Neonatal Research

Several recent articles which have some importance for diaphragmatic hernia management

Madenci AL, Sjogren AR, Treadwell MC, Ladino-Torres MF, Drongowski RA, Kreutzman J, et al. Another dimension to survival: predicting outcomes with fetal MRI versus prenatal ultrasound in patients with congenital diaphragmatic hernia. J Pediatr Surg. 2013;48(6):1190-7. Counselling parents about how likely it is that their infant may survive is very tricky. In general. And especially for diaphragmatic hernia patients where the mortality and morbidity are high, but the long term outcome is generally excellent. This study looks at several different indices of lung hypoplasia to determine which, if any, are better than others. It points out, as we already knew, but it bears emphasizing, that the lung head ratio differs during pregnancy, so rather than a single number, the ratio between observed lung-head ratio and the expected ratio is what is predictive of important outcomes. Also fetal MRI with calculation of lung volume (which also obviously should increase during gestation, so the percentage of normal is used as the measure) is a better discriminant of survival, need for ECMO, and ventilator dependency.

Walleyo A, Debus A, Kehl S, Weiss C, Schonberg SO, Schaible T, et al. Periodic MRI lung volume assessment in fetuses with congenital diaphragmatic hernia: prediction of survival, need for ECMO, and development of chronic lung disease. AJR American journal of roentgenology. 2013;201(2):419-26. In this study there were 226 mothers whoer fetuses had repeated fetal MRI. The percentage of expected lung volume was strongly assocaited with survival and need for ECMO. They didn’t compare to ultrasound, though.

Hagelstein C, Weidner M, Kilian AK, Debus A, Walleyo A, Schoenberg S, et al. Repetitive MR measurements of lung volume in fetuses with congenital diaphragmatic hernia: individual development of pulmonary hypoplasia during pregnancy and calculation of weekly lung growth rates. Eur Radiol. 2014;24(2):312-9. This article from the same group, presumably with many of the same babies, calculated the rate of antenatal lung growth. They found :

Individual development of FLV in patients with CDH during pregnancy is extremely variable. Follow-up MR-FLV measurements are advisable before deciding upon pre- and postnatal therapeutic options.

Babies could change from poor to reasonable prognosis lung volumes during pregnancy, and vice versa. Something to keep in mind.

Kays DW, Islam S, Larson SD, Perkins J, Talbert JL. Long-term Maturation of Congenital Diaphragmatic Hernia Treatment Results: Toward Development of a Severity-Specific Treatment Algorithm. Annals of Surgery. 2013;258(4):638-45. An observational study that suggests that one size does not fit all. Suggests different treatment algorithms for different severities of lesion, which makes sense, they suggest that less severe lesions should have delayed surgery, more severe lesoins should have surgery prior to ECMO, rather than after. Interesting but far from conclusive.

Kays DW, Islam S, Richards DS, Larson SD, Perkins JM, Talbert JL. Extracorporeal Life Support in Patients with Congenital Diaphragmatic Hernia: How Long Should We Treat? Journal of the American College of Surgeons. 2014;218(4):808-17. When you start ECMO for a diaphragmatic hernia, you know you may be in for the long run. this review of a large experience (96 ECMO cases of a total of 240), by the sam eauthors as the study above showed :

Of patients still on ECMO at 2 weeks, 56% survived, at 3 weeks 46% survived, and at 4 weeks, 43% of patients still on ECMO survived to discharge. After 5 weeks of ECMO, survival had dropped to 15%, and after 40 days of ECMO support there were no survivors.

Babies on ECMO up to 4 weeks still had good long term pulmonary outcomes. So be patient, but after 4 weeks it looks pretty desperate.

Wright T, Filbrun A, Bryner B, Mychaliska G. Predictors of early lung function in patients with congenital diaphragmatic hernia. J Pediatr Surg. 2014;49(6):882-5. This study looked at lung function in follow up of a group of very high risk CDH patients, during the first 3 years of life. They are actually pretty good. Things that you would expect to be predictors of somewhat poorer function (such as need for ECMO) were indeed, but even with those factors, only moderate abnormalities were found.

Garel C, Moutard ML. Main congenital cerebral anomalies: how prenatal imaging aids counseling. Fetal diagnosis and therapy. 2014;35(4):229-39. An excellent review article that can assist in prenatal counselling if you have a mother whose fetus has a CNS anomaly.

