Matty | February 4, 2009 - March 31, 2023
During my pregnancy with Matty we were in and out of hospital for various reasons. During a routine scan it was identified Matty had Talipes, otherwise known as a Club foot. We were assured this was isolated and not in relation to any other conditions. The pregnancy progressed to term and we were in and out of hospital for observations because Matty’s heart rate didn’t always fluctuate as it should, the doctors said he seemed a little ‘unwell’ and continued to monitor. He was born at full term very quickly in a couple of hours.
Matty was our second child so I already had awareness of what I expected that first night. I noticed Matty didn’t have a strong suck when feeding and struggled a little to latch on. I kept this to myself in an effort to get us back home and put it down to being overtired. As the days continued we noticed Matty slept all the time, and he didn’t seem to coordinate feeding well, the process of suck – swallow – breathe didn’t seem to come naturally. He was also not putting on weight, noticeably floppy and tired and not tracking our faces well, his vision seemed impaired in some way.
It was at this point that our journey started into the medical world of appointments, tests, procedures and investigations, and I gave up my career because the sheer number of appointments with various professionals and hospitals meant it was impossible to work.
Matty started treatment for his Talipes, using the Ponseti method of casting the foot and gradually changing it’s position week by week until corrected.
Matty had a feeding tube inserted and went onto a special milk which was kinder for reflux.
We worked hard with Occupational Therapists and Physiotherapists to help progress Matty towards key milestones and gain some core strength, he never did unfortunately meet the expected milestones and rarely made progress. We signed up to classes to help with development of motor skills, coordination and strength. Equipment started to arrive in our home, feeding pumps, medications for reflux and seizures, supportive systems for bathing, sleeping and sitting.
As our journey continued we still didn’t have a diagnosis for our darling boy, many things were looked at but nothing could be found. He was referred to as a SWAN (Syndrome without a name). We knew he had a rare disorder but technology hadn't yet caught up yet to find out what it was.
We were in and out of hospital with chest infections due to silent aspirations when feeding, despite having a nasal feeding tube. Matty had a procedure called a Fundoplication to help with his severe reflux and had a gastrostomy feeding tube inserted and the nasal feeding tube removed. Matty fell unwell after this procedure and suffered from sepsis and multiple organ failure. He was ventilated and transferred to ICU in Southampton. He underwent emergency life saving major surgery and had 30cm of his bowel removed. It is believed he experienced a necrotic bowel, we are unsure why. He had 2 stoma’s inserted and was in a grave position for many weeks whilst he recovered. To this day we still don’t know why he suffered the necrotic bowel, however we suspect it was some complication from the Fundoplication surgery the previous day.
After weeks in hospital Matty started to recover and grew stronger on some new milk which was easier for him to digest. After 3 months of living with an ileostomy stoma this was reversed with an operation.
From this point onwards our darling boys pain started. We are not sure whether this was due to a dysmotility of his bowel or whether it was due to bowel adhesions and scarring, but over the next weeks, months and years pain was a continued part of his daily life managed with medication but often unsuccessfully. We were in and out of hospital for suspected bowel obstructions where Matty would vomit dark green bile and have to be Nil by mouth for a few days/ week whilst he recovered. He had a severe gut dysmotility and had to have strong laxatives to help with this, he would become heavily constipated and we would eventually have to perform manual rectal bowel washouts at home with a catheter after enema’s became unsuccessful.
In 2015 Matty developed acute pancreatitis, with pseudo cysts, liver lesions, splenic collections, pleural effusion and collapsed lungs, he was in a bad way. He required a long term admission to great ormond street for 2 months to have interventional radiology treatment as pancreatitis in children is extremely rare and no other local hospitals were specialised to deal with Matty. We are not sure why this happened, Matty never followed any of the rule books! The team at the hospital suspected a medication may have caused this. After this acute episode Matty suffered with a great deal of pancreatic pain and eventually suffered chronic pancreatitis which needed treatment and procedures to keep bile ducts open to prevent blockages. The main cause of Mattys pain we believe was his chronic pancreatitis and gut dysmotility, as well as urinary retention which would eventually require us to be trained to catheterise him daily along with his regular bowel washouts for constipation.
Matty suffered many chest infections over the years and admissions to hospital for pneumonia, this would eventually lead to a dependency on oxygen at night times whilst sleeping.
Over the years Matty became highly medically complex, living in and out of hospital with open access and more and more conditions being added to his already very long list. His daily medication and feed regime was complex. We as a family became medically competent in all of his complex care and had a number of equipment items usually seen in hospital so we could keep him at home more.
