Magnus' story, 1-8 years old
Magnus Mortensen
SMA type 1, Respiratory Choices
(presentation prepared for a conference held by the Danish Muscular Dystrophy
Association)
We are the parents of Magnus Mortensen, SMA type 1. Magnus is now 8-years-old.
He began using a bi-pap when he was just over 1-year and has had a tracheotomy
since he was four.
The Danish Muscular Dystrophy Association asked us to speak about:
Our respiratory choices for Magnus
Our medical community's approach to Magnus's disease
Magnus's development – who is he today?
Content:
1. The Diagnosis: "Wait & See"
2. On our own in Egypt – one step forward
3. Magnus gets pneumonia
4. Tracheotomy
5. Magnus at 8
The Diagnosis: "Wait & See"
Magnus was diagnosed with SMA type 1 in September 2001. He was 10 months
old.
The first 10 months were a period of blissful ignorance. We lived in Egypt, Magnus was our first child and we had no other friends with children. We didn't know any better.
Magnus was "floppy" from the very beginning. He had a soft cry, could never really sit by himself and had unusual respiration.
The diagnosis that September in Denmark paralyzed us. From what we could read on the Internet, it seemed our son would be dead before he was two.
It was incomprehensible. Our child seemed basically healthy: he smiled, he ate, he giggled. How could he be afflicted with such a sinister disease?
For several weeks following the diagnosis, we were overloaded with SMA information. We met with the Muscular Dystrophy Association, the doctor who would become our neurologist, and a family with a daughter who had SMA.
Then we went back to Egypt with a suitcase full of kit: a brace to help him sit, a therapy ball, a special chair, and a CPAP.
We were sent on our way with the message that there was not much we could
do. "Wait and see how things develop and remember: pneumonia is a
very bad thing for these children."
On our own in Egypt – one step ahead
Back in Egypt (Charlotte worked for the UN) we were on our own –
away from the security of a medical system we knew and our network of
experts.
Unsatisfied with "wait & see" and still in need of more information we discovered a group of American mothers with a much more active approach to SMA. Through them we learned of a Doctor in New Jersey named Bach who had a novel method of dealing with SMA. We learned about dairy free diets, amino acid formulas and much more.
These mothers had weak SMA children who were alive well past 2-years-old.They gave us hope – we could do something.
One of the first things we learned from the American moms was to always
be one step ahead of the disease. For example:
• Magnus should get a g-tube before he lost his ability to swallow
• He should have a cough-assist and a bi-pap immediately so that
we could prevent colds from becoming pneumonia
• He needed to get on a diet that was simple to digest.
Most important, we came to understand that the absolute first priority with a weak SMA child is respiratory health – keep those airways clear and make certain the child can ventilate properly. Everything else is secondary - bracing, physical therapy all of it. We had a very VERY short time to get smart and get our care protocol in order.
The message was clear and it snapped us out of our post-diagnosis stupor. Get your respiration house in order or – at best – your child is going to lose the few abilities he has. But more likely, he will die.
With the help of our Egyptian pediatrician and our UN health insurance, we began collecting all the mysterious machines we had learned about: The Cough-Assist, a Bi-pap, suction units and a pulse oximeter. It was an odd task, still abstract – we had no idea just how important these tools would become.
Thinking back, were we consciously thinking about prolonging his life? Not really. We still could not perceive him as 'ill'. He was a happy, smiling, baby who happened to be a little weak.
And action gave us a feeling of control and security. We were no longer
waiting helplessly to see if pneumonia showed up. We were preparing ourselves
to act if a respiratory infection arrived.
Magnus gets pneumonia
In February 2002 we were in Denmark on vacation and Magnus contracted
pneumonia on the same day our bi-pap, cough assist and pulse oximeter
arrived from the United States.
He was 14-months-old at that time, could still eat by himself and had never been particularly ill.
This would be the first time the traditional Danish method for treating pneumonia in an SMA child – anti-biotics and CPAP – met with Dr. Bach's protocol – bi-pap and cough assist.
Magnus was admitted to a local hospital where treatment was restricted to CPAP.
When his condition deteriorated, he was transferred to a more specialized hospital.
A hospital-supplied bi-pap was waiting for us when we arrived -- along with 4 doctors, all curious to see the machine used with a 14-month old. They were skeptical that he would cooperate with it.
We put the mask on him and he looked up and smiled.
He was breathing more comfortably with the bi-pap but the pneumonia was not clearing. For three weeks he lay there with no improvement, the cough assist sitting in a closet next to his bed. The staff, completely unfamiliar with the machine, were highly skeptical and did not want it to be used.
