Hospital Staff Didn’t Give Emergency Cortisol to Teenager with Complex Special Needs

solu-cortef

 

A TEENAGER with complex special needs who died in hospital suffered a failure in basic medical communication, an inquest heard today.

Robin Brett, 18, of Blackmore Close, died in June 2014 in the Great Western Hospital after being admitted with chronic constipation and vomiting.

After a blood test indicated a raised white blood cell count, Robin went into cardiac arrest and died.

He had congenital adrenal hyperplasia (CAH) a metabolic disease and genetic defect of the adrenal gland and learning difficulties. He required daily care and medications.

Robin’s parents listed a string of errors they believe contributed to his death including failure to give him his regular medications, infrequent observations and the lack of regular and vital cortisol injections.

His heartbroken mother, Teresa, told the inquest in Salisbury that she had told hospital staff to give Robin an emergency dose of cortisol, which was not topped up after the recommended four hours.

She said: “I asked them to give him cortisol after I noticed he was becoming clammy and had a headache which is a sign of adrenal distress, I was just about to with the syringe in my hand when it was done and he instantly perked up.

 “But this wasn’t done again after four hours and I don’t know why.”

GWH staff nurse Hannah Porte who cared for Robin on his admission said she had concerns about his “alarmingly” high pulse rate when she did observations.

“I spoke with a doctor who assured me that because he had a pre-existing condition it wasn’t of great concern. That is our protocol and I felt comforted and reassured when they said that,” she said.

Robin was described as “rocking backwards and forwards and retching “ shortly before his cardiac arrest.

Mum Teresa broke down as she recalled her “sociable and friendly” son’s decline.

“He asked me to turn his DVD player off which was out of character in itself and he was clammy. All of a sudden he wasn’t breathing,” she said,

Registrar Fahreyer Alam, who examined Robin upon admission, said he could not provide an explanation as to why steroidal drugs were prescribed but not administered to Robin.

“They was nothing about his condition on examination which would link to adrenal crisis,” he said,

“The drugs were written on the drug chart and I cannot say why they were not given to him.”

Dr Alam said he had set an observation schedule of every two hours which he had articulated to nurses, and was not observed.

“All I can is there is an element of trust in the nurses and in a busy department we do have to relay things verbally and that is what you do,” he said.

When questioned by assistant coroner Dr Claire Balysz, Dr Alam said the effect of the seven week constipation may have put pressure on Robin’s vital organs.

“His heart and lungs were smaller than average and slightly underdeveloped. The faecal impaction made his colon stretch, it may have impacted his lungs and his heart and that is something the post mortem found,” he said.

Dr Alam and nurse Porte agreed that more was being done within the trust to improve the accuracy and accessibility of patient records, including a new observation system and the use of electronic prescribing and administration (EPMA) system.

Adapted from http://www.thisiswiltshire.co.uk/news/13843924.Robin_Brett__18__died_in_GWH_after_medics_failed_to_communicate__inquest_hears/

Congenital adrenal hyperplasia: Current surgical management at academic medical centers in the United States

J Urol. 2015 May;193(5 Suppl):1796-801. doi: 10.1016/j.juro.2014.11.008. Epub 2015 Mar 25.

Congenital adrenal hyperplasia: current surgical management at academic medical centers in the United States.

Abstract

PURPOSE:

Controversy exists on the necessity for and timing of genitoplasty in girls with congenital adrenal hyperplasia. Our knowledge of surgical preferences is limited to retrospective series from single institutions and physician surveys, which suggest a high rate of early reconstruction. We evaluated current surgical treatment for congenital adrenal hyperplasia at academic centers.

MATERIALS AND METHODS:

We queried the Faculty Practice Solutions Center database to identify all female patients younger than 18 years with a diagnosis of congenital adrenal hyperplasia between 2009 and 2012. Procedures were identified by CPT codes for vaginoplasty, clitoroplasty and other genital procedures. Reconstruction type, age at surgery and surgeon volume were analyzed.

RESULTS:

We identified 2,614 females in the database with a diagnosis of congenital adrenal hyperplasia who were seen at a total of 60 institutions. Of infants younger than 12 months between 2009 and 2011 as few as 18% proceeded to surgery within a 1 to 4-year followup. Of those referred to a pediatric urologist 46% proceeded to surgery. Of patients who underwent surgery before age 2 years clitoroplasty and vaginoplasty were performed in 73% and 89%, respectively, while 68% were treated with a combined procedure. A medium or high volume surgeon was involved in 63% of cases.

