Development of an intervention to increase adherence to nebuliser treatment in adults with cystic fibrosis: CFHealthHub.

BACKGROUND: Cystic fibrosis (CF) is a life-limiting genetic condition in which daily therapies to maintain lung health are critical, yet treatment adherence is low. Previous interventions to increase adherence have been largely unsuccessful and this is likely due to a lack of focus on behavioural evidence and theory alongside input from people with CF. This intervention is based on a digital platform that collects and displays objective nebuliser adherence data. The purpose of this paper is to identify the specific components of an intervention to increase and maintain adherence to nebuliser treatments in adults with CF with a focus on reducing effort and treatment burden. METHODS: Intervention development was informed by the Behaviour Change Wheel (BCW) and person-based approach (PBA). A multidisciplinary team conducted qualitative research to inform a needs analysis, selected, and refined intervention components and methods of delivery, mapped adherence-related barriers and facilitators, associated intervention functions and behaviour change techniques, and utilised iterative feedback to develop and refine content and processes. RESULTS: Results indicated that people with CF need to understand their treatment, be able to monitor adherence, have treatment goals and feedback and confidence in their ability to adhere, have a treatment plan to develop habits for treatment, and be able to solve problems around treatment adherence. Behaviour change techniques were selected to address each of these needs and were incorporated into the digital intervention developed iteratively, alongside a manual and training for health professionals. Feedback from people with CF and clinicians helped to refine the intervention which could be tailored to individual patient needs. CONCLUSIONS: The intervention development process is underpinned by a strong theoretical framework and evidence base and was developed by a multidisciplinary team with a range of skills and expertise integrated with substantial input from patients and clinicians. This multifaceted development strategy has ensured that the intervention is usable and acceptable to people with CF and clinicians, providing the best chance of success in supporting people with CF with different needs to increase and maintain their adherence. The intervention is being tested in a randomised controlled trial across 19 UK sites.

Intraocular hemorrhage after systemic thrombolytic therapy in a patient with exudative macular degeneration.

PURPOSE: To report a hemorrhagic complication from thrombolytic therapy in a patient with exudative macular degeneration. CASE REPORT: A 75 year old patient with exudative macular degeneration developed pain and loss of vision in the left eye shortly after receiving tissue plasminogen activator (t-PA) for a myocardial infarction. Examination revealed the patient to be in angle closure. A CT scan revealed the etiology of the angle closure to be a dense vitreous hemorrhage pushing the iris-lens diaphragm forward. Intraocular pressure was treated successfully, but the final visual acuity was only light perception. CONCLUSIONS: Thrombolytic therapy can lead to devastating intraocular hemorrhages. The presence of exudative macular degeneration may potentially increase the risk of developing such complications.

Surgical management of posteriorly dislocated silicone plate haptic intraocular lenses.

PURPOSE: To report a large series of delayed posterior dislocation of silicone plate haptic intraocular lenses after Nd:YAG laser capsulotomy and discuss the surgical management of this complication. METHODS: We reviewed the records of 11 consecutive patients (11 eyes) with delayed onset of posterior dislocation of a plate haptic silicone intraocular lens. The cause of the posterior capsular defect, time to dislocation, surgical management techniques, complications, and visual outcome were recorded. RESULTS: In eight of the 11 eyes, the silicone plate haptic intraocular lens dislocated an average of 1.8 months (range, 0 to 6.5 months) after Nd:YAG posterior capsulotomy. The other three eyes had surgical complications at the time of cataract extraction that compromised posterior capsular or zonular integrity and led to silicone plate haptic intraocular lens dislocation from 9 weeks to 6 months (mean, 3.6 months) postoperatively. Surgical management consisted of pars plana vitrectomy with intraocular lens repositioning (six eyes) or exchange (five eyes). The average follow-up period after intraocular lens repositioning or exchange was 6.5 months (range, 1 to 14 months). Best-corrected visual acuity at the last follow-up examination measured 20/40 or better in all but one eye that had preexisting macular disease. CONCLUSIONS: Cataract surgeons and patients should be aware of the potential for plate haptic silicone intraocular lenses to undergo delayed posterior dislocation through capsular defects. This complication can be managed effectively with vitrectomy and either repositioning or exchange of the implant. Postoperative visual acuity is generally excellent, and complications are minimal.

