Definition
Hypotonia, a condition of diminished muscle tone, is also referred to as “floppy infant syndrome” and may occur with or without muscle weakness. There are diverse etiologies including abnormalities of the central or peripheral nervous system, neuromuscular junction, or muscle; metabolic, endocrine, or nutritional disorders; connective tissue diseases; and chromosomal abnormalities. Perinatal hypoxia, hypotonic cerebral palsy, and nonspecific mental deficiency may all result in central hypotonia. There is also a condition referred to as benign congenital hypotonia, which is a diagnosis of exclusion, and improves or disappears entirely with age. Preterm infants as well will have age-appropriate hypotonia. Hypotonic babies often have feeding problems that result from abnormal or underdeveloped control of the oropharyngeal structures, contributing to an uncoordinated and/or weak suck.
Background
One of the more common causes of hypotonia, which we shall use as an example, is Down syndrome. Down syndrome is a genetic disorder caused by a trisomy of chromosome 21 resulting in hypotonia in more than 90% of cases. Associated oral abnormalities characteristically include malocclusion and a small mouth with a relatively large, protruding tongue, which when coupled to the hypotonia, result in significant associated feeding difficulties in some, but not all, of these children.
The Academy of Breastfeeding Medicine, American Academy of Pediatrics, World Health Organization, and other international organizations have recommended that all children should be breastfed, unless there is a medical contraindication. It is particularly important that Down syndrome and other hypotonic children be breastfed to minimize the risk of morbidities associated with artificial feedings, many of which they are at increased risk from by virtue of their condition. For example, in addition to the oral abnormalities and malocclusion, children with Down syndrome have developmental delay; are more susceptible to ear, respiratory, and other infections; and have an increased incidence of other congenital anomalies such as heart and gastrointestinal malformations. In looking at the effects of breastfeeding on these problems in a healthy population, approximately 44% of dental malocclusion can be attributed to lack of or short duration of breastfeeding, suggesting that breastfeeding promotes oral motor strength, a potential benefit to those children with Down syndrome and other causes of hypotonia. Breastfeeding helps with normal mouth and tongue coordination. Breastfeeding has also been shown to be protective against the development of ear and respiratory infections. Studies indicate that there is a positive neurocognitive advantage of breastfeeding, which is most pronounced in low birth weight and small for gestational age children who may score as many as eight points higher on intelligence tests than their formula-fed counterparts. As hypotonic babies may have disorders associated with neurocognitive impairment, the benefit of human milk feedings could make an important difference to their long-term outcome. Children with congenital heart disease who breastfeed have better growth, shorter hospital stays, and higher oxygen saturations than children with congenital heart disease who are formula fed. Again, this may suggest potential benefit to hypotonic infants with heart disease, seen in a significant proportion of babies with Down syndrome. Thus although children with Down syndrome and other forms of hypotonia have not been specifically studied, based on the wealth of information available from studies in the general population, they may be expected to benefit from breastfeeding and/or expressed breast milk.
Some mothers of children with Down syndrome express anxiety and fear at the time of their child’s diagnosis. Many express feeling “helpless” or frustrated that they were not able to breastfeed or felt as if they were not given support for breastfeeding. The ability to breastfeed their babies may empower these mothers.
Challenges to breastfeeding the hypotonic child exist, but many can successfully feed at the breast. No evidence exists that Down syndrome or other hypotonic infants feed better with the bottle than at the breast. Further, no evidence suggests that these children need to feed from a bottle before going to breast. Breastfeeding should be actively promoted and supported in these infants.
Sucking behavior, specifically in Down syndrome, has been documented to be less efficient than in normal-term infants with multiple parameters affected, including sucking pressure, frequency, and duration, as well as a deficiency in the smooth peristaltic tongue movement. When followed longitudinally over the first year, sucking pressure increased significantly by 4 months and again by 8 months. Frequency increased by 4 months. Duration did not increase over time, and peristalsis only normalized in the minority of infants who were restudied at 8 months. However, the overall result was improvement in sucking efficiency over the first year. Mothers tended to report that feeding problems were substantially improved by 3 to 4 months of age. Understanding this time frame allows practitioners to effectively support these mothers and babies to improve breastfeeding skills, and reach and maintain a sufficient milk supply that may enable them to ultimately successfully breastfeed, even with the presence of significant difficulties at the beginning.
Procedures
Education
- 1.
All mothers should be educated about the benefits of breastfeeding for themselves and their infants. A significant percentage of hypotonic infants can feed at the breast without difficulty.
- 2.
All babies should be followed closely both before and after discharge from the hospital for assessment of further needs.
