Our Pal - Amelia B

Contact Details

C/O 68 Boroughbridge Road
North Yorkshire
United Kingdom

Email Pal

NB: this is a forwarding address for the Pal, we do NOT disclose home addresses

Other Information

  • Parents/Guardians - Lisa
  • Interests - Ipad drawing, Billie Eilish (obsessed!) Meerkats, Descendants, Roblox, Game playing (on tech: X-box and Ipad but currently saving towards both a newer model iPad so she can use a proper apple pen and a Nintendo Switch!) cooking, Oreo chocolate, Maoam sweeties
  • Favourite Colour - Purple
  • Able to read? - Yes
  • Able to use hands? - Yes but restricted movement and weak/fatigued muscles
  • Visually/hearing impaired? - No
  • Suffers from any developmental delay? - No

Siblings - aged 3 to 16 wishing to receive post

  • George 24/09/2006
    • Interests:
      aming, (X-box) Minecraft, Airsoft, Model making and building, Junk modelling, loves learning things on space, aircraft and trains and has an incredible recall of factual info in these areas. He has also built himself a model railway on a track board. George is a fan of white chocolate and also partial to Maoam sweets too.
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About - Amelia B

Story Written 2019

Amelia is known as a SWAN – Syndrome Without A Name. She has multiple, complex medical conditions resulting from a neuromuscular disorder but as yet no unifying diagnosis. She is cognitively typical.

Shortly after birth Amelia was diagnosed with congenital upper limb arthrogryposis which caused contractures and restrictions in her upper body. She had less than 20 degrees range of movement in each arm, an obstructed airway and feeding difficulties as well as reflux.

She was initially referred to orthopaedics and physiotherapy then rapidly to paediatrics, dietician, speech and language and onwards to Great Ormond Street for multiple assessment, diagnostic and genetic testing.

Due to severe reflux, repeated chest infections and failure to thrive, aged two she had surgery to place a gastrostomy feeding tube and a Nissen’s fundoplication wrap to prevent refluxing into her lungs.

Aged three, Amelia was still failing to thrive and further investigations diagnosed gastroparesis (delayed gastric emptying with a wide range in severity of symptoms), a duodenal ulcer, multiple food intolerances and her diet was dairy, egg, soy, wheat and gluten free. We have over the years been able to reintroduce these foods in small amounts with varying degrees of success but limit consumption to prevent pain. Amelia has required yearly endoscopies since 2012 for management and diagnostic purposes.

In 2014, Amelia had her gastrostomy re-sited due to growth and gastric spasms. She experienced post-surgical complications and had to be re-admitted a few weeks later for a lengthy hospital stay and further surgery. Her recovery took over six months.

In recent months, Amelia has again experienced gastric spasms and an investigative procedure revealed this required intervention. In an effort to prevent a further re-site, Amelia went under anaesthesia to trial a different feeding device but unfortunately this has not been successful and we are therefore in further discussions with her surgeon.

Amelia has complex gastrointestinal and bowel difficulties caused by dysfunctional gut movement leading to frequent pain and severe constipation.

In 2016, Amelia had an ACE bowel stoma formed. Unfortunately Amelia’s site prolapsed on three occasions requiring further surgery and from late 2018 onwards, the washout procedures became unsuccessful because of the ongoing difficulties,

Amelia spent most of March and April as an in-patient on the gastro-intestinal ward at GOSH recently and in May 2019 had a colostomy procedure.

Amelia wears orthotics in shoes to minimise pronated (rolling) gait but Amelia’s ankles have become restricted in range in the last 6 months so she now requires overnight leg splints which have to be fitted before bed. She also wears arm splints overnight which we hope will prevent the need for surgery in later years. She requires daily physiotherapy on upper and lower limbs. This takes 15 to 20 minutes twice a day. Amelia eats small amounts orally but requires overnight feeds and at times daytime feeds to maintain growth. She has an unsafe swallow when fatigued and is at risk of choking so requires constant monitoring when eating/drinking. Past choking episodes have led to hospital admissions and airway/lung investigations under anaesthesia.