Vortex keratopathy associated with atovaquone.

PURPOSE: We studied a case of vortex keratopathy that was associated with the use of atovaquone. METHOD: A patient with acquired immunodeficiency syndrome (AIDS) with pulmonary Pneumocystis carinii was treated with 750 mg of atovaquone three times a day. RESULTS: Similar to previous findings of drug-induced vortex keratopathy, atovaquone vortex keratopathy is presumably caused by its lipophilic properties. This property is similar to that of other lipophilic agents such as amiodarone, chloroquine, chlorpromazine, quinacrine, and suramin. CONCLUSION: Atovaquone should be added to the list of agents causing vortex keratopathy involving the corneal epithelium.

Intraocular lymphoma presenting as retinal vasculitis.

We describe two unusual young patients with intraocular lymphoma who presented clinically with "retinal vasculitis." Brain biopsy specimens in both showed an angiocentric pattern of lymphocytic infiltration. Immunohistochemical studies were positive for T-cell markers. Both the retinal and the brain blood vessels were thus a preferential site of appearance of the malignant T-cells. Both cases responded favorably to aggressive chemotherapy with long-term survival. In the world's literature, there are 57 cases of intraocular lymphoma in which cell surface-marker studies were performed; of these, 53% proved to be B-cell lymphomas and 21% to be T-cell lymphomas. As more cases of intraocular lymphoma are studied with cell-typing and as our histochemical techniques improve, a correlation between the systemic and ocular findings and the cell type may be revealed.

Treatment of cytomegalovirus retinitis with intravitreal ganciclovir. Long-term results.

Long-term management of cytomegalovirus (CMV) retinitis by intravitreal injection of ganciclovir was evaluated in ten patients with acquired immune deficiency syndrome (AIDS). Patients were unable to tolerate systemic ganciclovir because of severe neutropenia (8 cases), catheter-induced sepsis (1 case), or the need to continue therapy for human immunodeficiency virus (HIV) with zidovudine (ZDV) (1 case). All patients had a favorable response to initial treatment. Cytomegalovirus retinitis progressed in four fellow eyes in which treatment was deferred. Vision improved or remained stable in all but one eye. Patients were followed for a mean of 4 months and received an average of 16.6 intravitreal injections in each eye. Relapse occurred late in the course while on maintenance treatment in five eyes (33%). There was no evidence of toxicity from repeated intravitreal injections. Treatment was very well tolerated. The only severe complication in a total of 249 injections was a single case of Staphylococcus epidermidis endophthalmitis which responded to intravitreal antibiotic treatment. Intravitreal ganciclovir is an effective alternative to systemic ganciclovir in those patients with severe neutropenia and in those patients who desire to remain on systemic ZDV.

Recovery of human immunodeficiency virus from ocular tissues in patients with acquired immune deficiency syndrome.

Human immunodeficiency virus (HIV) was recovered from multiple ocular tissues in three patients with acquired immune deficiency syndrome (AIDS). Consistently found in the retina, HIV was also detected in the conjunctiva, cornea, and iris. In two cases, HIV was detectable despite treatment with oral zidovudine. All three patients had bilateral cytomegalovirus (CMV) retinitis managed by intravitreal injection of ganciclovir. Culture of the retina for CMV was negative in all three cases. The finding of HIV in multiple ocular tissues is consistent with the neurotropic nature of the virus, and may explain some of the common ocular manifestations of AIDS such as AIDS retinopathy. Infection with HIV may predispose the retina to other opportunistic infections and may explain the high incidence of CMV retinitis in AIDS patients. This is the first report of HIV isolation from tissue within the eye.

