Nursing perspectives in meningococcal disease

In recent years, much media attention has been given to reporting meningcococcal disease. Dramatic headlines, however, often fail to give the true picture. This article explores the pathophysiology of meningococcal disease and gives an account of the nursing care and treatment received by a 6-month-old infant admitted to hospital with severe meningococcal purpura fulminans. A review of current therapy is presented.

of illness from a mild transient bacteraemia to a potentially fatal overwhelming sepsis. Meningococcal meningitis refers to inflammation of the meninges and cerebrospinal fluid (CSF) by the organism, while meningococcal sepsis or meningococcaemia indicates infection in the blood. A combination of both can occur. In its most severe form, a syndrome of septic shock with disseminated intravascular coagulation (DIC), known as meningococcal induced purpura fulminans, occurs. It remains unknown why people display such varying degrees of illness. It has been suggested that both the genetic make-up of the organism and host are responsible. Environmental factors are also thought to play a role (Kennedy & Duncan 1996). Meningococcus invades the bloodstream, then multiplies and releases endotoxin from the capsule. This initiates a complex cascade of chemical mediators which, although beneficial in the normal inflammatory response, in higher concentrations, can result in haemodyamic instability characterized by myocardial depression, capillary leak, peripheral shutdown, abnormal coagulation pathway and bone-marrow dysfunction, carrying with it a risk of mortality. Although a vaccine against this organism, that is effective against all serogroups has yet to be developed, (vaccines exist against serogroups A, C, W135, Y) the low incidence of neonatal infection suggests some immunity is passed transplacentally conferring protection for the first 6 months of life. The increased incidence of meningococcal disease in the 8-12 month age group supports this theory. As age increases, a decline in incidence occurs, therefore meningococcal sepsis is more c o m m o n in the paediatric population than in the adult population. Mortality with this condition is about 10%, but if it occurs in its most severe form, fulminating meningococcaemia, mortality often exceeds 50% (Smith et al 1998).

PATHOPHYSIOLOGY

CASE S T U D Y

Neisseria Meningitidis, the organism responsible for meningococcal illness is a uniquely human pathogen which is spread by droplet infection. It is a gram-negative aerobic diplococcus which has a polysaccharide capsule. It is classified into a number o f different serogroups i.e. A, B, C, D, W135, X, Y, according to this polysaccharide capsule. Serogroup B is the most c o m m o n cause of meningococcal disease in developed countries. Neisseria Meningitidis, is carried by 2-10% of the healthy population in their nasal mucosa. Invasion into the bloodstream, if it occurs, can result in varying degrees

Jane presented to the casualty department accompanied by her mother. Jane, who was on holiday from Australia, had previously been a healthy, good humoured baby but had been irritable since morning and refusing feeds in the afternoon. She appeared pale on arrival with signs of reduced peripheral perfusion. A core temperature of 39.5 c was noted, with a blood pressure (BP) of 104/49 and a heart rate of 180. She was lethargic. An intravenous (IV) cannula was sited and blood drawn for urea and electrolytes, full blood count and blood gas analysis. A urine specimen was obtained and a lumbar

Nursing perspectives in meningococcal disease ili!~

Eilish Moore and Norma Healy

Meningococcal sepsis is a potentially life threatening disease. Recent advances have led towards increased emphasis being placed on early identification and prompt aggressive management of these patients. This article outlines the disease pathology, describing a case study to illustrate the management and nursing care of a child with meningococcal sepsis. Current therapies are also discussed.

