My 14yo son has just been diagnosed Achalasia type 3

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Hello all! I plan to read throughout many of your stories as I can to attain information about this new diagnosis for my son but in the meantime, I have many questions. After having the Esophageal Manometry, it was confirmed that T has Achalasia Type 3. He is a very active and bright young man who is quite frustrated with this "inconvenience" as he calls it. It is difficult for me as his mother to get T to be completely honest about how poorly this is effecting his everyday life because he would much rather pretend it does not exist. His physician, while seeing the extreme results during the Manometry asked T "are you not having more pain than you have described?" She was surprised by his responses which is typically what I get from him..."doesn't hurt that bad" "it's not that big a deal".  So, this is my dilemma. How do I submit to such a radical surgery at this point in time when T doesn't seem to be ready for it? What are your experiences? I read about "progression", what does that look like? Is it better to have surgery before the progression or do I wait for more severe symptoms that T can no longer ignore? Here are his symptoms now: difficulty swallowing which he compensates for by eating smaller amounts of food at a time and he eats less often, pain in chest when eating although this is very inconsistent(which is why I think he can ignore). Also, he was having episodes of throwing up food he had just swallowed but this has not happened in about three months....how did he get this symptom to subside for now??? I said T is very active and I mean VERY...he is on two Lacrosse teams, one of them a travel team but he does NOT eat enough in my opinion for a pubescent athletic boy. He has recently thinned out but I'm suspicious of a growth spurt which is normal to puberty at this age and maybe nothing to do with Achalasia effects. How can I tell? I should also mention that T suffers from migraines and has since he was 4 years old. He was diagnosed at a young age with mild Chiari 1 Malformation. Anyone else out there with this anomaly as well? We are waiting for his follow up appointment to discuss the results of Manometry test. I do very much trust the physician he is seeing but I want to be prepared as best as possible in making a decision for T. Any feedback would be greatly appreciated. I know I rambled and probably missed important information but my head is spinning over this diagnosis. 

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  • Posted

    Hi Jttysmom

    I am Shivashiva's mum. I am amazed because what you have described is exactly what I am experiencing with my son. My son is 14 as well. He was diagnosed with Achalasia two months ago. His manometry results showed that he has type 1 and 2. The doctors have suggested for a balloon dilation. He has difficulty swallowing and makes sound at night time. I make sure that his head is raised when he sleeps by using double pillow. He has good days and bad days but does not complain. He does not think it's an issue which makes it difficult for us to make decisions. He is keen to get a dilation though. I would say go ahead with what the doctors suggest but always get second opinion. POEM the new less invasive surgery is something to consider. 

    I would like to be in touch as our sons are of the same age. All the best. 

    • Posted

      Shivashiva,

      Yes, as, I would also much like to be in touch with you as well. I am so thankful to be getting feedback about this condition. 

      Funny you should mention the noise, my son also has a funny sound that comes from his throatlike a gurgling sound. I haven't had him sleep with an incline yet. Why do you do this? Was it advised or are you combatting a specific symptom your son exhibits? My son hasn't complained of problems while sleeping recently but he has in the past thrown up after bedtime. I think because he his type 3 and this affects lower esaphogus he is less likely to have choking issues...not positive on this yet. Does your son have good days and bad? Today was a bad day for my son. He is running a fever from some virus we think and it took him almost an hour to eat a sandwich. I was shocked he ate it, typically he will give up. I watched him take a bite and walk around for a bit, come back to it and repeat. 

      Where do you live if you don't mind me asking? We are in the US. 

  • Posted

    Also I have read that there could be a link between anxiety and achalasia?
    • Posted

      Interesting. My son is a high stress kid. He is quite difficult in this area sometimes but the good side of this is that he does his best in everything. Great grades in school and works hard to perfect his skills in Lacrosse. But this translates into a stubborn streak as well. smile
  • Posted

    I do not have any knowledge, other than read about Achlasea. Last year I had a very bad reflux problem, I was really scared. I had difficulty swollowing, I would have to chew for ages so that it would go down. I am much better now, ten months on and I can eat normal. But I lost lots of weight, when you have a problem like this, it turns you somewhat off food. With your boy only being fourteen, I would ask your doctor if there is a nutritionist, that can advise you
  • Posted

    It does affect young people, and indeed athletes, but the advantage is that surgery results are dramatically better than if they let things run on for too long without treatment.   The longer it continues, the more the muscles, and nerves driving the muscles, of the swallowing system, get trained into not working properly.

