A Message From Sarah

Hi Everyone.

Mum and I are participating in the Walk to Cure Diabetes, an event that raises money to fund vital research into finding a cure for type 1 diabetes.

As you know, our family has been directly affected by this chronic illness and it is still a constant struggle. Type 1 diabetes is an autoimmune disease that is not caused by diet or lifestyle or through any fault of the sufferer. It can strike at any age but often occurs in childhood. Every day, five more Australians are diagnosed with the disease.

Type 1 diabetes affects every aspect of a person’s life and has a huge impact on families such as mine. A diagnosis of type 1 diabetes means constant finger prick tests and insulin injections, up to six times a day. This happens seven days a week, 365 days of the year. There are no sleep-ins, no holidays and no
time off. Just today Mum had to check my blood sugars 12 times! Children don’t grow out of type 1 diabetes and the threat of dangerous blood sugar highs and lows is
always present.

Perhaps the hardest aspect of the disease for us to deal with is the potential of long-term health complications. Even with the best care and management, people with type 1 diabetes often face complications such as blindness, heart disease, stroke and kidney damage.

That is why I am asking for your support. The Juvenile Diabetes Research Foundation’s Walk to Cure Diabetes raises money to fund the best and most
promising Australian research to find a cure for type 1 diabetes and help
families like mine.

Please give as much as you can – all donations, regardless of amount will bring us closer to a cure. To visit my personal fundraising web page click on the link below. When you donate a receipt will be automatically generated.

Thanks for listening to me, back to bed now, Mum is just about to come and take my BSL again!

http://walk.jdrf.org.au/teamParticipant.asp?participantID=571

The Artificial Pancreas

Im very excited.

I had a meeting with Sarah's Endocronologist. Her nice, friendly supportive endo, (not her origional Dr McMoron )

We planned this meeting a month ago after I was contacted by a Dr in the US claiming to have made a breakthrough in Pancreatic Agenesis research.

Firstly we discussed the events of last week and the condition in that Sarah was in. She agreed that it was unacceptable and can understand my feelings in why I feel they failed in there Duty of Care to Sarah and what we can do to ensure it wont happen again in the future.

We then moved on to this US research group. They have come across a new gene mutation called RFX6 Gene.

They found this gene mutation in 6 of 7 patients with Neonatal Diabetes. The patients all had a pancreas but the pancreas was either Annular or Hypoplastic which means that only a tiny portion was developed in fetal life.

All patients had Neonatal Diabetes but more interestingly they all had Malroation of the bowels, Severe IUGR at birth and Bilary Atreasia's.

Sarah was IUGR, Hypoglycemia within hours, had Malrotation of the bowel and had a micro colon which is a Bilary Atreasia.

The research this far and this part of the research is only at the mice stage, but that Islets Call Trasplants have transitioned the mice of insulin and this is a major breakthrough. It appears the RFX6 gene mutation causes all patients to be born without the Islets of Langerhams which are the insulin producing cells of the pancreas. Of course Sarah being completly Pancreatic Agenic means that she has no insulin producing cells, but this research indictes that an Islet cell transplant would be succesful.

We are quite a few years away from ICT therapy being approved for humans, but it is a major breakthrough for the few kids with this conidtion.

Something else that is quite exciting for us is the first stage of the Artificial Pancreas.

The first stage is now available in Australia. However upgrading Sarah's current Insulin pump to the new one is complicated. As hers is still under warranty under "Governemt Guidelines" we are unable to for another 2.5 years.

Well we found out in the last few days that there is a loophole in the system. If a newer technology becomes available that is better able to manage the condition then your health fund at there discretion may pay the benefit.

The new System is in 2 parts. 1 is the insulin pump itself. The second is an additional cannula that reads the blood sugar every 5 mins and uses blue tooth technology to send the reading to the insulin pump. the insulin pump then alerts the user ie me to rapid changes in blood glucose. So if Sarah's blood sugar was rapidly falling it would sound an alarm to alert us. If we were unable to take action ie we are asleep and the blood sugar falls below 2 it Automatically suspends insulin delivery for 2 hours.

