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Nursing Case study paroxysmal nocturnal hemoglobinuria

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Nursing Case study paroxysmal nocturnal hemoglobinuria

  1. 1. Paroxysmal Nocturrnal Hemoglobinuria PNH Nursingcasestudy.blogspot.comINTRODUCTION 1
  2. 2. Paroxysmal nocturnal hemoglobinuria (PNH) sometimes referred to asMarchiafava 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, andno familial 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. Othercomplications, 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 redcells or neutrophils during their prolonged remission, but a few PNH-affectedlymphocytes were detectable in 3 of 4 patients tested. 2
  3. 3. 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 Soliris (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 ofinformation, 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 providingtheir 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 and 3
  4. 4. skills 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 similarsituations 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. On the side of the College of Nursing this study can be a documentedguide for the students it can be a source of facts and knowledge not only for the 4
  5. 5. students within the college but open to all students who are interested onstudying about the disease. On the side of nursing profession, this study will serve as a symbol ofimportance of the nursing profession and the field of education on dealing withclient with paroxysmal nocturnal hemoglubinuria.Objectives (nurse centered)General Objectives The case study aimed to represent a comprehensive study of the chosenpatient’s condition called paroxysmal nocturnal hemoglubinuria and to knowsystematically the disease and its medical and nursing management andresponsibilities while taking care of the client.Specific objectives This study aims to: 1. Assess properly to determine the contributing factors regarding to the clients disease and identify any present abnormalities: a. Personal Data b. Family history of health and illness c. History of past illness d. History of present illness e. 13 areas of assessment 2. Gather the needed data that can help to understand how and why the disease occurs a. Diagnostic and Laboratory Procedures b. Anatomy and Physiology c. Pathophysiology book base and client centered 5
  6. 6. 3. Develop an individualized plan considering client characteristics or the situation and setting a specific, measurable, attainable, realistic and time bounded plan that reflect the onset, date of problem identified a. Planning (nursing care plan) 4. Provide an appropriate interventions for every problems encountered and monitor the client’s response to treatment and therapies through means of physical assessment and communication with the client a. Medical management b. Surgical management c. Nursing management 5. Judge the effectiveness of chosen interventions, nursing care, and the quality of care provided a. Client’s daily program in the hospital 6. Describe the general condition of the client upon discharge and know the take home medications, exercise, treatment for the client, provide health teachings and inform client for OPD follow-ups a. Discharge Planning 7. Broaden the knowledge of each member through further research about the latest news articles and journals regarding to the client disease a. Related literatureII. Nursing ProcessA. Assessment 6
  7. 7. 1. Personal Data a. Demographic Data Name: Mr. X Address: Victoria Tarlac Age: 33 year old Nationality: Filipino Civil Status: Married Occupation: Tricycle driver Religion: Born Again Christian Health Care Financing: Parents Date Admitted: February 10, 2009 Admitting Diagnosis: Paroxysmal Nocturnal Hemoglubinuria Final Diagnosis: Paroxysmal Nocturnal Hemoglubinuria b. Environmental Status The client is currently residing at Victoria, Tarlac for about 10 yearsnow. He lives with his family in a house made up of wood and concrete withcemented floor, located at a rice farm. Their forms of transportation arethrough tricycles, jeepneys, or just merely by walking. Garbage is disposedproperly through segregation which is then collected by the garbage collectorin their place. Their water source comes from a water pump. Their area is notcongested according to the patient. He is aware about his neighbors, but notmuch aware of the health source in their community. c. Lifestyle The client wakes up each morning around 8 - 10 o’clock and starts theday with a cup of coffee. After breakfast and rest, the client cleans the houseand their backyard. After cleaning the house, Mr. X always finds time to listento the radio and watch the television as one of his past time and is also hisway to rest and relaxed. The client’s food preferences were mostly pork, 7
  8. 8. poultry products and seldom eat vegetables. According to him, he only eats vegetables once a month. He said that even if their viand is vegetable, he insist her mother to cook other food, specifically meat or he sets aside the vegetables and only eats the meat. At noon, the client tends to sleep for about 4 hours per day. The client verbalized that he early goes to sleep at around 8 o’clock in the evening. He doesn’t use mosquito nets when sleeping because he said that it bothers him when he always urinates at night. He added that he doesn’t use any slippers inside their house but wears them outside. They used to put their left over foods in a basket. Meal time was the time where the family bonds and the time they get to know what happens within the whole day. The client also verbalized that he doesn’t have any vices. d. Social The client stated that he knows to speak and is able to understand Ilocano, Tagalog, and English. He verbalized that he use to attend to the Roman Catholic and Aglipayan Church but he claimed that he is a Born Again Christian. According to him, he is not a member of any organizations. e. Psychologic According to the client, financial problems and his disease are his primary stressors. He said that praying is his way to cope up with his problems; he believes that when he prays everything will be alright. The client speaks in a casual way during the interview and he said that he doesn’t say/speak bad words.2. Family History of Health and Illness 8
  9. 9. FATHER SIDE MOTHER SIDE ? ? ? ?Old Old Old Old 5 6 6 3 7 3 6 3 2 A&W A&W A&W A&W A&W suicide A&W A&W A&W 3 3 3 3 LEGEND A&W A&W PNH A&W Male Female Deceased Male Deceased Female Married Children Patient Alive & Well Paroxysmal Nocturnal A&W Hemoglubinuria PNH 3. History of Past Illness 9
  10. 10. According to the client, he first experienced to have the signs andsymptoms of PNH when he was at the age of 29. He said that he used to urinatefrequently at night with a tea colored urine; without pain when urinating, andurinates a large amount of urine but he doesn’t know the exact volume of urinebeing excreted. He assumed and told himself that it was just normal and he didnot tell it to his parents. Few days later, the other family members noticed that heis already pale in appearance, but he told them that it was just normal. The clientjust ignored his condition. Days passed by, he said that he always felt headache,abdominal pain, difficulty of breathing, fever and weakness. To relieve hisheadache and fever, he said that he took Medicol or Alaxan and Biogesic. Untilone day, he felt severe weakness and fell to the ground while sweeping theirbackyard. Because of the said incident, his family has decided to bring him to thehospital in their place in manila. He was sent to Philippine General Hospital. Hehad experienced to have blood transfusion (washed RBC) for several timesthere. The doctor prescribed him to take Ferrous Sulfate. According to the client,he continued to take Ferrous Sulfate as a supplement. He was admitted to manydifferent hospitals because of his condition, he was hospitalized for about 4 timesfor the past 4 years. First, he was admitted at PGH and the others are in TarlacProvincial Hospital. He also said that he does not go to the hospital for follow-upcheck-ups. According to him, he had chicken pox when he was in grade 4. He saidthat he had all the immunizations. According to him, he experience to havecough and colds only twice a year. He doesn’t have any allergies. According tohim, he did not have any other severe diseases in the past except his currentcondition.4. History of Present Illness Five days prior to admission the client stated that he experiencedshortness of breath, pallor for five days and generalized body weakness.According to the patient, when he is experiencing headache he takes a rest to 10