    It probably does restrict the intake of food and nutrition that a young active lad should be taking in.   So in the short term, high energy liquid food might be part of the answer.

    There is an element of having to face up to scary surgery when one does not know for certain what the result will be.   I think that takes a bit of courage, trust in the surgeon, and, ideally, communicating with other patients who have had it done.

    There is a link to a video on the website of the Oesoaphageal Patients Association under Oesoaphagus and Achalasia that may help, but it is linked to the POEM procedure which may not be relevant to you.

    • Posted

      This makes a lot of sense to me. I have already seen a progression with my sons condition and so why would I not suspect it will get worse and not better? I think I just haven't had enough time to process that surgery is really the only option. More importantly, I was told by a nurse where he had is Manometry that the two surgeons at that hospital do NOT have a good outcome reputation. Of course, this is one persons opinion but it did not give me great comfort of course.

      i will check out the video you linked. Do you know how I could begin searching for surgeons who specialize in this disorder? I live in the. US. 

    • Posted

      The video was produced by John Hopkins University but I do not know enough to be able to suggest possibilities in USA.
    • Posted

      I have just had a response from a suregon conatct in London as follows, and hope this helps:

      USC (University of Southern California) Tom DeMeester’s old department. UCSF (University of California San Francisco) Pelligrini's old dept; Marco Patti (University of Chicago). Jeff Peters in Rochester. All excellent

  • Posted

    Hi,

    Sorry to hear this. Our son (7yo) was also diagnosed with achalasia earlier this year but his condition was pretty bad and even had trouble drinking, so there wasn't really an option to not have surgery.

    The operation he had is called Heller Myotomy and it was carried out with keyhole surgery, which seems to be the standard procedure for this condition. As far as I understand means that the muscle of the lower sphincter is cut from the outside, i.e. not through the lining of the oesophogus, which means that he could soup half a day after the operation. In fact, we all ate a lot of soup in the weeks following the operation! We were pretty worried about the operation but he recovered remarkedly quickly without any complications and was back at school after two weeks. 

    The most important thing is probably to find a surgeon you trust to advise you. Any follow-on operations seem to be much more difficult so you want to make sure you find a surgical team with a good track record. There is also a question if a fundoplication should be done at the same time as myotomy is carried out. This helps to reduce reflux, but we were told that it is not required in 70% of cases and can also be done a later point if needed. The downside of a fundoplication is that you can't drink fizzy drinks afterwards. There seem to be different degrees of fundpolication you can have. In our case, the surgeon generally advised against it, but made a decision to carry out a minor fundoplication during the operation.This has worked really well for us so far. We haven't noticed any problems with reflux and our son is still enjoying fizzy drinks every now and then. It's also worth keeping in mind that your son may have regular check-ups after the operation. In our case, he is still under the supervision of the surgical team so it's handy that the hospital nearby.

    Things are vastly better after the operation but our son still struggles on some days. There seem to be a quite a few things you can do to make the symptoms less bad such as not eating too late, finding out which foods do not  'go down well' (such as sticky foods like marshmallow, nori and pizza), not eating in rush and having plenty to drink with the meal. We're still trying to find out what works best for him but it seems there is a quite a lot you can do. 

    It's a little difficult to find out from our son how bad his condition is on the days he struggles. I think he also prefers pretending that everything is fine even when his food doesn't go down easily, so we decided that we monitor his weight to help decide if we need to get more treatment for him (dilation would probably be the next thing).

    In our experience the operation isn't as scary as it may first appear and his quality of life is vastly better after the operation. Although achalasia doesn't seem to be a condition that you can get completely rid of, it sounds like many people can manage it quite well and this also seems to be the case for our son after the operation. 

    Good luck!

    • Posted

      Thank you for sharing about your son. Interesting to me that all the responses I have had so far in young age are male. 

