Modeling Motor Neurosciences Disease Essay

Question:

Discuss about the Modeling Motor Neurosciences Disease.

Answer:

Introduction:

According to the World Health Organization (WHO), palliative care is defined as an approach designed to help in the improvement of the quality of life of patients who are facing life-threatening diseases as well as their families. Palliative care helps in the relief and prevention of spiritual, psychosocial, and physical suffering through the treatment, assessment and timely identification of pain. This implies that palliative care can be relied upon to help in resolving a wide range of health issues such as affirmation of life; offering relief to painful and distressing symptoms; postponement of death; integration of spiritual and psychological care; enhancement of the quality of patient’s life; prolong life as well as helping the patients to enjoy an active life up to death; and providing the necessary assistance to the family members to endure the patient’s illness or unfortunate eventual death (Wiener, Weaver, Bell & Sansom-Daly 2015). Palliative care is not only restricted to the adult populations, but can also be extended to the children patients who can benefit from it in different healthcare facilities: children’s homes, hospitals, community health centers, and other tertiary care facilities in which the children’s spiritual, physical, and psychosocial needs are assessed and diagnosed before an appropriate intervention is provided.

Origin of Palliative Care

Palliative care is not a new phenomenon, but an old practice that has an expansive history that can be traced to as early as the 4th century during the origin of the Hospice Movement. Although palliative care is currently a separate form of care quite distinct from the traditional hospice care, it has a direct linked with the Dame Cicely Saunders-found Hospice Movement that was initially exclusively dedicated as a resting places for the travelers (Pratt & Wood 2015). However, as fate would have it, palliative care would later transform itself and by 1950s, it was tailored towards providing care for the dying patients.

This change was brought about by Dr. Cicely who, upon observing the dead patients, came with an idea that a modern hospice should be established to care for the dying patients. However, to accomplish this goal, Dr. Cicely emphasized that satisfactory palliative care should be delivered by a team of multidisciplinary professionals who have knowledge on pain relieving and could be entrusted with the provision of palatial care to the satisfaction of the patents, their carers and family members (Quill & Abernethy 2013). Despite all these efforts, hospice care would later transform to palatial care in 1974 when the terminology was coined by the Royal Victoria Hospital’s Dr. Balfour Mount. Dr. Mount was concerned about the level of end-of-life care provided to the patients. Since then, palatial care has become a recognized practice that is aimed at providing healthcare services to relieve the suffering of the patients as well as their respective families.

Motor Neuron Disease

Motor Neuron Disease (MND) refers to a neurological disease which progressively affects the brain and spinal cords nerves or neurons. When one is affected by MND, one experiences wasting and weakness since the nerve impulses tend to fail to reach the body muscles. It is for this reason that the condition negatively impacts on how an individual breathes, drinks, eats, talks, and walks. This is what was happening to Isobel because she was observed to be experiencing weaknesses in her arms and legs thus leaving no doubt that her deteriorating health are associated with the MND condition (Zimmermann, et al 2014). According to scientific research, MND condition affects people of all ages hence categorized into Kennedy’s Disease, Progressive Muscular Atrophy (PMA), Primary Lateral Sclerosis (PLS), Progressive Bulbar Palsy (PBP), and Amyotrophic Lateral Sclerosis (ALS).

Research has established that MND has many causes which may be hereditary or environmentally-triggered. Basically, it arises when the motor neuron cells of the spinal cord and brain dysfunctions. Familial or inherited conditions, which only account for 5% of the infections, are linked to genes that are transmitted from a parent to the offspring. With this in mind, it should be noted that scientists have found out that MND is caused by the abnormalities in the functioning of the glial cells, cell transport disruption, aggregates and RNA processing, and mitochondria as well as gene mutations as in the case of familial conditions (Gidwani, Joyce, Kinosian, Faricy-Anderson, Levy, Miller, Ersek, Wagner & Mor 2016). The changes in these body organs and cells contribute to the occurrence of the conditions because it alters the normal functioning in the body. MND has many pathophysiological changes in the body. It alters the functioning of the body Central Nervous System. For instance, it causes abnormalities in the functioning of glal cells in the body. Since glial cells are used in the supplying of motor neurons with nutrients, any abnormalities in the cells deprives the motor neurons or nutrients that they require to effectively operate in the body hence resulting into the MND condition.

