Nursing Care on Improving Postoperative Condition in CIPA Patients Combined with Charcot Spine

Background Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, hereditary, sensory autonomic neuropathy. There are few reports on CIPA combined with Charcot spine. Operation is a most effective method for CIPA patients who combine with Charcot spine, but those patients are easy to get hyperthermia and internal xation failure after operation. Patients’ decit teeth also make nutrition management dicult. In order to help patients to avoid these problems, we designed a series of preoperative and postoperative nursing means: 1. Rectal temperature monitoring and Temperature control 2. Use new posture management 3. Assess the nutritional status of patients and make a diet plan. In this study, we aimed to evaluate the effect of these nursing means. Methods retrospectively to examine the effect of the means.


Abstract
Background Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, hereditary, sensory autonomic neuropathy. There are few reports on CIPA combined with Charcot spine. Operation is a most effective method for CIPA patients who combine with Charcot spine, but those patients are easy to get hyperthermia and internal xation failure after operation. Patients' de cit teeth also make nutrition management di cult. In order to help patients to avoid these problems, we designed a series of preoperative and postoperative nursing means: 1. Rectal temperature monitoring and Temperature control 2. Use new posture management 3. Assess the nutritional status of patients and make a diet plan. In this study, we aimed to evaluate the effect of these nursing means.

Methods
We retrospectively analysed the records of 3 CIPA patients combined with Charcot spine who accepted our nursing means to examine the effect of the nursing means.

Results
Rectal thermometry was more accurate than axillary thermometry. Physical cooling prevented hyperthermia for CIPA patients after operation. None of 3 patients had internal xation failure. The BMI of one patient was only 14.0(malnutrition) before surgery, through nutritional intervention, all 3 patients' serum albumin levels returned to normal before discharge from the hospital.

Conclusions
In summary, this study has made a lot of suggestions that can improve the prognosis of CIPA patients with Charcot spine.

Background
Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, hereditary, sensory autonomic neuropathy, characterized by absence of reaction to painful stimuli [1], inability to sweat and mental retardation [2,3], it also can induce the destruction of the joint -neuropathic arthropathy (Charcot joint, Charcot spine). There are few reports associated with CIPA combined with the Charcot spine [4], and there is no standard treatment [5,6]. CIPA is a really dangerous disease, and most patients cannot live over 25 years [7]. Operation is a most effective and reasonable method for those patients combined; however, there are also many challenges for patients to have the operation. For example, besides sensory autonomic neuropathy, patients are also insensitive to pharmaceutical cooling [8], they are easy to get hyperthermia or convulsion, or they can die of secondary infection caused by hyperthermia. The absence of reaction to painful stimuli is easy to cause internal xation failure due to hypermobility. CIPA patients bleed more in 360 ° fusion operation, and they also have de cit teeth, which makes nutrition management di cult. We designed a series of nursing means to improve the prognosis of CIPA patients. The goals of this study are as follows: 1. to evaluate the effect of body position management. 2. to evaluate the effect of body temperature monitoring and control. 3. to assess the nutritional status of patients and make a diet plan.

