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Thank you to all of you who have helped us out by contributing and sharing Lainey's page. We appreciate everyone of you. I have increased the goal amount to try to further help Natasha & Jimmy out with future travel costs along with fees associated with the PWS Clinic.
UPDATE: Lainey's parents heard from Sick Kids Hospital on Monday. They were hoping to have the appointment on July 26th but they are not quite ready to open yet. The booking staff are now trying to get Lainey in for August 1st, 2020. With everything going on with Covid restrictions, the scheduling is a bit up in the air. Natasha and Jimmy have been given a guaranteed date of August 24th but remain hopeful that they may be able to go earlier.
***************************************Please share this page. Thank you.*****************************************
Lainey Jamieson Hynes was born on her due date of June 25, 2019 at 9:18pm weighing 6.8lbs. Her parents, Natasha Jessome and Jimmy Hynes were completely head over heels in love with their daughter.
After being discharged from the hospital, Natasha and Jimmy went back to the hospital for Lainey to have her hearing tested the following day. While there, they were talking to the nurse about how Lainey wasn’t nursing very much and how she was very sleepy. They tested her blood sugar levels and the results were low enough that they gave her formula right away to see if that would help. Lainey’s levels were tested after having the formula and they were in the normal range. They re-tested her levels after a few minutes and they were dropping faster than they should have. This resulted in her first stay in the Neonatal Intensive Care Unit.
While in the NICU, the doctor's were concerned with Lainey’s low muscle tone, sleepiness and her failure to gain enough weight. She had spinal taps, various blood tests, MRI, ECG, x-rays, and over all monitoring of her heart, oxygen and blood pressure. Lainey’s poor feet had more marks on them from the constant blood tests to check her sugar levels. There were multiple visits from a lactation consultant to see if she could get Lainey to have a full feeding without falling asleep. The second Lainey was close to her mother, she would fall asleep and wouldn’t eat. Natasha and Jimmy would have Lainey in a diaper and use cold cloths on her skin to try to keep her awake. She was very sleepy and it was hard to keep her awake for any feedings, even from a bottle.
There were countless visits from different doctors and specialists. No one was able to explain what was going on. Lainey was finally able to get on track with her feedings and she was gaining weight. The NICU team was happy with her progress and they were sent home.
Three days after being home, Lainey had a follow up appointment the family doctor. Once Lainey was weighed, her parents were told to take her back to the hospital because she lost more weight even though they were following the same routine that was being followed in the hospital. This resulted in Lainey's second NICU stay. While admitted, they did multiple series of blood work that sometimes was repeated numerous times a day, more x-rays and testing. A Geneticist came to see Natasha and Jimmy and wanted to test for a few things so they agreed. Again, Lainey's feeding were going well and she was gaining weight so they were released for the second time.
In total, Lainey spent 2 ½ weeks in the NICU. Natasha and Jimmy were scared and confused. It was harder than they could have imagined seeing Lainey’s small body poked and prodded so much and to have specialist after specialist confused on what was going on and why. She had her first 3 haircuts in the NICU in order to get her IV’s. They were unsuccessful in the many attempts to get the IV in her hands so they had to put it in her head. After coming home from her second admission, Lainey had weekly weight checks with the family doctor.
Exactly, one year ago when Lainey was just over 3 weeks old, Natasha and Jimmy received the news from Genetics that she had been diagnosed with a rare genetic syndrome called Prader-Willi Syndrome.
Prader-Willi Syndrome is a rare genetic disorder effecting 1/30000 people in Canada. PWS is a complex genetic disorder affecting appetite, growth, metabolism, cognitive function and behavior. People with PWS have a flaw in the hypothalamus part of their brain, which normally registers feelings of hunger and satiety. It is apparent that people with this flaw never feel full; they have a continuous urge to eat that they cannot learn to control. To compound this problem, people with PWS need less food than their peers without the syndrome.
PWS has been historically known as a two stage syndrome, with the first stage characterized by hypotonia (low muscle tone) and poor feeding in infancy, and the second stage exhibiting hyperphagia (an uncontrollable drive to eat), combined with weight gain on fewer calories.
PWS results from an abnormality on the 15th chromosome. It occurs in males and females equally and in all races. PWS can occur in any family, and cannot be prevented. There is no known cause.
Most of Lainey’s milestone will be delayed. Her parents were told that best case scenario, Lainey may sit up unsupported by one year of age and possibly walking by two years of age. To everyone's surprise, Lainey was sitting up just under 8 months of age. PWS is a spectrum disorder meaning that out of the countless possibilities and outcomes, we will not know exactly what to expect from Lainey until it presents itself.
To date, Lainey’s medical team consists of 20 doctors and specialists! As Lainey ages, her medial team will increase and so will the frequency of some of the appointments. She is currently followed by several doctors and specialists who see her very regularly.