INTRODUCTION

Eilish Moore, RGN, RSCN, Norma Healy, RGN, RSCN, St. Patrick's ICU, Our Lady's Hospital for Sick Children, Crurnlin, Dublin 12

(Requests for offprintsto ED1 or Nil) Manuscriptaccepted 4 May 1998 Intensive and Critical Care Nursing

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puncture was performed in a effort to identify the source o f infection. Chest and abdominal X-rays were also taken. While laboratory results were awaited a bolus o f Albumin 4.5% (10mls/kg) was given along with a dose o f Ceftaxime IV. A provisional diagnosis o f acute gastro-enteritis was made. Jane's CSF proved clear with no organisms growing, while her X rays were normal. Her blood picture however showed a marked metabolic acidosis, a low white cell count and platelet count. W i t h i n 3 hours o f her arrival in casualty, Jane had developed a petechial rash extending over her trunk and legs. She appeared increasingly tachypneoic and progressively more listless, facial oxygen was initiated. Skin scrapings were taken from one o f the lesions and further blood was taken for coagulation studies. Gramnegative diplococci grew from the skin scraping and her coagulation studies were abnormal with a prolonged prothrombin time and activated partial thromboplastin time and a rise in fibrin degradation products. A diagnosis o f meningococcal sepsis with a rapidly developing D I C was made. She was intubated and mechanically ventilated while transfer to intensive care (ICU) was arranged. Jane's mother was extremely distressed to witness this acute deterioration in her baby's condition and required a great deal o f support and reassurance as well as practical help in contacting Jane's father who was due to travel to Ireland to j o i n his family. H e was advised to travel as soon as possible. O n arrival in I C U , arterial and central line access was secured and a uretherat catheter passed to facilitate monitoring. An arterial blood gas (ABG) showed a pH7.1, standard bicarbonate (SBC) 15.2 and base excess (BE) 10.4. Large volumes o f Albumin 4.5% and cry stalloid fluid were infused. To facilitate ventilation Jane was given a muscle relaxant, pancuronium, and sedated with a Morphine infusion. A volume controlled mode o f ventilation was used. The rash was noted to be spreading and darkening in colour. Patchy areas appeared on both cheeks, extending over Jane's ears, her trunk and all four limbs showed involvement. In a effort to control the D I C , fresh frozen plasma (FFP) was commenced, as was a Heparin infusion at 10 units/kg/hr. Protein C was started initially with a test dose of 10iu/kg, and once no adverse reactions were noted a loading dose o f 100 i u / k g was given followed by a maintenance dose o f 15 iu/kg/hr. Jane displayed marked haemodynamic instability overnight necessitating the use ofinotropic drug therapy, Dopamine and Adrenaline infusions were commenced. Following a sudden episode o f oxygen desaturation a chest X-ray

revealed a large pleural effusion on the right side. A chest drain was immediately inserted draining 120 mls o f clear fluid. Despite increasing inotropic support Jane's BP remained labile and her urinary output was decreasing, so a decision was made to commence continuous veno-veno heamodiafiltration (CVVHDF). A vas-cath was inserted into her right internal jugular and haemofiltration commenced. Initially an even balance was maintained, i.e. ultrafiltrate losses were replaced ml for ml with replacement solution. Heparin was infused through the circuit to prevent the filter clotting. Dosage ranged from 10-30 iu/kg/hr, aiming to maintain a activated clotting time (ACT) of between 180-200. Her original heparin infusion was discontinued but Protein C was continued as her level was <30%. Regular blood sampling was performed to m o n itor electrolyte levels, allowing appropriate adjustments to be made to both the dialysis and replacements fluids. Boll o f sodium bicarbonate 8.4% were given as were transfusions o f blood and platelets. Jane's father arrived after a long flight to be greeted by a fairly grim scene; his daughter was practically unrecognizable amidst a web o f wires and tubing. He j o i n e d his wife at her bedside vigil. Frequent information updates were given to Jane's parents, treatment procedures and test results were interpreted. T h e y were encouraged to touch Jane and talk to her and invited to participate in her basic care routines. Despite the use of a warming blanket to help maintain body temperature, Jane's feet felt cool to the touch with a poor capillary refill. An urgent referral to the plastic surgeons was made as both legs were n o w very odematous and tense. Compartmental pressures were measured and found to be grossly elevated. Surgical decompression was felt to be too high a risk so it was advised to keep her lower limbs elevated and to measure thigh and calf girths frequently. T h o r o u g h examination o f all her skin lesions proved difficult as she displayed a marked intolerance to handling. N o further extension o f her rash was noted and efforts were made to relieve pressure areas gently and regularly. A second pleural effusion required drainage from the left side from which 35 mls o f clear fluid was drained. Despite remaining critically ill Jane began to improve over the next 24 hours. Once muscle relaxants were stopped Jane showed a good cough reflex, opened her eyes in response to noise and pupil reaction to light was brisk. It was decided to tentatively wean her from inotropic support. As Jane remained very odematous fluid was removed via the C W H D I = circuit beginning with 10 mls/hr and increasing to 30 mls/hr as tolerated. Total parental nutrition (TPN) was c o m -