  11. 11. relieve it and takes paracetamol if it is accompanied by fever. He also stated thatthe symptoms happen on a sudden onset. When he felt that he cannot handlethe severe body weakness and his parents noticed that he is very pale, hisparents have decided to take him to the hospital immediately. He was confined toTarlac Provincial Hospital on February 10 with an admitting diagnosis ofparoxysmal nocturnal hemoglobinuria.5. Physical Examination 13 Areas of Assessment I. Social Status Mr. X is a 33 year old man who’s currently residing at Victoria Tarlac together with his family. He is a jeepney driver for about two years now but due to his current condition, he cannot be able to continue his work. He was married one year ago and not yet bless with any children. He described his family as having a close ties wherein he believed that whatever problems and chaos that the family will encounter is can be solved by helping each other and through prayers. Financial aspect is sometimes the problem that the family undergone. But he verbalized that his salary is just enough to sustain their daily needs. He interacts with different people to their place and doesn’t have misunderstanding getting along with them. Despite his current condition, he still manages to interact with other patient and health workers during his confinement in the hospital. His wife is the one who stays and guide with him. The family perceived his condition as alerting and felt nervous about it. He is not a member or joined to any organizations in their place. The client is a Born Again Christian and regularly attends services. He believed that life is very important. In times of difficulties, he seldom goes and talked with his cousin, who is a Pastor and also his good friend to get some advice. 11
  12. 12. Norms Social support is involved in mitigating the human stressfulresponse and associated illness. It meets a fundamental human need orsocial ties, making life less stressful, thus indirectly contributing to goodhealth outcomes. Social responsibilities include forming new friendshipsand assuming some community activities. Social functioning of anindividual is to form relationships with others. Social support is aperception that one has an emotional and tangible resource to fall onwhen needed; perceived social support is being followed by the family toexpress the love of the family, financial aspect is one of the normalconstraints in the family. (Nursing fundamentals by Daniels; anintroduction to health and physical assessment in nursing by D’Amico andBarbarito)Analysis The patient’s social status can be described as normal; he hassupport system (the family) which he can turn to when facing difficultperiods particularly upon encountering emotional or coping crisis and hasa strong foundation of emotional stability. The client’s spiritual relationshipwith God is very strong and he has a strong faith with Him. He also hasclosed family ties and interacts well with others. He also communicateswith his fellowmen thus, he gain many friends.II. Mental Status • Physical Appearance and Behavior During the interview, Mr. X wears a shorts and shirt which areappropriate for his age and for the weather. We have observed that hewas not properly groomed, have untrimmed nails on both fingers and toesand with uncombed hair. He looks pale and weak. 12
  13. 13. Mr. X facial expressions were appropriate for his feeling and moodof conversation he was able to established good eye contact. When askedto walk, he exhibits an erect posture, a smooth gait and symmetrical bodymovements. He is cooperative throughout the interview and answered allquestions asked. • Level of Consciousness and Orientation The client was conscious, coherent and responsive during theinterview. He was oriented with the time, place where he is andrecognizes the persons who are with him. • Intellectual Function Mr. X is a graduate of 2 year Sea Man course. His ability to readand write matched his educational level. He was able to understand everyquestion that was asked from him and he was able to respond to themappropriately. He was able to remember past experiences during youngeryears and recall family history. • Speech Mr. X can speak Ilocano and Tagalog. He was able to speakspontaneously with coherent speech. He was able to express himself.Norms The patient should appear relaxed with appropriate amount ofconcern for the assessment. He should exhibit erect posture, a smoothgait and symmetrical body movements with regards to posture andmovements. The patient should be clean and well-groomed and shouldwear appropriate clothing for age, weather, and socio-economic status.Facial expression should be appropriate to the content of the conversationand should be symmetrical. The speech should have an effortless flow.The patient’s ability to read and write should match his educational level.He should be aware of self and the environment and should be able torespond appropriately to questions being asked. (Health Assessment andPhysical Examination 2nd Ed, Estes pp.656-663) 13
  14. 14. Analysis Based on the norms given, there were no major deviations fromnormal on the mental status of the patient. However, the patient has poorpersonal hygiene such as not properly groomed, untrimmed nails,uncombed hair which are associated by prolonged confinement in thehospital.III. Emotional Status During the interview, Mr. X told us that “pagkakasakit ay swerteswerte lang”. He considered that having a disease is just a bad luck(malas). It was noted that he has a positive coping and acceptance of hishealth condition. He has a strong faith in God that he considered prayersas his source of strength. Likewise, his relationship with his family is harmonious and conflictsare easily resolved. During his stay in the hospital, his family is alwaysthere beside him to support and serve whatever he needs. Aside from this,he also added that he usually talked to their ‘pastor’ which is his cousin,who is also his friend to asked for advice. He is also fond of watchingtelevision during his free time. This is also his means of entertainment anda sort of relieving stressful events in his life.Norms Emotional wellness is the ability to manage stress and to expressemotions appropriately. It involves the ability to recognize, accept andexpress feelings, and to accept one’s limitations. (Fundamentals OfNursing, Kozier, pg 173.) Normal coping pattern or emotions stability couldinclude acceptance of the problem, adjustment to it, expressing of self-perception and self-control of emotions, probable temporary use ofdefense mechanism and support system (Fundamentals of Nursing byKozier). Carrying out emotional feelings through words and facial 14