Baylis R, Ewald U, Gradin M, Nyqvist KH, Rubertsson C, Blomqvist YT. First time events between parents and preterm infants are affected by the designs and routines of neonatal intensive care units. Acta Paediatrica. 2014. How your unit is designed and organized affects the first interactions between parents and their preterm babies.

Finn D, Collins A, Murphy B, Dempsey E. Mode of neonatal death in an Irish maternity centre. Eur J Pediatr. 2014:1-5. A study which points out how different certain countries might be, the persistent influence of the Catholic church in Ireland makes pregnancy termination for any indication very rare, and largely impossible for fetal malformations. The babies who die in an Irish neonatal unit are therefore much more commonly babies with extremely high risk congenital anomalies, who receive comfort care. A much higher proportion of neonatal deaths being in this category than in our NICU, or others which have reported their experience. However there are also similarities, most deaths in the NICU occur after a decision to limit intensive care efforts, and only just over 10% occurred despite on-going full intensive attempts at life-sustaining efforts.

Niemarkt HJ, Kuypers E, Jellema R, Ophelders D, Hutten M, Nikiforou M, Kribs A, Kramer BW: Effects of less-invasive surfactant administration on oxygenation, pulmonary surfactant distribution, and lung compliance in spontaneously breathing preterm lambs. Pediatr Res 2014.In this study the clinical effects (on oxygenation) of surfactant therapy delivered via a thin catheter passed through the larynx were smiliar to the effects of standard intubation and surfactant, even though much less surfactant was delivered to the lungs, and the compliance was not as good in the thin catheter group.

Vasudevan C, Johnson K, Miall LS, Thompson D, Puntis J: The Effect of Parenteral Lipid Emulsions on Pulmonary Hemodynamics and Eicosanoid Metabolites in Preterm Infants: A Pilot Study. Nutrition in Clinical Practice 2013, 28(6):753-757. 5 preterm infants received a lipd emulsion based on olive oil, 10 others received standard soy-based emulsion. I don’t know why they weren’t randomized, even in a small pilot study, and no indication is given why some babies received the one oil or the other. Echocardiography was performed and eicosanoid analysis. Estimated pulmonary artery pressures fell faster in the olive oil group, the ratio between urinary thromboxane B2 and 6-keto-PGF1-alpha fell in the olive oil group and remained unchanged in the soy group. Interesting if limited data, confirming that we need to do a good clinical trial of fat emulsions from different sources.

In 2008 a Dr Michael Kahn published an article in the Prestigious New England Journal (which I abbreviate to PNEJM, for any new readers). It was entitled ‘etiquette-based medicine’. He made the introductory comment that although patients deserve compassionate doctors, maybe they would be satisfied with well-behaved ones. That might sound rather critical, but he went on to suggest a checklist of 6 items of common courtesy that he believed were often lacking in medical interactions, but which he thought were important for patients.

I could criticize the article for not being evidence based, but I won’t, because he was right; he also wasn’t in the least dogmatic about the list which he suggested should include these items.

1. Ask permission to enter the room; wait for an answer.

2. Introduce yourself, showing ID badge.

3. Shake hands (wear glove if needed).

4. Sit down. Smile if appropriate.

5. Briefly explain your role on the team.

6. Ask the patient how he or she is feeling about being in the hospital.

That might all sound like simple politeness, but writing from his own experience he knew these steps were often ignored. A more recent research project (free full text) has confirmed his suspicions. In 226 observed patient encounters the investigators found that none of the individual items were performed with a majority of patients, and, in about 1 third of the encounters, not a single one of the items were performed. Of course the physicians had given their consent to be in the study, and new that they were being observed, nevertheless, they still couldn’t manage the basics.

This is all a long preamble to our new publication. Over the years since Violette was hospitalised we have got to know a lot of physicians and nurses and ethicists who have had sick babies in the NICU, either as their parents or grandparents.  In our discussions with them we found that many had had individual or recurrent occurrences of poor communication, rudeness, or thoughtlessness of the medical (and sometimes the nursing) staff. We (particularly Annie Janvier and John Lantos who were the motivators and primary authors for this article, which was a real group effort for POST, Parents on the Other Side of Treatment) thought that the particular circumstances of the NICU are different enough that we should write a new checklist, of behaviours that should be routine and automatic, but are all-too-often lacking.

