Cystic Fibrosis And The Reckless Behavior Of The Patients Due To Psychological Damage

Abstract

Cystic fibrosis (CF) is a disorder of the cystic fibrosis transmembrane conductance regulator (CFTR) gene resulting in intra and extra- cellular imbalance in sodium and chloride concentrations. The people who struggle with CF have thick lung mucus, filling the airways with pus and risking them to bacterial infection. There is no cure for this, as people use different treatments for the specific symptoms. The age expectancy is 30 years old, fact which affects many children, teenagers and adults who are prone to have mental illness problems and act rebelliously because of reasons such as: social pressure, wish to be individual or the lost of hope. A lot of this destructive behavior ends up with them taking and trying illegal drugs, drinking alcohol and smoking cigarettes and marijuana. CF patients that already have a weak body shouldn’t take this risks which affect the already existent problems.

Due to ethical reasons there haven’t been many studies on how illegal drugs such as cocaine or hallucinogens affect the body. Alcohol has been linked to malnutrition, liver disease and pancreatitis. Smoking can lead to weaker lungs as it attacks leukocytes. Even second hand tobacco inhalation leads to malnutrition, not to mention first use of cigarettes. As for marijuana use, when it’s smoked It could have potential life threatening side effects like focal alveolitis or granuloma formation. However endocannabiods found in maternal milk could be ingested and THC could help with bronchodilatation, making it easier for CF patients to breathe.

Introduction

The first time Cystic fibrosis (CF) has been mentioned in our history was in medieval times, a few centuries ago. European folklore from around the 16th century affirms that the babies who tasted salty at birth will not survive much longer. At that time, this was also supposed to be linked with hexes and witches. 1 Nevertheless, CF was recognized as a disease later in the 20th century due to its genetic origins and humanity’s lack of knowledge on that subject. The first time CF got a name is in 1938 when Dr. Dorothy Andersen described CF as “cystic fibrosis of the pancreas” because of what she found when she performed biopsies on children who died quickly after birth because of malnutrition.

In 1948, Dr. Paul di Sant’Agnese, made a discovery during a New York summer heat wave. Infants in the emergency room had a high salt concentration in their sweat. By 1953 sweat electrolyte defect was discovered and sweat tests became available by 1959, disregarding the idea what CF was only a mucus disorder. Discoveries evolved, as well as science did, and by 1983 the basic problem of chloride transfer was put up into discussion. The gene that is responsible for CF was discovered in 1989, and has direct correlation to cell transport channels. Knowing the faulty gene, this opened the gates for new gene therapy and treatments.

Compared to medieval times, the age limit has significantly increased. Before the genetic discoveries, patients with CF used to die very early in their life. While life expectancy was 6 months, now it is up to 30, or even 40 years. 2 With intensive care, medication, activity, patients nowadays can live a longer life. Indeed the life they will lead will be a difficult one as symptoms of this disease are very difficult to endure and require a lot of strength, especially mentally, to go on. Since CF is a disease affecting the mucus of the lungs, patients present symptoms of wheezing and coughing. Many of them described the pain as constantly choking and not being able to breathe. It is painful, and even with treatment to lengthen life expectancy and help with the symptoms individually, there is still no cure. CF patients are also having gut problems and suffer from malnutrition and anorexia. Living a life knowing you’re going to die at around 40 years because of a disease with no cure has enormous effects on the psychology of the human, starting from the childhood way into adulthood.

Not to mention CF can come with other liver or pancreatic complications which affect also the digestive system. Patients require intensive care in all domains to live their fullest lives. Because of malnutrition, they even have a special diet which they should eat. All these physical limits have their toll on the human emotional state. CF patients without social support and help from doctors and family are prone to depression and have trouble accepting their diagnosis and future. There has been a few studies involving reckless behavior of teens and adults who suffer from CF. They try and escape the reality of the seriousness of the illness by confiding in cigarettes, cannabis, alcohol and other harmful drugs to such a weak system they have. In this paper there will be a more detailed insight of the risky behavior of CF patients involved drug abuse. We will go in the psychology of the patients and see how the genetic disorder affects their mental state. Knowing their life is shortened, the fact that people feel like they need to rebel against their nature is probably a human trait. But what are the consequences of taking risky drugs when the person suffers from CF?

The conscious decision of smoking and ingesting things that are against medical advice surely would have an unexpected bad result. Are drugs like cannabis actually effective in relieving pain like it was proved to happen in other chronic cases? And if that’s not the case, are CF patients taking them because of a mental health problem or just because of rebellious teenage acts?

