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Case study of a patient with terminal lung disease awaiting a single lung transplant

Margaret McCowan, Jane Moore

[Abstract]
[Background]
[Conclusion]
[References]

Abstract

Margaret McCowan is the Clinical Co-ordinator in the Respiratory Medicine Ward, and Jane Moore is the Clinical Co-ordinator in the Coronary Care Unit, at St. Vincent's Hospital, Sydney.

Two disease profiles which are known to progress to the stage where a lung transplant is required are Pulmonary Lymphangioleiomyomatosis and Pulmonary Sarcoidosis. A brief description of these diseases follows.

Pulmonary Lymphangioleiomyomatosis.

This very rare condition of young women is characterized by progressive dyspnoea, haemoptysis, recurrent pneuinothoraces and chylous effusions. Pathologically, there are widespread proliferations of immature smooth muscle throughout the peribronchial, perivascular and perilymphatic regions of the lung. The cause of the disease is unknown, but may be linked to an endocrine dysfunction. This possible causal link is made because of the occurrence of this disease in women of childbearing age only.

Diagnosis is made by chest x-ray (CXR), CT scan and lung biopsy. CXR will show hyperinflated lungs with fine linear and nodular densities that are more prominent in the lower lung fields. Lung biopsy will show nodular interstitial proliferation of smooth muscle in the lobtilar septs, pleura, walls of alveoli, small bronchi and bronchioles. Hormone receptors for progesterone or oestrogen are also sought to help determine the treatment of the disease.

Prognosis is poor. Following the time of initial diagnosis, the patient is expected to survive 3 - 10 years. Treatment is limited, but bilateral oophorectomy has been useful in patients with no hormone receptor found in the lung. If receptors are identified, specific hormone treatment has proved in some cases to be helpful. Single lung transplant is also an option, perhaps the only option in this progressive disease.

Murray, I.F. and Nadel, J.A., (1988: 1264).

Pulmonary Sarcoidosis

Sarcoidosis is a multisystemic granulomatous disorder which characteristically produces lymphadenopathy, pulmonary infiltration and skeletal, liver, eye or skin lesions. It is most common in young adults (24-40 years), and the incidence in women is twice that in men.

The cause remains unknown, although the following links have been suggested:

hypersensitivity response (possibly due to a T-cell imbalance) to such agents as atypical mycobacteria, fungi and pine pollen;
genetic predisposition;
immune system abnormalities;
exposure to chemicals such as zirconium or beryllium.

Diagnosis is made on the basis of results of CXR and lung biopsy. CXR shows bilateral hilar and right paratracheal adenopathy with or without diffuse interstitial infiltrates; occasionally, large nodular lesions in lung parenchyma. Lung (and or lymph node and skin) biopsy shows non-caseating granulomas and negative cultures for mycobacteria and fungi.

Treatment is steroids from one to two years to lifelong, and lung transplantation (for end stage disease).

D'Angelo, H., Gyetvan, C. and McCann, S. Eds, (1989: 114).

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Background

The following is a case study of the history of a patient awaiting single lung transplant.

Mary, a 33 year old registered nurse, is married with three children. Mary was a social smoker (mostly on weekends) until the age of 23 years. Her only known significant family history is that she has a mother with Allergic Lung Disease.

Mary has had a 13 year history of increasing shortness of breath, commencing in 1978, when she was diagnosed as having asthma due to the family history. At this time Mary had a normal CXR.

Over the next few years Mary maintained good health. While pregnant, in 1982 and 1984, Mary experienced increasing shortness of breath. This was attributed to asthma and pregnancy. In 1985, her CXR showed a local area of bronchiectasis and she was subsequently commenced on Prednisolone (oral steroid).

At this time Mary experienced a marked improvement in her symptoms. In 1988, during her third pregnancy, Mary again experienced shortness of breath. This was again attributed to asthma and pregnancy. Her health deteriorated and she was less able to care for her family because of increasing shortness of breath, fatigue, weight loss and nausea, and in 1989 the terminal diagnosis of pulmonary lymphangioleiomyomatosis was made.

