Cyclic Vomiting Syndrome and Cannabinoid Hyperemesis

Cyclic Vomiting Syndrome and Cannabinoid Hyperemesis comprehensively reviews the clinical features and pathophysiology of cyclic vomiting syndrome (CVS) and cannabinoid hyperemesis syndrome (CHS). This book differentiates the clinical presentation of CVS and CHS from other vomiting syndromes and provides the information necessary to diagnose and effectively treat these disorders.

Compiled by expert CVS/CHS clinicians and written by physicians and researchers from several disciplines, this reference provides the most updated, evidence-based approaches, and summarizes the latest research on CVS/CHS. Important topics such as the neural systems that drive nausea and vomiting, clinical features of CVS/CHS including its subtypes, insights into pathogenesis, as well as the curious association of hot-water bathing associated with both of these disorders are all explored.

This is a must-have reference for residents and fellows in training, as well as busy clinicians who care for patients with CVS and CHS across multiple care settings including ambulatory clinics, the emergency department, hospitals, and substance use/abuse treatment centers. It is also a useful reference for investigators with an interest in these vomiting disorders.

• Provides a comprehensive review of the diagnosis and management of CVS and CHS and the impact of these disorders on patients
• Outlines the pathophysiology and known factors that contribute to CVS and guides further investigation and treatment
• Explores the role of cannabis in CVS and CHS, reviews the current literature, and identifies knowledge gaps that need to be addressed

• Language: English
• Publisher: Academic Press
• Release date: Jul 14, 2022
• ISBN: 9780128217405

Book preview

1

Cyclic vomiting syndrome health experiences among patients and their caregivers

Kathleen A. Adams¹ and Ashley D. Jensen²,    ¹Cyclic Vomiting Syndrome Association and Medical College of Wisconsin, Milwaukee, WI, United states,    ²Cyclic Vomiting Syndrome Association, Milwaukee, WI, United states

Abstract

Cyclic vomiting syndrome (CVS) is an idiopathic, functional gastrointestinal disorder, now referred to as a disorder of gut–brain interaction appearing in adult and pediatric patients across the globe. Signs and symptoms of this illness mimic other illnesses such as food poisoning, gastroenteritis, or gastroparesis, making diagnosis difficult and often delayed for years. Patients are often given implausible diagnoses such as bulimia or psychogenic vomiting and are often dismissed by the medical community. However, the stereotypical and repetitive nature of the condition reveals the difference. Patients report abdominal pain, nausea, and vomiting as often as every 5–10 minutes occurring in cycles of varying frequency and potentially causing serious health complications. These complications can include electrolyte imbalances, peptic esophagitis, hematemesis, and/or gastrointestinal bleeding from mucosal lacerations termed Mallory–Weiss tears often requiring frequent hospital admissions. In adults, frequent, incapacitating episodes have been shown to cause job loss among 29% and disability among 32% by the time they are diagnosed.

Keywords

Cyclic vomiting syndrome; disorder of gut–brain interaction; mitochondrial DNA; Cyclic Vomiting Syndrome Association; CVS; hematemesis

1.1 A mother’s story

My daughter, M, was only 18 months old when the episodes began. They soon escalated into eleven years of indescribable undiagnosed episodes of misery lasting two to three days. She was hospitalized every six to eight weeks with symptoms of a ‘conscious coma’, relentless nausea and vomiting five to six times during the worst hour, often with blood and or bile present. She experienced drooling, pallor, and an insatiable thirst. This was frightening to all who witnessed her full-blown episode. At 42 years old now, M is mostly free of the episodes but is still significantly affected by the impact of those years.

Cyclic vomiting syndrome (CVS) is an idiopathic, functional gastrointestinal disorder, now referred to as a disorder of gut–brain interaction appearing in adult and pediatric patients across the globe. Signs and symptoms of this illness mimic other illnesses such as food poisoning, gastroenteritis, or gastroparesis, making diagnosis difficult and often delayed for years. Patients are often given implausible diagnoses such as bulimia or psychogenic vomiting and are often dismissed by the medical community. However, the stereotypical and repetitive nature of the condition reveals the difference. Patients report abdominal pain, nausea, and vomiting as often as every 5–10 minutes occurring in cycles of varying frequency and potentially causing serious health complications. These complications can include electrolyte imbalances, peptic esophagitis, hematemesis, and/or gastrointestinal bleeding from mucosal lacerations termed Mallory–Weiss tears often requiring frequent hospital admissions [1–3]. In adults, frequent, incapacitating episodes have been shown to cause job loss among 29% and disability among 32% by the time they are diagnosed [1,2].