Ocular complications associated with retrobulbar injections.

The authors describe six complications, of retrobulbar injections documented by fundus photography and fluorescein angiography. These include (1) injection of corticosteroid into the posterior ciliary arterial circulation resulting in emboli in the vasculature of the choroid and the optic nerve head; (2) injection of corticosteroid into the ophthalmic artery resulting in emboli in both the choroidal and retinal circulations; (3) presumed injection of lidocaine and air into the optic nerve sheath adjacent to the globe with extension anteriorly into the subretinal space and the space between the posterior vitreous and the internal limiting membrane; (4) occlusion of the central retinal artery without an associated retrobulbar hemorrhage; (5) trauma to and partial injection of lidocaine in the central retinal artery with embolization into the retinal circulation; and (6) presumed injection of lidocaine into the optic nerve sheath producing a combined central retinal vein and artery occlusion. Alternative techniques that might decrease the incidence of complications associated with retrobulbar injections are discussed.

Progression of diabetic retinopathy after pancreas transplantation for insulin-dependent diabetes mellitus.

We studied the effect of successful pancreas transplantation and consequent normoglycemia (mean total hemoglobin A1, 7.0 percent; range, 5.8 to 8.3) on visual function and diabetic retinopathy in 22 patients with Type I diabetes mellitus (study group). Sixteen similar patients in whom pancreas transplantation had been unsuccessful (mean total hemoglobin A1, 12.0 percent; range, 8.0 to 18.0) served as a control group. The majority of patients in both groups had advanced proliferative retinopathy. At a mean follow-up of 24 months we found no significant difference between the groups in the rate of progression of retinopathy, expressed as a score. Success of the transplantation did not prevent progression of retinopathy across the range of retinopathy studied. Progressive retinopathy was observed more commonly in patients with low retinopathy scores (nonproliferative or mild proliferative retinopathy) at base line in both the study group (13 of 17 eyes, or 76 percent) and the control group (7 of 12 eyes, or 58 percent). Further analysis suggested the possibility that after three years of euglycemia, the study group had less deterioration than the control group, particularly in eyes with advanced retinopathy. We observed no difference in the rate of loss of vision between the two groups. This study provides evidence that pancreas transplantation and subsequent normoglycemia neither reverse nor prevent the progression of diabetic retinopathy.

Use of intravitreal ganciclovir (dihydroxy propoxymethyl guanine) for cytomegalovirus retinitis in a patient with AIDS.

A patient with acquired immune deficiency syndrome with bilateral cytomegalovirus retinitis was treated with intravitreal 200-micrograms/0.1-ml doses of ganciclovir (9-[2-hydroxy-1-(hydroxymethyl) ethoxymethyl]guanine). The ganciclovir serum and intravitreal concentrations were measured with an enzyme-linked immunosorbent assay and pharmacokinetic factors were determined. There was no evidence of systemic absorption of ganciclovir from the eye. The elimination half-life of ganciclovir from the vitreous was estimated to be 13.3 hours. The intravitreal concentration remained above the ID50 of cytomegalovirus for approximately 62 hours after a single injection. Clinically, the patient retained useful vision in his right eye for three months. A total of 28 intravitreal injections were given on an outpatient basis under topical anesthesia and were well tolerated. There was no evidence of retinal toxicity from the drug.

Vitrectomy and endolaser for complications of proliferative diabetic retinopathy.

Argon laser endophotocoagulation capability during vitrectomy surgery represents a significant technological advance. This report details the initial experience with this technique at the University of Minnesota in 18 patients with particularly high-risk complications of proliferative diabetic retinopathy. Overall, 13 of the 18 eyes (72%) were stabilized anatomically. In comparison with preoperative levels, visual acuity improved in 11 eyes, remained unchanged in 1 eye and deteriorated in 6 eyes. The technique is used intraoperatively to treat existing, iatrogenic or purposeful retinal breaks and to do panretinal scatter therapy to reduce the incidence of postoperative neovascular complications affecting either the anterior or posterior segment of the globe.

