The loss of a child for any reason is unacceptable, heartbreaking, and tragic. I try on the idea of ‘there is always a silver lining’ but find the phrase just doesn’t fit in many cases. I do think, through our tears, we can learn something…so forgive me for ranting.

As parents, we need to be vigilant. We need to ask questions and expect answers. We need to be better informed about steroids and more knowledgeable about risk. We need to understand:

1. That complaints of belly pain should be investigated.
2. That 'bugs' at school are BUGS to a child on steroids and have a much greater effect due to immune suppression.
3. That if there are any concerns whatsoever, take your child to the ER and make sure everyone who touches your child knows he is on a steroid regimen (and whatever else he is taking)
4. That we actually need an 800 number of on-call experts to be available for guidance to primary care docs, ER docs – anyone who cares for our sons. I'm not sure about your house, by my children never got sick when the clinic was open, rather they got sick in the middle of the night or on a weekend when the 'regular' doctor was not on-call and a partner was taking is call. The Duchenne community has to have some ability to connect to experts 24/7, no matter the time of the day, the holiday or weekend. It is unforgivable that they see experts every six months to one year and then have no communication system in place for questions and/or emergencies. This is critical now for sure, and will become an even greater issue in the future as children participate in clinical trials, often traveling great distances and returning home in between visits.
5. The primary care doctor and rural ER may have insufficient understanding of Duchenne, no knowledge of the pathology, no experience with regimens (steroids, supplements, ace inhibitors, etc.), and will be totally lost if someone mentions (for instance) antisense oligonucleotides (or even exon skipping).

Steroids are the gold standard and we certainly don't want to have all parents stop steroids. At the moment, steroids are the ONLY drug that has been shown to have benefit in Duchenne. As you know, when boys start steroids, most (except for the nonresponders) boys improve in strength. They are able to do thing that have been impossible and out of their reach. They jump, they run, they ride bikes, and they are better able to keep up with peers.

But we have become pretty cavalier and I think we have all taken steroids for granted. Clearly the physicians discuss the predictable side effects: growth delay, delayed puberty, increased hair growth, and cushionoid face. We accept these side effects somehow, though, for our sons because data suggests they will walk longer – and walking, for most parents, is a goal in itself, no matter the consequences.

What is not typically said, stressed, or repeated often enough, is that steroids can cause other major problems such as ulcers and that they can mask fever and infection, sometimes until it is too late. Parents are not given adequate information or tools. Pain in the gut cannot ever be dismissed. Dystrophin is absent in the smooth muscle as well, and while few of our boys ever see a GI, the other side of the coin is that there are few GI doctors with experience in Duchenne. Parents need to have alarm bells in their head. If you are worried and your primary care doctor does not respond as you think he/she should, go to the ER.

As parents, we need to listen to what our child is saying to us, we need to understand what the doctors are giving our child, and we need to maintain a realistic perspective. As part of the Duchenne community, we have to be vigilant in educating physicians, in fighting for better “round the clock” care, and be prepared to work together to ensure a safe, healthy future for all of our sons.

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Comment by Lynn Bartels on August 8, 2008 at 12:12am
I was reading all of this and the other day my son woke up throwing up and I thought at the time that I was overreacting but i called the acute care clinic on base here and pushed them into giving me an appointment. They didn't want to do anything they said they let bugs run their course in children but i stressed the importance of him being able to take his meds so they ended up giving him fluids and he did improve. After reading the posts I am glad that I did push At the time I thought I was being overprotective and kind of a pain. I am glad now that I did though.
Comment by Linda on June 26, 2008 at 10:02pm
no one had an one at the er at the local hospital, his attending physicians when he was admitted to the hospital, the er at the children's hospital nor the attending physicans that he has when he was admitted there. they had their assumptions but when the tests were ran, they found nothing. i find it hard to believe that not a single person could tell me what was wrong with my son but that's what I was given. i've got appts with a new physican at home for all of my kiddos, plus the appts with a new mda clinic, along with my oldest annual cardiology appt so i will have tons and tons of questions and after 5 years of dealing with this, i learned to not ask..but demand answers to my questions. i'm just hoping someone could help me out while i'm here. i can't take him to the local doctor here...he used to be a vet and has already been sued for malpractice so in a couple of weeks they'll be seeing a doctor, and then there's the matter of getting the authorizations to be assigned a new pcm if we do let him see a doc here. i'm just wondering, how bad is this, they are fine right now...should i take them to an er now or should i wait for their physician...what should i do?
Comment by cheryl cliff on June 26, 2008 at 7:38pm
Hi Linda,

At the hospital did anyone provide an explaination as to why your son's stomach expanded? With that explaination, were you able to speak to a GI specialist and connect it to anything specific such as medications he is taking and or how was this related to DMD?

