The Girl Who Only Knows Life With Tubes

January 2015 family have been living under a lot of strain since the birth of their little girl Hannah Jones in June 2012. 

Her parent’s Katy and Ryan Jones live  in South Shields with their other two sons 8 year old Stephen and 3 year old Joshua. 

I write that they live in South Shields but sadly this family have been living separate lives since their amazing little girl was born, 80% of this little one’s life and her Mother Katy has been in the RVI or Birmingham hospital away from the rest of their family. 

Katy knew something was not quite right at 20 weeks pregnant she expressed her concerns on several occasions but was told not to worry.  At 25 weeks she refused to leave the hospital as she could not sit down at all, the pressure was so severe, at this point the doctors confirmed she was 5cms dilated and their little girl was born the next day weighing 1lb 9oz!

 

Hannah was very poorly she was unable to breathe on her own and was placed on a ventilator 20 minutes after being born. She had a tube fitted in her chest to drain the fluid and also suffered a bleed on the brain which caused Cerebral Palsy.   Such a worrying time for this family Hannah was so poorly it wasn’t until Hannah was 6 weeks old that her Mummy and Daddy could give her a cuddle.

Such a lot for this little body to suffer, at 5 months old she endured theatre to have a shunt placed in her brain as she suffered from Hydrocephalus :- a medical condition in which there is an abnormal accumulation of cerebrospinal fluid (CSF) in the ventricles of the brain.  This causes increased intracranial pressure inside the skull and may cause progressive enlargement of the head if it occurs in childhood, convulsion, tunnel vision and mental disability.  This was so severe in Hannah that she was operated on.  However in theater she suffered a second bleed.  Prior to this Hannah was showing signs of smiling, and her eyes were starting to follow, but after this bleed, this ability had to be worked on again from scratch this small milestone had been lost.

Hannah was weaned off the ventilator onto oxygen and amazingly was weaned off full breathing support by 6 months old.  However the most severe heath problem suffered owed to Hannah’s prematurity is the disease called Nectrotizing Enterocolitis the most serious intestinal disease among premature babies.  NEC occurs when tissue in the small or large intestine is injured or begins to die off.  This causes the intestine to become inflamed or in rare cases develop a hole.  When this happens, the intestine can no longer hold waste, so bacteria and other waste products pass through the intestine and enter the baby’s bloodstream or abdominal cavity. This can make a baby very sick, possibly causing a life-threatening infection.

Hannah’s was so severe that she had to have her large bowel and part of her small bowel removed.  This only left her with 47cms of her small bowel.  This meant that Hannah’s life was never going to be the “norm” she was not able to absorb nutrients out of oral feeds, this meant further surgery for this little girl who had gone through so much already.  Hannah was fitted with a central line this is a long, thin, flexible tube used to give medicines, fluids, nutrients, or blood products over a long period of time.  A catheter is often inserted in the arm or chest through the skin into a large vein. The catheter is threaded through this vein until it reaches a large vein near the heart.  This was fitted into Hannah’s chest so she could be fed Total Parental Nutrition also known as TPN.  This supplies all daily nutritional requirements but does not come without severe side effects and can cause the following life threatening complications;

Infection – Infection is a common cause of death in these patients, with a mortality rate of approximately 15% per infection, and death usually results from septic shock, Blood clots, Fatty Liver and Liver Failure – This is common with long term users – The pathogenesis is due to using linoleic acid (an omega-6 fatty acid component of soybean oil) as a major source of calories.Hunger pains and other re-feeding complications.

At this point of this family’s heart breaking journey, they had lived separate lives, the mother with her daughter in the RVI and the father at home with the boys.  Visiting was not very frequent owed to the risk of infection and the sheer expense of travelling to and from.  This put such a strain on them all.  A small light of hope after Hannah’s central line was fitted, they had the chance to go home, this was after Katy was fully trained in all nursing duties to manage  Hannah’s medical requirements.  Day 289 of Hannah being born Katy brought her daughter home with what seemed like the hospital with her.  The aim was to feed Hannah TPN via her central line Sadly this was not a long stay at home, since discharge Hannah has had a very stormy 2 years requiring 8 surgeries to try and improve her condition.  In 2014 alone they only managed 9 weeks at home, Katy and Ryan suffer terrible guilt not being able to be a full time parent to “all” of their children.  Katy has never left Hannah’s side she is the only one who is trained to take care of Hannah’s medical needs.

2014 was very distressing for them all, Hannah spent 6 months in PICU due to infection that turned septic, they were so worried, their little girl who had fought so much was fighting to stay alive.  The sheer spirit and determination of this little fighter and love from her family, got her through it and she defied all of the doctors!!

However when she was discharged from PICU on to a ward, this little girl had been so isolated she found even cuddles from her Mammy very distressing, she cried and cried.  This broke Katy’s heart, but as with some children who had suffered such trauma, they need re-habilitating back to some way type of normality.

She has had numerous admissions to the RVI and has had her central line replaced several times owed to infection.  In July last year Hannah was referred to Birmingham Children’s Hospital for assessment for a bowel transplant it was here that they discovered that she also had fibrosis of the liver. The doctors from Birmingham hospital have put a plan in place, at the moment Hannah is currently in hospital as they are trying a different way of feeding.  She already has a feeding tube into her stomach which is now fitted with a G Jet which will attempt to feed her milk into the only lower part of her bowel, whilst the other part is permanently on free drainage.  It is heart breaking that this is Hannah’s only way of life that she knows with tubes coming out of her little body.  She is attached to a machine 24/7, TPN being fed through her veins, feed going through to her stomach and a bag that is on permanent drainage.  This is her last hope as if this does not work she will be placed on the list for a stomach, bowel and liver transplant. On top of all of this worry they have been told that Hannah can only accept a match where the donor has passed away from stem cell death and is under 20kg.

Hannah is extremely vulnerable partly due to the fact that the RVI are unable to access veins to fit anymore Central Lines…this is her lifeline at the moment.  The only Doctors who are able to access the three veins that are left are those at Birmingham Hospital sadly these lines are not lasting long in her little body!  All her parents can do is keep her as safe as they can, each day they are just grateful she is still with them.