Nursing Diagnoses	Specifications	Aim	Nursing Interventions	Evaluation
Deficient Fluid Volume: Fluid loss due to loss of appetite, malnutrition and diarrhea	Poor oral intake, dryness of skin and mucous membranes, loss of body weight	Regulation of fluid intake in accordance with age and metabolic requirements. Regulation of signs and symptoms of dehydration.	- Intake and output was monitored. Noted incerased and decreased urinary output. - Infusion rate of parenteral fluids was monitored closely. - The patient was weighed daily. - Vital signs was monitored regularly. - Hydration and dehydration status were evaluated (fontanelle, mucous membrane, skin turgor, tears). - Laboratory findings and blood gas tests were checked.	The signs of dehydration tend to decrease, the oral mucous membrane is slightly moist.
Impaired Oral Mucous Membrane: Due to lack of fluid volume, common oral candida albicans, decayed teeth and O₂ support with reservoir mask.	Dryness and redness of the oral mucosa, common oral candida albicans	Ensuring the integrity and moistness of the oral mucosa.	- The infant’s oral mucous membrane was evaluated daily. - Redness in the mouth and sores on the tongue, tissue integrity, moistness were observed. - Moisturizers were applied to the rim and lips to prevent chapping and drying. - Oral care was applied with sodium bicarbonate diluted with physiological saline solution every six hours. - Mikostatin was administered as ordered by the doctor. - The mother was informed about oral care and its importance. - The child was hydrated.	There is still oral candida albicans and mild redness. The situation continues.
Imbalanced Nutrition: Less Than Body Requirements: Associated with anorexia and oral candida albicans	Losing weight, eating less than the recommended daily amount and switching to parenteral nutrition	Reaching the ideal nutritional level.	- The child was weighed every morning at the same time and in the same clothes. - Intake and output was monitored. Noted incerased and decreased urinary output. - Head circumference, height and weight percentile values were followed and recorded. - The child underwent central venous catheterization and TPN was started. - In addition, Peptamer food was added to the diet. - The amount of food given was recorded regularly. - Laboratory results were followed.	TPN and parenteral nutrition were discontinued on the 35th day of hospitalization. She continues to be fed with Peptamer formula.
Delayed Growth And Development: Immunodeficiency associated with adrenal insufficiency	Body weight and height below the expected percentile (height, body weight and head circumference below the 3% percentile)	Ensuring the child’s age-appropriate growth and development.	- A nutrition plan was created according to the needs of the child. - Height, body weight and percentile values were checked at regularly. - Its care is planned in the same time period. Frequent rest periods were planned.	Height, body weight and head circumference are below the 3% percentile. There is developmental delay. The child does not grow and develop in accordance with his age.
İmpaired Tissue (Skin) İntegrity: Because of edema, phimosis operation and invasive procedures	Damaged skin surface	Protection of skin integrity.	- Central venous catheter site and operation area were checked daily and wound care was performed. - The probe was changed every 3 - 6 hours when the patient had a vital monitor probe. - Unnecessary invasive intervention is avoided. - The position of the child was changed every 6 hours.	The patient does not have erythema or redness that disrupts skin integrity.
Fear: Due to the effort to cope with the rare and incurable disease, lack of knowledge, the abundance of painful interventions and prolonged hospitalization.	Child cries during treatments or sees healthcare worker and parent seems nervous	Ensuring the child’s adaptation to the healthcare worker and the hospital environment. Informing parents and supporting their participation in their child’s care.	- A calm and safe environment was provided for the child and family. - Parent was informed before all procedures. - It was ensured that the parent was with the child during the procedure. - It was tried to ensure that the child was taken care of by the same nurse as much as possible. - It is ensured that the child’s favorite toys are with him. - Parents were encouraged to voice their fears and concerns.	The child’s crying time during interventions decreased. It has been observed that the family trusts the health workers and exhibits more adaptive behaviors.
Diarrhea	Frequent (at least 3 times a day), watery and misshapen stools, increased intestinal sounds, continued antibiotic therapy	Normal consistency and number of stools.	- The child underwent central venous catheterization and TPN was started. In addition, Peptamer was added to the diet. - The amount of food given was recorded regularly. - Probiotic and Zinco syrup added to the treatment. - The child was hydrated. - The child was weighed every morning at the same time and in the same clothes. - Hydration and dehydration status were evaluated (fontanelle, mucous membrane, skin turgor, teardrop). - Laboratory findings and blood gas tests were checked regularly.	Diarrhea continues.
Acute Pain: Because of the painful procedures such as CVP and IV catheter insertion, endoscopy, colonoscopy, and diarrhea	Score of visual pain scale is 3, anxious look and groaning	To reduce or eliminate existing pain.	- Vital signs were checked regularly - Pain was evaluated with visual analog scale (VAS) and recorded. - Nonverbal pain behaviors were observed in the child. - Pain was tried to be reduced by using therapeutic techniques such as distracting the child’s attention and keeping favorite toys with him. - When necessary, analgesics were administered.	The child’s vital signs are stable. VAS pain score was evaluated as 2. The child continues to look anxious but there is no groaning.
Fatigue: Because of the immunodeficiency, adrenal insufficiency and hospitalization and persistent diarrhea	Weakness, constant fatigue, hypotonia, inability to walk	Relieving fatigue.	- The amount of light, heat and sound of the environment has been adjusted. - Unnecessary invasive procedures were avoided. - Interventions were planned to provide resting periods for the child.	Weakness, fatigue, hypotonia continues in the child.
Risk for Secondary Infection: Associated with presence of central venous catheter, gland dermatitis, surgical operation and prolonged hospitalization	CRP: 23 mg/dl (<0.5%)	Preventing the child from having a secondary infection other than the current infection.	- He was checked in terms of signs and symptoms (fever, chills, local or general skin discoloration) that may develop due to infection. - Hands were washed before and after each contact with the patient. Aseptic technique rules were followed in all invasive procedures. - Visitor restriction applied. - The family was informed about daily hygiene and diaper dermatitis. - Unnecessary invasive procedures were avoided. - The patient was started on topical treatment due to diaper dermatitis. (gentian violet)	The child has an infection. Intravenous antibiotic therapy is administered. No secondary infection was observed in the patient.CRP: 8.88 mg/dl (<0.5%)
Risk For Bleeding: INR: 1.33 and decrease in platelet level	Risky value of laboratory tests showing signs of bleeding	Absence of signs and symptoms of bleeding.	- Bleeding signs and symptoms were checked. - Vital signs were measured at regular intervals. - Laboratory findings were checked for Pt, Aptt, Inr and platelet count. Platelet transfusion was performed according to the results. - Unnecessary invasive procedures were avoided and the interventions were done gently. - The patient was protected against bumps and falls. - The caregiver parents were informed about the symptoms of bleeding and ways to protect themselves. - Kvit was applied at the order of the physician.	No signs of bleeding were observed in the child.
Risk of electrolyte ımbalances: Associated with resistant diarrhea and malnutrition	Caused by resistant diarrhea and malnutrition	Fluid electrolyte values have to be within normal limits, fluid intake and output have to be in balance	- Vital signs were checked regularly. - The daily fluid balance was checked. - Serum electrolyte levels were checked regularly.	For now the fluid intake and output are in balance.