My experience with Achalasia

The last 4 years have been a medical roller coaster. Achalasia is clearly not an easy disease to diagnose. I was eventually diagnosed with achalasia on 26 February 2014 at age 28. I am a fit female in the accounting profession and up until 4 months ago I had never heard of the condition. 

I have written this post for two reasons:

1) for fellow achalasia sufferers. As the condition is so rare, I found there was very little information and support as to what the sufferers go through. There are mostly medical sites with facts and figures but a patient needs more than that to understand the process and to be able to relate to someone who has had similar experiences. 

2) for family and friends to better understand the disease and what I have been going through. 

Here is how it all began and what I have done to treat it....

In the beginning...

I'll never forget the day approximately 4 years ago when I was home alone and I thought I was going to die. I suddenly got the most severe chest pain that was so crippling I could do nothing other than lie on my bed, writhing in pain. It seemed to never end and I was convinced that it was a heart attack. I was in too much pain to move and call for help. It lasted for probably about 20 minutes after which I was weak and exhausted.  

The next 3 and a half years kind of blend into one mess of chest pains, misdiagnoses and frustration. The chest pains came and went, from the extreme of having up to 7 in one night (they occurred most frequently in my sleep), to not having any for a few weeks. Getting them while driving or in public places was the worst. I would often end up with a red chest and neck afterwards from the pain. 

After about 2 years of this, I realised that drinking water on the onset of an attack could stop it. This really changed my life - when I had water with me. So I started carrying water with me wherever I went and managed to stop about 80% of the attacks in this way [Please note that these attacks are not necessarily as a result of the achalasia - see later].

Additionally, I struggled more and more with eating from about 2 year after the onset of the chest pains. It wasn't bad initially - the occasional whoopsy at the dinner table where I had to run to the bathroom and choked up food, sometimes it was so sudden I could not make it to the bathroom and sometimes it meant vomiting after a meal. But it was not too concerning or frequent yet.

So, what did the doctors say?? From my first appointment to my actual diagnosis, no one seemed to take me seriously. It was the most frustrating thing for me and for my boyfriend and family. They saw me suffering yet there was no medical reason given for it. Initially I had an ECG after my first chest pain and was told that my heart is happy and healthy. Go home. It was put down to stress. Anxiety. Take a sleeping pill. Have some calming tonic. Don't eat so close to bedtime. Allergies. Cut out dairy, gluten.

They said they couldn't do anything unless they saw the attack happening. So one day I got an attack on the way to work and drove straight to the very GP who told me he needs to see me while having an attack. I was crying in pain in the waiting room and eventually he saw me, while still having the attack, and told me he can't do anything until it stops. He just watched me until it stopped, then listened to my chest and took my blood pressure and told me I seem fine. I also went for a gastroscopy at some point and was sent home having been told I have a very healthy esophagus.

Towards my breaking point, the likely diagnoses moved to acid reflux (GERD) and heart burn. I took Nexium and it did nothing (neither did the sleeping pills in case you were wondering).

My breaking point

December 2013 I went home to Cape Town for the Christmas holidays. While I was there, my body went crazy and I suddenly could not keep food down. I was regurgitating my food almost every meal, and always at dinner time. I experimented to try and control it, thinking it was intolerance to certain foods. I cut out red meat, then salads, soups, dairy and vegetables one at a time, but nothing helped.  Sometime even water would not stay down.

When I got back home in January it continued and I decided to go to a new GP and not leave there until he believed me that something was really wrong. I gave him a rundown of my whole body from a sore toe to a split end in case he could link any symptoms. He listened properly to me and I left with anxiety pills, Nexium for reflux (just in case it helped me) and a recommendation to go and see a surgeon in hospital for further tests. Yay, this was progress!

The tests I had to get done and diagnosis

Please note that I am not a doctor and am just explaining as I understand everything based on my experiences and how everything was explained to me. 
I got an appointment with the surgeon who could find nothing wrong based on an initial checkup, so he recommended I have another gastroscopy in hospital. I was admitted the following week and had the gastroscopy under anesthetic in theater. My esophagus passed with flying colors. Perfectly healthy. The surgeon however noticed two things during the procedure: a slight narrowing of my lower esophagus and a single tertiary wave movement. 

I was sent for a C.T scan and someone working in radiology accidentally (??) told me there might be a tumor on the outside of my esophagus which is causing the narrowing and therefore I needed the scan, with contrast injected into me, to check it out. I was alone with no phone so this was very scary news and did not go down well (excuse the pun). There may have been some tears during the scan. Luckily the C.T scan came back clear (except for a few gallstones). 