I  reproduce the list here:

1. Say my baby’s name, regardless of how odd or misspelled it may be to you. Know my baby’s sex.

2. Don’t label my baby. My baby is not a diagnosis. She is not the “T-18,” the “23-weeker,” the “tiny critter,” or the “horrible BPDer in room 8.”

3. Say your name. Tell us who you are, what your profession is, and why you are here. Don’t assume we know and don’t assume we remember.

4. Listen to me. When you enter my baby’s bedside, acknowledge my presence. Sit down if you can. Ask me how I think my baby is doing. Embrace silence. Expect us to be upset. Don’t take it personally.

5. Speak my language. Every parent is different. Some of us want numbers, predictions, and statistics. Others don’t. We generally want to know whether our baby’s course is comparable with other babies with similar conditions or gestational age. Adapt your language to fit our needs.

6. Speak with one voice. We are overwhelmed with health care team members—nurses, students, residents, advanced practice nurses, respiratory therapists, and more. Limit the number of providers attending deliveries, difficult conversations, and code situations. Limit the number of people who examine my baby. Communicate with us in a consistent way.

7. Know my baby. We expect you to know everything about our baby.Take ownership and be responsible. Give us the results that are important to us the same day. Know the facts. Never tell us, “I’m just covering for today.”

8. Acknowledge my role. I contribute to my baby’s care too. I spend hours at the bedside; I pump my breast milk. I may be juggling a job or other children, operating on little sleep, and exercising continuous worry. Please understand and acknowledge this. Your acknowledgment of me in the role of a caring parent strengthens my resolve to be that good parent.

9. Don’t label me. Remember you are meeting me under the worst of circumstances. What is routine to you may be the greatest stress I’ve encountered in my life. Avoid the expression “difficult parents.” Instead, talk about “parents in a difficult situation.” If you feel the need to complain about a family, do so in privacy.

10. Know how important you are to me. I am placing my child’s life in your hands. Do not underestimate how important you are to our family.

We all know there are other issues in our interactions with parents that are important. We have already received one thoughtful and stimulating email that I will ask permission to quote in a future post. Our intention was to create a list of behaviours that should be as universal and automatic as knowing which end of a stethoscope to use. (I was going to say ‘as washing your hands’ but we are pretty pathetic at that!).

I think these kinds of behaviours should be taught as being essential in doctor-patient relationships. When I was being trained in medicine, modelling of good ‘bedside manner’ and general prinicples of doctor-patient relationships were certainly in the curriculum, but putting those general principles into action is clearly beyond some of our colleagues, senior and junior. I think we also need simple specific rules, and, yes, a checklist. If each time we interact with a family we keep these items in mind, families will be more content, we will be more satisfied and I am sure that our patients will be improved.

I am sure that most doctors and other health care professionals want to be good communicators, we should be teaching them how, and sometimes the simple basics will be all that is needed to make a big difference to parents.

I have had a very relaxing break, a long vacation with no blogging, indeed very intermittent internet access, and I did not even keep up with my scanning of the neonatal lierature.

But now back to full speed, or at least half, I have a few posts in the pipeline including one about a new publication in JAMA pediatrics, which will appear in a few minutes!

This is an interesting idea, I believe that initially it was thought that bubble CPAP might deliver some high frequency pressure oscillations to the lungs, as the bubbling in the circuit causes some small amplitude variations in the pressure in the circuit.  I think that has probably been debunked, there is likely no significant CO2 removal as a result of these small oscillations in pressure.

On the other hand if you hitch up your CPAP prongs to a real high frequency ventilator then you might be able to deliver some non-invasive high-frequency ventilation, at least while the airways are open.

This group of researchers (Mukerji A, Singh B, Helou SE, Fusch C, Dunn M, Belik J, et al. Use of Noninvasive High-Frequency Ventilation in the Neonatal Intensive Care Unit: A Retrospective Review. American journal of perinatology. 2014) report a retrospective group of babies who were treated with this kind of intervention. During high frequency non-invasive ventilation there was  a reduction in apneas, in oxygen requirements and in CO2. Of course the question is what would happen to a similar group  of babies treated with standard non-invasive techniques.

