Rachel – CVS, a storm in a teacup…

Rachel was born in January 2009, elated at her arrival, little did we know what was to come or what life had in store for us as a family.  She was always sick as an infant from birth; we were just shrugged off by any health professional we saw. It was the norm, “Infant colic” and she was just “possetting or had reflux”.  Call it a mother’s instinct; (having already been a parent for 10 years) something told me it was not the norm for this tiny little person to throw up with such force after each of her feeds that it would reach the lengths it was on the floor/walls. She would scream for hours on end, writhe, twist and squirm. She did not want to be picked up and yet not want to be put down at the same time. We were for months on end ignored at repeated pleas to doctors, told there was nothing wrong with her , we were just being paranoid, hysterical and over emotional. 

Strangely she thrived and never once lost any weight, she was meeting all her infant milestones and this continued until we started to wean her to solid foods.   This is when things started to become grim for her. At 13 months old Rachel took what we now know was her first “episode”. It lasted over a whole week. She lost so much weight and she was unrecognisable to the little girl that she was prior.  The vomiting was irrepressible; it just kept coming, over and over and over again. It was unlike anything I have ever experienced in my life (and have never experienced with her sisters who do not have CVS). She was so listless, pale, her eyes were purple all around and sunken within her thinning face, her little body ran out energy so quickly, it was simply incapable of taking care of her the way she needed it to and this was within 24 hours of onset. We called out hours and they sent a doctor out to assess her. She was admitted to hospital for dehydration which dealt with this immediately and Rachel made a complete turnaround. The “official” diagnosis at the time was “Viral Gastroenteritis”.

9 weeks later, Rachel experienced the same thing again. A sudden onset of paleness, she was shaking, this time I noticed a very peculiar smell on her breath but didn’t know what it was, she refused to eat or drink, she became immobile and listless very quickly and again vomiting commenced.  This time was different. It only lasted a couple hours and she rallied around rapidly. By the time we arrived at her appointment with the GP she looked perfectly fine. The GP said that due to the “virus” she had before sometimes an assault on the gut can leave a lasting impact on the folds of the stomach and it may take a few months for things to return to normal. We weren’t convinced.

For the next few years the same thing kept happening, she would keep doing this, becoming pale, listless, lying totally prostrated and exhausted, vomiting over and over again, sometimes for a few hours sometimes for a day at a time, sometimes a few days at time and then be perfectly fine until it happened again. And it kept happening. It would be either last thing at night before bed or first thing in the morning and even during the night. Every single time it occurred we would take her to the GP to be told the same thing over and over again. There is nothing wrong with your child. You are being paranoid. She is obviously just more prone to viral stomach upset than other children.  She has a tummy bug.

Rachel then started pre – school, and she had so many absences due to these “so called” tummy bugs that kept occurring repeatedly up to 19 times a year. This delayed her entry to school by a year.  She was in and out of the hospital/GP`s for appointments over these unexplained episodes of vomiting and not once did any medical professional pick up that she may be suffering from something else.  This went on for almost 5 years. 5 years of being ignored and dismissed and accused of being over cautious, paranoid and hysterical.

As parents we were angry, scared and so frustrated at having our concerns dismissed by every doctor we saw. We knew that it was not normal for a child to be sick 19 times in a 12 month period. What was happening to our little girl? So I took it upon myself to start searching for the answers.

Eventually I came across the CVSA-Nordic website. It explained what CVS was; it listed the symptoms and pattern of a typical “episode”. Could this be what was happening to our daughter? I started to read more into what CVS was. One website recommended if you suspect that your child or you were suffering from CVS to start keeping diaries of everything. And that is exactly what I did. I recorded;

▪ when her episodes started and when they ended

▪ how long they lasted

▪ tried to identify what the triggers might have been

▪ her presenting symptoms

▪ what medication we used

▪ what non medication methods we used

▪ notes on the episode itself

▪ what she had been doing

▪ what she had been eating up to 48 hours prior to the episode

▪ when her last bowel movement was

▪ how long her recovery was to her normal health

Very quickly we were able to ascertain and positively identify some triggers from the episode diaries within a matter of 12 weeks. Triggers such as; her diet, under/overeating eating, any kind of excitement, illness, allergies, physical exertion, weather and many more have been identified since. This enabled us to implement a regime at home that helped us to manage things a little better as we knew she was approaching the time to start school and  school absences are not looked upon kindly if they can not be explained.  

