27-year-old woman, who has been monitored in our unit since February 2004, when she was referred by her traumatologist at the age of 18.
History
- 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 the cervical, dorsal and lumbar paravertebral musculature, bilateral shoulder girdle, coxofemoral joints, thighs, knees and abdomen, resulting in intense pain and inability to ambulate, requiring a wheelchair to move around.

- Surgical intervention of exostosis at the level of the right knee, and bilaterally in the first metatarsophalangeal joint.
- Bilateral otosclerosis operated on at the age of 6. She is a hearing aid wearer.
- Iron deficiency anaemia probably secondary to chronic gastric bleeding due to treatment with NSAIDs and corticoids. On treatment with oral iron.
- Obesity, cushingoid appearance.
- Intolerance to metamizole.
- Reactive anxious-depressive syndrome.
- Episodes of hypercapnic respiratory failure secondary to restrictive ventilatory disorder due to her underlying disease, which have occasionally merited treatment with home NIV since 2004.
The latest complementary explorations performed were:
- Thoracic CT scan (February 2010): gross calcifications in soft tissue in the thoracic cavity in relation to the patient's underlying pathology. Cardiomegaly.
- Abdominal CT scan (April 2010): multiple muscle calcifications were seen in the pectoral, periscapular, dorsal, paravertebral and mainly in the left buttock area, suggestive of corresponding to her underlying disease.
- PFR (March 2011) FVC 1.57 (40 %); FEV1 1.36 (42 %); FEV1 86 %, GSA: (March 2011) P02 70, PC02 55.
Socio-functional status: Lives with parents. Student of business administration. She uses a wheelchair.
At her first visit she was prescribed a slow-release oral morphine, plus a permanent prednisone regimen. Previously, various NSAID and paracetamol regimens had been established, which were progressively ineffective, alternating between different types of opioids and different forms of presentation. Since February 2004, she has been monitored periodically, both for her baseline pain caused by the multiple heterotopic ossifications she presents, and also and fundamentally, for her painful thrusts secondary to inflammatory conditions in soft parts that give rise to new bone formations, thrusts caused by mild trauma, abnormal postures, viral conditions, etc.
We progressively withdrew the corticoids, leaving them confined only to the painful episodes due to neo-ossification, largely improving the cushingoid elements that he presented. Baseline control with transdermal fentanyl plus sublingual fentanyl for breakthrough pain. Lately, with transdermal fentanyl 100 mcg/h every 3 days, the pain was under control.
A few weeks ago she presented her most severe pain thrust since we attended her: due to a change of address, 2 days of family holidays, and with no other apparent cause, she started severe pain at the level of the left scapula, without fever and without any parallel respiratory symptoms. She went to the nearest hospital where she was examined and was found to have no respiratory or diffuse infectious component, attributing the symptoms to her underlying illness. She was given NSAIDs, pyrazolones and morphine, all by IV. After 24 hours, as no improvement was noted, she was referred to our centre. On arrival, VAS of 100 mm. The X-ray showed bone neoformation in the form of a bridge between the left scapula and costal grill, as well as accentuation of the right paracervical bone neoformation. We started a regimen of IV dexamethasone 4 mg c/6 hours + IV morphine 5 mg c/6 hours + desketoprofen 50 mg IV c/8 hours + oral diazepam 10 mg hour 22. Coverage with omeprazole, 40 mg/day. After 24 hours, the patient remained practically unchanged, so we increased the dosage of dexamethasone to 12 mg c/6 hours. After 24 hours there was improvement, VAS of 40 mm, we increased the dosage of dexamethasone in the morning to 20 mg, leaving the other 3 administrations at 12 mg. In addition, as she reported some dysaesthesia and paraesthesia on mobilisation of the painful region, we added oral pregabalin: 75mg-5mg-150 mg. Progressive improvement. After 3 days of this last regimen, VAS 0 mm. We began to reduce the dosage of dexamethasone, switching to oral administration, and morphine. Five days later, discharge home, with an oral regimen of paracetamol 1 gram c/8 hours + naproxen 500 mg 1-0-0 + pregabalin 75 1-0-2 + diazepam 10 mg 0-0-1 + montelukast 5 mg 0-0-1. Six days after starting the corticosteroids, the blood tests were normal.

From the time of discharge we carried out telephone follow-up, showing good evolution. No pain. She has resumed her university studies at a distance.