  15. 15. expressions are normal signs of present physical condition (NursingFundamentals by Daniels)Analysis The emotional state of the patient is well established. He does notshow any emotional feeling and weaknesses while in the hospital despitehaving a health condition. The patient manifest acceptance with regardsto his health condition and keep on being strong and enjoying life he hadnow and he spontaneously felt support from his family and friends. He isalso capable of controlling his emotions.IV. Motor Stability Prior to BT the patient experienced severe body weakness and hewas mostly confined on bed due to easy fatigability. After BT the patientregains his strength. He’s able to ambulate without assistance but stillcannot tolerate too much activity. The patient is able to transfer from bedto chair and vice versa.NORMS: Motor stability is the ability to move freely, easily, rhythmically, andpurposefully in the environment. People must move to protect themselvesfrom trauma and to meet their basic needs. It is vital to independence; afully immobilized person is vulnerable and dependent as an infant.(Fundamentals of Nsg. by Kozier)Analysis The patient was not able to tolerate too much activity and performADL’s due to easy fatigability. Blood transfusion is his way of regaining his strength.V. Body Temperature 15
  16. 16. The client’s general skin is warm to touch during the interview. Thefollowing table indicates the client’s body temperature. Date and Temperature (0C) Analysis hours 2/11/09 8 am 36.5 0C Normal 10 am 36.7 0C Normal 1:30 pm 36.8 0C Normal 3:00 pm 37.1 0C Normal 2/12/09 8 am 37.8 0C Abnormal 12 noon 38 0C Abnormal 2 pm 38.3 0C Abnormal 3:30 pm 38.4 0C Abnormal 4:30 pm 38 0C Abnormal 6 pm 37.8 0C Abnormal 10 pm 37.3 0C Normal 2/13/09 8 am 37.2 0C Normal 10 am 37.4 0C Normal 2 pm 37.5 0C Normal 5 pm 38.9 0C Abnormal 6 pm 38.7 0C Abnormal 8 pm 38.5 0C Abnormal 10 pm 37.9 0C Abnormal 2/14/09 6 am 38 0C Abnormal 8 am 37.8 0C Abnormal 10 am 37 0C Normal 2 pm 37 0C Normal 6 pm 37.2 0C Normal 2/15/09 6 am 38.2 0C Abnormal 6 pm 36.5 0C Abnormal 2/16/09 8 am 36.9 0C Normal 10 am 36.7 0C Normal 12 noon 37.2 0C Normal 1:30 pm 37.2 0C Normal 4 pm 37.2 0C Normal 10 pm 38.9 0C Abnormal 2/17/09 4 pm 38.5 0C Abnormal 10 pm 38.2 0C Abnormal 2/18/09 6 am 37.2 0C Normal 2 pm 38.8 0C Abnormal 5 pm 37.2 0C Normal 2/18/09 4 pm 37.3 0C Normal 10 pm 38.1 0C Abnormal 16
  17. 17. Norms A healthy persons body temperature fluctuates between 97°F(36.1°C) and 100°F (37.8°C), with the average being 98.6°F (37°C). Thebody maintains stability within this range by balancing the heat producedby the metabolism with the heat lost to the environment. Core bodytemperature was established by the temperature of blood perfusing thearea of the hypothalamus (body’s temperature control center) which cantrigger the body’s physiological response to temperature. (Healthassessment and physical examination 3rd edition by Mary Ellen ZatorEstes) Fever may suggest infections, and bleeding. A fever occurs whenthe thermostat resets at a higher temperature, primarily in response to aninfection. To reach the higher temperature, the body moves blood to thewarmer interior, increases the metabolic rate, and induces shivering.(www.fpnotebook.com/Hemeonc/Hemolysis/PrxysmlNctrnlHmglbnr.htm)Analysis During the stay in the hospital, client was experienced fever almostall the time. His fever is a response to what is happening to his body. Dueto his condition, because of inability of protein to bind into the cellmembrane whereas lacking of these complimentary protein act on the T-lymphocytes of the cell which are primary responsible for the immuneresponse. These complimentary proteins cannot bind on the cell, infectionmay possibly occur which is the primary cause f fever in the client.VI. Circulatory Status The client’s general skin color is pale in appearance including hisconjunctiva, lips, tongue, gums, palms and nails. His peripheral pulses areregular but apical pulse was very visible. No abnormal heart sound noted.Capillary refill is at the speed of 5 seconds for both fingers and toes. 17
  18. 18. The client’s blood pressure and pulse rate are noted in the followingtable: Date and hours Blood pressure (mmHg) Analysis 2/11/09 8 am 90/60 Abnormal 10 am 100/80 Abnormal 1:30 pm 100/60 Abnormal 3:00 pm 100/70 Abnormal 2/12/09 8 am 100/60 Abnormal 12 noon 100/60 Abnormal 2 pm 100/60 Abnormal 3:30 pm 110/60 Abnormal 4:30 pm 100/70 Abnormal 6 pm 110/70 Abnormal 10 pm 100/60 Abnormal 2/13/09 8 am 100/70 Abnormal 10 am 110/80 abnormal 2 pm 100/60 Abnormal 5 pm 130/90 abnormal 6 pm 120/70 normal 8 pm 110/70 abnormal 10 pm 90/60 Abnormal 2/14/09 6 am 90/70 Abnormal 8 am 100/70 Abnormal 10 am 100/70 Abnormal 2 pm 110/70 Abnormal 6 pm 110/70 Abnormal 2/15/09 6 am 110/70 Abnormal 6 pm 110/70 Abnormal 2/16/09 8 am 90/60 Abnormal 10 am 100/70 Abnormal 12 noon 100/70 Abnormal 1:30 pm 100/70 Abnormal 4 pm 120/70 Abnormal 10 pm 110/70 Abnormal 2/17/09 4 pm 120/80 Abnormal 10 pm 110/70 Abnormal 2/18/09 6 am 100/60 Abnormal 2/18/09 4 120/80 normal pm 10 pm 130/90 abnormal Date and hours Pulse rate 18
  19. 19. (beats per min) Analysis 2/11/09 8 am 89 Normal 10 am 86 Normal 1:30 pm 87 Normal 3:00 pm 88 Normal 2/12/09 8 am 95 Normal 12 noon 96 Normal 2 pm 98 Normal 3:30 pm 106 Abnormal 4:30 pm 100 Normal 6 pm 94 Normal 10 pm 96 Normal 2/13/09 8 am 94 Normal 10 am 86 Normal 2 pm 105 *Abnormal 5 pm 102 Abnormal 6 pm 92 Normal 8 pm 91 Normal 10 pm 99 Normal 2/14/09 6 am 94 Normal 8 am 98 Normal 10 am 99 Normal 2 pm 98 Normal 6 pm 87 Normal 2/15/09 6 am 87 Normal 6 pm 90 Normal 2/16/09 8 am 88 Normal 10 am 88 Normal 12 noon 87 Normal 1:30 pm 86 Normal 4 pm 88 Normal 10 pm 86 Normal 2/17/09 4 pm 88 Normal 10 pm 85 Normal 2/18/09 6 am 88 Normal 2/18/09 4 pm 106 Abnormal 10 pm 86 NormalNorms In a healthy young adult, the pressure at the highest of the pulse(systolic pressure) is approximately 120 mmHg, and the pressure at thelowest point of the pulse (diastolic pressure) is approximately 80 mmHg.The normal pulse rate of a healthy young adult is 60-100 beats per 19
  20. 20. minute. Normal capillary refill is at the speed of 2-3 seconds. Lips,conjunctiva, gums, nail beds and palms are should be pinkish in colour.(Fundamentals of Nursing by Barbara Kozier, et al.)Analysis Client’s blood pressure rates were mostly abnormal compared onthe normal values. Pulse rates were somehow normal but it can alsoexceed to normal values. The client pale appearance including hisconjunctiva, lips, tongue, gums, palms and nails may be an indicative ofpoor circulation of blood in the body. Because red blood cells areimmaturely breaking down or hemolysis happens with this condition, blooddoes not carry enough RBCs which are responsible for the red colorationof the body surfaces.VII. Respiratory Status Mr. X was admitted with a chief complaint of difficulty of breathing,weakness and pallor. Upon admission, O2 inhalation therapy was givenwith a rate of 1-2 lpm. Nail clubbing was present on both hands and feetnails. Breathing pattern is effortless and use of accessory muscles wasnoted during the interview. He has a regular breathing pattern. Noabnormal breath sounds heard. Resonant sound is heard duringpercussion. The thorax is slightly elliptical in shape. The ratio of the APdiameter to the transverse diameter is approximately 1:2. The patient’s respiratory rate throughout the hospital confinement: DATE AND TIME RATE INTERPRETATION 2-11-09 22 Abnormal 8AM 25 Abnormal 10AM 22 Abnormal 1:30PM 23 Abnormal 3-11PM 21 abnormal 02-12-09 21 Abnormal 8AM 26 Abnormal 12PM 25 Abnormal 20
  21. 21. 2PM 33 *Abnormal 3:30PM 25 Abnormal 6PM 28 Abnormal 10PM 28 Abnormal 2-13-09 6 am 26 Abnormal 8AM 35 Abnormal 10AM 26 Abnormal 2PM 24 Abnormal (3-11PM) 5PM 26 Abnormal 6PM 29 Abnormal 8PM 31 Abnormal 10PM 29 abnormal 2-14-09(11-7AM) 25 Abnormal 8AM 23 Abnormal 10AM 22 Abnormal 2PM 19 normal 3-11PM 20 normal 02-15-09(11-7AM) 20 normal 3-11PM 20 normal 2-16-09 (8AM) 30 Abnormal 10AM 25 Abnormal 12PM 27 Abnormal 1:30PM 25 Abnormal 4PM 26 Abnormal 10PM 30 Abnormal 2-17-09(4PM) 30 Abnormal 10PM 28 Abnormal 2-18-09(11-7AM) 26 Abnormal 7AM 25 Abnormal 10AM 24 AbnormalNorms Normal RR is 14-20 cycles per minute. Normal respirations areregular and even in rhythm. Depth of inspiration is unexaggerated andeffortless. Accessory muscle should not be used. Normal lung tissuesproduce resonant sound during percussion. Adventitious sounds shouldbe absent. The normal thorax is slightly elliptical in shape and the ratio of APdiameter to the transverse diameter is approximately 1:2 to 5:7. In other 21
