Pickwick Syndrome: Causes, Symptoms & Treatment

Pickwick syndrome is a condition that occurs in people who are extremely overweight. It is a form of obstructive sleep apnea.

What is Pickwick syndrome?

Pickwick syndrome takes its name from a character in the novel “The Pickwickians” by Charles Dickens. In this book, the coachman Little Fat Joe sleeps almost the entire time. Patients with Pickwick syndrome also suffer from extreme fatigue on a daily basis. Pickwick syndrome is also called obesity hypoventilation syndrome or obesity-related hypoventilation syndrome. It occurs exclusively in people with severe obesity, that is, with extreme overweight. The World Health Organization (WHO) refers to obesity as having a body mass index of more than 30. However, patients with Pickwick’s syndrome often have a BMI of more than 40 or 50. As a result of the obesity, hypoventilation syndrome develops. In hypoventilation, normal lung ventilation is abnormally decreased. The term hypoventilation is often used interchangeably with the term respiratory depression. However, hypoventilation actually refers more to lung ventilation, whereas in respiratory depression, respiratory control is impaired. Decreased ventilation of the lungs limits gas exchange, resulting in an undersupply of oxygen.

Causes

The main cause of Pickwick syndrome is pathologic obesity. The obesity creates a narrowing in the upper airways. The lungs also become constricted by the surrounding tissue masses. Pushing up the diaphragm, a very important mechanism for breathing, is made difficult by the masses of tissue that must be moved. So-called stenotic breathing occurs, especially at night. Patients have to breathe against the tissue. Due to the strain on breathing, the lungs are less ventilated and the alveoli receive less air. This condition is also known as alveolar hypoventilation. The decreased alveolar ventilation is also seen during the day. There is an undersupply of oxygen (hypoxemia). At the same time, however, too little carbon dioxide is exhaled, so that an excess of carbon dioxide develops in the blood in addition to the oxygen deficiency. This excess of carbon dioxide is also known as hypercapnia. It is believed that chronic hypercapnia serves to protect the respiratory pump. Normally, carbon dioxide levels are the strongest stimulus for breathing. However, the respiratory center becomes less responsive to chronic hypercapnia, causing a shift in the set point in respiratory regulation. Respiration is reduced and the oxygen content in the blood decreases. The body responds by producing more red blood cells (RBCs).

Symptoms, complaints, and signs

Respiratory weakness is most apparent at night in patients with Pickwick syndrome. It manifests as an accompanying and beat-related respiratory disturbance. Night sleep is not restful, resulting in marked daytime sleepiness with sleep attacks. Here the symptomatology resembles the sleep apnea syndrome. Breathing is irregular and there are periodic breathing pauses. These occur mainly during sleep. However, if Pickwick’s syndrome is pronounced, breathing may also be impaired during the day. Sleep disturbances and heavy snoring are also typical of the disease. Other important symptoms are an increase in the CO2 content of the blood (hypercapnia) and a decrease in the oxygen content of the blood (hypoxia). Furthermore, arterial hypertension (high blood pressure) develops. However, hypertension is found not only in the large body circulation, but also in the pulmonary circulation. In medical terminology, the increase in blood pressure in the pulmonary circulation is called pulmonary hypertension.

Diagnosis and course of the disease

Initial clues to Pickwick syndrome are provided by visual findings. Patients with Pickwick syndrome are conspicuous by their extreme obesity. Further diagnostic clues are provided by blood gas analysis. Blood gas analysis provides information about the gas distribution of carbon dioxide and oxygen in the blood. In patients with Pickwick’s syndrome, the oxygen content in the blood is lowered. The carbon dioxide content, on the other hand, is increased. Further examination procedures are performed to confirm the diagnosis. For example, long-term blood pressure is measured. Certain blood lipid values such as HDL, LDL and triglycerides are also determined. An ECG is performed to assess cardiac function.Echocardiography can also be used. Furthermore, X-ray diagnostics are used. Pulmonary function testing records the various lung volumes and other clinical measures.

Complications

A feared complication is the development of pulmonary hypertension. This is a constant high blood pressure caused by the compression of the pulmonary vessels. Due to the high pressure and triggered by obesity, there is also an increased risk of heart disease. This can lead, for example, to a weakness in the performance of the right heart. The arteries calcified by fat are to blame. The risk of suffering a heart attack is also increased as a result. Difficulty in breathing, which occurs not only during the day but also during sleep, leads to nocturnal cessation of breathing and even respiratory arrest. During the day, in the advanced stages of Pickwick’s disease, a bluish discoloration of the face (“blue bloater”) and constant shortness of breath may be added. The nocturnal breathing problems result in pronounced daytime fatigue. Some patients become permanently unable to work as a result and have to retire early. If there is an increase in the number of red blood cells (polyglobulia), there is an increased risk of thrombosis, in which blood clots form on the walls of the blood vessels. If these detach and travel upward, the dreaded pulmonary embolism develops. This also leads to shortness of breath and sudden heart failure.