The only way to know is by doing an RCT, which I hope is being done.

Kudos to Acta Paediatrica, for an article by a group of parents who were all faced with antenatal counseling in a situation of high risk for extremely preterm delivery. (Staub K, Baardsnes J, Hébert N, Hébert M, Newell S, Pearce R. Our child is not just a gestational age. A first-hand account of what parents want and need to know before premature birth. Acta Paediatrica. 2014)

The families (who I believe were all treated in Canadian perinatal centers : UPDATE: one of the families was actually seen in an Australian center) recount their experiences, the first is very distressing, an infant about to be born at 23 weeks gestation, with an estimated weight which is in the expected range for 23 weeks, who was not even given an option of attempted stabilization. Rather than giving Marcus a 20% chance of survival (a chance which is even higher than that in some parts of the world with a more positive attitude), he was given a 100% chance of dying. I would say that if fully informed parents shared in a decision that this decision was consistent with their values, and that they wished for comfort care only at birth, then it should be considered reasonable to provide that approach, but the family writing in this article was not given that as an option, they were given a ‘fait accompli’ : they were just told : we don’t do that here.

One of the babies, Domenica, should be dead according to the CPS guidelines; she was born some days before she reached 23 weeks gestation, and, according to our Professional Society, stabilization should not be offered. As you will see from the article she is doing really well.

The list of recommendations from the parents at the end of the article should be required reading for neonatologists everywhere. They should not be hugely surprising to ‘us’. but unfortunately as some of these stories show, they will surprise some health care professionals. I will reproduce them below for those who do not have full text access to the article, but if you are a health care provider and have access, please read the vignettes and the discussion that go before.

Recommendations for healthcare providers

1. Tell women that there is nothing they could have done to prevent the premature birth from happening.
2. Our unborn children have a story and are part of our hopes and dreams. Do not use gestational age to categorise our child. Ask us if our unborn baby has a name.
3. Each baby is different. Have a personalised approach. You can tell us what generally happens to children about to be born at the same stage of gestation as our child, but please also describe to us the particularities of our child that will influence his or her outcome.
4. Each family is different. We disagree that every family needs to receive extensive information about everything negative that may happen. Some parents want statistics, others want the general picture. Some parents want to make important decisions on their own, while others want recommendations. Please listen to us individually.
5. We need to trust you. Do not tell us that babies at 22 or 23 weeks do not survive. Do not tell us that most preterm infants are disabled. We rely on you to know your data. Reflect on the difference between medical numbers and medical values. Do not tell parents that their child will have a negative impact on their family. You do not have data to support these claims. Many clinicians decide that giving babies a chance is not worth it for a 10%-20% survival rate. Our discussions should be about values and not so much about percentages.
6. Give us balanced information. Tell us about what our children may, or may not, be able to do. Also tell us about the quality of life of other preterm infants have when they get older
7. Words are important. Before our child’s birth, do not ask us if we want to do “everything” or “nothing”. Have you ever met parents who wanted “nothing” for their child? Do not use the word resuscitation, as premature infants are not usually defibrillated. That is what we think resuscitation means when you use that language. Use the word stabilisation instead.
8. Do not take away the hope we have. There is always hope that we will deliver tomorrow. There is hope that we will be able to spend some time with our child. There is hope that we can survive the death of our child with positive memories. Do not abandon us. Instead, tell us that you will be there whatever happens.
9. Empower parents. When other family members are present, emphasise that strong families are important.
10. Be proud of the work you do. It is so important to us. You make it possible for us to share precious time with our babies. You are the heroes for so many families. You help babies survive and we are thankful for that.

One thing I want to re-iterate is the need to give balanced information. Most position statements and decision aids for extreme preterm delivery are designed to ensure that families know all of the bad things that can happen to extremely preterm infants.

It is analogous to a decision aid for prostate cancer therapy only listing death, infection, incontinence and impotence as potential outcomes of a prostatectomy, without mentioning that it might also save your life! How could anyone make a balanced, informed, decision if they only hear of the downside?

Surely our pride in our work (and I am proud of what I do, thank you Mme Staub et al) should lead us to acknowledge that most extreme preterm survivors so well, that, among survivors, disabilities are variable and often quite manageable, and that quality of life is acceptable to excellent for the large majority of those who survive, both those with and those without disability.