  22. 22. words, the normal adult is wider from side to side then front to back.( Health Assessment and PE, Estes pg. 451-470)Analysis The patient has RR greater than 20 cpm, which means that he istachypneic. Tachypneic is frequently present in hypermetabolic andhypoxic state. By increasing the RR, the body is trying to supply additionaloxygen to meet the body’s demands.VIII. State of Physical Rest and Comfort Mr. X usually wakes 6 o’clock in the morning and starts the day witha cup of coffee and continues to exercise by doing house hold chores. Theclient verbalized that he sometimes feels dizzy and difficulty of breathingwhile doing house chores. He can work as a driver and perform activitiesof daily living with full self care without the help of others. During vacanttime, he usually watches television as a form of relaxation plays basketballor just mingle around and talked to some friends. On a daily basis, hesleeps for about 7 to 8 hours at night and takes a 4 hours nap in theafternoon while resting from work. Mosquitoes from their housesometimes interrupt him but most of the time his rest and sleeping timewas not interrupted. He sometimes watches DVD’s to catch his sleep. Theclient usually feels hungry every time he woke up in the morning. During his stay in the hospital, he was mostly confined on bedwherein he cannot perform daily activities like eating, taking a bath,voiding, and getting dress and requires assistance from others. Heverbalized to feel fatigue and shortness of breath even when doing lightactivities. He usually sleeps for about 4 hours with some interruptions fromothers patients and health workers that provide cares and proceduresevery now and then. His sleep was also interfered whenever he feels theurge to void for about 10 times in a night. He appears lethargic, restlessand irritable, weak in appearance and yawns frequently. The environment 22
  23. 23. in the hospital is not conducive and is also one factor that the client cannotrest enough. The hospital room is not well ventilated, warm in temperatureand the weather is also hot making the client uneasy.Norms The sleep wake cycle is very important to young adults. Theyusually have an active lifestyle, and are thought to require 7 to 8 hours ofsleep each night but may do well on less. Maintaining a regular sleep-wake rhythm is more important than the number of hours actually slept. Sleep exerts physiologic effects on both the nervous system andother body structures. Sleep in one way restores normal levels of activityand normal balance among parts of the nervous system. It is alsonecessary for protein synthesis, which also allows repair processes tooccur. (Kozier et. al., Fundamentals of Nursing 7th edition)Analysis Client experienced no complete sleep hours and irregular sleeppattern. Compared with the normal values, client has an inadequateamount of sleep which made him to become emotionally irritable, havepoor concentration, and experiencing difficulty in making decisions. Theclient manifest discomfort from environmental temperature and lack ofventilation which also affects his sleep and comfort.IX. Reproductive Status Mr. X was circumcised when he was 12 years old. He verbalizedthat they don’t use any contraceptive method. The client doesn’t have anychildren yet. No abnormal findings were noted like tenderness,enlargement, or nodular growth on his penis and scrotum as stated by theclient. He verbalized that he is experiencing erectile dysfunction since thetime that he felt his illness which making their marriage sexual lie andfunction to be impaired. 23
  24. 24. Norms Penile erection is managed by two different mechanisms. The firstone is the reflex erection, which is achieved by directly touching the penileshaft. The second is the psychogenic erection, which is achieved by eroticor emotional stimuli. The former uses the peripheral nerves and the lowerparts of the spinal cord, whereas the latter uses the limbic system of thebrain. In both conditions, an intact neural system is required for asuccessful and complete erection. Stimulation of penile shaft by thenervous system leads to the secretion of nitric oxide (NO), which causesthe relaxation of smooth muscles of corpora cavernosa (the main erectiletissue of penis), and subsequently penile erection. Additionally, adequatelevels of testosterone (produced by the testes) and an intact pituitarygland are required for the development of a healthy erectile system.Analysis: As can be understood from the mechanisms of a normal erection,client’s impotence was develop due to hormonal deficiency, which isdisorder of the neural system, and lack of adequate penile blood supply orpsychological problems. Restriction of blood flow was arising fromimpaired endothelial function which makes the client impotence. Thisproblem makes the client to be emotionally worried thus he feels that hecannot perform his role as a husband to his wife and he cannot render hisworth in achieving their sexual satisfaction.X. Nutritional Status Mr. X weighs 58kg with a height of 5’7”. His computed body massindex is 20.67. Prior to admission, the patient usually eats pork and doesnot eat vegetables. Upon admission, he eats food served by the hospital.But he still doesn’t eat vegetables, he only eat meat. He doesn’t havedifficulty of eating because he has a good set of teeth. He drinks an 24
  25. 25. average of 8-10 glasses of water a day. The patient stated that he havelost his appetite that resulted to loss of weight from 68kg to 58kg. BMI= weight in kg m2 = 58 kgs. (1.675 m)2 = 58 kgs. 2.805625 BMI = 20.67Norms Nutrition is the sum of all the interactions between an organism andthe food it consumes. Nutrients are organic are organic and inorganicsubstances found in foods and are required for body functioning. Peoplerequire the essential nutrients in food for the growth and maintenance ofall body tissues and the normal functioning of all body processes. Several approaches attempt to approximate water needs for theaverage healthy adult living in a temperate climate. The Institute ofMedicine advises that man consume roughly 3 liters (about 13 cups) oftotal beverages a day and women consume 2-2 liters (about 9 cups) oftotal beverages a day. Many health professionals consider the BMI to be a more reliableindicator of changes in body fat stores and whether a person’s weightappropriate to height and may provide useful instrument of malnutrition. ABMI with a result of 16 is considered as malnourished; BMI of 16-19 isundernourished. BMI of 20-25 is normal. BMI; of 26-30 is over weight; BMIof 31-40 is moderately obese to severely obese and greater than 40 ismorbidly obese (Kozier)Analysis 25
  26. 26. The patient knows the right food to eat but he is not fond of eatingvegetable. He meets the daily water requirement. Due to his condition hedemonstrated loss of appetite and he loss weight of about 10 kilograms.Despite the client’s condition his BMI is within normal range.XI. Elimination Status Client used to urinate frequently (5- times in day and -10 times innight) with different volume which is most prominent in night time whereinhis urine becomes more tea like color in appearance without foul smell.Defecates 1 to 2 times per day with brownish color stool. Patientverbalized that she has no difficulty in voiding and defecating.Norms Normal urine output for an individual is 1200 to 1500 ml for 24hrs.With color clarity of straw, amber transparent, faint aromatic odor and nopresence of blood. (Fundamentals of Nursing by Kozier) Medications can have an impact on the client’s elimination healthand pattern. Diuretic increase urine production. Anti depressants,antihypertensive and some antihistamines and OTC cold medications maylead to urinary retention. (Nursing Fundamentals by Daniels)Analysis Tea colored urine present to the client is a manifestation of hiscondition where in there is an immature breakdown of RBCs in the bodywhich is eventually accumulates in the urine that makes it color tea like.Urine is more concentrated during night time because body is at rest anddoes not require a lot of movement unlike in daytime.XII. Sensory Status 26
