Parental Perspectives on Diagnosis of Cerebral Palsy

Case: Hospital course

Your second child, Anna, was a 25-week preterm infant. She was delivered by C-section due to abruptio placentae. Apgars were 5 and 8. She initially required ventilator support, but she was extubated on day of life 10. She had midline head positioning, judicious use of fluids, and no episodes of acute deterioration. A patent ductus arteriosus was diagnosed on day of life 3; it was successfully closed with indomethacin. She transitioned from total parenteral nutrition to enteral feeds of breast milk without significant problems. Serial cranial ultrasounds showed the following:

• day of life 7 - bilateral grade 2 intraventricular hemorrhage
• days of life 10 and 30 - no change
• time of discharge - resolving grade 2 hemorrhages, no periventricular leukomalacia

Anna was discharged home on day of life 96 on full feeds, vitamin D and iron, and breast milk fortified to 22 calories. You are delighted. Throughout Anna’s course, you have frequently been updated by Anna’s treatment team. Early on, especially with the news that Anna had grade 2 hemorrhages, you asked many questions regarding her developmental outcome. While grade 2 hemorrhages are not as severe as grades 3 and 4, the team could not assure you that Anna’s development would not be affected.

Moreover, Anna was born at 25 weeks, a risk factor for altered developmental outcome. In a meeting when Anna was 34 weeks corrected age, you re-discussed her current status, ongoing needs, and prognosis. You were informed that Anna, in addition to seeing her primary care provider, would also be seen in Neonatal Follow-up Clinic. This clinic would assess her developmental progress, her muscle tone, and any ongoing medical issues. An appointment was given for several months after discharge, when Anna was 3 months corrected age. A referral to early intervention was made at the time of discharge.

First neonatal follow-up visit at 3 months corrected age

Anna is gaining weight nicely. She is receiving Synagis^® as immunoprophylaxis against RSV. She is smiling and follows your face. You have noticed that her legs seem “strong” and that she “loves to stand.” Early intervention has contacted you; her evaluation is scheduled for the end of the week.

At her clinic visit, an examiner takes a 2-minute video of Anna as she rests on her back. The video is a General Movement Assessment (GMA) to assess her movement of her arms and legs, called fidgety movements. Anna has some movements, but not as many as would be expected for her age. She also is examined with the Hammersmith Infant Neurological Examination (HINE). The providers invite you to watch them as they go through various checks of Anna’s tone, reflexes, posture, and movement. The examiners say that Anna’s HINE score is “OK”; she is making progress but they, too, notice stiffness in her legs, that her toes curl under, and that her head control is not quite as good as would be expected. In regard to meeting developmental milestones, she is on target for baby thinking and language. The providers sit down with you and discuss their concerns and the importance of starting early intervention/physical therapy as soon as possible. Anna is making progress, but it is critical to initiate therapies now. Anna will be seen again in 3 months at which time it will be important to reassess her progress.

Second neonatal follow-up visit at 6 months corrected age

You report that physical therapy has begun, once a week. The therapist is concerned that the stiffness in Anna’s legs is not improving; she crosses her legs when held in vertical suspension and appears to tiptoe when held in standing position. You report to the team that Anna is not sitting independently, even briefly, and that her head control is not as good as her older sister’s when she was 6 months old.

The team again performs the HINE and examines Anna. They note the stiffness or increase in tone in the lower extremities. You ask, “Why are her legs stiff and why can’t she control her head better? My family has noticed that Anna is different from her older sister. What is it? Does it have a name?” The medical provider and physical therapist sit down with you. The doctor says, “Have you ever heard of cerebral palsy or CP?” You feel tears coming to your eyes. You ask, “Does she have it?” The doctor responds, “We do not know for certain right now, but we can tell you that based on our evaluation today, she is at high risk for developing CP. When we look back at Anna’s history, first, she was an extremely preterm baby. And you recall that she had the bleeding in the brain on her head ultrasound. Those issues put her at risk. Then, at her 3-month visit, she didn’t have those fidgety movements we talked about, plus we noted the stiffness in her legs. We wanted to see if the therapy would make a difference, but at this point we still note the stiffness. And today, on the HINE, she has a score that puts her in a category of children at high risk for CP. While this obviously is a difficult topic for you and your family, we feel it is important for you to know that she is at risk. We are not giving her the diagnosis today, but we want to see her back in 3 months for a reassessment.”