Signs and Symptoms

MND, as a condition, has a wide range of signs and symptoms that can be relied upon during its diagnosis. Some of the most common signs and symptoms associated with the MND conditions are muscle spasms; pains; eating difficulties, drinking challenges; bowel problems; discomfort; stiff joints; speech and communication difficulties; coughing; chocking; incontinence; swallowing difficulties; breathing difficulties; and cognitive difficulties in concentration, learning, and memory (Hardy & Rogaeva 2014). These changes are brought into the body because of the alterations in the normal functioning of the nervous system. Neurons play a very significant role in coordinating the activities of the Central Nervous System (CNS). Therefore, any abnormality in their functions can be disastrous to the body. It should therefore be noted that MND has a great influence on the Activities of Daily Living (ADL) of a patient. It interferes with daily ADL such as drinking, eating, learning, and communication. These are important activities that are done on day-to-day basis.

It is because of her MND condition that Isobel experienced swallowing, eating, drinking, walking difficulties alongside other challenges such as body pains, weaknesses, and incontinence. The disruptions in the normal functioning of the motor neurons can adversely affect Isobel’s CNS system including her brain and spinal cords which do control nearly all the cognitive and motor activities of the body (Pratt & Wood 2015). For instance, Isobel had to complain of pains because of the failure of the pain receptors to effectively discharge their duties by transmitting the messages from pain receptors to the brain. The same alterations should be linked to the eating, swallowing and drinking difficulties experienced by the patient. If the entire CCNS were fully functional, no such anomalies would have arisen. Therefore, it should be incumbent upon the physiotherapist to do something to relieve Isobel of the suffering.

Diagnosis

MND is a condition that can be managed if proper measures are taken. The first thing to do in the pursuit of medical intervention is a visit to the healthcare facility. It is recommended that an individual should pay a visit to the GP immediately one experiences the initial signs and symptoms of MND such as painless and progressive weakness of the grip. An appointment with a GP is crucial because it can provide a patient with an opportunity to be examined by a GP. This examination is important because it can be relied upon by the GP to make important decisions regarding the patient such as referral to an appropriate neurologist who is specialized in the brain or nervous system (Murray, Kendall, Boyd & Sheikh 2012). It is the neurologist who is then vested with the responsibility of carrying out additional tests and giving further opinion on what should be done to the patient.

It is therefore recommended for the MND patients like Isobel to cooperate with the GP and the neurologist since the diagnosis of the condition might be quite hard especially during the initial stages (Arnold, et al 2013). Researchers have attributed such difficulties to the fact that such symptoms might not have been caused by MND, but other common health conditions like peripheral neurolopathy in which the nervous system gets damaged by Type 2 Diabetes or a trapped nerve in which nerves get compressed as a result of the wearing and tearing of the bones.

Isobel should therefore visit a neurologist to diagnose her by carrying out a physical examination on her and also keenly observe her symptoms. At the same time, the neurologist can carry out a number of tests on the patients. These include blood test, lumbar puncture, musle biopsy, Electromyography (EMG), and Magnetic Resonance Imaging (MRI) scan. Whereas blood test can be relied upon to monitor the normal functioning of the body organs such as thyroid, MRI scan can be used for providing a finer picture of the spinal cord and brain of the patient (Smith et al 2012). Meanwhile, EMG is used in the measurement of the motor neurons electrical activities. Last, but not least, muscle biopsy is used in the testing if the challenge is actually located in the nerves or muscles. Before carrying out the diagnosis, the healthcare provider should have adequate information about the patient. for effective service delivery, the healthcare provider should be acquainted with Isobel’s ethnicity, race, nationality, medical history, age, gender, and religion.

Treatment

Even if it has been found out that MND has no known cure, it is a treatable condition. After accepting that the patient is suffering from the condition, it is recommended that appropriate treatment options should be applied so as to enable the patient to slow the progression and relieve the symptoms of the condition (Hardy & Rogaeva 2014). So, in Isobel’s case, after getting diagnosed with the condition, she should be introduced into a treatment plan. First, the patient deserves to be introduced to a multidisciplinary team of healthcare professionals to oversee her care. To be included in this team is the specialist nurse, palliative care professionals comprising of the neurologist.

From this point, the patient should be given an opportunity to engage in advance decision making because; failure to do might compromise her treatment since her condition might worsen to the extent that she might not be capable of contributing towards her treatment. Advance decision should not be overlooked since it can help in equipping the multidisciplinary care team with timely information on the patient’s choices regarding the resuscitation procedure; medication options; whether she should be treated at the hospice or at home; and if the patient is interested in donating any of her organs in case she dies (Novarino, Fenstermaker, Zaki, Hofree, Silhavy, Heiberg, Abdellateef, Rosti, Scott, Mansour & Masri 2014). These are important decisions that should be made by the patient because they involve her health. However, for efficiency purposes, the patient should be guided by her care team when making such decisions.

