Help Aidan Fight Rare Disease
Donation protected
Aidan is an 11 year old boy who suffers from Hollow Visceral Myopathy (HVM), a rare genetic condition with less than 2000 cases worldwide, more commonly known as Chronic Intestinal Pseudo Obstruction (this is the non-genetic version). This condition affects the motility of the smooth muscles of the digestive tract, and each case presents slightly different to the next person. This was diagnosed at the early age of six months and Aidan has had numerous hospitalisations and complications stemming from this disability.
Aidan at School
Aidan was diagnosed after an x-ray at the Tamworth Rural Referral Hospital, our local hospital, showed his bowel was backed up into his stomach, he was admitted to the hospital, where a colon wash was undertaken, this proved unsuccessful and we were flown to Sydney Children’s Hospital, Randwick for further testing and care. Aidan left hospital one month later, with a large quantity of medication, one of which was used for adults in the treatment of constipation but the dose we were advised to use was 4x the adult dose, this increased more and more over time as his bowel continued to deteriorate.
Within two years of this admission we had several more admissions and appointments for further testing, including an endoscopy to determine the function of his stomach. During this surgery, it was found that food eaten 10 days prior was whole and undigested in his stomach – leading to the conclusion of a very slow digestive tract.
At ten months of age it was discovered that Aidan cannot use his bladder to fully void and required 5-6 intermittent catheters per day and 2-3 toilet sessions to attempt voiding and encourage Aidan’s limited bladder function.
At seven years of age, Aidan was found to be sitting very low on the percentile chart despite significant care to his diet to encourage weight gain and as such it was decided that Aidan needed an Nasal Gastric (NG) tube to help his weight become more normal and stable. After twelve months, Aidan weight started to stabilise and he was taken off the NG and put on a strict diet to maintain his weight – this included 6 meals per day and 2 litres of fluid.
Aidan with Nasal Gastric (NG) Tube
In February 2013, Aidan was seeing his local Paediatrician when we were invited to speak to a Palliative Care Team, this sparked a long friendship with a huge amount of support as Aidan’s needs continued to change over the ongoing years. Newcastle Paediatric Palliative Care team, have liaised with a lot of organisation on our behalf helping them to cater to Aidan’s ongoing long term needs and helping us to try and give Aidan some semblance of normality. Due to the distance between Newcastle and Tamworth we often do video conference calls to allow us to monitor and manage each stage of Aidan needs rather than travelling four hours (one-way) to meet with them.
In July 2015, Aidan was admitted into John Hunter Hospital for Pain Management as his condition continued to deteriorate causing unbearable pain. This was a joint admission between the Palliative Care Team and the Pain Management Team, Aidan was able to learn strategies to cope with his pain and to give us tools to allow us to help Aidan when he was in a lot of pain.
On approximately 16 July 2016, Aidan was admitted to the local hospital, Tamworth Regional Referral Hospital, with a Urinary Tract Infection and suspected Pseudo Bowel Obstruction and was administered oral antibiotics to clear the infection. Aidan was released into his parent’s care on 20 July 2016, the day before we had to attend the Sydney Children’s Hospital, for an outpatients appointment to follow up from Aidan’s hospital admission on 13 May 2016, at the Sydney Children’s Hospital.
Aidan at the Sydney Children's Hospital
Upon presenting to the Sydney Children’s Hospital, Randwick, the Associate Professor of the Gastroenterology Department, Dr Arvi Lemburg inspected Aidan and immediately admitted him to the hospital. Dr Lemburg stated that Aidan’s stomach was distended and therefore was unable to properly digest and consequently any oral antibiotics would be useless to treat infections.
During this admission it was noted that Aidan's stomach would bloat to extreme sizes of over 80 cm and that his breathing was laboured due to the pressure of the distention. Furthermore, it was decided that surgery was required because Aidan’s body was having to internally eat itself to gain nutrients. This surgery could not be undertaken straight away due to the low weight Aidan was currently sitting at, creating a high risk to his life; therefore we were required to stay in the hospital for three months during which training was undertaken by the parents, to allow them to manage these early changes to his disability at home.
AIdan's Stomach & Bowel Distention (Bloating)
Due to the lack of motility in the bowel the doctors have had to put in a Ileostomy for bowel function, this bypasses the need to use his bowel and the high risk of pseudo obstructions that Aidan was suffering from prior to this surgery. Aidan also required a Gastrostomy to allow us to feed him as his stomach’s digestive rate was still very poor and he required more nutrition than he was able to absorb from normal food.