Despite all this he continued to smile and laugh and enjoy life when he could, he absolutely loved swimming, hot tubs and baths and playing with sensory toys and music. We tried hard to constantly improve the quality of his life and always tried to 'put the ability into his disability'.
We took Matty to America for a special Make a Wish to Disney which was magical, we stayed at a resort called Give Kids the World, the memories we share from this experience will last us a lifetime.
Matty was non-verbal and a full-time wheelchair user all of his life but he was able to communicate to us in other ways, either with tapping and patting or noises we became familiar with. He went to a very specialist school which we fought hard for him to attend which was just wonderful, here he would horse ride, swim, trampoline and be well cared for and looked after during the school day. Matty loved being warm and cosy and loved Christmas time with all the lights and sensory experiences. Matty was a sensory seeker and loved chewing and exploring with his mouth.
Sleep was an issue for Matty, he could never switch off so needed medication from an early age to enable him to get some rest. He was highly complex and we needed a care package which gave us support at home with paid carers we employed, who became like family to us.
Matty’s list of conditions were long, and he was very medicated as the years went on. His main conditions included:
Retinal dystrophy (registered blind but with some vision although not functionable)
chronic pancreatitis
Seizure disorder
Severe gut dysmotility
Diabetes
Heart murmur
Aspirational lung disease requiring night time oxygen
Unsafe swallow requiring regular suctioning
Autonomic dysfunction which meant he experienced low blood pressure and couldn’t regulate his body temperature
Stereotypies and compulsive behaviours
Sleep disorder
Dystonic movements
Scoliosis
Hip migration
Matty was 11 by the time we received the diagnosis of Baker Gordon Syndrome. He had been placed on a project called the 100,000 Genomes many years earlier, and after years we received the diagnosis of a genetic mutation, SYT1. We knew it was extremely rare and at the point of diagnosis he was one of about 13 worldwide known to have this rare disorder.
Matty’s health started to deteriorate over the years, particularly with feeding, eventually he had a Jejunostomy inserted so that he could be fed further down into his Jejunum rather than his stomach. Matty had a severe dysmotility in his bowel which meant feeding was extremely painful and gaining weight became an issue. He would experience severe pain whilst feeding and we would have to attach a bile bag to his stomach whilst he fed to relieve air and pressure, Matty fed for 20 hours a day connected to a pump of just 75ml and hour but even this was too much, sometimes we were unable to feed him due to pain and he would have to go into hospital for fluids. Again, we were in and out of hospitals all over the country, especially at major London hospitals regularly, to include Great Ormond Steet, Kings College and The Evelina, trying to help with both the pain and feeding issues as well as emergency treatment when he became dehydrated.
Over many years of sub optimal weight gain (not even on the 1st centile chart) and continued pain it became apparent we needed to consider TPN – Total Parenteral Nutrition. This requires a central line to be inserted and to be connected to an IV line feed for many hours in the day. Matty had received TPN before after his necrotic bowel surgery and I was not keen to go down this route as central lines come with a risk of infection and sepsis. Eventually in Jan 2023 my hand was forced in the end as Matty was 13 years old and only 21kg, he was 23kg back in 2015 at 6 years old, this shows how severely underweight he was. We went to hospital to have the central line inserted and to start the TPN Feed, which meant being in hospital for at least 6 months, we would then continue this feed at home after we had received training.
Matty had the line in a week and became septic with an infection from the line, he was ventilated and put on life support and transferred to Great Ormond Street hospital. After a week he came off the ventilator and started his recovery from this septic episode. The central line was removed and another one placed in his arm called a PICC Line. He continued his journey of TPN and started to gain a little weight, he had his 14th birthday in hospital, however I was not seeing the improvements with Matty that I had hoped we would see. He seemed pale and weak still but he had been through a lot.
We were still in hospital in March 2023 when Matty acquired another line infection and required ventilation with full life support and a transfer back to ICU in Great Ormond Street. He was septic again and suffering sepsis and multiple organ failure. We had only been at Great Ormond Street a couple of hours and Matty suffered his first cardiac arrest infront of my eyes. The team of medics got him back after an agonising 20 minutes of resuscitation. Over the next hour he battled 4 more cardiac arrests, the 3rd they managed to get him back again but on the 4th it was futile, our darling boy had died on 31st March 2023 at 9.30am.
His death certificate says Sepsis, Mutiple Organ Failure and Baker Gordon Syndrome as a contributing factor.
We are absolutely lost without him and we will never heal from this loss of our darling boy. Matty taught us so much throughout his life and we continue to be grateful for having him with us for the 14 years that he was.
Matty’s smile could light up a room and his infectious little giggle, we miss him desperately, beyond words, and we always will.