Background
Methods
Study design
Collaborative qualitative research
Description of the CF journey
Paediatric: | |
P1 = patient between 0 and 5 years | |
P2 = patient between 6 and 11 years | |
P3 = patient between 12 and 15 years | |
P4 = patient > 15 years and < 19 years (adolescents) | |
P5 = patient who has changed CF centre in the last two years | |
P6 = patient who received a CFTR modulator/potentiator | |
P7 = minor patient diagnosed with COVID-19 | |
P8 = minor patient in the paediatric transplant process | |
Adults: | |
P10 = patient > 18 and < 22 years of age post- transition from paediatric care | |
P11 = patient with late diagnosis in the last 4 years | |
P12 = 22–26-year-old patient stabilised at CF centre | |
P13 = patient with complications and progression of disease severity | |
P14 = patient travelling abroad for > 3 months in the last 3 years | |
P15 = patient planning to have a child or in the process of MAP | |
P16 = patient with children | |
P17 = patient who received a CFTR modulator/potentiator | |
P18 = adult patient diagnosed with COVID-19 | |
P19 = patient without major complications following transplant | |
P20 = patient with long-term complications following transplant |
Geographic data | |
Distance between home and centre: | |
0–5 km - between 5 and 20 km - between 20 and 50 km - over 50 km | |
Duration of travel (hours, minutes) from home to the centre (Hour, minutes): | |
Family characteristics | |
Language spoken in the family of the child or adult patient: | |
Marital status of the patient or parent responding to the survey: | |
Number of children living in the home: | |
Siblings with cystic fibrosis (number of children): … | |
Other medical conditions in the family (name the condition): …………. | |
Socio-professional characteristics | |
Employment status of the patient or parent responding to the survey: | |
- Salaried worker | - no professional activity |
- Self-employed | - on disability benefits |
- Student | - retired |
Socio-professional category of the patient or parent responding to the survey: | |
- Farmer or farm operator | intermediate profession |
- Craftsman or trader | - employee |
- Executive or intellectual profession | - worker |
- Student | |
Education level of the patient or parent responding to the survey: | |
- No diploma | - undergraduate degree |
- French certificate of general education | - master's degree |
- French vocational qualifications (CAP-BEP) | - PhD level |
- French general baccalaureate or professional baccalaureate | |
Specific criteria | |
- Activities with associations | - other: |
Paediatric Centre Investigator | # Patients interviewed | # Patients 0–5 y.o | # Patients / 6–11 y.o | # Patients 12–15 y.o | # Patients 16–19 y.o |
---|---|---|---|---|---|
P1 | P2 | P3 | P4 | ||
Lille Paediatric | 3 | 1 | 1 | 1 | – |
Paris R. Debré | 5 | – | 3 | – | 2 |
Strasbourg Paediatric | 5 | 2 | – | 2 | 1 |
Saint-Pierre LR | 5 | 1 | 2 | 1 | 1 |
Rennes Paediatric | 6 | 2 | 1 | 2 | 1 |
Bordeaux Paediatric | 3 | 1 | 1 | 1 | – |
Grenoble Paediatric | 4 | 1 | 2 | 1 | – |
S/TOTAL Paediatric | 31 | 8 | 10 | 8 | 5 |
Adult Centre Investigator | # Patients interviewed | # CF Patients 18–22 y.o | # CF Patients / 22 + y.o | # Transplant Patients | Of whom # Patients with child |
---|---|---|---|---|---|
P10 | P11-P12-13 | P19-20 | P15-16 | ||
Lille Adult | 12 | 2 | 7 | 3 | 3 |
Nantes Adult | 10 | 2 | 3 | 5 | 4 |
Lyon Adult | 6 | 1 | 3 | 2 | 3 |
Clermont-Ferrand | 6 | 2 | 3 | 1 | 3 |
Other CF centres | 2 | – | – | 2 | 2 |
S/TOTAL Adults | 36 | 8 | 15 | 13 | 15 |
TOTAL | 67 |
Study setting
-
In rural regions (Clermont-Ferrand or east of France or southwest), patients may live far from their center (more than 2-h drive) and may not have healthcare resources locally
-
In the Alps (Grenoble, Lyon) some families have to drive through the mountains to access their CF center which might be a problem in the winter
-
In Paris region as well as in the north (Lille), the centers follow deprived families, a few of them being immigrants from northern Africa
-
In La Réunion island, many families have specific beliefs related to genetic diseases, nutritional regimen in addition to a range of specific bacteria due to the local climate
Recruiting the study population
Collection and analysis of patient experience data
Semi-structured interviews
Data analysis
Ethics approval
Results
Sample characteristics
Interviews conducted with the study population sample