When should you see a doctor?

Obese people who notice irregular breathing, insomnia and other signs of a serious condition should consult the doctor. Pickwick syndrome results from extreme obesity and can be treated by weight loss. This requires early diagnosis, preferably before complications such as pulmonary or arterial hypertension or hypoxia have developed. Persons suffering from obesity should consult the physician if unusual complaints occur that go beyond the usual accompanying symptoms of obesity. Sleep apnea should be promptly evaluated by a physician. If respiratory arrest occurs as a result of sleep apnea syndrome, call 911. Pickwick’s syndrome is diagnosed by the primary care physician. For treatment of the underlying obesity, individuals should consult a nutritionist. Physiotherapeutic measures must be carried out for weight reduction, for which the sports physician or a physiotherapist is the right contact. In addition, a stomach reduction can be considered, which is performed as an inpatient procedure and requires follow-up care by a gastroenterologist. Any accompanying psychological disorders should be worked through with a therapist so that obesity can be alleviated in the long term and Pickwick’s syndrome can be remedied.

Treatment and therapy

It is imperative that patients with Pickwick syndrome reduce their weight. Weight reduction can be done conservatively with dietary changes. Alternatively, gastric bypass may be performed. In addition, patients must strictly avoid alcohol. Sleeping pills must also not be used despite the sleep disorders. Sleeping pills decrease respiratory drive and are therefore contraindicated in Pickwick syndrome. Since Pickwick’s syndrome can have life-threatening consequences depending on its severity, therapy is always started in specialized centers with a sleep laboratory. In milder cases, it is often sufficient if patients are positioned differently at night. In severe cases, positive nasal positive airway pressure therapy (nCPAP) is used. This involves nocturnal self-breathing. Very advanced cases can only be treated by home ventilation. In this case, patients are ventilated by machine. A life-threatening late consequence of extreme obesity, Pickwick syndrome can be fatal within a few years.

Outlook and prognosis

In general, the further course of Pickwick syndrome is very much dependent on the health condition of the affected person, so that a general prognosis cannot be given here. The course also depends very much on whether and how much weight the affected person loses and whether action is taken against the excess weight. If the underlying disease is not cured, the symptoms of Pickwick’s syndrome usually do not disappear and in many cases may even increase significantly.Therefore, a doctor should be contacted at the first symptoms and signs and treatment should be initiated to prevent further complications and discomfort. In the worst case, the enormous excess weight can lead to the death of the affected person if no action is taken against it. If the excess weight is reduced, the symptoms of Pickwick’s syndrome usually disappear. They can also be completely reduced if the excess weight is completely eliminated. In severe cases, the affected persons are dependent on ventilation by a machine. In general, a healthy lifestyle has a positive effect on the further course of the disease. The overweight itself can thereby also significantly limit the life expectancy of the affected person in many cases.

Prevention

Pickwick syndrome is a consequence of severe obesity. Therefore, overweight patients can prevent the syndrome with weight loss. For a normal body weight, a healthy and balanced diet is the absolute prerequisite. A wholefood diet with a high proportion of fruits and vegetables can counteract obesity. In addition, overweight people should ensure sufficient exercise. However, in the case of very severe overweight, a doctor should be consulted before weight reduction to support weight loss.

Aftercare

In most cases, only a few or very limited measures of aftercare are available to the person affected by Pickwick syndrome. Here, the patient should seek medical attention at the first signs and symptoms of the disease to avoid further complications or other medical conditions. In general, the further course of this disease depends very much on whether and how the affected person can reduce his excess weight, so that a general prediction is not possible. However, a healthy lifestyle with a balanced diet has a positive effect on the further course of the disease. A doctor can also draw up a diet plan for the person concerned, which should be followed in any case. The sleep complaints can be alleviated with the help of sleeping pills. The person affected should always take the correct dosage to avoid poisoning. In the case of overweight, a reduction is necessary in the long term, as this significantly reduces life expectancy. In some cases, sufferers are also dependent on the help and support of their own family in everyday life to prevent depression and other psychological upsets as well.

Here’s what you can do yourself

People who have Pickwick syndrome should seek medical treatment early. By constantly improving the quality of their sleep, sufferers can often alleviate the symptoms themselves. Regular sleep is particularly important. Sufferers should go to bed at the same time every day and sleep between seven and nine hours a day. Optimally, a sleep mask, earplugs and other aids are used to improve sleep quality. While good sleep hygiene cannot cure sleep apnea, it can significantly alleviate symptoms. Individuals who suffer from obesity should initiate dietary and exercise measures to correct weight problems in the long term. Pickwick syndrome patients who are in an advanced stage of the condition should not sleep without supervision so that emergency medical services can be called immediately in the event of a medical emergency. At best, the condition is treated at an early stage, which requires early diagnosis. Sufferers can often improve their symptoms by sleeping in a different position or adjusting their sleep patterns. Factors such as diet and body weight also have an influence on the development of Pickwick’s syndrome.