Anytime a DMD boy who is on steriods is having ANY sort of stomach issue the issue needs sorting out straight away without delay. The reason is the unfortunate example of the recent death in Wales. The steriods "mask" symptoms of illness or injury...the child taking the steriods often doesn't feel or experience illness or injury the same as non steriod takers. This can mean extreme ailments go unchecked because like the energizer bunny the kid keeps on going so parents and physicians don't think anything serious is happening. And, I think it is important to express to the doctors that the WHOLE body needs review. Check all the vitals even if it looks only like a stomach problem to make sure nothing is going on with the lungs or heart.
DMD kids require more intensive care and examination, they are more fragile throughout.
Comment by Linda on June 26, 2008 at 1:10am
I just read this and it really made me sit back. My younger son Matthew was diagnosed in February 2004 and was immediately put on Prednisone. In February 2005, he complained of stomach pain. He always had probs with constipation so I just thought he had to go to the bathroom. Then he started throwing up...again and again until he was dry heaving and his stomach began to expand. By the time we arrived at the emergency room, his stomach was literally the size of a small basketball and with tears in her eyes (thats what really freaked me out) the PA on call had him medi vaced (i know i didn't spell that right) to the Oklahoma City Children's hospital for surgery. Upon arrival, he was found to not need surgery and was given an enema and were sent home. On the two hour drive home, right before we turned into our house, I found that he was fevering and that night his stomach began to expand again. We went back to the ER and he was admitted. Test were ran and they could find nothing. Enemas were given but his stomach continued to expand and after inserting an NG went back down. A couple of days went by and we were taken by ambulance back to the Children's hospital and he was admitted. He stayed in the hospital for a week, with his stomach expanding and coming back down. Tests were ran and nothing was found. Eventually he just stopped and one week later we went home. Can you explain more into depth what happens with the boys who have complications with the steriods. This happened three years ago and he's been taken off of dairy products, put on Prilosec but occasionally still experiences stomach pain and now my oldest Avery who is 10 has been having stomach pains as well. Definetly going to the doc anyways, just hoping that you might be able to fill me in on a little more.

Comment by Pat Furlong on June 18, 2008 at 12:20pm
Doug Biggar has kindly agreed to share his 'medialert' - credit card version. We will have them available at the conference and following on-line. He suggests 2 for mom (because she has so much to carry!) and 1 for men (only one wallet!). Maybe one for others as well -teachers, grandparents, friends. I agree about the bracelet as well, though I realize some boys are reluctant to wear them because they are not 'cool'. Maybe a 'credit card' will work in that case as well.
Comment by Julie Gilmore on June 17, 2008 at 9:59am
Whilst we are on the subject of Medicalert bracelets not many mums (and dads) know that you are also entitled to get a medicalert that states you are the "Primary Carer of a special needs child". You can have a contact number registered with the issuer of the bracelet that enables emergency crews to contact this person if you are ever in an accident. This can be really important if you collect your child from school and they are in an EWC, as often alternative transport needs to be arranged.

If your child has reduced respiratory function, it is also important to discuss with their specialist the effects of opiate based pain medications such as morphine (as these supress the respiratory system even further).
In our situation Mitchells specialist is adament that his is not to have opiate based pain meds, so this also had to go on his medicalert bracelet as it is often the first thing doctors/paramedics administer in the event of an accident.......

Sometimes we laugh that his Medicalert tag will end up the size of a small country town just to accommodate all the information regarding his condition, but in an emergency, you can never give too much information - especially if you or your child are not in a position to offer the information yourselves....
Comment by Lee Ann Faeth on June 10, 2008 at 5:29pm
Dear Pat,

Cheryl's thank you says it all. I am so grateful that we have PPMD and can't imagine how lost parents of prior generations must have felt without having anywhere like this to turn to. Thank you also for posting this specific blog.

My son is going to start steroid treatment within the next couple of weeks and since making the decision in late April, I have had such a bad feeling in the pit of my stomach about starting him down this road, not only due to the side effects, but also out of concern that his local medical providers are not as informed as they should be. I am going to send a copy of my son's records and the Standard Care documents to all of my son's local medical providers now.
Comment by cheryl cliff on June 10, 2008 at 2:30pm
Thank you Pat!!

I will make sure my son has a bracelet and or card. I will also obtain copies to friends and family members as well. We have already registered on DuchenneConnect. Paul and I are really grateful you are there!
Comment by Jenny Garofalo on June 10, 2008 at 12:07pm
thank you so much for these great ideas and your wisdom. you are an amazing women and I hope I am half the mom you are. the idea about the bracelet is awesome. I ALWAYS worry if I am not with him what will happen in emergency if they cannot reach me.
Comment by Bains on June 10, 2008 at 11:49am
I was looking for medical alert braceler in the PPMD merchandise section. It will be nice to be able to purchase from here.

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