Nursing perspectivesin meningococcaldisease 93 menced in a effort to provide adequate protein, glucose and lipid supply to aid healing and guard against excessive weight loss and muscle wasting. Ventilatory support was reduced as Jane began to make a better inspiratory effort. Jane's central colour improved and her skin lesions generally appeared to be shrinking, becoming pinker around the edges. Dark necrotic areas o f skin on her left shoulder and the front o f her thighs remained areas o f concern. Some blisters occurred on these lesions and dry sterile dressings were applied. The circulation to both hands and feet remained adequate with pulses present. Emulsifying ointment was applied to dry areas and passive limb exercises carried out. Protein C infusion was discontinued after 7 days as her protein C level had reached 128% while Heparin continued to be infnsed as part o f the C V V H D F circuit. Jane continued to show signs o f improvement and she was n o w alert, fixing and following objects while showing increased tolerance to handling. Her vital signs were n o w stable despite reduced inotropic support. A sudden increase in her W C C on day 9 necessitated a full 'septic screen' and increased antibiotic cover was given. (Note: haemofiltration can mask a pyrexia). The femoral central line was removed as it was thought to be the possible source o f infection. Thankfully Jane responded well to treatment. Haemofihration was discontinued on day 10 as her urinary output was n o w adequate and her urea and electrolyte levels were satisfactory. Her vas-cath was removed. Jane was extubated and given nasal oxygen and, despite mild tachypneoa, delighted everyone by smiling. Chest X-ray showed some pulmonary oedema and oral Frusemide was ordered to correct this. Her T P N was gradually reduced as she tolerated increased volumes o f enteral feeds. Jane was transferred to an infant ward on day 12. It was thought that some skin grafting would be necessary to her left shoulder in the future. She continued to progress well generally but had occasional spikes in temperature which caused concern. Since no focus for infection had been found despite echocardiogram, full bone scan and C T scan, the pyrexias were thought to be due to an autoimmune response to her previous infection. A sudden onset o f reduced circulation to her left foot further complicated Jane's recovery. Doppler studies revealed a partial occlusion o f her left popiteal artery. Jane was readmitted to I C U where a Heparin infusion at 20 u n i t s / k g / h r was commenced. A diagnosis o£ autoimmune vasculitis was made and IV Sandoglobin was c o m menced at 2 grins/hr. Acetylsalicylic acid was also given orally. Perfusion to her foot gradually improved and the heparin infusion was

gradually discontinued over 48 hours. Leaving the unit for the second time, Jane looked w o n derful and despite some residual scarring on her cheeks, left shoulder and thighs, she was bright and responsive. She was discharged home soon after this, returning to outpatients with her parents who reported normal development, energy levels and an insatiable interest in her surroundings.

DISCUSSION Meningococcal sepsis is, therefore, a devastating and life-threatening condition. The speed with which it is diagnosed and treated is vital in order to prevent death and reduce long-term complications. Management must centre on correcting existing problems such as hypovolaemia, hypotension, acidosis, DIC, while recognizing the development o f any pulmonary, renal, neurological or skin complications.

