I should add that some kids can be fine, then vomit, and get pneumonia from vomit in the lungs.
Some SMA kids don’t get Nissens and are at higher risk of reflux aspiration pneumonia.
Some SMA kids go to the hospital multiple times per year with pneumonia.
As you can see, there are heavy ethical considerations.
How much do you treat, if at all? Some parents do basically nothing (i.e., palliative care). Some parents pull out all the stops and go with a trach & vent. Some parents want a few treatments but not other things. The SMA community stresses that there is no “right” way. Do what is best for your own family.
Our philosophy at this time, with limited knowledge and experience, is to do what we can to enhance her quality of life as our first priority. Make her happy. The side effect of that is that she may live beyond the average SMA1 life expectancy, especially given that she is a fairly “Strong 1.”
I have heard some people decide they want to extend life as long as possible for its own sake, ans they give little consideration to the quality of life. Quantity over quality. We don’t believe in that for our child. Although the decisions we make may extend her life, that is not truly the objective.
In case you were wondering, we also decided we are a “no trach” family. Our feeling at this time is that a trach does not enhance the quality of life. Other families obviously disagree as to their own children.
I imagine some families cast judgment upon other families based upon such ethical differences. A trach family might view a palliative family negatively as “just letting their child die.” A palliative family might view a trach family as “allowing their child to suffer needlessly.”
We adhere to the idea that there is no “right” way to make those decisions.
Today, she is happy. Hopefully, if we continue to treat her, and she becomes unhappy, she will let us know in her own way that it’s time and that it’s ok.
For what it’s worth, I think you’re absolutely correct in your thinking. I can tell you’ve done a ton of research and connected with other parents with this issue in their families, and that’s wonderful. At the same time, you realize that your daughter is an individual, with her own experience, strengths and weaknesses, and that’s awesome. It’s an important balance to maintain, to create a meaningful, happy life for her, not just a biological life.
If I may ask, are you a “want to know everything that could go wrong” person or a “don’t tell me about it unless it happens” person? Is your wife the same as you? As a future NICU nurse, I’m afraid this issue is going to be a tough one for me to navigate with parents. If you feel like it, could you share some of the ways the nursing staff has done well or poorly at sharing information about SMA in general and your daughter’s specific case with you?
Good luck with them, whatever your decisions are. I know they’ll hurt no matter what.
Wow, Bearflag, just…wow. Good on you for being a strong person through all of this. As an outsider looking in, my gut feeling is that I wouldn’t be able to cope with something like this, but perhaps if it were to happen with one of my children, I would because there would be no alternative.
Well, props to you and your family and I hope Getty lives a long and prosperous life.
Bearflag,
Have you visited the Dreammom blog? The writer has a son with a terminal genetic disorder. Her son has been in and out of hospitals many times (due to MRSA and pneumonia and other issues) and her experiences might be helpful for you. She has advice for parents on how to handle hospitalizations and other good info.
bearflag, y’all sound like you have a good plan going. as a member of the sdmb i know that you are a “knowledge is power” kind of person. using that knowledge and parental intuition y’all will make good decisions for wee getty, not a doubt in the world there.
stay strong and may you, your wife, and getty find joy and delight in each other minute by minute, hour by hour, day by day.
You have my best wishes and congratulations here, for your whole family. It’s not that I haven’t been wishing the best for everyone all along.
Knowledge is power. I couldn’t live in ignorance just waiting to react to negative events. I want to know the potential pitfalls and how to address them. An ounce of prevention … and all that jazz.
I have heard some parents are in denial or slip into severe depression. We can’t do that. There is no choice in our minds. Getty needs help to be happy, and we have to provide her that help.
I have heard stories of some parents denying their disabled kids medical equipment, like wheelchairs, because the parents can’t handle seeing the kid in that state. The parents don’t want to acknowledge the problem. They don’t want to be reminded of it. :smack:
I’ll get into the medical practitioners issue soon…
I was talking to an SMA parent today about pulse oximeters. They monitor vital stats and can set off an alarm if the stats drop. The primary vitals are heart rate, blood oxygen levels, and respiratory rate. We are trying to get a pulse ox.
This guy told me a story of an SMA family who had one SMA child and a healthy child. They decided to watch a movie at home. They put the kids on the floor and the parents sat on the couch. They didn’t use their pulse ox. They turned out the lights and enjoyed the movie. When the movie was over, they discovered their SMA child passed away sometime during the movie. The pulse ox could have saved a life.
Doctors and nurses is a big issue. I’m not sure where to begin.
When we first got the diagnosis, the doctors didn’t tell us much. They let us go home to let it sink in. When we came back, it was clear that we were learning quite a bit and we were eager to know more to start planning. However, when we asked about treatment options, they told us there would be time for that later. They just assumed we weren’t ready for it at the time, but we were. We wish we had more information earlier on.