The conversation continues. You are given more information. The providers ask if they can meet with other members of your family to answer questions. You are tearful but grateful to be aware of the at-risk status, putting a name on what you and your family have noticed is different about Anna from her older sister. You are encouraged to call the team at any time prior to the next visit. Physical therapy is increased to twice a week.

How would you want this scenario to play out?

• Would you want to hear that your child is at high risk for CP and worry about it for months, perhaps unnecessarily?
• Would you definitely prefer NOT to hear that your child is at high risk and just wait until a diagnosis is firm?
• Suppose you had never heard of CP? Would this be too much information to process if it is not a firm diagnosis?

What do we know about parents’ perspectives on the diagnosis of cerebral palsy?

A number of articles have addressed parents’ perspectives on the diagnosis of cerebral palsy. In brief, one investigator found that parental dissatisfaction with how the diagnosis was disclosed related to the diagnosis being made later, rather than earlier.1 In some cases, parents were frustrated and angry that there was a delay in diagnosis when they previously had expressed concerns to their provider. Another response was “everyone knew before I did.” Interestingly, one investigator found that some families actually are relieved to receive the diagnosis of a special need, and that they “are not crazy.”

This same investigator found that parental response to disclosure related not so much to the information that is given as to the manner in which it is imparted. Additionally, parents told at earlier time points were most likely to be satisfied, with earlier diagnosis allowing for earlier adjustment and acceptance of their child.

In a recent investigation, Katherine Guttmann, MD, MBE (former fellow in the Division of Neonatology at Children’s Hospital of Philadelphia), explored parental perspectives on
diagnosis and prognosis of neonatal intensive care unit graduates with cerebral palsy.2 In this select population, she found that the diagnosis was rarely seen as early, with 40% of parents surveyed (162/401) feeling the diagnosis was “a little too late” or “very delayed.” Interestingly, 59% of these parents felt the diagnosis provided benefits. This data suggests clinicians should aim for earlier diagnosis when appropriate.

In this regard, the diagnosis of CP currently occurs in children between 12 months to 24 months of age. The Cerebral Palsy Foundation is committed to moving the age of diagnosis to much earlier, as early as < 5 months. Why? Neuroplasticity of the brain is at its height in a child’s earliest months and years. The opportunity for robust responses to interventions diminishes as time passes. Thus, the earlier the diagnosis, the more likely the child will benefit from interventions.

NOTE: In the past year, utilizing the tools and training received with the endeavor “Toward the Earlier Diagnosis of Cerebral Palsy,” led by Andrea F. Duncan, MD, MS, Attending Neonatologist and Medical Director of our Neonatal Follow-Up Program, we have changed the time of diagnosis of cerebral palsy from 17 months to 8 months.

ON A PERSONAL NOTE: Regarding timing, in a totally informal, random sampling of young adults during pediatric resident teaching sessions, the author of this column posed the question as to whether these young trainees would or would not prefer to know if their own child was at high risk for CP. In 5 separate sessions with 5 to 7 trainees per session, every participant stated they would prefer to know, even if the diagnosis was not firm. Yes, it would be anxiety-provoking, but they would much rather know their child is at high risk than be in the dark.

Finally, in my experience, in addition to parents shedding many tears when discussing/conferring the diagnosis of CP, I have experienced the following reactions:

“CP? No, I never heard of it, except maybe on TV.”

“So, now I know. I knew something was not right. It helps to have a name for it.”

“CP. Yeah, I’ve heard of it. My cousin’s kid goes to that clinic.”

“I don’t understand how he got it.”

Given this set of responses, I hope it is clear that providers must, with great humility and sensitivity, adapt these difficult conversations accordingly.

References

1. Baird G, McConachie H, Scrutton D. Parents’ perceptions of disclosure of the diagnosis of cerebral palsy. Arch Dis Child. 2000;83(6):475-480.
2. Guttman K, Flibotte J, DeMauro SB. Parental perspectives on diagnosis and prognosis of neonatal intensive care unit graduates with cerebral palsy. J Pediatr. 2018;203:156-162.