During her first term of Primary School, her attendance fell to 71% through her episodes and illness triggering episodes on top of being ill which makes recovery for her longer than a child without CVS. We were summoned to her school and asked to explain why her attendance was so low by a member of the local councils Child and Young Persons Team. We explained everything to them. We were very open and honest. She asked if she could access Rachel’s medical records which we did not object to; we didn’t have anything to hide.

Around a week later we were contacted again and it was explained to us that they had been in contact with Rachel’s GP Surgery and they weren’t satisfied with our explanation. Upon contact with her GP Surgery they were notified that Rachel has only ever suffered from the usual “Childhood” ailments. We were notified there and then that if our daughter’s attendance at school did not improve immediately we would be subject to a stage intervention. This implied that we were not fulfilling our parental responsibilities and duty to our daughter with regard to her education. I was enraged and incensed that it could even be suggested that we were to send our daughter to school while in an episode. I asked for clarification if the resources would be put in place for our daughter, for her needs to be catered for and we were notified that her fitness to attend school would assessed by a teacher and if she is deemed fit enough to be there they would provide a “receptacle” for her throw up in if required.  We were in essence threatened to send our daughter to school despite the fact she was not fit to be there.

We refused to agree to this and contacted our GP which had recently changed. Our GP asked for an appointment with us to which we took all of our daughter’s episode diaries and some information we have printed from the CVSA-Nordic website.

At the appointment with the GP she informed us that no one from the Child and Young Persons Team had been in contact with her and if they had been she certainly wouldn’t have shared sensitive information pertaining to our daughter without obtaining our permission directly first. We discussed in length what had been going on with Rachel, showed everything we had and we finally had a medical professional who was really listening to us, who had some experience with CVS in children. She was 100% certain this is what our daughter was suffering from. From the point of that appointment, within weeks we had a referral to a Paed Gastroenterologist who gave our daughter a diagnosis of CVS. The relief, the comfort, the utter and sheer deliverance from the unknown to the known is a weight lifted that only other CVS parents can understand.

Unfortunately things haven’t got any better for our daughter now age 9 since her diagnosis. The frequency of her episodes hasn’t reduced. All preventative drug therapies to date have failed over the last 3 years (Cyproheptadine, Pizotifen, Propranolol). Ondansetron has unfortunate side effects but is effective in stopping her emesis (some of the time), so we now have Stemetil thrown in to deal with the unrelenting nausea that she is experiencing not only during her episodes but out with as well. And she has to deal with some other symptoms old and new out with her episodic period too. The newest and most recent symptom is the most worrying for us as her parents. She has developed muscle weakness to her lower limbs after undertaking physical exercise and after episodes since January this year when she had a particularly bad episode that lasted over a week. She has had an upper GI and MRI, plus blood cultures carried out that can not explain this, it continues to happen but there has been no follow up. We are hoping that her new consultant can delve into this issue more and offer more answers. We truly believe that if our daughter in her earlier years had been diagnosed with CVS instead of being left so long untreated things perhaps would be much different now.

What makes the difference to our life now after diagnosis?

To now have a supporting and responsive professional in our General Practitioner.  To have someone like her who understands, has some knowledge of CVS and really cares enough when they perhaps don’t know what to do but will do their best to find out or refer you to someone else who does. That makes all the difference. She advocates on our daughters behalf in situations when we cant or are unable to, but most importantly she sees our daughter as a person who she wants to help not just a patient that she wants to get in, deal with and roll right back out again. She treats us all with dignity, respect and with care. Everyone should have a GP like ours.   

What frustrates us the most?