So a few calls later and we find out the criteria for applying for "special consideration"

The claimant needs to have

* Used glucagon in the last 3 months
* Frequent Hypoglycemia
* Had an episode of Unresponsivness/Diabetic Coma
* Hypo Unawareness

Sarah meets all of these which is bad she does but good for this purpose

Now there is the pump side of it that health funds cover, but the optional CGMS system is not covered and comes at a cost of about $1500 with the cannulas priced at $75.00 each and they last for only 6 days. So the ongoing monthly cost is about $300.00

So we placed a call to our health fund who advised that if it was explained by the endo on how the integrated CGMS would benifit sarah and assist us to manage her condition that they could use "special consideration" to also pay for the CGMS.

So its really been good news. Our Endo is writing us a letter of support but things are looking positive for it to be approved

Admission # 25

So we have just come home from Admission # 25. An experience I never wish to happen again

Below is an email I have sent to the hospital administration

I have had a few days to reflect on an incident involving the medical care of my daughter.

She has a long and complex medical history at the hospital coming to the Neonatal Unit when she was 36 hours old. She has a precarious condition and was diagnosed with Pancreatic Agenesis (She was born without a Pancreas)

Last Thursday (19/08/2010) I bought her to the emergency department with a suspected Bowel Obstruction. Her care in emergency was not the concern, however late in the day it was decided that in order to wash her bowel out she would require an IV. 6 attempts were made at accessing her with no success. Her Gastro Consultant then made the decision that she would need to go to theatre for a washout and for a Central Line to be put in.

As she has Pancreatic Agenesis she is Obviously flagged as a Type 1 Diabetic and this would be the 5th time she would be going to theatre. We were admitted to the 5th Floor Medical unit. Shortly after arriving, the theatre nurse came to collect her.

Her BSL was checked prior to leaving the ward and her BSL meter handed to staff. I walked down to pre-op hold where I again checked her BSL and it was 9.6. Her Insulin Pump had been reduced to 50% of its normal rate as she was ion a fasting state and has a history of her BSL’s dropping under a GA.

She was returned to the ward 2 hours later. The recovery nurse said that she had woken up and had been kicking her legs around and all obs stable. She then left the ward

I touched Sarah’s hand for no reaction. I asked the nurse what her BSL was. There was nothing recordered on her theatre Observation chart.

I immediately took her BSL to find it was 1.4. Her nurse was checking her conscience state and she was unresponsive to Touch, Voice and painful stimulus.

There were no fluids running so the nursing staff administered 35mls of Dextrose into her central line and Sarah started coming out of the hypo

It was confirmed that no Monitoring of her Blood Glucose Levels had occurred in the time that Sarah was in the care of the Theatre Team.

As a parent of a child with a very complex and precarious medical condition, we place 100% faith in the medical staff. I can be in control of a lot, but in theatre I place my full trust in the medical staff

To witness what I witnessed was horrific, there is no other way to explain it. To watch your child completely lifeless with the only good thing being is she is still breathing is something that should never have occurred.

Sarah has had this condition since birth. Low bloods sugars are apart of daily life for us. In the 18 months since she first came home she has never fallen into an unresponsive state.

I guess I cannot understand why her blood sugar was not checked at any point while she was in there care. She is on an insulin pump which makes it fairly obvious that she is a Diabetic and I had a conversation with at least 3 theatre staff about the importance of monitoring her.

Being that Sarah has no Peripheral IV access left, we are left with the prospect of having to go to theatre for a central line should she become ill again. We have been very lucky this year and have been able to isolate her and she has avoided the nasty winter bugs, but it appears that she has picked up a bug on the ward this admission, so its likely that we may be facing the prospect within the week of having to bring her back in, if the infection gets into her chest as it has in the past, the only thing that has healed her has been IV Anti’s which puts us in the position of again placing her in the care of theatre staff and praying that they don’t repeat this mistake.