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FLUID RESUSCITATION Hypovotaemia exists because o f severe capillary leak. Consequently early fluid resuscitation is essential to prevent a serious loss o f the patient's intravascular volume. T w o large IV cannuatae must be sited immediately and Albumin 4.5% is commenced at 20 mis/kg bolus stat. and repeated as necessary. Crystalloid solution i.e. Ringer's solution, is also commenced at 100% maintenance. Fresh frozen plasma is reserved for patients with a severe coagulapathy. Hypotension is thought to be as a result o f hypovolaemia and vasodilatation and the depressing effect o£ the inflammatory response on the myocardium of the heart. Inotropes and vasoactive agents may be necessary to maximize cardiac output and peripheral perfusion. Dopamine and, if necessary, Adrenaline (both naturally occurring catecholamines) infusions are started to increase cardiac output and myocardial contracriliW, while Sodium Nitroprusside (a potent vasodilator) may be commenced to counteract the intense vasoconstriction associated with high doses ofinotropic drugs. Elective intubation is performed and assisted ventilation initiated to ensure adequate oxygenation and also correct the existing metabolic acidosis.

ANTI-COAGULANT

THERAPY

Disseminated intravascular coagulation leads to a sudden drop in the body's natural anticoagulant

94 Intensive and Critical Care Nursing levels Protein C, Protein S and Antithrombin III.) which causes diffuse deposition of fibrin strands in the small vessels resulting in poor tissue perfusion and subsequently skin necrosis and organ dysfunction. Smith et al (1998) suggest that severe acquired protein C deficiency is linked to limb amputation, skin grafting, organ failure and a sizeable mortality. Normal protein C levels are between 0.7-1.2 iu/ml or 80-150% of control. It is interesting to note that children less than 4 years have lower protein C levels than adults, which may explain the often prompt development of purpura and necrotic skin lesions in this age-group (Kennedy & Duncan 1996). Early administration of human activated protein C concentrate IV, i.e. within 4 hours of admission, where protein C levels are decreased or if pupuric skin lesions are evolving, is thought to return levels to normal as well as increase fibrinogen levels and decrease D.Dimers levels. Activated protein C when administered reverses D I C as it slows down excess thrombin formation by inactivating factors Va and VIIa. It also has profibrinolytic effects which help dissolve excess thrombi. In addition, it is thought to reduce the inflammatory response in the body. The reason for this is unknown. Often protein C levels return to normal within 24 hours of initiating therapy. Treatment with protein C in concentrated form avoids the need to give large volumes of FFP (Gerson et al 1993). To date no adverse reactions to protein C have been documented (Smith et al 1998, Rivard et at 1995, Rintalla et al 1996). The necessity of Heparin therapy in meningococcal sepsis is controversial. Kuppermann et al (1994) in a retrospective study o f patients with meningococcal purpura fulminans, found that the patients who were commenced on heparin therapy early had reduced incidence o f digit necrosis and amputation. Heparin is thought to be of benefit as it binds with Antithrombin IIl and increases the anticoagulant activity of antithrombin III significantly, which inhibits thrombin and thereby prevents coagulation. If antithrombin III levels are low, which is c o m m o n with meningococcaemia, the role of heparin is questionable (Kennedy & Durcan 1996).

