Devic’s Disease in Childhood

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Table of Contents

Introduction
Symptoms and Signs
Treatment Modalities
Appropriate Nursing Action
Patient Education
Complications and Issues in the Context of Pediatric Patients
Conclusion
References

Introduction

Devic’s disease, which is also known as neuromyelitis optica or NMO, is a dangerous medical disorder which affects the optic nerves and the spinal cord of patients, and is similar in function and symptoms to multiple sclerosis (Gocke, Ceylan, Multu, Altinsoy, & Koylu, 2013). It is a difficult disease to diagnose and the person afflicted with it suffers from inflammation of the optic nerves and the spinal cord, resulting in a potential for damage to visual and motor functions, as well as further complications.

While this is a rare disease, and mostly is found in adults over forty, in the recent years the numbers of afflicted children has been steadily increasing, with a strong female predominance noted by the pediatricians.

Also, until fairly recently the disease has often been misdiagnosed as multiple sclerosis. This makes Devic’s disease difficult to diagnose. The problem is augmented due to its symptoms also being similar to a number of other diseases, including acute demyelinating encephalomyelitis, systemic lupus erythematosis, mixed connective tissue disorder, inflammations caused by viruses, and pareneoplastic optic neuropathy (Nordqvist, 2015).

The purpose of this paper is to distinguish the unique features of Devic’s disease, particularly in children, define the symptoms and signs that differ it from other similar diseases, and define the measures that are needed to prevent and treat it.

Symptoms and Signs

NMO is a form of autoimmune disorder, and based on the nature of immune attacks, patients that suffered from the effects of Devic’s disease are affected by autoimmune attacks on the optic nerves and the spinal cord. In some cases, the autoimmune attacks are evident in both the optic nerves and the spinal cord area of the body. However, there are also cases wherein the autoimmune attacks focused only on the optic nerves or the spinal cord alone.

Due to the said immune attacks, those that are afflicted with NMO are often diagnosed with blurred vision. In advanced cases of the said disease, the patients suffered from loss of vision.

Aside from complications that resulted from the negative effects on the optic nerves, patients also reported feelings of weakness and numbness. In some cases there were also reports with regards to gall bladder problems or issues with regards to bowel movement.

Treatment Modalities

Unfortunately, currently there is no effective curative treatment for NMO. As a result, the treatment modalities are focused on quick detection and primary and secondary prevention of the disease.

The principle goals of Devic’s disease treatment are remission and irradication or mitigation of the symptoms associated with the relapse, long-term relapse prevention and, consequently, patient stabilization, and finally symptomatic therapy of the remaining symptoms (Gokce, Ceylan Multu, Altinsoy, & Koylu, 2013).

Due to the rarity of the disease, there is little chance of systemic evaluation of treatment efficiency through trials and analysis, which does mean that specifics of treatment might have to be changed if there the young patients show a negative response to specific type of steroids or other medicine.

Appropriate Nursing Action

Since there are diseases that exhibit the same signs and symptoms to NMO, nurses must learn the practical steps associated with diagnostic methodologies in order to rule out the above-mentioned conditions (Noseworthy, 2004).

In order to diagnose the disease, health practitioners usually use MRI technology and a NMO-IgG blood test scheme, which enable health care professionals to determine the onset of the disorder and see inflammation in the spinal cord (Irani, 2009). The blood test is particlurly useful, since even though research has demonstrated it to be erroneous in 30% of the studied cases, it can reveal that the patient does not suffer from MS and is a key tool in differentiating between NMO and MS.

Aside from the use of MRI and the specialized blood test, nurses assigned to this type of medical case must also understand the requirements in using lumbar puncture test or spinal tap in order to help deliver appropriate health care.

It is important to point out that there is not a single treatment protocol that can totally eliminate the said disorder. However, there were reported cases of improved health and well being after using intravenous corticosteroid therapy. Patients that did not benefit from the said therapy reported positive results after using a technique called plasmapheresis or plasma treatment. In a case wherein an NMO patient was rendered paraplegic the use of plasma exchange treatment method enabled the patient to move her lower extremities and after some time learned how to walk.

Treatment depends on how much the condition has affected the child, and how pronounced the symptoms are. The immune attacks need to be treated with intravenous or oral steroid and immune suppressant therapy, with the latter prescribed to prevent relapse.

There is a possibility of the children not responding to the mentioned above medicine. In this case, nurses and physicians would need to prescribe plasma exchange. Blood would need to be extracted from the body, and then returned with the blood cells replaced with a replacement solution (Devic’s Disease Treatment, n.d.)

Patient Education

With a disorder like Devic’s disease diagnosed, it is vital that nurses fully embrace their roles as educators when tackling both the young patients and their families or guardians. The nurses need to be prepared to employ all counseling and educational tools available to them, including verbal counseling, written materials, demonstrations, playing, on-the-phone counseling and practical training, in order to both account for child sensibilities and the parents’ need for factual information that would gently explain the situation to them and counsel them towards correct treatment of their child.

Two very different approaches need to be considered for the children and parents. For the child, their developmental stage needs to be considered, since with young children it has been proven that framing day-to-day management as games can serve to relieve a lot of mental stress off the child.

Parents require detailed counseling on the nature of the disease, as well as the treatment and medical procedures that the child will need to undergo in order to mitigate the symptoms and achieve recession. Both the parents and the pediatric patients will require emotional support to manage the information (Kelo, Martikainen, & Eriksson, 2013).

Complications and Issues in the Context of Pediatric Patients

If not timely treated, neuromyelitis optica can result in a wide range of severe and debilitating complications. These complications include damage to the optic due to untreated inflammation, and, as a result, permanent loss of vision; damage to the spinal cord, causing limb paralysis; muscle weakness and, as a result, breathing difficulties; brittle bones, as a result of prolonged steroid medication or lack of physical activities, and finally depression, as a result of the lifestyle associate with the constant need for treatment and numerous debilitations (Complications of neuromyelitis optica,2010)

Conclusion

Neuromyelitis optica is a very dangerous disorder, which is particularly threatening to young children. Since it does not have curative treatment, it relies on prevention and symptom treatment. It is vital that the disease is correctly diagnosed prior before complications developing. The nursing staff carries a lot of responsibility for the accurate diagnosis and timely treatment, since delays critically increase the dangers of permanent debilitation, and only efficiency and professionalism of the healthcare providers can increase the chances of some measure of recovery.

However, the patient will have to deal with the symptoms for the rest of their lives, and preventive, remission-prolonging treatment will need to become a part of their lifestyle.

References

Cleveland Clinic. (2016). Devic’s disease. Web.

Complications of neuromyelitis optica. (2010). Web.

Devic’s Disease Treatment (n.d.). Web.

Gokce, G., Ceylan O.M., Multu, F.M., Altinsoy H.I., & Koylu, T. (2013). Relapsing Devic’s disease in a child. Journal of Pediatric Neurosciences, 8(1): 146-149.

Irani, D. (2009). Cerebrospinal fluid in clinical practice. PA: Saunders-Elsevier.

Kelo, M., Martikainen, M., & Eriksson, E. (2013). Patient Education of Children and Their Families: Nurses’ Experiences. Continuing Nursing Education, 39(2):71-9.

Nordqvist, C. (2015). What is Devic’s disease? Web.

Noseworthy, J. (2004). Fifty neurologic cases from Mayo. New York: Oxford University Press.

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