The consequences for Sarah could be devastating and its myself and my husband that are left to repair the damage. I do not like to or want to come across as a difficult parent, but Sarah cannot yet advocate for herself so it’s my job to do it, but I feel very strongly about this incident

Sarah sees multiple teams at the RCH (Neonatology, General Medicine, Endocrine,Gastro,Cardiac) and I have been so frightened by this that I am literally petrified of her needing admission to RCH again. I believe an incident report has been submitted and I am writing to inform you of my feelings as I would hate for this to be swept under the carpet and for it to occur again to either Sarah or another patient with devastating consequences.

Welcome to NICU

I was reminded today that we are approaching our 2 year Diabetes "Anniversary" and its allowed me to do some reflections.

As I walked around cleaning the house today, the music on, Sarah plodding around the house getting into everything as I was putting it away. I stopped and just stared. My mind going back to those early day, the tears, the excitment, the nerves, the anticipation, the uncertain times, the sadness and the happy days.

In alot of ways when Sarah was born I was so overwhelmed by what was happening. I didnt even have time to absorb that fact that my Ob had just walked in the room and said "I need to get this baby out she is very sick", and that was it, people rushing everywhere.

Then I wake and am told I have a daughter, a very small daughter but she is alive and in NICU. I dont remember seeing her that night, I was wheeled through NICU on the way to the ward, but I was so drugged out all I could see was bubble wrap.

I do remember a few hours later when I was slightly more awake talking to a Dr from NICU. She explained that Sarah was doing as well as could be expected and she was on CPAP but coping extremely well given her tiny size. I also remember calling quite a few times overnight to check on her. I had quite a few friends and family that were calling me that night, probably trying to keep me pre-occupied and my mind off things and I will forever be grateful to those people.

The next day was Saturday. Michael bought the kids in. In the morning I begged the midwife to stop the Morphine drip and remove the Cathetor, I didnt care how much pain I was in I desperatly wanted to see my baby. What if things were worse than I was being told? I had to see her for myself. I didnt want anymore polariods, I wanted my baby. I HAD to lay my own eyes on her.

I did go and see her on the Saturday. I remember sitting there next to her, to scared to touch her but just staring. I had no idea how something so small could survive what she had, but what was breaking my heart was how was she going to keep fighting, she was so tiny. The photos really didnt do justice but I am not kidding when I say her fingers and toes where no bigger than grains of rice. Her skin was almost transparant. Her eyes still fused closed.

That day she was taken off CPAP and onto Low-Flow Nasal Prong oxygen, what I was to later learn is this is a massive step for a child her size and in her condition and there she was showing them all.

Again the Drs spoke to me. It was Dan Cazalaz, such a sweet caring Dr. They had concerns about her gut. Her abdo has become distended which is not uncommon on premature babies but its generally caused from feeding, so the treatment is to drop the volume of feeds down, only problem here was that they hadnt yet fed her. So they spoke about Anti-biotics and this was the first I heard mention of her having high blood sugars and requiring an insulin infusion.

The nexy day Sunday is where we see it all go wrong. Her abdo continued to swell and she went into respitory distress and they needed to ventilate her. I think that memory will stay with me forever. I had a friend come visit me in the hospital and I walked in on the Drs in the process of ventilating her. Drs everywhere, they were doing more xrays and this was the first time I cried since she was born. Only a few hours later Sarah would be on the way to the RCH for what was going to be a long road to home.

Arriving at the Childrens hospital was a surreal experience. Michael had gone with Sarah and the NETS team to the Childrens and I was left back at the Mercy really with no idea how Sarah was. She was assessed by the Neonatiologist John Mills upon arrival and he briefly spoke with Michael. Things didnt look to good and they would do everything they could. I discharged myself from the Mercy and arrived at the childrens at about 2 in the morning where I would stay for the next 7 days.

