Jack Schrooten
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Jack's First Year:
Jack was born on 11/4/98 at Missouri Baptist Medical Center, St. Louis, Missouri, at 33 weeks of age. After six weeks in the Special Care Nursery, Jack came home on ½ liter oxygen. Once home, Jack appeared to be doing well, although he was not an aggressive eater. Approximately three weeks after coming home, Jack had an apneic event and was admitted to St. Louis Children's Hospital. At this time, it was discovered that Jack had a vascular ring formed by the right aortic arch and an anomalous left subclavian artery.
On January 18, 1999, surgery was performed to divide the vascular ring. Jack came home two weeks after surgery on less than a ½ liter oxygen. Within two days of coming home, Jack was back in the hospital with a right upper lobe collapse. At this point, Jack was placed on nasal CPAP 24 hours a day for several weeks. After that, Jack was on nasal cannula during the day and CPAP at night. It was also noted during this hospital stay that Jack had an elevated right diaphragm (we have since discovered that this was a result of the surgery to divide the vascular ring). After three weeks in the hospital, Jack came home on nasal CPAP and supplemental oxygen. Jack appeared to be doing well although he did continue to have a difficult time eating. Two weeks after coming home Jack was again in respiratory distress and was admitted to St. Louis Children's. He was intubated on March 7, 1999.
After one week, the first attempt was made to extubate Jack. After Jack failed extubation, a decision was made to plicate Jack's diaphragm in an attempt to resolve the constant right upper lobe collapse. Following surgery, a second attempt was made to extubate Jack. Again, Jack failed extubation. At this point, the PICU staff asked for neuromuscular to consult since it appeared that Jack demonstrated low tone and muscle weakness. A muscle biopsy was performed and it was determined that Jack appeared to have an inflammatory myopathy. Jack was started on high dose steroids and after several weeks his CPK came down and Jack seemed to have slightly increased muscle strength. As a result another attempt was made to extubate Jack. When Jack failed this final attempt a decision was made to perform a tracheostomy so that Jack could come home. Within a week of being trached, Jack was on trach collar anywhere from 8 to 10 hours a day. When oral feedings were resumed it was noted that Jack aspirated his formula and a swallow study indicated that Jack had an uncoordinated and weak swallow. Surgery was performed to insert a g-button and Jack left St. Louis Children's PICU on June 8, 1999.
Jack's Neuromuscular Condition:
Jack's muscle biopsy was sent to the Mayo Clinic for review by Dr. Andrew Engle. Dr. Engle's diagnosis was "myopathy, active, chronic". Originally, Jack's neurologist had two working diagnoses, namely, post-vaccine myositis (because every time he ended up in respiratory distress it was after receiving immunizations) or congenital muscular dystrophy. Jack later had a brain MRI that revealed that he has moderate diffuse cerebral atrophy and absence of the inferior vermis and cerebellar tonsils. At the age of 13 months, it was discovered that Jack had cataracts. Jack has had the cataracts removed and now has lens implants. He wears bi-focal glasses and sees just fine. Based on the MRI and the cataracts, Jack has been diagnosed with a "congenital muscular dystrophy with eye and brain involvement". Jack does not fit the classic definition for "Eye-Muscle-Brain" type of congenital muscular dystrophy - which involves severe mental retardation and generally early death. I am skeptical that Jack has muscular dystrophy.
Based on Jack's muscle biopsy and the results of his EMG, Jack was accepted into a study at the
NIH for vaccine induced myositis. Because one of Jack's working diagnoses was vaccine induced myositis and because I'm not convinced that a vaccine did not have something to do with Jack's condition, he has not received any other vaccines since the initial ones he received in January 1999.
Jack's muscle tissue has been sent to Dr. Eric Hoffman at the NIH for his input as to what Jack's diagnosis and/or prognosis may be. Dr. Hoffman is a world renowned leader in the field of muscular dystrophy research. To date, we have received no information from Dr. Hoffman regarding his research of Jack.
Jack's Ventilator Progress:
Jack came home from Children's hospital on minimal ventilator support (rate 5, pressure support 4 and peep 4). Jack continued to tolerate trach collar for up to 10 hours a day during his first several months home. Jack then became quite sick with a respiratory infection and, as a result, he required increased ventilator support which made him unable to tolerate trach collar. During the winter of 1999-2000 Jack did not tolerate trach collar at all, as he was battling one respiratory infection after another.
Beginning in May, 2000, Jack started tolerating trach collar again. Eventually, Jack was able to tolerate being off the vent during all waking hours (approximately 8 hours a day). During a visit with the Jack's doctor in September, 2000, it was discovered that Jack's EtCO2 was 80. Based on this, Jack's vent settings were increased and no attempts have been made since September 2000, to wean Jack off his vent. Jack's current vent settings are: Rate 10; pressure control 24; pressure support 10 and peep 4 - Jack uses the Pulmonetic LTV 950.
May 2003
Jack is severely developmentally delayed. He cannot roll, sit or do anything for himself. He is much like a quadriplegic, although he has the use of his hands and he can move his legs. Just last month Jack got a power wheelchair and he is now learning to "drive" it. He loves the horn!