Kelly Ritchie, a psychologist at Sensosaurus, has been caring for Aidan since 2015. During the early appointments, Kelly administered a psychological test for Aidan, under DSM 5, which came back positive for depression, anxiety and Post-Traumatic Stress Disorder (PTSD). The PTSD and other mental health issues are a side-effect of his disability. We were seeing Sensosaurus, for Aidan’s mental health and pain management, prior to Aidan being admitted to the Sydney Children’s Hospital, however things spiralled further with his mental health during his time in the Sydney Children's Hospital and the decline of his disability.
Aidan about to fly with the Air Ambulance
On the 17 November 2016, Aidan was air-lifted to the Sydney Children’s Hospital, Randwick, as the surgeries, Aidan had previously, were not effective and further surgeries were required to prevent his disability progressing further. This required a further six weeks of training for Shane and myself. It is a prerequisite that two people must be trained to attend all aspects of Aidan’s care, and as such my husband moved to Sydney for a short period of time to attend this training. As stated before, Aidan lives a life at high risk of heart attack or stroke, due to the high risks and complex nature of his care, stemming from the recent implants that he had to have to save his life.
Aidan cannot eat more than 250 grams of food per day and this is only if his gastrostomy tube is functioning and Aidan has been able to void his stomach through this tube. Due to his inability to digest food, Aidan also needs to be feed through a Jejunostomy feeding tube, but we are unable to get full nutrition through this method of feeding and as such Aidan has a Hickman Central Venous Line (CVL) for addition feeds and fluid.
Aidan's CVL, Gastrostomy, Jejunostomy & Ileostomy
The CVL is tunnelled under the skin before running into the major vein of his neck and fed down towards the heart stopping mere millimetres before entering the heart. This line has a huge risk factor associated with it and puts him at high risk of blood clots, heart attack and/or stroke. The CVL is a double lumen and we run Home Parental Nutrition and IV fluids through it each night, seven nights a week. It can take an hour to set up a CVL, three nights a week, and must be done very carefully as risk of infection or contamination is high.
Aidan with a Stomach (Gastric) Drainage Bag
Very little is known about this disability and the long term effects it will have on Aidan’s body, due to the rarity and variances of this disability we struggle to obtain viable information about what to expect. The impact on Aidan being unable to go anywhere without either parent present and being unable to even enjoy an afternoon at a park due to a lack of proper sterile amenities if anything goes wrong is huge for all of us and Aidan cannot go to friends’ homes or have an outing without strict times being followed – every hour to two hours a medical procedure requiring a sterile environment is necessary.
Aidan's XMas at the Sydney Children's Hospital
Aidan’s disability has had a significant emotional and mental effect on his quality of life and this was exasperated when Aidan was re-admitted to the Children’s Hospital five hours away from his family and friends, except for his Mother. His Father, due to recent surgery to release the right psoas tendon, only a few months before, was unable to stay for longer than a few days at a time, as he was still undergoing treatment from his surgery in their home town.
Aidan was issued with an NDIS package on 28 October 2016. For the first six months of his NDIS plan, Aidan was gravely ill in hospital. This was Aidan’s second long stay at the Sydney Children's Hospital. During this time, Aidan required a large number of life-saving surgeries to prolong and sustain his life. As Aidan’s parents, we were provided over 90 hours of intense training to care for Aidan’s high needs, otherwise he would never have been permitted to leave the hospital to have some semblance of a normal life, including going to school again, of which he had missed a good nine months’ worth of, despite some hospital schooling.
Aidan requires 168 hours of care each week this equates to 24/7 day, per week care — this is not something any normal parent should be undertaking with their children, as it runs the risk of harm to both parent and child when a parent can no longer be a parent but a full time nurse.
Aidan's Daily Chart
One of Aidan's Medical Files at his local Hospital
Aidan also requires that his parents document everything carefully to prevent dehydration, over eating, excessive loss or worse a deterioration of Aidan health. Any hospital admission is a huge load for us, as not only does Aidan miss more schooling but he also needs to be fully supported by us as the staff at the Tamworth Rural Referral Hospital are unable to care for his needs.
Lee Akerman and The Butterfly Fulcrum team, have supported us from the beginning and have done amazing work to make sure we are fully supported and represented in the best way possible. Lee, has advocated hard for us to receive support from the NDIA and once Aidan is financially supported, we have instructed Lee to hire staff to help with cleaning, nursing and general care for Aidan’s wellbeing. He has also hired Michael Lakiss-Smith Night 'N' Day Comfort (a division of Minappi Pty Ltd), to make sure we are regularly supplied with Aidan’s medical needs.