Facilitation and Assessment of Feeding at the Breast in the Immediate Postpartum Period
- 1.
The first feeding should be initiated as soon as the baby is stable. There is no reason this cannot occur as early as the delivery room if the baby is physiologically stable.
- 2.
Kangaroo (skin-to-skin) care should be strongly encouraged. If the baby does not feed well, the touching may be stimulating, so that the baby is easier to arouse for feedings. Skin-to-skin care has also been shown to help increase mother’s milk supply, and it can assist with bonding, which may be especially important for these families.
- 3.
Assess the baby’s ability to latch, suck, and transfer milk. This assessment should involve personnel specifically trained in breastfeeding evaluation and management.
- 4.
Skin-to-skin contact will facilitate frequent attempts at the breast. For those attempts, particular attention should be given to providing good head and body support as the baby needs to spend effort sucking, not supporting body position. Use of a sling or pillows to support the infant in a flexed position allows the mother to use her hands to support both her breast and the infant’s jaw simultaneously.
- 5.
The “dancer hand” position (see Figure 14-1 ) may be helpful to the mother to try because it supports both her breast and her baby’s chin and jaw while the baby is nursing. This involves cupping her breast in the palm of her hand (holding her breast from below), with the third, fourth, and fifth fingers curling up towards the side of her breast to support it, while simultaneously allowing the baby’s chin to rest on the web space between her thumb and index finger (see Figure 14-1 ). The thumb and index finger can then give gentle pressure to the masseter muscle, which stabilizes the jaw. Additionally, pulling the jaw slightly forward may allow the infant to better grasp the breast and form a seal. The other hand is free to be used to support the baby’s neck and shoulders.
- 6.
Other strategies to help the infant latch and transfer milk may also be effective. Some mothers facilitate milk transfer with the technique of breastfeeding used in conjunction with hand compression. Instead of placing the thumb and index finger on the baby’s jaw for support (dancer position), the fingers are kept proximal to the areola, and milk is hand expressed as the baby suckles. A thin silicon nipple shield may be useful, if production is generous (> 500 mL/day) and mothers learn how to keep the reservoir filled by synchronizing breastfeeding with hand compression or using a nursing supplementation device simultaneously inside the shield. By making the mother aware of various techniques, aids, and ideas, she is empowered to experiment and discover the best repertoire to fit her and her baby’s individual needs.
- 7.
The mother, and family who is supporting her, should be counseled that more time may be necessary in the early weeks to complete a feeding. They should also know that in many cases the baby’s ability to feed will improve over the first weeks to months.
- 8.
Trained personnel must reassess the baby frequently (a minimum of once every 8 hours) because these babies must be considered high (breastfeeding) risk, similarly to the near-term baby (see ABM Protocol #10 Breastfeeding the Near-Term Baby). Encourage frequent nursing throughout the day as the ability to sustain suck may be impaired. Infants should go to breast as often as possible, aiming for at least 8 to 12 times per 24 hours. Prolonged periods of skin-to-skin contact will facilitate these frequent attempts at the breast. Assessments should include state of hydration and jaundice as possible complications of poor intake.
- 9.
Once transitional milk is present, test weighing with an appropriate digital scale may be an option to judge adequate milk transfer. Infants are weighed immediately prior to the feed on an electronic scale with accuracy at minimum 0.5 g, and then reweighed immediately after the feed with the exact same diaper, clothing, blankets, and so on, worn during the prefeed weight. Intake during the feed is reflected by weight gain, 1 g = 1 mL. Term infants with Down syndrome gain weight more slowly than normal full-term infants, so this must be taken into consideration during the early weeks and months. Growth charts specific for Down syndrome are found at http://www.growthcharts.com/charts/DS/charts.htm (last accessed 21 Jan. 2007).
- 10.
Consider alternative modes of feeding if the baby is unable to nurse at the breast or sustain adequate suckling, including the use of a cup, a spoon, or a wide-based silicone bottle. The use of a nursing supplementation aid alone (without a nipple shield—Section B6) may not be as helpful, as it works best with a baby who has an effective latch, the lack of which is often one of the significant problems of hypotonic infants.
- 11.
If supplementation is necessary, please see Academy of Breastfeeding Medicine Protocol #3 (Hospital Guidelines for the Use of Supplementary Feedings in the Healthy Term Breastfed Infant). If the baby is attempting to suckle, following each breastfeeding encounter with breast milk expression (see later), followed by spoon or cup feeding of the expressed milk to the baby, provides more stimulation to the breasts and more milk to the baby.
Preventative Measures to Protect a Milk Supply
- 1.