I hear many doctors just tell newly diagnosed parents to take their child home and just enjoy the time they have left. However, the truth is there is a lot a parent can do to have a healthy and happy SMA child. The doctors should present the spectrum of options and let the parents make informed decisions about whether to pursue them. Withholding such information is essentially a doctor making a value judgment for a family. Of course, this assumes the doctors know the option that are out there, which many doctors may not know.
The SMA community told us that our doctors and nurses likely do not know the ins and outs of SMA. The SMA community told us we would have to advocate for very basic things to convince our doctors and nurses. They said some routine practices of doctors and nurses can kill an SMA child. Be skeptical. Be informed. Be an advocate for your child. They said we we would soon know more about SMA than our doctors.
At the time, we didn’t believe a lay person would know better than a doctor. Now, we know. We have lived it.
We were the ones asking our doctors for a G-Tube. We were the ones asking our doctors to adopt certain bipap breathing machine protocols surrounding the surgery. When we took Getty into surgery, I was very active and involved in everything the doctors and nurses did. I knew every move they made and I asked them all kinds of questions about everything. I asked them to consider this and that before doing anything to ensure we came arrived at the best course of action.
I may have pissed off the hospital staff at first by being so proactive and inquisitive. After a few days, I think they came to understand that, for lay people, my wife and I were pretty well informed, we were able to assess potential implications medical decisions would have on an SMA baby, and we were valuable problem solvers. They altered many of the things they were thinking about doing based upon input from my wife and I. By the time we left the hospital, it felt almost as if as if we were a respected part of the medical team and not just pushy parents.
At one point, The intensivist asked me what I thought of a certain medical plan. He gave me his thoughts on his plan. I disagreed with him and made my argument to him. He listened to my argument and ended up agreeing with me. We went with my plan instead.
My father-in-law got a kick out of it. He was sitting in the back of the room laughing that I had completely turned the doctor around and I had the doctor agreeing with me.
I think the staff came to respect us. They would run all their plans by us for input. By the end of it, they told us that they had learned so much from their experience with our child. It was a bit of a struggle at first, but by the end of our hospital stay, we were all in agreement as to the best practices for an SMA baby, and it all went like clockwork once we got to that point. Then, we could relax and actually get to know each other.
I don’t think they were used to that from parents. One of them told us some parents just drop their kid off for surgery and they don’t come back until the surgery is over.
I’m rambling now.
Back to doctors …
I think our doctors were a very reactionary group. As my wife and I learned things, we had to push to get them done. I think the doctors have learned quite a bit from, and we appreciate that willingness to listen and learn.
We initially met with one surgeon and we gave him our ideas for surgery. He disagreed, but he gave us a holier-than-thou attitude about it. He took our questions and comments as a personal attack on him. Needless to say, we found a new surgeon asap, and he is wonderful. He is very matter-of-fact, but he is also willing to listen and learn. He is very willing to tell you his disagrees with your analysis and why… but he doesn’t get bent out of shape when you push the ideas through the ringer to arrive at some truth.
I heard that some doctors have a very hard time with SMA because they can’t “fix it.” They don’t want to deal with it. I understand that, but that shouldn’t affect a doctor’s duty to know things and to give the best informed care as possible.
At this point, I don’t know where I’m going with this. I think there are some lessons in there for you as a future nurse.
Doctors aren’t infallible.
Sometimes parents know their shit and are a valuable resource even though they are not medically trained.
Be willing to listen, learn, question, be questioned, and teach.
Treat people with respect.
Treat people as individuals.
A parents who is very involved in care is a concerned parent. I get it that some parents can be ignorant overprotective cranks when it comes to their sick kids, but try not to just leap to that conclusion. Give the parents a chance; then you can decide they are just stupid cranks.
I’m really rambling now. I hope there are some nuggets in there for you.
This is valuable advice to anyone who is responsible for someone else’s care. I found it out the hard way with both of my parents as they aged and became ill. My mother died of ALS (Lou Gehrig’s Disease). Just getting that diagnosis was remarkably difficult and I did tons of research and spoke to many doctors about her symptoms, possible diagnosis and treatment options. Neurologists were not pleasant to deal with and did not like diagnosing this- something they can’t “fix”. My father had multiple issues and was older when his medical issues caught up with him. Again, I had to educate myself and advocate strongly for treatment, rather than just palliative care. With “elder care”, there are many doctors who go with the “just make them comfortable cause they’re old and gonna die anyway” attitude. It sucks and you have to be pro-active and informed to ask the right questions and push for active treatment.