Molecular mechanisms and possible treatments In order to understand the terminality of this disease and why there isn’t a cure for it yet, we need to get a better understanding of the molecular mechanisms and treatments available. Knowing what can be done and how life can or can’t be improved plays a big part in the patient’s decision on how to approach the future. The CF gene resides on chromosome 7, on exon 27 and is 250 kB in length. This gene encodes a protein of 1,480 amino acids called cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is a transport protein channel for chloride transport located on the surface of the cells. CFTR can be found in the epithelium of our airways and in the sub mucosal glands. This channel is responsible for the regulated transport of chlorine and other anions in and out of the cell (by using the regulation of a cyclic adenosine monophosphate based transport, or CaMP) and also for the regulation of epithelial sodium channel (ENaC). So, when CFTR is defect, that leads to an imbalance in both chloride and sodium concentration in the cell. The dehydrated portions from the lack of sodium being absorbed in the cell and excess from the chloride intake obstruct the airways with mucus. Now the weak tissue is prone to bacterial infection and intense inflammatory response.

The treatment for CF, while it doesn’t cover treating the disease itself like most chronic inflammations, it focuses on the symptoms. Treatments nowadays are used both for airway clearing and providing aid in breathing and diet and nutrition to help the body get the nutrients it needs for survival. Therefore, treatments can go a few ways, neither of them completely efficient. First, there is nutritional repletion which consists in administering pancreatic enzymes for malnutrition prevention, as 85% of the patients suffer from pancreatic issues. By predicting the possibility of nutritional failure there are ways to increase pulmonary function as those 2 are linked. Another treatment pathway is the relief of air obstruction. In order to clean the airways from mucus and pus, a technique called “ketchup bottle” is used, where the patients are clapped and have their bodies vibrated which helps with airflow and viscosity of the mucus in the air conducts.

Another problem faced by the air pathway is inflammation and infection. For over 60 years culture specific antibiotics have been used to fight infection in CF patients. Nowadays, that method is still useful intravenous. There are trials of antibiotics being injected orally or in the form of spray, but they haven’t been quite that effective. If this treatment fails, a last resort for prolonging life is lung transplant. This procedure can’t be done often because of availability of transplants. Also, the even though the survival rate after the transplant is 80% after one year, it decreases to 50% after 4, so it still isn’t a conclusive lifelong treatment.

The Physiological effect CF has on patients

Childhood and adolescence are fragile years where a person evolves physically and mentally. In the case of children and teens with CF, another additional issue they might face is mental health problems. This could have many origins, such as the gut problems affecting the serotonin receptors or situational problems like facing life with an illness like this. Nevertheless, studies have proven that CF patients, especially young ones, suffer from different psychological defects such as depression or anxiety. One of those studies show that 59% of adolescents and young adults with CF suffer from anxiety and depression symptom. Psychological problems may even appear because of parental distress and pressure on the CF child, causing them to develop themselves a negative aspect on life. From 1966, studies have shown anxious parents and depressed children who talk about death and inability to enjoy anything around them because of their known future. Often children showed emotional numbness and hostility towards parents, teachers, and everyone helping them because their life was seen as a burden. An important fact in the impact on one’s life is hopefulness. A young person who cannot comprehend the severity of the impact on their life will show a better and more satisfactory life. Emotional wellbeing is linked to living a better life.

Another link of emotional distress which can cause reckless behavior is the striving independence of every teenager. Puberty is a time where young people strive to live to society’s expectations and live at their fullest, especially with the attention teenagers pay to social media as well as TV Shows. Anxious parents who are afraid for their child may constrict them from having a normal pubescent arch, resulting in anxiety between the CF diagnosed teenager and the caretaker. This is one of the flame that might ignite rebellious behavior in young adults and not only. Starting from the depression diagnostic, there is a strong link between people diagnosed with major depressive syndromes and alcohol and drug abuse, even in non-CF related cases8. Social pressure, losing hopefulness, not caring anymore about the future due to the possibility of an unforeseeable and unfavorable outcome, all of these mentioned lead to drug abuse in struggling CF patients.

Drug use and side effects

Drug intake is something that a lot of CF patients have tried. It is true that if you compare the statistics of people who have smoked or done drugs in both CF and non CF cases, CF patients do have a slightly difference in percentage. For example, in one study made comparing the behavior of adults who have CF to adults who do not, 35% of CF patients have tried illicit drugs compared to 37% of the comparison subjects. Although this comparison can be flawed as there is a big difference in the numbers of those who have this disease and those who don’t. For someone in a very fragile physical state, one would imagine that people won’t risk their wellbeing for something like alcohol or cigarettes. Surprisingly, studies show that a majority of the CF subjects have at least tried one of the dangerous chemicals and substances. Below there will be discussed 5 different categories of drugs and risky behavior that CF subjects have tried and how each one could potential worsen their health. 9 Smoking tobacco

One of the things that can be extremely dangerous for everybody, but especially people with such a lung condition, is smoking cigarettes. Smoking has been related to lung cancer throughout all our history. Unsurprisingly, tobacco has a negative effect upon a CF lung. Studies show that tobacco smoke affects the immune system targeting the T and B cells as well are the monocytes and neutrophils. When the bacterial function of the leukocytes is gone, the lungs are more prone to bacterial infection. Adding this possible outcome to an already weak tissue which could already have inflammation could worsen the condition of CF patients. But, mostly, tobacco effects nutrition. Even second hand smoking in children can affect their stature and the way they growth. There have been studies showing that tobacco smokers weight less than non smokers and once they stop, they gain around 5 kg. When dealing with an issue of malnutrition, the last thing needed to further cause complications is another weight loss factor. Around 45% males and 47% females who suffer from CF have tried cigarette smoking. 6% of those who have tried have continued smoking until the moment of the study. Another study does show that out of 173 subjects, around 11% are current smokers.