This diagnosis was based on Mary's history and CXR. Definitive lung biopsy was not attempted due to the risk of pneumothorax because of the presence of multiple bullae (air filled pockets in the pleural cavity) on CXR. At this time, steroids were discontinued and replaced by hormonal therapy (Provera). In 1990 Mary was assessed for a heart/lung transplant and was put on the active waiting list.

In February 1991, Mary presented at St. Vincent's Hospital in respiratory distress, unable to care for herself and family. On admission, the results of her arterial blood gases (A.B.G.'s), shown below (Table 1), reveal the presence of chronic respiratory acidosis. This is indicated by the elevated pCO₂, normal pH and elevated HCO₃. Mary was also hypoxic, evidenced by the low pO₂.

Normal Values
pH	7.38-7.42
pCO₂	38 - 42 mmHg
pO₂	95 - 100 mmHg
HCO₃	23 - 25 mEq/L
SO₂	97
Mary's values; February, 1991
pH	7.37
pCO₂	56 mmHg
pO₂	69 mmHg
HCO₃	32 mEq/L
S0₂	92

Table 1: Arterial blood gases, February, 1991

Mary initially depended on the nursing staff to give her minimal assistance only with daily living activities, but she soon deteriorated and required direct nursing care for at least one to two hours each morning. During her stay in hospital, Mary each morning attended the gym where, under the guidance of the Transplant Physiotherapist, she performed gentle arm movements to build up her respiratory muscles. She felt that missing her gym routine for just one day would mean that she had "given up". Her other exercise was the daily mandatory lap of the ward corridor, which could take her up to one hour to complete.

During her lengthy hospitalisation, Mary's husband, who was working in full time employment, was unbelievably supportive. He maintained the children at the family home, calling on his mother and friends, who organised a roster system, for assistance with collection and care of the children. He would visit Mary twice daily; in the morning prior to going to work, then, in the evening of each day, would bring all three children to visit their mother. The eldest son, who was nine years old was aware of the severity of Mary's illness. The other two children were aware only that their mother "lived in hospital" and was "not like other mothers".

Mary was on dependent on 35% oxygen for 24 hours/day. Whilst eating, she required 10 litres of oxygen via nasal prongs. Despite the oxygen therapy, Mary suffered from severe headaches and nausea. This is due to the elevated PCO₂, which at times, reaching as high as 88 (normal 38-42 mmHg). In May, Mary was transferred to Royal Prince Alfred Hospital for sleep studies, and returned to St. Vincent's Hospital in June, on nasal ventilation at night.

Mary's weight had decreased to 33 kgs, and this weight loss continued despite supplements of Ensure (a balanced liquid feeding supplement). At this time total parenteral nutrition (T.P.N) was considered but rejected after much discussion due to Mary's inability to lie flat for the central line insertion procedure, and also because of the added risk of infection and pneumothorax. The option taken to increase Mary's weight was to feed her via a gastrostomy tube. Mary was given Pulmocare (a balanced liquid feed, similar to Ensure) for 16 hours/day to maintain her weight for transplant. In July, Mary's A.B.G.'s were again taken. Results on spontaneous breathing had further deteriorated, as shown below (Table 2).

Normal Values		Spontaneous breathing	Nasal ventilation
pH	7.37	7.29	7.39
pCO₂	56 mmHg	88mmHg	62mmHg
pO₂	69 mmHg	58mmHg	103mmHg
HCO₃	32 mEq/L	42mEq/L	37mEq/L
B.E.	+1 to -1	+12	+10

Table 2: Arterial blood gases, July, 1991

As can be seen, Mary's pO₂ improved greatly on ventilation as opposed to spontaneous breathing, and her PCO₂ level was lowered. From the July A.B.G's shown above, a decision was made that Mary required more rest time using the nasal ventilation, so she had this rest period for about two hours each afternoon.

In August Mary's weight had increased to 37 kgs. Throughout the preceding three months, both staff and Mary despaired that the transplant would eventuate. The absence of a suitable donor and her continuing deterioration in health meant that Mary was preparing for what seemed to be an inevitable early death, without the chance at her only option for life, a healthy lung transplant.

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Conclusion

Finally on 6th September, 1991 (her birthday), she had her transplant; 1 year after placement on the waiting list. Mary had an uneventful recovery and is now doing well, having made a month long overseas trip with her family, moved house, attended the Transplant Games in Bathurst in 1992 and contributed much to the painting of the new house!