Timing of vitrectomy for active proliferative diabetic retinopathy.

Eyes with active proliferative diabetic retinopathy with dense sub-hyaloid hemorrhage and significant visual loss represent an appropriate indication for prompt vitrectomy. Twenty-six such eyes in 22 patients were operated. The results are compared to 49 diabetic eyes undergoing vitrectomy for other complications of diabetic retinopathy. The results demonstrated both an improved anatomic success rate (85% compared to 73%) and a higher rate of reading visual function (54% compared to 18%) in the early vitrectomy group, substantiating the study hypothesis.

Multifocal choroiditis associated with progressive subretinal fibrosis.

Five patients (all female, 14 to 34 years old) had an unusual form of multifocal choroiditis resulting in progressive subretinal fibrosis. This condition differed from other multifocal choroidal diseases such as acute multifocal placoid pigment epitheliopathy, birdshot retinochoroidopathy, and punctate inner choroiditis. Instead of forming atrophic or pigmented chorioretinal scars, the acute lesion healed with the formation of discrete, sharply angulated subretinal scars. In the macula the lesions coalesced, forming broad zones of subretinal fibrosis. Serous and hemorrhagic macular detachment may occur during the course of the disease with fluorescein angiographic features suggesting subretinal neovascularization. Subretinal neovascularization could not, however, explain the widespread scarring. Although progressive subretinal fibrosis may be considered an unusual outcome for a variety of other diseases, we believe that it is a distinct clinical entity distinguishable from other multifocal choroidal diseases. Retinal pigment epithelial hyperplasia may cause the condition.

Vitrectomy for double penetrating ocular injuries.

During the five-year period from 1977 to 1982, 15 consecutive patients with double penetrating ocular injuries were treated at the University of Minnesota. The 13 males and two females ranged in age from 5 to 38 years. Four patients had bilateral involvement. Vitrectomy techniques were used to stabilize the eyes and prevent or treat retinal detachment in 18 eyes. One eye was enucleated. Anatomic success was achieved in 11 eyes and visual success (visual acuity of 5/200 or better) in ten eyes. Surgical success was related to initial visual acuity, extent of vitreous hemorrhage, and the ability of the surgeon to excise completely the vitreous from the circumference of the exit wound.

Experimental retinal detachment. VI. The permeability of the blood-retinal barrier.

Fluorophotometry was used to study the permeability of the blood-retinal barrier in six monkeys with stable rhegmatogenous retinal detachments. Fluorescein transport was inhibited by probenecid. The rate of fluorescein disappearance (KvVv) was determined following intravitreal injection. In a separate experiment the equilibrium vitreous-plasma concentration ratio (Cv/Cp) was determined following intraperitoneal administration. Expressed in equivalent volumes of vitreous, the rate of fluorescein diffusion across the blood-retinal barrier (K'vVvCv/Cp) was 0.29 microL/min in control eyes and 0.73 microL/min in detached eyes. The rate of fluid movement across the blood-retinal barrier (K'vVv) [1 - (Cv/Cp)] was 2.89 microL/min in control eyes and 6.38 microL/min in detached eyes. Posterior movement of fluid contributes to retinal apposition under normal conditions and accounts for the rapid resolution of retinal detachment following closure of the retinal hole.

Retinal vascular changes in malignant melanoma of the choroid.

A review of fluorescein angiograms from 41 patients with malignant melanoma of the choroid showed that 11 patients (27%) had evidence of retinal vascular abnormalities. Abnormal retinal capillaries were found in eight cases, areas of capillary nonperfusion in seven cases, and microaneurysms in four cases. Lipid exudation, retinal neovascularization, and arteriovenous communication were noted in one case each. Histopathologic studies in eight cases showed extensive degeneration of the overlaying retina in all eight cases and extension through Bruch's membrane by the tumor in four cases. Abnormal capillary architecture was demonstrated by trypsin digest preparations from four cases.