Alcohol consumption It is mentioned above that CF patients already suffer from pancreas related issues. A common trait that both heavy drinkers and people with CF have is that both groups are prone to pancreatitis. Alcohol helps with the degradation of the pancreatic duct. 12 There is a study in which one day after ingesting alcohol, pulmonary function seemed to have gotten lower as patients experienced nausea and vomiting. Combined with other specific CF medication such as cephalosporin or chlorampenicol, the mixture provided the subjects’ side effects such as headaches. CF patients who suffer from the aforementioned pancreatitis have a high risk of developing liver diesease. Alcohol has been proven to damage the liver, studies dating back to 20th century. The ethanol has a toxic effect on hepatic cells. Moreover, alcohol has been linked to malnutrition. All these facts added up should be a clear indication that alcohol is not recommended for CF patients. And yet, 94% of males and 97% females have tried alcohol and 83% keep drinking regularly. Usually the age to which alcohol is tried the first time is 16 years. The habit continues in adulthood too. The majority of people in the study kept their alcohol consumption to normal units for healthy people, except a few cases who enjoy casual “binge drinking”.

Cannabis and marijuana derivates

When people smoke marijuana other than ingest it, it’s because metabolites are absorbed faster in the lungs and take a shorter time reaching the bloodstream. However, it has been shown that these metabolites are linked to lung diseases such as: focal alveolitis, formation of granulomas, obstructive airways etc. They have also been linked to possibility of teratogenic nature. Similar to smoking, it increases carbon monoxide and tar levels. In animal testing there has been observed that marijuana attacks the lungs defensin system. Some patients use it because of the short lived bronchodilator effects. Marijuana has been linked to helping with chronic pain, but for a CF patient smoking it will do more harm than any sort of pain relief long term. Around 31% of the case studies have used cannabis at least once. However cannabis might have positive outcomes If used and exploited correctly. Exploring the correct calorie intake and ways to avoid malnutrition, there is a constant search for supplements that can make life easier to patients. Cannabis is used for stimulating appetite.

Endogenous cannabiods have been found in maternal milk and they help newborns with maintaining a correct nutrition balance. This could help because endocannabiods are fatty acids derivates. Recent studies have hinted that the problem in CFTR malfunction could be a lipid problem. It is proposed that CFTR gene modelates the production of the endocannabiods because of the fatty acid synthesis. It is also proposed that, in addition to appetite stimuli, THC ingestion can help with inflammation and pain management as long as it’s not inhaled. 16 Illegal drugs Marijuana, while it might not be legal in the UK, it is legal in a lot of other countries and American states in which these studies have taken place.

While prior drug presented cases included legal drugs intake, there is no doubt that illegal drugs are still being taken by CF diagnosed patients and healthy people as well. The black market is prospering and synthetic drug production is rising up. A big problem in illegal drug trade is the purity of the products which can result in even more damage. As mentioned before, 35% of the people in the study have tried illicit drugs. The most common one after cannabis (which was considered illegal during the time of the study) is cocaine, with 14% of the patients trying it. 10% have tried ecstasy or other MDMA related drug and around 6% have tried hallucinogenic drugs. The earliest median age for drug use was 15 years old which involved sniffing glue or inhaling aerosols. After trying, around 3% continued taking drugs for recreational purposes4. There haven’t been enough studies on how illegal drug use has affected the body of someone suffering from CF. It is believed that the primal reason for that is the number of people who admit to doing it is rather low in the big picture. Also, a lot of studies around these drugs have been considered ethically ambiguous.

Conclusion

Cystic fibrosis is a life shortening and threatening genetic illness with no cure which affected a lot of people and still does. Focusing on the psychological aspects of the cystic fibrosis disease there was a noticeable increase in mental health problems amongst teenagers and adults due to the nature of life expectancy and the lost of hope because of the inexistence of a cure.

Truth is, there isn’t just one reason why CF patients result in having reckless behavior. It could be the depressive disorder, or simply because of rebellious acts. Certain is that, in the end, teenagers and adults and take drugs that could and do affect their health. These potentially dangerous substances complement CF symptoms and make them even worse for the patient. For example, alcohol and smoking cigarettes provoke a nutritional imbalance and weight loss, which CF patients already struggle with. In the studies presented, a large majority of the patients have tried at least once to smoke cigarettes or marijuana, drink alcohol or take illicit drugs.

The effect of this substance use and abuse has been clearly presented, as most chemicals further damage the lungs, pancreas and liver. However, in rare cases such as cannabis, the derivates of this plant could have positive outcomes such as pain relief if ingested in other ways than through lungs.

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