  27. 27. Client doesn’t wear any reading aid, his pupils size are 4mm equal.He has an intact visual acquity, sclera is anecteric and cardinal fields ofgaze are intact, in assessing corneal light reflex the reflected light seensymmetrically in the center of each cornea, conjunctiva is pale and moist.Reaction to light on both eyes is brisk. With uniform reaction toaccommodation. Mr. X has the ability to respond to light touch, superficialpain and temperature. His sense of smell is normal and he can distinguishfoul and fresh odor. Client’s both nostrils are patent, no evident swelling ofthe frontal and maxillary sinuses and excessive mucus discharges. Withregards to the auditory perception, Mr. X can hear spoken words w/ a 2feet distance away from the client. Lips are pale and dry, gums are pale-red in color, no bleeding and swelling noted. Buccal mucosa is pale incolor, smooth and moist, no lesions and halitosis noted. Tongue is alsopale in color, moist and rough, able to perform normal tongue movements,asked client to move tongue side to side up and down. Client candifferentiate food according to taste, gag reflex present. Tonsils aregraded 1+, uvula located on the midline (Normal, no signs ofinflammation).Norms The client should be able to perceive light touch, superficial pain,and temperature accurately and perceive the location of stimulus. Duringassessment of auditory perception the client should be able to hearspoken words from a distance of 2ft. Nostril should be patent, there shouldbe no evidence of swelling around the nose and eyes and lastly the clientshould distinguish and identify the odors w/ each nostril. Breath shouldsmell fresh; lips and membranes should be pink and moist w/ no evidenceof lesions and inflammations. Tongue should be in the midline of themouth; the dorsum of the tongue must be pink, moist and rough (from thetaste buds) and must be w/o lesions. It should move freely and thestrength of the tongue is symmetrically strong, buccal mucosa should be 27
  28. 28. moist, smooth and free from lesions. Gums should be pale-red stippledsurface on light skinned people. Gum margins should be defined, nopresence of swelling and bleeding. Normal tonsilar size is graded 1+ or2+, no swelling and exudates present, uvula in on the midline. Corneallight reflex (light reflex) should be symmetrically in the center of eachcornea. Both eyes should move smoothly and symmetrically in each of thesix fields of gaze conjunctiva must appear pinkish and moist. (Healthassessment and physical examination 3rd edition by Mary Ellen ZatorEstes). Adult’s pain perception and behavior exhibited when experiencingpain may be gender-based behaviors or by own interpretation of pain thatshe/he is feeling. (Fundamentals of Nursing by Kozeir)Analysis Client’s pale appearance of the skin and mucous membranes(conjunctiva and mucosa) may indicate signs of anemia or perfusebleeding.(Medical Surgical Nursing 11th Edition by Brunner andSuddarths) Due to his condition, he don’t have enough blood supplywherein his hemoglobin level is below normal (39 g/l compared to 120-10normal) thus making the client appearance to be pale. Hematocrit level(0.17) from a normal 0.37-0.47 value is also very low. Other than that,client does not show any significant deviations from the normal values andthus, considerately shows no sensory impairment.XIII. . Skin Appendages Mr. X’s skin was pale all over the body but most apparently on theface, mouth, lips, and conjunctiva. It is dry with minimize perspiration,rough and warm to touch. It has no lesions and it is non tender. It returnsto its original state rapidly when the skin is pinched and released. Scalpwas pale white and there were no signs of infestation or lesions. Nodandruff found. His hair is equally distributed, rough and black in color. Hehas untrimmed fingernails and toenails which pale in color and clubbing 28
  29. 29. was also evident on both his fingernails and toenails. They appearedconvex and wide and angle of the nail base was greater than 160 0. Nailsurface was smooth and its thickness was uniform throughout. Thevenipuncture site was located on his left cephalic vein.Norms Normally, the skin is a uniform whitish pink or brown color,depending on patient’s race. No skin lesions should be present. It shouldbe dry with minimize perspiration. Moisture on the skin will vary from onebody area to another with perspiration normally present on the hands,axilla, face, and in between the skin folds. Skin surface temperature bewarm and equal bilaterally. Hands and feet may be slightly cooler than therest of the body. Skin surfaces should be non tender. It should normallyfeel smooth, even and firm except where there is significant hair growth. Acertain amount of roughness can be normal. When the skin is pinched, itshould return to its original contour when released. The scalp should bepale white to pink in light-skinned individuals and light brown in dark-skinned individuals. There should be no sign of infestations or lesions.Seborrhea may be present. Hair may feel thin, straight, course, thick orcurly. It should be shinny and resilient when traction is applied. Normally,the nails have a pink cast in light skinned individuals and are brown indark skinned individuals. The nail surface should be smooth and slightlyrounded or flat. Its thickness should be uniform throughout, with nosplintering or brittle edges. The angle of the nail base should beapproximately 1600.Analysis Mr. X skin was pale which is due to low hemoglobin. Untrimmed toenails and fingernails indicate self care deficit and clubbing of the nailsresult from long-standing hypoxia. Mr. X also has poor peripheralcirculation which is indicated by slow capillary refill. 29
  30. 30. Client is at risk for infection with regards to the venipuncture hehad. 30
  31. 31. 6. Diagnostic and Laboratory ProceduresDIFFERENTIAL COUNTS:Hematology- This diagnostic test is a tool that provides information about the hematologic system of the patient. Diagnostic/ Date ordered Indications or Normal Analysis and Laboratory and date purposes Results values Interpretation of procedure results data FebruaryHemoglobin 10,2009 - is a measure of 31 g/l 120-180 Below normal range: the total amount of In response to 8:23 am hemoglobin in the decrease RBC, blood. It carries hemoglobin also oxygen to the cells decrease from the lungs and carbon dioxide away from the cells to the lungsHematocrit - measure the .092 L/L .370-.510 Below normal range: percentage of red can be a sign of the blood cells in 100 presence of ml of whole blood. hemorrhage, Determines if the anemia, client is hydrated or hyperthyroidism, dehydrated. dietary deficiency and pregnancy.RBC used to evaluate .90 T/L 4.2-6.3 Below normal range. MCV the size, weight Decreased RBC MCHC and hemoglobin result in lysis of RBC MCH concentration of due to lack of decay 31
  32. 32. RBC’s. Oxygen accelerating factor(CD55 and transportation is its CD59) on RBC. major function.WBC - determines the 8.1 G/L 4.1-10.9 Within normal range. Lymphocytes number of 0.225 0.6-4.1 low lymphocytes circulating WBC’s indicates decrease in the blood. It activity of the bone monitors the marrow presence of infection in the body.Platelet - platelets are the 168 G/L 140-440 Within normal range first line of protection against bleeding.Blood typing “A”RH Factor +DIFFERENTIAL COUNTS:Hematology- This diagnostic test is a tool that provides information about the hematologic system of the patient. Diagnostic/ Date ordered Indications or Normal Analysis and Laboratory and date purposes Results values Interpretation of procedure results data FebruaryHemoglobin 13,2009 - is a measure of 36 g/l 120-180 Below normal range: 32