I was told I could be discharged from hospital, disappointed and really starting to believe that this was all in my head and I needed a psychologist. Suddenly a man from radiology came to fetch me in a wheelchair (I still had a drip in) for further testing. The tertiary wave and narrowing that my surgeon had seen triggered something that made him want me to have a barium swallow as my final test for the day. I am so glad! 

A barium swallow is where you swallow a white, milky substance that tastes somewhere between a milkshake and a cleaning agent, while having video x-rays taken. The radiologist was shocked and suddenly wanted to get me a bucket as she thought I was going to vomit. I looked at the screen and saw that my initial swallowing is normal but no liquid was actually going down into my stomach. There was a clear tapering off of my esophagus and it was blocking the liquid going into my stomach. As a result, the liquid was building up in my esophagus to the point where I would need to vomit. In the below picture, the white substance cannot go any further down, except for a slight trickle:

This was a turning point. I was not diagnosed by any means, I was just relieved that it was not all in my head, even though the images were worrying. The word achalasia was not mentioned to me yet. My surgeon wanted to send me to a gastroenterologist for an esophageal manometry to test the contractions in my esophagus but there are only two doctors in Johannesburg that he recommended to do this and the wait was 6 weeks. This was an awful 6 weeks for the following reasons:

a) Google is not your friend when you are sick. Do not Google your symptoms. Google told me I was dying. 

b) My achalasia was so bad that my poor boyfriend had to sit through meals with me running off to the bathroom; I was embarrassed many times eating out in public to the point where I avoided it (and did I hear the word bulimia being whispered?!); and I eventually got so bad I had to go onto a liquid diet - soups and smoothies for a few weeks. This continued until my first treatment. 

You would think I would lose weight from all of this. I lost nothing. In fact, I even put on 1kg while on the liquid diet. Many achalasia sufferers lose so much weight before diagnosis. I'm not sure why this didn't happen to me. I think because I kept eating slowly throughout the day to ensure I had enough nutrients and energy, as I get low blood sugar easily. I kept at least something down each mealtime and never skipped meals.

Finally my time came for the esophageal manometry. This is where the doctor puts a thick tube with sensors on it through your nose down into your stomach and slowly pulls it up, taking measurements as you swallow water and biscuits. This tests the strength of your esophageal muscles from your stomach valve all the way up. 

I'm not going to sugar coat it - it is an awful test. Afterwards, I swore I would never go through it again. It feels like they are breaking your nose as the thick tube goes up through the bridge of your nose and your eyes water constantly. A syringe keeps squirting water into your mouth to swallow on command; and try swallowing biscuits with a tube down the back of your throat. 

The results were conclusive though - I have achalasia. This naming of the disease that I have was a big moment, scary as it was. It meant that steps could finally be taken towards healing me.

What is achalasia

You can go and get a more medical definition, but as it was explained to me, the esophagus is made up of muscles that contract and relax performing peristalsis to propel the food down into your stomach for digestion. A nerve ending which controls these muscles has died and as a result my lower esophageal muscles are in a state of almost permanent simultaneous contractions which means that there is a severe narrowing of my esophagus and essentially the valve into my stomach is always closed by these muscles. Because of this, food can only trickle into my stomach and it builds up during the day until it gets so high that I have to regurgitate the food - this is why it is usually worse at night time. My doctor says my esophagus is basically a reservoir of food that slowly is released into my stomach. There is no known cause of achalasia.

There are 3 types of achalasia. All 3 have high pressure (contracted muscles) in the lower esophagus where the pressure does not decrease when the patient swallows, thus not opening up to allow food down. However, they are distinguished as follows:
Type 1 - absence of movement from muscular contractions in the lower esophagus
Type 2 - simultaneous contractions of muscles, instead of a wave, therefore food is not propelled down and the stomach valve is blocked by contracted muscles
Type 3 -  vigorous achalasia with simultaneous spasms, often accompanied by pain from the spasms.

