Cystic Fibrosis Diagnosis During Infancy: A Balancing Act
Trudy Havermans, PhD; Linda Boulanger, RN; and Marijke Proesmans, MD PhD

Trudy Havermans

When parents receive the diagnosis of a chronic illness they have to say goodbye to a healthy child and meet a child with special needs. In Belgium, there is only neonatal CF screening in limited areas. About 70% of diagnoses are made within the first years of life (Belgian CF registry 2003).

During the first days and weeks parents are overwhelmed. They meet many healthcare workers in and outside the hospital and they are practically snowed under technical and wide-ranging information. Maybe even more overwhelming for parents is watching their child go through numerous (painful) investigations and procedures. Doctors and nurses are used to the hospital, taking (blood-) samples; x-ray investigations etc., but for many parents this is a new experience. Parents often express a loss of control over their child and their child’s life and they feel inadequate at parenting their child, taking care of their child the way they think they should or the way they would want to.

Over the past two months, we have had nearly ten new paediatric diagnoses of CF in or referred to our CF clinic in Leuven, Belgium. This is far more than we would normally have in such a time span, and it gave us the opportunity to reflect on our current strategy on starting-up CF care. It is important to note that we find this strategy helpful to follow within our setting! When providing first diagnosis care empathy is a central component. Within a context of empathic care every single member of our CF team focuses on their own specialisation. In the following a brief overview for the work division within our centre in Leuven:

1. The paediatricians aim to optimize treatment and stabilize the patients as soon as possible and inform parents about CF and it’s future perspectives;

2. The specialist CF nurse helps parents to find their way through the medical regime, guiding them with great care to obtain all the information they need, making sure they got the necessary equipment and that parents meet the members of the CF team and at the same time the nurse tries to protect parents from negative influences, e.g. wrong information on the internet, without avoiding discussion of difficult issues;

3. The dietician teaches parents about the child’s special needs with regard to food and eating, now and in the future, the enzymes and the vitamins and the extra care parents have to take with regard to their child’s diet;

4. The social worker helps parents struggle through mountains of paper work that has to be sorted (child benefit, hospital bills etc.); advice on job planning, help with social organizations, etc

5. The physiotherapists train parents to perform physiotherapy with their young child, teach them to do the therapy themselves and help parents to find a home-
physiotherapist. The physiotherapists also support parents to come to terms with
a completely new aspect of caring for a baby.

Linda Boulanger

6. The psychologist helps parents in their first attempts to come to terms with the diagnosis. Parents are encouraged to talk and the psychologist helps them to express their innermost worries and anxieties, their anger about the loss of control and the loss of security. The psychologist helps parents to cope with the loss of the image they had created of their future life with the new baby and helps them to carefully create a new picture, encouraging them to be optimistic but realistic.

As stated in the introduction, receiving the diagnosis of a chronic illness changes people’s lives. This confrontation is even more pronounced for apparently healthy babies diagnosed with CF after neonatal screening. Many parents deep down hope for weeks after the diagnosis that some mistake has been made, that the diagnosis is wrong.

The question is how best to provide CF care to parents in this agonizing situation. For sick children, hospital admission is urged by their pulmonary disease or need for major nutritional support; for babies with minimal symptoms such as after neonatal screening, one could argue that admitting a CF child is not a good initiative. Contra-indications for hospitalization include economical, social-emotional but also medical aspects. From a medical point of view, one could dispute that children should not unnecessarily be exposed to hospital bacteria. Hospitalization is expensive and often leads to parents having to take time of work. Some parents of asymptomatic children consider a hospital stay a waste of time. In addition, one could argue that patient and family need their home environment in times of stress, they need their family and friends close by.

Historically, since most patients were ill at diagnosis, a hospital stay was not questioned. Over the last years a few exceptions were made for a-symptomatic babies were parents as well as referring paediatricians argued strongly against a hospital stay. For these children however, we experienced the lack of a basic working relationship established between parents and the CF team during the first weeks after diagnosis. For example, we felt parents were less well informed and CF care had taken a bad start with stressful events happening, that could have been avoided. Sadly enough we also observed that some families minimized the CF diagnosis and treatment compliance was never properly established.

The question how to prevent these problems resulted in a discussion within our clinic about pro’s and con’s of hospitalization of all newly diagnosed CF patients. There is no perfect answer, but we have decided that the best way forward is to admit newly diagnosed CF children for at least three days in the hospital to start treatment “by the book”, even though they have no or minimal symptoms. Of course we do not really force people, but we advise them to come and stay in the hospital for several days. Whilst being with their child in the hospital, parents have the time and opportunity to meet members of the CF team, who help them in their first attempts to get to know CF and CF treatment and start the process of coming to terms with CF. “By the book” means that, coordinated by the specialist nurse, parents meet members of the CF team, they are taught about nebulizers, medication, physiotherapy, diet, and assisted with paperwork.

There is extensive literature on the need for information or on how to communicate a diagnosis to parents of chronically ill children. For example, parents have a great variety of information needs and the way a diagnosis is communicated may influence parents’ perception of the illness and the way they cope with the illness for a long period after diagnosis. Hummelinck and Pollock (2006) describe the importance of sensitively individualizing information provision to parents’ needs so as to address their requirements but not to unnecessarily increase their anxiety or insecurity. During hospitalization, parents are encouraged to talk about their individual needs and the goal is to establish a bond with the family. During hospitalization the focus is on CF and how each family in their own way can integrate this illness and the treatment into their family life.

Marijke Proesmans

Parents are encouraged to take time, and are not expected to learn everything within the week. They don’t have to “accept” CF within a few days, and they don’t have to become perfect CF parents within the month. It is vital that during hospitalization the message to take time is communicated to parents! After hospitalization parents have regular contact with the specialist nurse, not only during out-patient consultations. Parents can contact the CF team at any time and with any query. Because a relationship is established during hospitalization parents feel they can, and they do, contact us and consult us. They know who to ask for with their specific questions, they know faces of each individual CF team member.
As in many issues of CF care (ie home versus hospital IV therapy) it is a challenge to find the balance between ‘ideal treatment’ and ‘normal life’. Start-up of therapy after new CF diagnosis is a milestone and sets the tone for the rest of the journey one has to go with the patient and its family. We have opted for a ‘by the book’ starting-up of treatment, to create an individual bond with the family and to help then find a way to cope with the complexity of CF and CF care.