A 31-year-old male diagnosed in 2009 with Stiff-Strain Syndrome (SHR) atypical antipsychotics, disabling disease characterized by stiffness of the back and limbs due to continuous agonistic muscle contractions.
Its development involves antibodies directed against proteins of the organism involved in the GABAergical pathway leading to a negative effect of neurotransmission of gamma aminobutyric acid (GABA).
Its ethiopathogeny has been found to be four proteins (two presynaptic and two postsynaptic) as possible autoantigens.
Based on these immunological mechanisms involved in the physiopathogeny of the disease and in the non-response to immunomodulatory drugs plasmapheresis could be justified.
Personal history: Dorsal-lumbar scoliosis and cryptococcosis.
There is no conventional risk factor (non-DM, non-HTN, non-smoker).
Work in the maintenance and repair of vending machines.
1.
Establishment plan
The patient came to the emergency room in April 2009 complaining of severe dorsal pain radiating proximally and distally through the spinal cord without apparent triggering of 2 months of evolution treated by primary care with analgesia.
In the last two weeks she presented instability and weakness in the lower limbs.
Physical examination and analytical control were performed, as well as MRI of the dorsal spine with contrast, X-ray of the cervical and dorsal spine, chest X-ray, ECG and soft tissue ultrasound in the dorsal region diagnosed with Dorsal Pain.
In June of the same year he returned to the emergency room due to increased cervico-dorsal pain with inability to maintain an upright posture and muscle spasms in the lower limbs and lower limbs, causing falls and sleep pattern.
New diagnostic tests were performed (autoimmunity analysis and serology, complete spinal MRI, cerebral MRI with gadolinium, MR sacral-iliac joint, ultrasound consultation of soft parts and abdomen CT unit, CT unit).
Accomplished and unimproved patient to the rheumatology service for complete study of onconeuronals, AntiGAD and suspected unconfirmed Stiff-man Syndrome, initiating specific treatment
She was discharged with medication adjustment and rehabilitation recommendation.
Follow up for rheumatology and neurology consultations.
In the following two years, she was treated for possible Camptocormia with botulinum toxin injections every 4 months.
Presented Vitamin B deficiency
Throughout this period, they continued to perform diagnostic tests for the presence of a clear diagnosis (including genetic studies).
He was referred to the Neuromuscular Unit of the Neurology Service of the Hospital 12 de Octubre (Madrid), Unit of Movimento Consecutive Diseases of the Hospital Fundación Jiménez Díaz (Madrid).
The patient began to present an anxiety picture related to the progress of the disease.
In June 2012 due to the progressive worsening of the patient, hospitalization was decided.
The patient was no longer able to perform BADLs and used wheels to move due to total ventral flexion of the trunk and increased pain.
He was treated with several cycles of immunoglobulins.
In Octubre, 2012, after being diagnosed with Stiff-P bladder associated Camptocormia Syndrome, she was admitted for anuria with sphincter hypertonia and was discharged after placement of a catheter.
Percutaneous cytostomy was subsequently performed.
The pharmacological treatment used for this disease consists of drugs that increase GABA (anxiolytics, sedatives and antiepileptics), antispastic drugs, and immunomodulatory therapies.
In April 2013, a new hospital admission was decided for the initiation of therapeutic apheresis after failure of intravenous immunoglobulins.
On April 10, 2013, a provisional right jugular catheter was implanted and a first session of plasmapheresis was performed with replacement of 3000 cc of 5% albumin, membrane-separating plasma filter and anticoagulation with heparin.
The patient was informed about the care of the vascular access, highlighting the importance of maintaining the dry area, avoiding accidental catheter flushing, and communicating precociously the appearance of itching and/or pain in the site of insertion, etc.
The first week, apheresis was performed every other day, after which two weekly sessions were performed.
Given the good tolerance and excellent clinical response, apheresis procedure was performed once a week (Julio 2013).
The patient installed the standing position yielding the contracture of the abdominal muscles and recovering the upright posture, normalizing.
Cytostomy was removed, obtaining spontaneous and normal voiding.
On July 3, 2013, a radiocefaction arteriovenous fistula (AVF) was performed, which began to be used on August 6, 2013.
The patient is informed about the AVF care giving him all the necessary knowledge to detect possible complications.
After one month, the patient came to the dialysis unit complaining of pain in the AVF arm and the disappearance of thrill.
AVF thrombosis was detected and the patient recovered successfully in the vascular surgery service.
In September of the same year he suffered bacteremia due to S. Epidermis associated with temporary jugular catheter that was removed without incidents.
The response to apheresis therapy has been monitored by clinical evaluation, analysis of antibodies in plasma of anti-GAD67 and anti-GAD65, magnetic resonance imaging of the brain and neurophysiological study.
Currently the patient receives a monthly session of plasmapheresis chronically and diminishes the home medication.
1.
Care plan:
According to Virginia Henderson's care model 5, we established the nursing care process 6 focusing on those needs of patients who were not satisfied during the evolution of the disease, taking as a point of departure:
1.
Breathing normally: The patient breathed normally throughout the course of the disease.
Eupneic.
Imaging tests showed no findings.
Lost consciousness.
2.
Eating and drinking properly: The patient suffered progressive weight loss as the disease progressed.
3.
Constipation due to all body pathways: The patient presented problems of constipation due to pharmacological treatment to control the disease and an acute process in the urinary system.
4.
Moved and maintained adequate postures: difficulty walking due to intense pain caused by muscle contractures and trunk flexion having to use a permanent ventral wheel for displacement.
5.
Sleeping and resting: Continuous pain due to muscle spasms and the impossibility of maintaining an upright posture caused changes in the patient's sleep pattern.
6.
Choosing adequate clothing, dressing and dressing oneself out: Despite the continuous pain she experienced, she tried at all times to maintain independence, although sometimes she needed help to satisfy this need.
He wore comfortable clothing adapted to his needs.
7.
Maintaining body temperature within normal limits, adjusting clothing and modifying the environment: He experienced an episode of fever during bacteremia derived from temporary jugular catheter that was treated with antipyretics, recovering his catheter base.
8.
Maintaining body hygiene and skin integrity: The patient presented a bad appearance and cleanliness, often needing help during the bath time.
9.
Avoid environmental hazards and avoid injury to others: Numerous diagnostic tests and invasive treatments were performed.
10.
Communicating with others expressing emotions, needs, fears or opinions: During treatment the patient expresses his needs and emotions.
The family exercises the role of the caregiver without showing signs of strain, supporting him at each moment.
11.
Living according to their own values and beliefs: need satisfied despite pain and immobility
12.
Vital seclusion in something so that his work has a sense of personal accomplishment: due to the disabling evolution of the disease the patient could not work and expressed concern for his situation of progressive deterioration although he faced the process
13.
Establishment plan
14.
Learning, discovering or satisfying the curiosity that leads to normal development and using available resources showed interest in everything that was happening to him and asked about treatment with plasmapheresis.
The therapeutic regime has been implemented since the beginning of the disease.
