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Paroxysmal Nocturnal Hemoglobinuria Case Study

Paroxysmal Nocturnal Hemoglobinuria Case Study

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Published by romeo rivera

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Published by: romeo rivera on Jul 02, 2009
Copyright:Attribution Non-commercial


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INTRODUCTIONParoxysmal nocturnal hemoglobinuria
(PNH) sometimes referred to as
Marchiafava Micheli syndrome
is a descriptive term for the clinicalmanifestation of red cell breakdown with release of hemoglobin into the urine thatis manifested most prominently by dark-colored urine in the morning. The term"nocturnal" refers to the belief that hemolysis is triggered by acidosis during sleepand activates complement to hemolyze an unprotected and abnormal red cellmembrane. However, this observation later was disproved. Hemolysis is shownto occur throughout the day and is not actually paroxysmal, but the urineconcentrated overnight produces the dramatic change in color. PNH is nowknown to be a consequence of nonmalignant clonal expansion of one or severalhematopoietic stem cells that are deficient in GPI-anchor protein (GPI-AP)acquired through a somatic mutation of PIG-A.Paroxysmal nocturnal hemoglobinuria is a rare disease which affects 1 outof 5 million people. It has been suggested that, PNH may be more frequent inSoutheast Asia and in the Far East. Men and women are affected equally, and nofamilial tendencies exist.PNH may occur at any age from children (10%) as young as 2 years toadults as old as 83 years, but it frequently is found among young adults with amedian age at the time of diagnosis was 42 years (range, 16-75 year old). Inchildhood through adolescence, patients presented with more of the primaryfeatures of aplastic anemia than the normal adult population. Othecomplications, such as infections and thrombosis, occurred with equal frequencyin all age groups.The disease process is insidious and has a chronic course, with a mediansurvival of about 10.3 years. Twenty-two of the 80 patients (28%) survived for 25years. Of the 35 patients who survived for 10 years or more, 12 had spontaneousclinical recovery at which time no PNH-affected cells were found among the red
cells or neutrophils during their prolonged remission, but a few PNH-affectedlymphocytes were detectable in 3 of 4 patients tested.Laboratory diagnosis can include specialized test, such as sucrosehemolysis test, ham acid hemolysis test and fluorescent-activated cell analysis.Treatment is mainly supportive, consisting of transfusion therapy, anticoagulationtherapy, antibiotic therapy, corticosteroids therapy and supplement therapy whichincludes folic acid and iron. HSCT may be curative. Stress and strenuousactivities are contraindicated to the client. A change and adjustment in lifestyle isencouraged for the client to be able to function in his fullest potential, minimizethe effects of the disease and somehow live a normal life.On March 16, 2007, the U.S. Food and Drug Administration (FDA)approved
(eculizumab) for the treatment of PNH. This medicine works byblocking part of the immune system. It should help decrease the number of bloodtransfusions needed and the number of episodes of blood in the urine.During the year 2008 to 2009, only one case of PNH is recorded at theTarlac Provincial Hospital.
(TPH medical record).
Reason for choosing such case for presentation
 Paroxysmal Nocturnal Hemoglubinuria is a rare disease which reallycaptures the group’s interest among the other cases of the confined patients. Itgave a thrill for all of us since we do not have any idea about it and find it verychallenging.The researchers are eager to study about the disease due to lack of information, facts and studies. It is a new exploration. Our curiosity towards thecondition of our patient gave us a lot of questions just like how does the diseaseaffects an individual in different aspects; physically, emotionally, and socially andsomehow to help this client to promote and restore client wellness by providing
their needs and knowing the nursing responsibilities when caring the client. It isan opportunity for us to study this disease to equip the group with knowledge andskills to be able to manage future clients with the same disease in providing aquality nursing care.
Importance of the case study
This case study is made for different purposes whereas it connects thepast, present and something to do in the future time. It is intended to educate,inform and change untoward behaviors regarding the disease—ParoxysmalNocturnal Hemoglubinuria.This case study will help the client to recover faster and maintain holisticsense of wellness through applied effective management of the problemexperience by the client and it can also lessen the functional burden of the clientby understanding the treatment process and able to cope and adapt in thepresent condition and also the client will be able to know the importance of takingcare of own self.On the side of the group this case study can help each member to gainnew information about the disease and its etiology, pathophysiology, clinicalmanifestations as well as the standard medical and nursing management so thatwe may apply this newly-acquire knowledge to our client as well as similar situations in the future. The group will learn new clinical skills as well as sharpenour current clinical skills required in the management of the client withparoxysmal nocturnal hemoglubinuria. Through this study the group memberswill develop a sense of unselfish love and empathy in rendering nursing care tothe client so that the group may be able to serve future clients with a higher levelof holistic understanding as well as individual care.

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