Treatment options

With my doctor and with my surgeon, my options were discussed. I clearly could not do nothing as it was too severe. Here is a brief summary of my options:

a) Taking medication - not recommended

b) Balloon dilation - in theater a balloon is put down your throat and rapidly blown up at your stomach valve so that it rips the contracted muscles, breaking them apart, opening up the valve. Negatives of this procedure are that it may need to be performed a few times until they get the correct size balloon to break the muscles enough, and the muscles may grow back together and therefore this is quite often a short/medium term procedure. 

c) Botox - injected into the esophagus in theater to relax the muscles, thus opening up the esophagus. Not a long term treatment.

d) Heller Myotomy - An in-theater surgery, usually key-hole with 5/6 incisions in the stomach, where part of the esophageal muscles are cut away (being careful not to cut the esophageal lining) in the lower esophagus which opens the esophagus and allows food to fall into the stomach. Peristalsis does not occur as normal but eating is made easier. Then to prevent acid reflux from the permanently-open valve, Dor fundoplication (a partial wrapping of the stomach around the esophagus to make a low-pressure valve) is performed. 

e) Per Oral Endoscopic Myotomy (POEM) - not an option as not performed in South Africa. I could wait for a doctor to come from the States and meet with him but didn't want to wait. 

Botox

I was diagnosed a couple weeks before a trip to Thailand in April '14 for my boyfriends 30th birthday. I was advised that the botox treatment responds very differently in different patients and the length of effectiveness varies greatly among patients. I was told I could opt for the botox treatment as an initial treatment option for fast an effective reflief, but not as a long-term option. I can't remember the exact number, but my surgeon told me he does not want to perform any treatment on me as he has only seen achalasia about 3 times in his years of medicine (!!). 

There is a hospital in Cape Town that has a special interest in achalasia and gastroenterology so, since Cape Town is home, I decided to go there for the treatment where I also have the love and care of mommy, sister, family and friends. The staff and the professor who worked on me are amazing. They came in on a Saturday to meet with me and opened a wing of the hospital for me to do another esophageal manometry (noooooo!). They have more advanced software so they can not only determine if a patient has achalasia, but also the type of achalasia. 

The manometry was just as terrible the second time. And the third time. With my luck, I had to get it done twice in one sitting as the first one didn't give proper readings. I have type 2 achalasia which is good news as its apparently the best to treat but it does not explain my severe chest pains, as the painful spasms occur in type 3 (vigorous) achalasia usually. This led to questions about whether the achalasia and the chest pains are related, or just coincidentally occurring at the same time in my life, causing confusion in diagnosis. The thought at this point was that the chest pains are unrelated and the pains are from gallstones. 

Anyway, I went into theater on Monday 17th March for the botox treatment. This is performed under conscious sedation, meaning that you are awake during the procedure. You are meant to not really remember anything but for some reason I can still see and feel it all, even though I asked the anesthetist for more drugs due to discomfort. Its basically like a gastroscopy but there is a needle which they use to inject botox into several locations. I saw this all on the screen, it was quite cool. 

The botox worked wonders! I had the most incredible trip to Thailand and could eat almost normally with no regurgitation during the trip and for a few weeks after. However, I found myself waiting, wondering when the eating nightmare was going to begin again. The botox has been known to last up to 3 years in some patients, which I was really hoping for. It was a really great and effective option to go for, with immediate relief, since I was struggling so much initially. It did not stop the chest pains. 

However, after about 2 months, the symptoms started coming back - not badly, but they were still there and I was scared as to how long it would take for them to fully come back, so I called the professor in Cape Town and negotiated with work and we settled on 9 June 2014 as my surgery date. We had decided on the Heller Myotomy as my permanent treatment option as it is usually more effective that balloon dilation, and I am young and fit so should recover quickly. 

Heller Myotomy and recovery

Once again, I traveled to Cape Town for my surgery on Monday 9 June with the same professor, who I trust to perform the procedure on me.  This is my first big surgery ever and I was very nervous of the effectiveness, the pain, the recovery and the scars.

On the morning of my surgery, the professor described the surgery to me. I also had an ultrasound and it was decided that my gallbladder will be removed as well (if all goes well with the Heller Myotomy), as I have small gallstones which might be the cause of chest pains as they get stuck in a duct. I had no objection to my gallbladder being removed since the professor recommended it and it may well have to be removed at a later stage anyway.

Since I had the gallstones and achalasia at the same time, there was perhaps a confusion in diagnosis. And if the chest pains are from gallstones then it took 4 years to diagnose something that is easily diagnose-able with an ultrasound; and the achalasia then didn't take quite as long to diagnose since symptoms only started presenting themselves about two years after the chest pains. I may never know what caused the chest pains - whether it is the gallstones getting trapped in a duct, esophegeal spasms from the achalasia, or something completely different. I just hope and pray that they never return and that the combination of surgeries has cured it.

I was prepped for surgery, taken into the theater and put to sleep by noon. 

The procedure was approximately 4 hours (including the gallbladder and a mole removal by a plastic surgeon - I got a full service - apparently its usually at least 2 hours) and I was awake at 16:30, having been in recovery for a short while. 