o f CVVHF initially developed by Kramer et al (1977) has evolved to be a safe, effective method o f removing excess fluid and waste products from the critically ill patient. The value of this therapy in blood purifcation remains under debate, it is thought that some clearance o f cytokines may occur helping to control the body's response to endotoxin release. Gomez et al (1990) have demonstrated in animal studies effective removal of myocardial depressant factors in gram-negative sepsis. Using a pump system to establish the blood circuit, venous blood is passed through a filter with a synthetic semi-permeable membrane. The addition of a dialysis solution in a contracurrent direction through the filter enhances solute removal by diffusion, thus increasing the effectiveness of this therapy in a highly ureamic patient. Blood re-enters the patient via the venous catheter along with the appropriate replacement fluid. The continuous nature of this therapy allows for greater cardiovascular stability, by avoiding the large fluid shifts of conventional dialysis with the subsequent fluctuations in blood pressure. There is a 50% reduction in hypotensive episodes compared with haemodialysis (Henderson 1987). As with any extracorporal blood circuits, there are many challenges facing the I C U staff caring for a patient having CVVHF; maintaining line patency and adequate body temperature as well as minimizing the risk o f blood loss and sepsis are vitally important. Monitoring patient fluid status continuously involves recording all fluid lost through the filter as well as fluid added. These figures need to be considered in conjunction with overall fluid balance. Close attention to urea and electrolytes levels helps to guide necessary alteration to the therapy which may include the addition o f various electrolyte supplements to the replacement and/or dialysis fluid. For this therapy to be safe for a paediatric patient great care is needed in selecting appropriate lines and filter size. Accurate patient blood volume calculations as well as priming volumes o f the circuit need to be considered. C V V H F is an invasive therapy for it to be effective and safe, skilled personnel are required to manage the system.

A N T I B I O T I C THERAPY HAEMOFILTRATION Profound shock associated with meningococcal sepsis may lead to a significant reduction in renal perfusion, this coupled with a high metabolic rate in a toxic patient may result in rapidly rising levels o f waste products. The use

Once a diagnosis of meningococcal disease is suspected in a patient, a broad spectrum third generation cephalosporin, i.e. Cefotaximine, should be administered without delay because of its good penetration of" the blood-brain barrier until a sensitivity is available. Neisseria

Nursing perspectivesin meningococcaldisease 95

meningitidis is usually sensitive to Pencillin, a l t h o u g h e v i d e n c e exists to suggest that the o r g a n i s m is less sensitive to Perficillin t h a n it was previously ( K e n n e d y & D u n c a n 1996). T h e role o f steroids in m e n i n g o c o c c a l disease continues to be the source o f m u c h debate. It is t h o u g h t they i m p r o v e a u d i o l o ~ c a l a n d neurological o u t c o m e in m e n i n g o c o c c a l m e n i n N t u s . It is r e c o m m e n d e d to administer D e x a m e t h o s o n e IV prior to giving the first dose o f antibiotics, to p r e v e n t an increase in c y t o k i n e levels seen w i t h antibio t i c - i n d u c e d bacteriolysis. Close contacts o f t h e p a t i e n t w i t h t n e n i n g o coccal disease, i.e. family m e m b e r s , classmates a n d friends are advised to take R i f a m p i c i n orally w i t h i n 24 h o u r s for 2 days as c h e m o p r o phylaxis. It p r e v e n t s s e c o n d a r y spread o f this c o n d i t i o n as it eradicates Neisseria M e n i n g i t i d i s f r o m t h e n a s o p h a r y n x . It is w o r t h n o t i n g that t h e r e h a v e b e e n r e p o r t e d cases o f R i f a m p i c i n resistant meningococci. In these cases, C e f t r i a x o n e or c i p r o f l o x i n are r e c o m m e n d e d (Yagupsky et al 1993).

CONCLUSION As t h e case study demonstrates, c h i l d r e n w i t h meningocaemia may present with vague nonspecific s y m p t o m s t h e n s u d d e n l y d e t e r i o r a t e w i t h i n hours. D u r i n g r e c e n t years early i d e n t i fication o f h i g h risk patients has b e c o m e a priority. Critical care nurses face the c o n t i n u i n g challenge o f w o r k i n g towards i m p r o v i n g t h e m a n a g e m e n t o f these c h i l d r e n so that m o r b i d ity m a y b e f u r t h e r r e d u c e d . In particular, it is h o p e d to p r e v e n t skin damage, hopefully, t h e r e b y r e m o v i n g the n e e d for skin grafting in future years. The continuing education and training of I C U s t a f f i n t h e specialist skills r e q u i r e d to care for such patients is also a n o n - g o i n g challenge. It can h e a stressful a n d d e m a n d i n g t i m e for a critical care n u r s e w h o w h i l e m a n a ~ n g t h e h i g h l y t e c h n i c a l aspects o f t h e t h e r a p y also a t t e m p t s to offer the family i n f o r m a t i o n updates a n d e m o t i o n a l support. H o w e v e r , w e find that t h e r e w a r d o f seeing these c h i l d r e n leave t h e u n i t able to smile a n d w a v e g o o d b y e p r o v e s a powerful motivator. REFERENCES