Unlike other children, Lainey will not have your typical signs of being sick, having an ear infection, cold, etc. She will not have a fever or vomit like other children. And she has a high pain tolerance. With her happy laid back demeanour and her lack of a cry, we really need to pay close attention to her behaviours. Natasha and Jimmy have been instructed to take her to the Emergency Department if anything seems off with her to get checked. The only way of knowing for sure if something is going on is to have blood work, x-rays or other testing done. With lower tone, respiratory illnesses will result in hospital stays.
Lainey had two other hospital admissions since the ones in the NICU. The end of November she was admitted for a respiratory illness for 14 days. In May, she admitted again for 12 days and had received a second rare diagnosis. Lainey along with Prader-Willi Syndrome has Interstitial Lung Disease-Neuroendocrine Hyperplasia of Infancy (NEHI). These conditions are not related to one another.
It has been proven that Growth Hormones helps people with PWS to develop more normally, it helps with metabolism, cognitive function and it will give Lainey a better quality of life. Here in Nova Scotia, the Endocrinologists are not in favor of Growth Hormone treatment.
Unfortunately, there is no one that specializes in PWS in Nova Scotia. We were told that there are probably 10 people or less under the age of 18 with this diagnosis in our province. With that, Natasha had to prepare for almost every appointment with hours and hours if not days of prep to make sure she was asking the proper questions and to let Lainey’s care team know what her parents expect from them. Natasha has been to appointments where she is letting the specialists know about things they need to watch for and follow because Lainey is their first patient with PWS.
The family will be traveling to Toronto as early as this month to get a sleep study completed before having an in person appointment with an Endocrinologist at Sick Kids Hospital. Her parents are currently waiting on the date. Because of Covid-19, the appointments have been delayed. They may have to be in Ontario for July 26th! They are waiting on a phone call this coming Monday to confirm the date.
When in Ontario, the family will be going to a PWS clinic. There is a big expense with GH. We were told that the first year will be approximately $10,000-$12,000. We are hopeful that the Endocrinologist that specializes in PWS can help Lainey receive the treatment that she needs.
The PWS Endocrinologist, Dietitian along with the Sleep Clinic are all in Toronto. The family will have multiple trips to Ontario for follow ups and monitoring.
The family has been very fortunate to have the care team that they do. Lainey's pediatrician, family doctor and all of the doctors and specialist at the IWK have been unbelievably supportive.
To date, Lainey is sitting on her own, smiles non stop, has been rolling over, feeds herself finger foods or food that is put on a spoon or a fork. Her personality shines as big as her smile. Most importantly, she shows her family every day just how lucky they are to have her.
Lainey's journey has just started and our understanding and learning will be ongoing as Lainey gets older. PWS does not define Lainey or who she is. As her mother tells Lainey every day “defy all odds”.
XOXO
UPDATE: Lainey's parents heard from Sick Kids Hospital on Monday. They were hoping to have the appointment on July 26th but they are not quite ready to open yet. The booking staff are now trying to get Lainey in for August 1st, 2020. With everything going on with Covid restrictions, the scheduling is a bit up in the air. Natasha and Jimmy have been given a guaranteed date of August 24th but remain hopeful that they may be able to go earlier.
***************************************Please share this page. Thank you.*****************************************
Lainey Jamieson Hynes was born on her due date of June 25, 2019 at 9:18pm weighing 6.8lbs. Her parents, Natasha Jessome and Jimmy Hynes were completely head over heels in love with their daughter.
After being discharged from the hospital, Natasha and Jimmy went back to the hospital for Lainey to have her hearing tested the following day. While there, they were talking to the nurse about how Lainey wasn’t nursing very much and how she was very sleepy. They tested her blood sugar levels and the results were low enough that they gave her formula right away to see if that would help. Lainey’s levels were tested after having the formula and they were in the normal range. They re-tested her levels after a few minutes and they were dropping faster than they should have. This resulted in her first stay in the Neonatal Intensive Care Unit.
While in the NICU, the doctor's were concerned with Lainey’s low muscle tone, sleepiness and her failure to gain enough weight. She had spinal taps, various blood tests, MRI, ECG, x-rays, and over all monitoring of her heart, oxygen and blood pressure. Lainey’s poor feet had more marks on them from the constant blood tests to check her sugar levels. There were multiple visits from a lactation consultant to see if she could get Lainey to have a full feeding without falling asleep. The second Lainey was close to her mother, she would fall asleep and wouldn’t eat. Natasha and Jimmy would have Lainey in a diaper and use cold cloths on her skin to try to keep her awake. She was very sleepy and it was hard to keep her awake for any feedings, even from a bottle.
There were countless visits from different doctors and specialists. No one was able to explain what was going on. Lainey was finally able to get on track with her feedings and she was gaining weight. The NICU team was happy with her progress and they were sent home.
Three days after being home, Lainey had a follow up appointment the family doctor. Once Lainey was weighed, her parents were told to take her back to the hospital because she lost more weight even though they were following the same routine that was being followed in the hospital. This resulted in Lainey's second NICU stay. While admitted, they did multiple series of blood work that sometimes was repeated numerous times a day, more x-rays and testing. A Geneticist came to see Natasha and Jimmy and wanted to test for a few things so they agreed. Again, Lainey's feeding were going well and she was gaining weight so they were released for the second time.
