Conclusion

In short, AIP does not occur merely due to genetic factor, it will only trigger in the presence of both genetic and environmental factor. The treatment depends on the symptoms of porphyria that the patients have. Although porphyria usually cannot be cured, certain lifestyle changes may help to manage it. Undeniably, in most cases, people with AIP are capable of leading a normal healthy life. Despite that, they should be persistent in controlling their lifestyle, alcoholism and smoking must be avoided. Based on the case studies, it shows that patients with AIP are able to recover and lead a normal life after receiving the treatments. As an individual become older, the chance of experiencing acute attack reduces. However, the acute attack will still remain with the person and will not disappear in one's whole life.

Application of Theory for Case Studies #2

In this case, the transtheoretical model can fit in to explain the case. There are five stages which are the precontemplation, contemplation, preparation, action and maintenance.

Based on the case, the patient undergoes the precontemplation stage where he was not aware of the disease and has no intention to receive treatment and change lifestyle.

Next, in the contemplation stage, the patient experience acute attack and has the intention to receive treatment.

In the preparation stage, he does research on Acute Intermittent Porphyria and later found out that he has a family history on this disorder. Thus, he plans to receive treatment and change his lifestyle.

Then, in the action stage, the patient with AIP receives two treatments which are glucose intravenous and heme treatment. 

Lastly, in the maintenance stage, the patient is very aware and careful about his lifestyle after the treatment in order to prevent the recurrent of acute attack. 

Figure 37. Transtheoretical model

Case Studies #2

Figure 36. Case study

One of the patients in the case study was 39-years-old male who diagnosed with Acute Intermittent Porphyria (AIP) in 1998. This patient reported that he suffered from abdominal pain and constipation. More importantly, he also took many examinations from doctors to diagnose the disease or disorders was, however the results were normal. At the end of the test result, his spot urine sample also used to diagnose, ALA, PBG and Proto-PBG was positive. This is because his family members were diagnosed and suffered from AIP. In other words, it was medical history of AIP had found among his family. Besides that, patient received two types of treatments after he had diagnosed with AIP. The first type of treatment was he administered the glucose orally and had been monitored within three days. Unfortunately, he suffered the acute attack of AIP after he recovered. Due to the glucose did not function during his fasting for Ramadhan. Therefore, heme treatment had been replaced from glucose administration as treatment of AIP. He had completely recovered from AIP within two days after received intravenous heme (injection into the bloodstream). After the recovered from AIP, he became more careful in his lifestyle, such as prescribed the drug carefully.

Application of Theory for Case Studies #1

Based on case study 1, behavioral theory can be applied to cope with patients who do not want to make changes in their lifestyle. Behavioral theory is suggested by Skinner, which he more specifically named it as operant conditioning. Behavioral theory focuses on the leading factor to an action and the result or consequences of performing the action (McLeod, 2015). Skinner believed that the consequences will largely affect one to repeat or not repeat the particular action or behavior. 

In this case, positive reinforcement can be applied. Positive reinforcement is referred to the strengthening of good behavior by rewarding a person (McLeod, 2015). When the patient refrains himself from smoking or drinking alcohol, the physician can award or praise him with the thing he like. The happiness of getting reward may be a motivation for him to stop smoking or drinking alcohol. 

Next, positive punishment can also be used. Punishment is used to reduce undesirable behavior. However, it is not encouraged because there might be negative side effects. For example, punishment can lead to aggression or delinquent behavior. 

Case Studies #1

Figure 35. Case study

A 39-year-old male, was diagnosed of suffering from Acute Intermittent Porphyria. His spot urine sample was used for diagnosis, which his urine acetone and urobilinogen was positive and the ALA level was 25mg. Furthermore, his urine color turned into black after exposed to the sun. According to Sezgin (2016), he had reported experienced of severe abdominal pain, cold sweats. progressive muscle weakness, as well as constipation. Due to his family history, which included two brothers and a sister were also struggle of AIP, thus genetic inheritance was the primary factors of causing his AIP. Besides, he did not follow the advice of the physician, he also continued cigarette smoking and alcohol assumption, which possibly in triggering the recurrent attack of AIP. Moreover, he also used of the outpatient medication. For treatment, he had received intravenous dextrose infusion when he was confirmed of having AIP. Whereas he received heme cure at the third day of AIP attack. It was administered 3mg for a day, continued for three days. He was recovered at the 6th day. However, as he continued the behavior which will precipitate the attack of AIP, acute attack had occurred few years later.

Experience of Having Acute Intermittent Porphyria

This video is about a doctor who suffers from Acute Intermittent Porphyria. After recovering from the disorder, she described her experience of having AIP. In the video, she showed her photos to present the huge difference in her appearance before and after treatment. 

Figure 34. The photo of the doctor before and after treatment

In short, people with AIP should receive treatment immediately. As mentioned before, heme treatment is the most effective treatment for AIP and many people who suffer from AIP can lead a normal life after the heme treatment. Although it may not completely cured the disorder, but certainly the symptoms will be reduced. 

The Biopsychosocial Explanation of Acute Intermittent Porphyria

As the video mention, biopsychosocial model is a model to know the cause of mental illness and also the solution to it. The first part which is biologically, it includes heredity, genes, illness, and also mood, and the solution for this is medication, the second part which is psychological, it includes experience and thoughts and feelings, which mostly are negative thoughts and feelings, the solution for it is therapy. The next part is social, which includes family stress, and job stress and transition, the solution for it is to know the boundaries and self-care. 

Figure 30. Biopsychosocial model of health

Biopsychosocial model is a model that says that a disease is result from a combination from the aspect of biology, psychological and social factors, this model is use to improve the previous model which is the biomedical model that says that disease is result from exposure of pathogen (Engel, 1980). Just like Acute Intermittent Porphyria (AIP), this disease is caused by the combination of biology, psychological, and social factors (American Porphyria Foundation, 2010). Just like Acute Intermittent Porphyria (AIP), this disease is caused by the combination of biology, psychological, and social factors (American Porphyria Foundation, 2010). 

Biology factor in AIP says that this disease is passed down from generations to generations through the genes (Sezgin, 2016), in other words, it means that AIP is an inherited disease. Most compelling evidence, previous research indicated that 90% of the individual with AIP who usually W198X mutation in the genes (Andersson, Floderus, Wikberg, & Lithner, 2000; Bylesjö, Wikberg, & Andersson, 2009).

The next factor, which is psychological factor, such as being depress all the time or having too much stress emotionally and physcially or exhaustion, burn-out could also increase the risk of triggering the AIP in the body, provided if the person family generation have the history of AIP (Balwani & Desnick; Ventura, Cappellini, Biolcati, Guida, & Rocchi, 2014). Prior findings highlighted that hypertension was relatively highly associated with Acute Intermittent Porphyria (Bylesjö, Wikberg, & Andersson, 2009).

Figure 33. Alcoholism, smoking and drug abuse

The last factor that could increase the risk of AIP is social factors. Social factors includes smoking, drinking alcohol, and consuming medications or drugs such as barbiturates or  methyldopa could also increase the risk of triggering AIP  (Lopes, Valle, Taguti, Taguti, Betonico, & Medeiros, 2008). Other than that, past study revealed that working environment was the most common factor which lead to severe attack of AIP, especially among men (Bylesjö, Wikberg, & Andersson, 2009).  According to Bylesjö, Wikberg and Andersson (2009), 11 patients had reported that they had shifted work, solvents, stress, physical strain and exhaust fumes.