I often wondered what people saw in creating a blog for every day events in their life, did they not have friends to talk to....
I think i am a very lucky person to have great friends who are my support structure in life and always felt that they were enough for me, but here i am creating a blog to let out whats on my mind, so you ask where are my friends, well they are close by, at the end of the phone, posting on face book, the list goes on!!
The true reason behind my new blog is my beautiful six year old son Billy, he has just been diagnosed with a rare disease called primary intestinal lymphangiectasia....say that after a few drinks!!
Up until September 2010 he was a healthy bean, then he started getting a bloated tummy, oh course as a mum of two my instant diagnosis was constipation, bit of medicine and off to school you go!!
Oops little did i know my world was to be turned upside down.
By November 2010 it was apparent that constipation was not the issue, so i took him to our GP who ran a blood test on my birthday to be precise.
The blood test was in the morning and the GP was waiting on our doorstep when we arrived home, you kinda know when that happens the shit is about to hit the fan!
So our medical journey began, Billy's kidneys were tested, then we were sent to Birmingham's Children's hospital liver unit, one week later we were told his liver worked fine and home we went for what would be our last medical free Christmas.
We then returned back to our local hospital in February 2011 where quite thankfully for us Billy's consultant had seen this disease once before over 10 years ago and made a lucky guess that it was this disease. I should point out that there is roughly about 15 cases worldwide of this disease (something another parent who's child has this disease told us), so you see why i say a lucky guess
Billy was put on to a extremely low fat diet (10grams per day....check out the back of any food pack and you will see the challenge in that) with mct supplements.
I should explain what this disease does, when you eat fats, in particular long chain fats (present in most foods) the lymphatic vessels in the small bowel draw out the long chain fats along with other nutrients the body needs processes them and sends them through the small bowel and off around the body via the lymphatic system to nourish the body.
Billy's lymphatic vessels in his small bowel however have not been able to process these fats and have clogged the lymphatic vessels, which in turn have burst,as the small bowel is always in use they struggle to repair even with a low fat diet. So they leak fats, Vitamins, other nutrients and proteins the body need to grow and repair, also with the leakage he also loses lymphocytes which are key to a strong immune system,we know this is happening because Billy's abdominal cavities fill with fluid called ascities which extend to all his limbs and face, so without medical intervention his body would be up the creek with out a paddle and taking on water....
March the 14th....the day of no return, etched in my mind forever!
Billy had been doing half days at school due to lack of energy (well you would have lack of energy if your body's vit's and other goodies were leaking into your body cavities as loose fluid), however he came out of school happy with some energy, his teacher commented he had joined in at break time with play, something we had not seen for months as he had just gone into his shell because he felt so ill all the time (i have said it must feel like being hit with the flu). By 7pm that night a sickness and poop bug had got him.
When the body is fighting any bug/virus the lymphatic system goes in to overdrive to attack the germ cells, however as Billy's lymphatic system is leaking his body cavities just blew up with fluid, he was struggling to breath as his heart was being crushed by fluid and his body's electrolytes (magnesium, essential for heart function) were on the floor...another 24 hrs and i would be a mum of one not two boys.
The doctors tried something they were a little cautious about which was a albumin/diuretic iv infusion. Albumin is one of the main proteins Billy loses, this is needed to transport hydration in the blood stream....big player. Great news it worked in reducing the fluid build up in his body and gave him some va va voom back, this still continues to be our main support in this disease.
So what happened next, we got transferred to Sheffield Children's hospital for a video capsule (a pill with a video camera in it that captures 15 photos a second through the guts), the docs came back thinking in was not the disease we know it is, but just a narrowing of the bowel, so surgery was sheduled...yippee end of this journey....wrong!!
Billy under went a double ballooned endoscopy and laposcopy where his guts were examined inside then pulled out and inspected on the outside too...still feel a little queasy thinking about that.
There it was primary intestinal lymphangiectasia over 3 metres in diffuse patches over my beautiful boys small bowel, malnourished him and wasting his immune system...bloody bastard evil disease!!!Gggggrrrr
So here we are 5 weeks after that diagnosis, still in hospital, completely knackered, skint as my hubby has come out of work, not through choice we could just not manage being sent to different hospitals looking after our jobs and other son.
I work 16 hrs per week so with that and tax credits/child benefit we are carrying on, i think i had more money coming in when i was 16yrs old as a Saturday part timer, in fact i know i had more coming in.
So back to my original point why am i blogging when i have great friends, well i value them for a start and to have the conversation with them that you face the fact that your youngest son may die, they get lost for words and so would i if the shoe were on the other foot, the write up about this disease is incurable, life threatening, at best managed with diet and drugs, at worst fatal...Hhmm, bit of a conversation stopper me thinks! or tear jerker.
So to off load to a blog where chances are nobody will read is like free therapy.
Last week Billy had a Hickman line fitted to enable the doctors to deliver TPN feeds (a complete nutrition drip feed run over 20hrs) however this has failed, so after a stressful start to this week where surgeons have messed us about, i waved good bye to my hubby Chris and Billy tonight as they have been transferred to Sheffield Children's Hospital to hopefully get a successful Hickman line fitted while i wait at home as our other son Jack returns from a Spanish school trip tomorrow and fit my job in somewhere around that, i feel a shit mother as i don't know which son to give my time to the most or even how to divided my time so they both get some of me as i feel sorry for myself...pity party at my house!!!
I need to get myself in gear to raise funds to get changes made at my home to apply for funding to do TPN feeds at home (they like a wipe down type of home so bacteria can be destroyed), my friends and some family have been fab and got some funds going already, that makes me feel overwhelmed that they care about us enough to do that.
I have subjected myself to the disability living allowance form, OMG, i though i was quite a clever person until i started that...gloom was upon me within 10 minutes of doing this form. I have been assured that we will struggle to get this even though Billy's mobility is decreasing and he is wheelchair bound for outdoor trips...however i will share a little Britain moment we had, Chris and Billy got ward leave for a couple of hours and decided to go and apply for council tax benefit, so while Chris is explaining to the benefit advisor about how Billy is struggling with his mobility and how bad it is getting, Billy announces he needs the toilet gets out of the wheelchair and walks to the toilet....my hubby sat just mortified!!
So to sign off, as a very new and valued friend told me who's daughter has this disease "one day at a time"
and that's just what i do.x