Methods
Three CIPA patients with Charcot spine who received surgeries in our hospital from January 2010 to September 2019 were studied. In these 3 patients, besides lumbar vertebral injuries, CIPA can also do damage to the ankle, elbow, knee, and hip joint. The general information of the patients is shown in Table 1. The Mean operation age of these patients was 19.67 ± 5.03 (15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25). The average length of stay (ALOS) was 24.67 ± 6.03 days (19-31 days). The Median follow-up time was 14 months (6-124 months). The nursing measures is described in Table 2. Temperature monitoring: Rectal thermometry is recognized as the most accurate surrogate of body temperature, especially for hyperthermia patients [9]. We decided to monitor rectal temperature for these patients, compared the result of rectal temperature with axillary temperature. For rectal temperature monitoring, common ECG monitors (including ECG monitors of general company, Mindray Medical International, Philips, Siemens, and GE) can be used. It is necessary to prepare the temperature probe conversion line, which is suitable for the TEMP probe interface of the monitor in advance. A probe needs to be inserted into the rectum via the anus and kept in the patient's rectum. In order to ensure patients can tolerate and cooperate with rectal temperature monitoring after the operation: do a pre-operative rectal temperature monitoring for patients to assess their compliance and increase the likelihood of their postoperative cooperation. Measurement method: lubricate the probe and then put it into the intestinal cavity according to the clinical procedure. Caution: If resistance is felt while inserting the probe, stop the insertion and pull back the probe slightly. Do not force the probe in because improper placement may damage the patient's cavity mucosa. The probe has a scale, which can be used to determine the depth of probe placement and see whether the probe is falling off or retreating through the exposed scale.
Measure axillary temperature at 2 pm every day before surgery. Measure the rectal temperature and axillary temperature at 6 pm-6 am-2 pm-6 pm every day since one day prior to the operation, to identify hyperthermia in time.

Temperature control
Prepare temperature and humidity instruments for patients, because they can't reduce the temperature by perspiration.
Maintain the ambient temperature at 23 ± 2 ℃. Reduce the patient's cover at the same time. The ice bag is used for physical cooling to avoid hyperthermia after the operation. If the rectal temperature is maintained between 37.5 to 38.5℃, physical cooling is the main way to prevent hyperthermia or convulsion. If the rectal temperature is higher than 38.5 ℃ and the drop in body temperature is not substantial after physical cooling, it is necessary to use aspirin/lysine to lower the hypothalamus temperature set-point, despite the fact that CIPA patients are relatively insensitive to drug cooling.
The main points of nursing 2: Posture management

Bed rest
Preoperative: evaluate the degree of spinal cord compression by imaging and determine whether patients should have bed rest before surgery. One patient in this study had spinal cord compression before surgery, so he was required to lie in bed before the operation. Due to the mental retardation and stubborn character of CIPA patients, he did not understand why he needed to lie in bed before the operation. He insisted on getting up and going to the bathroom. Family members could fully understand the importance of bed rest after the communication and they were required to accompany the patient 24/7. Family members worked with the medical staff to ensure that patient lies in bed before the operation. Avoid further compression of the spinal cord or paralysis caused by activities.
Postoperative: Because of the absence of reaction to painful stimuli, the patient's body position cannot be limited by pain.
Teaching patients how to turn over and sit up can avoid the failure of internal xation caused by excessive activity.

Position change
The postoperative bedtime is relatively long because of the osteoporosis and operation. An X-ray should be taken before the patients get out of bed to make sure that the internal xation is stable.
From lying position to standing position Before going down to the ground, patients should put on the brace in bed rst, and then sit up beside the bed to avoid internal xation loosening or even displacement as their body position changes (see Fig. 1). They should not directly sit up from the supine position. Because at this time the bending force is too large, which can easily cause the loosening of internal xation.
From standing position to lying position when the patients lie down from sitting up, they should be wearing the brace. The brace can be removed after lying down. (see Fig. 2). During the whole process of sitting up and lying down, the brace can disperse the stress, reduce the pressure of internal xation, and minimize the risk of internal xation loosening.

Underground activities
Denervation of the bone can affect the healing. Teriparatide is used to promote the bone formation after the operation. Patients need to come back to the hospital for examination at the rst, third, and sixth months after the operation. The results of the X-ray can be used to determine the time when the brace can be removed. Before the internal xation is completely stable, the brace should be worn when changing the body position.
The main points of nursing 3: Assess the nutritional status of patients and make a diet plan Nrs2002 is used to evaluate the status of patients. CIPA patients always have tooth problems [5,10,11]. Therefore, evaluation of patients' teeth and chewing ability should be done before making the intervention plan. (Fig. 3) Data Collection These patients were followed up by phone, and their outpatient or medical records were reviewed.