  33. 33. the total amount of In response to 6:57 am hemoglobin in the decrease RBC, blood. It carries hemoglobin also oxygen to the cells decrease from the lungs and . carbon dioxide away from the cells to the lungsHematocrit - measure the .87 L/L . .370-.510 Below normal range: percentage of red can be a sign of the blood cells in 100 presence of ml of whole blood. hemorrhage, anemia, Determines if the hyperthyroidism, client is hydrated or dietary deficiency dehydrated. and pregnancyRBC used to evaluate 1.01 T/L 4.2-6.3 Below normal range. MCV the size, weight Decreased RBC MCHC and hemoglobin result in lysis of RBC MCH concentration of due to lack of decay RBC’s. Oxygen accelerating factor(CD55 and transportation is its CD59) on RBC. major function.WBC - determines the 6.9 G/L 4.1-10.9 Within normal range lymphocytes number of 1.2 0.6-4.1 circulating WBC’s in the blood. It monitors the 33
  34. 34. presence of infection in the body.Platelet - platelets are the 141 G/L 140-440 Within normal range first line of protection against bleeding.Blood typing “A”RH Factor +MCV - average volume 85.7 FL 80-97 Within normal range of individual RBC’sMCH - calculated 35.6 pg 26-32 above normal range. average weight of Due to macrocytic hemoglobin per anemia. RBCMHCH - average 414 g/l 310-360 above normal range. concentration or Due to macrocytic percentage of anemia. hemoglobin per RBCDIFFERENTIAL COUNTS: 34
  35. 35. Hematology- This diagnostic test is a tool that provides information about the hematologic system of the patient. Diagnostic/ Date ordered Indications or Normal Analysis and Laboratory and date purposes Results values Interpretation of procedure results data Feb. 14, 2009Hemoglobin - is a measure of 45 g/l 120-180 Below normal range: 7:05 am the total amount of In response to hemoglobin in the decrease RBC, blood. It carries hemoglobin also oxygen to the cells decrease from the lungs and carbon dioxide away from the cells to the lungsHematocrit - measure the .097 L/L .370-.510 Below normal range: percentage of red can be a sign of the blood cells in 100 presence of ml of whole blood. hemorrhage, Determines if the anemia, client is hydrated or hyperthyroidism, dehydrated. dietary deficiency and pregnancyRBC used to evaluate . 1.14 T/L 4.2-6.3 Below normal range. MCV the size, weight Decreased RBC MCHC and hemoglobin result in lysis of RBC MCH concentration of due to lack of decay RBC’s. Oxygen accelerating factor(CD55 and transportation is its CD59) on RBC. 35
  36. 36. major function.WBC - determines the 5.4 G/L 4.1-10.9 Within normal rangelymphocytes number of 1.4 0.6-4.1 circulating WBC’s in the blood. It monitors the presence of infection in the body.Platelet - platelets are the 127 G/L 140-440 Low platelet first line of indicates decrease protection against activity of the bone bleeding. marrowBlood typing “A”RH Factor +MCV - average volume 85.5 FL 80-97 Within normal range. of individual RBC’s Below normal range.MCH - calculated 39.5 pg 26-32 Due to macrocytic average weight of anemia. hemoglobin per RBC 464 g/l 310-360 Above normalMHCH - average range. 36
  37. 37. concentration or Due to macrocytic percentage of anemia. hemoglobin per RBCDIFFERENTIAL COUNTS:Hematology- This diagnostic test is a tool that provides information about the hematologic system of the patient. Diagnostic/ Date ordered Indications or Normal Analysis and Laboratory and date purposes Results values Interpretation of procedure results data Feb. 16, 2009Hemoglobin - is a measure of 58 g/l 120-180 Below normal 2:00 pm the total amount of range: In response hemoglobin in the to decrease RBC, blood. It carries hemoglobin also oxygen to the cells decrease from the lungs and carbon dioxide away from the cells to the lungsHematocrit - measure the .152 L/L .370-.510 Below normal range: percentage of red can be a sign of the blood cells in 100 presence of ml of whole blood. hemorrhage, Determines if the anemia, client is hydrated or hyperthyroidism, dehydrated. dietary deficiency and pregnancyRBC used to evaluate 1.80T/L 4.2-6.3 Below normal range. 37
  38. 38. MCV the size, weight Decreased RBC MCHC and hemoglobin result in lysis of RBC MCH concentration of due to lack of decay RBC’s. Oxygen accelerating factor(CD55 and transportation is its CD59) on RBC. major function.WBC - determines the 4.5 G/L 4.1-10.9 Within normal range Lymphocytes number of 1.2 0.6-4.1 circulating WBC’s in the blood. It monitors the presence of infection in the body.Platelet - platelets are the 104 G/L 140-440 Low platelet first line of indicates decrease protection against activity of the bone bleeding. marrowBlood typing “A”RH Factor +MCV - average volume 84.4FL 80-97 Within normal range of individual RBC’sMCH - calculated 32.2 pg 26-32 Above normal 38
  39. 39. average weight of range. hemoglobin per Due to macrocytic RBC anemia. Above normalMHCH - average 382 g/l 310-360 range. concentration or Due to macrocytic percentage of anemia. hemoglobin per RBCNursing responsibilities:Before • prepare the client • instruct client and family about requirements or restrictions(when and what to eat and drink, how long to fast) • explain to the client on how the procedure is done and why is it necessaryDuring • assist the client • use standard precautions and sterile technique as appropriate • use the correct procedure for obtaining the specimen • provide client comfort, privacy and safety • ensure correct labeling, storage and transportation of specimenAfter • nursing care of the client and follow-up activities and observations • compare previous and current test resultsBlood Chemistry Date Purpose Result Normal values Analysis 39
  40. 40. BUN 02-13-09 To asses for 18.71 2.9-8.2 mmol/L Elevated BUN and electrolyte creatinine level imbalance. indicates decreasedCreatinine 353.6 53-106mmol/L kidney perfusion.Nursing ResponsibilitiesBefore  Explain the test procedure and the importance of the test.During  Adhere to understand the precaution.  Apply pressure to the venipuncture site.  Explain that some bruising discomfort and swelling may appear at the site and that warm, moist compress can alleviate this. Monitor for signs of infection.After  Label the container and send to the laboratory.  Do hand washing after the test. 40
  41. 41. VII. Anatomy and Physiology ERYTHROPOIESIS Erythropoiesis is the development of mature red blood cells(erythrocytes). Like all blood cells, erythroid cells begin as pluripotential stemcells. The first cell that is recognizable as specifically leading down the redcell pathway is the proerythroblast . As development progresses, the nucleusbecomes somewhat smaller and the cytoplasm becomes more basophilic,due to the presence of ribosomes. In this stage the cell is called a basophilicerythroblast . The cell will continue to become smaller throughoutdevelopment. As the cell begins to produce hemoglobin, the cytoplasmattracts both basic and eosin stains, and is called a polychromatophilicerythroblast . The cytoplasm eventually becomes more eosinophilic, and thecell is called an orthochromatic erythroblast . This orthochromatic erythroblastwill then extrude its nucleus and enter the circulation as a reticulocyte .Reticulocytes are so named because these cells contain reticular networks ofpolyribosomes. As reticulocytes loose their polyribosomes they becomemature red blood cells.( www.som.tulane.edu) 41
  42. 42. Erythrocytes: (a) seen from surface; (b) in profile, forming rouleaux; (c) rendered spherical by water; (d) rendered crenate by salt. (c) and (d) do not normally occur in the body. RED BLOOD CELL, OR ERYTHROCYTE, is a hemoglobin-containingblood cell in vertebrates that transports oxygen and some carbon dioxide toand from tissues. Erythrocytes are formed in the red bone marrow andafterward are found in the blood. They are the most common type of bloodcell and the vertebrate bodys principal means of delivering oxygen from thelungs or gills to body tissues via the blood (Dean 2005). Erythrocytes consist mainly of hemoglobin, a complex moleculecontaining heme groups whose iron atoms temporarily link to oxygenmolecules in the lungs or gills and release them throughout the body. Oxygen can easily diffuse through the red blood cells cell membrane.Hemoglobin also carries some of the waste product carbon dioxide back fromthe tissues. The color of erythrocytes is due to the heme group ofhemoglobin. The blood plasma alone is straw-colored, but the red blood cellschange color depending on the state of the hemoglobin: when combined withoxygen the resulting oxyhemoglobin is scarlet, and when oxygen has been 42