I met Dr Mills the following morning. We sat down with John and another Dr who would be her general surgeon. They told us that Sarah looked to have a condition called NEC which is a condition in which the wall of the bowel becomes infected and "dies" the treatment is surgery to remove the dead colon, it has a high mortality rate and not every baby with NEC survives.

I remember walking into the hospital that night thinking "hey once they fix her bowel, she just needs to grow and will be home before we know it", Id do anything to go back to that now.

Day 9 became the day she was operated on, it was the longest 5 hours of my life. To know that they are operating not only on your 9 day old baby, but a baby that should still be growing inside you and is so tiny you cant even understand how they are going to manage to find her "colon" is one of the most terrifying experiences. "Was she strong enough", would she make it through?

Sarah was actually operated on at the bedside in NICU, and I think thats when I relised for the first time that we had a very sick baby, to know that she was so unstable that she couldnt be moved to theatre for the operation and that theatre would be coming to her.

I had met 2 very lovely mums in NICU, 2 mums that to this day we are pretty much in daily contact. We all had a very long road home and all our children whilst all have different complication we very much travel this continued road together. We cry together, we laugh together and we even scream together. All 3 of our children have had multiple surgeries and to know that when we are travelling a hard road, either one of us can pick up the phone and know that they understand and they get it is comforting.

There were several times in the first few uncertain weeks were I am positive the Drs didnt know if she would be there tomorrow, the problem was they simply didnt know what was wrong with her and that was a massive hurdle. When that surgery was in progress I remember seeing the Neonatiologist being called into her room and looking back it was obvious why now. The problem with treating Sarah was they couldnt believe that they were seeing let alone determine how they were going to save her.

It was late December when I met with Dr Mills again and he explained that there early suspicion was that Sarah had Cystic Fibrosis but during the surgery they were unable to locate her Pancreas and Gallbladder. He continued on by saying "Ive been a Dr for over 30 years and not only have I never met a child born without a Pancreas Ive never ever heard of it happening. After her surgery he contacted his father in the UK who is also a retired Paediatrician who had never come accross it either. He thought the most probable diagnosis was going to be Neonatal Diabetes and he explained how hard it is to stabilise diabetes in babies, let alone babies that were born at half there expected size and 9 weeks early.

So we went into Christmas with the faith that she did have a Pancreas, it was just that it was underdeveloped. Over Christmas however Sarah started going downhill at a rapid rate and she had also developed Nec for real this time. Thankfully they were able to treat it medically and she didnt require the removal of more of her Colon.

We met with John again in the early January and this is when we got her offical Diagnosis of "Pancreatic Agenesis" . He explained that along with No Pancreas and No Gall Bladder she also had a heart defect and a Abnormality of her Bile Ducts and her tests were showing that her Liver was failing. God Bless this man because he was so upfront with us. He told us that day that he didnt think she would survive and it would be a miracle if she ever came home.

The next few months really were at a standstill for me. I was grieving alot, grieving for what should have been, what was to come, what wasnt to come, but I had to remain strong for Sarah. I was really optimistic even when her Drs wernt. I felt if I lost that optimism then I would crumble.

I think too I was grieving for others. The thing with the RCH NICU is it is so very different to the other lvl 4 NICU's. It became apparent very early that there was only 3 reasons your baby was tranferred here.

1. Your child needed Surgery

2. They didnt know what was wrong and things wernt looking good

3. Palliative Care

In Sarah's 14 weeks there we met and said goodbye to 12 little angels. One that deeply touched my heart was little Cleo, she was born the day before Sarah at full term and Sadly she passed away 4 weeks after we achieved our Miracle by bringing Sarah home. Cleo would never leave the 4 walls of NNU, she would never have sunshine on her face and that broke my heart as only weeks earlier Cleo was getting ready to be discharged home.

So I sat there today watching Sarah pulls toys out right after I packed them away, I picked her up and I just sat there looking into her eyes, and giving her a massive cuddle.