I’m sorry you’ve had to learn this with your child, but good for you for staying on top of things. Doctors are human; some are decent people who are open-minded and interested in learning more regardless of the source of that info- and some are condescending assholes that think because they have a certain education, no one else can possibly know more (or differently) than they do. I’m glad you found one of the first type.
Yes, lots. Mostly confirming what I already believe, esp. with regard to doctors’ arrogance and fallibility and my strong belief that we’re (the health care team) essentially *employees *of the family and should involve them more in care and decisions than we do now.
The only sad part for me about working in the NICU is how many babies are alone almost all the time, save for the nurses. Where are the families?! But then I’m reminded that not all parents have the strength to be there. I think (just to present a tiny defense for a fairly indefensible practice) that we see those kids flying solo much more often than we see involved, educated parents, and so we assume you’re the former until you can prove to us you’re the later. It’s statistically more likely that you don’t want to know stuff than you do. I’m sorry you got caught in this net of assumptions. I hope that I can develop a better sense of who wants to know what when, or even develop some sort of questions that can help me figure that out sooner than they do now.
Thank you so much for sharing that, it really is helpful.
the hide their heads in the sand types out number the knowledge types. medical staff are more accustomed to the sand types.
i’m glad you are able to find doctors to work with you, and see you as a valued team member. at this point you and your wife are sma specialists.
doctors have to know about a wide spectrum of childhood issues, the bearflags are zeroed in on sma. y’all can look for every book, article, mention, personal story on it, doctors can’t. they have an overview knowledge, y’all have an indepth knowledge. working together is the best and most logical way to keep getty happy, healthy, and charming us all.
don’t worry about rambling, you are teaching us all very important things in your posts.
Bearflag70 - what you say above about being an advocate for your child’s health is true with almost all health issues, and not just the serious ones.
When my little one was diagnosed with JRA I can remember pushing for the anti-inflammatory injection in her knee since no one else seemed alarmed that she was learning not to use that leg properly. Now seven, she has no lingering effects except for when she skips, she does it on one leg only, unless we remind her to use both.
Doctors and nurses are well-trained and well-educated professionals, but you know your child.
Bearflag70 - what you say above about being an advocate for your child’s health is true with almost all health issues, and not just the serious ones.
When my little one was diagnosed with JRA I can remember pushing for the anti-inflammatory injection in her knee since no one else seemed alarmed that she was learning not to use that leg properly. Now seven, she has no lingering effects except for when she skips, she does it on one leg only, unless we remind her to use both.
Doctors and nurses are well-trained and well-educated professionals, but you know your child.
The first time my mom met my childhood orthopedist was in the hospital’s brace shop, where she could not convince the tech that something about the way they designed a back brace for me was hurting me. I’d been wearing braces since I was 18 months old so I’m quite sure that there really was something wrong with the thing, it’s not like I was unfamiliar with the concept, as I was six at this point.
The doc came in and asked her what was going on. She told him. He turned to the tech and said something like, “Change it the way she says to. She knows her child.” He also learned really quickly that he could use the big words with my mom and she understood them. He and everybody else there were really awesome.
You sound like you are an awesome advocate for Getty. Keep it up! There are good docs out there who will listen to you and work with you, the trick sometimes is finding them.
When my son went into hypertensive crisis (then eventually congestive heart failure) as a newborn, and had a mass on his left adrenal glad and I was asking questions, advocating, etc I asked a NICU nurse what happens to the babies of teenage moms or people who don’t have the knowledge I do or the strength to advocate for their child.
She said “We treat all patients alike and they get the same excellent service”
I was so greatful when we were air ambulanced to London ON.
Too late for edit, the point was to never stop being an advocate for Getty. I know you won’t you’ve been amazing so far. I think parents of sick babies are some of the strongest people in the world.
We are sometimes struck by the horror of the thought of where Getty might be right now but for our knowledge and advocacy. Once we get over that thought, we feel horrible for those who don’t have the connections, knowledge, or advocacy skills we have… those who simply do what the doctor says.
SMA kids can’t cough effectively because they are too weak. You keep the lungs clear with a cough assist machine. Get all the gunk up and out with a cough assist and a suction machine. We have a cough assist machine because we asked for it. It is considered a basic piece of SMA hardware, as we learned not from our doctors but through the SMA community.
Today, my wife got a call from a newly diagnosed parent whose SMA child had a rattle going on in the lungs. Her doc just sent her home with antibiotics. Our first reaction was this parent needs a cough assist machine ASAP to get that gunk out of there before it gets infected. None of her docs had ever told her that before. Now, she’s going back to her docs to try to get a cough assist.
We are glad that we are now learning the ins and outs of SMA, and we are not only helping Getty but also helping other newly diagnosed families. If we don’t know the answer to a question, we have a whole network of parents who have been doing this stuff for years, willing to lend others a hand. The SMA community is amazing.