One of the strategies used in the treatment of MND condition is medication. Among the most recommended drugs to use in treating a MND patient is riluzole. The drug is suitable for the treatment of MND condition because it can help in the reduction of the sensitivity of the motor neuron cells to the glutamate transmitter by slowing down its progressive damaging effects. When used, riluzole can be administered for around 3 months (Cortes, et al 2014). However, its side effects are rapid heartbeat, tiredness, and nausea. Some of the other medications used in the treatment of this condition are MND condition can be treated using a variety of options: gabapentin , quinine, Amitriptyline, baclofen, hyoscine hydrobromide, and non-steroidal anti-inflammatory drugs (NSAIDs).


On the other hand, there are other treatment options that can be used in the treatment of this condition. These include physiotherapy (for muscle cramps); percutaneous endoscopic gastrostomy (PEG) tube (for swallowing difficulties); non-invasive mechanical ventilation (NIV) (for breathing difficulties); or other complimentary therapies such as acupuncture. Complimentary therapy should also be considered in the treatment of the patient because it can play a significant role in stress reduction and making the patient much comfortable regardless of the painful experiences undergone. Therefore, when handling the patient, the healthcare team should use the most appropriate treatment strategy suitable for the patient M?ller, et al 2014).

As an MND patient, Isobel requires to get quality palatial care. To do this, the healthcare providers should adopt a holistic and multidisciplinary approach. An effective collaboration between different specialists is a recommended strategy in palatial care. At the same time, the palatial care providers should closely work with the patient’s carers to ensure that they are also involved in the care provision (Arbab, Baars & Geijsen 2014). This is necessary because palatial care is a psychologically, physically, and emotionally demanding exercise. Hence, adequate support is necessary right from the beginning up to the very end of care provision. However, in order to deliver quality care, the healthcare providers should deliver services that fully satisfy the psychosocial, spiritual, cultural, and physical needs of the patient as well as her family. This is what palatial care is all about. Special attention should be taken to address the needs of Isobel who is an aboriginal. And as an indigenous patient, Isobel requires to be given a holistic care that is responsive to, respects and recognizes her aboriginal culture. this is the only way through which she can be satisfied with the level of services provided to her.

References

Arbab, M., Baars, S. & Geijsen, N., 2014. Modeling motor neuron disease: the matter of time. Trends in neurosciences, 37(11), pp.642-652.

Arnold, E.S., et al., 2013, ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43. Proceedings of the National Academy of Sciences, 110(8), pp.E736-E745.

Cortes, C.J., et al., 2014. Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy. Neuron, 82(2), pp.295-307.

Gidwani, R., Joyce, N., Kinosian, B., Faricy-Anderson, K., Levy, C., Miller, S.C., Ersek, M., Wagner, T. & Mor, V., 2016. Gap between Recommendations and Practice of Palliative Care and Hospice in Cancer Patients. Journal of palliative medicine.

Hardy, J. & Rogaeva, E., 2014. Motor neuron disease and frontotemporal dementia: sometimes related, sometimes not. Experimental neurology, 262, pp.75-83.

M?ller, K., et al., 2014. Two novel mutations in conserved codons indicate that CHCHD10 is a gene associated with motor neuron disease. Brain, 137(12), pp.e309-e309.

Murray, S.A., Kendall, M., Boyd, K. & Sheikh, A., 2012. Illness trajectories and palliative care. Int Perspect Public Health Palliat Care, 30, pp.2017-19.

Novarino, G., Fenstermaker, A.G., Zaki, M.S., Hofree, M., Silhavy, J.L., Heiberg, A.D., Abdellateef, M., Rosti, B., Scott, E., Mansour, L. & Masri, A., 2014. Exome sequencing links corticospinal motor neuron disease to common neurodegenerative disorders. Science, 343(6170), pp.506-511.

Pratt, M. & Wood, M. eds., 2015. Art therapy in palliative care: The creative response. Routledge: New York.

Quill, T.E. & Abernethy, A.P., 2013. Generalist plus specialist palliative care—creating a more sustainable model. New England Journal of Medicine, 368(13), pp.1173-1175.

Smith, T.J., et al., 2012. American Society of Clinical Oncology provisional clinical opinion: the integration of palliative care into standard oncology care. Journal of clinical oncology, 30(8), pp.880-887.

Wiener, L., Weaver, M.S., Bell, C.J. & Sansom-Daly, U.M., 2015. Threading the cloak: palliative care education for care providers of adolescents and young adults with cancer. Clinical oncology in adolescents and young adults, 5, p.1.

Zimmermann, C., et al., 2014. Early palliative care for patients with advanced cancer: a cluster-randomised controlled trial. The Lancet, 383(9930), pp.1721-1730.

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