I havnt slept more than 5 hours in one stretch since she was born. Some night we dont sleep at all. Her diabetes is very hard to manage, as hard as we and her medical team try. We do what we can for her.

But by all accounts for me to be sitting here reflecting on this, talking about how Sarah was into lots of Mischief today is an absolute Miracle in itself.  At the start of this journey I didnt know how she was going to be able to breath on her own, I think I can honestly say I dont think that paed John Mills did either, and without him I dont think I would have been writing this. He searched high and low, he worked around the clock, when he got over the shock of what they had discovered he knew he was racing the clock to save this baby and he not only saved her, she in her own way is thriving.

Everynight I say a prayer for all of Sarah's little friends and there parents and I send some angel dust up to her friends who wernt able to stay but know that they are now at peace and will likely live in Sarah's heart forever.

I recently put this slideshow of Sarah together, if you have time pop over and have a look.

http://www.onetruemedia.com/shared?p=b797a26d0fd238564a921d&skin_id=1901&utm_source=otm&utm_medium=text_url

Sarah's 18 Month Growth and Development Assessment

Ive just received Sarah Growth and Assessment report in the mail and Im pleased as punch! It reads as Follows. 

I reveiwed Sarah in my medical outpatient clinic today with her Mother Karina. Sarah is now aged 18 months and has had a relatively good period in recent times. She has had a couple of months without incident, but over the last month there have been a few annoying problems such as an episode of of gastroentroenteritis, followed by an episode of tonsilittis that seemed to roll on to some upper respitory infection with cervical lymphadenitis. This all settled down then came an episode of Apnea with associated peri oral mottling while she was asleep. She had episodes like this about 12 months which I believe were very similiar and probably the same mechanism.

Sarah has had some seizures in the past and has had a videa EEG which was normal. Karina does have Buccal Midazolam at home to be administered should she have a prolonged seizure lasting more than 5 minitues.

Developmentally Sarah is doing Brilliantly. Her communication system is evolving well, with 5-10 single words, a lot of babble and good verbal responsiveness to verbal commands emerging. She commando crawls for effectively now, and sits stably. She pulls to stand and is beginning to cruise the furniture. She eats everything and is sleeping quite well.

The Endocronologists of course, are monitoring her diabetes and her recent HbA1c in July was 9.6, and I understand that her basal rate has been increased to try and bring this down a little. She is having some hypo's but these havnt been terribly troublesome.

Sarah is about to commence childcare two days a week which I think will be excellent for her Social development. Karina is trying to access an aide and I have sent a letter of support.

Sarah looks excellent today. She was happy and interacting delightfully, and played at a developmentally appropriate level on the floor. Her weight was 7.5kgs which is an increase of 220 grams over the past week.

Sarah has developed acholic stools (known to have schleriosing cholangitis). She is anicteric and remains well. I have asked for urgent review by gastroenterologists.

t is wonderful to see how well Sarah is doing. I am on sabatical for a few months and I look forward to seeing her again when I return.

 EB Bloggers
Powered By Ringsurf

The Dilemma of a Visit to the Emergency Room

One of the hardest things of having a child with a Rare condition is what happens in an Emergency. In an emergency you dont have time to call your "medical" team and you face the prospect of arriving at the emergency department with your child needing urgent medical care and explaining that your child has a rare condition.

You quite often met with black stares and even with eyes rolling and you wish for just one moment that it was a dream. But its no dream and you are quickly snapped back out of it. You stick to basics to get the immediate problem sorted.

Take a moment and think back to times when you have had to take your child to an ER room, a GP, a dentist. Your Given forms to complete, there is always a section on previous medical condition. Diabetes is always listed there but we always have to tick "other" and list Neonatal Diabetes/Pancreatic Agenesis.

I always try and give a very brief explanation. I say "Sarah has Pancreatic Agenesis, so she has Neonatal Diabetes and complete Pancreatic Insufficiency, she is on an Insulin pump and Creon supplements" Then the Drs, Nurses, students come flying in with questions.