Temperature
The changing trend of 3 patient's postoperative rectal and axillary temperature (Fig. 4).
Nutritional status the BMI of one patient was only 14.0(malnutrition) before surgery. Through nutritional intervention, all 3 patients' serum albumin levels returned to normal before discharge from the hospital( Figure 5).

Discussion
1. CIPA is a rare hereditary sensory autonomic neuropathy. It was rst reported by Swanson in 1963 [12]. There were few reports of CIPA in the previous literature. CIPA is more common in men, and all three patients in this study are female.
2. The diagnosis of CIPA is based on pain, temperature test and iodine starch sweating qualitative test [15]. Further diagnosis requires molecular biotechnology to nd out the location of gene mutation. In this study, all 3 cases are investigated by the above methods, and skin biopsies were performed in another hospital, which showed a loss of unmyelinated bers of peripheral nerve, denervation of skin nerve and abnormality of exocrine gland. All the evidence supports the CIPA diagnosis.
3. Rectal thermometry is recognized as the most accurate surrogate of body temperature, especially for hyperthermia patients [9]. CIPA has a great impact on body temperature, so it is necessary to monitor the temperature of patients after operation. The axillary temperature is not accurate when the core body temperature is abnormal. We must pay attention to prevent death due to secondary infection caused by hyperthermia.
CIPA, as a rare sensory autonomic neuropathy, affects sweating and heat dissipation. In this study, we rst tried to use rectal and axillary temperature to re ect the trend of postoperative temperature change. Secondly, we tried to explain the cause of the temperature change. The time course of temperature showed two peaks, on the rst day and 5-7 days after surgery, respectively. The changing trend of rectal and axillary temperature is similar, but the sensitivity of axillary temperature is not as good as that of the rectal temperature. Axillary temperature is easy to cause false-negative results, especially in the early postoperative period. It is suggested that rectal temperature should be used to re ect the temperature changes, especially in the early postoperative period.
The reasons for two peaks: the peak on the rst day after surgery is considered to be related to surgery; the second peak on the 5-7 days after surgery is considered to be a fever following the removal of the drainage tube. CIPA patients' bone healing is slow and poor [14,16,17], so they always get out of bed later than the regular patients. Before the removal of the drainage tube, the patients have to lay in the bed all the time. The surgical trauma is substantial, and there is much bleeding. After the removal of the drainage tube, there will be blood or uid accumulation in the body, which leads to the second peak.
4. CIPA is similar to spinal cord injuries, especially the cervical spinal cord injury, because they all have sensory autonomic neuropathy, which can affect sweating and heat dissipation. Therefore, our study uses physical cooling to prevent hyperthermia caused by spinal cord injury, and it has achieved good results. Although CIPA patients are insensitive to drug cooling, we can still use aspirin/lysine to reduce their temperature by lowering the temperature set-point. It is con rmed that to some extent the method of CIPA postoperative temperature control can be applied the treatment of spinal cord injury.
5. CIPA combined with Charcot spine should have 360 °fusion, postoperative position management is very important.
Vialle reported 9 cases of CIPA with Charcot spine and emphasized the importance of 360 °fusion. He pointed out that due to the lack of pain protection mechanism, the repeated excessive activity of patients is easy to cause the failure of internal xation even in case of a simple posterior fusion [18]. Therefore 360 °fusion was performed in all 3 CIPA patients.
360 °fusion can provide the most powerful stability to prevent the internal xation failure caused by excessive activity. 360 °fusion is important, but postoperative position management is also important. The position management is particularly important in the early postoperative period, especially in the period before the internal fusion is completely stable. Our method that ensure patients always wear braces during the whole process from lying position to standing position and from standing to lying position can disperse the stress of spine.
6. The BMI of one patient was only 14.0(malnutrition) before surgery. Through our nutritional intervention, this patient returned to normal before discharge from the hospital. This result can con rm that our approach can really help patients. Figure 1 The comparation of postoperative nomal spinal disease patient and CIPA pateient from lying to standing ; A nomal spinal disease patient; B CIPA pateient  The nutritional status of 3 patient