that he was improving and would often convince ourselves that he was looking better, but in hindsight, it’s hard to know what effect it really did have. The only objective criterion that we knew to look for was the condition of his skin. That did fluctuate during the course of the treatment. Some days it looked fantastic; pale and intact with little mottling. Other days it was fiery and flakey. He certainly turned the corner quickly around the time of starting photophoresis, from being on a respirator in ICU to out of hospital in two and a half weeks, but was that due to photopheresis or was he already heading in that direction? Although we were very grateful for the opportunity to try the therapy, despite the Doctor's and Max's best efforts, it didn't work out as we had imagined it might. In many ways, it was a relief for us all when the decision was made to finish it. No more risk of septic showers, no more weird and wonderful bacterial infections, no more blocked lines to be wrangled with and no more temperamental photophoresis machine.

Max’s adventures in photopherisis came to an end on May 30^th with the removal of his line. Having arrived at the hospital at 8 am – as a fasting and nauseous little man - Max had to wait until 5:30 pm before he went in to surgery. However, being the seasoned campaigner that he was, he wasn’t anxious at all.  He actually was demanding the ‘chocolate gas’ so that he could be put to sleep to get it all ‘over and done’ with ... because he was hungry!

As he still required the I.V. antibiotics, due to his thumb/hand wounds, he had a more temporary PICC line put into his arm. The antibiotic course finished on June 5^th, and pretty much straight away Max’s nausea and vomiting ceased. On June 25^th, the PICC line was removed. Max was line-free once again….this was certainly cause for celebration! 

PICC line

PICC line

As photophoresis had finished, some of the drugs that he had been able to drop from his regimen had to be reinstated, including steroids. At around this time he also began taking a new drug called Imatinib (Gleevec), to pick up where the photopherisis had left off in treating Max’s GVHD.  Gleevec (or Glivec) has been used for a number of years in the treatment of certain cancers, but recently it has also shown some promise amongst a number of patients in lessening the impact of skin GVHD. In this sense, it is still a relatively experimental treatment and there are known side effects and risks. That said, Max didn’t need an artificial appendage plugged directly into his blood supply network to support it, so with what he had been though over the past 9 months, this was a great relief.  

By the end of June, Max was certainly experiencing the effects of the reintroduction of steroids. Steroids are used primarily to suppress Max’s ‘ new’ immune system so that it does not attack his skin – which it still sees as being foreign. Other major side effects of steroids include the ‘moon face’ and an increase in appetite. So, as a result of restarting the steroids came the return of ‘Max the eating machine’, and we once again had the pleasure of listening to Max’s beautiful voice 24hrs as day….”I’M HUNGRY!!!!!.....”

In the pantry....Max's favourite place to be whilst on prednisolone/steroid treatment!

…luckily he had no issues with eating the majority of his 5^th Birthday cake!

Birthday Boy!

CAKEEEEEEE!

Next instalment:

Hand splints, influenza and School boy Max!