CONCLUSIONS:

Many patients with congenital adrenal hyperplasia in the database did not proceed to early reconstructive surgery. Of those referred to surgeons, who were possibly the most virilized patients, about half proceeded to early surgery and almost all underwent vaginoplasty as a component of surgery. About two-thirds of the procedures were performed by medium or high volume surgeons, indicative of the surgical centralization of disorders of sexual development.

Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

KEYWORDS:

adrenal glands; adrenal hyperplasia; congenital; disorders of sex development; reconstructive surgical procedures; virilism

PMID:
25817160
[PubMed – in process]

from http://www.ncbi.nlm.nih.gov/pubmed/25817160

Other Diseases

forums

Many of the people who post on the message boards suffer from other diseases, as well as Cushing’s. These links help to provide some information about these diseases.

~A ~

Acanthosis nigricans
This Topic on the Message Boards.

Acromegaly
This Topic on the Message Boards.

Addison’s Disease
This Topic on the Message Boards.

Adrenoleukodystrophy
This Topic on the Message Boards.


~B ~

Barrett’s esophagus


~C ~

Carney Complex
This Topic on the Message Boards.
New Support Group for Carney Complex.

Central Serous Retinopathy
This Topic on the Message Boards.

Congenital Adrenal Hyperplasia (CAH)
This Topic on the Message Boards.

Conn’s Syndrome
This Topic on the Message Boards.

Craniopharyngioma
This Topic on the Message Boards.


~D ~

Diabetes insipidus
This Topic on the Message Boards.


~E ~

Ectopic ACTH Syndrome
This Topic on the Message Boards.

Empty Sella
This Topic on the Message Boards.


~F ~

Fibromyalgia
This Topic on the Message Boards.


~G ~

Gigantism
This Topic on the Message Boards.


~H ~

Hirsuitism
This Topic on the Message Boards.

Hyperprolactinemia
This Topic on the Message Boards.

Hyperthyroidism
This Topic on the Message Boards.

Hypoalderostonism
This Topic on the Message Boards.

Hypocalcemia
This Topic on the Message Boards

Hypopituitarism
This Topic on the Message Boards.

Hypothyroidism
This Topic on the Message Boards.


~I ~

Insulin Resistance
This Topic on the Message Boards.


~K ~

Kidney Disease
This Topic on the Message Boards.


~L ~

Lyme Disease
This Topic on the Message Boards.


~M ~

Madelung’s Disease
This Topic on the Message Boards.

Menopause
This Topic on the Message Boards.

MEN Type 1
This Topic on the Message Boards.

Myasthenia Gravis
This Topic on the Message Boards.


~N ~

Nelson’s Syndrome
This Topic on the Message Boards.


~O ~

Osteopenia
This Topic on the Message Boards.

Osteoporosis
This Topic on the Message Boards.


~P ~

Panhypopituitarism
This Topic on the Message Boards.

PCOS
This Topic on the Message Boards.

Perimenopause
This Topic on the Message Boards.

Pheochromocytoma
This Topic on the Message Boards.

Pituitary dwarfism
This Topic on the Message Boards.

Premature menopause
This Topic on the Message Boards.

Primary pigmented nodular adrenocortical disease (PPNAD)
This topic on the Message Boards

Prolactinoma
This Topic on the Message Boards.

Pseudo Cushing’s
This Topic on the Message Boards


~R ~

Rathke’s cleft cyst
This Topic on the Message Boards.

ROHHAD (Rapid-Onset Obesity With Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation Presenting in Childhood)
This Topic on the Message Boards


~S ~

Sheehan’s Syndrome
This Topic on the Message Boards.

Stein-Leventhal Syndrome
This Topic on the Message Boards.


~T ~

Thymoma
This Topic on the Message Boards.

Thyroid Gland Disorders
This Topic on the Message Boards.

Turner’s Syndrome
This Topic on the Message Boards.


~V ~

Von Hippel-Lindau disease
This Topic on the Message Boards.


~Z ~

Zollinger-Ellison Syndrome

Diagnosing and Treating Cortisol Excess and Deficiency

From Day 1 of the 16th International Congress of Endocrinology and the Endocrine Society’s 96th Annual Meeting and Expo »

Chicago, IL – June 21, 2014

A phase 2 study of Chronocort®, a modified release formulation of hydrocortisone, in the treatment of adults with classic congenital adrenal hyperplasia

A Mallappa, L-A Daley, N Sinaii, C Van Ryzin, H Huatan, D Digweed, D Eckland, M Whitaker, LK Nieman, RJ Ross, DP Merke

Summary: Classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is characterized by cortisol and aldosterone deficiency and androgen excess. Current conventional glucocorticoid therapy is suboptimal as it cannot replace the normal cortisol circadian rhythm and inadequate or inappropriate suppression of adrenal androgens are common. In the preliminary results of a phase 2 study of Chronocort®, a modified release hydrocortisone capsule formulation, researchers found that Chronocort®, a novel modified release hydrocortisone capsule formulation, approximates physiological cortisol secretion, and improves biochemical control of CAH. Further analyses are underway.

