Harry's Journey

The Journey So Far...........birth to October 2003

Harry was born at 38 weeks following a fairly uneventful pregnancy.  Out of my three pregnancies this was probably the easiest.  I didn't put too much weight on, I felt fine once the sickness had passed and i worked until the end. In fact, i was in court (with work) giving evidence about three days before i had Harry.  I went into labour naturally, but it didn't progress and I opted for a C Section. When he was born he scored 9 on his apgar score, then five mins later he scored 10. He fed normally and was a content baby from the first night.  On the second day, the Paed Doc at Liverpool Women's Hospital found a small hole in his heart, but decided that it just needed a check up the following year.

The next day i went for a shower and left Harry with his Daddy. When i got back his Daddy said that Harry's arm had been twitching quite badly. It had stopped at this point so we thought no more of it. However this continued to happen. We were discharged when Harry was 4 days old.  Harry was a good baby, but he just wouldn't wake up. He never woke to feed in the night and he rarely opened his eyes in the day. The twitching continued and we asked the community midwife on a few occasions what she thought about it. Her reply was that she thought it was 'Normal baby behaviour'.

I remember that things started getting worse on May bank holiday weekend. On the Friday night Harry's leg started to twitch as well as his arm. The next day his head was twitching as well. This was happening about 6 times a day at this point. We were going to a friends baby's christening on the sunday and i remember Harry having a few of these episodes in the church and i knew that something wasn't right.  That night things took a turn for the worse. Whilst we were watching tv, Harry's leg, arm and head were twitching, then he turned to the left and his face contorted, his head turned back to the middle, then back to the left.  I went upstairs and found my 'Bounty' pregnancy book and looked up 'convulsion'. I think i knew at this point that it wasn't normal. My midwife came on the monday and again told me that it was 'normal baby behaviour'.  On the Tuesday, Paul was playing golf and i took Harry to be weighed. I mentioned to my health visitor what Harry was doing and she advised me to take him strait to A&E to get him checked out. I rang paul on the Golf course and we went to the hospital. We were just going to take him to a general hospital but when i rang my best friend Emma, she told me to take him to Alder Hey and i am just so glad that i followed her advice.

Harry didnt have any seizures whilst we were at the hospital but the Drs explained to us that they routinely keep all children in that 'twitch' or 'Jerk'.  We were a little bit dazed at this point as i think we were expecting to be given some medicine and sent home.  We actually didnt go home for weeks. The following day we saw a Neurologist, Dr Richard Appleton.  He asked lots of questions about what Harry was doing, how long each episode lasted and whether it was just one side of his body that was affected (it was mostly his left hand side).  He arranged for Harry to have a serious of tests and for him to have a CT scan.  Harry had 2 mri scans, a ct scan, ultrasound of the brain, numerous blood tests, chromozone tests, metabolic tests etc. All are normal. His first eeg was slightly abnormal, his 2nd was more abnormal and his 3rd showed seizures occuring from several areas of the brain (it was 5 hours long).

Dr Appleton suggested that we try a medicine called pyridoxine with Harry. It is vitamin B6, and some babys can have seizures when they are vit B6 deficient.  We went home from hospital on weekend leave and we actually thought it had worked, until the Sunday when he started having seizures again. At this point he was having about 5 a day that lasted about 30 seconds.  We went back to the hospital on the Monday morning and saw the Dr during the ward round.  The next drug we tried was carbamezapine.  This was a terrible time for us as Harry went from 4 seizures a day to one every couple of minites. He got that bad that he was given IV Phenobarb Medicine  over the weekend which sedated him.

To cut a long story short, all the meds we tried made him worse apart from phenobarb, vigabatin, phenytoin. Vigabatrin stopped the seizures for four days. We were so happy we thought we had cracked it.It was frustrating trying drugs and not having any good luck. Dr appleton and his team have been fantastic and we know we have one of the best neurologists in the uk. Dr Appleton ruled out all epilepsy syndromes one by one. In August 2003 he mentioned that there was one very, very rare condition that Harrys symtoms seemed to fit with. It was called migrating epilepsy of infancy. Sounds fairly harmless doesnt it? WRONG!!! It was one of the most devasting epilepsy syndormes that i had read about at that point. The only information i could find on the internet was a report by a Proff Du Lac in France. In this report he identified 12 kids worldwide. out of the 12 kids, 3 died. most never talked, walked, reached for anything, lost the ability to drink and swallow and developed microceaphaly.

We then had to wait until November when Dr Appleton met with Proff Du Lac. He agreed that it looked likely that Harry ahd Migrating Partial Epilepsy.  A drug was mentioned in Proff Du Lacs report and Dr Appleton arranged for Harry to start it. The drug was called stiripentol and was only available from france. We knew at this point that Harry was severely globally delayed and that he was registered blind. He couldnt hold his head up, reach for anything or smile.