Amelia attends mainstream school with practical and physical support. She is being taught to use a scribe, dictate and work on a computer to aid her school work and minimise her tiredness. Amelia is a part-time wheelchair user due to muscle weakness, fatigue and pain. She is hyper-mobile in lower extremities which causes joints to extend beyond their range and can lead to dislocations.

Amelia takes over 10 different medications up to four times per day depending on type/timings. She needs support with all aspects of dressing and undressing although this varies depending on her fatigue levels. On “bad” days she will not be able to dress herself at all. On “good/moderate” days she may only need support with buttons, zips, shoe fastening etc.

In recent months Amelia has struggled emotionally with being different to her peers, not being able to keep up with her friends and being tired. With the help of the community nurses and GOSH, we are seeking psychological support for her.

Above all else, she is an amazing young lady; wise beyond her years, compassionate, caring and vivacious. She loves drama and singing and is an excellent mimic.

Update 21st August 2020

After a lengthy admission at Great Ormond Street from May through July this year, Amelia has had to be brave and mature beyond her years. She lives with chronic pain which the gastroenterology team have explained cannot be completely controlled unfortunately as the stronger medications interfere with gut function. Her days are taken up with multiple medications, fluid balances and physiotherapy regimes. Night times are interrupted by medications, pump feeds and frequent wakings due to pain. In spite of all this, Amelia remains her feisty, funny and determined self (depending on your perspective and if you happen to be her parent, this can be both a blessing and a curse! 😁)
Hospital appointments continue to roll in fast and furiously, albeit mainly conducted online. When it has been necessary to attend in person, we have been very impressed with the measures taken by the NHS to safeguard and protect all. Thank you.
September 2020 sees the start of secondary school. Amelia is excited but also nervous, (as am I!) but no doubt her peers are feeling the same. We know she will tackle this with her usual aplomb and our thoughts and good wishes are with all in this situation…..especially Amelia’s teachers: May the force be with you!!  
I can’t pretend I am not nervous with COVID still very much present but balancing mental health, social and emotional needs and education alongside physical health is so vital and important, especially in this transitional year of secondary school.  We may have to review attendance as the year progresses but we are focused on the positives. 

Update 13th July 2020

A huge thank you to all the generous, kind and thoughtful people who have taken the time and trouble to send happy mail in all forms to Amelia & George B. It has been especially appreciated during COVID19 as no doubt like many other families, we are shielding. 

Amelia has had another lengthy stay in GOSH and we have only just come home. This admission has been very hard for many reasons and COVID precautions have meant that the usual distractions of visitors, therapy dogs, entertainment teams, even hospital schooling(!) have been much missed. On top of that, we are trying to get to to grips with being told that this is the best things can be and lower our expectations of Amelia being pain free. A hard thing for anyone to deal with, never mind at the age of 11.  Nonetheless in her indomitable way, Amelia will if not “Keep Calm,” certainly “Carry on!”       

Update 27th March 2020

Amelia has attended a number of appointments at Great Ormond Street in recent months. Her neuromuscular review was a mixed outcome: she has lost more movement range in her upper limbs with more contractures and joint immobility, developed spinal asymmetry, although fortunately this is mild at present and will continue to be monitored. 

Her stamina/power has reduced further and we are trying to focus on building up small activities where we can help her with this but it is tricky as muscle weakness and fatigue are sending her round in circles. The physiotherapist has suggested that we look at piano/key-board play to help with hand/arm therapy…we don’t have anywhere to put a great big one so will be looking into options for something portable….and ear plugs!! 😁   

Gastro issues remain problematic but Amelia keeps smiling. Thank you to everyone who gets in contact with us 🙂 and does so much for the children. Very greatly appreciated.

Update 1st October 2019

The monarch feeding device Amelia had placed in July to try and prevent needing a 3rd gastrostomy re-site has not worked out as planned and she is still experiencing pain and gastric spasms. We meet with her surgeon late October to see what thoughts are now.  Despite the ups and downs, Amelia remains her usual sassy self….which is both a good and a not so good thing depending on when she is giving out the sass!

George is still having lots of issues with his PEG-J and we meet with tissue viability and gastro nurses next week.