Aidan’s NDIS Plan had been confirmed as being revoked on 07 Sept 2017, and at this stage the family, with the help of Lee Akerman, are relying on the support of the public to help her continue to care for Aidan, until such time as a more permanent arrangement can be made.
Aidan at SchoolAidan was diagnosed after an x-ray at the Tamworth Rural Referral Hospital, our local hospital, showed his bowel was backed up into his stomach, he was admitted to the hospital, where a colon wash was undertaken, this proved unsuccessful and we were flown to Sydney Children’s Hospital, Randwick for further testing and care. Aidan left hospital one month later, with a large quantity of medication, one of which was used for adults in the treatment of constipation but the dose we were advised to use was 4x the adult dose, this increased more and more over time as his bowel continued to deteriorate.
Within two years of this admission we had several more admissions and appointments for further testing, including an endoscopy to determine the function of his stomach. During this surgery, it was found that food eaten 10 days prior was whole and undigested in his stomach – leading to the conclusion of a very slow digestive tract.
At ten months of age it was discovered that Aidan cannot use his bladder to fully void and required 5-6 intermittent catheters per day and 2-3 toilet sessions to attempt voiding and encourage Aidan’s limited bladder function.
At seven years of age, Aidan was found to be sitting very low on the percentile chart despite significant care to his diet to encourage weight gain and as such it was decided that Aidan needed an Nasal Gastric (NG) tube to help his weight become more normal and stable. After twelve months, Aidan weight started to stabilise and he was taken off the NG and put on a strict diet to maintain his weight – this included 6 meals per day and 2 litres of fluid.
Aidan with Nasal Gastric (NG) TubeIn February 2013, Aidan was seeing his local Paediatrician when we were invited to speak to a Palliative Care Team, this sparked a long friendship with a huge amount of support as Aidan’s needs continued to change over the ongoing years. Newcastle Paediatric Palliative Care team, have liaised with a lot of organisation on our behalf helping them to cater to Aidan’s ongoing long term needs and helping us to try and give Aidan some semblance of normality. Due to the distance between Newcastle and Tamworth we often do video conference calls to allow us to monitor and manage each stage of Aidan needs rather than travelling four hours (one-way) to meet with them.
In July 2015, Aidan was admitted into John Hunter Hospital for Pain Management as his condition continued to deteriorate causing unbearable pain. This was a joint admission between the Palliative Care Team and the Pain Management Team, Aidan was able to learn strategies to cope with his pain and to give us tools to allow us to help Aidan when he was in a lot of pain.
On approximately 16 July 2016, Aidan was admitted to the local hospital, Tamworth Regional Referral Hospital, with a Urinary Tract Infection and suspected Pseudo Bowel Obstruction and was administered oral antibiotics to clear the infection. Aidan was released into his parent’s care on 20 July 2016, the day before we had to attend the Sydney Children’s Hospital, for an outpatients appointment to follow up from Aidan’s hospital admission on 13 May 2016, at the Sydney Children’s Hospital.
Aidan at the Sydney Children's HospitalUpon presenting to the Sydney Children’s Hospital, Randwick, the Associate Professor of the Gastroenterology Department, Dr Arvi Lemburg inspected Aidan and immediately admitted him to the hospital. Dr Lemburg stated that Aidan’s stomach was distended and therefore was unable to properly digest and consequently any oral antibiotics would be useless to treat infections.
During this admission it was noted that Aidan's stomach would bloat to extreme sizes of over 80 cm and that his breathing was laboured due to the pressure of the distention. Furthermore, it was decided that surgery was required because Aidan’s body was having to internally eat itself to gain nutrients. This surgery could not be undertaken straight away due to the low weight Aidan was currently sitting at, creating a high risk to his life; therefore we were required to stay in the hospital for three months during which training was undertaken by the parents, to allow them to manage these early changes to his disability at home.
AIdan's Stomach & Bowel Distention (Bloating)Due to the lack of motility in the bowel the doctors have had to put in a Ileostomy for bowel function, this bypasses the need to use his bowel and the high risk of pseudo obstructions that Aidan was suffering from prior to this surgery. Aidan also required a Gastrostomy to allow us to feed him as his stomach’s digestive rate was still very poor and he required more nutrition than he was able to absorb from normal food.
Kelly Ritchie, a psychologist at Sensosaurus, has been caring for Aidan since 2015. During the early appointments, Kelly administered a psychological test for Aidan, under DSM 5, which came back positive for depression, anxiety and Post-Traumatic Stress Disorder (PTSD). The PTSD and other mental health issues are a side-effect of his disability. We were seeing Sensosaurus, for Aidan’s mental health and pain management, prior to Aidan being admitted to the Sydney Children’s Hospital, however things spiralled further with his mental health during his time in the Sydney Children's Hospital and the decline of his disability.