There are four phases of CVS: (1) inter-episodic or wellness, (2) prodromal (aura), (3) emetic, and (4) recovery. Treatment is based on the phase that the patient is experiencing when he/she presents to a health-care facility or provider [1,2,4,5]. Current literature presents CVS as a high-impact disorder affecting physical, emotional, and social health. This chapter will highlight what it is like to live with CVS and how this illness impacts the lives of sufferers and their caregivers.

1.2 Patient perceptions about causes of cyclic vomiting syndrome

Each patient will have different values and experiences and therefore will describe similar events in different ways. Each will act on those perceptions based on personal meaning. Understanding patient perceptions of their illness is crucial because perception influences individual illness management decisions and responses to therapy [6].

The pathogenesis of CVS has not yet been determined, but studies suggest that autonomic dysfunction, neuroendocrine dysfunction, dysregulation of brain–gut components, and mitochondrial dysfunction are responsible [7–12]. Boles et al. found polymorphisms on the mitochondrial DNA of pediatric-onset CVS, but this was not replicated in adults or confirmed [13]. Most patients believe that their illness is like any other vomiting illness until the episodes begin to recur in a recognizable pattern. Their beliefs include chemical imbalances and triggering events such as traumatic experiences, menses, mental or medical health issues, environmental exposures, and/or genetics [6].

1.3 Cyclic vomiting syndrome onset and symptoms

At the age of about 20, M’s probable diagnosis went from ‘undiagnosed cyclic vomiting’ to ‘undescribed mitochondrial mutation’. The condition manifested as mild cognitive disability and CVS. In this context, I was better able to understand her classic CVS symptoms of the years before. I could tell with dread, the first sound of the episode as M landed hard on the floor upstairs with a distinct thump. The monster had returned. The debilitating lethargy was interrupted with relentless nausea and repeated bouts of vomiting, drooling, pallor, and insatiable thirst. I now understand that devouring volumes of water helps bring on vomiting which relieves the nausea if only momentarily and dilutes the burning sensation of bile. Relief came in short stretches of blessed sleep.

As M’s mother, my self-talk was endless. This is far from normal; M can’t be the only one in the world who does this! This is physiology gone awry.

CVC is classified based on the onset of symptoms: pediatric-onset (prior to 18 years) and adult-onset (18 years or older) [2,13]. Whether you are an adult patient or a caregiver of a child with CVS, the initial episodes are described as a typical vomiting illness that will resolve on its own. After the first few episodes, it is recognized that this is not normal; there is a stereotypical pattern or cycle. The episodes return time and time again, most often in the early morning hours.

1.4 Receiving a diagnosis

The diagnosis was made based on M’s history and physical. Relief and optimism were monumental. As a result of that definitive diagnosis, M was started on amitriptyline along with frequent attention from a caring and knowledgeable physician. This led to a somewhat gradual but clear improvement in her condition. There are few words that can adequately describe the emotional impact of having this diagnosis.

Patients with CVS suffer for months, years, or even decades without a diagnosis. Because this is a chronic disorder, frequent care is sought in a variety of health-care venues, including emergency departments, urgent, primary, and/or specialty care clinics. Visit after visit, patients or caregivers describe their course of illness only to be told the tests are normal and that there is no answer for their symptoms. Because CVS is not well known, often misunderstood, and variable from patient to patient, clinicians often give up on the rigors of diagnosis and treatment. Recognizing a pattern of illness gleaned from the patient’s history is paramount in achieving the correct diagnosis and administration of proper treatment.

The following statement by Smith from 1934 describes the dynamics associated with this disorder: The mother of the patient on account of anxiety and loss of sleep, presents a pathetic figure as the curtain drops on the trying drama. The attending physician is subjected to the embarrassment of his conscious futility in answering the parent’s question as to what measures may be taken to prevent the next occurrence.