Methods:

  • The study objectives were to characterize pharmacokinetics and examine disease control following 6 months dose titration.
  • Serial profiling was obtained at baseline (conventional glucocorticoid) and every 2 months.
  • Twice-daily Chronocort® was initiated: 20 mg at 2300 h, 10 mg at 0700 h.
  • Dose titration was based on clinical status and optimal hormonal ranges (17OHP 300-1200 ng/dL, normal androstenedione (males: 40-150, females: 30-200 ng/dL), with androstenedione prioritized.
  • Chronocort® cortisol pharmacokinetic profile was the primary endpoint.
  • Secondary endpoints included biomarkers of disease control.

Results:

  • A total of 16 adults (8 females; age 29 ±13 years) with classic CAH (12 salt-wasting, 4 simple virilizing) participated.
  • Conventional therapy varied (5 dexamethasone, 7 prednisone, 4 hydrocortisone).
  • Chronocort® cortisol pharmacokinetic profile approximated physiological cortisol secretion.
  • Ten patients required Chronocort® dose adjustments (decrease in 8, increase in 2; mean hydrocortisone equivalent dose conventional vs 6 months: 16.1 ± 6.4 vs 14.7 ± 6.4 mg/m2).
  • Serial androstenedione levels were in the normal range in 8 (50%) of patients on conventional therapy compared with 12 (75%) on Chronocort® at 6 months.
  • The majority of patients on Chronocort® achieved 17O HP levels within the normal range, rather than within the mildly elevated range currently used for management.
  • At 6 months, Chronocort® resulted in lower 24-hr (P=0.02), morning (0700-1500; P=0.008), and afternoon (1500-2300; P=0.03) area-under-the-curve androstenedione compared with conventional therapy.
  • No serious adverse events occurred.
  • Common adverse events were headache, fatigue, early awakening, and anemia.
  • Three patients had unexpected carpal tunnel syndrome, which resolved with wrist splints.

From http://www.mdlinx.com/endocrinology/conference-abstract.cfm/ZZ5BA369FDE9DE4CED82CB6A7CD5BFD1BE/16521/?utm_source=confcoveragenl&utm_medium=newsletter&utm_content=abstract-list&utm_campaign=abstract-ICE/EN2014&nonus=0#

Cushing’s Awareness Challenge, Day 4

Another idea I borrowed from Robin – using a Wordle as inspiration for today’s post.
Even though I’m “in remission” since 1987, I’m still way too fatigued, napping every afternoon for several hours.  People think I should be normal since my pituitary surgery was so long ago.  Well, no.
me-tired
Just a few days ago, I posted this abstract on Severe fatigue in patients with adrenal insufficiency.  I don’t think that they needed to do this study at all.  Just ask any Cushie!

Abstract

BACKGROUND:

Fatigue is a frequently experienced complaint in patients with adrenal insufficiency (AI) and may be influenced by cortisol levels.

AIM:

The objective of this study was to determine the prevalence of severe fatigue in adrenal insufficiency (AI) patients, to assess which dimensions contribute to fatigue severity and to determine the association between salivary cortisol levels and momentary fatigue.

SUBJECTS AND METHODS:

We performed a cross-sectional study in the outpatient department of a university hospital. Included were 27 patients with congenital adrenal hyperplasia (CAH), 26 patients with primary AI (PAI), 24 patients with secondary AI (SAI) and 31 patients with adrenal insufficiency after treatment for Cushing’s syndrome (Cush-AI). Measurements included computerised questionnaires to determine fatigue severity and physical and psychosocial contributors. Patients took four saliva samples at home, in which cortisol levels were measured.

RESULTS:

Severe fatigue was experienced by 41 % of the CAH patients, 42 % of the PAI patients, 50 % of the SAI patients and 42 % of the Cush-AI patients. Psychological distress, functional impairment, sleep disturbance, physical activity, concentration problems and social functioning contributed to the subjective experience of fatigue. Salivary cortisol levels were not correlated with momentary fatigue.

CONCLUSIONS:

A considerable proportion of AI patients experience severe fatigue. Salivary cortisol level is not a significant predictor for momentary fatigue in AI patients.

PMID:
24615365
[PubMed – in process]

From http://www.ncbi.nlm.nih.gov/pubmed/24615365

 

Back to my nap now…

maryo colorful zebra