Its a very frustrating position to be put in, you know your childs condition inside back to front, but your in a critical situation most of the time and your in the ER because you can no longer deal with things at home. But yet you stand there in front of Drs and Nurses that not only have never met your child before but they have never heard of her condition. Yet you stand there knowing your childs life is in there hands. You are inredibly powerless in a way.

The point I am trying to make here I guess is about education. Every emergency visit for us is met with stares, we get glances from every direction. We get concerning glances from Drs, from nurses, from cleaners and yes even from other parents in the waiting room.

My child may look no more ill from yours, or she may be screaming at the top of her lungs and no I cant "Shut her up". We may bypass traige and be put to the front of the queue and taken through immediatly but that is because our childs condition can become critical in seconds. Its also because a simple cough or cold can be life threatening for Sarah.

You may be a person that works within the healthcare industry, ot studying in the field. I know sometimes we can come accross as being "difficult" parents, we dont get up each morning with the aim of making your day harder but as the parent of a child with a rare condition it is our job to make sure that our child's needs are met. We dont expect you to have a full understanding of there condition because lets face it, you are unlikely to come accross it again, but work with us. We live with it 24 hours a day 7 days a week. Talk to us, explain things to us. I am always happen to educate people on Sarah's condition, but understand when you show fear in your eyes, when you hesitate, when you become nervous you are making me even more nervous.

As a parent I will never growl under my breath whenever I see a child come to emergency not looking an worse than my own child who has been waiting for hours to be seen already go immediatly through for assessment.