During our recent visit to Jack's new pulmonologist (we moved from St. Louis to Phoenix in June, 2002), I told him that I wanted to start trying to wean Jack off his vent again. After "consulting" with several moms on the Tracheostomy message board, we are going to do a very slow and gradual vent wean. In the past we used to just take Jack off the vent and see how long he could "fly". With a more conservative approach, hopefully, Jack will make progress towards getting off the vent at least during the day.
Jack is a very happy and bright little boy. He has two older sisters and one younger brother. He receives all his schooling and therapy at home. Our family has adjusted fairly well with living with a child on a ventilator. We could never do it without the help of all the wonderful nurses we've had the last four years. Jack receives a minimum of 52 hours of nursing a week. I work full-time and, therefore, must use all my nursing for when I'm at work. The last four years have been difficult since, originally, none of Jack's doctors imagined the severity of his muscle weakness and they all assured me that he would be off the vent in no time. I struggle every day trying to make "peace" with the life that Jack must now live. But, as I tell everyone,
I just get up each day and do my best to "keep on keeping on".
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Jack and his sisters, Hilary (13) and Mary (11)
and his brother Eric (2)
December 2003
Update, January 2005:
This last year was one of heartache and new challenges. Jack was making great progress in learning to drive his power wheelchair and in using his communication board, however, in March, he suffered a major setback when he disconnected himself from his ventilator while his Dad was upstairs and didn't hear the alarms. It is unclear how long Jack was off his ventilator, but when his Dad found him, he was unconscious and non-responsive. Jack spent a week in the PICU and when he came home he could do little more than open his eyes. He was virtually paralyzed. The thing we missed the most was Jack's beautiful smile. It took about three months for the smile to return, but it IS back! Jack has regained some of what he lost physically, but he still has no purposeful use of his hands. As a result, he can no longer use his communication board, nor can he drive his wheelchair. Jack's neurologist said to give him a year to recover, so we still have a few months left before we reach the point of no return. Jack is making slow, but steady progress.
Jack continues to receive all his schooling and therapy at home. We don't really see any benefit to sending him to school and since it is a given that he will get sick if he does go to school, we've opted to keep him home. Jack has 7 different therapists who see him throughout the week. He has OT, PT, Speech and a teacher through the school district and OT, PT and Speech through our Department of Developmental Disabilities. Needless to say, there is never a dull moment at our house. I am also very fortunate to have wonderful nurses who provide excellent care for Jack and who have come to love him as we do.
In May, Jack took his very first plane trip - to St. Louis. Imagine having to carry onto the plane - Jack (all 52 pounds of him), his car seat, his ventilator, his 9-hour marine battery, his suction machine and a bag with all his supplies, PLUS his back-up vent, back-up battery, back-up suction machine, and all his back-up supplies. Before we took off, the pilot told me that I'd have to turn off Jack's ventilator during take off and landing - Yeah, right! The stress of getting Jack and all his equipment onto an airplane and having to deal with a completely freaked out pilot and flight crew was not worth the time we saved by flying. However, seeing all of Jack's doctors in St. Louis made the trip worth it. I've decided that it is in Jack's best interest (and mine) to take him back to St. Louis every summer to see the doctors who know him and who truly care about him. However, next time, we drive.
The highlight of our year was meeting many of the trach board families at the First Tracheostomy.com Pediatric Conference held in St. Louis. We especially enjoyed the opportunity to meet Cindy and Aaron. It was an awesome two days filled with lots of fun activities for the kids, as well as great presentations from some of the best pediatric airway and respiratory specialists in the country. We were very fortunate to have Dr. Robin Cotton as our keynote speaker. After the Conference was over, we reluctantly left all our friends and Jack's doctors in St. Louis to make our way back to Phoenix.
This year I was able to establish a foundation that I envisioned years ago. The name is "The Willow Tree Foundation" and its mission is to provide respite opportunities for parents of medically fragile children. The name was chosen for the reason that, in the raging storm, the willow tree bends, but it doesn't break - much like us parents. This next year will be spent applying for grant money and doing other fundraising. Stay tuned . . .
Jack celebrated his 6th birthday this year. After 6 years, I have to admit - I'm tired. I'm tired of the constant battles with Apria Healthcare. I'm tired of the 25+ EOBs I get each month from the insurance company, over half of which have been processed wrong. I'm tired of being talked down to by insurance company reps who treat me as if it is the first time I've ever called to dispute a claim. I'm tired of the reality that whether I go to work, get sleep or get out of the house without feeling guilty is dependent on whether I have a nurse in my house. Mostly, I'm tired of hearing how strong I am. There are times I just want to yell - "I am NOT strong, I'm really falling apart behind this façade of having it together". There are many days where I think that I can't do this any longer. Yet, I love Jack more than life itself and I will continue to give him all I can at any given moment. I know that each day I have with Jack, and with each of my children, is a gift. Jack is my hero and if he can keep on smiling in spite of his challenges, I certainly can do the same.
I approach this new year with hope for great things to happen - for Jack, for my other three children and for The Willow Tree Foundation. Thank you to all of my friends on the Trach Board for your unfailing support and encouragement. You have truly made it possible for me to "keep on keeping on". I love you guys!
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Christmas 2004
For more Jack updates, see Jack's Blog at: http://jack-schrooten.blogspot.com/