Aidan about to fly with the Air AmbulanceOn the 17 November 2016, Aidan was air-lifted to the Sydney Children’s Hospital, Randwick, as the surgeries, Aidan had previously, were not effective and further surgeries were required to prevent his disability progressing further. This required a further six weeks of training for Shane and myself. It is a prerequisite that two people must be trained to attend all aspects of Aidan’s care, and as such my husband moved to Sydney for a short period of time to attend this training. As stated before, Aidan lives a life at high risk of heart attack or stroke, due to the high risks and complex nature of his care, stemming from the recent implants that he had to have to save his life.
Aidan cannot eat more than 250 grams of food per day and this is only if his gastrostomy tube is functioning and Aidan has been able to void his stomach through this tube. Due to his inability to digest food, Aidan also needs to be feed through a Jejunostomy feeding tube, but we are unable to get full nutrition through this method of feeding and as such Aidan has a Hickman Central Venous Line (CVL) for addition feeds and fluid.
Aidan's CVL, Gastrostomy, Jejunostomy & IleostomyThe CVL is tunnelled under the skin before running into the major vein of his neck and fed down towards the heart stopping mere millimetres before entering the heart. This line has a huge risk factor associated with it and puts him at high risk of blood clots, heart attack and/or stroke. The CVL is a double lumen and we run Home Parental Nutrition and IV fluids through it each night, seven nights a week. It can take an hour to set up a CVL, three nights a week, and must be done very carefully as risk of infection or contamination is high.
Aidan with a Stomach (Gastric) Drainage BagVery little is known about this disability and the long term effects it will have on Aidan’s body, due to the rarity and variances of this disability we struggle to obtain viable information about what to expect. The impact on Aidan being unable to go anywhere without either parent present and being unable to even enjoy an afternoon at a park due to a lack of proper sterile amenities if anything goes wrong is huge for all of us and Aidan cannot go to friends’ homes or have an outing without strict times being followed – every hour to two hours a medical procedure requiring a sterile environment is necessary.
Aidan's XMas at the Sydney Children's HospitalAidan’s disability has had a significant emotional and mental effect on his quality of life and this was exasperated when Aidan was re-admitted to the Children’s Hospital five hours away from his family and friends, except for his Mother. His Father, due to recent surgery to release the right psoas tendon, only a few months before, was unable to stay for longer than a few days at a time, as he was still undergoing treatment from his surgery in their home town.
Aidan was issued with an NDIS package on 28 October 2016. For the first six months of his NDIS plan, Aidan was gravely ill in hospital. This was Aidan’s second long stay at the Sydney Children's Hospital. During this time, Aidan required a large number of life-saving surgeries to prolong and sustain his life. As Aidan’s parents, we were provided over 90 hours of intense training to care for Aidan’s high needs, otherwise he would never have been permitted to leave the hospital to have some semblance of a normal life, including going to school again, of which he had missed a good nine months’ worth of, despite some hospital schooling.
Aidan requires 168 hours of care each week this equates to 24/7 day, per week care — this is not something any normal parent should be undertaking with their children, as it runs the risk of harm to both parent and child when a parent can no longer be a parent but a full time nurse.
Aidan's Daily Chart One of Aidan's Medical Files at his local HospitalAidan also requires that his parents document everything carefully to prevent dehydration, over eating, excessive loss or worse a deterioration of Aidan health. Any hospital admission is a huge load for us, as not only does Aidan miss more schooling but he also needs to be fully supported by us as the staff at the Tamworth Rural Referral Hospital are unable to care for his needs.
Lee Akerman and The Butterfly Fulcrum team, have supported us from the beginning and have done amazing work to make sure we are fully supported and represented in the best way possible. Lee, has advocated hard for us to receive support from the NDIA and once Aidan is financially supported, we have instructed Lee to hire staff to help with cleaning, nursing and general care for Aidan’s wellbeing. He has also hired Michael Lakiss-Smith Night 'N' Day Comfort (a division of Minappi Pty Ltd), to make sure we are regularly supplied with Aidan’s medical needs.
Aidan’s NDIS Plan had been confirmed as being revoked on 07 Sept 2017, and at this stage the family, with the help of Lee Akerman, are relying on the support of the public to help her continue to care for Aidan, until such time as a more permanent arrangement can be made.
Fundraising team: Team Aidan (1)
Lee Akerman
Organizer
New South Wales
Aidan Jachim - Belinda
Team member