1.5 Triggers for episodes

For some, the triggers to episodes are elusive. Among those identified triggers are certain foods/alcohol, emotional or physical stress or excitement, overexertion, menses, public speaking, death of a pet, and lack of sleep. In children, episodes are often triggered by exciting, fun events such as holidays, birthdays, travel, or sleepovers. There is also the repetitive and unpredictable nature of CVS that plays directly into the anticipatory dread and anxiety which is itself a significant trigger. Once a patient has confidence in reliable medical care and a proven abortive treatment, this anticipatory anxiety about an impending attack of CVS is significantly decreased. The main goal of every CVS patient is to identify and avoid anything that might cause the next episode to recur. This learned knowledge of triggers greatly influences behavioral changes such as food and alcohol avoidance, strategies used to reduce stress and anxiety, change in sleeping and eating patterns, and avoidance of people and places that might trigger the dreaded cycle of debilitating symptoms. It becomes necessary to prioritize responsibilities, organize time, and establish good sleep hygiene techniques. Unfortunately, the changes that are made remarkably impact lives such as having to change educational and employment goals.

1.6 Impact of cyclic vomiting syndrome on the day-to-day life of patients

1.6.1 Physical impact

Oftentimes patients with CVS decline physically in ways that have not been described in the literature. These include stringy hair, loss of permanent teeth due to the frequent presence of acidic emesis, and deterioration of accessible veins often leading to the need for a series of central lines for repeated fluid replacement and sometimes significant weight loss. Many patients are malnourished despite being overweight. Unfortunately, patients also experience a significant decline in their overall well-being from the constant mental and emotional strain their bodies endure.

1.6.2 Emotional impact

CVS patients struggle with a chronic, cyclic course of illness that is devastating, painful, and misunderstood. The need to curb participation in school, work, and pleasure causes feelings of depression anxiety, fear, guilt, loss, and embarrassment. As you would expect, objective signs of CVS such as the act of vomiting or soiling themselves in front of others cause immense embarrassment. Many express personal fears regarding death, believing as they age that the attacks may become so severe, they may actually die from the illness. Intense feelings are expressed due to frequent episodes that lead to many hours hugging the toilet or hot-water bathing. This prevents patients from achieving normal living, goals, and dreams and disrupts their entire lives.

In adults and children, guilt is also expressed because of an awareness of the burden on caregivers who are taken away from other normal demands of family life. Many have lost or strained relationships with family and friends describing them as not understanding the illness, passing judgment, or losing interest altogether. However, several CVS patients have expressed that despite the burden, their caregivers (parents and other family members) have never wavered in their support and are often described as life-saving.

1.6.3 Impact on employment

The frequent, unpredictable symptoms of CVS financially impact patients and their families due to an inability to work at all or recurrent absenteeism. They have concerns not only for their medical well-being, but how they will financially support themselves. In addition, job stress and anxiety in the workplace have been described as a common trigger. Questions enter their minds: Will today be the day I attempt to travel on business, but instead find myself on the floor in the airport bathroom wrapped around the toilet desperate to stop the nausea, vomiting, and abdominal pain? Will today be the day I am fired from my job because I had to call in to work just one too many times? Or, on the contrary, will I be able to muster the courage to explain my illness to my boss and find he/she is caring and accommodating?

1.6.4 Impact on education

Significant absenteeism from school is common. In children, loss of school time and normal participation in childhood activity has enormous impact. Accommodations at school, home schooling, or tutoring are sometimes needed. For those who can enter college, there is the struggle with the decision to stay close to family as they often rely on them for health care during episodes. Others find a college that offers online degrees which removes the complication of not being able to be physically present.

1.7 Impact on caregivers of children with cyclic vomiting syndrome

The puke monster had control of our family consciousness. If M was in an episode, the cloud that hung over our home was palpable. Siblings coming home from school and father coming home from his day caring for his own patients would walk into the sound of relentless vomiting from a distant closed-door room. I hit the wall many times with no way over, under or around. In needing to find the emotional energy – my choices were few – insanity or coping. The other children and household demands continued – it had to be coping. I would find myself yet again, draped over M’s retching body mourning her agony. Constantly emptying the bucket, pulling her hair away from her face, trying to keep her clean in hopes she would be able to have some measure of rest.