If the infant is unable to successfully and fully breastfeed, or if the mother is separated from her infant (e.g., neonatal intensive care unit admission), lactation must be initiated and/or maintained through pumping or hand expression. Anticipating the initial difficulty a hypotonic infant will likely have with sustaining frequent and effective milk removal, insufficient milk production may be prevented by encouraging mothers to express milk shortly after delivery, ideally within 2 hours (certainly within the first 6 hours as is recommended with preterm mothers), and approximately every 3 hours thereafter. Aim to remove milk at least eight times in a 24-hour period, mimicking the stimulation of a vigorous term breastfeeding baby. Even if the baby shows some ability to go to breast, latch, and transfer milk, the mother will likely need to express or pump extra milk in the early weeks in order to build and maintain her milk supply at the higher level. A plentiful milk supply will enhance letdown for these less vigorous babies, and facilitate their feeding effort.
- 2.
Most of the research on initiating and maintaining milk supply by expressing milk has been done on mothers of preterm infants. The strongest determinant of duration and exclusivity of breastfeeding the preterm infant is the volume of milk produced by the pump-dependent mother, while insufficient milk production is the most common reason for cessation of efforts to provide milk for these infants. As the baby begins to improve with milk transfer, developing rhythms, and showing feeding cues, pumping times can be led by these cues (i.e., breast emptying by expression after each attempt at the breast). This pattern should continue until the couplet is reunited and/or the infant is able to sustain successful breastfeeding. It is critical that mothers be instructed on effective pumping, including both the use of a hospital-grade electric pump if available and manual expression.
- 3.
Extrapolating from preterm research for guidance in the hypotonic baby, the production of 500 mL/day is commonly cited as the minimum volume enabling premature babies < 1500 g to transition from tube or bottle feeding to successful, exclusive breastfeeding. Until studies are done in the hypotonic infant population, this is a minimum volume from which to start and can be adjusted based on calculations of intake necessary for growth.
- 4.
Simultaneous pumping of both breasts with a hospital-grade pump has been shown to be more effective than single pumping. Recent research suggests manually assisted pumping improves effective emptying and production in pump-dependent women. In contrast to the usual practice of passively depending on the pump to suction milk from the breast, manual techniques, used in conjunction with pumping, enable mothers to enhance emptying by using their hands for breast compression, massage, and expression.
- 5.
A pumping/feeding diary or log to enable health care providers to track maternal milk supply and intervene when needed can consist simply of a piece of paper with columns for date, time started pumping, time ended pumping, amount of milk expressed, and comments (such as where pumped, unusual stressors, etc.), or one can be ordered or used as a model from various websites, including: http://www.cpqcc.org/Documents/NutritionToolkit/NutritionToolkit.pdf , appendix “O” (last accessed 21 Jan. 2007).
At Discharge and in the Neonatal Period
- 1.
If the baby will remain hospitalized, the mother’s milk supply should be assessed daily including pumping frequency, 24-hour milk total, and any signs of breast discomfort. Carefully monitor the baby’s weight gain and consider supplementation as necessary.
- 2.
Inform mothers that sucking efficiency frequently continues to improve over the first year, such that the breastfeeding experience may “normalize” and may not continue to require interventions initially necessary for their own infant; for example, supplementation, pumping, more frequent nursing, and so forth.
- 3.
Provide information about local support groups for breastfeeding and for specific diagnoses such as Down syndrome families. Support and encouragement are particularly important for these mothers and families with the additional patience and time that is sometimes required to breastfeed these infants.
- 4.
Maternal milk supply is affected by ineffective or infrequent pumping/expressing. Although stress, fatigue, and pain are frequently cited as determinants of slow milk supply, recent evidence refutes this. However, it is not unreasonable to encourage maternal rest and analgesics as needed. Review and optimize breast milk expression frequency, schedule, and type of pump used, if necessary. A pumping diary/log (see earlier) can be useful.
- 5.
If maternal milk supply does not equal or exceed the infant’s needs or begins to slow despite optimal pumping, the use of galactogogues to enhance maternal milk supply may be considered. Please see Academy of Breastfeeding Medicine Protocol #9 (Use of Galactogogues in Initiating or Augmenting Maternal Milk Supply).
- 6.
In the presence of significant cardiac, gastrointestinal, or renal complications, it is sometimes necessary to increase the caloric density of breastmilk with extra fat, carbohydrate, or protein. If the mother’s milk supply is greater than the baby’s needs, a trial of feeding hindmilk (higher fat content, therefore more fat calories), either by expressing some of the foremilk before putting the baby to breast or, if supplements are being used, by pumping off a small volume of milk first (foremilk) and then in a separate container pumping the rest of the milk present (hindmilk) and feeding the baby only the hindmilk.
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