A 27-year-old woman, who has been monitored in our unit since February 2004, was referred by her traumatologist at the age of 18 years.
Background
- Fibrodysplasia ossificans progressiva.
Diagnosis at 6 years of age.
Fusion at the level of the vertebral bodies of the cervical spine, multiple ossifications at the level of cervical, dorsal, lumbar, scapular belt, bilateral disability relating to the wheels, thighs, intense knee and abdomen,
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- Surgical intervention of exostosis on the right knee and on the first bilateral metatarsophalangeal joint.
- Bilateral clubfoot operated at age 6.
Hearing aid holder.
- Iron deficiency anaemia probably secondary to chronic gastric bleeding with NSAIDs and corticosteroids.
Treatment with oral iron.
- Constipation, vomiting, constipation.
- Metamizole intolerance.
- reactive anxious-depressive syndrome.
- Episodes of hypercapnic respiratory failure secondary to restrictive ventilatory disorder due to their underlying disease, which have occasionally deserved treatment with home NIV since 2004.
The last complementary examinations performed were:
- chest x-ray (2010): illustrative markings on soft tissues of the chest cavity in relation to the patient's underlying disease.
Cardiomegaly.
- abdominal CT (April 2010): multiple muscle classifications are visualized in the pectoral, dorsal and dorsal territories, the underlying disease being primarily in the left glúte territory, suggestive of
- PFR (March 2011) CVF 1.57 (40 %); FEV1 1.36 (42 %); FEV1 86 %, GSA: (March 2011) P02 70, PC02 55.
Sociofunctional situation: lives with their parents.
Student of business administration.
wheels are mobilized.
On her first visit she was treated with slow-release oral morphine plus a permanent prednisone regimen.
Previously, various NSAI and paracetamol regimens had been established that were progressively ineffective, then alternating different types of opioids and different forms of presentation.
Since February 2004 it has been periodically controlled, both for its basal pain caused by the multiple heterotopic ossifications it presents, as well as fundamentally, by its painful lateral earth pressures secondary to new inflammatory conditions in soft parts.
Progressively, corticosteroids were removed, leaving them confined only to painful episodes due to neo-ossification, largely improving the quadroid elements presented.
Baseline control with transdermal fentanyl plus sublingual fentanyl for breakthrough pain.
The pain was controlled with transdermal fentanyl 100 mcg/h every 3 days.
A few weeks ago, the patient experienced more severe pain since we attended her: due to change of home, 2 day of family holidays, and without any other apparent cause, severe left scapular pain begins.
She came to the nearest hospital where the infectious or respiratory components were not found, attributing the picture to her background disease.
They medicate with NSAI, pyrazolones and morphine, all IV.
After 24 hours, as they did not perceive any improvement, they referred it to our center.
On arrival, the patient had a 100 mm VAS. X-ray showed a new bone formation in the form of a bridge between the left scapula and the pare costal joint, as well as a new bone formation for the right cervical spine.
We started dexamethasone IV 4 mg c/6 hours + morphine IV 5 mg c/6 hours + desketoprofen 50 mg IV c/8 hours + oral diazepam 10 mg hour 22.
Coverage with medication 40 mg/day.
After 24 hours, the dose of dexamethasone increased to 12 mg every 6 hours.
After 24 hours there is improvement, VAS of 40 mm, we increased the dosage of dexamethasone in the morning to 20 mg, leaving the other 3 administrations to 12 mg. In addition, as reported by some dysesthesias added and region pain to pregabalin 75 mg
Three days after the last dose, VAS 0 mm. We started decreasing the dosage of dexamethasone, orally, and morphine.
Five days after discharge, with oral regimen of paracetamol 1 gram c/8 hours + NSAID 500 mg 1-0 + pregabalin 75 1-0-2 + diazepam 10 mg 0-0-1 + montelukast 5 mg
Six days after starting corticoids, laboratory tests were normal.
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After discharge we performed telephone follow-up, showing good evolution.
No pain.
He has resumed his university studies at a distance.
