Jaden Manley, Family Story

Hello, my name is Dave and this is my son Jaden. We live in Mexico, NY. Jaden is 21 years old and we received his diagnosis of Classic Pelizaeus-Merzbacher Disease when he was only 18 months old. At the time we were very fortunate to receive the diagnosis so early. Jaden like many with PMD was initially misdiagnosed with Cerebral Palsy. My wife Julie and I had hoped to have additional children, however, Julie was tested and confirmed to be a carrier for PMD. Once we received the diagnosis we elected not to have additional children for fear that they may be affected as well. Although my wife does not come from a large family. There was no history of this disease in her family as far back as anyone could remember.

For the past 21 years every hope and dream that my wife and I had for our future changed the day we received the diagnosis. Our entire lives have been reshaped around providing the best possible care for our son. He has had to endure 5 surgeries in the past 6 years due to the progression of the disease. These surgeries include a feeding tube,

Osteotomys on both of his hips, Spinal Fusion and the installation of an intrathecal baclofen pump to manage his pain and spasticity.

Jaden is non verbal, non ambulatory, incontinent and relies on complete care and supervision for safety 24/7/365. We are very blessed in that though he has faced much adversity Jaden has a smile and personality that will light up any room. He tends to go with the flow much better than we do. Due to Jaden's size I have become the primary caregiver for Jaden over the last 6-7 years. Throughout his time in Public school Jaden has required a one to one aid to assist in all of his activities. At school he received all of his therapies as well as his social interaction with peers. Jaden has loved school all these years and they loved him as well. Jaden has now aged out of school. Now that school has ended Jaden is with me 24/7/365. In addition to being his caregiver I am now also his Physical Therapist, Occupational Therapist, Speech Therapist and his social entertainment.

The worst symptom for all of us I believe is the fact that he is non verbal. While we have tried many communication devices, because the disease affects his motor control there are really no good options. The inability for Jaden to tell us what he needs, wants or how he is feeling leaves us to do our best to guess. I have become pretty good at knowing what Jaden needs just by sounds or facial expressions and pure instinct. I can’t imagine how frustrating it has been for Jaden for 21 years to not be able to express himself to all those around him. This is especially difficult for us as caregivers that want to help when there is discomfort. You try your best not knowing if you are helping or actually making things worse?

The personal care required for Jaden also makes it difficult for him and the caregivers. Requiring complete assistance must be frustrating for Jaden. But it also means the caregivers' days are built completely around his schedule and routine. It takes an hour to get him up in the morning and in his wheelchair to start his day. Jaden requires 3 tube feedings per day that take 1 ½ to 2 hours to complete. Due to the amount of fluid he receives he also needs to be changed every 2 hours as he can not tell you when he needs to be changed. This makes travel very difficult and going out in public in general difficult. At the end of the day it takes an additional hour to get him ready for bed. Due to this schedule and the need to utilize the equipment in our home it is often easier to not leave the home. This leads to self imposed isolation. In fact I have not had a vacation since 2010 as it is actually more frustrating to try to go places and overcome the physical obstacles of his care than to just stay home. This is not great for Jaden or the caregivers.

However, as physically exhausting as the day to day care is. It becomes even worse when you disrupt the schedule and routine.

Now that Jaden has aged out of school there are no good day programs that would be able to accommodate his needs in our county. So I will be his full time caregiver as I try to work a full time job and also try to be a good husband. While we would be eligible to have some assistance come into the house that poses its own difficulties. While a physical break would be welcome, having a person lingering around our home caring for Jaden takes away any privacy you may have so you can not truly relax. So again it is easier to just take care of Jaden myself.

The biggest challenge that my wife and I have moving forward is finding a good long term care solution. Because we have no other children there are no siblings to provide care if anything happens to me. I am the youngest of 8 children.

My two oldest siblings have already passed. So we have no family option to provide care in a crisis. Our biggest fear is Jaden ending up in a hospital for months if anything happened to me and then being placed in the first (not the best) opening in a skilled nursing home. Should this happen he would be surrounded by many 80-90 year olds many with dementia which would not be a good social environment for him to thrive. We live in great fear that Jaden would be forced to live his remaining days in a bad situation should anything happen to me as my wife would not be able to care for him.

We pray that we will be able to see a treatment or a therapy in our lifetime even though it may not help Jaden. But short of a complete cure, a treatment that would ideally allow for communication, and lessen the physical aspects of the disease which would allow for more independence and a better quality of life for those with PMD as well as the caregivers would be a victory. Though a treatment for my son due to his age is not realistic. If I think about the risks we would be willing to take if there was an option available knowing what I know now and having the experience of living with PMD for 21 years. We would have been willing to accept any risk that did not involve putting my son in more pain for the opportunity to give him a chance at a better quality of life that would give him more independence and joy.