1.8 Perception about treatment in acute/urgent care

One major area of concern to patients occurs when they are seeking care during an episode. They find that health-care workers often do not recognize the symptoms as CVS even after a diagnosis has been made previously or have never heard of CVS. If CVS is recognized, many health-care providers hesitate to use phase-related treatment recommendations [6,14]. Patients are often accused of drug-seeking due to their request for opioids or reported marijuana use even though they state it is the only thing that works for their extreme anxiety, nausea, and abdominal pain. Others are accused of having mental illnesses due to observed or described self-help behaviors such as hot-water bathing or guzzle and vomit techniques used to alleviate symptoms [6].

1.9 Importance of health-care knowledge

The impact of CVS on our family was lessened by the ease and continuity with which M was cared for by her pediatrician. He arranged for her to be a direct admit to the hospital rather than the more common emergency room experience which can be both frustrating and lengthy. Throughout those eleven years without a diagnosis, this physician was ever present despite his own frustration. Just being consistently available, even though dumb founded himself, made an enormous difference in M’s and my emotional well-being. His search for answers was endless.

For those who have gone for years without a diagnosis, the impact of CVS on the family can be lessened by the ease and continuity of care provided by caring clinicians. Arranging for a direct admit to the hospital rather than the more common emergency room experience of waiting, questioning and speculation, and proven ineffective treatments is beneficial for those with frequent and refractory attacks. Being consistently available, even though, at the time, there may be no diagnosis, the approach of listening and attempting to find a diagnosis will make an enormous difference in the patient and caregiver’s emotional well-being. CVS patients need calmness, familiarity, and compassion.

Many patients, whether diagnosed with CVS previously or not, will present with an individualized description of their cycle of illness and some will provide a variety of identified triggers for their episodes. Because of the inconsistency in the way CVS patients present, clinicians may have difficulty in making an accurate diagnosis. Identifying and understanding CVS in addition to forming health-care relationships with patients is paramount. Patients who perceive a lack of knowledge among health-care workers even avoid care as a result and may succumb to complications from their illness [6]. Forming a partner-in-care relationship while demonstrating concern and respect improves health-care access and quality of life. Patients often report symptoms of anxiety and depression highlighting a need for a biopsychosocial or multidisciplinary model of care to address physical and mental health concerns. Health-care workers should have ongoing conversations regarding prophylactic, abortive, and self-management, including marijuana cessation or reduction in use [6].

1.10 Hope and support

About a year after M’s diagnosis, a handful of 5 families and 3 advising doctors met for 2 days of heavy-laden storytelling, discussion, and camaraderie. As a result, the Cyclic Vomiting Syndrome Association (CVSA) emerged. Now after 29 years of dogged determination by many and guided by a well-defined mission and a stellar medical advisory board, CVSA continues to support, educate, facilitate and fund research.

The Cyclic Vomiting Syndrome Association (CVSA) was established in 1993 by a handful of parents, caregivers, and interested medical professionals and can be accessed at https://cvsaonline.org. Since its inception, the CVSA, its associated medical advisory board, and volunteers have grown to serve the United States and Canada. We also liaise with CVSA chapters in the United Kingdom, Germany, Italy, and Scandinavia (CVSA Nordic). More recently, another CVSA chapter was formed by a patient advocate, Shun Emoto, in Tokyo, Japan. There is now a global presence of support for others coping with CVS. Conferences are held for patients and families every 2 years with up-to-date presentations by medical professionals and programs for pediatric patients and their siblings.

1.10.1 One example of the benefits of the Cyclic Vomiting Syndrome Association

In the early 1990s as part of a play therapy session at a conference of the Cyclic Vomiting Syndrome Association at the University of Missouri Health Services Children’s Hospital, the Puke Monster concept appeared. Children learn to blame the episodes on the monster as an entity outside of themselves rescuing children from being the embodiment of CVS. Conferences with play therapy continue to this day complete with revenge on the Puke Monster with silly string, water balloons, and