      Does your son have type 1,2 or3? I am reading scholarly articles on this condition that say type 3 has the lowest success rate for the Heller Myotemy. My son has type 3.

      Your son is so young to have to deal with such a big problem. Now that we have this diagnosis, so much of my sons history makes sense to me now. When he was born, for the first year of his life he could not hold down breast milk or formula without having two medications before he ate. The first was Zantac for the reflux and the second was Reglan which has been recalled off the market for his particular problem.  When he got older about age 3-7 he still had reflux issues and went through many test and the outcome of that was Lactose Intolerance and GERD. In the meantime, he developed migraines and an MRI revealed Chiari 1 Malformation at a very slight degree. We spent a couple years focusing on getting those migraines under control and the GERD played second fiddle to that problem. But then around the age of 11-12 he started complaining more about pain in his chest and he began what we would later call his "episodes". Tyler would sit down for a meal and depending on whether it was a good day or bad, he would throw up undigested food, come back to the table, try again until he either felt full enough or he would give up eating at that meal. We thought it was his. GERD getting worse. But after months of this we took him to a specialist near our home. He had an endoscopy, probe test, swallow study, barium study and then finally a Manometry over a 3-4 month period. Finally, an answer that made sense but one we weren't prepared for. He turns 14 next week and I'd say this has been a growing problem since birth. 

      I'm glad to hear about the success of your sons surgery. After reading what you said about keeping track of his weight, it confirmed that I need to take my son in for a check up with his regular pediatrician to see if he is on target for growth and weight. You have given me other things to think about as well, like the fundoplication. I need to educate myself more on this.

      I hope for continued success for your son. He sounds already that he is a brave young boy.

    • Posted

      That's a good point. The diagnosis was done through an barium swallow and xray and we were told that his case was a classical one with good chances of success through an operation. Seeing the x- ray was actually very helpful to understand his condition (although it was a bit of shock to see his dilated oesophagus). I think you're are completely right to check what the success rates for the different types of achalasia actually are.

      In our case the symptoms started fairly suddenly (burping like sounds during meals, small amounts of regurgitated food on the pillow in the morning every now and then) and then there was a fairly steady and quick progression over the next four months until he was hospitalised after dehydrating. We found just having the diagnosis was already a great relief to us!

      I wish you the best of luck with your son. Although it's a very nasty condition to have, it does seem that there is a lot you can do to make it less bad.  Maybe we've just been lucky so far but achalasia actually affects our son's life in a relatively minor way only a few months after finding out he had this condition. I hope things work out well for you!

  • Posted

    Hi, I'm very surprised how young your son is. I started having problems swallowing when I was 19. I'm 41 now. At the time, they had a hard time diagnosing me because they said it's very rare to see accholaysia in someone so young. Anyway, I wouldn't rush to have the surgery. If he can live with it for now and adjust his lifestyle, I would wait. You learn tricks to help ypu eat. Smaller bite sizes, drinking a lot of water during your meal. I had the surgery about 15 years ago. It didn't cure it, but it made it a little easier to eat. In the past year it has started to get worse and the dr says I need to have another surgery. I am holding off until it gets unbearable. I learn to live with it. I know what foods are harder to get down and I avoid them. I'm sure your son does the same. I hope this helped. Good luck
    • Posted

      Thank you for your response. I'm sorry to hear that your journey has been challenging with Achalasia. Which type do you have? My son has type 3. He also has already adopted behaviors to help him cope. For example, last night he ate a very warm bowl of soup and then was able to immediately eat 4 cookies with ease. Warm liquid helps and he takes advantage of those moments.

      I am very interested in hearing what his doctor will advise at his follow up appointment considering his age and this being a long term problem no matter what choice is made. I do know that I have read the quality of the Heller Myotemy matters for success but so does the particular circumstances of the patient. For example, type 3 has a low rate of long term success in Heller Myotemy.  It is hard for sure to make a choice like this for your child. I will definitely be considering all opinions and experiences. Thanks again for your feedback...it is very helpful to me.

  • Posted

    Btw, after I gad the Heller's procedure done...I would go once a year or so and have a dilation done....which helps temporarily but causes scar tissue in the esophagus. 

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