 

The Next Chapter, October 2003-April 2004

The stiripentol showed a big reduction in Harrys seizures and he went from having seizures all day to just having some before he went to sleep and when he woke. This lasted until December when there was an increase in Harrys seizures again. It was still a reduction in what he was orignally having.  I asked about starting the ketogenic diet in the December.  We met with Alison Terry (Dietician from Alder Hey) in the January to discuss the what the diet entailed and whether we wanted to go ahead with it.  We started the ketogenic diet about on the 1st March 2004.  We were dreading trying the diet  becuase we had heard about the side effects. But Harry did not have any side effects intially.  We believe that he became much more alert and happy.  His seizures  reduced. During the first few weeks he was not having any of the bigger seizures and just a few facial seizures e.g eye flutters, facial jerks.

 

July 2004 - October 2005

 

Harry continued responding well to the ketodiet.  His seizures were mostly of a night time when he was trying to get to sleep.  It was getting really noticable how delayed he was. He couldnt use his hands or hold his head up.  We got him a standing frame and although he seemed to hate we persevered.  He also developed severe reflux and to ease this we began feeding him by micromax pump which dripped the feed into his tummy via his gastrostomy tube over 1-2 hours.

We had a bad time during this period when Harry was withdrawing from Nitrazapam.  We still believe that he was having increased seizures when his next dose was due. We also didnt like how it made him floppy and lethargic. We had a horrible time withdrawing from this drug and i sat up with Harry in the night when he was shaking and had sweats. I was keen to get him off the drug as quickly as humanly possible. Within 3 weeks of the final dose Harry was more alert. I can honestly say that withdrawing nitrazpam was one of the best things we have done for Harry.

In april this year Harry started on Keppra. This had an immediate positve affect and Harry had a substantial decrease in his seizures. Again his alertness increased.

Although we had increased alertness and reduction in seizzures, Harry was following the pattern of migrating epilepsy as it was reported in Proff du lacs report. He had microceaphaly. His seizures came in clusters which we called 'bad days'. He appeared to have about 4 good weeks to each bad week. During a good week he would only have seizuress at night and none in the day. on his bad days he would start having seizures every few mins and we then gave his emergency meds.He would also have seizures all day on some bad days.

Harrys emergency med were changed from rectal diazapam to buccle medazalam. Our hospital was part of a study in which they tested the effectivness of the two meds in a&e for treating seizures. Buccle medazalam came out best in children, it stopped seizures quicker and for longer. It is also nicer to administer. It is drew up into a syringe and squirted into the buccle space in the cheek.

October 2005 - Current, you can now follow Harry's story on his blog

In October 2005 Harry got a really bad chest infection and ended up back in alder hey. At this time he also developed chronic diahoreah. He would literally be covered from his arm pits to his ankles. We kept hoping it would go but eventually went to see a gastro doc in January 2006. He was sheduled for surgery for a bowel and intestine biopsy due to the weight he had lost. He was 31lb in dec and in jan was only 24lb. He had the surgery on the 20th feb and also had his mickey button fitted.

We saw the neuro surgeon in December and he agreed to fit Harry with the VNS. We now just have to wait for funding approval for our local PCT. We are also considering the reflux operation as an option as harry continues to be sick frequently.

We started to lose the stable period we had enjoyed from about the end of november. His seizures increased and started lasting longer. This combined with the diahoreah made caring for him really hard work as we were up all night. We started reducing the keto diet in December time and immediatly we saw an increase in seizures and they became more 'nasty' and again lasting longer. The seizures are making him more and more tired and i feel so sorry for him when they happen. He now has anywhere up to 30 per day, lasting up to four mins.

We started having to use the rescue meds more and more and contacted Dr appleton recently. He is now waitng for a new medicine which he has ordered for Harry to try. It is called 'pottasium bromide', (i think). To be honest, we dont hold out much hope of any drug working long term for Harry as none of the others have. The doctors have said that we are now looking at palliative care options for Harry, to make his life comfortable as possible. He doesnt have a long life expectancy and we just want him to have a happy life. He has his own spa pool in a log cabin in the garden and he enjoys daily hydro therapy sessions, followed by a nice bath and then an aroma therapy massage before bed.

One interesting thing that has happened since Harry was first diagnosed is that i am hearing about more and more children with the condition. At one point i only knew of Harry and a boy in america, also on this site called cooper. Now there is two other children at alder hey, a little girl in london, a girl in canada, and two other baby boys in america.

With regards to Harrys develoment, he is basically quadriplegic. He can not use any of his limbs or hold his head up. His development is assesed as being at an 8 week old baby. He is registered blind, unable to eat and drink and has micro ceaphaly.




Additional Info

In addition to what is written above, every winter Harry suffers with up to 4 or 5 chest infections. These make him very poorly and we cope at home until his breathing gets bad, then we go the hospital and get his SATS checked. If they are low we are admitted, if not we go home. Hospitals are not our favourite place and fortunatley Dr Appleton has been fantastic at letting us go home. He knows that we are sensible and will call him if we are worried and dont hesitate to take him to hospital if he deteriorates.