Post transplant lung pathology showed that Mary did not suffer from Pulmonary Lymphangioleiomyomatosis, but Pulmonary Sarcoidosis, for which the nursing care is different only in the respect that continuing steroid therapy is indicated (unless awaiting transplantation).

The following care plan illustrates the nursing care given to Mary, prior to transplantation.

DIAGNOSIS	GOAL	NURSING ACTION	EVALUATION
Breathlessness Hypoxaemia Impaired gas exchange	To ensure no distress due to breathlessness To maintain 02 Saturation above 90% To ensure no signs of cyanosis	Allow patient to be in most comfortable position	Patient nursed in upright position supported by pillows
Potential for nutritional deficit, due to nausea breathlessness	To ensure adequate dietary intake, shown by stable or increased weight on weekly weighs	Refer to Dietitian Offer small, easy to eat high energy meals Encourage high energy drinks Give anti-emetics p.r.n. as ordered Care of gastrostomy feeding tube Give 02 at 10L/min. via nasal prongs whilst eating	Required supplemented gastrostomy feeds 16hr day to meet daily requirement Weight gain demonstrated
Potential for fluid imbalance	To provide adequate fluid status To ensure good skin turgor To ensure absence of dry cough	Encourage oral fluids to 2 litre/day	Patient hydration satisfactory, moist mouth, skin turgor No dry cough
Potential for constipation	To prevent constipation	Chart frequency of bowel movements Provide high fibre diet actions Provide adequate fluid intake Give aperients if indicated	Patient having daily bowel actions
Potential for skin breakdown due to: bed rest hypoxia	To maintain skin integrity	Provide 2/24 hourly position changes	Patient's skin intact and able to turn self
Social isolation	To maintain contact with family and friends To maintain awareness of events occurring outside the Hospital To allow the patient expression of her feelings	Continue 02 therapy Encourage frequent short visits with family and friends Encourage an interest in current affairs and /or world events Allow an avenue for the patient to express her feelings	Accessible contact with family and friends T.V. and newspaper accessibility
Inability to attend to own needs, due to: breathlessness hypoxia	To encourage the patient to continue with some aspects of daily care, by optimal utilisation of her energy	Provide continuous 02 therapy Ensure adequate rest periods Encourage patient contributions to decision- making Refer patient to Physiotherapy Offer positive reassurance Provide assistance as required and directed by patient Provide adequate nutrition/ gastrostomy feeding 16/24 hours	02 therapy 50% 24 hours per day 2 hours rest in afternoon Assisted shower and hygiene requirements
Alteration of self image, due to weight loss altered chest shape	To encourage the patient to accept changes and talk about them freely	Encourage communication Encourage with diet Assist with physiotherapy Administer muscle relaxants, e.g. Valium to reduce overuse of accessory muscles)	See Transplant Co-ordinator frequently Time to express fears and anxiety Talk with family and professionals about changes
Potential for spiritual distress	To provide awareness of the availability of the Pastoral Care Services	Contact Pastoral Care at patient request	Pastoral Care available to see twice weekly or more often if required
Pre-Transplant work-up	To ensure that patient consent is informed To ensure that the patient understands the principles of Lung Transplantation To provide education in the requirements for remaining on the active Transplant list To provide education in her role post-transplant To provide education on the risks associated with Transplant	Encourage patient to ask questions regarding the operation Instruct/ educate the patient in what she must do, e.g. walk the corridor/attend the gym, in order to remain on the list Inform the patient about her responsibilities post-operatively, such as: long-term medications required post transplant chest X-rays frequent brochoscopies, and peak flow recordings (and action to take if decreased) Advise patient that transplants are not successful in every case	Patient has adequate knowledge about pre- and post- Transplantation

Figure 1. Summary Nursing Care Plan for a Patient with Terminal Lung Disease Awaiting a Single Lung Transplant.

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References

Murray, J.F. and Nadel, J.A., (1988), Textbook of Respiratory Medicine, W.B. Saunders, Philadelphia.
D'Angelo, H.H., Gyetvan, C and McCann, S,. Editors, (1989), Respiratory Handbook, Springhouse Corporation, Springhouse, Pennsylvania.

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