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continue. Professional Care of Mother & Child, 3(8): 222-223 Carno M A 1994 Meningococcaemia: P..ecognizing and reducing complications in paediatric patients. AACN; Clinical Issues, 5(3): 278-288 Gerson W T, Dickerman J D, Bovii1E G, Golden E 1993 Severe acquired Protein C defciency in Purpura Fulminans associated with disseminated intravascular coagulation: Treatment with Protein C concentrate, Pediatrics, 91(2): 418-421 Gomez A, Wang R, Unruh H 1990 Haemofiltration reverses left ventricular dysfunction during sepsis in dogs. Anaesthesiology, 73:671-685 Henderson L 1987 Haemofiltration. The Kidney 20 (6) : 25 Kennedy N J, Duncan A W 1996 Acute meningococcaemia: Recent advances in management with particular reference to children. Anaesthesia & Intensive Care, 24( 2): 197-216 Kramer P, Wigger W, PdegerJ, Matthaei D & Scheler F 1977 Arteriovenous haemofiltration: A new and simple method for the treatment ofoverhydrated patients resistant to diuretics. IZdin.Wochenscher, ] (55): 1121-1122 Kuppermann N, Inkelis S, Saladino R 1994 The role of heparin in the prevention of extremitT and digit necrosis in meningococcal purpura fulminans. Pediatric Infectious Disease Journal, 13(10): 867-887 Nadal S, Habibi P, Levin M 1995 Management of meningococcal septicaemia. Care of the Critically Ill, / 19(i): 33-38 Rintalla E, Seppala O P, Kotilainen P, Rasi V,(1996 Protein C in the treatment of coagulapathy in m eningococcal disease. Lancet, 347 (9017): 1767 Rivard G E, David M, Farrell C, Schwarz H P 1995 Treatment of purpura fulminans in meningococcaemia with Protein C concentrate. The Journal of Pedriatrics, 126(4): 646 652 Smith O P, White B, P..afferty M e t al 1998 Successful use of Protein C concentrate, Heparin and haemodiafiltration in meningococcal induced purpura fulminans. Lancet. 350(9091) : 1590-1593 Yag~psy P, Ashkenazi S, Block C 1993 Rifampican resistant meningococci causing invasive disease and failure ofchemoprophylaxis. Lancet, 341(8853) : 1152-1153

FURTHER R E A D I N G

Bellomo P 1995 Continous hemofiltration as a blood purification in sepsis. New horizons, 3 (4) : 732-737 Best C, Walsh J, SinclalrJ, Beattie J 1996 Early hemodiafiltration in meningococcal septicemia. The Lancet 347(8995): 202 Hagland M 1994 Making sense ofcontinous renal replacement therapy. Nursing times 90(40): 37-39 Higley K 1996 Continous atriovenous hemofiltration: A case study. Critical care nurse 16 (5): 37-43 Kiely M A 1984 Coutinous Atriovenous Hemofiltration. Critical care nurse. 4 (4): 3943 Lieberman K V 1987 Continuous arteriovenous haemofiltration in children. Pediatric Nephrology, 1: 330-338 Thomson A P J, Sillsj A, Hart C A 1991 Validation of the Glasgow meningoceal septicemia prognostic score: A ten year retrospective survey. Critical care medicine 19 (1): 26-30 Williams V, Perldns L 1984 Continous ukrafiltration: A new ICU procedure for the treatment of fluid overload. Critical care nurse 4 (4): 44 49 Woodrow P 1993 Resource package: haemofiltration. Intensive and critical care nursing 9 (2): 95-107