Recovery in hospital: I woke up in my hospital room, in a lot of pain and immediately got a morphine injection, much to the entertainment of my family who were visiting, who say I was rather hilarious on it :) I don't remember much of that. I was awoken at 3am with a nurse checking my "stab wounds" as they called it. I was relieved to find that I didn't have a catheter and the nurse assisted me to the bathroom. 

My stomach was patched up with waterproof dressings so I couldn't see the cuts. There were 6 dressings, so I assumed 6 incisions including the extra one from the gallbladder. 

Stomach immediately post-surgery

I was meant to be on a liquid diet immediately after the Heller Myotomy but the hospital kept feeding me crispy roasts and sandwiches so I didn't eat too much but I wasn't very hungry. 

For 2 days and 2 nights post-surgery, I only got up to go to the bathroom and to wash myself with a tub next to my bed and put the hospital gown back on. I slept a lot, only on my back and wasn't able to read the books which I had brought with me. I got some pain on night 2 in my esophagus which may or may not be reflux, but I was told that is to be expected straight after the surgery, despite the stomach wrap. It has since gone away.

I stayed on a drip and in hospital for 2 nights, having my vitals checked every couple of hours. I was discharged after 2 nights. 

Recovery at home: Back at home I had so much love and care and was able to rest properly in bed and be fed tea and smoothies and soups. A massive thanks to mommy and sister sweet and all my amazing family and friends! The love, support, flowers and messages just kept on coming. And to my incredible boyfriend who has been with me through this whole process. I would have gone insane without you (perhaps I am anyway). There is an end in sight at last.

I really didn't have as much pain eating soft foods as I had expected and was fine with smoothies and soups and oats.  I was in bed most of the time, on my back, and got very easily tired and dizzy if I stood up for more than a few minutes. I woke up for the first 2 or 3 days at home feeling extremely nauseous and it was a slow process getting up as I had to take medication an hour before eating, then only could I eat, and only then could I take pain medication. 

I spent days in bed watching the Friends series from the beginning with my sister and sleeping in between and chatting to visitors. I could shower from the day I returned home as I had waterproof dressings on and was fine to do basic things like dress and dry my hair. The initial recovery was a lot quicker than I thought.

I stopped taking pain meds so often after about 5/6 days as I wasn't really so sore - more uncomfortable. A week post-surgery I got up a lot easier and was helping out a bit, making tea and moving round a bit easier, though I rested as much as possible. 

8 days post-surgery I flew back to Johannesburg with the help of my amazing boyfriend.  All the walking, and sitting upright and driving are not comfortable, so it was exhausting but nice to be back home. I can now sleep on my side for a while some of the time. 

9 days post-surgery I removed all the dressings at once to reveal my new stomach. Its not too bad. One of the cuts is bigger than expected and I have 7 in total - 2 appear to be really close together. There are stitches in all of them still which will dissolve. 

10 days post-surgery and I'm probably spending at least another week in bed to recover more (work is being amazing about it). I'm sticking to soft and mushy foods, now including overcooked pasta, until I feel comfortable eating more solid foods. I feel fine apart from the occasional painful spasm which I need to ask the professor about. Its just discomfort now and I need to work on getting my strength back and being able to move around more and sit up to be able to survive a day at work. 

I'm keeping the 'stab wounds' together with tape and covering with waterproof plasters. I can drive when I feel comfortable enough to, ensuring that I feel I will be fine to react fast if necessary. I haven't tried to walk further than the lounge since I got back and spend 95% of the day in bed, but I don't feel so dizzy when I do get up and stand for a while. I'm able to prepare a basic (mush) meal myself now. I can sit up but for short periods as its uncomfortable on the cuts. 

Update 7 weeks post surgery: I am eating like a champ. I cannot believe the difference. Meal times are amazing and the stress of eating in public has vanished. I am getting no reflux at all and have only had about 3 esophageal spasms since surgery. I am sleeping through the night without waking up from the severe chest pain and I don't have to always carry water with me just in case. 

My cuts are apparently healing nicely. I had to stop taping them as often, as I had a reaction to the Micropore. I still have 2 external stitches and the rest have dissolved. The stitches seem to have left almost a worse mark than the actual cuts on most of them. I'm putting on a scar gel hoping it will help in time. 

I'll always have the achalasia, but hopefully after this long and painful process, there is an end in sight. Eating may never be normal again but it should be a whole lot better. 

x

Please send questions if you are a fellow sufferer, I'm happy to try and assist any way possible! I hope this has been useful and I will update this with progress as to how I am doing as I go.