Finally, our favorite doctor came one night and said, "I think you need to try the cough assist because our treatment is not working." Magnus had had pneumonia for 4 weeks by that time and was slowly getting weaker.
Four days later, the pneumonia had cleared.
The cough assist had saved his life or, at the very least, prevented
further irreversible deterioration of his abilities. The cough assist
has been an important part of our lives ever since. And from that time
forward we have been known at the hospital as the "cough machine
people".
Tracheotomy
For Magnus's respiratory health, the biggest event has been his tracheotomy.
When Magnus turned 4 the hospital's respiration center recommended it was time for Magnus to get a respirator.
It seemed like a big decision at the time but given the situation, it ended up being a 'no brainer'.
At the time, Magnus was using the bi-pap almost constantly but was not getting proper ventilation. He was going to school and had a relatively active life but the bi-pap was always close by and the bi-pap 'sucks' if you have to use it all the time. It irritated his face and was effecting his facial development. The ventilation was not fully satisfactory and resulted in a lot of air in his stomach.
A trach seemed like a very good idea.
The operation went well and Magnus was even more excited about this new
"bi-pap", as he calls his respirator, than he was for the original.
Magnus at 8
Magnus now attends a school with a special section for the physically
handicapped. He attends 3-5 days a week depending on his health and the
health of his helpers.
He has never had another pneumonia.
He has been hospitalized several times in the last year because of a tendency for him to become dehydrated when infections cause him to vomit.
We more or less, have his respiratory health in as much control as possible. But he is still a fragile creature.
Psychologically, Magnus seems to still be a happy kid most of the time. We have not seen evidence yet that his disease has affected his quality of life.
There are many things he loves to do:
Lie and play with his arm in a special support device.
Watch Cartoon Network.
Play the game "Spore".
Draw with pen and paper as well as on his computer.
Play with his brother & sister.
Cuddle with his dog Cody.
Get tossed in the air.
Swim.
Tell jokes -- jokes we do not always understand.
Physically, Magnus is weaker than it is easy to imagine.
He can sit up in and drive his electric wheel chair while wearing a brace.
He can also use his computer from this position.
He prefers to lie.
He stands in a stander with his brace for 30 minutes or s everyday.
He is almost extremely difficult to understand when he speaks because
of the lack of facial muscles.
He eats through a g-tube.
He needs to be suctioned – superficially – 5-50 times a day.
When we occasionally look at our son from an 'outside' perspective we
do think, "damn that kid is handicapped."
And Magnus does get a lot of stares and comments from other children but he is good at responding. For example:
At a petting zoo, a child said, "Hey he is drooling!" Magnus
responded, "Yeah, I drool all the time."
At a family picnic, a cousin was asking him a series of rapid questions:
Why was his mouth open? Why couldn't he walk? Why did he talk funny? Magnus
response, "Because I am an alien." We asked him later did he
think he was in fact from outer space and he told us, "no, I just
said that to be funny."
Has it all been worth it?
Is Magnus's life worth living? As far as we can see, yes it is. Magnus
was born with his condition and seems to accept it. He is certainly as
interested in being alive as anyone we know.
Have we made conscious choice about prolonging his life? Not exactly. Our intervention was more motivated by short-term comfort. Longevity is a secondary result.
All the machines improve the quality of his life while he has it. Life is just better when you get the oxygen you need. Life is just better without the anxiety you might choke to death at any time on your own secretions. And life is just better when illness does not mean further deterioration of already diminished abilities because illness can be dealt with effectively.
When someone gets ill, we normally treat them with medicine. For weak muscular dystrophy children this treatment is just a little more mechanical.
Are we ever fed up, angry or frightened? All the time. But we never would have made a different choice and we value the life we have gotten.
Magnus's future?
We do not think too much about his future. Not concretely.
Though we did see and episode of an American television program, This
American Life*, which presented a 28-year-old named Mike with SMA. He
has a trach, can't swallow, can't speak and cannot sit upright but he
leads a rich life. He has a girlfriend, works as a journalist and plays
a lot of video games. The episode was about his desire to move out of
his mother’s house and live alone.
Seeing that made us hopeful. Looking at Mike's twisted body in the program
makes you think, "is that a life?" But what do we know.
Charlotte Gylling Mortensen, charlottegp@yahoo.com, tlf: 2548 8995
Joshua Mortensen, josh.mortensen@gmail.com, tlf: 2536 3262
* This American Life Season 2 episode 1 "Escape".