Often the real dangers are hidden under the surface

Unchartered Territory

Firstly I apologise to my blog followers for not updating in so long. As you can imagine from our Journey so far, life is shall I say interesting for us. There is barely a dull moment in this household!
I really havnt blogged much about my other children as I guess the purpose of this blog is to put down my rare feelings and emotions on life with Sarah, firstly it was a pregnancy journal and then well it turned into a place were I could blurt out my fears and then our journey in getting her home.
Our Eldest Child Kevin also has a Medical condition, a heart condition to be specific. It was never detected at birth and in fact wasnt diagnosed until he was 7. It was a very frustrating time. He had just been Diagnosed with ASD (Autism Spectrum Disorder) and his paed noticed he had a heart murmour, off we went for tests to learn that He had a Hole in his heard and the plumbing of his heart wasnt quite right. So he had a Atrial Septum Defect and PAPVD.
We then got a rude awakening to the health system in Australia and the Red Tape, I was tearing my hair out. There were waiting lists everywhere, infact the waiting list for a MRI was 5 months! It was ludicrious and I was furious.
It took us over 12 months to navigate the red tape and waiting lists and after 5 cancelled surgeries we finally had a date. Tuesday the 16th December 2008 he would have his surgery. It was going to fit in great, we could get him over his surgery by the time our baby arrived at the end of January.
Well as you know that didnt quite work out so well and with Sarah in NICU, we made the decision to cancel Kevins Surgery, somehow the idea of Sarah in NICU and Kevin in PICU wasnt so appealing.
So Kevin Finally underwent his surgery in Feb this year. It was a very stressful day, his surgery was expected to last 6-7 hours but instead it went into the early hours of the next day and it had taken them 12 hours. He however was a champion through the initial recovery and was discharged home after 5 days!
I tell you though watching 2 of your children undergo Open Heart Surgery within 3 months of each other was quite draining, as a parent its one of the hardest things you do. You hand your child over to a surgeon and until they surgeons emerge from theatre you have no idea what is going on. When Kevins surgery went so far over the expected time I become more and more anxious by the hour. I was relieved once I was able to get into PICU and see him. Although its so different seeing your baby on a Ventilator compared to seeing your 10 year old on a Ventilator.
It was also a time of stress because as you know Sarah's medical condition continues to be precarious and she can go downhill rapidly requiring admission to hospital. I had no idea how I would cope with both in hospital on Different wards and still 2 more at home to care for, thankfully it didnt happen and Sarah was farily stable at that time.
So onto Sarah. Im so happy to advise that we actually havnt had an admission for about 4 months now, we actually had a great run of 13 weeks where we didnt even have to take her to the emergency department!
Someone asked me recently is she more stable? I dont think she is, I think its more that we now know how to treat very complicated situations.We have dealt with almost every Diabetic complication there is, and its easier to treat her at home than in hospital.
In April Sarah Featured on the Good Friday Appeal as a follow-up story to the 2009 story. You can have a look at the link here Good Friday Appeal 2010   May and June were pretty uneventful and we just juggled the day to day complications at home, some days hairer than the day before, but we somehow managed and I can tell you it was such a relief to have some time alone, without the hospital admissions and the hospital appointments and this and that and this and that, I felt for probably the first time that I was able to be just a mother and not a Mother, Nurse, Carer.
July has been a very challnaging month. This month we have seen gastro, tonsillitis a viral ear/throat infection and Bronchiolitis, including our first experience with inserting an NG tube!
We seemed to be able to limp her through all that without an admission and I was very impressed with myself.
Then last Thursday Sarah had her Checkup with her Paed at the hospital. Now firstly her Paed is second to none. He isnt only her Dr either, he advocated for Sarah around the clock. He is very aware of our family unit and my desire to keep her home whenever possible.
So at the checkup he was very impressed, the last appointment was wasnt really sitting unassisted and this time she isnt just sitting on her own, she is now even crawling!!!!!!!!!!!!!
I'd discussed my concern with him about her Color, her skin has become very pale over the last 6 weeks and I was concerned that maybe her haemaglobin was dropping to low levels again.
He agreed that she did look pale but given her last FBC her levels were good at 126, he didnt want to have to draw blood if he didnt have to, so we decided to wait it out and see if her color got better over the next few weeks.
So as I was leaving, very happy that we got such a good report I asked one of those "by the way" questions.
I had noticed over the last week that her Stools have been very pale. Think the color of a Manila folder or the color of cream and yep thats the color Im talking about. Id only asked him as I had wanted to send off a sample to check her for Malabsorbtion.
Well he said can you go and change her and bring me a sample, but I hope its not as white as you are saying it is, if it is this could be serious.
So I bring the sample back, he takes one look and picks up the phone to page our Gastro Consultant.
So he explains to me that even by looking at the sample he can tell me its not malabsorbtion but what it is, is a problem with her Liver. There is no Bile in it and if there is no bile in it, the bile it pooling in her liver causing irreversable damage.
Many of you would remember that in Decmeber Sarah had an MRI which picked up a problem with her Liver. The condition is Primary Schloising Cholangitis. Its basically a condition where the bile ducts are malformed and the restrict the flow of bile. There is no cure for the condition, the only option is Liver Transplantation. However is mostly strikes people in their 30's-50's so there is very little data of Paediatric patients.
We were sent for Bloods on Friday which confirmed there appears to be some type of blockage in her bile ducts and the most likely explanation is that they have collapsed.
She is booked in for a Lover Ultrasound and Biopsy tomorrow morning, once that is done we will have a far clearer picture of whats going on, but her stools still remain white and she has now started itching all over, not a good sign. The most likely solution will be surgery this week to place stents in her Bile Ducts and start the process of Liver Transplantation.
And I guess that is what brings us into unchartered Territory, all the way through this journey Ive been able to have alook into a Crystal ball in a way. There is a little boy accross the tasmin that has the same condition and he is 6 months older than Sarah. But Finlay doesnt have ther Liver Condition and of the surviving children Sarah is the only one to survive with Liver problems, so we are on our own on this one.
So please say a prayer tonight for our little girl. I dont expect good news tomorrow, just have that feeling. But what I do know is we will fight this head on whatever the future holds for us.
 EB Bloggers
Powered By Ringsurf