  43. 43. released the resulting deoxyhemoglobin is darker, appearing bluish throughthe vessel wall and skin. Erythrocytes develop from committed stem cells through reticulocytes to mature erythrocytes in about seven days and live a total of about 120 days.he heme constituent of hemoglobin are broken down into Fe3+ and biliverdin.The biliverdin is reduced to bilirubin, which is released into the plasma andrecirculated to the liver bound to albumin. The iron is released into the plasmato be recirculated by a carrier protein called transferrin. Almost allerythrocytes are removed in this manner from the circulation before they areold enough to hemolyze. Hemolyzed hemoglobin is bound to a protein inplasma called haptoglobin which is not excreted by the kidney.(newworldencyclopedia.org) The G6PD(Glucose-6-dehydrogenase) gene provides instructions formaking an enzyme called glucose-6-phosphate dehydrogenase. Thisenzyme, which is active in virtually all types of cells, is involved in the normalprocessing of carbohydrates. It plays a critical role in red blood cells, whichcarry oxygen from the lungs to tissues throughout the body. This enzymehelps protect red blood cells from damage and premature destruction. glucose-6-phosphate dehydrogenase deficiency disrupt the normalstructure and function of the enzyme or reduce the amount of the enzyme incells.Without enough functional glucose-6-phosphate dehydrogenase, red bloodcells are unable to protect themselves from the damaging effects of reactiveoxygen species. The damaged cells are likely to rupture and break downprematurely (undergo hemolysis). Factors such as infections, certain drugs,and ingesting fava beans can increase the levels of reactive oxygen species,causing red blood cells to undergo hemolysis faster than the body canreplace them. This loss of red blood cells causes the signs and symptoms ofhemolytic anemia, which is a characteristic feature of glucose-6-phosphatedehydrogenase deficiency.( /ghr.nlm.nih.gov) 43
  44. 44. LYMPHOCYTE is a type of white blood cell (leukocyte) in thevertebrate immune system. The two main types of lymphocytes are T cellsand B cells, which function in the adaptive immune system. Otherlymphocyte-like cells are commonly known as natural killer cells, or NK cells,and are part of the innate immune system. The NK cells are sometimeslabeled "large granular lymphocytes," while the T cells and B cells are labeledas "small lymphocytes."Types of lymphocytes A stained lymphocyte surrounded by red blood cells viewed using alight microscope. The two main categories of lymphocytes are the B lymphocytes (Bcells) and T lymphocytes (T cell), both of which are involved in the adaptiveimmune system (Alberts 1989). B cells specifically are involved in the humoralimmune system and produce antibodies, while T cells are involved in the cell-mediated immune system and destroy virus-infected cells and regulate theactivities of other white blood cells (Alberts 1989). In essence, the function ofT cells and B cells is to recognize specific “non-self” antigens, during aprocess known as antigen presentation. Once they have identified an invader, 44
  45. 45. the cells generate specific responses that are tailored to maximally eliminatespecific pathogens, or pathogen infected cells. B cells respond to pathogens by producing large quantities ofantibodies that then neutralize foreign objects like bacteria and viruses. Inresponse to pathogens, some T cells, called "helper T cells," producecytokines that direct the immune response while other T cells, called"cytotoxic T cells," produce toxic granules that induce the death of pathogeninfected cells. The adaptive immune system, also called the "acquired immunesystem" and "specific immune system," is a response of the body wherebyanimals that survive an initial infection by a pathogen are generally immune tofurther illness caused by that same pathogen. The adaptive immune systemis based on dedicated lymphocytes. The basis of specific immunity lies in the capacity of immune cells todistinguish between proteins produced by the bodys own cells ("self" antigen—those of the original organism), and proteins produced by invaders or cellsunder control of a virus ("non-self" antigen—or what is not recognized as theoriginal organism). 45
  46. 46. Although the complement system has traditionally been considered partof the innate immune system, research in recent decades has revealed thatcomplement is able to activate cells involved in both the adaptive and innateimmune response. Complement triggers and modulates a variety of immuneactivities and acts as a linker between the two branches of the immuneresponse. In addition, the complement system maintains cell homeostasis byeliminating cellular debris and immune complexes. (www.nature.com) The complement system distinguishes "self" from "non-self" via arange of specialized cell-surface and soluble proteins. These homologousproteins belong to a family called the "regulators of complement activation(RCA)" or "complement control proteins (CCP)". The complement system isan enzyme cascade that helps defend against infection. Many complementproteins occur in serum as inactive enzyme precursors (zymogens); othersreside on cell surfaces. The complement system bridges innate and acquiredimmunity by Augmenting antibody (Ab) responses and immunologic memory,Lysing foreign cells, Clearing immune complexes and apoptotic cells.Complement components have many biologic functions (eg, stimulation ofchemotaxis, triggering of mast cell degranulation independent of IgE).(www.merck.com) Members of this family are: • complement receptor 1 (CR1 or CD35) • membrane cofactor protein (MCP or CD46) • C4b-binding protein (C4BP). • decay-accelerating factor (DAF or CD55) • factor H (fH) The complement system is an enzyme cascade that helps defend against infection. Many complement proteins occur in serum as inactive enzyme precursors (zymogens); others reside on cell surfaces. The 46
  47. 47. complement system bridges innate and acquired immunity by Augmenting antibody (Ab) responses and immunologic memory, Lysing foreign cells, Clearing immune complexes and apoptotic cells. Complement components have many biologic functions (eg, stimulation of chemotaxis, triggering of mast cell degranulation independent of IgE). (wikipedia.org) In addition, membrane components (decay-accelerating factor, CD55 andCD59, and membrane inhibitor of C8 and C9 insertion) are importantregulating proteins. The complement cascade is a dual-edged sword, causingprotection against bacterial and viral invasion by promoting phagocytosis andinflammation. Pathologically, complement can cause sub-stantial damage toblood vessels (vasculitis), kidney basement membrane and attachedendothelial and epithelial cells.( questdiagnostics.com) 47
  48. 48. 8. Pathophysiology 48
  49. 49. 49
  50. 50. B. PLANNINGNursing Priorities Based on Maslow’s Hiearchy of Needs: A. Enhance tissue perfusion 1. Ineffective Tissue perfusion: Peripheral r/t decreased hemoglobin concentration in blood B. Provide nutritional/fluid needs 2. Imbalanced nutrition: less than body requirements r/t decrease intake of essential nutrients C. Prevent complications brought about by disease 3. Activity Intolerance r/t imbalance between oxygen supply delivery and demand 4. Self-care deficit: Bathing/Hygiene r/t weakness and tiredness 5. Disturbed sleep pattern r/t excessive stimulation from environment 6. Anxiety r/t change in health status and role function 7. Risk for Infection r/t inadequate seco0.ndary defenses (decreased hemoglobin) D. Provide information about disease process, prognosis and treatment regimen 8. Deficient knowledge (PNH) r/t lack of exposure 50