The blatant judgement from others, whether that be another person, a doctor or a nurse, who does not understand and they do not care enough to do any research to try and better understand what CVS is, and what a sufferer is going through. Lack of knowledge I can forgive, but wilful ignorance I can not. There is no dignity and respect. The consideration that is given to the health related quality of life of the sufferer in the clinical setting or indeed between episodes is no where the standard it should be, things really do need to change in not only our hospitals but our individual GP Practices where CVS is concerned. The lack of knowledge and experience is alarming, but also it is more alarming how professionals allow their personal opinion to overrule professional obligation to treat a CVS patient based on what they believe rather than what they see. 

Rasheera – My Journey living with CVS

To be completely honest my CVS days are filled with memories of the emergency room and buckets filled with stomach vile and vomit. My mom having to lay cold towels on my head and body because I would sometimes run a fever. Her forcing me into a bath of cold water to help my temperature go down. My screaming and groaning throughout the nights because my stomach would be cramping so bad even though I had stopped vomiting. Somehow, I got used to the idea of sleeping with cups and bowls around my bed just in case I’d have to throw up. My mom would always tell me “ladybug, get you a cup or bowl to keep by your bed.” That was our strategy, we quickly found ways to help us adapt so when the episodes came we were as prepared as possible.

Me living with CVS not only affected my life, but it greatly affected everyone around me. It affected my mom who was a single parent. Many days she would have to leave work early to pick me up from school early because my episodes would be triggered.  We never knew when an episode would trigger, and we never knew if it was going to be an episode I could ride out in the hospital or at home.  We always prayed it would pass by smoothly but that was always up to my body.  Most of my episodes lasted anywhere from ten to fourteen days. The two-week long episode where the hardest, usually after a week if I wasn’t better I’d get checked into the nearest hospital and they pump my veins with fluids.

Many nights my mom spent laying on a cot by my hospital bed knowing she had to go to work the next day. My siblings were also greatly affected too.  During the times I got sick my siblings had to be left at home with a sitter. It was hard on all of us. It affected my siblings in small ways too not only having to adjust with having my mom gone to be with me but even at home. In having to cover my chores because I was too weak to get out of bed. They were forced to not only take over my chores but to also help clean up after me whenever I was going through an episode. Many times, my mom would send them into the room to check on me and they would grab the bucket and cups of throw up from around my bed, pour the throw up down the drain clean out the tub and bring it back to my room with liquids to drink. My older sister whom I shared a room with really tolerated a lot. She sometimes had to leave the room because I would get so sick I literally had to be quarantined.  As we got older we eventually had our own rooms but for minute we shared our space and she always knew first whenever I get sick.

I remember thinking at a young age how exhausting it was trying to talk. If I wasn’t throwing up, I was dry heaving, and if it was neither my body reeked with stomach cramps and extreme nausea to the point you couldn’t think. I would cry and tell my mom “nothing is coming out” and she would rub my hand and say, “I know baby, I know.” Living with CVS was secretly tormenting because other people couldn’t understand it was more than the stomach flu, but it was literally a life crippling syndrome that never seemed to leave me alone. Doctor’s had little resources or medical treatments during the peak of my syndrome. As I got older my episodes came less frequently and my body got stronger. My last vomiting episode was when I was 16 years old and that episode lasted for 10 days.

I’m not sure why I don’t have CVS anymore. I know if it wasn’t for m FAITH and FAMILY I wouldn’t have made it through those tough moments.  I still experience many issues in my digestive system and my stomach is extremely sensitive, so I don’t eat everything, but I am learning about different lifestyle choices and adding changes to my diet.  Knowing your triggers and stress reducing mechanisms help cut down on the symptoms a lot. My heart goes out to not only all the child and adult patients going through this syndrome but to all the loved ones on standby. It takes a lot of strength to watching your love ones suffer.  However, I can say I represent Hope that you can go into remission for this disease. Your life doesn’t have to revolve around CVS.  One of my prayers is that they continue to invest in drug treatments and research for CVS because no one should have to live with this debilitating disease.