Total | Number | Average | Mini | Maxi | |
---|---|---|---|---|---|
Paediatric | 39:34:44 | 31 | 1:16:36 | 42 min | 2h07' |
Adult | 44:37:00 | 36 | 1:12:21 | 27 min | 2h02' |
Mapping of PE domains and sub-domains
Descriptive results of two domains and their sub-domains
Management of CF care
My FEV was yo-yoing, the IV cure was always subject to discussion: when you don't feel sick, when it thwarts projects... The negotiation was tight. There is a big work of renunciation when you do not feel the benefit of the treatment even though there are side effects of antibiotics, allergies... Gradually, I can say that there has been a taming on both sides: following an article in PubMed, I obtained that my IV cures only last 10 days. The doctor has recognized that it is important to negotiate, to convince the patient of the benefit of the treatment.
It's simple, there are too many of us, there is no segregation (depending on the bacteria of the patients); there is a room to spit, it is called the spittoon, all the patients pass there one behind the other, no window no ventilation between two, no disinfection between patients.\
I was in a relationship, but at some point I was no longer independent at all, I didn't want to put the burden on my boyfriend, I went back to my parents, so that they could take care of me.
Domain of patient therapeutic education and shared decision-making
We were able to do lots of workshops, lots of questions. We all ate lunch together. We really spent the day from morning until evening with the parents, and a physiotherapist and a dietician from the CRCM who taught us all about how it works, where the disease started, which organs it could affect, and the evolution. At first, we were a little skeptical about going there, we didn't want to... well, not to cry... but we didn't want to spend a day where we were depressed all day. But they had really organized it so that we talk about the disease in general, and we can learn things we didn't know. There were a lot of things that we didn't know at all after a year... It was really very interesting.
On the day we changed centre, the paediatrician accompanied us, we were introduced to the whole adult care team and there was quite a friendly atmosphere. The transition went quite smoothly. We were able to talk with our old team and the new team... It happened at an age when change doesn't cause any trauma, it was not a problem. We were pretty well informed beforehand, and I was free to transition to the new centre whenever I felt ready. At the age of 18, I was happy to change centres.
The doctor who used to follow me came, then my new doctor introduced himself, they were able to talk. It was interesting. And afterwards, the whole team introduced themselves. An office was assigned to me on the day, and they all came in one by one. My parents came to the new centre on the first day, to see what it was like and meet the new team, but since then, they have not felt the need to come back and I have not either.
Now that I have transferred, I am more critical of the care I receive in the adult care service, which is not at all the same as in paediatrics services. For example, in paediatrics, I saw the physiotherapist every time I came to the centre. Now, this is no longer the case. I used to see many different care professionals in the paediatrics services, and I miss it at the adult care centre. Every time I come, I only see the pulmonologist and I do pulmonary function tests.
It's more the aerosols that are heavy, for my hair or skin concerns, I admit that I leave them aside a little, because having done everything else, in the evening I don't want to go to do the treatment, to pass the ointment on my head... There I let go a little, I don't necessarily do it all the time, I told the dermatologist. The accumulation of everything sometimes... I prioritize care a little.
And since all my blood sugar spikes were related to an infection, I wondered if it was COVID. But when I asked my doctor, he didn't want to do an additional blood test. I had to ask a doctor friend for a prescription, to see if there were antibodies... So I had to search on my own: I looked a lot on the internet to find out what are the reasons that increase blood sugar for normal diabetes and what can be the specific reasons for CF. And it's true that I was lucky, the most likely hypothesis was stress, but it could have been something else.