In total, Lainey spent 2 ½ weeks in the NICU. Natasha and Jimmy were scared and confused. It was harder than they could have imagined seeing Lainey’s small body poked and prodded so much and to have specialist after specialist confused on what was going on and why. She had her first 3 haircuts in the NICU in order to get her IV’s. They were unsuccessful in the many attempts to get the IV in her hands so they had to put it in her head. After coming home from her second admission, Lainey had weekly weight checks with the family doctor.
Exactly, one year ago when Lainey was just over 3 weeks old, Natasha and Jimmy received the news from Genetics that she had been diagnosed with a rare genetic syndrome called Prader-Willi Syndrome.
Prader-Willi Syndrome is a rare genetic disorder effecting 1/30000 people in Canada. PWS is a complex genetic disorder affecting appetite, growth, metabolism, cognitive function and behavior. People with PWS have a flaw in the hypothalamus part of their brain, which normally registers feelings of hunger and satiety. It is apparent that people with this flaw never feel full; they have a continuous urge to eat that they cannot learn to control. To compound this problem, people with PWS need less food than their peers without the syndrome.
PWS has been historically known as a two stage syndrome, with the first stage characterized by hypotonia (low muscle tone) and poor feeding in infancy, and the second stage exhibiting hyperphagia (an uncontrollable drive to eat), combined with weight gain on fewer calories.
PWS results from an abnormality on the 15th chromosome. It occurs in males and females equally and in all races. PWS can occur in any family, and cannot be prevented. There is no known cause.
Most of Lainey’s milestone will be delayed. Her parents were told that best case scenario, Lainey may sit up unsupported by one year of age and possibly walking by two years of age. To everyone's surprise, Lainey was sitting up just under 8 months of age. PWS is a spectrum disorder meaning that out of the countless possibilities and outcomes, we will not know exactly what to expect from Lainey until it presents itself.
To date, Lainey’s medical team consists of 20 doctors and specialists! As Lainey ages, her medial team will increase and so will the frequency of some of the appointments. She is currently followed by several doctors and specialists who see her very regularly.
Unlike other children, Lainey will not have your typical signs of being sick, having an ear infection, cold, etc. She will not have a fever or vomit like other children. And she has a high pain tolerance. With her happy laid back demeanour and her lack of a cry, we really need to pay close attention to her behaviours. Natasha and Jimmy have been instructed to take her to the Emergency Department if anything seems off with her to get checked. The only way of knowing for sure if something is going on is to have blood work, x-rays or other testing done. With lower tone, respiratory illnesses will result in hospital stays.
Lainey had two other hospital admissions since the ones in the NICU. The end of November she was admitted for a respiratory illness for 14 days. In May, she admitted again for 12 days and had received a second rare diagnosis. Lainey along with Prader-Willi Syndrome has Interstitial Lung Disease-Neuroendocrine Hyperplasia of Infancy (NEHI). These conditions are not related to one another.
It has been proven that Growth Hormones helps people with PWS to develop more normally, it helps with metabolism, cognitive function and it will give Lainey a better quality of life. Here in Nova Scotia, the Endocrinologists are not in favor of Growth Hormone treatment.
Unfortunately, there is no one that specializes in PWS in Nova Scotia. We were told that there are probably 10 people or less under the age of 18 with this diagnosis in our province. With that, Natasha had to prepare for almost every appointment with hours and hours if not days of prep to make sure she was asking the proper questions and to let Lainey’s care team know what her parents expect from them. Natasha has been to appointments where she is letting the specialists know about things they need to watch for and follow because Lainey is their first patient with PWS.
The family will be traveling to Toronto as early as this month to get a sleep study completed before having an in person appointment with an Endocrinologist at Sick Kids Hospital. Her parents are currently waiting on the date. Because of Covid-19, the appointments have been delayed. They may have to be in Ontario for July 26th! They are waiting on a phone call this coming Monday to confirm the date.
When in Ontario, the family will be going to a PWS clinic. There is a big expense with GH. We were told that the first year will be approximately $10,000-$12,000. We are hopeful that the Endocrinologist that specializes in PWS can help Lainey receive the treatment that she needs.
The PWS Endocrinologist, Dietitian along with the Sleep Clinic are all in Toronto. The family will have multiple trips to Ontario for follow ups and monitoring.
The family has been very fortunate to have the care team that they do. Lainey's pediatrician, family doctor and all of the doctors and specialist at the IWK have been unbelievably supportive.
To date, Lainey is sitting on her own, smiles non stop, has been rolling over, feeds herself finger foods or food that is put on a spoon or a fork. Her personality shines as big as her smile. Most importantly, she shows her family every day just how lucky they are to have her.
Lainey's journey has just started and our understanding and learning will be ongoing as Lainey gets older. PWS does not define Lainey or who she is. As her mother tells Lainey every day “defy all odds”.
XOXO
Organizer and beneficiary
Natasha Jessome
Beneficiary