  51. 51. Nursing Care Plans(Date Identified)Assessment Planning Intervention Expected OutcomeS After 6 1. Independent The pt. will> fatigue and shortness of breath hours of a. Assist client to semifowler’s position display anwhen doing light physical activities nursng R: To promote maximum lung expansion to increase inlike eating, urinating in bed pan, intervention, increase oxygenation and tissue perfusion. peripheral tissueoral and body hygiene and the client will perfusion aschanging clothes display an b. Assist client to do deep breathing exercises manifested by:> general body weakness increase in R: Helps regulate rate of breathing and anxiety to a. improvement peripheral conserve pt.’s energy. in capillary refillO tissue b. good> requires SO’s assistance when perfusion. c. Provide and quiet environment and provide peripheralaccomplishing ADLs comfort measures. pulses> pale conjunctiva, oral and nasal c.1 Change linens regularly. c. normal heartmucosa and integument c.2 Instruct SOs to minimize talking with the pt. rate and> carpal and tarsal clubbing c.3 Provide back massage as needed. respiratory rate> hair growth on fingers and toes c.4 Assist pt. in doing guided imagery and d. verbalizationabsent visualization relaxation techniques of improvement> capillary refill of 5 seconds in R: Helps promote rest and relaxation which in level offingernails, 4-5 seconds in toenails conserves pt.’s energy and decreases the body’s energy> Tachycardia = 105 bpm demand for oxygen. e. improvement> Tachypnea = 33 cpm in disposition> Hgb value = 36 g/l 2. Collaborative f.improvement of> Hct values = 0.17 a. Assist in obtaining specimen for laboratory Hgb/Hct values studies (Hb/Hct, RBC count, ABG)Nsg Dx R: Identifies deficiencies in RBC composition andIneffectiveTissue Perfusion: monitors the pt’s status in terms of oxygenationperipheral r/t decreased Hgb and perfusion. Also serves as a parameter forconcentration in blood client’s progress in achieving activity tolerance. 51
  52. 52. SE: b. Provide supplemental oxygen as indicated. PNH is a condition in which there R: Maximizing oxygen-carrying capacity of RBCsis a continuous autoimmune to transport to tissues of the body.destruction of RBCs. A significantdecrease in the total number of c. Administer packed RBC blood transfusion ascirculating RBCs would lead to indicated.inadequate amount of oxygen R: Increases the number of oxygen-carrying cellsperfused to the tissues of the body. to correct inadequate tissue perfusion.Poor perfusion at the peripheralswould cause clubbing, prolongedcapillary refill time, pale nailbeds,weak pulses and fatigue.Compensatory mechanisms liketachycardia and tachypnea helpincrease tissue perfusion which isalso evident in the pt. 52
  53. 53. Assessment Planning Intervention Expected OutcomeS: After 1 hour of 1. Independent: After appropriate nursing> fatigue and shortness of breath daily nursing a. Limit activities and decrease intervention, pt. willwhen doing light physical activities intervention, external stimulus. display a graduallike eating, urinating in bed pan, client will display R: Limitation decreases oxygen increase in activityoral and body hygiene and a gradual demand and decreasing stimulus tolerance as manifestedchanging clothes progressive promotes relaxation and decreases by:> frequently naps during daytime tolerance of anxiety which can also increase a. increase in capacity to(1-2 hours) physical activity oxygen demand. do ADLs w/o report of b. absence of chest painO: chest pain upon b. Assist patient to gradually and SOB while doing> confined to bed most of the time exertion increase activity level. Start from daily activities> pt. depends on assistance of SO simple ADLs like combing hair, c. improvement of skinin accomplishing ADLs like eating, brushing teeth and eating. Progress and nail color, peripheralurinating in bed pan, oral and body to mild activity like active-assistive pulses and capillary refillhygiene and changing clothes ROMs and then ambulating with which indications good> appears generally weak assistance. circulation> fingernails and conjunctiva pale R: Gradual increase in activity level d. increase in> tachycardia = 103 bpm ensures that the pt.’s heart is not independence> tachypnea = 33 cpm overworked and the complications of> low HB= 36 g/l prolonged immobility will be> low HCT= 0.17 prevented.Dx: c. Record and document pt.’s VSActivity intolerance [Level III] r/t before, during and after activitiesimbalance between oxygen supply and correlate with presence orand demand absence of SOB. R: Provides a baseline trend toSE: monitor pt.’s tolerance on thePNH is a condition in which the activity. Also provides a source forRBC count is decreased because of evaluation for the client’s progress tocontinuous hemolysis. Pale increase his activity tolerance.fingernails and conjunctiva as wellas low Hb/Hct indicates an d. Instruct pt. to avoid activitiesabnormally low RBC count. which increase abdominal pressure. (e.g. straining during defecation)An increase in physical activity will R: It can cause bradycardia whichcause the cells to increase their would decrease tissue perfusion todemand for oxygen to meet the all tissues including the myocardialincreased metabolic state. tissues.However, the amount of oxygen 53supplied by the RBC is decreasedbecause of the decrease in thenumber of circulating RBCs.
  54. 54. Assessment Planning Intervention Expected OutcomeS: After 8 hours Independent: After appropriate nursing> Frequent daytime naps of nursing a. Explain the necessity for therapeutic and intervention, client will report(1-2 hours) intervention monitoring procedures while the client is an improvement in> Feels that he lacks energy the client will hospitalized. sleep/rest pattern asand is always tired report an R: Pt. is more apt to be tolerant of manifested by:> Has difficulty in falling improvement disturbances by staff if he understands the a. verbalization of increaseasleep at night in sleep/rest reasons and importance of care. in energy and physical pattern. activityO: b. Restrict the intake of foods and fluids rich b. reduction or absence of> less than age-normed in caffeine yawning, irritability andtotal sleep time (7-8 hours) R: Increases pt.’s wakefulness and delay restlessness> lethargic falling asleep. c. increase in total time of> irritable and restless continuous, uninterrupted> yawns frequently c. Support continuation of usual bedtime night time sleep> weak in appearance rituals.> Frequent conversations R: Promotes relaxation and readiness forfrom SO sleep.> Interruption of rest and 54
  55. 55. sleep due to therapeutic d. Increase interaction time between pt. andand monitoring activities of SOs/staff during day and reduce physical andhealth care workers in mental activities late in the day and at night.hospital Minimize unnecessary disturbances during hours of sleep at night.Dx: R: Planned activities during daytime andDisturbed sleep pattern r/t reduction of stimulation during night timeexcessive stimulation from promotes continuous, uninterrupted sleep.environment e. Provide comfort measureSE: e.1 provide evening snack if availableExcessive environmental e.2 hygiene (bed bath and oral care)stimulus causes a disruption e.3 massage and back rubin the normal sleep-wake e.4 provide clean and comfortable bedcycle of the pt. Disturbance e.5 assist pt. to wear comfortable clothesin sleep esp. night time R: Promotes drowsiness, aid in relaxationreduces the length of REM and falling asleep.sleep. Insufficient REMsleep causes the pt. to feel f. Reduce fluid intake in the evening andfatigue and lack of energy. advice client to urinate/defecate beforeThe pt. also manifests sleeping if necessary.frequent yawning and R: Decreases the need to get up and go toirritability. The body bathroom during night time and preventscompensates for the interruption of REM sleep.insufficiency by takingdaytime naps which is alsoevident in the pt. 55

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