Tom, his FEV is mediocre at 60%. But fortunately the doctor has confidence, she believes us when we tell her that when he rides a bike or a trampoline he is not out of breath... When I have a problem, I send an email or I call, they will try to answer us during the day, at worst the next day. If they don't have the answer right away, we'll get a little email: the doctor isn't there, we're trying to contact her.
I have the impression that all the centres, I don't know if they all work like that, they draw the IV treatment quite quickly I have the impression. It always went well, and we saw the improvement afterwards. But for the parents, for the child, it's always complicated to say: come on, we're going to be on IV cure for 15 days. Now, we know what it is, we were a little scared the first time...
Main points from the patient experience collected from our study sample
Main points of the care pathways
-
The therapeutic partnership that patients and parents seek to build with the CF centre team contrasts with their lack of confidence or mistrust in the specialists they consult, outside the field of CF care.
-
The fight against CF is a daily struggle. At home, it relies on therapeutic patient education that focuses on preventing and supporting the management of warning signs of serious complications and crisis situations. Continuity of care between two visits to the centre relies on matters of responsiveness, on the care partnership between patients or parents and the care team, and ultimately, on the stability or deterioration of patients' health and quality of life.
-
The notable differences between paediatric and adult care concern: the multidisciplinary nature of paediatric care teams compared with adult care teams, which revolve around the pulmonologist—nurse coordinator collaboration; the place of the physiotherapist in adult care varies greatly between centres as well as the coordination of specialties outside the CF centre.
-
The perceived risk of cross-infection during hospital visits, depending on the physical organisation of consultations, and the level of respect of infection control measures in the hospital.
-
No significant experiences were reported by patients regarding disagreements between CF care team members about diagnosis or treatment.
-
Difficulties were reported during inpatient hospitalisation, sometimes serious, including declarations of adverse events. These difficulties are resolved by visits from CF centre consultants at patients' bedside.
-
Information on and access to new therapies varied between centres during phases of temporary compassionate use, which raises questions about access to information in patient records to verify eligibility.
-
Post-transplant follow-up is organised in different ways. It is either done at the transplant centre (TC), or alternates between the TC and CF centre nearest to the patient. These differences are discussed by the patients on social media, and do not seem to be based on scientific guidelines.
-
Transplant patients do not benefit from new therapies for CF and the lack of research involving this population contrasts with the fact that transplant patients are neither cured of CF, nor always in good health, and many factors make their future uncertain.
Main points of life with cystic fibrosis
Some difficult points in transitions
I would like to emphasize the kindness, the support which was great. We were completely in tears the first time we went to the hospital; the next week we were in "we're going to fight, they're going to fight with us" mode. See all this team that was dedicated to this disease. We are very lucky to have such good care.
Tom was born, he was not a big baby…The only thing that stuck out to me was that when I gave him a kiss, I said to the nurse: he has salty skin. She said don't worry, we haven't given him his first bath yet, that's got to be it. That's the only thing she said to me. Afterwards, when they came to give her the Guthrie test, I asked why it was done, she explained to me and said: it takes about a month if there is something, if in a month you haven't received anything, everything is fine. On November 19 I said to my husband: everything is fine, we haven't had any mail. And on November 21, I got the call from the doctor.
We were advised to change the sponge too, while doing the dishes. No more classic yellow sponge with green, it is better to use dishcloths or things like that that dry quickly. We were told to avoid plants in the house too, to avoid standing water in cups or things like that. In the car, on the way back from the CF centre, we decided to get rid of our pets and our plants. We were also told to disinfect all the siphons once a week, to prevent bacteria from forming in the siphons and taps. At first we were like, wow, that's a whole lot more to pay attention to. And finally, once you get used to it, it happens naturally.
“When you go to other people's house, it won't necessarily be as clean as your house, it's not too good to put them in a bubble either.”
When I was transplanted, I came back to my CF centre, we both talked a lot with the doctor, and that's when she told me that the announcements of transplants, she is really very bored to do them. She said to me: “you can't imagine how difficult it is to announce to your patients that you are trying to save their lives day by day and all of a sudden to tell them that you can't do anything anymore, and they have to go to a transplant center, and we don't know if the transplant will work. Maybe so, but there can be complications. I couldn't tell you everything. I couldn't tell you there are some who died.